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Peripheral Nerve Disease (peripheral + nerve_disease)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

Hereditary neuropathy with liability to pressure palsies associated with central nervous system myelin lesions

EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2001
J. Dac
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder most commonly caused by a 1.5-Mb deletion in chromosome 17p11.2 which contains the peripheral myelin protein-22 (PMP22) gene. Mutations resulting in functional loss of one PMP22 gene copy are less frequent. We present a 51-year-old patient with a l.5-Mb deletion in chromosome 17p11.2 who exhibited signs of peripheral as well as central nervous system lesions. He gave a history of recurrent episodes of limb numbness and weakness with spontaneous but incomplete recovery since age 20. His father and two brothers had similar symptoms. Neurological examination revealed signs of multiple mononeuropathy associated with frontal lobe, corticospinal tract and cerebellar dysfunction, as well as signs of initial cognitive impairment. Electrophysiological investigations showed a demyelinating peripheral nerve disease with multiple conduction blocks and conduction disturbances in both optic nerves. Magnetic resonance imaging of the brain revealed multiple subcortical and periventricular foci of myelin lesions. The association of central and peripheral nervous system lesions in this patient indicates a possible role of PMP22 not only in peripheral but also in central nervous system myelin structure. [source]

Ganglioside mimicry and peripheral nerve disease

MUSCLE AND NERVE, Issue 6 2007
Nobuhiro Yuki MD
Abstract Four criteria must be satisfied to conclude that a given microorganism causes Guillain,Barré (GBS) or Fisher (FS) syndrome associated with anti-ganglioside antibodies: (1) an epidemiological association between the infecting microbe and GBS or FS; (2) isolation in the acute progressive phase of illness of that microorganism from GBS or FS patients with associated anti-ganglioside IgG antibodies; (3) identification of a microbial ganglioside mimic; and (4) a GBS or FS with associated anti-ganglioside antibodies model produced by sensitization with the microbe itself or its component, as well as with ganglioside. Campylobacter jejuni is a definitive causative microorganism of acute motor axonal neuropathy and may cause FS and related conditions. Haemophilus influenzae and Mycoplasma pneumoniae are possible causative microorganisms of acute motor axonal neuropathy or FS. Acute and chronic inflammatory demyelinating polyneuropathies may be produced by mechanisms other than ganglioside mimicry. Muscle Nerve, 2007 [source]

Autonomic dysfunction in peripheral nerve disease

MUSCLE AND NERVE, Issue 6 2003
Phillip A. Low MD
Abstract Autonomic neuropathies are inherited or acquired neuropathies in which autonomic nerve fibers are selectively or disproportionately affected. Generally, sympathetic and parasympathetic fibers are both affected but there are exceptions. Acquired cases can be autoimmune; due to diabetes, amyloidosis, drugs, or toxins; or idiopathic. Autoimmune autonomic neuropathy is often subacute, sometimes associated with a neoplasm, and associated with high titers of antibody to ganglionic nicotinic acetylcholine receptor in about half of the severe cases. The molecular basis of inherited autonomic neuropathies is better known, including recent identification of the loci and genes of hereditary sensory and autonomic neuropathies types I, III, and IV. The inherited amyloid neuropathies are due to mutations of three proteins: transthyretin, apolipoprotein A1, and gelsolin. Non-invasive autonomic testing complements clinical and electrophysiological characterization of the autonomic neuropathies. Muscle Nerve 27: 646,661, 2003 [source]

Detection and analysis of Borna disease virus in Chinese patients with neurological disorders

EUROPEAN JOURNAL OF NEUROLOGY, Issue 3 2009
Q. Li
Background and purpose:, Borna disease virus (BDV) is a neurotropic RNA virus that is known to cause neurological disturbances in various animal species, potentially even humans. However, the association between BDV infection and human neurological disorders remains unclear. Methods:, Between August 2005 and March 2006, 65 patients with neurological disorders were enrolled into our study. The presence of BDV p24 RNA from peripheral blood mononuclear cells (PBMCs) was investigated by using nested reverse transcriptase PCR (RT-PCR) assay. Results:, Borna disease virus p24 RNA was detected from PBMCs in six patients with viral encephalitis by using nested RT-PCR assay. However, BDV p24 RNA was not detected in patients with multiple sclerosis or peripheral nerve diseases. Conclusion:, There might be possible associations between BDV infection and human viral encephalitis. [source]