Online Resources and Suggested Books for Advance Planning and End of Life

Hope Hospice has a terrific resource list on advance planning and end of life issues.  It’s divided into three sections:
* online resources for patients and families
* online resources for healthcare professional
* suggested books

I’ve copied it below.

Robin

——————–

www.hopehospice.com/resources.html

Resources for Patients and their Families

Caring Connections
http://www.caringinfo.org
(provides Advance Health Directive forms)

Coda Alliance
http://www.codaalliance.org

California Coalition for Compassionate Care
[Editor’s Note:  this is now at coalitionccc.org]

Completing A Life
http://www.completingalife.msu.edu/audioon/welcome.html

Family Caregiver Alliance
http://www.caregiver.org

‘The Five Wishes’
http://www.agingwithdignity.org/five-wishes.php

Hospice Foundation of America
http://www.hospicefoundation.org

Legacies
http://www.legacies.ca

New Lifestyles
http://www.NewLifeStyles.com
Free books that list local skilled nursing facilities, Board and Care homes and Assisted Living

Get Palliative Care
http://www.getpalliativecare.org

National Family Caregivers Association
http://www.nfcacares.org

Physician Orders for Life-Sustaining Treatment (POLST)
http://www.caPOLST.org

Resources for Healthcare Professionals

Academy of Hospice and Palliative Medicine
http://www.aahpm.org

American Hospice Association
http://www.americanhospice.org

Caring Connections
http://www.caringinfo.org

Certificate Program for End-of-Life Care
http://www.naropa.edu/
contemplativecare

End of Life/Palliative Education Resource Center
http://www.eperc.mcw.edu

The EPEC Project (Education in Palliative and End-of-Life Care)
http://www.epec.net

Hospice Foundation of America
http://www.hospicefoundation.org

Hospice and Palliative Nurses Association
http://www.hpna.org

National Hospice and Palliative Care Association
http://www.nhpco.org

Physician Orders for Life-Sustaining Treatment (POLST)
http://www.caPOLST.org

Suggested Reading

The 36 Hour Day (A Family Guide for the Care of People with Alzheimer’s/Dementia)
Nancy L. Mace and Peter V. Rabins

All Kinds of Love: Experiencing Hospice
Carolyn Jaffe and Carol H. Ehrlich

An Ocean of Time: Alzheimer’s Tales of Hope and Forgetting
Patrick Mathiasen, MD

By No Means: The Choice to Forgo Life-Sustaining Food and Water
Joanne Lynn (editor)

Caring in Remembered Ways: The Fruit of Seeing Deeply
Maggie Steincrohn Davis

Choices at the End of Life: Finding Out What your Parents Want Before It’s Too Late
Linda Norlander,RN, MS and Kerstin McSteen, RN, MS

Dying At Home: A Family Guide for Caregiving
Andrea Sankar

Dying Well: The Prospect of Growth at the End of Life
Ira Byock, MD

Dying with Dignity: A Plea for Personal Responsibility
Hans Kung and Walter Jens

Facing Death and Finding Hope: A Guide for the Emotional and Spiritual Care of the Dying
Christine Longaker

Final Gifts: Understanding the Special Awareness, Needs, and Communications of the Dying
Maggie Callanan and Patricia Kelly

The Four Stages of Hope: Using the Power of Hope to Cope With Dying
Cathleen Fanslow-Brunjes, MA, RN

Helping Grieving People: When Tears Are Not Enough: A Handbook for Care Providers
Shep J. Jeffreys, EdD.

I’m Here To Help: A Guide for Caregivers, Hospice Workers, and Volunteers
Catherine Ray

On Death and Dying
Elisabeth K bler-Ross, MD

Share the Care: How to Organize a Group to Care for Someone Who Is Seriously Ill
Cappy Capossela, Sheila Warnock and Sukie

Distinctive features of saccades in PSP

Researchers found that there were several differences between PSP patients and normal controls in terms of eye movements:

* Square-wave jerks are “more frequent, larger, and more markedly horizontal in PSP.”

Here’s a definition from the abstract: “Square-wave jerks (SWJs), consists of saccade pairs that appear purely horizontal on clinical inspection: the first saccade moves the eye away from the fixation target, and after a short interval, the second saccade brings it back toward the target.”

SWJs occur in healthy controls.

* In PSP, there is a “loss of a vertical component in fixational saccades.”

