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[Aphasia without amusia in a blind organist. Verbal alexia-agraphia without musical alexia-agraphia in braille].

A 77 year old right handed male was blind since the age of 2. He presented with an infarction involving the territory of the left middle cerebral artery involving the temporal and the inferior parietal lobes. He had learned to read and write language as well as read and write music in braille, ultimately becoming a famous organist and composer. There were no motor or sensory deficits. Wernicke's aphasia with jargonaphasia, major difficulty in repetition, anomia and a significant comprehension deficit without word deafness was present; verbal alexia and agraphia in braille were also present. There was no evidence of amusia. He could execute in an exemplary fashion pieces of music for the organ in his repertory as well as improvise. All his musical capabilities: transposition, modulation, harmony, rythm, were preserved. The musical notation in braille remained intact: he could read by touch and play unfamiliar scores, he could also read and sing the musical notes, he could copy and write a score. Nine months after the stroke his aphasia remained unchanged. Nevertheless he composed pieces for the organ which were published. Such data highly suggest the independence of linguistic and musical competences, defined as the analysis and organization of sounds according to the rules of music. This independence in an extremely talented musician leads to a discussion of the role of the right hemisphere in the anatomical-functional processes at the origin of musical competence. The use of braille in which the same constellations of dots correspond either to letters of the alphabet or musical notes supports the independence between language and music.

Aged↗

Severe central nervous system toxicity from high-dose cytarabine: expressive aphasia occurring after the second day of treatment.

A patient with acute erythroleukemia secondary to polycythemia vera was treated iv with high-dose cytarabine at a dose of 3 g/m2 every 12 hours. After the fourth dose, he developed an expressive (Broca's type) aphasia and somnolence, which progressed until after the treatment was stopped at the seventh dose. The somnolence cleared partially during the next 2 weeks but recurred after cytarabine was restarted at a dose of 100 mg/m2 daily for 7 days. The patient died 5 days later of refractory extreme thrombocytopenia and bleeding. The normal spinal fluid obtained during life and the postmortem findings were consistent with previously reported findings with central nervous system toxicity from high-dose cytarabine. Three unusual aspects of this case are early onset of symptoms, expressive aphasia as the dominant manifestation, and exacerbation of symptoms with standard doses (100 mg/m2/day).

Aphasia↗

[Features of speech dynamics in patients with different variants of motor aphasia in stroke].

Results of studying the peculiarities of speech restoration in patients with dynamic, efferent, afferent, and mixed motor aphasia are presented. These peculiarities were found to depend on the gravity of the speech disorder, time of the beginning and duration of the logopedic treatment, methods of the restorative training, relation between the impaired and preserved sides of the speech function. The studies of the peculiarities of the speech restoration process in various forms of motor aphasia have demonstrated that restorative training is necessary, has good prospects, and may contribute to a substantial social readaptation of the patients.

Aphasia↗

[Age and type of aphasia].

According to many studies, Broca's (or non fluent) aphasic patients are younger than Wernicke's (or fluent). Different hypotheses have been proposed to explain this age difference. However, since the studies in which an age difference was observed have not controlled all the variables--such as schooling and handedness--that may have an influence on speech and language impairments following cerebral lesion, it is legitimate to ask whether or not the explanations proposed are of any relevance. Accordingly, the present study aimed to investigate the age difference between Broca's and Wernicke's aphasics while achieving better control over potentially confounding variables. The subjects (9 Broca's and 14 Wernicke's) were selected from a data base according to the following selection criteria: aphasia type, handedness, localization of lesion and etiology (first CVA). The two groups revealed to be equivalent for sex distribution and schooling; post onset time was superior to three weeks for all subjects but one. Results showed that the distribution of age between Broca's and Wernicke's group were significantly different: there was a small representation of Broca's aphasics in older subjects while Wernicke's aphasia occurred at all ages.

Aged↗

Epileptic aphasia. First onset of prolonged monosymptomatic status epilepticus in adults.

