Short article on Mississippi politician who had PSP

Tuesday, December 25, 2007

Ex-Mississippi Lt. Gov. Evelyn Gandy dies
Associated Press

JACKSON, Miss. — Evelyn Gandy, the first woman in Mississippi ever elected to the offices of state representative, state treasurer, insurance commissioner and lieutenant governor, died yesterday at her home near Hattiesburg. She was 87.

In a time when few Mississippi women earned law degrees or entered politics, Gandy did both successfully.

Former colleagues said she was determined to be fair to those she served, and she displayed a quiet demeanor that belied her political strength.

“She was absolutely a public servant in the classic sense of the words,” said Jackson attorney John Corlew, who served in the Senate from 1976-80 when Gandy, a Democrat, was lieutenant governor. “She had no interest in doing anything but the utmost for the public good.”

Gandy had suffered from Progressive Supranuclear Palsy, a disease similar to Parkinson’s disease, said Carroll Ingram, a law partner of Gandy’s.

Wayne Dowdy, chairman of the Mississippi Democratic Party, said Gandy “left behind a legacy of major achievements.”

In 1943, Gandy was the only woman in her graduating class at the University of Mississippi School of Law. She was elected to the Mississippi House in 1947.

In 1960, she became the first woman elected state treasurer; 12 years later she became the first female state insurance commissioner. In 1976, she became the state’s first female lieutenant governor.

She fell short in two bids for the governorship, in 1979 and 1983.

Gandy is survived by several cousins. Funeral arrangements were incomplete yesterday.

Rare variant of PSP – pallido-nigro-luysial degeneration

Here’s an abstract about a very rare variant of progressive supranuclear palsy (PSP) – pallido-nigro-luysial atrophy (PNLA).  The article is based on brain bank research from Mayo Jacksonville and Mayo Rochester.  Researchers found:

“The clinical course of PSP-PNLA, however, was different, with earlier gait abnormalities and difficulty with handwriting, but later falls, rigidity and dysphagia than PSP.”

According to the full article, dizziness can also be a symptom of PSP-PNLA.

The abstract is copied below.



Brain. 2007 Dec 24 [Epub ahead of print]

Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy.

Ahmed Z, Josephs KA, Gonzalez J, Delledonne A, Dickson DW.
Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL and Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Pallido-nigro-luysial atrophy (PNLA) is a rare disorder that in many cases has histopathological features similar to progressive supranuclear palsy (PSP). In a pathological series of over 400 cases of PSP, eight cases were noted to have features similar to those described in PNLA, including severe atrophy and neuronal loss in the globus pallidus, substantia nigra and subthalamic nucleus, in addition to many axonal spheroids in the globus pallidus and substantia nigra. These eight cases of PSP-PNLA were compared to 11 typical PSP cases with quantitative neuropathologic indices and assessment of demographics, clinical features and the timing of clinical features. PSP-PNLA cases were younger, had longer disease duration and more often were not initially diagnosed with PSP; in the end, they did not differ from PSP with respect to any major clinical feature. The clinical course of PSP-PNLA, however, was different, with earlier gait abnormalities and difficulty with handwriting, but later falls, rigidity and dysphagia than PSP. Pathologically, the same types of lesions were detected in both PSP and PSP-PNLA, but there were differences in the distribution and density of tau-pathology, with less tau-pathology in motor cortex, striatum, pontine nuclei and cerebellum in PSP-PNLA. These clinical and pathological findings suggest that PSP-PNLA should be considered a variant of PSP.

PubMed ID#: 18158316  (see for the abstract only – available at no charge)


Another WSJ article on antipsychotics (12/20/07)

The Wall Street Journal is really hitting hard on this topic — nursing home use of antipsychotics. They’ve had two front page articles in 2 weeks. Here are some excerpts from today’s article:

* “Use of a new generation of antipsychotic drugs to control the behavior of dementia patients has surged in recent years, despite the Food and Drug Administration’s ‘black box’ warning labels that these drugs can increase the risk of death for elderly dementia sufferers.”

