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Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune encephalitis characterised by ataxia, ophthalmoplegia and altered consciousness. An overlap between BBE with Guillain-Barré syndrome (GBS) shows similar clinical and immunological features. We report a case of BBE with GBS overlap secondary toChlamydia pneumoniaeinfection. The triad of altered consciousness, ataxia and ophthalmoplegia were present in the patient. The investigations included cerebrospinal fluid cytoalbuminological dissociation, nerve conduction test that showed prolonged or absent F wave latencies, hyperintensity in the left occipital region on brain MRI and diffuse slow activity on the electroencephalogram. The chlamydia serology was positive indicating a postinfectious cause of BBE syndrome. He required artificial ventilation as his consciousness level deteriorated with tetraparesis, oropharyngeal and respiratory muscle weakness. Immunotherapy with intravenous immunoglobulin and methylprednisolone was commenced. He made good recovery with the treatment. Prompt recognition of this rare condition following chlamydia infection is important to guide the management.

In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. In the cases with Bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support of a central pathology whereas in Fisher syndrome, patients were areflexic in keeping with a peripheral aetiology. However, both authors recognised certain similarities to Guillain-Barré syndrome such as the presence of peripheral neuropathy and cerebrospinal fluid albuminocytological dissociation. The discovery of immunoglobulin G anti-GQ1b antibodies in patients with Fisher syndrome and later in Bickerstaff brainstem encephalitis was crucial in providing the necessary evidence to conclude that both conditions were in fact part of the same spectrum of disease by virtue of their common clinical and immunological profiles. Following this, other neurological presentations that share anti-GQ1b antibodies emerged in the literature. These include acute ophthalmoparesis and acute ataxic neuropathy, which represent the less extensive spectrum of the disease whereas pharyngeal-cervical-brachial weakness and Fisher syndrome overlap with Guillain-Barré syndrome represent the more extensive end of the spectrum. The conditions can be referred to as the 'anti-GQ1b antibody syndrome'. In this review, we look back at the historical descriptions and describe how our understanding of Fisher syndrome and Bickerstaff brainstem encephalitis has evolved from their initial descriptions more than half a century ago.

Background IgG anti-GQ1b antibodies are associated with Fisher syndrome (FS), Bickerstaff brainstem encephalitis (BBE), acute ophthalmoparesis and overlap of FS or BBE with Guillain–Barre syndrome (GBS) (FS/GBS or BBE/GBS). It has not been clearly established if the primary pathology of these disorders is demyelinating or axonal in nature. Rapid resolution of conduction slowing or block without signs of demyelination–remyelination has been reported in axonal subtypes of GBS that are associated with IgG anti-GM1 or -GD1a antibodies. We hypothesised that such reversible conduction failure would be also observed in FS and related disorders. Methods Serial nerve conduction studies were prospectively performed in 15 patients with FS and related conditions. Results Neither conduction block nor abnormal temporal dispersion was observed in any of the nerves at any point in all the patients. Conduction velocities for none of the nerves were in the demyelinating range. The amplitude of sensory nerve action potential was decreased in three FS, one FS/GBS and two BBE/GBS patients. Compound muscle action potential amplitudes were decreased in the two BBE/GBS patients. These decreases in amplitudes of sensory nerve action potential and compound muscle action potential promptly resolved without significant change in duration on serial studies. Conclusions Reversible conduction failure was seen in six of the 15 patients with FS and related disorders on serial nerve conduction studies. There were no signs of demyelination or remyelination in the 15 patients. The pathology appears to be primarily non-demyelinating. We believe these conditions form a continuous spectrum with axonal GBS.

Objective Clinical and immunological evaluation of ‘incomplete’ Bickerstaff brainstem encephalitis (BBE). Methods We studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside antibodies. Results Both patients displayed hypersomnolence and cerebellar-like ataxia in the absence of external ophthalmoplegia and carried high serum titres of IgG anti-GQ1b antibodies, strongly indicative of BBE. Conclusions Ophthalmoplegia can be absent or incomplete in BBE, and the absence of this clinical feature should not exclude BBE from the clinicians’ differential. Such cases of incomplete BBE could be defined as ‘ataxic hypersomnolence without ophthalmoplegia’.

Bickerstaff brainstem encephalitis (BBE) is characterised by the clinical triad of ophthalmoplegia, ataxia and consciousness disturbance, and the other features supportive of the diagnosis of BBE include antecedent infection, cerebrospinal fluid albuminocytological dissociation and the presence of serum IgG anti-GQ1b antibodies.1 The common clinical immunological profiles to BBE and Fisher syndrome (FS) strongly suggest that both conditions are part of the same spectrum of disease. BBE is a rare disorder, and so far there is only one epidemiological study of BBE; a nationwide survey conducted in Japan has shown that the annual incidence of BBE is …

Frequent detection of anti-GQ1b IgG antibodies shapes the disease concept ‘anti-GQ1b antibody syndrome,’ which has either peripheral nervous system (PNS) or central nervous system (CNS) origins.1 Various phenotypes are known in the PNS origin, which predominantly shows ophthalmoplegia (Miller Fisher syndrome (MFS)), limb weakness (Guillain-Barre syndrome (GBS) with ophthalmoplegia), or sensory ataxia (acute sensory ataxic neuropathy). In contrast, only one phenotype is known in the CNS origin, Bickerstaff brainstem encephalitis (BBE), which accounts for a major proportion of brainstem encephalitis with the clinical triad of ophthalmoparesis, ataxia and impaired consciousness.2 The relatively uniform clinical presentation in the CNS origin …

Bickerstaff brainstem encephalitis (BBE) is characterised by acute ophthalmoplegia, ataxia, and some features suggesting CNS involvement such as consciousness disturbance. In 1951, Bickerstaff and Cloake described three patients in their original report entitled ‘Mesencephalitis and rhomboencephalitis’, and in 1957 Bickerstaff expanded his series to eight patients and proposed the condition ‘brainstem encephalitis’, subsequently termed ‘BBE’. Between the two publications, in 1956 Miller Fisher described three patients with “an unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)”; this condition has been widely recognised as Fisher syndrome (FS). A detailed historical perspective of BBE and FS was presented in a review …