Paraneoplastic Myeloneuropathies

Shah, S. M.; Campo, Rocio Vazquez Do; Kumar, Neeraj; McKeon, Andrew; Flanagan, Eoin P.; Klein, Christopher J.; Pittock, Sean J.; Dubey, Divyanshu

doi:10.1212/wnl.0000000000011218

Cited by 32 publications

(18 citation statements)

References 40 publications

(55 reference statements)

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“…In the current case, this patient showed prominent response to immunomodulatory treatments without carcinoma management, which was rare in previous reports describing paraneoplastic syndrome. The nerve conduction study revealed prominent demyelinating features, which was not observed in previous studies [ 14 , 15 ]. The variation of the clinical phenotype and examination results from a “classical” paraneoplastic syndrome may be caused by the NF186 antibody.…”

Section: Discussioncontrasting

confidence: 61%

Paraneoplastic Syndrome Presenting Combined Central and Peripheral Demyelination Associated with Anti-CV2/CRMP5 and Anti-NF186 Antibodies: A Case Report

et al. 2023

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The anti-CV2/CRMP5 antibody is a well-characterized biomarker of paraneoplastic neurological syndrome. The anti-NF186 antibody is a recently discovered antibody associated with central or peripheral demyelination. The co-occurrence of these two antibodies has not been reported. Herein, we report a case with anti-CV2/CRMP5 and anti-NF186 antibodies in a 57-year-old male presenting with progressive numbness and weakness in his four limbs. At first admission, the spinal cord MRI showed a cervical cord demyelinating lesion and electrophysiological examination showed a mixed demyelinating and axonal polyneuropathy. Anti-CV2/CRMP5 and anti-NF186 antibodies were both detected in his serum. Initially, the patient showed a positive response to IVIG and glucocorticoid treatment. However, the syndrome relapsed and mass lesions in lung and mediastinum were detected at second admission. This time the anti-NF186 antibody was not detected but the anti-CV2/CRMP5 antibody was still present. IVIG and glucocorticoid treatment was no longer effective. This case illustrated that paraneoplastic syndrome should be considered when diagnosing patients with central and peripheral demyelination, and that the anti-NF186 antibody may help distinguish a subset of patients who can benefit from immunomodulatory treatments.

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Section: Discussioncontrasting

confidence: 61%

Paraneoplastic Syndrome Presenting Combined Central and Peripheral Demyelination Associated with Anti-CV2/CRMP5 and Anti-NF186 Antibodies: A Case Report

et al. 2023

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“…Blood and CSF laboratory studies are critical for establishing an accurate diagnosis of inflammatory myelopathy, not only for determining the role of inflammation as a pathogenic mechanism but also for providing a specific diagnosis in some types of myelitis. The discovery of specific pathogenic antibodies that target neural and glial antigens such as aquaporin 4, MOG, glial fibrillary acidic protein (GFAP), and other antigens linked to paraneoplastic disorders (eg, collapsin response mediator protein-5 [CRMP5]) [25][26][27] can be used as biomarkers of disease and define the subgroup of myelitis associated with autoimmune neurologic disorders. Testing of these autoantibodies should be centered on serologic studies rather than CSF because such autoantibodies are less frequent in CSF samples.…”

Section: Biomarkers In Autoimmune Inflammatory Myelopathiesmentioning

confidence: 99%

Clinical Approach to Myelopathy Diagnosis

Pardo

2024

CONTINUUM: Lifelong Learning in Neurology

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OBJECTIVE This article describes an integrative strategy to evaluate patients with suspected myelopathy, provides advice on diagnostic approach, and outlines the framework for the etiologic diagnosis of myelopathies. LATEST DEVELOPMENTS Advances in diagnostic neuroimaging techniques of the spinal cord and improved understanding of the immune pathogenic mechanisms associated with spinal cord disorders have expanded the knowledge of inflammatory and noninflammatory myelopathies. The discovery of biomarkers of disease, such as anti–aquaporin 4 and anti–myelin oligodendrocyte glycoprotein antibodies involved in myelitis and other immune-related mechanisms, the emergence and identification of infectious disorders that target the spinal cord, and better recognition of myelopathies associated with vascular pathologies have expanded our knowledge about the broad clinical spectrum of myelopathies. ESSENTIAL POINTS Myelopathies include a group of inflammatory and noninflammatory disorders of the spinal cord that exhibit a wide variety of motor, sensory, gait, and sensory disturbances and produce major neurologic disability. Both inflammatory and noninflammatory myelopathies comprise a broad spectrum of pathophysiologic mechanisms and etiologic factors that lead to specific clinical features and presentations. Knowledge of the clinical variety of myelopathies and understanding of strategies for the precise diagnosis, identification of etiologic factors, and implementation of therapies can help improve outcomes.

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“…Notably, only approximately a third demonstrates enhancement. 9 Only approximately 50% of cases show clinical improvement after treatment with combined tumor and immunosuppressive therapies; 25% show clinical stability; and the remaining 25% deteriorate despite all available treatments. 9…”

Section: Drs Handzic and Margolinmentioning

confidence: 99%

Anti-Ma2 Antibody-Mediated Paraneoplastic Cerebellar Degeneration and Myeloneuropathy Secondary to Lymphoma

Handzic,

Brossard-Barbosa,

Mandell

et al. 2023

Journal of Neuro-Ophthalmology

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A 61-year-old woman with a history of untreated low-grade B-cell lymphoma presented with blurry vision, unsteadiness, and worsening pain on touching skin of the upper trunk was enrolled. Blurry vision was attributed to oscillopsia from downbeat nystagmus, which later evolved into macrosaccadic oscillations. MRI brain and spine showed mild, longitudinally extensive T2 hyperintensity in the central gray matter of the spinal cord extending from the medulla to T11 level. Serum paraneoplastic panel was negative; however, she had very high titers of anti-Ma2 antibodies in cerebrospinal fluid. The diagnosis of paraneoplastic neurological syndrome was made. Empiric treatment with high dose of intravenous steroids followed by intravenous immunoglobulin infusions did not improve her symptoms. An extensive search for an underlying tumor commenced and was initially unrevealing. However, two-month follow-up positron emission tomography scan showed increased uptake in a right pulmonary nodule, which when biopsied confirmed diagnosis of extranodal marginal zone lymphoma. The final diagnosis was anti-Ma2 antibody-mediated paraneoplastic cerebellar degeneration and myeloneuropathy secondary to lymphoma.

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Paraneoplastic Myeloneuropathies

Cited by 32 publications

References 40 publications

Paraneoplastic Syndrome Presenting Combined Central and Peripheral Demyelination Associated with Anti-CV2/CRMP5 and Anti-NF186 Antibodies: A Case Report

Paraneoplastic Syndrome Presenting Combined Central and Peripheral Demyelination Associated with Anti-CV2/CRMP5 and Anti-NF186 Antibodies: A Case Report

Clinical Approach to Myelopathy Diagnosis

Anti-Ma2 Antibody-Mediated Paraneoplastic Cerebellar Degeneration and Myeloneuropathy Secondary to Lymphoma

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