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Tethered cord syndrome

2019-07-26T12:54:30Z

Roboval:/* Treatment */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/All-Disorders/Tethered-Spinal-Cord-Syndrome-Information-Page|title=Tethered Spinal Cord Syndrome Information Page {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref>

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Spina-Bifida-Fact-Sheet|title=Spina Bifida Fact Sheet {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref> In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.<ref name=":4">{{Cite web|url=https://csfinfo.org/videos/physician-lecture-videos/csf-lectures-archive/csf-disorders-klinge/|title=3rd CSF Disorders Symposium: The Occult Tethered Cord Syndrome {{!}} CSF|website=csfinfo.org|access-date=2019-07-26}}</ref>

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Constipation<ref name=":4" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />
* Foot/ankle deformities<ref name=":4" />
* Scoliosis<ref name=":4" />
* Decreased sensation<ref name=":4" />
* Asymmetry in neurological deficits<ref name=":4" />
* hyperreflexia/clonus<ref name=":4" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

=== Symptoms reported by EDS patients with tethered cord ===
* Vice like feeling around ribs
* Difficulty breathing or taking a deep breath
* Difficulty yawning
* Head feeling heavy
* Pressure in the back of the head
* Band like feeling around the forehead
* Inability to drive
* Vision changes such as tunnel vision
* Difficulty focusing eyes
* Sensation of pulling in the mid back, neck, skull, pelvis, and legs

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==
There is no standard technique in the surgical treatment of TCS. Generally, the lamina is removed, anywhere from L2 to S1, a durotomy is made, and electrical stimulation is used to confirm the absence of any nerve roots which may be associated with the filum. Finally, a microsurgical resection of the filum terminale (usually a 10 mm segment for pathology) is performed. The filum tends to be taut, and to briskly retract upon sectioning. However, findings are variable, and there is no evidence to suggest that the intraoperative findings predict or correlate with the surgical outcome and severity of the TCS. In some cases, it may be necessary to perform a lumbar stabilization across the motion segment in which the filum was sectioned. The resected filum should be sent for histopathological evaluation.<ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref>
[[File:04804D02-7E65-4543-9BAF-DA3B4CF140D6.jpg|thumb|a: Tethered cord syndrome: conus at the normal level (L1), fatty filum suggestive of tethered cord syndrome (Sagittal view lumbar spine, T1 weighted MRI). b: Tethered cord syndrome: the thickened filum terminale at the L2 level, just before division. (Intraoperative photograph of the lumbar spine thecal sac and the durotomy).]]

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

File:04804D02-7E65-4543-9BAF-DA3B4CF140D6.jpg

2019-07-26T12:53:10Z

Roboval:

a: Tethered cord syndrome: conus at the normal level (L1), fatty filum suggestive of tethered cord syndrome (Sagittal view lumbar spine, T1 weighted MRI). b: Tethered cord syndrome: the thickened filum terminale at the L2 level, just before division. (Intraoperative photograph of the lumbar spine thecal sac and the durotomy).

Tethered cord syndrome

2019-07-26T12:51:08Z

Roboval:/* Treatment */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/All-Disorders/Tethered-Spinal-Cord-Syndrome-Information-Page|title=Tethered Spinal Cord Syndrome Information Page {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref>

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Spina-Bifida-Fact-Sheet|title=Spina Bifida Fact Sheet {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref> In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.<ref name=":4">{{Cite web|url=https://csfinfo.org/videos/physician-lecture-videos/csf-lectures-archive/csf-disorders-klinge/|title=3rd CSF Disorders Symposium: The Occult Tethered Cord Syndrome {{!}} CSF|website=csfinfo.org|access-date=2019-07-26}}</ref>

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Constipation<ref name=":4" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />
* Foot/ankle deformities<ref name=":4" />
* Scoliosis<ref name=":4" />
* Decreased sensation<ref name=":4" />
* Asymmetry in neurological deficits<ref name=":4" />
* hyperreflexia/clonus<ref name=":4" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

=== Symptoms reported by EDS patients with tethered cord ===
* Vice like feeling around ribs
* Difficulty breathing or taking a deep breath
* Difficulty yawning
* Head feeling heavy
* Pressure in the back of the head
* Band like feeling around the forehead
* Inability to drive
* Vision changes such as tunnel vision
* Difficulty focusing eyes
* Sensation of pulling in the mid back, neck, skull, pelvis, and legs

