J Neurocrit Care.  2019 Jun;12(1):55-63. 10.18700/jnc.190083.

Primary central nervous system lymphoma with intramedullary spinal cord involvement mimicking inflammatory demyelinating disease

• Kim H
• Nam TS
• Levy M
• Lee KH
• Kim J
• Lee SJ

• KMID: 2452828
• DOI: http://doi.org/10.18700/jnc.190083

Abstract

BACKGROUND
Spinal cord involvement of primary central nervous system lymphoma (PCNSL) is rare in a young immunocompetent patient and can be misdiagnosed as an inflammatory demyelinating disease (IDD) of the central nervous system.
CASE REPORT
We report a case of PCNSL mimicking IDD in a previously healthy 46-year-old man with weakness in both hands for 1 week. Magnetic resonance imaging (MRI) of the cervical spinal cord revealed contrast-enhancing intraparenchymal and leptomeningeal lesions in the cervical spinal cord and medulla oblongata. Cerebrospinal fluid analysis revealed pleocytosis (37/mm³). The patient's symptoms and lesions improved with corticosteroid treatment. However, he developed semicomatose mentality 5 months later. Brain MRI, ventricular biopsy, and ¹â¸F -flurodeoxyglucose positron emission tomography/computed tomography confirmed PCNSL. The patient deceased 3 months later, despite high-dose methotrexate chemotherapy.
CONCLUSION
Persistent gadolinium-enhancing MRI lesions along the ventricular regions and spinal leptomeninges could differentiate PCNSL involving the spinal cord from IDD in the early stages of the disease.

Keyword

Primary central nervous system lymphoma; Neuromyelitis optica; Multiple sclerosis; Spinal cord; Magnetic resonance imaging

MeSH Terms

Biopsy
Brain
Central Nervous System*
Cerebrospinal Fluid
Cervical Cord
Demyelinating Diseases*
Drug Therapy
Electrons
Hand
Humans
Leukocytosis
Lymphoma*
Magnetic Resonance Imaging
Medulla Oblongata
Methotrexate
Middle Aged
Multiple Sclerosis
Neuromyelitis Optica
Spinal Cord*
Methotrexate

Figure

• 

  Fig. 1. Initial magnetic resonance imaging (MRI) of the brain and spinal cord 1 week after onset of symptoms. (A) Sagittal and (B, C) axial T2-weighted images show faint hyperintense multifocal lesions in the medulla oblongata (arrows) and cervical spinal cord (arrowhead). (D, E) High signal intensity in the medulla oblongata on T2-weighted images are not clearly detected on fluid-attenuated inversion recovery. No supratentorial or periventricular white matter lesion was seen on brain MRI (images not shown).

• 

  Fig. 2. Cervical spinal cord magnetic resonance imaging 3 weeks after the onset of symptoms. (A) Sagittal and (B, C) axial T2-weighted images show hyperintense lesions in the medulla oblongata and cervical spinal cord. (D) Gadolinium-enhanced T1-weighted images show leptomeningeal enhancement (arrowheads) and an intraparenchymal tadpole-like signal change (arrows) connected to the leptomeninges at the levels of (E) the medulla oblongata and (F) C2 vertebral body.

• 

  Fig. 3. Follow-up cervical spinal cord magnetic resonance imaging 6 months after the onset of symptoms. T2-weighted images (A, C) show marked decrease in multifocal lesions with no parenchymal enhancement (B, D). Gadolinium-enhanced T1-weighted sagittal images show persistent leptomeningeal enhancement (B, arrowheads).

• 

  Fig. 4. Brain computed tomography (CT) at the onset of the semicomatose status. Brain CT shows subependymal hyperdense lesions in all ventricles, including the right lateral ventricle, with hydrocephalus (A), and edema in the periventricular white matter and cerebral peduncle (B).

• 

  Fig. 5. Brain magnetic resonance imaging (MRI) after ventricular brain biopsy. (A, B) Axial T2-weighted images show hyperintensities in the hypothalamus and periventricular white matter. (C, D) Gadolinium-enhanced MRI shows multifocal subependymal nodular enhancing lesions.

• 

  Fig. 6. Pathologic findings. Hematoxylin and eosin staining (A) reveals large atypical lymphocytes mixed with small mature lymphocytes. Tumor cells were positive for CD20 (B, B-cell marker), with the Ki-67 labeling index approaching 80% (C), but negative for CD3 (D, T-cell marker) on immunohistochemical analysis (original magnification, ×200).

• 

  Fig. 7. 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) of the brain. 18F-FDG-PET shows abnormal FDG uptake in the right lateral ventricle (A, white arrow) and inferior 4th ventricle (C, black arrow). PET and coregistered PET/magnetic resonance fusion imaging shows asymmetric, mild hypermetabolic lesions along the body, atrium, and posterior horn of the right lateral ventricle with a maximum standardized uptake volume (SUVmax) of 4.4 (A, B), and moderate hypermetabolic lesions along the inferior 4th ventricle with an SUVmax of 5.6 (C, D).

• 

  Fig. 8. Brain magnetic resonance imaging (MRI) after high-dose methotrexate chemotherapy. (A, B) Axial T2-weighted images show complete resolution of the multifocal subependymal nodular lesions in the hypothalamus and ventricular regions. (C, D) Gadolinium-enhanced MRI shows diffuse pachymeningeal thickening with no leptomeningeal enhancement.

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