Constant groaning in PSP (case report)

This recently-published letter to the editor in a medical journal article describes “characteristic constant groaning” in late-stage PSP. The authors, members of a top PSP researcher team in the UK, believe that the constant groaning is “often misinterpreted as due to pain.”

They state:

“We have seen this phenomenon in at least 4 patients in the last two years. All of them presented with constant groaning only when they were in the advanced stages having lost ambulation and being confined to a wheelchair (equal to stage V). This phenomenon is very distressing for their caregivers…”

Indeed, over the last 7 years that I’ve been learning about PSP, many, many caregivers report this symptom of constant groaning on the PSP Forum and elsewhere. Sometimes it’s described as growling, moaning, or humming. I have read many guesses over the years as to why those with PSP do this including clearing the throat, warming up the throat before trying to speak, wanting to stay involved in the conversation, expressing a complaint about something, and comforting oneself.

My father (autopsy-confirmed PSP) had what we called the “moanin’ and groanin” symptom but it was NOT limited to late-stage PSP for him. He groaned for the last 18 months or so of his life. Sometimes it was very loud, and I’d ask him to keep it down a bit; he was able to adjust the volume. Or I’d put my hand back and forth over his mouth so that the sound alternated from loud to muffled; that would make Dad laugh and that stopped the moaning for a short while. Sometimes I’d moan along with him, and found it physically very hard to do for as long as he could do it for. For him, it started as a means of expressing a complaint. Later, it turned into something he did seemingly without a purpose. I’d call him the “ole groaner”; that would get a laugh (when he could laugh).

The authors state:

“[The] groaning in PSP can be seen as analogous to other ‘noise-making’ phenomena which have been described in patients with Alzheimer’s disease, vascular dementia and frontotemporal dementia, such as persistent screaming, perseverative vocalization, continuous chattering, muttering, etc., all together characterized as ‘inappropriate vocalization, due to frontal lobe damage or interruption of subcortical circuits’. What makes this groaning so characteristic of PSP is the combination of the characteristic spastic-hypokinetic dysarthria with perseverative vocalization due to frontal disinhibition…”

I’ve only seen it briefly mentioned in one medical journal article a few years ago (also authored by members of the UK team). Now, this is quite a bit of attention given to one little-discussed symptom.

When I posted this case report on the PSP Forum, the moderator Ed Plowman replied as follows with his experience about his late wife Rose:

Groaning and moaning is common in mid- to late-stage PSP. Rarely does it mean the patient is in pain (but go ahead and ask). It MAY be related to the same kind of progressive nerve damage that results in unintended and uncontrollable-by-the-patient laughter or crying in an earlier stage. Often, my late Rose was unaware of the moaning until I called attention to it; that would seem to get her mind focused elsewhere, and it would stop. I stopped making an issue of it except periodically when I would ask if she was in pain or feeling bad, and in time, that phase of the symptoms went away.

Here’s the citation for the case report, if you’d like to look it up:

Parkinsonism and Related Disorders. 2011 May 13. [Epub ahead of print]

Characteristic constant groaning in late stage progressive supranuclear palsy: A case report.

Stamelou M, Rubio-Agusti I, Quinn N, Bhatia K.
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, United Kingdom; Department of Neurology, Philipps University, Marburg, Germany.

PubMed ID#: 21571571 (see pubmed.gov for this citation only)

Robin

Hallucinations – rare in PSP and CBD

The authors review the prevalence of and treatment options for hallucinations in Progressive Supranuclear Palsy, Corticobasal Degeneration, and many other disorders. “Overall hallucinations are a rare symptom in PSP and CBD. However, if reported, VH [visual hallucinations] predominate. Questionnaire-based studies found hallucinations in 5–13% in PSP and in 5–21% in CBD. This was echoed by two autopsy-based studies with retrospective chart review where the prevalence was 7% for PSP and 0% for CBD.”

