How Quickly Does Progressive Supranuclear Palsy Progress & Is PSP Similar To ALS?

PSP is a progressive condition and the condition worsens as it progresses. PSP patients become severely disabled with a few years of onset of symptoms.1

Progressive supranuclear palsy is a rare progressive disorder that shows similar symptoms to Parkinson’s and ALS.2

Clinical pathology has been documented in several cases that affect movements, balance, and speech.3

Progressive supranuclear palsy (PSP) is a brain disorder that affects the brain cells that control the movement of the eyes. This eventually causes serious problems with trouble walking and maintaining balance. It is the most common atypical parkinsonism and Alzheimer’s that occurs once in every 100,000 people over age 60.

The condition is difficult to diagnose so there is a higher risk of progression resulting in choking, pneumonia, head injury, and frequent falls.

How Quickly Does Progressive Supranuclear Palsy Progress?

Progressive supranuclear palsy is characterized by the accumulation of tau protein that generally affects the frontal lobe, brainstem, cerebellum, and substantia nigra. It affects nearly 20000 people in the US. People with PSP experience changes in behavior, difficulties in controlling emotions, and troubled writing.

PSP is a progressive condition and the condition worsens as it progresses. Progressive supranuclear palsy patients become severely disabled within a few years of onset of symptoms. The prevalence ratio for new incidents for ages 50-99 is 5 in every 100,000, and the undefined prevalence ratio is 1 in every 10000 cases. The higher incidence is noticed in male patients who are in their early sixties.

Patients with this disorder usually progress with their symptoms and the typical life span is 5 years after the diagnosis of the condition. The average time for a person to experience first impairment is 4 years however in rare cases, especially in persons with pre-conditions or ailment, the major motor impairment happens even in two years. The coughing interval increases in patients especially after every meal and this is a typical symptom of pneumonia. Mortality is lower but when the person suffers from pneumonia, there is a higher incidence of death.

But, of course, frequent falls are more common resulting in multiple fractures however they are still ambulatory. The patient’s speech starts turning slurry after 5 years of diagnosis and they have reduced ability of concentration and memory.1

Is Progressive Supranuclear Palsy Similar To ALS?

Progressive supranuclear palsy (PSP) is a neurological disorder analogous to Parkinson’s syndrome in its outer form but to Alzheimer’s disease under the microscope.

Progressive supranuclear palsy is a rare progressive disorder show similar symptoms to Parkinson’s and ALS.

The onset of ALS on progressive supranuclear palsy patients is so subtle that the symptoms are incorrectly diagnosed for other conditions. However, since this is a progressive condition, the symptoms develop and grow with more weakness or gradually decline ineffectiveness.

Amyotrophic lateral sclerosis (ALS) is a rare neurological disease wherein you lose the ability to eat, walk, swallow, and eventually experience difficulties in breathing. The life expectancy in these cases is nearly 2-4 years. There is increasing evidence that supports common pathophysiology between ALS, and progressive supranuclear palsy. This is due to the mutation of the tau protein and has demonstrated overlapped presentations. Clinical theories state that incorporating clinical, pathological, and genetic analyses are crucial to have a better result and to determine the underlying pathophysiology.

In addition to progressive supranuclear palsy, there is a heterogeneous group of neurodegenerative disorders exemplified by the atypical metabolic rate of misfolded tau protein. This includes CBD and Alzheimer’s and hence they are termed as Tauopathies which results in progressive cell death.2,3

References:

  1. “Progressive Supranuclear Palsy Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, www.ninds.nih.gov/Disorders/patient-caregiver-education/fact-sheets/Progressive-Supranuclear-Palsy-fact-sheet.
  2. “Amyotrophic Lateral Sclerosis-Progressive Supranuclear Palsy Phenotype with Negative C9ORF72 Mutation.” MDS Abstracts, www.mdsabstracts.org/abstract/amyotrophic-lateral-sclerosis-progressive-supranuclear-palsy-phenotype-with-negative-c9orf72-mutation/.
  3. JC. Steele, JC. Richardson, et al. “Co-Morbidity of Progressive Supranuclear Palsy and Amyotrophic Lateral Sclerosis: a Clinical-Pathological Case Report.” BMC Neurology, BioMed Central, 1 Jan. 1964, bmcneurol.biomedcentral.com/articles/10.1186/s12883-019-1402-7.

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