Post-ictal psychosis in adolescent Niemann-Pick disease type C.
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.
Walterfang
Mark
M
Neuropsychiatry Unit, Royal Melbourne Hospital, Melbourne, VIC, Australia. mark.walterfang@mh.org.au
Kornberg
Andrew
A
Adams
Sophia
S
Fietz
Michael
M
Velakoulis
Dennis
D
D000293:Adolescent; D000367:Age Factors; D000927:Anticonvulsants / Q000627:therapeutic use; D002478:Cells, Cultured; D002788:Cholesterol Esters / Q000378:metabolism; D004951:Esterification; D005347:Fibroblasts / Q000378:metabolism; D005372:Filipin / Q000378:metabolism; D020233:Gait Disorders, Neurologic / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D052556:Niemann-Pick Disease, Type C / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D011618:Psychotic Disorders / Q000175:diagnosis / Q000209:etiology*; D012640:Seizures / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D016896:Treatment Outcome; D014635:Valproic Acid / Q000627:therapeutic use
D000927:Anticonvulsants; D002788:Cholesterol Esters; D014635:Valproic Acid; D005372:Filipin