Progressive Supranuclear Palsy
Progressive supranuclear palsy (PSP) is a rare degenerative disorder of the brain.
It destroys nerve cells in the parts of the brain that control eye movements, breathing and muscle coordination.
The nerve cell loss causes palsy or paralysis that slowly gets worse as the disease progresses. The palsy affects your ability to move the eyes, relax the muscles and control balance.
The major problems faced by patients of PSP include difficulty in swallowing, mood changes and sleep disturbances. The sleep disturbances associated with progressive supranuclear palsy include insomnia, frequent awakenings, sleep apnea, periodic limb movement disorder, REM behavior disorder and Hypersomnia.
The cause of progressive supranuclear palsy is not known. In few cases, the disease runs in families.
Progressive supranuclear palsy symptoms:
The symptoms of progressive supranuclear palsy usually appear slowly. Many people experience symptoms such as headaches, joint pains, fatigue, dizziness and depression.
- Frequent falls and clumsiness
- Very slow movements
- Inappropriate laughing or crying
- Memory loss and forgetfulness
- Unexplained balance problems
- Slurred speech
- Inability to hold urine in late stages
- Swallowing problems
- Stiff or awkward steps while walking
Sleep disturbances can be seen with PSP and this unusual sleep activity can be confusing and cannot mention to the family and spouse. It is important for the patient to undergo a sleep study to characterize the sleep disturbance.
Progressive supranuclear palsy diagnosis:
Diagnosis can be made by the doctor with the help of interview. You will be asked questions about when the symptoms have appeared, any other disorders are present, your medical problems, medications and about family medical history.
Multiple strokes or abnormal accumulations of fluid within the skull can cause balance problems. Magnetic resonance imaging (MRI) scans of the brain are needed to rule out these conditions.
Neuropsychological testing helps to determine person’s cognitive problems and strengths. It evaluates appearance, mood, anxiety level and experience of delusions or hallucinations.
This test assesses cognitive abilities such as memory, attention, orientation, use of language and ability to carry out various tasks and follow instructions. To observe the sleep patterns, a sleep test called polysomnography is conducted.
Progressive supranuclear palsy treatment:
Only few drugs have been found to have any effect on the symptoms of progressive supranuclear palsy. Drugs that show effect are L-dopa, amitriptyline, amantadine, desipramine and yohimbine. But, none of these drugs provide long lasting improvements and each drug can cause serious side effects.
For people with REM sleep behavior disorder, small amount of drug called clonazepam can be helpful. For sleep apnea, varieties of drugs are of some benefit. In severe cases, an apparatus that opens up the airway by means of continuous positive airway pressure (CPAP) can result in improved sleep.
Speech therapy can manage the swallowing and speech difficulty in progressive supranuclear palsy. Home environment should be modified to prevent injury from falls. Handrails should be installed in the bathroom. Dry eyes from infrequent blinking can be treated with drops and ointments.