Neurology

letters to the editor Plasma cell reaction in cerebrospinal fluid: An additional case report To the Editor: Glasser, Payne, a n d Corrigan’ described a 4-week-old girl with “meningoencephalitis, etiology unknown, probably viral,” a n d abnormal proliferation of p l a s m a c e l l s i n c e r e b r o s p i n a l f l u i d ICSF), r e p r e s e n t i n g t h e i n v i v o r e c a p i t u l a t i o n of t h e transformation of B lymphocytes induced i n vitro by mitogenic stimuli. We previously’-4 demonstrated t h a t 93 percent of h u m a n CSF lymphocytes b e a r T-cell surface markers (E-rosette formation), whereas only 3 percent a r e identified as B cells ( m e m b r a n e complement recept ors 1. Three years ago we observed a patient with cerebral metastases from ;I gastric cancer, with a cellular reaction in CSF similar to t h a t described by Glasser, P u y n e , a n d C o r r i g a n . ’ We d i d n o t p u b l i s h o u r observation since we w e r e n o t a b l e t o perform imniunofluorescence on CSF cells, a n d morphology did not represent in our minds a n absolutely discriminative criterion between p l a s m a cells a n d m e t a s t a t i c neoplastic cells. T h e paper of Glasser, P a y n e , a n d Corrigan’ gives u s t h e opportunity to publish our d a t a , since these authors, too, describe CSF plasma cells without definition of cellular immunoglobulin content. This 38-year-old woman suffered for 2 years from three episodes of intestinal bleeding, postprandial epigastric pain, and vomiting. Following the l a s t episode, headache, nuchal rigidity a n d photophobia developed. Examination d e m o n s t r a t e d paleness, mydriasis and bilateral Lasegue signs. E E G was diffusely slow. C‘SF pressure was increased, b u t protein and sugar content were normal a n d cultures were sterile. Thirty white blood cells per microliter were f o u n d i n t h e CSF. C y t o c e n t r i f u g e s m e a r s w e r e performed as described by G l a s s e r , P a y n e , a n d Corrigan.’ stained with Pappenheim a n d methyl g r e e n - p y r o n i n e . T h e d i ffe r e n t i a 1 c e 11 c ou n t w a s : Lymphocytes I percent, transitional lymphocytes 17 percent, plasmacytoid lymphocytes 40 percent, plasma cells 4 1p e i w n t a n d unclassifiable cells 1percent. O t h e r laborat,ory d a t a , including bone marrow biopsy a n d s e r u m immunoglobulins. showed only moderate anemia. After it few days. severe intestinal bleeding led to coma and death. Postmortem examination showed undifferenti:ited c a r c i n o m a of t h e s t o m a c h , w i t h metastases in rcgional lymph nodes, left ovary, l u m b a r spinal cord, arid meninges. OUT.case differs from t h a t of Glasser, Payne, a n d Corrigan.’ I n our case t h e r e were more m a t u r e cell forms in t h e CSF ( 4 1percent plasma cells compared to 3 er’spatient).T h e p l a s m a cell reaction i n the CSF of both cases is diffkult to explain. As reported previously,:’.‘ t h e great majority of CSF T lymphocytes bear IgG Fc receptors. Cells with these characteristics represent about ‘LO percent of peripheral blood T lymphocytes and. according t o Moretta a n d associates,s they can be identified as suppressor cells (i.e., as l y m p h o c y t e s s h o w i n g a b i l i t y to i n h i b i t B cell diffrrentiution to plasma cells). T h e prevalence of t h i s T

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NELJKOI,O(:Y 28 August 1978

cell subpopulation i n CSF would explain why, despite strong i m m u n e stimulation, a small n u m b e r of plasma cells usually generate i n t h e central nervous system. I n the case described by Glasser, Payne, a n d Corrigan,’ a n d in ours, proliferation of CSF plasma cells could be e x p l a i n e d by e i t h e r ( 1) g e n e t i c a l l y d e t e r m i n e d deficiency of suppressor cells in CSF or (2) a functional inhibition of these T cells induced by viruses or by neoplastic cell proliferation. Some doubt remains regarding both cases: C a n we be absolutely certain that plasma cells a n d their precursors can be identified solely on t h e basis of morphologic criteria? PAOLO EMILIO MANCONI M D Instituto tli Igient., Utziuersrtn di

Cnglinri

MARIA GIOVANNA MARROSU, M D A N D R E A SPISSU, M D Cliriitn Nc~urologsca,Uriiuersitn Cagliari

tii

PIER FELICE TODDE, M U ANTONELLO FERELLI IGI D Ospetlult~Otitologi~0 Caglinri 1 09100 Cagllarl, I t n h

References 1.

