HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Incomplete development of the brain in a newborn with methylmalonic aciduria.

Abstract
The investigation of the brain from a seven-day-old girl who died from a disorder in the metabolism of methylmalonic acid revealed a severe reactive gliosis of the cerebral white matter and the deeper layers of the cortex, incomplete development of the fetal granular layer of the cerebellum and the Bergmann glial cells, and delayed myelination of the cerebellum, the brainstem, and the cervical spinal cord. We suggest that some of the described neuropathological findings are an immediate consequence of a disturbed methylmalonic acid metabolism starting in utero, and not exclusively a secondary phenomenon due to accumulation of metabolites and acidosis postpartum.
AuthorsJ R Ostergaard, E Reske-Nielsen, E Nathan, K Rasmussen
JournalClinical neuropathology (Clin Neuropathol) 1991 Mar-Apr Vol. 10 Issue 2 Pg. 85-90 ISSN: 0722-5091 [Print] Germany
PMID2054982 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Glial Fibrillary Acidic Protein
  • Methylmalonic Acid
Topics
  • Astrocytes (chemistry)
  • Brain (abnormalities)
  • Brain Chemistry
  • Cerebellum (abnormalities, chemistry)
  • Female
  • Glial Fibrillary Acidic Protein (analysis)
  • Humans
  • Infant, Newborn
  • Metabolism, Inborn Errors (pathology)
  • Methylmalonic Acid (urine)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: