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Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Published online by Cambridge University Press:  02 December 2014

Martin Savard ,
Steve Verreault ,
Peter V. Gould ,
Vincent Bernier  and
Jean-Pierre Bouchard
Martin Savard
Affiliation:
The Hôpital de l’Enfant-Jésus du CHA, Laval University, Quebec City, Quebec, Canada
Steve Verreault
Affiliation:
The Hôpital de l’Enfant-Jésus du CHA, Laval University, Quebec City, Quebec, Canada
Peter V. Gould
Affiliation:
The Hôpital de l’Enfant-Jésus du CHA, Laval University, Quebec City, Quebec, Canada
Vincent Bernier
Affiliation:
The Hôpital de l’Enfant-Jésus du CHA, Laval University, Quebec City, Quebec, Canada
Jean-Pierre Bouchard
Affiliation:
The Hôpital de l’Enfant-Jésus du CHA, Laval University, Quebec City, Quebec, Canada
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Abstract

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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:

<span class='bold'>RÉSUMÉ:</span>

Le lymphome intravasculaire à grandes cellules (LIGC) représente un défi diagnostique. Il s’ accompagne de symptomes 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 reconnaìtre son tableau clinique typique parce qu’ il existe un traitement efficace.

<span class='bold'><span class='italic'>Méthode:</span></span>

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 cone médullaire (CM) avec encéphalopathie subséquente chez le patient.

<span class='bold'><span class='italic'>Résultats:</span></span>

Nous rapportons le cas d’ une femme de 61 ans présentant une paraplégie causée par une lésion du CM, qui a évolué un an plus tard vers une encéphalopathie et éventuellement un coma. Le diagnostic de LIGC a été confirmé à l’ autopsie. Ce cas est similaire à huit autres cas rapportés dans la littérature de lésions du CM associées à un LIGC et il est connu que 80 à 90% de ces patients évoluent vers une encéphalopathie s’ ils ne sont pas traités.

<span class='bold'><span class='italic'>Conclusions:</span></span>

Le LIGC est une cause connue mais rare de coma. Il est extrèmement important de poser le diagnostic parce que cette maladie est fatale si elle n’ est pas traitée. Ce tableau clinique (lésion du cone médullaire évoluant éventuellement vers une encéphalopathie) est caractéristique et l’ issue sera meilleure si cette pathologie est identifiée.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 35 , Issue 3 , July 2008 , pp. 366 - 371
Copyright
Copyright © The Canadian Journal of Neurological 2008

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