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Transient signs and symptoms of CNS involvement have been reported in Charcot–Marie–Tooth disease type 1X (CMT1X).1,2 We describe a patient with CMT1X and chronic progressive-relapsing inflammatory demyelinating CNS disease.
Case report.
A 44-year-old man was admitted because of subacute onset of gait ataxia, dysphagia, and dysarthria. He had experienced worsening stepping gait for the previous decade, and an EMG examination a year and a half before presentation had shown a demyelinating sensorimotor neuropathy. Otherwise, his medical history was unremarkable. Neurologic examination showed a wide based gait with ataxia worsening after eye closure, upper limb incoordination, and gaze-evoked nystagmus. There was weakness of foot dorsiflexion and toe extension. Tendon reflexes were absent. The patient was dysarthric and had dysphagia for liquids. EMG showed a mixed axonal-demyelinating polyneuropathy (table E-1 on the Neurology Web site at www.neurology.org). Visual evoked potentials (VEPs) were normal, whereas brainstem auditory potentials (BAEPs) showed alterations bilaterally (table E-1). Brain MRI showed multiple T2-hyperintense lesions in the pons, paraventricular …
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