Primary progressive aphasia




Primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired while other mental functions remain intact. It was first described as a distinct syndrome by M.-Marsel Mesulam in 1982. Primary Progressive Aphasias have a clinical and pathological overlap with the Frontotemporal Lobar Degeneration (FTLD) spectrum of disorders and Alzheimer's disease.


The list of signs and symptoms mentioned in various sources for Primary progressive aphasia includes the 4 symptoms listed below:

  • Reduced language skill
  • Difficulty using language
  • Difficulty understanding language
  • Difficulty finding right words

Note that Primary progressive aphasia symptoms usually refers to various symptoms known to a patient, but the phrase Primary progressive aphasia signs may refer to those signs only noticable by a doctor.


The following diagnosis criteria were defined by Mesulam:

  • Gradual impairment of object naming, syntax and word-processing
  • Premorbid language function is usually intact
  • Acalculia: inability to perform simple mathematical calculations
  • Ideomotor Apraxia: loss of the ability to execute or carry out learned purposeful movements


There is no approved treatment. But speech therapy can assist an individual with strategies to overcome difficulties. ″There are three very broad categories of therapy interventions for aphasia: restorative therapy approaches, compensatory therapy approaches, and social therapy approaches. Rapid and sustained improvement in speech and dementia in a patient with primary progressive aphasia utilizing off-label perispinal etanercept, an anti-TNF treatment strategy also used for Alzheimer's, has been reported. A video depicting the patient's improvement was published in conjunction with the print article. These findings have not been independently replicated and remain controversial.