Here’s my layperson definition of a saccade: It’s a constant eye movement that changes the position of gaze. When you stare at something, your eyes are not still. They are constantly moving back and forth as well as up and down.

In PSP, the eyes are no longer able to make these small adjustments up and down (vertically). This is part of the vertical supranuclear gaze palsy that is a trademark symptom of PSP. The authors suggest that “small saccades lose their vertical component in PSP, and this property may help clinicians differentiate PSP from other parkinsonian or cerebellar disorders.”

* In PSP, “normal fixational saccades (microsaccades) are rare.”

The saccades in PSP are not small as in healthy controls. The saccades are larger movements that meet the definition of “square-wave jerks.” As we learned in the first item above, the SWJs in PSP are more frequent and larger than are seen in healthy controls.

I’ve copied the abstract below.

Robin

This is the abstract:

Journal of Neuroscience. 2011 Mar 23;31(12):4379-87.

Distinctive features of saccadic intrusions and microsaccades in progressive supranuclear palsy.

Otero-Millan J, Serra A, Leigh RJ, Troncoso XG, Macknik SL, Martinez-Conde S.
Barrow Neurological Institute, Phoenix, Arizona, Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, Ohio, Institute of Clinical Neurology, University of Sassari, Sassari, Italy, California Institute of Technology, Pasadena, California, and University of Vigo, Vigo, Spain.

Abstract
The eyes do not stay perfectly still during attempted fixation; fixational eye movements and saccadic intrusions (SIs) continuously change the position of gaze.

The most common type of SI, square-wave jerks (SWJs), consists of saccade pairs that appear purely horizontal on clinical inspection: the first saccade moves the eye away from the fixation target, and after a short interval, the second saccade brings it back toward the target.

SWJs are prevalent in certain neurological disorders, including progressive supranuclear palsy (PSP).

Here, we developed an objective method to identify SWJs. We found that SWJs are more frequent, larger, and more markedly horizontal in PSP patients than in healthy human subjects.

Furthermore, the loss of a vertical component in fixational saccades and SWJs was the eye movement feature that best distinguished PSP patients from controls. We moreover determined that, in PSP patients and controls, the larger the saccade the more likely it was part of a SWJ. Furthermore, saccades produced by PSP patients had equivalent properties whether they were part of a SWJ or not, suggesting that normal fixational saccades (microsaccades) are rare in PSP.

We propose that fixational saccades and SIs are generated by the same neural circuit and that, both in PSP patients and in controls, SWJs result from a coupling mechanism that generates a second corrective saccade shortly after a large fixation saccade. Because of brainstem and/or cerebellum impairment, fixational saccades in PSP are abnormally large and thus more likely to trigger a corrective saccade, giving rise to SWJs.

PubMed ID#: 21430139 (see pubmed.gov for this abstract only)

5 Cases of Early Stage PSP (Pre-symptomatic)

Banner Sun Health in the Phoenix area encourages retirees in Arizona to donate their bodies and brains upon death. Many are neurologically normal. These neurologically normal brains are tremendous helpful in conducting research.

In this study of 277 brains donated to Banner Sun Health, they found five cases where the pathology seemed like “early PSP.” None of these five cases had any clinical symptoms of dementia, parkinsonism, or PSP. “Incidental” is the term neuropathologists give to such cases. So, incidental Lewy Body Disease is where the brain contains Lewy body pathology but there were no clinical symptoms of Parkinson’s Disease. And “incidental PSP” is where the brain has some PSP pathology but there were no PSP symptoms while the person was alive.

Robin

Parkinsonism & Related Disorders. 2011 Mar 18. [Epub ahead of print]

Neuropathological findings of PSP in the elderly without clinical PSP: Possible incidental PSP?

Evidente VG, Adler CH, Sabbagh MN, Connor DJ, Hentz JG, Caviness JN, Sue LI, Beach TG.
Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA.

Abstract
AIMS: We aimed to describe cases with incidental neuropathological findings of progressive supranuclear palsy (PSP) from the Banner Sun Health Research Institute Brain and Body Donation Program.

METHODS: We performed a retrospective review of 277 subjects with longitudinal motor and neuropsychological assessments who came to autopsy. The mean Gallyas-positive PSP features grading for subjects with possible incidental neuropathological PSP was compared to those of subjects with clinically manifest disease.

