Therapeutic Options in Treatment of Juvenile Huntington Disease: Difference to Adult Patients

Introduction: The DGN has published guidelines for the treatment of chorea and they are therapeutic recommendations for patients with Huntington disease (HD) and juvenile HD (jHD) as well. In less than 10% of the cases, the disease starts at the age younger than 21 years (jHD) and in 0.5 to 1% at the age younger than 10 years. We are reporting of our experience with five juvenile patients, of possibilities, problems, and differences compared with adults.

Casuistics: Patient 1 is a 17-year-old patient, with the symptoms of psychomotoric restlessness at night, during day bradykinesia, wheelchair–dependent, additional dystrophy. The therapy provided to the patient includes pramipexole, venlafaxine, quetiapine, valproic acid, L-Dopa, energy drinks, and accommodation at home.

Patient 2 is a 15-year-old patient, suffering from depression, mood disorders, avolition, bradykinesia, wheelchair–dependent, tremor, and pathologic EEG-pattern. The therapy provided to the patient includes the following: pramipexole, venlafaxine, quetiapine, valproate, and lithium; accommodation in psychiatry. Patient 3 is a 16-year-old patient, suffering from severe depression, impulse disorders, hallucinations, and tremor. The therapy provided to the patient includes the following: venlafaxine, risperidone, clozapine, and the residential accommodation for handicapped persons.

Patient 4 is a 10-year-old patient, with the symptoms of jumpiness at night, motor dysfunction, wheelchair–dependent, additional dystrophy. The therapy provided to the patient includes the following: clobazame, energy drinks, and the accommodation at home, sometimes palliative care unit.

Patient 5 is a 15-year-old patient, with school problems and depression. The therapy provided to the patient includes the following: psychotherapy, inclusion system in regular school, and accommodation at home.

Summary: Therapy plan of jHD requires an individual strategy, which is focused on the major symptom. In addition to the classic symptoms of HD, psychodynamic processes originating from adolescence contribute to the course of conflicts and determine treatment. Frequently, medication is used as off-label therapy. Because of small population of jHD studies are not practical.

Keywords: juvenile Huntington-Disease, therapeutic options, problems in youth patients.