Agraphia is an acquired neurological disorder causing a loss in the ability to communicate through writing, either due to some form of motor dysfunction or an inability to spell. The loss of writing ability may present with other language or neurological disorders; disorders appearing commonly with agraphia are alexia, aphasia, dysarthria, agnosia, acalculia and apraxia. The study of individuals with agraphia may provide more information about the pathways involved in writing, both language related and motoric. Agraphia cannot be directly treated, but individuals can learn techniques to help regain and rehabilitate some of their previous writing abilities. These techniques differ depending on the type of agraphia.
Agraphia can be broadly divided into central and peripheral categories. Central agraphias typically involve language areas of the brain, causing difficulty spelling or with spontaneous communication, and are often accompanied by other language disorders. Peripheral agraphias usually target motor and visuospatial skills in addition to language and tend to involve motoric areas of the brain, causing difficulty in the movements associated with writing. Central agraphia may also be called aphasic agraphia as it involves areas of the brain whose major functions are connected to language and writing; peripheral agraphia may also be called nonaphasic agraphia as it involves areas of the brain whose functions are not directly connected to language and writing (typically motor areas).
The history of agraphia dates to the mid-fourteenth century, but it was not until the second half of the nineteenth century that it sparked significant clinical interest. Research in the twentieth century focused primarily on aphasiology in patients with lesions from strokes.
Characteristics Agraphia or impairment in producing written language can occur in many ways and many forms because writing involves many cognitive processes (language processing, spelling, visual perception, visuospatial orientation for graphic symbols, motor planning, and motor control of handwriting).
Agraphia has two main subgroupings: central ("aphasic") agraphia and peripheral ("nonaphasic") agraphia. Central agraphias include lexical, phonological, deep, and semantic agraphia. Peripheral agraphias include allographic, apraxic, motor execution, hemianoptic and afferent agraphia.
Central Central agraphia occurs when there are both impairments in spoken language and impairments to the various motor and visualization skills involved in writing. Individuals who have agraphia with fluent aphasia write a normal quantity of well-formed letters, but lack the ability to write meaningful words. Receptive aphasia is an example of fluent aphasia. Those who have agraphia with nonfluent aphasia can write brief sentences, but their writing is difficult to read. Their writing requires great physical effort, lacks proper syntax, and often has poor spelling. Expressive aphasia is an example of nonfluent aphasia. Individuals who have alexia with agraphia have difficulty with both the production and comprehension of written language. This form of agraphia does not impair spoken language. Deep agraphia affects an individual's phonological ability and orthographic memory. Deep agraphia is often the result of a lesion involving the left parietal region (supramarginal gyrus or insula). Individuals can neither remember how words look when spelled correctly, nor sound them out to determine spelling. Individuals typically rely on their damaged orthographic memory to spell; this results in frequent errors, usually semantic in nature. Individuals have more difficulty with abstract concepts and uncommon words. Reading and spoken language are often impaired as well. Gerstmann syndrome agraphia is the impairment of written language production associated with the following structural symptoms: difficulty discriminating between one's own fingers, difficulty distinguishing left from right, and difficulty performing calculations. All four of these symptoms result from pathway lesions. Gerstmann's syndrome may additionally be present with alexia and mild aphasia. Global agraphia also impairs an individual's orthographic memory although to a greater extent than deep agraphia. In global apraxia, spelling knowledge is lost to such a degree that the individual can only write very few meaningful words, or cannot write any words at all. Reading and spoken language are also markedly impaired. Lexical and structural agraphia are caused by damage to the orthographic memory; these individuals cannot visualize the spelling of a word, though they do retain the ability to sound them out. This impaired spelling memory can imply the loss or degradation of the knowledge or just an inability to efficiently access it. There is a regularity effect associated with lexical agraphia in that individuals are less likely to correctly spell words without regular, predictable spellings. Additionally, spelling ability tends to be less impaired for common words. Individuals also have difficulty with homophones. Language competence in terms of grammar and sentence writing tends to be preserved. Phonological agraphia is the opposite of lexical agraphia in that the ability to sound out words is impaired, but the orthographical memory of words may be intact. It is associated with a lexicality effect by a difference in the ability to spell words versus nonwords; individuals with this form of agraphia are depending on their orthographic memory. Additionally, it is often harder for these individuals to access more abstract words without strong semantic representations (i.e., it is more difficult for them to spell prepositions than concrete nouns). Pure agraphia is the impairment in written language production without any other language or cognitive disorder.
