Primary progressive aphasia (Dementia)

Related Subjects: | Dementias |Abbreviated Mental Test Score (AMTS) |Alzheimer disease | Dementia with Lewy bodies | Frontotemporal dementia | Corticobasal degeneration | Creutzfeldt Jakob disease | Vascular Dementia | Primary progressive aphasia | Anti Dementia Drugs | AIDS Dementia Complex | Normal Pressure Hydrocephalus | Acetylcholinesterase inhibitors | Mental Capacity Act 2005 | Behavioural and Psychological Symptoms of Dementia

Aetiology

• A Dementia predominantly affecting the speech areas
• It is a form of Frontotemporal dementia
• Some forms inherited in an autosomal dominant manner
• Pathogenic variant in the GRN gene

Clinical

• Difficulty in finding words for unfamiliar items, without loss of language fluency, progressing to more severe word-finding difficulties and loss of semantic knowledge, for the items themselves as well as for their names.
• Anomia and word-finding difficulties predominate
• Predominantly language affected
• Can mimic a Broca type aphasia or a Wernicke type aphasia

Types

• Progressive non-fluent aphasia (PNFA)
• Semantic dementia (SD)
• Logopenic progressive aphasia (LPA)

Investigations

• FBC, U&E, ESR, CRP, LFT, TFT B12 folate, ECG, CXR
• CT/MRI scan to exclude stroke and shows atrophy in speech areas. It is usually accompanied by asymmetric atrophy of the temporal lobes, worse on the left
• EEG slowing
• PET scan shows decreased activity and reduced blood flow left perisylvian hemisphere and anterior temporal cortex

Management

• Speech and language therapy
• Referral to memory service