Fulminant Attacks Research Articles

Clinical Correlation of Multiple Sclerosis Immunopathologic Subtypes.

The goal of this work was to compare clinical characteristics across immunopathologic subtypes of patients with multiple sclerosis. Immunopathologic subtyping was performed on specimens from 547 patients with biopsy- or autopsy-confirmed CNS demyelination. The frequency of immunopathologic subtypes was 23% for pattern I, 56% for pattern II, and 22% for pattern III. Immunopatterns were similar in terms of age at autopsy/biopsy (median age 41 years, range 4-83 years, p = 0.16) and proportion female (54%, p = 0.71). Median follow-up after symptom onset was 2.3 years (range 0-38 years). In addition to being overrepresented among autopsy cases (45% vs 19% in biopsy cohort, p < 0.001), index attack-related disability was higher in pattern III vs II (median Expanded Disability Status Scale score 4 vs 3, p = 0.02). Monophasic clinical course was more common in patients with pattern III than pattern I or II (59% vs 33% vs 32%, p < 0.001). Similarly, patients with pattern III pathology were likely to have progressive disease compared to patients with patterns I or II when followed up for ≥5 years (24% overall, p = 0.49), with no differences in long-term survival, despite a more fulminant attack presentation. All 3 immunopatterns can be detected in active lesions, although they are found less frequently later into the disease due to the lower number of active lesions. Pattern III is associated with a more fulminant initial attack than either pattern I or II. Biopsied patients appear to have similar long-term outcomes regardless of their immunopatterns. Progressive disease is less associated with the initial immunopattern and suggests convergence into a final common pathway related to the chronically denuded axon.

Acute Pancreatitis in a Pregnant Women at 30 - 31 Weeks of Gestational Age with Complete Cure

Introduction: Acute pancreatitis is an acute inflammatory disease of the pancreas characterized clinically by upper quadrant pain and elevated levels of enzymes in the blood. Although The pathogenesis of pancreatitis is not fully understood, gallstone and chronic alcohol abuse is considered for two-thirds or more cases in the united stated. Case Presentation: In this case report, the researchers present a 29-year-old pregnant female G3P2 with 31 w, 2 d of gestational age, who was referring to maternity ward with upper quadrant pain, nausea, and vomiting. Her ultrasound, examination, and blood analysis showed acute pancreatitis due to hyperlipidemia. The patient underwent six plasmapheresis and medical treatment and was discharged with complete cure at 34 weeks of gestational age. Conclusions: Accurate assessment of the incidence and mortality of acute pancreatitis is difficult as mild pancreatitis may be subclinical and deaths may occur before the diagnosis of sever and fulminant attacks. Mortality rate is three percent in patients with interstitial pancreatitis and 17% in patients with pancreatic necrosis.

Numb Chin Syndrome with Four Fulminant Attacks in 16 Years

Numb Chin Syndrome with Four Fulminant Attacks in 16 Years

Evidence for the Role of B Cells and Immunoglobulins in the Pathogenesis of Multiple Sclerosis

The pathogenesis of multiple sclerosis (MS) remains elusive. Recent reports advocate greater involvement of B cells and immunoglobulins in the initiation and propagation of MS lesions at different stages of their ontogeny. The key role of B cells and immunoglobulins in pathogenesis was initially identified by studies in which patients whose fulminant attacks of demyelination did not respond to steroids experienced remarkable functional improvement following plasma exchange. The positive response to Rituximab in Phase II clinical trials of relapsing-remitting MS confirms the role of B cells. The critical question is how B cells contribute to MS. In this paper, we discuss both the deleterious and the beneficial roles of B cells and immunoglobulins in MS lesions. We provide alternative hypotheses to explain both damaging and protective antibody responses.

