Progressive supranuclear palsy is a rare disease that steadily 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 moves on. 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 frequently than women do. It has effects on three to 4 folks per million each year.
Prognosis for progressive supranuclear palsy: is affecting the brainstem, the basal ganglia, and the cerebellum. The brainstem is found at the apex of the spinal nerve. It controls the most simple functions required for survival-the involuntary ( unwilled ) movements such as breathing, 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 visual center. The fundamental ganglia are islands of nerve cells found deep within 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 shocks. The cerebellum is located 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. At first, the issue only happens in making an attempt to look down. As the disease progresses, capability to move the eyes right and left is also affected. However reflex or unwilled eye movements remain ordinary. Thus, when the patient’s head is tilted upwards, the eyes move to look down. These reflex movements remain standard until late in the course of the illness. The higher eyelids might be pulled back, the eyebrows raised, and the brow wrinkled, causing a typical wide-eyed stare. Rate of blinking may fall from the normal 20-30 per minute to 3 to 5 per minute. It becomes difficult to walk downstairs, to maintain eye contact during conversation, or to move the eyes up and down to read.
The earliest symptoms of PSP might be frequent falls or stiff, slow movements of the arms and legs. These symptoms may appear as much as 5 years before the characteristic vision issues. Walking becomes very clumsy, and some patients have a tendency to lean and fall backward. Facial muscles may 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 facial expression may be mistaken for senile dementia or Alzheimer’s illness. 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 disease, 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 basal 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 disease, but the pattern is somewhat different. Check out also cerebral palsy information.