S. Tuinier

Brain correlates of memory dysfunction in alcoholic Korsakoff's syndrome

OBJECTIVES To investigate the relation between anterograde amnesia and atrophy of brain structures involved in memory processing in alcoholic Korsakoffs syndrome. METHODS The volume of brain structures involved in memory processing was measured with MRI from 13 subjects with Korsakoffs syndrome, 13 subjects with chronic alcoholism without Korsakoffs syndrome, and 13 control subjects. The brain structures analysed were the hippocampus, the parahippocampal gyrus, the mamillary bodies, the third ventricle, and the thalamus. Brain volumes were correlated with the delayed recall of a verbal learning test. RESULTS Compared with subjects with chronic alcoholism and control subjects, subjects with Korsakoffs syndrome had a reduced volume of the hippocampus, the mamillary bodies, and the thalamus, and enlargement of the third ventricle. The impairment of delayed recall correlated with the volume of the third ventricle (r=−0.55, p=0.05) in the Korsakoff group. CONCLUSIONS Anterograde amnesia in alcoholic Korsakoffs syndrome is associated with atrophy of the nuclei in the midline of the thalamus, but not with atrophy of the mamillary bodies, the hippocampus, or the parahippocampal gyrus.

The psychopathological phenotype of Velo-cardio-facial syndrome

Velo-cardio-facial syndrome (VCFS) is mostly associated with deletions of chromosome 22q11, and is thought to be characterized by an increased frequency of major psychiatric disorders. Sixteen patients adults with VCFS and psychiatric symptoms were evaluated using a semi-structured investigation of history, symptoms, signs and behaviour. All available data were used in consensus meetings to obtain a classifiable diagnostic category. In contrast to other reports, no categorical diagnosis could be established. Instead, a quite specific psychological, behavioural and psychopathological constellation emerged that should most adequately be denominated as a VCFS-psychiatric syndrome. It is concluded that VCFS is associated with a specific psychopathological syndrome.

Citalopram in mentally retarded patients with depression: A long-term clinical investigation

The effect of citalopram was investigated in 20 mentally retarded patients suffering from a depressive disorder characterized by alterations in the domains of affectivity, motivation, motor activity and vital signs. The study followed a baseline-controlled open design. Citalopram was started in a daily dosage of 20 mg that was kept unchanged for 6 weeks. Thereafter dosage was adjusted to maximally 60 mg per day. Treatment effects were assessed according to the Clinical Global Improvement Scale (CGIS) after at least 6 months. In 12 of the 20 patients a moderate to marked improvement in all domains was observed upon treatment with 20-40 mg citalopram daily. Treatment for one year in the effective dose prevented recurrence of depressive symptomatology. Concomitant use of sedative anticonvulsants reduced responsiveness to treatment. No interactions were observed. It is concluded that citalopram is a well-tolerated, safe and effective antidepressant in mentally retarded subjects with depressive disorders.

Prader–Willi syndrome: cycloid psychosis in a genetic subtype?

Prader–Willi syndrome (PWS) is a genetically determined disorder associated with the loss of the paternal contribution to the proximal part of the long arm of chromosome 15. Its pathophysiology is dominated by hypothalamic dysfunctions. The psychopathological phenotype comprises affective and psychotic symptoms as well as an increase of pre-existent obsessive-compulsive behaviors. The present study comprises 19 PWS patients who were referred for neuropsychiatric evaluation because of psychotic deterioration. Patients were assessed by using the elements of semistructured symptom checklists. In the majority a genetic analysis was performed to detect the underlying chromosomal defect. In 16 of the 19 patients a diagnosis of cycloid psychosis could be established. The other three showed a bipolar affective disorder. Of the psychotic patients, 11 were diagnosed as UPD and one as del 15q11–13. The remaining four patients were diagnosed clinically. For various reasons the genetic etiology could not be established. In PWS patients with a psychotic disorder (cycloid psychosis) a disproportional number of UPD is found.

Integratie van opleiding en onderzoek in de opleiding tot psychiater.

In the training program for residents in psychiatry, scientific education and participation in research projects are relatively underdeveloped. This may result in a gap between recently acquired scientific knowledge and its implementation in clinical practice. Since the start of the residency training in psychiatry in 1989 in the Vincent van Gogh Institute for Psychiatry, several research lines have been developed especially concerning dimensional diagnostic procedures, evaluation of treatment effects, stress and psychopathology, pharmacokinetics of psychotropic compounds and disordered impulse and aggression regulation. It is concluded that active participation in research projects by both psychiatrists and residents in psychiatry is increasingly mandatory due to social-economic en scientific developments. Despite several countervailing factors, the Vincent van Gogh Institute for Psychiatry has succeeded in the creation of a scientific orientation in the residency program resulting in a number of publications.

Psychopathologie en Pathogenese van enkele behavioural phenotypes.

Description of psychopathological symptoms in mentally retarded subjects is of great heuristic value because of the association with a known genetic disorder as well as the heterogenous and atypical phenomenology. The concept of behavioural phenotypes postulates a specific relationship between gene defect and behaviour, although considerable overlap between syndromes and differences within syndromes concerning behavioural manifestations are frequently observed. Since syndrome specific psychiatric disorders can be established too, the term psychopathological phenotype seems to be appropriate.