Category Archives: LBD

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

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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)

Distinguishing Alzheimer’s from Dementia with Lewy Bodies

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Here’s an easy-to-read newspaper article from Science Daily (sciencedaily.com) on using “changes in alertness and cognition” to help distinguish Alzheimer’s Disease (AD) from Dementia with Lewy Bodies (DLB).  A local support gorup member suggested this is a good article to give family and friends about Lewy Body Dementia.

Robin
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www.sciencedaily.com/releases/2004/01/040116080751.htm

New Method Of Distinguishing Alzheimer’s From Lewy Body Dementia
Source: American Medical Association
Science Daily
Published 1/16/04

NEW YORK — Looking at specific changes in alertness and cognition may provide a reliable method for distinguishing Alzheimer’s disease (AD) from dementia with Lewy bodies (DLB) and normal aging, according a new study from the January 27, 2004, issue of Neurology, the official journal of the American Academy of Neurology, co-authored by Tanis J. Ferman, Ph.D., an expert on DLB.

Lewy bodies are round collections of proteins in the brain that are considered the pathological hallmark of Parkinson’s disease. Lewy bodies are never found in healthy normal brains. In Parkinson’s disease the Lewy bodies are largely localized to an area of the brain stem called the substantia nigra. In DLB, Lewy bodies are also found in brain’s cortex.

Although DLB accounts for as much as 20 to 35 percent of the dementia seen in the United States, treatment and diagnosis is often complicated by a lack of information about the disease. In the study, Dr. Ferman and colleagues examined episodes of fluctuation in cognition (problems in thinking or concentration) experienced by individuals with AD or DLB or normal older adults who had no signs of dementia.

“Fluctuating cognition is an important symptom of DLB but has been the center of some controversy because it is comprised of a number of behaviors, some common to all dementias and perhaps even found in normal aging,” said Dr. Ferman, assistant professor and clinical neuropsychologist in the department of psychiatry and psychology at the Mayo Clinic in Jacksonville, Fla. “Even though attempts have been made to carefully describe these behaviors, they have not been used reliably as diagnostic tools.” Dr. Ferman spoke today at an American Medical Association media briefing on Alzheimer’s disease in New York City.

Some of the common behaviors of DLB that comprise fluctuating cognition include episodes of confusion, excessive sleepiness, a waxing and waning of cognition, inattention, incoherent speech and varying ability to perform tasks. When this occurs, family members often describe their loved ones as “zoned out,” or “not with us.” This collection of behaviors is called fluctuations because these behaviors come and go. In the study, 200 normal older adults, 70 patients with AD and 70 patients with diagnosed DLB were compared on aspects of fluctuating cognition. Spouses, adult children or others involved with the subject on a day-to-day basis provided information.

Four characteristics significantly distinguished patients with DLB from persons with AD and normal elderly controls: daytime drowsiness and lethargy despite getting enough sleep the night before; falling asleep two or more hours during the day; staring into space for long periods and episodes of disorganized speech.

“For the normal elderly control group, one or two of these behaviors was found in only 11 percent of the group,” said Dr. Ferman. “For the patients with AD, one or two of these behaviors were not uncommon, but over 63% of the patients with DLB had three or four of these behaviors. This gives us a clear set of behaviors to use to reliably distinguish the fluctuations of Lewy body dementia from Alzheimer’s.”

“Medications that may be helpful to an Alzheimer’s patient may actually aggravate DLB symptoms such as hallucinations and symptoms of parkinsonism. Other medications that are only marginally helpful in AD sometimes have a dramatic impact on Lewy body dementia,” said Dr. Ferman. “It’s very important to diagnose correctly because proper treatment can help us manage symptoms and help caregivers cope.”

Both AD and DLB are dementias, that is, classified by a decline in thinking skills greater than expected by age that interferes with the activities of daily living, explained Dr. Ferman. In AD the first loss in thinking skills is in memory; in DLB the earliest loss appears to be with attention and visual perception. These differences may be related to different patterns of damage to the brain. In addition, patients with DLB may have fully formed hallucinations, Parkinson-like movement problems and/or fluctuating cognition. These symptoms may be present in late-stage AD, but one or all of them are present in early DLB.

