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April 2004

Kennedy Disease: Insights and Questions

Arch Neurol. 2004;61(4):603. doi:10.1001/archneur.61.4.603-a

I read with interest the ARCHIVES article on Kennedy disease (KD) by Sperfeld et al.1 First, I would like to emphasize 2 distinctive clinical features besides gynecomastia that are highly suggestive of the disease and distinguish patients with KD from patients with amyotrophic lateral sclerosis and other types of motor neuron disease. Facial and particularly perioral fasciculations are highly characteristic, if not clinically pathognomonic, of KD and are reportedly present in more than 95% of all patients. This feature is not a common finding in any other inherited or acquired degenerative disorder of the human nervous system. Also, careful neurological examination in most patients with KD reveals a decrease in vibratory perception, especially distally in the legs, and this feature is present even in patients without sensory symptoms.2

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