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
- Definition: Primary Progressive Aphasia is a form of dementia that predominantly affects the brain's language centers 🗣️, leading to a gradual decline in language abilities.
- Classification: A subtype of Frontotemporal Dementia (FTD) 🧩, which primarily impacts the frontal and temporal lobes.
- Genetic Factors: Some forms are inherited in an autosomal dominant manner 🧬.
- Genetic Mutation: Pathogenic variants in the GRN gene (Progranulin) are commonly associated, reducing progranulin levels needed for neuronal survival.
🩺 Clinical Features
- 🔎 Word-Finding Difficulties: Trouble naming unfamiliar items; worsens with time.
- 📛 Anomia: Difficulty naming objects → frequent pauses and hesitations.
- 🗨️ Language Predominance: Speaking, understanding, reading, and writing progressively impaired.
- Types of Aphasia Mimicked:
- 🧩 Broca's Type: Non-fluent, effortful speech with relatively preserved comprehension.
- 🧩 Wernicke's Type: Fluent but nonsensical speech with impaired comprehension.
- 📉 Progression: Worsening communication → affects social interaction and daily life.
🔄 Types
- 🗨️ Progressive Non-Fluent Aphasia (PNFA): Effortful, non-fluent speech + agrammatism.
- 📘 Semantic Dementia (SD): Loss of word meaning + object recognition difficulties.
- 🔤 Logopenic Progressive Aphasia (LPA): Impaired word retrieval + sentence repetition, but preserved grammar.
🔬 Investigations
- 🧪 Blood Tests: FBC, U&E, ESR, CRP, LFTs, TFTs, B12/Folate, ECG, CXR.
- 🧠 Neuroimaging:
- CT/MRI → Asymmetric temporal lobe atrophy (L > R).
- PET → ↓ metabolism in left perisylvian + anterior temporal cortex.
- 📉 EEG: May show generalized slowing.
- 🧾 Other tests as indicated to rule out alternative diagnoses.
💊 Management
- 🗣️ Speech & Language Therapy: Key for communication strategies.
- 🧑⚕️ Referral to Memory Services: Comprehensive care + cognitive assessments.
- 🤝 Supportive Care: Psychological support, caregiver education, daily living assistance.
- 💊 Pharmacological: No specific drugs; some dementia meds may help symptomatically.
- 📈 Regular Monitoring: Track progression + adjust care plans.
📚 References
- Rascovsky, K., Hodges, J. R., Knopman, D., et al. (2011). Semantic variant primary progressive aphasia and nonfluent variant primary progressive aphasia: Distinct patterns of white matter changes. Brain, 134(5), 1443-1454.
- Mackenzie, I. R., Mackenzie, W., Rosen, H. J., et al. (2011). Consensus paper: Frontotemporal dementia genetics. Acta Neuropathologica, 122(2), 231-245.
- Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., et al. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 1006-1014.
- Rascovsky, K., Hodges, J. R., Knopman, D., et al. (2011). Consensus criteria for the diagnosis of frontotemporal dementia. Neurology, 76(3), 335-346.
