eMedicine Specialties > Psychiatry > Adult
Huntington Disease Dementia: Treatment & Medication
Updated: Aug 20, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Acetylcholinesterase inhibitors (eg, rivastigmine, memantine) may have positive effects on cognition, although no treatment halts the progression of this illness. 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).
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.
- 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.
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.
Medication
Drugs used to manage psychosis and agitation in patients with dementia are intended to decrease psychotic symptoms (eg, paranoia, delusions, hallucinations) and associated or independent agitation, screaming, combativeness, or violence. The therapeutic goal is increased comfort and safety of patients, families, and caregivers.
Antipsychotics
Choice of agent is based on adverse-effect profile (patient specific). Atypical neuroleptics such as quetiapine and aripiprazole are the best studied. They are initiated at low doses and are given as standing doses rather than as needed. Use the lowest effective dose, and treat emergent adverse effects first by dose reduction. Younger and less frail individuals may tolerate and respond to somewhat higher doses. Periodically consider reducing or withdrawing antipsychotic medications.
Seroquel (Quetiapine)
Highly effective with data that indicates it has a low EPS profile (similar to placebo). Flipside is its initial sedating effect.
Adult
50-300 mg PO bid; give higher dose in PM or HS Dosages do not have to equal or exceed dosage regimens for bipolar disorders or the psychotic disorders
Pediatric
Not applicable
Warnings of use in the elderly as a class effect
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Sedation, dizziness, unsteadiness, hypotension
Aripiprazole (Abilify)
Mechanism of action is unknown, but is hypothesized to work differently than other antipsychotics. Thought to be a partial dopamine (D2) and serotonin (5HT-1A) agonist, and antagonize serotonin (5HT-2A). Additionally, no QTc interval prolongation noted in clinical trials.
Adult
10-15 mg PO qd; if needed, may increase dose gradually q2wk, not to exceed 30 mg/d
Pediatric
Not established
CYP450 3A4 and 2D6 isoenzyme substrate, thus, inhibitors (ie, ketoconazole, quinidine, fluoxetine, paroxetine) or inducers (ie, carbamazepine) may increase or decrease serum levels, respectively
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Common adverse effects include headache, anxiety, somnolence, or insomnia; rare reports of tardive dyskinesia and neuroleptic malignant syndrome; may cause orthostatic hypotension, seizure, dysphagia, or suicidal ideation; hyperglycemia may occur and in some cases be extreme, resulting in ketoacidosis, hyperosmolar coma, or death
Quetiapine (Seroquel)
Indicated for schizophrenia. May act by antagonizing dopamine and serotonin effects.
Adult
Initial: 25 mg PO bid/tid and increase to a range of 300-400 divided bid/tid by fourth day
Maintenance: 150-750 mg/d
Pediatric
Not established
May antagonize levodopa and dopamine agonists; phenytoin, thioridazine, and other liver enzyme inducers may reduce levels
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May induce orthostatic hypotension associated with dizziness, tachycardia, and syncope; has been associated with neuroleptic malignant syndrome
Risperidone (Risperdal)
Effective against agitation and psychosis in elderly patients, even at very low doses, which may limit EPS. Binds to dopamine D2 receptor with 20 times lower affinity than for 5-HT2 receptor. Improves negative symptoms of psychoses and reduces incidence of EPS.
Adult
0.5-2 mg PO qd; titrate at 1-wk intervals prn; not to exceed 16 mg/d
Pediatric
Not established
Avoid alcohol; caution with other drugs that may prolong QT interval; coadministration with carbamazepine may decrease effects; may inhibit effects of levodopa; clozapine may increase levels
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause extrapyramidal reactions, hypotension, tachycardia, and arrhythmias
Clozapine (Clozaril)
May be a good choice for individuals who cannot tolerate EPS of conventional antipsychotic agents. May inhibit serotonin, muscarinic, and dopamine effects.
