PREGNANCY AND DELIVERY IN CHARCOT-MARIE-TOOTH DISEASE TYPE-1

We report the obstetric complications and the influence of pregnancy a nd delivery in 21 Charcot-Marie-Tooth disease type 1 (CMT 1) patients with 45 gestations. Sixteen patients had subjective disabilities from childhood or youth, and five with late onset had subclinical CMT when they became pregnant. The rate of obstetric complications in the study group was in accordance with that of the normal population, and there was no deleterious effect on fetal outcome. Of the 21 participants, 3 8% (8 of 21) reported an exacerbation of CMT in at least one pregnancy . These patients noted increasing weakness in 81% (17 of 21) of their gestations. A temporary worsening occurred in 35% (6 of 17) of these p regnancies, and neurologic disabilities persisted after 65% of the del iveries (11 of 17). Patients who had pregnancy-associated progression in the first gestation (7 of 21) experienced similar deterioration in subsequent pregnancies (10 of 11), ie, there is a high risk for recurr ence of exacerbations. Four women (19%) stated that their last deliver ies were responsible for either an exacerbation or the onset of the ne uropathy. The remaining nine patients (43%) denied any effect of their gestations on the progression of the neuropathy. Among the patients w ho had subjective disabilities from childhood or youth, the risk of a noticeable exacerbation in at least one pregnancy was 50% (8 of 16) an d affected 81% of their gestations (17 of 21), whereas there was no in fluence of pregnancy in the five patients with adult onset of CMT, alt hough the first symptoms were noticed postpartum in two of them. Neith er age at onset, age at delivery, interval between onset and delivery, or order or number of gestations are useful criteria for predicting a pregnancy-related progression of CMT 1.