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Αλέξανδρος Γ. Σφακιανάκης

Thursday, November 26, 2020

Multiple cranial nerve enlargement in Charcot-Marie-Tooth disease

alkiviadis.1961 shared this article with you from Inoreader

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Hypertrophy of peripheral nerves are commonly reported in patients with demyelinating forms of Charcot-Marie-Tooth disease (CMT).1 Herein, an under-recognised association with hypertrophy of multiple cranial nerves in a patient with CMT is described.

A 39-year-old otherwise healthy man presented with a 2-month history of tension-type headaches without other associated neurological symptoms. Examination showed normal visual acuity but mild blunting of the nasal disc margins bilaterally on funduscopy. No other crani al nerve abnormalities were elicited. In the lower limbs, there was prominent pes cavus deformity involving both feet (figure 1A) associated with subtle ankle dorsiflexion weakness and impaired distal proprioception. Power in the upper limbs was normal and deep tendon reflexes were globally depressed.

Neurophysiological examination () revealed absent sensory responses in the upper and lower limbs while distal motor responses in the lower limbs were reduced in amplitude. Distal motor latencies were significantly prolonged in the upper and...

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