MSA Overview in Continuum, August 2016

An overview of diagnosing atypical parkinsonian disorders, including multiple system atrophy (MSA), was published in the August 2016 issue of Continuum, a journal for neurologists.

The article describes MSA as follows:

MSA is characterized by variable presentations of parkinsonism, cerebellar and pyramidal signs, and autonomic dysfunction. … Two clinical phenotypes are generally distinguished by predominant parkinsonism (MSA–parkinsonian type [MSA-P]) or predominant cerebellar ataxia (MSA–cerebellar type [MSA-C]). Median age of onset for MSA is 58 years of age, which is younger than that of PSP and CBD. No MSA cases have been identified younger than age 30, whereas for PSP the cutoff age is 50 years. Disease progression is faster than in PD and mean survival is approximately 6 to 9 years, consistent with more widespread neurodegeneration.

There’s nothing new in the article (except the very brief mentions of dementia in MSA – a bit more on that below) but it provides a good summary of current knowledge about MSA.  The article discusses:

* MSA-P symptoms, including parkinsonism, tremor (distal, myoclonic), rigidity, dystonia, anterocollis, early falls, dysarthria, dysphonia, autonomic failure (69%), dyskinesia (orofacial common).  A case study of someone with MSA-P is provided.

* MSA-C symptoms, including limb ataxia, gait ataxia, early falls, dysarthria (ataxic), dysphagia, gaze impairment, emotionality

* MSA pathology

* MSA diagnostics

* MSA treatments

Some excerpts are copied below.

The author lists “progressive dementia” as a possible symptom of MSA-C.  And, the author points to an article from 2010 that estimates “dementia in 14% to 16% of patients with MSA.”  I’ll have to read that 2010 article to see if there’s autopsy confirmation and if they really mean “dementia” or “cognitive impairment.”ed below.

The abstract is available at no charge. Amazingly, the full article seems to be available at no charge; grab it while you can! See:

Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
PubMed ID#: 27495201

Link to Abstract

Link to Full article

Happy reading!

Robin


Excerpts from
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.

KEY POINTS

* Multiple system atrophy–parkinsonian type may be differentiated from Parkinson disease by its more symmetrical appearance, atypical tremor, dystonia (antecollis), early dysarthria/dysphonia, gait and postural instability, dysautonomia, and rapid progression.

* Multiple system atrophy–cerebellar type is one of the most common causes of sporadic, adult-onset ataxia and is distinguished by parkinsonism, dysautonomia, and rapid progression.

* Pharmacologic treatment of orthostatic hypotension may include enhancing blood volume with fludrocortisone or desmopressin or adding drugs that increase vascular resistance such as midodrine, droxidopa, or pyridostigmine.

 

CBD Overview in Continuum, August 2016

An overview of diagnosing atypical parkinsonian disorders, including corticobasal degeneration (CBD), was published in the August 2016 issue of Continuum, a journal for neurologists.

The article describes CBD as follows:

CBD is an atypical parkinsonian syndrome … that presents with varied phenotypes. The classic presentation with asymmetric rigidity, dystonia, and ideomotor apraxia is now referred to as CBS… Typically, marked asymmetry of involvement is the most striking feature and helps differentiate CBD from other degenerative disorders. The most common presenting feature is asymmetric hand clumsiness followed by early bradykinesia, a frontal syndrome, tremor, and rigidity. The mean onset of disease occurs in the sixth decade, and prognosis is generally poor with a mean survival of about 7 years from diagnosis. 

There’s nothing new in the article but it provides a good summary of current knowledge about CBD. The article discusses:

* CBD symptoms, including marked asymmetry, focal rigidity,
coarse rest/action tremor, limb dystonia (followed by
contractures), alien limb phenomenon, hand, limb, gait, or
speech apraxia, myoclonus, cortical sensory loss, language
deficits, frontal/cortical dementia, bulbar impairment, and
postural instability.

* four types of CBD including:

> CBS
> frontal behavioral-spatial syndrome
> nonfluent/agrammatic primary progressive aphasia
> PSP syndrome

One case study is provided of someone with corticobasal-PSP
syndrome.

* CBD pathology

* CBD diagnostics

* CBD treatments

A very short excerpt is copied below.

The abstract is available at no charge. Amazingly, the full article seems to be available at no charge; grab it while you can! See:

Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
PubMed ID#: 27495201

Link to Abstract

Link to Full article

Happy reading!

Robin


Excerpts from
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.

KEY POINTS

* The most common presenting features for corticobasal degeneration are asymmetric hand clumsiness or apraxia followed by early bradykinesia, frontal syndrome, tremor, and rigidity.

* Ideomotor apraxia is defined by an inability to perform a skilled motor task despite having intact language, motor, and sensory function. Examples include inability to imitate gestures or mime a certain task (eg, use a screwdriver or cut with a pair of scissors). This type of apraxia can be difficult to distinguish from limb-kinetic apraxia, which is frequently seen in parkinsonisms, but is independent of modality (imitation versus miming).

 

PSP Overview in Continuum, August 2016

An overview of diagnosing atypical parkinsonian disorders, including progressive supranuclear palsy (PSP), was published in the August 2016 issue of Continuum, a journal for neurologists.

The article describes PSP as follows:

It is the most common form of atypical parkinsonism, comprising about 5% to 6% of those patients presenting with parkinsonism. The estimated prevalence and annual incidence of PSP is about 5 per 100,000 in individuals between the ages of 50 and 99 years, but is likely higher due to misdiagnosis and under-recognition. The average age of onset is typically in the sixties (average age of 63 to 66 years), and the mean survival from diagnosis is reported between 5 to 8 years. Hallmarks of the disease include prominent, early postural instability, unexplained falls, vertical supranuclear palsy, and progressive dementia.

