The sudden onset of muscle weakness and a flaccid paralysis can be due to several causes, such as polyneuropathies, myelopathies, myopathies or neuromuscular junction dysfunction. The most common etiologies are infectious, inflammatory or toxic so differential diagnosis is needed to identify the underlying cause and treat it properly. Guillain–Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide. It represents a continuous spectrum of related acute immune-mediated polyneuropathy. Acute motor and sensory axonal neuropathy (AMSAN) is a rare axonal subtype of GBS, characterized by antibody-mediated axonal degeneration of motor and sensory nerves [6]. Molecular mimicry between C. jejuni lipooligosaccharide and human gangliosides is considered one of the main mechanisms in the pathogenesis of GBS and related disorders. In this case report, we describe a patient with fulminant AMSAN unresponsive to treatment, which progressed to a “brain death”-like state with absent brainstem reflexes that lasted for 40 days.

A case of acute motor and sensory axonal neuropathy mimicking brain death / Libonati, Laura; Fiorini, Ilenia; Cambieri, Chiara; Ceccanti, Marco; Inghilleri, Maurizio. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-3478. - (2021). [10.1007/s10072-021-05051-2]

A case of acute motor and sensory axonal neuropathy mimicking brain death

Laura Libonati;Ilenia Fiorini;Chiara Cambieri;Marco Ceccanti;Maurizio Inghilleri
2021

Abstract

The sudden onset of muscle weakness and a flaccid paralysis can be due to several causes, such as polyneuropathies, myelopathies, myopathies or neuromuscular junction dysfunction. The most common etiologies are infectious, inflammatory or toxic so differential diagnosis is needed to identify the underlying cause and treat it properly. Guillain–Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide. It represents a continuous spectrum of related acute immune-mediated polyneuropathy. Acute motor and sensory axonal neuropathy (AMSAN) is a rare axonal subtype of GBS, characterized by antibody-mediated axonal degeneration of motor and sensory nerves [6]. Molecular mimicry between C. jejuni lipooligosaccharide and human gangliosides is considered one of the main mechanisms in the pathogenesis of GBS and related disorders. In this case report, we describe a patient with fulminant AMSAN unresponsive to treatment, which progressed to a “brain death”-like state with absent brainstem reflexes that lasted for 40 days.
2021
neuropathy, brain death, neurophysiology, AMSAN
01 Pubblicazione su rivista::01i Case report
A case of acute motor and sensory axonal neuropathy mimicking brain death / Libonati, Laura; Fiorini, Ilenia; Cambieri, Chiara; Ceccanti, Marco; Inghilleri, Maurizio. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-3478. - (2021). [10.1007/s10072-021-05051-2]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1477877
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