* “About 30% of nursing-home residents are on antipsychotic drugs, according to the Centers for Medicare & Medicaid Services, most of them on newer ones called atypical antipsychotics.”

* “The challenge of caring for rising numbers of seniors who suffer from dementia and the behavior problems that can stem from it has provoked a wrenching debate among nursing-home operators, regulators and families. There are few effective medicines to manage the outbursts of Alzheimer’s patients — behavior that can overwhelm family members trying to care for a loved one at home, and strain the resources of those trying to maintain order in nursing facilities.”

* “Medicaid in 2005 spent $5.4 billion on atypical antipsychotic medicines — more than it spent on any other class of drugs… Atypical antipsychotics are approved for schizophrenia and bipolar disorder. But in what is known as ‘off label’ use, doctors often prescribe the drugs to elderly people with dementia.”

* “According to CMS, nearly 21% of nursing-home patients who don’t have a psychosis diagnosis are on antipsychotic drugs. A 2005 study, published in the Archives of Internal Medicine, found antipsychotics were prescribed not only for psychosis, but for depression, confusion, memory loss and feelings of isolation, says the study’s author Becky Briesacher.”

* “Family members can object to the use of such drugs. But they risk having the facility threaten to discharge their relative on grounds that they pose a danger to themselves or others.”

Here’s the link to the article:

Nursing Homes Struggle To Kick Drug Habit
New Therapies Sought For Dementia Sufferers;
Music and Massages
Wall Street Journal
By Lucette Lagnado
December 20, 2007; Page A1


Article on use of antipsychotics (WSJ 12-4-07)

This will be of interest to those who are taking or those whose loved ones are taking antipsychotic medication. (Traditional antipsychotics include Haldol; atypical antipsychotics include Seroquel, Zyprexa, Risperdal, and Clozaril). This is a recent front-page Wall Street Journal article on the subject that has been getting a lot of attention. Here are a few excerpts from the article:

* “In recent years, Medicaid has spent more money on antipsychotic drugs for Americans than on any other class of pharmaceuticals — including antibiotics, AIDS drugs or medicine to treat high-blood pressure.”

* “Nearly 30% of the total nursing-home population is receiving antipsychotic drugs, according to the Centers for Medicare & Medicaid Services, known as CMS. In a practice known as ‘off label’ use of prescription drugs, patients can get these powerful medicines whether they are psychotic or not.”

* “Nursing homes often find it difficult to balance the demands of caring for certain patients against the pressure to keep staff costs down. The economics of elderly care can work in favor of drugs, because federal insurance programs reimburse more readily for pills than people.”

* “America is facing a public health crisis over the care of those with dementia, Dr. Pollock says. ‘We are left with the atypicals because we have nothing else,’ he says. These drugs have a role to play, he says, but ‘nonpharmacologic treatments’ should be tried first.”

* “The Food and Drug Administration issued a ‘black box’ warning on using the drugs for dementia patients in 2005. … Some doctors are now switching back to older, cheaper antipsychotics, such as Haldol, the FDA says. The older drugs had fallen into disuse, but don’t have a black-box warning. Now, the FDA says it’s weighing putting a black-box warning on those drugs, too.”

Here’s a link to the article:

Prescription Abuse Seen In U.S. Nursing Homes
Powerful Antipsychotics Used to Subdue Elderly; Huge Medicaid Expense
By Lucette Lagnado
Wall Street Journal
December 4, 2007; Page A1


“Doctor is touched by…woman’s devotion” to her husband with locked-in syndrome

Many of us in the PSP and CBD communities feel as though our family members are “locked in.”  This is a sweet article in the LA Times by a physician who was touched by a woman’s devotion in caring for her husband with locked-in syndrome.



