Monthly Archives: August 2016

Conditions with symptoms similar to dementia

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Conditions with symptoms similar to dementia

This post may be of interest to those dealing with dementia-like symptoms and wondering if there are other causes besides neurological disorders.

An article, in Next Avenue, is about non-neurodegenerative conditions that cause dementia-like symptoms.  See:

www.nextavenue.org/dementia-symptoms/

Is It Dementia? Maybe or Maybe Not
Other conditions come with similar symptoms, experts say
Next Avenue
By Emily Gurnon, Health & Caregiving Editor
August 3, 2016

Lots of conditions present with dementia-like symptoms — depression, medication side effects, nutritional deficiencies, or metabolic problems, for example.  The key to knowing if it’s dementia or something else is testing.  Sometimes that’s neuropsychological testing, blood tests, depression testing, or neuro-imaging.  Or changing medications.

My father had progressive supranuclear palsy (confirmed through brain donation).  When we saw the first neurologist, I was very surprised when the neurologist ordered a syphilis test.  He said he wanted to be as sure as he could be that it was PSP and not something else.  Sure enough, syphilis is listed below as a condition causing dementia-like symptoms.

Robin

 

Managing difficult behaviors, hallucinations, and delusions with validation and redirection

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This post will be of interest to those caregivers coping with dementia.

The title of an article on ElderCare Online suggests it’s about managing difficult behaviors. Actually, the article offers practical tips for managing difficult behaviors, hallucinations, and delusions.

Tips are given for dealing with the situation where a family member with dementia sees a rabbit on the sofa, asserts that you are not the spouse, insists that it’s time for breakfast (even though it’s “really” time for dinner), wants to re-arrange the cupboards, or gets “underfoot” all day long.

Some of these tips are based on Naomi Feil’s validation therapy. The author explains: “First, the idea behind validation therapy is to ‘validate’ or accept the values, beliefs and ‘Reality’ of the dementia person – even if it has no basis in your reality.”

And, the author says: “The key is to ‘agree’ with what they want but by conversation and ‘steering’ get them to do something else without them realizing they are actually being redirected. This is both validation and redirection therapy. Does this always work? NO! But it has a pretty high success rate because it is so non-confrontational.”

Three examples are given about how a conversation might go when the person with dementia wants to find his car keys, starts leaving the house, or wants to use the phone.

Based on the copyright, it looks like this article was written in 2000. Someone referred to it recently on an online Alzheimer’s support group. It’s posted to ElderCare Online (ec-online.net). See:

www.ec-online.net/community/activists/difficultbehaviors.htm

Using Validation Therapy to Manage Difficult Behaviors
by Jan Allen, CSW, MSE
ElderCare Online (ec-online.net)

Robin

 

NPF overview of six atypical parkinsonism syndromes

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NPF overview of six atypical parkinsonism syndromes

Here’s the National Parkinson Foundation (parkinson.org) overview of atypical parkinsonism syndromes.  In addition to the four that are part of Brain Support Network – PSP, CBD, MSA, and DLB – NPF also includes drug-induced parkinsonism and vascular parkinsonism.

I’m not sure when this webpage was created.

Robin

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www.parkinson.org/understanding-parkinsons/diagnosis/What-are-the-different-types-of-atypical-Parkinsonism-Syndromes

What are the Different Types of Atypical Parkinsonism Syndromes?
National Parkinson Foundation

Progressive Supranuclear Palsy (PSP)

* PSP is one of the more common forms of atypical Parkinsonism.

* Symptoms of PSP usually begin after age 50 and progress more rapidly than PD.

* These symptoms include: imbalance and frequent falls early on in the disease, rigidity of the trunk, voice and swallowing changes and eye-movement problems including the ability to move eyes up and down.

* Dementia develops later in the disease. There is no specific treatment for PSP.

* Dopaminergic medication treatment is often tried and may provide some modest benefit.

* Other therapies such as speech therapy, physical therapy, and antidepressants are important for management of patients with PSP.

* No laboratory/brain scan testing exists for PSP. In rare cases, some patients may have shrinking of a particular part of the brain, called the “Pons”, which can be seen on an MRI of the brain.

Corticobasal Degeneration (CBD)

* CBD is the least common of the atypical causes of Parkinsonism

* CBD develops after age 60 and progresses more rapidly than PD.

* The initial symptoms of CBD include asymmetric bradykinesia, rigidity, limb dystonia and myoclonus (rapid jerking of a limb), postural instability, and disturbances of language.

* There is often marked and disabling apraxia of the affected limb, where it becomes difficult or impossible to perform coordinated movements of the affected limb even though there is no weakness or sensory loss. Sometimes this can be so severe that the movements of the affected limb cannot be controlled and is called ‘alien limb’ phenomenon.

