Bikes and Ladders

Posted by & filed under Weekly Journal.

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

Posted by & filed under Weekly Journal.

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.

Lewy Body Disease?

Posted by & filed under Weekly Journal.

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

Posted by & filed under Weekly Journal.

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.

Sickness

Posted by & filed under Weekly Journal.

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.

Illness without a name

Posted by & filed under Weekly Journal.

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.

Parkinson’s disease versus Dementia with Lewy bodies

Posted by & filed under Weekly Journal.

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

Posted by & filed under Weekly Journal.

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.

Job

Posted by & filed under Weekly Journal.

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

Posted by & filed under Weekly Journal.

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.

Close

Your Name (required)

Your Email (required)

Subject

Your Question