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Views & Reviews Demyelinating Disease After Neurologically views & reviews Demyelinating disease after neurologically complicated primary Epstein-Barr virus infection P.F. Bray, MD; K.W. Culp, BS; D.E. McFarlin, MD; H.S. Panitch, MD; R.D. Torkelson, MD; and J.P. Schlight, BS Article abstract-This report describes five patients who, following a neurologically complicated primary Epstein- Barr virus infection, developed progressive or relapsing neurologic deficits. The sequelae in four patients followed 4 to 12 years led to the diagnosis of multiple sclerosis (MS). The fifth patient presented with acute disseminated sclerosis and exhibits diffuse neurologic deficits that have persisted for 2 years. We suggest that the diagnosis of an unexplained acute neurologic or psychiatric syndrome should raise the question of a primary EBV etiology. A precisely timed sero- logic and hematologic study of the blood is imperative to capture the essential evidence. The data presented represent a clinical association between a neurologically complicated primary EBV infection and both chronic and acute demyeli- nating disease. The evidence does not justify a conclusion that EBV virus causes MS. NEUROLOGY 1992;42:278-282 Antibodies to the ubiquitous Epstein-Barr virus The acute neurologic syndromes accompanying (EBV) are in the blood of patients wherever EBV infections take different clinical forms, includ- humans inhabit the planet. Almost all children in ing aseptic meningitis, encephalitis, optic papillitis, the underdeveloped countries are infected, silently cranial neuritis (especially Bell’s palsy), brainstem for the most part, by age 4 years. Until recently, encephalitis, cerebellitis, acute psychosis, severe the virus has maintained a balanced and economic encephalopathy with and without increased relationship with its host. However, in northern intracranial pressure (including Reye’s syndrome), Europe, United States, and Canada, where there is transverse myelitis, postinfectious polyneuritis less overcrowding and better sanitation, about (Guillain-Barre syndrome), brachial neuropathy one-half the population is not infected until the and, rarely, dysautonomia and movement disor- teen years or early adult life, when kissing ders. Although the pathogenesis remains unclear, becomes more habitual and the exchange of saliva almost all parts of the nervous system appear vul- allows the seeding of the uninfected person with nerable to involvement in IM.5-7There have been virus. Approximately one-half of these EBV- repeated descriptions of inflammatory infiltrates seronegative persons develop clinical, heterophile- and diffuse neuronal degenerative changes from positive, infectious mononucleosis (IM), and the brains of fatal cases, and similar pathologic other half experience a clinically unrecognized changes were present in one person convalescing EBV infection. from uncomplicated IM who died secondary to trau- A clinical association between glandular fever or ma.8 The virus has been recovered from CSF in two IM and concurrent acute neurologic complications documented cases of IM-associated encephaliti~,~J~ was documented over 50 years ago,1,2and when but fortunately, most patients with CNS complica- EBV was identified as the causal agent 25 years tions recover fully. late^-,^.^ the same association was ~onfirmed.~-~In Sumaya et all1 were among the first to publish exceptional cases, the disorder can be fatal for dif- serologic data that suggested a relationship ferent systemic reasons or be associated with acute between MS and EBV. Similar studies by us12J3 central or peripheral nervous system syndromes. showed that 100% of MS patients are seropositive Estimates of the frequency of neurologic complica- and that their blood antibody titers to the transfor- tions vary from 1 to 5%.536 mation EBV nuclear antigen (EBNA) and the lytic From the Departments of Neurology and Pediatrics (Dr. Bray, and K.W. Culp and J.P. Schlight), University of Utah, Salt Lake City, UT; the Section on Neuroimmunology (Dr. McFarlin), National Institute of Neurologic Disease and Stroke, NIH, Bethesda, MD; the Department of Neurology (Dr. Panitch), University of Maryland, Baltimore, MD; and the Division of Pediatric Neurology (Dr. Torkelson), University of Nebraska, Omaha, NE. Supported in part by the Clipped Wings (flying personnel, United Airlines), Richard Uriarte, and the Utah Association of Life Underwriters Received May 13, 1991. Accepted for publication in final form July 29, 1991. Address correspondence and reprint requests to Dr. Patrick F. Bray, 50 No. Medical Drive, University Hospital, Salt Lake City, UT 84132 278 NEUROLOGY 42 February 1992 viral capsid antigen (VCA) are significantly elevat- Results. A narrative description of the five ed. patients is given below. Over the