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Lookup NU author(s): Dr Yi NgORCiD,
Professor Grainne Gorman
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© 2023 Elsevier B.V.Stroke-like episode is a paroxysmal neurological manifestation which affects a specific group of patients with mitochondrial disease. Focal-onset seizures, encephalopathy, and visual disturbances are prominent findings associated with stroke-like episodes, with a predilection for the posterior cerebral cortex. The most common cause of stroke-like episodes is the m.3243A > G variant in MT-TL1 gene followed by recessive POLG variants. This chapter aims to review the definition of stroke-like episode and delineate the clinical phenomenology, neuroimaging and EEG findings typically seen in patients. In addition, several lines of evidence supporting neuronal hyper-excitability as the key mechanism of stroke-like episodes are discussed. The management of stroke-like episodes should focus on aggressive seizure management and treatment for concomitant complications such as intestinal pseudo-obstruction. There is no robust evidence to prove the efficacy of L-arginine for both acute and prophylactic settings. Progressive brain atrophy and dementia are the sequalae of recurrent stroke-like episode, and the underlying genotype in part predicts prognosis.
Author(s): Ng YS, Gorman GS
Editor(s): Horvath R; Hirano M; Chinnery PF
Publication type: Book Chapter
Publication status: Published
Book Title: Mitochondrial Diseases
Print publication date: 16/06/2023
Online publication date: 20/02/2023
Acceptance date: 02/04/2022
Series Title: Handbook of Clinical Neurology
Publisher: Elsevier B.V.
Place Published: Amsterdam
PubMed id: 36813321
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