RESULTS: There were 5 cases with histopathological findings suggestive of PSP, but no parkinsonism, dementia or movement disorder during life. Cognitive evaluation revealed 4 of the 5 cases to be cognitively normal; one case had amnestic mild cognitive impairment (MCI) in her last year of life. The mean age at death of the 5 cases was 88.9 years (range 80-94). All 5 individuals had histopathologic microscopic findings suggestive of PSP. Mean Gallyas-positive PSP features grading was significantly lower in subjects with possible incidental neuropathological PSP than subjects with clinical PSP, particularly in the subthalamic nucleus.

CONCLUSIONS: We present 5 patients with histopathological findings suggestive of PSP, without clinical PSP, dementia or parkinsonism during life. These incidental neuropathological PSP findings may represent the early or pre-symptomatic stage of PSP. The mean Gallyas-positive PSP features grading was significantly lower in possible incidental PSP than in clinical PSP, thus suggesting that a threshold of pathological burden needs to be reached within the typically affected areas in PSP before clinical signs and symptoms appear.

Copyright © 2011 Elsevier Ltd. All rights reserved.

PubMed ID#: 21420891

Painful stimuli in face differentiates PSP and MSA-P

An Italian research team has put out a research paper comparing TCR (trigemino-cervical reflexes) in MSA and PSP.

TCRs are “electromyographic responses induced by electrical stimulation of the trigeminal nerve and recorded in the neck muscles.” Neurologically-normal people have TCRs in response to painful stimuli in the face; the head visibly retracts.

A research paper published in 2008 from the same Italian team showed that TCRs were absent in PSP, probably due to brainstem degeneration. TCRs were detected “in patients with PD, albeit showing a longer reflex latency than that found in healthy controls… These results suggest that absence of TCRs may be useful in supporting the diagnosis of PSP and may represent a potential tool for distinguishing PSP from other parkinsonian syndromes.”

Since MSA and PSP can look alike, the team decided to “investigate the usefulness of TCRs in differentiating PSP from MSA.” The authors note that the “differential diagnosis of these two conditions can be difficult,
especially when specific neurological signs, such as dysautonomy or vertical gaze palsy, are not obvious.”

The researchers compared 10 people with a clinical diagnosis of MSA-P to 10 people with a clinical diagnosis of PSP. (The neurophysiologist testers were unaware of the clinical diagnoses.) They found that all 10 patients with a clinical diagnosis of MSA-P demonstrated TCRs, and all 10 patients with a clinical diagnosis of PSP has no TCRs. “An important result of the comparison of the two groups of patients in our study is that no differences were found in terms of disease duration and severity.” The authors believe that PSP and MSA-P have different levels of brainstem degeneration.

The authors conclude: “Trigemino-cervical reflex recording is a rapid neurophysiological method, which could assist in the differential diagnosis between PSP and MSA-P.” The authors call for another study to include MSA-C patients.

This finding will be of interest to the PSP folks: “we found that PSP patients have high pain thresholds, although this feature cannot differentiate these subjects from MSA patients.” The authors speculate this is due to dysregulation of the pain processing contro.”

One weakness of the study is that these are clinical diagnoses. As the authors point out, PSP and MSA can be confused.

I’ve copied the abstract below.

Robin

Clinical Neurophysiology. 2011 Mar 9. [Epub ahead of print]

The contribution of trigemino-cervical reflexes in distinguishing progressive supranuclear palsy from multiple system atrophy.

Serrao M, Di Fabio R, Bartolo M, Perrotta A, Tassorelli C, Coppola G, Davassi C, Padua L, Sandrini G, Pierelli F.
Department of Medical and Surgical Science and Biotechnology, “Sapienza” University of Rome ICOT – Polo Pontino, Latina, Italy; Rehabilitation Centre Policlinico Italia, Rome, Italy.

Abstract

OBJECTIVE: Trigemino-cervical reflexes (TCRs) are electromyographic responses induced by electrical stimulation of the trigeminal nerve and recorded in the neck muscles. Trigemino-cervical reflexes are detectable in Parkinson’s disease, whereas they are absent in progressive supranuclear palsy (PSP), an atypical parkinsonism associated with brainstem degeneration. To date, no study has investigated TCRs in multiple system atrophy (MSA), another atypical parkinsonism associated with brainstem involvement, which resembles PSP.

METHODS: To understand whether TCRs are helpful in differentiating PSP from MSA, we compared the TCRs recorded in 10 PSP patients with those obtained from 10 patients diagnosed as having probable MSA, parkinsonian type (MSA-P).

RESULTS: Trigemino-cervical reflexes were not recorded in any of the PSP patients, while they were clearly detectable in all the MSA-P patients.

