Progressive Supranuclear Palsy || When Tauopathy Causes Neurological Disoder

in StemSocial2 months ago

To Begin today, I will give two illustrations about a condition, then we are going to discuss this condition.

(i). A person who sits on a hospital bed, looking straight with his head straight, and he isn't blinking his eyes. This patient can move his eyeballs sideways to follow an object (for example; a pen), but cannot look upwards or downwards when the material is being moved across his face upwards and downwards. The eyes are able to move left and right, the head displays a stiff palsy where movement is impossible.

(ii). A person who is having difficulty checking his wristwatch to tell the time, because his head is stiff. When a second person help to flex the head downwards, the eyeball moves in the opposite direction looking upward (like that of a doll), preventing the patient from being able to look at the time.

pexels image ||

The two examples above discusses a condition expressed by patients with Progressive Supranuclear Palsy, First identified by Neurologists, J. Clifford Richardson, John C. Steele, and Jerzy Olszewski, in a manuscript published in 1964, titled Progressive Supranuclear Palsy - A Heterogeneous Degeneration Involving the Brain Stem, Basal Ganglia and Cerebellum With Vertical Gaze and Pseudobulbar Palsy, Nuchal Dystonia and Dementia.

Numbers and Clinical Manifestations Serve as Proof

An article titled Prevalence and natural history of progressive supranuclear palsy, published in 1988, which was a research done in New Jersey, showed that the prevalence ratio for Progressive Supranuclear Palsy, was 1.39/100,000. The research was carried out on 799,022 patients in chronic care facilities. In 1999, another research was carried out in the United Kingdom, showing a prevalence rate of 6.4 per 100,000 people. In recent time, psp association, there are about 4000 people living with Progressive Supranuclear Palsy, at any given time. Progressive Supranuclear Palsy (PSP) is usually common among the older population, within the ages of 50 and 89 years old. With less prevalence on people below the age of 40 years. Progressive Supranuclear Palsy is a familial disease, which can be found from one generation to another, or in relatives.

When it comes to symptomatology, patients with Progressive Supranuclear Palsy are reported to have Supranuclear opthalmoplegia / ocular motor dysfunction, Postural Instability (imbalance, walking difficulty, frequent fall), Idiopathic Parkinson’s disease, Frontal lobe cognitive dementia (Behavioral/cognitive impairment), Corticobasal Syndrome, Pseudobulbar Palsy (speech/language disorders), and Cervical Dystonia. These were confirmed in some cases via PSP autopsy.

A Little Pathophysiology Explains it

Progressive Supranuclear Palsy is clinical, and according to Neoropathology, patients suffer from PSP as a result of Taupathy. A condition where TAU protein is deposited in the neurons of the brain as well as in the Astrocyte of Glial cells in the brain. The amount of TAU in the area of the brain determines the severity, and the location where the protein is present determines the type of symptoms that the patients will exhibit. TAU can be deposited in the subthalamic nucleus found between the midbrain and diencephalon, globus pallidus, red nucleus, striatum of the basal ganglia, substantia nigra, dentate nucleus, pontine tegmentum in the brain stem, oculomotor nucleus, and medulla..

Tau is a protein that is known to bind the neurons of Microtubules, repeatedly in neurofibrillary tangles (4R-tau), thereby allowing them to meet and bind with other neurons. (NCBI). Basically, this neurological disorder is as a result of 4 repeat TAU (4R-TAU) deposition in the brain neuron, causing neuronal loss in the part of the brain. Progressive Supranuclear Palsy, may first look like Parkinsons disease but overtime, the syndrome begins to manifest properly.

Diagnosis Allows Easy Explanation to Patients

According to a 2017 study there are four functional domains when diagnosing Progressive Supranuclear Palsy. These domains are the degree of ocular dysfunction, Degree of Postural instability, Degree of Akinesia, and the Degree of cognitive dysfunction. Since Tau protein (4R-TAU) which are produced as a result of splicing of MAPT (microtubule-associated protein) gene, can be deposited at different part of the brain, different variants of PSP exists. These are PSP-Speech and Language (where speech is affected), PSP-Frontal, PSP-corticobasal syndrome, PSP-Progressive gait freezing which includes freezing while walking or doing activities, and PSP-Parkinsonism.

In All Fairness, There Should Be Treatments and Cure

You see, it isn't fair at this time, because Progressive Supranuclear Palsy cannot be cured, but it can be managed depending on the diagnosis. Medications such as Amantadine can be useful for patients experiencing reduced axial rigidity, and mobility. Also, drugs like Levodopa will be helpful for patients with akinetic rigidity (Motor symptoms). In other to manage this disease, a lot of medical practitioners including; Specialist nurses, General medical practitioners, Speech and Language therapist, physiotherapist, Dietician, and psychiatrists will be involved in helping.

Image 1 ||Pexels Image


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