Annals of Indian Academy of Neurology
IMAGES IN NEUROLOGY
Year
: 2007  |  Volume : 10  |  Issue : 3  |  Page : 189--190

"Batwings dilatation" of sylvian fissures in glutaric aciduria type I


VV Ashraf1, K Sudha2,  
1 Department of Neurology, Malabar Institute of Medical Sciences, Govindapuram PO, Calicut - 673 016, Kerala, India
2 Department of Pediatrics, Malabar Institute of Medical Sciences, Govindapuram PO, Calicut - 673 016, Kerala, India

Correspondence Address:
V V Ashraf
Department of Neurology, Malabar Institute of Medical Sciences, Govindapuram PO, Calicut - 673 016, Kerala
India




How to cite this article:
Ashraf V V, Sudha K. "Batwings dilatation" of sylvian fissures in glutaric aciduria type I.Ann Indian Acad Neurol 2007;10:189-190


How to cite this URL:
Ashraf V V, Sudha K. "Batwings dilatation" of sylvian fissures in glutaric aciduria type I. Ann Indian Acad Neurol [serial online] 2007 [cited 2021 Jan 15 ];10:189-190
Available from: https://www.annalsofian.org/text.asp?2007/10/3/189/34803


Full Text

A previously healthy 11-month-old girl presented with fever, diarrhea, seizures and thereafter lost milestones. The pregnancy and delivery as well as growth and development had been normal until the present illness. On admission, the baby was afebrile, irritable, hypotonic with extreme weakness of the neck muscles. She had macrocephaly.

CT scan of the brain demonstrated, marked dilatation of the sylvian fissures, in the form of batwings and marked regression of the temporal lobes and widening of CSF spaces around the frontal lobes. [Figure 1] MR imaging of the brain, apart from the above findings, showed T2 hyperintensities in both the lentiform nuclei and the diffuse white matter changes. [Figure 2] Arterial blood gas analysis was normal. Blood and urine qualitative examination for abnormal metabolites showed significant organic acids in both. A quantitave organic academia workup by HPLC showed marked urinary excretion of glutaric acid, diagnostic of glutaric aciduria type I.

 Discussion



The presentation of Glutaric academia type I is variable and often confusing. Typically the disease presents in infancy, mimicking acute encephalitis as in our patient. The most striking finding on brain imaging in glutaric academia type I is the presence of very wide CSF spaces anterior to the temporal lobes and within the sylvian fissures. [1] This "Batwings dilatation" of sylvian fissure was first described as a characteristic finding in glutaric academia in 1987. [2] The above radiological findings if combined with basal ganglia lesions is almost pathognomonic of glutaric academia type I. [3] The finding of "Batwings dialatation" of sylvian fissures and wide CSF spaces anterior to temporal lobes in a child with suspected encephalitis, should alert the clinician of the possibility of Glutaric academia type I.

References

1Mandel H, Braun J, el-Peleg O, Christesen E, Berant M. Glutaric aciduria type I: Brain CT features and diagnostic pitfall. Neuroradiology 1991;33:75-8.
2Amir N, El-Peleg O, Shalev RS, Christensen E. Glutaric aciduria type I: Clinical heterogeneity and neuroradiological features. Neurology 1987;37:1654-7.
3Brismar J, Ozand TP. CT and MR of the brain in glutaric acidemia type I. Am J Neuroradiol 1995;16:675-83.