MSA Description for Emergencies

[Guest guest]

For some time I have been watching the posts for a concise and accurate

written description to provide to medical professional who don't know what

MSA is all about.

When I say concise, I would think one or two pages at the most. When I say

accurate, it would be easily verified; e.g. with references.

I have been faithfully building a file of written documents on MSA using

posts from the list and searches on the net. At this point, it is a good

reference library. However, none of these print outs seem to fit the bill

as described above.

I was reminded of this need when I had to take Terry to the ER recently

after a rather bad fall. I was able to satisfy their need this time from

my own knowledge with a verbal description.

Has anyone else filled this need?

Sennewald Charlottesville, Virginia

[Guest guest]

& Beth, The best 2 page MSA intro/info I have found is from Parkinson

Report, Spring 1998 by NPF Center of Xcellence at Vandy members

on, Blaser, , Ariel Deutch ancient but

comprehensive and an easy read for anyone.

Perhaps Pam B. or Don S. could get this updated or find something similar

for us to use wherever possible.

A new explanation of MSA, Parkinson's Plus and the alphabet soup of related

disorders would be helpful.

LA Louise in Springhill where upper 90's temp with similar humidity competes

with AC's and the available power supply.

[Guest guest]

I agree that having a MSA description for emergencies would be very

helpful. I too could have used one recently when my husband was having a

sleep study done and passed out while going to the bathroom. The people

at the sleep center called for an ambulance and had my husband taken to

the emergency room. All of this could have been avoided if they had a

brief description of MSA. It also would have been nice to have a

description to give to the personnel in the emergency room.

Beth

On Sun, 07 Jul 2002 13:36:00 -0400 Sennewald perrys@...>

writes:

> For some time I have been watching the posts for a concise and

> accurate

> written description to provide to medical professional who don't

> know what

> MSA is all about.

>

> When I say concise, I would think one or two pages at the most.

> When I say

> accurate, it would be easily verified; e.g. with references.

>

> I have been faithfully building a file of written documents on MSA

> using

> posts from the list and searches on the net. At this point, it is a

> good

> reference library. However, none of these print outs seem to fit

> the bill

> as described above.

>

> I was reminded of this need when I had to take Terry to the ER

> recently

> after a rather bad fall. I was able to satisfy their need this time

> from

> my own knowledge with a verbal description.

>

> Has anyone else filled this need?

>

> Sennewald Charlottesville, Virginia

>

>

>

>

> If you do not wish to belong to shydrager, you may

> unsubscribe by sending a blank email to

>

> shydrager-unsubscribe

>

>

>

>

>

[Guest guest]

,

The write up by Dr. Jankovic that was posted recently is good. Did you see

it?

It's at http://www.bcm.tmc.edu/neurol/jankovic/educ_msa.htm

and here's a copy:

Multiple System Atrophy

ph Jankovic, M.D.

What is Multiple System Atrophy (MSA)?

Multiple system atrophy (MSA) refers to syndromes with features that overlap

with three previously-described neurodegenerative disorders: Shy-Drager

syndrome (SDS), striatonigral degeneration (SND), and olivopontocerebellar

atrophy (OPCA). As a group, all three types of MSA are characterized by

parkinsonism, especially rigidity, poverty of movement, and loss of balance.

However, the resting tremor which is often an early feature of true

Parkinson's disease is not a prominent feature of MSA. Other symptoms

associated with MSA can include varying amounts of weakness and spasticity

and disturbance of the central autonomic nervous system causing

lightheadedness or fainting spells upon standing (because of a marked fall

in blood pressure), incontinence of bowel and bladder, and impotence in

males. These symptoms are further discussed as they relate to the three

types of MSA below.

