Monthly Archives: January 2012

Delayed gastric emptying in MSA

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In this Japanese study, “gastric emptying” was studied in 25 patients with multiple system atrophy (MSA), 20 patients with ataxia, and 20 healthy volunteers.

The authors concluded:

“These results suggested that gastric emptying was significantly delayed in patients with MSA, and the delay already appeared in the early stage of the disease. Delayed gastric emptying is one of the autonomic failures and may be a clinical marker of MSA.”

Since these autonomic symptoms can also occur in Parkinson’s Disease and Lewy Body Dementia, I think a good follow-on study would be to see if delayed gastric emptying occurs any earlier in MSA than in other disorders.

The abstract to the article is copied below

Robin

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Journal of Neurology. 2012 Jan 5. [Epub ahead of print]

Is there delayed gastric emptying in patients with multiple system atrophy? An analysis using the (13)C-acetate breath test.

Tanaka Y, Kato T, Nishida H, Yamada M, Koumura A, Sakurai T, Hayashi Y, Kimura A, Hozumi I, Araki H, Murase M, Nagaki M, Moriwaki H, Inuzuka T.
Department of Neurology and Geriatrics, Graduate School of Medicine, Gifu University, Gifu City, Japan.

Abstract
Autonomic failure is one of the criteria according to the second consensus statement for the diagnosis of multiple system atrophy (MSA). Gastrointestinal symptoms are frequent complaints in patients with MSA and may be associated with reduced gastrointestinal motility due to autonomic nervous system dysfunction. However, there are few reports on gastric emptying in patients with MSA. We investigated gastric emptying in 25 patients with MSA, 20 patients with sporadic adult-onset ataxia of unknown etiology (SAOA), and 20 healthy volunteers using the (13)C-acetate breath test. Gastric emptying function is estimated by this test as the half-emptying time (HET) and peak time of the (13)C-%-dose-excess curve (T (max)), with expirations collected for 4 h after a test meal and determination of (13)CO(2) content using an infrared (IR) spectrophotometer. The HET and T (max) of gastric emptying were significantly delayed in patients with MSA as compared to those in SAOA and controls (p < 0.01). The HET and T (max) were not significantly different between SAOA and controls. No correlation existed between the HET or T (max) and the duration or severity of the disease in MSA patients. These results suggested that gastric emptying was significantly delayed in patients with MSA, and the delay already appeared in the early stage of the disease. Delayed gastric emptying is one of the autonomic failures and may be a clinical marker of MSA.

PubMed ID#: 22218651   (see pubmed.gov for this abstract only)

 

Explaining “high,” “intermediate,” and “low” chances of DLB

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One of Brain Support Network’s mission is to help families with brain donation arrangements.  We’ve seen lots of neuropathology reports come back with various descriptions of “Lewy body disease” and the cover letters indicate the person had “Lewy body dementia.”  Within the “comments” section of the reports terms such as “low,” “intermediate,” and “high” are used.  This post attempts to explain that terminology.

Most of the neuropathology reports I read are from the Mayo Clinic in Jacksonville.  The neuropathologist there, Dr. Dennis Dickson, is one of the top in the world.  He co-authored the diagnostic criteria for Dementia with Lewy Bodies.  That criteria can be found in this important paper:

Neurology. 2005 Dec 27;65(12):1863-72.
Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium.
McKeith IG, many others, and Consortium on DLB.

Interestingly, neuropathologists don’t say that someone had Lewy Body Dementia (or Dementia with Lewy Bodies) as that’s a clinical diagnosis, not a neuropathologic one.  They may say something like “based on the clinical picture, the level of Lewy bodies, and the level of Alzheimer’s pathology, the LIKELIHOOD this donor had Dementia with Lewy Bodies is high/intermediate/low.”

There’s a chart in the McKeith paper that neuropathologists rely on in making this statement. The table is titled “Assessment of the likelihood that the pathologic findings are associated with a DLB clinical syndrome.”  On one axis is “Lewy body type pathology,” where the choices are Brainstem, Limbic (transitional), and Diffuse neocortical.  On the other axis is “Alzheimer’s type pathology,” where the choices are NIA-Reagan Low (Braak stage 0-2), NIA-Reagan Intermediate (Braak stage 3-4), and NIA-Reagan High (Braak stage 5-6).

If the Lewy body pathology is Limbic or Diffuse and the amount of Alzheimer’s pathology in the brain is low, the “likelihood that the pathologic findings are associated with a DLB clinical syndrome” are “high.” Conversely, if the LB pathology is Brainstem or Limbic and the amount of Alzheimer’s pathology in the brain is high, the “likelihood that the pathologic findings are associated with a DLB clinical syndrome” are “low.”

Here’s a related post about the severity of Lewy bodies in the brain that explains brainstem, limbic (transitional), and diffuse.

Typically, Lewy Body pathology and Alzheimer’s pathology co-occur in the brain of someone with Lewy body dementia.  In my layperson’s mind, the low-intermediate-high language is basically a gauge to say which pathology is more important in terms of overall pathologies found in the brain — is it Lewy Body pathology or Alzheimer’s pathology?

Let me know if you have questions!