CONCLUSIONS: Trigemino-cervical reflex recording is a rapid neurophysiological method, which could assist in the differential diagnosis between PSP and MSA-P.

SIGNIFICANCE: This study further improves our understanding of the different neuronal functioning of extrapyramidal disorders. TCRs monitoring may be useful to support the diagnosis of atypical parkinsonisms especially when clinical evidence is uncertain.

Copyright © 2011. Published by Elsevier Ireland Ltd.

PubMed ID#: 21396886 (see pubmed.gov for this abstract only)

Lack of thirst distinguishes PSP from MSA-P and PD

Hypodipsia is “abnormally diminished thirst.” This German research team (one of the best in the world for studying PSP) concludes that hypodipsia is helpful in differentiating PSP from Parkinson’s and MSA-P.
Robin

Movement Disorders. 2011 Mar 7. [Epub ahead of print]

Hypodipsia discriminates progressive supranuclear palsy from other parkinsonian syndromes.

Stamelou M, Christ H, Reuss A, Oertel W, Höglinger G.
Department of Neurology, Philipps University, Marburg, Germany.

Abstract
BACKGROUND: The objective of this study was to evaluate whether the sensation of thirst differs between patients with progressive supranuclear palsy (PSP), multiple system atrophy with predominant parkinsonism (MSA-P), and Parkinson’s disease (PD).

METHODS: We administered a standardized thirst questionnaire to age-, sex-, and stage-matched patients with probable PSP, PD, and MSA-P and healthy controls (HC), n = 15/group. In an independent cohort (n = 10/group), we provoked thirst by infusing hypertonic NaCl in age-, sex-, and stage-matched patients with PSP, PD, and MSA-P and recorded plasma osmolality and thirst (visual analog scale).

RESULTS: On questioning, 73% of PSP patients reported a reduced sensation of thirst (hypodipsia) compared with previous years (HC, 0%; PD, 7%; MSA-P, 7%; P < .0001). On NaCl infusion, PSP patients reported significantly lower thirst than did PD and MSA-P patients for all times from 20 to 95 minutes (P < .05). The thirst score at 25 minutes discriminated individual PSP patients well from PD and MSA-P patients.

CONCLUSIONS: Hypodipsia appears helpful in differentiating PSP from PD and MSA-P.

Copyright © 2011 Movement Disorder Society.

PubMed ID#: 21384428 (see pubmed.gov for this abstract only)

Neurodegenerative disease misclassified as psychiatric

This UCSF study says more about the behavioral variant of frontotemporal dementia than it does about PSP, CBD, or the other neurodegenerative diseases included in the research. The researchers’ conclusion makes a couple of points:

* “Neurodegenerative disease is often misclassified as psychiatric disease. …[When] patients with neurodegenerative disease are initially classified with psychiatric disease, the patient may receive delayed, inappropriate treatment and be subject to increased distress.”

* “Physicians should consider referring mid- to late-life patients with new-onset neuropsychiatric symptoms for neurodegenerative disease evaluation.”

Robin

Journal of Clinical Psychiatry. 2011 Feb;72(2):126-33.

The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease.

Woolley JD, Khan BK, Murthy NK, Miller BL, Rankin KP.
UCSF, San Francisco, CA.

Abstract
OBJECTIVE: To identify rates of and risk factors for psychiatric diagnosis preceding the diagnosis of neurodegenerative disease.

METHOD: Systematic, retrospective, blinded chart review was performed of 252 patients with a neurodegenerative disease diagnosis seen in our specialty clinic between 1999 and 2008.

Neurodegenerative disease diagnoses included
* behavioral-variant frontotemporal dementia (n = 69),
* semantic dementia (n = 41), and
* progressive nonfluent aphasia (n = 17) (all meeting Neary research criteria);
* Alzheimer’s disease (n = 65) (National Institute of Neurologic and Communicative Disorders and Stroke-Alzheimer’s Disease and Related Disorders Association research criteria);
* corticobasal degeneration (n = 25) (Boxer research criteria);
* progressive supranuclear palsy (n = 15) (Litvan research criteria); and
* amyotrophic lateral sclerosis (n = 20) (El Escorial research criteria).

Reviewers remained blinded to each patient’s final neurodegenerative disease diagnosis while reviewing charts. Extensive caregiver interviews were conducted to ensure accurate and reliable diagnostic histories. For each patient, we recorded history of psychiatric diagnosis, family psychiatric and neurologic history, age at symptom onset, and demographic information.

