Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 64-year-old right-handed woman whose initial symptom was slowly progressive aphasia without generalized dementia and who was subsequently diagnosed as having corticobasal degeneration (CBD). Neurological examination revealed disturbed vertical gaze, dysarthria, rigidity of the right upper extremity, and bilateral instinctive grasp reaction. Neuropsychological assessment disclosed Broca's aphasia, buccofacial apraxia, and memory disturbance. MRI of the brain showed atrophy of the frontotemporal lobes, which was more severe on the left than on the right, especially the left inferior frontal gyrus. In most reported cases of CBD, the initial symptom is motor dysfunction of the unilateral upper or lower extremity. However, we should be cautious that among cases with CBD, there have been rare cases that begin with progressive aphasia alone. In our case, the atrophied region of the cerebral cortex was most severe around the left inferior frontal gyrus. In a few reported cases with the initial symptom of aphasia, the atrophied region corresponded considerably to the type of the aphasia. On the other hand, in those whose initial symptom was mainly motor dysfunction of the unilateral extremity, the atrophied region was remarkable in the posterior part of the frontal lobe and parietal lobe. Therefore, we suggest that in CBD the distribution of the cerebral cortical lesions differs in accordance with whether the initial symptom is motor disturbance or aphasia, and that the type of aphasia corresponds to the location of the cortical lesion.
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PMID:[A case of corticobasal degeneration presenting with primary progressive aphasia]. 921 26

We report three cases of effortful echolalia in patients with cerebral infarction. The clinical picture of speech disturbance is associated with Type 1 Transcortical Motor Aphasia (TCMA, Goldstein, 1915). The patients always spoke nonfluently with loss of speech initiative, dysarthria, dysprosody, agrammatism, and increased effort and were unable to repeat sentences longer than those containing four or six words. In conversation, they first repeated a few words spoken to them, and then produced self initiated speech. The initial repetition as well as the subsequent self initiated speech, which were realized equally laboriously, can be regarded as mitigated echolalia (Pick, 1924). They were always aware of their own echolalia and tried to control it without effect. These cases demonstrate that neither the ability to repeat nor fluent speech are always necessary for echolalia. The possibility that a lesion in the left medial frontal lobe, including the supplementary motor area, plays an important role in effortful echolalia is discussed.
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PMID:Effortful echolalia. 953 94

Apraxia of speech and Broca's aphasia both affect voice onset time (VOT) whereas phonemic vowel length distinctions seem to be preserved. Assuming a close cooperation of anterior perisylvian language zones and the cerebellum with respect to speech timing, a similar profile of segment durations must be expected in ataxic dysarthria. In order to test this hypothesis, patients with cerebellar atrophy or cerebellar ischemia were asked to produce sentence utterances including either one of the German lexial items "Rate" (/ra:t(h)e/, 'installment'), "Ratte" (/rat(h)e/, 'rat'), "Gram" (/gra:m/, 'grief'), "Gramm" (/gram/, 'gramm'), "Taten" (/t(h)atn/, 'actions'), or "Daten" (/datn/, 'data'). At the acoustic signal, the duration of the target vowels /a/ and /a:/ as well as the VOT of the word-initial alveolar stops /d/ and /t/ were determined. In addition, a master tape comprising the target words from patients and controls in randomized order was played to three listeners for perceptual evaluation. In accordance with a previous study, first, the cerebellar subjects presented with a reduced categorical separation of the VOT of voiced and unvoiced stop consonants. Second, vowel length distinctions were only compromised in case of the minimal pair "Gram"/"Gramm." In contrast to "Rate"/"Ratte", production of the former lexical items requires coordination of several orofacial structures. Disruption of vowel length contrasts would, thus, depend upon the complexity of the underlying articulatory pattern.
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PMID:Phonemic vowel length contrasts in cerebellar disorders. 1009 44