Epileptic aphasia in adults is a rarely described syndrome. Its occurrence in individuals without a clear-cut history of seizures raises diagnostic difficulties with important therapeutic implications. Two such cases are reported in which the diagnosis was confirmed by EEG with a dramatic therapeutic response to anticonvulsant medication. The EEG criteria leading to detection of the epileptic nature of the syndrome are detailed. Maintaining a high index of suspicion in cases with fluctuating symptoms remains crucial for early diagnosis and management, especially in terms of differentiating epileptic aphasia from transient ischemic phenomena.

Aged↗

The prognostic significance of sound recognition in receptive aphasia.

Among 42 aphasics with notable to severe aural verbal comprehension defects in the acute stage of aphasia, 20 with normal sound recognition showed rapid and almost complete recovery of aural comprehension, regardless of how severely aural comprehension was initially impaired. Among 22 receptive aphasics with impaired sound recognition in the acute stage of aphasia, only 32% recovered rapidly; 45% had impairment two years after onset. Results indicated that intact sound recognition predicts rapid and complete recovery of aural comprehension.

Adult↗

Global aphasia without hemiparesis.

Three patients acutely developed global aphasia, but did not manifest the typical accompanying right hemiparesis. Computed tomography and magnetic resonance imaging studies demonstrated that the patients had two discrete left hemisphere lesions, one in the anterior language cortices or language-related subcortical areas, and one in the posterior language cortices. Cerebral angiography showed that two patients had complete occlusion of the left internal carotid artery, and the third had an intraluminal "clot" in the supraclinoid portion of the left internal carotid, findings suggestive of an embolic etiology. Our cases indicate that global aphasia without hemiparesis predicts two discrete lesions and a particularly good recovery of speech and language.

Adult↗

Subcortical structures in aphasia. An analysis based on (F-18)-fluorodeoxyglucose, positron emission tomography, and computed tomography.

Subcortical structural damage that includes the anterior and posterior internal capsule, caudate, thalamus, lenticular nuclei, and insula has been shown to cause aphasias. A critical question that has not been resolved is whether the role of these structures on behavior is a direct one or whether it is indirect through the cortex. We have used pathway analysis to evaluate computed tomography, glucose metabolic, and language data from 47 aphasic patients to answer this question. For fluency (from the Western Aphasia Battery), subcortical structural damage had direct and indirect (through frontal lobe) effects on the behavior. For a comprehension task (sequential commands), subcortical damage had no direct effect and only a slight indirect effect through the temporal lobe. Thus, both direct and indirect effects of subcortical damage can be demonstrated for specific behavioral measures.

Adult↗

Hemianesthesia and aphasia. An anatomical and behavioral study.

A 61-year-old right-handed man with a history of lacunar cerebrovascular disease and hypertension had the sudden onset of right-sided numbness and difficulty speaking. Neurologic evaluation revealed a dense right hemianesthesia that included the face, trunk, arm, and leg. Neuropsychological examination documented a conduction aphasia, which resolved nearly completely several months later. Computed tomographic and magnetic resonance imaging studies showed a lesion in the left hemisphere that involved the posterior insula and disrupted thalamocortical connections but entirely spared the thalamus proper. We suggest that the combination of hemianesthesia and aphasia indicates a white matter lesion subjacent to inferior parietal and posterior temporal cortices.

Aphasia↗

Acquired childhood aphasia. Outcome 1 year after onset.

The effects of the variables age at onset, cause, severity and bilaterality of lesion, and type of aphasia on course and outcome were investigated in a group of 28 aphasic children. Analysis of spontaneous speech and tests of auditory verbal comprehension were used to determine the presence of aphasia. The severity of the cerebral lesion was assessed using a rating scale for computed tomographic scans. Most of the children had not recovered completely 1 year after onset. Recovery was significantly different according to etiological categories. Complete recovery was seen in the majority of traumatic cases.

Adolescent↗

The role of brain single photon emission computed tomography in the diagnosis of primary progressive aphasia.

