Mimics and chameleons in Guillain–Barré and Miller Fisher syndromes

Abstract

Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS) have several subtypes, together forming a continuous spectrum of discrete and overlapping syndromes. Such is the heterogeneity within this spectrum that many physicians may be surprised to learn that these disorders are related pathophysiologically, and therefore share certain clinical features. These include history of antecedent infection, monophasic disease course and symmetrical cranial or limb weakness. The presence of cerebrospinal fluid albuminocytological dissociation (raised protein, normal cell count), antiganglioside antibodies and neurophysiological evidence of axonal or demyelinating neuropathy also support a diagnosis in many cases, but should not be relied upon. Mimics of GBS and MFS can broadly be divided into those presenting with symmetrical limb weakness and those presenting with brainstem signs. MFS and the pharyngeal-cervical-brachial variant of GBS are frequently mistaken for brainstem stroke, botulism or myasthenia gravis, whereas Bickerstaff's brainstem encephalitis is often diagnosed as Wernicke's encephalopathy. Chameleons or atypical presentations of GBS-related disorders include: paraparetic GBS, bifacial weakness with paraesthesias, acute ataxic neuropathy, acute ophthalmoparesis, acute ptosis and acute mydriasis. Many neurologists may also not be aware that deep tendon reflexes remain present or may even appear brisk in up to 10% of patients with GBS. Correct diagnosis of GBS-related disorders helps to avoid unnecessary investigations and allows early immunotherapy if appropriate.

Guillain-Barré syndrome: causes, immunopathogenic mechanisms and treatment

“…West Nile virus and Herpes simplex virus can cause extensive necrotizing myelopathy [83]. West Nile virus and Herpes simplex virus can cause extensive necrotizing myelopathy [83].…”

Guillain-Barré syndrome mimicking botulism in early disease course

“…GBS has a broad spectrum of clinical presentations, including the phenotypes of Bickerstaff encephalitis, Miller Fisher syndrome, and pharyngeal-cervical-brachial variant, described as a botulism mimic 3. The pharyngeal-cervical-brachial variant may occur with anti-GT1a antibodies (although testing is not widely available) and presents with descending paralysis, as opposed to the classic ascending pattern seen in typical GBS.…”

Th17 cells and their cytokines serve as potential therapeutic target in experimental autoimmune neuritis

“…Guillain–Barré syndrome (GBS) is a heterogeneous, immune‐mediated illness that afflicts the peripheral nervous system (Doorn, Ruts, & Jacobs, ). Pathologically, GBS is mainly divided into demyelinating and axonal form, with demyelination form predominating in most countries and regions (Wakerley & Yuki, ). Although plasma exchange and intravenous immunoglobulin were proved to be effective, 20% of GBS patients still suffered from severe disability, and approximately 2%–10% of them died prematurely (Hughes & Cornblath, ).…”