Other Movement Disorders

Parkinson's and ParkinsonismProgressive Supranuclear PalsyMultiple System Atrophy

Parkinson’s is part of an umbrella of conditions known as Parkinsonism. The main symptoms of Parkinson’s are also the symptoms of a number of conditions which are grouped together under the term ‘Parkinsonism’. Parkinson’s is the most common form and is sometimes referred to as idiopathic Parkinson’s disease, which means its cause is not clearly known.

There are no special tests that can prove absolutely whether someone has a Parkinson’s or other Parkinsonism syndrome. This diagnosis is usually based on clinical examination and medical history.

People with typical Parkinson’s usually should show an initial good response to levodopa. People with other causes of Parkinsonism, on the other hand usually do not respond as well.

When this is the case, the terms ‘Parkinsonian syndrome’, ‘atypical Parkinsonism’ or ‘Parkinson’s plus’ may be used by the doctor. Symptoms that allow the doctor to make a specific diagnosis may only appear as the condition develops.

Parkinson’s Disease and Movement Disorder Society (PDMDS), India provides support and information for all people with Parkinsonism not only those with Parkinson’s, so people with an alternative diagnosis can join the society and use our services.

There are basically four broad categories of Parkinsonism.

Primary/Idiopathic Parkinson’s – This is the most common type of Parkinson’s. Here the causes of Parkinson’s are not known.

Secondary Parkinson’s – This type of Parkinson’s occurs as a side effect to certain drugs (prolonged use of medications for psychiatric disorders etc), certain toxins, after a head injury or brain tumor etc.

  • Drug – induced Parkinsonism
  • Post encephalitic Parkinsonism
  • Arterio scelerotic Parkinsonism

Hereditary Parkinson’s – In rare cases Parkinson’s can be passed on within families. Remember that simply having a family member with Parkinson’s does not mean that another family member will get Parkinson’s.

Parkinson – Plus Syndromes – Parkinson – plus syndromes are a group of neurological (brain) diseases that are similar to Parkinson’s and also have additional unique features that distinguish them from Parkinson’s.

These are

  • Progressive Supranuclear Palsy
  • Multiple System Atrophy – (now diagnosed as MSA-P and MAS-C) which include;
    – Striato Nigral Degeneration (SND),
    – Sporadic Olivo Ponto Cerebellar Atrophy (OPCA),
  • Cortico Basal Ganglionic Degeneration (CBGD)
  • Diffuse Lewy Body Disorder

Progressive Supranuclear Palsy, sometimes called as Steele-Richardson-Olszewski, is a rare degenerative neurological condition often presenting with similar symptoms to Parkinson’s. The condition is so named as it begins slowly and continues to get worse (i.e. progressive) and causes weakness (i.e. palsy) by damaging certain parts of the brain above pea-sized structures called nuclei that control eye movement. (i.e. supranuclear)

Key Features
PSP is usually seen in people between the ages of 50-60 or above. The most obvious sign of the disease is an inability to move the eyes properly, which occurs because of lesions in the area of the brain that coordinates eye movements. Such inability could appear as an inability or reduced ability to look up or down, light sensitivity, slow blinking of eyelids and difficulty maintaining eye contact. Some patients may describe this effect as a blurring. Patients also often show alterations of mood and behavior, including depression and apathy. Other key signs of PSP include, unsteady walking and frequent unexplained falls (mainly backward), stiffness of the neck and limbs, slow slurred and/or quiet speech, difficulty swallowing and a generalized slowness of movements. Sometimes mild dementia is also observed.

Diagnosing PSP
The diagnosis of PSP may be difficult. However, the key to establishing the diagnosis of PSP is the identification of early gait instability, with frequent backward falls and difficulty moving the eyes, which are considered the hallmark of the disease.

Causes of PSP
The cause of PSP is not known. Scientists have come up with several theories as to why the brain would degenerate in such a way. PSP could result from exposure to a virus that enters the body and then takes years to produce obvious effects or be the result of complex genetic mutations. None of these theories, however, have been proven.

Treatment for PSP
Although currently there are no specific treatments for PSP, when people with PSP present with Parkinson’s symptoms including tremors, slowness, stiffness, and balance problems they may respond in a moderate manner to anti-Parkinsonian treatment such as Levodopa. Another group of drugs that has some application in PSP treatment are antidepressant medications.

