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01-06-2019 | Stroke | Original Communication

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features

Authors: Yu Hongo, Juntaro Kaneko, Hiroki Suga, Daisuke Ishima, Eiji Kitamura, Tsugio Akutsu, Yuya Onozawa, Naomi Kanazawa, Tomohide Goto, Kazutoshi Nishiyama, Takahiro Iizuka

Published in: Journal of Neurology | Issue 6/2019

Abstract

Objectives

To investigate a diversity of stroke-like episodes (SLEs) in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and report a disseminated form of SLEs (D-SLEs) attributed to a cluster of disseminated small cortical lesions.

Methods

We retrospectively reviewed the clinical information of 27 MELAS patients seen at Kitasato University Hospital between January 1990 and April 2018. Among those, we selected 13 patients with m.3243A>G mutation [median age at onset, 35 years (11–68 years), two pediatric onset < 17 years] who had at least one SLE. SLEs were classified into classic or non-classic based on characteristic features of stroke-like lesions.

Results

44 SLEs were identified during a median observational period of 119 months (3–240 months). Among those, 29 (65.9%) were classic SLEs (C-SLEs) mainly attributed to a single continuous lobular lesion incongruent to vascular territory and occasionally accompanied by a gradual spread associated with hyperperfusion and persistent seizure activity. The remaining 15 were non-classic attributed to sparsely distributed (n = 10), disseminated (n = 4) or cerebellar lesions (n = 1). C-SLEs developed in all patients but non-classic SLEs in 5; D-SLEs developed in 4 patients accounting for 4 of 44 SLEs (9.1%). Non-classic SLEs developed more frequently in pediatric-onset than in adult-onset patients (12/15 vs. 3/29, p < 0.0001). SLEs began with acute onset of symptoms in 42 SLEs (95.5%), but D-SLEs of 2 adult-onset patients began with ill-defined subacute-onset fluctuating encephalopathy.

Conclusions

This study showed a diversity of SLEs in patients with m.3243A>G mutation. Further studies are required to elucidate the pathophysiological mechanisms of non-classic SLEs including D-SLEs.

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Title: A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features
Authors: Yu Hongo
Juntaro Kaneko
Hiroki Suga
Daisuke Ishima
Eiji Kitamura
Tsugio Akutsu
Yuya Onozawa
Naomi Kanazawa
Tomohide Goto
Kazutoshi Nishiyama
Takahiro Iizuka
Publication date: 01-06-2019
Publisher: Springer Berlin Heidelberg
Keywords: Stroke
Cranial MRI
Published in: Journal of Neurology / Issue 6/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09283-3

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features

Abstract

Objectives

Methods

Results

Conclusions

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusions

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