Paraneoplastic Syndromes in Neuro-ophthalmology

Gordon, Lynn K.; Dinkin, Marc

doi:10.1212/con.0000000000000788

Cited by 15 publications

(16 citation statements)

References 100 publications

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“…Ophthalmic involvement including PON and cancer associated retinopathy is common in PNS, even presenting as the initial clinical manifestation of PNS in some cases with malignant tumors [ 1 – 4 , 7 – 8 ]. However, many other optic neuropathies should be excluded before PON was diagnosed in malignant tumor patients with optic nerve involved, which including ischemic anterior ON, papilloedema due to intracranial metastasis, MCON, ION, compressive ON, toxic or malnutritional ON, and demyelinating optic neuritis [ 1 – 4 , 6 – 8 ]. Therefore, early diagnosis of PON is challenging in clinical practice.…”

Section: Discussionmentioning

confidence: 99%

“…The PNS Euronetwork defined six antibodies (anti-Hu, Yo, CV2/CRMP-5, Ri, Ma2 and amphiphysin) as “well characterized onconeural antibodies”, and five antibodies (anti-Tr, ANNA3, PCA2, Zic4, and mGluR1) as “partially characterised onconeural antibodies”, which could confirm or support the diagnosis of PNS [ 1 – 3 , 10 – 16 ]. However, the seropositivity of paraneoplastic antibodies in PON patients is still under evaluation due to lack of enough volume of PON patients [ 1 – 4 , 8 , 10 – 16 ]. In our study, serum paraneoplastic antibodies were detected in only 44.4% (4/9) PON patients.…”

Section: Discussionmentioning

confidence: 99%

“…Optic neuropathy (ON) such as papilloedema secondary to intracranial metastasis is not uncommon in patients with malignant tumors, however, other optic neuropathies such as paraneoplastic optic neuropathy (PON), infiltrative optic neuropathy, and demyelinating optic neuritis, are rare in malignant tumor patients, and their early diagnosis is quite challenging in clinical practice [ 1 – 4 ].…”

Section: Introductionmentioning

confidence: 99%

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Serum and Cerebrospinal Fluid Testing in Optic Neuropathy Patients with Malignant Tumors

et al. 2022

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Purpose. To evaluate the value of serum and cerebrospinal fluid (CSF) testing in optic neuropathy (ON) patients with malignant tumors. Methods. Fourteen patients clinically diagnosed as ON with malignant tumors but without intracranial or orbital mass in MRI were included in this study. Detailed medical records including medical history, complete ophthalmic examination, colour fundus photography, visual field test, orbital MRI examination, serum and CSF testing data were collected and analyzed. The diagnosis of paraneoplastic optic neuropathy (PON) based on the 2004 recommended criteria of the paraneoplastic syndrome- Euronetwork consortium for paraneoplastic neurological disorders, and current adaption for neuropathies. All patients underwent serum tests for pathogens and autoantibodies including antinuclear antibodies, anticardiolipin antibodies, antineutrophil cytoplasmic antibodies, AQP4-Ab and MOG-Ab, as well as CSF tests for malignant cells under microscope. Serum paraneoplastic antibodies were detected in PON patients. Monkey cerebellar tissue-based assay was used to detect unknown serum anti-neuron antibodies in PON patients with negative paraneoplastic antibody testing results. Results. Fourteen ON patients were classified as four groups based on their clinical and MRI characteristics, as well as serum and CSF testing results: [1] definite PON, 6 cases (11 eyes); [2] possible PON, 3 case (5 eyes); [3] meningeal carcinomatosis-associated optic neuropathy (MCON), 4 cases (6 eyes); [4] infiltrative optic neuropathy (ION), 2 cases (2 eyes). Malignant cells were found under microscope in CSF samples from MCON and ION patients, contrast to no malignant cells in CSF samples from PON cases. All 14 ON patients with malignant tumors showed negative results in serum tests for pathogens and autoantibodies. Serum paraneoplastic antibodies were tested in PON patients, anti- CV2, anti-Yo, and anti- amphiphysin were detected positive in 2, 1, and 1 case, respectively, in definite PON group, whereas no serum paraneoplastic antibody detected in possible PON group. Two unknown serum antineuronal antibodies (an anti- Purkinje cell antibody and an anti-granular cell antibody) were detected using monkey cerebellar tissue-based assay in 2 of 5 PON patients with negative paraneoplastic antibody test results. Conclusions. Serum and CSF tests are of great importance in differentiating different subtypes of ON with malignant tumors. Current diagnosis of PON still depends on combination of clinical and MRI manifestations, as well as serum and CSF tests. Tissue-based assay may help to detect new biomarkers for ON etiology and diagnosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Serum and Cerebrospinal Fluid Testing in Optic Neuropathy Patients with Malignant Tumors

