Hemiparkinsonism-hemiatrophy syndrome

Hemiparkinsonism-hemiatrophy syndrome (HPHA) is a rare neurological condition in which a person develops Parkinson-like symptoms — such as tremor, stiffness, and slow movement — on one side of the body, along with noticeable shrinkage (atrophy) of that same side of the body. The body atrophy typically involves the limbs and sometimes the face, and it is usually present from early in life or becomes apparent during childhood or young adulthood. The parkinsonian symptoms tend to appear later, often in early to middle adulthood, and they primarily affect the same side as the body shrinkage. Unlike typical Parkinson's disease, HPHA tends to start at a younger age and progresses more slowly. Brain imaging often reveals asymmetric changes, such as shrinkage of one side of the brain (cerebral hemiatrophy) or abnormalities in the basal ganglia, which are brain structures involved in movement control. The exact cause of this syndrome is not fully understood, but it is thought to result from brain injury or abnormal brain development early in life rather than a specific genetic mutation. Treatment is mainly symptomatic. Levodopa and other Parkinson's disease medications may help with stiffness and slowness, though the response can be variable and sometimes less robust than in typical Parkinson's disease. Physical therapy, occupational therapy, and supportive care play important roles in maintaining function and quality of life. There is currently no cure for this condition.

Bring these to your next appointment

• Q1.Is my condition likely to progress, and if so, how quickly?,Would levodopa or other Parkinson's medications help my symptoms?,What physical therapy exercises would be most beneficial for me?,Are there any clinical trials or research studies I could participate in?,Should I have regular brain imaging to monitor changes over time?,Could deep brain stimulation be an option for me in the future?,What can I do to maintain my independence and quality of life long-term?

Common questions about Hemiparkinsonism-hemiatrophy syndrome