A 30-year-old man was hospitalized with dysarthria and weakness of his right arm and leg. Three months previously, he had noticed numbness and weakness of his right shoulder, which spread to involve his left leg but which improved after 8 months. On admission, neurological examination revealed limb kinetic apraxia and constructive apraxia of the right hand, motor aphasia, dysarthria, and spastic quadriplegia. Sensory examination revealed hyperalgesia and dysesthesia in the right arm and left leg. Deep tendon reflexes were hyperactive in all four extremities. And he had bilateral Babinski signs. Laboratory examination revealed pH 7.38, PCO2 46.1 Torr, PO2 93.4 Torr, BE 1.7, and blood lactate, 9.0 mg/dl (normal 5-20 mg/dl). Cerebrospinal fluid lactate level was 20.0 mg/dl. pyruvate 1.34 mg/dl. and protein 83 mg/dl. Blood lactate and pyruvate values were markedly elevated after aerobic exercise. T2WI brain MRI showed scattered high signal lesions in the left precentral and postcentral gyrus, right paracentral lobes, both superior frontal gyri, and right superior temporal gyrus. Right biceps brachi biopsy showed almost complete cytochrome c oxidase (COX) deficiency. There were no ragged-red fibers. There was marked decrease of COX activity: 2.7 nmol/min/mg-mitochondrial protein (normal range: 33.0 +/- 16.1, n = 7) in the biopsied muscle. Open brain biopsy (after permission from the patient and his family) revealed gliosis and perivascular infiltration of lymphocytes and macrophages without vascular proliferation. There was no mitochondrial DNA mutations, deletion or duplication, including tRNA-Leu 3243, 8993, 3271, 9176, 3291, and tRNA-Lys 8344, 8356, and 8363. From these findings, a diagnosis of COX deficiency presenting as MELAS-like episodes was done. His mother also showed abnormality on aerobic exercise test, but she had no episode of stroke or neurological dysfunction. Six months later, his aphasia and apraxia of the right hand had resolved, and at discharge he was able to ambulate with a cane. Ten months later, he returned to his work. There has been no recurrence of neurologic symptoms over the next 3 years and 10 months. This patient appears to represent a rare case of adult onset COX deficiency presenting as MELAS-like episodes.
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PMID:[MELAS-like episodes in an adult case with cytochrome c oxidase deficiency]. 1523 72

Background. The purpose of this study was to characterize the speech and language disturbances seen in patients with traumatic brain injury aroused from long-term coma at the Rehabilitation Clinic of the Bydgoszcz Academy of Medicine. Material and methods. The research covered 94 patients (30 women, 64 men, average age 34.7). Speech and language disturbances were diagnosed with basic neuropsychological batteries and authorial methods, and classified as akinetic mutism, global aphasia, sensory aphasia, motor aphasia, dysarthria, or dysphonia. The patients were examined 3 times: at admission, after one month, and after two months. Results. The patients displayed various speech and language disturbances. In the first examination, 47 persons (50.0%) presented with dysarthria. Post-traumatic aphasia was seen in 28 persons (29.8%). Akinetic mutism was somewhat less common (12 persons, 12.8%) and dysphonia least common (6 persons, 6.3%). In the second examination there were numerous shifts between groups, and 15 persons no longer had any disturbances (16.0%). These trends continued in the third examination. Dysphonia was seen in 4 persons (4.3%), akinetic mutism in only 3 (3.2%). 23 persons (24.5%) did not show any speech and language disturbances. Conclusions. Patients aroused from long-term coma display various speech and language disturbances. A significant number have dysarthria and/or aphasia, somewhat fewer have akinetic mutism. Dysphonia is also seen. Mutism often resolves spontaneously, as does dysphonia, while the symptoms of aphasia and dysarthria are more persistent.
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PMID:Disturbances of speech and language in patients aroused from long-term coma subsequent to traumatic brain injury. 1767 75

A classical tenet of clinical neurology proposes that cerebellar disorders may give rise to speech motor disorders (ataxic dysarthria), but spare perceptual and cognitive aspects of verbal communication. During the past two decades, however, a variety of higher-order deficits of speech production, e.g., more or less exclusive agrammatism, amnesic or transcortical motor aphasia, have been noted in patients with vascular cerebellar lesions, and transient mutism following resection of posterior fossa tumors in children may develop into similar constellations. Perfusion studies provided evidence for cerebello-cerebral diaschisis as a possible pathomechanism in these instances. Tight functional connectivity between the language-dominant frontal lobe and the contralateral cerebellar hemisphere represents a prerequisite of such long-distance effects. Recent functional imaging data point at a contribution of the right cerebellar hemisphere, concomitant with language-dominant dorsolateral and medial frontal areas, to the temporal organization of a prearticulatory verbal code ('inner speech'), in terms of the sequencing of syllable strings at a speaker's habitual speech rate. Besides motor control, this network also appears to be engaged in executive functions, e.g., subvocal rehearsal mechanisms of verbal working memory, and seems to be recruited during distinct speech perception tasks. Taken together, thus, a prearticulatory verbal code bound to reciprocal right cerebellar/left frontal interactions might represent a common platform for a variety of cerebellar engagements in cognitive functions. The distinct computational operation provided by cerebellar structures within this framework appears to be the concatenation of syllable strings into coarticulated sequences.
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PMID:The contribution of the cerebellum to speech production and speech perception: clinical and functional imaging data. 1778 16

