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Published on: June 15, 2018
Mimics and chameleons in Guillain-Barré and Miller Fisher syndromes
Benjamin R Wakerley1, Nobuhiro Yuki2
1Department of Neurology, Gloucestershire Royal Hospital, Gloucester, UK.
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.
Insights
Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) are related disorders with overlapping features. Recognizing their spectrum and atypical presentations is crucial for accurate diagnosis and timely immunotherapy.
Area of Science:
- Neurology
- Immunology
Background:
- Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) are heterogeneous neurological disorders.
- These conditions form a spectrum with shared pathophysiological mechanisms and clinical features.
Purpose of the Study:
- To highlight the pathophysiological relationship and overlapping clinical features of GBS and MFS.
- To discuss diagnostic challenges, mimics, and atypical presentations of GBS-related disorders.
Main Methods:
- Review of clinical features, diagnostic criteria, and differential diagnoses for GBS and MFS.
- Analysis of common and atypical presentations, including mimics.
Main Results:
- GBS and MFS share features like antecedent infection, monophasic course, and weakness.
- Diagnostic support includes CSF albuminocytological dissociation, antibodies, and neurophysiology, but these are not definitive.
- Mimics include stroke, botulism, myasthenia gravis, and Wernicke's encephalopathy; atypical GBS presentations exist.
Conclusions:
- Accurate diagnosis of GBS-related disorders is essential to avoid unnecessary tests.
- Early immunotherapy can be initiated promptly upon correct diagnosis.
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