Valproic acid for myoclonic epilepsy in POLG1 carriers can be fatal

Josef Finsterer

doi:10.5114/fn.2021.104542

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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abstract:

Letter to the Editor

Valproic acid for myoclonic epilepsy in POLG1 carriers can be fatal

Josef Finsterer

¹

Klinik Landstrasse, Messerli Institute, Vienna, Austria

Folia Neuropathol 2021; 59 (1): 17-18

DOI: https://doi.org/10.5114/fn.2021.104542

Online publish date: 2021/03/31

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With interest we read the article by Tarka et al. about the autopsy findings of an 8-year-old female with mitochondrial disorder (MID) due to the compound heterozygous variants c.2243G>C and c.2542G>A in POLG1 [1]. The patient manifested clinically with mental retardation, developmental regression, and myoclonic epilepsy, for which she received valproic acid (VPA) [1]. Neuropathological studies after death from acute pancreatitis and liver failure revealed bilaterally symmetric degenerative lesions of the accessory olivary nuclei in addition to typical features of Alpers-Huttenlocher disease (AHD) [1]. It was concluded that pancreatitis prior to liver failure is unusual [1]. The study is appealing but raises comments and concerns.

keywords:

Valproic acid for myoclonic epilepsy in POLG1 carriers can be fatal

Josef Finsterer 1

POLG1, epilepsy, Alpers-Huttenlocher disease, liver failure, mitochondrial disorder

Josef Finsterer

¹