1/40. Neurological manifestations of chronic hepatitis c.hepatitis c virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement c4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. complement c3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis c associated vasculitis should be considered in the differential diagnosis.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/40. Severe lead-induced peripheral neuropathy in a dialysis patient.Toxic neuropathy caused by lead (manifested as wrist drop) was a frequent phenomenon before 1925. In modern times, it is a distinct rarity. We report herein a Hispanic woman who developed end-stage renal failure, followed by wrist drop, in whom the maximal total blood lead was 69 microg/dL. Measurements of lead in her tibia and calcaneus by K-x-ray fluorescence, however, showed markedly elevated values. The wrist drop cleared after four treatments with intravenous calcium sodium edetate (Ca EDTA). in vitro studies of (210)Pb uptake by red blood cells (RBC) after incubation with normal or uremic plasma indicated that (210)Pb uptake was inhibited by uremic plasma. These studies suggest the presence of a transport inhibitor in uremia that modifies the distribution of lead between plasma and RBC, leading to lower overall blood values.- - - - - - - - - - ranking = 1.0762263145074keywords = blood cell (Clic here for more details about this article) |
3/40. Fulminant peripheral neuropathy with severe quadriparesis associated with vincristine therapy.OBJECTIVE: To report a case of fulminant neuropathy with severe quadriparesis associated with vincristine chemotherapy. CASE SUMMARY: A 48-year-old white man with acute lymphoblastic leukemia was started on an induction chemotherapeutic regimen that included intravenous vincristine. He received a total of 6 mg of vincristine over two weeks during induction chemotherapy. Over the next two weeks, he developed a fulminant peripheral neuropathy with severe quadriparesis. DISCUSSION: Although commonly associated with peripheral neuropathy, vincristine neurotoxicity only rarely involves instances of fulminant peripheral neuropathy with severe quadriparesis. guillain-barre syndrome is also associated with leukemia and may present as a fulminant peripheral neuropathy with severe quadriparesis. CONCLUSIONS: Fulminant neuropathy with severe quadriparesis occurring in patients with leukemia being treated with vincristine (and who do not have coexistent charcot-marie-tooth disease) is more likely due to guillain-barre syndrome than to vincristine neurotoxicity.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/40. Treatment of neuropathic pain with venlafaxine.OBJECTIVE: To report a case of successful treatment of neuropathic pain with venlafaxine. CASE REPORT: A 39-year-old white woman presented with neuropathic back pain. The patient obtained 50% pain relief with consecutive use of amitriptyline, desipramine, and imipramine. Anticholinergic effects prompted a switch to extended-release venlafaxine 75 mg/d. Pain relief was as effective with this therapy as with the tricyclic antidepressants. The absence of adverse effects allowed the patient to discontinue all laxatives. DISCUSSION: Venlafaxine is an antidepressant that inhibits reuptake of norepinephrine and serotonin. This is the major mechanism by which tricyclic antidepressants relieve neuropathic pain. Venlafaxine does not bind to muscarinic-cholinergic, histaminic or alpha1-adrenergic receptors responsible for the common adverse effects seen with tricyclic antidepressants. CONCLUSIONS: This report describes the efficacious use of venlafaxine in the treatment of neuropathic pain. Double-blind, randomized, controlled trials are needed to explore this further.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/40. Neurolymphomatosis associated with muscle and cerebral involvement caused by natural killer cell lymphoma: a case report and review of literature.We report a biopsy-proven case of neurolymphomatosis (NL) presenting with sensory motor axonal polyneuropathy, polymyositis, and cerebral involvement. Ours is the second reported case of NL caused by natural killer-cell lymphoma defined by morphology and immunophenotyping. For 3 months, the patient developed stocking-glove distribution of hypesthesia, subacute progressive weakness and mental deterioration. EMG showed severe sensorimotor mixed axonal-demyelinating polyradiculoneuropathy. Lumbar puncture revealed mildly high protein level with normal glucose and cell count. sural nerve biopsy demonstrated lymphomatous axonal neuropathy and muscle biopsy was indicative of lymphomatous polymyositis. brain MRI revealed multiple white matter lesions, consistent either with progressive multifocal leukoencephalopathy or cerebral lymphoma. bone marrow biopsy showed neoplastic infiltrates. The patient died of multiple organ failure prior to initiation of chemotherapy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/40. Optico-cochleo-dentate degeneration associated with severe peripheral neuropathy and caused by peroxisomal D-bifunctional protein deficiency.The clinical, neuroradiological, neuropathological and biochemical findings in a patient with optico-cochleo-dentate degeneration (OCDD; OMIM 258700) are presented in a severe case succumbing at the age of 4 years. The electron microscopic and biochemical data showed for the first time that OCDD may occur as the phenotypic expression of D-bifunctional protein deficiency, i.e., a peroxisomal disorder. The boy was born as the first child of healthy, consanguineous parents of Turkish origin. No other family members were affected. The main clinical symptoms consisted of muscle hypotonia ("floppy infant"), generalized epileptic fits, hypacusis, rotatory nystagmus, insufficient pupillary reactions, and mental retardation. Fibroblast cultures revealed D-bifunctional protein deficiency. Neuropathological examination displayed moderate frontoparietal and insular microgyria, and atrophy of the cerebellum. Loss of neurons was severe in the granular layer, the Purkinje cell band of the cerebellum, and rather complete in the dentate nucleus. A corresponding loss of myelinated fibers associated with characteristic periodic acid-Schiff-positive macrophages was most prominent in the white matter of the cerebellum. There was additional severe loss of myelinated fibers in the central portions of the optic nerve, reduction of the nerve fiber density in the cochlear nerve, and reduction of myelinated nerve fibers by about 80-90% in the sural nerve, which has not been studied in previous cases. At the electron microscopic level, characteristic inclusions mainly in perivascular macrophages and astrocytes were the most prominent finding. The inclusions usually showed a bilaminar structure, whereas trilaminar structures, typically seen in adrenoleukodystrophy, and multilaminar structures were less frequently seen.