Adult
25 mg PO qd/bid; then increase to a therapeutic target dose of 300-450 mg/d after 2 wk
Pediatric
Not established
Epinephrine and phenytoin may decrease effects; TCAs, neuroleptics, CNS depressants, guanabenz, and anticholinergics may increase effects; do not use with bone marrow depressants
Documented hypersensitivity; WBC count <3500/µL before or during therapy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not discontinue abruptly; perform WBC testing q2wk for duration of therapy
Thioridazine (Mellaril)
Medium-potency antipsychotic with less EPS but more sedation than haloperidol. More sedating and may be given at bedtime for a patient with difficulty falling asleep. First-line drug for treatment of evening psychosis (sundowning) or multiple affective symptoms (eg, agitation, anxiety, depressed mood, tension, sleep disturbance, fears) in elderly patients, patients with dementia, or both.
Adult
25-100 mg PO qd
Pediatric
<2 years: Not recommended
>2 years: 25 mg PO qd; not to exceed 3 mg/kg/d
May cause false-positive pregnancy test result; some anticholinergics may reduce effects of medication; may increase toxicity of CNS depressants, TCAs, and antihypertensives (eg, propranolol, pindolol); may decrease effects of guanethidine
Documented hypersensitivity; severely depressed state
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients may experience thermoregulatory changes and stomach upset; orthostasis may lead to falls in elderly patients; caution in narrow-angle glaucoma and severe cardiac or liver disease
Benzodiazepines
Sometimes not ideal agents for geriatric patients. May have higher likelihood of adverse effects and potentially fewer benefits than antipsychotics; however, can be useful in treating agitation when anxiety is prominent. Most common adverse effects are sedation, ataxia, amnesia, confusion, and paradoxical anxiety. Long-acting agents must be used with caution, and dose increases should be gradual. If benzodiazepines are used for an extended period (eg, 1 mo), they should be tapered rather than stopped abruptly, owing to the risk of withdrawal.
Lorazepam (Ativan)
Sedative hypnotic with short onset of effects and relatively long half-life. By increasing action of GABA, which is a major inhibitory neurotransmitter in the brain, may depress all levels of CNS, including limbic and reticular formation. Does not require oxidative metabolism in the liver and has no active metabolites.
Benzodiazepine of choice in the ED; can be given PO, SL (for rapid effect in panic attack), and IM or IV (mixed in same syringe with antipsychotic). Has longer CNS effects than diazepam and is preferred over antipsychotics for treatment of psychosis secondary to acute intoxication with hallucinogens, cocaine, PCP, and stimulants. Can be used as adjunctive therapy in nonorganic acute psychosis in which DOC is a high potency antipsychotic.
Adult
0.5-2 mg PO/IV/IM q6-8h
Pediatric
0.02-0.05 mg/kg PO/IV/IM; not to exceed 4 mg/dose
Toxicity of benzodiazepines in CNS increases when used concurrently with alcohol, phenothiazines, barbiturates, and MAOIs
Documented hypersensitivity; preexisting CNS depression; hypotension; narrow-angle glaucoma
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal or hepatic impairment, myasthenia gravis, organic brain syndrome, or PD; drug or alcohol abuse; suicidal tendencies
Anticonvulsants
Given the limited data, cannot be recommended with confidence for treatment of agitation in patients with dementia. Nonetheless, a therapeutic trial of one of these agents may be appropriate for some patients who are nonpsychotic, especially those who are mildly agitated or unresponsive to antipsychotics. Monitoring patients for symptoms of toxicity and discontinuing administration if a toxic symptom is identified or if no improvement is observed are critical. The more efficacious agents have been the newer ones (ie, zonisamide, oxcarbazepine, levetiracetam), which are GABA modulators and have less toxicity, with no need to monitor levels.
Zonisamide (Zonegran)
Indicated for adjunctive treatment of partial seizures with or without secondary generalization. Evidence indicates that it is also effective in myoclonic and other generalized seizure types.
