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April 2004

Kennedy Disease: Insights and Questions—Reply

Arch Neurol. 2004;61(4):603-604. doi:10.1001/archneur.61.4.603-b

In reply

We absolutely agree with Dr Paparounas that perioral fasciculations are characteristic of KD, but none of our patients showed perioral fasciculations in the early stages of the disease. Early stages are characterized by the presence of other features, as described in detail in our article.1 All of our patients, however, showed perioral fasciculations in later disease stages. No data were available for when the fasciculations became manifest for the first time. In our study, there were 10 patients in whom generalized fasciculations occurred many years before weakness or atrophy developed. Concerning sensory involvement, our data showed that a dimished vibration sense was a clinical finding in 50% of the patients in later stages of the disease. Although excellent reports have described sensory involvement in KD, no information about the manifestation age is available.2,3 Patients described in these reports were in later disease stages as shown by age and the presence of weakness, muscular atrophy, fasciculations, and tremor.

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