An overview of diagnosing atypical parkinsonian disorders, including progressive supranuclear palsy (PSP), was published in the August 2016 issue of Continuum, a journal for neurologists.
The article describes PSP as follows:
It is the most common form of atypical parkinsonism, comprising about 5% to 6% of those patients presenting with parkinsonism. The estimated prevalence and annual incidence of PSP is about 5 per 100,000 in individuals between the ages of 50 and 99 years, but is likely higher due to misdiagnosis and under-recognition. The average age of onset is typically in the sixties (average age of 63 to 66 years), and the mean survival from diagnosis is reported between 5 to 8 years. Hallmarks of the disease include prominent, early postural instability, unexplained falls, vertical supranuclear palsy, and progressive dementia.
There’s nothing new in the article but it provides a good summary of current knowledge about PSP. The article discusses:
* PSP symptoms, including falls, eye problems, facial appearance, dysarthria, dysphagia, apathy, depression, disinhibition,
emotional lability, dementia, and perseveration.
* the five types of PSP, including:
> Richardson syndrome (classic PSP)
> PSP-parkinsonism
> PSP-pure akinesia with gait freezing
> PSP-corticobasal syndrome (PSP-CBS) (or primary nonfluent
aphasia)
> PSP-behavioral variant of frontotemporal dementia (FTD)
One case study is provided of someone with PSP-parkinsonism.
* two other possible PSP variants with features that overlap with
either primary lateral sclerosis (PLS) or cerebellar ataxia.
* PSP pathology
* PSP diagnostics
* PSP treatments
A very short excerpt is copied below.
The abstract is available at no charge. Amazingly, the full article seems to be available at no charge; grab it while you can! See:
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
PubMed ID#: 27495201
Link to Abstract
Link to Full article
Happy reading!
Robin
Excerpts from
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
KEY POINTS
* Key features of progressive supranuclear palsy include early gait instability, unexplained falls, supranuclear gaze palsy, axial rigidity, dysarthric speech, and dementia.
* The rocket sign occurs in patients with progressive supranuclear palsy who have lost insight into their postural instability and “rocket” out of their chair without assistance, resulting in a high risk for falling.
* Early signs of supranuclear gaze palsy in patients with progressive supranuclear palsy include slowed vertical saccades and reduced optokinetic nystagmus. Square-wave jerks, or minute saccadic eye movements, may also be present, representing fixation instability.
* To assess for the applause sign, a clinician can demonstrate three claps to the patient and ask him or her to copy. The applause sign is present if the patient claps more than three times and continues (perseverates).
* The approach to treatment of patients with progressive supranuclear palsy should include a multidisciplinary team and involve physical, occupational, and speech therapy; psychiatry; neuropsychology; social work; and palliative care.
* Levodopa therapy should be tried in most progressive supranuclear palsy cases, with a levodopa dose of up to 1200 mg/d in divided doses as tolerated. Partial response is possible in early progressive supranuclear palsy, particularly in progressive supranuclear palsy–parkinsonism.