A. What is Shy-Drager Syndrome (SDS)?

Shy-Drager Syndrome (SDS) is a form of MSA named after the two physicians

who first described it in 1960: Dr. Milton Shy from the National Institutes

of Health and Dr. Glenn Drager from the Baylor College of Medicine in

Houston. Patients with SDS often experience symptoms of fainting or

dizziness upon changing position (such as sitting up or standing up from a

laying down position), impotence (loss of erectile function in males), and

urinary incontinence. The light-headedness and fainting result from a drop

in blood pressure that occurs upon standing, which in turn decreases the

amount of blood flowing to the brain. When the blood pressure drop becomes

severe, the blood flow to the brain becomes insufficient to maintain

consciousness, and the patient passes out. When the head is lowered, as when

the patient collapses to the floor, the amount of blood reaching the brain

increases as the blood pressure rises, and the patient regains

consciousness.

The above symptoms occur because SDS damages the autonomic nervous system,

the part of the nervous system which controls autonomous functions that are

essential to survival. These functions include regulation of heart rate and

breathing, in addition to control of intestinal, urinary, and sexual

functions. The autonomic nervous system also controls skin and body

temperature, and the body's response to stress. Because the autonomic

nervous system also controls constriction and dilation of the pupils of the

eye, as well as focusing the eye, SDS patients sometimes complain of blurry

vision.

SDS is more common in men than women, and symptoms often first begin when

patients are in their 50's. Among the first 2000 patients referred to the

Baylor College of Medicine Parkinson's Disease Center and Movement Disorders

Clinic, SDS was the cause of parkinsonism in 1.8% of the patients. Patients

later develop parkinsonian features, including slowed and shuffling gait,

difficulty with balance when standing or walking, mild (if any) tremor,

slurred speech, constipation, and loss of control of bowel and bladder.

Patients also may develop inappropriate crying and laughing spells,

hoarseness and interference with breathing from vocal cord paralysis, and

longer than normal gaps in breathing during sleep (known as sleep apnea).

Blood pressure can drop severely upon standing (for example down to 70/40

and lower). Patients may be troubled by dry mouth, dry skin, abnormal

sweating, and they may have difficulty controlling normal body temperature.

Eventually they can develop irregularities in heart rate, and problems with

chewing, swallowing, and breathing. The course of the disease is variable.

While some patients live for up to 20 years after the onset of symptoms,

most patients reach severe disability within 7 to 8 years.

Diagnosis is sometimes aided by testing autonomic nervous system function,

for example, by measuring blood pressure and heart rate with the patient

laying down compared with standing up. A polysomnogram, or sleep study, can

document sleep apnea (gaps in breathing during sleep). Brain imaging with CT

and MRI are usually helpful in differentiating typical Parkinson's disease

from atypical parkinsonism.

Finding a cure for this chronic, progressive disease will not be possible

until the cause(s) is identified. The treatment of SDS is difficult because

levodopa can lower further the already low blood pressure. There may be no

improvement even with large doses of levodopa. Treatments that lessen the

postural blood pressure changes include increasing the amount of salt in the

diet, use of fludrocortisone (a steroid hormone which increases blood

pressure) or midodrine, and daytime use of support hose such as Jobst

stockings (to decrease pooling of blood in the legs). Because these methods

can increase blood pressure to undesirably high levels when the patient is

lying down, the head of the patient's bed should be elevated at least 30

degrees. Other medicines including indomethacin (Indocin), non-steroidal

anti-inflammatory drugs, beta blockers, and central stimulants are sometimes

used with variable success. Rarely, a cardiac pacemaker to treat irregular

heartbeat, and surgery to place feeding or breathing tubes are needed.

B. What is Striatonigral Degeneration (SND)?

Striatonigral degeneration (SND) is another disorder included in the general

category of MSA. As with all MSA patients, SND patients have parkinsonian

symptoms such as stiffness and muscular rigidity, slowed movement, and

marked postural instability. Tremor is usually mild, if present at all. SND

patients often have early onset falling, speech which is severely slurred

and soft, excessive snoring, abnormal pauses between breaths during sleep

(sleep apnea), difficulty breathing, and varying levels of muscle stiffness.

