Results 201 to 210 of about 4,617 (234)

Hereditary Sensory and Autonomic Neuropathies

2015
The hereditary sensory and autonomic neuropathies (HSAN) are a group of rare, clinically and genetically heterogeneous disorders. A numerical classification (type I to V) based on clinical phenotype and pattern of inheritance is widely used but additional entities have been described.
Felicia B. Axelrod, Horacio Kaufmann
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Hereditary sensory and autonomic neuropathies

2013
Hereditary sensory and autonomic neuropathies (HSN/HSAN) are clinically and genetically heterogeneous disorders of the peripheral nervous system that predominantly affect the sensory and autonomic neurons. Hallmark features comprise not only prominent sensory signs and symptoms and ulcerative mutilations but also variable autonomic and motor ...
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Molecular pathogenesis of hereditary motor, sensory and autonomic neuropathies

Current Opinion in Neurology, 2001
The hereditary motor, sensory and autonomic neuropathies are a heterogeneous group of neurological diseases. The classification of such is presently in a state of change. The original classification system was based on clinical findings whose limitations are being unfurled with increasing insights into the molecular basis of these disorders.
C L, Bennett, P F, Chance
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Hereditary Sensory and Autonomic Neuropathies

2001
Abstract The hereditary sensory and autonomic neuropathies (HSAN) represent a rare group of disorders. A classification of the conditions belonging to this group has been in evolution for decades. At present, the categorization of patients is based on the mode of inheritance, clinical manifestations, and electrodiagnostic and ...
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Hereditary sensory and autonomic neuropathy type I (Thévenard's disease)

Skeletal Radiology, 1996
Radiographic and MR findings of two cases of hereditary sensory and autonomic neuropathy type I are reported. This rare disease has a non-specific radiographic and MR appearance. Differential diagnosis includes other types of hereditary and acquired sensory neuropathies affecting small myelinated and unmyelinated nerve fibers, as well as vascular ...
Allmann, Karl-Heinz, Leu, Hansjoerg, Burg, Günther, Hodler, J.   +3 more
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Hereditary sensory and autonomic neuropathy type IV orthopaedic complications

Journal of Pediatric Orthopaedics B, 2009
Painless fractures with delayed healing or abnormal callus formation require exclusion of a systemic disorder. We report a 9-year-old girl with hereditary sensory and autonomic neuropathy type IV who developed bone changes in the hind foot after a protracted healing of a tibia fracture. Osteomyelitis was considered as a possible cause of destruction of
Ivo, Marik, Miroslav, Kuklik, Dana, Kuklikova, Kazimierz, Kozlowsk   +3 more
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Hereditary Sensory and Autonomic Neuropathy With Autonomic Crises

Journal of Child Neurology, 2011
Hereditary sensory and autonomic neuropathies have different phenotypes. We report 2 cousins with differing clinical courses of a hereditary sensory and autonomic neuropathy. The progressive disease in case 1 is dominated by loss of sensation, autonomic crises, and pain.
Anne, Koy, Rainer, Freynhagen, Ertan, Mayatepek, Daniel, Tibussek   +3 more
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Hereditary sensory and autonomic neuropathy with ataxia and late onset

Clinical Neurology and Neurosurgery, 1994
We report two brothers affected by a dominantly inherited form of hereditary sensory and autonomic neuropathy (HSAN), characterized by clinical features of sensory ataxia, and by late onset in the 6th decade. Sural nerve biopsy in the proband showed almost complete loss of myelinated fibers, and relative sparing of unmyelinated fibers.
A, Marbini, G, Pavesi, G, Cenacchi, A, Mazzucchi, P, Preda, F, Gemignani   +5 more
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Beyond neuropathy in hereditary sensory and autonomic neuropathy type V: cognitive evaluation

European Journal of Neurology, 2008
Background and purpose:  Hereditary sensory and autonomic neuropathy (HSAN) type V is a very rare disorder. It is characterized by the absence of thermal and mechanical pain perception caused by decreased number of small diameter neurons in peripheral nerves.
D C, de Andrade, S, Baudic, N, Attal, C L, Rodrigues, P, Caramelli, A M M, Lino, P E, Marchiori, M, Okada, M, Scaff, D, Bouhassira, M J, Teixeira   +10 more
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Cutaneous innervation in hereditary sensory and autonomic neuropathy type IV

Neurology, 2000
The authors investigated immunocytochemically the innervation of a skin biopsy in a rare case of hereditary sensory and autonomic neuropathy type IV. A few protein gene product 9.5-, growth-associated protein 43-, calcitonin gene-related peptide-, and substance P-immunoreactive nerve fibers were observed in the deeper regions of the dermis ...
VERZE', Laura, VIGLIETTI, Carla Maria, PLUMARI L., CALCAGNI M., STELLA M., SCHRAMA LH, PANZICA, Giancarlo   +6 more
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