Seizures, dyslexia, and dysgraphia of psychogenic origin

Dyslexia and dysgraphia of various kinds are recognized sequelae of cerebral lesions. Although refusal to read or write may occur in a number of psychiatric disorders, to our knowledge dyslexia and dysgraphia as the sole manifestations of a psychogenic disorder have not previously been reported. Our patient had psychogenic symptoms, initially including seizures but subsequently consisting solely of dyslexia and dysgraphia.     

Progressive aphasia with Lewy bodies

Dementia with Lewy bodies (DLB) may include both Alzheimer and Lewy body pathology, but has never been reported to cause primary progressive aphasia. We report a 69-year-old woman who died 11 years after presenting with the syndrome of progressive aphasia. Six years after aphasia onset she developed visual hallucinations, and subsequently parkinsonism. Autopsy examination revealed Alzheimer's disease (AD), cortical Lewy bodies, and depigmentation and Lewy bodies in the substantia nigra and locus ceruleus. The aphasia most likely reflected the initial onset of AD, and the psychosis and parkinsonism most likely reflected the subsequent onset of Lewy body pathology. This first reported case of progressive aphasia occurring within the context of AD and Lewy body pathology uniquely illustrates the clinical and pathological nosological relationships between these two disease processes, and demonstrates a limitation of the general term, 'DLB'.     

Characteristics of dyslexia and dysgraphia in a Chinese patient with semantic dementia

We describe a 44-year-old Chinese-speaking patient with semantic dementia (SD), who demonstrates dyslexia and dysgraphia. The man was administered a series of neuropsychological inspections, including general language tests and reading and writing examinations. The patient demonstrated surface dyslexia when reading single Chinese characters aloud. While most writing errors demonstrated by the patient were orthographically similar errors and noncharacter responses, such as pictograph, logographeme, and stroke errors, rather than phonologically plausible errors that were homophonous or different only in tone from the targets. We suggest that the type of acquired dysgraphia demonstrated by Chinese-speaking SD patients is determined by the unique features of the Chinese writing system.     

Phonological dyslexia and dysgraphia: cognitive mechanisms and neural substrates

To examine the validity of different theoretical assumptions about the neuropsychological mechanisms and lesion correlates of phonological dyslexia and dysgraphia, we studied written and spoken language performance in a large cohort of patients with focal damage to perisylvian cortical regions implicated in phonological processing. Despite considerable variation in accuracy for both words and non-words, the majority of participants demonstrated the increased lexicality effects in reading and spelling that are considered the hallmark features of phonological dyslexia and dysgraphia. Increased lexicality effects were also documented in spoken language tasks such as oral repetition, and patients performed poorly on a battery of phonological tests that did not involve an orthographic component. Furthermore, a composite measure of general phonological ability was strongly predictive of both reading and spelling accuracy, and we obtained evidence that the continuum of severity that characterized the written language disorder of our patients was attributable to an underlying continuum of phonological impairment. Although patients demonstrated qualitatively similar deficits across measures of written and spoken language processing, there were quantitative differences in levels of performance reflecting task difficulty effects. Spelling was more severely affected than reading by the reduction in phonological capacity and this differential vulnerability accounted for occasional disparities between patterns of impairment on the two written language tasks. Our findings suggest that phonological dyslexia and dysgraphia in patients with perisylvian lesions are manifestations of a central or modality-independent phonological deficit rather than the result of damage to cognitive components dedicated to reading or spelling. Our results also provide empirical support for shared-components models of written language processing, according to which the same central cognitive systems support both reading and spelling. Lesion-deficit correlations indicated that phonological dyslexia and dysgraphia may be produced by damage to a variety of perisylvian cortical regions, consistent with distributed network models of phonological processing.     

Deep dyslexia in a patient with crossed aphasia

A single case study of a 33-year-old right-handed man who suddenly developed left hemiplegia and nonfluent aphasia is reported. Extensive testing of the patient's reading ability revealed the symptom complex of deep dyslexia. The relevance of crossed deep dyslexia in a reader of Italian is discussed.     

