Family Affair

Posted by & filed under Weekly Journal.

Every chair and stool in the small room has a person in it and the nurse and social worker stand against one wall. Few patients bring so many family members, but this man has Huntington’s disease, a genetic neurological disease characterized by chorea or spontaneous uncontrollable movements, and such patients tend to have non- typical families. The patient sits in his wheelchair. His face is unshaven; he wears glasses and keeps his hands fixed in his lap between his knees.
The nurse taking his weight and blood pressure notes he has lost fourteen pounds since his last appointment in June. The patient’s sister mentions this to the physician when he enters the room. The doctor asks the patient whether he has had trouble swallowing, and he shakes his head. The ex- wife comments when she comes by with meals he has been sleeping in bed, and she thinks he has been sleeping too much, and not eating enough. The doctor gazes at the patient telling him he sleeps like a teenager, while he informs the family in the room patients with HD have increased metabolic needs. He wheels his seat in front of the wheelchair, asking the stubbly- faced man how much exercise he gets, commenting he could use an exercise routine and to consider riding a stationary bike or walking every day. The sister remarks,
Well that went over well…
The patient rolls his eyes, as the sister concedes she has tried to get him to walk around the apartment complex he lives in, to no avail.
The doctor recommends Boost, Ensure or smoothies; added calories will thwart further weight loss. The patient’s voice is unintelligible, yet the physician knows him and the remark he makes. Heavier patients with HD tend to have an easier course of illness. The physician asks about the time the patient retires to bed at night, and the patient responds when he feels tired, around one, and sometimes later. The physician nods in understanding, commenting under those conditions one might sleep later in the morning, but not until 4:00pm. He examines the list of the patient’s medications on the computer and suggests decreasing the nighttime risperidone dose by half.
On physical examination, the patient feels rigid. The doctor inquires about when he first received his diagnosis, noting he is in the rigid phase of illness, which occurs after in advanced stages of the disease when most chorea has subsided. The ex- wife and sister agree it has been ten to eleven years.
Wearing a black dress and blue glasses, the social worker comments she is beginning a support group for those with HD, and their families. They will meet in the Orlando area. She takes several email addresses so various arms of the family will be informed about future activities revolving about Huntington’s disease.

Navigating the Seas: Parkies of the Caribbean

Posted by & filed under Weekly Journal.

We are eighty or so people of the three thousand on the Navigator of the Seas bound for the Cayman Islands and Cozumel. Scheduled are two days of lectures, workshops, dinners and gatherings organized by a travel agent and small group of women from the Parkinson Research Foundation, with Doctor Sanchez- Ramos specialist in movement disorders, Mary Spremulli- speech therapist and Eman Nakshabendi- dietician.
Dressed in pirate garb with ornate boots and a tricornered hat, Larry Hoffheimer, founder of the PRF and Brian Curro the director, welcome the group and explain briefly how the conference will proceed. Shorty after, we disband to our staterooms and are soon called by the captain to participate in the safety drill. The raging pirate theme draws heads as two perspiring buccaneer vixens pass passengers waiting shoulder to shoulder in the scorching sun of Broward County and Brian Curro attends the required drill dressed in his brigand costume, appropriately sweating into his eight-inch beard which adorns his naked and quite hairy ample belly. Others less ostentatiously dressed mingle among the ship’s population, though with some knowledge of Parkinson’s disease you may notice them. Some have the Parkinson jiggle of the hand, others the forward bent posture and quick steps. Some use wheel chairs, others use walkers, most have a caregiver at their elbow, or not far away. Many blend in, their disease going unnoticed by the general population.
In the conference rooms on days at sea we are something of a family, all somehow involved in an illness that is progressive, debilitating and has no cure. After twenty minutes or so in the warm meeting room, some heads nod forwards in slumber, while family members remain attentive to the details of the physician’s lecture. Mary Spremulli gives a lecture on Methods to Improve and Strengthen Your Voice on the second day. She shows before and after video clips of several of her patients to illustrate the changes that are possible after regular work with a fifty-minute exercise class that combines voice and physical movement. Eman Nakshabendi gives a lecture on the daily requirements for optimum health and addresses other concerns like the maximum dose of vitamin D. There is a bootcamp workout and yoga for patients and at the end, a session in which all are invited to probe the panel of healthcare professionals with questions.
On the eve of the final day we gather again and under the influence of rum punch, appetizers and gold coins of milk chocolate Larry Hoffheimer thanks all for attending and reiterates the major goal of the PRF is education. He invites us to attend again, and mentions another seminar to be held in Tampa, Florida in March, 2011. Before we break apart to attend the nightly show, peruse the venues of live music or visit the casino, we vote on the most dapper pirate. Three elaborate pirate patients walk the plank strutting their swords, mustaches, patched eyes and striped trousers. The guy with blousy striped pantaloons, an ample white smock with romantically wide sleeves, eye patch and kerchief draws whistles from the crowd. He stands bent at the waist though he was tall once, he face is static as a mask and his long legs take small shuffling steps.

