Cases reported "Polyradiculoneuropathy"

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1/24. guillain-barre syndrome presenting with severe musculoskeletal pain.

    Three children with guillain-barre syndrome are described who presented with musculoskeletal pain to an orthopaedic clinic or to a paediatric rheumatologist at a children's hospital. In each case, osteomyelitis of the spine was considered to be the most likely diagnosis and bone scans were performed in two of the three patients. Two of the three children required care in an intensive care unit, within hours of diagnosis. Outside of the specialty of neurology, the presentation of guillain-barre syndrome with severe muscle pain is not generally well known. With greater awareness of this particular presentation, dangerous delays in diagnosis and inappropriate investigations will be minimized.
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2/24. Immune-mediated neuropathy and myopathy in post-streptococcal disease: electron-microscopical, morphometrical and immunohistochemical studies.

    A 22-year-old man suffered from a complete flaccid tetraparesis and an immune complex-mediated rapid progressive glomerulonephritis after group A streptococcal infection. serum creatine kinase was excessively elevated and myoglobinuria occurred. Nerve conduction studies revealed evidence of axonal neuropathy. Recovery was satisfactory within 18 months. sural nerve and peroneus muscle biopsies were performed in the 4th and 14th week of the disease. light microscopy of the sural nerve showed an incipient axonal type of neuropathy in the first biopsy. Ultrastructurally, wallerian degeneration and endoneurial inflammatory cells were present. In the muscle biopsy, few atrophic fibers and altered blood vessels without further anomalies were found. In the second sural nerve biopsy, macrophages were numerous, some of which were immunoreactive for HLA-DR, and only a few myelinated and some unmyelinated nerve fibers remained. Muscle fibers in the second biopsy showed high-grade atrophy and myofibrillar abnormalities. immunohistochemistry revealed diffuse endoneurial immunoglobulin deposition in the first sample, while in the later biopsy specimen, deposits of IgG, and kappa and lambda light chains were visible in circumscribed endoneurial areas. Immune-mediated neuropathy and myopathy are not well-known complications of streptococcal disease. This is, to our knowledge, the first detailed report on morphological findings in muscle and nerve in such a disorder.
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keywords = macrophage
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3/24. Early guillain-barre syndrome without inflammation.

    A patient with typical acute guillain-barre syndrome died 72 hours after his first symptoms occurred, and an autopsy was performed 8 hours after his death. Extensive sampling of cranial and peripheral nerves, sensory ganglia, and autonomic nerves showed only minimal inflammatory lymphocytic and macrophage infiltrates. This case, one of the earliest studied extensively, represents an extreme example of a noninflammatory mechanism that has been proposed in some cases of Gullain-Barre syndrome.
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4/24. Motor nerve biopsy in severe guillain-barre syndrome.

    We undertook a biopsy of a terminal branch of the musculocutaneous nerve in a man with severe guillain-barre syndrome and very small distally evoked action potentials. The biopsy showed pronounced subperineurial edema, macrophage infiltration, and many axons that had been completely demyelinated, some associated with intratubal macrophages. The biopsy unequivocally identified the pathological process as primary demyelination, not axonal degeneration, and was more informative than previous reports of sural nerve biopsies in patients with guillain-barre syndrome.
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ranking = 1276.642235513
keywords = macrophage
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5/24. Noninflammatory knee joint effusions in spinal cord-injured and other paralyzed patients. Four case studies.

    Four patients with knee effusions are described. Three had spinal cord injuries and the fourth had guillain-barre syndrome. Of the spinal cord-injured patients, the effusions were unilateral in one case and bilateral in another. The third patient developed effusions on two separate occasions with bilateral involvement once and unilateral involvement once. The patient with guillain-barre syndrome had bilateral knee joint effusions. All effusions were tapped and revealed type I synovial fluid except for one joint that contained some blood cells. Two of the three spinal cord-injured patients had heterotopic ossification at the knees documented by 99mTe bone scans and two were lying prone for the treatment of decubitus ulcers. Similar knee joint effusions have been described by several authors in the past. The etiology is unknown; however, we offer the hypothesis that paralysis of the muscles adjusting intra-articular knee joint structures may be the cause. It is important for clinicians to recognize that benign knee joint effusions are likely to be encountered in paralyzed patients. They can be evaluated with a simple synovial fluid analysis.
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keywords = bone
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6/24. Acute inflammatory demyelinating polyneuropathy following bone marrow transplantation.