Studies have shown that a deep brain nucleus known as the " putamen " (part of

the basal ganglia of the brain) is particularly damaged by SND, in addition

to the substantia nigra (the part of the brain particularly affected in

typical Parkinson's disease). Some patient's symptoms improve somewhat in

response to high doses of levodopa.

C. What is Olivopontocerebellar Atrophy (OPCA)?

The term olivopontocerebellar atrophy (OPCA) refers to a diverse group of

degenerative disorders which feature progressive parkinsonism (stiffness and

rigidity, slow movement, postural instability) in combination with tremors

and incoordination that worsen with movement (this form of incoordination is

called ataxia). This action tremor is distinctly different from the resting

tremor seen in typical Parkinson's disease, and arises from involvement of a

part of the brain, known as the cerebellum. Some OPCAs occur in families

(genetically inherited); the OPCAs which do not run in families (are

" non-familial " and " not-inherited " ) are the ones included by the term " MSA " .

The sporadic (non-familial) form of OPCA occurs equally in men and women.

Age at onset ranges from childhood to age 66. The first symptom is usually

ataxia (incoordination) and loss of balance, followed later by parkinsonian

symptoms. A few years after onset, patients can develop memory problems,

difficulty with eye movements, slurred speech, difficulty with swallowing,

incontinence, weakness, loss of muscle mass, and muscle stiffness.

Brain imaging studies in OPCA, such as CT and MRI scans, demonstrate

decreased size of the cerebellum and brainstem structures (atrophy). As with

all MSAs, the main treatment is a trial of high dose levodopa in an attempt

to alleviate some of the parkinsonian symptoms. Unfortunately the response

to levodopa is usually poor.

REFERENCES

General:

Jankovic J: Parkinson's plus syndromes. Mov Disord 1989;4:S95-S119.

Jankovic J. Treatment of parkinsonian syndromes. In: Kurlan R, ed. Treatment

of Movement Disorders, J.B. Lippincott Company, Philadelphia, PA,

1995:95-114.

Multiple System Atrophy:

Quinn N: Multiple system atrophy. In: Marsden CD, Fahn S. Movement Disorders

3, Butterworth-Heinemann, Oxford, 1994:262-281.

Gilman S. Multiple System Atrophy. In: Jankovic J, Tolosa E, eds.

Parkinson's Disease and Movement Disorders, 3rd edition. and

Wilkins, Baltimore, 1998: 245-262.

Berciano J. Olivopontocerebellar atrophy. In: Jankovic J, Tolosa E, eds.

Parkinson's Disease and Movement Disorders, 3rd edition. and

Wilkins, Baltimore, 1998:263-297.

MSA Description for Emergencies

> For some time I have been watching the posts for a concise and accurate

> written description to provide to medical professional who don't know what

> MSA is all about.

>

> When I say concise, I would think one or two pages at the most. When I

say

> accurate, it would be easily verified; e.g. with references.

>

> I have been faithfully building a file of written documents on MSA using

> posts from the list and searches on the net. At this point, it is a good

> reference library. However, none of these print outs seem to fit the bill

> as described above.

>

> I was reminded of this need when I had to take Terry to the ER recently

> after a rather bad fall. I was able to satisfy their need this time from

> my own knowledge with a verbal description.

>

> Has anyone else filled this need?

>

> Sennewald Charlottesville, Virginia

>

>

>

> If you do not wish to belong to shydrager, you may

> unsubscribe by sending a blank email to

>

> shydrager-unsubscribe

>

>

>

>

>

[Guest guest]

Pam:

Do you recognize the article Louise is talking about below?

Message: 16

Date: Sun, 7 Jul 2002 13:48:38 -0500

Subject: Re: MSA Description for Emergencies

& Beth, The best 2 page MSA intro/info I have found is from Parkinson

Report, Spring 1998 by NPF Center of Xcellence at Vandy members

on, Blaser, , Ariel Deutch ancient but

comprehensive and an easy read for anyone.

