Management Guidelines
Evaluations Following Initial Diagnosis
To establish the extent of disease and needs in an individual diagnosed with Parkin type early-onset Parkinson disease (PARK-Parkin), the following evaluations (if not performed as part of the evaluation that led to the diagnosis) are recommended:
Assess the presence and the severity of parkinsonian signs, non-motor features, and treatment-related complications using the Unified Parkinson's disease rating scale (UPDRS) or the Movement Disorder Society (MDS) UPDRS .
Assess the presence of atypical signs, such as hyperreflexia and dystonia.
Evaluate the degree of response to treatment.
Assess for cognitive or behavioral problems.
Consider consultation with a clinical geneticist and/or genetic counselor.
Treatment of Manifestations
To date, the treatment of PARK-Parkin does not differ from that of Parkinson disease of other etiologies. No specific guidelines are currently available.
The motor impairment usually responds very well to low doses of dopaminergic medication; the response is typically sustained even after long disease duration. To reduce or delay side effects, levodopa doses should not exceed the levels required for satisfactory clinical response.
On average, the response to low doses of levodopa is excellent and sustained. The likelihood of developing levodopa-induced dyskinesias is higher than in individuals with parkinsonism resulting from other etiologies.
Source: GeneReviews — "Parkin Type of Early-Onset Parkinson Disease"
