title: Paroxysmal tonic eye deviation: an atypical presentation of hypothalamic hamartoma.
abstract: Hypothalamic hamartoma is a rare developmental non-neoplastic malformation, often characterised by early onset gelastic seizures and later progressive cognitive and behavioural deterioration. In this case study, we have examined a child who presented with an atypical onset of benign paroxysmal gaze deviation between two to three months of age. The patient subsequently developed gelastic seizures at age 13. Based on the observation that hypothalamic hamartomas do not involve any functional region involved in eye motility, we speculate that both gaze deviation and gelastic seizures are a manifestation of the epileptogenic nature of the hypothalamic hamartoma. [Published with video sequences].
authors.affiliation: Department of Child Neurology and Psychiatry, University of Pavia, Pavia, Italy.
authors.forename: Ivana
authors.initials: I
authors.lastname: Olivieri
authors.forename: Federica
authors.initials: F
authors.lastname: Teutonico
authors.forename: Simona
authors.initials: S
authors.lastname: Orcesi
authors.forename: Grazia
authors.initials: G
authors.lastname: Papalia
authors.forename: Carla
authors.initials: C
authors.lastname: Uggetti
authors.forename: Stefano
authors.initials: S
authors.lastname: Bastianello
authors.forename: Umberto
authors.initials: U
authors.lastname: Balottin
authors.forename: Pierangelo
authors.initials: P
authors.lastname: Veggiotti
mesh_terms: D000293:Adolescent; D000375:Aging; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000209:etiology*; D006222:Hamartoma / Q000503:physiopathology*; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000503:physiopathology*; D007223:Infant; D015835:Ocular Motility Disorders / Q000209:etiology; D012640:Seizures / Q000209:etiology