July 2015 - Brain Support Network

Food stagnation and esophageal dysmotility in MSA

Date July 26, 2015

Recently, there was a Japanese study published in Dysphagia (a journal) comparing esophageal involvement in MSA and ALS. They investigated something called “food stagnation,” which I’m assuming is where food gets stuck in the esophagus. Researchers looked at 16 MSA patients with dysphagia and 16 ALS patients with dysphagia.

The paper wasn’t really about treatment but more about the need to use videofluorography to analyze the esophageal phase in those with MSA (or ALS) to see how severe food stagnation is. In this small study, food stagnation occurred in 100% of the MSA patients. It was only present in 25% of the ALS group. Aspiration pneumonia and sudden death are concerns. I think sudden death is due to suffocation on regurgitated food.

Those with MSA are warned not to lie down right after eating. And the researchers expressed some concern that CPAP machines may exacerbate food regurgitation. They note that this concern is “purely speculative.”

I’ve copied the abstract below.

Someone on the ShyDrager Yahoo!Group had asked about “esophageal dysmotility.” While I was looking up a definition of that term, I stumbled across a useful website called wisegeek.com. They have a good webpage on esophageal dysmotility:

wisegeek.org/what-is-esophageal-dysmotility.htm

There was a short paragraph about treatment:

“Dysmotility disorders of the esophagus are generally incurable. The main objective of treatment is therefore symptom management and relief. Several types of medication can be used for dysmotility treatment, including calcium channel blockers and botulinum toxin. Dietary modifications can help relieve symptoms, and some disorders can be improved with surgery.”

I posted some of this info on the ShyDrager Yahoo!Group. One of our local BSN group members, Don, posted that his wife was harmed by botox injections. She received injections in the salivary glands to treat excess salivation. As a result, her swallowing went “from difficult to impossible.” She had to be hospitalized and came home with a feeding tube. Don notes that this does not happen to everyone. Indeed, we’ve had many group members who have received botox injections for drooling to no ill effect. But it’s certainly a risk.

Robin

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Abstract

Dysphagia. 2015 Jul 24.

Esophageal Involvement in Multiple System Atrophy.
Taniguchi H, Nakayama H, Hori K, Nishizawa M, Inoue M, Shimohata T.

Abstract
The prevalence of esophageal involvement and its impact on clinical manifestations in patients with multiple system atrophy (MSA) remains unknown. We recruited 16 consecutive patients with dysphagia associated with MSA (MSA group) and 16 consecutive patients with dysphagia associated with amyotrophic lateral sclerosis (ALS group). We assessed the presence or absence of food stagnation within the esophagus using videofluorography. Food stagnation within the esophagus was observed in 16 patients (100 %; 7 severe, 9 mild) in the MSA group and in 4 patients (25 %; 4 mild) in the ALS group (P < 0.001). Follow-up videofluorography revealed that food stagnation in patients with MSA could exacerbate during the disease course. Patients with MSA and severe food stagnation showed a wide range of intraesophageal stasis by videofluorography. Among the 16 patients in the MSA group, 4 developed aspiration pneumonia and 1 died of suffocation associated with food regurgitation during continuous positive airway pressure therapy. In conclusion, food stagnation within the esophagus occurs more frequently in MSA patients with dysphagia than in ALS patients with dysphagia. Because food stagnation can cause serious complications such as aspiration pneumonia and suffocation, patients with MSA should be evaluated by videofluorography, especially those with stagnation in the esophageal phase.

PMID: 26205436

Learning to communicate and enjoy time with someone with dementia

Date July 16, 2015

Posted by Robin Riddle

This is a very sweet article from The New York Times about a son who learns to communicate and enjoy time with his mother with Alzheimer’s.

Here are the key excerpts:

My oldest brother [Mark] … talks to my mom by phone every day. He enjoys trading gibberish with her in their incomprehensible conversations. “She’s completely unplugged from reality and disoriented to time and space,” Mark says. “It doesn’t make any sense, so why not have a good time?” At first, I worried Mark’s antics were making fun of my mom — laughing at her, rather than with her. Then I tried it. I stopped concentrating on the content of her words and, instead, went along with every twist. Once she said she wanted to go somewhere for a picnic. “Why, Mom?” I asked. She said: “So we can boil the chicken.” Rather than grilling her about what she meant, I asked what kind of chicken we should boil and where we should hold this picnic.