2.

Glasser L, Payne C, Corrigan JJ: The in vivo development of plasma cells: A morphologic study ofhuman cerebrospinal fluid. Neurology (Minneap) 27:448-459,1977 Manconi PE, Zaccheo D, Bugiani 0 , et al: T anti B lymphocytes in normal cerebrospinal fluid. N Engl J Med 294:49, 1976

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Manconi P E , Marrosu MG, C i a n c h e t t i C, e t a l : Subpopulations of T lymphocytes in human cerebrospinal fluid. (Abstr) Eleventh World Congress of Neurology, Amsterdam. Amsterdam-Oxford, Excerpta Medica, 1977, P 97

Manconi PE, Zaccheo D, Bugiani 0,et al: Surface markers of lymphocytes from human cerebrospinal fluid. Predominance of T lymphocytes bearing receptors for Fc segment of IgG. Eur Neurol (in press) Moretta L, Webb SR, Grossi CE: Functional analysis of two human T cell subpopulations: Help and suppression of B cell responses by T cells bearing receptor sfor iqM or IgG. J Exp Med 146:184-200, 1977

Reply from the authors: Plasma cells have been identified in the CSF in m a n y pathologic conditions.] We consider this a local cellular i m m u n e response to a variety of antigens. Autopsy findings i n meningeal carcinomatosis typically show a mild lymphocytic infiltrate in the leptomeninges2 a n d reactive cells a r e frequently seen i n t h e CSF accompanying tumors. These cells include lymphocytes, plasma cells, siderophages, eosinophils a n d monocytes. Dr. Manconi a n d h i s colleagues h a v e shown t h a t both B a n d T lymphocytes a r e present i n normal CSF b u t t h a t T lymphocytes a r e present i n higher percentages t h a n i n t h e peripheral blood.3 T h e cells shed into t h e CSF a r e

a s s u m e d t o b e a n a c c u r a t e r e f l e c t i o n of t h e leptomeningeal lymphocyte population. Both populations of cells are capable of being stimulated by a variety of antigens. The nature of the antigenic stimulus is one of the variables determining the relative response of B and T lymphocytes. Since T cells have a role in regulating the activation of B lymphocytes, it is plausible t h a t the intensity of the plasmacytic reaction reflects a n alteration in either suppressor or helper T cell function. Although there is a higher percentage of mature plasma cells in Dr. Manconi’s case of metastatic carcinoma, the absolute number of mature plasma cells is small and less than in the case we reported ( 1 2 per microliter compared with 46 per microliter). The increased relative numbers of transitional cells in our case may be related t o the intensity of the cellular response.’ I t is difficult to give a satisfactory scientific answer to the s e m a n t i c i n n u e n d o s of t h e p h r a s e “absolute certainty.” The light- and electronmicrographs in our case ofprobable viral meningitis leave no doubt as t o t h e lymphocytic and plasma cell nature of the cellular e l e m e n t s described. Differentiation of exfoliated neoplastic epithelial cells from plasma cells should not present a problem. However, Dr. Manconi and his colleagues might find i t helpful t o compare the inflammatory infiltrate in the leptomeninges t o t h e CSF cytology t o confirm the presence of mature plasma cells and to estimate their relative numbers.

and, histologically, the eosinophilic spongiosis pointed to this diagnosis. Unfortunately the authors misquote us. According t o them, Beutner, Jordon and Chorzelski’ “suggest that since this autoantibody binds with some complement in vitro there is a strong etiologic relation.” In the report they cite, we actually stated t h a t pemphigus antibodies “appear to fix complement in vivo (though apparently not i n vitro).” This statement was subsequently confirmed by Jordon and associates,2 who verified the failure of pemphigus antibodies to fix complement in vitro. This discrepancy between the statement of Noguchi and Nishitani and the literature they cite is of fundamental importance because acantholytic lesions can be induced in skin explants maintained in organ culture in the presence of pemphigus antibodies. “Lesion” production occurs in the explants i n the absence of c ~ m p l e r n e n t . ~Indeed, -~ the F a b fragment of pemphigus antibodies (which is incapable of binding complement) can induce the production of acantholytic lesions in the tissue culture system.4 ERNEST H . REUTNER, M.D. TADEUSZ P. CHORZELSKI, M.D.

References 1.

2. LEWIS GLASSER, M.D. CLAIRE P A Y N E , Ph.D. JAMES J . CORRIGAN. J R . , M.D

References 1. 2. 3.

