1/49. 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 = nervous system (Clic here for more details about this article) |
2/49. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 2.5227249919163keywords = brain (Clic here for more details about this article) |
3/49. Optic disc edema in neonatal onset multisystem inflammatory disease (NOMID).PURPOSE: To inform ophthalmologists about neonatal onset multisystem inflammatory disease (NOMID), a rare condition with ophthalmologic manifestations. methods: We report a single case of NOMID with optic disc edema. RESULTS: A 28-month-old child with neonatal rash, arthropathy, central nervous system (CNS) involvement, and optic disc edema was diagnosed with NOMID. CONCLUSIONS: The finding of posterior uveitis or optic disc edema in a child with juvenile onset arthritis may allow the differentiation of NOMID from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 3.9484292967842keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/49. Intracranial inflammatory tumors: a survey of their various etiologies by presentation of 5 cases.Due to similar clinical and neuroradiological features, intracranial inflammatory tumors (IITs) are frequently misdiagnosed as brain neoplasms, from which they notably differ in respect to therapy and prognosis. In this article, five cases of such tumors are presented. Three of the patients with brain tumors (cases 3, 4 and 5) presented a history of 'pararheumatic' syndromes but no diagnosis of defined immunopathies. On the basis of radiological findings, all processes were classified as genuine brain neoplasms, but histology showed reactive inflammatory features. The possible etiologies of these 'tumors' are discussed on the basis of all clinical and histological data of the patients. The spectrum of diseases potentially leading to the manifestation of an IIT is reviewed. Additionally, the presentation of case 5, who developed a highly malignant B-cell-lymphoma 6 months after the removal of an IIT without any histological signs of atypia, shows that this differential diagnosis always has to be kept in mind.- - - - - - - - - - ranking = 7.568174975749keywords = brain (Clic here for more details about this article) |
5/49. Lymphocytic hypophysitis in a patient with systemic lupus erythematosus.A case of lymphocytic hypophysitis in a patient with systemic lupus erythematosus is described. A 20-year-old woman was admitted to our hospital with generalized myalgia and facial rash in May 1998. The patient had a medical history, physical examination, and laboratory findings compatible with systemic lupus erythematosus (SLE). headache and nausea had developed 3 months previously and worsened over the following months. Hormonal investigation showed hypopituitarism except for prolactin. A magnetic resonance image of the brain showed a mass lesion in the pituitary fossa. A trans-sphenoidal surgical procedure was performed which revealed a dark-yellowish hematoma. Microscopic examination showed diffuse infiltration of lymphocytes and plasma cells with fibrosis in the anterior pituitary. Post-operatively the patient's headaches and nausea resolved. This indicates that lymphocytic hypophysitis may be associated with SLE.- - - - - - - - - - ranking = 2.5227249919163keywords = brain (Clic here for more details about this article) |
6/49. Lymphocytic adenohypophysitis mimicking a pituitary macroadenoma.A 24-year-old woman developed headache and rapidly progressive visual disturbances during the last trimester of her first pregnancy. Magnetic resonance tomography (MRI) of the brain documented an intra- and suprasellar mass lesion. For preservation of vision, transsphenoidal microsurgical decompression was performed. Immediately postoperatively, visual acuity improved and hemianopia resolved. Histological examination yielded the diagnosis of primary lymphocytic adenohypophysitis. This is a rare inflammatory pituitary disease. There are no typical clinical, laboratory, or radiological findings that allow precise preoperative diagnosis. Even though this autoimmune disorder is principally steroid-responsive, an improvement of visual disturbances under steroid therapy cannot be predicted. Therefore, surgery is justified not only to establish the diagnosis, but also to restore vision by decompression of the optic nerves and the chiasm.- - - - - - - - - - ranking = 2.5227249919163keywords = brain (Clic here for more details about this article) |
7/49. Human herpesvirus 6-meningoencephalitis in an hiv patient with progressive multifocal leukoencephalopathy.Human herpesvirus 6 (HHV6) has been reported as a rare cause of meningoencephalitis and leukoencephalitis. We present an hiv-infected patient with lesions of progressive multifocal leukoencephalopathy (PML), but also meningoencephalitis apparently due to HHV6. immunohistochemistry for HHV6 antigens and in situ polymerase chain reaction for HHV6 genome showed many positive lymphocytes and microglia in the meningeal and cortical lesions. More importantly, dead and dying neurons were conspicuous; some were undergoing neuronophagia and some displayed evidence of HHV6 infection. A pathogenic role for this almost universal, and usually commensal, virus in inflammatory brain lesions and PML is briefly discussed.- - - - - - - - - - ranking = 2.5227249919163keywords = brain (Clic here for more details about this article) |
8/49. Fetal varicella syndrome: disruption of neural development and persistent inflammation of non-neural tissues.Primary varicella zoster virus (VZV) infection during pregnancy is rare. If it occurs between the 8th and 20th week of gestation, fetal varicella syndrome results in 1-2% of the fetuses. We report about a varicella infection that affected a pregnant mother in the 12th week of gestation. At 33 weeks, a premature girl was born with destruction of neurons in spinal cord, spinal ganglia and plexus myentericus, and secondary developmental disturbance including mummification of one arm and segmental intestinal atresia. The brain did not show any abnormalities. However, VZV dna could be detected by PCR in tissues from the brain and spinal ganglia. Chronic necrotizing inflammation was found in the placenta, fetal membranes, and one ovary. These locations showed nuclear inclusions which by in-situ-hybridization were proven to be VZV derived. This case demonstrates that in the fetal age, 'neurotropism' of VZV signifies severe destruction but not necessarily persistent inflammation of neural tissue. However, due to the inefficient fetal immune system, inflammation can go on for weeks, preferentially in non-neural tissues.- - - - - - - - - - ranking = 5.0454499838327keywords = brain (Clic here for more details about this article) |
9/49. The CINCA syndrome: a rare cause of chronic arthritis and multisystem inflammatory disorders.Chronic infantile neurological cutaneous articular (CINCA) syndrome is a rare disorder with neonatal onset characterised by a chronic progressive inflammatory process with skin rash, articular and central nervous system involvement. This primary systemic inflammatory disorder should be distinguished from juvenile rheumatoid arthritis (JRA). Although the articular findings are characteristic features of CINCA syndrome, there is a certain degree of variability in the articular involvements which are not always symmetrical nor is the degree of severity uniform. The etiology of CINCA syndrome remains unknown. No single treatment has been found to be effective. This syndrome is known in the American medical literature as infantile onset multisystem inflammatory disease (IOMID).- - - - - - - - - - ranking = 3.9484292967842keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/49. Tumefactive fibroinflammatory lesion of the neck with progressive invasion of the meninges, skull base, orbit, and brain.SUMMARY: Tumefactive fibroinflammatory lesions of the head and neck are rare. CNS involvement has not been reported. We present a histologically proven case of a tumefactive fibroinflammatory lesion that originated in the left side of the neck and progressed over 2 years to involve the meninges, the cavernous sinuses, the right temporal lobe, and the right orbit. The lesion caused destruction of the skull base and a subdural hematoma. The relationship of the present lesion to idiopathic hypertrophic pachymeningitis and tolosa-hunt syndrome is discussed.- - - - - - - - - - ranking = 10.090899967665keywords = brain (Clic here for more details about this article) |
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