Neuropediatrics 2006; 37 - THP61
DOI: 10.1055/s-2006-945884

ETIOLOGICAL AND NEUROIMAGISTIC ASPECTS IN INFANTILE SPASMS

F Buruiana 1, D Plesca 1, R Teleanu 1, AM Davitoiu 1, M Moiceanu 1, L Cretu 1, M Stefanescu 1, I Stancea 1, D Dragomir 1
  • 1“Dr. Victor Gomoiu” Children's Hospital, Bucharest, Romania

Objectives: Establishing the etiology and the correlation between etiological factors and neuroimagistic aspects.

Material and Methods: A total of 77 infants and small children were diagnosed with infantile spasms on the basis of the three clinical and electrical criteria.

Results: From a total of 77 cases, 10 (12%) had idiopathic IS, the remaining 67 cases (88%) beeing symptomatic. Among the cases with symptomatic IS, a great majority was associated with tuberous sclerosis (15 cases-22%), followed by the IS secondary to perinatal hypoxic-ischemic encephalopathy (14 cases-20%); 9 cases (13%) had anomalies of neuronal migration, of which 6 cases with lisencephaly and 3 cases with Aicardi syndrome. Other congenital CNS malformations were found in 3 cases. IS secondary to an acute CNS infection (bacterial meningoencephalitis) were found in 3 cases (4%). Two patients had chromosomal anomalies (Down syndrome and 4p15.1 deletion) and other two had phenotipic features suggestive of chromosomal anomalies. In 25 cases (37%) no etiological factor could be established. The analysis of the CT/RMN images revealed: periventricular calcifications (15 cases), as well as other lesions suggesting tuberous sclerosis; anomalies of neuronal migration were found in 9 patients; patients with sequellae after PHIE had various lesions: porencephalic cyst, cortico-subcortical atrophies with or without cerebral calcifications, multicystic encephalomalacy; This type of lesion was also found in patients with CNS infections and as a sequellae after a fronto-temporal hematoma. The most often encountered neuroimagistic aspect was diffuse cortico-subcortical atrophy.

Conclusion: There is a great variety of etiological factors which are responsible for IS. The most frequent etiological factors found in the studied patients were cerebral malformations (including especially tuberous sclerosis) and perinatal hypoxic-ischemic encephalopathy.