CRMP-5

BACKGROUND: The diagnosis and treatment of autoimmune optic neuritis (ON) has improved with the accessibility and reliability of aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing, yet autoantibody-negative ON remains common. This study describes the demographic, clinical, and outcome data in patients with isolated ON across the pediatric and adult cohort. METHODS: A retrospective chart review of University of Utah Health patients with the International Classification of Diseases (ICD) code of ICD-9 377...

BACKGROUND AND OBJECTIVES: Pathologic descriptions of peripheral nerve involvement in paraneoplastic neuropathies are sparse, mostly from autopsies focusing on CNS and dorsal root ganglia tissues. Here, we describe the clinicopathologic features of peripheral nerve biopsies in patients with paraneoplastic neurologic syndromes to expand the currently limited knowledge. METHODS: Retrospective review of the Mayo Clinic electronic medical record from 1995 to 2022 for patients identified to have subacute onset neuropathy with paraneoplastic antibodies identified in our neuroimmunology laboratory having available nerve biopsies performed at the time of diagnosis...

A 68-year-old man with small-cell lung cancer developed anti-collapsin response-mediator protein (CRMP)-5 antibody-related paraneoplastic neurological syndrome (PNS) presenting with ataxia and chorea during treatment with durvalumab. As a result of steroid therapy, anti-CRMP-5 antibodies became negative, hyperintense lesions on brain magnetic resonance imaging disappeared, and neurological symptoms improved. After resuming durvalumab, he became unable to walk due to neurological adverse events (nAEs). There have been no reported cases manifesting PNSs and nAEs as a result of the same immune checkpoint inhibitors (ICIs) administered at different times...

Limbic encephalitis is a well-defined clinical disorder among paraneoplastic neurological syndromes. Although it is not always possible to identify specific autoantibodies in limbic encephalitis, the presence of anti-neuronal nuclear antibody type 1 (ANNA1 or anti-Hu), anti-Ma2, collapsin response mediator protein 5 (CRMP-5-IgG or anti-CV2), anti-GABAB  receptors and anti-amphiphysin antibodies are often detected. A 66-year-old male patient with complaints of forgetfulness was evaluated in our clinic after having seizures...

Thymomas are associated with autoimmune disease, most commonly myasthenia gravis, and rarely with autoimmune encephalitis. More recently, viral triggers including COVID-19 have also been implicated in autoimmunity. We present a case of antibody-positive autoimmune encephalitis that developed in the setting of COVID-19 in a patient with thymomatous myasthenia gravis.

Optic neuritis with CRMP-5 IgG is a paraneoplastic inflammation of the optic nerve associated with lung cancer, mostly small-cell lung cancer. We present the case of a patient with lung adenocarcinoma who developed progressive bilateral visual loss a few months after immune-chemotherapy with pembrolizumab and after Covid-19 vaccination. Positive CRMP-5 IgG were detected in blood sample and complete work-up - including brain MRI - did not show any progression. High dose systemic corticoids were administered with transient improving, followed by intravenous immunoglobulins, methotrexate and rituximab but despite negativization of CRMP-5 IgG, the patient had a progressive visual loss...

Autoimmune chorea syndromes represent a vast array of paraneoplastic, parainfectious and idiopathic disorders. It is increasingly apparent that familiarity with these disorders is critically important, as they may be treatable or may be part of a syndrome requiring further work-up and monitoring. These disorders are mediated by an aberrant immunologic attack with resultant neuronal dysfunction, manifesting as chorea. These conditions are typically accompanied by other neurologic or systemic manifestations. In this review we outline the clinical features, epidemiologic factors, and delineate the specific antibodies associated with each of these autoimmune mediated disorders...

OBJECTIVES: Population-based epidemiologic data for paraneoplastic neurologic syndromes (PNSs) in the United States are lacking. Our objective was to evaluate the incidence, prevalence, and associated morbidity of PNS. METHODS: We performed a population-based epidemiology study in Olmsted County, Minnesota, with patients identified between January 1, 1987, and December 31, 2018, using the medical records linkage system of the Rochester Epidemiology Project (REP) who met the definite/probable 2021 PNS criteria and 2004 PNS criteria...

Paraneoplastic neurological syndrome (PNS) is a heterogeneous group of neurological disorders caused by immune-mediated inflammatory mechanisms. We herein report a 77-year-old man with CV2/CRMP5-antibody-related PNS associated with a gastrointestinal stromal tumor (GIST). He was admitted for forgetfulness and delusional behavior. His neurological symptoms were subacute, and a whole-body examination revealed a gastric GIST. Serology showed CV2/collapsin response mediator protein (CRMP)-5 antibodies. Partial gastrectomy was performed for the GIST, and the neurological symptoms and serum CV2/CRMP5 antibodies disappeared...

We describe an uncommon cause of paraneoplastic optic neuropathy in adenocarcinoma of the lung. A 45-year-old healthy woman presented with seizure and encephalitis, followed by an acute visual loss in both eyes for 1 week. Her visual acuity was no perception of light in the right eye and hand movement in the left eye. There was a generalised restriction of extraocular muscle movements in both eyes. Funduscopy showed a bilateral pale optic disc. A paraneoplastic antigen autoimmune profile showed a positive anti-CV2/CRMP-5 antibody...