RESULTS: A total of 28.2% of patients with a neurodegenerative disease received a prior psychiatric diagnosis. Depression was the most common psychiatric diagnosis in all groups.

Behavioral-variant frontotemporal dementia patients received a prior psychiatric diagnosis significantly more often (50.7%; P < .001) than patients with Alzheimer’s disease (23.1%), semantic dementia (24.4%), or progressive nonfluent aphasia (11.8%) and were more likely to receive diagnoses of bipolar disorder or schizophrenia than were patients with other neurodegenerative diseases (P < .001). Younger age (P < .001), higher education (P < .05), and a family history of psychiatric illness (P < .05) increased the rate of prior psychiatric diagnosis in patients with behavioral-variant frontotemporal dementia.

Cognitive, behavioral, and emotional characteristics did not distinguish patients who did or did not receive a prior psychiatric diagnosis.

CONCLUSIONS: Neurodegenerative disease is often misclassified as psychiatric disease, with behavioral-variant frontotemporal dementia patients at highest risk.

While this study cannot rule out the possibility that psychiatric disease is an independent risk factor for neurodegenerative disease, when patients with neurodegenerative disease are initially classified with psychiatric disease, the patient may receive delayed, inappropriate treatment and be subject to increased distress.

Physicians should consider referring mid- to late-life patients with new-onset neuropsychiatric symptoms for neurodegenerative disease evaluation.

© Copyright 2011 Physicians Postgraduate Press, Inc.

PubMed ID#: 21382304

Spectrum of Frontotemporal Degeneration – 3/2/11 Webinar Notes

I listened to yesterday’s AFTD (Association for Frontotemporal Degeneration) webinar on the spectrum of frontotemporal degeneration, thinking it was truly about the “spectrum.”  It was NOT about the spectrum in that the speaker announced early on that he would be ignoring PSP and CBS (two movement types of FTD), and concentrating on the three other types of FTDs – bvFTD, PNFA, and SD.  So my notes may not be of much interest to the PSP and CBD communities.

—————-

Notes by Robin Riddle about

The Spectrum of Frontotemporal Degeneration
AFTD webinar
Wednesday, March 2, 2011
Speaker – Mario Mendez, MD, PhD, Director of the FTD and Neurobehavior Clinic, UCLA

Organization of Talk
1. What is frontotemporal degeneration (FTD)?
2. What are the clinical syndromes of FTD?
3. Principles of Management
4. Resources and Referral

Brief History of FTD Syndromes
1892: Arnold Pick described 6 patients with FTD
1911: Alzheimer describes the neuropathology
DARK AGES
1993+: Renaissance: Epidemiology, Clinical Criteria
1997: Age of Tauopathy: FTDP-17, abnormal tau
2006: Age of TDP43 protein and progranulin gene
2009+: NEW AGE OF EXPLORATION, THERAPY

Epidemiology of FTD Syndromes

1. 5-8% of dementias; 13.5-15% if onset < 65
AD, VaD, and DLB are more common than FTD
Second most common dementia among young people

2. Prevalence/Incidence in 45-64 yr age group: 15/100,000 and 1-2/100,000

3. Usual onset in 50’s (mean onset 57-58)
Strikes people in their prime
Devastating time to develop a dementia: raising a family; making an income

4. Slightly more men than women

5. Duration shorter than AD (mean 8 years)
There are different series with different means and members.
Mean duration of about 7.6 years makes the most sense to him.
Semantic Dementia may be longer (9 years).

Three Key Points on FTD

1. Variable phenotypes and syndromes:
Behavioral variant FTD (bvFTD) involves social brain
Progressive nonfluent aphasia (PNFA)
Semantic dementia (SD)
Others: FTD-MND (10%), CBS (asymmetric motor findings; ideomotor apraxia), PSP

CBS and PSP are related syndromes. They are movement disorders. This talk will focus on the main syndromes.

2. Variable neuropathology: misfolded protein. The most common abnormal inclusions (clumps of protein) are with TDP-43 (45%), tau (40%), and FUS.

3. 15% autosomal dominant genetic mutations: in MAPT, PRGN, VCP, FUS, CHMP2B, TARDBP (this last one is with TDP-43).

Variable phenotypes and syndromes: Some people are very frustrated by this!

