Five Approximate Phases of Lewy Body Dementia (as seen by caregivers)

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This post is about five phases of Lewy Body Dementia, according to a group of caregiver spouses.

Some members of the LBD_caringspouses Yahoo!Groups list have been working on the “LBD Approximate Phases” for a year now.  I just received from Sue Lewis this final version of the Phases.  This caregivers’ view of the five phases of LBD is a worthwhile document, especially for those new to the world of LBD.  They’ve been working on this list on and off for so long that, at this point, they are not inviting your suggestions on how to modify the document.  But they welcome any general comments (primarily positive ones, I think)!  You can send comments to me and I’ll pass them on (unless you know Sue Lewis’ contact info from the LBD_caringspouses list).

Robin

 

LBD APPROXIMATE PHASES AS SEEN BY CARING SPOUSES
August 2007

Facilitated by Sue Lewis of West Virginia, edited by June Christensen, Kansas

GLOSSARY OF ACRONYMS

ADL: Activities of Daily Living – dressing/bathing/ feeding oneself
BP: Blood Pressure
CG: Caregiver
DME: Durable Medical Equipment–wheel chair, shower chair
DPOA: Durable Power of Attorney
LBD: Lewy Body Dementia
LO: Loved One
LW: Living Will
MPOA: Medical Power of Attorney
PCP: Primary Care Physician
POA: Power of Attorney
REM: Rapid Eye Movement sleep disorder
URI: Upper Respiratory Infection
UTI: Urinary Tract Infection
WCh: Wheelchair

PHASE I POSSIBILITIES

Most caregivers are concerned/worried that something is not right. Please note that symptoms from later stages can appear at this early phase. At the end of this phase, dementia is becoming difficult to deny

  • Possible REM sleep disorder
  • Restless Leg Syndrome
  • Hallucinations
  • Possible Parkinson’s disease diagnosis
  • Myoclonus (involuntary jerking) 
  • Increased daytime sleep – two+ hours
  • Loss of sense of smell (Anosmia)
  • Vision problems
  • Hearing loss
  • Speech problems ·
  • Impaired physical coordination (ataxia)
  • Shuffling gait
  • Slowness of movement
  • Altered posture (called Lewy Lean) 
  • Chronic runny nose
  • Impaired comprehension and cognition
  • Inability to learn new tasks
  • Loss of initiative and interests
  • Diminished alertness
  • Short-term memory loss but able to hide (mask) symptoms or engage in show time ·
  • Mood: Fluctuations
  • Depressed/anxious
  • Paranoia
  • May accuse spouse of infidelity, aggression
  • Able to engage independently in leisure activities
  • Handwriting is affected
  • Impaired ability to handle financial responsibilities · Still may be able to work but driving skills often compromised

 

PHASE II POSSIBILITIES

Most caregivers are worried that something is wrong and seek medical attention. May be given an incorrect diagnosis (Alzheimer’s, Multisystem atrophy, Multi-Infarct Dementia, Depression, Parkinson’s). Please note that symptoms from later or earlier stages can appear at this phase. It is strongly suggested that caregivers consult with an elder law attorney at this phase. At least, have a Power of Attorney and Medical Power of Attorney document on the patient. Family, friends, caregivers may successfully take financial advantage of LO. Caregivers need to familiarize themselves with all finances and assets to possibly consult with a financial advisor.

  • Ambulates/transfers without assistance but increased risk for falls/requires walker
  • Leaning to one side (Lewy Lean)
  • Possible fainting
  • Able to perform most ADLs without assistance
  • Some autonomic dysfunction (changes in BP, sweating, fainting, dry mouth)
  • Occasional episodes of incontinence (one or two a month)
  • Constipation
  • Parkinson’s symptoms may be controlled with medication
  • Increased difficulty in:
    • Finding words (aphasia)
    • Organizing thoughts
    • Reading & comprehension
    • Following TV programs
    • Operating home appliances
  • May be able to administer own medications
  • Able to follow content of most conversations
  • Able to be left unsupervised for two or more hours
  • Delusions
  • Capgras Syndrome (seeing or thinking that a person or objects have been replaced by another identical one)
  • May be more depressed
  • More paranoid and more agitated

 

PHASE III POSSIBILITIES

Most caregivers have the correct diagnosis. Caregiver and patient actively grieve. Caregivers need regular planned respite. Caregiver needs require regular preventive health care. Caregivers may need home health aide assistance to maintain LO in the home. Patient is at risk for long-term care due to: psychological symptoms, personal safety risk, and caregiver safety risk. The needs of the patient affect personal finances. Please note that symptoms from later or earlier stages can appear at this phase.

