motor neuron disease

Introduction

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; &

• 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

• structural lesions
  • parasagittal or foramen magnum tumors
  • cervical spondylosis
  • Chiari malformation or syrinx
  • spinal cord arteriovenous malformation
• infections
  • bacterial
    • tetanus
    • Borrelia
  • viral
    • poliomyelitis
    • Herpes zoster
    • HTLV-1
• toxins
  • metals:
    • lead
    • aluminum
  • drugs:
    • strychnine
    • phenytoin
  • electric shock
  • X-irradiation
• immunologic
  • plasma cell dyscrasia
  • autoimmune polyradiculopathy
• paraneoplastic syndromes
• metabolic
  • hypoglycemia
  • hyperparathyroidism
  • hyperthyroidism
  • vitamin deficiency:
    • vitamin B12
    • folate
    • vitamin E
  • malabsorption
• hereditary biochemical disorders
  • androgen receptor defect (Kennedy's disease)
  • hexosaminidase A deficiency
  • infantile alpha-glucosidase deficiency (Pompe's disease)
  • hyperlipidemia
  • hyperglycinuria
  • methylcrotonylglycinuria

More general terms

• neuromuscular disease; myoneural disease
• neurodegenerative disease

More specific terms

• 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)

Patient information

motor neuron disease patient information