Psychomotor Retardation, Brain Calcifications and West’s Syndrome in an Infant

Introduction: West’s syndrome is a severe epileptic syndrome. It may be secondary to brain damage or of unknown aetiology. We report one case of West’s syndrome in an infant and underlined the difficulties in aetiological research with poor technical platform.

Observation: A 2-year-4-month-old infant was admitted in neurology on September 7, 2012 for spasms seizures and chewing for one year. At 1 year of age, the infant had had several episodes of convulsions in the context of fever. He had no particular neonatal history, he was born at term. He is the second child of a sibling of two children. At the physical examination there was no staturo-ponderal delay but a psychomotor delay. Standing and walking were impossible. There was axial hypotonia. Oral language was mutic with a few poorly articulated syllables. Eye movement and swallowing were normal. Sphincter control did not exist. Brain CT scan showed multiple calcifications involving the gray nuclei and brain atrophy. Anti-epileptic treatment with vigabatrin and corticosteroid therapy in addition to functional rehabilitation did not improve the outcome.

Discussion: West syndrome has several aetiologies. Psychomotor retardation is always present in this syndrome. It is described as the infantile spasms that occur in infants under 1 year of age. There are two major groups in this syndrome, namely the structural forms, which are the consequences of severe antenatal or perinatal brain damage, and the forms of unknown aetiology. The structural forms are the most pejorative; they are marked by psychomotor development delay and the existence of a brain lesion that precedes the onset of the disease. The electroencephalographic (EEG) displayed a disorganization recording with irregular slow waves. This pattern is described as hypsarhythmic.

Conclusion: The management of West syndrome is difficult. The prognosis remains severe especially in the structural form.

Keywords: West’s syndrome; Cerebral calcifications; Hypsarhythmia