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==
There is no standard technique in the surgical treatment of TCS. Generally, the lamina is removed, anywhere from L2 to S1, a durotomy is made, and electrical stimulation is used to confirm the absence of any nerve roots which may be associated with the filum. Finally, a microsurgical resection of the filum terminale (usually a 10 mm segment for pathology) is performed. The filum tends to be taut, and to briskly retract upon sectioning. However, findings are variable, and there is no evidence to suggest that the intraoperative findings predict or correlate with the surgical outcome and severity of the TCS. In some cases, it may be necessary to perform a lumbar stabilization across the motion segment in which the filum was sectioned. The resected filum should be sent for histopathological evaluation.<ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Austin|first2=Claudiu|last3=Benzel|first3=Edward|last4=Bolognese|first4=Paolo|last5=Ellenbogen|first5=Richard|last6=Francomano|first6=Clair A.|last7=Ireton|first7=Candace|last8=Klinge|first8=Petra|last9=Koby|first9=Myles|date=2017|title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref>

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

Tethered cord syndrome

2019-07-26T12:23:38Z

Roboval:/* Symptoms reported by EDS patients with tethered cord : */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/All-Disorders/Tethered-Spinal-Cord-Syndrome-Information-Page|title=Tethered Spinal Cord Syndrome Information Page {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref>

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Spina-Bifida-Fact-Sheet|title=Spina Bifida Fact Sheet {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref> In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.<ref name=":4">{{Cite web|url=https://csfinfo.org/videos/physician-lecture-videos/csf-lectures-archive/csf-disorders-klinge/|title=3rd CSF Disorders Symposium: The Occult Tethered Cord Syndrome {{!}} CSF|website=csfinfo.org|access-date=2019-07-26}}</ref>

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Constipation<ref name=":4" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />
* Foot/ankle deformities<ref name=":4" />
* Scoliosis<ref name=":4" />
* Decreased sensation<ref name=":4" />
* Asymmetry in neurological deficits<ref name=":4" />
* hyperreflexia/clonus<ref name=":4" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

=== Symptoms reported by EDS patients with tethered cord ===
* Vice like feeling around ribs
* Difficulty breathing or taking a deep breath
* Difficulty yawning
* Head feeling heavy
* Pressure in the back of the head
* Band like feeling around the forehead
* Inability to drive
* Vision changes such as tunnel vision
* Difficulty focusing eyes
* Sensation of pulling in the mid back, neck, skull, pelvis, and legs

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

Tethered cord syndrome

2019-07-26T12:20:05Z

Roboval:Added symptoms referred to by Dr. Klinge as indicators of tethered cord

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/All-Disorders/Tethered-Spinal-Cord-Syndrome-Information-Page|title=Tethered Spinal Cord Syndrome Information Page {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref>

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body.<ref>{{Cite web|url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Spina-Bifida-Fact-Sheet|title=Spina Bifida Fact Sheet {{!}} National Institute of Neurological Disorders and Stroke|website=www.ninds.nih.gov|access-date=2019-07-26}}</ref> In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.<ref name=":4">{{Cite web|url=https://csfinfo.org/videos/physician-lecture-videos/csf-lectures-archive/csf-disorders-klinge/|title=3rd CSF Disorders Symposium: The Occult Tethered Cord Syndrome {{!}} CSF|website=csfinfo.org|access-date=2019-07-26}}</ref>

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Constipation<ref name=":4" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />
* Foot/ankle deformities<ref name=":4" />
* Scoliosis<ref name=":4" />
* Decreased sensation<ref name=":4" />
* Asymmetry in neurological deficits<ref name=":4" />
* hyperreflexia/clonus<ref name=":4" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

=== Symptoms reported by EDS patients with tethered cord : ===
* Vice like feeling around ribs
* Difficulty breathing or taking a deep breath
* Difficulty yawning
* Head feeling heavy
* Pressure in the back of the head
* Band like feeling around the forehead
* Inability to drive
* Vision changes such as tunnel vision
* Difficulty focusing eyes
* Sensation of pulling in the mid back, neck, skull, pelvis, and legs

