Stroke-like Episodes in Inherited Neurometabolic Disorders

Stroke-like episodes (SLEs) are significant clinical manifestations of metabolic disorders affecting the central nervous system. Morphological equivalents presented in neuroimaging procedures are described as stroke-like lesions (SLLs). It is crucial to distinguish SLEs from cerebral infarction or i...

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Main Authors: Natalia Będkowska, Aneta Zontek, Justyna Paprocka
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:Metabolites
Subjects:
Online Access:https://www.mdpi.com/2218-1989/12/10/929
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author Natalia Będkowska
Aneta Zontek
Justyna Paprocka
author_facet Natalia Będkowska
Aneta Zontek
Justyna Paprocka
author_sort Natalia Będkowska
collection DOAJ
description Stroke-like episodes (SLEs) are significant clinical manifestations of metabolic disorders affecting the central nervous system. Morphological equivalents presented in neuroimaging procedures are described as stroke-like lesions (SLLs). It is crucial to distinguish SLEs from cerebral infarction or intracerebral hemorrhage, mainly due to the variety in management. Another significant issue to underline is the meaning of the main pathogenetic hypotheses in the development of SLEs. The diagnostic process is based on the patient’s medical history, physical and neurological examination, neuroimaging techniques and laboratory and genetic testing. Implementation of treatment is generally symptomatic and includes L-arginine supplementation and adequate antiepileptic management. The main aim of the current review was to summarize the basic and actual knowledge about the occurrence of SLEs in various inherited neurometabolic disorders, discuss the possible pathomechanism of their development, underline the role of neuroimaging in the detection of SLLs and identification of the electroencephalographic patterns as well as histological abnormalities in inherited disorders of metabolism.
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spelling doaj.art-ba3ce8eba60f43ecacc02d0b246c243b2023-11-24T01:15:23ZengMDPI AGMetabolites2218-19892022-09-01121092910.3390/metabo12100929Stroke-like Episodes in Inherited Neurometabolic DisordersNatalia Będkowska0Aneta Zontek1Justyna Paprocka2Students’ Scientific Society, Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, PolandStudents’ Scientific Society, Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, PolandDepartment of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, PolandStroke-like episodes (SLEs) are significant clinical manifestations of metabolic disorders affecting the central nervous system. Morphological equivalents presented in neuroimaging procedures are described as stroke-like lesions (SLLs). It is crucial to distinguish SLEs from cerebral infarction or intracerebral hemorrhage, mainly due to the variety in management. Another significant issue to underline is the meaning of the main pathogenetic hypotheses in the development of SLEs. The diagnostic process is based on the patient’s medical history, physical and neurological examination, neuroimaging techniques and laboratory and genetic testing. Implementation of treatment is generally symptomatic and includes L-arginine supplementation and adequate antiepileptic management. The main aim of the current review was to summarize the basic and actual knowledge about the occurrence of SLEs in various inherited neurometabolic disorders, discuss the possible pathomechanism of their development, underline the role of neuroimaging in the detection of SLLs and identification of the electroencephalographic patterns as well as histological abnormalities in inherited disorders of metabolism.https://www.mdpi.com/2218-1989/12/10/929stroke-like episodestroke-like lesioninherited errors of metabolismneurometabolic disordersMELASMERRF
spellingShingle Natalia Będkowska
Aneta Zontek
Justyna Paprocka
Stroke-like Episodes in Inherited Neurometabolic Disorders
Metabolites
stroke-like episode
stroke-like lesion
inherited errors of metabolism
neurometabolic disorders
MELAS
MERRF
title Stroke-like Episodes in Inherited Neurometabolic Disorders
title_full Stroke-like Episodes in Inherited Neurometabolic Disorders
title_fullStr Stroke-like Episodes in Inherited Neurometabolic Disorders
title_full_unstemmed Stroke-like Episodes in Inherited Neurometabolic Disorders
title_short Stroke-like Episodes in Inherited Neurometabolic Disorders
title_sort stroke like episodes in inherited neurometabolic disorders
topic stroke-like episode
stroke-like lesion
inherited errors of metabolism
neurometabolic disorders
MELAS
MERRF
url https://www.mdpi.com/2218-1989/12/10/929
work_keys_str_mv AT nataliabedkowska strokelikeepisodesininheritedneurometabolicdisorders
AT anetazontek strokelikeepisodesininheritedneurometabolicdisorders
AT justynapaprocka strokelikeepisodesininheritedneurometabolicdisorders