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JCI Insight 2019 Apr 4;4(7). Epub 2019 Apr 4.

Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, Toulouse, France.

Paraneoplastic neurological disorders result from an autoimmune response against neural self-antigens that are ectopically expressed in neoplastic cells. In paraneoplastic disorders associated to autoantibodies against intracellular proteins, such as paraneoplastic cerebellar degeneration (PCD), current data point to a major role of cell-mediated immunity. In an animal model, in which a neo-self-antigen was expressed in both Purkinje neurons and implanted breast tumor cells, immune checkpoint blockade led to complete tumor control at the expense of cerebellum infiltration by T cells and Purkinje neuron loss, thereby mimicking PCD. Read More

Neurol Sci 2019 Mar 29. Epub 2019 Mar 29.

Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy.

The diagnosis of sporadic adult onset ataxia is a challenging task since a large collection of hereditary and non-hereditary disorders should be taken into consideration. Sporadic adult onset ataxias include degenerative non-hereditary, hereditary, and acquired ataxias. Multiple system atrophy and idiopathic late cerebellar ataxia are degenerative non-hereditary ataxias. Read More

Turk Pediatri Ars 2018 Dec 1;53(4):263-266. Epub 2018 Dec 1.

Pediatric Hematology and Oncology Clinic, Kanuni Sultan Süleyman Training and Research Hospital, İstanbul, Turkey.

Lymphomas are solid tumors characterized by the malignant proliferation of lymphoid cells. Neurologic signs encountered in patients with Hodgkin's lymphoma can be due to the direct spread of tumor to the nervous system, secondary to chemotherapy or radiation, secondary to tumor mass compression, infectious causes and paraneoplastic syndromes. Paraneoplastic neurologic syndromes are rarely encountered in patients with Hodgkin's lymphoma and non-Hodgkin's lymphoma. Read More

Eur J Case Rep Intern Med 2018 21;5(3):000780. Epub 2018 Mar 21.

Department of Internal Medicine, Villarrobledo General Hospital, Villarrobledo, Albacete, Spain.

We present the case of a 57-year-old man with ataxia and clinical and radiological features of cerebellar degeneration. Computed tomography showed a mediastinal mass and the patient was diagnosed with thymic carcinoma. Paraneoplastic cerebellar degeneration is aninfrequent disorder and its association with thymic carcinoma very rare. Read More

Brain Nerve 2019 Feb;71(2):167-174

Department of Neurology and Strokology, Nagasaki University Hospital.

A 63-year-old female who developed dizziness, diplopia and subsequent gait disturbance from September X-1 year was analyzed. The first neurological findings in May X year revealed cerebellar ataxia, weakness in the proximal limbs, decreased tendon reflexes, and autonomic symptoms (ADL:mRS 3). Furthermore, an incremental phenomenon was observed in the repetitive nerve stimulation test, and she was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) based on the serum P/Q-type calcium channel (VGCC) antibody positivity. Read More

Methods Mol Biol 2019 ;1941:225-255

Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.

Immune-mediated inflammation of the brain has been recognized for more than 50 years, although the initial descriptions were mainly thought to be secondary to an underlying neoplasm. Some of these paraneoplastic encephalitides express serum antibodies, but these were not thought to be pathogenic but instead have a T-cell-mediated pathophysiology. Over the last two decades, several pathogenic antibodies against neuronal surface antigens have been described in autoimmune encephalitis, which are amenable to immunotherapy. Read More

Curr Oncol 2018 12 1;25(6):e585-e591. Epub 2018 Dec 1.

Department of Medicine, Division of Medical Oncology, University of Ottawa, Ottawa, ON.

Breast cancer is the most common cancer in women, with 15%-25% of those tumours overexpressing the human epidermal growth factor receptor 2 (her2), which is associated with more aggressive disease. On rare occasions, patients present with a paraneoplastic syndrome months to years before their cancer diagnosis. Paraneoplastic cerebellar degeneration (pcd) is associated with fewer than 1% of cancers and is strongly associated with breast and gynecologic malignancies. Read More

Cerebellum 2018 Dec 18. Epub 2018 Dec 18.

Division of Movement Disorders, Department of Neurology, Yale School of Medicine, Yale University, New Haven, CT, USA.