In watching a wife tenderly care for her husband, who can blink but can’t move, a doctor is touched by the woman’s devotion.
By Steve Dudley, Special to The Times
December 17, 2007

In a corner of a room he shares with three other residents at the nursing home, a patient with a curious illness stares out the window at an empty bird-feeder.

He holds his arms in a curious, characteristic way — elbows and wrists hyperflexed so that his hands are tucked under his chin, like a child in prayer.

Mr. Fletcher has been bedridden for years. He can’t talk, can only moan. He can’t move other than to blink or shift his gaze. The only sign of responsiveness is that his eyes will sometimes follow stimuli, either of voice or hand movement.

Although he cannot move, his sensations are still intact. This means that Mr. Fletcher can still feel pain like you or I, or have that annoying itch that he cannot scratch. And people like Mr. Fletcher have their reasoning fully intact. They can think, dream, hope and reflect upon their endless prison.

Mr. Fletcher has what is commonly referred to as “locked-in syndrome” — the result, occasionally, of a rare form of stroke, happening viciously and suddenly, often striking down people in the prime of life after head trauma.

In Mr. Fletcher’s case, it happened more slowly. He has supranuclear palsy, a degenerative condition of the central nervous system that usually afflicts the very elderly, although Mr. Fletcher is only in his early 60s. It’s a cousin of Parkinson’s and Alzheimer’s diseases, an insidious condition with symptoms that might start as mild gait abnormalities or difficulty with eye movements and gradually develop over years.

Nobody knows what causes it, and treatments are essentially palliative: You really can’t do a thing for those who suffer from it other than to keep them comfortable.

Mr. Fletcher has been admitted for some rehabilitation as he transitions from the hospital, after a partial bowel obstruction, back to his home. A team of therapists, nurses and aides and, most importantly, his wife, is here to care for him. I suspect that his wife, the one with the least formal training, understands him best.

I’d like to tell you about her.

I am visiting another patient in Mr. Fletcher’s room when Mrs. Fletcher arrives and proceeds to wash him with a moistened towel.

Carefully and tenderly, she alternates between wiping and drying, turning him first one way, then another. Patiently, meticulously and ever so gently, she bathes him.

After she finishes with the bathing, she rubs her husband down with lotion, massaging it into his skin until it is supple. Then the shaving begins. She lathers his face and, with smooth, efficient strokes, manages to remove a three-day growth of stubble. You can see his eyes sparkle as she winds up her ministrations.

I try not to stare. It seems intrusive, to watch them together, intimate, in an interaction almost as private as making love.

She is caring for this man for all she is worth. Being with him, cleaning him, giving of herself to him and receiving at the most a blink or grunt in return.

Now that’s love. That’s commitment. In a time when people get divorced for the most superficial of reasons, it is a tremendous encouragement to see true love in action.

Mr. Fletcher is a handsome black man with beautiful ebony skin. His wife is Vietnamese. I imagine they met during the Vietnam War when he was a dashing GI and she was a scared young woman whose homeland was being split in two.

As I watch her bathe him, I reflect on what their early years together might have been like. Running on the beach, staying out till dawn, picnics, favorite restaurants and lots of dancing — they look like a couple who used to dance a lot.

I read of a young guy over in France, barely in his 40s, who became locked-in after a stroke. He had been a magazine editor. With the patience of Job, he dictated a book by blinking to a scrivener.

He referred to himself as feeling like a captive in a diving bell. One can only imagine the terror and loneliness of being in this state. You’re completely dependent upon others for all your needs, though you cannot voice any of them. Too warm? Too cold? Too bad.

And the people who are there to help often treat you as someone who cannot understand, as if you’re in a coma.

I’ve seen only one other patient who was locked-in, and that was back in medical school. We were on “physical diagnosis” rounds. That’s where a group of sleepy-eyed doctors in training dutifully follow their chief resident all over the hospital looking for interesting cases.