  • Ambulation/transfers are impaired
  • Needs assistance with some portion of movement
  • At risk for falls
  • Increase of Parkinsonism symptoms
  • Increase of autonomic dysfunctions
  • Frequent episodes of incontinence (two+ per week)
  • Needs assistance/supervision with most ADLs
  • May require DME
  • Speech becomes impaired
  • Projection (volume) may decrease
  • Able to follow content of most simple/brief conversations or simple commands
  • Increased difficulty with expressive language
  • Able to be left unsupervised less than one hour but unable to work or drive
  • Unable to administer medication without supervision
  • Unable to organize or participate in leisure activities
  • Inability to tell time or comprehend time passing
  • Mood fluctuations (depressed, paranoid, anxious, angry) requiring medical monitoring
  • Increased confusion
  • Delusions
  • Increased Capgras Syndrome · Severity of symptoms may increase or decrease

 

PHASE IV POSSIBILITIES

Caregiver at high risk for chronic health/joint problems. Self-care is paramount to providing patient care. The needs of the patient require the assistance of a home-health aide/private-duty aide two-to-seven days per week. Increased patient needs may require potential for long-term care placement. Patient may be declared mentally incapacitated. Caregiver may need to explore Hospice services.

  • Needs continuous assistance with ambulation/transfers
  • High risk for falls
  • May need electronic lift recliner chair
  • Needs assistance with all ADLs
  • Requires 24-hour supervision
  • Autonomic dysfunctions need regular medical monitoring
  • Incontinent of bladder and bowel
  • Unable to follow content of most simple/brief conversations or commands
  • Speech limited to simple sentences or one-to-three-word responses
  • Parkinson’s symptoms need regular medical monitoring
  • Choking, difficulty swallowing (dysphasia), aspiration, excessive drooling
  • Increased daytime sleeping
  • Hallucinations prevalent but less troublesome

 

 PHASE V POSSIBILITIES

Caregiver is actively grieving. Potential for increased caregiver stress. Hospice assistance is strongly suggested. Caregiver will need hands-on support from others to maintain LO at home. Caregiver may need to honor decisions made earlier on the Living Will.

  • Dependent for all ADLs
  • Patient may require hospital bed
  • Hoyer lift or Mo-lift, suction machine, etc.
  • Assistance with repositioning
  • High risk for URI, pneumonia, and UTI
  • Skin breakdown
  • May have a fever
  • Difficulty swallowing with possible decision for feeding tube necessary
  • Muscle contractions – hands, legs, arms; lean to either side very pronounced
  • May need nutritional supplements
  • Ensure/Boost/Carnation Instant Breakfast
  • Unable to follow simple commands
  • Decreased or no language skills
  • Constant delusions
  • Fluctuations less frequent and more severe

Palliative Care and PD (presentation by Susan Heath, RN)

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These are the notes I took from Susan Heath’s presentation and Q&A session at the June 29, 2007 Caregivers Appreciation Luncheon that five members from our Atypical Parkinsonism support group attended.

Susan Heath is an RN with the SF VA and works with their Parkinson’s Disease Research, Education and Clinical Center.  She is the co-organizer of the PD support group meeting held at the SF VA.

Her presentation was on the topic of Palliative Care and PD, but I found it much wider than that in terms of scope.  The short Q&A had nothing to do with palliative care.

You can find a copy of her presentation materials at:

https://web.archive.org/web/20110915013038/http://www.ppsg.org/PPSG_2/Docs/Susan%20Heath%20Presentation_June2007.pdf

Here are my notes on her presentation on palliative care and Parkinson’s Disease (PD).  (I’m not going to repeat the info that is on the slides.)

Robin

————–

Robin’s Notes from

Palliative Care and PD
by Susan Heath, RN, SF VA
June 29, 2007

“Palliative care” is about the relief of suffering.  It includes “hospice” but it also includes “modern medicine.”  It is not synonymous with “hospice.”

“A good death is an accomplishment.”

“People don’t die ‘of’ PD, they die ‘with’ PD.”

There is ONE paper by a German pathologist named Braak who suggests that PD is infectious.  (She emphasized the word “one.”)  Braak believes that PD gets into the gut and works its way up to the brain.  Polio is like this.

There are subtypes of PD.  Two examples are the akinetic-rigid type and the tremor dominant type.

People with PD have a sensory integration problem.  One example of this is that those with PD are not aware they are speaking softly (hypophonia).  A second example is that those with PD don’t know how to pack a car well any more to take a trip.

“Young onset PD has a genetic component.”

