Pearls & Oy-sters: Bibrachial Amyotrophy From a Dural Tear
Yuyao Sun1, Shivani Pahwa1, Rani Priyanka Vasireddy1
1From the Departments of Neurology (Y.S., R.P.V., F.D.R.), Neurosurgery (S.P., F.D.R.), and Radiology (S.P., A.B., F.D.R.), University of Kentucky, Lexington.
Neurology
|March 14, 2024
Summary
Bibrachial amyotrophy, often mistaken for ALS, can be caused by dural tears leading to spinal cord compression. Early diagnosis requires vigilance for this rare condition.
Area of Science:
- Neurology
- Neurosurgery
- Radiology
Background:
- Bibrachial amyotrophy presents as upper extremity weakness, with diverse underlying causes.
- Amyotrophic Lateral Sclerosis (ALS) is a common differential diagnosis for this phenotype.
Observation:
- A patient with bibrachial amyotrophy initially diagnosed with ALS showed atypical clinical progression and electrodiagnostic findings.
- Further investigation revealed dural tears with cerebrospinal fluid (CSF) leak, causing extradural fluid collection, ventral myelopathy, and intracranial hypotension.
Findings:
- Dural tears can mimic ALS, presenting with bibrachial amyotrophy and characteristic "owl's eye" T2 hyperintensities in spinal cord anterior horn cells on MRI.
- This case highlights the diagnostic challenge in differentiating dural tear-induced myelopathy from ALS.
Implications:
- Clinical and radiological vigilance is crucial for identifying rare dural tears presenting as bibrachial amyotrophy.
- Accurate diagnosis of dural tears is essential for appropriate management and to avoid misdiagnosis of neurodegenerative diseases like ALS.
