title: [Differential diagnoses of West syndrome].
abstract: This study describes the clinical and electroencephalographic characteristics of epileptic spasms, and more especially those that occur during the first two years of life (infantile spasms). West syndrome has been clearly defined as the association between infantile spasms with an electroencephalographic pattern of hypsarrhythmia. Although intellectual deficit appears in almost all cases in which infantile spasms are not controlled with medication, this is a developmental aspect of the condition and not a manifestation that must necessarily be present in order to define the syndrome. The analysis of the interictal and ictal electroencephalogram readings, together with the clinical characteristics of the spasms and the neurological examination of patients, provides some orientation as regards the causations. Despite the spectrum that the title of this work focuses on, the study does not cover the treatment of early infants with West syndrome. Emphasis is placed on the differential diagnoses of West syndrome with other epileptic syndromes that manifest in the first two years of life, and more especially with a series of abnormal non-epileptic motor phenomena that occur in early infants. All these last non-epileptic disorders are displayed in a table, but benign myoclonus of early infancy or Fejerman syndrome is given as a paradigmatic example for the differential diagnosis. The primordial aim is to prevent neurologically healthy early infants from receiving antiepileptic drugs and even adrenocorticotropic hormone or corticoids due to a mistaken diagnosis.
authors.affiliation: Hospital de Pediatria SAMIC. Prof. Dr. J.P. Garrahan, Buenos Aires, Argentina.
authors.forename: Natalio
authors.initials: N
authors.lastname: Fejerman
mesh_terms: D017668:Age of Onset; D001927:Brain Diseases / Q000150:complications; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020820:Dyskinesias / Q000175:diagnosis; D004421:Dystonia / Q000175:diagnosis; D004569:Electroencephalography; D020936:Epilepsy, Benign Neonatal / Q000175:diagnosis; D006801:Humans; D007223:Infant; D009207:Myoclonus / Q000175:diagnosis; D020447:Parasomnias / Q000175:diagnosis; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology