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.
Teunissen LL, Notermans NC, Franssen H, van Engelen BGM, Baas F, Wokke JHJ. Disease Course of Charcot-Marie-Tooth Disease Type 2 and Comorbidity—Reply. Arch Neurol. 2004;61(9):1470. doi:10.1001/archneur.61.9.1470-b