We present two cases of primary progressive aphasia studied with neuropsychologic measures, computed tomography or magnetic resonance imaging, and single-photon emission computed tomography with technetium Tc99m-labeled hexamethylpropyleneamine oxime. Clinical and neuropsychologic observations revealed a marked, progressive loss of language functions over time with relative preservation of nonlanguage cognitive functions in both patients. The brain single-photon emission computed tomographic scan revealed marked left frontal and minimal left temporal and parietal hypoperfusion in case 1 and marked left posterior frontal and minimal left temporal hypoperfusion in case 2. The value of brain single-photon emission computed tomography in distinguishing primary progressive aphasia from Alzheimer's disease is described.

Aged↗

Long-term prognosis of poststroke aphasia studied with positron emission tomography.

OBJECTIVE: To evaluate positron emission tomography with the use of fludeoxyglucose F 18 as a predictor of the long-term outcome of poststroke aphasia. BACKGROUND: Positron emission tomography shows functional deficits after stroke even in morphologically intact brain regions. The regional metabolic impairment can be related to the clinical deficit. Little is known about whether regional hypometabolism early after stroke predicts the long-term prognosis of stroke sequelae. PATIENTS AND METHODS: Twenty-two patients with language disturbance caused by a single lesion in the territory of the left middle cerebral artery were studied with fludeoxyglucose positron emission tomography and with a neuropsychological test battery that included a test of receptive language (Token Test) and a test of word fluency (/f/, /a/, /s/ test). The neuropsychological test was readministered about 2 years after the initial test. RESULTS: Regional cerebral metabolic rates of glucose measured early after stroke showed a highly significant correlation with the results of the 2-year follow-up test. The receptive language disorder best correlated with cerebral metabolic rates of glucose in the left superior temporal cortex, and word fluency correlated with cerebral metabolic rates of glucose in the left prefrontal cortex. CONCLUSION: Cerebral metabolic rates of glucose in speech-relevant brain regions measured early after stroke are a predictor of the eventual outcome of aphasia.

Aged↗

Treatment of aphasia.

Approximately 1 million people have aphasia in the United States today, yet with properly targeted therapy in selected patients effective communication can be restored. Current approaches to treatment of aphasia include psycholinguistic theory-driven therapy, cognitive neurorehabilitation, computer-aided techniques, psychosocial management, and (still on an experimental basis) pharmacotherapy.

Aphasia↗

Corticosteroid-responsive postmalaria encephalopathy characterized by motor aphasia, myoclonus, and postural tremor.

OBJECTIVES: To study the clinical spectrum of an acute severe encephalopathy occurring in 2 patients after recovery from falciparum malaria infection and to compare it with the reported clinical features of the postmalaria neurological syndrome. DESIGN: Case report. SETTING: Tertiary care hospital. PATIENTS: Two patients presented with acute onset of fluctuating motor aphasia, severe generalized myoclonus, and postural tremor. Additional signs were cerebellar ataxia, and in 1 patient, generalized epileptic seizures. Magnetic resonance imaging of the brain revealed patchy white matter lesions in 1 patient. Clinically, the patients' conditions continued to worsen until corticosteroids were introduced, the use of which induced a rapid, albeit incomplete, recovery. CONCLUSIONS: We describe a new, severe variant of the still poorly defined postmalaria neurological syndrome. We propose a preliminary classification of this syndrome, according to its clinical characteristics, as follows: a mild or localized form, characterized by isolated cerebellar ataxia or postural tremor; a diffuse, but relatively mild encephalopathic form, characterized by acute confusion or epileptic seizures; and a severe, corticosteroid-responsive encephalopathy that is characterized by motor aphasia, generalized myoclonus, postural tremor, and cerebellar ataxia.

Adrenal Cortex Hormones↗

Differential capacity of left and right hemispheric areas for compensation of poststroke aphasia.

