Keratoconus associated with myelinated retinal nerve fibers is not frequent and the relationship between the two pathologies is difficult to explain, therefore studies and further investigation are required. The etiology of each condition may suggest the role of genetic factors. Follow-up is important to evaluate the progression of keratoconus and myelination. Here we describe the unusual coexi...

Two Japanese persons with consanguinous parents had a motor and sensory neuropathy of the hypertrophic type with excessive myelin outfolding in the myelinated fibers. A morphometric analysis of the biopsied sural nerve was made. Excessive myelin outfolding, segmental demyelination, and remyelination and decrease in the density of both large and small myelinated fibers were evident. Using linear...

A case of angioimmunoblastic lymphadenopathy with dysproteinaemia complicated by a subacute peripheral neuropathy is described. Clinically the neuropathy was mainly motor, but pain and paraesthesiae in the legs were also present. Sural nerve biopsy indicated decreased numbers of myelinated fibres, mainly the largest, without actual signs of degeneration or regeneration. However, the occurrence...

Introduction It has been known that a receptor potential may occur at the non-myelinated terminals in the frog muscle spindle and the receptor currents may be integrated in a Ranvier node of the myelinated branches at which afferent impulses may initiate. i) The hypothesis that the receptor potential may be caused by an inward flow of sodium ions) is supported by recent works on mammalian muscl...

A new nerve fiber model has been developed, incorporating electrical characteristics of human myelinated sensory nerve fibers. It will be used to determine the response of myelinated somatosensory nerve fibers in the spinal cord to electrical stimulation with an epidurally placed electrode. Index terms Spinal cord stimulation, human sensory nerve fiber, simulated action potential, chronaxie dia...

In human and experimental diabetes, the relationship between molecular abnormalities in perikarya of sensory neurons and structural abnormalities in their distal axons is largely unexplored. In this study we examined neurofilament (Nf) and tubulin messenger RNA (mRNA) expression and their incorporation into distal sensory axons during progressive streptozotocin-induced diabetes in rats. After 2...

Primary afferents are sensitized to mechanical stimuli following in vivo inflammation, but whether sensitization of mechanically gated ion channels contributes to this phenomenon is unknown. Here we identified two populations of murine A fiber-type sensory neurons that display markedly different responses to focal mechanical stimuli of the membrane based on their expression of calcitonin gene-r...

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PURPOSE Alagille syndrome is frequently associated with optic disc anomalies. This is the first report of a patient with Alagille syndrome and myelinated retinal nerve fibers. METHODS A 5-year-old female patient was referred to the Centre for Ophthalmology before a liver transplantation. Ocular examinations including slit lamp examination and funduscopy as well as anterior segment and fundus ...

We analyzed clinical and pathological disease in 2 peripheral myelin protein-22 (PMP22) overexpressing mouse models for 1.5 years. C22 mice have 7 and C3-PMP mice have 3 to 4 copies of the human PMP22 gene. C3-PMP mice showed no overt clinical signs at 3 weeks and developed mild neuromuscular impairment; C22 mice showed signs at 3 weeks that progressed to severe impairment. Adult C3-PMP mice ha...