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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.70 no.7 São Paulo July 2012 



Good obstetric outcome in a patient with Segawa disease


Bom resultado obstétrico em uma paciente com doença de Segawa



Takashi Watanabe; Shigeki Matsubara

Department of Obstetrics and Gynecology, Jichi Medical University, Tochigi, Japan




Dear Editors,

This Journal has repeatedly been dealing with Parkinson's disease and its related disorders, in which cerebral dopamine shortage is a considered culprit for the diseases' manifestation. Segawa disease, first described by Japanese pediatric neurologist Dr. Segawa in 19761, is an autosomal dominant trait characterized by progressive dystonia with marked diurnal fluctuation. Cerebral dopamine shortage is also its considered culprit. Levodopa improves the neurological symptoms. Carbidopa blocks extracerebral decarboxylation of levodopa and thus increases brain uptake of levodopa. Levodopa monotherapy or combination therapy of levodopa and carbidopa is usually employed1. Since Segawa disease manifestation usually starts before reproductive age, neurologists may sometimes be consulted as to pregnancy possibility/outcome or they may have a chance to treat pregnant women associated with this disorder. How pregnancy affects this disorder (or vice-versa) and the appropriate drug selection during pregnancy remains unclear.

We previously reported a primiparous woman with Segawa disease who had a good obstetric outcome2. She received levodopa 1,250 mg/day throughout the pregnancy and gave birth to a healthy infant. She was included among the six patients with nine pregnancies that we summarized2. Of the six patients, five remained asymptomatic under levodopa treatment, whereas the remaining woman not receiving levodopa showed symptom deterioration with stillbirth. Of the nine pregnancies, levodopa monotherapy was performed in six, all with good obstetric outcomes. The one woman receiving combination therapy had two consecutive miscarriages and, subsequently, received levodopa monotherapy during her third pregnancy, which resulted in a healthy infant4. We concluded that during pregnancy levodopa should be continued, and levodopa monotherapy and not combination therapy may be the treatment of choice2.

We report here another primiparous woman with Segawa disease. At the age of eight, bilateral clubfeet appeared. Her brother had already been diagnosed as having Segawa disease. With her condition diagnosed as Segawa disease, administration of levodopa 300 mg/day was started, which completely ameliorated her symptoms. After the age of 20, she had taken levodopa 100-300 mg/day, with the dose modified according to her condition. At the age of 32, she became pregnant and continued to receive levodopa 100-300 mg/day throughout her pregnancy, which well controlled the disease. She vaginally gave term birth to a healthy 3,694 g infant with an Apgar score of 8 at 5 minutes, with an uneventful postpartum course. Levodopa 100-300 mg/day was continued after delivery.

No English or Portuguese reports have been published since our 2009 report3. The present patient is the seventh reported case. This successful pregnancy outcome supports our previous proposal of levodopa monotherapy and continuation of levodopa throughout pregnancy and puerperium. Carvidopa increases the levodopa concentration in organs4, which may be associated with the miscarriage reported; however, miscarriages during combination therapy may have been coincidental. Further study is needed to confirm our speculation.



1. Sagawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H. Hereditary progressive dystonia with marked diurnal fluctuation. In: Eldridge R, Fahn S (eds). Advances in Neurology. Vol.14. New York: Ravan Press, 1976:215-233.         [ Links ]

2. Segawa M, Nomura Y, Nishioka M. Autosomal dominant guanosine triphosphate cyclohydrolase I deficiency (Segawa Disease). Ann Neurol 2003;54:S32-S45.         [ Links ]

3. Watanabe T, Matsubara S, Baba Y, Tanaka H, Suzuki T, Suzuki M. Successful management of pregnancy in a patient with Segawa disease: case report and literature review. J Obstet Gynaecol Res 2009;35:562-564.         [ Links ]

4. Nomoto M, Kaseda S, Iwata S, Osame M, Fukada T. Levodopa in pregnancy. Mov Disord 1997;12:261.         [ Links ]



Shigeki Matsubara
Department of Obstetrics and Gynecology, Jichi Medical University
3311-1 Yakushiji, Shimotsuke, Tochigi
329-0498 Japan

Received 23 January 2012
Received in final form 02 February 2012
Accepted 09 February 2012



Conflict of interest: There is no conflict of interest to declare.

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