Symmetrical necrosis of the basal ganglia in methylmalonic acidaemia
| Autor: | Jean-Jacques Martin, A. M. Roodhooft, W. Blom, E. R. Baumgartner, K. J. Van Acker |
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| Rok vydání: | 1990 |
| Předmět: |
Male
Pathology medicine.medical_specialty Necrosis Brain damage Globus Pallidus Central Nervous System Diseases Basal ganglia medicine Humans In patient Methylmalonic acidaemia Child Acidosis medicine.diagnostic_test business.industry Magnetic resonance imaging Fibroblasts Combined Modality Therapy Magnetic Resonance Imaging Malonates Globus pallidus nervous system Child Preschool Pediatrics Perinatology and Child Health medicine.symptom business Tomography X-Ray Computed Metabolism Inborn Errors Methylmalonic Acid |
| Zdroj: | European journal of pediatrics. 149(8) |
| ISSN: | 0340-6199 |
| Popis: | In a patient with methylmalonic acidaemia (MMAA), persistent neurological symptoms were observed in addition to the acute episodes of metabolic dysequilibrium. CT scan and magnetic resonance imaging revealed bilateral symmetrical necrosis of the globus pallidus. Different episodes of metabolic decompensation, one with severe acidosis, had occurred. Persistent neurological symptoms in patients with MMAA who are appropriately treated suggest irreversible brain damage which appears to occur preferentially at the level of the basal ganglia. |
| Databáze: | OpenAIRE |
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