FTD Common Syndromes:

1. bvFTD: behavioral variant ~ 50%
A. often present with apathy, abulia, or detachment. MDs commonly diagnose these people as depressed when, in fact, these people are developing problems in the frontal lobe. Need to screen for depression.
B. Disinhibition or impulsivity. People violate social boundaries. Causes distress in families. He calls this “Private behaviors in public.”
C. FTD-MND (motor neuron disease)

2. Language predominant variants
A. PNFA: Progressive non-fluent aphasia ~ 25%
B. SD: Semantic dementia ~25%

3. CBS or PSP: parkinsonism symptoms

The FTD Spectrum With 5-Year UCLA FTD Clinic Experience:
FTD (behavioral variant), n=118
Primary Progressive Aphasia (non-fluent), n=48
Semantic Dementia, n=19
Corticobasal syndrome, n=11
Progressive supranuclear palsy, n=9
(Literature: MND in about 15%)

Frontotemporal Degenerations
Frontotemporal Dementia: 56.7%; Age at Onset 57.5 (9.7); Male sex 63.5%; Initial MMSE 22.7 (6.6)
Progressive Nonfluent Aphasia: 18.7%; Age at Onset 59.3 (8.2); Male sex 66.7%; Initial MMSE 21.5 (7.08)
Semantic Dementia: 24.6%; Age at Onset 63.0 (9.7); Male sex 39.1%; Initial MMSE 22.5 (7.0)
From Johnson et al, Arch Neurol 2005;62:925-30

bvFTD – Core diagnostic features
A. Insidious onset and gradual progression
B. Early decline in social interpersonal conduct
C. Early impairment regulation of personal conduct
D. Early emotional blunting: Acting as if they don’t care. Not caring about others.
E. Early loss of insight: No acceptance that anything is wrong.

From: Neary et al, Neurology 1998;51:1546-51;
SE 85%, SP 99% among 34 patients in Knopman et al, 2005

These diagnostic criteria are difficult to operationalize! How do you assess “early decline in social interpersonal conduct”?

Symptoms in 53 bvFTD Patients – at onset and after 2 years:
Decline in social conduct: 39.6% onset; 83% 2 years
Impaired personal regulation: 69.8% onset; 88.7% 2 years
Emotional blunting: 35.8% onset; 94.3% 2 years
Lack of insight: 58.5% onset; 100% 2 years
Compulsive-like behaviors: 45.3% onset; 88.7% 2 years
Logopenia and anomia: 41.5% onset; 96.2% 2 years
Hyperorality (other KBS) 0% onset; 20.8% 2 years

From Mendez & Perryman, 2002

Results of 134 referrals to the UCLA FTD & Neurobehavior Clinic for possible bvFTD:
23 (17.2%) initially met criteria for bvFTD
40 converted to bvFTD by two year f/up
36 had psychiatric disorder
17 had Alzheimer’s disease
9 had another neurological disorder [Anoxic encephalopathy (2), prion disease (2), Hashimoto’s encephalopathy, neurosarcoidosis, NPH, paraneoplasticsyndrome, sleep apnea syndrome]
9 without final diagnosis

Note how few met the criteria for bvFTD!

International Consensus Criteria for bvFTD

I. Neurodegenerative Disease: progressive deterioration behavior and/or cognition

II. Possible bvFTD (3 of A-F present…so 3 out of 6)

A. Early behavioral disinhibition (1 or more)
1. Socially inappropriate behavior
2. Loss of manners or decorum
3. Impulsive, rash or careless actions

B. Early apathy or inertia

C. Early loss of sympathy or empathy (1 or more)
1. Diminished response to others people’s
needs and feelings
2. Diminished social interest, interrelatedness
or personal warmth

D. Early perseverative, stereotyped or compulsive/ritualistic behavior
1. Simple repetitive movements
2. Complex, compulsive or ritualistic
3. Stereotypy of speech

E. Hyperorality and dietary changes (1 or more)
1. Altered food preferences
2. Binge eating, increased consumption of alcohol or cigarettes
3. Oral exploration or consumption of inedible objects

F. Neuropsychological profile (all 3 present)
1. Deficits in executive tasks
2. Relative sparing of episodic memory
3. Relative sparing of visuospatial skills

These are much better. They will be approved soon.

C: has some similarities with autistic spectrum disorders.

The clinical diagnosis should be corroborated with neuro-imaging. Scan shows disproportionate frontal lobe hypometabolism. The imaging is getting better and better.

UCLA uses the FDDNP ligand. FDDNP-PET shows different distribution of cortical pathology in AD and FTD.