Mom’s private caregiver, Ellen Knapp, … taught me how to talk to Mom in this new phase of her life. The technique is called validation, and Ellen learned it from a veteran author and psychologist, Naomi Feil. As Ms. Feil explains it to me in a phone interview, this translates to “accepting whatever behavior the person has and trying to become a part of it.” For example, a carpenter with Alzheimer’s might pound his fist against a wall. The wrong way to talk to him would be to say, “Why are you pounding the wall? Stop it!” The right way would be to ask, “Is that wood made out of oak or pine?”

I’m not exactly a Jedi knight of this technique, but I find it relaxes Mom and makes her more easygoing and less frustrated. The key, for me, is to stop judging or trying to analyze or change her behavior. That means fewer questions about her past, even if that subject is difficult for me to avoid — in the past, she was my mom! That’s what I really want to talk to her about, not chickens and picnics. Occasionally I can’t resist and I ask whether she remembers Dad, her husband of 54 years. Sometimes she says yes. Sometimes she says no. It’s a dead end either way.

Here’s a link to the article:

www.nytimes.com/2015/07/12/opinion/sunday/my-mother-lost-and-found.html

SundayReview | Opinion
New York Times
My Mother, Lost and Found
By Steve Knopper
July 11, 2015

Well worth reading…

Robin

“When DLB, PD, and PSP masquerade as MSA” (Mayo autopsy study)

Date July 4, 2015

Posted by Robin Riddle

We’ve been waiting for this sort of article to be published for a long time. And we need similar articles for all the disorders in our group, not just MSA. The article calls into question diagnostic criteria (for PD and PSP) and calls into question assumptions made by clinicians (for MSA and PD).

A fascinating article was published in the journal Neurology this week. It’s from Mayo researchers and focuses on 134 patients who had clinical diagnoses of MSA and donated their brains between 1998 and 2014. As you may know, Brain Support Network helps people nationally donate a loved one’s brain. We were responsible for about 25% of those 134 (clinical) MSA brain donations!

Of the 134, 125 had adequate medical records to review. (It’s so important to provide complete medical records when you are donating a loved one’s brain! Sadly, not everyone does.) Of the 134 patients, only 62% actually had MSA upon brain autopsy! The most common misdiagnosis was DLB (37% – wow!), followed by PSP (29%) and Parkinson’s Disease (15%). (18% had other diagnoses including CBD and vascular parkinsonism.)

Those who actually had DLB and PD, not MSA, had autonomic failure — which is what led the clinicians astray in their diagnosis. (According to the PD diagnostic criteria, severe dysautonomia in early stages is an exclusionary criteria.)

Those who actually had PSP, not MSA, had cerebellar ataxia — which is what led the clinicians astray in their diagnosis. (According to the PSP diagnostic criteria, cerebellar ataxia is an exclusionary criteria.) Apparently, there is an atypical form of PSP called PSP-cerebellar. The authors note that a recent study “has shown that older onset, early falls, and vertical gaze palsy without dysautonomia may differentiate PSP-C from MSA-C.”

We have certainly seen this in our local support group — people diagnosed with MSA but it turns out they have DLB, PD, or PSP. Happens all the time. In our local sample, the diagnostic accuracy is closer to 50%. Clinicians do not seem to understand that:

(a) autonomic failure can occur in DLB,

(b) hallucinations and dementia do not occur in MSA, and

The diagnostic accuracy was not different between general neurologists and movement disorder specialists.

Retrospectively, the 125 patients with clinical records were diagnosed with probable MSA, possible MSA, or unknown. 49 patients met the criteria for probable MSA, 35 for possible MSA, and the remaining 41 were not assigned due to lack of clinical information (such as whether the patient was responsive to levodopa). Of those with probable MSA or possible MSA, the diagnostic accuracy was 71% and only 60% respectively.

