g(m1)-ganglioside and Muscular-Atrophy

Motor neuropathy with multifocal conduction blocks represents a recently identified autoimmune disorder of the peripheral nerve myelin. Association of motor neuropathies or neuronopathies with thyroid disorders, such as hyperthyroidism, hypothyroidism or thyroid neoplasms has been rarely described. We studied a 61-year-old man with a 2-year-history of slowly progressive weakness of the left limbs with atrophy and fasciculations. Nerve conduction velocity studies revealed multifocal motor conduction blocks. Serum IgM titer of antibodies against GM1 was elevated (1:1280; n.v. up to 1:640). Thyroid studies were compatible with Hashimoto's thyroiditis. Therapy with high dose intravenous immunoglobulins was followed by a prompt clinical recovery. Then the disease assumed an intravenous immunoglobulins dependent course with a full clinical, but transient, recovery. This is the first observation of an association of multifocal motor neuropathy with high titers of GM1 and Hashimoto's thyroiditis and reinforces the multifocal motor neuropathy autoimmune origin as well as the repeated clinical recoveries after intravenous immunoglobulins. This case also suggests to deeply investigate the thyroid function in patients with multifocal motor neuropathy.

Topics: Demyelinating Diseases; Electromyography; Enzyme-Linked Immunosorbent Assay; Fasciculation; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Male; Middle Aged; Motor Neuron Disease; Muscle Weakness; Muscular Atrophy; Neural Conduction; Thyroiditis, Autoimmune

Topics: Adult; Arm; Demyelinating Diseases; Diagnosis, Differential; Electromyography; France; G(M1) Ganglioside; Humans; Immunoglobulin M; Male; Median Nerve; Motor Neuron Disease; Muscular Atrophy; Neural Conduction; Peripheral Nervous System Diseases; Recurrence

We report the results of immunosuppressive treatment with intravenous cyclophosphamide in 12 patients with lower motor neuron syndrome and elevated titers of serum autoantibodies to GM1 ganglioside. All patients had lower motor neuron dysfunction including proximal or distal weakness, fasciculation and muscle atrophy, but no upper motor neuron dysfunction such as hyperreflexia, spasticity or Babinski's sign. Electrophysiological studies revealed no evidence of conduction block, but EMG findings of acute or chronic denervation in the limbs were present. Serum biochemistry and immunological studies were negative for M protein. After a 6-month follow-up, despite a fall in antibody titer, there was no significant clinical improvement in any of the patients.

Topics: Aged; Autoantibodies; Cyclophosphamide; Electromyography; Female; G(M1) Ganglioside; Humans; Immunoglobulin M; Immunosuppressive Agents; Male; Middle Aged; Motor Neuron Disease; Motor Neurons; Muscular Atrophy; Syndrome