1/29. The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports.A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral dna from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/29. Stealth virus epidemic in the Mohave Valley: severe vacuolating encephalopathy in a child presenting with a behavioral disorder.An infectious illness, attributed to atypically structured cytopathic "stealth" viruses, occurred in 1996 in the Mohave Valley region of the united states. A stealth virus-infected child from this region has developed a severe noninflammatory, vacuolating (spongiform) en cephalopathy. The illness initially presented as a behavioral problem without overt neurological signs. Extensive investigations, including repeated magnetic resonance imaging, two brain biopsies, and stealth virus cultures, have helped define the disease process occurring in this child. Significant clinical benefit with apparent retardation of disease progression occurred during a 6-week course of ganciclovir therapy. The potential contributing role of stealth virus infections in children presenting with behavioral problems needs to be addressed.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/29. HLA-mismatched CD34-selected stem cell transplant complicated by HHV-6 reactivation in the central nervous system.We report here a patient who suffered from PCR- confirmed human herpesvirus type 6 (HHV-6) meningoencephalitis after allogeneic purified CD34 cell transplantation from his HLA-mismatched sibling donor, even though he had been on intense prophylaxis with i.v. ganciclovir (GCV), acyclovir (ACV) and gamma-globulin containing a specific antibody against HHV-6. Serological evaluation disclosed that both the donor and recipient had IgG antibody against HHV-6 before transplantation. His blood WBC count started to transiently increase on day 10, and all blood components had decreased by day 20. He then developed a severe headache and high blood pressure, and sporadic abnormal neurological findings including nystagmus and delirium. An analysis of cerebrospinal fluid (CSF) revealed 8 cells/microl, a glucose level of 130 mg/dl and a protein level of 201 mg/dl (normal, 50 mg/dl) on day 26. At the time, HHV-6 was detected only in CSF by a PCR-based method and he was diagnosed as having meningoencephalitis due to the local reactivation of HHV-6. Although he failed to respond to high-dose therapy with ACV (60 mg/kg/day) and gamma-globulin, the dna of this virus disappeared from the CNS upon treatment with GCV (30 mg/kg/day) combined with the intraventricular infusion of alpha-interferon. His clinical course was further complicated with meningoencephalitis due to staphylococcus epidermidis, and he died of tentorial herniation on day 79 without the recovery of blood components. This experience may indicate that intense prophylaxis to prevent reactivation of HHV-6 in the CNS is essential for the management of such profoundly immunosuppressed patients.- - - - - - - - - - ranking = 33.578620117303keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/29. Postinfectious gastroparesis related to autonomic failure: a case report.BACKGROUND AND AIM: Severe dysautonomia may be secondary to viral infections, resulting in impaired autoimmune, cardiovascular, urinary and digestive dysfunction. Herein, we present a case of a 31-year-old white female patient who had severe gastroparesis related to autonomic failure following an episode of acute gastroenteritis. This seems to be the first report providing thorough assessment of the enteric and autonomic nervous system by analysis of full-thickness small intestinal biopsies, cardiovagal testing and autopsy. HOSPITAL COURSE: This patient affected by a severe gastroparesis was treated with antiemetics, prokinetics, analgesics and gastric electrical stimulation to control symptoms. nutritional support was made using jejunal feeding tube and, in the final stage of disease, with total parenteral nutrition. Autonomic studies revealed minimal heart rate variability and a disordered Valsalva manoeuvre although the enteric nervous system and the smooth muscle layer showed a normal appearance. Hospital courses were complicated by episodes of bacteraemia and fungemia. serum antiphospholipid antibodies were noted but despite anticoagulation, she developed a pulmonary embolism and shortly thereafter the patient died. autopsy revealed acute haemorrhagic candida pneumonia with left main pulmonary artery thrombus. Sympathetic chain analysis revealed decreased myelinated axons with vacuolar degeneration and patchy inflammation consistent with guillain-barre syndrome. The evaluation of the enteric nervous system in the stomach and small bowel revealed no evidence of enteric neuropathy or myopathy. CONCLUSION: A Guillain-Barre-like disease with gastroparesis following acute gastroenteritis is supported by physiological and autonomic studies with histological findings.- - - - - - - - - - ranking = 7.0683761927757keywords = nervous system (Clic here for more details about this article) |