We popped into this guy’s room. I can’t remember his name. For that matter, I don’t know if we were ever told. He was just the interesting case on the eighth floor. As we lined the bed, we were grilled by a supervising resident about the case before us. This drill, known as “pimping,” is endorsed as a useful, hands-on, didactic exercise — and it may serve its purpose, but more commonly it devolves into brash showmanship at best and downright intellectual bullying at worst.

And we were pimped about the poor gentleman who was locked-in. He just lay there, hearing every word we spoke about him.

Not once did anyone address him directly or acknowledge that he was a person of any real worth. He was simply an exercise in learning, a specimen.

As I watch Mrs. Fletcher, I reflect on that experience. I am struck by the contrast between a devoted wife and a group of doctors-in-training making their cursory visit before moving on to the next interesting case.

She is so kind to him, devoted, caring. I suppose that is what the marriage vows mean when they speak of “in sickness and in health.” Here is tangible evidence of someone being faithful to that promise. I’m sure it is way more than she bargained for, but she dispatches her duties willingly, without resentment.

When you’re young, you take your health for granted. Slowly, it is taken away from even the strongest of us. That day, Mrs. Fletcher taught me volumes about the timelessness of love and the gift of health.

Steve Dudley is a family physician in Seattle.



Gaze Control and Foot Kinematics: PSPers Climbing Stairs

This abstract about a progressive supranuclear palsy (PSP) study was published recently on PubMed.  The conclusions were not surprising.

Here’s one:

“Deficits in gaze control may influence stepping behaviors and increase the risk of trips or falls during stair climbing.”

The full abstract is copied below.



Physical Therapy. 2007 Dec 11 [Epub ahead of print]

Gaze Control and Foot Kinematics During Stair Climbing: Characteristics Leading to Fall Risk in Progressive Supranuclear Palsy.

Di Fabio RP, Zampieri C, Tuite P.
Department of Physical Medicine and Rehabilitation, and Graduate Programs in Neuroscience, University of Minnesota, Minneapolis, MN.

BACKGROUND AND PURPOSE: Does gaze control influence lower-extremity motor coordination in people with neurological deficits? The purpose of this study was to determine whether foot kinematics during stair climbing are influenced by gaze shifts prior to stair step initiation.

SUBJECTS AND METHODS: Twelve subjects with gaze palsy (mild versus severe) secondary to progressive supranuclear palsy were evaluated during a stair-climbing task in a cross-sectional study of mechanisms influencing eye-foot coordination. Infrared oculography and electromagnetic tracking sensors measured eye and foot kinematics, respectively. The primary outcome measures were vertical gaze fixation scores, foot lift asymmetries, and sagittal-plane foot trajectories.

RESULTS: The subjects with severe gaze palsy had significantly lower lag foot lift relative to lead foot lift than those with a mild form of gaze palsy. The lag foot trajectory for the subjects with severe gaze palsy tended to be low, with a heading toward contact with the edge of the stair. SUBJECTS:with severe gaze palsy were 28 times more likely to experience “fixation intrusion” (high vertical gaze fixation score) during an attempted shift of gaze downward than those with mild ocular motor deficits (odds ratio [OR]=28.3, 95% confidence interval [CI]=6.4-124.8). Subjects with severe gaze shift deficits also were 4 times more likely to have lower lag foot lift with respect to lead foot lift than those with mild ocular motor dysfunction (OR=4.0, 95% CI=1.7-9.7).

DISCUSSION AND CONCLUSION: The small number of subjects and the variation in symptom profiles make the generalization of findings preliminary. Deficits in gaze control may influence stepping behaviors and increase the risk of trips or falls during stair climbing. Neural and kinematic hypotheses are discussed as possible contributing mechanisms.

PMID: 18073265  (see for this abstract)

“For many, ‘Do Not Resuscitate’ too painful to discuss

This article is on DNR orders; it’s from this Monday’s Boston Globe. Everyone should discuss these orders with family members!

