Couple from Rotan

The couple comes from Rotan, the islands off the coast on Honduras, to see the movement disorder specialist; there are no practicing neurologists in the country. The patient is rugged, tall and has a receding hairline and wide shoulders. His wife is his equal in beauty, slender with dark long hair, pale fair skin and a thick very English accent. She makes eye contact easily with the physician behind the desk, while her spouse’s eyes travel about the small room with fluorescent lighting and insufficient ventilation.
As the doctor sits, the wife explains it has been about six months since her husband first complained about the the fourth child in their home. She adds they have three children; the youngest is an eight year-old boy. It was dinnertime and the family sat down together. The fourth child was a boy named Hector, and he was making a horrible mess with the spaghetti on his plate. Her spouse became enraged at the illusory boy, for not responding when he insisted on him using a napkin. Later on in the evening she tried to sooth her spouse by telling him he must have been having a hallucination, because they have only three children. When her spouse had calmed he recognized they did truly have only three children, but he remained confused about the boy at the table.
His eyes drift to the doctor and he explains there is something wrong, and it began perhaps nine months previous, when he noticed himself unsteady on his feet. He had been playing golf, had drunk a beer and fell in a sand pit. The unsteadiness remained and never completely went away, though sometimes he is less stable than others.
The physician asks whether he feels stiff, and he nods and adds he feels slow, like a man older than he is. He feels slow in the head, sometimes forgetting his children’s names. In her seat the wife states her spouse is thirty- eight and used to be a professional rugby player, and remains active, completing his seventh Iron Man competition six months ago.
The doctor inquires whether he trained for the event through the symptoms, and he nods agreeing, stating his times were proof there was something wrong- he finished more than an two hours later than his previous race. The physician approaches the patient asking him to sit on the examination table. He looks at his eyes, asking the patient to follow the pen as he moves it within the limits of his gaze. He brings the pen up high above the patient’s eyebrows and the brown eyes travel some before they move no higher. And he has difficulty gazing downwards without flexing his neck forward. The physician comments that he appears unable to gaze in vertical directions. The symptom has a name, supranuclear palsy, which occurs in a variant of Parkinson’s disease known as Progressive Supranuclear Palsy (PSP).
In the hallway the patient walks with narrow based small steps. The physician explains after watching the patient, the syndrome derives its name for the characteristic traits of PD, along with several other notable problems- rigidity, especially of the axial muscles (neck and back), postural instability, and impaired voluntary movement of the eyes. In PSP, there may be a mild dementia, but in his case, the hallucinations and dementia make the Doctor suspicious of two other parkinson plus syndromes: cortical-basal-ganglionic degeneration and dementia with Lewy bodies. In dementia with Lewy bodies, the hallucinations can occur even before medication is started. In cortical-basal-ganglionic degeneration the parkinsonism is complicated by problems with voluntary gaze and dementia. In all of these parkinson plus cases, some patients respond to Sinemet, feeling decreased rigidity and slowness, though there is no cure for the progressive illness. The wife looks at her hands in her lap, as her husband asks whether anything he can do would have an effect on the course of the disease. The doctor looks at him directly and says the majority of patients who receive the diagnosis require some type of walking aid after three to five years, and are either wheelchair or bed bound in eight years. The doctor looks down at his own hands momentarily and comments all the physical activity he gets has probably forestalled the illness to some degree, and he should remain as active as possible and the medication may promote movement, by making him feel less rigid. The patient nods and the physician explains how to gradually increase the dose to achieve a therapeutic effect.

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