How To Diagnose Progressive Supranuclear Palsy & What Is The Best Medicine For It?

The first clinicopathologic descriptions of progressive supranuclear palsy (PSP) were published in 1963 and 1964.(1)

The most common symptoms at disease onset were postural instability and falls (63%), dysarthria (35%); bradykinesia ( 13% ).(1)

How To Diagnose Progressive Supranuclear Palsy?

Progressive supranuclear palsy is a degenerative condition for which the diagnosis making is clinical. It is because there is no specific test present that can confirm as well as specify the diagnosis of PSP at the same time. How to make the diagnosis on clinical features, the hallmark symptom of PSP such as supranuclear ophthalmoplegia, downward gaze palsy of the eye should be present along with the supporting history such as presence of frequent falls, abnormal and wide gait, inability to see the path clearly, etc. Other clinical features such as neck dystonia, pseudobulbar palsy may also be present which can help in further strengthening the diagnosis.(2)

Once the diagnosis is made by the physician based on clinical features, it can be supported and specified with the help of radiological, biochemical, and histological examination. In the radiological examination, MRI and SPECT can be performed which may show brain atrophy, periaqueductal grey matter changes, etc.

Biochemical examination of the cerebrospinal fluid could also help detect the protein levels especially tau proteins which are confirmatory once the clinical diagnosis is made. The most specific and sensitive test for the supporting diagnosis of progressive supranuclear palsy is a histological examination of the brain tissue. It is used for the detection of neurofibrillary tangles, cerebral plaques in the brain tissue which gets accumulated due to abnormal tau proteins and increases with age.

What Is The Best Medicine For Progressive Supranuclear Palsy?

To start the patient on any medication, it is very important to exclude the differential diagnosis of this condition especially such as parkinsonism which is a fairly treatable disease. Although there has been widespread research upon this condition but a complete and permanent cure has not been found yet. Many of the cases respond to various dopaminergic and anticholinergic agents but do so for a short duration only. There have been no drug developed which can stop the progression of this condition and this condition is fairly an untreatable one.

Few groups of drugs can be used such as dopamine agonists like bromocriptine, cabergoline, pramipexole, ropinirole, etc., anticholinergic agents like trihexyphenidyl, benztropine, etc., tricyclic antidepressants amitriptyline, nortriptyline, methysergide, etc.(3)

In a few of the cases, where the pharmacological therapy has faltered badly and there has been no symptomatic relief to the progressive supranuclear palsy patient with symptoms seriously damaging the quality of life, there has been advocation for the usage of electroconvulsive therapy. Although only indirect electroconvulsive therapy which is given under the effect of anesthesia is allowed.(4)

Conclusion

Like most of the other degenerative conditions especially involving brain and spinal cord, progressive supranuclear palsy is also progressive with limited modes of diagnosis and treatment. Specific diagnosis and complete treatment are not possible in any of the cases of this condition. Diagnosis making is most of the time, based on clinical examination and history. It can be adequately supported by radiological, biochemical, and histological evidence but it cannot be confirmed on such test reports because a variety of other diseases also consists of a similar presentation on these tests.

After the recent advances in the field of pharmacology, these degenerative conditions have become a new taste for the neurologists. Scientists have been trying to completely stop the progression of this condition but have not found success yet. Drugs like dopamine agonists can provide relief on a short term basis.

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