Serial electroencephalographic findings in patients with MELAS

Fujimoto, Shinji; Mizuno, Kumiko; Shibata, Hideo; Kanayama, Manabu; Kobayashi, Masanori; Sugiyama, Naruji; Ban, Kyoko; Ishikawa, Tatsuya; Itoh, Tamiko; Togari, Hajime; Wada, Yoshiro

doi:10.1016/s0887-8994(98)00088-5

Cited by 17 publications

(13 citation statements)

References 15 publications

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“…The right occipital lobe showed the area of DWI and FLAIR sequence hyperintensity. A summary of related literature on EEG findings and seizure types in mitochondrial disorders are shown in Table 1 [3,4,[7][8][9][10]. This highlights the fact that our patient is a rare MELAS case of acute cortical blindness episode with Anton-Babinski syndrome due to occipital lobe seizures with clinical, radiological and electroencephalographic concordance (Figures 1 and 2).…”

Section: Discussionsupporting

confidence: 53%

Mitochondrial Myopathy, Encephalopathy, Lactic acidosis and Stroke-Like Episodes Syndrome Presenting With Anton-Babinski Syndrome and Concurrent Occipital Lobe Seizures

Ewida¹,

Ahmed²,

Luo³

et al. 2021

Cureus

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Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a complex group of disorders with multisystem involvement that have a wide range of biochemical and genetic defects. The earliest symptoms of MELAS typically include easy fatigability, muscle weakness, encephalopathy with stroke-like episodes, recurrent headaches and seizures. The pathogenesis of stroke-like episodes manifesting as focal deficits like acute cortical blindness is not fully understood. We present an eight-yearold, right-handed boy with MELAS confirmed by the presence of pathogenic missense variant mutation (mt.3243A>G) presenting with acute intermittent reversible episodes of cortical blindness and Anton-Babinski Syndrome secondary to concurrent occipital lobe seizures captured during video electroencephalography (V-EEG) monitoring, in addition to the neuro-imaging which was not consistent with acute ischemic stroke. This case highlights the importance of the V-EEG monitoring besides clinical testing and radiographic correlation during acute cortical blindness episodes in MELAS as occipital lobe seizures could be a part of the symptomatology.

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Section: Discussionsupporting

confidence: 53%

Mitochondrial Myopathy, Encephalopathy, Lactic acidosis and Stroke-Like Episodes Syndrome Presenting With Anton-Babinski Syndrome and Concurrent Occipital Lobe Seizures

Ewida¹,

Ahmed²,

Luo³

et al. 2021

Cureus

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“…When complicated by a stroke‐like episode, focal epileptiform discharges appear in the region of the lesion. Except for the unusual location (typically posterior brain regions), there is nothing to distinguish it from other causes of a focal epileptic discharge 34 . Patients with the myoclonic epilepsy with ragged red fibres syndrome typically show progressive slowing of background rhythms with symmetrical posterior polyspike and wave complexes often related to myoclonic jerks.…”

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confidence: 99%

Mitochondrial diseases in childhood: a clinical approach to investigation and management

Kisler

Whittaker

McFarland

2010

Develop Med Child Neuro

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ATPAdenosine triphosphate COX Cytochrome c oxidase MELAS Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes mtDNA Mitochondrial DNA nDNA Nuclear DNA POLG1 Polymerase gamma gene SNHI Sensorineural hearing impairmentMitochondrial diseases are a common cause of inherited neurological disorders in children. Although dysfunction of the central nervous system is prominent, multisystem involvement also occurs. Diagnosis relies on characteristic clinical features, an understanding of mitochondrial genetics, and a logical, informed approach to investigations. There is a significant body of recent literature on advances in mitochondrial genetics and the investigation of mitochondrial diseases. However, to our knowledge there remains a paucity of published information on the management of these disorders. Management of the complex constellation of neurological and multisystem clinical features is challenging, and is reliant on a multidisciplinary approach. The care of the child and family is dependent on clear communication between health professionals from primary, secondary, and tertiary care as well as specialist input from quaternary services. The aim of this review is to provide paediatric neurologists, paediatricians, and allied health professionals with a structured approach to the diagnosis and management of children with suspected or confirmed mitochondrial disease.

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“…[2] Both focal and generalized seizures can occur in patients with MELAS; however, focal seizures are the predominant type, especially when patients had SLEs. [1,13,14] Based on the current recommendations for AED selection for epilepsy, [15,16] LEV and CBZ are the most commonly prescribed for patients with MELAS. However, concerns have been raised due to the mitochondrion-toxicity of some AEDs.…”

Section: Discussionmentioning

confidence: 99%

Levetiracetam administration is correlated with lower mortality in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes

Zhang

Zhao

et al. 2019

Chinese Medical Journal

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Background: Studies on the relationship between antiepileptic drug (AED) administration and clinical outcomes in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) remain scarce. Levetiracetam (LEV) is an AED that is neuroprotective in various neurologic disorders. This study aimed to determine the impact of LEV on the outcome of MELAS. Methods: A retrospective, single-center study was performed based on a large cohort of patients with MELAS with a history of seizures ( n = 102). Decisions on antiepileptic therapies were made empirically. Patients were followed up for 1 to 8 years (median, 4 years) and divided into 2 groups based on whether LEV was administered (LEV or non-LEV). The modified Rankin scale (mRS) scores and mortality risks were analyzed in all patients. Results: LEV, carbamazepine, benzodiazepines, topiramate, oxcarbazepine, valproate, and lamotrigine were administered in 48, 37, 18, 13, 11, 9, and 9 patients, singly or in combination, respectively. The mean mRS score of the LEV group ( n = 48) was lower than that of the non-LEV group ( n = 54; mean ± standard deviation, 2.79 ± 1.47 vs . 3.83 ± 1.93, P = 0.006) up to the end of the study. Nevertheless, there was no difference in the proportion of subjects without disability (mRS ranging 0–1) between the groups ( P = 0.37). The multivariate regressions revealed that LEV treatment was associated with lower mRS scores (odds ratio 0.32, 95% confidence interval [CI] 0.15–0.68, P = 0.003) and mortality rates (hazard ratio 0.24, 95% CI 0.08–0.74, P = 0.013). There was a significant difference in the Kaplan-Meier survival curves between the groups (χ 2 = 4.29, P = 0.04). Conclusions: The LEV administration is associated with lower mortality in patients with MELAS in this retrospective study. Further laboratory research and prospective cohort studies are needed to confirm whether LEV has neuroprotective effects on patients with mitochondrial diseases.

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Serial electroencephalographic findings in patients with MELAS

Cited by 17 publications

References 15 publications

Mitochondrial Myopathy, Encephalopathy, Lactic acidosis and Stroke-Like Episodes Syndrome Presenting With Anton-Babinski Syndrome and Concurrent Occipital Lobe Seizures

Mitochondrial Myopathy, Encephalopathy, Lactic acidosis and Stroke-Like Episodes Syndrome Presenting With Anton-Babinski Syndrome and Concurrent Occipital Lobe Seizures

Mitochondrial diseases in childhood: a clinical approach to investigation and management

Levetiracetam administration is correlated with lower mortality in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes

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