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Inflammatory Neuropathies (inflammatory + neuropathy)
Selected AbstractsAUTOMIC FAILURE AND NORMAL PRESSURE HYDROCEPHALUS IN A PATIENT WITH CHRONIC DEMYELINATING INFLAMMATORY NEUROPATHYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002M. Laurà A 75-year-old man with HCV hepatitis developed at the age of 70 presented with rest and action tremor localized at both hands and progressive cognitive impairment with memory loss. Four years later he begun to complain of progressive fatigue, occasional falls, numbness at the extremities and orthostatic hypotension. One month after admission, he rapidly worsened with inability to walk, mainly because of autonomic failure. Neurological examination revealed gait disturbances, including a wide base of support and short stride, slurred speech, reduction of upward gaze, rest and action tremor at both hands, intrinsic hand muscle and anterior tibialis muscle wasting and weakness on both sides, absent deep tendon reflexes, loss of vibration sense at lower limbs, and bilateral pes cavus. Routine laboratory studies, autoantibodies, thyroid function, neoplastic markers and immunoelectrophoresis were normal. Cryoglobulins were absent, whereas CSF protein content was increased (142 mg/dl). Autonomic nervous system investigation detected severe orthostatic hypotension. Nerve conduction studies showed absent sensory potentials and a marked reduction of compound motor action potential amplitudes and of motor conduction velocities. A sural nerve biopsy revealed remarkable onion bulb-like changes, endoneurial and perivascular infiltrations of inflammatory cells. Psychometric tests showed mild cognitive impairment. Brain MRI was consistent with normotensive hydrocephalus. The findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, autonomic nervous system involvement and normal pressure hydrocephalus. A condition of multiple system atrophy (MSA) might be taken into account, even if somatic peripheral nerve involvement may rarely occur in MSA. Moreover the normal pressure hydrocephalus could be due to the high protein content in CSF (Fukatsu R et al., 1997). [source] Treating nerves: a call to armsJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2008Richard A. C. Hughes Abstract The process of proving that new treatments for peripheral nerve diseases work has often been slow and inefficient. The lack of adequate evidence for some existing treatments has been highlighted by Cochrane systematic reviews. This article uses four different conditions to illustrate the need for more research. Both corticosteroid injections and surgical decompression of the median nerve are efficacious in carpal tunnel syndrome, but whether corticosteroid injections avoid the need for operation needs to be discovered. Corticosteroids are efficacious for Bell's palsy, but the role of antiviral agents needs clarification, which should come from ongoing trials. Intravenous immunoglobulin (IVIg) and plasma exchange are both efficacious in Guillain-Barré syndrome, but corticosteroids are not. More trials are needed to discover the best dose of IVIg in severe cases and whether mild cases need treatment. In chronic inflammatory demyelinating polyradiculoneuropathy, corticosteroids, IVIg and plasma exchange are all efficacious, at least in the short term, but trials are needed to discover whether and which other immunosuppressive agents help. The Peripheral Nerve Society has formed a standing committee, the Inflammatory Neuropathy Consortium (http://pns.ucsd.edu/INC.htm), to facilitate the trials needed to answer the remaining questions in the inflammatory neuropathies. [source] Immunotherapy of idiopathic inflammatory neuropathiesMUSCLE AND NERVE, Issue 3 2003Peter D. Donofrio MD Abstract Evaluation of peripheral neuropathy is a common reason for referral to a neurologist. Recent advances in immunology have identified an inflammatory component in many neuropathies and have led to treatment trials using agents that attenuate this response. This article reviews the clinical presentation and treatment of the most common subacute inflammatory neuropathies, Guillain,Barré syndrome (GBS) and Fisher syndrome, and describes the lack of response to corticosteroids and the efficacy of treatment with plasma exchange and intravenous immunoglobulin (IVIG). Chronic inflammatory demyelinating polyneuropathy, although sharing some clinical, electrodiagnostic, and pathologic similarities to GBS, improves after treatment with plasma exchange and IVIG and numerous immunomodulatory agents. Controlled trials in multifocal motor neuropathy have shown benefit after treatment with IVIG and cyclophosphamide. Also discussed is the treatment of less common inflammatory neuropathies whose pathophysiology involves monoclonal proteins or antibodies directed against myelin-associated glycoprotein or sulfatide. Little treatment data exist to direct the clinician to proper management of rare inflammatory neuropathies resulting from osteosclerotic myeloma; POEMS syndrome; vasculitis; Sjögren's syndrome; and neoplasia (paraneoplastic neuropathy). Muscle Nerve 28: 273,292, 2003 [source] | ||||||||||