The authors argue that because of the need for treatment, “the distinction of disease-inherent hallucinations from medication-associated perceptual disturbances is highly relevant.” This article addressed medication-associated hallucinations along with delirium-associated hallucinations and surgery-associated hallucinations. Both delirium- and surgery-associated hallucinations may be due to medication as well.

I’ve copied the abstract of the article below.

Robin

CNS Neuroscience & Therapeutics. 2011 Feb 16. [Epub ahead of print]

Hallucinations in Neurodegenerative Diseases.

Burghaus L, Eggers C, Timmermann L, Fink GR, Diederich NJ.
Department of Neurology, University of Cologne, Cologne, Germany Cognitive Neurology Section, Institute of Neurosciences and Medicine (INM3), Research Center Jülich, Jülich, Germany Department of Neurology, Centre Hospitalier de Luxembourg, Luxembourg City, Luxembourg.

Abstract
Background: Patients with neurodegenerative disease frequently experience hallucinations and illusionary perceptions. As early symptoms, hallucinations may even have diagnostic relevance (i.e., for the diagnosis of Lewy Body Dementia).

In the later course of the disease, hallucinations may appear as characteristic symptoms and often constitute a particular challenge for therapeutic endeavors.

Here, the distinction of disease-inherent hallucinations from medication-associated perceptual disturbances is particularly relevant.

Results: Synucleinopathies and tauopathies have different risk profiles for hallucinations. In synucleinopathies hallucinations are much more frequent and phenomenology is characterized by visual, short-lived hallucinations, with insight preserved for a long time. A “double hit” theory proposes that dysfunctionality of both associative visual areas and changes of limbic areas or the ventral striatum are required.

In contrast, in tauopathies the hallucinations are more rare and mostly embedded in confusional states with agitation and with poorly defined or rapidly changing paranoia. The occurrence of hallucinations has even been proposed as an exclusion criterion for tauopathies with Parkinsonian features such as progressive supranuclear palsy.

Conclusion: To date, treatment remains largely empirical, except the use of clozapine and cholinesterase inhibitors in synucleinopathies, which is evidence-based. The risk of increased neuroleptic sensitivity further restricts the treatment options in patients with Lewy Body Dementia. Coping Strategies and improvement of visual acuity and sleep quality may be useful therapeutic complements.

© 2011 Blackwell Publishing Ltd.

PubMed ID#: 21592320 (see pubmed.gov for this abstract only)

Quality of Life in PSP – Lower in Women, Depressed, etc/

This is a *very* interesting article on quality of life in PSP and MSA. The research team is one of the top in the world studying PSP. (They are not as well known for their MSA research.)

The researchers use the term health-related quality of life (HrQoL). This term “describes different aspects of self-perceived well-being which are affected by…disease and its treatment.” “HrQoL is an important aspect in the healthcare of chronic diseases such as neurodegenerative disorders.”

Study participants completed a generic HrQoL instrument, the EQ-5D; this instrument has been validated for use in parkinsonian disorders. “The EQ-5D comprises five questions that relate to five dimensions of health: mobility, self-care, usual activities, pain/discomfort and anxiety/depression. Each dimension is divided into three levels of severity (1 = no problem, 2 = moderate problem, 3 = severe problem). … The study participants also rated their current HrQoL on a visual analogue scale (VAS thermometer type) ranging from 0 (worst imaginable health state) to 100 (best imaginable health state).”

One hundred and one German patients were initially recruited. Some patients were excluded. In the end, 86 patients completed the study — 18 MSA-C, 28 MSA-P, and 40 PSP.

The researchers found:

* Factors of reduced HrQoL were “female gender, <12 years of education, disease severity, a decreased number of persons in the household and depression.”

Regarding education, the authors note: “This may reflect insight and expectations of patients, which are known to have influence on quality of life in other chronic diseases.” And, the authors state: “educational programs on APS could contribute to improvement of HrQoL in these diseases.”

Disease severity was measured by the UMSARS IV (Unified MSA Rating Scale, part IV), even in the PSP patients. Depression was measured by the BDI (Beck Depression Inventory).

