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Morvan Syndrome and Diffuse Large B-Cell Lymphoma in the Central Nervous System
- Source :
- The neurologist. 25(3)
- Publication Year :
- 2020
-
Abstract
- Introduction The origin of contactin-associated protein-like 2 (Caspr2) antibodies in patients with Morvan syndrome is currently unknown. This case report investigated a possible association between the production of Caspr2 antibodies and aberrant proliferation of B lymphocytes. Case report We admitted a critically ill 65-year-old female patient with a suspected infection of the central nervous system (CNS). In addition to acquired neuromyotonia and CNS involvement, Caspr2 antibodies detected in her serum led to the presumptive diagnosis of Morvan syndrome. However, steroid and immunoglobulin treatment did not result in a satisfactory therapeutic outcome. On the basis of findings from immunohistochemistry, flow cytometric analysis, and immunoglobulin/T-cell receptor gene rearrangement detection of cerebrospinal fluid cells, we also made a concurrent diagnosis of diffuse large B-cell lymphoma in the CNS of this patient. The patient then received 4 cycles of rituximab and methylprednisolone therapy with an interval of 2 weeks, which temporarily led to a near-complete remission of her symptoms. Upon follow-up, her symptoms relapsed at 3 months after the last treatment with rituximab and methylprednisolone. Conclusions This is a first reported case of a patient who was concurrently diagnosed with Morvan syndrome and diffuse large B-cell lymphoma in the CNS. Additional studies are needed to determine whether aberrantly proliferating B lymphocytes are responsible for the production of Caspr2 antibodies.
- Subjects :
- Pathology
medicine.medical_specialty
Nerve Tissue Proteins
030204 cardiovascular system & hematology
03 medical and health sciences
0302 clinical medicine
Cerebrospinal fluid
medicine
Humans
Aged
biology
business.industry
Membrane Proteins
Gene rearrangement
medicine.disease
Syringomyelia
Lymphoma
Methylprednisolone
biology.protein
Immunohistochemistry
Rituximab
Female
Isaacs Syndrome
Lymphoma, Large B-Cell, Diffuse
Antibody
business
Diffuse large B-cell lymphoma
030217 neurology & neurosurgery
medicine.drug
Subjects
Details
- ISSN :
- 23312637
- Volume :
- 25
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- The neurologist
- Accession number :
- edsair.doi.dedup.....4bb32d35795fcf196b65642d181b2085