past 6 years, we have watched for Patient 1. In 1977, a 30-year-old woman devel- patients with definite laboratory evidence of a pri- oped IM complicated by cerebellitis and brainstem mary EBV infection and in whom other diseases encephalitis. Two positive HA tests were demon- that might mimic a primary EBV infection have strated within 1 month after the onset of her sys- been excluded. Over this observation period, we temic IM symptoms. Over the ensuing 10 months found five patients with laboratory evidence of pri- the disease ran a relapsing-remitting course. CSF mary EBV infection whose courses were complicat- electrophoresis performed after her third relapse ed by acute neurologic syndromes and in whom showed four OCB. An EM1 CT head scan revealed progressive or relapsing neurologic deficits have bilateral frontal lobe white matter lesions. This been observed. patient was reported in abstract form in 1977.20 She was followed and treated in an outpatient MS Methods. The five patients to be reported all exhibited clinic at the National Institutes of Health for 10 hematologic or serologic evidence of a primary EBV years. At age 41 years, her disability had pro- infection, and were included only if an acute neurologic gressed markedly and she was placed in a nursing or psychiatric syndrome was documented, the cause was home. She died at the age of 42 years without otherwise obscure, and neurologic complications persist- autopsy. ed. All patients exhibited CSF evidence of inflammation and quantitative or qualitative immunoglobulin abnor- Patient 2. An ll-year-old girl presented with a malities. Patients with postinfectious polyneuritis were severe acute illness and neurologic findings sug- excluded. gesting brainstem and cerebellar deficits. A serum In making the clinical diagnosis of MS, we adhered to HA test was positive, and CSF examination dis- the standard riter ria.'^^'^ Clinicians have used the terms closed 48 mononuclear cells, a total protein of 38 “remitt i n g- re 1a p s i n g ” and “chronic pro gre s s i v e ” to mg/dl, and OCB on electrophoresis. Bilateral visual describe most cases, but differentiation of these two clini- and brainstem auditory evoked responses were cal types may be difficult and our patients had features abnormal, revealing delayed latencies. For 7 years of both. after the acute illness, she had a relapsing-remit- Three laboratory methods were used to identify a pri- ting course; serial head MRIs showed an increasing mary EBV infection. A standard heterophile antibody (HA) test was used. Manual differential white blood cell number and size of patchy, bilateral, cerebral white counts were performed and special attention was given matter lesions. She is now seriously incapacitated to the presence and percentage of large atypical lympho- and carries the clinical diagnosis of MS. cytes. A complete EBV antibody profile was performed, Patient 3. Within 1 month after making two sui- including an indirect immunofluorescence study of titers cide attempts and being treated with an antide- to VCA (IgG, IgA+IgM), early antibody-diffuse pressant, this 16-year-old girl gradually developed (IgG+IgA), early antibody-restricted (IgG), and EBNA alteration of mood and cognition, and impaired (IgG), and to a negative control. To insure reproducibili- vision. Her first examination revealed bilateral ty, assays were performed in both our laboratory and pyramidal signs, and her CSF exhibited 25 that of Werner and Gertrude HenleI6 at the University of mononuclear cells, increased CNS IgG synthesis, Pennsylvania. Even in patients who exhibit typical clini- cal, hematologic, or HA evidence of primary EBV infec- and OCB in the IgG zone. After treatment for pre- tion, our experience and that of the Henles (personal sumed herpes encephalitis without benefit, she communication) indicate that EBV-specific IgM may be showed progressive deterioration in cognitive func- difficult to demonstrate. This probably results from the tion and diffuse EEG slowing along with a fourfold large molecular size of IgM, which may impede the entry rise in serum anti-EBVCA IgG. A differential of the IgM antibody through the cell membrane, and the leukocyte count at the time of symptom onset difficulty of obtaining commercial fluorescein-labeled showed 12% “atypical lymphocytes.” A brain biopsy IgM that is not contaminated with VCA IgG; the latter performed 2% months after disease onset showed may lead to competition for antigenic binding sites to inflammation and marked loss of myelin, and the IgM molecules within the cell.’? No patient was included pathologist considered the findings most consistent in the study who did not fulfill the serologic or hemato- logic criteria for a primary EBV infection. Other causes with acute MS; an EBV DNA probe study was not for
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