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2008 (19)
2009 (21)
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Tingling and Numbness
Monday, December 21, 2009

The patient feels she is walking on sponges. The tingling and numbness in the arms and legs never goes away. The movement disorder doctor comments both thyroid illness and diabetes can affect the nerves, resulting in sensations of numbness and tingling.  The patient is relatively healthy, and does not have diabetes. To combat rheumatoid arthritis the patient takes methotrexate, which Wikipedia states received approval for that treatment in 1988. The online site does not mention changes in sensation, as a possible adverse effect on the central nervous system. Other sources of information do show that rheumatoid arthritis can result in peripheral neuropathy. Diagnosed with low thyroid function, the patient has taken synthroid for years. Emedicinehealth.com lists numbness and tingling of the hands or feet under hypothyroid symptoms, one might experience during pregnancy, an unlikely scenario for a patient over sixty years of age.
The specialist conducts the physical examination, finding some cogwheel rigidity in the muscles of the arms. Fine motor skills are slower than normal, though the patient demonstrates them well. Facial expression appears somewhat frozen, and speech lacks the whispered quality frequently heard in patients. The patient does not shuffle, though gait is slow and she admits she loses her balance quite easily. Neither the patient nor family member mention falls. While demonstrating gait in the hallway, the patient turns en bloc, not freely but turning the feet stepping repeatedly around an axis.
The physician notes the lower body symptoms are more expressive of Parkinson's, than upper body symptoms, a hallmark of vascular Parkinson's, or lower body PD. The subtype differs from true PD, in that the patient may not suffer from a lack of dopamine in the brain, though the symptoms resemble the true illness. He inquires whether the patient feels the prescribed medications have been helpful. The patient reports she had seen little change in her symptoms.
After some discussion, they agree to a plan; try to consume a vegetarian diet for two weeks to assess whether medications are effective. With little to no proteins in the diet, the levodopa should have better access to brain where it is made into dopamine and should have an effect if the patient is dopamine- depleted. If the patient has no lack of dopamine, the medications will do little to ameliorate symptoms. If there is no change the patient should increase the dose of medications and assess that effect. If after the increase in dose the patient still feels no reduction in symptoms, she will stop taking the Comtan, and gradually reduce the levodopa/ carbidopa until weaning herself from the drug. At this point, one may say with some certainty whether the patient has her own stores of dopamine, or if she has become depleted of the neurotransmitter, as is typical in Parkinsons disease.
The specialist addresses the tingling and numbness in the extremities by prescribing gabapentin, also called Neurontin and Gabarone. He says the drug is quite benign, in terms of its side effects, though initially it makes one drowsy. He advises the patient to begin taking it at nighttime, and to increase the dose by taking a pill during the day as she becomes tolerant to the sleepiness. The physician asks the patient to return in four months so they can reassess the situation.
Job
Friday, November 20, 2009

Just because you may be plagued by one illness, does not exempt you from acquiring another. So it is that 5% of patients with HIV contract symptoms of Parkinson's disease. Whether due to exposure of typical things like pesticides, well- water, or heavy metals, or because an HIV patient may have prior exposure to neuroleptic medications and opportunistic infections, it is unclear how and whether one illness relates to the other. Parkinsonism has also been reported as the presenting symptom of HIV. In a recent issue of the journal, Movement Disorders a healthcare team from the Salford Royal Hospital, UK reported on a patient with HIV who received a diagnosis of idiopathic Parkinson's disease and suffered from dyskinesia. The patient underwent treatment with highly- active antiretroviral therapy and experienced resolution of the parkinsonian symptoms.

In 2001, the patient was a 40- year old homosexual man in a relationship, with no history of injectable drug use or foreign travel. He described a three-year history of parkinsonism on one side of the body.  He reported having difficulty writing and noticed the loss of manual dexterity due to tremor in the left hand. Examination noted the presence of seborrhoeic dermatitis of the face. He had signs of upper and lower limb resting and postural tremor, though slowness of movement and rigidity were confined to the left side of the body. He had full facial expression, and no problems with eye movements.

