Teaching NeuroImages: Longitudinally extensive transverse myelitis in MELAS

Teaching NeuroImages: Longitudinally extensive transverse myelitis in MELAS

Author Souza, Paulo Victor Sgobbi de Autor UNIFESP Google Scholar
Pinto, Wladimir Bocca Vieira de Rezende Autor UNIFESP Google Scholar
Oliveira, Acary Souza Bulle Autor UNIFESP Google Scholar
Abstract A 16-year-old girl presented with acute-onset flaccid quadriparesis with urinary incontinence. Medical history unveiled generalized epilepsy since age 7 years and status epilepticus 3 years before. Multisystem compromise was absent. Family history was unremarkable. Two days after admission, generalized refractory status epilepticus occurred. Neuroimaging studies disclosed laminar cortical necrosis and longitudinally extensive transverse myelitis (LETM) in cervical and thoracic spinal cord levels (figure). Muscle biopsy disclosed subsarcolemmal mitochondrial proliferation and ragged-red fibers (figure). Genetic testing confirmed m.A3243G mutation in the MTTL1 gene, diagnostic of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).(1) LETM can be seen extremely rarely in MELAS.(2</SUP)
xmlui.dri2xhtml.METS-1.0.item-coverage Philadelphia
Language English
Date 2016
Published in Neurology. Philadelphia, v. 86, n. 4, p. E37-E37, 2016.
ISSN 0028-3878 (Sherpa/Romeo, impact factor)
Publisher Lippincott Williams & Wilkins
Extent E37-E37
Origin https://doi.org/10.1212/WNL.0000000000002313
Access rights Open access Open Access
Type Editorial
Web of Science ID WOS:000369264100003
URI https://repositorio.unifesp.br/handle/11600/58674

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