* No laboratory/brain scan tests exist to confirm the diagnosis of CBD. CBD is a clinical diagnosis.

* There is no specific treatment for CBD.

* Supportive treatment such as botulinum toxin (Botox) for dystonia, antidepressants, speech and physical therapy may be helpful.

* Levodopa and dopamine agonists (common PD medications) seldom help.

Multiple System Atrophy (MSA)

* MSA is a larger term for several disorders in which one or more system in the body deteriorates.

* Included in the category of MSA are: Shy-Drager syndrome (DSD), Striatonigral degeneration (SND) and OlivoPontoCerebellar Atrophy (OPCA).

* In 2007, a new classification was proposed with two major subtypes: MSA- P (similar to SND) in which parkinsonian signs predominate and MSA-C a cerebellar dysfunction type which resembles OPCA. The term Shy-Drager Syndrome is now rarely used.

* The mean age of onset is in the mid-50s.

* Symptoms include: bradykinesia, poor balance, abnormal autonomic function, rigidity, difficulty with coordination, or a combination of these features.

* Initially, it may be difficult to distinguish MSA from Parkinson’s disease, although more rapid progression, poor response to common PD medications, and development of other symptoms in addition to Parkinsonism, may be a clue to its diagnosis.

* No laboratory/brain scan testing exists to confirm the diagnosis of MSA.

* Patients respond poorly to PD medications, and may require higher doses than the typical PD patient for mild to modest benefits.

Dementia with Lewy bodies (DLB)

* Did you know that Dementia with Lewy Bodies is second to Alzheimer’s as the most common cause of dementia in the elderly?

* DLB is a neurodegenerative disorder that results in progressive intellectual and functional deterioration.

* Patients with DLB usually have early dementia, prominent visual hallucinations, fluctuations in cognitive status over the day, and Parkinsonism.

* It is not uncommon for patients to present with cognitive problems particularly language problems, known as aphasia.

* Other cognitive changes in patients with DLB include deficits in attention, executive function (problem solving, planning) and visuospacial function (the ability to produce and recognize figures, drawing or matching figures).

* There are no known therapies to stop or slow the progression of DLB.

Drug-induced Parkinsonism

* Side effects of some drugs, especially those that affect dopamine levels in the brain such as anti-psychotic or anti-depressant medication, can actually cause symptoms of Parkinsonism.

* Although tremor and postural instability may be less severe, this condition may be difficult to distinguish from Parkinson’s disease.

* Medications that can cause the development of Parkinsonism include:
– Antipsychotics
– Metaclopramide
– Reserpine
– Tetrabenazine
– Some calcium channel blockers
– Stimulants such as amphetamines and cocaine
– Usually after stopping those medications Parkinsonism gradually disappears over weeks to months

Vascular Parkinsonism

* Multiple small strokes can cause Parkinsonism.

* Patients with this disorder are more likely to present with gait difficulty than tremor, and are more likely to have symptoms that are worse in the lower part of the body.

* Some will also report the abrupt onset of symptoms or give a history of step-wise deterioration (symptoms get worse, then plateau for a period).

* Dopamine is tried to improve patients’ mobility although the results are often not as successful.

* Vascular Parkinsonism is static (or very slowly progressive) when compared to other neurodegenerative disorders.

Page reviewed by Dr. Joash Lazarus, NPF Movement Disorders Fellow, Department of Neurology at Emory University School of Medicine.

Tau drug failed in Alzheimer’s trial (despite what newspapers say)

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Tau drug failed in Alzheimer’s trial (despite what newspapers say)

This post may be of interest to three groups:
* those of you following experimental drug therapies for PSP and CBD, both tauopathies (disorders of the protein tau);
* those of you fascinated (frustrated?) by the disconnect between research results and the popular press; and
* those of you tracking low diagnostic accuracy for Alzheimer’s Disease.

Some of you might remember there was an experimental drug called Rember, based on methylene blue.  The latest version of Rember is called LMTM, TauRx0236, or LMT-X.  Clinical trials of LMT-X in Alzheimer’s recently ended.  LMTM is made by TauRx Therapeutics.

For background, see our May 2015 blog post with an update on LMTM:

www.brainsupportnetwork.org/methylene-blue-update/

According to the Alzforum, at a late July 2016 Alzheimer’s conference in Toronto, a researcher “reported that LMTM failed to slow cognitive or functional decline in people with mild to moderate Alzheimer’s disease. On the main primary results slide, disease progression curves for both doses of drug and the placebo were practically identical.”  And, yet, later in the presentation, the researcher continued to claim that there was a strong benefit on cognition from the drug in a subgroup.