Symptoms of right- sided illness and compromised balance were apparent approximately a year later. A clinician recommended dividing the 800 mg/ day of levodopa into smaller doses. The amendment made some improvement, though marked motor fluctuations, and dyskinesia appeared within six months. The patient began taking amantadine along with continuous subcutaneous apomorphine infusion. The method of delivering medication to the body allowed a reduction in levodopa to 300 mg/day and mildly improved motor complications.

In 2004, the patient underwent an excisional biopsy of the parotid glands due to bilateral swelling. Results showed benign cystic changes in cells. An HIV test came back positive. Other testing provided a CD4 count of 150 (normal for HIV+ is 500) and a viral load of >750,000 (the very upper limit). Neuropsychological testing showed mild impairment of short- term memory. The patient began highly- active anti-retroviral therapy: lamivudine/ zidovudine and lopinavir/ ritonavir with a marked reduction in his viral load. Symptoms of parkinsonism improved over the following months allowing him to discontinue the apomorphine infusion, and gradually withdraw from levodopa. In a follow- up appointment in April 2008, the patient exhibited no resting tremor or bradykinesia. Rigidity was absent at rest and with co-activation.

HIV- infected patients with increased cell loss in the substantia nigra have been described, though these patients have been free of neurological abnormalities. PET studies have shown increased metabolism levels in the basal ganglia in early HIV infection, with reduced metabolism occurring in advanced illness. The authors concede the abnormalities they describe in the patient could be explained by reversible dopaminergic dysfunction secondary to HIV infection; though the span of time the patient suffered from symptoms, seems exceedingly long. They advocate HIV should remain in the differential diagnosis of young- onset PD, given its potential resolution by highly active anti- retroviral therapy.

Kobylecki C. et al. Letter to Editor HIV- associated parkinsonism with levodopa- induced dyskinesia and response to highly- active antiretroviral therapy. Movemnt. Dis. Early view  11 Nov. 2009.

Rasagiline- Induced Spontaneous Ejaculation
Friday, November 13, 2009

Journals publish letters sent to their editors to enlighten clinicians, students and others about unusual patient cases. The following story describes an atypical response to the medication, rasagiline. Prescribed for patients with PD, it is thought to harbor neuro- protective effects.

The sixty- five year old patient was born with spina bifida, but had no dysfunction of bowel, bladder, or sexual function. The patient received a diagnosis of PD at age sixty- one and took 2mg rasagiline to treat his symptoms.  A month after initiating treatment with levodopa he experienced spontaneous ejaculation occurring in clusters, every ten minutes for thirty minutes. Such episodes happened every two to seven days. Ejaculation took place without an erection and with no stimulation. In between these episodes he had regular sexual activity with no autonomic problems.

On his neurological exam, the clinician found left- sided rigidity, and slowness of movement without a resting tremor. The dorsiflexors were mildly weak, and he had decreased proprioception of vibration in the toes of both feet. The lower body had decreased reflexes.

The episodes of spontaneous ejaculation continued even when the dose of rasagiline dropped by half, but stopped when he discontinued the drug. Without rasagiline, the patient noted his symptoms worsened, and his clinician subsequently prescribed a four-month course of 10 mg of selegiline daily, to counteract additional movement concerns. After a period of four months, without sexual side effects, the patient began rasagiline anew, at 2 mg per day. Within a month spontaneous ejaculations re-occurred. The patient elected to stay on the medication, and still experiences unprompted orgasms. He also noted orgasms associated with planned sexual activity are more explosive, with less recovery time needed between such events.

Apart from hypersexuality, the prevalence and range of sexual dysfunction in those with PD, gets little attention. However, numerous reports of sexual problems occurring after the use of dopamine agonists, specifically apomorphine and ropinirole exist. This report, the authors believe, is the first of its kind.

Researchers noted dopamine's role in inducing an erection when apomorphine was undergoing drug trials for the treatment of alcoholism. Apomorphine is a powerful dopamine receptor agonist and has been marketed as a treatment for human erectile dysfunction. Bromocriptine and ropinirole, two other dopamine agonists are reported to have improved erectile dysfunction, as well.

