He with his wife’s support, had prepared to undergo deep brain stimulation. He lay on the surgical table. The surgeon had installed the electrical lead within his brain when he suddenly stopped breathing. Over three minutes passed before he was revived. Apparently, they sewed his skull up and left the wires within his head. The tremor which moves both his hands goes unabated. The tremor continues with the doctor’s recommendation to try Artane, or trihexyphenidyl. The physician inquires whether he’s had vivid nightmares. The patient nods and his wife agrees, “Oh, yes.” The doctor observes the medication is doing little to calm the tremor. It has some rather nasty side effects in people older than sixty; it can interfere with short-term memory and in some men may inhibit the bladder and result in urinary retention. The doctor informs the couple, patients with frequent nightmares are also likely experiencing mild cognitive decline. He thinks it’s time to phase out the Artane, which may contribute to the muddled mind. Another medication capable of silencing action tremors is the barbiturate, Primidone. It’s sedative effects would put the patient in a very sleepy state, where performing any function with the hands might be risky. The doctor urges the couple to consider returning for a fresh surgical consultation, he believes the best solution to the tremor in his hands is still deep brain stimulation. Coming to the side of the patient the physician tests the fluidity of movement in his wrist. There is no ratchet-like movement. The patient demonstrates that slow movement of his hands calms the tremor- he takes both hands and makes deliberate circles in front of his body. The doctor enthusiastically exclaims he should try a Tai Chi class. The doctor demonstrates the measured motion of the exercises. The wife comments the previous physical therapy sessions were especially helpful. She could see improvements. He stood straighter and almost walked normally. She volunteers that their home has undergone some renovation, in order to allow her spouse movement throughout the interior. Several interior steps have been fitted with a gradually incline, so her husband can access all levels. When they are out in the world her husband prefers the walker, though when they are going long distances he uses the motorized scooter. He tries to maintain his independence. Another concern is his back. He recently underwent a set of injections to the nerve roots to dull the pain that makes standing unbearable. The pain impacts his ability to move and thwarts what motivation he has to exercise. The doctor recommends another round of physical therapy and encourages the patient to get out and walk. The bent man looks at his hands and comments he used to be an illustrator. Now he can’t even write his name. The doctor suggests wearing a set of wrist weights. People with tremor have found the added weight dampens movement. He may even find he can use his hands some. The doctor reminds them an alternative solution is to retry the surgical option.
The small man has been plagued with tremor since his diagnosis of Parkinson’s disease more than twenty years ago. He comes in to the examination room with his wife and a packet of information under an arm. He has the blank visage of a PD patient. When the doctor questions what he’s brought with him though, his expression breaks and he looks almost excited. With the papers in his hands, they flutter about and his wife intercepts them. The patient takes a seat. The hands are moving and distracting him and his wife places the pages on the physician’s desk. The doctor pauses and looks at what they have brought in.
The information from the Michael J. Fox Foundation for Parkinson Research internet site and describes a study of 183 patients with essential tremor or tremor brought on by PD. The study and results were presented in a poster session two years ago, on November 2, 2009. The American Society for Radiation Oncology released a report for the press. They called the intervention, “a less invasive way to eliminate tremors caused by Parkinson’s disease and essential tremor than deep brain stimulation and radiofrequency treatments, and is as effective, according to a long-term study presented November 2, 2009 at the 51st Annual Meeting of the American Society for Radiation Oncology. “The study shows that radiosurgery is an effective and safe method of getting rid of tremors caused by Parkinson’s disease and essential tremor, with outcomes that favorably compare to both DBS and RF in tremor relief and risk of complications at seven years after treatment,” Rufus Mark, M.D., an author of the study and a radiation oncologist at the Joe Arrington Cancer Center and Texas Tech University, both in Lubbock, Texas said. “In view of these long-term results, this non-invasive procedure should be considered a primary treatment option for tremors that are hard to treat.”
The doctor shakes his head and comments that destroying a small region of the brain to control involuntary movements is not a new idea. There’s evidence that prehistoric man drilled holes in the skull, perhaps to release evil spirits or to still tremor. He explains to the patient and his wife that this method uses a beam of radiation to essentially thermo-coagulate an area of tissue, and goes under different names- Cyberknife, Gamma Knife and Synergy are a few. The target is the Ventralis Intermedius nucleus or VIM. Different strengths of radiation have been tried, with varying results.
