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 Progression of dysarthria + dysphagia in DLB, PSP, etc 
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Post Progression of dysarthria + dysphagia in DLB, PSP, etc
This will be of interest to those dealing with dysarthria (speech problems) and dysphagia (swallowing problems), which, according to the article should be 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.
Cognitive Neuropharmacology Unit, The Champlain Building, 6410 Rockledge Dr, Suite 600, Bethesda, MD 20817-1844, USA.

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


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


Sun Apr 22, 2007 12:52 am
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This is one of the best articles written on MSA, DLB, PSP, and CBD because all the cases are pathologically-confirmed. But it's very depressing reading.

I just realized that the full article is available for free online:
http://archneur.ama-assn.org/cgi/reprint/58/2/259 --> PDF version
http://archneur.ama-assn.org/cgi/content/full/58/2/259 --> HTML version


Tue Aug 26, 2008 3:35 am
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An example of a swallowing issue is when there's choking on saliva or fluid draining in the back of the throat.

I don't think the existence of sinus draining, mucus, or phlegm has any bearing on whether there's a swallowing issue.


Tue Aug 26, 2008 12:58 pm
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
In this study of 14 autopsy-confirmed DLB patients, the average survival time after the onset of dysphagia was 10 months. The range was 3-17 months.


Tue Feb 08, 2011 1:46 am
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
Very interesting, Robin. Thanks for bringing this up again.

As you say, it is especially valuable because all of these cases were confirmed diagnosis. On the other hand, the study involved fewer than a hundred cases total, for all the diseases. This is interesting and valuable, but with it based on only 14 DLB cases, I'm not sure how representative of the entire Lewy Body population it is.

Again, this points up the need for more brain tissue with well-documented histories, so that suggestive research like this can be extended.

(Coy's documented history will show a diagnosis of dysphagia based on in-hospital swallowing tests, in 2003. He's still going strong. Of course, we don't have the DLB confirmation yet. 8) )

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Jeanne, 68 cared for husband Coy, 86. RBD for 30+ years; LDB since 2003, Coy at home, in early stage, until death in 2012


Tue Feb 08, 2011 11:09 am
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
I notice that the condition with the most examples, PSP with 24, also had the biggest range, from 9 to 96 months. I would bet a few Swiss chocolate bars that when more cases are studied, the ranges will increase for the other conditions.

I do think this is an extremely valuable piece of research and I am grateful that this kind of study is going on. I just don't think the results are any where near conclusive nor can they be applied directly to our LOs. It is interesting to know the average (of 14 cases) but we all still have to remember Your Mileage May Vary! :lol:

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Jeanne, 68 cared for husband Coy, 86. RBD for 30+ years; LDB since 2003, Coy at home, in early stage, until death in 2012


Tue Feb 08, 2011 3:35 pm
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
In fact, one CBD patient never had dysphagia.

I do think it's useful to know that *on average* within a small study, survival time was not very long after the onset of dysphagia. It simply serves as another reminder to get ones affairs in order and live life to the fullest.


Tue Feb 08, 2011 4:24 pm
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
Thank heavens that these questions are being studied.

In the past year since my mother was diagnosed, if you can call it that, I have learned that there is nothing predictable about LBD, and in fact it's not possible to say whether my mother has LBD, or a combination of LBD and something else, or something else entirely. And it even has been suggested to me more than once that because of her symptoms, she really might not have LBD at all, despite a thorough neuro workup at a major teaching hospital.

So the idea of understanding what is happening to her and where it will lead becomes more and more absurd. I have completely given up planning or expectations of any kind. All I know is she is deteriorating and therefore the SNF is the right place for her.

Surely it would be such an immense help to all the patients and their families if someone could tell them what is going on and what to expect.

Julianne


Tue Feb 08, 2011 4:57 pm
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
This is one of the best posts I've ever seen here! Thank you!

Certainly the key is to do a better job diagnosing patients while they are alive. Hopefully some biomarkers can be developed for that.

And it's very important to continue to do research like this with larger sample sizes. So little research is done on autopsy-confirmed DLB patients that I really shake my head and wonder how we are ever going to move forward.


Tue Feb 08, 2011 6:01 pm
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Post Re: Progression of dysarthria + dysphagia in DLB, PSP, etc
Thanks, Robin. Those thoughts have been floating around in my head for a while now, and they all came together when I reacted to reading this post. I think these are things that everyone with a newly-diagnosed LO needs to realize. It saves a lot of anguish and frustration to understand how elusive the answers are.

I totally agree--better diagnostic techniques are so desperately needed. Maybe as the huge baby boomer generation moves toward old age, they and their families will push for this.

Julianne


Tue Feb 08, 2011 6:36 pm
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