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/40. Masked deficit of vitamin B12 in the patient with heterozygous beta-thalassemia and spastic paraparesis.The spinal cord, brain, optic nerves and peripheral nerves may be affected by vitamin B12 (cobalamin) deficiency. Deficiency of vitamin B12 also causes megaloblastic anaemia, meaning that the red blood cells are usually larger than normal. In this paper we report a 16-year old girl who was referred to us for the evaluation of mild paraparesis and paresthesias marked by tingling "pins and needles" feelings and general weakness. The patient, her parents and sisters were on a strict vegan diet, which made us believe that vitamin B12 deficiency may be the possible cause of the neurologic clinical manifestations. The serum level of vitamin B12 was low, but there was no macrocytosis in the routine blood examination. The electrophoresis of haemoglobin was pathologic, there was 3.7% of HbA2 and 11.6% of HbF (heterozygous form of beta-thalassaemia). When megaloblastic anaemia occurs in combination with a condition that gives rise to microcytic anaemia, many megaloblastic features may be masked. Instead of being macrocytic, the anaemia could be normocytic or even microcytic. Vitamin B12 deficiency is a diagnosis that must not be overlooked. This case report turns the light on the fact that increased MCV is a hallmark in vitamin B12 deficiency, but it is not an obligatory sign.- - - - - - - - - - ranking = 1.0762263145074keywords = blood cell (Clic here for more details about this article) |
8/40. Peripheral neuropathy in vanishing white matter disease with a novel EIF2B5 mutation.The authors describe an infant with vanishing white matter disease with demyelinating peripheral neuropathy. sequence analysis of EIF2B5 gene showed that the patient was a double heterozygote, with novel missense mutation CGA-->CAA in codon 269 of exon 6, resulting in the replacement of an arginine residue with glutamine.- - - - - - - - - - ranking = 5keywords = white (Clic here for more details about this article) |
9/40. Leukoencephalopathy as a rare complication of hepatitis c infection.We report the case of a 64-year-old female patient with hepatitis c infection (HCV), who developed Sjogren's disease and sensory peripheral neuropathy. Clinical conditions worsened over three years with central nervous system involvement characterised by transient third cranial nerve paresis and mild selective impairment of attention and memory. brain magnetic resonance imaging showed diffuse periventricular and lobar white matter hyperintensity. Laboratory findings included mixed cryoglobulinaemia (type II), cryocrit 1.47%, low serum levels of complement c4 and high levels of rheumatoid factor, HCV 1b genotype, high HCV mRNA levels in serum and cerebrospinal fluid. skin biopsy showed evidence of vasculitis. After one year of plasmapheresis, immunosuppressant therapy and occasional corticosteroid treatment, neurological symptoms improved, skin biopsy changed and inflammation parameters normalised, suggesting that neurological symptoms might be related to the high levels of mixed cryoglobulins.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/40. chromium deficiency, glucose intolerance, and neuropathy reversed by chromium supplementation, in a patient receiving long-term total parenteral nutrition.A white female, now age 40 and receiving total parenteral nutrition for more than 5 years, developed unexpected 15% weight loss after 3 1/2 years of regimen, together with peripheral neuropathy confirmed by nerve conduction measurements. An intravenous glucose tolerance test showed that the fractional rate (K) had decreased to 0.89%/min (normal greater than 1.2). There was observed during this glucose infusion a borderline normal insulin response with a fall in plasma free fatty acids and in plasma leucine. During daily infusion of well over 400 g of glucose, the respiratory quotient was 0.66. chromium balance was negative. chromium levels were, in blood 0.55 ng/ml (normal 4.9 to 9.5) and in hair 154 to 175 ng/g (normal greater than 500). Regular insulin daily (45 micron) in the infusate nearly maintained euglycemia but despite this, and even with further glucose intake to restore weight loss, intravenous glucose tolerance test (K) and respiratory quotient were unchanged. Administration of insulin was then stopped and 250 microng of Cr added to the daily total parenteral nutrition infusate for 2 weeks. After this the intravenous glucose tolerance test (K) and respiratory quotient became normal (1.35 and 0.78, respectively). Over the next 5 months insulin was not needed and glucose intake had to be reduced substantially to avoid overweight. In this period nerve conduction and well-being returned to normal. With a maintenance addition of chromium to the total parenteral nutrition infusate (tentatively this addition is 20 microng/day) the patient has remained well for 18 months (to July 1976). These results suggest that relatively isolated chromium deficiency in man, hitherto poorly documented, causes 1) glucose intolerance, 2) inability to utilize glucose for energy, 3) neuropathy with normal insulin levels, 4) high free fatty acid levels and low respiratory quotient and, 5) abnormalities of nitrogen metabolism.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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