However, a variety of other forms of therapy can help control the symptoms of stiffness, imbalance, swallowing difficulties etc. A multi disciplinary team including; Physiotherapists, Speech therapists, Occupational therapists, Dieticians etc would benefit the person greatly as is seen with persons with Parkinson’s.

PSP does also predispose patients to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia), choking, head injury, and fractures caused by falls, thus requiring good attention to the medical and nutritional needs of the individuals to minimize such complications.

The diagnosis of PSP has significant impact not only on the person but also those close to the person with PSP. Progressive disability results in an increasing need for care and support for the person. However such a loss of independence can also be unsettling and challenging for the individual.
Caregivers and families too may feel stressed, frustrated and chronically tired thus making the condition harder to cope.

Support services for both the person with PSP and their caregivers is vital to uphold the quality of care, encourage communication and a positive attitude towards ones treatment and care, and also maintain the pleasurable nature of the relationship.

Multiple System Atrophy (MSA) is a group of related syndromes that make up a rare progressive neurological disorder presenting with similar symptoms to Parkinson’s. The condition is marked by a combination of symptoms affecting movement, blood pressure, and other body functions; hence the label Multiple System Atrophy.

MSA was the name for disorders once known individually as Striato Nigral Degeneration (SND), Sporadic Olivo Ponto Cerebellar Atrophy (OPCA), and the Shy-Drager Syndrome. Following a consensus conference in 1998, new diagnostic criteria were adapted. It was then recommended that patients be designated as having MSA- C if cerebellar features predominate and MSA-P if Parkinsonian features predominate, instead of using the earlier terms of SND and OPCA respectively. The term Shy- Drager syndrome was found to no longer be useful as autonomic failure is invariably present.

Key Features
MSA is characterized by symptoms of autonomic nervous system failure; such as lightheadedness, blurred vision or fainting spells due to a fall in blood pressure when standing (orthostatic hypotension), constipation, erectile failure in men, urinary retention and or loss of bowel/bladder control. However, autonomic dysfunction may not always be the presenting feature of MSA. Patients with MSA may also present with in coordination or difficulties with balance (called cerebellar ataxia) or early bulbar and swallowing difficulty.

These symptoms may be combined with tremor, slowed movements, rigidity, slurred speech, swallowing difficulties and loss of muscle coordination.

Some people with people with MSA may also experience depression and/or anxiety and may develop a dementia.

MSA affects both men and women, primarily in their 50’s, athough it has been seen to affect people younger and older than this as well. Progressive in its nature, the condition may progress swiftly or slowly. Most importantly however, every individual may experience a different pattern of signs and symptoms as is the case with Parkinson’s and other movement disorders.

Diagnosing MSA
The diagnosis of MSA is complicated due to the similarities in the nature of its initial symptoms with those of Parkinson’s. However its hallmark of symptoms related autonomic nervous system failure distinguishes it from Parkinson’s and other similar conditions.

Thus it is imperative that the diagnosis be made by a specialist, usually a Neurologist. Since there are no specific tests for the condition, a period of observation is often needed before making such a diagnosis.

Causes of MSA

There is no known cause of MSA. Extensive research in this area is underway; however no proven cause has yet been established.

Treatment for MSA
Currently, there is no specific treatment for MSA. However, anti-Parkinsonian drugs such as Levodopa, used to treat rigidity may offer some help. Orthostatic hypotension may be treated with fludro cortisone and other drugs that raise blood pressure.

A routine of stretching and exercise can help retain muscle strength and range of movement. Thus People with MSA, as is the case with Parkinson’s, may benefit from working with a multi disciplinary team that could include Physiotherapists, Speech Therapists, Dieticians, Continence Nurses, Occupational Therapists.

Increased dietary fiber intake or use of laxatives may relieve constipation, and drugs or a penile implant may help with male impotence. An artificial feeding tube or breathing tube may be surgically inserted for management of swallowing and breathing difficulties.

The diagnosis of MSA has significant impact not only on the person but also those close to the person with MSA. Progressive disability results in an increasing need for care and support for the person. Such a loss of independence can also be unsettling and challenging for the individual.

Caregivers and families too may feel stressed, frustrated and chronically tired, thus making the condition harder to cope.

Support services for both the person with MSA and their caregivers is vital to uphold the quality of care, encourage communication and a positive attitude towards ones treatment and care, and also maintain the pleasurable nature of the relationship.