Abstract
A 10-month-old girl with mild developmental delay became hemiplegic after seizures. Cranial CT scan and magnetic resonance imaging (MRI) revealed no lesions related to vascular diseases, but brain atrophy on the right side was remarkable. Digital subtraction angiography showed slightly decreased visualization of peripheral branches of the right medial cerebral artery. Propionic acidaemia was diagnosed on the basis of high plasma levels of propionic acid and its metabolites and the elevated urinary excretion of these acids. With therapy, the levels of these acids fell, and her left hemiplegia disappeared 3 months later.
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Abbreviations
- AIH:
-
acute infantile hemiplegia
- PPA:
-
propionic acidaemia
- PA:
-
propionic acid
- DSA:
-
digital subtraction angiography
- IMP:
-
N-isopropyl-p-[123I] iodoamphetamine
- SPECT:
-
single-photon emission computed tomography
- MRI:
-
magnetic resonance imaging
References
Cheema-Dhadli S, Leznoff CC, Halperin ML (1975) Effect of 2-methylmalonate on citrate metabolism: implications for the management of patients with propionic acidemia and methylmalonic aciduria. Pediatr Res 9:905–908
Dave P, Curless RG, Steinman L (1984) Cerebellar hemorrhage complicating methylmalonic and propionic acidemia. Arch Neurol 41:1293–1296
Gebarski SS, Gabrielson TO, Knake JE, Latack JT (1983) Cerebral CT findings in methylmalonic and propionic acidemias. AJNR 4:955–957
Heidenreich R, Natowicz M, Hainline BE, Berman P, Kelley RI, Hillman RE, Berry GT (1988) Acute extrapyramidal syndrome in methylmalonic acidemia: “Metabolic stroke” involving the globus pallidus. J Pediatr 113:1022–1027
Menkes JH (ed) (1985) Textbook of child neurology. Lea & Febiger, Philadelphia, pp 592–595
Shigematsu Y, Sudo M, Momoi T, Inoue Y, Suzuki Y, Kameyama J (1982) Changing plasma and urinary organic acid levels in a patient with isovaleric acidemia during an attack. Pediatr Res 16:771–775
Steinman L, Clancy RR, Cann H, Urich H (1983) The neuropathology of propionic acidemia. Dev Med Child Neurol 25:87–94
Wolf B, Hsia YE, Sweetman L, Gravel R, Harris DJ, Nyhan WL (1981) Propionic acidemia: a clinical update. J Pediatr 99: 835–846
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Shigematsu, Y., Mori, I., Nakai, A. et al. Acute infantile hemiplegia in a patient with propionic acidaemia. Eur J Pediatr 149, 659–660 (1990). https://doi.org/10.1007/BF02034758
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DOI: https://doi.org/10.1007/BF02034758