Central motor conduction in hereditary motor and sensory neuropathy and hereditary spastic paraplegia.
Conduction of the central motor pathways (CMCT) by magnetic stimulation of the motor cortex (TMS) was performed in 17 patients with hereditary motor sensory neuropathy (HMSN) and 2 siblings with hereditary spastic paraplegia (HSP). CMCT was prolonged in two patients with HMSN I with associated pyramidal features and in two subjects with HMSN II without clinical pyramidal signs. CMCT may be abnormal in HMSN due to central motor pathways involvement or altered spinal excitability with increased synaptic delay. CMCT was normal in the upper limbs in patients with HSP but increased in the legs. Diagnostic yield of TMS increased in less disabled cases with HSP when selective conduction at the spinal level (C7-S1) was calculated. Abnormal spinal conduction in HSP is consistent with degeneration of the crossed corticospinal tracts at the thoracic level found in neuropathologic observations.
Cruz Martínez
A
A
Unidad de Electromiografía, Clínica La Luz, Madrid, Spain.
Tejada
J
J
D000293:Adolescent; D000328:Adult; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D011712:Pyramidal Tracts / Q000503:physiopathology*; D011930:Reaction Time / Q000502:physiology; D015419:Spastic Paraplegia, Hereditary / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D013116:Spinal Cord / Q000503:physiopathology*; D013569:Synapses / Q000502:physiology; D009435:Synaptic Transmission / Q000235:genetics / Q000502:physiology*