The wife reads from the sheet in her hand, “With a median follow-up of seven years, 84 percent had significant or complete resolution of tremors. In patients with Parkinson’s disease, 83 percent had near or complete tremor resolution, while those with essential tremor had 87 percent of this degree of tremor resolution.”
The patient is not able to undergo deep brain stimulation, as he has metal bits embedded in his body and brain from his years spent in Vietnam. DBS surgery requires the patient have an MRI of the brain, something not possible for a person with metal shards, since the magnetic field may cause the metal to move, creating internal bleeding. Stereotactic surgery can be performed with the use of a CT, rather than an MRI, to position the beam of radiation.
She was diagnosed with Parkinson’s disease in 1992. Today her husband pushes her in a wheelchair. Her body is alive with movement. Her torso stretches diagonally across the seat, her arms twisting at her sides. At times her legs extend straight out from her hip joints. The motion doesn’t dissuade her from speaking. Her voice is strong, not whispery. Her questions and comments reveal the clarity of her mind.
The husband pulls his chair up to the physician’s desk and takes several sheets he’s folded, from his pocket. The first lists the medications his wife takes. The doctor explains the patient has symptoms that are difficult to treat. The dyskinesias she endures occur at peak dose and end dose. They occur randomly while she is on and off. The doctor presses the husband to document his wife’s movements throughout the day, for two weeks. While he prepares a chart he describes the “on” and “off” states to the caregiver. The husband questions the state his wife is currently in- though she writhes in motion, her muscles are rigid. Forty minutes later, her movements are more contained. Her feet rest on the floor and the muscles at her wrists and elbows are supple.
She is slender. Whether she was always so slim is questionable. The doctor asks whether she has trouble eating and she responds she must cut her meat into very small pieces. Her peach-colored blouse looks like linen and is carefully ironed. Her skin is olive, her hair dark and she smiles.
The doctor says they must slowly cut back on the Sinemet. The patient will become increasingly more rigid, but they plan to replace the decreasing medication with ropinirole, until the Neupro patch arrives in July. The dopamine agonist brings on less dyskinesia. The physician directs the spouse to decrease the Sinemet, so that in two weeks time she has weaned herself from the medication. At the same time he is to continually increase the ropinirole until she is taking seven pills daily. Though the Neupro patch may provide some relief for her dyskinesias, she is a suitable candidate for neurosurgery. Such patients typically have complex motor fluctuations, as does the patient’s wife. The doctor turns the sheet of paper over and draws a diagram of the surgery. He describes the electrodes that send signals to the brain. The wires run under the skin down the neck and connect to a battery pack that is worn in the chest. Bilateral surgery requires two packs, that are hidden beneath the skin on either sides of the torso.
The doctor prints out refills for the prescriptions, a consult with the neurosurgeon and a request for physical therapy with attention to breathing exercises. He asks the caregiver to call in two weeks and report on how his wife fares without Sinemet, and only ropinirole to control her symptoms.
The patient is petite, delicate and dresses fashionably. She used to wear high heels when coming to her appointments. Then she fell twice. Today she wears red flats.
The symptoms of Parkinson’s disease are curtailed by the three times per day allotment of Stalevo, and the dispersible levodopa she places on her tongue every morning. The pain, pulling and burning in her dystonic trapezius is almost more than she can bear.
Dystonia is a condition in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. Dytonia can be an early sign of PD and can also develop as a complication of levodopa treatment of PD Treatment of dystonic muscles is difficult and has been limited to minimizing the symptoms, since no cure is available.
Some dystonias improve with levodopa and wax and wane with the dose of medication. The current patient suffers continually from the acute muscle spasm, regardless of timing with her medication. The muscle contraction lies between her shoulders, her upper back, and neck. It does not lessen. For two years she has carried the muscles cramps. Relief for her comes in Botox shots applied to the muscles of her back and neck. The shots themselves are painful. In order to get the correct muscle, the needle must be inserted, then reinserted, until the firing muscle is targeted, and then the botox is released. Individual muscle cells must take up the paralyzing venom and after several days the muscle is finally soothed into smooth flat tissue. The botox shots are usually given at three months intervals. Today the patient states her pain is substantial, yet the spasms have not drawn her head to one side. When she stands to walk, one shoulder is noticeably higher. Her arm swing is limited. Her next botox appointment is still several weeks away.
