A rare neurodegenerative disorder characterized by isolated and gradual dissolution of language function that progresses to dementia (Mesulam M).
Associated with difficulty naming objects, word endings, junctions, pronouns, and verb tenses.
Initially the process begins with difficulty finding words, with progression to impaired grammatical structure and comprehension.
Process begins in middle age.,
Personality, memory , visual processing are reserved until advanced stages of the disease.
Three classifications exist: progressive nonfluent aphasia is characterized by progressive difficulty with production of speech, semantic dementia characterized by a disorder of word and object meaning, and lohopenic progressive aphasia which is marked by word finding and repetition difficulty.
The process interefers ability to memorize word lists, and to solve reasoning tasks.
Recalling daily events, and social skills remain intact at diagnosis.
Patients seek medical attention because of word finding disabilities, abnormal speech patterns and spelling errors.
Language is the the prominent abnormality for the first two years of PPA.
Associated with atrophy of the central part of the brain’s left hemisphere where the language center is housed.
This site may have scar tissue and abnormal proteins that reduce the brain’s activity.
There is a relationship in the left hemisphere between brain atrophy and impairments in language performance.
PPA associated with perisylvian portions of the inferior frontal and temporalparietal regions known as Broca’s and Wernicke’s areas, respectively, as well as surrounding of the frontal, parietal, temporal cortex display atrophy, EEG slowing, impaired blood flow, and decreased glucose use.
Anomia, the difficulty with word finding and impaired object naming, is the most common sign of PPA.
With time spontaneous speech becomes increasingly dysfluent.
May be associated with speech 2242ors including simplification, circumlocution, phonemic paraphasic mistakes.
Agrammatism with progressive telegraphic type speech pattern may develop.
Comprehension for grammatically complex language becomes difficult.
Patients eventually develop difficulty with comprehension and may become mute.
Initially deficits may be restricted to expressive language functions.
Progressive expressive aphasia is the deterioration of normal language function that causes individuals to lose the ability to communicate fluently while still being able to comprehend single words and intact other non-linguistic cognition.
It is found in a variety of degenerative neurological conditions including Pick’s disease, motor neuron disease, corticobasal degeneration, frontotemporal dementia, and Alzheimer’s disease.
Depression is a common association.
In following years a more global dementia may supervene, as maybe behavioral dysfunction and symptoms of motor neuron disease or cortical basal degeneration.
Within 10 years patients can become mute and lose the ability to understand both written and spoken language.
Associated with learning disabilities and certain gene mutations.
Patients with dyslexia are at higher risk.
Family related cases have been reported.
Diagnose his by clinical evaluation, and there is no specific test to diagnose the process.
Neuroimaging studies exclude other pathological states.
Neuropsychological testing is not diagnostic.
May have associated Alzheimer’s disease.
No efficacious medical management exists.
Speech and language therapy can be helpful