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

Tethered cord syndrome

2019-07-26T11:46:10Z

Roboval:/* Symptoms */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body. In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

=== Symptoms reported by EDS patients with tethered cord : ===
* Vice like feeling around ribs
* Difficulty breathing or taking a deep breath
* Difficulty yawning
* Head feeling heavy
* Pressure in the back of the head
* Band like feeling around the forehead
* Inability to drive
* Vision changes such as tunnel vision
* Difficulty focusing eyes
* Sensation of pulling in the mid back, neck, skull, pelvis, and legs

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

Tethered cord syndrome

2019-07-26T11:37:19Z

Roboval:/* Onset */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body. In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.

== Onset ==
Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]

Tethered cord syndrome

2019-07-26T11:35:57Z

Roboval:/* Symptoms */

[[File:Tethered cord drawing.png|thumb]]
'''Tethered cord syndrome''' is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can be congenital or acquired and appear in childhood or adulthood. It is considered progressive.

It causes spinal cord hypoperfusion, electrophysical changes and metabolic changes, including impaired glucose metabolism.<ref name=":2">{{Cite journal|last=Colohan|first=Austin R. T.|last2=Zouros|first2=Alexander|last3=Siddiqi|first3=Javed|last4=Yamada|first4=Shoko M.|last5=Yamada|first5=Brian S.|last6=Pezeshkpour|first6=Gholam|last7=Won|first7=Daniel J.|last8=Yamada|first8=Shokei|date=2007-08-01|title=Pathophysiology of tethered cord syndrome and similar complex disorders|url=https://thejns.org/view/journals/neurosurg-focus/23/2/foc-07_08_e6.xml|journal=Neurosurgical Focus|language=en-US|volume=23|issue=2|pages=1–10|doi=10.3171/FOC-07/08/E6|issn=1092-0684}}</ref><ref>{{Cite journal|last=Colohan|first=Austin R. T.|last2=Yamada|first2=Shokei|date=2009-01-01|title=Tethered Cord Syndrome|url=https://thejns.org/view/journals/j-neurosurg-spine/10/1/article-p79.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=10|issue=1|pages=79–80|doi=10.3171/2008.10.SPI15714L}}</ref><ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref>

There are a range of conditions associated with tethered cord. The most dramatic is spina bifida, where the spinal cord does not complete it's development and is visible on the outside of the body. In a frank tethered cord, the cord is not visible on the outside of the body, but has not fully developed and is anchored to the structures inside the spinal column. Think of a tongue tie, where instead of having a free tongue, there is a thick web that attaches the tongue to the base of the mouth. This is similar to what a tethered cord is like. In some cases, the base of the spinal cord, the conus, is much lower than it should be, and this is how neurosurgeons typically diagnose tethered cord. However, the cord can still be tethered without the conus being below the level of the first lumbar vertebrae - this is referred to as an occult or hidden tethered cord, or a tight filum.

== Onset ==
Some researchers, nothing that less severe spinal cord traction may remain asymptomatic in childhood<ref name=":3">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>, hypothesize that the age of symptom onset is related to the amount of cord stretch. Factors in adult onset tethered cord syndrome include: transient stretching of the spine, mechanical constriction/narrowing of the spinal canal, and spinal trauma, all in the presence of an already tightly tethered conus medullaris, such as might occur during natural childbirth or in an automobile accident.<ref name=":3" />

