Progressive peripheral agraphia

We describe RW, a patient who presented with writing difficulty that deteriorated over time. While her graphemes were typically legible, her writing was extremely slow, and her letters were written in an inconsistent and heterogeneous manner (e.g. each "a" in the word "banana" was produced in a different way). Her mental imagery of letters was impoverished, and she also produced allographic errors in her writing. She had some spelling errors as well, but many of these were due to omissions, perseverations, and motor operations. A positron emission tomography scan demonstrated superior parietal occipital and superior frontal defects that were more evident on the left than the right. Our observations are consistent with the hypothesis that RW has a deficit retrieving physical letter forms as manifested by her heterogeneous and slow production of letter forms. This disruption of grapheme retrieval is associated with interruption of a superior frontal-parietal system in the left hemisphere.     

Progressive Peripheral Agraphia

We describe RW, a patient who presented with writing difficulty that deteriorated over time. While her graphemes were typically legible, her writing was extremely slow, and her letters were written in an inconsistent and heterogeneous manner (e.g. each ‘a’ in the word ‘banana’ was produced in a different way). Her mental imagery of letters was impoverished, and she also produced allographic errors in her writing. She had some spelling errors as well, but many of these were due to omissions, perseverations, and motor operations. A positron emission tomography scan demonstrated superior parietal occipital and superior frontal defects that were more evident on the left than the right. Our observations are consistent with the hypothesis that RW has a deficit retrieving physical letter forms as manifested by her heterogeneous and slow production of letter forms. This disruption of grapheme retrieval is associated with interruption of a superior frontal-parietal system in the left hemisphere.     

Ideational agraphia: a single case study.

A case study investigates the writing, spelling and praxic skills of one patient who was found to have a selective impairment in his ability to write letters and words in the absence of difficulty in copying these same letters and words. He appeared to have difficulty accessing the correct motor programmes or sequences for writing which we term "ideational agraphia" analagous to the syndrome of ideational apraxia. These findings are related to Margolin's information-processing model of writing.     

Writing Kanji without semantics in a case with probable Alzheimer's disease]

We examined a patient (NM) with probable Alzheimer's disease who showed phonologically plausible errors in kanji (logogram) writing. In semantic tasks, she showed no deficits in pointing or naming but had difficulty in more complex tasks such as proverb comprehension. In reading aloud of kanji words, she could read most kanji words correctly and showed little phonologically plausible reading errors. She performed poorly in lexical decision and on-reading of one-letter kanji (Sino-Japanese pronunciation derived from the Chinese language at the time of borrowing). Writing to dictation demonstrated no mistakes in kana letters and words, but many errors in kanji, which were phonologically equivalent but semantically inappropriate. To explore the relationship between the writing errors in kanji words and comprehension of the word meanings, we selected 33 words that she made phonologically plausible writing errors. We gave her the following five tasks using these words; 1) to ask meanings of the words, 2) to dictate the words, 3) to dictate sentences including these words, 4) to discriminate appropriate target words from distracters including her own erroneous responses, and 5) to write these words again. She showed no consistent errors in these tasks. In some occasions, she could write correct kanji words without understanding word meanings. She also showed phonologically plausible writing errors in spite of describing correct word meanings. In Japanese, word meaning deficits like Gogi aphasia were thought to cause phonologically plausible writing errors. As the impairments of word meanings in NM are comparatively mild, the underpinning of her kanji agraphias might be different from that of phonologically plausible errors in Gogi aphasia. It would be suggested that she frequently wrote phonologically equivalent errors because of her lexical deficits in spite that her phonological processing was preserved. Furthermore, she would not necessarily use the semantics (word meanings) of kanji words during dictation.     

Letter selection and letter assembly in acquired dysgraphia.

OBJECTIVE: We explored the constituents of the graphemic buffer in a patient with acquired dysgraphia and tested the hypothesis that the graphemic buffer is composed of 2 dissociable components: letter selection and letter assembly. BACKGROUND: Research on dysgraphia has established the graphemic buffer as a component of the spelling mechanism, and the buffer is considered a short-term memory store that is critical for letter production. However, little is known about the components within the buffer. METHOD: We devised 2 spelling tasks that rely differentially on letter selection and letter assembly. In the selection task, our patient produced the letters that composed a target word, but she did not have to provide serial position information. In the assembly task, B.H. was given all the letters of a target word and was asked to spell the word by arranging the letters in the proper serial order. RESULTS: Compared to spelling to dictation, our patient did not benefit from being given letter identity information (ie, assembly task), but her performance improved significantly when position information was available (ie, selection task). CONCLUSIONS: Based on these data, and the comparison of her performance with another dysgraphic patient, we propose that the graphemic buffer engages in both letter selection and letter assembly.     

