Skip to main content

Prognostic Factors for Survival in Multiple System Atrophy


An 88 year-old male with probable multiple system atrophy-parkinsonism (MSA-P) with at least 4 years of gait instability complicated by fall and cervical fracture, sick sinus syndrome status post pacemaker placement, postural hypotension requiring midodrine and fludrocortisone, achalasia status post BOTOX® injection and dilation, urinary retention status post chronic indwelling Foley catheter presented to the Geriatrics outpatient clinic to explore hospice care. In a brief background search of MSA, I found that there are 2 distinct presentations of MSA – cerebellar and parkinsonian. In discussing his prognosis with the patient, I wondered if in patients with MSA, does parkinsonism phenotype predict better survival than the cerebellar phenotype? And are there other disease specific or patient specific features that predict survival in MSA patients? 


I searched the terms “multiple system atrophy, survival” on PubMed Clinical Queries (website url), which I have often found more useful than regular PubMed general search. To narrow my results, I selected the “Prognosis” category and “Broad” scope for the search. I found a systematic review of pathologically confirmed cases of MSA identified through search of MEDLINE and other published reference lists from 1887 to 2/1995.

Individual patient data was reviewed, although there was no mention about assessment for validity of the individuals case reports and case series. Of the total of 433 cases included in the analysis, 358 cases were reported from 1971 through 1994. Chi-squared test for heterogeneity showed significant heterogeneity in age of onset, autonomic features, parkinsonian features, pyramidal features and MSA type across the board. The mean age onset was at 54.2 years of age, and mean survival around 6 years. Majority of the cases (> 80%) had autonomic features or parkinsonism.

The only significant predictor of increased survival were younger age at onset, more recent publication, and presence of cerebellar features. The results were not altered when accounting for the life expectancy of the general population over time from 1887 through 1970 and from 1971 through 1994. There was a trend toward worse survival when the case had more than one clinical features (parkinsonism, autonomic, cerebellar, pyramidal)

Back to the Patient: Using the information from this systematic review and patient’s clinical picture of likely at least a 4 year of disease duration and multiple clinical features, his clinical presentation confers to worse survival. When this was discussed with him and his family, in light of his rapid functional decline over the last 10 months, our patient agreed to enroll into hospice.

Take-home Points:
1. Age of onset for MSA is around 54 years. Older age of onset is a predictor for worse survival.

2. Cerebellar predominant feature is a predictor for increased survival.

3. Recent publication date is a predictor for increased survival, although there may be unaccounted confounder not included in this analysis. Clinical relevance of this finding is limited.

by: Pei Chen, MD
Department of Geriatrics and Palliative Medicine
Icahn School of Medicine at Mount Sinai

This post is part of the #GeriCases series, in which we discuss a clinical case in geriatrics and the attempt to provide patient-centered care with the use of best available evidence. 

Picture courtesy: Paco CT

Ravishankar Ramaswamy, MD
@RavRamaswamy
Section Editor, #GeriCases

Comments

Anonymous said…
Please see New England Journal of Medicine review article on Multiple System Atrophy - January 15, 2015

https://www.facebook.com/ProfGregorKWenning

http://www.nejm.org/doi/full/10.1056/NEJMra1311488

Popular posts from this blog

Practical Advice for the End of Life: A Podcast with BJ Miller

This week we talk with BJ Miller, hospice and palliative care physician, public speaker, and now author with Shoshana Berger of the book "A Beginner's Guide to the End."

As we note on the podcast, BJ is about as close as we get to a celebrity in Hospice and Palliative Care.  His TED Talk "What Really Matters at the End of Life" has been viewed more than 9 million times.  As we discuss on the Podcast, this has changed BJ's life, and he spends most of his working time engaged in public speaking, being the public "face" of the hospice and palliative care movement.

The book he and Berger wrote is filled to the brim with practical advice.  I mean, nuts and bolts practical advice.  Things like:
How to clean out not only your emotional house but your physical house (turns out there are services for that!)Posting about your illness on social media (should you post to Facebook)What is the difference between a funeral home and mortuaryCan I afford to die?  …

Caring, and the Family Caregivers We Don’t See

Over lunch at a restaurant in Manhattan, my father and I talked about long-term care insurance and the emergence of senior centers and nursing homes across the U.S. that offer a variety of ethnic cuisines and cultural events, catering not only to a growing population of adults over 65, but also, to an increasingly diverse population of adults who call the U.S. their home. This conversation was different from many similar ones before it – we weren’t talking about my research; we were talking about our own lives.
My parents immigrated to the U.S. in the late ‘70s and early ‘80s, following their parents’ advice on professional opportunities that seemed unimaginable in India at the time. Although they considered moving back soon after to care for their aging parents and to raise children, they ultimately decided to stay in the U.S. As I chronicled earlier, my paternal grandparents lived with us until I completed middle school, at which point they returned to India and lived with my mater…

Top 25 Studies in Hospice and Palliative Care (#HPMtop25)

by: Kara Bishoff (@kara_bischoff )

Back in 2015 we wrote a post asking for input on what articles should belong on a list of the top 25 articles in hospice and palliative care.   We decided to focus on hospice palliative care studies and trials - as opposed to review articles, consensus statements and opinion pieces.

Here’s what we came up with. It was hard to pick just 25! We highly prioritized clinical utility and tried to achieve diversity & balance. Many others are worthy of inclusion. Take a look and let us know if you have suggested changes for next year.

Module 1: Symptom Management
Randomized, Double-Blind, Placebo-Controlled Trial of Oral Docusate in the Management of Constipation in Hospice Patients. Tarumi Y et al. JPSM, 2013.Once-Daily Opioids for Chronic Dyspnea: A Dose Increment and Pharmacovigilance Study. Currow DC et al. JPSM, 2011.Effect of palliative oxygen versus room air in relief of breathlessness in patients with refractory dyspnoea: a double-blind, randomise…