Juvenile Huntington Disease

Juvenile Huntington's disease (HD), also called early-onset HD, is an incurable genetic disorder that affects people under the age of 20. Juvenile HD accounts for around 10 percent of all HD cases. Although a defect in the same gene causes both early-onset and adult-onset HD, the symptoms are different.

Symptoms

• Children with juvenile HD may suffer from generalized or partial seizures, behavioral changes, dementia, learning difficulties, muscle rigidity and problems with swallowing and speech.

Diagnosis

• It is hard to diagnose juvenile HD because other unrelated conditions might also cause similar symptoms. Doctors need to carefully study the patient's medical and family history before making the diagnosis.

Treatment

• Anticonvulsive drugs are used to treat seizures. Physical therapy (such as pool therapy) can help prevent muscular atrophy and improve movement. A combination of counseling and drugs can help with psychological and behavioral problems.

Progression

• The progression of juvenile HD depends on the age of the patient. The sooner the symptoms occur, the faster the disease progresses. Most children with juvenile HD require round the clock assistance and care.

Prognosis

• Juvenile HD is a degenerative disease that gets worse with age. Patients of juvenile HD usually die within 10 years of the emergence of symptoms.