Cavernous angioma of the cerebellum and cerebellar atrophy--case report.
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A cavernous angioma of the cerebellum occurred in a 55-year-old female presenting with a 3-year history of slowly progressive cerebellar signs and symptoms. Computed tomography and magnetic resonance imaging revealed a lesion in the left cerebellar nuclei and atrophic change of the affected cerebellar hemisphere. The final diagnosis was made at operation. The cerebellar nuclei and white matter were probably affected by the slowly expanding lesion, with repeated hemorrhage leading to degeneration of the afferent and efferent fibers, and subsequent atrophy of the affected cerebellar hemisphere. (+info)
Evidence of bilateral temporal lobe involvement in primary progressive aphasia: a SPECT study.
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Primary progressive aphasia (PPA) is rare. Only limited series have been reported with SPECT or PET. Moreover, in the majority of studies, the left-to-right asymmetry ratio was used, leading to difficulties in right hemisphere analyzes. METHODS: Twenty-nine patients with clinical criteria of PPA (Mesulam and Weintraub) were included and compared with 12 control subjects. Complete language examination was performed in all patients. SPECT was performed on a double-head gamma camera after intravenous injection of hexamethylpropyleneamine oxime (22 patients and 12 control subjects) or ethylcysteinate dimer (7 patients). Nineteen regions of interest (ROIs) were drawn on each hemisphere in all patients using the Talairach atlas. The perfusion index (PI = cortex-to-cerebellum ratio) was calculated for each ROI. Atrophy was quantified on MRI by consensus of 3 observers in 16 cortical ROIs. ANOVAs were used to compare the PI between (a). patients and control subjects, (b). patients with (n = 15) or without (n = 14) lexicosemantic abnormalities (LS+ vs. LS-) and patients with (n = 19) or without (n = 10) arthric disorders (A+ vs. A-), and (c). patients with or without atrophy. RESULTS: In the 29 patients, the PI was significantly lower in the left temporopolar, left lateral temporal, left Wernicke, left parietal, and right lateral temporal cortex when compared with control subjects (P < 0.001). In LS+ patients versus control subjects, the PI significantly decreased in the left temporal cortex (lateral temporal; medial temporal; temporopolar; Wernicke), left Broca, left parietal, and right lateral temporal cortex (P < 0.001). In addition, LS+ versus LS- comparison showed a significant decrease in the left lateral, left medial temporal, and left Broca cortex (P < 0.001). In comparison with control subjects, the PI was not significantly different in A+ patients, whereas in A- patients the PI was significantly decreased in the left and right lateral temporal cortex, left Wernicke, and left parietal cortex. Moreover, the PI significantly decreased in the left lateral temporal region in A+ patients compared with A- patients. Finally, in patients without atrophy, the PI significantly decreased in the right and left lateral temporal cortex and the left parietal cortex (P < 0.01). CONCLUSION: Our study demonstrates that right-handed patients with PPA present a decreased perfusion in the bilateral temporal cortex. Moreover, in these regions, morphologic abnormalities are preceded by perfusion abnormalities. Finally, our results show that large left temporal dysfunction occurs in patients with LS disorders. (+info)
FOXP2 expression during brain development coincides with adult sites of pathology in a severe speech and language disorder.
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Disruption of FOXP2, a gene encoding a forkhead-domain transcription factor, causes a severe developmental disorder of verbal communication, involving profound articulation deficits, accompanied by linguistic and grammatical impairments. Investigation of the neural basis of this disorder has been limited previously to neuroimaging of affected children and adults. The discovery of the gene responsible, FOXP2, offers a unique opportunity to explore the relevant neural mechanisms from a molecular perspective. In the present study, we have determined the detailed spatial and temporal expression pattern of FOXP2 mRNA in the developing brain of mouse and human. We find expression in several structures including the cortical plate, basal ganglia, thalamus, inferior olives and cerebellum. These data support a role for FOXP2 in the development of corticostriatal and olivocerebellar circuits involved in motor control. We find intriguing concordance between regions of early expression and later sites of pathology suggested by neuroimaging. Moreover, the homologous pattern of FOXP2/Foxp2 expression in human and mouse argues for a role for this gene in development of motor-related circuits throughout mammalian species. Overall, this study provides support for the hypothesis that impairments in sequencing of movement and procedural learning might be central to the FOXP2-related speech and language disorder. (+info)
Dexamphetamine treatment in stroke.