et al. 2022

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“…The data were collected by medical staff/doctoral students with study/research grants from the European project on paraneoplastic neurological syndromes. On the basis of the criteria of the Paraneoplastic Neurologic Syndrome Euronetwork guidelines [ 5 ], PNS patients were identified according to clinical presentation with one of the following neurological syndromes: cerebellar degeneration [ 21 ]; encephalomyelitis, or limbic encephalitis [ 22 ]; polyneuropathy [ 23 ]; retinal degeneration [ 24 ]; motor neuron disease [ 25 ]; opsoclonus-myoclonus [ 26 ]; peripheral nervous disorders, including sensory neuro-/neuronopathy, dysautonomia and neuromyotony [ 27 ]; and Lambert-Eaton myasthenia syndrome, polymyositis and necrotizing myelopathy [ 28 , 29 ]. …”

Section: Methodsmentioning

confidence: 99%

Paraneoplastic Neurological Syndromes: Study of Prevalence in a Province of the Lombardy Region, Italy

Lorusso¹,

Precone²,

Ferrari³

et al. 2020

JCM

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Paraneoplastic neurological syndromes (PNSs) are a heterogeneous group of rare immune-mediated diseases associated with cancer. The aim of this study was to investigate the prevalence of PNSs in the province of Brescia. PNS prevalence was calculated using the Lombardy regional hospital admission records from 1998 to 2003. We used the website “Epidemiologic and Economic Atlas of Hospital Activities in Lombardy” and the “International Statistical Classification of Diseases and Related Health Problems”. In the province of Brescia, we found 54 cases of PNSs, 29 with subacute neuropathies, five with paraneoplastic cerebellar degeneration and 20 with encephalomyelitis. Peripheral nervous system diseases were the most frequent neurological disorders. In Lombardy, the number of PNS patients admitted was 322 (133 with encephalomyelitis, 21 with paraneoplastic cerebellar degeneration, 166 with polyneuropathies and two with optic degeneration). In Lombardy, the prevalence of PNSs was 25 in 100,000 hospital admissions and 5.92 in 100,000 for the Lombardy population. Our results show a discrete presence of PNS patients in the province of Brescia and in the Lombardy region as a whole.

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“…Management of postinfectious and autoimmune etiologies of OMS includes immunosuppressive therapies such as steroids, intravenous immunoglobulin, or plasma exchange. In paraneoplastic OMS, tumor-directed treatment is the primary modality with the aforementioned immunosuppressive therapies used as an adjunct (4).…”

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confidence: 99%

Opsoclonus in Uremia With Resolution After Hemodialysis

Changa¹,

Rucker²,

Drummond

2021

Journal of Neuro-Ophthalmology

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A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus and spontaneous opsoclonus. He had no known risk factors for the development of opsoclonus and upon undergoing hemodialysis, experienced near resolution of his eye movement abnormalities, thus highlighting a possible link between the uremic state and opsoclonus.

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Paraneoplastic Syndromes in Neuro-ophthalmology

Cited by 15 publications

References 100 publications

Serum and Cerebrospinal Fluid Testing in Optic Neuropathy Patients with Malignant Tumors

Serum and Cerebrospinal Fluid Testing in Optic Neuropathy Patients with Malignant Tumors

Paraneoplastic Neurological Syndromes: Study of Prevalence in a Province of the Lombardy Region, Italy

Opsoclonus in Uremia With Resolution After Hemodialysis

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