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Case Reports
. 2024 Oct 23;24(1):410.
doi: 10.1186/s12883-024-03906-7.

Pregnancy and delivery after functional hemispherectomy for Rasmussen's encephalitis: a case report

Elena Jost  1 , Waltraut M Merz  2 , Patrick A Kupczyk  3 , Laura Tascón Padrón  2 , Eva C Weber  4 , Christian G Bien  5 , Philipp Kosian  2
Affiliations
Case Reports

Pregnancy and delivery after functional hemispherectomy for Rasmussen's encephalitis: a case report

Elena Jost et al. BMC Neurol. .

Abstract

Background: Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive seizures and unilateral cerebral atrophy with onset during childhood and unknown etiology. When medical therapy appears refractory, surgical disconnection of the affected hemisphere is indicated. Quality of life after functional hemispherectomy is largely good, affected females may therefore pursue pregnancy. However, data on pregnancy and delivery in RE post hemispherectomy is extremely rare.

Case presentation: We present the case of a patient with left functional hemispherectomy for RE at the age of seven, who experienced two successful pregnancies. In both pregnancies, her post-surgical symptoms including right-sided spasticity, cephalgia, dizziness, and impairment of vision and speech deteriorated but improved to pre-pregnancy level after delivery. Neurologic sequelae post-hemispherectomy overlapped with clinical signs of preeclampsia and required close diagnostic surveillance during both pregnancies.

Conclusion: There are no data on the interaction between RE, hemispherectomy and pregnancy, making maternal and fetal risk assessment difficult. Due to the complexity of the condition and symptoms, management of RE in pregnancy remains highly challenging and requires an interdisciplinary approach. This is the first case description of two successful pregnancies in a woman with RE and status post-hemispherectomy. Further evidence is urgently required to improve counseling and management of affected women.

Keywords: Case report; High-risk pregnancy; Pre-existing condition; Rare disease; Rasmussen’s encephalitis.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
MRI showing Rasmussen’s encephalitis after left functional hemispherectomy. MRI in transverse plane (A, FLAIR sequence) and coronal plane (B, T2-TSE sequence): Extensive left hemispheric T2-hyperintense defect areas involving the cortex, ex vacuo, dilatation of the left lateral ventricle and ipsilateral midline shift reflecting unilateral cerebral atrophy
See this image and copyright information in PMC

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