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Bulbar palsy
About: Bulbar palsy is a research topic. Over the lifetime, 419 publications have been published within this topic receiving 8273 citations.
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TL;DR: Thirty-second speech samples were studied of at least 30 patients in each of 7 discrete neurologic groups, each patient unequivocally diagnosed as being a representative of his diagnostic group, leading to results leading to these conclusions.
Abstract: Thirty-second speech samples were studied of at least 30 patients in each of 7 discrete neurologic groups, each patient unequivocally diagnosed as being a representative of his diagnostic group. Three judges independently rated each of these samples on each of 38 dimensions of speech and voice using a 7-point scale of severity. Computer analysis based on the means of the three ratings on each patient on each dimension yielded results leading to these conclusions: (1) Speech indeed follows neuroanatomy and neurophysiology. There are multiple types or patterns of dysarthria, each mirroring a different kind of abnormality of motor functioning. (2) These patterns of dysarthria can be differentiated; they sound different. They consist of definitive groupings of certain dimensions of speech and voice, deviant to distinctive degrees. (3) Five types of dysarthria were delineated: flaccid dysarthria (in bulbar palsy), spastic dysarthria (in pseudobulbar palsy), ataxic dysarthria (in cerebellar disorders), hypokine...
1,179 citations
TL;DR: It is confirmed that BBE constitutes a clinical entity and additional clinical and laboratory features of BBE are provided, indicative that the two disorders are closely related and form a continuous spectrum.
Abstract: Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia. Ninety-two per cent of the patients involved had had an antecedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and generally a good outcome. Serum anti-GQ1b IgG antibody was positive in 66%, and MRI showed brain abnormality in 30% of the patients. Another striking feature was the association with flaccid symmetrical tetraparesis, seen in 60% of the patients. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Limb weakness in the BBE cases studied was considered the result of overlap with the axonal subtype of Guillain-Barre syndrome. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. A considerable number of BBE patients have associated axonal Guillain-Barre syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
339 citations
01 Jan 2000
TL;DR: The hypothesis is that patients who present with bulbar palsy and amyotrophy, rather than corticospinal and corticobulbar features, may be most susceptible to the development of FTD.
Abstract: A motor neuronopathy complicating frontotemporal dementia (FTD) has been recognised and designated FTD/ motor neurone disease (MND). FTD is characterised by profound character change and altered social conduct, and executive deficits, reflecting focal degeneration of the frontal and temporal neocortex. The motor neuronopathy comprises bulbar palsy and limb amyotrophy. The major histological change is typically of microvacuolation of the cerebral cortex, with atrophy of the bulbar neurones and anterior horn cells of the spinal cord. Ubiquitinated inclusion bodies occur in large pyramidal cortical neurones and in surviving cranial nerve nuclei and anterior horn cells. Evidence is emerging that some patients with classical MND/ amyotrophic lateral sclerosis (ALS), who are thought not to be demented, develop cognitive deficits in the realm of frontal executive functions. Moreover, frontal lobe abnormalities have been demonstrated by neuroimaging. The findings point to a link between FTD/ MND and cMND/ALS and suggest that a proportion of patients with cMND/ALS go on to develop FTD. Patients with cMND/ALS may not be equally vulnerable. The hypothesis is that patients who present with bulbar palsy and amyotrophy, rather than corticospinal and corticobulbar features, may be most susceptible to the development of FTD. © 2000 Published by Elsevier Science B.V.
191 citations
TL;DR: In this article, a motor neuronopathy complicating frontotemporal dementia (FTD) has been recognised and designated FTD/motor neurone disease (MND), which is characterised by profound character change and altered social conduct, and executive deficits, reflecting focal degeneration of the frontal and temporal neocortex.
188 citations