We reviewed 10 patients (5 males, 5 females) with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. The age of symptom onset ranged from 3 months to 12 years. All had lactic acidosis, multiple stroke-like events with secondary neurological deficits, radiological changes of
Stroke-like episodes in familial mitochondrial encephalomyopathy: clinical and biochemical aspects
✍ Scribed by M. S. Damian; H. Reichmann; H.-J. Schütz; W. Dorndorf; W. Schachenmayr
- Publisher
- Springer
- Year
- 1991
- Tongue
- English
- Weight
- 1013 KB
- Volume
- 238
- Category
- Article
- ISSN
- 0340-5354
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✦ Synopsis
Acute episodes of focal neurological dysfunction are a well-recognized complication of the mitochondrial encephalomyopathies. Because of rapid remission, biochemical tests and other diagnostic procedures are mostly performed after the acute phase. We report the case of a patient suffering from mitochondrial disease manifesting primarily with seizures, progressive deafness and dementia, who experienced multiple stroke-like episodes. Other members of the family with evidence of mitochondrial dysfunction are presented briefly. EEG and biochemical findings in the acute stage are correlated with clinical symptoms, showing characteristics distinct from the chronic illness. The possible involvement of dietary factors in the provocation of stroke-like episodes is discussed and regulation of glucose intake suggested as a strategy in the prevention of stroke-like episodes.
📜 SIMILAR VOLUMES
## Abstract In this study we present incidence, point prevalence, and mortality figures of mitochondrial encephalomyopathies in a population‐based study of children from western Sweden. Through the screening of registers and review of medical records, we identified 32 patients under 16 years of age