Primary progressive aphasia

Primary progressive aphasia (PPA) is a group of disorders characterized by progressive language and speech difficulties.^[1] It was first described as a distinct syndrome by M.-Marsel Mesulam in 1982.^[2] Primary Progressive Aphasias have a clinical and pathological overlap with the Frontotemporal Lobar Degeneration (FTLD) spectrum of disorders and Alzheimer's disease.

Classification

Three classifications of primary progressive aphasia have been described.^[3][4][5] In the classical Mesulam criteria for primary progressive aphasia, there are 2 variants: a non-fluent type Progressive Nonfluent Aphasia (PNFA) and a fluent type Semantic Dementia (SD).^[1][6] A third variant of primary progressive aphasia, Logopenic Progressive Aphasia (LPA)^[7] is an atypical form of Alzheimer's disease.

Diagnostic criteria

The following diagnosis criteria were defined by Mesulam ^[8]

• 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

Risk Factors

There are no known environmental risk factors for the progressive aphasias. However, one observational, retrospective study suggested that vasectomy could be a risk factor for PPA in men.^[9] These results have yet to be replicated or demonstrated by prospective studies.

PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration, including PPA, are at slightly greater risk of developing PPA or another form of the condition.^[10]

Treatment

There is no approved treatment. 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.^[11] 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.

See also

References

Further reading

• Amici S, Ogar J, Brambati SM et al. (Dec 2007). "Performance in specific language tasks correlates with regional volume changes in progressive aphasia". Cognitive & Behavioral Neurology 20 (4): 203–11. doi:10.1097/WNN.0b013e31815e6265. PMID 18091068.  CS1 maint: Explicit use of et al. (link)
• Gliebus G (March 2010). "Primary progressive aphasia: clinical, imaging, and neuropathological findings". Am J Alzheimers Dis Other Demen 25 (2): 125–7. doi:10.1177/1533317509356691. PMID 20124255. 
• Henry ML, Beeson PM, Alexander GE, Rapcsak SZ (February 2012). "Written language impairments in primary progressive aphasia: a reflection of damage to central semantic and phonological processes". J Cogn Neurosci 24 (2): 261–75. doi:10.1162/jocn_a_00153. PMC 3307525. PMID 22004048. 
• Henry ML, Gorno-Tempini ML (December 2010). "The logopenic variant of primary progressive aphasia". Current Opinion in Neurology 23 (6): 633–7. doi:10.1097/WCO.0b013e32833fb93e. PMC 3201824. PMID 20852419. 
• Reilly J, Rodriguez AD, Lamy M, Neils-Strunjas J (2010). "Cognition, language, and clinical pathological features of non-Alzheimer's dementias: an overview". J Commun Disord 43 (5): 438–52. doi:10.1016/j.jcomdis.2010.04.011. PMC 2922444. PMID 20493496. 
• Rohrer JD, Knight WD, Warren JE, Fox NC, Rossor MN, Warren JD (January 2008). "Word-finding difficulty: a clinical analysis of the progressive aphasias". Brain 131 (Pt 1): 8–38. doi:10.1093/brain/awm251. PMC 2373641. PMID 17947337. 

External links

• FAQ on PPA from IMPPACT, the International PPA Connection
• PPA information from the UCSF Memory and Aging Center
• Northwestern Cognitive Neurology and Alzheimer's Disease Center

Topics related to Primary progressive aphasia Pathology of the nervous system, primarily CNS (G04–G47, 323–349) Inflammation Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic Spinal cord Myelitis: Poliomyelitis Demyelinating disease Transverse myelitis Tropical spastic paraparesis Epidural abscess Both/either Encephalomyelitis Acute disseminated Meningoencephalitis Brain/ encephalopathy Degenerative Extrapyramidal and movement disorders Basal ganglia disease Parkinsonism PD Postencephalitic NMS PKAN Tauopathy PSP Striatonigral degeneration Hemiballismus HD OA Dyskinesia Dystonia Status dystonicus Spasmodic torticollis Meige's Blepharospasm Athetosis Chorea Choreoathetosis Myoclonus Myoclonic epilepsy Akathisia Tremor Essential tremor Intention tremor Restless legs Stiff person Dementia Tauopathy Alzheimer's Early-onset Primary progressive aphasia Frontotemporal 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