Is the Illness PD??

Posted by & filed under Weekly Journal.

The couple is still not sure whether the spouse benefits from taking Sinemet. His expression is immobile and he sits like a statue in the seat across from the doctor, not twitching, not blinking, not moving at all. The wife answers most questions after considering them, she raises her hand, adjusts her glasses, pats her husband on the wrist, puts her head down as she thinks. All the simple random movements people make while in conversation, the husband lacks.
The patient feels he is rigid, but on physical examination, the muscles surrounding the joints are loose and supple. The patient walks well, swinging both arms though he takes small steps and turns using two feet, rather than spinning on one foot. The physician comments patients with lower body PD, also called vascular PD retain their ability to swing their arms.
Behind the desk, the physician considers the amount of Sinemet the patient takes daily, noting it is a moderate dose. He inquires into what the spouse has for breakfast and explains that dietary proteins and Sinemet compete for uptake into the brain. In order to maximize the effect of medication, many patients need to cut back on the proteins they ingest during breakfast and lunch, and have their day’s allotment of protein rich food in the evening, when they do not intend to go to the county fair, or dancing.
The wife responds they are accustomed to eating a large breakfast with eggs, bacon and cereal, as well as coffee with milk. Near three o’clock, they have their main meal and later in the evening, they have something light. The spouse realizes this is the reverse of what the physician is advocating. The patient walks in the mornings after breakfast, and he has an exercise routine he begins at five in the afternoon; he seems not to feel especially slow or encumbered by the amount of protein he takes in.
The doctor concedes he may not have true Parkinson’s disease, but may have a variant, like vascular PD, which responds less to dopaminergic medication. The physician urges the pair to try to cut down on the morning proteins, to see whether the spouse feels some difference- less slowness and less rigidity. If he is averse to cutting back on proteins, he can increase the daily dose of Sinemet to 2.5 tablets 4 times/day. They may also try accomplishing a blend of the two strategies, decreasing proteins a bit, and increasing the Sinemet a bit, with the goal to find out whether the medication decreases the patient’s rigidity and slowness of movement. If the patient feels no benefit from the medication, there is no reason to continue taking it. Sinemet is only for the relief of symptoms; it does not affect the underlying course of any disease.

Is this PD?

Posted by & filed under Weekly Journal.

The physician prefaces the clinical visit stating the patient has an atypical type of PD. Arriving with her daughter, the patient reveals her balance has gotten noticeably worse since December, her daughter nods her eyebrows high. The physician concedes Sinemet has little affect on gait and balance.
Two years ago, the older woman was hospitalized when she had an episode of being unable to move from her bed. The daughter explains the root cause was due to a urinary infection, and her mother decompensated. After several days, she recovered use of both limbs and was walking soon after. Looking for a potential stroke, physicians ordered an MRI of the brain, which revealed two old lacunar strokes, and diffuse changes due to insufficient blood flow caused by chronic microvascular disease. The doctor behind the desk explains when these sorts of changes occur in the front of the cortex, a syndrome of lower body parkinsonism results. Slowness and rigidity affects the legs much more than the hands and arms. People with this diagnosis typically have a full arm swing, yet small shuffling steps.
The patient agrees this seems to be her scenario; her feet and legs are much more impaired than her hands and arms. The low volume of the patient’s speech causes the physician to ask whether she has always had such a light voice. The daughter nods agreeing her voice has changed, and looks over at her mother who points out that sometimes she talks too fast and people fail to understand her. The doctor nods declaring the speech pattern is similar to how patients walk, with quick small steps, and he begins to explain that Parkinson’s disease impairs sensory integration. How do we know this is true? He answers his own question, stating when a patient with PD acquires additional sensory input, for example, a therapist instructs the patient to step directly on red stripes on the floor, by concentrating on the lines and placing the foot on each one, the patient is able to pass through a doorway she previously froze in midway.
Both the patient and her daughter are uncertain Sinemet is really helping her symptoms. In prior times, the doctor states, a patient would be admitted to the hospital for a drug holiday. All the medications would be completely stopped and then gradually reintroduced at much smaller doses. It is clear then, whether Sinemet actually relieves slowness and rigidity. Instead, because drug holidays are deemed extremely dangerous, the patient can now slowly cut back on the Sinemet, by eliminating a dose every other day. The goal is to discover whether the patient suffers from a deficiency of dopamine. He warns this may take several weeks, because some dopamine terminals may retain the ability to store an excess of the neurotransmitter. At any point in the process, the patient may decide she feels more agile with the medication, and then gradually resume building towards her customary dose. Discovering what exactly the patient’s body needs means she will not be taking medication needlessly.

