Intravascular lymphomatosis presenting with a conus medullaris syndrome
mimicking disseminated encephalomyelitis

Schwarz, Štefan; Zoubaa, Saida; Knauth, Michael; Sommer, Clemens; Storch-Hagenlocher, B.

doi:10.1093/neuonc/4.3.187

Cited by 16 publications

(5 citation statements)

References 13 publications

(25 reference statements)

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“…On the other hand, as steroid treatment is generally known to be effective to some extent in both diseases, the efficacy of steroids is not useful for distinguishing the two diseases. However, as was seen in the present case, steroid therapy was not very effective in all four of the previously reported patients who received it ( 3 - 8 ) ( Table 3 ). This may be due to the fact that irreversible vascular-related lesions frequently develop in the spinal cord of patients with IVLBCL ( 3 , 5 , 12 , 13 ), meaning that efficacy and functional outcome would depend on the size of the stroke lesion in each case.…”

Section: Discussionsupporting

confidence: 60%

“…However, as was seen in the present case, steroid therapy was not very effective in all four of the previously reported patients who received it ( 3 - 8 ) ( Table 3 ). This may be due to the fact that irreversible vascular-related lesions frequently develop in the spinal cord of patients with IVLBCL ( 3 , 5 , 12 , 13 ), meaning that efficacy and functional outcome would depend on the size of the stroke lesion in each case. Therefore, to improve the functional prognosis, IVLBCL needs to be identified early before the lesion expands.…”

Section: Discussionsupporting

confidence: 60%

“…It may be challenging to clinically establish a diagnosis of IVLBCL and myelopathy due to its rarity. So far, only 6 IVLBCL cases with long spinal cord lesions extending over three or more vertebral segments have been reported ( Table 3 ) ( 3 - 8 ). The clinical presentation in the present case resembled that in the previously reported cases, all of which demonstrated subacute disease onset and primary progression of a motor and sensory deficit, accompanied by longitudinal lesions on spinal MRI ( Table 3 ) ( 3 - 8 ).…”

Section: Discussionmentioning

confidence: 99%

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Long Spinal Cord Lesions Caused by Venous Congestive Myelopathy Associated with Intravascular Large B-cell Lymphoma

Miura

Saito

et al. 2021

Intern. Med.

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Intravascular large B-cell lymphoma (IVLBCL) is a subtype of B-cell lymphoma, characterized by lymphoma cell proliferation within small blood vessels. We herein describe a rare case with long spinal cord lesions caused by venous congestive myelopathy associated with IVLBCL. An 81-year-old man presented with paraplegia of the lower limbs and sensory disturbances. Magnetic resonance imaging revealed intramedullary longitudinal T2-hyperintensity lesions in the thoracic cords. The patient died three months after disease onset, and a neuropathological analysis revealed predominantly atypical B-lymphocytes located sparsely in the veins of the spinal cord. IVLBCL should be considered in the differential diagnoses of long spinal cord lesions.

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Section: Discussionsupporting

confidence: 60%

Section: Discussionsupporting

confidence: 60%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Long Spinal Cord Lesions Caused by Venous Congestive Myelopathy Associated with Intravascular Large B-cell Lymphoma

Miura

Saito

et al. 2021

Intern. Med.

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“…43 Peripheral nerve involvement has been described as well. 44 Reported signal characteristics include T1: isointense to the spinal cord/T2: hyperintense (contrasts with the characteristic low T2 signal intensity that is seen in intracranial lesions)/T1 Cþ (Gd): usually solid and homogeneous enhancement. 45 The patient evolved with loss of strength and hemiparesis on the right side of the body and superficial hemiparesthesia on the left side, thus suggesting BSS, which was confirmed by imaging tests.…”