Tremor is an involuntary, rhythmic, oscillatory movement of a body part. It is a central feature of a range of diseases resulting from pathological changes in the cerebellum. Interestingly, in modern times, the terms "cerebellar tremor" and "intention tremor" are often used synonymously and interchangeably. Read More

Ann Indian Acad Neurol 2018 Oct-Dec;21(4):311-314

Department of Neurosciences, Institute of Neurosciences/Internal Medicine, Indraprastha Apollo Hospitals, New Delhi, India.

Paraneoplastic syndromes are a rare heterogeneous group of disorders that are indicators of the underlying occult malignancy. Subacute cerebellar ataxia in a patient with a known cancer is often due to metastatic invasion or other complications of the cancer, such as infection, coagulopathy, metabolic and nutritional deficits, or side effects of treatment. When tumor- and treatment-related causes have been excluded, the patient is considered to suffer from paraneoplastic cerebellar degeneration (PCD). Read More

J Neurol Sci 2019 01 8;396:184-186. Epub 2018 Nov 8.

Institute of General Pathology, University Hospitals of Modena, Italy.

J Neuroimmunol 2019 Jan 8;326:14-18. Epub 2018 Nov 8.

Department of Pathology, Nottingham University Hospitals NHS Trust, Queen's Medical Centre, Nottingham NG7 2UH, UK.

Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls. SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC. Read More

Curr Oncol Rep 2018 Nov 10;20(11):92. Epub 2018 Nov 10.

Neurology Division, Hospital das Clínicas, University of São Paulo School of Medicine, Sao Paulo, Brazil.

The disorders of the central nervous system associated with cancer by remote immune-mediated mechanisms are a heterogeneous group. These disorders encompass the classic paraneoplastic disorders and the recently recognized autoimmune encephalitis associated with antibodies against neuronal cell surface or synaptic proteins that occur with or without cancer association. In the last decade, the new surge of interest in neuronal diseases associated with anti-neuronal antibodies led to the rapid discovery of new forms of disease that have different manifestations and were not previously suspected to be immune mediated. Read More

Neurology 2018 Nov;91(19):e1831-e1833

From the Departments of Neurology (S.-U.L., J.-Y.C., J.-S.K.) and Laboratory Medicine (E.Y.S.), Seoul National University College of Medicine; Dizziness Center, Clinical Neuroscience Center, and Department of Neurology (S.-U.L., H.-J.K., S.-W.O., J.-Y.C., J.-S.K.), and Research Administration Team (H.-J.K.), Seoul National University Bundang Hospital, Seongnam; and Department of Laboratory Medicine (E.Y.S.), Seoul National University Hospital, South Korea.

Gan To Kagaku Ryoho 2018 Oct;45(10):1510-1512

Dept. of Gastrointestinal Tract Surgery, Fukushima Medical University.

A case complicated with colorectal and prostate cancers in paraneoplastic(subacute)cerebellar degeneration(PCD)is extremely rare. We report a retrospective case of rectal carcinoma with paraneoplastic cerebellar degeneration. A 79-year-old man with Parkinson's disease was unable to walk because of paralysis. Read More

Cerebellum 2019 Apr;18(2):245-254

French Reference Center for Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, F-69677, Bron, France.

To describe autoantibodies (Abs) against tripartite motif-containing (TRIM) protein 9 and 67 in two patients with paraneoplastic cerebellar degeneration (PCD) associated with lung adenocarcinoma. Abs were characterized using immunohistochemistry, Western blotting, cultures of murine cortical, and hippocampal neurons, immunoprecipitation, mass spectrometry, knockout mice for Trim9 and 67, and cell-based assay. Control samples included sera from 63 patients with small cell lung cancer without any paraneoplastic neurological syndrome, 36 patients with lung adenocarcinoma and PNS, CSF from 100 patients with autoimmune encephalitis, and CSF from 165 patients with neurodegenerative diseases. Read More

Child Neurol Open 2018 1;5:2329048X18795546. Epub 2018 Oct 1.

National Pediatric Myoclonus Center and National Pediatric Neuroinflammation Organization, Inc., Orlando, FL, USA.

Paraneoplastic cerebellar degeneration is rare and noteworthy in children. In this 7-year-old, it was documented to have occurred within a year of ataxia presentation. The instigating cancer was stage III adrenal adenocarcinoma, remitted after surgical resection at age 2. Read More

Radiologe 2018 Dec;58(12):1080-1090

Abteilung für Neuroradiologie, Kantonsspital Baden, Baden, Schweiz.

