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Case No. 402
2016-03-01~2016-03-31
출제자 : 이경미
Hit : 561
| Period | 2016-03-01~2016-03-31 |
|---|---|
| Diagnosis | MELAS |
| Clinical information | 25yr/Male C.C> 2달전부터 두통, 말이 어눌하고 잘 알아 듣지 못한다 Fig 5,6은 2달전 FLAIR영상임. |
| Discussion | -Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) -Inherited disorder by mutation in mitochondrial DNA (mtDNA) -Clinical profile Classic triad: Lactic acidosis, seizures, stroke-like episodes Sensorineural hearing loss, muscle weakness, acute onset headache Age : old childhood/early adulthood (mean onset :15 yrs) -Natural History Recurrent stroke-like events with either permanent or reversible neurologic deficits -Treatment Cofactor and supplement therapy Acute: Stroke-like cortical lesions "Shifting spread" (appearance, disappearance, reappearance elsewhere) Lesions cross typical vascular territories Location Stroke-like: Parietooccipital > temporoparietal Calcifications: Basal ganglia (BG) Size: Variable, progressive, multifocal CT NECT: Symmetric BG calcification CECT: Variable gyral enhancement MRI Acute T2WI & FLAIR : hyperintense cortex/subcortical WM Cortical abnormalities cross vascular territories Parietal and occipital lobes (M/C) Gyral enhancement on T1 C+ MRA – no vascular occlusion Chronic Multifocal lacunar-type infarcts, Symmetric BG calcification, WM volume loss, Progressive atrophy of parietooccipital cortex. MRS: Lac "doublet" at 1.3 ppm(60-65%) |
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Correct Answer
Semi-Correct Answer |