Experiencing ejaculation without an erection is curious. The author notes animal studies have shown dopamine aids ejaculation. For ejaculation to occur without an erection, different dopamine receptor sub types may come into play, as stimulation of D2 receptors in the rat's pre- optic area by the D2/D3 receptor agonist, quinelorane, promotes ejaculation. Piribedil, another D2 and D3 receptor agonist significantly increases rat ejaculation with less effect on erection. Further support that D3 receptors in the brain modulate orgasm and the period between ejaculations, appeared when technicians injected a selective D3 receptor antagonist into the medial pre- optic area of the rat brain, abolishing ejaculations without affecting erections. It is also possible rasagiline increases central dopamine at either the brainstem or medial pre- optic area to increase signals to the spinal cord ejaculation center. The author concedes the patient is unique due to his underlying spina bifida, suggesting spinal cord or peripheral involvement is responsible for the aberrant reaction to rasagiline. As neurologists inquire more frequently into the sexual function of their patients, and with increasing use of rasagiline, more instances of the unusual side effect should occur.

Chuang R.S. and Lang A.E. Letters to the Editor Rasagiline- Induced Spontaneous Ejaculation. Movemnt. Dis. V.24 I.14 Oct. 30, 2009.
Going Solo
Friday, November 06, 2009

A patient arrived for the first time at clinic today with striking symptoms of Parkinson's disease. A very detailed history of when the first signs occurred followed, with acute insight into what presented and how the initial feeling of pain in one arm got interpreted as a muscle strain. After noticing the pain persisted throughout the day, for several days, then months the patient noted decreased strength in the arm. Objects once easily manipulated spilled onto the kitchen floor.

The patient lay on the bed at night wondering whether the spouse would notice the bed shaking. The inner tremor continued as did the pain in the arm. The specialist comments that studies have been done on patients who feel an inner sense of tremor, and that the symptom, once thought to correlate with anxiety, was shown to correspond with depression.

The patient nods in understanding and seeks to relate more of the history; bringing a foot into the air as one does in ascending a step, has become problematic.  In order to perform the feat, the patient is forced to hang on to the railing. Akin to this issue, is the inability to rise from a chair without using the arms. In eating lunch, the patient finds his arm has lost the fluidity of movement it had. It is almost as though the arm and hand holding the fork, move in ratchet-like motion.

The physician states two over -the- counter measures may be useful are coenzyme Co Q10 at 900-1200 mg/day and creatine at 20 grams/day. The two have been found to improve mitochondrial function. The doctor comments the autonomic nervous system becomes involved in PD, and patients typically find themselves contending with constipation. The patient nods in agreement, relating some relief of the symptom through a remedy by Planetary Herbal Formulas, something called Triphala. Frequent urination is another symptom the patient notes, occurring at night. The doctor responds that many patients comment on this.

The patient concedes that the weight loss through healthy eating of many fruits, vegetable and whole grains was intended, thirty three pounds came off rather easily in the last year. The physician asks the patient to move to the examination table. While moving, the patient clasps every stable surface on the way to the exam table. The physician asks to see the patient walk, and the feet appear to be magnetically fastened to the floor. The patient has no arm swing. To gauge the ability to recover from a perturbation in balance, the physician cues the patient about what he plans to do, then jostles the patient's shoulders. The patient throws the arms wide and has extreme difficulty righting the torso under the feet. Problems with recovering from a balance disturbance typically present themselves in Stage III illness.

Back in the confines of the small office, the specialist explains that when a patient's signs or symptoms create impairments that conflict with working, or performing routine activities, he prescribes medications to dampen symptoms of PD. Most drugs have secondary affects. Though both creatine and Co-Q10 will do nothing to quiet the issues, they may delay the worst of the illness. Rasagaline, or Azilect is a prescription medication proven to delay the need for levodopa in PD patients. An MAO inhibitor, it carries a black- box warning because it may bring on a hypertensive crisis when combined with red wine and aged cheeses.

The patient stretches an arm over the notes the physician provides, and divulges that rather than taking the drugs more time needs to be given to an alternative method. The medications will be there, available when hope runs out that other means may alleviate the most troubling symptoms. Yes, the physician agrees, this is a patient decision; yet he wants the patient to return in four months to check on progress.

