Lewy Body Disease?

The son and daughter of the patient bring her to the movement disorder specialist with a provisional diagnosis of Lewy Body Disease. Frederic Lewy gave his name to the bits of protein he found in the cytoplasm of cells throughout the brain in the early 1900s. The illness is characterized by dementia, fluctuations in cognition- attention, alertness and thinking ability from day to day, and Parkinsonism.

Lewy Body Disease might have made a more simple patient case. The actual patient had a history of high blood pressure, neurosurgery to correct a potentially fatal aneurysm, which Wikipedia defines as a "blood-filled dilation (balloon-like bulge) of a blood vessel caused by disease or weakening of the vessel wall." Neuroradiology discovered a tumor on her cerebellum while performing an MRI to ascertain the state of the aneurysm. The children of the patient noted she had trouble finding the appropriate words for objects, following surgery, an observation that coincides with an aneurysm on the left side of the brain, the language center. After surgery, patients routinely receive Dilantin, prophylactically, to avoid seizures caused by minute scarring of cortical tissue. The patient never took the medication and suffered a seizure in the presence of her 85-year-old husband. The children also reported an incident in which the patient, "went crazy" after her medications were changed. Though the craziness subsided with new drugs, she still suffered from significant mental changes, for example she could not report her correct age.

With all her troubles, the children of the patient commented she was in good health and rarely needs medical attention. On physical examination, the specialist noted tremor in her left hand and arm with less involvement on the right side. He felt some rigidity, more on the left than right, when the patient was distracted by performing a concurrent activity- in this case; she tapped her palm on her thigh. Parkinsonism also appeared as decreasing amplitude in repeated finger to thumb tapping. Of note also were brisk reflexes and upward going toes- a Babinski sign indicative of brain disease, specifically damage to the corticospinal tracts. The patient felt incapable of independent walking, so the physician gauged her standing and balance skills. Without support, she could stand with feet touching, though the left side of her body twitched noticeably. With her eyes closed, she stood considerably less still and when jostled she lost balance quickly.

The patient complained most about the tremor on the left side of her body. This symptom is what the specialist addressed, by recommending a medication that successfully quells tremor that would not interact with her other medications. To complicate matters a bit more, the doctor learned the patient refuses to take any medications given to her by her husband, with whom she lives. The patient fears he is trying to kill her. For this reason, an independent person must visit the home bringing the necessary drugs.

When the dilemma unfolded, the physician commented Medicare pays for home health to dispense medications. He wrote a prescription for this service, as well as for physical therapy. The patient left the office on the arm of her son, and was clearly unstable with tremor rocking the left side of her torso.

Hints of PD

She just wanted to be told she didn't have Parkinson's disease. Unfortunately, the doctor couldn't dispel her worries. She did have tremor; what sort of tremor is questionable. Sometimes causes are unclear, time helps clarify issues, though few people can ever pinpoint the cause of Parkinson's disease. Those who can identify a true cause of the illness may subscribe it to having a genetic mutation, though familial patterns of Parkinson's disease are quite rare.

The patient suffered from temporal arteritis, inflammation of the temporal artery. The harrowing condition has symptoms like tenderness of the scalp and hair, pain in the jaw and when chewing, and loss or blurriness of vision. Doctors prescribe oral steroids to bring inflammation under control, because vision can be lost and never regained if flow through the ophthalmic artery gets disrupted and the patient has no secondary means of blood circulation to the eye. For some reason, women are three times more likely to suffer from this problem than men. Rheumatoid arthritis or systemic lupus erythematosus may coincide with the ailment; the patient lived with the former.

Upon examination, the doctor noticed brisker reflexes on the right side as well as slight rigidity when engaged in a simultaneous task. Though the patient reported a tremor that could shake wine from her glass, the tremor during examination was mild and fine. Might arteritis be the cause of her physical symptoms? Rigidity may result from inflammation in the brain, but the spouse reported tremor was worsening. With the course of steroids, inflammation should resolve, causing rigidity to improve.

Worsening tremor is unlikely to result from inflammatory processes in the brain. If the patient suffers from a Parkinsonian syndrome then tremor should gradually worsen and involve both sides of the body at rest, or other symptoms should appear. With the request to return to clinic in six months to a year, the physician prescribed Selegiline with the hope of forestalling and diminishing a possible loss of dopaminergic neurons.

Dad- a Clinic Day without PD Patients

My father used to be 5'10", now he's 5'6" or so. With my high heels on, I am as tall as he is. He's got that hunched over Parkinsonian posture. I tried to fix it by putting my shin near his spine and pulling his shoulders back. He shouted at me. I couldn't get him to extend his chest at all, his shoulders and pectorals have very little 'give'.

At home, he has a regular exercise routine, but the day I saw him he and his generation opted to eat dinner in the hotel restaurant, rather than walk the block and a half to the grill and microbrewery. When we did walk together, I took his arm, hoping that a little support would encourage a more spirited pace. Usually it failed, but at least he could hear me. He stops when he talks. I acquired the habit of either not talking, or monopolizing all the words, so we could continue moving.

