The phenomenology and diagnosis of psychiatric illness in people with Prader–Willi syndrome

2008 ◽  
Vol 38 (10) ◽  
pp. 1505-1514 ◽  
Author(s):  
S. Soni ◽  
J. Whittington ◽  
A. J. Holland ◽  
T. Webb ◽  
E. N. Maina ◽  
...  

BackgroundPsychotic illness is strongly associated with the maternal uniparental disomy (mUPD) genetic subtype of Prader–Willi syndrome (PWS), but not the deletion subtype (delPWS). This study investigates the clinical features of psychiatric illness associated with PWS. We consider possible genetic and other mechanisms that may be responsible for the development of psychotic illness, predominantly in those with mUPD.MethodThe study sample comprised 119 individuals with genetically confirmed PWS, of whom 46 had a history of psychiatric illness. A detailed clinical and family psychiatric history was obtained from these 46 using the PAS-ADD, OPCRIT, Family History and Life Events Questionnaires.ResultsIndividuals with mUPD had a higher rate of psychiatric illness than those with delPWS (22/34 v. 24/85, p<0.001). The profile of psychiatric illness in both genetic subtypes resembled an atypical affective disorder with or without psychotic symptoms. Those with delPWS were more likely to have developed a non-psychotic depressive illness (p=0.005) and those with mUPD a bipolar disorder with psychotic symptoms (p=0.00005). Individuals with delPWS and psychotic illness had an increased family history of affective disorder. This was confined exclusively to their mothers.ConclusionsPsychiatric illness in PWS is predominately affective with atypical features. The prevalence and possibly the severity of illness are greater in those with mUPD. We present a ‘two-hit’ hypothesis, involving imprinted genes on chromosome 15, for the development of affective psychosis in people with PWS, regardless of genetic subtype.

2006 ◽  
Vol 96 (1-2) ◽  
pp. 127-131 ◽  
Author(s):  
Richard Rende ◽  
Boris Birmaher ◽  
David Axelson ◽  
Michael Strober ◽  
Mary Kay Gill ◽  
...  

1988 ◽  
Vol 153 (3) ◽  
pp. 376-381 ◽  
Author(s):  
John L. Waddington ◽  
Hanafy A. Youssef

The demography, psychiatric morbidity, and motor consequences of long-term neuroleptic treatment in the 14 children born to a father with a family history of chronic psychiatric illness and a mother with a late-onset affective disorder resulting in suicide are documented. Twelve siblings lived to adulthood, nine of whom were admitted to a psychiatric hospital in their second or third decade, and required continuous in-patient care; five remaining in hospital, with long-term exposure to neuroleptics, had chroniC., deteriorating, schizophrenic illness and emergence of movement disorder. Two siblings showed no evidence of psychosis but developed a late-onset affective disorder. The implications for the issues of homotypia, vulnerability to involuntary movements, and interaction with affective disorder are discussed.


2003 ◽  
Vol 15 (1) ◽  
pp. 32-37 ◽  
Author(s):  
W. M. A. Verhoeven ◽  
S. Tuinier ◽  
L. M. G. Curfs

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.


1988 ◽  
Vol 152 (6) ◽  
pp. 839-842 ◽  
Author(s):  
Ulrike Schmidt ◽  
David Miller

HIV disease often leads to neuropsychiatric disturbance, either through direct infection of the brain by the virus or through CNS disease secondary to immunodeficiency. Neuropsychiatric complications of AIDS and AIDS-related disorders may present clinically as acute or chronic organic mental syndromes, or may mimic functional psychiatric illness, in particular depression, anxiety, or psychotic states. Two cases of hypomanic states in homosexual men suffering from AIDS are reported. Neither of the two men had a personal or family history of affective disorder. In one man, hypomanic symptoms were caused by early HIV encephalopathy; he rapidly developed typical HIV dementia with a marked downhill course. In the second case, a clear connection between the hypomanic symptoms and direct HIV brain involvement was not established.


The Lancet ◽  
2002 ◽  
Vol 359 (9301) ◽  
pp. 135-136 ◽  
Author(s):  
Harm Boer ◽  
Anthony Holland ◽  
Joyce Whittington ◽  
Jill Butler ◽  
Tessa Webb ◽  
...  

1991 ◽  
Vol 5 (1) ◽  
pp. 47
Author(s):  
G D Pearlson ◽  
C A Ross ◽  
W D Lohr ◽  
BW Rovner ◽  
G A Chase ◽  
...  

PEDIATRICS ◽  
1979 ◽  
Vol 63 (4) ◽  
pp. 517-519
Author(s):  
Gerald S. Golden

Two children are reported who had recurrent attacks of impairment of time sense, body image, and visual analysis of the environment. These occurred with a clear state of consciousness and in the absence of any evidence of an encephalitic process, seizures, drug ingestion, or psychiatric illness. Both children had recurrent headaches; one was clearly migrainous. There was a family history of migraine in both cases. These children represent examples of the Alice in Wonderland syndrome in juvenile migraine.


1995 ◽  
Vol 8 (1) ◽  
pp. 43-46 ◽  
Author(s):  
R. M. Lawrence ◽  
J. C. Hillam

We describe two cases of Binswanger's disease of pre-senile onset which presented with affective and psychotic symptoms well before the appearance of cognitive deterioration and neurological signs, initially evading an accurate diagnosis. Psychiatrists should be aware of white matter disease and its role in the pathogenesis of psychiatric illness. Particular attention should be given to a history of hypertension as a risk factor in the early identification of these cases.


Author(s):  
Nikole Benders-Hadi

This chapter on postpartum psychosis notes that the risk of postpartum psychosis in the general population is very rare at less than 1%. In a mother with a known history of schizophrenia, this risk increases to 25%. Psychotic symptoms appearing postpartum may also be evidence of a bipolar disorder. The presence of elevated mood, increased activity levels and energy, poor sleep, and a family history of manic episodes all increase the likelihood that a bipolar disorder is present. Women with a personal or family history of a bipolar disorder are at an elevated risk of developing a mania or depression with psychotic symptoms postpartum. Postpartum psychosis due to any cause is a psychiatric emergency and treatment should be initiated early and aggressively to ensure the safety of mother and infant. Hospitalization and/or separation of the baby and mother may be necessary. The use of medication to treat schizophrenia or bipolar disorder during pregnancy may decrease the risk of a postpartum psychosis. With appropriate postpartum medication and support, the majority of women experiencing postpartum psychosis recover well and the risk of recurrent psychotic symptoms can be greatly reduced.


1993 ◽  
Vol 163 (4) ◽  
pp. 492-498 ◽  
Author(s):  
Christian Guth ◽  
Peter Jones ◽  
Robin Murray

Early-onset affective disorder is associated with obstetric complications and a high familial risk of psychiatric illness, in particular psychosis. In a matched case-control study, we investigated 47 adult in-patients with major depressive disorder or bipolar 1 disorder, who had earlier in life presented to a child psychiatry department. Cases were matched on sex, social class and ethnic group with 47 controls, who were admitted to hospital for affective disorders in adult life but had no psychiatric contact before the age of 21. We found that both psychiatric disorder in first-degree relatives and a history of obstetric complications were associated with early onset. Childhood symptoms did not predict the type of adult affective disorder.


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