Parkinson's disease versus Dementia with Lewy bodies
Thursday, October 29, 2009
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.

Trim and in her Early Seventies
Monday, October 19, 2009

She fell between two cars three years ago and just recently finished the final treatment to her jaw and teeth. The accident resulted in a fractured jaw and arm; not simple fractures but compound, and they didn't heal well. The jawbone had to be rebroken, aligned and held in place with a titanium plate. Three years later, she noticed the tremors in her mouth and hands.  At night, she felt her entire body twitching.

The first neurologist the patient saw diagnosed the tremors as Parkinson's disease and prescribed Mirapex. The medication can increase patient compulsivity, and receives blame for instigating gambling habits in people ordinarily not prone to wagers. The husband of the patient vouches that the personality of his spouse changed when she began the drug. Now, when she gets an idea in her mind she must follow it to the end. Delaying or minimizing the chore is insufficient, the patient feels compelled to carry out whatever it is her mind has settled on.

Generally, the rule with prescribing levodopa containing medications is, not to begin treatment with Sinemet in patients younger than 60 years of age, because half all patients treated with levodopa medications develop dyskinesia in three to five years. Dyskinesia is uncontrollable, often fluid movements of the limbs. The patient in her early seventies might have received the drug and been more completely relieved of her symptoms. In the physician's opinion, tremor is really a cosmetic issue, embarrassing to the patient but usually not disabling. The patient admits that when she becomes aware of her tremor there are things she does to subdue it.

The movement disorder specialist would like the patient to begin taking amantadine twice a day for symptoms, because it may slow progression of the disease. He relates he would like to taper to only one Mirapex, right before retiring for the night. He also states that if the patient feels symptoms are worse, she might try a course of Sinemet. Now confused by how many drugs the physician is recommending, the doctor clarifies; always take the amantadine twice a day, take the Mirapex before bed and if worse begin weaning onto Sinemet. Sinemet is one of the drugs best to begin gradually. He writes out a schedule, the first several days the patient takes a single pill at breakfast. After that, the patient steps up to taking a total of two pills, once at breakfast and again at lunch, this continues for several more days. The second step takes the patient up to three pills total, at breakfast, lunch and dinner.

The specialist notes the patient has mild symptoms of PD. She has rigidity in both arms and makes small excursions when performing repetitive fine hand gestures. Strolling in the hallway, the doctor notes her gate is solid and her arm swing full. Perhaps the course of illness will remain mild. He requests the couple return in three months, so he can check the status of the patient and make any changes in medications. Encouraged that she walks for an hour on the treadmill, the doctor emphasizes the role physical activity has in enhancing physical function.

Illness without a name
Friday, September 25, 2009

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.