A case of left thalamic infarction with aphasia and dysgraphia in Kanji]

We reported an 11-year-old girl with left thalamic infarction causing aphasia and dysgraphia. The lesion corresponded to the perfusion area of the tuberothalamic and paramedian arteries. Confrontation naming and word finding were impaired, but phonological cuing was very helpful despite the absence of amnesia. Dysgraphia was observed only in Kanji (morphogram) writing, and was also improved by visual cuings. These findings suggested difficulty in memory retrieval, in which the left thalamus might have some role.     

Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: A neuropsychological and neuroimaging analysis

There is currently considerable interest in the clinical spectrum of progressive nonfluent aphasia (PNFA) and progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high‐resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP‐PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP‐PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. © 2010 Movement Disorder Society     

Using voice recognition software to treat dysgraphia in a patient with conduction aphasia

Background: Augmentative and alternative communication (AAC) has primarily been utilised for motor speech deficits or as an aid for communicating basic needs in the acute stages of aphasia rehabilitation. However, AAC strategies can be employed for individuals with aphasia across a wide range of severity levels and at different stages of recovery. For individuals with chronic impairments in language, voice recognition software can further enhance communication by providing an alternative means for written expression.

Aims: To investigate the functional and linguistic effects of a treatment program that used voice recognition software to improve written communication in an individual with chronic aphasia.

Methods & Procedures: A 65-year-old woman with conduction aphasia participated in a prospective case study. During baseline testing the individual was evaluated on measures of language (i.e., Boston Diagnostic Aphasia Examination, 3rd edition, Goodglass, Kaplan, & Baressi, 2001 Goodglass, H., Kaplan, E. and Barresi, B. 2001. Boston Diagnostic Aphasia Examination, 3rd, Baltimore, MD: Lippincott Williams & Wilkins.  [Google Scholar]; Boston Naming Test, Kaplan, Goodglass, & Weintraub, 2001 Kaplan, E., Goodglass, H. and Weintraub, S. 2001. Boston Naming Test, 2nd, Philadelphia, PA: Lippincott Williams & Wilkins.  [Google Scholar]), computer skills, and functional communication (i.e., ASHA FACs, Frattali et al., 1995 Frattali, C., Thompson, C. K., Holland, A. L., Wohl, C. B. and Ferketic, M. K. 1995. American Speech-Language-Hearing Association Functional Assessment of Communication Skills for Adults, Rockville, MD: American Speech-Language-Hearing Association.  [Google Scholar]; Quality of Communication Life Scales, Paul et al., 2004 Paul, D., Frattali, C., Holland, A., Thompson, C., Caperton, C. and Slater, S. 2004. Quality of Communication Life Scale (ASHA QCL), Rockville, MD: American Speech-Language-Hearing Association.  [Google Scholar]). A cursory examination of the oral peripheral structure was administered. Treatment consisted of 10 one-hour sessions of training on the Dragon NaturallySpeaking^© Program. Following treatment, measures of language, computer skills, quality of life, and functional communication were re-administered. Further, to evaluate if skills in written expression could be utilised to communicate via e-mail, a distance-learning program was implemented.

Outcomes & Results: Findings revealed that, with intensive instruction, the participant was able to independently access the computer and the Dragon NaturallySpeaking^© Program. At the conclusion of the treatment phase, functional writing abilities approximated spoken communication. Some minor changes in reading, repetition, and conversation were suggested on formal and informal assessment measures. The participant made substantial gains in using the program in her activities of daily living, but only achieved minor success in demonstrating her skills during distance learning.

Conclusions: The potential benefits of AAC devices may change throughout the course of recovery from aphasia. New technologies may facilitate gains in communication in individuals with aphasia throughout their lifespan. This study demonstrated that intensive training in the use of voice recognition software can enhance functional writing in an individual with chronic aphasia. Although marked progress in written expression was achieved, transfer of skills for use on the Internet was limited. Clinical management should include assessment of various assistive technologies across different modalities of communication for people at different stages of recovery from aphasia.     