“As our understanding and ability to recognize Lewy body dementia has improved, there has been an explosion of research,” said Dr. Ferman. “As we develop effective treatments to prevent or delay progression of DLB, early diagnosis will be key.”

Note: This story has been adapted from a news release issued by American Medical Association.

Gastrointestinal and Urinary Dysfunction

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This post is of interest to those dealing with gastrointestinal problems, urinary dysfunction, problems with saliva, dysphagia, etc.

This article on gastrointestinal and urinary dysfunction in PD was published today in PDF News.  PDF = Parkinson’s Disease Foundation.  The author is Dr. Ron Pfeiffer, the same neurologist who spoke on non-motor symptoms at last year’s PD Symposium in the Bay Area.

Here’s a  link to the article online:

www.pdf.org/en/spring07_gastrointestinal_and_urinary_dysfunction_in_pd

Gastrointestinal and Urinary Dysfunction in PD
By Dr. Ronald Pfeiffer
PDF News, Spring 2007

I’ve copied a few excerpts below on dysphagia and stomach problems.

Robin

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Excerpts from:

Gastrointestinal and Urinary Dysfunction in PD
By Dr. Ronald Pfeiffer
PDF News, Spring 2007

Dysphagia
Difficulty swallowing, or dysphagia, is a very common problem in Parkinson’s.  At least 50 percent (some studies even suggest over 80 percent) of people with PD experience difficulty in swallowing, and an even greater percentage show abnormalities on x-ray tests of swallowing.

Difficulty swallowing is usually due to the lack of coordination among the many muscles in the mouth and throat that must work together in perfect precision to produce normal swallowing.  When food gets stuck in the mouth, the person may have to try several times to complete a swallow.  The muscles in the back of the throat — and in the esophagus — may also lose coordination, and individuals who have difficulty swallowing are at increased risk for food or liquid to get into the windpipe.  From there, it can get into the lungs (called aspiration), which can result in pneumonia.

Although treatment of dysphagia can be difficult, speech/swallowing therapists can instruct patients on swallowing techniques and on designing changes in food consistency that reduce the risk of aspiration.  Some improvement in coordination of the muscles used in swallowing may be achieved through adjustments in PD medications.  Only very rarely is it necessary to place a feeding tube.

Stomach problems
Impaired ability to empty the contents of the stomach, called gastroparesis, is another potential gastrointestinal complication of PD.  This may produce a bloated sensation and cause people to feel full even though they have eaten very little.  Sometimes nausea may develop.

Failure of the stomach to empty in a timely fashion may also impair or delay the effectiveness of PD medications, especially levodopa, since levodopa is absorbed from the small intestine and cannot get to its destination if it is trapped in the stomach.
Treatment of gastroparesis in Parkinson’s has not been extensively studied.  Domperidone is an effective medication, but unfortunately it is not available in the US.

Treatment routes that bypass the stomach, such as transdermal drug delivery by skin patch, may become available in the near future.  Another potential treatment under investigation involves a form of levodopa designed to be delivered directly into the small intestine via a feeding tube.

Progression of dysarthria + dysphagia in DLB, CBD, MSA, and PSP

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This will be of interest to anyone dealing with dysarthria (speech problems) and dysphagia (swallowing problems), which, according to the article should be everyone within one year of disease onset.  (I thought dysarthria meant slurred speech.  But, according to this article, it can also mean hypophonic speech or monotonic speech.)

This article is about dysarthria and dysphagia in autopsy-confirmed cases of DLB, CBD, MSA and PSP, all APDs (Atypical Parkinsonian Disorders).  PD cases are part of the study as well.

Here’s the citation and abstract:

Archives of Neurology. 2001 Feb;58(2):259-64.  

Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

Muller J, Wenning GK, Verny M, McKee A, Chaudhuri KR, Jellinger K, Poewe W, Litvan I.

BACKGROUND: Dysarthria and dysphagia are known to occur in parkinsonian syndromes such as Parkinson disease (PD), dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Differences in the evolution of these symptoms have not been studied systematically in postmortem-confirmed cases.