Sickness
Monday, July 13, 2009
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.
Lewy Body Disease?
Saturday, June 13, 2009
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
Friday, June 05, 2009
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
Saturday, May 30, 2009
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
Friday, May 08, 2009
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.
Cotton and Linen
Tuesday, May 05, 2009
A white blouse, natural undyed linen skirt and huarache sandals; the patient's appearance hints at affluent comfort. Her height, shoulder length white hair and the pleasant proportions of her face require the attention of those around her. Making eye contact with those passing in the hall, her facial expression does not change. Dyskinesia gently rocks her head from side to side. The husband is shorter and rounder. Her legs are long, perhaps a model's legs in younger years.
The doctor inquires into her health, commenting on the movements of the head. He has never seen her so mobile. They are in Sun City now, their winter place. But New York beckons. Their return North means physical therapy with a previous therapist who worked on her neck and shoulder. Years ago a surgeon placed metal plates to stabilize the cervical vertebrae of the neck, but the excess motion of dyskinesia creates pain that moves through her shoulder and down the left arm. The doctor comments deep brain stimulation can readily improve the unwanted movements. She comments she takes a blood thinner, Coumadin and avoids green leafy vegetables for their wealth of vitamin K- a blood coagulator. No, surgery is not an option for her.
As the doctor types information into the computer system the pace of his words slow. She reaches into her bag for the diary, where she has recorded her physical condition for the past month. There are days when she froze repeatedly, other days when she was "on" and forgot to take the medication. The doctor re-emphasizes the times when symptoms are known to worsen; with stress, any colds or flu, dental work...From the occurrence of movements and the time since the last pills the physician deduces she suffers from peak dose dyskinesia. If she cut back her dose a bit, relied more on agents that prolong dopamine's affect, or spaced the doses out more through the day she might experience less dyskinesia. The husband shakes his head, commenting a large party is approaching and his wife worries
Test Pilot
Tuesday, May 05, 2009
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
Undaunted
Tuesday, May 05, 2009
Undaunted
The patient begins explaining as soon as the doctor sits and the list is long. He thinks he's always had a tremor of the hands but now he thinks it may be worse; sometimes he has troubling controlling the mouse on the computer so the cursor sprints across the screen. He has a low body temperature, usually about 96 degrees. Another autonomic sign is erectile dysfunction..
On examination the doctor finds some rigidity in the muscles of the right arm, a hint of rigidity in the left arm but none in the wrists. His gait is fluid, with an arm swing. Facial expressions are complete. His eye movements are full, but then he has only one eye; he lost the left one when he was seven, when he accidentally stuck a knife in it. He was also hit by a car and spent a year in the hospital trying to acquire appropriate healing of the left tibia- leg bone. As a child he watched his brother die when he had a seizure and never recovered. His father died before age thirty-five and two of the patient's daughters also died. Yet he is not depressed, he's an optimist. We laugh. So much death and he is undaunted.
He is a working engineer, and he's past retirement age, at 72. Traveling he uses his Irish passport; in Libya they have negative associations about Americans and he travels a lot; India, Northern Africa... He speaks French, some Arabic, Spanish, some Italian and he used to speak Gaelic.
He worries about his enlarging waistline, and the doctor asks him whether he has had his thyroid tested. He admits the skin of his arms gets very dry, unless he uses lotion his skin flakes like the scales of a fish.
The doctor explains a study he is in which seeks a biomarker for the progression of Parkinson's disease. The patient is a wonderful candidate because he is early in the disease process, if he has Parkinson's. The only way to be sure about the diagnosis and whether he has a deficit of dopamine, is to gauge the response to levodopa.. Yet the doctor hesitates to give him medications when he functions so well, choosing instead to give him a drug thought to delay onset of symptoms, Selegiline. A prescription for physical therapy will help him form an exercise routine to keep him active.
Rigid and Akinetic
Tuesday, May 05, 2009
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.
Contrasting Patients
Tuesday, May 05, 2009
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
Tuesday, May 05, 2009
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.
Shakes and Pains
Tuesday, May 05, 2009
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.
Two Men and a Mute
Friday, January 09, 2009
Shy Drager's Syndrome takes away the body's ability to regulate blood pressure so there is a lot of lightheadedness and falling. That's why the spouse wears tight hose and uses the wheel chair in the middle of the night. It's all happened before. The doctor likes to give these patients erythropoietin, a substance that promotes formation of red blood cells. The patient also takes Florinef, a drug that causes the kidney's to hold onto salt, thereby increasing blood volume.
The patient asks the medical student whether she's a dancer. Her blond hair hangs straight down past her shoulders and she responds easily, replying when she was younger. The spouse teaches dance, and has owned a dance studio for forty years.