Two Cases

Posted by & filed under Weekly Journal.

The fabric of her black trousers drapes over the angles of her thin thighs. The nurse did not weigh her today. She is known to have her blood pressure drop, and if she stands, she’s likely to pass out. Her torso bends forwards making a large hump form in the middle of her back. The caregiver urges her to sit upright in the chair, and holds her arm on the arm rest as the doctor summarizes her medical history, and how he thinks he can help her today.

He had thought she suffered from a variant of Parkinson’s disease, multiple system atrophy, though he is unsure whether she truly fits the diagnosis’ criteria. An MRI would enlighten him, but would probably not change her medical treatment; which is largely to keep her as comfortable and content as possible. To do this he would like to increase the Seroquel she takes at night, to ensure she sleeps for several hours without waking. Her left leg no longer moves and has to be placed on the footrest of the wheelchair. Her left hand has a resting tremor, and no reflexes can be coaxed from her. She is ninety- two and her family does not want to her to have a feeding tube placed, and her caregiver says she eats well.

From behind his desk, the physician asks the caregiver whether the patient ever speaks, and she nods and confides she tends to be bossy. The woman caretaker wears a short sleeve shirt exposing the tattooed calligraphy on both her arms. She has milk chocolate brown skin and long twisted dreadlocks worn away from her face. When the patient’s stringy hair falls over her face she brushes it back over one ear.

The second patient is tall, brown- haired and looms over the doctor as she strides into the small office and sits in the far chair. Her half- sister has accompanied her today and has gone to search for the patient’s purse, which hangs from the nurse’s shoulder, who takes the patient’s blood pressure. The patient comments her sister is probably lost, and the nurse wearing wine- colored scrubs, comments she will go look for her. She hands a large chrome and leather purse to the patient and leaves the room rolling equipment with her.

While the patient and doctor speak about her illness the patient’s face moves spontaneously. She grimaces slightly and extends her head backwards. She takes the movements in stride as the doctor comments her face seems to be moving more than formerly, and the patient adds she has noticed more movements of her face and f

When having a pedicure, the man at her feet had problems; her feet, ‘flapped around’. Switching the subject, she confides she was upset the staff at the VA failed to inform her that her husband, would not die right away after being diagnosed with rapidly progressive ALS. She began planning his funeral. She planned the funeral for fourteen months before someone enlightened her he would probably outlive her.

The half- sister who is tanned, slender and six years older appears, smiling. Her hair is cropped short and she wears a bright pink bow on the right side of her curly head. She explains she and her sister had different fathers, though they once all lived in Detroit. It was a different city then. The patient tells us she could take the bus all day. There was an amusement park and a theater; it was a proud city on the lake and a river.

The physician recommends speech therapy and Risperdal which will dampen the patient’s chorea, and she seems amenable to both. The physician inquires if the nurse coordinator has any cards the patient might carry, which say she has Huntington’s Disease- explaining her staggering and other symptoms, if she is stopped by the police or worried neighbors while walking her four dogs.

Patient from Boston

Posted by & filed under Weekly Journal.