== Symptoms ==
*[[File:Tethered cord symptoms (Part II).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part I)]][[File:Tethered cord symptoms (Part I).jpg|thumb|Chiari Neurological Center’s tethered cord questionnaire (Part II)]]
* Leg pain<ref name=":0">{{Cite journal|last=Wilberger|first=James E.|last2=Pang|first2=Dachling|date=1982-07-01|title=Tethered cord syndrome in adults|url=https://thejns.org/view/journals/j-neurosurg/57/1/article-p32.xml|journal=Journal of Neurosurgery|language=en-US|volume=57|issue=1|pages=32–47|doi=10.3171/jns.1982.57.1.0032}}</ref>
* Leg weakness
* Leg numbness<ref name=":1">{{Cite web|url=https://www.seattlechildrens.org/conditions/brain-nervous-system-mental-conditions/tethered-spinal-cord/|title=Tethered Spinal Cord|website=Seattle Children’s Hospital|language=en|access-date=2019-06-02}}</ref>
* Lower back pain<ref>{{Cite journal|last=Gupta|first=S. K|last2=Khosla|first2=V. K|last3=Sharma|first3=B. S|last4=Mathuriya|first4=S. N|last5=Pathak|first5=A|last6=Tewari|first6=M. K|date=1999-10-01|title=Tethered cord syndrome in adults|url=http://www.sciencedirect.com/science/article/pii/S0090301999001214|journal=Surgical Neurology|volume=52|issue=4|pages=362–370|doi=10.1016/S0090-3019(99)00121-4|issn=0090-3019}}</ref>
* Numbness under soles of feet
* Rectal pain<ref name=":0" />
* Urinary urgency or incontinence<ref name=":0" />
* Urinary retention<ref>{{Cite web|url=https://www.hindawi.com/journals/crior/2015/926185/|title=An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain|last=Saita|first=Kazuo|last2=Yamaguchi|first2=Takehiko|date=2015|website=Case Reports in Orthopedics|language=en|access-date=2019-06-02|last3=Chikuda|first3=Hirotaka|last4=Akiyama|first4=Toru|last5=Kanda|first5=Shotaro}}</ref>
* Bowel dysfunction<ref name=":0" />
* Pulling sensation (on brain or upper spine, from below)
* Scoliosis<ref name=":1" />

=== Signs and symptoms in Children<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-06-02}}</ref> ===
* Lesion on the lower back
* Fatty tumor or deep dimple on the lower back
* Skin discoloration on the lower back
* Hairy patch on the lower back
* Back pain, worsened by activity and relieved with rest
* Leg pain, especially in the back of legs
* Leg numbness or tingling
* Changes in leg strength
* Deterioration in gait
* Progressive or repeated muscle contractions
* Leg deformities
* Spine tenderness
* Scoliosis (curvature of the spine)
* Bowel and bladder problems
* Difficulty running<ref>{{Cite journal|last=Kothbauer|first=Karl F.|last2=Lew|first2=Sean M.|date=2007|title=Tethered Cord Syndrome: An Updated Review|url=https://www.karger.com/Article/FullText/98836|journal=Pediatric Neurosurgery|language=english|volume=43|issue=3|pages=236–248|doi=10.1159/000098836|issn=1016-2291|pmid=17409793}}</ref>

== Diagnosis ==

=== Imaging ===
If a tethered cord is suspected, one or more tests may be necessary to confirm the diagnosis. The most common is a lumbar MRI, but a [[myleogram]], CT scan, or ultrasound may also aid in diagnosis<ref>{{Cite web|url=https://www.aans.org/|title=Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments|website=www.aans.org|language=en|access-date=2019-07-26}}</ref>

=== '''Occult tethered cord syndrome''' ===
{{Video|id=https://youtu.be/MF0i0CGKkMI|service=youtube|dimensions=550|description=|alignment=right|urlargs=}}Occult tethered cord syndrome describes patients with the signs and symptoms of tethered cord syndrome but who have normal neuroimaging. These cases are often diagnosed via a urodynamics study, which can reveal neurogenic bladder.<ref>{{Cite journal|last=Metcalfe|first=P. D.|last2=Luerssen|first2=T. G.|last3=King|first3=S. J.|last4=Kaefer|first4=M.|last5=Meldrum|first5=K. K.|last6=Cain|first6=M. P.|last7=Rink|first7=R. C.|last8=Casale|first8=A. J.|date=2006-10|title=Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale|url=https://www.ncbi.nlm.nih.gov/pubmed/16945660|journal=The Journal of Urology|volume=176|issue=4 Pt 2|pages=1826–1829; discussion 1830|doi=10.1016/j.juro.2006.04.090|issn=0022-5347|pmid=16945660}}</ref>

