А rare clinical case of pregnancy and childbirth in a patient with an implanted neurostimulator for Оppenheim torsion dystonia (DYT-1)

Abstract

The problems of planning, follow-up of pregnancy and childbirth in women with inherited extrapyramidal neurological disorders, including torsion dystonia, are represented by isolated cases and receive little literature coverage. Dystonia is a rare disease, the incidence of its various types is 300–400 patients per million (0.03%). Idiopathic torsion dystonia is a group of genetically determined hyperkinetic disorders, which refers to an extrapyramidal pathology of the central nervous system with a progressive course of the disease. This article presents the case follow-up of pregnancy and childbirth in a patient with torsion dystonia and chronic neurostimulation 1.5 years after implantation of the ActivaRC neurostimulation system without drug therapy. The patient has given birth via elective caesarean section while receiving neurostimulation via the ActivaRC system. This clinical observation shows that the physiological course of pregnancy in patients with this diagnosis is possible in case of its advance planning and correction of the DBS programming. A multidisciplinary approach to the management of a patient with generalized torsion dystonia and chronic neurostimulation made it possible to achieve a favourable pregnancy outcome for both mother and fetus. Follow-up of pregnancy in such patients requires a correction of the DBS programming and choosing the optimal timing and method for delivery. The routine follow-up clinical examination by a neurologist should be carried out both in the planning stage and throughout the entire period of pregnancy (end of the first trimester, weeks 21–23, 32–34), as well as in the postpartum period.