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Emergency: requires immediate attention
Acute motor axonal neuropathy
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Emergency: requires immediate attention

Acute motor axonal neuropathy

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Contributors: Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Guillain-Barré syndrome (GBS) represents a spectrum of rare acute immune-related polyneuropathies with varying features and presentations. It is divided into 2 main subtypes: demyelinating and axonal. Demyelinating forms include acute inflammatory demyelinating polyneuropathy (AIDP) and clinical variant Miller Fisher syndrome (MFS). Axonal forms include acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN).

AMAN is characterized by symmetric limb weakness, facial and oropharyngeal muscle weakness, areflexia, and respiratory insufficiency. A variant is AMSAN, which involves damage to axons of sensory neurons in addition to those of motor neurons and is characterized by acute onset of quadriparesis, distal sensory loss, areflexia, and respiratory insufficiency. There can also be autonomic dysfunction in both diseases. AMAN typically progresses to nadir more rapidly than AIDP.

The exact etiology is unclear, but GBS typically presents days to weeks after an infection, most commonly Campylobacter jejuni. Other reported triggers include cytomegalovirus, influenza, Epstein-Barr virus, and human immunodeficiency virus (HIV). Of note, GBS has been reported in patients with probable Zika virus infection in South America, French Polynesia, Latin America, and the Caribbean. Rarely, GBS has been associated with surgery or immunization.

AMAN occurs more frequently in China and Japan. It often progresses more rapidly than AIDP. Recovery is unpredictable; some patients recover completely in days, while others have slow and incomplete recovery.

Codes

ICD10CM:
G61.0 – Guillain-Barre syndrome

SNOMEDCT:
715770009 – Acute motor axonal neuropathy

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Therapy

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Last Reviewed: 02/08/2018
Last Updated: 03/13/2018
Copyright © 2019 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Acute motor axonal neuropathy
Print 1 Images
Acute motor axonal neuropathy (Acute motor axonal neuropathy) : Flaccid paralysis, Muscle weakness
Copyright © 2019 VisualDx®. All rights reserved.