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Fascicles of the sural nerve from each of 20 diabetic patients, mostly with maturity-onset diabetes, were studied by biochemical and pathological techniques, and results were compared to values found in nerve specimens from 15 healthy persons. The sorbitol and fructose content was much more variable in diabetic than in healthy nerves. More than one-third of(More)
The reported prevalence of diabetic polyneuropathy varies from 5 to 80%. This unsatisfactory state may relate to evaluation of different patient groups, different minimal criteria for the diagnosis of neuropathy, and different degrees of surveillance. To made matters worse, patients with polyneuropathy tend to be equated ignoring differences in severity. To(More)
Preceding the joint meeting of the 19th annual Diabetic Neuropathy Study Group of the European Association for the Study of Diabetes (NEURODIAB) and the 8th International Symposium on Diabetic Neuropathy in Toronto, Canada, 13-18 October 2009, expert panels were convened to provide updates on classification, definitions, diagnostic criteria, and treatments(More)
The magnitude of the health problem from diabetic neuropathies remains inadequately estimated due to the lack of prospective population-based studies employing standardized and validated assessments of the type and stage of neuropathy as compared with background frequency. All Rochester, Minnesota, residents with diabetes mellitus on January 1, 1986, were(More)
The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic(More)
Dejerine-Sottas syndrome is a hypertrophic, demyelinating neuropathy which appears to demonstrate autosomal recessive inheritance in most pedigrees. Clinical symptoms are similar but more severe than Charcot-Marie-Tooth disease type 1 (CMT1), of which the major subtype, CMT1A, results either from duplication of a 1.5-megabase DNA region in chromosome(More)
Sixteen cases of osteosclerotic myeloma and peripheral neuropathy (SM-PN) were reviewed. The neuropathy resembled chronic inflammatory-demyelinating polyneuropathy with predominantly motor disability, high CSF protein levels, and low motor nerve conduction velocities. Twelve of the 16 patients had detectable levels of monoclonal serum proteins, all with(More)
The natural history of intraneural perineurioma has been inadequately studied. The aim of this study was to characterize the clinical presentation, electrophysiologic and imaging features and outcome of intraneural perineurioma. We ask if intraneural perineurioma is a pure motor syndrome that remains confined to one nerve and should be treated by surgical(More)
We used a laser Doppler velocimeter for measurement of skin blood flow in 63 healthy control subjects and in patients with dysautonomias. We measured vasoconstrictor responses to inspiratory gasp, standing, Valsalva maneuver, and cold stimulus. An abnormal profile was defined in terms of the percentage of abnormal test results, the results of individual(More)
DNA methyltransferase 1 (DNMT1) is crucial for maintenance of methylation, gene regulation and chromatin stability. DNA mismatch repair, cell cycle regulation in post-mitotic neurons and neurogenesis are influenced by DNA methylation. Here we show that mutations in DNMT1 cause both central and peripheral neurodegeneration in one form of hereditary sensory(More)