About 15% of patient receiving botox eventually build anti-bodies to the medication, which reduces or completely blocks the beneficial effects of botox. In these cases patients can use the alternate toxin, called type B. There is always the possibility that botox may flow into other, nonspasmotic tissue. In such instances, the paralyzing agent may cause her head to sag. She may experience muscle weakness; as some portion of the muscle fibers have been immobilized.
Aside from the dystonia, the doctor encourages her to take two of her 15 mg. mirtazapine pills. The drug is an antidepressant and taken at night, improves the quality of sleep. Her spouse is looking into acupuncture for the burning pain the dystonia brings.
The spouse comments her husband’s cognitive ability has taken a definite decline since her appointment six months ago. Gesticulating with her hand she shows how she has been shown the process of decline will be; horizontal for some time then a dip to a new horizontal plane. The doctor enlightens the couple the patient is probably the most documented patient he has, who he believes suffers from dementia with Lewy Bodies.
The patient is sturdy. At rest in the chair, he has no spontaneous movement. By his side, his wife types on her laptop while she listens to the doctor who speaks to the patient. He bought a mobile home for ten thousand dollars one day, and drove it home. The contract had no three days qualifying clause, so they could not return it. The wife concedes the children and grandchildren use it occasionally. She estimates it’s used four times a year. Driving has become a sore point, though he has a driver, who takes him where he needs to go. He thinks he may take a driving course, though the doctor discourages him, citing the fact that patient’s with parkinsonian syndromes lack “normal” spatial relations. Studies have shown patients tend to favor one side of the road, listing towards the curb, or the center of the thoroughfare. Whether or not they still retain the ability to shift quickly, they are hazardous to others.
His voice is soft, and the doctor leans across the desk to hear him. He would like to know what he can expect next, with the illness he has. The doctor comments there have been longitudinal studies on patients with parkinson’s disease, but none that he is aware of, of those diagnosed with Lewy body dementia. He speaks about a larger study enrolling people who suffered from dementia. Only seven to eight percent of those were thought to suffer from Lewy Body disease. Alzheimer’s disease is a more common cause of dementia.
None of the parkinsonian medications are useful for his symptoms. The doctor states they would worsen his quality of life by adding to the mental confusion, and tendency to hallucinate. Namenda and Aricept are two medications he takes to forstall mental decline. Vigorous physical exercise might help his condition, if he could motivate himself to do it. His wife agrees diet and exercise can go a long way to alleviating many ills, though she knows her spouse well. He attends silver sneakers, and a yoga class. The doctor encourages his yoga, and commends him for retaining interests he can engage in. Another desire is to ride horses. He has found a place, whether they will allow him on a horse, his wife is not sure.
Excerpt from article comparing DLB and AD
“Although cross-sectional studies have suggested similarities between DLB and AD in overall level of cognitive dysfunction, patients with DLB are reported to have more pronounced executive, attentional, and visuospatial deficits early in the course of the dementia. Several studies suggest that patients with DLB perform better than patients with AD on verbal recall tasks, but others have found no differences. Patients with DLB may have more difficulty with the free recall of declarative information in the context of relatively intact recognition memory. There have been fewer studies comparing progression of cognition in DLB and AD, and their results have been somewhat equivocal. In 3 studies, the rate of decline in global cognitive function was similar in AD and DLB Another noted faster decline in DLB. With regard to everyday functioning, 1 retrospective study found that patients with DLB were more likely to be institutionalized than patients with AD. However, both groups displayed a similar time to reaching an end point of moderate to severe functional impairment, as measured by the Blessed Dementia Rating Scale (BDRS).”