A study was done by the Univ of IA on driving.  It was determined that 70% of those with an H&Y score between 2.5 and 3.0 are bad drivers.

After an H&Y score of 3.0, it is safer for PD patients NOT to walk.

“PD patients can’t multi-task, especially in advanced stages.”

“How do you know if your loved one has swallowing problems?  If they are choking or aspirating with meals.”  (Robin’s note:  I don’t like this answer because, with silent aspiration, there is no sound!  If you hear no sound but you suspect there might be aspiration, the only way to know is to have a modified barium swallow study done.)

The time to discuss advanced care planning is when you don’t need it, not when you need it.

A permanent NG tube is uncomfortable.

Based on her years of experience as an ICU RN, she says that the “guilty, non-involved family member” wants the most aggressive treatment.  Involved family members don’t want to prolong a loved one’s life.

Here are my notes on the Q&A session:

Mirapex is conducting a study called “Dominion,” which is looking into what relationship there is between compulsive behaviors and dopamine agonists, such as Mirapex and Requip.

In Europe (France?), there’s an injectable form of levodopa (Sinemet) available.

The SF VA will be getting a sleep center soon.

She mentioned a caregiver stress survey.

Visual Disturbances in PD

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The Colorado Neurological Institute produces a publication called the CNI Review. The Fall ’05 issue contains a couple of articles that our group might find interesting. One is “Visual Disturbances in Parkinson’s Disease and Intervention,” written by an optometrist. All of the atypical Parkinsonism disorders in our group can experience these visual problems. I read about this today on a multiple system atrophy-related online support group.

Here’s what someone on that MSA-related online support group had to say about this article on visual disturbances:

“The important thing I got from the article was that double vision is
intolerable and it gives 3 options for treatment. To me the word
INTOLERABLE is what is important. We didn’t take my Dad’s complaints
seriously enough when he complained about the double vision. We just
thought it was one of those MSA symptoms that he would have to live
with. Finally, my Dad told the ophthalmologist himself and he was given
the prescription for the Fresnel Prism Overlay. Works well for him.”

You can find the full publication (5MB+) online: (takes about a minute to download, even with high-speed internet access)

https://web.archive.org/web/20060213033243/http://thecni.org/reviews/17-fall05-all.pdf

Visual Disturbances in Parkinson’s Disease and Intervention
by Thomas Politzer, O.D, FCOVD, FAAO
CNI Review
Fall 2005

The visual disturbances article starts on page 10 of the CNI Review (which is page 12 of the PDF). Ironically, in the section on reading difficulty and skipping lines, there seems to be a problem in the article. I think some text is missing.

Robin

“Diagnostic criteria of dementia” (Canadian journal article)

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I’ve been looking lately into the definition of dementia. When caregivers of those with progressive supranuclear palsy (PSP) call me for the first time, I often ask “does your loved one have dementia,” knowing that at least half of those with PSP have dementia as a primary symptom. Often the caregivers will say “no,” and then go on to tell me how their loved one can no longer balance a checkbook, make investment decisions, or make any sort of decisions. Perhaps these caregivers are embarrassed to say that their loved ones are demented. Or perhaps the only kind of dementia they are aware of is Alzheimer’s Disease, and they know their loved ones don’t have that. Or perhaps we are using different definitions or criteria.

The only standard definition of dementia I’m aware of is the DSM IV criteria. (DSM = Diagnostic and Statistical Manual for Mental Disorders, Fourth Edition, published by the American Psychiatric Association, Washington, DC.) According to a University of Alberta website:

“Dementia is a clinical state characterized by loss of function in multiple cognitive domains. The most commonly used criteria for diagnoses of dementia is the DSM-IV. Diagnostic features include: memory impairment and at least one of the following: aphasia, apraxia, agnosia, disturbances in executive functioning. In addition, the cognitive impairments must be severe enough to cause impairment in social and occupational functioning. Importantly, the decline must represent a decline from a previously higher level of functioning. Finally, the diagnosis of dementia should NOT be made if the cognitive deficits occur exclusively during the course of a delirium.”

(Wikipedia definitions: aphasia = loss of the ability to produce and/or comprehend language; apraxia = loss of the ability to execute or carry out learned purposeful movements, despite having the desire and the physical ability to perform the movements; agnosia = loss of knowledge or loss of the ability to recognize objects, persons, sounds, shapes, or smells)

The problem is that there are many different types of dementia (70 or 80 types) and their characterizations are all so different. And, as the abstract below indicates, not all types of dementia have memory impairment.

Robin

——————

The Canadian Journal of Neurological Sciences. 2007 Mar;34 Suppl 1:S11-8.

