Assuntos
Cooperação Internacional , Atrofia de Múltiplos Sistemas/fisiopatologia , Doenças Raras/fisiopatologia , Descoberta de Drogas/tendências , Europa (Continente) , Humanos , Atrofia de Múltiplos Sistemas/tratamento farmacológico , Doenças Raras/tratamento farmacológico , América do Sul , Estados UnidosRESUMO
The clinical features of multiple system atrophy (MSA) include four domains: autonomic failure/urinary dysfunction, Parkinsonism, cerebellar ataxia, and corticospinal tract dysfunction. Although the diagnosis of definite MSA requires pathological confirmation, magnetic resonance imaging (MRI) studies have been shown to contribute to the diagnosis of MSA. Although pyramidal tract dysfunction is frequent in MSA patients, signs of pyramidal tract involvement are controversially demonstrated by MRI. We evaluated the pyramidal involvement in 10 patients (7 women) with clinically probable MSA, detecting the presence of spasticity, hyperreflexia, and Babinski sign, as well as demonstrating degeneration of the pyramidal tract and primary motor cortex by MRI in all of them. Our article also discusses key radiological features of this syndrome. In MSA, pyramidal tract involvement seems to be more frequent than previously thought, and the clinicoradiological correlation between pyramidal tract dysfunction and degeneration may contribute to the understanding of the clinical hallmarks of MSA. MRI may also add information regarding the differential diagnosis of this syndrome.
Assuntos
Imageamento por Ressonância Magnética , Atrofia de Múltiplos Sistemas/patologia , Degeneração Neural/patologia , Tratos Piramidais/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/complicações , Atrofia de Múltiplos Sistemas/fisiopatologia , Degeneração Neural/complicações , Degeneração Neural/fisiopatologia , Doença de Parkinson Secundária/diagnóstico , Doença de Parkinson Secundária/etiologia , Doença de Parkinson Secundária/fisiopatologia , Reflexo Anormal/fisiologia , Índice de Gravidade de DoençaRESUMO
Multiple system atrophy (MSA) is characterized by parkinsonian, cerebellar and pyramidal features along with autonomic dysfunction in different combinations. Onset of dysarthria during the first year of the manifestation of a parkinsonian syndrome suggests the diagnosis of MSA. The aim of this study was to characterize the voice and the speech of patients with MSA. We studied five MSA patients with a mean age of 51.2 years. Each patient was submitted to a neurological and a specific speech and voice assessment. The latter consisted of the following: clinical interview, myofunctional examination, and perceptual speech evaluation. Speech and voice complaints occurred at an average time of 1.1 year after the onset of the motor symptomatology. All MSA patients had the mixed type of dysarthrophonia, where hypokinetic, ataxic and spastic components were seen in each of the patients, although hypokinetic component predominated among the others. Our findings are different from what is commonly seen in Parkinson's disease in which hypokinetic component is the only abnormal finding. We think that specific speech and voice assessment is important to establish the diagnosis and to choose the best management of MSA patients.
Assuntos
Atrofia de Múltiplos Sistemas/complicações , Distúrbios da Fala/diagnóstico , Distúrbios da Voz/diagnóstico , Adulto , Disartria/diagnóstico , Disartria/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/fisiopatologia , Doença de Parkinson/complicações , Doença de Parkinson/fisiopatologia , Medida da Produção da Fala , Distúrbios da Voz/etiologiaRESUMO
Patients with basal ganglia diseases may exhibit ideomotor apraxia. To define the nature of the impairment of the action production system, we studied a repetitive gesture of slicing bread by three-dimensional computergraphic analysis in eight nondemented patients with Parkinson's disease in the "on" state, five with progressive supranuclear palsy and four with multiple system atrophy. Two patients with Parkinson's disease and two with progressive supranuclear palsy showed ideomotor apraxia for transitive movements on standard testing. A Selspott II system was used for kinematic analysis of wrist trajectories and angular motions of the shoulder and elbow joints. Patients with Parkinson's disease, progressive supranuclear palsy, and even some with multiple system atrophy exhibited kinematic deficits in the spatial precision of movement and velocity-curvature relationships; in addition, they failed to maintain proper angle/angle relationships and to apportion their relative joint amplitudes normally. Spatial disruption of wrist trajectories was more severe in patients with ideomotor apraxia. We posit that the basal ganglia are part of the parallel parieto-frontal circuits devoted to sensorimotor integration for object-oriented behavior. The severity and characteristics of spatial abnormalities of a transitive movement would therefore depend on the location and distribution of the pathologic process within these circuits.