Clinical Issue: Autoimmune disorders of the central nervous system (CNS) are common but are also a heterogeneous group of diseases. The most common form is multiple sclerosis (MS), others are clinically isolated syndrome (CIS), acute demyelinating encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD). Paraneoplastic syndromes are rare and tumor-associated, they are not induced by direct invasion of tumor tissue but by tumor-associated autoantibodies mostly against specific CNS proteins, e. Read More

Mayo Clin Proc Innov Qual Outcomes 2018 Mar 1;2(1):74-77. Epub 2018 Feb 1.

Division of Hematology and Oncology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL.

Immune-mediated encephalitis related to immune checkpoint inhibitor therapy is a rare but increasingly described condition that can cause significant morbidity. There are several reported cases in the literature but no previously described cases of immune-mediated cerebellitis. We describe a case of acute cerebellitis that developed in a 20-year-old man with primary refractory Hodgkin lymphoma being treated with the immune checkpoint inhibitor nivolumab. Read More

Curr Neuropharmacol 2019 ;17(1):33-58

University of Washington, School of Medicine, Seattle, WA 98109, United States.

Immune-mediated cerebellar ataxias (IMCAs), a clinical entity reported for the first time in the 1980s, include gluten ataxia (GA), paraneoplastic cerebellar degenerations (PCDs), antiglutamate decarboxylase 65 (GAD) antibody-associated cerebellar ataxia, post-infectious cerebellitis, and opsoclonus myoclonus syndrome (OMS). These IMCAs share common features with regard to therapeutic approaches. When certain factors trigger immune processes, elimination of the antigen( s) becomes a priority: e. Read More

Intern Med 2019 Feb 12;58(4):619-620. Epub 2018 Sep 12.

Department of Neurology, Kitano Hospital, The Tazuke Kofukai Medical Research Institute, Japan.

BMJ Case Rep 2018 Aug 27;2018. Epub 2018 Aug 27.

Department of Surgery, All India Institute of Medical Sciences, New Delhi, India.

Carcinoma breast presenting with paraneoplastic cerebellar degeneration is a rare scenario. We report a case of a 52-year-old woman, which is a follow-up case of completely treated carcinoma breast presenting with paraneoplastic cerebellar degeneration which, on investigation, revealed metastatic disease with recurrence at previous scar site and metastasis to contralateral axilla. The patient was given pulse methyl prednisolone therapy and underwent wide local excision of nodule and right axillary lymph node dissection with 14 cycles of trastuzumab and paclitaxel as adjuvant therapy. Read More

Mol Clin Oncol 2018 Aug 25;9(2):163-167. Epub 2018 May 25.

Department of Neurology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu 210029, P.R. China.

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication of cancer characterized by rapid development of cerebellar ataxia. We herein present a case of a 67-year-old female patient with PCD caused by breast cancer. The patient presented with progressively worsening cerebellar deficits that had been misdiagnosed for several months prior to the identification of the anti-Yo autoantibodies in the serum. Read More

Noro Psikiyatr Ars 2018 4;55(2):189-194. Epub 2018 May 4.

Department of Neurology, Istanbul University, Faculty of Medicine, İstanbul, Turkey.

Introduction: Anti-neuronal antibodies (ANA) are found in paraneoplastic neurological syndrome and autoimmune encephalitis patients. Our aim was to analyze prognostic factors related with ANA seropositivity.

Methods: Twenty-seven consecutive ANA seropositive patients were included in the study. Read More

BMJ Case Rep 2018 Jun 21;2018. Epub 2018 Jun 21.

Department of Neurology, G B Pant Institute of Post Graduate Medical Education and Research, New Delhi, India.

Paraneoplastic cerebellar degeneration is a rare dysfunction of the cerebellum associated with malignancy. Nevertheless, it is the most common paraneoplastic syndrome affecting the brain. A 50-year-old woman presented to the neurology outpatient department (OPD) with symptoms of cerebellar dysfunction since 4 months and complaints of a painless lump in the right breast and drooling from mouth since 1 month. Read More

BMJ Case Rep 2018 Jun 15;2018. Epub 2018 Jun 15.

Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK.

