An overview of psychiatric symptoms in Huntington’s disease

Abstract

Huntington's disease (HD) is an inherited autosomal dominant disorder characterized by neurologic, cognitive, and psychiatric symptomatology. Psychiatric symptoms in HD are often amenable to treatment, and relief of these symptoms may provide significant improvement in quality of life. This review will briefly describe neurologic, neuropsychologic and brain imaging data, and then review psychiatric syndromes seen in HD and their treatment.

Neuroprotection for Huntington’s Disease: Ready, Set, Slow

“…7,8 There is no therapy proven to delay onset or slow progression, 9,10 and the best current medical care has a positive impact by focusing multidisciplinary attention on symptom management and caregiver support and by maximizing function and quality of life. [11][12][13][14] Because of the early functional decline, the chronic and increasingly intensive care required, and the profound multigenerational impact on entire families, 15 HD disproportionately consumes medical, social, and family resources. The principal target populations for neuroprotective therapies are those who are premanifest (not yet symptomatic but known to possess a huntingtin gene with the causative CAG expansion) as well as those who are manifest (overtly symptomatic), but not yet so advanced that there is a vastly diminished quality of life to preserve.…”

The Functional Implications of Motor, Cognitive, Psychiatric, and Social Problem-Solving States in Huntington's Disease

“…It results from an aberrant trinucleotide (cytosine-adenine-guanine [CAG]) expansion on chromosome 4 at 4p16.3 that codes for the production of glutamine at the N-terminal of the protein huntingtin (Anderson & Marder, 2001). Although the normal function of huntingtin is unknown, it has been hypothesized that its aggregation causes a toxic gain of cellular function that results in the symptomatic presentation of HD (Anderson & Marder, 2001). …”

“…The striatum-specifically the caudate, putamen, and globus pallidusdegenerates most markedly in HD (Gómez-Tortosa et al, 2001). A decrease of medium spiny -aminobutryic acid (GABA) neurons in the caudate and globus pallidus, as well as diminished striatal D1 and D2 receptor binding, has been observed in HD (Anderson & Marder, 2001). More recently, it has been recognized that the neuronal circuitry that connects areas of the frontal cortex (e.g., the orbitofrontal cortex) and subcortical regions (e.g., the caudate) is affected also (Anderson & Marder, 2001;Marshall et al, 2007;Rosenblatt & Leroi, 2000;Tekin & Cummings, 2002;Thieben et al, 2002;van Duijn, Kingma, & van der Mast, 2007).…”

“…However, few researchers conceive of HD as a psychiatric disorder; it has been suggested that this view of HD has resulted in many late or misdiagnoses, because between 24% and 79% of the patients manifest psychiatric signs as disease prodromes (Anderson & Marder, 2001;Marshall et al, 2007;van Duijn et al, 2007). In light of the presence and significance of psychiatric and cognitive symptoms in HD, it is important to examine these facets and their everyday implications carefully…”

Delayed Huntington's Disease Diagnosis in Two Alcoholic Patients With a Family History of "Parkinson's Disease"

“…1 As the earliest pathological changes in Huntington's disease are in the associative portion of the striatum, the domains first affected by the disease may be cognitive and psychiatric rather than motor.…”