Parkinson’s Plus Syndromes

Question 1

What is multiple system atrophy?

Answer 1

Multiple system atrophy (MSA) is a rare neurodegenerative disorder, caused by cell loss in certain areas of the brain and the spinal cord, leading to a variety of symptoms affecting especially the functions of the autonomic nervous system and the motor system.

Question 2

What is the classification of MSA?

Answer 2

There are 2 predominant types of multiple system atrophy

• MSA-P - Predominant Parkinsonian features
• MSA-C - Predominant Cerebellar features

Shy-Drager syndrome is a type of multiple system atrophy- not used anymore as almost every patient is affected by autonomic or urinary dysfunction.

Question 3

What are the clinical features of MSA?

Answer 3

Parkinsonism
Autonomic disturbance
-Erectile dysfunction: often an early feature
-Postural hypotension
-Atonic bladder
Cerebellar signs

Question 4

What is the management of MSA?

Answer 4

Currently, no therapy can reverse or halt progression of the disease. Management is symptomatic and targets Parkinsonism and autonomic failure.

Question 5

What is progressive supranuclear palsy?

Answer 5

Progressive supranuclear palsy (PSP) is a neurodegenerative syndrome which was first described in 1964.

It affects cognition, eye movements and posture. Characteristics include supranuclear, primarily vertical, gaze dysfunction accompanied by extrapyramidal symptoms and cognitive dysfunction. The cause is unknown.

Question 6

What are the clinical features of PSP?

Answer 6

postural instability and falls
-Patients tend to have a stiff, broad-based gait
Impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
Parkinsonism
-Bradykinesia is prominent
Cognitive impairment
-Primarily frontal lobe dysfunction