OBJECTIVE: To study differences in the evolution of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

PATIENTS AND METHODS: Eighty-three pathologically confirmed cases (PD, n = 17; MSA, n = 15; DLB, n = 14; PSP, n = 24; and CBD, n = 13) formed the basis for a multicenter clinicopathological study organized by the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Cases with enough clinicopathological documentation for the purpose of the study were selected from research and neuropathological files of 7 medical centers in 4 countries (Austria, France, England, and the United States).

RESULTS: Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months). Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months). Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs) (specificity, 100%) but failed to further distinguish among the APDs. Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months, P =.7) and latency to a complaint of dysphagia was highly correlated with total survival time (rho = 0.88; P<.001) in all disorders.

CONCLUSIONS: Latency to onset of dysarthria and dysphagia clearly differentiated PD from the APDs, but did not help distinguish different APDs. Survival after onset of dysphagia was similarly poor among all parkinsonian disorders. Evaluation and adequate treatment of patients with PD who complain of dysphagia might prevent or delay complications such as aspiration pneumonia, which in turn may improve quality of life and increase survival time.

PubMed ID#: 11176964  (see pubmed.gov for this abstract)

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These results were the most interesting (and depressing):

“Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs).”

“Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months).”

“Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months).”

“Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months).”

On this last point, here’s the actual chart on this from the article:

Survival Time After Onset of Dysphagia, months
PD        24 (2-61)
CBD     49 (25-89…..including a single patient with dysphagia but without dysarthria)
DLB     10 (3-17)
MSA     15 (6-68)
PSP       18 (6-96)

Based on this, I don’t understand why DLB’s short survival time isn’t highlighted.

Here are some excerpts from the article’s Comment section:

“(Early) dysarthria and perceived swallowing dysfunction are not features of PD.”

“[Dysarthria] as a presenting symptom has been described in clinical series of CBD, MSA, and PSP.  In PD and DLB, hypphonic/monotonous speech represented the most frequent type of dysarthria, whereas imprecise or slurred articulation predominated in CBD, MSA, and PSP.”

“In a clinical study of CBD, Rinne et al described dysarthria as one of the initial symptoms in 11% of the patients, which is close to our findings.  At follow-up, on average 5.2 years, dysarthria was diagnosed in 70% of the patients…  According to our findings, dysarthria occurred in almost every patient with CBD.”

“In agreement with our results, Quinn described the speech of patients with MSA as more severely affected than that of patients with PD, with slurring dysarthria, as well as the low volume and monotone of parkinsonism.”

“In both PSP and MSA, progressive dysarthria is believed to represent a manifestation of brainstem and cerebellar involvement.  In fact, PET studies revealed marked hypometabolism in the cerebellum and brainstem of patients with MSA, which correlated with dysarthria.”

“In our study, dysphagia was associated with concomitant dysarthria in all parkinsonian patients except one.  This sequence of dysphagia following dysarthria has also been reported in clinical studies of PD, MSA, and PSP.”

“…Golbe et al reported dysphagia after a median of 1 year after the onset of dysarthria in PSP.”

In all these disorders, “bronchopneumonia has been reported as a leading cause of death, which may be subsequent to silent aspiration resulting from dysphagia.”

“Most of our patients with MSA and PSP complained of a swallowing dysfunction, in contrast to patients with PD, CBD, and DLB…  Impaired lingual proprioception is hypothesized to contribute to the unawareness of swallowing difficulties in PD and might in part explain significantly longer latencies to dysphagia in our PD cases.  In contrast, patients with PSP were reported to be keenly aware of swallowing problems, including those with cognitive impairment.”

“(The) similarly short remaining survival time in PD and PSP after the onset of perceived dysphagia suggests that this symptom represents a reliable marker for the onset of functionally relevant swallowing abnormalities in both disorders.”

“Our findings of increased latency to dysarthria and dysphagia and similar time interval from onset of dysphagia to death in patients with PD compared with patients with APDs suggest that extrastriatal and nondopaminergic lesions represent an important factor for the development of dysarthria and dysphagia.  Indeed, Bonnet et al reported that dysarthria, gait, and postural stability had a decreased levodopa response in patients with long-standing PD who still benefited from the levodopa effects on tremor, rigidity, and akinesia.  Whereas the APDs are characterized by multiple system neuronal degenerations, in PD disease progression is determined by a progressive dopaminergic deficit arising from the selective neuronal degeneration of the substantia nigra pars compacta.”

Robin