Progressive supranuclear palsy is also called Steele-Richardson-Olszewski syndrome. (1)
The only proven risk factor for progressive supranuclear palsy is age. The condition typically affects people around the age of 60.(1)
What Are The First Symptoms Of Progressive Supranuclear Palsy?
Progressive supranuclear palsy is a condition that produces a lot of symptoms but is mostly related to the movement of body parts. This occurs because it is associated with a lesion at the site of basal ganglion which is the control and refining center for the movements taking place other than the movements done by pyramidal tracts, hence also known as extrapyramidal movements(2).
The most common symptom of presentation of progressive supranuclear palsy is a defect in eye movements. It may present in the form of gaze disorder or ophthalmoplegia. The downward gaze is the earliest to get involved followed by upward gaze palsy. It creates difficulty in seeing downwards and the patient presents with a history of frequent falls/difficulty in walking.
The next cardinal feature of progressive supranuclear palsy is pseudobulbar palsy. In this, there is paralysis of ninth, tenth, and eleventh cranial nerve areas but these are supranuclear in origin, which means the palsy is characterized by rigidity and upper motor neuron type. It leads to increased gag reflex and complaints of dysphagia by the patient.
Another important feature of progressive supranuclear palsy is prominent neck dystonia. In this, there is a sustained contraction of the neck muscles to an extent that it becomes painful and the patient is unable to change the position.
Few other symptoms which usually get unnoticed in such patients are gait disturbance, behavioral abnormalities, personality changes, memory impairment, etc.
These are present in this condition is associated with other related conditions or syndromic association such as corticobasal degeneration etc.(3)
How Do You Test For Progressive Supranuclear Palsy?
Most of the patients of progressive supranuclear palsy present at the age of more than 60 years because it is a degenerative disease and worsens with age. The diagnosis of this condition is made by clinical examination generally. The first step is the thorough history taking for the symptoms and the sequence of presentation. It is very helpful to ask the family history because it is also known to consist of genetic components.
The next step is the complete physical examination of the individual. Looking for the signs related to the symptoms of presentation such as loss of gaze, exaggerated gag reflex, powerful and sustained neck muscle contraction, etc.
To confirm the diagnosis of progressive supranuclear palsy radiological, as well as biochemical examination, can be used. The radiological examination includes magnetic resonance imaging which can show non-characteristic but specific changes in the grey matter of the brain. The biochemical examination includes cerebrospinal fluid investigations especially for the abnormal tau proteins which are the core proteins responsible for this condition. This investigation’s sensitivity can be increased by the use of polymerase chain reaction which can amplify the abnormal proteins and increase its detection rate. It is also almost diagnostic for this condition along with the presence of clinical features but not singularly because it can represent in other degenerative brain conditions also.
Progressive supranuclear palsy is a progressive and degenerative brain condition that can produce a variety of symptoms depending upon the site of involvement where the abnormal proteins have deposited. The most common symptom is the gaze abnormality especially the download gaze and history of recurrent falls in association with it. Many of the symptoms such as behavioral change, personality artifacts can go unnoticed because these are present in almost all the individuals of the elderly age group.
Although biochemical tests can be done for confirming the diagnosis is made usually on the clinical features only.
- Does Progressive Supranuclear Palsy Run In Families & Is It Worse Than Parkinson’s?
- What Leads To Progressive Supranuclear Palsy & Can It Be Cured?
- Can Progressive Supranuclear Palsy Go Away On Its Own & What Are Its Natural Remedies?
- Is Progressive Supranuclear Palsy A Progressive Disease & Alternative Treatments For It?
- What Happens To Untreated Progressive Supranuclear Palsy & When To Go To Doctor For It?
- How Long Will It Take To Recover From Progressive Supranuclear Palsy & How Long Does The Symptoms Last?
- What Are The Ways To Prevent Progressive Supranuclear Palsy & Does It Reoccur?