Alzforum said:

Many researchers in the room, and later in the hallways, were dismayed not only at the way the data were being parsed, but also at media stories about the drug’s ostensible “success.” Outlets such as The Times, New Scientist, and the Huffington Post variously announced the arrival of the first drug to halt Alzheimer’s, or that the drug slowed disease by 80 percent, even while leading Alzheimer’s researchers at AAIC were challenging the company’s leader, Claude Wischik, for overstating the trial’s result.

Paul Aisen, with USC, suggested that not very many people in the LMTM clinical trial actually had Alzheimer’s!  Alzforum said:

Aisen further pointed out that the fraction of people in the trial who carried an ApoE4 allele—48, 42, and 53 percent among the placebo, low-, and high-dose LMTM, respectively—seemed low. “In ADCS trials, ApoE4 prevalence among people with mild to moderate AD is typically 60-70 percent,” he said. “Since there was no biomarker to support the diagnosis of Alzheimer’s disease, the low ApoE4 numbers make one wonder about the accuracy of the diagnosis,” Aisen said. “If you rely solely on clinical diagnosis, then you had better have highly experienced clinicians,” he added. In ADNI, the bapineuzumab and first set of solanezumab Phase 3 trials, about a quarter of participants, especially the ApoE4 non-carriers, were later found to be amyloid-negative.

Here are all the details, if you’d like more:

www.alzforum.org/news/conference-coverage/first-phase-3-trial-tau-drug-lmtm-did-not-work-period

In First Phase 3 Trial, the Tau Drug LMTM Did Not Work. Period.
Alzforum
29 July 2016

Robin

Baylor’s Overview of Atypical Parkinsonism

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Baylor’s Overview of Atypical Parkinsonism

Joseph Jankovic, MD, is a movement disorder specialist at Baylor College of Medicine in Houston.  This webpage from 2011 is an overview of atypical parkinsonism.  There’s a quick overview of Parkinson’s Disease (PD) and then Dr. Jankovic explains how the atypical parkinsonism forms differ from PD.  Most of the text is about progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).  There is some info about dementia with Lewy bodies (DLB), cortico-basal ganglionic degeneration (CBD), and vascular parkinsonism.  Copied below is most of the text from the webpage and a link to it.

You might also want to check out Dr. Jankovic’s table of differential diagnosis.  He indicates which symptoms or conditions are present or not present in various atypical parkinsonism forms.  Copied below is a link to the table plus my comments about the table.

Thanks to Sue Buff, in the PD community, for pointing out this webpage to me.

Robin

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www.bcm.edu/healthcare/care-centers/parkinsons/conditions/atypical-parkinsonism

Atypical Parkinsonism
Baylor College of Medicine
by Joseph Jankovic, MD
2011

Parkinson’s disease is a progressive brain disorder which usually produces some combination of the following symptoms:
* Tremor at rest
* Slowed movement
* Stiffness or rigidity of muscles
* Unsteadiness when standing or walking
* Freezing” or sudden loss of ability to move (as if the feet are “glued” to the floor).

Parkinson’s disease (PD) results from dying off (degeneration) of certain nerve cells found in a deep part of the brain (brainstem) called the substantia nigra. The reason for the cell death is unknown. These cells produce a neurotransmitter chemical called dopamine. It is the depletion of dopamine in the brain that results in symptoms of PD. Initially, Parkinson’s symptoms improve with dopamine replacement in the form of levodopa (Sinemet). Although the nerve cells in the substantia nigra, which make dopamine, are dying, the nerve cells in the basal ganglia (striatum), which are normally stimulated by dopamine, have well preserved dopamine receptors and are therefore still responding to dopamine. The cornerstone of medical treatment of PD is administration of levodopa, which is taken up and converted into dopamine by the brain.

“Atypical” Parkinsonism and How It Differs From Parkinson’s Disease

Parkinsonism refers to a set of symptoms typically seen in Parkinson’s disease, but caused by other disorders. Atypical parkinsonism includes a variety of neurological disorders in which patients have some clinical features of PD, but the symptoms are caused not only by cell loss in the substantia nigra (the brain area most affected in classic PD), but also by additional degeneration of cells in the parts of the nervous system that normally contain dopamine receptors (striatum). In other words, the patients look like they have PD, but the cause of their symptoms is different from that of classic PD. Patients with atypical parkinsonism have symptoms similar to PD, including resting tremors, slowed movement, stiffness, gait difficulty and postural instability, but in addition have symptoms and signs that are not typically present in PD, hence the term “Parkinsonism plus syndrome.”

