Summary
A case of mitochondrial encephalomyopathy (MELAS) with mental disorder is reported. The SPECT study using123I-iodoamphetamine (IMP) and MRI study revealed abnormality in the left parieto-occipital areas without abnormality in the brain CT or brain scintigram. These findings suggest a localized dysfunction of the brain capillary endothelium in association with the cerebral involvement of mitochondrial encephalomyopathy.
Similar content being viewed by others
References
Bydder GM, Steiner RE, Young IR, Hall AS, Thomas DJ, Marshall J, Pallis CA, Legg NJ (1982) Clinical NMR imaging of the brain: 140 cases. AJR 139:215–236
Goda S, Hamada T, Ishimoto S, Kobayashi T, Goto I, Kuroiwa Y (1987) Clinical improvement after administration of coenzyme Q10 in a patient with mitochondrial encephalomyopathy. J Neurol 234:62–63
Hart ZH, Chang C, Perrin EVD, Neerunjun JS, Ayyar R (1977) Familial poliodystrophy, mitochondrial myopathy, and lactate acidemia. Arch Neurol 34:180–185
Hayes DJ, Hilton-Jones D, Arnold DL, Galloway G, Styles P, Duncan J, Radda GK (1985) A mitochondrial encephalomyopathy. A combined31P magnetic resonance and biochemical investigation. J Neurol Sci 71:105–118
Hill TC, Holman BL, Lovett R, O'Leary DH, Front D, Magistretti P, Zimmerman RE, Moore S, Clouse ME, Wu JL, Lin TH, Baldwin RM (1982) Initial experience with SPECT (singlephoton computerized tomography) of the brain using N-isopropyl I-123 p-idoamphetamine: Concise communication. J Nucl Med 23:191–195
Holliday PL, Climie ARW, Gilroy J, Mahmud MZ (1983) Mitochondrial myopathy and encephalopathy: Three cases —A deficiency of NADH-CoQ dehydrogenase? Neurology 33: 1619–1622
Kobayashi Y, Miyabayashi S, Takada G, Narisawa K, Tada K, Yamamoto TY (1982) Ultrastructural study of the childhood mitochondrial myopathic syndrome associated with lactic acidosis. Eur J Pediatr 139:25–30
Kobayashi M, Morishita H, Sugiyama N, Yokochi K, Nakano M, Wada Y, Hotta Y, Terauchi A, Nonaka I (1987) Two cases of NADH-coenzyme Q reductase deficiency: relationship to MELAS syndrome. J Pediatr 110:223–227
Kuriyama M, Umezaki H, Fukuda Y, Osame M, Koike K, Takeishi J, Igata A (1984) Mitochondrial encephalomyopathy with lactate-pyruvate elevation and brain infarctions. Neurology 34: 72–77
Mukoyama M, Kazui H, Sunohara N, Yoshida M, Nonaka I, Satoyoshi E (1986) Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes with acanthocytosis: a clinicopathological study of a unique case. J Neurol 233:228–232
Ohama E, Ohara S, Ikuta F, Tanaka K, Nishizawa M, Miyatake T (1987) Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy. Acta Neuropathol 74: 226–233
Pavlakis SG, Phillips PC, DiMauro S, De Vivo DC, Rowland LP (1984) Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol 16:481–488
Shapira Y, Harel S, Russell A (1977) Mitochondrial encephalomyopathies. A group fo neuromuscular disorders with differences in oxidative metabolism. Isr J Med Sci 13:161–164
Winchell HS, Horst WD, Braun L, Oldendorf WH, Hattner R, Parker H (1980) N-Isopropyl-(123I)p-Iodoamphetamine: single pass brain uptake and washout; Binding to brain synaptosomes and localization in dog and monkey brain. J Nucl Med 21: 947–952
Yamamoto T, Beppu H, Tsubaki T (1984) Mitochondrial encephalomyopathy: fluctuating symptoms and CT. Neurology 34: 1456–1460
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Suzuki, T., Koizumi, J., Shiraishi, H. et al. Mitochondrial encephalomyopathy (MELAS) with mental disorder. Neuroradiology 32, 74–76 (1990). https://doi.org/10.1007/BF00593949
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00593949