Interictal Schizophrenia-like Psychosis in a Patient With Double Cortex Syndrome
Abstract
CASE REPORT
History of epilepsy and psychosis. The patient is a right-handed 39-year-old Japanese woman. Her family members (parents and two brothers) had normal cognitive functions and no neuropsychiatric disorders and have adapted themselves to the community well. She entered an ordinary elementary school, although her early developmental milestones were somewhat delayed.At age 10, she developed complex partial seizures (CPS), characterized by aura, loss of consciousness with eye deviation to the left, occasional brief tonic movement of both arms, and subsequent generalized tonic-clonic seizures (GTC). She suffered epileptic seizures 3 to 4 times per month. Since her first seizure, she has shown a progressive mental decline, which necessitated her transfer to a special class at age 12. Since graduating from a special high school for mentally handicapped persons, she has commuted to a workshop for the disabled.At age 25, she developed delusions of persecution and reference without any preceding explanatory psychosocial events. She had visual hallucinations of ghosts and monsters and probable auditory hallucinations. She exhibited some unusual mannerisms (walking around talking to herself and staring extendedly at photos of a popular singer) and obsessive behaviors (flushing the toilet every 15 minutes). During these episodes, her consciousness was clear and her orientation was intact. Administration of an antipsychotic drug (levomepromazine, 150 mg per day) improved her mental state within 2 months. She had three more psychotic episodes with similar symptoms, and levomepromazine was found to be effective again. Since her first psychosis occurred, the frequency of seizures has decreased to 1 to 2 times per year for the past 15 years. Her personality has not altered throughout her clinical course except during psychotic episodes. She has taken 600 mg of carbamazepine per day (serum level; 9.7 μg/ml).Investigations. EEG showed well-developed 9-Hz to 11-Hz occipital rhythms and bilateral epileptiform discharges with predominance over the left frontotemporal region. During the four psychotic episodes, EEG has not normalized. Brain MRI revealed bilateral bands of heterotopic gray matter in the centrum semiovale, separated from cortical gray matter by layers of white matter (Figure 1). Diffuse atrophy of the cerebral hemispheres was also observed, although the gyral and convolutional patterns of the overlying cortex were normal. The limbic structures were unremarkable. Interictal brain SPECT images with [99mTc]HMPAO have been reported elsewhere.7 Briefly, they revealed identical or increased perfusion of the subcortical laminar heterotopia, compared with that of the overlying cortex, and hypoperfusion of the left temporal lobe. Neurological examinations were unremarkable. Tanaka-Binet IQ Scale score was 38 at age 35 years.Chromosome aberration. The patient and her mother underwent chromosomal and genetic studies after giving written informed consent. Cytogenetic examination using GTG banding revealed that the patient had a pericentric inversion of chromosome 9, a karyotype of 46XXinv(9)(p12q13). Her mother also had this chromosomal abnormality.Mutation analysis. Genomic DNA was extracted from white blood cells. To scan regions of DCX covering the branch site of each intron and the 5′-upstream sequences of exon 1A, we determined the following sequences by using GenomeWalker Kits (Clontech, Palo Alto, CA, USA): 325 bp upstream of exon 1A and 146 bp of intron 1B 3′-portion (DDBJ/EMBL/GenBank Accession Nos.: AJ005591–AJ005597). Polymerase chain reactions (PCRs) were performed by using a Gene Amp 9700 thermal cycler (PE Applied Biosystems, Chiba, Japan) under the conditions shown in Table 1. PCR products were sequenced directly in both directions with a BigDye terminator kit and an ABI 377 DNA sequencer (PE Applied Biosystems). Mutations in the gene DCX were not detected in this patient or her mother.
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