1/101. Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy.Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/101. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
3/101. Acute inflammatory neuropathy in charcot-marie-tooth disease.The authors report an association between acute inflammatory neuropathy and previously undiagnosed Charcot-Marie-Tooth 1A disease in a 15-year-old girl. sural nerve biopsy study showed hypertrophic neuropathy with endoneurial infiltrates of macrophages and lymphocytes. This association may be coincidental, but a particular susceptibility to damage of these peripheral nerves cannot be excluded. This report confirms the importance of pes cavus as a sign of long-standing sensorimotor neuropathy.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
4/101. Probable lymphocytic hypophysitis diagnosed by short-term serial computed tomography and gallium-67 scintigraphy--case report.A 61-year-old female presented with headache, malaise, and left oculomotor nerve paralysis. Computed tomography (CT) demonstrated a diffuse pituitary mass and enlarged pituitary stalk with homogeneous contrast enhancement. Her symptoms gradually resolved without treatment. gallium-67 scintigraphy showed abnormal uptake in the pituitary lesion. Serial CT every 2 weeks after admission showed homogeneous contrast enhancement and shrinking of the pituitary mass to a normal size 12 weeks after the onset. The final diagnosis was lymphocytic adenohypophysitis without biopsy. recurrence has not been observed for 8 years after discharge. The patient did not need hormone replacement therapy. Histological examination is not always necessary to diagnose probable lymphocytic adenohypophysitis with the characteristic feature of rapid onset, abnormal gallium-67 uptake in the lesion, and resolution of symptoms in the acute stage with shrinking of the lesion on neuroimaging.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
5/101. Serious infections from bacillus sp.Serious infections caused by organisms of the genus bacillus developed in seven patients. Five drug abusers had either endocarditis or osteomyelitis, one leukemic patient had necrotizing fasciitis, and one patient had a ventriculoatrial shunt infection with recurrent bacteremia. All patients recovered. Experience with these cases reemphasizes the importance of not dismissing bacillus organisms as culture contaminants, especially when isolated from blood, body fluids, or closed-space infections.- - - - - - - - - - ranking = 0.29476702764232keywords = organ (Clic here for more details about this article) |
6/101. Unusual complications in an inflammatory abdominal aortic aneurysm.An unusual case of an inflammatory abdominal aortic aneurysm (IAAA) associated with coronary aneurysms and pathological fracture of the adjacent lumbar vertebrae. The associated coronary lesions in cases of IAAA are usually occlusions. In the present case, it was concluded that a possible cause of the coronary aneurysm was coronary arteritis and the etiology of the pathological fracture of the lumbar vertebrae was occlusion of the lumbar penetrating arteries due to vasculitis resulting in aseptic necrosis. Inflammatory AAA can be associated with aneurysms in addition to occlusive disease in systemic arteries. The preoperative evaluation of systemic arterial lesions and the function of systemic organs is essential.- - - - - - - - - - ranking = 0.14738351382116keywords = organ (Clic here for more details about this article) |
7/101. Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis.We present the MR imaging findings in a 43-year-old male patient with bilateral idiopathic sclerosing inflammation of the orbit. Bilateral enhancing retrobulbar masses, with concentric compression of the retrobulbar segment of the left optic nerve, were seen. MR imaging proved to be the only means to distinguish between the different intraorbital structures and to determine the exact site of optic nerve compression. To our knowledge, this is the first documented case of MR imaging findings of this entity.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
8/101. immunotherapy of cancer patients with bacillus Calmette-Guerin: summary of four years of experience in japan.Active immunotherapy with living BCG was conducted on 98 patients with various types of cancer. The candidates for this therapy were patients with residual or inoperable cancer of the colorectum, liver, breast, biliary tract, lung, and other organs with a follow-up of 4-58 months. Eleven of the 98 (11%) were able to survive for as long as 37-58 months (mean survival time 42.5 months) because of this treatment and are still living. Another 11 patients are also alive more than 24 months after starting treatment. Thirty-seven patients, however, succumbed within 12 months despite BCG immunotherapy. On the other hand, 37 patients in the control group, who shared the same clinical status and did not receive BCG therapy during this period, underwent unhappy courses for 2-12 months (mean survival time 8.7 months). The pretreatment immunoresponsiveness of these 98 patients was suppressed, as measured by the following immunologic parameters: T-cell subpopulation in the peripheral blood, stimulation index of PHA, and skin tests to DNCB, KLH, PPD, and PHA. All of these parameters improved shortly after initiation of BCG injections in 22 patients who survived more than 24 months. In contrast, in patients who died within 12 months, immunoresponsiveness remained suppressed throughout the course. This result has suggested that there was an apparent correlation between the effectiveness of BCG and immunoresponsiveness. In addition, a good correlation was observed between the duration of inflammatory reactions at BCG injection sites and clinical prognoses. Moreover, it was shown that a relatively high amount of BCG (20-80 mg as an initial dosage) and repeated injections of living BCG were necessary to obtain a sufficient enhancing effect on the immunocompetency of these late-stage cancer patients. The most conventional criterion used to determine an optimal time for booster injections of BCG was measurement of the PPD-evoked skin reaction at the BCG injection site, that is, Koch's phenomenon. When a marked flare-up reaction of more than 2.5 X 2.5 cm in size was observed, the effect of BCG was considered to be continuing, and no additional booster injection was needed. The mean interval between the first and second BCG injections was 6.2 /-1.1 months in patients who survived more than 2 years. In contrast, the duration of this reaction was only transient in ineffective cases. The most frequent side effects of this therapy were fever and malaise; these complications occurred in 62% of the cases. No severe side effects, such as dissemination, anaphylactic shock, or granulomatous hepatitis, have been experienced throughout this study, even in patients to whom a total dosage of more than 200 mg of living BCG were injected.- - - - - - - - - - ranking = 0.14738351382116keywords = organ (Clic here for more details about this article) |
9/101. Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult.Inflammatory infiltration of intestinal myenteric plexuses (i.e. myenteric ganglionitis), along with severe intestinal motor abnormalities, may accompany paraneoplastic syndromes, neurological disorders and gastrointestinal infections, although rare cases can be idiopathic. In this report, we describe the case of a patient who presented with chronic intractable vomiting and weight loss associated with idiopathic myenteric ganglionitis mainly involving the stomach. Tissue analysis showed that the inflammatory infiltrate comprised T lymphocytes (CD4 and CD8 ), and peptide immunolabelling revealed a marked decrease of substance p/tachykinin immunoreactive staining in nerve fibres and myenteric neurones. Following systemic steroid therapy, the patient's symptoms dramatically improved, and after one year of follow-up his general condition remains satisfactory. The possible mechanisms leading to symptom generation and gastric dysmotility in the context of an idiopathic myenteric ganglionitis are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
10/101. Lymphocytic adenohypophysitis mimicking a pituitary macroadenoma.A 24-year-old woman developed headache and rapidly progressive visual disturbances during the last trimester of her first pregnancy. Magnetic resonance tomography (MRI) of the brain documented an intra- and suprasellar mass lesion. For preservation of vision, transsphenoidal microsurgical decompression was performed. Immediately postoperatively, visual acuity improved and hemianopia resolved. Histological examination yielded the diagnosis of primary lymphocytic adenohypophysitis. This is a rare inflammatory pituitary disease. There are no typical clinical, laboratory, or radiological findings that allow precise preoperative diagnosis. Even though this autoimmune disorder is principally steroid-responsive, an improvement of visual disturbances under steroid therapy cannot be predicted. Therefore, surgery is justified not only to establish the diagnosis, but also to restore vision by decompression of the optic nerves and the chiasm.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
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