title: Continuous giggling and autistic disorder associated with hypothalamic hamartoma.
abstract: We present the case of a child affected since early infancy from recurring episodes of giggling mixed with stereotypical behaviours, mingled with head drops, and eventually with falls, in the context of an autistic disorder. Scalp video-EEG recordings revealed an epileptic encephalopathy with generalized slow spike-and-wave complexes alternating with electrodecremental periods, which generally corresponded to the onset of the aforementioned clinical sequences. A resection of a hypothalamic hamartoma was achieved at the age of two. Since then, after 22 months of follow-up, the child is totally free from the giggling and the drops, and the autistic behaviour significantly improved during the second year of follow-up. This case illustrates the difficulties to recognize some subtle ictal manifestations during infancy and childhood, and encourages the early surgical treatment of hypothalamic hamartomas when associated with epileptic encephalopathy and when technically possible.
authors.affiliation: Clinical Neurophysiology section, Niño Jesús University Hospital, Madrid, Spain. mperez.hnjs@salud.madrid.org
authors.forename: Angeles
authors.initials: A
authors.lastname: Pérez-Jiménez
authors.forename: Fransisco J
authors.initials: FJ
authors.lastname: Villarejo
authors.forename: María Concepción
authors.initials: MC
authors.lastname: Fournier del Castillo
authors.forename: Juan José
authors.initials: JJ
authors.lastname: García-Peñas
authors.forename: Mar
authors.initials: M
authors.lastname: Carreño
mesh_terms: D001321:Autistic Disorder / Q000150:complications / Q000523:psychology*; D001519:Behavior / Q000502:physiology*; D004569:Electroencephalography; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000523:psychology* / Q000601:surgery; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000523:psychology* / Q000601:surgery; D007223:Infant; D019635:Neurosurgical Procedures; D011569:Psychiatric Status Rating Scales; D013239:Stereotyped Behavior