An etiologic evaluation of 86 patients with infantile spasms is presented and the place of cranial magnetic resonance imaging (MRI) findings within this spectrum is discussed. A total of 103 cranial MRIs, performed between 4 and 72 months of age, were analyzed and classified according to the etiologic and pathophysiologic aspects. Ninety-one percent of cases were diagnosed as symptomatic infantile spasms, and hypoxic-ischemic encephalopathy was the primary cause (30%). The most common involvement was thinning of the corpus callosum in 43 patients (50%), followed by dilation of cerebral ventricles in 32 (36%), delayed myelination in 23 (26.7%), lesions of diencephalic deep gray matter in 17 (19.7%), and enlargement of the subarachnoid space in 12 (14%). Thin corpus callosum and diffuse atrophy were changes mainly associated with hypoxic-ischemic encephalopathy, whereas delayed myelination seemed to be independent from a specific etiology. The results showed that cranial MRI may provide considerable information regarding not only the etiology but also the pathophysiology of infantile spasms. (J Child Neurol 2003;18:241—246).
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References
1.
Commission on Pediatric Epilepsy of the International League Against Epilepsy: Workshop on infantile spasms. Epilepsia1992;33: 195.
2.
Langenstein I., Willig RP, Kuehn D.: Cranial computed tomography (CCT) findings in children with ACTH and dexamethasone: First results. Neuropediatrics1979; 10:370-384.
3.
Muroi J., Okunuo T., Kuno C., et al: An MRI study of the myelination pattern in West syndrome. Brain Dev1996;18:179—184.
4.
Barkovich AJ , Kjos BO, Jackson DE, Norman D.: Normal maturation of the neonatal and infant brain: MR imaging at 1.5T. Radiology1988;166:173—180.
5.
Barkovich AJ: Pediatric Neuroimaging: Normal Development of the Neonatal and Infant Brain, Skull, and Spine, 2nd ed. New York, Raven Press, 1995;9—54.
6.
Okumura A., Watanabe K., Negoro T., et al: Evolutional changes and outcome of West syndrome: Correlation with magnetic resonance imaging findings. Epilepsia1998;39:46—49.
7.
Ohtahara S., Ohtsuda Y., Yamatogi Y., et al: Prenatal etiologies of West syndrome. Epilepsia1993;34:716—722.
8.
Aicardi J., Sabril IS: Investigator and peer review groups: Vigabatrin as initial therapy for infantile spasms. Epilepsia1996;37:638—642.
9.
Aydinli N., Çalişkan M., Özmen M., Tonguç E.Neuroradiologic aspects of West syndrome. Pediatr Neurol1998; 19: 211—216.
10.
Glaze DG, Hrachovy RA, Frost JD, et al: Prospective study of outcome of infants with infantile spasms treated during control studies of ACTH and prednisone. J Pediatr1988;112:389—396.
11.
Cusmai R., Ricci S., Pinard JM, et al: West syndrome due to perinatal insults. Epilepsia1993;34:738—742.
12.
Ozawa H., Hashimoto T., Endo T., et al: West syndrome with periventricular leukomalacia: A morphometric MRI study. Pediatr Neurol1998;19:358—363.
13.
Rutherford MA, Pennock JM, Schiwieso JE, et al: Hypoxic ischaemic encephalopathy: Early magnetic resonance imaging findings and their evolution. Neuropediatrics1995;26:183—191.
14.
Johnston MV , Hoon AH: Possible mechanisms in infants for selective basal ganglia damage from asphyxia, kernicterus, or mitochondrial encephalopathies . J Child Neurol2000;15:588—591.
15.
Riikonen R. : Infantile spasms: Infectious disorders. Neuropediatrics1993;24:274—280.
16.
Riikonen R. : Cytomegalovirus infection and infantile spasms . Dev Med Child Neurol1978;20:570—579.
17.
Riikonen R. , Donner M.: Incidence and aetiology of infantile spasms from 1960 to 1976: A population study in Finland. Dev Med Child Neurol1979;21:333—343.
18.
Steinlin MI , Nadal D., Eich GF, et al: Late intrauterine cytomegalovirus infection: Clinical and neuroimaging findings. Pediatr Neurol1996;15:249—253.
19.
Boagert P., Chiron C., Adamsbaum C., et al: Value of magnetic resonance imaging in West syndrome of unknown etiology. Epilepsia1993;34:701—706.
20.
Lombroso CT : A prospective study of infantile spasms: Clinical and therapeutic correlations. Epilepsia1983 ;24:135—158.
21.
Chiron C., Dulac O., Beaumont D., et al: Therapeutic trial of vigabatrin in refractory infantile spasms . J Child Neurol1991;6:52—59.
22.
Matsumato A. , Watanabe K., Negoro T., et al: Infantile spasms: Etiologic factors, clinical aspects and long term prognosis in 200 cases. Eur J Pediatr1981;135:239—244.
23.
Watanabe K. , Negoro T., Aso K., Matsumoto A.: Reappraisal of interictal electroencephalograms in infantile spasms. Epilepsia1993;34: 679—685.
24.
Riikonen R. : Infantile spasms: Some new theoretical aspects . Epilepsia1983;24:159—168.
25.
Yalcinkaya C., Dincer A., Gunduz E., et al: MRI and MRS in HMG-CoA lyase deficiency . Pediatr Neurol1999; 20: 375—380.
26.
Curatolo P., Pelliccia A., Cotroneo E.: Prognostic significance of CT findings in West syndrome, in Akimoto H (eds): Advances in Epileptology: XIII Epilepsy International Symposium. New York, Raven Press, 1982, 191—194.
27.
Singer WD, Haller JS, Sullivan LR, et al: The value of neuroradiology in infantile spasms. J Pediatr1982;100:47—50.
28.
Sheth RD, Schaefer GB, Keller GM, et al: Size of corpus callosum in cerebral palsy. J Neuroimaging1996;6:180—183.
29.
Natsume J., Watanabe K., Maeda N., et al: Cortical hypometabolism and delayed myelination in West syndrome . Epilepsia1996;37: 1180—1184.
30.
Schropp C., Staudt M., Staudt F., et al: Delayed myelination in children with West syndrome: MRI study . Neuropediatrics1994;25: 116—120.
31.
Watanabe K. : West syndome: Etiological and prognostic aspects . Brain Dev1998;20:1—8.
32.
Avanzini G., Sancini G., Canafoglia L., Franceschetti S.: Maturation of cortical physiological properties relevant to epileptogenesis , in Spreafico R, Avanzini G, Andermann F (eds): Abnormal Cortical Development and Epilepsy. London, John Libbey and Company, 1999,63-75.
33.
Connors BW, Gutnick MJ: Intrinsic firing patterns of diverse neo-cortical neurons. Trends Neurosci1990;13:99—104.
34.
Chiron C., Dulac O., Bulteau C., et al: Study of regional cerebral blood flow in West syndrome. Epilepsia1993;34:707—715.
35.
Valk J., Knaap MS: Magnetic Resonance of Myelin, Myelination and Myelin Disorders. Berlin, Springer Verlag, 1989.
36.
Dulac O.: Infantile spasms: A pathophysiological hypothesis, in Nehlig A, Motte J, Moshé SL, Plouin P (eds): Childhood Epilepsies and Brain Development. London, John Libbey & Company, 1999,93-102.
37.
Chugani HT, Shields WD, Shewmon DA, et al: Infantile spasms I. PET identifies focal cortical dispenses in cryptogenic cases for surgical treatment. Ann Neurol1990;27:406—413.
