On the publication of “Neurological Features of Bechet Syndrome: A Case Series.” This article was published in Neurology on April 18, 2016.
OBJECTIVE: To better understand the variant clinical neurological findings of Bechet Syndrome.
BACKGROUND: Behçet’s disease is a systemic disease with the triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis. It can involve the neurological systems and often occurs as a chronic meningoencephalitis. However, its clinical features are varied and may mimic other neurological diseases. Comparison of the different clinical features may help the differential diagnosis and treatment. DESIGN/METHODS: The authors reviewed 3 cases of Bechet syndrome with different clinical features. The average age was 41.3 years and 2 of the 3 patients were female. The diagnosis of Behcet was made more than one year in 2 cases and acutely in 1 case. Patients’ medical records including the CSF analysis, image studies and electrophysiological data were reviewed. RESULTS: In case A with meningoencephalitis, patient developed acute cognitive impairment after a trip in Turkey. Neurological exam revealed upper motor neuron (UMN) signs. MRI brain demonstrated patchy leptomeningeal hyperintensities with laminar necrosis. CSF analysis showed lymph dominant pleocytosis. Patient’s mental status improved after large dose of steroid treatment. In case B with peripheral neuropathy, the patient was diagnosed with Behcet disease 2 years ago. She was admitted for acute onset left upper extremity weakness and numbness. MRI brain ruled out stroke. Her symptoms resolved quickly with oral steroid therapy. In case C with optic neuropathy, the patient was diagnosed with Behcet disease 7 years ago after an episode of blurry vision, oral and scrotum ulcers. He was admitted for recurrent visual disturbance after he stopped steroid treatment. Neurological exam was significant for UMN signs. MRI brain demonstrated T2 hyperintensities at the brainstem.
CONCLUSIONS: This case series study helps to understand better the variant neurological features of Behcet’s disease, which will facilitate the diagnosis and appropriate treatment.