Adult - Diagnosis

Research

Paraneoplastic Neurological Diseases

Prof.Angela Vincent, Christian G Bien

Summary

Review article

Paraneoplastic neurological syndromes (PNS) are neurological disorders which are the indirect effect of a tumour, ie. in which there is no direct involvement of the tumour or its metastases or its treatment. Although some conditions can be caused by, for instance, alterations in the levels of circulating hormones or growth factors, the PNS are now generally thought of as immune-mediated conditions.

ACNR • VOLUME 7 NUMBER 5 • NOVEMBER/DECEMBER 2007

Inflammatory Diseases of the CNS I: Encephalitis

Dr Ingrid Mazanti Professor Roy O.Weller

Summary

Neuropathy article

Encephalitis can be defined as diffuse inflammation of brain tissue, or spinal cord (encephalomyelitis or just myelitis). Two major forms of encephalitis occur: those due to virus infections and those due to autoimmune inflammation - Acute Disseminated Encephalomyelitis (ADEM).

ACNR • VOLUME 4 NUMBER 3 JULY/AUGUST 2004

Viral encephalitis: a clinician’s guide
Tom Solomon, Ian J Hart, Nicholas J Beeching

The management of patients with suspected viral encephalitis has been revolutionised in recent years with improved imaging and viral diagnostics, better antiviral and immunomodulatory therapies, and enhanced neurointensive care. Despite this, disasters in patient management are sadly not uncommon. While some patients are attacked with all known antimicrobials with little thought to investigation of the cause of their illness, for others there are prolonged and inappropriate delays before treatment is started. Although viral encephalitis is relatively rare, patients with suspected central nervous system (CNS) infections, who might have viral encephalitis, are not. In addition, the increasing number of immunocompromised patients who may have viral CNS infections, plus the spread of encephalitis caused by arthropod-borne viruses, present new challenges to clinicians. This article discusses the Liverpool approach to the investigation and treatment of adults with suspected viral encephalitis, and introduces the Liverpool algorithm for investigation and treatment of immunocompetent adults with suspected viral encephalitis (available at www.liv.ac.uk/braininfections).

Pract Neurol 2007; 7: 288–305
doi:10.1136/jnnp.2007.129098

This article cites 56 articles, 30 of which can be accessed free at:
http://pn.bmj.com/cgi/content/full/7/5/288#BIBL

Diagnostic Testing for Encephalitis, Part I*

Julie E. Reznicek, D.O.,1 and Karen C. Bloch, M.D.,2 1Department of Medicine, 2Departments of Medicine and Preventive Medicine, Division of Infectious Diseases, Vanderbilt University School of Medicine, Nashville, Tennessee

Abstract Encephalitis is characterized by both its pleomorphic clinical presentation and its diagnostic challenges. Fever, headache, and alteration of consciousness are classically present; however, the diversity of neurological symptoms can make it difficult to distinguish encephalitis from other infectious and non-infectious central nervous system conditions. Identification of a specific pathogen has important therapeutic and prognostic implications for an individual patient and has broader public health significance in potentially identifying a need for prophylaxis of contacts or environmental control of arthropod vectors. Despite the availability of nucleic acid amplification-based tests, a specific pathogen is identified in less than 50% of cases. This two-part review will focus on issues in diagnostic testing, such as specimen selection and optimal use of serology and PCR techniques for pathogens causing encephalitis in North America. Part I introduces basic features of encephalitis, including the initial evaluation of patients with possible encephalitis, and then discusses aspects of diagnostic testing for encephalitis caused by specific agents, including herpes simplex virus, the non-simplex Herpesviridae, enteroviruses and agents associated with tick-borne diseases, specifically, Rocky Mountain spotted fever and ehrlichiosis.

Clinical Microbiology Newsletter 32:3,2010

Limbic encephalitis: a clinician’s guide

Jonathan M Schott
Honorary Research Fellow, Dementia Research Centre, Institute of Neurology University College London, UK and Specialist Registrar, Department of Neurology, Royal Free Hospital, London UK

Limbic encephalitis typically presents with subacute development of memory impairment, confusion, and alteration of consciousness, often accompanied by seizures and temporal lobe signal change on MRI. There is however no clear consensus as to the definition; even traditional distinctions between "encephalitis" and "encephalopathy", and between "delirium" and "dementia" may be blurred in such patients.

