Diphtheritic neuropathy

Ajitesh Rai MD (Dr. Rai of Saint Louis University has no relevant financial relationships to disclose.)
Yi Pan MD PhD (Dr. Pan of St. Louis University has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released October 11, 2004; last updated April 26, 2016; expires April 26, 2019

This article includes discussion of diphtheritic neuropathy, diphtheritic paralysis, diphtheritic polyneuritis, and diphtheritic polyneuropathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Diphtheritic neuropathy is an acute demyelinating polyneuropathy developing 3 to 5 weeks after severe diphtheria infection. It is a biphasic illness with initial bulbar and other cranial nerve involvement followed later by motor weakness in extremities and autonomic dysfunction. Symptoms are caused by an exotoxin, which inhibits protein synthesis and subsequent myelin production. There are a large number of differential diagnoses, the most important being Guillain-Barre syndrome. Diagnostic workup consists of confirming infection and neurophysiological evidence of demyelination. Management is conservative with attention paid to respiration and the airway, which may require mechanical ventilation if significantly impaired.

Key points

 

• Diphtheritic neuropathy is an acute demyelinating polyneuropathy.

 

• Latency in development of polyneuropathy varies from 2 to 6 weeks.

 

• Typical presentation is early weakness in bulbar, followed by weakness in trunk, and later weakness in extremities.

 

• Effective control includes universal immunization with diphtheria toxoid throughout life.

Historical note and terminology

Diphtheritic neuropathy is an acute demyelinating polyneuropathy, which has long been recognized as the most common severe complication of Corynebacterium diphtheriae infection. Diphtheria was recognized in 5th century BC by Hippocrates. The early history of diphtheritic neuropathy was reviewed by Rolleston a century ago (Rolleston 1904). A definite relationship between diphtheria attack and subsequent paralysis was demonstrated during epidemics by Chomel in 1743, Ghisis in 1749, and Bard in 1789. The first monograph on diphtheritic paralysis was written by Maingault in 1854. Maingault was followed by other observers. The best known was Trousseau who collected 90 cases. He found paralysis of palate in 70 cases, generalized paralysis in 64 cases, and other symptoms including amaurosis, strabismus, sensory deficits, paralysis of lower limbs, truck, neck, rectum, and bladder (Rolleston 1904). The first histological investigation was made by Meyer (Meyer 1881). Currently, diphtheritic neuropathy is a rare disease in Western Europe and the United States. The most recent reported cases were from an epidemic in the former Soviet Union, which occurred from 1990 to 1995 (Piradov et al 2001).

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