A 71-year-old man presented with quadriplegia, seizures, dysarthria, motor aphasia and urinary incontinence lasting for several years. The development of proteinuria and increased susceptibility to infections brought the physician's attention to possible underlying autoimmune diseases. Laboratory investigations revealed evidence for systemic lupus erythematosus (SLE) and antiphospholipid syndrome. Imaging studies showed obstructive hydrocephalus. Several courses of methylprednisolone therapies followed by maintenance therapy with low-dose steroid, ventriculoperitoneal shunt, and antihypertensives improved the proteinuria and dysarthria but not the urinary incontinence or dementia. A thromboembolic event in the central nervous system secondary to phospholipid antibodies or lupus activity may represent a pathogenetic basis for hydrocephalus. When encountering a patient with hydrocephalus but without apparent predisposing factors, it is always important to include SLE as a differential diagnosis.
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PMID:Hydrocephalus in an elderly man with systemic lupus erythematosus. 1951 33

Little is known about the clinical usefulness of diffusion tensor tractography (DTT) for the arcuate fasciculus (AF) in stroke patients with aphasia. Using DTT, we attempted to investigate the clinical usefulness of the AF in patients with aphasia. Five stroke patients and 7 age- and sex-matched normal subjects were recruited for this study. We recruited stroke patients with language dysfunction who had lesions in the left corona radiata and basal ganglia level. DTT for the AF was reconstructed using DTI-studio software. Korean-Western Aphasia Battery (K-WAB) was used for measurement of language function. Patient 1, who showed mild dysarthria, revealed a normal left AF in terms of integrity and DTT parameters. In patient 2, with conduction aphasia, the left AF showed partial injury; however, the integrity of the left AF was spared. Patients 3 and 4, who had no brain lesions at Broca's area on conventional brain MRI, showed disruptions of the left AF over the stroke lesions after originating from Wernicke's area and they presented with Broca's aphasia. Patient 5 revealed global aphasia on K-WAB and the left AF was not reconstructed due to severe injury and Wallerian degeneration. We found that DTT for the AF could provide useful information on the presence or severity of injury of the AF, which could not be detected on conventional brain MRI in stoke patients. In addition, it could be helpful in classification of the aphasia type of stroke patients.
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PMID:The clinical application of the arcuate fasciculus for stroke patients with aphasia: a diffusion tensor tractography study. 2214 64

The aim of the study was to determine the incidence and types of speech disorders in patients with acute stroke. The study was performed in 936 acute stroke patients admitted to University Department of Neurology, Tuzla University Clinical Center, in the period from January 1, 2007 through December 31, 2008. Out of 936 study patients, speech disorders were verified on admission in 771 (82.37%) patients. Dysarthria was present in 540 (57.69%) and aphasia in 231 (24.67%) patients. In the group with speech disturbances, dysarthria was present in 70.04% and aphasia in 29.96% of patients. During hospital stay, lethal outcome was recorded in 51 patients, significantly higher in the group with speech disorders (P = 0.004). At discharge from the hospital, speech disorders persisted in 671 (75.81%), dysarthria in 468 (69.75%), and different types of aphasia in 203 (30.25%) of 885 surviving patients. Among patients with aphasia at both admission and discharge, global aphasia was most common, followed by motor aphasia (Broca's aphasia) and nominal aphasia. Although the rate of patients with speech disorders was lower at discharge, the difference was not statistically significant. On admission, 82.37% of patients were considered to have a speech-language disorder, dysarthria being most common. Concerning the type of aphasia, global aphasia was most frequent. Study results suggested the importance and need of speech-language therapy in the early rehabilitation of post-stroke patients; it should be initiated during their hospital stay and continued at long-term.
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PMID:Incidence and types of speech disorders in stroke patients. 2264 78

A 41-year-old male with a history of human immunodeficiency virus (HIV) infection developed motor aphasia, dysarthria, and right hemiparesis. A magnetic resonance imaging scan of the brain revealed a cerebral infarction in the territory of the left middle cerebral artery. The laboratory data showed decreased levels of protein S and protein C. Transesophageal contrast-enhanced echocardiography revealed a patent foramen ovale (PFO). Prothrombotic states, such as protein S and C deficiency, have been reported in HIV-infected patients. In addition, previous studies have reported prothrombotic states to be risk factors for PFO-related cerebral infarction. An association between combined protein S and C deficiency caused by HIV infection and PFO-related cerebral infarction was suggested in our patient.
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PMID:Cerebral infarction in an HIV-infected patient with combined protein S and C deficiency and a patent foramen ovale. 2366 14


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