1/112. 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 = 1keywords = nerve (Clic here for more details about this article) |
2/112. Autonomic nerve tumour with skeinoid fibres: ultrastructure of skeinoid fibres examined by quick-freezing and deep-etching method.A case of gastrointestinal autonomic nerve tumour with skeinoid fibres (SFs) of the jejunum in a 79-year-old Japanese man, was examined by the quick-freezing and deep-etching (QF-DE) method. The tumour consisted of spindle cells with immunohistochemical reactions for vimentin, NSE and CD34. Electron microscopically, features of the neural cells of the myenteric plexus were observed. The QF-DE method demonstrated intercellular meshwork structures, consisting of thin filaments (7-15 nm), with granular deposits. Fully developed parts of the deposits formed nodular aggregates composed of irregularly surfaced thick fibrils (30-48 nm) with a tendency to linear arrangement (SFs). We detected many interconnecting thin filaments (ICTFs) between the SFs, which were pre-existing components in the meshwork, avoiding the granular deposits. The focal thickening formed by the connection between SFs and ICTFs revealed a periodicity typical of SFs (33-45 nm). We conclude that SFs are formed by decoration of the granular deposits along pre-existing intercellular meshwork structures.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
3/112. I-123 MIBG cardiac imaging in diabetic neuropathy before and after epalrestat therapy.I-123 metaiodobenzylguanidine (MIBG) scintigraphy is a new method to evaluate cardiac sympathetic nerve disturbance in patients with diabetes mellitus. Epalrestat specifically inhibits aldose reductase and improves diabetic neuropathy. The authors report a case of improvement in cardiac sympathetic dysfunction using MIBG scintigraphy with epalrestat therapy. In this case, epalrestat effectively reversed diabetic neuropathy, and MIBG scintigraphy was useful to evaluate the effect of epalrestat.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/112. High mitotic index associated with poor prognosis in gastrointestinal autonomic nerve tumour.AIMS: Three gastrointestinal autonomic nerve tumours (GANT) were characterized by immunohistochemistry and flow cytometry. Two of the three cases occurred in the small intestine, while the third was found in the stomach. Besides the immunohistochemical and ultrastructural description, the aim of this study was to examine the relation between the known and accepted predictive factors (ploidy data, the S-phase fraction, the mitotic and MIB-1 index and the size of the tumour) and the survival of the patients. methods AND RESULTS: The immune profile showed that 3/3 cases were vimentin and NSE, 2/3 were synaptophysin and PGP 9.5 positive, while 1/3 also showed S100 positivity. Ultrastructurally, all the cases had dense core granules, one of them contained skenoid fibres. The flow cytometry revealed diploid dna in all cases, however, significant differences could be seen in the proliferative activity of the individual neoplasms. CONCLUSIONS: In spite of the published data of gastrointestinal stromal tumours (GIST) generally, neither the MIB-1 index and the ploidy data nor the size of the primary tumour helped to predict the clinical progression of the examined GANTs. However, the high proliferative activity (57 mitoses/10 HPF) and the elevated S-phase fraction (24%) was associated with advanced, metastatic and recurring disease in case 3. On the basis of these three cases, high mitotic activity is the most reliable factor in predicting aggressive clinical behaviour.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
5/112. GANT-like gastrointestinal pacemaker cell tumours with oncocytic features.We describe two cases of gastrointestinal stromal tumours with prominent oncocytic features. Both had features consistent with differentiation towards the interstitial cells of cajal (CC). They were composed of nests and bundles of cells with abundant, deeply granular, eosinophilic cytoplasm. Immunohistochemical investigations revealed positivity with c-kit, vimentin and CD34 antibodies in both neoplasms. Ultrastructurally the neoplastic cells showed characteristic features of CC; they had synapse-like structures and dense core cytoplasmic granules. Oncocytic features were confirmed by immunohistochemistry using anti-mitochondrion antibody in both cases and by electron microscopy in one case (case 1). Although the CC are frequently described as mitochondrion-rich cells, oncocytic changes have not previously been reported as a feature of gastrointestinal autonomic nerve tumour (GANT)-like stromal tumours.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
6/112. Schwannoma of the cervical sympathetic chain. The virginia experience.We present 4 cases of schwannomas arising from the cervical sympathetic chain. These lesions are uncommon and most often present as an asymptomatic solitary neck mass. Preoperative diagnosis can be difficult, even with the aid of computed tomography, magnetic resonance imaging, ultrasound, and angiography. While a paraganglioma can often be ruled out, exact determination of the nerve of origin is frequently elusive until the time of surgery. Operative excision remains the treatment of choice, often requiring sacrifice of a portion of the sympathetic chain. Postoperative Horner's syndrome is common, but does not appear to have an adverse effect on the patient.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
7/112. Clinico-pathological features of postural hypotension in diabetic autonomic neuropathy.We report the clinico-pathological features and management of a 49-year-old male with a 30-year history of Type 1 diabetes mellitus who had nephropathy (proteinuria 1.81 g/24 h, creatinine 136 micromol/l), proliferative retinopathy and severe somatic and autonomic neuropathy. A sural nerve biopsy demonstrated marked myelinated fibre loss with unmyelinated fibre degeneration and regeneration combined with extensive endoneurial microangiopathy. The management of the patient's blood pressure problems (supine hypertension) and symptomatic postural hypotension is discussed.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
8/112. Gastrointestinal autonomic nerve (GAN) tumor of the rectum.Gastrointestinal autonomic nerve (GAN) tumor is a relatively newly defined member of the gastrointestinal stromal tumor family, which is a rare group of mesenchymal neoplasms originating in the bowel wall throughout the entire gastrointestinal tract. A thorough search of the literature revealed only one brief mention of this tumor arising in the rectum. We present a full description of such a tumor and summarize the clinical characteristics, therapeutic measures used, biological behavior, and outcome of this unique case.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
9/112. 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 = 0.5keywords = nerve (Clic here for more details about this article) |
10/112. Plexosarcoma: endoscopic ultrasound and electron-microscopic characteristics of a stromal tumor.Endoscopic ultrasound is useful for managing submucosal masses; however, some of these lesions can be difficult to classify except with full histological and electron microscopic evaluation. A 72-yr-old woman was seen with upper GI bleeding. endoscopy showed a 1.7-cm sessile ulcerated submucosal mass in the duodenal bulb. Endoscopic ultrasound revealed an echolucent submucosal mass arising from the fourth echolayer, the muscularis propria of the duodenal wall. These findings suggested that the lesion was a leiomyoma. The patient eventually had the lesion resected because of recurrent bleeding. Histologically it was a spindle cell tumor that on electron microscopy showed neuronal elements consistent with a plexosarcoma, or gastrointestinal autonomic nerve tumor. These lesions account for some one third of all gastrointestinal stromal tumors. Despite their low grade malignant histologic appearance, local recurrence or hepatic metastases occur in about 70% of patients.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
| | Next -> |