The patient is from Boston and she pronounces the word head with two syllables. She wears her grey hair short around her face and the frames of her glasses are stylish and complement her eyes. Her spouse is small and Italian. He wears a hearing aid in his left ear, a turquoise polo shirt and he speaks loudly with the accent of a hitman. She scans the three women observing and tells them not to get old. She is seventy- five, but has the energy and demeanor of someone younger.
She says she has trouble eating, and adds perhaps that’s not a bad thing; referring to her rounded shape. The doctor asks exactly what seems to be the problem, because the typical parkinsonian tremble occurs at rest, not when engaged in activity. That sort of tremor is an action tremor, or an essential tremor, what Katherine Hepburn suffered from. He asks her to perform several dexterity measures, and her tremor appears slight, hardly incapacitating. The physician points at her and tells her she is not being honest with him and she concedes, that she feels the tremor is larger than it perhaps appears.
Though she currently takes two and a half pills of Sinemet three times a day, she feels little effect from the medication. The doctor asks what she eats for breakfast, and she tells him she had a bowl of cereal with milk. His eye brows shoot up and he exclaims the amino acid building blocks of proteins in the milk compete with the levodopa for access from blood to brain. That may be why she has noted no benefit from the medication. If she is uncertain whether her medications are working, she should attempt a purely vegetarian diet for a week or two, with as little protein as possible to see if the levodopa is working to alleviate her symptoms. A second solution is to increase the dosage of Sinemet (levodopa/carbidopa). He tests her arms and feels some cogwheel rigidity in the muscles. She writes a sentence for him to demonstrate the state of her penmanship, and her handwriting is miniscule. Yes, the physician nods, her writing is typical of someone with Parkinson’s.
The doctor asks the spouse whether he sees any improvement in his wife’s symptoms and he shrugs. He comments later she has told him she feels unable to move from her chair, and the physician nods and states initiation of movement is hard for those with PD, though when they get moving it becomes hard to stop. She mentions she exercises every morning and the other day performed thirty-one repetitions of sitting to standing. The physician praises her for exercising and encourages her to continue, telling her that is the best thing she can to for herself. He writes a new dosage schedule to increase her medication and arranges for a followup visit.

My Dad and PD

Posted by & filed under Weekly Journal.

For brief seconds, it appears he wears makeup to convince the world he is almost eighty. Odd patches of increased pigmentation dots his cheeks, brow and hairline. What is this disguise and why is my father wearing this old face?

In December he will turn eighty and my sisters would like to visit and celebrate. He sees no reason for a party, whether we would travel a hundred miles, or thousands. We discuss this on the walk home from the Indian restaurant; one of the few places open on a Sunday evening. His right knee is new, he calls it his DuPont knee. His swing is somewhat shorter on this side than the left, his shoe hits the ground at mid-foot under him, rather than hitting with his heel, in front. For the past few days, the left knee has been bothering him. He can’t recall how he might have strained it. The pace is slow, half as slow as the normal walking tempo. His chin tends to drift downward, and his gaze turns towards the ground, and his wife reminds him to put his chin up, and for a bit, his eyes settle on the scenery in front of him.

I see the tremor in his left hand when he is due for medication. He performs most movements slowly; bringing individual salads to the dinner table one at a time, rather than carrying a salad bowl in either hand. When he holds a mug or cup, the liquid inside lists precariously to the side.

In the middle of the night I hear him talking in his sleep. He’s having a conversation with someone. He talks loudly and articulately, then pauses as though listening for the person’s reply. He’s speaking again, and I wonder whether he’s really on the phone. I don’t understand the words, only the cadence of sentences and I drift back to sleep wondering about all the times I heard my father on the phone, while I slept in the adjoining room. Later he tells me the house we lived in had only eight hundred square feet.

When he was younger he cut his hair short, so short he looked almost balding. Now his straight white hair stands up. It covers his entire head and I muss my hand through it and tell him his hair is wonderful longer. His eyebrows retain some darker shades of grey and grow like wild bushes high and curling towards his brow. He seems content. When I ask him what he is doing when I catch him in his chair, he tells me he ‘s sitting. I take the chair in the other corner and sit with him.

Couple from Rotan

Posted by & filed under Weekly Journal.

