Introduction
Progressive supranuclear palsy is an uncommon brain disorder that causes serious problems with walking, balance and eye movements, and later with swallowing. The disorder results from deterioration of cells in areas of brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy is also called Steele-Richardson-Olszewski syndrome.
Progressive supranuclear palsy worsens over time and can lead to life-threatening complications, such as pneumonia and swallowing problems. There’s no cure for progressive supranuclear palsy, so treatment focuses on managing the signs and symptoms.
Signs & symptoms
The characteristic signs and symptoms of progressive supranuclear palsy include:
A loss of balance while walking especially a tendency to fall backwards
An inability to aim your eyes properly causing poor eye contact or spillage of drinks during conversation
Late signs and symptoms of progressive supranuclear palsy vary and may mimic those of Parkinson’s disease and dementia. They generally get worse over time and may include:
- Stiffness and awkward movements
- Falling
- 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
The cause of progressive supranuclear palsy isn’t known.
Deterioration of cells in certain areas of brain, that control body movements and thinking is present. These cells are found to have abnormal amounts of a protein called tau. Clumps of tau are also found in other neurodegenerative disorders, such as Alzheimer’s disease.
The only proven risk factor for progressive supranuclear palsy is age.
Pathophysiology
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, some rare familial clusters have been reported. 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.
Subcortical white matter showing tau-positive perinuclear glial inclusions.
Subcortical white matter showing tau-positive perinuclear glial inclusions.
Abnormality of tau protein
PSP has been considered to be a tau protein disorder. Cortical fibrillary tangles of PSP are similar to those observed in Alzheimer disease with regard to the presence of an abnormally phosphorylated tau protein. Tau is a component of a microtubule-associated protein that is responsible for axonal transport of vesicles. The mechanism whereby this is involved in PSP has yet to be determined. PSP overlaps with corticobasal degeneration (CBD) in this regard, and the latter may have a stronger association with tau protein abnormalities than does PSP.
Tau proteins exist in 6 isoforms encoded by a single gene. Different electrophoretic patterns have been identified in the various disorders associated with tau abnormalities. Thirty-two mutations have been identified in more than 100 families. About half of the known mutations have their primary effect at the protein level. They reduce the ability of tau protein to interact with microtubules and increase its propensity to assemble into abnormal filaments. The other mutations have their primary effect at the RNA level and perturb the normal ratio of 3-repeat to 4-repeat tau isoforms. When studied, this change resulted in a relative overproduction of tau protein with 4 microtubule-binding domains in the brain.
Different patterns of abnormal Tau protein have been found in Alzheimer’s disease, PSP, Pick disease , and frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). The chromosomal region containing MAPT has been shown to evolve into 2 major haplotypes, H1 and H2. The more common haplotype, H1, is overrepresented in patients with PSP and CBD.
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
There is no cure for progressive supranuclear palsy. Treatments are available to help ease symptoms of the disorder. They include:
- Parkinson’s disease medications – which increase levels of a brain chemical involved in smooth, controlled muscle movements. The effectiveness of these medications is limited and usually temporary, lasting about two to three years.
- Botox therapy – which may be injected in small doses into the muscles around eyes. Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms.
- 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
Patients with PSP tend to have progressive deterioration. Confinement to bed or a wheelchair is typically necessary within 8 years. Eventual death usually follows a severe fall, pulmonary embolus, or aspiration pneumonia.
Complications
Complications of progressive supranuclear palsy result primarily from slow and difficult muscle movements. These complications may include:
- Falling, which could lead to head injuries, fractures and other injuries
- Difficulty focusing the eyes, which also can lead to injuries
- Difficulty sleeping
- Difficulty looking at bright lights
- 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
Disease & Ayurveda
Clinical manifestations of progressive supranuclear palsy happens when there is Vaatakopa which deviates stability. It is seen mostly in Vaata prakruti people and old age.
Nidana
Physical
Dry, cold and old food items
Excess travelling & exertion
Trauma/injury
Exposure to wind
Loss of sleep
Psychological
Trauma or grief
Fear
Anxiety
Stress
Either dhaatukshaya(degeneration of body tissues) or aavarana (obstruction in body channels) will block or redirect Vaata from its normal path.
Purvaaroopa
Not mentioned
Samprapti
Due to the causative factors, Vaatadosha vitiates and move irregularly all over the body and cause imbalance in circulation & metabolism. When it enters the nervous & locomotor system, and gets lodged there, the disease is developed.
Lakshana
Instability in thoughts & opinions
Fear
Lack of confidence
Weakness of body
Deterioration of muscles
Tremors
Loss of balance & coordination
Confusion
Disturbed sleep
Divisions
Not mentioned
Prognosis
Saadhya if it is a new case developed in young healthy persons without any complications. Asaadhya or yaapya in others.
Chikithsa
Ayurvedic treatment for this disease mainly aims on balancing the deranged Vaatadosha. In niraama condition, brumhana therapy is advised. In cases with aama, first aamapaachana and then agnideepana should be done before strating brumhana therapy.
Samana
Abhyangam
Moordhapichu
Brumhanam
Sodhana
Snehapanam
Swedanam
Snehavasti
Kashayavasti
Commonly used medicines
Mridweekadi kashayam
Brahmeedrakshadi kashayam
Drakshadi kashayam
Brahmarasayanam
Chyavanaprasam
Kallyanakaghrutam
Saaraswataghrutam
Yogendra Ras
Brands available
AVS Kottakal
AVP Coimbatore
Vaidyaratnam oushadhasala
SNA oushadhasala
Home remedies
Practising meditation and auto-suggestion techniques at home
Diet
- 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
Behaviour:
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 body to be in normal temperature.
Yoga
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 bioavailability of the medicine and food ingested and leads to positive health.
Yoga can maintain harmony within and with surroundings.
Suryanamaskara
Pavanamuktasana
Vajrasana
All the exercises and physical exertions must be decided and done under the supervision of a medical expert only.
Research articles
https://pubmed.ncbi.nlm.nih.gov/29455271/
https://pubmed.ncbi.nlm.nih.gov/10824045/
These statements have not been evaluated by the Food and Drug Administration, United States. This product is not intended to diagnose, treat, cure or prevent any disease. Please consult your GP before the intake.
Writer:
Dr. Rajesh Nair, the co-founder and chief consultant of Ayurvedaforall.Com, is a graduate of prestigious Vaidyaratnam Ayurveda College (affiliated with the University of Calicut), Kerala, India. Additionally, he holds a Postgraduate Diploma in Yoga Therapy from Annamalai University.
Dr. Nair offers consultation at two busy clinics in and around Haripad, Alleppey, Kerala, the southern state famous worldwide for authentic ayurvedic treatment and physicians. While offering consultation on all aspects of ayurvedic treatments Dr. Nair has a special interest in Panchkarma, Yoga, and Massage.
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