A 31-year-old man presented with a subacute cerebellar syndrome of unknown aetiology. Investigations including a paraneoplastic antibody screen were negative and a working diagnosis of possible vasculitis was concluded. After 1 month, he re-presented with worsening of his symptoms and a neck lump. Read More

Handb Clin Neurol 2018 ;154:129-149

Research, Neurology, and Pathology Services, Veterans Affairs Medical Center and Departments of Neurology and Pathology, Albany Medical College, Albany, NY, United States. Electronic address:

This chapter summarizes the neuropathologic features of nonneoplastic disorders of the adult cerebellum. Gait ataxia and extremity dysmetria are clinical manifestations of diseases that interrupt the complex cerebellar circuitry between the neurons of the cerebellar cortex, the cerebellar nuclei (especially the dentate nuclei), and the inferior olivary nuclei. The cerebellum is a prominent target of several sporadic and hereditary neurodegenerative diseases, including multiple system atrophy, spinocerebellar ataxia, and Friedreich ataxia. Read More

Medicine (Baltimore) 2018 Jun;97(24):e10717

West China Hospital, Sichuan University, Chengdu, P.R. China.

Rationale: Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological deficit affecting the cerebellum. Anti-Yo antibody positive PCD is a rare occurrence most likely associated with gynecologic or breast malignancies. The identification of the underlying tumor is a diagnostic challenge in many of these patients. Read More

Br J Cancer 2018 Jul 14;119(1):105-113. Epub 2018 Jun 14.

Brain and Spine Institute (ICM), Experimental Neuro-Oncology Department, Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06, 75013, Paris, France.

Background: Paraneoplastic neurological syndromes are rare conditions where an autoimmune reaction against the nervous system appears in patients suffering from a tumour, but not linked to the spreading of the tumour. A break in the immune tolerance is thought to be the trigger.

Methods: The transcriptomic profile of 12 ovarian tumours (OT) from patients suffering from paraneoplastic cerebellar degeneration (PCD) linked to anti-Yo antibodies (anti-Yo PCD OT) was compared with 733 ovarian tumours (OT control) from different public databases using linear model analysis. Read More

J Fr Ophtalmol 2018 Jun 9;41(6):554-559. Epub 2018 Jun 9.

Université Rennes, INSERM, INRA, CHU de Rennes, Institut NUMECAN (Nutrition Metabolisms and Cancer), 35000 Rennes, France. Electronic address:

Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Although these syndromes are rare, the clinical manifestations can herald an unsuspected, underlying malignancy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Read More

Handb Clin Neurol 2018 ;155:313-332

French Reference Centre for Paraneoplastic Neurological Syndromes, Lyon Neurological Hospital, Lyon, France; Institut NeuroMyoGene, Université Claude Bernard Lyon 1, Lyon, France. Electronic address:

Immune-mediated cerebellar ataxia (CA) comprises a group of rare diseases that are still incompletely described, and are probably underdiagnosed. Both acute and progressive progressions are possible. Different syndromes have been identified, including CA associated with anti-GAD antibodies, the cerebellar type of Hashimoto encephalopathy, primary autoimmune CA, gluten ataxia, opsoclonus-myoclonus syndrome, and paraneoplastic cerebellar degenerations. Read More

Oncotarget 2018 May 8;9(35):23975-23986. Epub 2018 May 8.

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

Cerebellar degeneration related protein 1 (CDR1) is expressed in the cerebellum, and CDR1 antibodies have been associated with paraneoplastic cerebellar degeneration (PCD). In this study, we examined CDR1 expression in cerebellum and in ovarian and breast tumors, as well as the intracellular localization of CDR1 in cancer cells in culture. CDR1 was strongly expressed in the cytosol and dendrites of Purkinje cells and in interneurons of the molecular layer in cerebellum. Read More

J Fr Ophtalmol 2018 May 18;41(5):e181-e185. Epub 2018 May 18.

Ophthalmology Service, CHU de Rennes, 35033 Rennes cedex, France. Electronic address:

Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Read More

Lung India 2018 May-Jun;35(3):245-247

Department of Internal Medicine, Jamaica Hospital Medical Center, Jamaica, NY 11418, USA.

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor and several paraneoplastic syndromes have been related to it. We report the case of a 60-year-old male initially admitted to rule out cerebral vascular accident with the final diagnosis of SFT associated with paraneoplastic cerebellar degeneration and hypoglycemia. The diagnosis was confirmed by computed tomography-guided lung biopsy. Read More

Neuropathol Appl Neurobiol 2019 Feb 24;45(2):141-156. Epub 2018 May 24.

