Progressive supranuclear palsy is a rare disorder of the brain that can cause serious issues with walking, balancing, coordination and eye movements, and important functions like swallowing will be difficult in later stages. The disorder results from the deterioration of cells in areas of the brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy is otherwise known as Steele-Richardson-Olszewski syndrome. This is a progressive disorder that worsens over time and can lead to life-threatening complications, including pneumonia and swallowing problems. A permanent cure for progressive supranuclear palsy has not been found yet, treatment focuses on the management of the signs and symptoms.
Signs & symptoms of Supranuclear Palsy
It starts as a loss of balance while walking especially a tendency to fall backwards. There will be difficult to focus the eyes properly, causing poor eye contact or spillage of drinks during the conversation.
Late stages show varied manifestations, sometimes resembling Parkinson’s disease and dementia. They get worse over time in most cases and may include:
- Stiffness and awkward movements
- Problems with speech and swallowing
- Sensitivity to light
- Sleep disturbances
- Loss of interest in pleasurable activities
- Impulsive behaviour, tantrums possibly including laughing or crying for no reason
- Difficulties with memory, reasoning, problem-solving and decision-making
- Depression and anxiety
- A surprised or frightened facial expression, resulting from rigid facial muscles
Causes of Supranuclear Palsy
The exact cause of progressive supranuclear palsy is not yet known.
Deterioration of cells in certain areas of the brain, that control body movements and thinking is present. These cells are found to have abnormal amounts of a protein called Tau, the same as in other neurological conditions Alzheimer’s disease etc. Age is a risk factor for PSP.
Pathophysiology of Supranuclear Palsy
Progressive Supranuclear Palsy (PSP) is reportedly the second most common cause of idiopathic parkinsonism. Generally, there is no family history and no strong genetic component is known in this idiopathic condition. However, family history is reported in rare cases. The disease usually begins when patients are in their 50s to mid-60 s.
PSP is associated with neuronal loss, gliosis, and neurofibrillary tangles in the pretectal area, substantia nigra, subthalamic nucleus, globus pallidus, superior colliculus, and substantia innominata. Degeneration of multiple neurotransmitter systems leads to a more diffuse disorder than idiopathic Parkinson’s disease. The cholinergic and adrenergic systems are involved in addition to the dopaminergic system. Tau-positive glial inclusions are a consistent pathologic finding. Coiled bodies, which are small round cells of oligodendrocytic origin found in white matter, are also seen in a widespread distribution.
PSP is known to be a tau protein disorder. Tau is a component of a microtubule-associated protein, responsible for the axonal transport of vesicles. The mechanism whereby this is involved in PSP is not yet understood clearly.
Ruling out Parkinson’s disease is very important. Diagnosis of PSP is made rather than Parkinson’s disease if you experience:
- A lack of shaking
- A poor or no response to Parkinson’s medications
- Difficulty moving your eyes, particularly downward
An MRI and a positron emission tomography (PET) scan are also recommended in most cases
Treatments for Supranuclear Palsy
There is no cure for progressive supranuclear palsy. Treatment options are administered to help ease discomforts due to the disorder. They include:
- Parkinson’s disease medications – which increase levels of a brain chemical involved in smooth, controlled muscle movements. These medications have limited effects which are usually temporary.
- Botox therapy – which may be injected in small doses into the muscles around the eyes. Botox treatment stops the chemicals signalling the muscles to contract and can improve spasm in the eyelids.
- Eyeglasses with bifocal or prism lenses, which may help ease problems with looking downward.
- Speech and swallowing evaluation to learn safer swallowing techniques.
- Physical therapy and occupational therapy to improve balance.
Prognosis of Supranuclear Palsy
Patients with PSP tend to have progressive deterioration. In most cases, the patient is confined into a bed or a wheelchair within 8 years. Death may be due to a severe fall, pulmonary embolus, or aspiration pneumonia.
Complications of Supranuclear Palsy
Complications of progressive supranuclear palsy happen due to slow and difficult muscle movements in most cases. These complications may include:
- Falling, which could lead to head injury, fractures and other serious injuries
- Difficulty in focusing the eyes, which also can lead to injuries
- Difficulty in sleeping
- Difficulty looking at bright lights
- The problem in swallowing, which can lead to choking or inhaling food or liquid into the airway (aspiration)
- Pneumonia, which can be caused by aspiration which can be fatal
- Impulsive behaviours and tantrums
Ayurvedic Concept of Supranuclear Palsy
Clinical manifestations of progressive supranuclear palsy happen when there is Vaatakopa which deviates stability. It is seen mostly in Vaata prakruti people and old age.
Nidana of Supranuclear Palsy
Dry, cold and old food items
Excess travelling & exertion
Exposure to wind
Loss of sleep
Trauma or grief
Either dhaatukshaya(degeneration of body tissues) or aavarana (obstruction in body channels) will block or redirect Vaata from its normal path.
Purvaaroopa of Supranuclear Palsy
Samprapti of Supranuclear Palsy
Due to the causative factors, Vaatadosha vitiates and move irregularly all over the body and cause an imbalance in circulation & metabolism. When it enters the nervous & locomotor system and gets lodged there, the disease is developed.
Lakshana of Supranuclear Palsy
Instability in thoughts & opinions
Lack of confidence
Weakness of body
Deterioration of muscles
Loss of balance & coordination
Divisions of Supranuclear Palsy
Prognosis of Supranuclear Palsy
Saadhya if it is a new case developed in young healthy persons without any complications. Asaadhya or yaapya in others.
Chikithsa of Supranuclear Palsy
Ayurvedic treatment for this disease mainly aims at balancing the deranged Vaatadosha. In niraama condition, brumhana therapy is advised. In cases with aama, first aamapaachana and then agnideepana should be done before starting brumhana therapy.
Commonly used medicines for Supranuclear Palsy
Home remedies for Supranuclear Palsy
Practising meditation and auto-suggestion techniques at home
Diet for Supranuclear Palsy
- To be avoided
Heavy meals and difficult to digest foods – cause indigestion.
Junk foods- cause disturbance in digestion and reduces the bioavailability of the medicine
Carbonated drinks – makes the stomach more acidic and disturbed digestion
Refrigerated and frozen foods – causes weak and sluggish digestion by weakening agni (digestive fire)
Curd – causes vidaaha and thereby many other diseases
- To be added
Light meals and easily digestible foods
Green gram, soups, buttermilk boiled with turmeric, ginger and curry leaves
Freshly cooked and warm food processed with cumin seeds, ginger, black pepper, ajwain etc
Better to avoid exposure to excessive sunlight wind rain or dust.
Avoid lifting heavy weights and other vigorous physical activities.
Maintain a regular food and sleep schedule.
Avoid bath in cold water immediately after exercise. Allow the body to be at normal temperature.
Yoga for Supranuclear Palsy
Stretching exercises, meditation for relaxation and following a specific Yogacharya with yoga asanas like suryanamaskara, pavanamuktasana, vajrasana, etc are recommended for improving circulation and digestion.
Regular exercise helps improve the bioavailability of the medicine and food ingested and leads to positive health.
Yoga can maintain harmony within and with surroundings.
All the exercises and physical exertions must be decided and done under the supervision of a medical expert only.
Research articles of Supranuclear Palsy