Skip to main content
Log in

Subdural hemorrhage as an initial sign of glutaric aciduria type 1: A diagnostic pitfall

  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

The case of a 9-month-old girl with glutaric aciduria type 1 (GA 1) is reported. On initial presentation at 6 months of age, the patient demonstrated bilateral subdural hemorrhages and widening of the basal cisterns. After neurosurgical intervention the subdural effusions regressed; their etiology remained unclear. At the age of 9 months the patient presented again because of progressive loss of psychomotor abilities and a dystonic movement disorder. Cerebral MRI revealed regressive subdural hematoma, but marked frontotemporal atrophy as well. Because of a suspected metabolic disorder, urinary analysis of organic acids was performed. This repeatedly showed marked excretion of glutaric acid, 3-hydroxyglutaric acid and glutaconic acid, indicating a diagnosis of GA 1. Considering our patient's history, we recommend the inclusion of GA 1 in the differential diagnosis of patients with unexplained subdural hematoma and neurological deficits.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Goodman SI, Markey SP, Moe PG, Miles BS, Teng CC (1975) Glutaric aciduria: a “new” disorder of amino acid metabolism. Biochem Med 12:12–21

    PubMed  Google Scholar 

  2. Amir N, El-Peleg O, Shalev R, Christensen E (1987) Glutaric aciduria type 1: clinical heterogeneity and neuroradiologic features. Neurology 37: 1654–1657

    PubMed  Google Scholar 

  3. Haworth JC, Booth FA, Chudley AE, DeGroot GW, Dilling LA, Goodman SI, Greenberg CR, Mallory CJ, Mc Clarty BM, Seshia SS, Seargeant LE (1991) Phenotypic variabilities in glutaric aciduria type 1: report of fourteen cases in five Canadian kindreds. J Pediatr 118: 52–58

    PubMed  Google Scholar 

  4. Christensen E (1983) Improved assay of glutaryl-CoA dehydrogenase in cultured cells and liver: application to glutaric aciduria type I. Clin Chim Acta 129: 91–97

    PubMed  Google Scholar 

  5. Aicardi J, Goutieres F, Saudabray JM, Ogier H (1985) CT scans of infants with glutaric aciduria. Dev Med Child Neurol 27:401–406

    PubMed  Google Scholar 

  6. Dunger DB, Snodgrass GJA (1984) Glutaric aciduria type 1 presenting with hypoglycemia. J Inherit Metab Dis 7: 122–124

    PubMed  Google Scholar 

  7. Iafolla AK, Kahler SG (1989) Megalencephaly in the neonatal period as the initial manifestation of glutaric aciduria type 1. J Pediatr 114: 1004–1006

    PubMed  Google Scholar 

  8. Kimura S, Hara M, Nezu A, Osaka H, Yamazaki S, Saitoh K (1994) Two cases of glutaric aciduria type 1: clinical and neuropathological findings. J Neurol Sci 123: 38–43

    PubMed  Google Scholar 

  9. Mandel H, Braun J, El-Peleg O, Christensen E, Berant M (1991) Glutaric aciduria type 1. Brain CT features and a diagnostic pitfall. Neuroradiol 33: 75–78

    Google Scholar 

  10. Yager J, McClarty BM, Seshia SS (1985) CT scan findings in an infant with glutaric aciduria type 1. Dev Med Child Neurol 30: 808–820

    Google Scholar 

  11. Osaka H, Kimaura S, Nezu A, Yamazaki S, Saitoh K, Yamaguchi S (1993) Chronic subdural hematoma, as an initial manifestation of glutaric aciduria type-1. Brain Dev 15: 125–127

    PubMed  Google Scholar 

  12. Drigo P, Burlina AB, Battistella A (1993) Subdural hematoma and glutaric aciduria type 1. Brain Dev 15: 460–461

    PubMed  Google Scholar 

  13. Friede RL (1989) Developmental neuropathology, 2nd edn. Springer, New York Berlin Heidelberg, pp 198–208

    Google Scholar 

  14. Phillips JY (1955) Cerebral cortical damage incident to chronic subdural hematoma in infancy. Bull Los Ang Neurol Soc 20: 30–36

    Google Scholar 

  15. Shimoji T, Satoh K, Ishii S (1992) A pathogenesis of chronic subdural hematoma to the subdural membrane. Neurol Surg 20: 131–136

    Google Scholar 

  16. Herzberger E, Rotem Y, Braham J (1956) Remarks on thirty-three cases of subdural effusion in infancy. Arch Dis Child 31: 44–50

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Woelfle, J., Kreft, B., Emons, D. et al. Subdural hemorrhage as an initial sign of glutaric aciduria type 1: A diagnostic pitfall. Pediatr Radiol 26, 779–781 (1996). https://doi.org/10.1007/BF01396200

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01396200

Keywords

Navigation