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

Aim: Neurodegeneration is associated with dysfunction of calcium buffering capacity and thereby sustained cellular and mitochondrial calcium overload. Paraneoplastic cerebellar degeneration (PCD), characterized by progressive Purkinje neurone degeneration following paraneoplastic Yo antibody internalization and binding to cerebellar degeneration-related protein CDR2 and CDR2L, has been linked to intracellular calcium homeostasis imbalance due to calbindin D malfunction. Therefore, we hypothesized that Yo antibody internalization affects not only calbindin calcium binding capacity, but also calcium-sensitive mitochondrial-associated signalling, causing mitochondrial calcium overload and thereby Purkinje neurone death. Read More

Brain Nerve 2018 Apr;70(4):371-382

Tokyo Medical University Hachioji Medical Center, Department of Neurology, Tokyo Medical University Hachioji Medical Center.

The cerebellum is one of the main targets in the central nervous system for autoimmunity. Immune-mediated cerebellar ataxias include gluten ataxia, GAD antibody-associated cerebellar ataxia, Hashimoto's encephalopathy, and paraneoplastic cerebellar degeneration. Autoimmune cerebellar ataxia may be of either insidious or subacute onset, and vertigo or transient neurological symptoms occur in some patients before the onset of the disease, in contrast to spinocerebellar degeneration. Read More

Brain Nerve 2018 Apr;70(4):341-355

Department of Neurology and Strokology, Nagasaki University Hospital.

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction. Approximately 50-60% of patients with LEMS have a tumor, most often small cell lung cancer (SCLC), making LEMS a paraneoplastic neurological syndrome. In Japan, the clinical picture is a male: female ratio of 3:1; mean age, 62 years (17-80 years); and 61% of LEMS patients have SCLC (SCLC-LEMS), with the remainder of patients having no cancer. Read More

Rev Neurosci 2018 09;29(7):745-755

Department of Neurochemistry and Neuropathology, Poznan University of Medical Sciences, 49, Przybyszewskiego Str., 60355 Poznan, Poland.

Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Read More

Cerebellum 2018 Aug;17(4):387-391

School of Medicine, University of Washington, Seattle, WA, 98109, USA.

The cerebellum characteristically has the capacity to compensate for and restore lost functions. These compensatory/restorative properties are explained by an abundant synaptic plasticity and the convergence of multimodal central and peripheral signals. In addition, extra-cerebellar structures contribute also to the recovery after a cerebellar injury. Read More

Wien Med Wochenschr 2018 May 6;168(7-8):193-198. Epub 2018 Feb 6.

Krankenhaus Agatharied GmbH, Norbert Kerkel Platz, Sankt-Agathastraße 1, 83734, Hausham, Deutschland.

Paraneoplastic neurological syndromes describe a very heterogeneous group of neurological disorders. Paraneoplastic cerebellar degeneration is frequently associated with gynecological tumors, lung cancer and Hodgkin lymphoma. The following case report shows the typical course of this disease as well as the diagnostic and therapeutic challenges. Read More

Rinsho Shinkeigaku 2018 Feb 31;58(2):83-87. Epub 2018 Jan 31.

Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University.

In this independent clinical study, we analyzed retrospectively the clinical features of 9 cases (6 male and 3 female) of Lambert-Eaton myasthenic syndrome that were administered 3,4-diaminopyridine (3,4-DAP). Four cases showed no cancer and 5 cases had small cell lung carcinoma. Seven cases were positive for anti voltage-gated calcium channel antibodies. Read More

World Neurosurg 2018 Apr 10;112:117-122. Epub 2018 Feb 10.

Universidad Icesi, Faculty of Medicine, Cali, Colombia; Clinical Research Centre, Cali, Colombia; Department of Neuroscience, Fundación Valle del Lili Hospital, Cali, Colombia. Electronic address:

Background: Paraneoplastic cerebellar degeneration (PCD) is a rare complication of some malignant cancers. It is most commonly described in women with gynecologic or breast malignancies; however, there have been reports in other types of cancers. Symptoms include ataxia, dysarthria, and tremors, which could be the first manifestations of an underlying malignancy. Read More

Internist (Berl) 2018 Feb;59(2):151-158

Klinisches Institut für Neurologie, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich.

