Neurology · 1985 · 38 citations · 0 references
Life SciencesGeneticsNeurologyHuman GeneticsMedical GeneticsNeurogeneticsGenetic DisordersHereditary NeuropathiesMendelian Neurological DisordersMotor NeuropathyNeurogenetic DisordersSensory DisordersAutosomal Dominant Hereditary NeuropathyAutosomal Dominant InheritanceDominant Inheritance ModelsHereditary Motor Neuron DiseaseNerve Conduction VelocitySensory Nerve ConductionSensory-motor NeuropathyX-linked Dominant Inheritance
The inheritance of the hereditary motor and sensory neuropathies (HMSN) is usually autosomal dominant. We studied a kinship with a pattern of X-linked dominant inheritance. The phenotype was similar to HMSN of the "intermediate" type. Men were more severely affected than women, and hypertrophic nerves were not found. Nerve conduction was very slow in men, but it was mildly slow or normal in women. No male-to-male transmission was found in six generations.