The last episode lasted about 15 minutes. She was ready to call 911. It began with shaking in the arms. The trembling got more violent, his eyes rolled back in his head, and his torso straightened in the wheelchair. His breathing changed and he lost bladder control. The wife has described the episodes before, apparently with less emotion. The doctor worries about seizures and recommends an EEG and MRI. He comments it is rare to have PD and seizures appear together, they tend to be inversely related - though hypoxia can induce them.
The man with the thick hair and heavy rectangular glasses asks about scotch and water. He never remembers the seizures, even right after they've occurred. When would you like a drink? The physician asks. Before and after dinner the patient answers, but the wife returns and vetoes all scotch with a wave of her hand. He won't be able to walk, she claims. No scotch.
He recalls a meeting he recently attended. Hugo Chavez was there and he didn't look well. He and his wife had just returned and their bags were still in the hall near the front door. It was a dream, his wife reminds him. He looks at her. As the doctor hands her a new prescription, she turns to her husband, explaining there will be three more pills to add to the regular five. He never believes he needs to take them- she comments, he thinks I am poisoning him.
The next patient comes to clinic with his son. They are speaking Greek when the team enters. He is small and bald and when the doctor asks him to walk down the hallway, he jogs. He exercises everyday. The doctor asks about the hallucinations, and the patient replies he sees them all the time, all kinds of people. Sometimes they walk next to him. Mostly they are happy. Women appear and beckon to him. Some are naked and lie in bed with him and his wife. He has woken in bed, wet with semen. When the dreams occur, he says he feels like dancing and waltzes side to side, his arms carrying an invisible partner.
The patient in the wheelchair hasn't used her feet in a long, long time. They are crooked underneath her white socks, with permanent contractures. Her head almost sits on her chest cavity, the bowing of her upper back is so pronounced. The mother who has been reading a magazine, doesn't comment on the others in the room as the doctor asks permission to let the medical student and writer attend. Clearly, she is bothered. The doctor laughs softly as the stuffed bear the girl holds in her lap speaks. He looks at the girl with short-cropped dark hair, and asks her a question. He wants her to hold out her hands and demonstrates. The girl creeps her right hand towards her mother's left arm and squeezes. Does she speak? He asks. No.
With cerebral palsy from birth, the daughter never speaks. Since the last appointment, the medicines have calmed her dystonic movements. Then the mother leans forward and asks for a recommendation for a Spanish- speaking psychiatrist. She says her daughter screams when people touch her, when she tries to change her diaper or brush her teeth, or change her clothes, or bathe her. When the daughter is tired of family company, she screams. She constantly grinds her teeth and refuses to drink. Mother puts her in her room, closing the door to scream alone. She fears neighbors will call the police, fears they think she is abusing her daughter. Mother confides she gave her daughter three times the dose of a drug to make the child manageable for the appointment.
What kind of quality of life is that? The doctor asks. Screaming is clearly no way to live.
Hushed
Friday, January 09, 2009
The patient's voice is so soft it's hard to believe he's not pulling some sort of joke. The voice or the lack of one doesn't change the doctor's regular speech pattern. He wants to know what medications the patient takes. The patient responds slowly and softly, indicating he doesn't know the dose of the pill he takes. The doctor gazes down at the chart at the list of medications and asks the patient how the drugs got in the chart.
"I remembered them." The doctor shakes his head in agreement but he's not convinced the patient is lacking more than a voice. He asks the medical student to get a mini- mental form, then asks the patient whether he knows where he is and what the date is. Satisfied somewhat the doctor questions the man about why he takes two pills per day. The pale man responds he had the sensation he was wearing long gloves on his forearms so he stopped increasing the dosage.
With two pills per day, the doctor can't say whether a patient would experience any relief from symptoms. He's irritated. It's been six months since the patient's last visit and he still can't determine whether the man is benefiting from the drugs. The doctor writes out a drug schedule, increasing half a pill every three days until the man takes up to two tablets three times per day. It's an outline, or a staircase the patient can go up and down on. The physician clarifies he wants the patient on a larger dose to determine whether levodopa is helping the symptoms or not. Signs of illness appear a little worse.
The man's noticed a slight drool from the side of his mouth, his facial expression seldom changes and his blood pressure is quite low. So low, the clinician worries an increased dose of levodopa will send it plummeting; he writes a prescription for florinef, which will keep blood volume high so the slight man won't faint when he stands up. Another worrisome symptom is anemia; patients with Shy- Drager Syndrome frequently are anemic. A stool softener, I notice in the chart, another sign of autonomic nervous system involvement.
We watch the slender man walk down the hallway. Is it the cell phone hooked to the belt that makes his left arm jut out? He breaks the turn in fractions rather than gliding through it. The forward head and rounded back catch in the doctor's teeth. He will recommend physical and speech therapy and will see him again in six months.