Magnetic Resonance Imaging in MELAS Syndrome

Overview

Journal Neuroradiology

Specialties Neurology
Radiology

Date 1990 Jan 1

PMID 2398945

Citations 9

Authors

L Rosen

S Phillips

D Enzmann

Affiliations

Soon will be listed here.

Abstract

MELAS syndrome is a distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Computed tomography (CT) and magnetic resonance (MR) findings are reviewed in a patient with MELAS. Serial CT studies demonstrated multiple "migrating" infarcts in various stages of evolution involving primarily the posterior temporal and occipital regions. MR was more sensitive than CT in demonstrating the number and extent of cortical lesions in this disease entity.

Citing Articles

Adult-onset mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome with progressive sensorineural hearing loss: A case report.

Trang T, Chien P, Dung B, Thu N, Truc N, Khang V Radiol Case Rep. 2021; 16(7):1865-1869.

PMID: 34093932 PMC: 8166901. DOI: 10.1016/j.radcr.2021.04.049.

Migratory stroke-like lesions in a case of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and a review of imaging findings.

Cai S, von Coelln R, Kouo T Radiol Case Rep. 2016; 11(4):425-429.

PMID: 27920873 PMC: 5128397. DOI: 10.1016/j.radcr.2016.09.003.

The appearance of ADCs in the non-affected areas of the patients with MELAS.

Liu Z, Liu X, Hui L, Zhao D, Wang X, Xie S Neuroradiology. 2010; 53(4):227-32.

PMID: 20556599 DOI: 10.1007/s00234-010-0729-y.

MELAS: a new disease associated mitochondrial DNA mutation and evidence for further genetic heterogeneity.

Hanna M, Nelson I, Morgan-Hughes J, Wood N J Neurol Neurosurg Psychiatry. 1998; 65(4):512-7.

PMID: 9771776 PMC: 2170312. DOI: 10.1136/jnnp.65.4.512.

Neuroradiological features of six kindreds with MELAS tRNA(Leu) A2343G point mutation: implications for pathogenesis.

Sue C, Crimmins D, Soo Y, Pamphlett R, Presgrave C, Kotsimbos N J Neurol Neurosurg Psychiatry. 1998; 65(2):233-40.

PMID: 9703178 PMC: 2170193. DOI: 10.1136/jnnp.65.2.233.

References

Kobayashi Y, Miyabayashi S, Takada G, Narisawa K, Tada K, Yamamoto T . Ultrastructural study of the childhood mitochondrial myopathic syndrome associated with lactic acidosis. Eur J Pediatr. 1982; 139(1):25-30. DOI: 10.1007/BF00442074. View

Yamamoto T, Beppu H, Tsubaki T . Mitochondrial encephalomyopathy: fluctuating symptoms and CT. Neurology. 1984; 34(11):1456-60. DOI: 10.1212/wnl.34.11.1456. View

Fukuhara N, Tokiguchi S, Shirakawa K, Tsubaki T . Myoclonus epilepsy associated with ragged-red fibres (mitochondrial abnormalities ): disease entity or a syndrome? Light-and electron-microscopic studies of two cases and review of literature. J Neurol Sci. 1980; 47(1):117-33. DOI: 10.1016/0022-510x(80)90031-3. View

Kearns T, SAYRE G . Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases. AMA Arch Ophthalmol. 1958; 60(2):280-9. View

Riggs J, SCHOCHET Jr S, Fakadej A, Papadimitriou A, DiMauro S, Crosby T . Mitochondrial encephalomyopathy with decreased succinate-cytochrome c reductase activity. Neurology. 1984; 34(1):48-53. DOI: 10.1212/wnl.34.1.48. View

Kuriyama M, Igata A . Mitochondrial encephalopathy, lactic acidosis, and strokelike syndrome (MELAS). Ann Neurol. 1985; 18(5):625-6. DOI: 10.1002/ana.410180525. View

Pavlakis S, Phillips P, DiMauro S, De Vivo D, Rowland L . Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol. 1984; 16(4):481-8. DOI: 10.1002/ana.410160409. View

Hasuo K, Tamura S, Yasumori K, Uchino A, Goda S, Ishimoto S . Computed tomography and angiography in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes); report of 3 cases. Neuroradiology. 1987; 29(4):393-7. DOI: 10.1007/BF00348922. View

Shapira Y, Harel S, Russell A . Mitochondrial encephalomyopathies: a group of neuromuscular disorders with defects in oxidative metabolism. Isr J Med Sci. 1977; 13(2):161-4. View

10.

Land J, Morgan-Hughes J, Clark J . Mitochondrial myopathy. Biochemical studies revealing a deficiency of NADH--cytochrome b reductase activity. J Neurol Sci. 1981; 50(1):1-13. DOI: 10.1016/0022-510x(81)90038-1. View

11.

SCHOTLAND D, DiMauro S, Bonilla E, Scarpa A, Lee C . Neuromuscular disorder associated with a defect in mitochondrial energy supply. Arch Neurol. 1976; 33(7):475-9. DOI: 10.1001/archneur.1976.00500070017003. View

12.

HART Z, Chang C, PERRIN E, Neerunjun J, Ayyar R . Familial poliodystrophy, mitochondrial myopathy, and lactate acidemia. Arch Neurol. 1977; 34(3):180-5. DOI: 10.1001/archneur.1977.00500150066013. View

13.

Driscoll P, Larsen P, Gruber A . MELAS syndrome involving a mother and two children. Arch Neurol. 1987; 44(9):971-3. DOI: 10.1001/archneur.1987.00520210065021. View

14.

Morgan-Hughes J, Hayes D, Clark J, Landon D, Swash M, Stark R . Mitochondrial encephalomyopathies: biochemical studies in two cases revealing defects in the respiratory chain. Brain. 1982; 105 (Pt 3):553-82. DOI: 10.1093/brain/105.3.553. View