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An Interesting Case of Nuchal Rigidity
- Christina Boettcher, Clemens Warnke, Stephan Macht, Hans-Peter Hartung, Bernd C. Kieseier
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 38 / Issue 3 / May 2011
- Published online by Cambridge University Press:
- 02 December 2014, pp. 516-517
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A 34 year-old female without prior relevant medical history presented to our emergency room with a two-day history of neck pain, odynophagia and elevated body temperature up to 38.2° C. Her general practitioner had started the patient on antibiotic treatment with ciprofloxacin and referred her to our emergency department for further diagnostic workup and treatment of suspected meningitis. Initial neurological examination revealed neck rigidity and head pain without further focal neurological signs. Body temperature was 37.7 °C, blood analyses revealed a normal leukocyte count (8.900/μl) and normal C-reactive protein (<0.3mg/dl), blood cultures were sterile. Lumbar puncture was without pathological findings (<1 leukocytes/μl, normal levels for glucose, lactate and protein). Cervical magnetic resonance imaging could rule out osseous injury and cervical myelopathy, however a small prevertebral, retropharyngeal fluid collection was visible (Figure 1a-c). This prompted the diagnosis of acute retropharyngeal calcific tendinitis (RCT). This diagnosis was confirmed by conventional x-ray showing the presence of a characteristic amorphous calcification in the retropharyngeal space anterior to the C1-C2 segments (Figure 1d). Treatment with i.v. methylprednisolone (250mg) was started and pain was reduced shortly after the first infusion. The patient was discharged on tapered oral methylprednisolone and non-steroidal anti-inflammatory drug (NSAID) treatment. Symptoms resolved completely within a week.
Inflammatory Demyelinating Brain Lesions Heralding Primary CNS Lymphoma
- Leila Husseini, Andreas Saleh, Guido Reifenberger, Hans-Peter Hartung, Bernd C. Kieseier
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 39 / Issue 1 / January 2012
- Published online by Cambridge University Press:
- 02 December 2014, pp. 6-10
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Brain biopsy plays a crucial role in the exploration of suspect white matter lesions in the differential diagnosis of primary central nervous system lymphoma (PCNSL) and inflammatory demyelination. We present the case of a previously healthy, immunocompetent woman, aged fifty-nine, who developed a histologically confirmed demyelinating white matter lesion months prior to the manifestation of a PCNSL. Similar cases of “sentinel lesions” preceding a PCNSL have been reported. In a literature review, we compared the diagnostic features that may be useful to differentiate a PCNSL from inflammatory demyelinating disease in older age. We conclude that the occurrence of large, contrast-enhancing cerebral lesions in older patients with a relapsing-remitting disease course and steroid-resistant vision disorders should lead to the consideration of a PCNSL.
7 - Schwann cells as immunomodulatory cells
- Edited by Patricia Armati, University of Sydney
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- Book:
- The Biology of Schwann Cells
- Published online:
- 13 August 2009
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- 15 February 2007, pp 118-125
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Summary
INTRODUCTION
The nervous system has long been considered an immunologically privileged site. This concept was based on the premises that: (1) there is a more or less strict anatomic separation between the systemic immune compartment (blood) and the neural tissue; (2) molecules required for antigen presentation are absent under normal circumstances; (3) there is no lymphatic drainage; and (4) immune surveillance by T cells is lacking. It is now obvious that most of these assumptions are not tenable. The blood–nerve barrier (BNB) does restrict access of immune cells and soluble mediators to a certain degree; however, this restriction is not complete, either anatomically (e.g. the BNB is absent or relatively deficient at the roots, in the ganglia and the motor terminals) or functionally. Activated T lymphocytes can penetrate intact barriers irrespective of their antigen specificity, and, under certain circumstances, release cytokines that upregulate the expression of major histocompatibility complex (MHC) class II molecules, key molecules required for antigen presentation. In the central nervous system (CNS) tissue-resident neuroglial cells are present that actively participate in the regulation of immune responses within the tissue. In recent years, several lines of evidence have pointed to Schwann cells as immunocompetent cells within the peripheral nervous system (PNS), which, in addition to their physiological roles, exhibit a broad spectrum of immune-related functions and might be involved in the local immune response in the PNS. In this chapter we will elaborate on the expanding recognition of Schwann cells as immunocompetent cells that form part of the local immune circuitry within the PNS. Interestingly, present data suggest that the entire spectrum of an immune response can be displayed by Schwann cells; recognition of antigens, presentation of antigens, mounting an immune response, and, finally, terminating an immune response within the inflamed peripheral nerve.
92 - Immune mechanisms in neurological disease
- from PART XII - AUTOIMMUNE DISORDERS
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- By Hans-Peter Hartung, Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany, Klaus V. Toyka, Department of Neurology, Julius-Maximilians-University, Würzburg, Germany, Bernd C. Kieseier, Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany
- Edited by Arthur K. Asbury, University of Pennsylvania School of Medicine, Guy M. McKhann, The Johns Hopkins University School of Medicine, W. Ian McDonald, University College London, Peter J. Goadsby, University College London, Justin C. McArthur, The Johns Hopkins University School of Medicine
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- Book:
- Diseases of the Nervous System
- Published online:
- 05 August 2016
- Print publication:
- 11 November 2002, pp 1501-1526
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Summary
The following chapter reviews principles of immunology to provide an understanding of how components of the nervous system are recognized by the immune system, how an autoimmune response is mounted, how immune cells and mediators enter the nervous tissue, and how tolerance against neural antigens is induced, maintained, and broken. Most of the general principles have been discovered since the 1980s but only with the new technology of targeted deletions and mutations (permanent and conditional knockout, knockin) can these principles be systematically explored at the molecular level. A brief discussion of multiple sclerosis, the Guillain–Barré syndrome, and myasthenia gravis will follow, while clinical aspects and disease-specific pathomechanisms of immune-mediated neurological disorders are presented in greater detail in individual chapters. Therapeutic consequences based on immunological principles are discussed in Chapter 93.
Categories of the immune response
The immune system is a multifaceted system of cells and molecules with specialized tasks in defending the organism from external agents, infectious or toxic. Moreover, the immune system plays a pivotal role in maintaining antigenic homeostasis in the body. Two types of responses to invading organisms can take place: an acute response launched within hours, and a delayed response occurring within days. The immediately responding system is termed innate immune system, and it evolves stereotypically and at the same magnitude regardless how often the infectious agent is encountered. In contrast, a more delayed response is delivered by the adaptive or acquired immune system and provides a more specific immunologic reaction which improves in efficiency on repeated exposure to a given infective agent, capitalizing on the formation of immunological memory. The immune system has traditionally been divided into innate and adaptive systems, each containing different cellular and molecular components. The main distinction between these two systems lies in the mechanisms and receptors used for immune recognition. These two systems are not separated, but are functionally connected allowing for intensive interactions (Carroll & Prodeus, 1998; Ochsenbein & Zinkernagel, 2000).
The innate immune system
During evolution, the innate immune system appeared before the adaptive immune system, and some form of innate immunity probably exists in all multicellular organisms. Characteristically, innate immune responses consist of all the immune defence mechanisms that do not require immunologic memory. Genetically, the molecular mediators and their receptors are highly conserved between species as far apart as Caenorhabditis elegans, Drosophila, and mammals.