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

Progressive supranuclear palsy is a disease of middle age. Symptoms usually begin in the 60s, barely before age 45 or after age seventy five. Men develop PSP more often than ladies do. It affects 3 to four folks per million annually.

Prognosis for supranuclear palsy: is affecting the brainstem, the fundamental ganglia, and the cerebellum. The brainstem is found at the head of the spinal cord. It controls the most elementary functions required for survival-the involuntary ( unwilled ) movements such as respiring, blood pressure, and pulse rate. The brainstem has three parts : the medulla oblongata, the pons, and the midbrain. The parts affected by PSP are the pons, which controls facial nerves and the muscles that turn the eye outward, and the midbrain, the visual center. The basal ganglia are islands of nerve cells located deep in the brain. They’re concerned in the initiation of voluntary ( willed ) movement and control of emotion. Damage to the basal ganglia causes muscle rigidity ( 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 annihilated. The term supranuclear means that the damage is done above ( supra ) the nuclei. Patients with PSP have problems with voluntary ( willed ) eye movement. Initially, the problem only happens in trying to look down. As the disease advances, capability to move the eyes right and left is also affected. However reflex or unwilled eye movements remain standard. Thus, when the patient’s head is leaned upwards, the eyes move to look down. These reflex movements remain ordinary until late in the course of the illness. The higher eyelids might be pulled back, the eyebrows raised, and the brow wrinkled, causing a normal wide-eyed stare. Rate of blinking may decline from the normal 20-30 per minute to three to five 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 arms and legs. These symptoms may appear as much as five years before the characteristic vision issues. Walking becomes very ungainly, and some patients have a tendency to lean and fall backward. Facial muscles might be weak, causing slurred speech and difficulty swallowing. Sleep may be disturbed and thought processes slowed. Although memory remains intact, the slowed speech and thought patterns and the rigid facial expression may be mistaken for senile dementia or Alzheimer’s illness. Emotive reactions may become exaggerated and unbecoming, and the patient may experience anxiety, depression, and agitation.

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