The couple comes from Rotan, the islands off the coast on Honduras, to see the movement disorder specialist; there are no practicing neurologists in the country. The patient is rugged, tall and has a receding hairline and wide shoulders. His wife is his equal in beauty, slender with dark long hair, pale fair skin and a thick very English accent. She makes eye contact easily with the physician behind the desk, while her spouse’s eyes travel about the small room with fluorescent lighting and insufficient ventilation.
As the doctor sits, the wife explains it has been about six months since her husband first complained about the the fourth child in their home. She adds they have three children; the youngest is an eight year-old boy. It was dinnertime and the family sat down together. The fourth child was a boy named Hector, and he was making a horrible mess with the spaghetti on his plate. Her spouse became enraged at the illusory boy, for not responding when he insisted on him using a napkin. Later on in the evening she tried to sooth her spouse by telling him he must have been having a hallucination, because they have only three children. When her spouse had calmed he recognized they did truly have only three children, but he remained confused about the boy at the table.
His eyes drift to the doctor and he explains there is something wrong, and it began perhaps nine months previous, when he noticed himself unsteady on his feet. He had been playing golf, had drunk a beer and fell in a sand pit. The unsteadiness remained and never completely went away, though sometimes he is less stable than others.
The physician asks whether he feels stiff, and he nods and adds he feels slow, like a man older than he is. He feels slow in the head, sometimes forgetting his children’s names. In her seat the wife states her spouse is thirty- eight and used to be a professional rugby player, and remains active, completing his seventh Iron Man competition six months ago.
The doctor inquires whether he trained for the event through the symptoms, and he nods agreeing, stating his times were proof there was something wrong- he finished more than an two hours later than his previous race. The physician approaches the patient asking him to sit on the examination table. He looks at his eyes, asking the patient to follow the pen as he moves it within the limits of his gaze. He brings the pen up high above the patient’s eyebrows and the brown eyes travel some before they move no higher. And he has difficulty gazing downwards without flexing his neck forward. The physician comments that he appears unable to gaze in vertical directions. The symptom has a name, supranuclear palsy, which occurs in a variant of Parkinson’s disease known as Progressive Supranuclear Palsy (PSP).
In the hallway the patient walks with narrow based small steps. The physician explains after watching the patient, the syndrome derives its name for the characteristic traits of PD, along with several other notable problems- rigidity, especially of the axial muscles (neck and back), postural instability, and impaired voluntary movement of the eyes. In PSP, there may be a mild dementia, but in his case, the hallucinations and dementia make the Doctor suspicious of two other parkinson plus syndromes: cortical-basal-ganglionic degeneration and dementia with Lewy bodies. In dementia with Lewy bodies, the hallucinations can occur even before medication is started. In cortical-basal-ganglionic degeneration the parkinsonism is complicated by problems with voluntary gaze and dementia. In all of these parkinson plus cases, some patients respond to Sinemet, feeling decreased rigidity and slowness, though there is no cure for the progressive illness. The wife looks at her hands in her lap, as her husband asks whether anything he can do would have an effect on the course of the disease. The doctor looks at him directly and says the majority of patients who receive the diagnosis require some type of walking aid after three to five years, and are either wheelchair or bed bound in eight years. The doctor looks down at his own hands momentarily and comments all the physical activity he gets has probably forestalled the illness to some degree, and he should remain as active as possible and the medication may promote movement, by making him feel less rigid. The patient nods and the physician explains how to gradually increase the dose to achieve a therapeutic effect.

Tremor Predominant Parkinson’s Disease

Posted by & filed under Weekly Journal.

