From PNS to CNS: characteristics of anti-neurofascin 186 neuropathy in 16 cases

Xie, Chong; Wang, Ze; Zhao, Nan; Zhu, Dezhi; Zhou, Xiajun; Ding, Jie; Wu, Yifan; Yu, Hao; Guan, Yangtai

doi:10.1007/s10072-021-05101-9

Cited by 8 publications

(6 citation statements)

References 34 publications

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“…However, the presence of the anti-NF186 antibody was intriguing. The anti-NF186 antibody was previously reported in patients with either demyelinating/axonal peripheral neuropathy or CNS demyelination [ 4 , 7 ]. A previous study revealed some patients with paraneoplastic syndrome had antibodies against DRG neurons, motor neurons, or Schwann cells, though anti-NF186 antibody was not detected [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionmentioning

confidence: 99%

“…Neurofascin 186 (NF186) is a cell adhesion molecule located on axons of the central and peripheral nervous system. Recent studies indicated that the presence of antibodies against NF186 may indicate acquired peripheral neuropathy or CNS demyelination [ 4 , 5 , 6 , 7 ]. However, NF186 was not detected in the CCPD cases.…”

Section: Introductionmentioning

confidence: 99%

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 spectrum of the diseases associated with neurofascin 186 antibodies is limited mainly to peripheral neuropathy ( 1 ) such as chronic demyelinating inflammatory polyneuropathy ( 2 ) as well as subacute nodopathy ( 3 ) and to amyotrophic lateral sclerosis ( 4 ). Recently, also central nervous system (CNS) involvement in neurofascin 186 autoantibody-associated peripheral neuropathy has been demonstrated ( 5 ). However, cognitive impairment has been rarely reported in association with neurofascin 186 antibodies ( 5 ), and there might be structures in the CNS involved in primary neuroinflammation in the peripheral nervous system.…”

Section: Introductionmentioning

confidence: 99%

“…Recently, also central nervous system (CNS) involvement in neurofascin 186 autoantibody-associated peripheral neuropathy has been demonstrated ( 5 ). However, cognitive impairment has been rarely reported in association with neurofascin 186 antibodies ( 5 ), and there might be structures in the CNS involved in primary neuroinflammation in the peripheral nervous system. Here we report two patients predominantly presenting with cognitive impairment as a clinical phenotype in which primary neuroinflammatory locus is probably not the peripheral nervous system.…”

Section: Introductionmentioning

confidence: 99%

Case report: Amnestic mild cognitive impairment in multiple domains associated with neurofascin 186 autoantibodies: Case series with follow-up and review

Hansen

Sagebiel

Rentzsch³

et al. 2023

Front. Psychiatry

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BackgroundNeurofascin 186 autoantibodies are known to occur with a diseased peripheral nervous system. Recently, also additional central nervous system (CNS) involvement has been reported in conjunction with neurofascin 186 autoantibodies. Our case enlarges the spectrum of neurofascin 186 antibody-related disease to include mild cognitive impairment (MCI).MethodsWe report here a case after having examined the patient files retrospectively, including diagnostics such as blood and cerebrospinal fluid (CSF) analysis involving the determination of neural autoantibodies, brain magnetic resonance imaging (MRI), brain fluorodesoxyglucose positron emission tomography (FDG-PET), and extensive neuropsychological testing.ResultsWe report on two patients with MCI. Brain MRI showed cerebral microangiopathy in both patients, but brain FDG-PET demonstrated pathology in the right prefrontal cortex, in the right inferior parietal cortex, and in both lateral occipital cortices in one patient. Neurofascin 186 antibodies were detected in serum in both patients, and neurofascin 186 autoantibodies were also detected in the CSF of one of these patients. At follow-up six month later, neurofascin 186 autoantibodies disappeared in one patient while persisting in the other.ConclusionWe report on two individuals presenting MCI associated with neurofascin 186 antibodies, thus expanding the potential spectrum of neurofascin 186-associated disease. This report supports the recommendation to consider also neurofascin 186 autoantibodies in not just peripheral nerve disease, but also in disorders involving CNS autoimmunity. More studies are needed to clarify the lack of association between neurofascin 186 autoantibodies and cognitive decline.

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“…Meanwhile, antibodies against NF186 were found in less than 3% of patients with AN [ 5 , 9 , 10 , 11 , 18 , 19 , 20 , 21 ]. Delmont et al reported that anti-NF186 IgG was related to a subset of AN patients with subacute-onset, sensory ataxia, demyelination with conduction block, cranial nerve involvement and concomitant autoimmune disorders.…”

Section: Introductionmentioning

confidence: 99%

Anti-NF155/NF186 IgG4 Antibody Positive Autoimmune Nodopathy

et al. 2022

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Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) seropositive for autoantibodies against nodal and paranodal proteins display distinct clinical presentations. In the latest study, CIDP with autoantibodies against paranodal proteins was defined as autoimmune nodopathy (AN). We herein present a case of 39-year-old male with anti- neurofascin (NF) 155 and NF186 IgG4 antibody with gait disturbance and tremor, who was followed up for 4 months and demonstrated clinical improvements after apparently effective rituximab therapy. In addition, a literature review was conducted to investigate the clinical characteristics of anti-NF155/NF186-positive AN.

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From PNS to CNS: characteristics of anti-neurofascin 186 neuropathy in 16 cases

Cited by 8 publications

References 34 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

Case report: Amnestic mild cognitive impairment in multiple domains associated with neurofascin 186 autoantibodies: Case series with follow-up and review

Anti-NF155/NF186 IgG4 Antibody Positive Autoimmune Nodopathy

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