The term limbic encephalitis was initially coined to describe patients presenting with amnesia, psychiatric disturbances, and often seizures, and who had postmortem evidence both of occult neoplasia and fairly selective inflammation within the temporal lobes.1 More recently, however, it has also been used to describe patients with a similar phenotype but in whom an infectious or non-paraneoplastic autoimmune cause has been proven or suspected. Even in "typical" paraneoplastic limbic encephalitis, selective involvement of the limbic structures (hippocampus, amygdala, hypothalamus, insular and cingulate cortex) is often not proven histologically, but has been inferred from the clinical presentation . . .

Practical Neurology 2006;6:143-153; doi:10.1136/jnnp.2006.091827

Tick-borne encephalitis complicated by a polio-like syndrome following a holiday in central Europe

AENDEKERK R. P. P. (1) ; SCHRIVERS A. N. A. (1) ; KOEHLER P. J. (1) ;
(1) Department of Neurology, De Wever Hospital, P.O. Box 4446, 6401 CX Heerlen, PAYS-BAS

Abstract

We report a case of Central European tick-borne encephalitis (CETE) in a 54-year-old man, presenting with fever and neurological complications following a holiday in Austria. A disease resembling paralytic poliomyelitis may develop with upper and lower extremity paralysis, as is the case in our patient. Our patient was most likely infected by eating goat's cheese, made of unpasteurised goat's milk. The diagnosis was confirmed by a positive IgM antibody response to the virus in the serum.

CLINICAL NEUROLOGY AND NEUROSURGERY 1996, vol. 98, no3, pp. 262-264 (5 ref.)

The Management of Encephalitis: Clinical Practice Guidelines by the Infectious Diseases Society of America

Allan R. Tunkel, Carol A. Glaser, Karen C. Bloch, James J. Sejvar, Christina M. Marra, Karen L. Roos, Barry J. Hartman, Sheldon L. Kaplan, W. Michael Scheld, and Richard J. Whitley

Guidelines for the diagnosis and treatment of patients with encephalitis were prepared by an Expert Panel of the Infectious Diseases Society of America. The guidelines are intended for use by health care providers who care for patients with encephalitis. The guideline includes data on the epidemiology, clinical features, diagnosis, and treatment of many viral, bacterial, fungal, protozoal, and helminthic etiologies of encephalitis and provides information on when specific etiologic agents should be considered in individual patients with encephalitis.

Read guidelines

Factors influencing PCR detection of viruses in cerebrospinal fluid of patients with suspected CNS infections

NWS Davies, LJ Brown, J Gonde, D Irish, RO Robinson, AV Swan, J Banatvala, RS Howard
J Neural Neurosurg Psychiatry 2005;76:82-87. doi:10.1136/jnnp.2004.045336

Background:Polymerase chain reaction (PCR) is used to detect viruses in the cerebrospinal fluid (CSF) of patients with neurological disease. However data to assist its use or interpretation are limited.
Objective: We investigated factors possibly influencing viral detection in CSF by PCR which will also help clinicians interpret positive ad negative results.
Methods: CSF from patients with was tested for human herpesviruses types 1-6, JC virus, enteroviruses, and Taxoplasma gondii. The likelihood of central nervous (CNS) infection was classified as likely, possible, or unlikely. PCR findings in these categories were compared using single variable and logistic regression analysis.
Results: Of 787 samples tested, 97 (12%) were PCR positive for one or more viruses. Of episodes likely to be CNS viral infections, 30% were PCR positive compared to 5% categorised as unlikely. The most frequent positive findings were Epstein Barr virus (EBV), enteroviruses, and herpes simplex virus (HSV). Enteroviruses and HSV were found predominantly in the likely CNS viral infection group, whereas EBV was found mainly in the unlikely group. Positive PCR results were more likely when there were 3-14 days between symptom onset and lumbar puncture, and when CSF white cells count was abnormal, although a normal CSF did not exclude a viral infection.
Conclusions: Th diagnostic yield of PCR can be maximised by using sensitive assays to detect a range of pathogens in approximately timed CSF samples. PCR results, in particular EBV, should be interpreted cautiously when symptoms cannot readily be attributed to the virus detected.

:Last modified: September 2010