We were very lucky in this regard. I signed the paperwork to place Dad on DNR on a Tuesday, using his Living Will and POLST as legal and moral support for the decision. He died that Friday, peacefully. … o_discuss/

For many, ‘Do Not Resuscitate’ too painful to discuss
Relatives, doctors often delay in offering patients the option

By Patricia Wen, Globe Staff
The Boston Globe
December 3, 2007

CHELMSFORD – The gray official form, labeled “Do Not Resuscitate,” lay for months at the bottom of Linda Batson’s dresser drawer. She had wanted her ailing 85-year-old mother to sign it.

Last Christmas, Batson’s mother, suffering from a terminal liver disease, had nearly collapsed inside St. Mary’s Church. She had later told her only daughter that she was ready, if her heart stopped, to “join Dad.” Batson knew that a DNR order would make clear to medical workers her mother’s desire to forgo emergency measures, and relieve her of the burden of communicating those final wishes.

Batson had trouble raising the subject to her mother, however. At Batson’s request, her mother’s longtime doctor had discussed the DNR form during an office visit in June, then handed them the gray document saying, “Go home and think about it.” But, as the two watched soap operas and took strolls in the following weeks, Batson put off the talk. Her mother grew weaker.

One evening this fall, she turned to her daughter.

“Linda’s going to make supper,” she said matter-of-factly from her living room chair.

“I’m Linda!” Batson replied.

“No,” her mother said. “You’re my mother.”

For families facing the impending death of a loved one, few topics trigger more anguish than the Do Not Resuscitate order. The subject can be so painful that relatives and doctors wait too long, until the patient’s mental capacity wanes and the tough decision is left to family members. There is little ambiguity in a DNR order: Emergency medical staff must withhold CPR and other life-reviving treatments if the patient’s heart or breathing stops, allowing death.

“This is the part of medicine where there’s finality,” said Dr. Wayne Saltsman, the Lahey Clinic geriatrician for Batson’s mother, Lee Russell, and her late husband. “That’s why these discussions are so hard to have. We’re talking about the end.”

The reluctance among doctors and family members to initiate these talks runs so deep that, three decades after DNR orders were introduced, their use remains spotty. Now the Department of Public Health is exploring whether to adopt a new kind of form, used in six other states, that could make the process easier. Called POLST (Physician Orders for Life-Sustaining Treatment), the document asks for a patient’s preferences on CPR, but also allows patients to make decisions about use of other life-sustaining interventions, such as intravenous fluids, antibiotics, and breathing machines.

The order would have to be signed by a doctor or nurse practitioner, and the bright pink form is designed to be carried by a patient and honored at hospitals and nursing homes, as well as by emergency medical technicians.

With a Massachusetts DNR order, in contrast, patients’ wishes must be redocumented each time they enter a new hospital, nursing home, or other medical facility, and a physician’s signature is required each time.

Also, a special DNR must be obtained for the home, if the patient wishes to die there without the interference of EMTs responding to a 911 call.

“We can’t withhold CPR if the Do Not Resuscitate order is not filled out completely,” said Lieutenant Christopher Stratton of Boston’s Emergency Medical Services.

In some cases, he said, his ambulance crews have had no choice but to try to revive an elderly patient in cardiac arrest, even though family members insisted their loved one wanted to be left alone or they showed incomplete, or unsigned, DNR forms.

Stratton said properly completed DNR orders for the home should be placed prominently, such as on the refrigerator door.

A DNR order is typically discussed only with the elderly and patients with serious chronic conditions. Among end-of-life documents, it is the least well known. According to a 2005 survey of Massachusetts residents over the age of 50, roughly one out of three had never even heard of DNR orders. Yet, nearly everyone was familiar with healthcare proxies (a legal document that authorizes a person to make medical decisions for someone who is incapacitated) and living wills (written guidelines for end-of-life care), though that document is nonbinding in Massachusetts.

