Progressive muscular atrophy

from Wikipedia, the free encyclopedia
Classification according to ICD-10
G12.2 Motor neuron disease
ICD-10 online (WHO version 2019)

The Progressive muscular atrophy (PMA) is a rare form of motor neuron disease and is a variant of amyotrophic lateral sclerosis viewed (ALS).

Although only the lower motor neuron is affected, the course of the disease is the same as that of ALS. The differentiation from ALS appears clinically and pathologically difficult.

distribution

The disease is usually not familial. The male sex is more often affected. Transitions in ALS can occur.

Clinical manifestations

Clinical criteria are:

Postmortem, intraneuronal bunina bodies and ubiquitin-positive inclusions can be detected, which are established morphological features of ALS.

Differential diagnosis

A distinction must be made between amyotrophic lateral sclerosis (ALS) and the hereditary forms of spinal muscular atrophy (SMA).

Prospect of healing

The prognosis is determined by the respiratory impairment.

history

The name was coined in 1850 by the French neurologist François-Amilcar Aran (1817–1861).

Individual evidence

  1. ↑ a b c T. Meyer, C. Münch u. a .: Progressive muscular atrophy. An underdiagnosed variant of amyotrophic lateral sclerosis. In: The neurologist. 78, 2007, p. 1383, doi: 10.1007 / s00115-007-2288-y .
  2. ↑ a b c National Institute of Neurological Disorders and Stroke ( Memento of the original from April 13, 2014 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.ninds.nih.gov
  3. ↑ Yuichi Riku, Naoki Atsuta et al. a .: Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis. In: BMJ Open. 4, 2014, p. E005213, doi: 10.1136 / bmjopen-2014-005213 .
  4. ^ FA Aran: Recherches sur une maladie non encore décrite du système musculaire (atrophie musculaire progréssive). In: Archives générales de médecine, Paris, 1850, Vol. 24, pp. 172-214.

Web links