Apathy comes. He is content to read the paper all day, drinking black coffee. I hear he does little housework and does not help with chores. When these things are pointed out, he shrugs his shoulders. My Dad has always favored well-cooked meat. At the hotel restaurant, he requested well- done bacon; it looked like jerky, he was delighted. He ate his bacon with dry toast- also well- done. I informed him the protein in the meat would affect how well his medications worked. He has never been science - oriented, he is an English and History teacher. I told him about neurotransmitters and how dopamine and proteins compete for the same sites, but I don't think he knew what I was saying, except that I thought the bacon was a bad choice.

Ages ago, I found a map of Wyoming or North Dakota with a town bearing our family surname. I showed it to him at the hotel breakfast.

"Yeah, I knew about that." He wasn't impressed.

On certain nights of the week when his wife works late, he cooks dinner. It can be a contentious event, apparently. Dad would not disclose what was for dinner on a recent night; he'd already had one argument about it. He likes meat and potatoes and tolerates a vegetable. How do the bowels of this man work? He lives primarily on bread and meat, and drinks no water, just black coffee during the day.

I am the delinquent one, the daughter who has never visited him, and he is nearing 78. At the end of our visit, I hug him and tell him I love him. He may have become apathetic, but he is teary to see his three daughters leave.

Hope is Hard to Kill

The patient hoped there was something in her a neurosurgeon could fix, to alleviate the tremor of her left hand. Unfortunately, she lived with several poorly aligned vertebrae in the cervical area and the doctor could see no reason on the MRI for a tremor. He wasn't the first physician to tell her this. Two neurosurgeons and a neurologist confided they saw no cause for tremor on the images of her brain and upper spinal column. Still hope is hard to kill. Clinically, the tremor she endured would be classified as mild, yet because it was her arm and hand, she conceived the movement as pronounced and problematic.
An older woman- she thought herself so, her birthday three years before the physician behind the desk, she suspected her tremor might be part of aging. The doctor assured her she was not old, and tremor is not a natural consequence of increasing age. He pointed with both hands to his head of dark hair shot through liberally with graying streaks, encouraging her to change her thinking on that matter.
With a Latin surname, a Florida speech pattern and the pale eyes and skin, I assumed she was a descendant of an original Florida family, but I was wrong. She still used the name of her former husband. Her manner so mild, I feared she would cry, outnumbered in the examination room by the clinical coordinator, the physician and myself.
The specialist recommended amantadine for the tremor, two pills a day- one at breakfast another at dinner or before bed. The drug has other properties as well as reducing tremor; it is anti- viral, so patients experience less flu and colds while taking it. While Azilect might stabilize symptoms, it is also an MAO inhibitor and possibly problematic, as one must avoid certain foods- aged meats, cheeses, and certain other drugs. He also strongly advised an antidepressant to lighten the cloud she carries over her head.
The MRI mages produced an unusual finding, a calcified meningioma: a very slow- growing tumor of tissue involving the tissue of the meninges. The doctor fished into the large envelope searching for a report to see what the radiologist concluded, and confirmed his own diagnosis. Women apparently have a greater quantity of such tumors.
Small, round, light splotches speckled the brain on the MRI. The doctor gave this a medical term, "leukoareosis". Apparently, leaky blood vessels in the brain show up on MRIs due to their excess fluid, a natural consequence of high blood pressure. They also, cannot be blamed for instigating the tremor of the left hand.

Rigid and Akinetic

He is a model of rigid akinetic Parkinson's disease, as opposed to the tremor dominant type. A compact man with a receding hairline of thick wavy and whitening hair, his nose is hooked. The skin of his face is pale and wraps his cheekbones tightly. He admits he's never suffered from tremor, a good thing when welding. He's an artist, primarily a sculptor. He calls himself an art teacher. The doctor asks to see his work, noting he appreciates art. All sorts of toxic substances- manganese, cobalt and solvents increased the risk he would acquire the illness. Today he is either
under- medicated or does not have PD. He sits like a rock in the yellow plastic chair. As he speaks he moves his mouth, but he doesn't fidget, scratch, blink or shift his weight. He shoots his left eyebrow up, once.

The patient and the doctor begin their words at the same time, more than once their voices overlap. When a pronounced delay in response sends the room to silence, the wife tries to help by reminding her spouse of the sequence of events. It has been six or seven years since the diagnosis, the initial symptoms are hard to recall. Still symptoms, diagnosis and treatment began within a period of a year. Not good, in the opinion of the physician...He can't recall feeling any improvement with medications. The specialist declares the patient is inadequately medicated or doesn't respond to levodopa.

He begins drawing a chart, describing how to increase the dose of Sinemet from one pill, four times daily to a possible maximum of ten pills daily. The idea is to increase the dosage, then observe the response the body makes on the following two days. Always increase the dose by half a pill, in a staircase fashion, halting progression if feeling nausea. If nausea continues at that dose, then step backward a half pill. The object is to discover what the correct dose is to alleviate most symptoms. Fluidity of movement, increased arm swing in walking, loosening of joints are as three aspects for possible improvement.

