1/14. Human herpes virus type 7 dna in the cerebrospinal fluid of children with central nervous system diseases.Human herpes virus type 7 (HHV-7) has been associated with unspecific febrile syndrome, exanthem subitum (ES), viral rashes and Epstein-Barr virus (EBV) like syndrome. Neurological complications such as hemiplegia or seizures have been described in a few children with ES. Whether HHV-7 may also affect the CNS in the absence of ES is unknown. In this study, we investigated CSF samples from children with different neurological diseases for the presence of HHV-7 specific dna. A HHV-7 specific nested polymerase chain reaction (PCR) was established amplifying a 478 bp dna sequence of the glycoprotein U23 of HHV-7 strain SB. 68 children with CNS diseases with inflammatory CSF findings (n = 24), CNS diseases without inflammatory CSF findings (n = 18) and febrile seizures (n = 26) were examined. A total of 26 children with infectious diseases in the absence of neurological disease and 11 children without signs of a peripheral infection and without neurological disease served as controls. The CSF samples of six children from the study groups were HHV-7 PCR positive, but none from the controls. These children were diagnosed with aseptic meningitis (n = 1), viral encephalitis/meningoencephalitis (n = 2), facial palsy (n = 1), vestibular neuritis (n = 1) and febrile seizure (n = 1). CONCLUSION: These results indicate that human herpes virus type 7 infection is associated with central nervous system disease in children and should be considered in children whether inflammation in the cerebrospinal fluid is present or not.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
2/14. An isolated fourth ventricle in neurosarcoidosis: MRI findings.We report on an isolated enlargement of the fourth ventricle in a patient with neurosarcoidosis which developed 3 years after the insertion of a ventriculo-atrial shunt. Repeated MRI images were obtained in a patient with known neurosarcoidosis between 1995 and 2000. Imaging findings were correlated to the medical course of the patient, who developed a hydrocephalus and a trapped fourth ventricle consecutively. The isolation was presumably due to granulomatous inflammation of the ependyma surrounding the fourth ventricular outlets. The isolated fourth ventricle was responsible for a deterioration of neurological status. Neurosarcoidosis is a severe complication in sarcoidosis patients. An isolated enlargement of the fourth ventricle is a rare complication in clinically deteriorated patients with neurosarcoidosis and ventricular drainage, which may require neurosurgical treatment.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
3/14. Eales' disease with neurological involvement. Part 2. pathology and pathogenesis.Detailed neuropathologic examination was carried out on 1 case of Eales' disease with CNS involvement, in the form of retinal vasculopathy, followed first by signs of brain stem and cerebellar disease and then by a myelopathy, with death 4 years later from retinal infection. There was mild chronic inflammation in the retina, and sub-total demyelination of one optic nerve. The brain stem and cerebellum showed extensive vasculopathy, with various stages of venous change extending from proliferation and dilatation to haemorrhage, or to thickening with hyalinisation. The perivenular brain tissue, particularly of the cerebellum, often showed demyelination, with relative axon preservation, but no inflammation. Similar, but less pronounced venopathy was seen in the dorsal cord. There was ascending degeneration of Goll's columns and descending degeneration of the lateral columns.- - - - - - - - - - ranking = 2keywords = inflammation (Clic here for more details about this article) |
4/14. Primary angiitis of the central nervous system presenting with subacute and fatal course of disease: a case report.BACKGROUND: Primary angiitis of the central nervous system is an idiopathic disorder characterized by vasculitis within the dural confines. The clinical presentation shows a wide variation and the course and the duration of disease are heterogeneous. This rare but treatable disease provides a diagnostic challenge owing to the lack of pathognomonic tests and the necessity of a histological confirmation. CASE PRESENTATION: A 28-year-old patient presenting with headache and fluctuating signs of encephalopathy was treated on the assumption of viral meningoencephalitis. The course of the disease led to his death 10 days after hospital admission. Postmortem examination revealed primary angiitis of the central nervous system. CONCLUSION: Primary angiitis of the central nervous system should always be taken into consideration when suspected infectious inflammation of the central nervous system does not respond to treatment adequately. In order to confirm the diagnosis with the consequence of a modified therapy angiography and combined leptomeningeal and brain biopsy should be considered immediately.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
5/14. Anakinra therapy for CINCA syndrome with a novel mutation in exon 4 of the CIAS1 gene.We report on a patient with chronic infantile neurological cutaneous and articular (CINCA) syndrome. sequence analysis revealed a novel missense mutation in exon 4 of the CIAS1 gene. The patient was unresponsive to several treatments including prednisolone, immunosuppressants (azathioprine and cyclosporin), disease-modifying antirheumatic drugs (DMARDs: penicillamine, salazopyrin and methotrexate) and the tumour necrosis factor-alpha (TNF-a)-blocker infliximab. At 32 mo of age, administration of the recombinant human interleukin-1 receptor antagonist anakinra commenced, which caused an immediate and marked improvement in the clinical symptoms and laboratory test results. Continuous inhibition of the inflammation required a dose of 1.0 mg/kg every 12 h. CONCLUSION: Following the diagnosis of CINCA syndrome, anakinra treatment should be commenced as the first line of therapy.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