Background: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. Read More

J Neuroimmunol 2018 Feb 18;315:28-32. Epub 2017 Dec 18.

Center for Sleep Sciences and Medicine, Stanford University, 3165 Porter Drive, Palo Alto, CA 94304, USA. Electronic address:

Anti-Yo paraneoplastic cerebellar degeneration (PCD) is a devastating autoimmune complication of gynecological cancers. We hypothesized that as for other autoimmune diseases, specific HLA haplotypes are associated. We conducted high resolution HLA typing of Class I/Class II in 40 cases versus ethnically matched controls. Read More

Acta Neuropathol 2018 04 3;135(4):569-579. Epub 2018 Jan 3.

Institut NeuroMyogène, Equipe Synaptopathies et Autoanticorps (SynatAc), INSERM U1217/UMR CRS 5310, Lyon, France.

Paraneoplastic cerebellar degenerations with anti-Yo antibodies (Yo-PCD) are rare syndromes caused by an auto-immune response against neuronal antigens (Ags) expressed by tumor cells. However, the mechanisms responsible for such immune tolerance breakdown are unknown. We characterized 26 ovarian carcinomas associated with Yo-PCD for their tumor immune contexture and genetic status of the 2 onconeural Yo-Ags, CDR2 and CDR2L. Read More

CNS Neurol Disord Drug Targets 2017 Dec 20. Epub 2017 Dec 20.

Royal Hallamshire Hospital. United Kingdom.

The identification of an increasing number of immune mediated ataxias suggests that the cerebellum is often a target organ for autoimmune insults. The diagnosis of immune mediated ataxias remains challenging as there is significant clinical overlap between immune mediated and other forms of ataxia. Furthermore the classification of immune mediated ataxias requires further clarification particularly for those ataxias where no specific antigenic trigger and associated antibodies have been identified. Read More

J Neurol Sci 2018 01 7;384:36-37. Epub 2017 Nov 7.

Department of Neurology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave, Bethesda, MD 20889, United States.

Neurology 2018 Jan 8;90(2):e103-e110. Epub 2017 Dec 8.

From the Departments of Neurology (D.D., A.G., S.J.P., E.P.F., A.M., C.J.K.) and Laboratory Medicine and Pathology (V.A.L., S.J.P., E.P.F., J.E.S., A.M., C.J.K.), Mayo Clinic, Rochester, MN.

Objective: To establish the phenotype and clinical outcomes of collapsin response-mediator protein-5 (CRMP5) autoimmune neuropathy in comparison with anti-neuronal nuclear antibody type 1 (ANNA1)-immunoglobulin G (IgG) neuropathy.

Methods: Patients with CRMP5-IgG and/or ANNA1-IgGs were identified in our service-line testing, and medical records were reviewed.

Results: One hundred five patients with CRMP5-IgG neuropathy (88% smokers; 69% having cancer, most commonly small cell lung cancer [75%]) were identified and compared to 51 patients with ANNA1-IgG neuropathy, 27 with coexisting CRMP5-IgG. Read More

Int J Neurosci 2018 Aug 18;128(8):721-728. Epub 2017 Dec 18.

a Clinical Neuroimmunology and Neurochemistry, Department of Neurology , Hannover Medical School , Hannover , Germany.

The paraneoplastic cerebellar syndrome presents as severe neuroimmunological disease associated with malignancies. Antibodies against antigens expressed by tumor cells cross-react with proteins of cerebellar Purkinje cells leading to neuroinflammation and neuronal loss. These antineuronal antibodies are preferentially investigated by serological analyses while examination of the cerebrospinal fluid is only performed infrequently. Read More

Scand J Immunol 2018 02;87(2):109-110

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

Parkinsonism Relat Disord 2017 Nov 13;44:106-109. Epub 2017 Oct 13.

Island Hospital, 308 Macalister Road, 10450 Penang, Malaysia. Electronic address:

Purpose Of Review: To provide an overview of paraneoplastic autoimmune disorders presenting with various movement disorders.

Recent Findings: The spectrum of paraneoplastic autoimmune disorders has been expanding with the discovery of new antibodies against cell surface and intracellular antigens. Many of these paraneoplastic autoimmune disorders manifest as a form of movement disorder. Read More