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Neurology Jan 2021To test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurologic syndromes we retrospectively reviewed clinical, radiologic, and... (Observational Study)
Observational Study
OBJECTIVE
To test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurologic syndromes we retrospectively reviewed clinical, radiologic, and serologic features of 32 patients with concomitant paraneoplastic spinal cord and peripheral nervous system involvement.
METHODS
Observational study investigating patients with myeloneuropathy and underlying cancer or onconeural antibody seropositivity.
RESULTS
Among 32 patients with paraneoplastic myeloneuropathy, 20 (63%) were women with median age 61 years (range 27-84 years). Twenty-six patients (81%) had classified onconeural antibodies (amphiphysin, n = 8; antineuronal nuclear antibody [ANNA] type 1 [anti-Hu], n = 5; collapsin response mediator protein 5 [CRMP5] [anti-CV2], n = 6; Purkinje cell cytoplasmic antibody type 1 [PCA1] [anti-Yo], n = 1; Purkinje cell cytoplasmic antibody type 2 [PCA2], n = 2; kelch-like protein 11 [KLHL11], n = 1; and combinations thereof: ANNA1/CRMP5, n = 1; ANNA1/amphiphysin, n = 1; ANNA3/CRMP5, n = 1). Cancer was confirmed in 25 cases (onconeural antibodies, n = 19; unclassified antibodies, n = 3; no antibodies, n = 3). Paraneoplastic myeloneuropathies had asymmetric paresthesias (84%), neuropathic pain (78%), subacute onset (72%), sensory ataxia (69%), bladder dysfunction (69%), and unintentional weight loss >15 pounds (63%). Neurologic examination demonstrated concomitant distal or asymmetric hyporeflexia and hyperreflexia (81%), impaired vibration and proprioception (69%), Babinski response (68%), and asymmetric weakness (66%). MRI showed longitudinally extensive (45%), tract-specific spinal cord T2 hyperintensities (39%) and lumbar nerve root enhancement (38%). Ten of 28 (36%) were unable to ambulate independently at last follow-up (median 24 months, range 5-133 months). Combined oncologic and immunologic therapy had more favorable modified Rankin Scale scores at post-treatment follow-up compared to those receiving either oncologic or immunologic therapy alone (2 [range 1-4] vs 4 [range 2-6], < 0.001).
CONCLUSIONS
Paraneoplastic etiologies should be considered in the evaluation of subacute myeloneuropathies. Recognition of key characteristics of paraneoplastic myeloneuropathy may facilitate early tumor diagnosis and initiation of immunosuppressive treatment.
Topics: Adult; Aged; Aged, 80 and over; Autoantibodies; Female; HEK293 Cells; Humans; Male; Middle Aged; Paraneoplastic Polyneuropathy; Paraneoplastic Syndromes, Nervous System; Retrospective Studies
PubMed: 33208548
DOI: 10.1212/WNL.0000000000011218 -
The Pan African Medical Journal 2017Paraneoplastic dermatoses are a spectrum of cutaneous manifestations which may precede, coincide with or follow the diagnosis of cancer. Our study aims to remind...
Paraneoplastic dermatoses are a spectrum of cutaneous manifestations which may precede, coincide with or follow the diagnosis of cancer. Our study aims to remind clinicians that desquamative erythrodermia is a form of paraneoplastic dermatosis which may occur during hematologic malignancies. Hence the importance of a complete assessment to identify a neoplastic process in the presence of these clinical signs and especially when they are suspect.
Topics: Aged; Hematologic Neoplasms; Humans; Male; Paraneoplastic Syndromes; Skin Diseases
PubMed: 28748019
DOI: 10.11604/pamj.2017.27.18.12273 -
Journal of Investigative Medicine High... 2024Cutaneous paraneoplastic syndromes due to Hodgkin lymphoma present with a wide spectrum of clinical manifestations from generalized pruritus to exfoliative erythroderma....
