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motor neuron disease
Disorders characterized by degeneration of motor neurons within the central nervous system (CNS) including: 1) anterior horn cells of the spinal cord; 2) brainstem motor nuclei; &
3) neurons of the motor cortex. These disorders have been classified as primary motor neuron disease of which amyotropic lateral sclerosis (ALS) is the only example & secondary motor neuron disorders.
Etiology:
1) structural lesions
- parasagittal or foramen magnum tumors
- cervical spondylosis
- Chiari malformation or syrinx
- spinal cord arteriovenous malformation
2) infections
a) bacterial
- tetanus
- Borrelia
b) viral
- poliomyelitis
- Herpes zoster
- HTLV-1
3) toxins
a) metals:
- lead
- aluminum
b) drugs:
- strychnine
- phenytoin
c) electric shock
d) X-irradiation
4) immunologic
- plasma cell dyscrasia
- autoimmune polyradiculopathy
5) paraneoplastic syndromes
6) metabolic
a) hypoglycemia
b) hyperparathyroidism
c) hyperthyroidism
d) vitamin deficiency:
- vitamin B12
- folate
- vitamin E
e) malabsorption
7) hereditary biochemical disorders
- androgen receptor defect (Kennedy's disease)
- hexosaminidase A deficiency
- infantile alpha-glucosidase deficiency (Pompe's disease)
- hyperlipidemia
- hyperglycinuria
- methylcrotonylglycinuria
Specific
amyotrophic lateral sclerosis (ALS); Lou Gerig's disease
infantile-onset ascending spastic paralysis
monomelic amyotrophy; benign focal amyotrophy; Hirayama syndrome; O'Sullivan-McLeod syndrome
primary lateral sclerosis (PLS)
progressive bulbar palsy
progressive lower motor neuron disease (PLMND)
progressive pseudobulbar palsy
spinal muscular atrophy (SMA)
General
neurodegenerative disease
neuromuscular disease; myoneural disease
References
- Harrison's Principles of Internal Medicine, 13th ed.
Companion Handbook, Isselbacher et al (eds), McGraw-Hill
Inc. NY, 1995, pg 733-736
- National Institute of Neurological Disorders and Stroke (NINDS)
NINDS Motor Neuron Diseases Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Motor-Neuron-Diseases-Information-Page