Aphasia
- Author: Howard S Kirshner, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA more...
Background
Aphasia is an acquired disorder of language due to brain damage. Aphasia does not include (1) developmental disorders of language, often called dysphasia in the United States; (2) purely motor speech disorders, limited to articulation of speech via the oral-motor apparatus, referred to as stuttering, dysarthria, and apraxia of speech; or (3) disorders of language that are secondary to primary thought disorders, such as schizophrenia.
Encompassed under the term aphasia are selective, acquired disorders of reading (alexia) or writing (agraphia). Closely related to aphasia are the family of disorders called apraxias (disorders of learned or skilled movements), agnosias (disorders of recognition), acalculias (disorders of calculation ability), and more global neurobehavioral deficits such as dementia and delirium. Such related syndromes may coexist with aphasia or exist independently.
Pathophysiology
Aphasia may occur secondary to brain injury or degeneration and involves the left cerebral hemisphere to a greater extent than the right. Language function lateralizes to the left hemisphere in 96-99% of right-handed people and 60% of left-handed people. Of the remaining left-handed people, about one half have mixed hemisphere language dominance, and about one half have right hemisphere dominance. Left-handed individuals may develop aphasia after a lesion of either hemisphere, but the syndromes from left hemisphere injury may be milder or more selective than those seen in right-handed people.
Most aphasias and related disorders are due to stroke, head injury, cerebral tumors, or degenerative diseases. The neuroanatomic substrate of language comprehension and production is complex, including auditory input and language decoding in the superior temporal lobe, analysis in the parietal lobe, and expression in the frontal lobe, descending via the corticobulbar tracts to the internal capsule and brainstem, with modulatory effects of the basal ganglia and the cerebellum.
Aphasia syndromes have been described based on patterns of abnormal language expression, repetition, and comprehension. These classical syndromes have been roughly correlated with specific left hemisphere locations, though clear overlaps and individual differences make the aphasia syndromes limited in specificity. Patients may lose the ability to produce speech, to comprehend speech, to repeat, and to hear and read words in many nuanced ways. Classical aphasia syndromes (see Aphasia syndromes in History) include global, Broca, Wernicke, and conduction aphasia, as well as transcortical motor, transcortical sensory, and transcortical mixed aphasia. Pure alexia and optic aphasia are often discussed with the classical aphasias.
Language function can be parsed in several important ways other than assignment to the classical aphasia syndromes. A variety of types of evidence have noted that certain specific language functions (such as naming pictures) activate widespread neural networks involving many parts of both hemispheres of the brain. Producing, receiving, and interpreting speech requires specific and distinct cognitive processes such as phonologic decoding and encoding, orthographic decoding and encoding (for reading), lexical access, lexical-semantic representations of words, and semantic interpretation of language. Differentiation of these processes involves testing patients with different aphasia types and attempting to find double dissociations among groups of patients to determine the neurologic basis of specific cognitive processes.
The lesion method, the principal source of information about aphasia from autopsy studies in the 19th and early to mid 20th centuries and from brain imaging modalities since the 1970s, remains a useful source of information. However, it has been abetted by cortical stimulation studies, mainly in patients with epilepsy, and functional neuroimaging, such as fMRI and PET scanning often carried out during language testing in healthy individuals, to determine the language function of specific areas of the brain.
Epidemiology
Frequency
United States
Data on incidence of aphasia in the United States are limited. Aphasia occurs in a variety of cerebrovascular, traumatic, and degenerative conditions. Stroke is likely the most common cause of aphasia, and it has been estimated that about 20% of stroke patients develop aphasia. More than 700,000 strokes occur in the United States each year, and approximately 170,000 new cases of aphasia every year are related to stroke. The number of patients with language disorders secondary to traumatic brain injury, brain tumors, and other brain lesions such as arteriovenous malformations is not precisely known. Patients with neurodegenerative disorders such as Alzheimer disease and frontotemporal dementia frequently manifest language deficits. The prevalence of Alzheimer disease in the United States is approximately 5 million cases.
Mortality/Morbidity
Aphasia is a condition, not a disease; therefore, it has no attributable mortality rate.
Race
No reliable data exist on the incidence of aphasia in different racial groups. Within disease entities, however, such differences are well known. In stroke, for example, African Americans have almost a 2-fold higher incidence as compared with whites. In addition, specific types of stroke, such as cerebral hemorrhage, lacunar infarctions, and intracranial artery stenoses, are known to be more common in African Americans than Caucasians. One might therefore surmise that poststroke aphasias would be more common in African Americans.
