A German patient and his Austrian wife have recently moved from Iowa, and need a new neurologist. His voice resonates in the small room with only a hint of German lilt left. He has the voice for radio, but he is a test pilot. The doctor is in education mode conferring to the patient all things related to Parkinson’s and discusses the personality type others have given to the stereotypical sufferer. Well controlled, averse to adventure, given to detail and methodical work, the patient admits that describes himself.
The wife describes the onset of symptoms a few years ago; trembling in a hand that in time involved the foot as well. Today there is little evidence of any symptom. The doctor feels some cog- wheel rigidity in the muscles at the elbow joint on the left side otherwise the patient’s symptoms are very well disguised by medications- Mirapex and Sinemet. The movement disorder physician commends the patient’s management, he is doing the right things- exercising daily… He would add something thought to slow illness- perhaps coenzyme Q10 or deprenyl.
The doctor conducts a physical exam. Performing the finger to nose task, first with the right hand and then left, I note the patient’s hands. His skin is taught and smooth, unlined and young- looking. He is in his sixties and he has the hands of a young man. The wife has skin appropriate to someone of her age, and a flare for fashion evident in pale pink clogs with an open toe and well- cut red hair. She is un-intimidated by the doctor and presses him on why he speaks so much of animal research.
The patient’s gait is flawless and his arm swing full and loose. ‘Maybe I don’t have PD…’ the patient comments as the physician emphasizes how well his symptoms are covered. The doctor doesn’t give the comment any reply. All witnessed the wife mimicking the tremor of the hand, and how the movement eventually affected the left foot. If he doesn’t have Parkinson’s he has something close enough
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.
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.
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.
Six month follow- ups help the doctor and patient stay on top of shifting symptoms, though more frequent appointments are possible. The doctor has said he likes movement disorders because there are no emergencies. Though the first incident of freezing may feel catastrophic to the patient, it passes. They are bumps in the road. Sometimes the journey is lightened by sharing it.
The Parkinson’s disease coordinator speaks with the patient about attending possible support group meetings in his area. He responds, stating not all patients want to talk about their problems. Not deterred, she explains the sessions will be run according to group preference. Some people may want lectures, others may just want the social time.
The shooting pain in the left knee is new and it worries him. It never occurs when he’s doing Tai chi and it moves up, not down, as the doctor would prefer. Pain moving downward may emanate from the spine, a bony prominence can easily impinge on the fibers of a nerve, sending scintillating pain down through the leg. That’s not it. This is pain moving up towards the thigh and it’s fast, not throbbing and deep as the pain patients with PD describe. Both his mother and father are diabetic…The patient climbs onto the examination table and the physician tries to replicate the sensation. Both legs have bruises at the lower mid- shin. The tightness of the muscles in the patient’s legs is extreme; the doctor comments he would like him to continue with physical therapy.
Parkinson’s disease in this patient is evident in the constant tremor of his hands. Six months ago, it was not as prominent as it is today. He describes shaving, his right hand wavering towards his cheek. The doctor recommends wearing wrist weights to dampen the movements, the patient nods. Tremor is one of the hardest symptoms to suppress, and he takes a distinct drug to soften the constant shaking. Yet he walks well, with head and shoulders erect, an arms swing, and ample step size.
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.
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.
A patient reported today the Neupro patches they requested from a Spanish pharmacy were returned because the dry ice required for shipping required special consent and paperwork. The patient continues to try and balance his medications, attempting to find a happy equilibrium between over medication and dyskinesia, and under medication resulting in more slowness, rigidity and episodes of freezing. The specialist in movement disorders commented freezing episodes usually occur independent of medication dosages. However, the patient claims Sinemet relieves such episodes. To manage the balancing act, the physician recommended cutting back on the Requip; he prescribed 1 mg tablets, to replace the 2 mg dose.
The patient had been interested in deep brain stimulation for controlling dyskinesia, and had visited a specialist in another city for a consultation. The clinical physician commented, of the six patients he has that have undergone surgery, only two continue to do well with their implants. He emphasized the surgery doesn’t cure anything, and the operation comes with considerable risks; settings require adjusting and not all clinical staff are capable of superior post- operative care. While training is required to adjust settings, a spectrum of talent is employed to maximize the beneficial effects of neurostimulation.
The patient scheduled for 2:00pm sat in the waiting room for an hour and a half before the staff realized the allotted patient appointment drifted past. Consequently, the patient was in an ugly frame of mind. Though the reason for the visit was called, “Tremors” the patient exhibited none of the characteristic motions. Instead, the patient described spontaneous, involuntary movements like sudden face slaps, jerks, or kicks. The physician inspecting the patient’s medical record noted the patient took a wide variety of antidepressants, and pain pills. Several of the daily medications are capable of producing involuntary movements and slurred speech. While the patient waited two months for an examination by the movement disorder specialist, hoping that the doctor would be able to identify the ailment and how to fix it. The patient’s hopes crushed, tears flowed. The specialist stated he was the wrong doctor to be handling the case
and in his opinion the medications were the cause of the involuntary movements.
