Table 2

Motor neurone disorders (modified from Kato et al, 2003, with permission)

Genetically determined forms of motor neuron disorder
Familial MND (ALS) (see table 1)
Brown-Vialetto-Van Laere syndrome (early onset bulbar and spinal ALS with sensorineural deafness)
Fazio-Londe syndrome (infantile progressive bulbar palsy)
Hexosaminidase deficiency
Hereditary spastic paraplegia (many forms, including ALS2, ALS4)
Spinal muscular atrophy (SMA)

  • Proximal childhood and later onset forms of SMA (types 1–4), SMN gene related

  • Distal SMA (various forms)

  • Distal SMA with vocal cord involvement (Harper Young syndrome)

  • Adult onset proximal SMA (unrelated to SMN gene mutations)

X linked bulbar and spinal muscular atrophy (Kennedy’s disease) Hereditary motor and sensory neuropathy (predominantly motor forms)
Multisystem disorders with occasional anterior horn cell involvement (e.g. SCA3)
Algrove syndrome
Apparently sporadic (idiopathic) forms of motor neuron disorder Sporadic MND (progressive bulbar palsy; classical limb onset MND/ALS; progressive spinal muscular atrophy)
Primary lateral sclerosis
Distal sporadic focal spinal muscular atrophy (“Hirayama syndrome”)
Atypical juvenile onset MND in South India (“Madras” form of MND)
Western Pacific and other similar forms of MND/ALS (Guam, Kiipeninsula, New Guinea)
Guadeloupe PSP-dementia-MND/ALS syndrome
MND with frontotemporal dementia (MND-dementia syndrome; Pick’s disease with MND)
Multiple system atrophy
Progressive supranuclear palsy
Corticobasal ganglionic degeneration
Acquired forms of motor neuron disorder
HTLV-1 associated myelopathy (HAM)
HIV-associated MND syndrome
Creutzfeld-Jacob disease (amyotrophic forms)
Multifocal motor neuronopathy
Acute poliomyelitis
Lead, mercury toxicity
Neurolathyrism (caused by Lathyrus sativa, containing b—oxalyl-L-aminoacid, BOAA)
Konzo (due to toxic cyanogenic cassava)
Radiation (e.g. cervical and lumbosacral radiculopathy)
Post- polio progressive muscular atrophy syndrome
Autoimmune disorders (e.g. Sjögren’s disease)
Endocrinopathy (hyperthyroidism, hyperparathyroidism, hypoglycaemia)