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

01 Pubblicazione su rivista
Libonati Laura, Fiorini Ilenia, Cambieri Chiara, Ceccanti Marco, Inghilleri Maurizio
ISSN: 1590-3478

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.

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