Motor conduction block and high titres of anti-GM1 ganglioside antibodies: pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome.
A patient with a progressive lower motor neuron syndrome and neurophysiological evidence of motor axon loss, multifocal proximal motor nerve conduction block, and high titres of anti-ganglioside GM1 antibodies. Neuropathological findings included a predominantly proximal motor radiculoneuropathy with multifocal IgG and IgM deposits on nerve fibres associated with a loss of spinal motor neurons. These findings support an autoimmune origin of this lower motor neuron syndrome with retrograde degeneration of spinal motor neurons and severe neurogenic muscular atrophy.
Adams
D
D
Centre Hospitalier, Universitaire Vaudois, Lausanne, Switzerland.
Kuntzer
T
T
Steck
A J
AJ
Lobrinus
A
A
Janzer
R C
RC
Regli
F
F
D000368:Aged; D001323:Autoantibodies / Q000097:blood; D004797:Enzyme-Linked Immunosorbent Assay; D005677:G(M1) Ganglioside / Q000276:immunology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D016472:Motor Neuron Disease / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D009431:Neural Conduction / Q000502:physiology; D009469:Neuromuscular Junction / Q000503:physiopathology; D010525:Peripheral Nerves / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology
D001323:Autoantibodies; D005677:G(M1) Ganglioside