A case of motor neuron disease with progressive aphasia and dementia]

We report a 71-year-old woman showing rapidly progressive non-fluent aphasia and dementia accompanied by motor neuron disease (MND). There was no family history of dementia or motor neuron disease. There was 10 months history of dysarthria and dysphagia. On examination, she showed profound difficulty in articulation. Her comprehension was impaired in that she was unable to obey three-stage command. Her written language was also impaired with phonological spelling errors, syntactic errors, and perseveration. Neuroradiological investigations showed atrophic changes and hypoperfusion of left temporal and bilateral parietal region revealed by MRI and SPECT, respectively. Her subsequent decline was rapid. It might be likely that aphasia is much more common in dementia with bulbar MND than is currently recognized because bulbar palsy might mask the language disorder.     

Dysgraphia for letters: a form of motor memory deficit?

A case of pure dysgraphia is presented in which the patient could accurately copy letters which she could not write. The patient did not show any evidence of significant reading or speech impairment or any buccofacial or limb apraxia. Both oral and "block spelling" performance were intact. The writing impairment, which was bilateral, appeared to consist of a memory difficulty for the motor movements associated with letters. The dysgraphia was shown to be specific to letters as the patient was able to transcribe certain numbers and patterns which were similar to letters in their visuospatial complexity. It is suggested that dysgraphia for letters may represent a specific type of motor memory deficit, dissociable from copying skills and the ability to draw letter-like forms.     

Disconnection agraphia in a case of multiple sclerosis: the isolation of letter movement plans from language

We report the case of a left-handed man (MCR), who presented with a peripheral agraphia as an early sign of multiple sclerosis. His left-handed writing was neologistic, whilst oral spelling, typing and spelling with the right hand were intact. Structural MRI scanning revealed a lesion of the body of the corpus callosum. Dichotic listening tests indicated that MCR displayed left hemisphere dominance for language. It is proposed that MCR represents a case of a disconnection syndrome in which right hemisphere systems that provide the basis for movement templates during left-handed writing are isolated from left hemisphere language systems. Analysis of left-handed writing indicated that peripheral movement control was highly structured with both individual letter frequency and sequential dependencies between letters represented within these motor control units. This case represents an opportunity to explore the mechanisms of movement control for writing and to examine the characteristics of isolated letter templates.     

Right-sided neglect influences the writing of Kanji: A case study

Neglect is the failure to report, respond, or orient to novel or meaningful stimuli presented to the side opposite a brain lesion. Here we describe the case of a 73-year-old woman who experienced a writing deficit in the right-sided component of Kanji letters (morphograms) of the Japanese language. Magnetic resonance imaging of the brain showed an infarction in the left middle temporal and occipital gyrus. The patient wrote recognizable, well-formed but paragraphic Kanji letters in both spontaneous and dictated writing. Most letters are composed of two elements: “hen” (left) and “tsukuri” (right). Neographism in tsukuri was her most frequent error. When she was instructed to answer orally hen and tsukuri, she made more mistakes related to tsukuri than to hen. Unilateral spatial neglect in memory or imagery was reported. We concluded that unilaterally disrupted processing of internal representations affected the writing of Kanji letters in this patient.     

A case of pure agraphia due to left parietal lobe infarction]

We reported a case of an 86-year old woman with pure agraphia due to the left parietal lobe infarction. The characteristics of agraphia were as follows. Most errors in Kana and Kanji writing to dictation and copying were no response. She was able to write only numerals from 1 to 12 precisely. Most errors in numerals were substitution. One unrecognizable numeral was found. She succeeded in pointing to nine among ten single Kana letter named by the examiner with the systematic table of the Japanese syllabary, but missed in pointing to Kana words. It took more time for the patient to point to single Kana letter than the control. Magnetic resonance imaging showed a cerebral infarction in the left parietal lobe which included a part of superior parietal lobule and supramarginal gyrus. We classified pure agraphia with parietal lobe infarction into two types in our previous report. In one type (type 1), letters in writing are poorly formed, but the ability to make words with the methods other than writing are reserved. The only deficit of graphic motor pattern could cause Type 1 agraphia. In another type (type 2), letters in writing were well-formed, but spelling with anagram or typing was disturbed. The deficits of writing process other than graphic motor pattern could cause Type 2 agraphia. This typing seems to be effective not only in Kana but also in Kanji. In this report, we investigated the differences of lesion between two types out of some references. Type1 agraphia seems related to lesion of left superior parietal lobule, while Type 2 agraphia seems related to lesion of left supramarginal gyrus. This case had the features of type 2 agraphia at least, and the compatible lesions.     