5/29. An informative case of Graves' disease with implications for schizophrenia.The aetiology of schizophrenia and the other psychoses is not yet established. The Knight model, based on genetic and other evidence, proposes that schizophrenia is an autoimmune disease, caused by the development of forbidden clones of B lymphocytes that secrete autoantibodies that accidentally stimulate cell surface receptors on certain neurons, affecting the limbic system of the brain. An unusual defect in a Maori man with Graves' disease rendered him unresponsive to the usually effective antithyroid drugs, prompting his being treated with prednisone, a non-specific immunosuppressant agent. This was highly successful, reducing the blood level of the causative thyroid-stimulating autoantibodies with reduction of thyroid hormone levels and thyroid gland size. Unfortunately, high dosage prednisone can be used for only a month, because of steroid toxicity. A research pathway to effective therapy of receptor-mediated autoimmune diseases, which probably include the psychoses, is now apparent. It involves finding the autoantibodies, then cloning of their antigenic targets, as has been done for Graves' disease. This will provide knowledge of the peptide sequences necessary for constructing therapeutic agents for selectively destroying the pathogenic forbidden clones. Meanwhile, usage of short-term therapy with prednisone could be helpful in the management of schizophrenia and should be explored.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/29. Transient acute obstructive hydrocephalus of unknown origin in a 13-month-old infant.We report on a case of a 13-month-old female infant who was admitted to our department with the chief complaints of drowsiness and vomiting. A history of an unspecified viral infection was reported. Clinical examination was negative for focal neurological signs or signs of central nervous system infection. Initial CT scan revealed obstructive hydrocephalus, and shunting was scheduled. dexamethasone treatment was started. Eight hours after admission the child almost restored his baseline mental status and the operation was postponed. The dexamethasone treatment was discontinued 3 days later. Follow up CT and MRI scans were normal. We discuss the case and the possible causes of transient hydrocephalus in children.- - - - - - - - - - ranking = 8.3946550293258keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/29. 'Locked-in syndrome' for 27 years following a viral illness: clinical and pathologic findings.We describe a man who, after a presumed encephalitic illness, was "locked-in" for 27 years. His CT and autopsy findings showed atrophy of the brainstem and a cystic lesion at the base of the pons. He survived longer than most other patients in a similar state.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/29. central nervous system involvement in patients with rotavirus gastroenteritis.Two children with rotavirus gastroenteritis are presented. The first case developed a fatal Reye's syndrome and the other one encephalitis with slow recovery. The rotavirus diagnosis was made in both cases by electron microscopy and a significant rise in antibody titres to nebraska calf diarrhea virus was seen in one of the two patients.- - - - - - - - - - ranking = 9.4245015903677keywords = nervous system (Clic here for more details about this article) |
9/29. 'Locked-in coma' in postinfective polyneuropathy.A patient with postinfective cranial and peripheral polyneuropathy exhibited the electroencephalographic and behavioral features of "alpha coma". The relation of this form of extensive peripheral disconnection to those cases with central disconnection due to pontomesencephalic lesions is discussed. We conclude that in both situations further evaluation of brain stem and cortical function is necessary to determine whether or not consciousness is preserved, rather than relying solely on the presence of ocular movements and reactivity of the electroencephalogram.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/29. viruses, neurodevelopmental disorder and childhood psychosis.Four cases are described of prepubertal boys in whom the convergence of neurodevelopmental disorder, viral infection and psychosis seemed more than coincidental. review of the literature highlights the possibility that viral infection of the central nervous system may play a contributory role in childhood psychosis. Whilst it is essential to avoid a reductionist stance when investigating these difficult conditions, the emergence of potent anti-viral treatments and sophisticated methods of identifying the presence of viral infection should encourage us to consider more carefully the relevance of viruses in childhood psychosis.- - - - - - - - - - ranking = 8.3946550293258keywords = central nervous system, nervous system (Clic here for more details about this article) |
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