Huntington Disease Dementia Treatment & Management

Updated: Sep 11, 2018
  • Author: Idan Sharon, MD; Chief Editor: David Bienenfeld, MD  more...
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Treatment

Medical Care

Acetylcholinesterase inhibitors (eg, rivastigmine, memantine) may have positive effects on cognition, although no treatment halts the progression of this illness. A recent review detailing all studies investigating the effectiveness of acetylcholinesterase inhibitors revealed that there is little evidence of the benefit of such medications. [11]

Symptomatic treatment is aimed at minimizing the distressing movements. Pharmacological intervention is available for the behavior and/or psychologic disturbances, chorea, and weight loss. Psychologic symptoms may require major antipsychotic drugs for control. Treatment for patients with depression is used to improve mood, functional status, and quality of life. Research has shed greater understanding on the disease mechanism; however, promising avenues in gene therapy and neurotransplantation are still only in their incipient stages.

Cognitive impairment

Patients who are cognitively impaired require a multidisciplinary treatment approach, which must be based on a solid alliance with the patient and family.

Ongoing assessment should include periodic monitoring of the development and evolution of cognitive and noncognitive psychiatric symptoms and their response to intervention.

Safety measures include (1) evaluation of suicidal tendency and the potential for violence; (2) recommendations regarding providing adequate supervision, preventing falls, and limiting the hazards of wandering; (3) vigilance regarding neglect or abuse; and (4) restrictions on driving and the use of other dangerous equipment.

Also, helping patients and their families plan for financial and legal issues is important.

Psychosis

Intervention should be guided by the patient's level of distress and risk to the patient or caregivers.

In addition to distress, if agitation, combativeness, or violent behavior is causing danger to the patient or others, psychopharmacologic treatment is indicated with atypical neuroleptics and mood stabilizers (anticonvulsants known as GABA agents).

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Surgical Care

One experimental strategy that may offer hope in the neurodegenerative disorder of HD has been neural transplantation. Fetal human striatal implants to replace lost neurons and/or prevent the degeneration of neurons destined to die most likely will be the first transplantation strategy attempted in clinical trials.

A study conducted in France examined whether grafts of human fetal striatal tissue could survive and have detectable effects in 5 patients with mild-to-moderate HD. [12]

  • After 2 years of preoperative assessment, patients were grafted with human fetal neuroblasts into the right striatum then, after a year, the left striatum.

  • Final results were assessed 1 year later on the basis of neurological, neuropsychological, neurophysiological, and psychiatric test results.

  • The final position emission tomography scan assessment showed increased metabolic activity in various subnuclei of the striatum in 3 of 5 patients, contrasting with the progressive decline recorded in the other 2 patients in the series, as seen in patients with untreated HD. Motor and cognitive functions were improved or maintained within the normal range, and functional benefits were seen in activities of daily living in these 3 patients but not in the other 2.

  • Fetal neural allografts could be associated with functional, motor, and cognitive improvements in patients with HD.

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Consultations

Consultation with social service agency personnel is warranted. As the patient's dependency increases, caregivers may begin to feel more burdened. Families should be counseled regarding when to consider and plan for additional support at home or for possible transfer to a long-term care facility. A referral for some form of respite care (eg, home health aid, daycare, brief nursing home stay) with the help of social service agency personnel may be helpful.

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