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Query: UMLS:C0003635 (apraxia)
 2,817 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The speech of a patient with aphemia (pure anarthria) resulting from a penetrating brain wound was studied using linguistic and acoustic observations as well as electromyographic recordings from four labial muscles. The results are discussed in relation to phonetic disintegration's syndrome and apraxia of speech which, respectively, enhance linguistic disorders and motor programming disturbance.
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PMID:Aphemia after a penetrating brain wound: a case study. 187 79

We have attempted to draw some parallels between syndromes of adult acquired aphasia and of childhood developmental dysphasia. There appear to be two syndromes that are almost exact duplicates in the adults and the children: (a) pure word deafness and verbal auditory agnosia, and (b) aphemia and verbal dyspraxia. Two other syndromes seem to have rather close but not exact counterparts: Broca's aphasia and the phonologic-syntactic deficit syndrome, and transcortical sensory aphasia and the semantic-pragmatic deficit syndrome. There are two dysphasic syndromes, the phonologic production deficit syndrome and the lexical-syntactic deficit syndrome, that do not seem to have close adult counterparts. Neither of these dysphasic syndromes has been defined in adequate linguistic detail, and it is possible that their description may have to be modified when more data become available. Whether these comparisons between dysphasias and aphasias have heuristic value for guiding external validation studies of the clinically defined dysphasic syndromes of preschool children remains to be determined. Our purpose was to formulate hypotheses as to which cerebral systems are likely to be dysfunctional in children with clinically defined dysphasic syndromes. We recognize that the disorders of language acquisition and those of overlearned adult language have fundamental differences, and that plasticity of the child's developing brain introduces further complexities. Nevertheless, it seems reasonable to think that there are constants in brain organization that span all ages. Looking for language deficits common to aphasic adults (whose lesions can usually be delineated with contemporary neuroimaging techniques) and to dysphasic children (in whom there are rarely any neurologic clues) may be a fruitful way to begin to define the cerebral correlates of the children's deficits.
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PMID:Syndromes in developmental dysphasia and adult aphasia. 245 53

A case of mutism due to left hemisphere infarction is described. Recovery revealed mild motor dysphasia. Review of the literature showed that the case resembles aphemia but is unique by virtue of its duration, and the absence of associated apraxia and paresis.
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PMID:Mutism following left hemisphere infarction. 621 Mar 46

Aphemia, also called anarthria or severe apraxia of speech, is a rare disorder of speech production usually resulting from vascular lesions affecting the inferior premotor cortex of the left hemisphere. A patient presenting with aphemia as the sole manifestation of primary progressive aphasia (PPA) is reported.
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PMID:Pure progressive aphemia. 835 Jan 14

There has been considerable recent interest in frontal lobe epileptic syndromes, and less attention paid to occipital and parietal epilepsies. The occipital and parietal lobes have arbitrary anatomical borders. The prinicpal seizure symptomatology includes somatosensory (paresthetic, painful, thermal, sexual, apraxia, disturbances of body image); visual (amaurotic, elementary and complex hallucinations, illusions) and other phenomena (anosognosia, apraxia, acalculia, alexia, aphemia, confusional states, gustatory, vertiginous, adversive, oculoclonic and eyelid flutter). The seizure symptoms are of varying localizing and lateralizing value and seizure discharges may spread rapidly and perceived symptoms may reflect secondary spread rather than the primary site of seizure onset. Recognized parietal and occipital epilepsy syndromes include benign epilepsy of childhood with centrotemporal spikes, benign epilepsy of childhood with parietal evoked spikes, benign occipital epilepsy of childhood, migraine/epilepsy syndromes, and epilepsy with bilateral occipital calcification. In addition, occipital and parietal epilepsy may be on the basis of any underlying structural lesion. There is frequently a poor correlation between clinical and EEG features. MRI and functional imaging often reveals underlying pathology. There have been no specific trials of different antiepileptic drugs for occipital and parietal seizures. Surgical treatment has its place, with attention to the risk of causing a fixed neurological deficit.
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PMID:Parietal and occipital lobe epilepsy: a review. 850 83

Among the dozen known mutations in the PrP gene which segregate with the inherited prion diseases, only 2 mutations have been described in Israel so far: the codon 200 mutation in Creutzfeldt-Jakob disease (CJD) affected Libyan Jews, and the codon 102 mutation in 1 Jewish Gerstmann-Straussler-Scheinker (GSS) affected pedigree of German origin. We report here 2 unrelated CJD178 cases affected by a unique phenotype: aphemia, apraxia, uncontrolled laugh and no ataxia. As opposed to other CJD178 patients, in these patients, the signal transduction protein 14-3-3, recently suggested as a CJD marker, was detected in the cerebrospinal fluid samples by immunostaining. The D178N mutation, known to be linked to 2 different phenotypes: Fatal Familial Insomnia (FFI) and CJD, was not described so far among Jews. The phenotype reported here, although it shares a common Va1129/Asn178 haplotype with the previously described CJD178, may point to a different clinical subtype of CJD178.
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PMID:Identification in Israel of 2 Jewish Creutzfeld-Jakob disease patients with a 178 mutation at their PrP gene. 953 35

