Huntington's disease and its association with psychopathology

Abstract

We review several aspects of Huntington's disease (HD), with a special focus on the psychopathological manifestations often identified in patients with this disorder. We discuss the evidence for a higher-than-average prevalence of psychosis, depression, and obsessive-compulsive disorder (OCD) in individuals with HD or at risk for the illness and analyze the possible significance of these findings. Particular emphasis is placed on OCD, in view of the neuroanatomical impairment that this condition shares with HD, the symptomatic similarities between these disorders, and recent findings of an excess of OCD in HD-affected families. We hypothesize that precise characterization of the psychiatric status of some HD patients showing psychopathological manifestations and their families might help to distinguish different clinical subtypes of the disorder. This approach could hold promise in improving the management of HD in the future.

Fluoxetine in the treatment of Huntington's disease

“…It can be argued that some clinical markers, such as the presence of OCD in this instance, might possibly help pinpoint distinct subgroups of HD patients, each with its specific neurotransmitting and neuroanatomical patterns of involvement (De Marchi and Mennella 2000). This is in agreement with recent evidence that HD is not as homogeneous as previously believed (see Nance 1996 for a review).…”

The neuropsychology of obsessive compulsive disorder: the importance of failures in cognitive and behavioural inhibition as candidate endophenotypic markers

“…Though this hyperactivation may represent a relatively disease-specific finding, abnormalities in structures within the lateral orbitofrontal loop have been reported in other psychiatric disorders with notably different symptomatology-including mood disorders, other anxiety disorders, and basal ganglia disorders. There is a growing body of evidence that obsessive-compulsive symptoms are found with unexpectedly high frequency in Tourette's syndrome (Como, 1995), Huntington's disease (De Marchi and Mennella, 2000), and perhaps also Parkinson's disease (see Alegret et al (2001) and Maia et al (2003)). Cortico-subcortical neural loops are not completely functionally segregated, and overlapping neurobiology may account for this overlap in symptoms.…”

An overview of psychiatric symptoms in Huntington’s disease

“…However, Watt and Seller [73] found 12% prevalence in a sample of 65 HD patients. De Marchi and Mennella [74] suggest this lower prevalence found in newer work is due to more stringent definitions of schizophrenia in newest versions of the DSM-IV, especially because psychosis as part of a mood disorder is now less likely to be classified as schizophrenia. Also, some older samples of HD patients may have shown overrepresentation of those with schizophrenia, because the populations studied were in psychiatric hospitals.…”