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 exchange is recommended as initial therapy.
Highlights
Our observations suggest that immunoadsorption plasmapheresis should be used only when antiganglioside antibodies are detected
Bickerstaff brainstem encephalitis (BBE), first described in 1951, is a rare post-infectious neurological syndrome characterized by ophthalmoplegia, ataxia and symptoms suggesting central nervous system involvement, such as impaired consciousness, extensor plantar responses and hemisensory loss [1]
Various treatments have been reported for BBE, such as 1) a combination of steroids and plasma exchange, 2) various types of plasmapheresis without steroids, 3) steroids only, 4) a combination of steroids and intravenous immunoglobulin (IVIg) and plasma exchange, and 5) nonspecific immunotherapy [3]
Summary
Bickerstaff brainstem encephalitis (BBE), first described in 1951, is a rare post-infectious neurological syndrome characterized by ophthalmoplegia, ataxia and symptoms suggesting central nervous system involvement, such as impaired consciousness, extensor plantar responses and hemisensory loss [1]. BBE shares common features with Miller Fisher syndrome (MFS), both clinically and immunologically, and accumulating evidence suggests that BBE and MFS are part of a continuous spectrum of variable central and peripheral nervous system involvement [2]. Various treatments have been reported for BBE, such as 1) a combination of steroids and plasma exchange, 2) various types of plasmapheresis without steroids, 3) steroids only, 4) a combination of steroids and intravenous immunoglobulin (IVIg) and plasma exchange, and 5) nonspecific immunotherapy [3]. We describe a patient with anti-GQ1b antibodynegative BBE who was successfully treated with IVIg and methylprednisolone
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