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Bibrachial Amyotrophy Due to Spontaneous Spinal Cerebrospinal Fluid Leak

To identify the key insights or developments described in this article
1 Credit CME

A 61-year-old woman presented to the emergency department with neck pain after a fall in the nursing home, where she had lost her balance while trying to move clothing in her closet with her feet. She had a 7-year history of progressive neck, shoulder, and upper extremity weakness and atrophy, for which several diagnoses had been entertained, including amyotrophic lateral sclerosis, person-in-the-barrel syndrome, and upper motor neuron disease of unknown etiology. Electromyography had shown chronic neurogenic changes in the cervical myotomes. There was no history of headache or prior trauma. At presentation, she had severe atrophy of her neck, shoulder, and upper extremity musculature. Occasional fasciculations were noted. Motor examination showed flaccid paralysis (Medical Research Council score of 0 of 5) of the upper extremities except for right thumb abduction (score of 4 of 5) (Video). Lower extremity strength was normal. Deep tendon reflexes were absent in the upper extremities and normal in the lower extremities. Magnetic resonance imaging (MRI) showed an extensive ventral cervicothoracic extradural cerebrospinal fluid (CSF) collection with posterior displacement of the spinal cord (Figure 1A). Brain MRI findings were normal. Digital subtraction myelography localized the ventral CSF leak to the thoracic 11-12 level (Figure 1B). At surgery, through a posterior intradural approach, a ventral dural tear (Figure 2) was found, and this was repaired. The patient recovered well from surgery, and postoperative MRI showed complete resolution of her CSF leak. At last follow-up 40 months postoperatively, stable motor examination findings were noted.

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Article Information

Corresponding Author: Wouter I. Schievink, MD, Department of Neurosurgery, Cedars-Sinai Medical Center, 127 S San Vicente Blvd, 6th Floor, Los Angeles, CA 90048 (schievinkw@cshs.org).

Published Online: February 20, 2023. doi:10.1001/jamaneurol.2022.5388

Conflict of Interest Disclosures: None reported.

References
1.
Schievink  WI .  Spontaneous intracranial hypotension.   N Engl J Med. 2021;385(23):2173-2178. doi:10.1056/NEJMra2101561PubMedGoogle ScholarCrossref
2.
Mihaylova  TG , Biondo  A , Zak  I , Lewis  RA .  Cervical anterior horn cell loss from subdural hygroma—a late sequelae of spontaneous CSF leak.   Neurology. 2009;72(suppl 3):A310.Google Scholar
3.
Schievink  WI , Maya  M , Moser  F , Nuño  M .  Long-term risks of persistent ventral spinal CSF leaks in SIH: superficial siderosis and bibrachial amyotrophy.   Neurology. 2021;97(19):e1964-e1970. doi:10.1212/WNL.0000000000012786PubMedGoogle ScholarCrossref
4.
Morishima  R , Takai  K , Ando  T , Nakata  Y , Shimizu  T , Taniguchi  M .  Brachial multisegmental amyotrophy caused by cervical anterior horn cell disorder associated with a spinal CSF leak: a report of five cases.   J Neurol. 2019;266(11):2679-2684. doi:10.1007/s00415-019-09469-9PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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