Parkinsonism-plus. Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is one of the most severe diseases in the parkinsonism-plus group.

I will remind you why these diseases are classified into a separate group and why they are called that. They belong to neurodegenerative diseases (like Parkinson’s disease and Alzheimer’s disease), meaning there is continuously progressive degeneration (atrophy) of brain cells in the brain. The cause is the accumulation of specific proteins, alpha-synuclein (as in the case of Parkinson’s disease, for example), or tau protein (as in the case of PSP).

«”Parkinsonism-plus” – because neurons die almost simultaneously in several different areas of the brain. This leads to the almost simultaneous appearance of several different symptoms and syndromes (in addition to the parkinsonism syndrome, as in the case of Parkinson’s disease). For example, speech disorders, swallowing difficulties and choking while eating, instability when walking and early falls, eye movement disorders, memory decline, and early development of dementia.

Compare – in Parkinson’s disease, for a long time, the only pronounced manifestation remains parkinsonism syndrome (slowness, muscle stiffness, resting tremor). Other symptoms (dementia, falls, swallowing and speech problems), if they appear, occur much later.

The problem is that often in the first 1-2 years of the development of any neurodegenerative disease, we can never be sure of the diagnosis, and frequently, over time, new symptoms appear that may lead to reconsidering the diagnosis towards another, more malignant and aggressively progressing disease from the parkinsonism-plus group.

The main, frequently occurring symptom of PSP is impaired eye movement. The patient finds it difficult to move their eyes, especially up and down. This becomes noticeable with the forced position of the head and neck (with the nose slightly raised upwards) and the patient’s particular facial expression (with eyebrows raised as if surprised, and infrequent blinking). To look to the sides or upwards, the patient has to turn their head, which is always difficult with PSP –   because due to pronounced stiffness in the neck muscles, turning the head becomes very difficult

Unfortunately, treatment for progressive supranuclear palsy does not always bring clinical effect and improvement. This is because dopaminergic deficiency is not predominant in the pathogenesis of this disease, unlike Parkinson’s disease. Therefore, antiparkinsonian tablets, if they help, have a small effect and more often require large doses. Therefore, the general recommendation for treating PSP is to use the maximum tolerated doses of the most effective drug (levodopa)

Therefore, clinical rehabilitation and identification/treatment of all symptoms are very important in the management of a patient with Parkinson’s disease. This means preventing hypodynamia and stiffness, physical therapy (active and passive), massage, fall prevention, and the use of rehabilitation aids (walkers, canes, chairs, handrails), diet therapy, speech therapy, and much more.

Stay healthy!

Gulnara Nailevna Akhmadeeva,
PhD in Medicine,
neurologist specializing in Parkinson’s
V.S. Buzaev International Medical Centre