Saltsman, who tries to raise the subject of DNR orders for all his elderly patients, said families ignore them at considerable risk. For the elderly and very sick, aggressive CPR often breaks fragile bones, and causes internal bleeding.

One national study found that less than 5 percent of chronically ill elderly patients revived by CPR live long enough to be discharged from the hospital, and the fraction that survive are a far weaker version of themselves, often neurologically impaired.

“There is a strong likelihood they are not the same person as they were before,” Saltsman said.

Difficult image to bear

For Batson, it was the image of her frail mother being revived and connected to a breathing machine without being able to speak that caused her to dwell on a signed DNR order. She and her brother were committed to having their mother spend her final months in her home, not a nursing home. They had to make sure their mother’s wish to die without CPR would be honored under all circumstances, including if an EMT arrived in her Chelmsford apartment.

When autumn came, Batson had come to the dreaded realization that she – as healthcare proxy – might be the one to have to sign the order. Her mother’s mind was clearly declining precipitously, especially after a brief hospitalization in July for chest pains. By late summer, Batson was living full time with her mother, chasing away fictitious cats that her mother claimed were roaming the apartment. Batson also had put up “No Children Allowed” signs outside the bedroom door, playing along after her mother complained that children were hiding under her bed.

Batson regretted that she had not pulled the DNR form out of the drawer, pushed away her fears, and talked directly with her mother about the document. Her mother had not raised the topic either, and at times Batson wondered whether her silence had some kind of meaning. But then she would remember that when her father was dying, her mother had been clear that she wanted to go peacefully. So Batson said nothing, wanting her mother’s dwindling days to focus on happier times.

She often flashed back to the mother of her past. Married to Edward Russell, the Tennessee native raised two children in Lexington while working full time as a lab technician. A slender woman with brown hair, she was a talented seamstress who tended the garden with her husband. She was at a loss when her husband of 61 years died in 2005, though she stayed busy keeping up with the lives of her two grown children, three grandchildren, and four great-grandchildren.

Throughout the past year, it was so hard to bring up any topic that would remind Batson – and her mother – of the end. But having worked in elder care, Batson has always believed the elderly deserve the right to control their own end-of-life medical decisions as much as possible and sign their own DNR forms.

“I wished I had done it earlier,” she recalled. “We should have just done it.”

On the Monday morning before Thanksgiving, Saltsman rang their apartment buzzer, responding to Batson’s request to make a house call on her mother.

When the doctor greeted Russell, who held a walker, she recognized him instantly, letting out her trademark giggle. Batson said her mother has always adored Saltsman, chair of Lahey’s geriatric medicine department, usually responding to any encounter with him “as if Elvis had arrived.”

For at least a half an hour, he talked with Russell in the living room, and he quickly detected her mind was not all there. She could not remember that Thanksgiving was fast approaching. Batson’s mother also asserted that her brother was in the apartment, but he was nowhere in sight. Also, she talked about longstanding plans to move to a new home, even though there were no such plans.

Russell’s liver condition, called primary sclerosing cholangitis, can cause a troubling buildup of toxins that creates mental confusion.

“My concern is whether she has the mental capacity to make informed decisions,” he said later. “She did not have it.” This meant Russell was not legally able to sign the DNR form.

Saltsman left Russell in the living room with her home health aide, and joined Batson in the kitchen. He had long believed a DNR order was the right thing for Russell, and now it was entirely up to Batson. She had already given this topic so much thought, and she was sure her mother would not want to be revived with chest compressions and plastic tubes.

At the kitchen table, Batson told Saltsman she was ready to complete the DNR form. The doctor signed, and then Batson added her signature.

The document is now taped to the refrigerator door.

French case report: probable DLB but autopsy confirmed CJD

Here’s an interesting case report in a French journal where the patient was initially thought to have probable dementia with Lewy bodies (DLB), then suffered an epileptic event, and upon autopsy was proven to have Creutzfeldt-Jakob disease (CJD or “mad cow disease” in humans). The abstract is copied below.