The additional problems may include inability to look up and down (vertical gaze palsy) and early postural instability, such as seen in progressive supranuclear palsy (PSP), the most common form of atypical parkinsonism. Patients with PSP often have the “procerus sign” which is a particular “worried” facial expression.

The second most common form of atypical parkinsonism is multiple system atrophy (MSA), previously also referred to as Shy-Drager syndrome or olivopontocerebellar atrophy. Patients with MSA are typically distinguished from those with PD by the presence of autonomic features such as unstable blood pressure which falls drastically upon standing (orthostatic hypotension), early disturbance of sexual, bladder, and bowel dysfunction, reddish-bluish discoloration of skin (e.g. the “cold hand” sign), and marked sleep disturbance (e.g. acting out of dreams and sleep apnea). Other typical features of MSA include forward head tilt (anterocollis) or a body tilt when sitting (Pisa sign), loss of coordination, and rapidly progressive course with inability to ambulate usually within the first 3-5 years after onset. MSA is divided into MSA-C (cerebellar variant) which has more symptoms of ataxia (incoordination) and MSA-P (parkinsonian variant) which has more parkinsonian symptoms but is unresponsive to the usual therapy for Parkinson’s disease (levodopa).

Another common cause of atypical parkinsonism is “vascular parkinsonism” caused by multiple, usually very minute, strokes. These patients usually have more symptoms in the lower extremities (lower body parkinsonism) with walking difficulties, balance problems and falls.

Other forms of atypical parkinsonism are Dementia with Lewy bodies (DLB) and cortico-basal ganglionic degeneration (CBD). Patients with DLB have, in addition to the parkinsonian features, early dementia (preceding or co-occurring with the parkinsonian symptoms), visual hallucinations (seeing people, animals or objects that are not real), their symptoms fluctuating, with “good days” and “bad days.” Patients with CBD usually present with asymmetric stiffness, apraxia (inability to carry out learned purposeful movements), alien limb (the hand or the leg seem to have “a mind of their own”), and limb dystonia (abnormal sustained muscle contractions causing abnormal postures and twisting). Poor or no response to levodopa is common feature to all forms of atypical parkinsonism.

In contrast to typical PD, in which dopamine receptors are spared, patients with atypical parkinsonian disorders have lost their dopamine receptors and therefore they do not respond to levodopa as well as those with typical PD. This can be demonstrated by special imaging such as positron emission tomography (PET) and dopamine transporter imaging (DAT-SPECT). MRI may be also helpful in differentiating PD from atypical parkinsonism.

Causes

There are probably many causes of atypical parkinsonism, but no one specific cause has been identified. There are many articles (see references) that describe some of the research into the causes of these disorders. Although it is not yet known what causes atypical parkinsonism, only one member of a family is usually affected and, therefore, these disorders are thought to be sporadic and not inherited. This is in contrast to typical PD where genetic factors seem to be quite important. The various atypical parkinsonian syndromes are classified according to the patterns of damage they produce in the nervous system, the constellation of clinical symptoms they cause, and their natural course.

Support Groups for Patients and Their Families

Patients affected with atypical parkinsonism and their families should consider joining national or local PD support groups. Membership in these organizations facilitates exchange of information and members can obtains “tips” that may be useful in coping with the physical and mental disabilities associated with these disorders. Also, many local support groups offer exercise therapy which may be helpful to some patients. With the emotional support of family members and with expert medical management guided by a knowledgeable and compassionate physician, many patients with atypical parkinsonism can lead enjoyable and productive lives.
www.bcm.edu/healthcare/care-centers/parkinsons/conditions/atypical-parkinsonism/parkinsonism-plus-syndromes

Table 1 – Parkinsonism Plus Syndromes: Differential Diagnosis

Robin’s note:  This table identifies which symptoms or conditions are present or not present in atypical parkinsonism disorders.  The symptoms or conditions include bradykinesia, rigidity, gait disturbance, tremor, ataxia, dysautonomia, dementia, dysarthria/dysphagia, dystonia, eyelid apraxia, limb apraxia, motor neuron disease, myoclonus, neuropathy, oculomotor deficit, sleep impairment, asymmetric findings, levodopa response, levodopa dyskinesia, family history, putaminal T2 hypo-intensity, and Lewy bodies.   Disorders include PD, PSP, MSA-P, MSA-C, CBD, DLB, and PDACG (parkinsonism-dementia-ALS complex of Guam).

Robin’s evaluation:  I disagree with some of this chart.  For example, tremor is present in PSP-parkinsonism.  And tremor can be present in DLB.