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Unusual presentation of a primary spinal lymphoma
  1. K A Jellinger1,
  2. W Grisold2
  1. 1L Boltzmann Institute of Clinical Neurobiology, PKH/B-Building, Baumgartner Hoehe 1, A-1140 Vienna, Austria
  2. 2L Boltzmann Institute of Neuro-Oncology, Kaiser Franz Josef-Spital, Kundratstraβe 3, A-1100 Vienna, Austria
  1. Correspondence to:
 Dr K A Jellinger;
 kurt.jellinger{at}univie.ac.at
  1. A Wilkening3,
  2. M Brack3,
  3. F Heidenreich3,
  4. R Dengler3,
  5. K Weiβenborn3,
  6. A Brandis4
  1. 3Department of Neurology, Medical School of Hannover, Carl-Neuberg Strasse 1, D-30625 Hannover, Germany
  2. 4Department of Neuropathology

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    Wilkening et al1 described the unusual history of a formerly healthy immunocompetent white woman initially presenting with an S1 syndrome with lymphocytic pleocytosis in the CSF, leading to the misdiagnosis of meningoradiculitis. Repeated spinal MRI disclosed an epidural mass lesion, and histological examination of the tumour biopsy confirmed the diagnosis of primary spinal lymphoma of Burkitt's type. The authors emphasise that in every case with suspected meningoradiculitis including neuroborreliosis, meningeal lymphoma should be considered as an important differential diagnosis. In view of the occasionally difficult diagnosis of primary meningeal lymphoma we briefly present the clinicopathological report of a primary malignant lymphoma with almost selective involvement of the spinal roots: A woman aged 65 years without known systemic lymphoma presented with a painful focal right brachial plexus lesion and, 2 weeks later, developed progressive motor neuropathy with flaccic paraparesis of the lower limbs. Laboratory tests were negative except for her CSF showing increased protein content and 85 lymphocytes/mm3, not expressing the B cell tumour marker CD 20. Nerve conduction studies suggested subchronic demyelinating neuropathy without motor conduction block. Cranial and cervical CT and MRI were negative. Sural nerve biopsy disclosd a demyelinating neuropathy with acute myelin loss, some remyelination with incipient onion bulb formation, and wallerian degeneration, but without inflammatory or neoplastic infiltration. Muscle biospy showed neurogenic muscle atrophy. Despite high dose corticosteroid and intravenous immunoglobulin treatment, her condition deteriorated rapidly with develoment of flaccid paraplegia and paresis of the right arm. Repeated CSF analysis showed increasing protein content and pleocytosis up to 180 cells/mm3, mainly transformed but with no neoplastic lymphocytes, again none being immunopositive for the B cell markers CD 19 and CD 20. Extensive search for extraneural malignancy including repeated cranial, spinal, and abdominal CT and MRI scans were all negative. The patient died 6 months after the onset of neurological symptoms. Necropsy disclosed diffuse B cell lymphoma2 restricted to the cauda equina with massive infiltration of the anterior lumbal spinal nerve roots and dorsal ganglia, and very mild neoplastic infiltrates in the schiadic and femoral nerves associated with severe myelin loss. There was no involvement of the spinal cord, brain, meninges, or cranial nerves. The right brachial plexus showed severe demyelination without onion bulb formation and perivascular infiltrates exclusively formed by T lymphocytes. Search for systemic malignant lymphoma was negative except for an isolated small focal area of B cell lymphoma in the thyroid gland.3 As in the case reported by Wilkening et al,1 this finding widens the range of possibilities and differential diagnostic difficulties of selective involvement of the spinal roots (and peripheral nervous system) by primary malignant non-Hodgkin's lymphomas, often also referred to as “neurolymphomatosis”3–5 making an extensive diagnostic investigation necessary.

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    Authors' reply

    We greatly appreciate the interesting comments of Jellinger and Grisold who describe another unusual case of primary spinal lymphoma. In their case, by contrast with that presented by us the spinal roots were selectively involved without the formation of a mass lesion during the disease. Moreover, CSF studies in their case showed increased protein content and pleocytosis but no neoplastic cells, which had been found in our patient. Together, the case presented by us and the case of Jellinger and Grisold underlines the fact that the range of primary cerebral and especially primary spinal lymphoma is very wide with different clinical presentation, location of manifestation, prognosis, and probably of aetiology. More cliniconeuropathological studies on primary spinal lymphoma are necessary to better understand this disease and to develop therapeutic strategies for its different types.

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