Karina Stavitsky, BS; Adam M. Brickman, PhD; Nikolaos Scarmeas, MD; Rebecca L. Torgan, BS; Ming-Xin Tang, PhD; Marilyn Albert, PhD; Jason Brandt, PhD; Deborah Blacker, MD; Yaakov Stern, PhD The Progression of Cognition, Psychiatric Symptoms, and Functional Abilities in Dementia With Lewy Bodies and Alzheimer Disease. Arch. of Neur. Oct. 2006, Vol 63, No. 10
He’s just twenty-six. He’s slumped over staring at his hands in his lap when he recounts the tremor of his left hand. It appeared when he began working in New York City. He noticed it first when taking the train from Brooklyn into the city. At first he thought he’d had too much coffee. Then he thought he was simply anxious about the new position he’d taken at the law firm. As the day progressed he forgot about the tremor. The next day, it was there again, in the morning on the train ride to work. He’d been grasping a pole to steady himself in the crowded cab, when he noted his left hand moving spontaneously, intermittently touching a woman’s thigh. She turned, as he did to see his resting left hand fluttering like a leaf. He apologized to the woman and she nodded to him when she saw he could not control the movement.
The doctor asks about other symptoms; constipation, depression, sleeping problems, anxiety or loss of smell. The patient glumly smiles and nods. He claims he has all of those. The loss of smell, he recalls dates back to the days when he lived in a fraternity on campus. A fire started in the kitchen on the main floor, only when the smoke detector went off in the bedroom next to his and his house mates pounded on his door, did he wake and exit through a second-story window. He never smelled the smoke.
Constipation has been a problem for him since he was a child. As a teenager he began eating All Bran cereal and drinking a quart of water after every meal. Those habits he thinks have solved the issue, though he still has occasional irregularity.
He shrugs when asked about depression, admitting he has never been a very sunny character. His girlfriend has told him he seems depressed, but he thinks he has always felt more blue, than happy.
The doctor asks him to come to the examination table, where he pulls out the step. The young man stands, fixes his brown hair behind an ear and sits at the end. As the doctor takes his arm he asks about the quality of his sleep.
After some moments, he confides he’s always slept fitfully. While he’d like to blame his poor sleep on anxiety over work, he recalls sleeping poorly while in school. As a child and teenager he walked in his sleep. His housemates in the fraternity frequently reported finding him wandering through house, with no apparent purpose. He has lashed out at his girlfriend while sleeping. She tells him he has conversations in his sleep.
The doctor moves his hand at his wrist, then his arm at his elbow, feeling for fluidity of movement then accesses the left side. He pauses there as he moves the joints, then looks to the medical student and asks her what she feels when she moves the arm. She takes the patient’s arm, closes her eyes and moves it back and forth. Opening her eyes she looks to the physician and reports some friction in the motion. The doctor nods and asks about other health issues, while he scans the form the patient has left on his desk.
The doctor gazes at him and reports, though he is young he has some rigidity, a sign of Parkinson’s disease. Diagnosis requires a patient have three of the cardinal symptoms; slowness, rigidity, tremor. His age forbids the use of standard dopaminergic therapy. The doctor would like him to begin exercising regularly, getting his heart rate up to eighty percent of his maximum, in a daily routine. He’d like the patient to begin taking coenzyme Q10 in a relatively high dose. He asks whether the symptoms interfere with his ability to work. The patient states he’d like to hide the tremor as much as possible from those at work. This was the reason for the appointment in Florida. The doctor nods in understanding and begins explaining the options for medication.
He’s down to 117 pounds and he used to weigh 150. His hands form the large belly he had. The doctor across from him cautions him not to lose anymore. He had been a borderline diabetic, and now he no longer has that diagnosis. Dyskinesias rock him in the seat but his is undeterred, and he asks the doctor whether decreasing the dose of Sinemet will help conserve his existing dopamine-producing neurons.
Behind the desk the physician responds, saying that Sinemet had at one time been thought to be detrimental to the neurons that produce dopamine. Scientists had thought levodopa might have a toxic affect on the dwindling population, but that idea has been discarded based on research that has shown that advancing illness affects other neurotransmitters, in addition to dopamine. The doctor even states some think that the levodopa gets converted to dopamine in the brain may actually have some protective value on the declining population of dopamine-rich neurons.
