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Abstract Details

Diverse Neurological Manifestations in Neuro-Behcet’s Disease: A Case Series
Autoimmune Neurology
P11 - Poster Session 11 (11:45 AM-12:45 PM)
2-001
To describe the various neurological presentations in Behcet’s disease.
Behcet’s disease is a chronic, relapsing multisystem vasculitis. Nervous system
involvement is observed in less than 10% of cases and it can occur as parenchymal or non-parenchymal disease.
Retrospective chart review.

Patient 1: A 23-year-old female with a known diagnosis of Behcet’s disease (not on immunosuppressive therapy) presented with sudden onset unsteadiness, and was found to have a small right middle cerebellar peduncle infarct. Notably, she had no other vascular risk factors. Her CSF showed lymphocytic pleocytosis (TNC 29; lymphocytes 98%, protein 29, glucose 58). Cerebral angiogram demonstrated no vascular irregularities.

Patient 2: A 27-year-old male with chronic uveitis and oral and genital ulcers presented with subacute personality changes, balance issues and abnormal movements. MRI brain revealed hyperintense lesions involving internal capsules, thalami, midbrain and left cerebellar peduncle associated with contrast enhancement in some of these regions. CSF analysis revealed elevated white blood cell count (TNC 25; lymphocytes 77%, monocytes 18%, protein 48, glucose 57).

Patient 3: A 20-year-old female with a known diagnosis of Behcet’s disease (on daily prednisone 10 mg and azathioprine 100 mg) presented with progressively worsening headache and was found to have filling defects in the left transverse sinus and superior sagittal sinus concerning for venous sinus thrombosis.

All patients were treated with high dose IV steroids (from 3-5 days) and were subsequently started on immunosuppressive therapy (patient 3 was switched from azathioprine to adalimumab).

Neuro-Behcet’s disease represents a rare manifestation of Behcet’s syndrome with considerable heterogeneity in clinical presentations including brainstem syndrome, meningoencephalitis, myelopathy, stroke like syndromes and others. In this case series, three patients demonstrate the spectrum of neurological involvement: acute ischemic stroke in the right cerebellar peduncle, multifocal parenchymal brain lesions with predominant brainstem syndrome and cerebral venous sinus thrombosis.

Authors/Disclosures
William R. Meador, MD
PRESENTER
Dr. Meador has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for ForensisGroup. The institution of Dr. Meador has received research support from PCORI. The institution of Dr. Meador has received research support from ROCHE/GENENTECH.
Rana Abushamma, MBBS Dr. Abushamma has nothing to disclose.
Shruti P. Agnihotri, MD An immediate family member of Dr. Agnihotri has stock in Pfizer. The institution of Dr. Agnihotri has received research support from Roche/ Genentech.