The authors argue that greater attention needs to be paid to the treatment of depression. Perhaps this conclusion was drawn because only 80% of the depressed patients were receiving adequate antidepressant therapy.

* “With increasing disease severity, the proportion of married patients decreased and the proportion of divorced patients rose.”

* “The dimensions [on the EQ-5D] most affected were ‘selfcare’ and ‘usual activities’. Severe problems in these dimensions were reported by 38.4 and 57.0% of the patients, respectively.”

* “[Pain] was more problematic in MSA than in PSP…”

I’ve copied the abstract below.

Robin

Neurodegenerative Diseases. 2011 May 12. [Epub ahead of print]

Health-Related Quality of Life in Multiple System Atrophy and Progressive Supranuclear Palsy.

Winter Y, Spottke AE, Stamelou M, Cabanel N, Eggert K, Höglinger GU, Sixel-Doering F, Herting B, Klockgether T, Reichmann H, Oertel WH, Dodel R.
Department of Neurology, Philipps University Marburg, Marburg, Germany.

Abstract
Objective: Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), known as atypical parkinsonian syndromes (APS), are neurodegenerative disorders with severe disability and decreased life expectancy.

Little is known about the health-related quality of life (HrQoL) and its determinants in patients with those disorders. The objective of our cross-sectional study was to evaluate the HrQoL in patients with APS and to identify the determinants of HrQoL.

Methods: A total of 101 consecutive patients with MSA (n = 54) and PSP (n = 47) were recruited in four German neurological centers.

Disease severity was assessed using the Hoehn and Yahr stages and the Unified MSA Rating Scale.

The HrQoL was evaluated using the EuroQol instrument (EQ-5D and EQ-VAS). Independent determinants of HrQoL were identified in multiple regression analyses.

Results: The mean EQ-VAS score was 52% lower than that reported for the general population (36.9 ± 18.3 vs. 77.4 ± 19.0).

Of the study participants, 63% reported severe problems in at least one dimension of the EQ-5D.

Cerebellar dysfunction was associated with a more considerable reduction of HrQoL.

Independent determinants of reduced HrQoL were female gender, <12 years of education, disease severity, a decreased number of persons in the household and depression.

Conclusions: The HrQoL in MSA and PSP is considerably reduced. While therapeutic options in the treatment of motor symptoms remain restricted, greater attention should be paid to the treatment of depression, which was identified among independent determinants of HrQoL. Independent determinants of HrQoL should be considered when developing healthcare programs aimed at improving the HrQoL in APS.

Copyright © 2011 S. Karger AG, Basel.

PubMed ID#: 21576919 (see pubmed.gov for this abstract only)

“New insights” into PSP

This recently-published paper is about “new insights” into atypical parkinsonism, including MSA, DLB, PSP, and CBD. The paper is based upon medical journal articles published in 2010. Two of the three authors are well-known Austrian researchers in the atypical parkinsonism community.

The authors describe atypical parkinsonian disorders (APDs) as “characterized by relentlessly progressive and levodopa refractory parkinsonism associated with a range of distinctive atypical features.”

Here are the key points made in the article related to PSP:

* “During the last years, infrequent variants due to PSP tau pathology have been separated from the ‘classical’ syndrome (Richardson syndrome). These variants that challenge the concept of clinicopathologic PSP include PSP-parkinsonism (PSP-P), pure akinesia with gait freezing (PAGF), corticobasal syndrome (CBS), and progressive nonfluent aphasia. … The regional differences in pathological severity almost certainly account for the clinical differences and logically correlate with the different clinical features.”

* “Familial PSP is rare and sometimes related to familial frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17).”

* “Male sex, older age at disease-onset and higher PSP rating scale scores were independent predictors of shorter survival.”

* “A natural history study confirmed previous observations of high levels of cognitive impairment associated with PSP occurring in up to 50% of patients at early disease stages. Furthermore, the cognitive profile characterized by frontal-executive dysfunction was similar to a control cohort of patients with MSA.”

