1/80. 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 = area (Clic here for more details about this article) |
2/80. 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 = 852.54851420352keywords = plexus (Clic here for more details about this article) |
3/80. intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection.We report the association of neurological and intestinal disorders with the reactivation of Epstein-Barr virus (EBV) in a child. This previously healthy 13-yr-old boy presented with pharyngitis and acute abdominal ileus. laparotomy excluded a mechanical obstruction. Postoperatively, he suffered from prolonged intestinal obstruction, pandysautonomia, and encephalomyelitis. Histological examination of the appendix and a rectal biopsy taken 3 months after the onset showed an absence of ganglion cells (appendix) and hypoganglionosis (rectum), with a mononucleate inflammatory infiltrate in close contact with the myenteric neural plexuses. EBV-PCR was positive in the blood and cerebrospinal fluid, and in situ hybridization with the Epstein-Barr virus encoded rna probe showed positive cells throughout the appendix wall including the myenteric area, in a mesenteric lymph node, and in the gastric biopsies. EBV spontaneous lymphocytic proliferation was noted in the blood. The serology for EBV showed previous infection but anti-early antigen antibodies were present. No immunodeficiency was found. Neurological and GI recovery occurred after 6 months of parenteral nutrition and bethanechol. The omnipresence of EBV associated with the neurointestinal symptoms suggest that the virus was the causal agent. This is the first documented case of acquired hypoganglionnosis due to EBV reactivation.- - - - - - - - - - ranking = 853.54851420352keywords = plexus, area (Clic here for more details about this article) |
4/80. 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 = 852.54851420352keywords = plexus (Clic here for more details about this article) |
5/80. Hormonal and cardiovascular reflex assessment in a female patient with pure autonomic failure.We report the case of a 72-year-old female with pure autonomic failure, a rare entity, whose diagnosis of autonomic dysfunction was determined with a series of complementary tests. For approximately 2 years, the patient has been experiencing dizziness and a tendency to fall, a significant weight loss, generalized weakness, dysphagia, intestinal constipation, blurred vision, dry mouth, and changes in her voice. She underwent clinical assessment and laboratory tests (biochemical tests, chest X-ray, digestive endoscopy, colonoscopy, chest computed tomography, abdomen and pelvis computed tomography, abdominal ultrasound, and ambulatory blood pressure monitoring). Measurements of catecholamine and plasmatic renin activity were performed at rest and after physical exercise. Finally the patient underwent physiological and pharmacological autonomic tests that better diagnosed dysautonomia.- - - - - - - - - - ranking = 5.0401052566995keywords = chest (Clic here for more details about this article) |
6/80. Statuslike SUNCT in two young women.OBJECTIVE: To describe the statuslike pattern of SUNCT (short-lasting unilateral neuralgiform pain with conjunctival injection and tearing) in two young women. BACKGROUND: sunct syndrome is a rare condition characterized by a short-lasting periocular pain associated with marked autonomic symptoms. Twenty-five cases have been reported in the literature with a high preponderance of males and a mean age of 51 years. The frequency of episodes shows a wide variability, not just among individuals but also in the same patient, and a statuslike pattern of almost continuous attacks has been described. methods: We report the cases of two young women (aged 26 and 23 years) with typical SUNCT features who suffered bouts of up to 60 paroxysms of pain per hour. Paraclinical investigations showed no abnormalities. CONCLUSIONS: Although unusual, paroxysms in SUNCT may overlap into a clinical status. A strong relationship with hormonal changes was noted in one patient. In both cases, the pain was refractory to treatment with indomethacin, carbamazepine, and hypnotics, and only intravenous methylprednisolone with oral carbamazepine may have been partially effective in one case.- - - - - - - - - - ranking = 115.04382741719keywords = pain (Clic here for more details about this article) |
7/80. Bowel perforation due to gastrointestinal autonomic nerve tumour associated with neurofibromatosis type 1.Gastrointestinal autonomic nerve (GAN) tumours form a distinct subcategory of gastrointestinal stromal tumours, and are thought to originate from the enteric autonomic plexus. To date, a total of 45 cases have been documented in the literature; a few of these cases were associated with neurofibromatosis type 1 (NF-1). Bowel perforation due to other gastrointestinal stromal tumours has been reported only twice, but never in association with a GAN tumour. We describe a 40-year-old woman with NF-1 who had bowel perforation due to a GAN tumour. The patient underwent radical surgical resection and remained tumour free for at least 4 years, which may indicate a good prognosis.- - - - - - - - - - ranking = 852.54851420352keywords = plexus (Clic here for more details about this article) |
8/80. Familial association of autonomic and gastrointestinal symptoms.Autonomic dysfunction occurs in the adult population with irritable bowel syndrome, but this association is not recognized in children. A mother and son with functional abdominal pain unresponsive to conventional treatment had complete resolution of symptoms with treatment directed at the autonomic dysfunction identified by testing. The authors recommend autonomic testing in patients with functional abdominal pain and suggest that autonomic dysfunction plays a direct and intrinsic role in the mechanism of these disorders and their symptoms.- - - - - - - - - - ranking = 57.521913708594keywords = pain (Clic here for more details about this article) |
9/80. cluster headache sine headache: two new cases in one family.We report two cases of cluster headache sine headache occurring in a family of cluster headache sufferers. A 54-year-old female, suffered for 15 years from short-lasting, right-sided attacks of eye reddening, lacrimation, rhinorrea, palpebral oedema and miosis, seven to eight times per day, in periods of 20-30 days. She did not experience headache, either with or without these phenomena. Her son suffers from periorbital, short-lasting, right-sided painful attacks accompanied by eye reddening, lacrimation, rhinorrea and nasal stiffnesss, occurring in periods of 30-35 days. Soon after the end of the current cluster period he still had daily attacks lasting 10-15 min, with the oculo-nasal autonomic phenomena only. The occurrence of cluster headache sine headache and cluster headache in the same family strongly suggests that the first condition is related to the cluster headache syndrome. cluster headache sine headache should be considered as a subtype of the cluster headache syndrome.- - - - - - - - - - ranking = 28.760956854297keywords = pain (Clic here for more details about this article) |
10/80. Resolution of diabetic autonomic neuropathy.Many consider diabetic autonomic neuropathy to be an irreversible complication of diabetes of long duration. Three patients developed symptoms of autonomic neuropathy which subsequently resolved. Their autonomic neuropathy was not associated with long duration of diabetes, but with weight loss. Each had marked weight loss and resolution occurred on regaining remembered premorbid weight. A woman aged 20 was admitted with anorexia nervosa (weight loss 6 kg). She complained of feeling bloated. Gastroenterological investigations showed delayed gastric emptying. RR ratio (respiration and standing) was abnormal. Resolution occurred after two years. A male aged 18 developed diabetic symptoms, which were overlooked. Twelve months later he presented underweight and ketonuric; insulin treatment was started but within one month he became impotent. Resolution occurred after 18 months. An 80 year old man presented after six months trial of diet and sulphonylurea therapy. He was underweight, had ketonuria, and such muscle loss that he was unable to sit unaided. insulin treatment was started. He developed severe symptomatic postural hypotension. This resolved six months later by which time he had regained his normal weight. These cases illustrated symptomatic autonomic neuropathy occurring in relation to weight loss with resolution on recovery of normal weight, a temporal pattern mimicking that of acute cachectic painful neuropathy. Treatment of autonomic neuropathy should be like that of cachectic neuropathy, that is with an expectation of recovery and should include strategies to regain premorbid weight and achieve glycaemic control.- - - - - - - - - - ranking = 28.760956854297keywords = pain (Clic here for more details about this article) |
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