Perhaps Pam B. or Don S. could get this updated or find something similar

for us to use wherever possible.

A new explanation of MSA, Parkinson's Plus and the alphabet soup of related

disorders would be helpful.

LA Louise in Springhill where upper 90's temp with similar humidity competes

with AC's and the available power supply.

Sennewald Charlottesville, Virginia

[Guest guest]

,

It sounds like this article:

http://www.parkinson.org/atrophy.htm

Take care, Bill Werre

======================================

>

>

> Date: 2002/07/08 Mon PM 04:09:21 CDT

> To: shydrager

> Subject: Re: MSA Description for Emergencies

>

> Pam:

>

> Do you recognize the article Louise is talking about below?

>

> Message: 16

> Date: Sun, 7 Jul 2002 13:48:38 -0500

>

> Subject: Re: MSA Description for Emergencies

>

>

>

> & Beth, The best 2 page MSA intro/info I have found is from Parkinson

> Report, Spring 1998 by NPF Center of Xcellence at Vandy members

> on, Blaser, , Ariel Deutch ancient but

> comprehensive and an easy read for anyone.

[Guest guest]

:

Not sure if this is what your looking for.

Take Care Vera

web site==MSA

Multiple System AtrophyThis term abbreviated MSA defines a specific syndrome within the larger less well defined category of multiple system degenerations. The features of the MSA include: parkinsonism, cerebellar or corticospinal signs, orthostatic hypotension, impotence, and urinary incontinence or retention, usually preceding or within two years after the onset of the motor symptoms. The previous division into Shy Drager Syndrome (SDS), Striato Nigral Degeneration (SND), and Olivopontocerebellar Atrophy (OPCA) has been dropped. Immunohistochemistry demonstrates that these three disorders share a common pathology. The latest diagnostic criteria are shown in the table below.Wenning et. al. determined that the combination of autonomic insufficiency, speech or bulbar dysfunction, absence of dementia, postural instability with falls, poor response to levodopa, and absence of levodopa-induced confusion gave a diagnostic sensitivity and specificity greater than 90%.Median age of onset of MSA is about age 55 years (range of 33 to 76). It affects men slightly more than women. Nearly half of patients are disabled or wheelchair bound within 5 years of the onset of motor symptoms. Mean survival is 6 to 7 years. 80% of MSA patients develop predominant parkinsonism (MSA-P) and 20% develop predominant cerebellar signs (MSA-C). The latter statistics are likely skewed by referral patterns to movement disorder centers. There is considerable overlap. Cerebellar features are present in over 40% of patients with SND type, and parkinsonism is detectable in 50% of OPCA type patients. 30% and 65% of MSA patients had a good levodopa response at some stage. Between 13% and 30% maintained some response through the course of the illness. 25% to 50% of those treated with levodopa had dyskinesias (particularly orofacial) and dystonia, even if they did not experience improvement in motor state.Autonomic symptoms were the initial feature in 41% of patients, but ultimately 97% of patients developed some degree of autonomic dysfunction. The most frequent autonomic symptom in men was impotence and in women urinary incontinence. Orthostatic hypotension occurred in 68%.A mild restriction of downgaze may develop in about 10% of MSA cases.. Anterior horn cell loss may occur but is uncommon. Anal sphincter EMG (90% have an abnormality) is a sensitive and specific diagnostic test for MSA. Even less frequently seen is a mild sensory neuropathy. Classic rest tremor is uncommon (29%) Cerebellar signs occurred in 54% of patients and upper motor neuron signs in 49% of the cases. SND type generally demonstrates more tremor, pyramidal signs, and myoclonus than OPCA type. Severe dementia is uncommonRespiratory stridor (which ultimately occurs in 1/3 of cases) in combination with parkinsonism is highly suggests MSA until proved otherwise; although stridor can also occur in PD, it is exceptionally rare.