* “The NINDS-SPSP (National Institute of Neurological Disorders­Society for Progressive Supranuclear Palsy) criteria were established 15 years ago focusing on the classic Richardson syndrome presentation.”

Copied below is the abstract.

Robin

Current Opinion in Neurology. 2011 May 13. [Epub ahead of print]

New insights into atypical parkinsonism.

Wenning GK, Krismer F, Poewe W.
Department of Neurology, Medical University, Innsbruck, Austria.

Abstract

PURPOSE OF REVIEW:
Atypical parkinsonian disorders (APDs) comprise a heterogenous group of disorders including multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Based on literature published in 2010, we here review recent advances in the APD field.

RECENT FINDINGS:
Genome-wide association studies have provided robust evidence of increased disease risk conferred by synuclein and tau gene variants in MSA and PSP. Furthermore, advanced imaging tools have been established in the differential diagnosis and as surrogate markers of disease activity in patients with APDs. Finally, although therapeutic options are still disappointing, translational research into disease-modifying strategies has accelerated with the increasing availability of transgenic animal models, particularly for MSA.

SUMMARY:
Remarkable progress has been achieved in the field of APDs, and advances in the genetics, molecular biology and neuroimaging of these disorders will continue to facilitate intensified clinical trial activity.

PubMed ID#: 21577106 (see pubmed.gov for this abstract only)

“New insights” into CBD

This recently-published paper is about “new insights” into atypical parkinsonism, including MSA, DLB, PSP, and CBD. The paper is based upon medical journal articles published in 2010. Two of the three authors are well-known Austrian researchers in the atypical parkinsonism community.

The authors describe atypical parkinsonian disorders (APDs) as “characterized by relentlessly progressive and levodopa refractory parkinsonism associated with a range of distinctive atypical features.”

Here are the key points made in the article related to CBD:

* “The clinical presentation of CBD is variable and includes the CBS, PSP, progressive aphasia, and frontotemporal dementia. However, CBD research is hampered by the rarity of the disorder, patients being scattered over a large geographic area.”

* “A recent clinicopathological study showed that only the minority of postmortem confirmed CBD cases were diagnosed correctly in life (five out of 19 cases) and that the clinical syndrome of CBS is not specific for CBD. Intriguingly, most of the pathologically confirmed CBD cases were characterized clinically by a PSP-like presentation with delayed onset of vertical supranuclear gaze palsy.”

* “Intriguingly, an Alzheimer’s disease-like CSF pattern was associated with early memory decline in patients with CBS according to a recent study.”

Copied below is the abstract.

Robin

Current Opinion in Neurology. 2011 May 13. [Epub ahead of print]

New insights into atypical parkinsonism.

Wenning GK, Krismer F, Poewe W.
Department of Neurology, Medical University, Innsbruck, Austria.

Abstract

PURPOSE OF REVIEW:
Atypical parkinsonian disorders (APDs) comprise a heterogenous group of disorders including multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Based on literature published in 2010, we here review recent advances in the APD field.

RECENT FINDINGS:
Genome-wide association studies have provided robust evidence of increased disease risk conferred by synuclein and tau gene variants in MSA and PSP. Furthermore, advanced imaging tools have been established in the differential diagnosis and as surrogate markers of disease activity in patients with APDs. Finally, although therapeutic options are still disappointing, translational research into disease-modifying strategies has accelerated with the increasing availability of transgenic animal models, particularly for MSA.

SUMMARY:
Remarkable progress has been achieved in the field of APDs, and advances in the genetics, molecular biology and neuroimaging of these disorders will continue to facilitate intensified clinical trial activity.

PubMed ID#: 21577106 (see pubmed.gov for this abstract only)

Study into use of feeding tubes – surveying families

Though the two studies mentioned in this post refer to feeding tubes in those with dementia, many issues raised in this email are relevant to those without dementia as well.

