Adult-onset of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome presenting as acute meningoencephalitis: A Case Report

Journal of Emergency Medicine

Volumn 43, Issue 3, 2012, Pages

  Hsu, Yu Chuan ^a   Yang, Fu Chi ^a   Perng, Cherng Lih ^a   Tso, An Chen ^a   Wong, Lee Jun C ^b   Hsu, Chang Hung ^a  

^a TRI SERVICE GENERAL HOSPITAL   (Taiwan)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

Author keywords

epilepsy; MELAS; meningoencephalitis; mitochondrial disease; valproic acid

Indexed keywords

BRAIN PROTEIN; CEFTRIAXONE; GLUCOSE; LACTIC ACID; MITOCHONDRIAL DNA; PHENYTOIN; PYRUVIC ACID; RIBOFLAVIN; UBIDECARENONE; VALPROIC ACID; VANCOMYCIN;

ADULT; AGITATION; ANTIBIOTIC THERAPY; ARTICLE; AUDIOGRAPHY; CASE REPORT; CEREBROSPINAL FLUID ANALYSIS; DISEASE SEVERITY; DRUG SUBSTITUTION; DRUG WITHDRAWAL; ELECTROENCEPHALOGRAPHY; EMERGENCY WARD; ERYTHROCYTE COUNT; FEVER; GENE MUTATION; GLUCOSE BRAIN LEVEL; GRAND MAL SEIZURE; HEADACHE; HEMISPHERE; HUMAN; LEUKOCYTE COUNT; LUMBAR PUNCTURE; MALE; MEDICAL HISTORY; MELAS SYNDROME; MENINGOENCEPHALITIS; NUCLEAR MAGNETIC RESONANCE IMAGING; NUCLEOTIDE SEQUENCE; PATIENT MONITORING; PERCEPTION DEAFNESS; PHYSICAL EXAMINATION; PRIORITY JOURNAL; PROTEIN CEREBROSPINAL FLUID LEVEL; SIDE EFFECT; SPEECH DISORDER; TRANSTHORACIC ECHOCARDIOGRAPHY; VERBAL COMMUNICATION;

ANTICONVULSANTS; DIAGNOSIS, DIFFERENTIAL; DNA, MITOCHONDRIAL; HUMANS; MALE; MELAS SYNDROME; MENINGOENCEPHALITIS; MIDDLE AGED; POINT MUTATION; POLYMERASE CHAIN REACTION; POLYMORPHISM, RESTRICTION FRAGMENT LENGTH; VALPROIC ACID;

EID: 84866081361     PISSN: 07364679     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jemermed.2009.10.021     Document Type: Article

References (25)

1. S.G. Pavlakis, P.C. Phillips, S. DiMauro, D.C. De Vivo, and L.P. Rowland Mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes: a distinctive clinical syndrome Ann Neurol 16 1984 484 488
2. P.F. Chinnery, N. Howell, R.N. Lightowlers, and D.M. Turnbull Molecular pathology of MELAS and MERRF The relationship between mutation load and clinical phenotypes Brain 120 1997 1713 1721 (Pubitemid 27443048)
3. S. Dimauro, and G. Davidzon Mitochondrial DNA and disease Ann Med 37 2005 222 232 (Pubitemid 40780440)
4. M. Leão Valproate as a cause of hyperammonemia in heterozygotes with ornithine-transcarbamylase deficiency Neurology 45 1995 593 594
5. C.M. Lin, and P. Thajeb Valproic acid aggravates epilepsy due to MELAS in a patient with an A3243G mutation of mitochondrial DNA Metab Brain Dis 22 2007 105 109 (Pubitemid 46354826)
6. M. Schwartz, and J. Vissing Paternal inheritance of mitochondrial disease N Engl J Med 347 2002 576 580
7. P.F. Chinnery, and D.M. Turnbull Epidemiology and treatment of mitochondrial disorders Am J Med Genet 106 2001 94 101
8. M. Hirano, and S.G. Pavlakis Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS): current concepts J Child Neurol 9 1994 4 13 (Pubitemid 24022131)
9. D.M. Sproule, and P. Kaufmann Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome Ann N Y Acad Sci 1142 2008 133 158
10. D.C. Wallace Mitochondrial diseases in man and mouse Science 283 1999 1482 1488
11. Y. Tamagawa, K. Kitamura, and H. Hagiwara Audiologic findings in patients with a point mutation at nucleotide 3,243 of mitochondrial DNA Ann Otol Rhinol Laryngol 106 1997 338 342 (Pubitemid 27175025)
12. K. Auré, C. Jardel, and A. Lombès Mitochondrial diseases: molecular mechanisms, clinical presentations and diagnosis investigations Ann Pathol 25 2005 270 281 (Pubitemid 41665408)
13. W.S. Kunz The role of mitochondria in epileptogenesis Curr Opin Neurol 15 2002 179 184 (Pubitemid 34286903)
14. A.A. Morris Mitochondrial respiratory chain disorders and the liver Liver 19 1999 357 368 (Pubitemid 29457212)
15. C.W. Bazil, and T.A. Pedley Clinical pharmacology of antiepileptic drugs Clin Neuropharmacol 26 2003 38 52 (Pubitemid 36184114)
16. M.J. Brodie, and J.A. French Management of epilepsy in adolescents and adults Lancet 356 2000 323 329 (Pubitemid 30459597)
17. L. Rumbach, J.M. Warter, A. Rendon, C. Marescaux, G. Micheletti, and A. Waksman Inhibition of oxidative phosphorylation in hepatic and cerebral mitochondria of sodium valproate-treated rats J Neurol Sci 61 1983 417 423 (Pubitemid 14234269)
18. S. Ponchaut, F. Van Hoof, and K. Veitch Cytochrome aa3 depletion is the cause of the deficient mitochondrial respiration induced by chronic valproate administration Biochem Pharmacol 43 1992 644 647
19. Y. Goto, S. Horai, and T. Matsuoka Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation Neurology 42 1992 545 550
20. C.W. Lam, C.H. Lau, J.C. Williams, Y.W. Chan, and L.J. Wong Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy Eur J Pediatr 156 1997 562 564 (Pubitemid 27289858)
21. S. Ponchaut, F. Van Hoof, and K. Veitch Valproate and cytochrome c oxidase deficiency Eur J Pediatr 154 1995 79
22. M. de Toledo, J. Díaz-Guzmán, D.A. Pérez- Martínez, R.A. Sáiz-Díaz, A. Rodríguez-Vallejo, and Y. Campos MELAS syndrome masquerading as herpes encephalitis: genetic diagnosis Rev Neurol 33 2001 148 150 (Pubitemid 32885050)
23. D.R. Johns, A.G. Stein, and R. Wityk MELAS syndrome masquerading as herpes simplex encephalitis Neurology 43 1993 2471 2473 (Pubitemid 24004529)
24. A. Ogata, and K. Mitsumori MELAS presenting herpes simplex encephalitis (Japanese) Nippon Rinsho 60 Suppl 4 2002 629 631
25. S.R. Sharfstein, M.F. Gordon, R.B. Libman, and E.S. Malkin Adult-onset MELAS presenting as herpes encephalitis Arch Neurol 56 1999 241 243 (Pubitemid 29076190)