Agraphia can occur separately or co-occur and can be caused by damage to the angular gyrus.
Peripheral Peripheral agraphias occurs when there is damage to the various motor and visualization skills involved in writing. Apraxic agraphia is the impairment in written language production associated with disruption of the motor system. It results in distorted, slow, effortful, incomplete, and/or imprecise letter formation. Though written letters are often so poorly formed that they are almost illegible, the ability to spell aloud is often retained. This form of agraphia is caused specifically by a loss of specialized motor plans for the formation of letters and not by any dysfunction affecting the writing hand. Apraxic agraphia may present with or without ideomotor apraxia. Paralysis, chorea, Parkinson's disease (micrographia), and dystonia (writer's cramp) are motor disorders commonly associated with agraphia. Hysterical agraphia is the impairment in written language production caused by a conversion disorder. Reiterative agraphia is found in individuals who repeat letters, words, or phrases in written language production an abnormal number of times. Perseveration, paragraphia, and echographia are examples of reiterative agraphia. Visuospatial agraphia is the impairment in written language production defined by a tendency to neglect one portion (often an entire side) of the writing page, slanting lines upward or downward, and abnormal spacing between letters, syllables, and words. The orientation and correct sequencing of the writing will also be impaired. Visuospatial agraphia is frequently associated with left hemispatial neglect, difficulty in building or assembling objects, and other spatial difficulties.
Causes
Agraphia has a multitude of causes ranging from strokes, lesions, traumatic brain injury, and dementia. Twelve regions of the brain are associated with handwriting. The four distinct functional areas are the left superior frontal area composed of the middle frontal gyrus and the superior frontal sulcus, the left superior parietal area composed of the inferior parietal lobule, the superior parietal lobule and the intraparietal sulcus and lastly the primary motor cortex and the somatosensory cortex. The eight other areas are considered associative areas and are the right anterior cerebellum, the left posterior nucleus of the thalamus, the left inferior frontal gyrus, the right posterior cerebellum, the right superior frontal cortex, the right inferior parietal lobule, the left fusiform gyrus and the left putamen. The specific type of agraphia resulting from brain damage will depend on which area of the brain was damaged.
Phonological agraphia is linked to damage in areas of the brain involved in phonological processing skills (sounding out words), specifically the language areas around the sylvian fissure, such as Broca's area, Wernicke's area, and the supramarginal gyrus.
Lexical agraphia is associated with damage to the left angular gyrus and/or posterior temporal cortex. The damage is typically posterior and inferior to the perisylvian language areas.
Deep agraphia involves damage to the same areas of the brain as lexical agraphia plus some damage to the perisylvian language areas as well. More extensive left hemisphere damage can lead to global agraphia.
Gerstmann's syndrome is caused by a lesion of the dominant (usually the left) parietal lobe, usually an angular gyrus lesion.
Apraxic agraphia with ideomotor apraxia is typically caused by damage to the superior parietal lobe (where graphomotor plans are stored) or the premotor cortex (where the plans are converted into motor commands). Additionally, some individuals with cerebellar lesions (more typically associated with non-apraxic motor dysfunction) develop apraxic agraphia. Apraxic agraphia without ideomotor apraxia may be caused by damage to either of the parietal lobes, the dominant frontal lobe, or to the dominant thalamus.
Visuospatial agraphia typically has a right hemisphere pathology. Damage to the right frontal area of the brain may cause more motor defects, whereas damage to the posterior part of the right hemisphere leads predominantly to spatial defects in writing.
Alzheimer's disease
Agraphia is often seen in association with Alzheimer's disease (AD). Writing disorders can be an early manifestation of AD. In individuals with AD, the first sign pertaining to writing skills is the selective syntactic simplification of their writing. Individuals will write with less description, detail and complexity, and other markers, such as grammatical errors, may emerge. Different agraphias may develop as AD progresses. In the beginning stages of AD, individuals show signs of allographic agraphia and apraxic agraphia. Allographic agraphia is represented in AD individuals by the mixing of lower and upper case letters in words; apraxic agraphia is represented in AD patients through poorly constructed or illegible letters and omission or over repetition of letter strokes. As their AD progresses, so does the severity of their agraphia; they may begin to form spatial agraphia, which is the inability to write in a straight horizontal line, and there are often unnecessary gaps between letters and words.