Dr. Joan Teno, a community health physician and researcher at Brown University and Dr. Susan Mitchell, of the Hebrew Senior Life Institute for Aging Research, have researched feeding tube decisions over the last several years. Previously they found that “some American hospitals inserted no feeding tubes ­ zero ­ in patients with advanced dementia over an eight-year period while others [inserted feeding tubes in] one in three.” In their 2010 study, they concluded: “Among nursing home residents with advanced cognitive impairment admitted to acute care hospitals, for-profit ownership, larger hospital size, and greater ICU use was associated with increased rates of feeding tube insertion, even after adjusting for patient-level characteristics.”

Wanting to explore the discrepancy in feeding tube placements, these two researchers and a team surveyed family member, after their relatives’ deaths, on decisions they had made regarding feeding tubes. All of the deceased relatives had dementia. The researchers talked to nearly 500 people from five states — MA and MN, where there are low rates of feeding tube use, and AL, FL, and TX, where there are high rates of feeding tube use.

About 10% of the deceased relatives received a feeding tube. The New York Times reports that:

* “Of patients who received feeding tubes, 13.7 percent of family members reported that doctors had inserted the tubes without seeking permission at all.”

* “But even when medical providers raised the issue and a feeding tube was inserted, 12.6 percent of the family respondents said they had felt pressured by the physician to agree to the procedure, and more than half believed that the physician strongly had favored tube insertion.”

* “Moreover, these talks tended to be cursory: More than 40 percent of respondents said the discussion had lasted less than 15 minutes, and roughly a third said no one had mentioned the risks involved.”

* “In the end, about a third of these families said they thought the feeding tube did improve their loved ones’ quality of life, while only 23.4 percent said they regretted the use of the tube.”

Dr. Teno addresses why there’s a push for feeding tubes: “My guess is, we’ve changed hospital medicine to focus on discharge. How do we move someone quickly out of the system? You put the feeding tube in, you send people back to the nursing home.”

Dr. Paula Span, who wrote the New York Times article on the study, believes that the push for feeding tubes results from doctors not wanting to talk about death with their patients.

Here’s a link to the NYT article (with some interesting reader comments posted):
http://newoldage.blogs.nytimes.com/2011 … -in-haste/

And I’ve copied the abstract of the recent article authored by Teno, Mitchell, et al, below as well as their 2010 article on the differences in hospitals’ rates of feeding tube placement.

Robin

Decision-Making and Outcomes of Feeding Tube Insertion: A Five-State Study

Joan M. Teno MD, MS, Susan L. Mitchell MD, MPH, Sylvia K. Kuo PhD, Pedro L. Gozalo PhD, Ramona L. Rhodes MD, MPH, Julie C. Lima PhD, MPH, Vincent Mor PhD

Journal of the American Geriatrics Society, 59. Article first published online: 3 MAY 2011

OBJECTIVES: To examine family member’s perceptions of decision-making and outcomes of feeding tubes.

DESIGN: Mortality follow-back survey. Sample weights were used to account for oversampling and survey design. A multivariate model examined the association between feeding tube use and overall quality of care rating regarding the last week of life.

SETTING: Nursing homes, hospitals, and assisted living facilities.

PARTICIPANTS: Respondents whose relative had died from dementia in five states with varying feeding tube use.

MEASUREMENTS: Respondents were asked about discussions, decision-making, and outcomes related to their loved ones’ feeding problems.

RESULTS: Of 486 family members surveyed, representing 9,652 relatives dying from dementia, 10.8% reported that the decedent had a feeding tube, 17.6% made a decision not to use a feeding tube, and 71.6% reported that there was no decision about feeding tubes. Of respondents for decedents with a feeding tube, 13.7% stated that there was no discussion about feeding tube insertion, and 41.6% reported a discussion that was shorter than 15 minutes. The risks associated with feeding tube insertion were not discussed in one-third of the cases, 51.8% felt that the healthcare provider was strongly in favor of feeding tube insertion, and 12.6% felt pressured by the physician to insert a feeding tube. The decedent was often physically (25.9%) or pharmacologically restrained (29.2%). Respondents whose loved ones died with a feeding tube were less likely to report excellent end-of-life care (adjusted odds ratio=0.42, 95% confidence interval=0.18–0.97) than those who were not.

