1/29. Chronic inflammatory demyelinating polyradiculoneuropathy with histologically proven optic neuritis.Although patient series of clinical, electrophysiological, or magnetic resonance imaging evidence for involvement of the central nervous system in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been published, the histological proof has never been reported. We present the case of a 46-year-old male patient who developed CIDP in his early 20s and who died of relapsing severe pneumonia. In late stages of the disease the patient presented visual loss and bilateral atrophy of the optic nerve. Neuropathological examination revealed severe peripheral neuropathy consistent with CIDP and central involvement with bilateral optic neuritis. This is the first case reporting CIDP and histologically proven optic neuritis.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/29. Post-infectious central and peripheral nervous system diseases complicating mycoplasma pneumoniae infection. Report of three cases and review of the literature.Three patients with a central and peripheral nervous system disease complicating a mycoplasma pneumoniae (M. pn.) infection are presented. Patient 1 suffered from bilateral optic neuritis as well as acute guillain-barre syndrome recovering after plasmapheresis. The two other patients suffered from severe haemorrhagic leukoencephalitis (Hurst) which only could be contained by aggressive decompressive craniectomy with duraplasty. All three illnesses were clearly shown to be associated with M. pn. infection.Our three patients represent the full scale of central nervous (CNS) (cerebral and myelitic) as well as peripheral nervous system (PNS) (GBS, optic neuritis) manifestation of a disease caused by the same pathogenetic - post-infectious - mechanism; pathogenic CNS and PNS epitopes might be shared in post-infectious neurological disease following M. pn. infection.- - - - - - - - - - ranking = 2.1689097506642keywords = nervous system (Clic here for more details about this article) |
3/29. Temporal arteritis: a spectrum of ophthalmic complications.Seven patients of temporal arteritis with eye involvement have been presented. These cases represent a spectrum of disease from intermittent diplopia with minimal 6th nerve weakness through mild retinal ischemia with recovery to permanent bilateral blindness. Temporal arteritis should be suspected when any form of ocular ischemia is suspected by history or found on examination of an elderly person. An early diagnosis may protect the vision in both eyes if vision is normal at the time of diagnosis. If vision in one eye is decreased because of ischemia, the vision in the other eye can usually be retained if proper therapy is instituted. Furthermore, adequate therapy may even result in improvement in vision in the involved eye. patients with biopsy proven temporal arteritis should be continued on steroid therapy until the active disease is quiescent. Inactivity should be determined by carefully monitoring the ESR while steroids are being tapered. If the ESR rises, it is indicative of continued inflammation and if steroids are not continued, the eyes remain at risk as seen in Case 5. If the ESR remains elevated for a year or more despite continuation of high steroid levels, consideration should be given to repeating the temporal artery biopsy. Temporal arteritis should be considered in the differential diagnosis of any multisystem disease in older patients. Even central nervous system involvement may occur concomitantly, since the intracranial vessels are not immune from the disease process. tuberculosis, systemic syphilis and more recently the collagen vascular diseases have been dubbed the "great imitators" and "the protean diseases." We suggest that the same terminology can be applied to temporal arteritis. Temporal arteritis can affect any organ. Moreover, there is a wide spectrum of variation in the degree of involvement of any particular tissue as illustrated by these 7 cases of ocular involvement.