Cutaneous paraneoplastic syndromes due to Hodgkin lymphoma present with a wide spectrum of clinical manifestations from generalized pruritus to exfoliative erythroderma. We summarize the clinical findings and outcomes of 14 patients with Hodgkin lymphoma and associated cutaneous paraneoplastic syndromes treated at Mayo Clinic over the past 3 decades. Cutaneous paraneoplastic syndromes may be present at the time of lymphoma diagnosis, whereas in other patients, it may appear at the time of relapse, including patients with initial absence of cutaneous manifestations during the initial lymphoma presentation. Our results indicate that complete resolution of the paraneoplastic syndrome is associated with significantly improved overall survival. Recognition of cutaneous paraneoplastic syndromes is a crucial surrogate of relapsed malignancy and treatment requires targeting the underlying malignancy.
Topics: Humans; Hodgkin Disease; Paraneoplastic Syndromes; Male; Female; Middle Aged; Adult; Aged; Young Adult; Skin Diseases; Adolescent
PubMed: 38767173
DOI: 10.1177/23247096241255840 -
The Israel Medical Association Journal... Oct 2018
Topics: Autoantibodies; Humans; Opsoclonus-Myoclonus Syndrome; Paraneoplastic Syndromes, Nervous System
PubMed: 30324785
DOI: No ID Found -
Journal of Thoracic Oncology : Official... Sep 2014Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these... (Review)
Review
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to take proper care of these patients.
Topics: Humans; Paraneoplastic Syndromes, Nervous System; Thymoma
PubMed: 25396312
DOI: 10.1097/JTO.0000000000000300 -
Annals of Neurology Jul 2016Neuroblastoma is a childhood cancer derived from cells of neural crest origin. The hallmarks of its enigmatic character include its propensity for spontaneous regression... (Review)
Review
Neuroblastoma is a childhood cancer derived from cells of neural crest origin. The hallmarks of its enigmatic character include its propensity for spontaneous regression under some circumstances and its association with paraneoplastic opsoclonus, myoclonus, and ataxia. The neurodevelopmental underpinnings of its origins may provide important clues for development of novel therapeutic and preventive agents for this frequently fatal malignancy and for the associated paraneoplastic syndromes. Ann Neurol 2016;80:13-23.
Topics: Disease Progression; Humans; Molecular Targeted Therapy; Neoplasm Regression, Spontaneous; Neural Crest; Neuroblastoma; Neurodevelopmental Disorders; Opsoclonus-Myoclonus Syndrome; Paraneoplastic Syndromes, Nervous System
PubMed: 27043043
DOI: 10.1002/ana.24659 -
Blood Mar 2022Neurologic complications of lymphoid cancer can be challenging to recognize and treat. The nervous system can be affected directly by hematogenous or local spread of... (Review)
Review
Neurologic complications of lymphoid cancer can be challenging to recognize and treat. The nervous system can be affected directly by hematogenous or local spread of lymphoma. Indirect neurologic effects of lymphoma include paraneoplastic syndromes and vascular complications. Lymphoma treatments can also cause neurologic complications. Early identification and treatment are crucial to stabilize or reverse neurologic deficits, prevent further nervous system injury, and optimize overall oncologic therapy. This article provides an overview of the different neurologic complications of lymphoma and its treatments, in addition to presenting case studies that emphasize commonly encountered clinical scenarios.
Topics: Humans; Lymphoma; Neoplasms; Nervous System Diseases; Paraneoplastic Syndromes
PubMed: 34479368
DOI: 10.1182/blood.2019003690 -
Italian Journal of Dermatology and... Apr 2021Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease,...
Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease, characterized by polymorphous clinical features, including mucocutaneous erosions, blisters, lichenoid papules, and erythemas. Several autoantibodies have been detected in serum of PAMS patients, including antiplakins, anti-alpha-2-macroglobulin like 1, and antidesmogleins autoantibodies. The mortality rate of PAMS is up to 90%. This is due on the one hand to the poor response to treatments and on the other hand to the delay in the diagnosis and to the prognosis of the underlying neoplasia.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Neoplasms; Paraneoplastic Syndromes; Pemphigus
PubMed: 33070576
DOI: 10.23736/S2784-8671.20.06675-4 -
Anais Brasileiros de Dermatologia 2018Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory...
Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory bowel diseases, vaccination, medications and neoplasms. Hematologic malignancies are the diseases most related to paraneoplastic Sweet's syndrome, but this clinical entity can also be found occasionally in some solid tumors, including genitourinary tract tumors. We report a rare case of paraneoplastic Sweet's syndrome associated with the diagnosis of cervical cancer.
Topics: Diagnosis, Differential; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Sweet Syndrome; Uterine Cervical Neoplasms
PubMed: 30066769
DOI: 10.1590/abd1806-4841.20187353 -
Journal For Immunotherapy of Cancer Mar 2024Patients with paraneoplastic syndromes (PNS) are excluded from clinical trials involving immune checkpoint inhibitors (ICIs) due to safety concerns. Moreover, real-world...
BACKGROUND
Patients with paraneoplastic syndromes (PNS) are excluded from clinical trials involving immune checkpoint inhibitors (ICIs) due to safety concerns. Moreover, real-world data on efficacy and safety is scarce.
METHODS
In this retrospective study, data were collected on patients with PNS and solid tumors receiving ICI between 2015 and 2022 at nine institutions. Patients were classified into: Cohort 1 (pre-existing PNS before ICI initiation), cohort 2 (PNS during ICI treatment), and cohort 3 (PNS after ICI discontinuation). Patients with metastatic non-small cell lung cancer (NSCLC) (mNSCLC) from cohort 1 were matched to patients who were PNS-free at each institution up to a 1:3 ratio for age, sex, type of ICI, use of concurrent chemotherapy, and number of lines of systemic therapy prior to ICI initiation. Kaplan-Meier method was used to assess overall survival (OS) and time-to-next treatment (TTNT).
RESULTS
Among 109 patients with PNS treated with ICIs, median age at ICI initiation was 67 years (IQR: 58-74). The most represented cancer type was NSCLC (n=39, 36%). In cohort 1 (n=55), PNS exacerbations occurred in 16 (29%) patients with median time to exacerbation after ICI of 1.1 months (IQR: 0.7-3.3). Exacerbation or de novo PNS prompted temporary/permanent interruption of ICIs in 14 (13%) patients. For cohort 2 (n=16), median time between ICI initiation and de novo PNS was 1.2 months (IQR: 0.4-3.5). Treatment-related adverse events (trAEs) occurred in 43 (39%) patients. Grade ≥3 trAEs occurred in 18 (17%) patients. PNS-directed immunosuppressive therapy was required in 55 (50%) patients. We matched 18 patients with mNSCLC and PNS (cohort 1) to 40 without PNS, treated with ICIs. There was no significant difference in OS or TTNT between patients with mNSCLC with and without PNS, although a trend was seen towards worse outcomes in patients with PNS. TrAEs occurred in 6/18 (33%) and 14/40 (35%), respectively. Grade ≥3 trAEs occurred in 4 (22%) patients with PNS and 7 (18%) patients without PNS.
CONCLUSIONS
Exacerbations of pre-existing PNS occurred in 29% of patients treated with ICIs and both exacerbations and de novo PNS occur early in the ICI course. TrAE from ICIs were similar between patients with and without PNS. Our data suggest that pre-existing PNS should not preclude consideration of ICI therapy although patients may not derive the same clinical benefit compared with patients without PNS.
Topics: Humans; Middle Aged; Aged; Immune Checkpoint Inhibitors; Carcinoma, Non-Small-Cell Lung; Retrospective Studies; Lung Neoplasms; Paraneoplastic Syndromes
PubMed: 38448038
DOI: 10.1136/jitc-2023-008724