CONCLUSION: Based on the perceptions of bereaved family members, important opportunities exist to improve decision-making in feeding tube insertion.

PubMed ID#: 21539524 (see pubmed.gov for this abstract only)

Hospital Characteristics Associated With Feeding Tube Placement in Nursing Home Residents With Advanced Cognitive Impairment

Joan M. Teno, MD, MS; Susan L. Mitchell, MD, MPH; Pedro L. Gozalo, PhD; David Dosa, MD, MPH; Amy Hsu, BA; Orna Intrator, PhD; Vincent Mor, PhD
Author Affiliations: Center for Gerontology and Health Care Research, Warren Alpert School of Medicine, Brown University, Providence, Rhode Island (Drs Teno, Gozalo, Dosa, Intrator, and Mor and Ms Hsu); Institute for Aging Research, Hebrew Senior Life, Harvard Medical School, Boston, Massachusetts (Dr Mitchell); and Providence Veterans Affairs Medical Center, Providence, Rhode Island (Drs Dosa and Intrator).

JAMA (Journal of the American Medical Association). 2010;303(6):544-550.

ABSTRACT
Context
Tube-feeding is of questionable benefit for nursing home residents with advanced dementia. Approximately two-thirds of US nursing home residents who are tube fed had their feeding tube inserted during an acute care hospitalization.

Objective
To identify US hospital characteristics associated with higher rates of feeding tube insertion in nursing home residents with advanced cognitive impairment.

Design, Setting, and Patients
The sample included nursing home residents aged 66 years or older with advanced cognitive impairment admitted to acute care hospitals between 2000 and 2007. Rate of feeding tube placement was based on a 20% sample of all Medicare Claims files and was assessed in hospitals with at least 30 such admissions during the 8-year period. A multivariable model with the unit of the analysis being the hospital admission identified hospital-level factors independently associated with feeding tube insertion rates, including bed size, ownership, urban location, and medical school affiliation. Measures of each hospital’s care practices for all patients with serious chronic illnesses were evaluated, including intensive care unit (ICU) use in the last 6 months of life, the use of hospice services, and the ratio of specialist to primary care physicians. Patient-level characteristics were also considered.

Main Outcome Measure
Endoscopic or surgical insertion of a gastrostomy tube during a hospitalization.

Results
In 2797 acute care hospitals with 280 869 admissions among 163 022 nursing home residents with advanced cognitive impairment, the rate of feeding tube insertion varied from 0 to 38.9 per 100 hospitalizations (mean [SD], 6.5 [5.3]; median [interquartile range], 5.3 [2.6-9.3]). The mean rate of feeding tube insertions per 100 admissions was 7.9 in 2000, decreasing to 6.2 in 2007. Higher insertion rates were associated with the following hospital features: for-profit ownership vs government owned (8.5 vs 5.5 insertions per 100 hospitalizations; adjusted odds ratio [AOR], 1.33; 95% confidence interval [CI], 1.21-1.46), larger size (>310 beds vs <101 beds: 8.0 vs 4.3 insertions per 100 hospitalizations; AOR, 1.48; 95% CI, 1.35-1.63), and greater ICU use in the last 6 months of life (highest vs lowest decile: 10.1 vs 2.9 insertions per 100 hospitalizations; AOR, 2.60; 95% CI, 2.20-3.06). These differences persisted after controlling for patient characteristics. Specialist to primary care ratio and hospice use were weakly or not associated with feeding tube placement.

Conclusion
Among nursing home residents with advanced cognitive impairment admitted to acute care hospitals, for-profit ownership, larger hospital size, and greater ICU use was associated with increased rates of feeding tube insertion, even after adjusting for patient-level characteristics.

