Novel variants in a patient with late-onset hyperprolinemia type II: diagnostic key for status epilepticus and lactic acidosis

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Novel POLG variants associated with late-onset de novo status epilepticus and progressive ataxia

Mitochondrial disease is phenotypically and genetically heterogeneous with an estimated prevalence of 1 in 4,300. Mutations in the POLG gene, encoding the catalytic subunit of DNA polymerase gamma, are an important cause of mitochondrial disease. The spectrum of clinical manifestations in POLG-related mitochondrial disease is variable, with disease onset ranging from adulthood-onset dominant or...

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Lactic Acidosis in Status Asthmaticus

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Type II hyperprolinemia: a case report.

Hyperprolinemia type II (HP II) is a rare inherited metabolic disease due to the deficiency of pyroline-5-carboxylate dehydrogenase. It is generally believed to be a benign condition although some patients have neurological problems such as refractory convulsions. Here we report a six-year-old girl with HP II who admitted to our hospital with recurrent seizure refractory to multiple antiepilept...

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ژورنال

عنوان ژورنال: BMC Neurology

سال: 2019

ISSN: 1471-2377

DOI: 10.1186/s12883-019-1583-0