Angiotrophic large cell lymphoma mimicking multiple sclerosis associated transverse myelitis

Ormsby, Adrian H.; Prayson, Richard A.; Heard, Robert

doi:10.1016/s0967-5868(99)90036-x

Cited by 9 publications

(4 citation statements)

References 13 publications

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“…Notably lymphadenopathy was absent. 5,8,25 Paraclinical findings [4][5][6] usually encountered in this condition are elevated blood LDH without alteration of the remaining liver function tests (85 -90%), elevated ESR (80%) and CSF protein with presence of oligoclonal bands 4 (only reported in a nine patients series with seven of them positive). In our case, blood LDH and CSF protein were increased on the first investigation.…”

Section: Commentmentioning

confidence: 99%

Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Savard

Verreault²,

Gould³

et al. 2008

Can. j. neurol. sci.

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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...

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Section: Commentmentioning

confidence: 99%

Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Savard

Verreault²,

Gould³

et al. 2008

Can. j. neurol. sci.

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“…Primary B cell lymphomas of the CNS (PCNSL) are relatively rare; they account for less than 1% of CNS tumours. Although lymphomas and MS lesions may cause similar MRI changes [ 52 , 66 ], these two entities can be distinguished easily by histology. B cell lymphomas are easy to diagnose due to their high cellularity, angiocentric growth pattern (Fig.…”

Section: Tumoursmentioning

confidence: 99%

Diagnosis of inflammatory demyelination in biopsy specimens: a practical approach

Kuhlmann¹,

2008

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Multiple sclerosis is the most frequent demyelinating disease in adults. It is characterized by demyelination, inXammation, gliosis and a variable loss of axons. Clinically and histologically, it shares features with other demyelinating and/or inXammatory CNS diseases. Diagnosis of an inXammatory demyelinating disease can be challenging, especially in small biopsy specimens. Here, we summarize the histological hallmarks and most important neuropathological diVerential diagnoses of early MS, and provide practical guidelines for the diagnosis of inXammatory demyelinating diseases.

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“…1,8,10 Antemortem diagnosis of human cases has been achieved following brain 1,4,8,11,12 or muscle [13][14][15] biopsy. Although it was not evident in this mare, neural manifestations are present in .80% of people affected by the condition 16,17 and, in human and small animal cases, have included ocular dysfunction, seizures, ataxia, circling, involuntary micturition and defecation, dementia, paresis, pain or altered pain sensation (hypesthesia/paresthesia/dysesthesia), and peripheral neuropathy. 5,8 The condition may be recognized if vascular flow abnormalities are evident during neuroimaging procedures.…”

mentioning

confidence: 67%

“…3,19,20 Endotheliotrophic lymphoma has been reported most commonly as a B-cell-derived lymphoma 8,11 and polymerase chain reaction techniques have been used to document immunoglobulin heavy chain rearrangement. 19,21,22 Proliferations of T cells, as was evident in this mare, have been reported in dogs 6,7 and humans, 5,[23][24][25][26] as have rare cases of histiocytic intravascular lymphoid accumulations. 27 The mechanism whereby malignant lymphocytes home to endothelial cells is unknown, but probably relates to the expression of endothelial adhesion molecules.…”

mentioning

confidence: 68%

Angiotrophic T‐Cell Lymphoma as a Cause of Regenerative Anemia in a Horse

Raidal

Clark

Raidal

2006

Veterinary Internal Medicne

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Angiotrophic large cell lymphoma mimicking multiple sclerosis associated transverse myelitis

Cited by 9 publications

References 13 publications

Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy

Diagnosis of inflammatory demyelination in biopsy specimens: a practical approach

Angiotrophic T‐Cell Lymphoma as a Cause of Regenerative Anemia in a Horse

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