We describe a patient with progressive aphemia with agrammatism that was later overlaid with buccofacial apraxia and pseudobulbar palsy. Pathological findings were consistent with those of classic Pick's disease with argyrophilic inclusions and neuronal achromasia, except for restricted cortical atrophy in the frontal operculum posterior to the pars opercularis (Brodmann Area 44). In addition, major neuronal loss was confined to the premotor cortex and the anterior half of the precentral gyrus (Area 6), which apparently explained the aphemia. The present case demonstrated that classic Pick's disease can show quite limited cortical atrophy in a patient who clinically presents with progressive aphemia. Also, our patient differed from the progressive non-fluent aphasia patients reported as having Pick's disease, who were all Pick variants, revealing that classic Pick's disease can be included in the spectrum of progressive aphasia syndrome.
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PMID:Progressive aphemia in a patient with Pick's disease: a neuropsychological and anatomic study. 974

A 69-year-old women was admitted to Tokyo Medical and Dental University Hospital because of slowly progressive difficulty in speech production over 8-years. On admission, her spontaneous speech was non-fluent, limited to one-syllable utterance, and severely efforty. But her visual and auditory comprehension was preserved. There was no significant general intellectual deterioration. Severe buccofacial apraxia, but no swallowing was observed. So we considered her difficulty in speech as aphemia. Three-dimensionally reconstructed surface MR image clearly showed severe atrophy in the posterior part of the left inferior frontal gyrus and the lower part of the left pre-central gyrus. The FDG-PET demonstrated a focal hypometabolism in the same region. The lesion in this area was suggested to be a cause of speech production difficulty in this case.
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PMID:[Slowly progressive aphemia--a case report]. 1007 30

Damage to the anterior peri-intrasylvian cortex of the dominant hemisphere may give rise to a fairly consistent syndrome of articulatory deficits in the absence of relevant paresis of orofacial or laryngeal muscles (apraxia of speech, aphemia, or phonetic disintegration). The available clinical data are ambiguous with respect to the relevant lesion site, indicating either dysfunction of the premotor aspect of the lower precentral gyrus or the anterior insula in the depth of the Sylvian fissure. In order to further specify the functional anatomic substratum of this syndrome, functional magnetic resonance imaging (fMRI) was performed during reiteration of syllables differing in their demands on articulatory/phonetic sequencing (CV versus CCCV versus CVCVCV). Horizontal tongue movements and a polysyllabic lexical item served as control conditions. Repetition of the CV and CCCV monosyllables elicited a rather bilateral symmetric hemodynamic response at the level of the anterior and posterior bank of the central sulcus (primary sensorimotor cortex), whereas a more limited area of neural activity arose within this domain during production of lexical and nonlexical polysyllables, significantly or exclusively lateralized toward the left hemisphere. There is neurophysiological evidence that primary sensorimotor cortex mediates the "fractionation" of movements. Assuming that the polysyllables considered are organized as coarticulated higher-order units, the observed restricted and lateralized cortical activation pattern, most presumably, reflects a mode of "nonindividualized" motor control posing fewer demands on "movement fractionation." These findings may explain the clinical observation of disproportionately worse repetition of trisyllabic items as compared to monosyllables in apraxia of speech. The various test materials failed to elicit significant activation of the anterior insula. If at all, only horizontal tongue movements yielded a hemodynamic reaction extending beyond the sensorimotor cortex to premotor areas. Since limbic projections target the inferior dorsolateral frontal lobe, the enlarged region of activation during horizontal tongue movements might reflect increased attentional requirements of this task.
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PMID:Articulatory/phonetic sequencing at the level of the anterior perisylvian cortex: a functional magnetic resonance imaging (fMRI) study. 1104 68

Aphemia is a disorder with prominent speech abnormality. Since its description by Broca, there has been debate regarding the neuropsychological disorganization underlying aphemia: is aphemia an articulatory disorder or a language disorder? We describe a patient with markedly impaired articulation, but preserved receptive and written language function and buccal-facial coordination. The location of his stroke was in the left precentral gyrus, undercutting a small area of motor and premotor cortex. This case suggests that aphemia can occur as an isolated articulation deficit without language involvement or more widespread bulbar apraxia, and may be a severe form of apraxia of speech.
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PMID:Aphemia: an isolated disorder of articulation. 1151 58


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