Category Archives: PSP

Argyrophilic grain disease – Brazilian Aging Brain Study Group description

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This interesting review of argyrophilic grain disease (AGD) was recently published by the Brazilian Aging Brain Study Group.  (One of the leaders of the Brazilian study is now at UCSF.)  The full article is available at no charge here:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5618985/

The abstract notes:

Argyrophilic grain disease (AGD) is an under-recognized, distinct, highly frequent sporadic tauopathy, with a prevalence reaching 31.3% in centenarians. The most common AGD manifestation is slowly progressive amnestic mild cognitive impairment, accompanied by a high prevalence of neuropsychiatric symptoms. … AGD is frequently seen together with Alzheimer’s disease-type pathology or in association with other neurodegenerative diseases. 

Generally, clinical symptoms of AGD are memory impairment (like Alzheimer’s) and neuropsychiatric symptoms.  The mild cognitive impairment progresses very slowly, in most cases.  Common neuropsychiatric symptoms include personality changes (rudeness, stubbornnes, egocentric behavior, disinhibition, obsession, apathy) and depression.  The neuropsychiatric symptoms may pre-date the memory impairment.  Given these symptoms, common clinical diagnoses are Alzheimer’s, frontotemporal dementia, and progressive supranuclear palsy.

The article notes:

A study comparing Parkinson’s disease patients with and without AGD, and studies showing a high prevalence of AGD in individuals with late-onset schizophrenia and delusional disorders, corroborate the association of AGD with psychiatric symptoms.  Asaoka et al. reported an AGD case exhibiting delusions and hallucinations at clinical presentation.  A few reports implicate AGD as the underlying condition in patients featuring clinical frontotemporal dementia. In such cases, AGD pathology is widespread in the brain, as opposed to the majority of AGD cases where involvement is restricted to limbic structures.

There are no clinical diagnostic criteria for diagnosing someone with AGD during life.  The article addresses this by noting, “At any rate, AGD is virtually unknown to the clinical community because most cases do not present any known distinctive clinical symptoms.”  Confusingly for clinicians, AGD can be present in those with cognitive impairment or neuropsychiatric symptoms.  It is a postmortem diagnosis.

Pathologically, we at Brain Support Network see AGD co-occurring with other neurodegenerative diseases, especially other tauopathies such as progressive supranuclear palsy (PSP).

AGD commonly occurs in older people. But AGD can be seen in younger people.  The article states,  “In the series of the Brain Bank of the Brazilian Aging Brain Study Group, 6.7% of individuals aged between 50 and 60 had AGD.”

There is some thought that the presence of AGD may ward of the development of other pathologies, such as Alzheimer’s Disease.  The authors note:

The reasons why AGD does not develop with prominent clinical features have yet to be clarified. Recent evidence of lack of tau acetylation in AGD suggest a protective role of this entity against spread of other neurodegenerative conditions, particularly Alzheimer’s disease and is a hypothesis currently being investigated.

This review article adds greatly to our understanding of AGD.

Robin

Pain more severe and common in PD and MSA compared to PSP

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In this small study, these patient populations were compared — 21 with multiple system atrophy (MSA), 16 with progressive supranuclear palsy (PSP), and 65 with Parkinson’s Disease (PD).  Europeans researchers showed that pain was “significantly more common and more severe in PD and MSA compared to PSP.”

The full abstract is copied below.

Robin

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Brain & Behavior. 2015 May;5(5):e00320. Epub 2015 Mar 25.

Pain in multiple system atrophy and progressive supranuclear palsy compared to Parkinson’s disease.

Kass-Iliyya L, Kobylecki C, McDonald KR, Gerhard A, Silverdale MA.

Abstract
BACKGROUND:
Pain is a common nonmotor symptom in Parkinson’s disease (PD). The pathophysiology of pain in PD is not well understood. Pain characteristics have rarely been studied in atypical parkinsonian disorders such as Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP).

AIM OF THE STUDY:
We aimed to evaluate pain intensity, location, and associated symptoms in atypical parkinsonian disorders compared to PD.

METHODS:
Twenty-one patients with MSA, 16 patients with PSP, and 65 patients with PD were screened for pain using question 1.9 of the MDS-UPDRS. Pain intensity was quantified using the short form McGill Pain Questionnaire (SFMPQ). Pain locations were documented. Motor disability was measured using UPDRS-III. Affective symptoms were assessed using the Hospital Anxiety and Depression Scale (HADS).

RESULTS:
Pain was significantly more common and more severe in PD and MSA compared to PSP (P < 0.01). Pain locations were similar with limb pain being the most common followed by neck and back pain. Pain intensity correlated with HADS scores but not motor severity.

