Abstract
The combination of axial spasms in clusters, hypsarrhythmia, and psychomotor delay
beginning in the first year of life defines West syndrome. Variants of this classical
triad comprise variations of age of onset ranging from the first month to 4 years,
spasms that may be asymmetrical or combined with focal seizures, asymmetrical, synchronous
or fragmented hypsarrhythmia, and psychomotor function which may be delayed, deteriorated
or normal. These variations mainly seem to depend on etiology, and specific patterns
have been identified for the various causes. Most causes relate to non-progressive
uni- or multifocal cortical lesions, although some are due to inborn errors of metabolism.
Ten to 20% exhibit no evidence of brain lesion and are considered idiopathic. This
condition is intermediary between epilepsy in which the disorder is limited to paroxysmal
events during which time the patient returns to his prior condition, and status epilepticus
in which the paroxysmal activity is not interrupted. Here, there are both paroxysmal
events and a continuous non-convulsive paroxysmal activity that contributes to the
deterioration. In the present understanding of pathophysiology, spasms seem to involve
subcortical structures, whereas hyparrhythmia affects cortical areas, also causing
psychomotor deterioration. Deafferentation of subcortical structures by the continuous
spiking and slow wave activity could account for release of autonomic activity in
the basal ganglia. Cortical paroxysmal activity could be caused by age-related hyperexcitability
linked to the development of cortical neuronal networks throughout infancy. The mode
of action of steroid and vigabatrin therapies, the two therapies with demonstrated
efficacy, can be explained on this basis.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- The descriptive epidemiology of infantile spasms among Atlanta children.Epilepsia. 1999; 40: 748-751
- Epidemiologic features of infantile spasms in Iceland.Epilepsia. 1994; 35: 802-805
- A retrospective study of spontaneous remission and long-term outcome in patients with infantile spasms.Epilepsia. 1991; 32: 212-214
- Enregistrement ambulatoire de l'EEG pendant 24 heures dans les spasmes infantiles épileptiques.Rev EEG Neurophysiol Clin. 1987; 17: 309-318
- Ictal clinical electroencephalographic findings of spasms in West syndrome.Epilepsia. 1993; 34: 671-678
- Atlas of electroencephalography. Epilepsy. Addison-Wesley, Cambridge, MA1952 (p. 2)
- Sleep characteristics in infantile spasms.Neurology (Cleveland). 1981; 31: 688-694
- Evidence of late onset infantile spasms.Epilepsia. 1998; 39: 32-39
- L'encéphalopathie myoclonique infantile avec hypsarythmie (syndrome de West). Masson, Paris1964
- A prospective study of infantile spasms: clinical and therapeutic correlations.Epilepsia. 1983; 24: 135-158
- Prognosis for seizure control in infantile spasms preceded by other seizures.Brain Dev. 1990; 12: 306-309
- The origin of infantile spasms: evidence from a case of hydranencephaly.Dev Med Child Neurol. 1972; 14: 644-656
- Traitement des spasmes infantiles par vigabatrin en première intention et monothérapie: à propos de 70 nourrissons.Arch Pediatr. 1998; 5: 731-738
- Mental and behavioural outcome of infantile epilepsy treated by vigabatrin in tuberous sclerosis patients.Epilepsy Res. 2000; 38: 151-160
- Autistic regression in relation to limbic pathology and epilepsy: report of two cases.Dev Med Child Neurol. 1993; 35: 166-176
- Infantile spasms: III. Prognostic implications of bitemporal hypometabolism on positron emission tomography.Ann Neurol. 1996; 39: 643-649
- Neuropsychological aspects of tuberous sclerosis: relation to epilepsy and MRI findings.Dev Med Child Neurol. 1991; 33: 698-705
- Quantification of diffuse and focal delta activity in hypsarrhythmia.Brain Dev. 1990; 12: 310-315
- Agenesis of the corpus callosum, infantile spasms, ocular anomalies (Aicardi's syndrome). Clinical and pathological findings.Neurology. 1976; 26: 1152-1158
- Infantile spasms: a review of the literature and a study of 112 cases. Heinemann Medical, London1964
- Reappraisal of interictal electroencephalograms in infantile spasms.Epilepsia. 1993; 34: 679-685
- Infantile spasms.in: Vinken P.J Bruyn G.W Handbook of clinical neurology. North-Holland, Amsterdam1974: 219-234
- Long-term outcome of West syndrome: a study of adults with a history of infantile spasms.Epilepsia. 1996; 37: 367-372
- Treatment of infantile spasms: results of a population-based study with vigabatrin as the first drug for spasms.Epilepsia. 1999; 40: 950-957
- Predicting favorable outcome in idiopathic West syndrome.Epilepsia. 1993; 34: 747-756
- The idiopathic form of West syndrome.Epilepsia. 1993; 34: 743-746
- Localization of focal cortical lesions influences age of onset of infantile spasms.Epilepsia. 1996; 37: 1068-1071
Lortie A, Plouin P, Chiron C, Delalande O, Dulac O. Focal cortical dysplasia in infancy. In preparation.
- Electroencephalographic aspects of hemimegalencephaly.Dev Med Child Neurol. 1989; 31: 377-383
- L'épilepsie dans l'agyrie–pachygyrie classique.Rev EEG Neurophysiol Clin. 1983; 13: 232-239
- Infantile spasms and Menkes disease.Epil Disord. 2000; 2: 227-230
- A propos de 21 cas d'hypsarythmie de Gibbs. Son traitement spectaculaire par l'ACTH.Acta Neurol Belg. 1958; 58: 130-141
- Infantile spasms: hypothesis-driven therapy and pilot human infant experiments using corticotropin-releasing hormone receptor antagonists.Dev Neurosci. 1999; 21: 281-289
- A simple, effective and well-tolerated treatment regimen for West syndrome.Dev Med Child Neurol. 1994; 36: 863-872
- Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis.Epilepsy Res. 1997; 26: 389-395
- Randomised, placebo-controlled study of vigabatrin as first-line treatment of infantile spasms.Epilepsia. 1999; 40: 1627-1633
- Genetic predisposition to West syndrome.Epilepsia. 1993; 34: 732-737
- Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment.Ann Neurol. 1990; 27: 406-413
- Infantile spasms and West syndrome. Saunders, London1994
- The development of the corpus callosum in cats: a light- and electron-microscopic study.J Comp Neurol. 1988; 276: 132-156
- The effects of epileptic cortical activity on the development of callosal projections.Dev Brain Res. 1994; 77: 251-255
- Positron emission tomography of human brain functional development.Ann Neurol. 1987; 22: 487-497
- Changes in regional cerebral blood flow during brain maturation in children and adolescents.J Nucl Med. 1992; 33: 696-703
- The right brain hemisphere is dominant in human infants.Brain. 1997; 120: 1057-1065
- CA3 driven hippocampal–entorhinal loop controls rather than sustains in vitro limbic seizures.J Neurosci. 1997; 17: 9308-9314
- Magnetic resonance of myelin, myelination, and myelin disorders. Springer, Berlin1989
- Immunocytochemical characterization of long-term persistent immune activation in human brain after herpes simplex encephalitis.Neuropathol Appl Neurobiol. 2000; 26: 285-294
- Brain functional imaging SPECT in agyria–pachygyria.Epilepsy Res. 1996; 24: 109-117
- Etude neuropsychologique prospective des spasmes infantiles. Journées Parisiennes de Pédiatrie. Flammarion, Paris1989
Article info
Publication history
Accepted:
August 28,
2001
Received:
August 17,
2001
Identification
Copyright
© 2001 Published by Elsevier Inc.