Bouchard RW.
Clinique de mémoire et unité de recherche Alzheimer, CHA Hôpital de I’Enfant-Jésus, Québec, QC, Canada.

In the past two decades there has been a tremendous effort among clinicians and searchers to improve the diagnostic criteria of the dementias on the basis of the differential neurological and neuropsychological profiles. This was an obligatory requirement for clinical trials and the development of treatments. Over the years it became rapidly evident that the cohorts of patients in studies had some degree of heterogeneity, making it difficult to interpret the results of some studies, particularly in the vascular dementias and the mild cognitive impairment (MCI) group. For example, many sub-types of the vascular group were included in clinical trials, such as the cortical strokes, the lacunar states and the diffuse white matter disease cases, and some of the patients might have had also mixed pathology. In addition, the standard DSM IV criteria for dementia no longer represent our present knowledge of the clinical profile of some of the dementias such as vascular dementia (VaD) and fronto-temporal dementia where the memory impairment is not necessarily the first requirement. To improve the validity of clinical trials and eventually help developing more appropriate treatments, we revised the present diagnostic criteria and made recommendations for some changes in the context of the 2nd Canadian Conference on the Development of Antidementia Therapies, held in 2004 and reviewed in the light of the recent literature as of early 2006. It is expected that in the near future, these dementia criteria for clinical trials will have to be revised again in order to include specific subtypes of the dementias as well as biomarkers, structural and functional imaging.

PubMed ID#: 17469675 (see pubmed.gov for the abstract only)

Riluzole study – MSA+PSP

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This news article was sent to me by a local support group member whose mother with MSA died a few months ago. She regularly reads Medscape and saw a news article on the results of a study of the drug riluzole in MSA and PSP. Rather than being disappointed by the results (that showed that riluzole had no effect on survival), she finds it cheering to know that researchers are actually studying these diseases. Also, she says: “The interesting point is the study design, which included a lot of people fairly early in their diseases and may be a good model for future drug studies.”

The study was done through the European group NNIPPS (Neuroprotection and Natural History in Parkinson Plus Syndromes). The lead author was P. Nigel Leigh, MD, PhD, from King’s College, London. I don’t know of an equivalent group in the US that is looking into all the atypical parkinsonism disorders.

Here’s a link to the news article, and a copy of the full text. To gain access to anything on Medscape, you must log in, which requires (free) registration.

http://www.medscape.com/viewarticle/557908

NNIPPS: No Benefit of Riluzole on Survival in MSA and PSP

Susan Jeffrey
Medscape Medical News 2007.

June 7, 2007 (Istanbul) — A randomized trial of the neuroprotectant drug riluzole (Rilutek, Sanofi-Aventis) in patients with the “Parkinson plus syndromes” of multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) shows no effect on survival, either in the population as a whole or within the PSP or MSA strata, with treatment vs placebo.

However, the study supports the idea that large trials in neurodegenerative disorders can use survival as an end point and that it is feasible to include patients with these conditions quite early in their disease using the Parkinson-plus-syndrome concept.

The concept, for which this group has developed and validated diagnostic scales based on these data, “may be worthwhile when we have drugs that are promising,” lead author P. Nigel Leigh, MD, PhD, from King’s College, London, United Kingdom, who presented the results on behalf of the Neuroprotection and Natural History in Parkinson Plus Syndromes (NNIPPS) Consortium, told attendees here.

“It has simple, pragmatic diagnostic criteria, which I suggest have some merit,” he added, as well as high sensitivity and specificity for these difficult-to-diagnose conditions. The results were presented here at the 11th International Congress of Parkinson’s Disease and Movement Disorders.

No Survival Benefit

PSP and MSA often present as Parkinson plus syndromes — that is, having some of the classical features of Parkinson’s disease but with some additional features. Early in the disease course, it can be very difficult to distinguish these conditions from each other, but for any intervention to be useful, it is important to enroll patients before diagnosis of PSP vs MSA is definitive.

PSP and MSA each have roughly the same frequency as amyotrophic lateral sclerosis (ALS), Dr. Leigh told Medscape, and “so are not insignificant adversaries.” The idea of NNIPPS was to design a trial that would give a definitive answer, he said. “The whole field is deviled by small trials that never, ever, could have gotten a result. If we’re going to choose a drug that’s not ideal, like riluzole — no one was really expecting it to be a cure — let’s at least be 80% or 90% sure that we’re going to know in the end that it doesn’t work or it does work.”