Neuromyelitis Optica IgG Serostatus in Fulminant Central Nervous System Inflammatory Demyelinating Disease

The aquaporin-4-specific serum autoantibody neuromyelitis optica (NMO) IgG is a validated biomarker distinguishing NMO spectrum disorders from multiple sclerosis (MS). Because fulminant attacks are more common in NMO spectrum disorders than in MS, some investigators suggest that NMO IgG may be a marker of destructive demyelination rather than a disease-specific biomarker. To our knowledge, this study is the first to compare NMO IgG serostatus among patients with fulminant central nervous system inflammatory demyelinating disease (CNS IDD). To determine whether NMO IgG distinguishes patients with NMO spectrum disorders from those with other fulminant corticosteroid-refractory CNS IDD. Descriptive historical cohort. Neuroimmunology laboratory and neurology practice, Mayo Clinic College of Medicine, Rochester, Minnesota. Patients Serum samples from 74 patients who underwent plasmapheresis between February 24, 1993, and November 22, 2007, for a corticosteroid-refractory CNS IDD were tested for NMO IgG by indirect immunofluorescence assay. Two blinded observers scored serum samples tested at 1:120 dilution. Clinical data were obtained by medical record review. Preplasmapheresis serum samples were available from 74 patients (ratio of women to men, 2:5); the mean interval between blood draw and plasmapheresis was 13 days. At the time of plasmapheresis, the mean age of patients was 46 years (age range, 7-80 years); the mean Expanded Disability Status Scale score was 7.0 (score range, 3.5-9.5 [10.0 is death]). Diagnoses included MS (18 patients with definite and 11 patients with probable), longitudinally extensive transverse myelitis involving at least 3 vertebral segments (20 patients), NMO (14 patients), transverse myelitis involving fewer than 3 vertebral segments (8 patients), optic neuritis (2 patients), and acute disseminated encephalomyelitis (1 patient). Neuromyelitis optica IgG was detected in 20 patients (27%) (10 with longitudinally extensive transverse myelitis, 9 with NMO, and 1 with recurrent optic neuritis) and was not detected in any patient with MS, short transverse myelitis, monophasic optic neuritis, or acute disseminated encephalomyelitis. Neuromyelitis optica IgG is a specific biomarker for NMO spectrum disorders and is not simply a marker of destructive CNS IDD.

Lesion pathology predicts response to plasma exchange in secondary progressive MS

Plasma exchange has been evaluated for treatment of acute inflammatory demyelinating syndromes of the CNS, e.g., acute relapsing multiple sclerosis (MS).1–3 It was recently reported that the pathologic pattern type II4 predicts the response to plasmapheresis treatment.5 Notably, however, these investigators had studied patients with fulminant attacks of MS. In a commentary,6 these findings were put in the context of older studies on the utility of plasma exchange in MS. Here, we report on a 32-year-old woman with a >6-year history of MS who had entered the chronic progressive phase 17 months before starting plasma exchange therapy. Prior to this, the patient had …

Attack-related severity: a key factor in understanding the spectrum of idiopathic inflammatory demyelinating disorders

Understanding the spectrum of idiopathic inflammatory demyelinating disorders (IIDD) is a fundamental issue for the diagnosis and treatment of these disorders as well as for the approach to their pathogenesis. The spectrum of IIDD is usually classified according to clinical course and lesion distribution. We compared the demographic features, clinical characteristics, laboratory findings, and genetic backgrounds between 193 Japanese patients with and without clinically or radiographically fulminant attacks who all satisfied the diagnostic criteria for multiple sclerosis (MS). “Fulminant attacks” in the current study represent attack-related clinically or radiologically severe relapses but do not necessarily mean severe disability. Patients with fulminant attacks were clinically and immunogenetically distinct from those free of such attacks, and the previously described characteristics of the opticospinal form of MS (OSMS) or neuromyelitis optica (NMO) were mostly shared by patients with fulminant attacks. HLA profiles were similar among patients with fulminant attacks irrespective of the lesion distributions. The GG homozygous and G alleles of the CTLA4 gene A/G coding SNP at position 49 in exon 1 were significantly more common in patients with fulminant attacks than in those without. Attack-related severity may be an important factor if validated by prospective studies defining criteria and establishing relationships to disease course and treatment regimens.

Treatment of ulcerative colitis.

