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The Multi System Degenerations
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The information below is directed to patients and caregivers. More detailed information, with more medical terminology is available by clicking the individual disorders below
Progressive Supranuclear Palsy(Parkinson's, falls, and poor eye movement) This is a drug resistant
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 (CBGD)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), actionmyoclonus
(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. Informational Links: WE MOVE re CBDG Mediconsult Site: The Multi-System Atrophies 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 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.
WE MOVE Info Link:
http://www.wemove.org/msa.html 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. Striato-Nigral Degeneration (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 facies, 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.
(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. Summary 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. |
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