Multiple System Atrophy: Clinical Diagnostic Criteria

Striato Nigral Degeneration Type(predominant parkinsonism)Sporadic adult-onset (age 30 years or above)Possible Non/poorly levodopa responsive parkinsonism*ProbableAbove**, plus severe symptomatic autonomic failure*** or cerebellar signs or pyramidal signs or pathological sphincter EMG Definite Post mortem confirmed

OLIVOPONTOCEREBELLAR Type(predominantly cerebellar)Sporadic adult-onset (age 30 years or above)PossibleCerebellar syndrome with parkinsonism ProbableSporadic adult-onset cerebellar syndrome*(with or without parkinsonism or pyramidal signs) plus severe symptomatic autonomic failure*** or pathological sphincter EMG Definite Post mortem confirmed

* Without DSM III dementia, generalised tendon areflexia, downgaze PSNP or other identifiable cause.** Moderate or good, but often waning, response to levodopa may occur, in which case multiple atypical features need to be present.*** Postural syncope or presyncope and/or urinary incontinence or retention not due to other causes.Sporadic: one other case of typical clinical IPD among 1st or 2nd degree relatives allowable.

Frequency of individual clinical features in 203 cases of MSA

From Wenning, et al, MSA: A Review of 203 Pathologically proven Cases Movement Disorders Vol 12 No 2 1997 – 133-147

Autonomic symptomsUrinary incontinence 55% Postural faintness 51%Impotence 47%Recurrent syncope 18%Fecal incontince 12% Parkinsonism Akinesia 83% Tremor 67% Rigidity 63% Best L-Dopa response Poor 72% Good 28% Last L-Dopa response Poor 95% Good 5% Dyskinesias Orofacial 15% Limbs 10%Fluctuations 24%

Cerebellar Signs Gait ataxia 49% Limb ataxia 47% Intention Tremor 24% Nystagmus 23% Pyramidal signs Hyperreflexia 46% Babinski 41% Spasticity 10% Other features Intellectual deterioration Mild 22% Moderate 2% Severe 0.5% Stridor 13% Dystonia 12% Anisocoria 8% Contractures 7%

General References On Clinical Features of Parkinsonisms

*Handbook of Clinical Neurology Vol 49 Extrapyramidal disorders: Vinken, Bruyn, Klawans eds. Elsevier Science publishers 1996Movement Disorders a Comprehensive Survey;: Weiner, Lang A. eds. Futura publishing company 1989Neurodegenerative Diseases, Calne, D., eds. W.B. Saunders Company 1994Parkinson's Disease and Movement Disorders, Jankovic, Tolosa ,eds. Urban & Scharzenberg 1988Clinics of North America Volume 83 Number 2 March 1999

MSAWenning, et al, MSA: A Review of 203 Pathologically Proven Cases. Movement Disorders Vol 12 No 2 March 1997 – 133-147Quinn, N; Lecture notes in AAN syllabus on Multiple System Atrophy AAN 1999 TorontoMargery H. Mark Lumping and splitting the parkinson plus syndromes Neurologic Clinics Volume 19 Number 3 August 2001

On Sun, 07 Jul 2002 13:36:00 -0400 Sennewald <perrys@c...>

writes:

> For some time I have been watching the posts for a concise and

> accurate

> written description to provide to medical professional who don't

> know what

> MSA is all about.

>

> When I say concise, I would think one or two pages at the most.

> When I say

> accurate, it would be easily verified; e.g. with references.

>

> I have been faithfully building a file of written documents on MSA

> using

> posts from the list and searches on the net. At this point, it is a

> good

> reference library. However, none of these print outs seem to fit

> the bill

> as described above.

>

> I was reminded of this need when I had to take Terry to the ER

> recently

> after a rather bad fall. I was able to satisfy their need this time

> from

> my own knowledge with a verbal description.

>

> Has anyone else filled this need?

>

> Sennewald Charlottesville, Virginia

>