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In reply

We thank Gemignani and Marbini for their important comments on our 5-year prospective study of disease course in CMT2. First, it is unlikely that comorbidity played an important role in the neurological deterioration of our patients; comorbidity causing polyneuropathy was scarce in our cohort. Two patients had previously been treated for hypothyroid disease. During follow-up, 2 other patients developed type 2 diabetes mellitus.

Second, as the authors mention, sensory complaints and abnormalities are not uncommon in CMT.¹ We divided sensory complaints into positive symptoms (tingling), negative symptoms (numbness), and pain; pain was therefore separately scored. Nociceptive pain due to musculoskeletal stress may have played a role because 9 of 43 patients had shoulder, back, hip, or knee complaints.

Finally, it is indeed surprising that quality of life is unchanged in these patients despite the slow progression of polyneuropathy. General quality-of-life questionnaires may not be useful to measure the . . . [Full Text of this Article]

AUTHOR INFORMATION
Laurien L. Teunissen, MD, PhD; Nicolette C. Notermans, MD, PhD; Hessel Franssen, MD, PhD; Baziel G. M. van Engelen, MD, PhD; Frank Baas, MD, PhD; John H. J. Wokke, MD, PhD

RELATED ARTICLE

Disease Course of Charcot-Marie-Tooth Disease Type 2 and Comorbidity
Franco Gemignani and Adriana Marbini
Arch Neurol. 2004;61(9):1470.
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