Arber, C.

Czaplinski, A.

Steck, A.

Sutter, R.

Tichelli, A.

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R. SutterC. ArberA. TichelliA.J. SteckA. CzaplinskiCranial and peripheralneuropathydue to leptomeningealinfiltration in a patientwith Waldenstrom’smacroglobulinemiaReceived: 20 April 2006Received in revised form: 26 August 2006Accepted: 7 September 2006Published online: 3 August 2007Sirs: Lymphoplasmacytic lym-phoma or Waldenstrom’s macro-globulinemia (WM) is a chronicmature B-cell lymphoproliferativedisorder of plasmacytoid lympho-cytes usually involving bone mar-row, lymph nodes and spleen. Aserum monoclonal immunoglob-ulin M (IgM) may be present andmay be associated with hypervis-cosity and/ or cryoglobulinemia.We present the case of a 70 yearold male who was diagnosed withWM. Bone marrow infiltration attime of diagnosis was 40%, themonoclonal IgM kappa in periph-eral blood was 11.5g/l, and cryo-globulines were present. A therapywith four cycles of R-CHOP (rit-uximab, cyclophosphamid, doxo-rubicin, vincristin, and prednison)was administered. The patient re-sponded well to therapy andachieved a good partial remissionwith only a residual bone marrowinfiltration of 5–10% and themonoclonal IgM kappa in periph-eral blood was 0.7 g/l.Six months later the patientgradually developed persistentheadaches, nuchal rigidity, doublevision, dysphagia and dysarthria.The clinical examination atadmission showed meningism andsevere bulbar paralysis along withsymptoms of sensori-motor poly-neuropathy.Examination of cerebrospinalfluid disclosed 106 cells/mm3, ele-vated protein level (3160 mg/l) andelevated beta-2-microglobulin(6.35 mg/l). Cytological analysesrevealed atypical lymphocytes andflow cytometry analysis showed akappa monoclonal CD19 positiveB-cell population. Bone marrowexamination showed a persistentmultifocal infiltration with lym-phoplasmacytic lymphoma (15% ofnucleated cells). Transformation ina diffuse large B cell lymphoma wasexcluded. The serum monoclonalIgM kappa was 0.4 g/l, cryoglobu-lines were low, and serum anti-MAG (myelin associated glycopro-tein) antibodies were negative. Thebrain MRI showed a mild thicken-ing of leptomeninges without tu-mor mass or meningealenhancement. In addition the MRIof the lumbar spinal cord aftergadolinium injection showed anincreased signal in perimedullaryand cauda equina roots regions, aswell as enlargement of the latter(Fig. 1 and 2).We interpreted the symptomsas cranial and peripheral neurop-athy due to leptomeningeal andnerve root infiltration and the pa-tient was given a palliative radia-tion-therapy of the brain and thecauda with simultaneous steroidtherapy. The patient’s conditionimproved with a clear decrease ofbulbar and polyneuropathicsymptoms. Although these thera-peutic interventions were estab-lished, the patient died elevenweeks after diagnosis.Sensori-motor neuropathy re-lated to the antibody activity of themonoclonal IgM to the MAG is awell-known neurological compli-cation of WM [2, 3]. However,involvement of the peripheralnervous system due to leptomen-ingeal infiltration by neoplasticcells is very rare and has been re-ported in few cases only [1, 4–6].Remarkably, in contrast to othernon-Hodgkin’s lymphomas wheremeningeal involvement developsin the setting of progressive dis-ease, in our patient meningealinfiltration occurred in a stablepartial remission phase of the WM.In conclusion, we hypothesizethat WM may progress in thenervous system without transfor-mation into a more malignant B-cell-lymphoma (Richter syn-drome) even if the initial chemo-therapy leads to significantremission of the disease.Dr. R. Sutter (&) Æ A.J. Steck ÆA. CzaplinskiDept. of NeurologyUniversity of BaselPatersgraben 44031 Basel, SwitzerlandE-Mail: sutte r@uhbs.chC. Arber Æ A. TichelliDept. of Hematology, University of Basel,SwitzerlandFig. 1 Axial T1-weighted MRI of lumbar spinalcord after gadolinium showed an increased signalin perimedullary regionsLETTER TO THE EDITORSJ Neurol (2007) 254:1122–1123DOI 10.1007/s00415-006-0405-7rReferences1. Abad S, Zagdanski A-M, Brechignac S,Thioliere B, Brouet JC, Mariette X (1999)Neurolymphomatosis in Waldenstro¨m’smacroglobulinaemie. Br J Haematol 106:100–1032. Braun PE, Frail DE, Latov N (1982)Myelin-associated glycoprotein ist theantigen for a monoclonal IgM in poly-neuropathy. J Neurochem 39: 1261–12653. Dellagi K, Dupouey P, Brouet JC, Bille-cocq A, Gomez D, Clauvel JP, SeligmannM (1983) M. Waldenstrom’s macro-globulinemia and peripheral neuropa-thy: a clinical study of 25 patients. Blood62: 280–2854. Hug A, Haas J, Storch-Hagenlocher B,Wildemann B (2004) LeptomeningealeTumorzellinfiltration als Erstmanifesta-tion eines Immunozytoms (M. Wald-enstro¨m). Nervenarzt 75: 1012–10155. Massengo S, Riffaud L, Morandi X,Bernard M, Verin M (2003) Nervoussystem lymphoid infiltration in Wald-enstro¨m’s macroglobulinemia. A casereport. J Neuro-Oncol 62: 353–3586. Noel J, Gille M, Garbar Ch, Martens M,Verhulst D, Vandeput Y (2002) Primarycentral nervous system lymphoma withWaldenstro¨m’s macroglobulinaemia.Eur Neurol 47: 184–185Fig. 2 Sagittal T1-weighted MRI of lumbar spinal cord after gadoliniumshowed an increased signal in perimedullary and cauda equina roots regions1123

Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

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Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

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