TITLE:
A case of Bickerstaff brainstem encephalitis successfully treated with intravenous immunoglobulin and methylprednisolone after unsuccessful immunoadsorption plasmapheresis
AUTHORS:
Takeshi Imai, Takahiro Shimizu, Yuta Hagiwara, Toshikazu Hirayama, Yasuhiro Hasegawa
KEYWORDS:
Bickerstaff Brainstem Encephalitis;Intravenous Immunoglobulin; Methylprednisolone;Immunoadsorption Plasmapheresis
JOURNAL NAME:
Open Journal of Clinical Diagnostics,
Vol.3 No.1,
March
20,
2013
ABSTRACT: Bickerstaff brainstem encephalitis (BBE) is a rare post-infectious neurological syndrome for which an effective treatment strategy has not been established. Here, we report a case of a 71-year-old male who suffered from an upper respiratory tract infection, and 7 days later, developed numbness of the bilateral upper and lower limbs, unsteady gait and dysarthria. Brain magnetic resonance imaging was normal, nerve conduction study and cerebral spinal fluid analysis were nonspecific. Based on the clinical features, we tentatively diagnosed Guillain-Barré syndrome and started immunoadsorption plasmapheresis. However, consciousness progressively declined to coma level within 10 days. Electroencephalogram showed diffuse slowing, and auditory evoked brainstem response (ABR) demonstrated absence of waves II, III and V. Serum anti-GQ1b IgG autoantibody and anti-GM1b IgG autoantibody were negative. Subsequently, we diagnosed BBE, and clinical symptoms resolved after treatment with intravenous immunoglobulin and methyllprednisolone. On day 62, neurological symptoms were remarkably alleviated with an improvement in ABR. Our observations suggest that immunoadsorption plasmapheresis should be used only when anti-ganglioside antibodies are detected. Combination therapy with intravenous immunoglobulin and methylprednisolone or plasma exchangeis recommended as initial therapy.