Adult
100 mg/d PO for 2 wk, then increase by 100 mg/d PO q2wk; not to exceed 400 mg/d; may be given qd or bid
Pediatric
Not established
May increase serum carbamazepine levels; carbamazepine may increase concentrations; phenobarbital may decrease levels
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause drowsiness, weight loss, ataxia, nausea, and slowing of mental activity
Oxcarbazepine (Trileptal)
Indicated for treatment of seizures. Pharmacological activity is primarily by the 10-monohydroxy metabolite (MHD) of oxcarbazepine. May block voltage-sensitive sodium channels, inhibit repetitive neuronal firing, and impair synaptic impulse propagation. Anticonvulsant effect also may occur by affecting potassium conductance and high-voltage activated calcium channels. Drug pharmacokinetics are similar in older children (>8 y) and adults. Young children <8 y) have a 30-40% increased clearance compared with older children and adults. Children <2 y have not been studied in controlled clinical trials.
Adult
Adjunctive therapy: 600 mg/d PO divided bid initially; may increase by a maximum of 600 mg/d qwk; recommended daily dose is 1200 mg/d; monitor patients for adverse anticonvulsant effects
Conversion to monotherapy: 600 mg/d PO divided bid initially; gradually reduce dose of concomitant anticonvulsants in approximately 3-6 wk and gradually increase oxcarbazepine dose in 2-4 wk; may increase oxcarbazepine dose prn by maximum increment of 600 mg/d qwk; monitor patients closely during transition phase for adverse anticonvulsant effects
Initiation of monotherapy: 600 mg/d PO divided bid initially; increase dose by 300 mg/d PO q3d to 1200 mg/d; monitor patients for adverse anticonvulsant effects
Pediatric
Adjunctive therapy
<4 years: Not established
4-16 years: 8-10 mg/kg/d PO divided bid, not to exceed 600 mg/d; gradually increase to target dose over 2 wk
Target dose (based on body weight)
20-29 kg: 900 mg/d
29.1-39 kg: 1200 mg/d
>39 kg: 1800 mg/d
May decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin, and valproic acid; when given in doses >1200 mg/d, may significantly increase phenytoin and phenobarbital serum concentrations; can reduce serum concentrations of oral contraceptives and make them ineffective; can increase clearance of felodipine
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Can cause adverse cognitive effects (eg, psychomotor slowing, impaired concentration, impaired speech and language); in persons with impaired renal function (CrCl <30 mL/min), begin at half usual starting dose; dose increments should be made more slowly; can cause hyponatremia (sodium <125 mmol/L); among persons with hypersensitivity to carbamazepine, 25-30% will have hypersensitivity to oxcarbazepine; rapid withdrawal can cause exacerbation of seizures; observe for adverse effects and monitor plasma levels of concomitant anticonvulsants during dose titration
Levetiracetam (Keppra)
Used as add-on therapy for partial seizures. Mechanism of action unknown. Has favorable adverse effect profile, with no life-threatening toxicity reported.
Adult
1000 mg/d PO bid (500 mg bid); may increase 1000 mg/d q2wk to maximum recommended daily dose of 3000 mg; long-term experience at doses >3000 mg/d is relatively minimal, and no evidence indicates doses >3000 mg/d offer additional benefit
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment; major adverse effects include somnolence, asthenia, incoordination, mild leukopenia (3%), and behavioral changes (eg, anxiety, hostility, emotional lability, depression and psychosis [1-2%], depersonalization)
Antidepressants
Patients with depression should be considered for treatment even when the diagnostic criteria for major depression are not met. Evaluate patient for neurovegetative signs, suicidal ideation, and other indicators of major depression because these may indicate a need for safety measures. Successful treatment of depression, partially or fully, can resolve cognitive deficits. SSRIs tend to have a more favorable adverse effect profile than cyclic agents. The choice of an antidepressant generally is based on adverse effect profile and general medical and psychiatric status of each patient. SNRIs, which now include venlafaxine (Effexor XR) and duloxetine (Cymbalta), are first-choice agents.
