Abstract Details

Title Subacute Segmental Lower Motor Neuron Syndrome Associated with Spinal Cord Gray Matter T2-Hyperintensities in 2 Patients with Well-Controlled HIV Infection

Topic Infections/AIDS/Prion Disease

Presentation(s) P03 - (-)

Poster/Presentation
Number 250

Objective

Background BACKGROUND: Motor neuron disease in HIV can mimic amyotrophic lateral sclerosis. Brachial amyotrophic diplegia (BAD) has also been described in HIV patients. Only 3 cases of BAD have been associated with non-enhancing, bilateral, T2-hyperintense gray matter lesions on MRI, one in a patient with untreated HIV.

Design/Methods DESIGN/METHODS: Case 1: A 62 year-old virally suppressed HIV-positive man presented with 5 days of a limp and painful left hand and forearm. His MRI showed T2-hyperintensities in the central gray matter of the cervical cord. Neurophysiologic studies demonstrated acute denervation in the left C8-T1 myotomes. After 1 week of prednisone, his strength improved slightly. Two years later his contralateral limb was affected. MRI was unchanged. Neurophysiologic studies demonstrated acute denervation in the right C8 myotome. Case 2: A 53 year-old virally suppressed HIV-positive man with hepatitis C presented with 2 months of weakness and numbness in the left 4th and 5th digits and pain to the elbow. MRI identified a T2-hyperintensity in the gray matter of the spinal cord at C5-6. Neurophysiologic studies demonstrated acute denervation in the left C7-T1 myotomes.

Results RESULTS: Both patients were treated with monthly intravenous immunoglobulin (IVIg). The first patient did not worsen after starting IVIg. The second patient's pain and sensory changes disappeared within 2 weeks. His weakness resolved after 6 months.

Conclusions CONCLUSIONS: We describe a subacute, painful myelopathy different from HIV vacuolar myelopathy. The distal upper extremity weakness and pain is atypical for BAD. MRI shows gray matter pathology consistent with the patients' lower motor neuron syndrome. One patient may have improved with IVIg, suggesting a para-infectious mechanism of motor neuron damage.

Authors/Disclosures
Michael R. Wilson, MD, FAAN (University of California San Francisco) PRESENTER	Dr. Wilson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Wilson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ouro Medicines. Dr. Wilson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Vertex Pharmaceuticals. Dr. Wilson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Indapta Therapeutics. Dr. Wilson has received personal compensation in the range of $50,000-$99,999 for serving as an officer or member of the Board of Directors for Delve Bio. Dr. Wilson has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cambridge Medical Experts. Dr. Wilson has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Dunham Hallmark. Dr. Wilson has stock in Delve Bio. The institution of Dr. Wilson has received research support from Genentech / Roche. The institution of Dr. Wilson has received research support from NIH. The institution of Dr. Wilson has received research support from Novartis. The institution of Dr. Wilson has received research support from National Multiple Sclerosis Society. The institution of Dr. Wilson has received research support from Fanconi Anemia Research Foundation. The institution of Dr. Wilson has received research support from Department of Defense. The institution of Dr. Wilson has received research support from Chan Zuckerberg Initiative. The institution of Dr. Wilson has received research support from Kyverna Therapeutics. Dr. Wilson has received intellectual property interests from a discovery or technology relating to health care. Dr. Wilson has received intellectual property interests from a discovery or technology relating to health care. Dr. Wilson has received personal compensation in the range of $10,000-$49,999 for serving as a Expert Witness with US Dept of Justice.
	No disclosure on file
	No disclosure on file
Nagagopal Venna, MBBS, FAAN (Massachusetts General Hospital)	Dr. Venna has nothing to disclose.

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