Cp. Tsai et al., IMMUNOSUPPRESSIVE TREATMENT IN LOWER MOTOR-NEURON SYNDROME WITH AUTOANTIBODIES AGAINST GM1 GANGLIOSIDE, European neurology, 33(6), 1993, pp. 446-449
We report the results of immunosuppressive treatment with intravenous
cyclophosphamide in 12 patients with lower motor neuron syndrome and e
levated titers of serum autoantibodies to GM 1 ganglioside. All patien
ts had lower motor neuron dysfunction including proximal or distal wea
kness, fasciculation and muscle atrophy, but no upper motor neuron dys
function such as hyperreflexia, spasticity or Babinski's sign. Electro
physiological studies revealed no evidence of conduction block, but EM
G findings of acute or chronic denervation in the limbs were present.
Serum biochemistry and immunological studies were negative for M prote
in. After a 6-month follow-up, despite a fall in antibody titer, there
was no significant clinical improvement in any of the patients.