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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Progressive dysarthria is a common sign of several degenerative disorders of the central nervous system; it may also be a distinct nosographic entity. We identified nine patients in which progressive dysarthria remained the sole neurological sign for at least 2 years after onset. At least a year after hospital admission, the following diagnoses were made: two cases of corticobasal degeneration, one of frontotemporal dementia, one of primary progressive aphasia, one of motor neuron disease (MND)-dementia, one of ALS, and one of ALS-aphasia. In the remaining two patients progressive dysarthria remained the only neurological sign at latest examination. We conclude that in most cases progressive dysarthria is the presenting sign of an established neurodegenerative disease (generally degenerative dementia or motor neuron disease), although the possibility that progressive dysarthria is a distinct entity cannot be excluded. To clarify this issue, studies (probably multicenter) on more patients with longer clinical follow-up and pathological confirmation are required.
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PMID:Progressive dysarthria: definition and clinical follow-up. 1459 92

The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.
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PMID:Corticobasal degeneration as a cognitive disorder. 1463 61

The feasibility of a novel instrument, the Functional Communication Scale (FCS), was determined for individuals with moderate-to-mild cognitive-communication deficits secondary to traumatic brain injury (TBI). A group design including 30 adults with confirmed diagnosis and communication problems was utilized. Conversational samples with each participant were videotaped and rated for 13 FCS items. Three raters with diverse clinical experiences rated the elicited samples. Results identified significant and positive relationships between the cognitive-communication severities and the total FCS scores. Significant inter- and intra-rater reliability scores were found for the three raters. The FCS also determined significant differences between individuals with and without concurrent aphasia or dysarthria. No obvious differences were found between males and females nor between individuals with the primary diagnosis of TBI vs other neurological aetiologies. These findings have implications for assessing the adequacy of functional communication of individuals who are candidates for community re-entry.
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PMID:Functional communication screening in individuals with traumatic brain injury. 1466 Feb 35

We report a rare case of recurrent brain abscess associated with congenital pulmonary arteriovenous fistula. A 52-year-old man was admitted to our hospital in October, 1999 because of a sudden stroke-like onset of right hemiparesis, right hemiparesthesia, dysarthria and sensory aphasia. He had a history of previous brain abscess in the right cerebellar hemisphere. It had been removed in 1991. CT scan at the time of the current admission disclosed a low-density area in the left parietal region. The mass was ring-enhanced after injection of contrast medium. On MRI the mass lesion was depicted as low-intensity on T1-weighted image and high-intensity on T2-weighted image. The mass was ring-enhanced after administration of Gd-DTPA. In spite of conservative treatment the size of the abscess increased considerably with marked surrounding edema. The brain abscess was successfully treated with aspiration and drainage, and the residual mass was resected. The patient also had a history of arteriovenous fistula in the lower lobe of his right lung. This had been excised in 1965. However, he had no signs, symptoms or family histories of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Contrast enhanced CT scan of the chest showed nodular lesions connected to vascular shadows in the right lower lung field. Pulmonary angiograms also revealed multiple arteriovenous fistulas in the lower lobe of the right lung. He was not dyspneic or cyanotic, but his hypoxia, polycythemia, and recurrent brain abscess were thought to be caused by pulmonary arteriovenous fistula. The fistulas were embolized with coils via a percutaneous catheter. Pulmonary arteriovenous fistula should be treated aggressively either by surgery and/or by coil embolization in order to prevent the complication of brain abscess.
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PMID:[Recurrent brain abscess associated with congenital pulmonary arteriovenous fistula: a case report]. 1497 25

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

A 61-year-old man had been treated for malignant fibrous histiocytoma with the pulmonary and the lymph node metastasis in the department of orthopedics in our hospital. He was admitted to our department because of an acute onset of conscious disturbance and non-fluent aphasia. Diffusion-weighted imaging (DWI) showed high signal intensity areas in the bilateral cerebella, thalami and posterior lobes. T2WI did not show any mass effects. Enhanced CT did not reveal any enhanced lesion. He was diagnosed as having cerebral embolism, and his conscious disturbance was improved after medication. Eight weeks later, he presented dysphagia, dysarthria, and ataxia in his extremities. DWI showed multiple lesions of low signal intensity located at the identical place where had showed high signal intensity in the initial DWI. T2WI showed high signal intensity area with mass effect. It was indicated that cerebral metastasis might grow after tumorgenic embolism. This is a rare case that tumor emboluses were developed to the metastatic brain tumors.
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PMID:[A case of malignant fibrous histiocytoma with metastatic brain tumors after tumorgenic embolism]. 1538 7