Sex
Not enough data are available to evaluate differences in the incidence and clinical features of aphasia in men and women. Some studies suggest a lower incidence of aphasia in women because they may have more bilaterality of language function. Differences may also exist in aphasia type, with more women than men developing Wernicke aphasia.
Age
Age may be an important factor in recovery. Some studies suggest that recovery from aphasia due to a stroke is less favorable in patients older than age 70 than in younger patients. However, at any age, recovery of various degrees can occur, even at times remote from the brain injury.
Conroy P, Scowcroft J. Decreasing cues for a dynamic list of noun and verb naming targets: A case-series aphasia therapy study. Neuropsychol Rehabil. Jan 16 2012;[Medline].
Alexander MP, Naeser MA, Palumbo C. Broca's area aphasias: aphasia after lesions including the frontal operculum. Neurology. Feb 1990;40(2):353-62. [Medline].
Kreisler A, Godefroy O, Delmaire C, et al. The anatomy of aphasia revisited. Neurology. Mar 14 2000;54(5):1117-23. [Medline].
Naeser MA, Helm-Estabrooks N, Haas G, et al. Relationship between lesion extent in 'Wernicke's area' on computed tomographic scan and predicting recovery of comprehension in Wernicke's aphasia. Arch Neurol. Jan 1987;44(1):73-82. [Medline].
Hillis AE, Gold L, Kannan V, et al. Site of the ischemic penumbra as a predictor of potential for recovery of functions. Neurology. Jul 15 2008;71(3):184-9. [Medline].
Caramazza A. Minding the facts: a comment on Thompson-Schill et al.'s "A neural basis for category and modality specificity of semantic knowledge". Neuropsychologia. 2000;38(7):944-9. [Medline].
Geschwind N. Disconnexion syndromes in animals and man. I. Brain. Jun 1965;88(2):237-94. [Medline].
Damasio AR, Damasio H. The anatomic basis of pure alexia. Neurology. Dec 1983;33(12):1573-83. [Medline].
Naeser MA, Alexander MP, Helm-Estabrooks N, et al. Aphasia with predominantly subcortical lesion sites: description of three capsular/putaminal aphasia syndromes. Arch Neurol. Jan 1982;39(1):2-14. [Medline].
Fromm D, Holland AL, Swindell CS, et al. Various consequences of subcortical stroke. Prospective study of 16 consecutive cases. Arch Neurol. Oct 1985;42(10):943-50. [Medline].
Alexander MP, Naeser MA, Palumbo CL. Correlations of subcortical CT lesion sites and aphasia profiles. Brain. Aug 1987;110 (Pt 4):961-91. [Medline].
Weiller C, Willmes K, Reiche W, et al. The case of aphasia or neglect after striatocapsular infarction. Brain. Dec 1993;116 (Pt 6):1509-25. [Medline].
Crosson B. Subcortical Functions in Language and Memory. New York, NY: Guilford; 1992.
Dejerine J. Contribution a l'etude anatomo-pathologique et clinique des differentes varieties de cecite verbales. Memoires Societe Biologie. 1892;4:61-90.
Benton AL. The fiction of the Gerstmann syndrome. J Neurol Neurosurg Psychiatry. 1961;24:961-991.
Benton AL. Gerstmann's syndrome. Arch Neurol. May 1992;49(5):445-7. [Medline].
Thompson CK, Cho S, Price C, Wieneke C, Bonakdarpour B, Rogalski E, et al. Semantic interference during object naming in agrammatic and logopenic primary progressive aphasia (PPA). Brain Lang. Jan 12 2012;[Medline].
Lidzba K, Staudt M, Zieske F, Schwilling E, Ackermann H. Prestroke/poststroke fMRI in aphasia: Perilesional hemodynamic activation and language recovery. Neurology. Jan 11 2012;[Medline].
Berthier ML, Green C, Lara JP, Higueras C, Barbancho MA, Dávila G, et al. Memantine and constraint-induced aphasia therapy in chronic poststroke aphasia. Ann Neurol. May 2009;65(5):577-85. [Medline].
Alexander MP, Benson DF, Stuss DT. Frontal lobes and language. Brain Lang. Nov 1989;37(4):656-91. [Medline].
Alexander MP, Hiltbrunner B, Fischer RS. Distributed anatomy of transcortical sensory aphasia. Arch Neurol. Aug 1989;46(8):885-92. [Medline].
