title: Neurodegenerative disorder masquerading as psychosis in a forensic psychiatry setting.
abstract: A man presenting in his 50s, following conviction for a non-violent crime, to forensic psychiatric services, and then to a neuropsychiatry service with an unusual presentation of psychosis: second person auditory hallucinations, grandiose delusions and somatic delusions. Detailed collateral and family history revealed a background of progressive cognitive deficit and a family history of motor neuron disease. MRI of the brain revealed asymmetrical parieto-occipital volume loss and genetic testing demonstrated a pathogenic expansion of the chromosome 9 open reading frame 72 (C9ORF72) gene consistent with familial frontotemporal dementia caused by a hexanucleotide repeat expansion at C9ORF72, a recently discovered cause of familial frontotemporal dementia/motor neuron disease. This form of frontotemporal dementia should be considered as an important potential differential diagnosis for patients presenting with psychotic symptoms in later life, in whom a detailed family history and thorough cognitive assessment is essential.
authors.affiliation: Division of Psychiatry, University College London, London, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Sommerlad
authors.affiliation: Camden and Islington NHS Foundation Trust, London, UK.
authors.forename: James
authors.initials: J
authors.lastname: Lee
authors.affiliation: Dementia Research Centre, University College London, London, UK.
authors.forename: Jason
authors.initials: J
authors.lastname: Warren
authors.affiliation: Department of Neuropsychiatry, University College London Hospitals, London, UK.
authors.forename: Gary
authors.initials: G
authors.lastname: Price
mesh_terms: D000073885:C9orf72 Protein; D003415:Crime; D003702:Delusions / Q000209:etiology; D003937:Diagnosis, Differential; D005555:Forensic Psychiatry; D057180:Frontotemporal Dementia / Q000175:diagnosis / Q000235:genetics / Q000523:psychology*; D006212:Hallucinations / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D011506:Proteins / Q000235:genetics; D011618:Psychotic Disorders / Q000175:diagnosis / Q000209:etiology*
chemical_list: D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; D011506:Proteins