Motor neuron disease Madras

Classification according to ICD-10
G12.2	Motor neuron disease
ICD-10 online (WHO version 2019)

The motor neuron disease Madras , MMND for short , ( English Madras motor neuron disease ) belongs to the motor neuron diseases and is characterized by weakness beginning in adolescence with atrophy of the limbs together with various cranial nerve palsies and sensorineural hearing loss .

distribution

The frequency is not known, so far almost 200 patients have been described, mostly from southern India. The disease occurs sporadically and nothing is known about heredity. This special form represents around 4% of all forms of motor neuron diseases .

causes

Changes in mitochondrial DNA are suspected.

Clinical manifestations

The main clinical symptoms are:

Skinny figure
Atrophy of the muscles in the distal parts of the limbs
Involvement of facial and swallowing muscles, cranial nerves VII, IX and XII
Pyramid sign .
Hearing loss

diagnosis

The diagnosis is based on clinical findings. In electromyography there is a chronic partial denervation .

Differential diagnosis

The following are to be distinguished:

therapy

A causal treatment is currently not possible. Providing hearing aids is considered helpful.

Prospect of healing

The disease is slowly progressing , but does not affect life expectancy.

literature

E. Meenakshisundaram, K. Jagannathan, B. Ramamurthi: Clinical pattern of motor neuron disease seen in younger age groups in Madras. In: Neurology India. Vol. 18, December 1970, p. Suppl 1, ISSN 0028-3886 , p. 109 ff., PMID 5508105 .
A. Nalini, K. Thennarasu, BK Yamini, D. Shivashankar, N. Krishna: Madras motor neuron disease (MMND): clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971-2007). In: Journal of the neurological sciences. Vol. 269, No. 1-2, June 2008, ISSN 0022-510X , pp. 65-73, doi: 10.1016 / j.jns.2007.12.026 , PMID 18261745 .
A. Nalini, A. Pandraud, K. Mok, H. Houlden: Madras motor neuron disease (MMND) is distinct from the riboflavin transporter genetic defects that cause Brown-Vialetto-Van Laere syndrome. In: Journal of the neurological sciences. Vol. 334, No. 1–2, November 2013, ISSN 1878-5883 , pp. 119–122, doi: 10.1016 / j.jns.2013.08.003 , PMID 24139842 , PMC 4068726 (free full text).

Individual evidence

↑ ^a ^b ^c ^d ^e Madras Motor Neuron Disease. In: Orphanet (Rare Disease Database).
^ ^A ^b M. Gourie-Devi, TG Suresh: Madras pattern of motor neuron disease in South India. In: Journal of Neurology, Neurosurgery, and Psychiatry . Vol. 51, No. 6, June 1988, ISSN 0022-3050 , pp. 773-777, PMID 3404185 , PMC 1033146 (free full text).
↑ M. Gourie-Devi, A. Nalini: Madras motor neuron disease variant, clinical features of seven patients. In: Journal of the neurological sciences. Vol. 209, No. 1-2, May 2003, ISSN 0022-510X , pp. 13-17, PMID 12686396 .

[Orpha-1] Madras Motor Neuron Disease. In: Orphanet (Rare Disease Database).

[Gourie-2] A ^b M. Gourie-Devi, TG Suresh: Madras pattern of motor neuron disease in South India. In: Journal of Neurology, Neurosurgery, and Psychiatry . Vol. 51, No. 6, June 1988, ISSN 0022-3050 , pp. 773-777, PMID 3404185 , PMC 1033146 (free full text).

[3] M. Gourie-Devi, A. Nalini: Madras motor neuron disease variant, clinical features of seven patients. In: Journal of the neurological sciences. Vol. 209, No. 1-2, May 2003, ISSN 0022-510X , pp. 13-17, PMID 12686396 .