We report a 55-year-old right-handed Japanese man with motor neuron disease and dysgraphia of kana letters. He was admitted to our hospital because of dysarthria and dysphasia. On admission, the results of general physical examination were within normal limits. Neurological examination revealed severe dysarthria, dysphasia, impaired movement of the tongue without fasciculation and slight distal muscle weakness in the bilateral upper limbs. There were no fasciculation of the muscle. Deep tendon reflexes were hyperactive without Babinski's signs. Sensation, coordination, and gait were normal. Neurophysiological studies demonstrated normal motor nerve conduction velocities and sensory action potential. The results of needle electromyography of the upper limbs were compatible with motor neuron disease (MND). Magnetic resonance imaging (MRI) showed atrophy of the bilateral temporal region of the brain. 99mTc-HMPAO SPECT (Single Photon Emission Computed Tomography) showed reduced uptake of tracer in the bilateral temporal region. On neuropsychological examination, his behavior was normal, and orientation and intelligence were also preserved, but his speech was severely impaired. Reading comprehension was slightly impaired. In regard to writing comprehension, he had no difficulty in copying of words though dictation was found to be impaired. He omitted one kana letter in a word. Agraphia is accompanied by various factors such as aphasia, dementia, agnosia, alexia. But in this case at least for early stage, agraphia existed without other higher cortical dysfunction. He did not show severe dementia in his early stage of his disease, but developed it later in the disease's progression. In this case, agraphia might be due to the atrophic changes in the temporal lobe.
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PMID:[A case of dementia with motor neuron disease associated with agraphia--the omission of kana letters]. 1556 82

A 35-year-old female ingested a lethal dose of potassium cyanide in a suicide attempt. She survived following antidote therapy and intensive care. Following artificial coma she presented with an agitative state for several days followed by akinetic mutism, buccofacial and ideomotoric aphasia. Severe rigid-akinetic syndrome, dysarthria, dysphagia and generalized dystonia developed weeks later. MRI revealed lesions in the caudate and lentiform nuclei, precentral cortex, and cerebellum. SPECT by [123-I] 2 beta-carbomethoxy-3-beta-(4-iodophenyl)-Tropan on two occasions revealed progressive loss of dopamine transporter suggestive of nigral neuronal apoptosis. Striatal and frontal hypometabolism and hypoperfusion were found by FDG-PET and HMPAO SPECT.
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PMID:Cyanide-induced akinetic rigid syndrome: clinical, MRI, FDG-PET, beta-CIT and HMPAO SPECT findings. 1573 73

Fourteen patients with nonfluent progressive aphasia (NFPA) performed a picture description task in both spoken- and written-output conditions, as well as tests of confrontation naming, spelling to dictation and reading aloud of single words and text. Relative to controls, the patients' spoken and written picture descriptions were reduced in length, speed and amount of information. Of particular interest, and accounting for the first part of the article's title, was a pervasive pattern of poorer spoken and written output associated with the requirement to produce more; this was true when 'more' meant either (a) longer vs. shorter single words or (b) connected language vs. single words. Deficits in spoken and written naming were largely parallel and modality-specific output impairments (such as dysarthria in speech or letter-formation problems in writing) seemed to account for the minority of cases who exhibited a discrepancy. Most patients showed no evidence of agrammatism or reduced verb production in their speech, which typically had normal proportions of content and function words as well as nouns and verbs. By contrast, some degree of telegraphic output was observed in the written narratives of a number of patients. Our results argue against several candidates for the main functional locus of impairment in NFPA, but it is likely that deficits in grammatical processing, working memory, planning/executive skills, speech motor abilities and phonological processing all play a role.
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PMID:When more yields less: speaking and writing deficits in nonfluent progressive aphasia. 1578 53

This review summarizes clinical and imaging features associated with primary progressive aphasia (PPA). We investigate the hypothesis that these patients can be divided into subgroups of progressive non-fluent aphasia (PNFA) and semantic dementia (SD), based on their linguistic profiles and related imaging studies, and examine whether each of these major subgroups can be further subdivided. We focus on several critical features within each progressive aphasic subgroup. In PNFA, we examine agrammatism, phonologic disorder, and impaired verb processing to determine whether this syndrome is related to a modality-specific impairment in word formation and articulation, or a conceptual deficit that interferes with grammatical processing. In SD, we examine impaired semantic memory, limited remote memory, and anomia to assess whether this syndrome is due to a modality-neutral interruption of semantic memory, or the degradation of various material-specific representations of object features and words. We conclude that there is sufficiently consistent and converging evidence from clinical and imaging studies to support the claim that PNFA and SD are distinct subgroups of PPA. However, there does not appear to be sufficient evidence at this point to support further discrimination within these progressive aphasic subgroups. Testing hypotheses about finer-grained syndromes such as progressive dysarthria or progressive anomia has important consequences for improving our understanding of language organization and the neural basis for language.
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PMID:Primary progressive aphasia: a review. 1584 55


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