The doctor reviews the note he wrote after the previous visit. The only new presently available progress for patients with Parkinson’s disease is the Neupro patch. The company has said the patch would be available soon. For more than a year patients have been told that news. Now it seems it will be released in July. The patch provides a dopamine agonist that is taken up through the skin, at a steady rate for twenty-four hours. The method of absorption bypasses the digestive system. A dopamine agonist will not compete with dietary proteins for entry to the brain. The alternate source of dopamine will allow the patient to reduce the Sinemet he takes, thereby reducing the dyskinesias.
The doctor checks the rigidity in his arms and finds none. The patient is completely on. His joints are supple. He’s brought in a page of questions for the doctor. His handwriting is large and loose, atypical for a PD patient. He wonders whether he is taking too much vitamin D; 4000 mg per day. The doctor isn’t clear on the current information on the vitamin, it seems to be always changing.
An article by Salynn Boyles at Web MD health news from March of 2011, notes a study of patients with PD
found a high prevalence of vitamin D insufficiency, though levels did not continue to decline as the disease progressed.
The research is one suggesting a link between low vitamin D levels and Parkinson’s disease. A study from Finland published in 2010, showed people with the lowest levels of vitamin D were significantly more likely to develop Parkinson’s over almost three decades of follow-up, compared to people with the highest blood levels of the vitamin.
It’s not clear if vitamin D insufficiency raises Parkinson’s risk or if having high levels of the vitamin is protective, says study researcher Marian L. Evatt, MD, of Emory University School of Medicine and the Atlanta Veterans Affairs Medical Center.
“More research is needed to figure this out,” she tells WebMD. “There is certainly an association, but we can’t say if it is causal.”
In the newly published study, researchers examined the prevalence of vitamin D insufficiency in untreated patients with early Parkinson’s disease. They found 69.4% of patients had vitamin D insufficiency and 26% had vitamin D deficiency.
She says she’s a freelance writer. Woman’s Day recently published an article she wrote about care giving. She smiles and says it’s ironic, she never thought her own spouse would be the caregiver. The man is classically handsome, tall with dark hair and an easy smile. He pats her hand, when she divulges this information. Her dark hair is pulled back into a loose ponytail that hangs forward over her shoulder. The toes of her black shoes are so pointy, they cannot possibly house a foot. Talkative and anxious, she engages the medical student and me while the doctor takes an emergency call from another patient. The man by her side is silent, though he sits with a pleasant aspect on his face and demeanor.
Outside the doctor converses on the phone, we hear his words, ‘The only real emergencies in Parkinson’s disease are falls leading to fractures which should be handled in the emergency room . . . No. Take the dissolvable medication and lie down. Turn on soothing music. Listen to yourself breathing, inhale to the count of four, hold for three seconds and exhale to the count of four. NO. I’m in clinic, I have to go. Call me in an hour.’
The doctor enters, apologizing for his lateness. The patient begins talking about the tremor in her right hand, and the doctor sits, scanning the forms she’s filled in, and interrupts her with an upright finger. He asks for a moment to look over her paperwork. Though he begins questioning her immediately, ‘So you really are quite healthy . . .You exercise, you eat well, your blood pressure and blood work are normal. You’re fifty-five?’
The doctor smiles, ‘That would make this early onset Parkinson’s, if that is indeed what you have.’ He asks what symptoms she has and when they began. It’s been three months since she noticed the jiggling in her right hand. She was using the keyboard, typing when she felt her right hand acted more like her left, less useful. A subtle change, she admits, but she saw the tremor in the morning when folding laundry.
The doctor asks her to sit on the examination table. He takes her right arm in his hand and flexes the joint at the elbow. Moving it back and forth, he takes the other side and makes the same motion. He isn’t sure. He returns to her right side and states there may be some slight rigidity there, but it surely isn’t definite. Performing the other tasks, he asks her to return to her seat.
At his desk he says her symptoms, specifically the feeling her right hand was performing like her non-dominant side, sounds like very early Parkinson’s disease, but she lacks any of the cardinal symptoms- slowness, rigidity and resting tremor. He encourages her to maintain her exercise regimen, as that may be helping to put off the full onset of the illness. She may be very sensitive to alterations in her body and be able to detect early changes, though she may not manifest the illness at all. He would like to continue watching her, and asks her to return in six months. She’s content with his appraisal and shakes his hand before leaving.