The disease often begins in the “social brain.”

How does FTD affect social behavior?
1. Acquired sociopathy (Mendez et al, 2003; Miller et al, 1997)
2. Reduced understanding of social concepts (Zahn et al, 2009)
3. Reduced self-referential emotions (Sturm et al, 2006)
4. Reduced recognition of facial emotions (Rosen et al, 2005)
5. Reduced empathy (cognitive-OF, emotional-TL; Rankin et al, 2005)
6. Reduced theory of mind (Gregory et al, 2002; Lough et al, 2006)
7. Reduced recognition of humanness (Mendez et al, 2005,2006)

Sociopathy in 16 FTD Patients seen at UCLA:
3 Unsolicited sexual approach or touching
3 Traffic violations including hit-and-run accidents
2 Physical assaults
1 Shoplifting
1 Deliberate non-payment of bills
1 Pedophilia
1 Indecent exposure in public
1 Urination in inappropriate public places (eg, on neighbor’s lawn)
1 Stealing food
1 Eating food in grocery store stalls
1 Breaking and entering into others’ homes (eg, in order to play the piano)

Phineas Gage: famous patient; iron rod damaged his frontal lobe. He illustrates the disinhibited subtype.

International Guidelines for the Diagnosis of Primary Progressive Aphasia – Workgroup Results
Three main variants:
• PNFA ­ progressive non-fluent aphasia: Now nonfluent/agrammatic variant PPA
• SD ­ semantic dementia: Now semantic variant PPA
• PLA- progressive logopenic aphasia: Now logopenic variant PP. This is often AD.

Progressive NF Aphasia
• Grammatical difficulty in language production
• Reduced motor speech — effortful, hesitant, phonemic abnormality
• Apraxia of speech frequently present
• Reduced comprehension of syntactically complex sentences
• Spared word comprehension and object recognition
• Atrophy inferior left frontal-anterior insula region

[There were lots of examples of agrammatic speech. See the PDF for all of those.]

Semantic Dementia
• Multimodal semantic deficits (modality-specific)
• Semantic anomia – decreased category fluency & decreased word comprehension
• Abnormal person and object recognition
• Surface dyslexia and regularization, typicalization errors
• Decreased specificity, general (superordinate) word preference
• Bizarre food choices or fads and rigidity
• Atrophy of temp polar, perirhinal “recognition” cortex

He calls this the “what is” disease as patients ask “what is a bottle?,” “what is this?”

Pyramids and palm trees test.
Drawings become more generic. Drawing of peacock becomes more generic.

Logopenic Variant PPA: Not discussing today as this is typically AD.

Evolution of FTD’s (2-5 yrs)
• FTDbv: 1/2 become PNFA; 1/4 become CBS/PSP
• PNFA: 1/2 become FTDbv; 1/3 become CBS/PSP
• CBS/PSP: 1/2 become FTDbv; 1/2 become PNFA
• SD: 3/4 become FTDbv
• Differences still persist at end of life
From Kertesz et al, 2007; Snowden et al, 2007

MAPT and PRGN
• 6% Microtubule-associated tau gene (C’17) mutations
• Progranulingene (C’17) mutations in 10%; TAR-DNA-binding protein-43 (TDP-43) in ubi+ inclusions

Good review in the journal Neurology about genetic testing with genetic counseling.
Most people don’t benefit from genetic testing.
There is genetic testing for MAPT and PRGN.

[There was a slide on CSF biomarkers, different from the slide in the PDF.]

His management approach:
Primarily symptomatic
SSRIs helpful for disinhibition and repetitive or compulsive behaviors
Trazodone for more disruptive behaviors
Methylphenydate useful in activating some who lack initiation or are apathetic
AchI used in AD can make things worse in FTD (disinhibition, repetitive behaviors)

Long list of drugs tested in FTD

Caregiver support is extremely important:
* Behavioral, functional, financial, and legal counseling
* Provide an “external executive”
* Use AFTD (theaftd.org) and Alzheimer’s Association resources
* Speech, occupational, physical therapy
* Establish dedicated support groups
* Daycare, respite care and nursing care

Conclusions
* FTD is common in those <65 who develop changes in social behavior or language
* The clinical syndromes vary and overlap. The main syndromes are bvFTD, PNFA, and SD. Others are FTD-MND, CBS, and PSP.
* The neuropathology varies.
* 15% are genetic and include MAPT, PRGN, FUS, valosin, and TARBP genes. Must provide genetic counseling for the entire family.
* Management is primarily symptomatic. Focus on treating disruptive behaviors with a limited psychoactive agents such as SSRIs.
* Contact AFTD

Questions and Answers:

Q: Is there a standard evaluation?