The authors note that “Correctly diagnosed patients with MSA had a younger age at onset and age at death than patients with PD or PSP.” Duration of symptoms wasn’t different.

The authors looked at frequency of clinical features among the various confirmed diagnoses.

“Comparing MSA and DLB, urinary incontinence, limb ataxia, nystagmus, and pyramidal signs were more frequent in MSA. Cognitive impairment and visual hallucinations were more frequent in DLB. Comparing MSA and PD, urinary incontinence was less frequent and visual hallucinations were more frequent in PD. Comparing MSA and PSP, urinary incontinence, constipation, orthostatic hypotension, and RBD were more frequent in MSA. Vertical gaze palsy was more frequent in PSP. Frequency of levodopa responsiveness and average Mini-Mental State Examination score were not different among the groups.”

This aspect of “levodopa responsiveness” is interesting to me because we are always told that one symptom of MSA, PSP, DLB, and CBD is that there’s either no or a poor response to levodopa, as compared to PD. I asked Dr. Neng Huang, BSN’s medical advisor, about this recently as we have a local group member whose husband was diagnosed with MSA during life but PD on brain autopsy. Dr. Huang pointed out that “poor tolerance” of levodopa is not the same thing as “poor response.” Many people have side effects when taking levodopa and want to discontinue it. This seems to be viewed in the movement disorder specialist’s mind as being non-responsive rather than poor-tolerance.

The abstract makes an interesting statement about DLB. Apparently not everyone with DLB confirmed through autopsy had dementia during life! If someone doesn’t have dementia, they don’t qualify for a DLB diagnosis during life. The authors wonder if the cognitive impairment was overlooked because few of the DLB-masquerading-as-MSA patients had neuropsychological evaluations.

The researchers point out that a 62% diagnostic accuracy isn’t adequate for either patient care or MSA research. Obviously, we won’t have success with MSA research if only 62% of the participants actually have MSA!

Those are the highlights. The abstract is copied below. You can now find the full paper for free online:

www.ncbi.nlm.nih.gov/pmc/articles/PMC4534078/

Robin

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Here’s the abstract:

Neurology. 2015 Jul 2. [Epub ahead of print]

When DLB, PD, and PSP masquerade as MSA: An autopsy study of 134 patients.

Koga S, Aoki N, Uitti RJ, van Gerpen JA, Cheshire WP, Josephs KA, Wszolek ZK, Langston JW, Dickson DW.

OBJECTIVE:
To determine ways to improve diagnostic accuracy of multiple system atrophy (MSA), we assessed the diagnostic process in patients who came to autopsy with antemortem diagnosis of MSA by comparing clinical and pathologic features between those who proved to have MSA and those who did not. We focus on likely explanations for misdiagnosis.

METHODS:
This is a retrospective review of 134 consecutive patients with an antemortem clinical diagnosis of MSA who came to autopsy with neuropathologic evaluation of the brain. Of the 134 patients, 125 had adequate medical records for review. Clinical and pathologic features were compared between patients with autopsy-confirmed MSA and those with other pathologic diagnoses, including dementia with Lewy bodies (DLB), Parkinson disease (PD), and progressive supranuclear palsy (PSP).

RESULTS:
Of the 134 patients with clinically diagnosed MSA, 83 (62%) had the correct diagnosis at autopsy. Pathologically confirmed DLB was the most common misdiagnosis, followed by PSP and PD. Despite meeting pathologic criteria for intermediate to high likelihood of DLB, several patients with DLB did not have dementia and none had significant Alzheimer-type pathology. Autonomic failure was the leading cause of misdiagnosis in DLB and PD, and cerebellar ataxia was the leading cause of misdiagnosis in PSP.

CONCLUSIONS:
The diagnostic accuracy for MSA was suboptimal in this autopsy study. Pathologically confirmed DLB, PD, and PSP were the most common diseases to masquerade as MSA. This has significant implications not only for patient care, but also for research studies in MSA cases that do not have pathologic confirmation.