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/29. Possible role of corticosteroids in nervous system plasticity: improvement in amblyopia after optic neuritis in the fellow eye treated with steroids.OBJECTIVE: amblyopia consists of reduced visual function in the absence of organic disease, caused by deficient visual stimulation, most commonly due to squint or refractive error. amblyopia is thought to be reversible up until the age of approximately 8 years (critical period) and is usually treated with occlusion of the fellow eye. There is recent evidence for visual system plasticity extending beyond the critical period, supported by reports of improvement in visual acuity in the amblyopic eye after loss of vision in the contralateral eye. This suggests that the adult visual system exhibits sufficient plasticity to allow such improvement. We describe here improvement in visual acuity in three amblyopic patients after they received high-dose intravenous glucocorticoids for optic neuritis in the contralateral eye. methods: Clinical and neurological evaluation added. RESULTS: In all cases, the improvement was sustained, even after the recovery from the optic neuritis. CONCLUSIONS: Because steroids affect neural plasticity, we hypothesize that they facilitate and enhance visual improvement in amblyopia, a quality that may be tested in future controlled trials.- - - - - - - - - - ranking = 1.4459398337761keywords = nervous system (Clic here for more details about this article) |
5/29. Aseptic meningitis and optic neuritis preceding varicella-zoster progressive outer retinal necrosis in a patient with AIDS.Varicella-Zoster Virus (VZV) is the second most common ocular pathogen in patients with hiv infection. VZV retinitis is estimated to occur in 0.6% of patients with hiv infection and may occur in one of two clinical syndromes. The first is the acute retinal necrosis syndrome, which also may be seen in immunocompetent hosts. The second clinical syndrome occurs in patients with CD4 cell counts typically < 50 x 10(6)/l and is termed progressive outer retinal necrosis. VZV retinitis has been reported to occur simultaneously with other VZV central nervous system manifestations such as encephalitis and myelitis in hiv-infected patients. In addition, VZV retrobulbar optic neuritis heralding VZV retinitis has recently been described in hiv-infected patients who had suffered a recent episode of dermatomal herpes zoster. Herein we report the case of an hiv-infected individual who presented with VZV meningitis and retrobulbar optic neuritis that preceded the onset of progressive outer retinal necrosis. We also review of the literature of seven additional reported cases of retrobulbar optic neuritis preceding the onset of VZV retinitis.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/29. optic neuritis in the Landry-Guillain-Barre-Strohl syndrome.A patient with typical Landry-Guillain-Barre-Strohl syndrome (LG-BS) developed bilateral optic neuritis. Laboratory studies showed hypersensitivity to both central and peripheral nervous tissue myelin. The occurrence of optic neuritis is presumably due to autohypersensitivity to central nervous tissue myelin. The initial lesions of the LG-BS syndrome in the peripheral nerves might have liberated sequestered antigens that cross-reacted with central nervous system myelin.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/29. Combination of high-dose intravenous immunoglobulins and itraconozole in treating chronic mycotic demyelinating optic neuritis.Mycotic demyelinating optic neuritis is a neurological disorder of the visual system caused by mycotoxins released by indoor toxic molds. Although the health effect of indoor toxic mold on the population worldwide is now one of the "emerging diseases", its involvement in chronic demyelinating optic neuritis has not been reported. Most of the neurological and immunologic abnormalities associated with toxic mold mycotoxins are very difficult to treat successfully, especially neural demyelination of the central and peripheral nervous systems. This paper presents the case of a 42-year-old white female, in whom chronic demyelinating optic neuritis with persistent visual defects due to chronic exposure to toxic molds was diagnosed at the age of 34 years. In spite of all the therapeutic services given to her for over 8 years, her illness persisted and was difficult to treat. However, we successfully treated her with a combination of intravenous immune globulin (IVIG) and itraconozole (Sporanox) when all other treatment modalities failed. This is probably the first report where persistent toxic mold-induced neurological and immunologic disorders were successfully treated with a combination of itraconozole and IVIG.- - - - - - - - - - ranking = 0.36148495844404keywords = nervous system (Clic here for more details about this article) |