As previous functional neuroimaging studies could not settle the controversy regarding the contribution of dominant and subdominant hemisphere to recovery from poststroke aphasia, language performance was related to H2(15)O-positron emission tomographic activation patterns in 23 right-handed aphasic patients 2 and 8 weeks after stroke. In patients classified according to the site of lesion (frontal, n = 7; subcortical, n = 9; temporal, n = 7) and in 11 control subjects, flow changes caused by a word repetition task were calculated in 14 regions representing eloquent and contralateral homotopic areas. These areas were defined on coregistered magnetic resonance imaging scans and tested for significance (Bonferroni corrected t test, alpha = 0.0036). At baseline, differences in test performance were only found between the subcortical and temporal group. The extent of recovery, however, differed and was reflected in the activation. The subcortical and frontal groups improved substantially; they activated the right inferior frontal gyrus and the right superior temporal gyrus (STG) at baseline and regained left STG activation at follow-up. The temporal group improved only in word comprehension; it activated the left Broca area and supplementary motor areas at baseline and the precentral gyrus bilaterally as well as the right STG at follow-up, but could not reactivate the left STG. These differential activation patterns suggest a hierarchy within the language-related network regarding effectiveness for improvement of aphasia; ie, right hemispheric areas contribute, if left hemispheric regions are destroyed. Efficient restoration of language is usually only achieved if left temporal areas are preserved and can be reintegrated into the functional network.

Adult↗

Deterioration of naming nouns versus verbs in primary progressive aphasia.

Disproportionate impairment of naming nouns versus verbs and the opposite pattern have been reported in cases of focal brain damage or degenerative disease, indicating that processing of nouns and verbs may rely on different brain regions. However, it has not been clear whether it is the spoken word forms or the meanings (or both) of nouns and verbs that depend on separate neural regions. We tested oral and written naming of nouns and verbs, matched in difficulty, in patients with nonfluent primary progressive aphasia (nonfluent PPA; n = 15), fluent primary progressive aphasia (fluent PPA; n = 7), and amyotrophic lateral sclerosis with frontotemporal dementia (ALS-FTD; n = 6). Patients with nonfluent PPA and ALS-FTD, both individually and as groups, were significantly more impaired on verb naming than on noun naming and significantly more impaired on oral naming than written naming. Patients with fluent PPA showed the opposite pattern for both word class and modality, significantly more impaired naming of nouns versus verbs and significantly more impaired written versus oral naming. Results indicate that separate regions of the brain are essential for access to oral and written word forms of verbs and nouns, and that these neural regions can be differentially damaged in separate forms of PPA.

Aged↗

Anterior temporal laterality in primary progressive aphasia shifts to the right.

In aphasia due to stroke, language-related activity shifts not only to undamaged cortex within the dominant hemisphere but also toward right-sided areas homotopical to the left-sided lesion. We examined whether a rightward shift takes place in primary progressive aphasia (PPA). Nineteen PPA patients participated, 19 healthy subjects and 14 patients with amnestic mild cognitive impairment who served as controls. Subjects underwent neuropsychological assessment, structural magnetic resonance imaging (MRI), and a functional MRI with a factorial design: words versus pictures and associative-semantic versus visuoperceptual task. Measures of neuropsychological performance were entered as regressors into a multiple linear regression analysis, with response amplitude during the associative-semantic versus control conditions as outcome variable. Language competence correlated negatively with responses in the right anterior temporal cortex and positively with volume and responses in the left-sided homotope. In normal subjects, anterior temporal activation was more extensive to the left than the right (laterality index [LI], +0.64; standard error [SE], 0.11). Laterality was inverted in PPA with word comprehension deficit (LI, - 0.34; SE, 0.19), with an intermediate pattern in PPA without comprehension deficit (LI, +0.23; SE, 0.14). The rightward laterality shift previously reported in aphasic stroke extends to PPA, in particular, when comprehension is deficient.

Aged↗

Language deficits after apparent clinical recovery from childhood aphasia.

Twenty-seven children with childhood injury to the left hemisphere were tested for language function and compared with appropriate controls. Eleven children had incurred their lesions before the age of 1 year, 16 afterward. The group with perinatal injury to the left hemisphere did not show a specific aphasic deficit even though they were mildly cognitively impaired. The group of children with later injury to the left hemisphere showed aphasic deficits if the original injury had caused a language defect; otherwise the left hemisphere injury was not associated with specific disturbances in language function. The average age at time of lesion in those children who had recovered from aphasia was 4.7 years. We conclude that even when childhood aphasia results from a unilateral nonprogressive lesion, recovery of language is less complete than has been generally supposed.

Adolescent↗