The Experts below are selected from a list of 960 Experts worldwide ranked by ideXlab platform
Anand I Rughani - One of the best experts on this subject based on the ideXlab platform.
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lower motor neuron findings after upper motor neuron injury insights from postoperative supplementary motor area syndrome
Frontiers in Human Neuroscience, 2013Co-Authors: Jeffrey E Florman, Hugues Duffau, Anand I RughaniAbstract:Hypertonia and hypereflexia are classically described responses to upper motor neuron injury. However, acute hypotonia and areflexia with motor deficit are hallmark findings after many central nervous system insults such as acute stroke and spinal shock. Historic theories to explain these contradictory findings have implicated a number of potential mechanisms mostly relying on the loss of descending corticospinal input as the underlying etiology. Unfortunately, these simple descriptions consistently fail to adequately explain the pathophysiology and connectivity leading to acute Hyporeflexia and delayed hypereflexia that result from such insult. This article highlights the common observation of acute Hyporeflexia after central nervous system insults and explores the underlying anatomy and physiology. Further, evidence for the underlying connectivity is presented and implicates the dominant role of supraspinal inhibitory influence originating in the supplementary motor area descending through the corticospinal tracts. Unlike traditional explanations, this theory more adequately explains the findings of postoperative supplementary motor area syndrome in which hyporeflexive motor deficit is observed acutely in the face of intact primary motor cortex connections to the spinal cord. Further, the proposed connectivity can be generalized to help explain other insults including stroke, atonic seizures, and spinal shock.
Jeffrey E Florman - One of the best experts on this subject based on the ideXlab platform.
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lower motor neuron findings after upper motor neuron injury insights from postoperative supplementary motor area syndrome
Frontiers in Human Neuroscience, 2013Co-Authors: Jeffrey E Florman, Hugues Duffau, Anand I RughaniAbstract:Hypertonia and hypereflexia are classically described responses to upper motor neuron injury. However, acute hypotonia and areflexia with motor deficit are hallmark findings after many central nervous system insults such as acute stroke and spinal shock. Historic theories to explain these contradictory findings have implicated a number of potential mechanisms mostly relying on the loss of descending corticospinal input as the underlying etiology. Unfortunately, these simple descriptions consistently fail to adequately explain the pathophysiology and connectivity leading to acute Hyporeflexia and delayed hypereflexia that result from such insult. This article highlights the common observation of acute Hyporeflexia after central nervous system insults and explores the underlying anatomy and physiology. Further, evidence for the underlying connectivity is presented and implicates the dominant role of supraspinal inhibitory influence originating in the supplementary motor area descending through the corticospinal tracts. Unlike traditional explanations, this theory more adequately explains the findings of postoperative supplementary motor area syndrome in which hyporeflexive motor deficit is observed acutely in the face of intact primary motor cortex connections to the spinal cord. Further, the proposed connectivity can be generalized to help explain other insults including stroke, atonic seizures, and spinal shock.
Hugues Duffau - One of the best experts on this subject based on the ideXlab platform.
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lower motor neuron findings after upper motor neuron injury insights from postoperative supplementary motor area syndrome
Frontiers in Human Neuroscience, 2013Co-Authors: Jeffrey E Florman, Hugues Duffau, Anand I RughaniAbstract:Hypertonia and hypereflexia are classically described responses to upper motor neuron injury. However, acute hypotonia and areflexia with motor deficit are hallmark findings after many central nervous system insults such as acute stroke and spinal shock. Historic theories to explain these contradictory findings have implicated a number of potential mechanisms mostly relying on the loss of descending corticospinal input as the underlying etiology. Unfortunately, these simple descriptions consistently fail to adequately explain the pathophysiology and connectivity leading to acute Hyporeflexia and delayed hypereflexia that result from such insult. This article highlights the common observation of acute Hyporeflexia after central nervous system insults and explores the underlying anatomy and physiology. Further, evidence for the underlying connectivity is presented and implicates the dominant role of supraspinal inhibitory influence originating in the supplementary motor area descending through the corticospinal tracts. Unlike traditional explanations, this theory more adequately explains the findings of postoperative supplementary motor area syndrome in which hyporeflexive motor deficit is observed acutely in the face of intact primary motor cortex connections to the spinal cord. Further, the proposed connectivity can be generalized to help explain other insults including stroke, atonic seizures, and spinal shock.
Sandro Salvarani - One of the best experts on this subject based on the ideXlab platform.
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Paraneoplastic cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma.
Functional Neurology, 2014Co-Authors: Roberto Fancellu, Elena Corsini, Gaetano Bernardi, Paolo Buzzo, Maria Luisa Ferrari, Alberto Garaventa, Mauro Truini, Eleonora Lamantea, Sandro SalvaraniAbstract:We describe a case of cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. A 28-year-old woman developed progressive ataxia with Hyporeflexia at the age of 19. Brain MRI showed progressive cerebellar atrophy. Neurophysiological studies, screening of immune-mediated ataxias, oncological markers, vitamin E and genetic tests for spinocerebellar ataxia types 1,2,3, Friedreich ataxia and POLG1 were negative. Anti-Hu antibodies were positive in Western blot and indirect immunofluorescence (1:640). Total-body computed tomography revealed a mediastinum mass; the histological diagnosis was maturing ganglioneuroma. Immunohisto - chemistry showed a mild reaction between the tumor and the patient’s serum, and no reaction between the tumor and control serum. After surgery, serum anti-Hu titer decreased, while ataxic symptoms initially worsened and then stabilized. Ganglioneuroma is a benign tumor, usually derived from the maturation of a neuroblastoma. The benign histology and the presence of anti-Hu antibodies could be related to the positive oncological prognosis and to the slow clinical course mimicking a degenerative ataxia.
Edward M Kaye - One of the best experts on this subject based on the ideXlab platform.
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pelizaeus merzbacher disease presenting as spinal muscular atrophy clinical and molecular studies
Annals of Neurology, 1994Co-Authors: Marvin R. Natowicz, Edward M Kaye, Rhonda F Doll, Frances I SmithAbstract:Two brothers with profound neonatal hypotonia and Hyporeflexia and electrodiagnostic testing consistent with lower motor neuron pathology were found to have a leukodystrophy. Using single-strand conformational polymorphism analysis and direct sequencing, a mutation within exon 3 of the gene encoding proteolipid protein (Gly73 Arg substitution) was previously detected in both brothers and their mother, establishing the diagnosis of Pelizaeus-Merzbacher disease. Despite reported sparing of the peripheral nervous system in Pelizaeus-Merzbacher disease, we suggest that proteolipid protein gene products may influence the development of anterior horn cells or peripheral nervous system myelin and that some individuals affected with this disease may present with clinical and electromyographic features suggestive of neonatal spinal muscular atrophy.