He’s lived with the tremor for thirty years, since he was twenty-six. In those thirty years he has consulted several physicians. Whether he ever received any medication is an unanswered question. His attitude seems to be stoic, with the assumption that medicine has little to offer him. The tremor rocks his head back and forth. When he brings his chin to his chest the movement stops, and he can raise his head slowly before it resumes.
He’s convinced the agent that he used to clean jet engines, a supreme degreaser, is the cause. A toxic overdose from submerging his upper body in the solvent, on a regular basis, he believes is the culprit. Whether he is right or not is immaterial. The tremor haunts him, unrelenting. Sometimes while he lies in bed he feels his body shaking inside.
The doctor is certain he doesn’t suffer from Parkinson’s disease. His facial muscles are expressive, mobile. Actions are fluid. There is no slowness to him, if anything he does things quicker than normal. His voice is full volume, without hesitation. The tremor encompasses both hands.
On physical examination the muscles surrounding his joints are supple, his eye movements are normal, dexterity not compromised, though he complains his handwriting has degenerated. The doctor asks him to copy a spiral winding out from a point inside. The pen in his hand makes regular jolts as the tremor moves his hand. It’s the work of someone who has essential tremor. The same regular movement that made Katherine Hepburn’s head shake and voice waver.
An electrical engineer, he has the tenacity to draw his work at the computer. To accomplish this, he needs to subdue the tremor. He overcomes the tremor by pushing firmly downward. After three hours or so, his entire arm is aching.
Clearly, he’s ready for a change. The doctor prescribes Inderal, a beta blocker. He asks the patient to begin with one pill for three days then escalate to two pills for three days, than add the third pill for three days. After several weeks he’s to switch to a single pill of 60mg, that is a long-acting. The doctor asks him to call him in a month and report on his status, rather than give him another appointment. In the meantime, he requests a blood test, to look for heavy metal toxicity and an MRI of the brain, with contrast to rule out other more noxious diagnoses.
Her white hair, curled at the ends, her green cotton sweater and yellow capri’s attest to her casual sense of style. She stretches her long legs out in front of the chair she slumps in and tells the doctor her concerns. The book in her arm, has the title something like, Fifty Medications the Doctor’s Don’t Tell You About. She’s got the pages which mention Parkinson’s disease, tagged. The doctor checks the back and looks up the pages that speak to his specialty. Published in 1993, the text has nothing the physician is unaware of. A patient would hope her neurologist, who also has a PhD in Pharmacology, knows his craft.
The patient has none of the resting tremor, the stony facial expression, or slowness that regularly accompanies the illness. Her major concern is her ability to walk. She is hesitant in expressing it as a concern, she ventures really she fears her balance is declining. She’s never fallen. Several years ago she underwent some ‘walking therapy’ and felt it really helped. The doctor agrees she would benefit from physical therapy and writes her an order for PT. He also urges her to begin a daily regimen for her cardiovascular health. With a pacemaker and coronary artery disease, he asks her to check with her cardiologist before beginning. He’d like her to begin using a stationary bicycle. In the beginning, this would be a five minute commitment, though he would like her to increase her time by five minutes every week until she is spending twenty minutes on the bike. Additionally, he’d like her to raise her heart rate to eighty percent of her maximum for her age. With a pencil, he figures out what this figure would be.
He encourages her to participate in the yoga class being offered at her community center. The activity is very good for stretching out the joints and muscles, especially helpful for those with PD, who tend to get stiff and slumped. Balance is also addressed. Swimming is another activity that would complement her health.
Her spouse depends on her. She is the caregiver. She knows it will be her downfall, she’s aware of the demands that tax her. She thought he was talking in his sleep, but in fact he was wheezing. It was five in the morning. He seldom wakes her, generally.
On physical examination her limbs are loose, without rigidity. In medical terms she would be regarded as, “on”. Freezing also bothers her. She asks about it, but the doctor confesses, there are no medications that simply address freezing. She has written down the prescription for exercise, and mentions her handwriting has deteriorated, though that simply must be her age. The doctor faces the computer screen, typing in her renewals for medication and does not comment.
In her eighties, this patient has beauty in her face. Her blue eyes and features have a life behind them that is hidden from those of us who are not her friends, though we may see hints of her joy.