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We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with
OBJECTIVE
To characterize the dysarthria in patients with corticobasal degeneration (CBD) and determine if analysis of speech in isolation helps to distinguish CBD patients from patients with Parkinson's disease (PD).
METHODS
60 subjects were assessed by means of perceptual analysis of speech: 15
The authors evaluated dysarthria and orofacial apraxia (OFA) in 10 patients with a clinical diagnosis of corticobasal degeneration (CBD). Nine patients were slightly dysarthric according to the French version of the Frenchay Dysarthria Assessment, which evaluates the motricity of the components of
Spinal and bulbar muscular atrophy (SBMA) is a hereditary neuromuscular disease affecting only males characterized by progressive muscular atrophy and weakness in bulbar and limb muscles. The present study aimed to evaluate the features of velopharyngeal dysfunction (VPD) in SBMA subjects by an
OBJECTIVE
To characterize the dysarthria in patients with multiple system atrophy (MSA).
METHODS
Motor speech examinations, consisting of oral motor, oral agility, and perceptual speech analysis, were performed on 46 patients with MSA.
METHODS
University department of neurology referral
Clear speech refers to intentionally modifying conversational speech to maximise intelligibility. This study aimed to compare the speech behaviour of patients with progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and Parkinson's disease (PD) under conversational and clear speech
OBJECTIVE
To characterize the clinical and neuroradiologic features of a new spinocerebellar ataxia, SCA-12, in the index family.
BACKGROUND
The authors recently linked SCA-12 to a novel CAG repeat expansion on chromosome 5q31-33 that is located within the 5' region of PPP2R2B, a gene encoding a
We report an autosomal recessive neurodegenerative disorder in 25 white members from a large inbred Brazilian family, 22 of whom were evaluated clinically. This condition is characterized by (1) subnormal vision secondary to apparently nonprogressive congenital optic atrophy; (2) onset of
Perceptual analysis is not sufficient enough to identify specific dysarthria types. In order to improve the discrimination between dysarthria types, we developed a standardized evaluation of different functions controlling speech motor performances. This was applied to 90 patients suffering from
We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was
A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide,