Epileptic spasms without hypsarrhythmia. A new case report and review of the literature
Navas-Sánchez, P.; Martínez-Antón, J.; Bauzano-Poley, E.
Revista de Neurologia 51(10): 592-596
2010
ISSN/ISBN: 0210-0010 PMID: 21069638 Document Number: 640446
The term 'epileptic spasm' must be used to refer to a type of seizure that is typically found in childhood. Clinically, it is expressed as brief axial contractions, in flexion, extension or mixed, which can be symmetrical or asymmetrical and usually appear in clusters. The best-known epileptic syndrome associated with the appearance of grouped spasms is West's syndrome. They may also appear in other generalised epilepsies, epilepsies with periodic spasms or even seizures that are similar to grouped spasms in certain partial epilepsies. A 6-month-old girl with paroxysmal episodes of cluster spasms, for whom no pattern of hypsarrhythmia was observed in the electroencephalogram. The physical examination, neuroimaging and metabolic studies did not offer any pathological findings of interest. The psychomotor development of the patient prior to the onset of the seizures was normal. Following treatment with several different antiepileptic drug regimes, finally control over the seizures was accomplished with a combination of valproic acid and vigabatrine. There are cases like this, with cluster spasms, which fall within the age bracket at which West's syndrome typically occurs and which, nevertheless, do not present the electroencephalographic characteristics of hypsarrhythmia (neither typical nor atypical) or neuropsychological impairment. In these patients it is not clear whether we are dealing with a variant related with West's syndrome or not, since two of the three criteria required for its diagnosis are not fulfilled.