Robin

Severity of Lewy body pathology in the brain

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One of Brain Support Network’s mission is to help families with brain donation arrangements.  We’ve seen lots of neuropathology reports come back with various descriptions of “Lewy body disease.”

A Lewy body is an abnormal clump of alpha-synuclein protein.  These clumps were described by Dr. Frederic Lewy, a neurologist.

Lewy bodies are present in the brains of those with Parkinson’s Disease and Lewy Body Dementia.  It can be somewhat of a fine line to determine if someone has Parkinson’s Disease or Lewy Body Dementia because both disorders include Lewy bodies in the brain.  Neuropathologists are guided by WHERE in the brain the Lewy bodies are located in order to make a diagnosis.

Most of the neuropathology reports I read are from the Mayo Clinic in Jacksonville.  The neuropathologist there, Dr. Dennis Dickson, considers four levels of Lewy Body Disease severity:

* incidental
* brainstem
* transitional
* diffuse

Other brain banks occasionally call “diffuse Lewy body disease” something like “neocortical Lewy body disease.”

“Incidental” means that there are a small number of Lewy bodies in the brain and these are largely irrelevant.  There are no clinical symptoms.  Something like 10% of neurologically normal people are found to have had an incidental level of Lewy bodies in the brain.

“Brainstem” means that the Lewy bodies are in the brainstem.  A diagnosis of “brainstem Lewy body disease” is basically Parkinson’s Disease.

Let’s skip to “diffuse.”  A diagnosis of “diffuse Lewy body disease” means that the Lewy bodies are diffuse throughout the brain — even in the cortex of the brain.  Generally, it’s appropriate to say that someone with a DLBD diagnosis had Lewy Body Dementia, which means either Parkinson’s Disease Dementia or Dementia with Lewy Bodies.

In between “brainstem” and “diffuse” is transitional. This is an intermediate stage of Lewy body disease where the Lewy bodies are outside of the brainstem but not diffuse throughout the brain.  Some brain banks use the term “limbic” rather than “transitional” to refer to the fact that the Lewy bodies are in the limbic system of the brain.  Often those with TLBD had Lewy Body Dementia as well. Perhaps dementia symptoms were not as severe as someone with DLBD.

Interestingly, neuropathologists don’t say that someone had Lewy Body Dementia. They may say something like “based on the clinical picture, the level of Lewy bodies, and the level of Alzheimer’s pathology, the LIKELIHOOD this donor had Dementia with Lewy Bodies is high/intermediate/low.”  This means that Lewy Body Dementia is a clinical diagnosis — not a pathological one.

Here’s a related post on the topic of “likelihood” of having dementia with Lewy bodies:

Let me know if you have questions!

Robin

 

Article on caregiver loss and grief

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I picked up a copy of Preserving Your Memory magazine today at the doctor’s office.  An article on dealing with loss was worth reading.  Though it’s about dealing with losses when your loved one has dementia, I think the article applies to dealing with losses when your loved one has any sort of medical condition or when you are dealing with any sort of medical condition.

Here are some of the key points made in the article:

* We “now rely more on what we call the ‘dual process model’—where a grieving person tries to live life in the new reality while at the same time coping with a sense of loss…”

* “Grief is not about death, but about loss.”

* “If grief is the conflicting feelings caused by the end of or change in a familiar pattern of behavior, then any changes in relationships with people, places, or events can cause the feelings we call grief.”

* The ambiguity of caring for a living, breathing person while grieving the loss of that person’s former self can be overwhelming and confusing.

* Anticipatory grief. In this phase, caregivers grieve in the face of the disease and brace for what’s to come.

The article offers some “tips for healthy grieving.”

Here’s a link to the article, which starts on page 10:

www.alzinfo.org/wp-content/uploads/2011/12/PYM_Winter12.pdf 

Loss: The relationship between dementia and grief is a complicated one.
Preserving Your Memory (Magazine)
Winter 2012

Robin

Cerebral amyloid angiopathy – overview

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One of Brain Support Network’s mission is to help families with brain donation arrangements.  We’ve seen a few neuropathology reports come back with the term “cerebral amyloid angiopathy” (CAA). This is often seen on reports when the brain donor was diagnosed during life with Lewy body dementia, but no Lewy bodies can be found. We often see it in those with have a history of strokes.

“Angiopathy” means a disease of the blood vessels. The term commonly refers to conditions where small blood vessels are damaged and burst open. 

“Cerebral” refers to the brain.

And “amyloid” refer to the protein amyloid. This is one of the proteins found in Alzheimer’s Disease, by the way. But CAA is not Alzheimer’s.

In CAA, amyloid proteins have accumulated in the walls of blood vessels in the brain. The person with CAA is at high risk of suffering a stroke. And, for unknown reasons, the person with CAA is at high risk of developing dementia.

Probable CAA can be diagnosed during life on the basis of imaging. The diagnosis can be confirmed through a biopsy (while the person is alive) or an autopsy (when the person has died). In the case of a biopsy, a vascular surgeon can take a sample of a blood vessel in the brain, and this sample is studied for signs of amyloid.

Sometimes, CAA is inherited. You would have some sense if it runs in your family by knowing if other relatives have similar symptoms.