Stroke-like episodes in mitochondrial encephalomyopathy with lactic acidosis

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Abstract

We described two patients (female, 47 years and male, 42 years) with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). Diagnosis was confirmed by genetic study (A3243G mitochondrial DNA mutation was found), muscle biopsy and elevated lactate level in the blood. Clinical picture of stroke-like episodes was presented by symptoms of mainly involvement of the posterior brain area (fluent and amnestic aphasia, hemianopia, paresis, ataxia), as well as by headache and epileptic seizures. In the first patient stroke-like episodes recurred but their symptoms almost completely regressed with time. The second patient had severe residual neurological deficit. Other clinical manifestations included hearing loss, memory deterioration, cardiomyopathy, fatigue and type 1 diabetes mellitus. MRI in acute period of stroke-like episodes found cortical lesions mainly in the posterior parts of the brain. They completely disappeared in the first case, but persisted in the second patient. The differential diagnosis of stroke-like episodes and ischemic stroke and approaches to treatment are discussed.

 

About the authors

Lyudmila A. Kalashnikova

Research Center of Neurology

Author for correspondence.
Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

Larisa A. Dobrynina

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

Alla V. Sakharova

Research Center of Neurology

Email: kalashnikovancn@yandex.ru
Russian Federation, Moscow

Roxana P. Chaykovskaya

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

M. F. Mir-Kasimov

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

Rodion N. Konovalov

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
ORCID iD: 0000-0001-5539-245X

Cand. Sci. (Med.), senior researcher, Neuroradiology department

Russian Federation, 125367 Moscow, Volokolamskoye shosse, 80

Аlla A. Shabalina

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
ORCID iD: 0000-0001-9604-7775

D. Sci. (Med.), leading researcher, Head, Laboratory of hemorheology, hemostasis and pharmacokinetics (with clinical laboratory diagnostics)

Russian Federation, Moscow

M. M. Kostyreva

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

V. V. Gnezditsky

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

S. V. Protsky

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
Russian Federation, Moscow

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Copyright (c) 2010 Kalashnikova L.A., Dobrynina L.A., Sakharova A.V., Chaykovskaya R.P., Mir-Kasimov M.F., Konovalov R.N., Shabalina A.A., Kostyreva M.M., Gnezditsky V.V., Protsky S.V.

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