Progressive nonfluent aphasia

Presentation

The main clinical features are signature language progressive difficulties with speech production. There can be problems in different parts of the speech production system, hence patients can present with articulatory breakdown, phonemic breakdown (difficulties with sounds) and other problems. However, it is rare for patients to have just one of these problems and most people will present with more than one problem. Features include:

• Hesitant, effortful speech
• Apraxia of speech
• Stutter (including return of a childhood stutter)
• Anomic aphasia (word retrieval failures)
• Phonemic paraphasia (sound errors in speech e.g. 'gat' for 'cat')
• Agrammatism (using the wrong tense or word order)

As the disease develops, speech quantity decreases and many patients become mute.

Cognitive domains other than language are rarely affected early on. However, as the disease progresses, other domains can be affected. Problems with writing, reading, and speech comprehension can occur, as can behavioural features similar to frontotemporal dementia.

Diagnosis

Imaging studies have shown differing results which probably represents the heterogeneity of language problems than can occur in PNFA. However, classically atrophy of left perisylvian areas is seen. Comprehensive meta-analyses on MRI and FDG-PET studies identified alterations in the whole left frontotemporal network for phonological and syntactical processing as the most consistent finding. Based on these imaging methods, progressive nonfluent aphasia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, frontotemporal dementia and semantic dementia.

Classification

Some confusion exists in the terminology used by different neurologists. Mesulam's original description in 1982 of progressive language problems caused by neurodegenerative disease (which he called primary progressive aphasia (PPA) included patients with progressive nonfluent (aphasia, semantic dementia, and logopenic progressive aphasia.

Management

No cure or treatment for this condition has been found. Supportive management is helpful.

References

References

1. M. Hunter Manasco. (2014). "Introduction to Neurogenic Communication Disorders". Elsevier Health Sciences.
2. (March 2011). "Classification of primary progressive aphasia and its variants". Neurology.
3. (July 2007). "Towards a nosology for frontotemporal lobar degenerations-a meta-analysis involving 267 subjects". NeuroImage.
4. (June 1982). "Slowly progressive aphasia without generalized dementia". Annals of Neurology.
5. (April 2001). "Primary progressive aphasia". Annals of Neurology.
6. (March 2011). "Classification of primary progressive aphasia and its variants". Neurology.
7. (November 2010). "The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants". Current Neurology and Neuroscience Reports.
8. (September 2011). "Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship". Neuropsychology Review.
9. (2015). "Treatment of Primary Progressive Aphasia". Current Treatment Options in Neurology.