In recent years new standards for the treatment of ulcerative colitis have evolved. This review updates evidence based therapy for the various clinical situations as well as some novel approaches. The literature search was based on Medline, Cochrane database (CD-ROM) and handsearch of relevant papers including quoted literature. There is clear-cut evidence-based support for the use of local 5-aminosalicylates in mild/moderate distal and oral 5-aminosalicylates in extensive ulcerative colitis. The administration of corticosteroids is definitely indicated in severe disease. Fulminant attacks are treated by intravenous cyclosporine or colectomy. In chronic active disease azathioprine is probably helpful. Relapse prevention again is a domain of 5-aminosalicylates or, as a novel development, E. coli Nissle. The various meta-analyses as well as the controlled trials performed in the various clinical situations typical for the manifestations of ulcerative colitis form a solid base of evidence to guide individual treatment decisions.

Abnormal root development, probably due to erythema multiforme (Stevens-Johnson syndrome)

A case report is presented of a patient in whom nearly all teeth of the permanent dentition still present had short roots, while some teeth had never been formed at all. It may be concluded from the typical differences in length between the roots of the various teeth that this condition must be due to complete cessation of the growth of the teeth at the age of 7 or 8 years. Since the patient suffered a fulminant attack of erythema multiforme (S tevens-J ohnson syndrome) at this age, and since no other possible explanation of the short roots has been found, it is concluded that this clinical condition may have been the reason for the short root anomaly found. Damage or even destruction of the epithelial root sheath during this disease may be assumed to be the direct cause of the failure of full root development.

Permanent Anonychia After Stevens-Johnson Syndrome

Loss of nails with scarring that leads to anonychia may occur on a congenital basis, as in the nail-patella syndrome and the dystrophic epidermolysis bullosa, or on an acquired basis, as seen after severe trauma, as well as in the case of impaired peripheral circulation, in lichen planus, and in bullous drug eruptions.1Any drugs that induce bullae can cause nail changes or nail loss due to destruction of the nail matrix.1 To our knowledge, hitherto nothing has been written about the nail changes in Stevens-Johnson syndrome. Therefore, we will present such a case report of a patient with permanent nail loss. Report of a Case A 29-year-old man was admitted to the dermatology department April through July 1971 with a fulminant attack of Stevens-Johnson syndrome. Physical examination results showed suppurative conjunctivitis on both eyes and edema and redness of the eyelids. On the lips and around the

X-RAY FINDINGS IN THE PEDIATRIC SURGICAL EMERGENCIES

Clinical course of Hirschsprung's diasease (aganglionosis) is fairly variable depending upon its length of the aganglionic segment and accompanying enterocolitis. Cremin and co-workers subdivided it into five groups.Case histories and x-ray findings of two cases of the long segment aganglionosis were presented. The first case showed rather typical x-ray findings and its diagnosis was readily established. A transverse colostomy was made successfully, but he died of a fulminant attack of enterocolitis about a year later. The second case had normal calibered aganglionic segment without showing irregular bizarre contraction. The site of caliber change was also not apparent, but there was a caliber difference between the ascending colon and the descending colon. The diagnosis was made as a stenosis in the splenic flexure and a side-to-side anastomosis between the dilated transverse colon and the collapsed sigmoid colon was carried out. His postoperative course was complicated with continuous vomiting, increasing abdominal distention, wound infection and entero-cutaneous fistulas. He succumed on 15th postoperative day. Autopsy revealed the long segment aganglionosis extending to the left half of the transverse colon.

Fulminating ulcerative colitis with colonic wall necrosis.

Chronic ulcerative colitis is a disease of protean manifestations involving many anatomical systems but with the distinguishing features of colonic inflammatory mucosal ulceration, fibrosis of the bowel wall, and relative uninvolvement of the colonic serosal surfaces. Of the many complications of ulcerative colitis, there are a group of catastrophic conditions occurring within the colon, namely, perforation, massive bleeding, and carcinoma. 1 To these obstruction might well be added, although it overlaps with the complications named, for the obstruction may be neoplastic, cicatricial, or it may be adynamic. This latter occurs in fulminating attacks, dangerous especially because dilation tends to accentuate colonic wall necrosis and thus leads to perforation and peritonitis. This report is directed to a review of actual colonic wall perforation or threatened colonic wall necrosis in 13 ulcerative colitis patients at the State University of Iowa Hospitals, 11 in fulminant attacks and 2 with acute focal perforations. Patients