Paroxetine mesylate (Paxil)
Selectively inhibits presynaptic serotonin reuptake. Besides use in patients with depression, Ranen et al used sertraline in the treatment of severe aggressiveness in HD. Complete cessation of agitative behavior was maintained on follow-up studies.
Adult
10-40 mg PO qd, usually dosed PM secondary to sedating effects
Pediatric
Not established
Increases toxicity of MAOIs, diazepam, tolbutamide, and warfarin; avoid alcohol; may be potentiated by cimetidine; caution with other CNS drugs and drugs metabolized by CYP2D6 isoenzyme (eg, TCAs, flecainide, propafenone)
Documented hypersensitivity; during or within 14 d of MAOIs
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in preexisting seizure disorders and recent myocardial infarction, unstable heart disease, and hepatic or renal impairment; monitor for mania or hypomania; suicidal tendencies
Venlafaxine (Effexor)
Indicated for treatment of depression. May treat depression by inhibiting neuronal serotonin and norepinephrine reuptake. In addition, causes beta-receptor down-regulation.
Adult
IR: 75 mg/d PO divided bid/tid with food; increase in 75-mg/d increments q4d to 225-375 mg/d
ER: 75 mg/d PO with food; increase in 75-mg/d increments q4d to 225 mg/d
Pediatric
Not established
Cimetidine, MAOIs, sertraline, fluoxetine class IC antiarrhythmics, TCAs, and phenothiazine may increase effects
Documented hypersensitivity; patients taking MAOIs or have taken them within 14 d of initiating therapy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients may experience hypertension; fatal reaction may occur if taken concurrently with an MAOI; exercise caution in patients with cardiovascular disorders
Duloxetine (Cymbalta)
Potent inhibitor of neuronal serotonin and norepinephrine reuptake. Antidepressive action is theorized to be due to serotonergic and noradrenergic potentiation in CNS.
Adult
20 mg PO bid; may increase to 60 mg/d administered qd or divided as 30 mg bid
Pediatric
Not established
Metabolized by CYP1A2 and CYP2D6; coadministration with drugs that inhibit CYP1A2 (eg, fluvoxamine, cimetidine, ciprofloxacin, enoxacin) may increase blood levels and toxicity; coadministration with drugs that inhibit CYP2D6 (eg, paroxetine, fluoxetine, quinidine) may increase blood levels and toxicity; moderately inhibits CYP2D6 and may decrease elimination of CYP2D6 substrates (eg, TCAs, phenothiazines [eg, thioridazine], type IC antiarrhythmics [eg, propafenone, flecainide]); coadministration with MAOIs may cause serious, sometimes fatal reactions that include hyperthermia, rigidity, myoclonus, autonomic instability, and mental status changes including extreme agitation, delirium, and coma (see Contraindications)
Documented hypersensitivity; uncontrolled narrow-angle glaucoma; do not administer with 14 days after stopping MAOI or initiate MAOIs within 5 d after stopping duloxetine
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Observe closely for clinical worsening and suicidal ideation when initiating treatment or following dosage change; gradually decrease dose when discontinuing, do not abruptly discontinue; caution with hepatic impairment or end-stage renal disease; may cause slight blood pressure increase; may activate mania or hypomania; common adverse effects include nausea, dry mouth, constipation, decreased appetite, fatigue, somnolence and increased sweating
More on Huntington Disease Dementia |
| Overview: Huntington Disease Dementia |
| Differential Diagnoses & Workup: Huntington Disease Dementia |
 Treatment & Medication: Huntington Disease Dementia |
| Follow-up: Huntington Disease Dementia |
| References |
| « Previous Page | Next Page » |
References
American Psychiatric Association. Practice guideline for the treatment of patients with Alzheimer''s disease and other dementias of late life. American Psychiatric Association. Am J Psychiatry. May 1997;154(5 Suppl):1-39. [Medline].
