Progressive supranuclear palsy is a rare disease that gradually destroys nerve cells in the parts of the brain that control eye movements, respiring, and muscle coordination. The loss of nerve cells causes palsy, or paralysis, that slowly worsens as the illness advances. The palsy has effects on ability to move the eyes, relax the muscles, and control balance.

Progressive supranuclear palsy is a disease of middle age. Symptoms generally begin in the 60s, rarely before age 45 or after age 75. Men develop PSP more often than girls do. It is affecting three to four people per million each year.

Progressive supranuclear palsy prognosis: is affecting the brainstem, the fundamental ganglia, and the cerebellum. The brainstem is located at the top of the spinal nerve. It controls the most elementary functions needed for survival-the involuntary ( unwilled ) movements like respiring, blood pressure, and heartbeat rate. The brainstem has 3 parts : the medulla oblongata, the pons, and the midbrain. The parts influenced by PSP are the pons, which controls facial nerves and the muscles that turn the eye outward, and the midbrain, the visible center. The fundamental ganglia are islands of nerve cells found deep in the brain. They are involved in the initiation of voluntary ( willed ) movement and control of emotion. Damage to the basal ganglia causes muscle stiffness ( spasticity ) and tremors. The cerebellum is found at the base of the skull. It controls balance and muscle coordination.

Vision is controlled by groups of cells called nuclei in the brainstem. In PSP, the nuclei continue to function, but the mechanisms that control the nuclei are demolished. The term supranuclear means that the damage is done above ( supra ) the nuclei. Patients with PSP have trouble with voluntary ( willed ) eye movement. Initially, the issue only happens in trying to look down. As the illness progresses, ability to move the eyes right and left is also influenced. However reflex or unwilled eye movements remain standard. Thus, when the patient’s head is tilted upwards, the eyes move to look down. These reflex movements remain standard till late in the course of the disease. The upper eyelids could be pulled back, the eyebrows raised, and the brow wrinkled, causing a standard wide-eyed stare. Rate of blinking may fall from the normal 20-30 per minute to three to 5 per minute. It becomes tough to walk downstairs, to maintain eye contact during conversation, or to move the eyes up and down to read.
The earliest evidence of PSP might be frequent falls or stiff, slow movements of the legs and arms. These symptoms may appear as much as five years before the characteristic vision Problems. Walking becomes very clumsy, and some patients have a tendency to lean and fall backward. Facial muscles might be puny, causing slurred speech and difficulty swallowing. Sleep may be troubled and thought processes slowed. Although memory remains intact, the slowed speech and thought patterns and the rigid countenance could be mistaken for senile dementia or Alzheimer’s disease. Emotional responses may become exaggerated and unfit, and the patient may experience anxiety, depression, and agitation.

The cause of PSP isn’t known. Most people who develop PSP come from families with no history of the illness, so it doesn’t seem to be inherited, except in certain rare instances. People who have PSP seem to lack the neurotransmitters dopamine and homovanillic acid in the fundamental ganglia. Neurotransmitters are chemicals that help carry electric impulses along the nervous system. Broadcasting structures in brain cells called neurofibrils become disorganized ( neurofibrillary tangles ). Neurofibrillary tangles are also found in Alzheimer’s illness, but the pattern is rather different. Check out also cerebral palsy info.