    Acute inflammatory demyelinating polyneuropathy (AIDP) appeared in two patients following allogeneic bone marrow transplantation (BMT). In one transplanted patient (but not in the donor) T cells were sensitized against peripheral nervous system myelin. This could reflect a change in the T cell repertoire in a different milieu. In this context, AIDP might be part of graft-versus-host disease or another associated autoimmune disorder following BMT.
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ranking = 5
keywords = bone
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7/24. Skeletal muscle ultrastructural alterations in a case of guillain-barre syndrome.

    The structural study of a muscle biopsy from a case of guillain-barre syndrome showed the typical findings of neurogenic atrophy and several abnormalities not commonly observed in denervated muscle as fibre necrosis, capillary alterations and infiltration of macrophages and occasional mast cells. The histopathological picture was similar to that found in the muscular compromise of some autoimmune diseases. The possibility for an immune mechanism in the muscle damage of guillain-barre syndrome is discussed.
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keywords = macrophage
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8/24. A clinicopathological study of the guillain-barre syndrome. Nine cases and literature review.

    The postmortem findings are reported from 9 cases of the guillain-barre syndrome with survival between 10 days and 1 yr. In 8 cases there was multifocal loss of myelin throughout the peripheral nervous system with relative preservation of axons. In 1 case there was predominant loss of axons. Inflammatory mononuclear cell infiltration was present in the peripheral nervous system of all cases except 1 case surviving a year. The extent and severity of cell infiltration was variable, usually being less prominent than in previous reports, and sometimes sparing nerves in which myelin destruction was severe. Vesicular dissolution of myelin noted by electron microscopy was considered to be a postmortem artefact. In cases examined within 30 days after the onset, immunohistochemical studies with monoclonal antibodies identified more leucocytes (PD7/2B11 ) and T cells (UCHL1 ) in the endoneurium than in cases examined later or control cases. These findings and recent single case reports indicate that the pathology of the Guillian-Barre syndrome is variable. This variability may reflect differences in pathogenesis, with greater cell-mediated immunity in some cases and greater antibody targeted macrophage-mediated demyelination in others.
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keywords = macrophage
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9/24. immobilization hypercalcemia associated with Landry-guillain-barre syndrome. Successful therapy with combined calcitonin and etidronate.

    Two patients with immobilization hypercalcemia associated with Landry-guillain-barre syndrome had marked hypercalciuria (890 and 1136 mg/d [22.2 and 28.3 mmol/d]) and radiologic evidence of generalized osteopenia. Parathyroid hormone levels were either low or normal by C-terminal radioimmunoassay. Subtotal parathyroidectomy was performed in the one patient, with no improvement in serum or urinary calcium levels. A bone biopsy specimen revealed decreased cellular activity in the first patient and increased bone resorption in the second patient. Treatment with intravenous saline, furosemide, oral phosphate supplementation, mithramycin, and calcitonin alone was ineffective in lowering serum or urinary calcium levels. However, when subcutaneous calcitonin combined with oral etidronate disodium was used, a reduction in the serum calcium level was observed within two days of therapy. Within one week of the start of this combined therapy, the calcium level returned to normal and urinary calcium excretion was substantially reduced.
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keywords = bone
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10/24. neuritis cordis due to the acute polyneuritis of the guillain-barre syndrome.

    Three patients with the guillain-barre syndrome which followed the course of Landry's acute ascending paralysis died a sudden cardiac death. Autonomic dysfunction had appeared clinically, consisting of sphincter disturbances in one patient and fluctuating blood pressure and bradycardia in the other. In a twenty-three year old female patient cardiac function had been inconspicuous, apart from tachycardia, but the ECG showed S-T segment depression and flat T waves. Postmortem examination revealed acute inflammatory demyelinating polyradiculoneuritis involving the peripheral autonomic nervous system and especially the nerves of the heart. Immunohistochemically, the inflammatory cell infiltrations of this neuritis cordis consisted of macrophages (MAC 387 positive) and T lymphocytes (UCHL1 positive). No indication of a direct viral infection of the inflamed cardiac nerves was detectable by immunohistochemistry (HSV, CMV, influenza virus) nor by electron microscopy. The neuritis cordis was classified as an inflammatory cardio-neuropathy secondary to a patchy acute polyneuritis of the guillain-barre syndrome, involving the autonomic nervous system. myocarditis could be discounted, and the neuritis cordis was thought to be responsible for the sudden cardiac death.
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keywords = macrophage
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