PURPOSE OF REVIEW: This article reviews the current classification system of primary spinal cord tumors and explores evolving diagnostic and therapeutic strategies for both primary tumors and metastatic tumors to various compartments of the spinal cord. RECENT FINDINGS: The 2016 World Health Organization classification system allows for more precise prognostication of and therapy for spinal cord tumors and has identified new entities, such as the diffuse midline glioma, H3 K27M mutant...

We report the case of a lady who presented with 3 weeks of visual floaters and optic disc swelling. Subsequent investigations revealed deep white matter changes on brain imaging, and enlarged mediastinal nodes. The presence of anti-CRMP-5 antibodies finally led to the diagnosis of a paraneoplastic syndrome, and mediastinal lymph node biopsy confirmed the diagnosis of small-cell lung cancer. The learning points from this case include that optic neuritis can be the only presenting feature of a paraneoplastic neurological syndrome, and the usefulness of anti-neuronal antibody measurement as a diagnostic marker of an underlying paraneoplastic disease process...

Patients with anti-CV2/collapsin response mediator protein (CRMP)5 antibodies present with more frequent chorea, cerebellar ataxia, uveo/retinal symptoms, and Lambert-Eaton myasthenic syndrome or myasthenia gravis. Chronic intestinal pseudo-obstruction (CIPO) is an intestinal motility dysfunction disease dysmotility that is caused by a neuromuscular disease with recurrent or persistent intestinal obstruction in the absence of mechanical obstruction. We report the case of a patient with CRMP5 antibody-positive paraneoplastic neurological syndrome (PNS) that is associated with autonomic dysfunction (presenting most remarkably as CIPO)...

Optic neuritis (ON) is a common manifestation of central nervous system demyelinating disorders such as multiple sclerosis (MS). The last two decades have seen increasing recognition of atypical optic neuritis syndromes, driven in large part by characterization of demyelinating diseases associated with antibodies to aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG). Given their increased importance in the workup of ON, familiarity with serological tests for ON has become essential for ophthalmologists...

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a rare neurological condition with many associated risk factors. The presentation varies and consists of seizures, impaired visual acuity or visual field deficits, disorders of consciousness, headaches, confusion and focal neurological deficits. The diagnosis relies on clinical presentation and MRI findings. Treatment and prognosis are related to the underlying etiology. CASE PRESENTATION: We present a 58-year-old woman with ovarian cancer who developed symptoms and radiologic signs of PRES with no apparent trigger other than a sudden increase in blood pressure for the first time in her life and before any treatment has begun...

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Autoimmunity against collapsin response-mediator protein-5 (anti-CRMP-5) has been associated with ocular inflammation in paraneoplastic syndrome. We present a 59-year-old Caucasian man with optic neuritis and vitreous cells in both eyes (OU), at different stages. Despite the fact that the patient did not have any systemic disease, we suspected a paraneoplastic syndrome and requested CRMP-5-IgG and a mediastinoscopy. After performing the tests, a small cell lung carcinoma was diagnosed. Autoantibody CRMP-5-IgG positivity and optic neuritis combined with vitreous inflammation was defined as a paraneoplastic entity, avoiding vitreous biopsy and allowing us to suspect malignancy before systemic symptoms appeared...

CRMP-5-associated GTPase (CRAG), a short splicing variant of centaurin-γ3/AGAP3, is predominantly expressed in the developing brain. We previously demonstrated that CRAG, but not centaurin-γ3, translocates to the nucleus and activates the serum response factor (SRF)-c-Fos pathway in cultured neuronal cells. However, the physiological relevance of CRAG in vivo is unknown. Here, we found that CRAG/centaurin-γ3-knockout mice showed intensively suppressed kainic acid-induced c-fos expression in the hippocampus...

PURPOSE: To explore the presence of serum anti-retinal antibodies (ARAs) in the Chinese patients with presumed autoimmune retinopathy (AIR). METHODS: Twenty-three Chinese patients with presumed AIR, disease controls including 40 RP patients, 22 bilateral uveitis patients, 18 acute zonal outer occult retinopathy (AZOOR) patients, and 30 healthy donors were included. Serum samples of all the subjects were obtained and analyzed for the presence of four ARAs including recoverin, α-enolase, carbonic anhydraseII (CAII), and collapsin response-mediated protein (CRMP)-5 by Western bolt assay...

Anticollapsin-responsive mediator protein 5 (CRMP-5) IgG is an antibody generally associated with small-cell lung cancer, which is known to cause paraneoplastic neurological syndromes, including encephalitis, myelitis and neuropathy. HIV escape is a phenomenon in which a patient with low or undetectable levels of HIV RNA in plasma is found to have elevated levels in cerebrospinal fluid (CSF). We present a case of a 58-year-old HIV-positive woman with undetectable plasma viral load who developed a subacute flaccid paraparesis...