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Resident University Clinic of Psychiatry Graz Medical University Graz, Austria
Professor University Clinic of Psychiatry Graz Medical University
Professor Head of the Department University Clinic of Psychiatry Graz Medical University
Staff Scientist University Clinic of Psychiatry Graz Medical University Auenbruggerplatz 31 8036 Graz, Austria fax 43 316 385 3556 raphael.bonelli{at}klinikum-graz.at
Published Online, March 1, 2005. www.theannals.com, DOI 10.1345/aph.1E501
Case Report. A 30-year-old woman with genetically proven Huntington's disease (55 CAG repeats) experienced increasing hyperkinesias and highly disabling rigidity. She had been treated with different high-dose neuroleptics for several years. We performed antipsychotic drug holidays in our patient for 3 years; 6 times inpatient and 4 times outpatient. The duration of neuroleptic break varied between 3 and 14 days. We used the motor section of the United Huntington's Disease Rating Scale (UHDRS) to evaluate the amelioration of her movement disorders: oculo-motor function, oro-lingual function, fine motor tasks, parkinsonism, dystonia, chorea, and statics and gait.2 In the worst case, the patient can gain 124 points for the whole UHDRS motor section; a bettering of symptoms would decrease the score. In every procedure, the subjective well-being of the patient, as well as the objective scores in UHDRS motor scale, improved significantly (Table 1), and the patient could be initiated on reduced doses of neuroleptic medication.
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The drug holiday procedure was managed as follows. On day 1, we stopped all antipsychotic medication and administered amantadine infusions (beginning with 0.2 g twice daily up to 0.4 g twice daily) to reduce rigidity and benzodiazepines for reduction of chorea. Hyperkinesias and rigidity improved promptly. At the recurrence of choreatic movements (usually about the seventh day of treatment), we reduced and stopped amantadine infusions, and neuroleptics were restarted in low doses.
Discussion. Theories of transmitter and deregulation of basal ganglia show a clear association in the underlying mechanism of Parkinson's disease and Huntington's disease. Both diseases lead to destruction of neurons and uninhibited transmitter distribution in neighbor areas, resulting in unbalanced movements.3 Drug holidays are often used in the treatment of Parkinson's disease to ameliorate the effects of chronic levodopa by resensitizing dopamine receptors in the striatum. Levodopa therapy can then be perpetuated at lower doses with fewer of the adverse effects that usually accompany long-term use of the drug.1 Some studies show a significant improvement of dyskinesia and reduced adverse effects, often lasting up to 3 years.1 A theory in Huntington's disease that neurodegeneration is caused by a relative excess of excitatory neurotransmitters such as glutamate led to the attempt of using amantadine,4 which has been used in Parkinson's disease.5
The use of antipsychotic medications in Huntington's disease is limited due to their diminishing effect in symptom progression. New strategies to ameliorate motor disorders and the subjective well-being of the patient are needed. Our findings show that neuroleptic-resistant hyperkinesias and rigidity associated with Huntington's disease improve during an antipsychotic break. Drug holidays seem to be an adequate therapy to reduce symptoms and antipsychotic doses.
References
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