Progressive Supranuclear Palsy
This is a drug resistant parkinsonism.
It is characterized by the inability to move the eyes in an up and down movement, neck dystonia
(a peculiar posturing of the neck causing the head to be fixed skyward), dysphagia (difficulty swallowing) and dysarthria (difficulty speaking), and a tendency to suddenly lose balance.
The facial expression may take on a surprised look with elevation of the eyebrows.
Early symptoms are often vague and include
mild disequilibrium with slowing, easy fatigability, minor personality changes and subtle visual symptoms such as blurring or double vision. With disease progression, walking becomes slow and
deliberate with broad-based steps and progressive loss of balance. The facial expression is fixed and associated with frowning, and rapid blinking can occur.
Loss of memory and other problems
with concentration is frequent. Tremor is rare. Common personality changes are apathy, depression and irritability.
Complaints often associated with the
problem moving the eyes up or
down include the inability to read, write, eat properly, or dress. Going down stairs can be very difficult. In advanced stages the eyes may not move at all. As the disease progresses falling becomes more frequent, often without warning. Swallowing often becomes the major problem putting the patient at risk of choking and developing pneumonia.
This diagnosis is made on the basis of
findings on the in office neurologic examination. The characteristic eye movement problem and shrinkage of the brainstem on CT scan can confirm the diagnosis.
Cortico-basal-ganglionic Degeneration (CBD)
Rigid Apraxic Syndrome
This is a rare drug resistant
parkinsonism. It is characterized by the marked asymmetry of the Parkinsonian features until late in the disease. The patients will frequently have apraxia (inability to properly use a limb for complex
tasks despite normal power and only mild incoordination), action myoclonus (i.e. jerky abnormal movements superimposed on normal movements), "alien limb phenomenon"(one limb seems to have a
mind of its own, sometimes actively interfering with planned movements), and stimulus sensitive myoclonus (involuntary jerking in response to light touch).
Early on the condition may be misdiagnosed
as Parkinson's disease. Marked often painful rigidity may occur late in this disorder. Tremor is not as common as in Parkinson's disease.
CT scanning, or MRI may identify local atrophy (shrinkage) of the surface (cortex) of the brain late in this condition.
The Multi-System Atrophies (MSA)
This group of disorders includes what used to be called:
Olivopontocerebellar Atrophy (OCPA)
Striato-Nigral Degeneration (SND)
Shy-Drager syndrome (SDS).
The Multi-System Atrophies are a group of progressive neurodegenerative disorders that early on can mimic, or be mistaken for, Parkinson's Disease. This
diagnosis is often made only after patients fail to improve with medication or develop atypical features.
In Parkinson's Disease only one main group of nerve cells die at an abnormally fast rate. These cells produce Dopamine, a neuro-chemical that stimulates parts of the brain responsible for movement. Without dopamine "like a motor without oil", the body slows, stiffens, and can start to shake. In Parkinson's Disease replacing dopamine in pill form results in improved mobility. In the Multi-System Atrophies a variety of groups of nerve cells die off. The cells that dopamine stimulates are involved. In this situation the "motor is damaged and just putting more oil in doesn't usually help". Thus dopamine replacement doesn't resolve the many problems associated with these disorders account for approximately 20% of all cases of parkinsonism.
Although early on these disorders may be
difficult for doctors to tell apart, as time goes on they develop certain distinguishing features. They all share the unfortunate characteristic of responding poorly to anti-Parkinson medications.
MSA - Previously called Shy-Drager Syndrome
Parkinson's and loss of autonomic function
In addition to the nervous system
responsible for movement there is a nervous system responsible for automatic body functions (autonomic nervous system). The autonomic nervous system looks after the regulation of the blood
pressure, control of the urinary bladder and sexual function. Shy Drager syndrome is a problem with a failure of the autonomic nervous system together with parkinsonism.
This disorder is twice as common in males than females and life expectancy is 7 to 10 years. It usually begins after age 50.
Patients may present with rigidity,
masked facies, resting tremor, bradykinesia, loss of balance, dysarthria and dysphagia, emotional lability (uncontrollable emotions) and a shuffling gait. They may respond temporarily to
anti-parkinsonian medication, but problems with the urinary bladder (urinary urgency and retention) and sexual
function (impotence) become evident. Inability to maintain blood pressure results in low blood pressure particularly after meals. This can result in fainting or just weakness, poor concentration, headaches, blurred vision and confusion. Medications for Parkinson's may induce or aggravate the low blood pressure.
Medications are available to attempt
to improve the low blood pressure. Pressure stockings for the legs as well as increased dietary salt can help.
MSA-C - previously called Olivo-ponto-cerebellar Atrophy (O.P.C.A.)
Parkinsonism plus balance problems
This is a progressive parkinsonism
associated with "ataxia" (unsteadiness and lack of coordination) usually beginning in middle age with generalized slowing, loss of coordination and tremor. Disturbances in speech, eye
movement as well as muscular rigidity occur.
OPCA may present with rigidity,
resting tremor, slowness of muscle movement, a fixed facial expression and a shuffling gait which are typical of Parkinson's.
Most patients become wheelchair bound
within a few years of onset. Dysarthria (difficulty speaking) is a symptom in almost every case; it is frequently an early sign and is at times so severe that speech
becomes unintelligible. Swallowing can also become a major problem putting the patient at risk of choking and developing pneumonia.
The presence of ataxia mixed with
parkinsonism, features on CT scan (brainstem and cerebellar shrinkage) and failure to respond to anti-Parkinsonian medication help in diagnosing this disorder. This disorder can be inherited.
MSA-P = previously called Striato-Nigral Degeneration (SND)
Mimic of Parkinson's but poor response to medications
Often this is hard to distinguish from
Parkinson's Disease. The typical Parkinsonian rest tremor occurs less commonly. An MRI can sometimes identify changes in the brain that can suggest this diagnosis.
Patients may develop rigidity with
generalized bradykinesia, masked face, a shuffling gait and quiet, muffled speech. Swallowing problems occur as the disease advances.
Dystonic (muscular twisting) postures
are noticed, as are abnormalities of neck posture with a marked forward tilt of the head on the neck. Personality changes such as anxiety and irritability are often seen. The response to
medication is disappointing. This disorder can steadily progress, unaltered by medication. Survival can be threatened within 4 - 5 years.
All of these conditions are extremely
frustrating for patients as well as their caregivers because of the difficulty improving the majority of their symptoms. This can often lead to patients seeking additional specialist opinions,
as well as unproven treatments. Maintaining an ongoing
relationship with a familiar family physician or specialist is extremely important to guide the ongoing care of a patient with any of these complicated group of disorders.