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A Body In The Mind

“Are you simply a frustrated doctor? Perhaps deep down what you wanted to be was a neurosurgeon?” are comments I have occasionally fielded. As it happened I never yearned to be a medical doctor, and while clinical neuropsychology does have many things in common with neurology, neurosurgery, and psychiatry, equally valid might have been the comment, “Are you a frustrated detective?” Most research has an element of detective work, whatever the field, and clinical neuropsychology is no exception. For me, it is the mix of working as a clinician and a researcher simultaneously that is the attraction of clinical neuropsychology. And these two aspects of the discipline are at their powerful best when the clinician or researcher comes across a patient with a neurological disorder or a collection of symptoms that are rare. If the patient is willing and is well enough to be tested, the neuropsychologist has an opportunity to discover something new about the way the mind works, and hopefully the careful research assessments will also provide detailed information that can improve the patient’s rehabilitation programme.

The neuropsychologist usually begins with a broad assessment that highlights the intact ability areas and those areas where the patient is now experiencing difficulties, because of their brain damage or neurological illness. Further assessments of those problem areas narrow down the possible reasons for the problems, step by step. Every new round of assessments involves reading previous research in the area, and thinking about how this patient’s results confirm or are different from past published studies of similar cases. New tests often need to be designed. Of course in this situation, tests are unlikely to be standardized against other people of the same age, sex and cultural group. At this level the detective neuropsychologist is looking for clues and following them up at the individual level.

Noting how long and detailed these assessments often are, neuropsychologists are often criticized for putting patients through lengthy and tiring procedures. My experience is that patients often look forward to this time with the neuropsychologist. This assumes, of course, that the neuropsychologist is a skilled clinician and knows how to spread out the assessments and schedule tests so that the patient is not left with a feeling of failure after any session, is never assessed when he is tired, and is given easily understood and helpful feedback. At least when the patient sees the neuropsychologist walking in the door again, he knows he isn’t about to get another needle!

The following opening excerpt from “The Man Who Misplaced His Body” (Chapter 5 of my book TROUBLE IN MIND) is an illustration of the neuropsychologist working as detective. Being a detective before the time of the internet incorporated considerably more leg work—or library work—than today, but the principles remain unchanged. This single case of a very rare disorder in which the patient loses the ability to know where, on the human body, the different body parts are situated, suggests that humans have a map of the human body hard-wired into their brains. (The reference for the original research article documenting this case is
Ogden, J.A. 1985. Autotopagnosia: Occurrence in a patient without nominal aphasia and with an intact ability to point to parts of animals and objects. Brain, 108, 1009-1022.)

Excerpt: Page 115-121

“Have you seen a case of Gerstmann ’s syndrome?” a neurologist asked me one day when she met me on the ward where I was assessing patients for a postdoctoral research project.

“No,” I answered, trying to remember exactly what Gerstmann’s syndrome was, but already feeling the anticipatory buzz that invariably accompanied the possibility of discovering for myself a “new” neuropsychological disorder.

“Well, you might like to check out a man who was admitted last week with a left parietal tumor of some sort. He’s very alert and has no language problems, but he has left–right confusion, he can’t write or calculate, and he gets his fingers muddled up.”

I grinned at the neurologist. “I’d better read up about it first, but I’d love to assess him.”

“Thought you might! Don’t wait too long. I’ve put him on steroids, so his symptoms might disappear in a few days as the swelling goes down. I think he’s got apraxia as well, so you’d better add that to your reading list.”

I thought I’d introduce myself to the patient first and see if he was willing and well enough to carry out a few basic tests. Then I’d spend the rest of the day, and into the night if necessary, in the university medical library, reading everything I could find on Gerstmann’s syndrome and apraxia. I flicked through the patient’s medical file to check his admission details and read what the neurologist and nurses had written about him. Julian was a 59-year-old automobile and boat mechanic who lived with his wife, Anne, in a small seaside town in the sunny far north of New Zealand. He spent his spare time fishing and boating, and it had been after a weekend sailing trip that Julian had first noticed that his right arm was weak. He put this down to tiredness and advancing age, but soon after, Anne became concerned when she noticed he was having difficulty writing. Then he had two minor car accidents when he veered to the right, and within three months his right arm weakness had spread to his right leg until he was unable to stand alone. He was also becoming increasingly confused. Anne finally managed to get him to see his local doctor, and that same afternoon Julian found himself on a flight to Auckland. On being admitted to the Auckland Hospital neurology and neurosurgery ward, he had a computed tomography (CT) scan of his brain. This revealed a large, well-defined cystic tumor with surrounding swelling in the left parietal region of his brain, displacing the corpus callosum (the large fiber tract joining the two hemispheres) to the right.

The neurologist had noted that on admission, Julian demonstrated a right visual field deficit and had a mild weakness of his right arm and leg but no loss of tactile sensation. His comprehension seemed unimpaired and his speech was fluent and normal. He had difficulty telling his left from his right and was unable to subtract 7 from 100. He was strongly right-handed, so not surprisingly his writing with his left hand was clumsy, but in addition his spelling was shockingly bad, whereas his wife said he had been a good speller previously. When the neurologist tested Julian’s ability to identify his fingers by asking him to close his eyes and name the finger she was touching—little finger, index finger, and so on—he made a number of errors. In the light of his left–right confusion, acalculia (inability to calculate), agraphia (inability to write), and finger agnosia (inability to “know” and therefore name his fingers), the neurologist had queried Gerstmann’s syndrome. She had started Julian on a course of steroid medication to reduce the swelling around the tumor.