The specialist recommended the patient phone the social worker and begin the process of applying for disability. Clearly, it would be hard to keep a telephone position with a slurred voice, and inability to write in a timely manner. The good news was the patient suffered from no neurological degenerative sickness; the bad news was the patient was too depressed to appreciate good physical health.
New patients at the clinic receive one-hour appointments. The expert in movement disorders sees patients referred from other physicians, due to unusual symptoms involving motion of the body. The most prevalent of movement disorders is essential tremor, affecting 4% of the population over the age of forty. Parkinson’s disease is the second most common neurodegenerative illness after Alzheimer’s disease. The patient was probably referred to the specialist to rule out Parkinson’s disease, as some symptoms might make a case for the illness, deteriorating handwriting, shaking of the right hand, lack of sleep, and tiredness.
At fifty-one years old, the patient had a list of other complaints that spoke to some other diagnosis, entirely. With several car accidents in the history, and a laminectomy of L4-L5 for nerve root compression, with another possible surgery looming due to compression of the spinal cord in the cervical area, it was hard to separate symptoms possibly caused by damage to nerve roots, from an active disease process. Might the sensory symptoms; numbness and tingling of the right side of the face be due to compression high in the vertebra of the neck, while the same symptoms in the legs be due to compression addressed by the past surgery? Complicated migraines may be accompanied by sensory symptoms like numbness and tingling, and migraines were something the patient had endured since quite young.
The physician explained that in neurology doctors tend to work with a cluster of symptoms that suggest an illness. In Parkinson’s disease a patient needs to have two of the four cardinal signs of the illness; slowness of movement, rigidity, tremor of the hand or foot, and impaired postural reflexes.
In trying to ascertain a diagnosis, the patient had undergone an MRI, spinal tap and nerve conduction studies. On MRI, the brain was noted to harbor several areas of concern, specifically low-density areas that might have been present in someone who had suffered a stroke, or had high blood pressure and diabetes. The physician noted the signals in the brain might also be due to mini strokes brought on by migraines. Nerve conduction studies were found to be normal, as was the lumbar puncture; two tests that might have pointed to a demyelinating illness like multiple sclerosis. The patient also underwent testing to see whether the problem might be Lyme’s disease, tests were negative.
While examining the patient, the foot and leg moved spontaneously. The term for such unintended random movements is myoclonic jerks. When forceful and prolonged the motions cause the patient’s muscles to ache a day later. A former physician noted the patient’s hands were five degrees cooler than the average skin temperature. The neurology exam was normal except the patient could not stand with eyes closed and feet together, or perform heel to toe gait.
The most bothersome problem of all however, was failing memory. The cognitive changes were not something the doctor could gauge in the hour allocated; he referred the patient for neuropsychological testing to determine what areas of cognition were affected. Testing may give some clue about the process of the illness and point in a direction to explore further.
A patient usually comes to the movement disorder clinic with the diagnosis of Parkinson’s disease because there’s a quirky symptom that puzzles the general neurologist. Small idiosyncrasies about the presentation of illness and the onset of symptoms can mean the difference between Parkinson’s disease and Dementia with Lewy bodies, a variant diagnosis. Perhaps you think, so what? The diagnosis of an illness describes a certain course. The diagnosis gives a range of issues that typically present themselves with progression of illness, and they vary.
A patient comes to the clinic diagnosed with PD yet the first indication of illness was hallucinations. Standard patients with PD may experience hallucinations when they are sleep deprived, over-medicated or in advanced stages of illness. The patient’s symptoms are readily apparent upon physical examination; cogwheel rigidity exists in the muscles of the arms, there is slowness of movement and the hands exhibit a resting tremor. It has not been two years since diagnosis. The most astounding symptom is the loss of substantial weight in two months. PD patients lose weight over the years, not in sudden drops.
The specialist concedes the patient’s symptoms are not typical for PD. He would like the patient evaluated by a neuropsychologist who will evaluate cognition, or mental faculties and access what the deficits are. The family members who have come with the patient attest to the loss of memory, and increased reliance on others to remember, and perform basic functions such as grocery shopping.
He would like to target two issues in the clinical session- he’d like to improve sleeping and ensure the patient is eating adequately. To improve sleeping, he tells the patient to open the drapes during the day, let in the sun and try not to nap. At night, he writes a prescription of the antidepressant, Remeron or its generic, myrtazapine. The medication may take up to a month to have anti-depressant effects, but in the meanwhile, it increases appetite and makes patients sleepy. It is to be taken at night.
The essential difference between Parkinson’s disease and Dementia with Lewy bodies is the distribution of the protein masses, called Lewy bodies. While patients with PD also harbor the plaques, they begin low in the brain, eventually migrating to inhabit cells higher in the cortex as the patient becomes demented at end stage illness. In the other scenario, the mental decline evident early on occurs because Lewy bodies have invaded the cortex of the brain and impaired its normal functioning.