Leigh disease (subacute necrotizing encephalomyelopathy): report of one case.

T. S. Wu, C. C. Lee, J. T. Lin, H. H. Hsu, S. T. Lin, Y. J. Jong, E. Y. Shen

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A case is reported of a 16-month-old girl who presented with generalized hypotonia, ptosis and persistent low grade fever after a previous pneumonia. Brain CT and MRI showed symmetric necrotizing lesions in the basal ganglia, substantia nigra and periaqueduct area. Lactate and pyruvate levels were elevated in both the blood and cerebrospinal fluid. Biopsy of the rectus femoris muscle for electron microscopic examination revealed some distortion of the mitochondrial cristae. Biochemical study showed normal respiratory chain enzymes. Leigh disease was considered from the neuroradiological findings and morphological investigations.

Original languageEnglish
Pages (from-to)301-307
Number of pages7
JournalActa Paediatrica Sinica
Volume34
Issue number4
Publication statusPublished - Jul 1993

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Leigh Disease
Muscle Hypotonia
Quadriceps Muscle
Substantia Nigra
Electron Transport
Basal Ganglia
Pyruvic Acid
Cerebrospinal Fluid
Lactic Acid
Pneumonia
Fever
Electrons
Biopsy
Muscles
Brain
Enzymes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Wu, T. S., Lee, C. C., Lin, J. T., Hsu, H. H., Lin, S. T., Jong, Y. J., & Shen, E. Y. (1993). Leigh disease (subacute necrotizing encephalomyelopathy): report of one case. Acta Paediatrica Sinica, 34(4), 301-307.

Leigh disease (subacute necrotizing encephalomyelopathy) : report of one case. / Wu, T. S.; Lee, C. C.; Lin, J. T.; Hsu, H. H.; Lin, S. T.; Jong, Y. J.; Shen, E. Y.

In: Acta Paediatrica Sinica, Vol. 34, No. 4, 07.1993, p. 301-307.

Research output: Contribution to journalArticle

Wu, TS, Lee, CC, Lin, JT, Hsu, HH, Lin, ST, Jong, YJ & Shen, EY 1993, 'Leigh disease (subacute necrotizing encephalomyelopathy): report of one case.', Acta Paediatrica Sinica, vol. 34, no. 4, pp. 301-307.
Wu TS, Lee CC, Lin JT, Hsu HH, Lin ST, Jong YJ et al. Leigh disease (subacute necrotizing encephalomyelopathy): report of one case. Acta Paediatrica Sinica. 1993 Jul;34(4):301-307.
Wu, T. S. ; Lee, C. C. ; Lin, J. T. ; Hsu, H. H. ; Lin, S. T. ; Jong, Y. J. ; Shen, E. Y. / Leigh disease (subacute necrotizing encephalomyelopathy) : report of one case. In: Acta Paediatrica Sinica. 1993 ; Vol. 34, No. 4. pp. 301-307.
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