1/14. The spectrum of chronic inflammatory demyelinating polyneuropathy.research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/14. An unusual neuropathy in a diabetic patient: evidence for intravenous immunoglobin-induced effective therapy.We report the case of a 68-year old type-2 diabetic male patient who was admitted to hospital for progressive weakness in the right lower limb. Although his metabolic control was good, he lost more than 20 kg of weight. Despite intensive physio- and vitaminotherapy, his neurological condition kept on degrading with a severe amyotrophy and pain of the right thigh. He was unable to walk and to stand alone. Besides a yet known sensitive polyneuropathy, the electrophysiological study revealed an obvious motor involvement with signs of demyelination and axonal degeneration. Combined with the albuminocytologic dissociation observed in the cerebrospinal fluid, these specific clinical and electrophysiological features led us to postulate a diagnosis of inflammatory neuropathy. The patient underwent a treatment by methylprednisolone and immunoglobins that rapidly induced a striking improvement of his neurological condition. This case report illustrates that rare forms of neuropathy such as inflammatory neuropathies close to chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in diabetic patients and superimpose on the more commonly described forms of neuropathies. It recalls the importance of recognizing CIDP-like neuropathies because unlike other forms of neuropathy, inflammatory neuropathies are perfectly curable.- - - - - - - - - - ranking = 0.19937338905446keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/14. Severe chronic sensory-motor polyneuropathy: coexistence of 3 unrelated etiologies in a type 1 diabetic patient. A case report and review of the literature.We present the case of a 58-year-old man, who has suffered from type 1 diabetes mellitus since he was young. He had monoclonal IgM kappa gammopathy of undetermined significance and high anti-MAG antibody titer. He developed a polyneuropathic picture with the clinical and laboratory features of chronic inflammatory demyelinating polyneuropathy within the span of approximately 2 years. He benefited from IV administration of high doses of immunoglobulins. Investigation of all parameters, but particularly of the clinical phenotype, can lead to a better definition of the polyneuropathic picture, especially for therapeutic and prognostic purposes.- - - - - - - - - - ranking = 0.19937338905446keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/14. Unusual neurophysiological and immunological findings in myasthenia gravis: a case report.We describe the case of a male patient with ocular myasthenia gravis who developed a diabetic neuropathy similar to chronic inflammatory demyelinating polyradiculoneuropathy associated with transient generalized 'myokymic' discharges and distal weakness. He had antibodies against acetylcholine receptor and GQ1b ganglioside, but not anti-voltage-gated K( ) channel antibodies. Serial electrophysiological and immunological findings showed that diabetes was involved in the immune-mediated mechanism of peripheral neuropathy. We hypothesize that the concomitant appearance of distal motor weakness and decreased compound muscle action potentials upon repetitive nerve stimulation, together with increased distal motor latency and generalized peripheral nerve hyperexcitability, were all related to transient serum positivity to anti-GQ1b antibodies.- - - - - - - - - - ranking = 0.19937338905446keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/14. Complete bilateral relapsing ophthalmoplegia in a diabetic patient with a sensory-motor distal polyneuropathy.The clinicopathological study of a case of relapsing complete bilateral external ophthalmoplegia associated with a sensory-motor polyneuropathy is presented. No other causes apart from diabetes mellitus were ascertained. The sural biopsy demonstrated an axonal as well as demyelinating neuropathy. The physiopathology of the rare cases of diabetic multiple bilateral cranial nerve palsies is discussed.- - - - - - - - - - ranking = 0.0015665273638451keywords = demyelinating (Clic here for more details about this article) |
6/14. Relapsing inflammatory demyelinating polyneuropathy in a diabetic patient.Inflammatory demyelinating polyradiculoneuropathies exhibit well-known ultrastructural lesions of the peripheral nerve, both in acute cases, i.e., guillain-barre syndrome, and in relapsing, sub-acute and chronic cases. We present a case of relapsing inflammatory demyelinating polyradiculoneuropathy in a diabetic patient with a biopsy exhibiting these lesions, as well as a widening of the outermost myelin lamellae in some fibers. Such associated lesions are classic in experimental inflammatory demyelinating polyradiculoneuropathies, but have not been reported in human pathology.