Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Abstract

366Intravascular large cell lymphoma (ILCL) is an extremely rare disorder, with a little more than one hundred cases reported in the literature, which remains difficult to diagnose. Cardinal manifestations are neurological (encephalopathy, stroke and myelopathy), cutaneous and constitutional. It was first described by Pfleger and Tappeiner in 1959 under the name angioendotheliomatosis systemisata proliferans because the authors thought the lesion was endothelial in origin. 1 However, it is now recognized that the tumor is rather an intravascular proliferation of lymphomatous cells, obstructing small vessels. 2,3 Unfortunately, the vast majority of neurological cases lack other manifestations, in particular cutaneous lesions. 4 Moreover, there ABSTRACT: Background: Intravascular large cell lymphoma (ILCL) is a diagnostic challenge, with neurological, cutaneous and constitutional symptoms. The natural history is usually an evolution to a comatose state. As invasive procedures are usually required for diagnosis, recognizing the typical clinical pattern is critical since an effective treatment is available. Method: After an extensive literature review of the subject, we report a case of ILCL, analyzing clinical, laboratory, radiological and pathological data. We will also give a special attention to the clinical picture of a conus medullaris (CM) lesion with subsequent encephalopathy in the same patient. Results: We report here a 61-year-old woman with a paraplegia caused by a CM lesion, evolving about one year latter to encephalopathy and eventual coma, with the diagnosis of ILCL confirmed by autopsy. The present case is similar to eight other cases in literature who had CM lesion associated with ILCL, knowing that 80-90% of these patients will eventually evolve to encephalopathy without treatment. Conclusions: ILCL is a recognized but rare cause of coma. Diagnosing it is tremendously important since it is fatal if left untreated. We propose that this specific picture (conus medullaris lesion, eventually evolving to encephalopathy) is quite characteristic and will directly result in better outcome if recognized.RÉSUMÉ: Lymphome intravasculaire avec syndrome du cône médullaire suivi d'une encéphalopathie. Le lymphome intravasculaire à grandes cellules (LIGC) représente un défi diagnostique. Il s'accompagne de symptômes neurologiques, cutanés et généraux. La maladie évolue habituellement vers un état comateux. Comme on doit avoir recours à une procédure invasive pour établir le diagnostic, il est très important de reconnaître son tableau clinique typique parce qu'il existe un traitement efficace. Méthode : Nous avons procédé à une revue de littérature exhaustive et nous rapportons un cas de LIGC avec analyse des données cliniques, biochimiques, radiologiques et anatomopathologiques. Nous portons une attention particulière au tableau clinique de la lésion du cône médullaire (CM) avec encéphalopathie subséquente chez le patient. Résultats : Nous rapportons le cas d'une femme de 61 ans présentant une paraplégie causée p...

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

“…As in our case, a subacute onset progressive paraparesis due to spinal cord involvement may be the presenting manifestation of the disease [10,20,21]. A predilection for the conus medullaris has been reported syndrome [8,16,20,22]. In our patient disease evolution was accompanied by extension of the intramedullary signal change up to the conus.…”

“…Additionally IVL, unlike inflammatory myelitis, affects the elderly [3,[5][6][7]10]. IVL should also be a consideration when a myelopathy is followed by encephalopathy or other manifestations of multifocal disease [7,22]. An encephalopathy antedated our patients' demise.…”

Intravascular lymphoma preceded by urinary retention and diagnosed by random skin biopsy

“…Twenty-nine (38 %) of 77 patients (including patients in the author's hospitals) experienced spinal cord and nerve root vascular syndromes, such as paraparesis, pain, and incontinence. However, there have been only six reports of IVL to our knowledge, presenting with urinary retention/incontinence as initial symptoms (Table 1) (Nakahara et al 1999;Yamashiro et al 2001;Schwarz et al 2002;Legeais et al 2004;Savard et al 2008;Tsugawa et al 2010). In these seven reports, definite diagnosis of IVL was made by autopsy in three cases, muscle biopsy in one case, brain biopsy in one case, and bone marrow biopsy in one case.…”

Primary spinal epidural non-Hodgkin’s lymphoma presented with spinal cord compression syndrome

“…They mainly occur in adults past the fourth or fifth decade [1]. This entity is even rarer in children [1,5,6]. The following report illustrated a PSENL in a young boy presented with spinal cord compression syndrome in the absence of clinical suspicion of lymphoma.…”

“…Primary spinal epidural non-Hodgkin's lymphomas (PSENLs) are relatively rare cause of spinal cord compression syndrome [1][2][3][4]. They mainly occur in adults past the fourth or fifth decade [1]. This entity is even rarer in children [1,5,6].…”