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1/63. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

    The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/63. Polyneuropathy as initial manifestation of systemic sclerosis (scleroderma).

    We report the first case of a young female patient who developed a sensory-motor polyneuropathy, without any skin or internal involvement characteristic of SSc, but with a serological positivity of antitopoisomerase I antibodies. After 4 years she developed a rapid skin tightening with lung involvement, in a full blown picture of the diffuse subset of SSc. The case suggests that the peripheral nervous system deserves more attention, in particular in the earliest phase of SSc.
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ranking = 0.55740190804403
keywords = sclerosis
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3/63. Following the clues to neuropathic pain. Distribution and other leads reveal the cause and the treatment approach.

    Neuropathic pain can seem enigmatic at first because it can last indefinitely and often a cause is not evident. However, heightened awareness of typical characteristics, such as the following, makes identification fairly easy: The presence of certain accompanying conditions (e.g., diabetes, hiv or herpes zoster infection, multiple sclerosis) pain described as shooting, stabbing, lancinating, burning, or searing pain worse at night pain following anatomic nerve distribution pain in a numb or insensate site The presence of allodynia Neuropathic pain responds poorly to standard pain therapies and usually requires specialized medications (e.g., anticonvulsants, tricyclic antidepressants, opioid analgesics) for optimal control. Successful pain control is enhanced with use of a systematic approach consisting of disease modification, local or regional measures, and systemic therapy.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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4/63. Hereditary motor and sensory neuropathy with minifascicle formation in a patient with 46XY pure gonadal dysgenesis: a new clinical entity.

    This case report is of a patient with 46XY pure gonadal dysgenesis, who presented with chronic progressive motor and sensory polyneuropathy. The sural nerve biopsy exhibited minifascicle formations accompanied by a marked decrease in myelinated fibers. This is the first report of polyneuropathy with minifascicle formations in 46XY pure gonadal dysgenesis. Because a similar polyneuropathy was recently reported in a case with 46XY partial gonadal dysgenesis, it is possible that these cases represent a new type of hereditary motor and sensory neuropathy associated with gonadal dysgenesis.
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ranking = 4.7353216814018
keywords = chronic progressive
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5/63. Familial childhood onset neuropathy and cirrhosis with the 4977bp mitochondrial dna deletion.

    The common 4977 base pair mitochondrial deletion has been identified in association with a number of distinct clinical phenotypes. These include the kearns-sayre syndrome, the Pearson marrow-pancreas syndrome, and chronic progressive external ophthalmoplegia. We report the clinical and pathological findings in two siblings in whom the 4977 base pair mitochondrial dna deletion was identified in muscle-derived mitochondrial dna. One sibling manifested early onset liver and renal failure, and both developed prominent peripheral sensorimotor neuropathy. These clinical findings have not been previously described in association with the 4977bp mtDNA deletion and thus represent a further expansion of the spectrum of mitochondrial disease.
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ranking = 4.7353216814018
keywords = chronic progressive
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6/63. cholesterol emboli neuropathy.

    We report the clinical and pathologic features of a patient with peripheral neuropathy that was the first clinical expression of cholesterol emboli syndrome (CES). biopsy of skeletal muscle and peripheral nerve revealed cholesterol clefts in lumens of small arteries, necrotizing arteritis, and severe degeneration of peripheral and intramuscular nerves. At autopsy, the peripheral nervous system was extensively affected by similar changes. We conclude that (1) peripheral neuropathy may be the initial manifestation of CES. Presumably, deposition of cholesterol leads to arteritis. (2) The underlying pathology of CES neuropathy is chronic axonal degeneration, possibly due to chronic ischemia of epineurial arteries. (3) Muscle biopsy is important in the antemortem diagnosis of CES. Nerve biopsy may show involvement of epineurial vessels. (4) CES may resemble polyarteritis nodosa clinically and pathologically. (5) CES may be under-recognized and should be included in the differential diagnosis of any neuropathy of uncertain cause, particularly when there is a history of vascular catheterization, or severe aortic atherosclerosis.
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ranking = 0.13935047701101
keywords = sclerosis
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7/63. A rare Cu/Zn superoxide dismutase mutation causing familial amyotrophic lateral sclerosis with variable age of onset, incomplete penetrance and a sensory neuropathy.

    We present three members of a pedigree with familial amyotrophic lateral sclerosis (FALS) who have a rare mutation in exon 4 of Cu/Zn superoxide dismutase (SOD1) codon position 89, converting alanine to valine. This mutation was associated with incomplete penetrance and variable age of onset. The onset of the disease was late in two of our patients and early in the other. Two of our patients had symptoms and/or signs of an associated painful sensory neuropathy. The incomplete disease penetrance seen with this mutation (and others reported in the literature) emphasizes the potential value for obtaining an SOD1 genotype in patients with ALS, even if there is no apparent family history.
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ranking = 0.69675238505504
keywords = sclerosis
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8/63. Progressive atypical peripheral neuropathy following nephrectomy in a patient with renal cell carcinoma.

    Peripheral neuropathy or amyotropic lateral sclerosis can be associated with renal cell carcinoma. We report a 63-year-old male patient with renal cell carcinoma who developed an atypical, progressive neuropathy after nephrectomy.
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ranking = 0.13935047701101
keywords = sclerosis
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9/63. neurologic manifestations of progressive systemic sclerosis.

    Neurologic involvement in progressive systemic sclerosis is considered uncommon. We retrospectively examined the prevalence and nature of neurologic complications in 50 patients with progressive systemic sclerosis. In 20 (40%), neurologic abnormalities were detected, with a total of 28 neurologic manifestations. All levels of the central and peripheral nervous system were affected: muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%). Of note were the presence of myelopathy in four patients and inclusion-body myositis in two. In 10 patients (20%), no definable cause of the neurologic dysfunction could be identified, apart from progressive systemic sclerosis. Thus, neurologic presentations of progressive systemic sclerosis are much more common than previously reported and may be due to direct involvement of the nervous system by a primary pathologic process in a significant number of patients.
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ranking = 1.1148038160881
keywords = sclerosis
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10/63. Deblocking effect of TRH-T in three cases of chronic progressive multifocal neuropathy.

    Three cases of acquired demyelinating multifocal neuropathy with persistent conduction blocks are reported. In one of them protyreline tartrate (TRH-T) had an evident deblocking effect; similar but milder effects were seen in the other two cases. The course and consistency of this effect is analyzed on the grounds of clinical and electromyographic findings. The nature of the THR-T responsive conduction block is analysed as a mild variant of CIDP with regard to its occurrence in a late and chronic phase without signs of active immune processes.
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ranking = 18.941286725607
keywords = chronic progressive
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