Appell J, Kertesz A, Fisman M. A study of language functioning in Alzheimer patients. Brain Lang. Sep 1982;17(1):73-91. [Medline].
Auerbach SH, Allard T, Naeser M, et al. Pure word deafness. Analysis of a case with bilateral lesions and a defect at the prephonemic level. Brain. Jun 1982;105:271-300. [Medline].
Bakar M, Kirshner HS, Wertz RT. Crossed aphasia. Functional brain imaging with PET or SPECT. Arch Neurol. Oct 1996;53(10):1026-32. [Medline].
Benson DF, Ardila A. Aphasia. A clinical perspective. 1. First ed. New York: Oxford University Press; 1996:1-441.
Benton AL, Hamsher K De S, Varney NR, et al. Contributions to Neuropsychological Assessment. New York: Oxford University Press; 1983.
Benton AL, Hamsher KD. Multilingual Aphasia Examination. Iowa City, IA: AJA Associates; 1989.
Bhogal SK, Teasell R, Speechley M. Intensity of aphasia therapy, impact on recovery. Stroke. Apr 2003;34(4):987-93. [Medline].
Boatman D, Gordon B, Hart J, et al. Transcortical sensory aphasia: revisited and revised. Brain. Aug 2000;123 (Pt 8):1634-42. [Medline].
Bookheimer S. Functional MRI of language: new approaches to understanding the cortical organization of semantic processing. Annu Rev Neurosci. 2002;25:151-88. [Medline].
Bowers D, Bauer RM, Heilman KM. The nonverbal affect lexicon: theoretical perspectives from neuropsychological studies of affect perception. Neuropsychology. 1993;7:433-44.
Broca P. Remarques sur le siege de la faculte du language articule, suivies d'une observation d'aphemie. Bull Soc Anat Paris. 1861;2:330-57.
Catani M, Jones DK, ffytche DH. Perisylvian language networks of the human brain. Ann Neurol. Jan 2005;57(1):8-16. [Medline].
Catani M, Mesulam M. The arcuate fasciculus and the disconnection theme in language and aphasia: history and current state. Cortex. Sep 2008;44(8):953-61. [Medline].
Cummings JL, Benson F, Hill MA, et al. Aphasia in dementia of the Alzheimer type. Neurology. Mar 1985;35(3):394-7. [Medline].
Damasio AR. Aphasia. N Engl J Med. Feb 20 1992;326(8):531-9. [Medline].
de Boissezon X, Peran P, de Boysson C, et al. Pharmacotherapy of aphasia: myth or reality?. Brain Lang. Jul 2007;102(1):114-25. [Medline].
De Renzi E, Vignolo LA. The token test: A sensitive test to detect receptive disturbances in aphasics. Brain. Dec 1962;85:665-78. [Medline].
DeWitt LD, Grek AJ, Buonanno FS, et al. MRI and the study of aphasia. Neurology. Jun 1985;35(6):861-5. [Medline].
Freedman M, Alexander MP, Naeser MA. Anatomic basis of transcortical motor aphasia. Neurology. Apr 1984;34(4):409-17. [Medline].
Freund CS. Uber optische Aphasie und Seelenblindheit. Archiv Psychiatrie Nervenkrankheiten. 1889;20:276-97.
Goodglass H, Kaplan E. The Assessment of Aphasia and Related Disorders. Philadelphia, PA: Lea and Febiger; 1972.
Goodglass H, Wingfield A, Hyde MR, et al. Category specific dissociations in naming and recognition by aphasic patients. Cortex. Mar 1986;22(1):87-102. [Medline].
Hall DA, Anderson CA, Filley CM, et al. A French accent after corpus callosum infarct. Neurology. May 13 2003;60(9):1551-2. [Medline].
Hillis AE. Aphasia: progress in the last quarter of a century. Neurology. Jul 10 2007;69(2):200-13. [Medline].
Jacobs DH, Shuren J, Gold M, et al. Physostigmine pharmacotherapy for anomia. Neurocase. 1996;2:83-92.
Kaplan E, Goodglass H, Weintraub S. The Boston Naming Test. Philadelphia, PA: Lea and Febiger; 1978.
Kertesz A. Western Aphasia Battery. London, Ontario: University of Western Ontario Press; 1980.
Kertesz A, Sheppard A. The epidemiology of aphasic and cognitive impairment in stroke: age, sex, aphasia type and laterality differences. Brain. Mar 1981;104:117-28. [Medline].
Kirshner HS. Behavioral neurology. Practical science of mind and brain. 1. 2. Boston. Butterworth Heinemann. 2002;1-474.
