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  • 1
    ISSN: 1432-0428
    Keywords: Streptozotocin diabetes ; blood-nerve barrier ; non-enzymatic glycation ; serum proteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The permeation of native and non-enzymatically glycated albumin and immunoglobulin G into the endoneurium of the sciatic nerve of rats was examined in acute experiments. Low amounts of native albumin entered both in control and streptozotocin-diabetic animals with no significant difference between them. The entry of glycated albumin was significantly greater both in control and diabetic rats, especially in the former. Permeation of native and glycated immunoglobulin G was not detectable over the time course of the experiment. It is concluded that glycation of albumin enhances its permeation into the nerve. This may be relevant to the increased amounts of endoneurial albumin that are detectable in human diabetic neuropathy.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1991), S. 354-358 
    ISSN: 1432-0533
    Keywords: Peripheral neuropathy ; Chediak-Higashi syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical features of a brother and sister with the Chediak-Higashi syndrome (CHS) are reported. Both showed evidence of a sensory neuropathy associated with central nervous system involvement. Nerve conduction studies indicated an “axonal” neuropathy. Sural nerve biopsy in the brother demonstrated a loss of myelinated nerve fibres, particularly those of larger size, and of unmyelinated axons. In contradistinction to some previous reports, giant lysosomes in Schwann cells were not observed and there were no inflammatory changes. Electron microscopy and teased-fibre studies showed no evidence of demyelination. It is concluded that the neuropathy of CHS is of axonal type. Its mechanism remains obscure.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1459
    Keywords: Chronic inflammatory demyelinating polyneuropathy ; Cranial nerve lesions ; Magnetic resonance imaging ; Nerve biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Five cases of chronic inflammatory demyelinating polyneuropathy are described in which cranial nerve involvement accompanied a more generalized neuropathy. Clinical, electrophysiological, radiological and nerve biopsy findings are presented. Cranial nerve lesions in this form of polyneuropathy may be related to lesions of the peripheral nerves or of the central nervous system, when they may be accompanied by MRI evidence of more widespread CNS demyelinating lesions. In cases of early onset, the occurrence of focal cranial nerve lesions may serve to distinguish chronic inflammatory from inherited demyelinating polyneuropathies.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1619-1560
    Keywords: sympathetic skin response ; autonomic failure ; cholinergic dysautonomia ; dopamine-beta-hydroxylase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The sympathetic skin response (SSR) detects changes in the electrical potential in the skin in response to physiological and electrical stimuli and, therefore, may indicate the integrity of sympathetic cholinergic neural pathways to sweat glands. This has been evaluated in 21 patients with three different forms of peripheral autonomic failure. Of these, 15 had pure autonomic failure (PAF) without additional neurological features; investigations indicated both sympathetic and parasympathetic failure. Four patients had pure cholinergic dysautonomia (PCD), with clinical and laboratory features indicating only cholinergic failure. Two siblings had dopamine-betahydroxylase (DBH) deficiency with only sympathetic adrenergic failure. None was on drugs affecting cholinergic function. Ten normal individuals were aged-matched with PAF patients and studied as controls. The SSR was recorded from the palmar hand and plantar foot surfaces, using previously described techniques, in response to physiological (auditory, cough and inspiratory gasp) and electrical stimuli. Nerve conduction studies excluded an associated motor or sensory neuropathy. The SSR was present in all normal individuals, and in both patients with DBH deficiency who had preserved cholinergic and subdomotor function. It was absent in all 15 PAF and all four PCD patients with impaired cholimergic function. Therefore, we conclude that the SSR reflected sympathetic cholinergic function in these three different groups with peripheral autonomic failure. Clin Auton Res 8:133–138
    Type of Medium: Electronic Resource
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