HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Watanabe, Y. Articles by Nishimura, T. Search for Related Content PubMed PubMed Citation Articles by Watanabe, Y. Articles by Nishimura, T.

SPECT Findings in Mitochondrial Encephalomyopathy

Department of Radiology, National Toneyama Hospital
Division of Tracer Kinetics and Nuclear Medicine, First Department of Internal Medicine and Department of Pediatrics, Osaka University Medical School, Osaka, Japan

Correspondence: For correspondence or reprints contact: Tsunehiko Nishimura, MD, PhD, Division of Tracer Kinetics, Osaka University Medical School, 2-2 Yamadaoka, Suita Osaka 565, Japan.

ABSTRACT

We investigated the alterations in regional cerebral blood flow (rCBF) in mitochondrial encephalomyopathy (MEM), using [¹²³I]N-isopropyl-p-iodoamphetamine (IMP) or ^99mTc-hexamethyl propyleneamine oxime SPECT in 10 MEM patients. Methods: Four of the patients had MEM with lactic acidosis and strokelike episodes (MELAS), 2 had Kearns-Sayre syndrome (KSS), 1 had myoclonic epilepsy with ragged red fibers (MERRF) and 3 had cytochrome C oxidase deficiency (CCOD). Cerebral perfusion reserve was obtained from 6 patients (3 MELAS, 1 MERRF, 1 KSS, 1 CCOD) for a comparative analysis using the split-dose ¹²³I-IMP SPECT method before and after the injection of acetazolamide. Results: All patients with MELAS showed focal hypoperfusion in the parietal and/or occipital lobes. Follow-up studies (3 MELAS patients) revealed extension or improvement in the abnormal perfusion. The hypoperfused lesions were correlated with abnormal CT/MRI findings. Perfusionwas normal in 1 MERRF, 2 KSS and 3 CCOD patients, whereas CT/MRI findings in 1 MERRF,1 KSS and 1 CCOD patient were abnormal. The cerebral perfusion reserve in 3 MELAS patients was decreased significantly compared with that in patients with other types of MEM (MELAS 7.4%, other MEM 33.8%; p < 0.05). Conclusion: The rCBF was altered specifically in patients with MELAS, suggesting that brain perfusion SPECT will be useful in diagnosing and assessing such patients. The decreased cerebral perfusion reserve in patients with MELASmay representan important feature of the pathogenesis of the strokelike episodes. The SPECT findings of patients with other types of MEM (MERRF, KSS and CCOD) were normal.

Key Words: mitochondrial encephalomyopathy • lactic acidosis • SPECT • cerebral perfusion reserve

This article has been cited by other articles:

				S. Ito, W. Shirai, M. Asahina, and T. Hattori Clinical and Brain MR Imaging Features Focusing on the Brain Stem and Cerebellum in Patients with Myoclonic Epilepsy with Ragged-Red Fibers due to Mitochondrial A8344G Mutation AJNR Am. J. Neuroradiol., February 1, 2008; 29(2): 392 - 395. [Abstract] [Full Text] [PDF]

				T. Iizuka, F. Sakai, N. Suzuki, T. Hata, S. Tsukahara, M. Fukuda, and Y. Takiyama Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome Neurology, September 24, 2002; 59(6): 816 - 824. [Abstract] [Full Text] [PDF]

				D HILL, S WINTERSGILL, L STOTT, B CADGE, and J GRAHAM Cochlear implantation in a profoundly deaf patient with MELAS syndrome J. Neurol. Neurosurg. Psychiatry, August 1, 2001; 71(2): 281 - 281. [Full Text]

				M. J. Molnar, A. Valikovics, S. Molnar, L. Tron, P. Dioszeghy, F. Mechler, and B. Gulyas Cerebral blood flow and glucose metabolism in mitochondrial disorders Neurology, August 22, 2000; 55(4): 544 - 548. [Abstract] [Full Text] [PDF]