CONCLUSIONS:
Pain is more common and more intense in PD and MSA than PSP. Differences in distribution of neurodegenerative pathologies may underlie these differential pain profiles.

PMID: 25874161

Mayo Clinic study of thousands of brains reveals tau as driver of AD

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This post is about Alzheimer’s though it’s likely of interest to those dealing with PSP and CBD, and of some interest to those dealing with LBD.  Both PSP and CBD are tauopathies, or disorders of the protein tau.  LBD is not a tauopathy but it commonly co-occurs with the largest tauopathy – Alzheimer’s Disease.

This Mayo Clinic press release from last Tuesday is focused on Alzheimer’s Disease (AD).  AD is a disorder of two proteins – tau and amyloid.  There has long been a dispute in the researcher community as to which tau or amyloid is the “culprit” in AD.  (The two camps are called the tauists and the baptists.  bap = beta amyloid protein.)

In this major study of 3600 donated brains at the Mayo Clinic in Jacksonville, Mayo researchers conclude that tau is the primary culprit.  And efforts should focus on halting tau.

This is great news for the PSP and CBD communities because if researchers can solve the problem of tau building up, then they may be able to treat PSP and CBD.

At the link, there’s a one-minute video with Dr. Melissa Murray explaining the findings.  I usually find such videos worthwhile but this one was too short and too much of a summary to be of interest to me.  Your opinion may be different!

newsnetwork.mayoclinic.org/discussion/mayo-clinic-study-of-thousands-of-brains-reveals-tau-as-driver-of-alzheimers-disease/

MAYO CLINIC PRESS RELEASE
24-MAR-2015
Mayo Clinic study of thousands of brains reveals tau as driver of Alzheimer’s disease

Robin

 

“Her mother seemed to have classic dementia. Or did she?”

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This is a good article about the symptoms of normal pressure hydrocephalus (NPH).  Often, NPH is misdiagnosed as progressive supranuclear palsy or Lewy body dementia!

In this case, the sufferer’s cognitive symptoms improved immediately after brain suergery.

Here’s a link to the full article:

https://www.washingtonpost.com/national/health-science/her-mother-seemed-to-have-classic-dementia-or-did-she/2015/03/02/89b1ec1e-a736-11e4-a06b-9df2002b86a0_story.html

Her mother seemed to have classic dementia. Or did she?
By Roni Caryn Rabin
Washington Post
March 2, 2015

Guide on how to diagnose PSP, etc. (Neurologic Clinics, Feb. 2015)

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I ran across an interesting medical journal article today that provides a guide for neurologists on how to diagnose three atypical parkinsonism disorders — PSP (progressive supranuclear palsy), CBD (corticobasal degeneration), and MSA (multiple system atrophy.

The full article is available online for a fee.  See:

www.neurologic.theclinics.com/article/S0733-8619(14)00080-2/fulltext

“Atypical Parkinsonism: Diagnosis and Treatment”
Maria Stamelou, MD, PhD and Kailash P. Bhatia, FRCP
Neurologic Clinics 33 (2015) 39-56

Here’s a short overview of PSP from the article:

“PSP is a neurodegenerative disease characterized by symmetric parkinsonism, supranuclear palsy of vertical gaze, early postural instability with falls backwards, subcortical dementia, dysarthria, and dysphagia.  The prevalence of PSP is approximately 5 per 100,000, and men and women are equally affected. Average age at onset is 63, and mean time from symptom onset to death is 7 years. No pathologic proven cases have begun before the age of 40.”

The authors ask the question – why is it so hard to diagnose these atypical parkinsonian disorders accurately during life?  Their answers:

“[The] early differential diagnosis is complicated by patients with pathologically proven PSP, CBD, or MSA that may present clinically with phenotypes other than the classic ones.  Conversely, patients with the classic AP [atypical parkinsonism] phenotypes may turn out to have other pathologic abnormalities.” 

So, basically, it’s really hard to tell all of these disorders apart.

The authors ask:  “if it’s not PSP, CBD, or MSA, what could it be?”  One disorder it could be is Parkinson’s Disease (PD).  What else could it be if it’s not PD?  Very commonly, it’s one of the other atypical parkinsonian disorders — PSP, CBD, MSA, and DLB (Dementia with Lewy Bodies).

What else could it be if it’s not one of the other atypical parkinsonism disorders?  For PSP and CBD, very commonly it’s FTD (frontotemporal dementia).

I recommend shelling out some money on this article.

Robin