Takamichi Hattori

To investigate the pathophysiological role of anti-GM1 antibody in Guillain-Barré syndrome (GBS), we reviewed sequential nerve conduction studies of 345 nerves in 34 GBS patients. Statistically significant correlation between IgG anti-GM1 antibodies and electrodiagnoses was found. Sixteen IgG anti-GM1-positive patients were classified as having acute motor or acute motor sensory axonal neuropathy (AMAN or AMSAN) (12 patients), as having acute inflammatory demyelinating polyneuropathy (AIDP) (3 patients), or as undetermined (1 patient) by electrodiagnostic criteria. Besides axonal features, there was rapid resolution of conduction slowing and block. In 3 patients initially diagnosed as having AIDP, conduction slowing was resolved within days, and 1 of them and 3 AMAN patients showed markedly rapid increases in amplitudes of distal compound muscle action potentials that were not accompanied by prolonged duration and polyphasia. The time courses of conduction abnormalities were distinct from those in IgG anti-GM1-negative AIDP patients. Rapid resolution of conduction slowing and block, and the absence of remyelinating slow components, suggest that conduction failure may be caused by impaired physiological conduction at the nodes of Ranvier. Reversible conduction failure as well as axonal degeneration constitutes the pathophysiological mechanisms in IgG anti-GM1-positive GBS. In both cases, immune-mediated attack probably occurs on the axolemma of motor fibers.

We report a case of adrenomyeloneuropathy (AMN) in which serial urodynamic studies showed neurogenic bladder dysfunction. The patient was in good health until the age of 12, when he began to lose his hair. At age 25 he started to have urinary urgency, difficulty in voiding, occasional fecal incontinence, erectile impotence, and progressive gait disturbance. In his first admission to our hospital age 31, he was intelligent but childish. He showed diffuse baldness, spastic paraparesis, and disturbed vibratory sensation. Serum cortisol response to corticotropin (ACTH) was low and serum levels of very long chain fatty acids were increased. Nerve conduction studies and sural nerve biopsy showed the presence of peripheral neuropathy. These findings confirmed the diagnosis of AMN. The first urodynamic study showed residual urine volume of 50 ml, impaired bladder sensation, and detrusor hyperreflexia. At age 38 he needed diapers because he became apathetic and demented, and could no longer stand by himself. MRI disclosed high signal intensities in the bilateral cerebral white matter. The second urodynamic study showed residual urine volume of 200 ml and decreased bladder capacity with marked detrusor hyperreflexia. Demyelinating lesions of the peripheral nerve and white matter of the spinal cord and the cerebrum may be mainly responsible for the micturitional disturbance in our patient with AMN.