Monthly Archives: October 2013

MSA Diagnosis and Treatment (Neurologist Study-Guide, Oct 2013)

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“Continuum” is a journal published by the American Academy of Neurology.  Its subtitle is “Lifelong Learning in Neurology.”  It’s a study-guide for neurologists.  Every year or maybe every other year they have an issue devoted to movement disorders.  Within that issue, there’s always an article on three parkinsonian syndromes — PSP, MSA, and CBD.  This year’s article has two authors — Dr. Irene Litvan, who was the keynote speaker at our October 2012 atypical parkinsonism symposium, and Dr. David Williams, a rising star in the atypical parkinsonism community.  (Unfortunately for us, Dr. Williams lives in Australia.)

The study-guide is available at no charge online:

www.ncbi.nlm.nih.gov/pmc/articles/PMC4234134/

Continuum (Minneap Minn). 2013 Oct; 19(5 Movement Disorders): 1189–1212.
Parkinsonian Syndromes
David R. Williams, MD, MBBS, PhD, FRACP and Irene Litvan, MD, FAAN

The MSA-related section of the study-guide has four parts:

#1 – an overview of how MSA is diagnosed.  This section may include too much medical lingo for many in our group.

#2 – a very short section on “natural history.”  This is easy-to-read.

#3 – treatment paradigm.  I suggest you concentrate on this section.  Though it has quite a bit of medical jargon, I think it’s understandable.  It refers to a “multidisciplinary team approach.”

#4 – a case report of someone with MSA-P.  A video is available of someone with rigidity.

Robin

5 Types of PSP and Treatment (Neurologist Study-Guide, Oct 2013)

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“Continuum” is a journal published by the American Academy of Neurology.  Its subtitle is “Lifelong Learning in Neurology.”  It’s a study-guide for neurologists.  Every year or maybe every other year they have an issue devoted to movement disorders.  Within that issue, there’s always an article on three parkinsonian syndromes — PSP, MSA, and CBD.  This year’s article has two authors — Dr. Irene Litvan, who was the keynote speaker at our October 2012 atypical parkinsonism symposium, and Dr. David Williams, a rising star in the atypical parkinsonism community.  (Unfortunately for us, Dr. Williams lives in Australia.)

The study-guide is available at no charge online:

www.ncbi.nlm.nih.gov/pmc/articles/PMC4234134/

Continuum (Minneap Minn). 2013 Oct; 19(5 Movement Disorders): 1189–1212.
Parkinsonian Syndromes
David R. Williams, MD, MBBS, PhD, FRACP and Irene Litvan, MD, FAAN

The PSP-related section of the study-guide has three parts:

#1 – an explanation of the five clinical subtypes of PSP, which are:

  • Richardson syndrome
  • Progressive supranuclear palsy-parkinsonism
  • Progressive supranuclear palsy-pure akinesia with gait freezing
  • Progressive supranuclear palsy-corticobasal syndrome
  • Progressive supranuclear palsy-frontotemporal dementia

Videos are available of someone with Richardson syndrome (Dr. Litvan is heard/seen) and of someone with PSP-parkinsonism (Dr. Williams is heard/seen).  Other than the videos, I think most of the explanation of the five subtypes and both of the case write-ups will include too much medical lingo for many in our group.

#2 – a very short section on “natural history.”  This is easy-to-read.

#3 – treatment paradigm.  I suggest you concentrate on this section.  Though it has quite a bit of medical jargon, I think it’s understandable.  Interesting to me is the suggestion to use solifenacin (Vesicare) for bladder issues.  I first heard about this prescription medication years ago from a local PSP support group member.  A suggestion is also made about using botox for various symptoms.  We didn’t try botox of neurogenic bladder, but we did use it to good effect for treatment of dystonic postures and severe rigidity.

Robin

 

Different Types of Alzheimer’s Disease (based on Mayo Brain Bank)

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A couple of years ago (September 2011), the Mayo Clinic published interesting research on the different types of Alzheimer’s Disease, based on neuropathologic findings.  Of course there’s the “typical” type of AD that most of us are familiar with.  There are two other types that are new to me —

  • hippocampal sparing (HpSp)
  • limbic predominant (LP)

One of Brain Support Network’s missions is to assist families around the United States with brain donation.  We have helped over 150 families donate a loved one’s brain.  We occasionally see these two non-typical AD diagnoses on neuropathology reports.  We look forward to more publications describing these two non-typical types of Alzheimer’s.

In this study, 889 cases of AD at the Mayo Clinic brain bank were examined.  Just over 11% of the cases were hippocampal sharing, and 14% of the cases were limbic predominant.  So these two non-typical AD types represent 25% of all AD cases.

Because the “hippocampal sparing (HpSp)” type had less hippocampal atrophy than typical AD, this meant that memories were relatively more preserved than with typical AD.  “Patients with hippocampal sparing AD were younger at death (mean 72 years) and a higher proportion of them were men (63%). … HpSp had a shorter disease duration. … A non-AD clinical diagnosis was more frequent (30%) in HpSp than LP and typical AD. Examples of non-AD clinical diagnoses included behavioral variant of frontotemporal dementia (12%) and other focal cortical syndromes (13%), such as progressive nonfluent aphasia, semantic dementia, posterior cortical syndrome, and corticobasal syndrome, as well as parkinsonian disorders (4%), such as progressive supranuclear palsy and Parkinson’s disease dementia.”

“[Those] with limbic-predominant AD were older (mean 86 years)and a higher proportion of them were women (69%).”  Disease duration was the same as with typical AD.  “LP most often had an antemortem clinical diagnosis of AD (94%). Of the 6% of LP who were given a non-AD clinical diagnosis, only a few were considered to have behavioral variant of frontotemporal dementia (n=2) or focal cortical syndromes (n=1), but they were comparable on parkinsonian diagnoses (n=4).”

“Neither initial, nor final MMSE scores assessed within three years of onset and death, respectively, were different among the AD types. When MMSE scores were evaluated with respect to disease duration, apparent differences were observed for HpSp. Figure 4 shows rapid progression of deterioration on MMSE in HpSp, with many patients progressing from normal to impaired values in a few years, declining at a rate of 5 points per year on the MMSE. On average, HpSp cases showed a steeper slope than both LP and typical AD.”

As for genetics, the researchers found:

“Microtubule-associated protein tau (MAPT) H1H1 genotype was more common in limbic-predominant AD (70%) than in hippocampal sparing AD (46%, but did not differ significantly between limbic-predominant and typical AD (59%). Apolipoprotein E (APOE) ɛ4 allele status differed between AD subtypes only when data were stratified by age at onset.”

The researchers argue that these distinct AD clinicopathological subtypes “should be considered when designing clinical, genetic, biomarker, and treatment studies in patients with AD.”

The full article is available online at no charge:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3175379/

Robin