Bachoud-Levi AC, Remy P, Nguyen JP, et al. Motor and cognitive improvements in patients with Huntington''s disease after neural transplantation. Lancet. Dec 9 2000;356(9246):1975-9. [Medline].
Bamford KA, Caine ED, Kido DK, et al. A prospective evaluation of cognitive decline in early Huntington''s disease: functional and radiographic correlates. Neurology. Oct 1995;45(10):1867-73. [Medline].
Barami K, Hutchins KD, Lyman WD. Neurotransmitter distribution in the second trimester fetal human corpus striatum. Neurol Res. Jan 2001;23(1):16-22. [Medline].
Barnoy S, Tabak N. Israeli nurses and genetic information disclosure. Nurs Ethics. May 2007;14(3):280-94. [Medline].
Biglan K, Shoulson I. Juvenile-onset huntington disease: a matter of perspective. Arch Neurol. Jun 2007;64(6):783-4. [Medline].
Chou KL, Borek LL, Friedman JH. The management of psychosis in movement disorder patients. Expert Opin Pharmacother. May 2007;8(7):935-43. [Medline].
Folstein S. Huntington's Disease: A Disorder of Families. Baltimore, Md: Johns Hopkins University Press; 1989.
Folstein SE. The psychopathology of Huntington''s disease. Res Publ Assoc Res Nerv Ment Dis. 1991;69:181-91. [Medline].
Frank S, Biglan K. Long-term fetal cell transplant in Huntington disease: stayin' alive. Neurology. Jun 12 2007;68(24):2055-6. [Medline].
Garcia Ruiz PJ, Mena MA, Sanchez Bernardos V, et al. Cerebrospinal fluid homovanillic acid is reduced in untreated Huntington''s disease. Clin Neuropharmacol. Feb 1995;18(1):58-63. [Medline].
Gilroy J. Basic Neurology. 3rd ed. New York, NY: McGraw-Hill; 2000.
Gustavson AR, Cummings JL. Cholinesterase inhibitors in non-Alzheimer dementias. J Am Med Dir Assoc. Nov-Dec 2003;4(6 Suppl):S165-9. [Medline].
Hahn-Barma V, Deweer B, Durr A, et al. Are cognitive changes the first symptoms of Huntington''s disease? A study of gene carriers. J Neurol Neurosurg Psychiatry. Feb 1998;64(2):172-7. [Medline].
Hakimian R. Disclosure of Huntington''s disease to family members: the dilemma of known but unknowing parties. Genet Test. 2000;4(4):359-64. [Medline].
Harper P. Huntington's disease. In: Harper P, ed. Major Problems of Neurology. 2nd ed. Philadelphia, Pa: WB Saunders; 1996.
Harris GJ, Codori AM, Lewis RF, et al. Reduced basal ganglia blood flow and volume in pre-symptomatic, gene- tested persons at-risk for Huntington''s disease. Brain. Sep 1999;122 ( Pt 9):1667-78. [Medline].
Hodges JR, Salmon DP, Butters N. Differential impairment of semantic and episodic memory in Alzheimer''s and Huntington''s diseases: a controlled prospective study. J Neurol Neurosurg Psychiatry. - Butters N;53(12):1089-95. [Medline].
Jason GW, Suchowersky O, Pajurkova EM, et al. Cognitive manifestations of Huntington disease in relation to genetic structure and clinical onset. Arch Neurol. Sep 1997;54(9):1081-8. [Medline].
Kallail KJ, Godfrey NE, Suter G, Anthimides L. A multidisciplinary approach to the management of Huntington''s disease. Kans Med. Nov 1989;90(11):309-11. [Medline].
