MENINGO-ENCEPHALITIS WITH NEUROPATHY; BICKERSTAFF/FISHER SYNDROME AN UNDER RECOGNISED ENTITY. (29th November 2012)

• Record Type: Journal Article
• Authors: Damodaran, D; Varma, A
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 83(2012)Supplement 2
• Page Start: A22
• Page End: A23
• Publication Date: 2012-11-29
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2012-304200a.85
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : A 69-year-old patient who had mild URTI a week ago presented with back pain, nausea and progressive weakness of the limbs. Examination revealed mild confusion and subtle eye closure weakness. Motor system examination revealed MRC Grade 4 weakness proximally and distally in his limbs. Mild rigidity in the upper limbs with subtle finger nose incoordination and arreflexic limbs with mute plantar. CT and MRI scan of the brain was unremarkable apart from mild atrophic changes CSF protein was 2.4 g with normal CSF constituents Virology and autoimmune screen were either negative or normal Over the next couple of weeks symptoms progressed. NCS/EMG showed reduced sensory responses of both limbs and the CMAPs of both lower limbs were delayed, raising the possibility of demyelination. He was managed with immunoglobulin followed by pulsed methylprednisolone and then a tapering dose of steroids over the next 2–3 months. He improved gradually and made a full recovery. Discussion: This case illustrates the importance of characterising meningoencephalitis with neuropathy as a distinct syndrome like Bickerstaff/fisher syndrome when infection and systemic autoimmune disorders have been ruled out, Two thirds of these patients have the anti-Gq1b +ve. Our patient was not +ve anti Gq1b but clinical syndrome, electrophysiology and response to treatment suggest this diagnosis. These patients should be treated with immunoglobulin on an urgent basis. The Peripheral Nervous system examination is frequently cursory in patients with encephalitis and neuropathy confluent of the syndrome may be missed.