PPAs and language assessment

Dre. MP Thibodeau, MD, FRCPCGeriatrician

CHUM, MontréalCGS-resident day, April 2018

Disclosure

๏ No relationship with financial sponsors

๏ No financial or in-kind support

๏ No potentiel for conflict of interest

Objectives

๏ Recognize the specificities of the 3 PPA variants

๏ Name the relevant aspects which should be assessed on history

๏ Develop skills in order to perform a bedside language assessment

What is a PPA ?๏ It’s Primary !

• Not vascular, cancer or trauma

๏ It’s Progressive !

• Neurodegenerative disease

๏ It’s an Aphasia !

• Language dominant disease

Clinical family

FTD

PSPsCBSMNDPPAbvFTD

NFvSv

Lancet Neurol. 2011 Feb;10(2):162-72.

AgvAoS

Lv

Inclusion criteria for PPACriteria 1–3 must be answered positively

1. Most prominent clinical feature is difficulty with language

2. These deficits are the principal cause of impaired daily living activities

3. Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease

Mesulam, 2001

Exclusion criteria for PPA1. Pattern of deficits is better accounted for by other nondegenerative nervous system or medical disorders

1. NO stroke on imaging !

2. Cognitive disturbance is better accounted for by a psychiatric diagnosis

3. Prominent initial episodic memory, visual memory, and visuoperceptual impairments

4. Prominent, initial behavioral disturbance

Epidemiology๏ Not well defined

๏ Age of onset later than bv-FTD

๏ Male=female

๏ Survival: 8-10 y

Karageourgiou et al. Semin Neuro, 2014

video 1

Non Fluent variantNFv-PPA clinical criteria

1. Agrammatism

1. Telegraphic speech, syntax errors

2. Apraxia of speech

1. Effortful, halting speech with inconsistent speech sound errors and distortions

3. Comprehension

1. Impaired comprehension of syntactically complex sentences

2. Spared single-word comprehension

3. Spared object knowledge

2/3

Classification of primary progressive aphasia and its variants. Neurology. 2011 Mars.

1/2

Non Fluent variantNFv-PPA imaging criteria

Imaging must show one or more of the following results:

1. Predominant left posterior fronto-insular atrophy on MRI or

2. Predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET

NFv-PPAOther key features

๏ Pt often frustrated by difficulties

๏ Good functional status

๏ More EPS, neurological deficits

๏ More problems with praxias

1. Bucco-facials

๏ Posterior cerebral function preserved

2. Apraxia of limb, calculations, visuo-spatial

video 2

Semantic variantSv-PPA Clinical Criteria

Both of the following core features must be present:

1. Impaired confrontation naming

1.Follows hierarchy

2.Worst when asked to identify description

2. Impaired single-word comprehension

1.Word-image association

2.Syntaxic comprehension preserved

Classification of primary progressive aphasia and its variants. Neurology. 2011 Mars.

Semantic variantSv-PPA Clinical Criteria

At least 3 of the following must be present:

1. Impaired object knowledge

1.particularly for low frequency or low-familiarity items

2. Surface dyslexia or dysgraphia

3. Spared repetition

4. Spared speech production

1.grammar and motor speech

Semantic variantSv-PPA imaging Criteria

Imaging must show one or more of the following results:

1. Predominant anterior temporal lobe atrophy

2. Predominant anterior temporal hypoperfusion or hypometabolism on SPECT or PET

Semantic VariantOther key features

๏ Bilateral disease

๏ More behavioral symptoms

1. More conscious of deficits than FTD, but can become agressive when confrontation

๏ Interest for art and shiny things !

๏ Forward digit span preserved helps differentiate early stages from Lv

๏ Normal physical examen even late in disease as opposed to NFv

Right Sv-PPA๏ Behavioral syndrome

1. Cold and distant

2. Rigidity

๏ Trouble identifying others emotions

๏ Healthy eating habit change !

๏ Prosopagnosia

๏ Loss of insight

๏ Compulsion with verbal stimuli

Last variant

Logopenic variantLv-PPA clinical criteria

1. Impaired single-word retrieval in spontaneous speech and naming

2. Impaired repetition of sentences and phrases

1.Phonological memory impairment

1.Forward digit span deficits

3.Speech (phonologic) errors in spontaneous speech and naming

4.Spared single-word comprehension and object knowledge

5.Spared motor speech

6.Absence of frank agrammatism

Classification of primary progressive aphasia and its variants. Neurology. 2011 Mars.

2/2

3/4

Logopenic variantLv-PPA imaging criteria

Imaging must show at least one of the following results:

1. Predominant left posterior perisylvian or parietal atrophy on MRI

2. Predominant left posterior perisylvian or parietal hypoperfusion or hypometabolism on SPECT or PET

Logopenic variantOther key features

๏ Complex calculation déficits

๏ Can evolve to a more diffuse disease

1. PPA +

๏ Co-exists w PCA

2. visuoperceptual pb

๏ More often AD

On a brain…

Sv

LvNFv

Loss of word meaning

Comprehension deficits

Repetition deficits

Motor speech disorder

Agrammatism

Bedside evaluation

Bad news….๏ No simple validated tool !

๏ PALS, ACE-III, DTLA, Sydney language battery (SYDBAT)

What to ask• Past history

• Handedness• Learning disabilities

• Family history of FTD, PPA, mouvement disorder

• Speech problems

• Stuttering, change in voice, dysarthria

• Language problems

• Word retrieval

• Paraphasias

• Loss of word meaning

• Comprehension deficits

• Trouble reading and writing

• Behavioral problems

• Neurological Sx

What to test๏ Spontaneous language

๏ Cookie theft

๏ Image naming

๏ Apraxia of speech

•Hippopotamus, eccentricity, caterpillar…

๏ Dysarthria

•Pa-ta-Ka

What to test ๏ Word comprehension

๏ Semantic associations

๏ Word and sentence repetition

๏ Sentence Comprehension

๏ Surface dyslexia and dysgraphia

1. Words and non-words

2. Earth, ghost, choir

๏ MMSE, MoCA

๏ FAB

Leyton, Brain 2011; 134:3030

Vandenberghe 2016, Alzheimer's Research & Therapy, 8:16

Mixed variant…

It’s normal…๏ 1/6 cases don’t fit in a category

๏ Don’t be afraid to say follow-up necessary

๏ Write what features support which Dx

Sajjadi et al. 2012

“The enemy now has a name and I can fight that enemy.”

What is the importance of identifying the right subtype ?

๏ Speech therapy and neuropsy

๏ Implication for treatment

1. CholI

๏ Impact on follow-up

2. Physical signs

3. Physical therapy

4. Behavioral pb approach

Pathological family

FTD\FTLD

MNDPPABvFTD PSPSCBS

TAU

TDPTDPFUSAD

NFvSv Lv

ADTAU

TDP TAUTAU

MacKenzie, Acta Neuropathol (2010) 119:1–4

TDPAD

AD

What to remember ?

Sv

LvNFv

Loss of word meaning

Comprehension deficits

Repetition deficits

Motor speech disorder

Agrammatism

Questions ?Thank you !

Key references๏ Gorno-Tempini et al. Classification of primary progressive aphasia and its variants Neurology 76:1006–1014

๏ Leyton et al, PALS, 2011

๏ Vandenberghe Alzheimer's Research & Therapy (2016) 8:16

๏ Karageourgiou et al. Semin Neuros, 2014