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Pregnancy and delivery in Charcot-Marie-Tooth disease type 1

From the Institute for Human Genetics (Drs. Rudnik-Schöneborn, Röhrig, and Zerres), University of Bonn, Bonn, Germany, and the Molecular Genetics Laboratory (Dr. Nicholson), Department of Medicine, University of Sydney, Concord Hospital, Concord, Australia.

We report the obstetric complications and the influence of pregnancy and 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, 38% (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 pregnancies, and neurologic disabilities persisted after 65% of the deliveries (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 recurrence of exacerbations. Four women (19%) stated that their last deliveries were responsible for either an exacerbation or the onset of the neuropathy. The remaining nine patients (43%) denied any effect of their gestations on the progression of the neuropathy. Among the patients who had subjective disabilities from childhood or youth, the risk of a noticeable exacerbation in at least one pregnancy was 50% (8 of 16) and affected 81% of their gestations (17 of 21), whereas there was no influence of pregnancy in the five patients with adult onset of CMT, although the first symptoms were noticed postpartum in two of them. Neither 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

Address correspondence and reprint requests to Dr. Sabine Rudnik-Schöneborn, Institut fur Humangenetik der Universität, Wilhelmstr. 31, D-53111 Bonn, Germany.

Received October 14, 1992. Accepted for publication in final form February 8. 1993.

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