NFAV-PPA

The diagnostic criteria for nonfluent/agrammatic variant PPA (NFAV-PPA) developed by an international group of PPA investigators use two diagnostic categories: clinical diagnosis and imaging-supported diagnosis. The criteria must be met as defined without meeting any of the exclusion criteria for primary progressive aphasia (PPA).

Primary Progressive Aphasia (PPA)

Criteria 1-3 must be answered positively for a PPA diagnosis.

1. Most prominent clinical feature is difficulty with language: e.g., word-finding deficits, paraphasias, effortful speech, grammatical and/or comprehension deficits
2. These deficits are the principal cause of impaired daily living activities: e.g., problems with communication activity related to speech and language, such as using the telephone; or performing routine job responsibilities that require verbal communication
3. Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease.

Exclusion Criteria

Criteria 1-4 must be answered negatively for a PPA diagnosis.

1. Pattern of deficits is better accounted for by other non-degenerative nervous system or medical disorders: e.g., neoplasm, cerebrovascular disease, hypothyroidism
2. Cognitive disturbance is better accounted for by a psychiatric diagnosis: e.g., depression, bipolar disorder, schizophrenia, pre-existing personality disorder
3. Prominent initial episodic memory, visual memory and visuo-perceptual impairments: e.g., inability to copy simple line drawings
4. Prominent initial behavioral disturbance: e.g., marked disinhibition, emotional detachment, hyperorality or repetitive/compulsive behaviors

Diagnostic Criteria for Nonfluent/Agrammatic Variant PPA (NFAV-PPA)

Also known as progressive nonfluent aphasia (PNFA) and agrammatic PPA or PPA-G

Clinical Diagnosis of NFAV-PPA

At least one of the core features (1 and 2) must be present, and at least two of the following other features (3-5) must be present.

1. Agrammatism in language production
2. Effortful, halting speech with inconsistent distortions, deletions, substitutions, insertions, or transpositions of speech sounds, particularly in polysyllabic words (often considered to reflect "apraxia of speech")
3. Impaired comprehension of syntactically complex sentences
4. Spared single word comprehension
5. Spared object knowledge

Imaging-Supported NFAV-PPA Diagnosis

Both of the following criteria must be present.

1. Clinical diagnosis of NFAV-PPA
2. Imaging must show one or more of the following results:
1. Predominant left posterior fronto-insular atrophy on MRI
2. Predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET