Shy Drager’s Syndrome = Multiple System Atrophy with Orthostatic Hypotension = Parkinson’s Plus Synd

The patient is a trim and tall man who spent his youth working the family farm in South Dakota. He recalls driving the truck through the fields, spraying herbicide from two enormous containers on either side of the vehicle across multiple lines of plants, no shirt, breathing and feeling the mist settling on his bare skin. The Environmental Protection Agency states, the product 2,4-D is a herbicide used on a number of crops. “At high concentrations, it affects the central nervous system in humans, with symptoms including stiffness of arms and legs, incoordination, lethargy, anorexia, stupor, and coma.” The movement disorder specialist remarks that this may be a factor that may have contributed to the development of his illness many decades later.
Asked about the earliest symptoms, the patient reported a three month interlude in his life in which he did little more than crawl in and out of bed, his fatigue was so overwhelming. Subsequently diagnosed with chronic fatigue syndrome, the patient recuperated to a large degree. The patient who is too young for retirement, describes feeling energetic for about an hour and a half every morning before feeling tiredness overtake him. He currently works less than two days per week.
Another predominant symptom is his lightheadedness. The doctor checked his blood pressure and reported it was quite low, taken in the sitting position. Blood pressure usually drops when standing. Medications for Parkinson’s, levodopa and dopamine agonists both lower blood pressure in patients. The physician encouraged the patient to salt his food liberally in the weeks ahead, take his blood pressure twice a day, and see whether the lightheadedness dissipates. If adding salt is ineffective he might try wearing compression hose, sometimes called T.E.D. hose, which prevent the pooling of blood in the lower body when a person stands. The garments are tight and hot, and hard to tolerate in the heat of Florida. The last resort is a medication, Florinef, which causes the kidneys to retain sodium, which in turn causes the body to retain water, thereby increasing blood pressure.
The patient received a diagnosis of probable PD less than a year ago, though the presentation of his symptoms, namely the bothersome low blood pressure is a hint of another less favorable illness. Symptoms of trembling in the wrist and hand began on the left side, and now involve the right side. Bilateral symptoms place the patient at stage two, on the Hoehn and Yahr staging scale. The patient commented that his voice has lost volume; it used to be less whispery. His face lacked expression, as well. On physical examination the physician noted the patient’s limited shoulder motion, he commented about the rigidity of axial, or trunk musculature, though his neck appeared supple. Both the physician and student were able to elicit rigidity in the arm muscles, and in gait, the patient had full arm and leg swing. Tandem gait, walking heel to toe, was more difficult and the patient spread his arms to compensate for the narrower base of support.
The less favorable diagnosis is what used to be called Shy Drager’s Syndrome. Patients who can tolerate levodopa, may benefit from the medication. Toleration comes by managing the constant tendency for blood pressure to slide downwards. The physician asked the patient to return in six months. Time will tell whether the patient suffers from an atypical presentation of Parkinson’s, or a more complicated illness.
The National Institute of Neurological Disorders and Stroke breaks Shy Drager’s Syndrome into three possible types; Parkinsonian-type includes symptoms of Parkinson’s disease such as slow movement, stiff muscles, and tremor; the cerebellar-type, which causes problems with coordination and speech; and the combined-type, which includes symptoms of both parkinsonism and cerebellar failure. Problems with urinary incontinence, constipation, and sexual impotence in men happen early in the course of the disease. Other symptoms include generalized weakness, double vision or other vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating.


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