The patient who is a stocky wide- shouldered, healthy looking man, says he’s never slept through an entire night in his life. While making an extraordinary statement his face remains unexpressive, placid. His eyebrows and mouth convey no indication of emotion. The physician behind the desk shakes his head and comments the need for sleep varies considerably between people. Though few people respond to Seroquel so strangely. The first time the patient took the drug he slept for twenty-four hours straight. He describes trembling with cold for several hours on one occasion, on a separate night he blames the prescription for extreme tension, which made him pace through the house for hours. Clearly, he responds aberrantly to the medication.
The wife comments he thrashes during the night, sometimes calling out or shouting in his sleep; she reports this occurs three to four times a month. The doctor comments no one with PD has a normal sleep pattern and he describes how rapid eye movement sleep shifts to become out of phase with other sleep architecture- or wave patterns, allowing the person with PD to be able to move while dreaming. Essentially, the person is able to enact dream content, which is not possible in someone else whose sleep architecture is unaffected by illness.
The wife states they have been married forty- seven years. Her spouse leans towards her and comments they have been fighting for forty- six. An amiable banter flows between them. The wife offhandedly states her husband is an artist and describes the fine work he has been involved in, commenting he never has tremor when he’s working, it’s only later on when he’s resting. The physician responds,
“… that’s why it’s called a resting tremor…”
The patient asks whether he can increase the Mirapex he takes while experiencing stressful events, for example he is due to undergo eye surgery to for glaucoma- to release pressure on the orbits of his eyes. It requires the ophthalmologist create holes in the membrane at the back of the eye. The doctor with a head full of wavy and graying hair nods,
“Certainly, there is no contraindication to the drug.”
On physical examination, the doctor finds some rigidity on the man’s left side, and in the neck. When asked to raise his shoulders, he barely shrugs. His walk has little arm swing on either side, and when they retake their seats, the doctor asks how long ago was it when you first came in?
The wife thinks it was 2008, and the records on the computer confirm her guess. In 2008 the patient stated he had experienced symptoms of illness for five years prior to visiting a neurologist. He still gets sufficient symptomatic relief from Mirapex (pramipexole) a dopamine agonist, to avoid Sinemet. The doctor comments that most people require Sinemet after a year or two of treatment with an agonist, and he is an unusually fortunate case.

Teacher with Drug-Induced Parkinsonism

Posted by & filed under Weekly Journal.

The patient taught middle school for forty years, and she sits without leaning on the back of her chair. She comes for a consultation about whether she has Parkinson’s disease. Her husband has come with her, as a witness to the changes he has seen in her health. Her falls scared both of them. In the most recent, the patient carried groceries in each arm. She fell straight forward and broke her nose. When on the floor she was unable to rise without assistance.

The medical history of the patient has some red flags for the doctor; the patient doused her garden with spectricide and the toxin caused her thyroid to quit functioning. He mentions that there is a relationship, though not a causal one, between Parkinson’s disease and exposure to pesticides, heavy metals and other environmental toxins. She comments she has suffered from trigeminal neuralgia, inflammation of the fifth cranial nerve that produces intermittent bouts of shooting pain to the side of her face and jaw. Her husband notes he has seen tremor in her hands and a stiff walk, while the patient says she has experienced left- sided weakness, fatigue, forgetfulness, and problems with bladder control.

The patient has taken some medications that may have deleterious side effects. Pravastatin, being one of the cholesterol- lowering statins, recently made the news for its under- reported tendency to invoke muscle pain and weakness, especially in the legs. Her primary care doctor added Abilify, a novel tranquilizer to her medications when he thought Prozac was inadequate for her symptoms of depression. Abilify can block dopamine receptors and produce some signs and symptoms of parkinsonism and in addition can induce tardive dyskinesia in patients, uncontrollable movements of the face, tongue or other body parts and these may wane if discontinued, or become permanent with continued treatment.

On physical examination, the doctor finds no stiffness or rigidity in her muscles, and her gait is normal with a full arm swing. He comments that he can detect no signs of parkinsonism. When he places a tuning fork on the bones of her foot, and she is unable to feel the metal buzzing, though she can feel the vibration in the knuckles of her hands. She is able to discern whether her toes are up or down, but her perception of temperature is also impaired. The doctor tells her that she does not have the clinical features of Parkinson’s disease now. She may have had some symptoms of parkinsonism while taking Abilify, but those have gone away after stopping the medication. Based on her examination he diagnoses a peripheral neuropathy to explain some of her symptoms. The cause of her neuropathy will require more extensive evaluation. A B-12 deficiency, low thyroid function, medications or toxic insult are possible causes of neuropathy. He conjectures a toxic bath, like the kind she experienced, might result in a neuropathy, though the lower extremity problem resembles what a diabetic patient might incur. He urges her to see another physician whose specialty is the peripheral nervous system. He hands the patient and her husband a referral form. The other specialist will thoroughly explore the function of other nerves (nerve conduction studies), and order the appropriate blood and other tests that will aid in ruling out other disorders.


Your Name (required)

Your Email (required)


Your Question