There’s nothing new in the article but it provides a good summary of current knowledge about PSP. The article discusses:

* PSP symptoms, including falls, eye problems, facial appearance, dysarthria, dysphagia, apathy, depression, disinhibition,
emotional lability, dementia, and perseveration.

* the five types of PSP, including:

> Richardson syndrome (classic PSP)
> PSP-parkinsonism
> PSP-pure akinesia with gait freezing
> PSP-corticobasal syndrome (PSP-CBS) (or primary nonfluent
aphasia)
> PSP-behavioral variant of frontotemporal dementia (FTD)

One case study is provided of someone with PSP-parkinsonism.

* two other possible PSP variants with features that overlap with
either primary lateral sclerosis (PLS) or cerebellar ataxia.

* PSP pathology

* PSP diagnostics

* PSP treatments

A very short excerpt is copied below.

The abstract is available at no charge. Amazingly, the full article seems to be available at no charge; grab it while you can! See:

Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
PubMed ID#: 27495201

Link to Abstract

Link to Full article

Happy reading!

Robin


 

Excerpts from
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.

KEY POINTS

* Key features of progressive supranuclear palsy include early gait instability, unexplained falls, supranuclear gaze palsy, axial rigidity, dysarthric speech, and dementia.

* The rocket sign occurs in patients with progressive supranuclear palsy who have lost insight into their postural instability and “rocket” out of their chair without assistance, resulting in a high risk for falling.

* Early signs of supranuclear gaze palsy in patients with progressive supranuclear palsy include slowed vertical saccades and reduced optokinetic nystagmus. Square-wave jerks, or minute saccadic eye movements, may also be present, representing fixation instability.

* To assess for the applause sign, a clinician can demonstrate three claps to the patient and ask him or her to copy. The applause sign is present if the patient claps more than three times and continues (perseverates).

* The approach to treatment of patients with progressive supranuclear palsy should include a multidisciplinary team and involve physical, occupational, and speech therapy; psychiatry; neuropsychology; social work; and palliative care.

* Levodopa therapy should be tried in most progressive supranuclear palsy cases, with a levodopa dose of up to 1200 mg/d in divided doses as tolerated. Partial response is possible in early progressive supranuclear palsy, particularly in progressive supranuclear palsy–parkinsonism.

 

After caregiving (when a loved one dies)

Someone in our local support group who just lost a parent sent me this article from Grandparents.com.  Though the article is from Grandparents.com, I think the article applies to any caregiving relationship.

The author of the article quotes the author of a book on caregiving:  “Caregiving is a never-ending test of your strength, until one day it stops and the feeling of ‘what do I do now?’ mixed with sadness begins.”

Here are some “tips for transitioning back to your own life after a loved one you’ve been caring for, dies”:

1.  Seek Out Support – A bereavement support group…can be very helpful.

2.  Know that Guilt is Normal – Caregivers commonly feel guilt: guilt that you may have been angry at your loved one for getting sick in the first place; guilt that you didn’t do enough; and guilt that you couldn’t save them. … It is also normal to feel relief when your loved one dies, both because they are no longer suffering and because you no longer have to carry the responsibility of their care.

3.  Take Care of Yourself – When you’re caregiving, it’s all too easy to let your own needs fall to the wayside. … Make that dentist appointment that has been on your to-do list for months, get your haircut, and, while you’re at it, do something a little extravagant for yourself like getting a massage or buying a new handbag.

4.  Welcome the Extra Time – Family, friends, and faith organizations are where most people start to reconnect with the world.

5.  Reach Out to Friends

6. Enjoy Quality Time with Your Spouse

Here’s a link to the article:

www.grandparents.com/family-and-relationships/caregiving/life-after-caregiving

After Caregiving: How to Fill the Void
Tips for transitioning back to your own life after a loved one you’ve been caring for, dies.
Grandparents.com
By Anne Fritz
Un-dated

Robin

Tips for reducing dementia-related behavior (anger, etc)

This post may be of interest to those dealing with dementia-related behavior.

Friend of BSN’s, social worker Ann Blick Hamer, forwarded me this useful article today.  The topic is dementia-related behaviors, which includes anger, swearing, or aggressive outbursts.  The author suggests:

“As hard as it may be, remind yourself that the cursing or anger that might appear to be directed at you is really a symptom of the disease. Not only may it help you feel a little better when a challenging behavior occurs, but it can help safeguard the relationship between you and the person in your care.”

The author offers three tips to reducing dementia-related behaviors:

1. Get out your detective’s hat and looking glass

You don’t need to become a private investigator, but it helps to know that a great number of challenging behaviors are caused by an unmet physical or emotional need.

2. Approach & Connect – the Right Way

[Did] you know that with dementia, a person’s visual field shrinks to the size of binoculars by mid-stage? In late stage dementia it even reduces to monocular vision.

[Always] approach from the front so they can see you first.

3. Use the Environment to Your Advantage

Paying attention to settings, sounds, sights, lighting, or even smells can greatly impact your care outcomes.

Here’s a link to the article:

www.pineseducation.org/3-essential-tips-to-reducing-challenging-behaviors-in-dementia-care

3 Essential Tips to Reducing Challenging Behaviors in Dementia Care
Pines of Sarasota Education & Training Institute
02 August 2016

Note that the online version has a link to a list of the top ten “unmet needs” of those with dementia.  These “unmet needs” include physical needs (such as hunger, thirst, tired, temperature, pain) and emotional needs (such as anger, sadness, loneliness, and being scared).

Robin