Spelling errors among children with ADHD symptoms: The role of working memory

Research has shown that children with attention deficit/hyperactivity disorder (ADHD) may present a series of academic difficulties, including spelling errors. Given that correct spelling is supported by the phonological component of working memory (PWM), the present study examined whether or not the spelling difficulties of children with ADHD are emphasized when children's PWM is overloaded. A group of 19 children with ADHD symptoms (between 8 and 11 years of age), and a group of typically developing children matched for age, schooling, gender, rated intellectual abilities, and socioeconomic status, were administered two dictation texts: one under typical conditions and one under a pre-load condition that required the participants to remember a series of digits while writing. The results confirmed that children with ADHD symptoms have spelling difficulties, produce a higher percentages of errors compared to the control group children, and that these difficulties are enhanced under a higher load of PWM. An analysis of errors showed that this holds true, especially for phonological errors. The increased errors in the PWM condition was not due to a tradeoff between working memory and writing, as children with ADHD also performed more poorly in the PWM task. The theoretical and practical implications are discussed.     

Remediation of written language

Written communication involves the linking of ideas and words to their appropriate spellings, and then moving the hand to actually write the desired words. Acquired impairments of writing are referred to as agraphia and can result from damage to various stages of the writing process. These impairments may reflect degraded knowledge about spelling and the correspondences between sounds and letters or difficulty with the selection and formation of letters. Characteristic features of agraphia syndromes are reviewed in this article, along with example approaches to remediation. The treatments variously serve to strengthen weakened abilities, take advantage of residual skills, and develop compensatory strategies.     

Spelling dyslexia: a deficit of the visual word-form.

A patient with spelling dyslexia read both words and text accurately but slowly and laboriously letter by letter. Her performance on a test of lexical decision was slow. She had great difficulty in detecting a 'rogue' letter attached to the beginning or end of a word--for example, ksong--or in parsing two unspaced words, such as applepeach. By contrast she was immune to the effects of interpolating extraneous coloured letters in a word, a manipulation that affects normal readers. Therefore it is argued that this patient had damage to an early stage in the reading process, to the visual word form itself.     

Where do perseverations come from?

RB, a patient with probable Alzheimer's disease, makes continuous perseverations of single letters when writing (e.g. fruit-->fruuit), particularly on high frequency letters. An analysis of her errors reveals that her perseverations do not reflect letter substitutions or transpositions, nor do they suggest difficulty with geminates. No continuous perseverations were found in oral production, in graphic and simple motor tasks, and in oral spelling. RB's data do not support an attention deficit as the basis of her continuous perseverations. It is proposed that a deficit at the level of abstract letter representations is the source of RB's perseverations. The implications of this conclusion for accounts of perseveration and of spelling models are discussed.     

Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome]

A case of unusual Landau-Kleffner syndrome was reported. She was an 8 year-old girl and showed non-verbal agnosia, diffuse EEG abnormalities and convulsions. Her responses to both verbal and non-verbal sounds remained inconsistent and unstable. When a continuous spike-wave complexes on EEG was detected, she paid no attention to any sound in spite of her fair consciousness. Auditory brainstem response and magnetic resonance imaging of her brain were normal. Auditory agnosia was correlated well with EEG abnormalities, and valproic acid and clonazepam were effective for EEG improvement. After the EEG improvement, clinical responses to sounds recovered well; firstly she could pay attention to sounds and then she could distinguish between verbal and non-verbal sounds. Finally, she could speak a few words after the learning letters.     

An epilepsy case presenting temporal aphonia and prosodic deficit with left hemiparesis]

A 53-year-old right-handed woman was admitted to a hospital with a seizure, exhibiting loss of consciousness and paroxysmal myoclonic movement. MRI showed no abnormalities of her brain. Electroencephalography revealed sporadic sharp and slow wave complexes, starting from the right central region. Cerebral perfusion SPECT revealed increased blood supply in the right hemisphere. She received diagnosis of epilepsy and was treated with phenytoin. After she regained her consciousness, she had no voice and presented with left hemiparesis which was interpreted to be Todd's palsy. She gradually recovered in using her voice, to full normalization of affective prosody in 11 days. The left hemiparesis recovered first in the upper and later in the lower extremities. Her symptoms could be interpreted to be vocal and prosodic disturbance, but not aphasia, because she could understand speach and communicate with writing. The lesions responsible for prosodic deficit are still controversial. The symptoms observed and the findings obtained may indicate that the deficiency of the affective prosody may be caused by the dysfunction of the medial surface of the non-dominant frontal lobe in this case.