Bickerstaff’s brainstem encephalitis, Miller Fisher syndrome and Guillain-Barré syndrome overlap in an asthma patient with negative anti-ganglioside antibodies

Abstract

BackgroundBickerstaff’s brainstem encephalitis (BBE), together with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) were considered to form a continuous clinical spectrum. An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody.Case presentationHere we report a 20-year-old male patient with overlapping BBE, MFS and GBS. The patient had a positive family history of bronchial asthma and had suffered from the condition for over 15 years. He developed BBE symptoms nine days after an asthma exacerbation. During the course of illness, he had significantly elevated IgE levels in both serum and cerebrospinal fluid. Serologic analysis of antibodies against ganglioside complexes (anti-GDIa, anti-GDIb, anti-GM1, anti-GM2, anti-GM3, anti-GQIb and anti-GTIb antibodies) showed negative results.ConclusionsSince asthma has recently been related to autoimmune disease, our case supports an autoimmune mechanism underlying the clinical spectrum composed of BBE, MFS and GBS. However, contrary to a proposed anti-GQ1b antibody syndrome, we would suggest that pathogenesis of this clinical spectrum is not limited to anti-ganglioside antibodies.

A rare mimic of acute stroke: rapidly progressing Miller-Fisher Syndrome to acute motor and sensory axonal neuropathy variant of Guillain-Barre Syndrome

“…There are only three previously documented cases of MFS progressing to AMSAN, of which two were males, with an age range of 20–72 years (table 1). Unlike our patient, all three previously reported cases had comorbid BBE during the course of their illness 5 14 15. Anti-GQ1b antibodies have been shown to be present in patients with MFS and BBE, explaining the overlap between these two diseases 16.…”

“…The presence of these antibodies in the two patients with MFS-AMSAN-BBE overlap is evidence that the three conditions may actually be part of the same disease spectrum 5 14. However, these antibodies may not be solely responsible for this clinical spectrum as one patient tested negative for all the antibodies 15…”

Peripheral neuropathy in limbic encephalitis with anti‐glutamate receptor antibodies: Case report and systematic literature review

“…Besides, anticipating Guillain–Barré syndrome (GBS) had also been reported in patients with anti‐NMDA receptor encephalitis (Tojo et al., ; Pruss et al., ). Moreover, GBS may also develop concurrently with acute disseminated encephalomyelitis (Bernard, Riou, Rosenblatt, Dilenge, & Poulin, ), Bickerstaff's brainstem encephalitis (Odaka et al., ; Han et al., ), and combined central and peripheral demyelination (Ogata et al., ; Cortese et al., ). In clinical practice, peripheral symptoms may be masked by severe CNS dysfunctions (Joubert et al., ).…”

Bickerstaff brainstem encephalitis, acute transverse myelitis, and acute motor axonal neuropathy: diagnostic and treatment challenges in patients with concomitant syndromes. Clinical observation

“…В последующем подобные синдромы стали называться перекрестными, что подразумевало перекрытие симп-томов 2 и более заболеваний предположительно одной природы, таких как СГБ, СЭБ и СМФ [9,17,18]. Се-годня описаны разные сочетания перечисленных со-стояний как у взрослых, так и у детей [5,9,10,[19][20][21]. При обсуждении вопроса о перекрытии симптомов перечисленных форм дизиммунных расстройств обра-щено внимание на клиническую схожесть СЭБ и СМФ.…”