Peter S: The plasma cells of the cerebrospinal fluid. J Neurol Sci 4:227-239, 1967 Gonzalez-Vitale JC, Garcia-Bunuel R: Meningeal carcinomatosis. Cancer 37:2906-2911, 1976 Manconi PE, Zaccheo D, Bugiani 0, et al: T and B lymphocytes in normal cerebrospinal fluid. N Engl J Med 294:49, 1976

Myasthenia and pemphigus To the Editor: We have read with great interest the paper by Noguchi and Nishitani on “Immunologic studies of a case of myasthenia gravis associated with pemphigus vulgaris after thymectomy” (NEUROLOGY

26:1075-1080, 1976). T h e clinical and histologic features of the bullous eruption described were consistent with the diagnosis of pemphigus foliaceus rather t h a n pemphigus vulgaris. Clinically, the lack of mucous membrane involvement

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4. 5.

Beutner E H , Jordon RE, Chorzelski T P : The immunopathology of pemphigus and bullous pemphigoid. J Invest Dermatol 51:63-80, 1968 Jordon RE, Sams WM, Diaz G, et al: Negative complement immunofluorescence in pemphigus. J Invest Dermatol 57:407-410, 1971 Michel B, KO CS: Effect of pemphigus or bullous pemphigoid sera and leucocytes on normal human skin in organ culture. An in vitro model for the study of bullous diseases. J Invest Dermatol 62:541-542, 1974 Schiltz J, Michel B: Production of epidermal acantholysis in normal human skin in vitro by the IgG fraction from pemphigus serum. J Invest Dermatol 67:254-260, 1976 Deng JS, Beutner EH, Shu S: Pemphigus antibody action on skin explants. Kinetics of acantholytic changes and stability of antigens in tissue cultures of normal monkey skin explants. ( i n press)

R e p l y from the authors: We are grateful t o Drs. Chorzelski and Beutner for pointing out the mistake in our paper, in which we stated that the antiepithelial antibody binds complement in vitro, instead of in vivo. This was a typographical error, which we believe was made by the printer. We also appreciate the suggestion of Drs. Chorzelski and Beutner concerning the diagnosis of the skin lesion. I n addition t o frank myasthenia gravis and histologically proven t h y m o m a , t h e p a t i e n t showed a proteous cutaneous manifestation after partial thymectomy, and finally died in a myasthenic crisis on January 18, 1977, after a 9-year illness. Permission for autopsy was not obtained.

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T h e initial stage of t h e cutaneous disorder resembled dermatitis herpetiformis. However, t h e first s k i n biopsy showed definite suprabasal bullae with eosinophilic spongiosis. consistent with t h e histologic diagnosis of pemphigus vulgaris. As Emmerson' pointed out, eosinophilic spongiosis does not always rule out t h e diagnosis of pemphigus vulgaris. T h e second biopsy w a s performed when the patient improved after methylprednisolone therapy a n d showed acantholytic bulla formation in a superficial portion o f t h e epidermis, as indicated by Drs. Chorzelski a n d Beutner. Therefore, this case C O L I ~be ~ categorized a s a transitional a n d peculiar form of pemphigus. These diagnostic problems have been discussed from t h e dermatologic point of view in another paper.'

858 NEUROLOGY 28 August 1978

H iVlSHITIAM , M D S NOGUCHI, M D Dcpartrric,nt of .VcJurologt Kitario Hospital Osaka, Japan

References 1.

2.

Emmerson RW, Wilson-Jones E: Eosinophilic spongiosis in pemphigus; A report of unusual histological change in pemphigus. Arch Dermatol 97:252-257, 1968 Tagami H, Imamura S, Noguchi S, et al: Coexistence of peculiar pemphigus, myasthenia gravis and malignant thymoma. Dermatologica 152:181-190, 1976

Plasma cell reaction in cerebrospinal fluid: An additional case report PAOLO EMILIO MANCONI, MARIA GIOVANNA MARROSU, ANDREA SPISSU, et al. Neurology 1978;28;856 DOI 10.1212/WNL.28.8.856 This information is current as of August 1, 1978 Updated Information & Services

including high resolution figures, can be found at: http://www.neurology.org/content/28/8/856.1.citation.full.html

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Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1978 by the American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.

Plasma cell reaction in cerebrospinal fluid: an additional case report.

Neurology letters to the editor Plasma cell reaction in cerebrospinal fluid: An additional case report To the Editor: Glasser, Payne, a n d Corrigan’...
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