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Gorno Tempini2011 PDF
Gorno Tempini2011 PDF
I. Clinical diagnosis of nonfluent/agrammatic variant PPA I. Clinical diagnosis of semantic variant PPA
At least one of the following core features must be Both of the following core features must be present:
present:
1. Impaired confrontation naming
1. Agrammatism in language production
2. Impaired single-word comprehension
2. Effortful, halting speech with inconsistent speech
sound errors and distortions (apraxia of speech) At least 3 of the following other diagnostic features must
be present:
At least 2 of 3 of the following other features must be
present: 1. Impaired object knowledge, particularly for low-
frequency or low-familiarity items
1. Impaired comprehension of syntactically complex
sentences 2. Surface dyslexia or dysgraphia
Both of the following criteria must be present: Both of the following criteria must be present:
Speech/language
function Task Behavioral measures Variant in which impaired
Speech
production
Grammar Picture description task; story retelling Grammatical structure; mean length of utterance; Nonfluent/agrammatic variant
(e.g., picture aided); constrained-syntax speech rate; accuracy of content; melody;
sentence production task prosody; specific error types in word selection;
articulation
Motor speech Motor speech evaluation, including Effortfulness; hesitations; presence of apraxia of Nonfluent/agrammatic variant
multiple repetitions of multisyllabic speech or dysarthria; specific types of speech
words; diadochokinesis of speech sound errors; factors that affect articulation (e.g.,
articulators; spontaneous speech word length in syllables)
Confrontation Single-word retrieval in response to Error rate; delay in naming; factors that affect Severe deficit in semantic variant
naming pictures, sounds, foods, and odors naming accuracy (e.g., familiar vs unfamiliar with semantic errors; moderate
items, nouns vs verbs, semantic category); error impairment in logopenic variant
types (e.g., semantic errors, phonemic errors) with phonemic errors
Repetition Oral repetition of words, pseudowords, Factors that affect repetition accuracy (e.g., Logopenic variant with
phrases, and sentences predictability of the phrase, sentence length, phonological errors
grammatical complexity); error types
Sentence Matching orally presented sentences Factors that affect comprehension (e.g., Nonfluent/agrammatic variant,
comprehension to pictures; answering yes/no grammatical complexity; reversibility of the effect of grammatical complexity;
questions; following directions sentence, e.g., The boy was kicked by the girl vs logopenic variant, length and
The ball was kicked by the girl) frequency effect
Single-word Word-to-picture matching; Word-to- Factors that affect comprehension (e.g., Semantic variant
comprehension definition matching; Synonym familiarity; frequency; grammatical word class)
matching
Object/people Picture-picture matching; odd-one-out; Factors that affect object knowledge (e.g., Semantic variant
knowledge semantic associations; gesture-object familiarity, semantic category)
matching; sound-picture matching
Reading/spelling Lists including regular and irregular Factors that affect reading/spelling accuracy Semantic variant with
word lists, from various word classes, (e.g., regularity, frequency, word class); error “regularization” errors; logopenic
matched for other factors; types (e.g., regularization, phonologically variant phonologic errors
pseudowords matched to words in plausible errors; articulatory distortions)
length
usually associated with greater right temporal atro- variant PPA, FTLD-TDP type pathologic changes
phy and early behavioral changes, such as loss of em- will be the most common finding.15,20-23
pathy and compulsions.45
Logopenic variant PPA (also known as logopenic pro-
Surface dyslexia and dysgraphia are features of the
gressive aphasia or as logopenic PPA). The logopenic
semantic variant and refer to an impairment in read-
variant is the most recently described variant of
ing and writing words with “irregular” or atypical
PPA.11,50 The criteria are summarized in table 4.
relationship between spelling and pronunciation.46
Word retrieval (in spontaneous speech and confron-
Patients typically “regularize” such words, so that
tation naming) and sentence repetition deficits are
“sew” is read as /su/.
the core features of the logopenic variant. Spontane-
Sparing of repetition and motor speech, even
ous speech is characterized by slow rate, with fre-
when semantic deficits are prominent, are the other 2
quent pauses due to significant word-finding
features. Although language production is usually
grammatically accurate, it can at times contain some problems, but there is no frank agrammatism.
“paragrammatic” errors, such as substituting less ap- Speech production deficits are therefore distinct
propriate closed class words or inflections, for in- from those of patients with the nonfluent variant,
stance, “I know what they’re doing but I can’t think who also speak in a slow and halting manner, but
the words what they’re doing.”47 with output that is dysprosodic, and marked by mo-
Anatomically, the semantic variant has been asso- tor speech errors or agrammatism.8,50,51 The confron-
ciated with atrophy in the ventral and lateral portions tation naming impairment is usually less severe in the
of the anterior temporal lobes bilaterally, although logopenic than in the semantic variant, and errors are
damage is usually greater on the left.11,14,34,48,49 usually phonologic in nature.11 A useful differentiat-
Cases will be defined as semantic variant PPA ing feature between these 2 variants is also the rela-
with definite pathology when patients present with tive sparing of single-word comprehension in
the above mentioned clinical features, imaging- logopenic patients. Consistent with the hypothesis
supported or not, and a known histopathologic diag- that a phonologic short-term memory deficit is a key
nosis. Based on the literature, in the case of semantic cognitive mechanism underlying most language defi-
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