Kirshner HS. 1-532. In: Handbook of neurological speech and language disorders. First ed. New York: Marcel Dekker, Inc; 1995.
Kirshner HS, Alexander M, Lorch MP, et al. Continuum: Disorders of speech and language. Baltimore MD: Lippincott Williams & Wilkins CONTINUUM.; 1999.
Kirshner HS, Casey PF, Henson J, et al. Behavioural features and lesion localization in Wernicke's aphasia. Aphasiology. 1989;3:169-176.
Kirshner HS, Hughes T, Fakhoury T, et al. Aphasia secondary to partial status epilepticus of the basal temporal language area. Neurology. Aug 1995;45(8):1616-8. [Medline].
Kirshner HS, Kistler KH. Aphasia after right thalamic hemorrhage. Arch Neurol. Oct 1982;39(10):667-9. [Medline].
Kirshner HS, Tanridag O, Thurman L, et al. Progressive aphasia without dementia: two cases with focal spongiform degeneration. Ann Neurol. Oct 1987;22(4):527-32. [Medline].
Lazar RM, Antoniello D. Variability in recovery from aphasia. Curr Neurol Neurosci Rep. Nov 2008;8(6):497-502. [Medline].
Love T, Swinney D, Walenski M, et al. How left inferior frontal cortex participates in syntactic processing: Evidence from aphasia. Brain Lang. Dec 2008;107(3):203-19. [Medline].
Mariën P, Paghera B, De Deyn PP, et al. Adult crossed aphasia in dextrals revisited. Cortex. Feb 2004;40(1):41-74. [Medline].
Martin PI, Naeser MA, Theoret H, et al. Transcranial magnetic stimulation as a complementary treatment for aphasia. Semin Speech Lang. May 2004;25(2):181-91. [Medline].
Mega MS, Alexander MP. Subcortical aphasia: the core profile of capsulostriatal infarction. Neurology. Oct 1994;44(10):1824-9. [Medline].
Mesulam MM. Primary progressive aphasia. Ann Neurol. Apr 2001;49(4):425-32. [Medline].
Morris HH, Luders H, Lesser RP, et al. Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation. Neurology. Jul 1984;34(7):877-83. [Medline].
Nicholas M, Obler L, Albert M, et al. Lexical retrieval in healthy aging. Cortex. Dec 1985;21(4):595-606. [Medline].
Nishio S, Takemura N, Ikai Y, et al. Sensory aphasia after closed head injury. J Clin Neurosci. May 2004;11(4):442-4. [Medline].
Ojemann G, Ojemann J, Lettich E, et al. Cortical language localization in left, dominant hemisphere. An electrical stimulation mapping investigation in 117 patients. 1989. J Neurosurg. Feb 2008;108(2):411-21. [Medline].
Price CJ, Wise RJ, Watson JD, et al. Brain activity during reading. The effects of exposure duration and task. Brain. Dec 1994;117 (Pt 6):1255-69. [Medline].
Robey RR. A meta-analysis of clinical outcomes in the treatment of aphasia. J Speech Lang Hear Res. Feb 1998;41(1):172-87. [Medline].
Schiff HB, Alexander MP, Naeser MA, et al. Aphemia. Clinical-anatomic correlations. Arch Neurol. Nov 1983;40(12):720-7. [Medline].
Shuren JE, Hammond CS, Maher LM, et al. Attention and anosognosia: the case of a jargonaphasic patient with unawareness of language deficit. Neurology. Feb 1995;45(2):376-8. [Medline].
Tanridag O, Kirshner HS. Language disorders in stroke syndromes of the dominant capsulostriatum-- a clinical review. Aphasiology. 1987;1:107-117.
Thompson C. Functional neuroimaging: applications for studying aphasia. In: LaPointe LL eds. Aphasia and related neurogenic language disorders. 3rd ed. New York: Thieme; 2005:19-39.
Thompson CK, den Ouden DB. Neuroimaging and recovery of language in aphasia. Curr Neurol Neurosci Rep. Nov 2008;8(6):475-83. [Medline].
Wertz RT, Weiss DG, Aten JL, et al. Comparison of clinic, home, and deferred language treatment for aphasia. A Veterans Administration Cooperative Study. Arch Neurol. Jul 1986;43(7):653-8. [Medline].
Yang ZH, Zhao XQ, Wang CX, et al. Neuroanatomic correlation of the post-stroke aphasias studied with imaging. Neurol Res. May 2008;30(4):356-60. [Medline].