Section: Discussionmentioning

confidence: 99%

B-Cell Lymphoma Intramedullary Tumor: Case Report and Systematic Review

Gonsalves¹,

Raffa²,

Sousa

et al. 2023

Asian J Neurosurg

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Intramedullary tumors represent the major cause of spinal cord injuries, and its symptoms include pain and weakness. Progressive weakness may concomitantly occur in the upper and lower limbs, along with lack of balance, spine tenderness, sensory loss, trophic changes of extremity, hyperreflexia, and clonus. The study protocol was in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. A systematic search of the MEDLINE electronic database was performed to identify the studies reporting the clinical features of children and adults who presented with an intramedullary lymphoma. Twenty-one studies were included, reporting 25 cases. Manuscripts were excluded if the full-text article was not available, original data were not reported (e.g., review articles), or if the main disease was not intramedullary lymphoma. A structured data extraction form was employed to standardize the identification and retrieval of data from manuscripts. To enlighten the discussion, a case is also presented. An 82-year-old woman with Fitzpatrick skin type II, diagnosed and treated for non-Hodgkin's lymphoma 7 years ago, was admitted with mental confusion and memory loss for the past 2 months—evolving with recurring falls from her own height. One day before admission, she displayed Brown-Séquard syndrome. An expansive lesion from C2 to C4 in the cervical spinal cord was found and a hypersignal spinal cord adjacent was described at the bulb medullary transition to the C6–C7 level. A primary spinal cord tumor was considered, as well as a melanoma metastasis, due to the lesion's flame pattern. The patient presented a partial recovery of symptoms and a reduction of the spinal cord edema after being empirically treated with corticosteroids, but the lesion maintained its extent. Subsequently, a large diffuse B-cell lymphoma with nongerminal center was found in open body biopsy, infiltrating neural tissue. The main objective of the present study is to report a surgical case treated for a large diffuse B-cell lymphoma, in addition to presenting the results of a systematic review of primary intramedullary spinal cord lymphoma.

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“…Considering the few published case reports of this disease, the clinical features of PISCL are unclear, resulting in complicated diagnosis and delayed therapy. In some cases, diagnosis of this disease is not confirmed until an autopsy is performed [2,3].…”

Section: Introductionmentioning

confidence: 99%

Nomograms Predict Survival Outcome of Primary Intramedullary Spinal Cord Lymphoma Patients

Yang

2019

Med Sci Monit

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BackgroundPrimary intramedullary spinal cord lymphoma (PISCL) is a rare cause of myelopathies. Considering its poor prognosis, it is essential to determine the appropriate treatment strategies and to develop nomograms to predict survival outcome for PISCL patients.Material/MethodsData were collected from the Surveillance, Epidemiology and End Results (SEER) database. We used 364 patients to investigate overall survival (OS) and 289 patients for cancer-specific survival (CSS). Kaplan-Meier method was to evaluate correlations of survival with different treatment strategies and clinicopathologic factors. Univariate and multivariable analyses were conducted to assess OS and CSS based on different variables. Risk factors were integrated to build nomograms.ResultsMost of the 414 PISCL patients diagnosed with positive histology had diffuse B cell lymphoma, were under 60 years old, were male, were of white race, had 1 primary tumor, were married, were low stage, and had previously undergone chemotherapy. We found that radiation therapy had no effect on patient OS and CSS, and patients receiving chemotherapy alone tended to have better OS and CSS in comparison with other groups. In addition, we showed that clinicopathologic factors, including histologic type, age, stage, and marital status, could serve as independent prognostic factors for PISCL patient OS and CSS. These factors were utilized to construct nomograms. The calibration curves demonstrated good agreement. The concordance indexes for OS and CSS were 0.672 (P=0.024) and 0.683 (P=0.029), respectively.ConclusionsPractical nomograms were established for patients’ OS and CSS. Besides, this study can guild clinician to make the right decision for appropriate treatment of PISCL patients.

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Intravascular lymphomatosis presenting with a conus medullaris syndrome mimicking disseminated encephalomyelitis

Cited by 16 publications

References 13 publications

Long Spinal Cord Lesions Caused by Venous Congestive Myelopathy Associated with Intravascular Large B-cell Lymphoma

Long Spinal Cord Lesions Caused by Venous Congestive Myelopathy Associated with Intravascular Large B-cell Lymphoma

B-Cell Lymphoma Intramedullary Tumor: Case Report and Systematic Review

Nomograms Predict Survival Outcome of Primary Intramedullary Spinal Cord Lymphoma Patients

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