The patient and his wife appear for their appointment. They have a jovial quality uncharacteristic of patients coming for a second opinion on Parkinson’s disease. The issue the patient has with speech is evident right away. It seems he must summon a certain energy to pronounce words, or to string words together. There is some lapse of time between questions and his response and there are none of the small inarticulations people make in standard speech; the small sounds of hm… ah…and well… are all missing. Talking and writing are the two major impairments that brought him to see a neurologist, a few years ago. The wife mentions since that time she has noticed the patient’s speaking ability has deteriorated. She explains the patient sometimes tells her yes, when he means no. In addition, the patient admits his memory is not as good as it was.
The patient’s general health is remarkable for hyperlipidemia- high cholesterol and triglycerides in the blood, high blood pressure, and diabetes. The patient manages all three problems with medications. The three problems contribute to give the patient small vessel disease- the smallest blood vessels that shuttle blood between arteries and veins are blocked, consequently, the patient’s brain has multiple small areas which do not receive sufficient blood supply.
The patient has had the relevant tests. A PET scan revealed his brain has normal oxygen consumption, indicating no signs of Alzheimer’s disease. A recent MRI suggested possible atrophy of the temporal lobes. Neuropsychological testing indicated the patient had losses in executive function, and should not be handling money; at the time he was the sole owner and director of a company with fifty employees. However, the patient scoffed at the results saying the tests were overly tedious, and he disliked the tester. Results also indicated the patient’s IQ must have become compromised, as his apparent IQ did not harmonize with his position as a functioning executive. However, the movement disorder specialist fails to discuss this fact with the patient and spouse. Inquiring later about why he skipped lightly over this, the specialist noted the patient had scoffed at the results of neuropsychological tests, denying their truthfulness or value.
On physical exam, the specialist notes no rigidity, nor slowness of movement. He does find the patient has limited range of eye movements when gazing downwards, which a sign of an atypical form of PD known asprogressive supranuclear palsy (PSP). Though the patient’s speaking ability is affected, he lacks many other signs typical of the disorder. Though he is a heavy man, with a substantial belly, he comes to a standing position easily from sitting in a chair. His gait is fluid, his arm swing full, and his balance normal. The specialist explains that this may be a very early case of the illness.
PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson’s disease. Hence it is also called Steele-Richardson-Olszewski syndrome, for the three who focused on the disorder. The initial complaints are typically vague and an early diagnosis is always difficult. The primary complaints fall into these categories: 1) unsteady walking or abrupt and unexplained falls without loss of consciousness; 2) visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble; 3) slurred speech; and 4) various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood. http://www.ninds.nih.gov/disorders/psp/detail_psp.htm
In a review article, Dr. Marsel Mesulam described Primary Progressive Aphasia as having an insidious onset with gradual progressive impairment in finding words, naming objects, and comprehending words while engaged in conversation. Though not evident in the first years, patients exhibit prominent apathy, disinhibition, loss of recent memory, visuospatial impairments, visual- recognition problems, inability to perform simple mathematical calculations, and inability to perform pantomime movement as instructed. Dr. Mesulam notes that some patients may have signs and symptoms of illness confined to the area of language for as many as 10 to 14 years, before other problems emerge. Nowhere in the review does he mention problems with eye movements. (New Eng. J. of Med. Vol. 349:1535-1542 N.16 Oct. 16, 2003)
According to the National Institute of Neurological Disorders and Stroke, Corticobasal Degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy of multiple areas of the brain. Progressing gradually, the initial symptoms, which typically begin at or around age 60, first appear on one side of the body, but eventually affect both sides. Symptoms are similar to those found in Parkinson disease, such as poor coordination, an absence of movements, rigidity, disequilibrium or impaired balance, and limb dystonia- abnormal muscle postures. Other symptoms such as cognitive and visual-spatial impairments, apraxia- loss of the ability to make familiar, purposeful movements, hesitant and halting speech, myoclonus- muscular jerks, and dysphagia- difficulty swallowing, may also occur. The patient eventually becomes unable to walk. From the writer’s perspective as an outsider, the patient seems least likely to suffer with this diagnosis. . (http://www.ninds.nih.gov/disorders/corticobasal_degeneration/corticobasal_degeneration.htm)
The movement disorder specialist recommends the patient and his wife make an appointment with another specialist whose area is cognitive neurology. This authority would be able to establish areas in which the patient has cognitive impairments. He also recommends the patient undergo another MRI within a year, to see whether changes in the brain have progressed.
Sometimes a diagnosis is unclear; sometimes a person has more than one diagnosis. The patient asks whether some pill will prevent further decline in his condition, and the specialist says there is nothing he can recommend. However, eating fruits and vegetable and performing daily exercise are general health recommendations for all. From the expression on his face, the patient stopped listening at the mention of fruits and vegetables.