Abstract
Sensory neuronopathies (SN) represent a specific subgroup of peripheral nervous system disorders characterized by selective damage to dorsal root ganglia neurons. In contrast to polyneuropathies, SN usually present with multifocal non-length-dependent sensory deficits, ataxia and normal muscle strength. In the last few years, SN have been increasingly recognized in clinical practice associated to different etiologies. Despite this, several relevant issues about clinical findings, diagnosis, pathogenesis and therapy still need to be addressed. For instance, almost half of all SN patients are still labeled as idiopathic. Non-motor manifestations and particularly dysautonomia were not properly investigated in these patients. On clinical grounds, there is still no validated scale to measure the severity of sensory ataxia. Available data on pathogenesis and therapy are scant as well. In this setting, we have designed this research project in an attempt to address these specific questions and ultimately to improve the care of these patients. We propose a multimodal approach, including clinical, neurophysiological, immunological, molecular genetics and neuroimaging analyses. For UNICAMP, this project will strongly support the establishment of a new independent research group devoted to neuromuscular diseases and peripheral neuropathies. (AU)
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