A: We have standard scales we use, including neuropsychological tests. UCLA has its own MRI sequence or series.

Q: Once diagnosed, do you declare them incapacitated?

A: Yes/No. Capacity is state-dependent. The communication to the patient and family: “the patient is incapacitated.” Patients are more incapacitated than their neuropsychological tests will tell you. The patient is at risk for poor decision-making.

Q: Is there a correlation with traumatic brain injury?

A: TBI is not an established risk factor for FTD.

Many with TBI are injured in their frontal lobes so some of the symptoms may be the same.

Q: Neuropathological differences?

A: [didn’t understand question or answer!]

 

Acalculia in 2 autopsy-proven CBD cases

UPenn researchers recently got published a report of two cases of acalculia in autopsy-confirmed CBD. Acalculia is an impairment in the ability to calculate or performing simple mathematical tasks, such as adding or subtracting.

I don’t consider this particularly new information but the authors say that “While the original case descriptions mentioned acalculia, few studies have investigated this, and reports of acalculia in autopsy-proven CBD are very rare. We detail 2 autopsy-defined CBD cases with acalculia to emphasize that CBD compromises cognitive functioning due to disease that includes parietal cortex.”

This paragraph from the Discussion section is interesting: “In 15 patients with autopsy-proven CBD that included the 2 cases detailed here, acalculia was noted in 28.6%, although this was thought to be an underestimation since calculations were not often examined. … Patients with CBS have significant impairments estimating and comparing quantities, performing calculations with small numerosities, and using quantity knowledge to support word meaning. … MRI in CBS regularly shows parietal atrophy, including areas associated with number knowledge.”

I’ve copied the citation below.

Robin

Neurology. 2011 Feb 15;76(7 Suppl 2):S61-3.

Acalculia in autopsy-proven corticobasal degeneration.

Pantelyat A, Dreyfuss M, Moore P, Gross R, Schuck T, Irwin D, Trojanowski J, Grossman M.
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.

FTD Webinar, Wed 3/2, noon CST, registration open

Editor’s note:  See our notes from the webinar here.

Sorry for the short notice on this. I just received an email on this webinar from the AFTD — which now stands for the Association for Frontotemporal Degeneration (rather than Dementia). The AFTD now has a new web address as well; you can find them online at theaftd.org. (ftd-picks.org re-routes you to theaftd.org.)

You are invited to participate in this webinar:

The Spectrum of Frontotemporal Degeneration
(tomorrow) Wednesday, March 2
noon to 1pm central time
speaker – Dr. Mario Mendez, Director of the FTD and Neurobehavior Clinic at UCLA
register here – http://cme.edocendo.com/

The registration page gives these objectives for the webinar:

“Those attending this presentation will receive information that should allow them to:
– Have a working knowledge of frontotemporal degeneration (FTD) and how to diagnosis these syndromes;
– Comprehend the major clinical syndromes of FTD: bvFTD, PPA, CBS, PSP;
– Learn principles of management of FTD, particularly addressing the unique burden on caregivers and family;
– Know where to find resources and when to refer patients with FTD.”

When you register, you first have to create an account, which requires entering a username and password. Then you fill out a form that asks for your name, last four digits of SSN, your degree, your company, your address, and your phone #. (There is no accuracy-checker.) You are then sent an email which tells you how to join the webinar tomorrow.

This webinar is sponsored by AFTD. Most of the audience will be physicians and other medical professionals. (CME — continuing medical education — credits are available for MDs.)

There’s a short 4-question pre-test on FTD you can take.* Based on that pre-test and other MD-oriented webinars I’ve attended, my guess is that most people here will be able to understand much of what is said.

Robin

—————-

* The Spectrum of Frontotemporal Degeneration Pre-Test

The following test must be completed prior to participating in “The Spectrum of Frontotemporal Degeneration” CME activity. This test is required for you to receive your CME credit.

1. FTD has its usual onset in the elderly (>65 years of age)?
A. True
B. False

2. FTD is clinically indistinguishable from Alzheimer disease?
A. True
B. False

3. The diagnosis of FTD relies on clinical criteria rather than laboratory tests?
A. True
B. False

4. The most common presentations of FTD are personality changes or language problems?
A. True
B. False