The doctor hands the patient the medication progression chart, requesting he return in three months. He is not free, yet. Both patient and spouse agree to provide their blood for a study searching for possible biomarkers in PD. This entity in blood would change along with the progression of illness, providing another source to document disease progression. Physicians determine the progression of illness by physical exam, but it gets tricky when patients rely on medication to be fully functional. Visual scanning techniques can also document loss of dopamine in the substantia nigra, but the procedure is costly, and dopamine-producing cells dwindle naturally with aging before producing parkinsonian symptoms.
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Bikes and Ladders

Bikes and Ladders
There are no symptoms when he's working. He climbs ladders and walks on roofs. When he comes home he freezes between doorways, as he does at the movies. He and his wife wait to be among the last to exit, to avoid the rush of people. He comments he doesn't sleep much, but it doesn't bother him. His energy level is high. It's been ten years since his diagnosis and his gait appears unaffected by the illness now; he has a natural arm swing and his steps are fluid. I ask the doctor whether he questions the diagnosis, and he says no. The patient responds to levodopa. The only troublesome time is around four in the morning after a bowl of cereal when the medication never seems effective; he shuffles. The doctor asks if he pours milk in the bowl. He does.

Milk protein is an especially competitive amino acid, and competes with levodopa for space on receptors. Other proteins will have a similar action, making levodopa much less effective. It's hard to fathom the patient doesn't know this. Both indicate this is the first time they have heard dietary proteins can interfere with uptake of the medication. The clinical coordinator nods and mentions this is something a support group can be helpful with. She is in the process of setting up a support group for patients in their area, and she gathers their contact information.

The next patient has had the illness since his mid thirties. He sits with his right shoulder drawn up towards his ear as his left hand flails and the right hand is stuck in a dystonic spasm. He recounts a story of hospitalization after falling backwards in his yard. They gave me morphine, he notes. The doctor raises his eyebrows and asks whether he liked the sensation. No, he thought they were trying to put him to sleep. They did x-rays of his chest after listening to his lungs. He had six x-rays, they only needed to take two, he relays indignantly. They put him on a course of several antibiotics, with names this long- he gestures a distance of about a foot. This is his worst nightmare; he will die in a hospital of pneumonia. He was strapped to a gurney, journeying into the belly of the hospital deep underground with patients lining the hallways all waiting for testing. Before they let him go, they made him sign a paper promising to take another two bags of IV antibiotics. He is in the midst of writing an angry letter to the hospital- a lawyer still.

The caregiver accompanying him is blond and well dressed, and has pink lipstick. Her face is pleasant and her demeanor quiet but assertive. She makes notes while the clinician speaks. Asking him to clarify what symptoms of an "off" episode look like. The answer is complicated because the patient experiences wearing off symptoms in one arm with dyskinesia on the opposing side. Learning about Parkinson's will be rough with this patient as the model.

He wants to ride his new bike, it has two wheels, not three, as the doctor would have preferred. It was lightning when it arrived, but he had to try it out before the rain began. The pretty caregiver found him a block away frozen in the pouring rain.

Contrasting Patients

Dyskinesia of the diaphragm is what he suffers from. The area below his rib cage seizes, moving the t-shirt that covers him, I think of a belly dancer's abdomen and wonder whether the doctor will lift the t- shirt. The pulling can get so intense he buckles over, while sitting. He was diagnosed only two years ago, and the disease affects both sides of the body. Dyskinesia affects both hands, and they move spontaneously while he sits in the examination room. Amantadine helps the dyskinesia and he's been without it since Sunday.
As the doctor examines the patient he notes the dark freckles that cover the area from the ankles up towards the knee. It's a side effect of Amantadine. In lighter skinned people the color appears reddish- purple. Since he's been without the medication the swelling in the feet has diminished.
The doctor recommends rotigotine and the clinical coordinator goes to search for samples. He explains to the daughter and patient what he would like to try- diminish the Sinemet while adding rotigotine, to see whether the new combination will help eliminate the excess movements. As the physician explains he writes the steps down on a sheet of paper he discovered in the printer.
This patient is almost floating. She enters and sits and speaks with the coordinator mentioning the book she recommends to all with a relative or spouse with PD, but can't remember the title. She wears green patterned long shorts and a yellow shirt. Her glasses sit on her nose and her skin is pale and clear and a hint of pink covers her cheeks. In her animated speech she kicks her legs out from below the chair.
The doctor searches the computer for the note he dictated last, while she speaks. There was a time when she fell into a fit of depression. She had reached fifty, got divorced and her children were away. 'You have to dig yourself out, anyway you can.' She is doing much better these days and would feel even better if she didn't have to spend $485. every month on the Neupro patch she acquires from Canada. This month she called twelve pharmacies before finding the medication at the thirteenth. They gave her free shipping.
The doctor encourages her, telling her she looks very well. She's had the illness for fifteen years and now sees symptoms of the illness on her left side. She confides she has been living with her boyfriend for the past eleven years. He is a calming influence and doesn't mind waiting until her medications are working, to leave the house.
When the patient has left, the doctor comments he has seen her for a long time. He remembers the husband who was Italian and reminded him of a mobster, wearing a baseball hat and a large belly. When I leave I note her new partner; a tanned fit man also wearing a ball cap.