8/29. Non-MS recurrent demyelinating diseases.The introduction of MRI has shown that the acute, recurrent (R), and multiphasic (M) forms of disseminated encephalomyelitis (DEM) are more common than suspected in adults, and that their MR images are sufficiently characteristic in most instances to make differentiation from multiple sclerosis (MS) possible. In addition, a number of clinical features of DEM are rarely seen in MS: fever, malaise, nausea, vomiting, positional vertigo, convulsions, aphasia, meningism, bilateral optic neuritis, and CSF leukocytosis and elevated protein. CSF oligoclonal bands are usually absent. It is remarkable that confusion between R- and MDEM and MS persists despite the numerous published reports on recurrent DEM dating back 70 years, many illustrating the characteristic MRIs. There are many case reports of DEM erroneously diagnosed as MS, Schilder's, Marburg's, Devic's, and Balo's disease, and, in particular brain tumors. It is probable that acute DEM is occasionally mistaken for a clinically isolated symptom of MS. Possible mechanisms for recurrence include localization at the site of a previous injury to the nervous system, or by the phenomenon of molecular mimicry. The importance of differentiating R- and MDEM from MS is greater today due to the recommendation that immunodulatory treatment be initiated in patients with a clinically isolated syndrome, or when the occurrence of a second clinical episode establishes the diagnosis of MS.- - - - - - - - - - ranking = 0.36148495844404keywords = nervous system (Clic here for more details about this article) |
9/29. CNS MR and CT findings associated with a clinical presentation of herpetic acute retinal necrosis and herpetic retrobulbar optic neuritis: five hiv-infected and one non-infected patients.INTRODUCTION: This report demonstrates the spectrum of central nervous system (CNS) abnormalities observed on MR imaging and CT studies in 6 patients with clinical or pathologic diagnoses of acute retinal necrosis (ARN) and retrobulbar optic neuritis (RBON-H) resulting from herpes zoster Virus and cytomegalovirus. We discuss the etiologic and pathophysiologic implications regarding these findings. methods: Standard MR imaging sequences of the whole brain and selected high-resolution images of the orbits and globes, from 6 patients, were reviewed by three neuroradiologists for consensus interpretation of the findings. Special sequences augmenting disease were obtained in individual cases. Axial CT images were obtained from two patients using 5mm sequential slices. RESULTS: MR imaging findings showed both T2 signal brightening and contrast enhancement in one or both optic nerves, optic tracts and lateral geniculate bodies, as well as the postsynaptic optic radiations and optic cortex. Similar findings were observed in the superior colliculus, lateral midbrain and cerebellum, with multiple potential etiologic possibilities regarding pathways of dissemination. Low T2* signal (indicating magnetic field susceptibility effects) and CT hyperdensity, consistent with prior hemorrhage, were also observed in the optic tracts, optic radiations and lateral geniculate bodies. Post-contrast enhancement was observed in the meninges and Meckle's cave in one hiv negative patient. CONCLUSION: These cases demonstrate CNS imaging findings associated with RBON that are temporally-related to ARN. They support the hypothesis that RBON can either precede or follow ARN and implicate transneuronal, transsynaptic and/or transcerebrospinal fluid viral spread by the herpetic family.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/29. Intravenous immunoglobulin and interferon: successful treatment of optic neuritis in pediatric multiple sclerosis.optic neuritis is a common clinical condition that causes loss of vision. It can be clinically isolated or can occur as one of the manifestations of multiple sclerosis. multiple sclerosis is a severe disabling demyelinating disease of the central nervous system, which is rare among children. The treatment of optic neuritis has been investigated in several trials, the results of which have shown that corticosteroids speed up the recovery of vision without affecting the final visual outcome. Treatment of neurologic disorders with intravenous immunoglobulin is an increasing feature of our practice for an expanding range of indications, including multiple sclerosis. Owing to its anti-inflammatory properties, intravenous immunoglobulin can be beneficial in the treatment of acute relapses and in the prevention of new relapses of multiple sclerosis. To our knowledge, there is only one experience of treatment of optic neuritis with intravenous immunoglobulin in multiple sclerosis, even if therapeutic trials are used in the therapy of multiple sclerosis. We report on a girl with optic neuritis and multiple sclerosis in whom treatment with intravenous immunoglobulin at first alone and subsequently associated with interferon achieved great improvement in visual acuity.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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