The nursing notes were also interesting. When Julian was admitted, he had great difficulty putting the correct arms and legs into his pajamas, even when the garments were held in place for him. He became quite frustrated and told the nurse that he seemed to have “lost his marbles.” His problem sounded like dressing apraxia. Apraxia is the inability to carry out, on command, learned skilled movements such as waving, brushing one’s hair, or, as in Julian’s case, dressing, despite good comprehension, full cooperation, and intact—or reasonably intact—motor and sensory systems.

Armed with the neurologist’s and nurses’ notes and my basic neuropsychology tool kit, I went to see Julian, who was sitting up in bed with his weak right arm propped on a pillow but was looking quite chirpy. I explained that I was keen to assess him for research purposes, and he said he would be delighted to “have a go” at my tests; he was bored already stuck here in the hospital when he could be out in his boat. I began with some of the standard tests of vocabulary, comprehension, and general information and found he had no problems at all with these. To assess visual neglect, a disorder where the patient doesn’t attend to the space and the objects in it on the side opposite to his or her brain damage (see Chapter 3), I gave Julian five simple paper-and-pencil tests. He showed a mild right-sided neglect on only one. He did show symptoms of right-sided body neglect (another type of neglect where the patient is not interested in body parts opposite to the brain damage), which probably exacerbated the appearance of weakness of his right arm and leg. For example, although his right arm lay motionless at his side most of the time, when he was asked to concentrate on moving that arm or grasping my hand with his right hand, he could do so with near-normal strength. Another symptom of right-sided neglect was his request that people stand on his left because he did not like them standing on his right.

I asked him to point to his left ear. He looked confused and then, using his good left arm, pointed to the right side of his neck. This was a surprise, as I had been expecting left–right confusion but not body-part confusion. So I did some further spontaneous testing and quickly discovered that he really couldn’t seem to locate the different parts of his body, or indeed the different parts of my body. At that point Julian’s lunch was delivered, and I sat with him for a while, observing that by holding his fork in his left hand he managed to spear the food on both sides of his plate—cold meat, beans, and tomato sections—and deliver it to his mouth successfully. This was another sign that he didn’t have significant visual neglect. I left him in peace, promising him I’d be back first thing next morning, and made a beeline for the medical school library.

In 1984 there was no rapid Internet providing electronic access to every journal article at the touch of a computer button. So when I got to the library I began my research by pulling out heavy neurological tomes and reading the classic descriptions of disorders. Next I checked for research articles under topic titles by going through card files stacked in the thousands in filing cabinets. Once I had a list of article references scribbled on my pad, I climbed down into the library basement, which housed most of the journals I would need—some over sixty years old. It was a dusty treasure trove of rolling shelves, and I could spend hours sitting on the floor between the shelves, a stack of dark green, blue, and black books of bound journals beside me, devouring the detailed descriptions of neurological disorders and syndromes written by the meticulous clinical researchers of the past. It always made me feel like a real researcher poised at the brink of an exciting new discovery. That “me” would never have believed that one day, only twenty years or so into the future, the contents of that entire archive of journals, extending back nearly a century, would be able to fit onto a single 100-gigabyte hard drive in a tiny computer which could sit on my desk! But for me that feeling of being part of the history of discovery—even if only on the very periphery—is not as acute when hundreds of research articles can be downloaded, categorized, and summarized in a flash without my needing to venture into the door of a real library or even take out a pen.

Sitting there on the library floor, along with reading about Gerstmann’s syndrome and apraxia, I discovered some classic references to an even stranger disorder called autotopagnosia, which literally translated means “not knowing the topography of oneself.” This very rare disorder was associated either with generalized brain damage or with lesions of the left parietal lobe. The main symptom was an inability to point on verbal command to human body parts, either one’s own or those of another person (or a doll or picture of a human), although patients varied with regard to the exact symptoms they displayed. It took me quite a while to untangle the different hypotheses that had been postulated over the last sixty or more years to explain this strange disorder. In some cases the problem seemed to be a language-related impairment where the patient had lost the names of body parts but not those of other things. This made some sense, as the brain damage that gave rise to the disorder invariably included the posterior part of the left, “language” hemisphere. Then there were cases where the patient had a more general difficulty pointing to the parts of any objects, not just body parts. For example, a patient with this sort of problem, when asked, could point neither to a person’s nose nor to the wheel of a car. This suggested that the disorder was at least in part a problem with the visuospatial system—a problem with locating object parts within a whole. This characterization also made “brain” sense, as the parietal lobe in the left as well as in the right hemisphere is associated with visuospatial functioning.

Having read everything I could find and having photocopied many articles, my next task was to come up with a battery of tests to assess Julian’s many fascinating disorders—in particular, tests that would help me define the parameters of Julian’s difficulties with pointing to body parts. Of course, as with all higher cognitive disorders, I knew that a complex disability such as autotopagnosia could occur for more than one underlying reason. Did Julian have a category-specific naming problem for body parts that prevented him from pointing to them on command, and if so did it extend to the body parts of other animals as well as humans? Perhaps his difficulty was more general, extending to a problem pointing to the parts of inanimate objects as well? Could he name body parts when shown them in isolation yet have difficulty locating them on a complete human body? There were no standard neuropsychological tests for this purpose; I would need to design my own. Given that Julian had already been started on steroids, time was of the essence; very likely his more dramatic symptoms would resolve as the brain swelling reduced. The other sad possibility was that his tumor might prove to be malignant and his condition could deteriorate. The chances of my ever having the opportunity to assess a patient with autotopagnosia again were very slim. There were very few cases reported in the literature, probably because patients with left parietal tumors or strokes large enough to cause autotopagnosia would usually have extensive language problems that would mask the more subtle symptoms of autotopagnosia.
End of excerpt

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