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Post‐ictal psychosis in adolescent Niemann‐Pick disease type C
Author(s) -
Walterfang Mark,
Kornberg Andrew,
Adams Sophia,
Fietz Michael,
Velakoulis Dennis
Publication year - 2010
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-009-9021-x
Subject(s) - psychosis , ictal , context (archaeology) , psychiatry , medicine , pediatrics , population , age of onset , niemann–pick disease, type c , psychology , disease , epilepsy , biology , paleontology , environmental health
We describe the presentation of an adolescent with juvenile‐onset Niemann‐Pick disease type C (NPC) who presented with post‐ictal psychosis in the context of a developing seizure disorder. After demonstrating mild gait disturbance beginning at the age of 4 years, he was diagnosed with NPC at age 12 on the basis of 95% of cultured fibroblasts staining positive for filipin and a reduced fibroblast cholesterol esterification rate. He then developed a seizure disorder at age 15, where clusters of seizures produced typical psychotic symptoms, including hallucinations and delusions. His seizure disorder responded to valproate, which resulted in a settling of his psychotic symptoms. Whilst post‐ictal psychosis is rarely reported prior to the age of 16, NPC in adolescents and adults is particularly psychotogenic and may increase the risk for post‐ictal psychosis in the pediatric population.