PubMed ID#: 20145231 (see pubmed.gov for this abstract as well as a link to the full article at no charge)

PSP-related blog post by Florida woman

Several years ago, I met June Keith through the PSP Forum (forum.psp.org).  June lives in Key West, Florida.  I helped her make arrangements to donate her mother’s brain upon death.  June’s mother Shirley died last year.  PSP was confirmed upon brain autopsy.  Surprisingly, though Shirley was 77 years old, she had no other brain pathologies besides PSP.

This is the first Mother’s Day June is experiencing without her mother.  On her “Postcards from Paradise” blog, June posted today about her mother and PSP.  I’ve copied the full blog post below as well as a link to the blog.  There are several old photos posted to the blog online.

Robin

——————————-

juneinparadise.blogspot.com/2011/05/rest-in-peace-mom.html

Postcards from Paradise
a blog by June Keith

Thursday, May 5, 2011

Rest In Peace, Mom

On this Mother’s Day, my first one without my mother, I want to sound an alert in her honor. I want to talk about Progressive Supranuclear Palsy (PSP), the mysterious and cruel illness that tortured and ultimately killed her. PSP is a tragic disease, not only because of its torturous course. It’s also difficult to recognize. Sadly, researchers seem no closer to understanding PSP today than they were when Mom was finally diagnosed in 2007. Its cause remains a mystery.

PSP is a disease of the brain, attacking six people in 100,000. PSP slowly kills off parts of the brain that relate to vision, swallowing, balance and speech. Because it occurs so infrequently, many physicians simply know nothing about it. That was Mom’s case. At first her vision went bad. She lost her ability to do crossword puzzles due to what she described as fuzzy eyesight. She lost her balance and tumbled frequently, seemingly without reason. She could not eat a meal without dropping half of it on the shelf formed by her amble bosom. Her weight skyrocketed as she became increasingly immobile. She also became incontinent, a fact I was to sorrowfully learn on a car trip from Florida to Nova Scotia. It was in Georgia that I pulled off the Interstate to find a drug store and buy her first package of adult diapers. She wore them for the rest of her life.

Yet not one of the many doctors who examined her could find a problem with her health. Yes, she had lost her ability to walk without the aid of a walker. And yes, her vision was strangely off. But she passed every colonoscopy and endoscopy and vision test and cognition test and blood test with flying colors.  She went for nights without sleeping. Dozens of medications were prescribed to help her to sleep. None worked. She became a habitual television watcher, a past-time that she’d long considered to be a big waste of time. She dressed in house dresses and stayed indoors to avoid the embarrassment of falling in public.

As her health continued to fail, with no reason that any medical test or procedure could find, she considered that she was simply wearing out, that she’d burned the candle at both ends for many long years and this was the result of all of that. She’d been a nurse and worked the night shift for 35 years. She worked while her husband and three kids slept because she liked to party by day. She skied in the winter. She beached in the summer.

As her habits began obviously to change, we scolded her for eating too fast, for not taking care of herself, for not exercising. Walk, Mom! Get out and do things, we urged. She said she couldn’t. We thought she was lazy, or depressed. We agonized when the phone rang, expecting the bad news from Sebring, Fla. of another mishap involving Mom, who was supposedly quite healthy.

Claiming that her lovely home had become too big for her to handle, she sold it and bought a tiny trailer in a seniors-only trailer park. She had the floors redone. The walls painted. She bought new kitchen appliances. But it was still a trailer, and we were mystified as to why she chose such a modest dwelling when she could afford so much more.  Then I understood. I observed that when she walked from the living room to her bedroom she used the walls to keep her balance. Everywhere in that trailer she had a place to hold on to, to keep her on her feet.

What was wrong with her? I took her to eye doctors, five total, and not one could find a problem with her eyes. By then she was incapable of reading or writing. In the hospital, after a fall, a nurse asked me “is your mother blind?” We were advised to place her in a nursing home, but she countered that she was healthy. Every test said so. Why would she give up her independence when there was nothing wrong with her? If she fell and broke her neck, so be it.

We hired people to come into her home and clean, cook and take care of her dog. Her condition continued to baffle us, and her. Four uneasy years passed.

