From the Guidelines
Progressive supranuclear palsy (PSP) is a rare neurodegenerative brain disorder characterized by the gradual deterioration of brain cells in areas controlling eye movements, balance, and other motor functions, with a prevalence of around 5/100,000 1.
Key Characteristics of PSP
- The hallmark symptom is difficulty with downward eye movements, which progresses to affect all directions of gaze 2.
- Patients typically experience frequent falls, stiffness, slow movements, slurred speech, difficulty swallowing, and changes in personality or cognition.
- PSP is caused by the abnormal accumulation of tau protein in brain cells, similar to Alzheimer's disease but affecting different brain regions.
- The condition usually begins after age 60 and progresses more rapidly than Parkinson's disease, with which it is sometimes confused 1.
Diagnosis and Management
- Correctly diagnosing a Parkinsonian syndrome on clinical features alone can be quite challenging, and imaging remains an essential diagnostic tool in the evaluation of a patient presenting with Parkinsonian symptoms 1.
- Treatment focuses on managing symptoms with medications like levodopa (though response is typically limited), antidepressants for mood changes, and botulinum toxin for eye problems.
- Physical, occupational, and speech therapy are crucial parts of management.
Prognosis
- Most patients require significant care within 3-5 years of diagnosis, and the condition typically leads to serious complications within 6-10 years.
- The neurological examination aims to identify motor signs that may be associated with FTD or FTD-related disorders, such as parkinsonism, oculomotor disorders or ALS, which can help distinguish PSP from other conditions 2.
From the Research
Definition and Characteristics of Progressive Supranuclear Palsy
- Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder characterized by postural instability and falls, vertical supranuclear gaze palsy, parkinsonism with poor levodopa response, pseudobulbar palsy, and frontal release signs 3.
- PSP is a disorder of tau protein aggregation, with a clinical spectrum wider than originally described, including a phenotype resembling Parkinson disease in about a third of cases 4.
- The classic syndrome of PSP is recognized by a combination of down gaze palsy with progressive rigidity and imbalance leading to falls 5.
Clinical Features and Diagnosis
- The clinical features of PSP include motor symptoms (100%), cognitive/behavioral symptoms (89%), systemic and bulbar symptoms (80%), and sleep disturbances (60%) 3.
- The diagnosis of PSP remains clinical, although magnetic resonance imaging and cerebrospinal fluid measures are showing promise as early-stage screening tools 4.
- The 2017 International Parkinson and Movement Disorder Society PSP criteria outline 14 core clinical features and 4 clinical clues that combine to diagnose one of eight PSP phenotypes with probable, possible, or suggestive certainty 6.
Treatment and Prognosis
- Treatment of PSP is supportive, with coenzyme Q-10 showing preliminary symptomatic efficacy and levodopa providing transient, modest benefit 4.
- The presence of sleep disturbances, and possibly hallucinations, is associated with increased death risk 3.
- The median survival of PSP patients is approximately 7 years, with a mean onset age of 63 4.