Kirkwood SC, Su JL, Conneally P, Foroud T. Progression of symptoms in the early and middle stages of Huntington disease. Arch Neurol. Feb 2001;58(2):273-8. [Medline].
Kremer B, Goldberg P, Andrew SE, et al. A worldwide study of the Huntington''s disease mutation. The sensitivity and specificity of measuring CAG repeats. N Engl J Med. May 19 1994;330(20):1401-6. [Medline].
Marder K, Zhao H, Myers RH, et al. Rate of functional decline in Huntington''s disease. Huntington Study Group. Neurology. Jan 25 2000;54(2):452-8. [Medline].
Mayeux R, ed. Dementias: Advances in Neurology. 38. New York, NY: Raven; 1983.
Meiser B, Dunn S. Psychological impact of genetic testing for Huntington''s disease: an update of the literature. J Neurol Neurosurg Psychiatry. Nov 2000;69(5):574-8. [Medline].
Nance MA. Huntington disease: clinical, genetic, and social aspects. J Geriatr Psychiatry Neurol. Summer 1998;11(2):61-70. [Medline].
Nehl C, Paulsen JS. Cognitive and psychiatric aspects of Huntington disease contribute to functional capacity. J Nerv Ment Dis. Jan 2004;192(1):72-4. [Medline].
Ramaswamy S, Shannon KM, Kordower JH. Huntington's disease: pathological mechanisms and therapeutic strategies. Cell Transplant. 2007;16(3):301-12. [Medline].
Ranen NG, Lipsey JR, Treisman G, Ross CA. Sertraline in the treatment of severe aggressiveness in Huntington''s disease. J Neuropsychiatry Clin Neurosci. Summer 1996;8(3):338-40. [Medline].
Ribaï P, Nguyen K, Hahn-Barma V, Gourfinkel-An I, Vidailhet M, Legout A. Psychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patients. Arch Neurol. Jun 2007;64(6):813-9. [Medline].
Rosenstein LD. Differential diagnosis of the major progressive dementias and depression in middle and late adulthood: a summary of the literature of the early 1990s. Neuropsychol Rev. Sep 1998;8(3):109-67. [Medline].
Sharma P, Savy L, Britton J, et al. Huntington''s disease: a molecular genetic and CT comparison. J Neurol Neurosurg Psychiatry. Feb 1996;60(2):206-8. [Medline].
Sorensen SA, Fenger K, Olsen JH. Significantly lower incidence of cancer among patients with Huntington disease: An apoptotic effect of an expanded polyglutamine tract?. Cancer. Oct 1 1999;86(7):1342-6. [Medline].
Stahl SM, Thiemann S, Faull KF, et al. Neurochemistry of dopamine in Huntington''s dementia and normal aging [retracted by Stahl SM, Thiemann S, Faull KF, Barchas JD, Berger PA. In: Arch Gen Psychiatry 1989 Aug;46(8):758]. Arch Gen Psychiatry. Feb 1986;43(2):161-4. [Medline].
Starkstein SE, Brandt J, Bylsma F, et al. Neuropsychological correlates of brain atrophy in Huntington''s disease: a magnetic resonance imaging study. Neuroradiology. 1992;34(6):487-9. [Medline].
Swash M. Clinical Neurology. New York, NY: Churchill Livingstone; 1991.
Tian J, Herdman SJ, Zee DS, Folstein SE. Postural stability in patients with Huntington''s disease. Neurology. Jun 1992;42(6):1232-8. [Medline].
Zakzanis KK. The subcortical dementia of Huntington''s disease. J Clin Exp Neuropsychol. Aug 1998;20(4):565-78. [Medline].
Further Reading
Keywords
Huntington disease, dementia, HD, Huntington's disease, movement disorders, progressive dementia, psychiatric illness, behavioral disturbance, mental illness, psychosis, depression, schizophrenia, benign familial chorea, inherited ataxias, neural acanthocytosis, familial Alzheimer disease, AD, Parkinson disease, Parkinsonism, PD