One day my husband Michael said “I think I know what’s wrong with your mother.”  He’d read an obituary of a woman who’d died after a courageous struggle with Progressive Supranuclear Palsy. He’d researched the subject and was soon certain that Mom was a victim of PSP.  He was right. She was.

The description of PSP matched mom’s symptoms exactly. We got Mom to a neurologist who confirmed our suspicions. (The first neurologist she saw had insisted she had Alzheimer’s Disease, a diagnosis that in no way matched her symptoms.) The prognosis was ugly and sad. Mom’s main risk factors were falling and choking. PSP patients often died of pneumonia from inhaling food, he said. He also told us that there was nothing to be done. She would die. Slowly.

That was in June, 2007. She died in a nursing home in August, 2010, completely blind, wheelchair bound, and eating a diet of pureed food—the most horrible insult of all, she said. She broke a hip. Then she got pneumonia. She survived all of that and four months later broke her other hip. She died in her sleep, days later, hours after being released from the hospital.

She had a nice nest egg by the time she retired at age 62. She’d carefully saved her money, and even inherited a bit from her own mother, but she died nearly penniless, having spent a fortune on her care during the last five years of her life. Running out of money was a constant fear. And the fear of dying by choking to death was always with her, too, she told me.

I’m telling this story to inform as many people as we possibly can of the disease of PSP. We were surprised and heartbroken at how many medial professionals failed to recognize Mom’s illness. Ultimately it was my husband who diagnosed her, and he is a songwriter.

We donated our mom’s brain to Florida Brain Bank and learned from her autopsy that her disease was definitely PSP with no other disease process. A neurologist in Key West told me that Mom’s was only the fourth case of PSP in his career. He said PSP had probably shortened her life by ten years. She was around 70 when she began her decline. She was 77 when she died.

Others who have died of PSP are Dudley Moore, the actor, and Teresa Brewer, a singer, who died at the same age as Mom. Doubtless there are hundreds of people suffering right now with this strange and hellish illness that goes unrecognized more often than not. Once we knew what Mom was suffering from we hooked up with the PSP forum, where the various features of PSP are discussed daily. It gave us immeasurable relief.

I’m telling this story with the hope that it will help someone, somehow, or some way, to recognize or to manage this particularly horrendous illness that my amazing mother handled with remarkable humor, grace and courage.

Happy Mother’s Day, Mom. Rest in well-deserved peace.

List of PSP-like syndromes

In a case report* of a 71-year-old Spanish woman with rheumatoid arthritis who developed many PSP symptoms (including vertical gaze palsy), there’s a long list of PSP-like syndromes:

Parkinson’s Disease

Multiple system atrophy

Whipple disease
Creutzfeldt-Jakob disease
Caribbean parkinsonism
Niemann-Pick disease type C
Frontotemporal dementia associated with chromosome 17

Cerebral hemosiderosis

Flunarizine and amiodarone

Corticobasal Degeneration

Cerebral amyloid angiopathy and motor neurone disease
Dementia with Lewy bodies

Prion disease
Lytico-Bodig disease
Cerebral autosomal dominal arteriopathy with subcortical infarcts and leukoencephalopathy
Bilateral hypoxic-eschemic striopallidal lesions
Neurosarcoidosis

Pallidonigroluysian Atrophy

Obstructive hydrocephalus

Clebopride (antidopaminergic drug)
Occupational exposure to lead batteries
Cerebrovascular disease
Neurosyphilis
Intracranial dural arteriovenous fistula

Richardson syndrome

* Neurologist. 2011 May;17(3):136-40.
Rheumatoid meningitis mimicking progressive supranuclear palsy.
Aguilar-Amat MJ, Abenza-Abildúa MJ, Vivancos F, Rodríguez de Rivera FJ, Morales-Bastos C, Gandía-Gonzalez ML, Pérez-Zamarrón A, Arpa J.
Departments of *Neurology †Neuropathology ‡Neurosurgery, La Paz University Hospital, Madrid, Spain.
PMID: 21532380