- - - - - - - - - - ranking = 0.37937723851039keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/14. Acute inflammatory demyelinating polyneuropathy in a diabetic patient: predominance of vesicular disruption in myelin sheaths.A diabetic woman underwent an incision of the right big toe for an abscess and developed a typical guillain-barre syndrome 48 h later. A biopsy of a peripheral nerve, performed 10 days later, showed modifications usually seen in diabetic patients, as well as the characteristic ultrastructural modifications of the guillain-barre syndrome (GBS). Moreover, 22% of myelinated fibers exhibited vesicular disruption of the myelin sheaths. This lesion is rarely encountered on the biopsies of peripheral nerve in GBS and concerns only a few myelinated fibers. Such a prominence of myelinic vesicular disruption and its occurrence in a diabetic patient are discussed.- - - - - - - - - - ranking = 0.25187380743103keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
8/14. Compression injury of the sciatic nerve during a prolonged surgical procedure in a diabetic patient.patients with diabetes mellitus and an underlying mixed axonal and demyelinating polyneuropathy are particularly prone to compression neuropathies with consequent axonal degeneration and a poor prognosis for recovery. This hazard should be taken into account whenever a prolonged surgical procedure is contemplated. An illustrative case is presented.- - - - - - - - - - ranking = 0.0015665273638451keywords = demyelinating (Clic here for more details about this article) |
9/14. Successful treatment of neuropathies in patients with diabetes mellitus.OBJECTIVES: To report and characterize two forms of disabling progressive peripheral neuropathy in patients with diabetes mellitus, which respond to anti-inflammatory and/or anti-immune treatment. DESIGN: review of clinical, electrophysiologic, and pathologic findings and results of treatment. SETTING: University medical center. patients: Twenty-one patients with diabetes mellitus to whom we gave anti-inflammatory and/or anti-immune treatment for progressive peripheral neuropathy during the past 6 years. MAIN OUTCOME MEASURES: patients were interviewed and examined at intervals before and after beginning treatment with intravenous immunoglobulin (n = 15), prednisone (n = 13), cyclophosphamide (n = 5), plasma exchange (n = 3), and azathioprine (n = 1) (alone or in combination). RESULTS: Fifteen patients had evidence of axonal neuropathy by electrophysiologic studies (group A). All 15 patients had non-insulin-dependent diabetes mellitus, 10 patients had weight loss, and 13 patients had prominent involvement of thighs and/or thoracic bands consistent with diabetic amyotrophy or mononeuropathy multiplex. Small vessel disease was seen in all 10 patients who underwent biopsy, with perivascular or vascular inflammation seen in seven patients. Six patients had demyelinating neuropathy by electrophysiologic criteria (group B). All these patients had insulin-dependent diabetes mellitus, and no one had weight loss. The process was asymmetric in three patients and involved thoracic or abdominal regions in two patients. Onion bulbs were seen in all four patients who underwent biopsy, but no vascular inflammation or occlusion was seen. In all patients in both groups, worsening of their conditions stopped and improvement started after beginning treatment. CONCLUSION: Neuropathies responsive to anti-inflammatory and/or anti-immune therapy in patients with diabetes mellitus include (1) multifocal axonal neuropathy caused by inflammatory vasculopathy, predominantly in patients with non-insulin-dependent diabetes mellitus, indistinguishable from diabetic proximal neuropathy or mononeuropathy multiplex, and (2) demyelinating neuropathy indistinguishable from chronic inflammatory demyelinating polyneuropathy, predominantly in patients with insulin-dependent diabetes mellitus.- - - - - - - - - - ranking = 0.20250644378215keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/14. Accelerated neuropathy of renal failure.OBJECTIVE--To describe a syndrome of rapidly evolving polyneuropathy in patients with severe renal failure. DESIGN--Retrospective case series of four patients. SETTING--In-hospital evaluations and personal examinations of patients. patients--Four patients with severe or end-stage renal failure who were receiving peritoneal dialysis. RESULTS--These patients had an acute or subacute syndrome characterized by generalized limb weakness over days or weeks, severe imbalance, diminished reflexes, and numbness. Spinal fluid protein levels were elevated and some demyelinating features were noted on electrophysiological testing. Improvement occurred with more frequent peritoneal dialysis in one patient and transplantation in another, but the neuropathy progressed in the other two in whom diabetes may have played a role. CONCLUSION--This partly reversible acute uremic neuropathy, which is probably caused by the metabolic disturbances of end-stage renal failure, simulates guillain-barre syndrome or chronic inflammatory demyelinating polyneuropathy and may be complicated by diabetic neuropathy.- - - - - - - - - - ranking = 0.20093991641831keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
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