== Treatment ==

== Pathophysiology ==

=== Metabolism ===
Tethered cord is associated with impaired glucose metabolism in spinal cord tissue,<ref name=":2" /> changes in the reduction/oxidation ratio of [[cytochrome oxidase]].<ref>{{Cite journal|last=Yamada|first=Shoko M.|last2=Won|first2=Daniel J.|last3=Yamada|first3=Shokei|date=2004-02-01|title=Pathophysiology of tethered cord syndrome: correlation with symptomatology|url=https://thejns.org/view/journals/neurosurg-focus/16/2/foc.2004.16.2.7.xml|journal=Neurosurgical Focus|language=en-US|volume=16|issue=2|pages=1–5|doi=10.3171/foc.2004.16.2.7|issn=1092-0684}}</ref> and reduced ATP production.<ref>{{Cite journal|last=Sullivan|first=Stephen|last2=Park|first2=Paul|last3=Stetler|first3=William R.|date=2010-07-01|title=Pathophysiology of adult tethered cord syndrome: review of the literature|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1080.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E2|doi=10.3171/2010.3.FOCUS1080|issn=1092-0684}}</ref> Energy loss due to neural membrane stretching contributes to leakage of [[sodium]], [[potassium]] and [[calcium]].<ref>{{Cite journal|last=Yamada|first=Shokei|last2=Iacono|first2=Robert P.|last3=Andrade|first3=Terry|last4=Mandybur|first4=George|last5=Yamada|first5=Brian S.|date=1995-04-01|title=Pathophysiology of Tethered Cord Syndrome|url=http://www.sciencedirect.com/science/article/pii/S1042368018304650|journal=Neurosurgery Clinics of North America|series=Spinal Dysraphism|volume=6|issue=2|pages=311–323|doi=10.1016/S1042-3680(18)30465-0|issn=1042-3680}}</ref>

A study of energy cost of walking in adolescents with tethered cord, as measured by oxygen uptake, found that “energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.”<ref>{{Cite journal|last=Bruinings|first=A. L.|last2=Berg‐Emons|first2=H. J. G. Van Den|last3=Buffart|first3=L. M.|last4=Heijden‐Maessen|first4=H. C. M. Van Der|last5=Roebroeck|first5=M. E.|last6=Stam|first6=H. J.|date=2007|title=Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2007.00672.x|journal=Developmental Medicine & Child Neurology|language=en|volume=49|issue=9|pages=672–677|doi=10.1111/j.1469-8749.2007.00672.x|issn=1469-8749}}</ref>

=== Blood flow ===
People with tethered cord syndrome have reduced blood flow to the spinal cord.<ref name=":2" />

“Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.”<ref>{{Cite journal|last=Rekate|first=Harold L.|last2=Theodore|first2=Nicholas|last3=Kalani|first3=M. Yashar|last4=Filippidis|first4=Aristotelis S.|date=2010-07-01|title=Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome|url=https://thejns.org/view/journals/neurosurg-focus/29/1/2010.3.focus1085.xml|journal=Neurosurgical Focus|language=en-US|volume=29|issue=1|pages=E9|doi=10.3171/2010.3.FOCUS1085|issn=1092-0684}}</ref>

In a study of five children undergoing surgery for tethered cord syndrome group, spinal cord blood flow prior to untethering was a mean of 12.6 ml/min per 100 g of tissue. It increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue.<ref>{{Cite journal|last=Danto|first=Joseph|last2=Greenberg|first2=Burt M.|last3=Rosenthal|first3=Alan D.|last4=Schneider|first4=Steven J.|date=1993-02-01|title=A Preliminary Report on the Use of Laser-Doppler Flowmetry during Tethered Spinal Cord Release|url=https://academic.oup.com/neurosurgery/article/32/2/214/2804055|journal=Neurosurgery|language=en|volume=32|issue=2|pages=214–218|doi=10.1227/00006123-199302000-00010|issn=0148-396X}}</ref>

== Co-morbidities ==
* [[Ehlers-Danlos syndrome]]
* [[Craniocervical instability]]

== See also ==
* [[Craniocervical instability]]

== Learn more ==

* “[https://thejns.org/spine/view/journals/j-neurosurg-spine/10/1/article-p79.xml Tethered Cord Syndrome],” ''Journal of Neurosurgery'', ''2009.''

== References ==
<references />
[[Category:Neurology]]
[[Category:Diagnoses]]
[[Category:Neurological disorders]]