They decided to use survival as an end point for this trial. “Everybody said, you can’t do it, it takes too long, but I think we’ve shown you can do it. It’s tough, and it takes a long time, but maybe that will be worth it when we do have really good drugs,” he noted.

Still, they had started out with the hope that riluzole might be effective. The natural history of MSA and PSP is poorly understood, they note in their abstract, but excitotoxicity may contribute to the neuronal damage. Riluzole, already approved for use in ALS, is a glutamate-release inhibitor and has been shown to prolong survival by about 2 months in clinical ALS trials and possibly between 6 and 19 months in retrospective data.

The NNIPPS trial, then, was a European multicenter, randomized, and stratified trial of riluzole in a dose of 50 to 200 mg per day vs placebo in patients with PSP and MSA. At the same time, investigators meant to investigate the natural history of these conditions presenting as Parkinson plus syndromes, acquiring prospective data on diagnostic criteria, MRI changes, and pathology.

The primary outcome was survival at 36 months; secondary outcome measures included functional status, cognition, quality of life, healthcare costs, and MRI abnormalities.

Power calculations were based on published estimates of survival in these conditions. In total, they enrolled 766 subjects from participating centers in the United Kingdom, France, and Germany. Six were later excluded because they either were found not to have proper informed consent or did not actually receive the drug, leaving 362 patients diagnosed clinically as PSP and 398 as MSA.

They used their own NNIPPS diagnostic criteria rather than the retrospectively validated existing criteria, he pointed out, because “we decided they were operationally extremely difficult in the setting of a clinical trial; they’re too complicated, too complex,” he said. In addition, “they’re very good on specificity but they’re weak on sensitivity, and we wanted sensitivity as well.”

Median survival from onset was 7.8 years for PSP and 8.7 for MSA, with 171 deaths occurring in the PSP group and 171 in the MSA group.

Blinded Data

In his presentation of the primary results here, Dr. Leigh noted that they have not yet unblinded the riluzole/placebo comparison because they hope still to do the other secondary analyses in a blinded fashion. Instead, he simply presented the survival results as “treatment A” vs “treatment B.” Looking at the survival curves, however, he pointed out, “It doesn’t take much to show you that there was no significant difference, with the log rank clearly not significant. There was no difference, despite having adequate power.”

Analysis of the intent-to-treat population showed no evidence of a significant treatment effect of riluzole either in the population as a whole or in the PSP or MSA strata.

“So the very least we can say is that riluzole does not work in this condition, but maybe this is a model for looking at other drugs, which we hope indeed it will be,” he said.

Pathological diagnosis was confirmed in 112 cases where brain tissue has been donated and analyzed; another 20 brains have been donated but not yet analyzed, he noted. Overall, pathology-confirmed diagnosis showed that clinical diagnosis using the NNIPPS diagnostic criteria had an overall positive predictive value of 90% for PSP and 93% for MSA, “so we were encouraged by that,” Dr. Leigh noted.

In an interview, Dr. Leigh pointed out that an earlier session here on the genetics of movement disorders underlines the “scary” genetic diversity of these diseases, making finding specific treatments for each subgroup with a different molecular mechanism a daunting task. “If you’re going to find disease-modifying therapy in neurodegenerative diseases, the real challenge is to try to break across the boundaries of individual diseases and find common mechanisms.”

They were disappointed with these results not only because they had hoped to help patients, but because if it had been positive that would have pointed to a common mechanism and provided some insight into how riluzole may be working in ALS as well, since this has not been well described.

“Still, I think, after recovering from the bad weekend when we got the news, that there’s still a huge resource there,” Dr. Leigh said.

Rich Resource

Despite the fact that the trial was ultimately negative, NNIPPS provides a rich data set for understanding motor, cognitive, neuroimaging, and genetic factors in MSA and PSP.

For example, they devised and validated 2 diagnostic scales, the Parkinson Plus Scale and the Short Motor Scale, specifically designed for early use before a diagnosis of PSP or MSA is certain. They also have quality-of-life data and health costs that can be used in healthcare planning, he noted.

In addition, they have 633 baseline MRIs in these patients, with follow-up scans done at the end of the study in 187 patients; 521 DNA samples; and brains now from 131 patients, prospectively collected and being analyzed. Dr. Leigh invited any researchers interested in accessing these data to contact him about it.

The study was supported by the European Community. Study drug was provided by Sanofi-Aventis. Dr. Leigh disclosed has worked as a consultant for ONOPharma, Teva, Trophos, and GlaxoSmithKline.

11th International Congress of Parkinson’s Disease and Movement Disorders: Oral Platform Presentations 4602: Abstract 10. June 3-7, 2007.