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Reducing disability and dependency after a stroke is an important clinical objective. We examine what is known about the use of dexamphetamine in patients recovering from an acute stroke, and consider whether further clinical studies should be undertaken. Dexamphetamine has repeatedly been shown to enhance recovery after experimental brain injury in animals, the best effects being seen when dexamphetamine is combined with lesion-specific motor training or sensory stimulation. Postulated mechanisms for these beneficial effects in animals are in keeping with contemporary theories of neurophysiological rehabilitation in man. There have been few clinical studies of dexamphetamine during rehabilitation after an acute stroke. Four controlled trials demonstrated a tendency to an improved outcome when dexamphetamine was paired with therapy and administered 3-30 days after an ischaemic stroke. However, clinical studies to date have been small, included only highly selected patients, and have not addressed possible confounding effects of the drug on mood and untreated depression. Dexamphetamine has previously been used under supervision in medically ill patients and appears to be safe and well-tolerated. There is a need for well-designed studies to assess further the safety and efficacy of dexamphetamine in rehabilitation after stroke. (+info)
The child who does not talk.
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The majority of normal children will have developed some speech by the age of two years. Significant delay in speech development may be the result of (1) deafness, (2) mental retardation, (3) faulty innervation of the speech organs, (4) aphasia, (5) autism, (6) a family trait, (7) hospitalism, (8) parental neurosis, or (9) some combination of these factors. Each nonspeaking child needs an individually planned study for precise diagnosis and recommendation for treatment. (+info)
Effect of amphetamines on speech defects in the mentally retarded.
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In a double-blind study, 106 mentally retarded patients with speech defects were given 15 mg. of d-Amphetamine daily, or a placebo, for a three-month period. Speech defect types were: Stuttering, immature production, oral inaccuracy, lisp, psychotic, mongoloid, aphasia, deafness, and cleft palate. Only the stutterers showed obvious improvement in comparison with the placebo group. Three severe, long-term stutterers showed such dramatic improvement that their whole course in life has been changed. (+info)
Common speech problems encountered in general practice.
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The authors consider speech and communication in the light of whole patient care and point out that defects may be signs and symptoms of underlying organic disease. They describe the four classifications of speech disorders-articulation, rhythm, voice and language, with an indication of the speech therapy required and duration of treatment. Special emphasis has been given to those speech problems which are seen by the family physician; these are usually of the articulation group. A short discussion of stuttering and aphasia is given. Emphasis is put on the direction of treatment by the physician and the use of well-qualified personnel as members of the rehabilitation team. (+info)
Early phonological awareness and reading skills in children with Down syndrome.
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Increasingly, children with Down syndrome receive literacy instruction with the expectation of acquiring functional reading skills. Unfortunately, little is known about the processes underlying literacy skills in this special population. Phonological awareness contributes to literacy development in typically developing children, however, there is inconclusive evidence about these skills in younger children with Down syndrome. 9 children with Down syndrome (5.6-8.10 years) participated in this investigation. Due to the paucity of standardised phonological awareness measures for children with special needs, in particular children with Down syndrome, a variety of tasks were adapted from the literature. The assessment battery examined the skills of phonological awareness, literacy, speech production, expressive language, hearing acuity, speech perception, and auditory-visual memory. The results suggest that children with Down syndrome are at risks for reading acquisition difficulties due to reduced phonological awareness skills. These deficits are in addition to delays caused by reduced cognitive skills. Only one of the participants was able to demonstrate rhyme awareness, which may have been due to task effects. Written word recognition ability was correlated with tests of phonemic awareness, and error analysis of the spelling and non-word reading tasks suggested grapheme-phoneme connections deficits. Further research is needed to determine the best methods of assessment and intervention for phonological awareness in children with Down syndrome. (+info)