Progressive dysgraphia in a case of posterior cortical atrophy

Dysgraphia (agraphia) is a common feature of posterior cortical atrophy (PCA). However, detailed analyses of these spelling and writing impairments are infrequently conducted. LM is a 59-year-old woman with dysgraphia associated with PCA. She presented with a two-year history of decline in her writing and dressmaking skills. A 3D T1-weighted MRI scan confirmed selective bi-parietal atrophy, with relative sparing of the hippocampi and other cortical regions. Analyses of LM's preserved and impaired spelling abilities indicated mild physical letter distortions and a significant spelling deficit characterised by letter substitutions, insertions, omissions, and transpositions that was systematically sensitive to word length while insensitive to real word versus nonword category, word frequency, regularity, imagery, grammatical class and ambiguity. Our findings suggest a primary graphemic buffer disorder underlies LM's spelling errors, possibly originating from disruption to the operation of a fronto-parietal network implicated in verbal working memory.     

Non-convulsive status epilepticus presenting with Wernicke's aphasia

Ictal aphasia in adults is a rare phenomenon. Most reported cases manifest with non-fluent (Broca) aphasia. Ictal fluent (Wernicke) aphasia is less common. We report a 47-year-old, right-handed woman that presented with recurrent episodes of non-convulsive seizures in the form of Wernicke's aphasia for 2 weeks. An MRI of the brain showed an old cerebral infarction in the left parieto-occipital area. Scalp EEG revealed continuous periodic sharp waves at the left temporal regions with diffusion to the whole left hemisphere and at occasions to the right. This is followed by variable periods of post ictal slowing. Recurrence of the described ictal pattern was noted. Management of status epilepticus was started in the form of intravenous diazepam and a loading dose of phenytoin and phenobarbitone. After treatment, she improved clinically and the EEG improved with disappearance of the left temporal ictal rhythm and normalization of the EEG background. Thus, establishing the diagnosis of non-convulsive partial status epilepticus manifesting as ictal aphasia.     

Corticobasal degeneration presenting with progressive conduction aphasia

We report the case of a woman with primary progressive aphasia (PPA) presenting with conduction aphasia. Neurological findings showed bilateral finger tremor and signe de poignet figé in her right hand. Memory, orientation, and activities of daily living were well preserved. Linguistic examination showed severe impairment in repetition, fluent spontaneous speech with phonemic paraphasia, and relatively well preserved comprehension. Limb-kinetic apraxia and parkinsonism were not observed during the course of her illness. T1-weighted magnetic resonance imaging revealed severe atrophy of the left temporal lobe and dilatation of the left Sylvian fissure. Neuropathological findings demonstrated the most severe atrophy in the left superior temporal gyrus and Gallyas–Braak-positive or phosphorylated tau-immunoreactive cytoskeletal structures, which were consistent with corticobasal degeneration (CBD). We speculate that the progressive conduction aphasia of our patient might have been caused by left temporal lobe impairment. We suggest that progressive conduction aphasia may be a feature of CBD presenting with PPA.     

Sub-types of deep dyslexia: a case study of central deep dyslexia

A case study is reported of a female patient (JAH), who following a left middle cerebral artery infarct, presented with the cardinal symptoms of deep dyslexia and deep dysphasia (semantic errors when reading and repeating words aloud, respectively). Detailed assessment revealed impaired performance across modalities for many tasks, but particularly those tasks that depend on an intact store of semantic knowledge. Her acquired dyslexia is best characterised as deep dyslexia of a central sub-type.     

Primary progressive aphasia presenting as conduction aphasia.

We report a case of a woman with primary progressive aphasia (PPA) who presented with conduction aphasia. A 60-year-old, right-handed, Japanese female suffering from progressive aphasia had difficulty in repeating words and phrases. She displayed phonemic paraphasias but had preserved comprehension and had no cognitive or behavior disorder for more than 6 years after the onset of the condition. She was able to continue to work successfully and to perform all her normal daily activities. T1-weighted magnetic resonance imaging revealed minute dilatation of the left inferior horn and sulci in the left hemisphere, and positron emission tomography revealed mild hypometabolism in the left supramarginal gyrus and its surrounding areas. Therefore, she was diagnosed as suffering from PPA presenting as conduction aphasia. We believe that the progressive conduction aphasia of the patient belongs to one of the fluent forms of PPA, and the ability to continue normal work along with the clinical portrayal of preserved memory and cognition skills may be features of a form of PPA presenting as conduction aphasia.