Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins.
For patients with relapsingremitting multiple sclerosis (RRMS), there are currently six approved medications that have been shown to alter the natural course of the disease. The approved medications include three beta interferon formulations, glatiramer acetate, natalizumab and mitoxantrone. Treating aggressive forms of RRMS and progressive disease forms of MS still presents a great challenge to neurologists. Intense immunosuppression has long been thought to be the only feasible therapeutic option. In patients with progressive forms of MS, lymphoid tissues have been detected in the central nervous system (CNS) that may play a critical role in perpetuating local inflammation. Agents that are currently approved for patients with MS have no or very limited bioavailability in the brain and spinal cord. In contrast, cyclophosphamide (CYC), an alkylating agent, penetrates the bloodbrain barrier and CNS parenchyma well. However, while CYC has been used in clinical trials and off-label in clinical practice in patients with MS for over three decades, data on its efficacy in very heterogeneous groups of study patients have been conflicting. New myeloablative treatment paradigms with CYC may provide a therapeutic option in patients that do not respond to other agents. In this article we review the scientific rationale that led to the initial clinical trials with CYC. We will also outline the safety, tolerability and efficacy of CYC and provide neurologists with guidelines for its use in patients with MS and other inflammatory disorders of the CNS, including neuromyelitis optica (NMO). Finally, an outlook into relatively novel treatment approaches is provided.
Multiple sclerosis (MS) is an acquired inflammatory demyelinating disease of the CNS that is typically diagnosed in the second or third decade of life. It is generally believed that over the last few decades the life expectancy of patients with adult onset MS (AOMS) has approached that of the general population as a result of better medical and nursing care. Thus, an increasing number of MS patients are entering or have reached senescence. A second group of elderly patients with MS that may be very different in terms of disease pathogenesis are patients with late onset MS (LOMS). The diagnosis in LOMS patients can be challenging because of a large number of age-associated MS differential diagnoses, atypical presentations, a low index of suspicion and the lack of diagnostic criteria specific to this age group. Also, specific problems these patients encounter have only recently become a focus of attention. Changes in renal and hepatic function with age, in addition to the coexistence of medical co-morbidities, require special attention in the management of elderly patients with MS. In this review we outline the characteristics of senescent AOMS and LOMS patients. In addition, we discuss therapeutic strategies in elderly patients with MS based on our knowledge of immunosenescence and age-associated characteristics of this disorder. Given the overall aging of the population, focusing on these two patient groups appears highly relevant.
Sjögren syndrome (SS) has been known to manifest with neurological complications, most frequently of the peripheral nervous system, and often in advance of xerostomia and xerophthalmia. There has been one case report of a patient with SS presenting with acute motor neuropathy similar to Guillain-Barré syndrome (GBS). We report the case of a patient who developed rapidly fulminant acute motor axonal neuropathy (AMAN) with positive anti-GM1 antibody at high titers in association with serological and pathological evidence of SS without xerostomia or xerophthalmia.
Chronic cerebrospinal venous insufficiency (CCSVI) was recently proposed as a contributing factor in the pathology of multiple sclerosis. This concept has gained remarkable attention, partly because endovascular neurointervention has been suggested as a treatment strategy. This review summarizes available evidence and provides a critical analysis of the published data. Currently, there is inconclusive evidence to support CCSVI as an etiological factor in patients with multiple sclerosis. Endovascular procedures should not be undertaken outside of controlled clinical trials. Keywords: chronic cerebrospinal venous insufficiency, multiple sclerosisThe origin of the hypothesis Chronic cerebrospinal venous insufficiency (CCSVI) as a potential etiopathogenic entity in multiple sclerosis (MS) has recently been suggested and gained significant attention.Over the past few years, Zamboni and colleagues were the first to propose and explore this novel and controversial idea [Zamboni et al. 2009b,c,d]. The theory of CCSVI suggests that MS may be causally related to an inflammatory or immune reaction to iron that accumulates in the central nervous system (CNS) secondary to insufficiency of cervical and cerebral venous blood vessels [Zamboni, 2006]. The same investigators detected venous insufficiency by ultrasound and transcranial Doppler. These patterns of venous insufficiency in cranial and cervical blood vessels were seen in patients with MS and absent in control cohorts.In 2010, Zivadinov and colleagues studied 16 patients with relapsing remitting MS (RRMS) and eight matched, healthy controls by 3 T scanner using susceptibility-weighted imaging sequence [Zivadinov et al. 2010]. According to the authors, all 16 patients with MS fulfilled the diagnosis of CCSVI compared with none of the healthy controls. In patients with MS higher iron concentrations were found in the thalamus, globus pallidus, and hippocampus. Iron concentration measures were related to longer disease duration and increased disability, and increased MRI lesion burden and brain atrophy. Similar results were reported by Haacke and colleagues in the same year . Subsequent studiesAs mentioned above, the proposal of CCSVI as an etiological factor or disease modifier in MS is controversial. Subsequent to the initial observations, other investigators conducted their own studies to determine the existence and prevalence of CCSVI in patients with MS and control cohorts.One positive study by Al-Omari and Rousan showed that in 25 patients with MS there was evidence of CCSVI in 84%, whereas none of the 25 controls displayed evidence of cerebral or cervical venous abnormalities [Al-Omari and Rousan, 2010]. There was no blinding of the investigators in this study.All other published studies, however, could not reproduce the findings published by Zamboni and coworkers [Doepp et al. 2010;Krogias et al. 2010;Sundstrom et al. 2010;Wattjes et al. 2010;Yamout et al. 2010]. Doepp and colleagues found no evidence of CCSVI in patients with MS using Doppler criteria [...
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