Psychologie and Neuropsychiatrie du Vieilessement. 2007 Sep;5 Suppl 1:10-8.

[Pitfalls in the diagnosis of epilepsy in the elderly.][Article in French]

Harston S.
Pôle de Gériatrie, CHU de Bordeaux, France.

The current increase of life expectancy amplifies the visibility of the higher prevalence of epilepsy during the course of aging, first described in the 1970’s. The epileptic symptoms do not fundamentally differ after the age of 75 from those met in younger adults, but the increase in the frequency, on one hand, of other neurological conditions (especially dementias and their psycho-behavioral complications, and strokes) and, on the other hand, of non-specific geriatric syndromes such as delirium, can result in diagnostic mistakes detrimental for appropriate geriatric care. We report the case of a seventy-eight year old female patient who initially presented as a probable dementia with Lewy bodies, then featured a status epilepticus mainly revealed by a delirium first related to an iatrogenic event, followed by partial recovery, then presented a reversible non-situational status epilepticus, and was finally proven to have a Creutzfeldt-Jakob’s disease. If dementia is nowadays considered as one of the major elements to be taken into account in the organization of neurological and geriatric care, a pluridisciplinary approach should, in the same way, better define the place of diagnosis and care of epilepsy in older patients.

PubMed ID#: 17875496 (see for English-language abstract only)

PSP Blurb in Neurology Now, Nov/Dec ’07 (by Golbe)

Neurology Now is a publication for patients and families on neurological topics. This month’s issue has an “Ask the Experts” question on PSP. It’s written by Dr. Golbe and has no content we haven’t already seen.

I have emailed Dr. Golbe in the past as to why he recommends anticholinergics (Ambien, Detrol, Oxybutynin) to those with PSP, and have never received a reply. Maybe someone else can give it a shot!? … -00023.htm

Neurology Now: Volume 3 (6), November/December 2007, p 37

My husband was just diagnosed with progressive supranuclear palsy. Is there any treatment for this disease?

Answer by Lawrence I. Golbe, M.D., professor of neurology at the University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School. (He is also director of Research and Chair of the Scientific Advisory Board of CurePSP, the Society for Progressive Supranuclear Palsy.)

Progressive supranuclear palsy is a rare brain disorder that causes serious and permanent problems with control of gait and balance. People with this disease may also experience changes in their mood and behavior (such as depression and apathy), as well as difficulty with organizing mental tasks and dealing with abstractions. These problems slowly worsen over time. Eventually, most people also develop problems with swallowing and eye movement.

The drugs levodopa-carbidopa (Sinemet) or amantadine (Symmetrel) may provide modest and temporary relief for the limb stiffness that interferes with movement. However, dosages of amantadine beyond 100 mg twice a day may cause confusion, and if there is no benefit within a week, you should stop taking it. The levodopa-carbidopa should be titrated up (meaning the doctor will increase the dosage level until it produces an effect) to at least 1,200 mg per day before concluding that it is ineffective.

Donepezil (Aricept) or other memory drugs intended for Alzheimer’s disease may help with dementia symptoms. Depression may be treated with antidepressants, sleepiness with modafinil (Provigil), and insomnia with a sleeping medication such as zolpidem (Ambien)-however, it usually is not a good idea to take modafinil and zolpidem together because they have opposite effects. The urinary urgency may respond to tolterodine (Detrol) and oxybutynin (Ditropan), though these drugs often worsen the constipation that comes with the disease.

A swallowing evaluation, during which a doctor will usually take an x-ray of the person swallowing, should be performed as soon as he or she starts to cough on liquids. The disinhibition that is often part of this disease is a result of degeneration of the brain’s frontal lobe and may cause people to overload the mouth or to suddenly stand and run while eating.

While physical therapy helps little with balance, it can help doctors determine the best devices to aid gait and can be also be used to prescribe a safe exercise regimen to improve joint flexibility.