Progressive Supranuclear Palsy (PSP) is caused by the progressive death of nerve cells in the brain, leading to difficulty with balance, movement, vision, speech and swallowing.
- Progressive – it gets steadily worse over time
- Supranuclear – it damages parts of the brain above the pea-sized ‘nuclei’ that control eye movements
- Palsy – it causes weakness
Signs and Symptoms
It is important to know that PSP does not affect everybody in the same way. Early symptoms may include loss of balance and unexpected falls (usually backwards), stiffness and eye problems. These can include difficulties in looking up or down, focusing, double or tunnel vision and dislike of bright lights. Some people can experience behavioral and cognitive changes – depression, apathy, clumsiness, or tiny, cramped handwriting.
The voice can become soft and lack volume, speech and swallow problems can develop as the condition progresses. In some cases there are inappropriate emotional outbursts such as laughing or crying. Falls become more frequent with time. Incontinence can be present.
Diagnosis and Treatment
PSP is difficult to diagnose because of the complexity of the disease and variation in symptoms. Early on, symptoms may resemble those of other neuro-degenerative diseases such as Parkinson’s disease, Alzheimer’s disease, Motor Neuron disease or Multiple System Atrophy. As a result many people are initially misdiagnosed.
PSP is diagnosed by clinical observation and by excluding other conditions. On referral to hospital clinical assessment involves a battery of blood tests, MRI, scans, psychological, motor, and neurological tests to rule out Alzheimers, Motor Neuron Disease, Stroke, tumor, and other conditions. Motor problems may present as Parkinson’s disease so to rule out this patients are put on Levadopa. Parkinson’s disease responds well to this drug.
There is no effective treatment for PSP so managing symptoms is key to controlling the progression of this disease. Dopamine levels in the brain are diminished in PSP so Levadopa along with other drugs are usually prescribed to improve balance and relieve stiffness. Extra nutrient treatments may be necessary at different stages. In general laxatives are prescribed for constipation and to ensure that there is good drug absorption for control of symptoms. Antidepressants may be prescribed short term. Prism glasses and botulism injections may be used for eye problems. While PSP is not life threatening in itself swallowing difficulties increases the risk of choking and aspiration pneumonia. Regular exercise and good diet is necessary..
It is also important to see a neurologist with experience of PSP to get a definite diagnosis. PSP is complex; persons with PSP require a multidisciplinary team to provide the necessary care.
Having a diagnosis of PSP is emotionally upsetting, you will need time to get used to the diagnosis. Family support will be very important at this stage and throughout all stages of the condition. You will also need advice, information, and support.
PSPA Ireland provides information advice and support group meetings for patients, carers and families whose life is affected by Progressive Supranuclear Palsy.
See Contact Us.