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.