6/14. diagnosis and management of isolated angiitis of the central nervous system.Isolated angiitis of the central nervous system (IAC) is usually a fatal inflammatory disease with a predilection for small blood vessels. Recurrent cerebral infarction leading to death within a few years is the usual course, but this may be significantly altered by aggressive immunosuppressive therapy with prednisone and cyclophosphamide. Other diseases may, however, present with similar clinical and angiographic features. Because antemortem diagnosis suggests a therapy, establishing the criteria for diagnosis is important. This report describes clinical, angiographic, and biopsy features, and therapy of five successfully treated patients with IAC. The following specific criteria are recommended for establishing an antemortem diagnosis of IAC: (1) clinical pattern of headaches and multifocal neurologic deficits present for at least 6 months, unless the deficits are severe at onset or rapidly progressive; (2) cerebral angiography demonstrating segmental arterial narrowing; (3) exclusion of systemic inflammation or infection; and (4) leptomeningeal/parenchymal biopsy demonstrating vascular inflammation or exclusion of alternate diagnoses. Based upon the successful management of these five previously unreported patients, as well as others in the literature, the following treatment regimens are recommended for the initial 6 weeks of therapy: (1) prednisone 40 to 60 mg/day, and (2) cyclophosphamide 100 mg/day.- - - - - - - - - - ranking = 2keywords = inflammation (Clic here for more details about this article) |
7/14. Simulataneous occlusion of the central retinal artery and vein.Combined CRAO/CRVO in our patients occurred with rapid visual loss, usually over a few hours, associated with evidence of inflammation and/or cellular infiltration of the retrobulbar portion of the optic nerve. The ophthalmoscopic appearance was characteristic, with papilledema and hemorrhages of various types in the posterior pole. The retina also showed ischemic changes, with a milky-white color and cherry-red macula. fluorescein angiography, when possible, showed no retinal vascular flow, and normal choroidal flow. After six to eight weeks, optic atrophy was evident and the retinal vessels were markedly narrowed or obliterated. The macula showed typical cystic changes. Neovascularization often developed, leading to neovascular glaucoma as the end result.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
8/14. The neuropathology of acquired immune deficiency syndrome.We reviewed the neuropathologic characteristics of 52 cases of acquired immune deficiency syndrome (AIDS) at autopsy. Histologically significant neuropathologic lesions were found in 38 cases. We believed that infection was the predominant pathologic process in 26 cases; occasionally, multiple infectious agents were present. This included toxoplasma encephalitis (n = 16), fungal abscess (n = 1), tuberculous abscess (n = 1), progressive multifocal leukoencephalopathy (n = 2), cysticercosis (n = 1), and escherichia coli meningoencephalitis (n = 1). Microglial nodules or perivascular inflammation suggested that encephalitis was the most likely cause in five cases. In two additional cases, a primary demyelinating process that was apparently related to cytomegalovirus was present. Vascular or hypoxic diseases were present in nine cases. The findings included intracerebral hemorrhage (n = 1), subarachnoid hemorrhage (n = 2), infarction (n = 2), diffuse hypoxic changes (n = 5), cerebral edema (n = 1), and rare thromboemboli with extravasation of RBCs (n = 1). One case of primary lymphoma was observed. The CNS lesions were the proximate cause of death in 15 patients. The CNS complications of AIDS are varied and often are the major manifestation of the syndrome.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
9/14. eosinophil cationic protein (ECP) in the cerebrospinal fluid.ECP (eosinophil cationic protein) has been measured by means of a specific radioimmunoassay in the cerebrospinal fluid (CSF) from 210 individuals with various diseases affecting the central nervous system. In the same specimens lactoferrin and albumin were measured as well, as indicators of neutrophil-involved inflammation and damage to the blood-brain barrier. From a patient reference group (n = 39) the upper "normal" limit for ECP was estimated to 1.7 microgram/l. In patients with acute cerebrovascular disease (n = 108) ECP levels were elevated in 38% of the cases which was a significantly (P less than 0.001) greater proportion than seen for lactoferrin (7%). In patients with acute infections of the CNS (n = 30) 67% had raised ECP levels with significantly higher levels (P less than 0.001) in those having bacterial infections. The ECP levels were significantly correlated (P less than 0.001) to the lactoferrin-levels in the whole infectious group. In patients with tumours (n = 25) raised levels of ECP were found in 67% of those with malignant and in 6% of those having benign tumours. This difference was statistically significant (P = 0.001). The ECP levels were closely related to those of lactoferrin (P less than 0.001) and albumin (P less than 0.005). Of the patients with multiple sclerosis (n = 19) 25% had raised ECP levels. This proportion was not significantly different from those having raised lactoferrin levels. In three patients extremely high ECP levels (70-455 micrograms/l) were found and a causal relationship between ECP and the brain tissue damage in these patients is suggested. In comparison with the neutrophil-related data the findings suggest a preferential involvement of eosinophils in some diseases affecting the central nervous system.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
10/14. Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI.intracranial hypotension is a cause of diffuse enhancement of the pachymeninx with gadolinium, which often is associated with subdural fluid collections. We reviewed the results of meningeal biopsy in six patients with intracranial hypotension and diffuse pachymeningeal enhancement to correlate the MRI findings with histopathologic observations and to explain the abnormalities seen on MRI. Grossly, the dura mater was unremarkable in all patients, as were the leptomeninges, except for one patient with prolonged (18 months) intracranial hypotension in whom the arachnoid was thickened and opaque. Microscopically, the dura mater was entirely normal on its epidural aspect; however, a fairly thin zone of fibroblasts and thin-walled small blood vessels in an amorphous matrix was noted on the subdural aspect. In the patient with longstanding symptoms, diffuse benign arachnoidal cell proliferation was also noted, probably a reaction triggered by longstanding changes in the subdural area, as noted in the five other patients. There was no evidence of inflammation, infection, or metastatic neoplasia. These findings suggest that in intracranial hypotension, the dural-meningeal abnormalities probably represent reactive secondary phenomena, likely related to hydrostatic changes in the CSF, and not a primary meningeal process.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
| | Next -> |