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Cerebellum Ataxias 2021 Jun 13;8(1):16. Epub 2021 Jun 13.

Academic department of neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.

Background: Most immune-mediated cerebellar ataxias, including those associated with gluten sensitivity (Gluten Ataxia), tend to present subacutely and usually progress gradually. Acute presentations with rapid progression outside the context of paraneoplastic cerebellar degeneration require prompt diagnosis and early access to disease-modifying immunotherapy in order to avert severe and permanent neurological disability.

Case Presentations: We describe three cases of rapid-onset Gluten Ataxia, an immune-mediated cerebellar ataxia due to gluten sensitivity. Read More

J Clin Neurosci 2021 Jul 21;89:336-342. Epub 2021 May 21.

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China. Electronic address:

Paraneoplastic neurological syndromes (PNS) are rare immune-mediated disorders, and the detection of onconeural antibodies is helpful for PNS diagnosis. The aim of this study was to investigate the clinical characteristics of patients with PNS with positive onconeural antibodies in a single center in Hubei, China. We retrospectively analyzed the clinical characteristics of 54 patients with positive onconeural antibodies from January 2016 to September 2020. Read More

Cerebellum Ataxias 2021 Jun 5;8(1):14. Epub 2021 Jun 5.

Department of Nuclear Medicine, Aalborg University Hospital, Aalborg, Denmark.

Background: Paraneoplastic cerebellar degeneration (PCD) is a classic neurological syndrome where the presence of Anti-Tr/DNER antibodies is strongly associated with Hodgkin Lymphoma (HL). Awareness of the syndrome is important because with prompt treatment the prognosis of HL is good. The diagnosis can be a challenge in some patients. Read More

Rev Med Liege 2021 May;76(5-6):413-418

Professeur émérite, Université de Liège, Belgique.

Neurological paraneoplastic syndromes are non-metastatic complications of systemic cancers, often resulting from an immune response triggered by the crossed expression of neuronal antigens by tumour cells. Several neurological syndromes such as cerebellar degeneration, sensory neuronopathy, limbic encephalitis, encephalomyelitis or the Lambert-Eaton myasthenic syndrome are most often paraneoplastic and require prompt cancer screening, particularly if the patient shows risk factors for cancer. Although there are many exceptions to this rule, a given syndrome is often associated with a particular antibody and the corresponding tumour. Read More

J Neurooncol 2021 Jun 2. Epub 2021 Jun 2.

Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Unit 0431, Houston, TX, 77030, USA.

Introduction: Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from tumor-associated autoimmunity against antigens within the cerebellum. Anti-Purkinje cell cytoplasmic antibody 1 (PCA-1), or anti-Yo, is the most commonly linked antibody and is classically associated with breast and ovarian cancers.

Methods: Medical records of patients at our institution who developed PCA-1 associated PCD were reviewed. Read More

Neurol Res Pract 2021 May 24;3(1):30. Epub 2021 May 24.

Department of Neurology, University Hospital Essen, Essen, Germany.

Anti-glial nuclear antibody (AGNA) is an onconeuroal antibody targeting the nuclei of Bergmann glia in the cerebellum and Anti-SRY-related HMG-box 1 (SOX1). It is highly specific for small cell lung cancer (SCLC) and correlates to the appearance of paraneoplastic neurological syndromes such as Lambert-Eaton myasthenic syndrome (pLEMS) and paraneoplastic cerebellar degeneration (PCD) amongst others. Herein, we present a SCLC patient with rapidly progressive PCD, LEMS and axonal polyneuropathy associated with AGNA/SOX1-antibodies, successfully treated with plasma-exchange (PLEX). Read More

Brain Nerve 2021 May;73(5):611-619

Department of Medical Education, Tokyo Medical University.

Autoimmune mechanisms insult the cerebellum, resulting in the development of cerebellar ataxias (CAs). These immune-mediated cerebellar ataxias (IMCAs) include gluten ataxia, postinfectious cerebellitis, paraneoplastic cerebellar degeneration (PCD), antiglutamate decarboxylase 65 antibody-associated CA (anti-GAD ataxia), and primary autoimmune cerebellar ataxia (PACA). Cell-mediated mechanisms are assumed to be involved in PCD, whereas accumulating evidence shows that anti-GAD antibodies decrease the amount of GABA released which leads to functional synaptic deficits. Read More

Leuk Lymphoma 2021 May 17:1-2. Epub 2021 May 17.

Department of Neuroscience, Aziz Sancar Institute for Experimental Medicine, Istanbul University, Istanbul, Turkey.

Neurol Clin Pract 2021 Feb;11(1):33-42

Division of Movement Disorders (NW, MA, DH), Rush University Medical Center, and Biostatistical Analysis (YL, BO), Department of Neurological Sciences, Rush University Medical Center, Chicago, IL.

Objective: To determine the differences in outcomes of adult patients with ataxia initially evaluated for paraneoplastic cerebellar degeneration (PCD) as inpatients or outpatients.

Methods: In this retrospective cohort analysis, diagnosis, workup, and functional outcomes based on the change in the modified Rankin Scale (mRS) score were compared between patients with ataxia who underwent workup for PCD initially as inpatients vs outpatients between March 2011 and June 2018 at Rush University Medical Center.

Results: There were 78 patients included in the analysis; 59% were women, and the average age at symptom onset was 57 ± 19. Read More

Ann Indian Acad Neurol 2021 Jan-Feb;24(1):32-39. Epub 2021 Feb 9.

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs.

Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center. Read More

Acta Derm Venereol 2021 May 10;101(5):adv00452. Epub 2021 May 10.

Department of Medical Sciences and Human Oncology, Section of Dermatology, University of Turin, Via Cherasco 23, IT-12124 Torino, Italy. E-mail:

Cureus 2021 Mar 3;13(3):e13677. Epub 2021 Mar 3.

Hematology/Oncology, Magee Women's Hospital, University of Pittsburgh Medical Center, Pittsburgh, USA.

Paraneoplastic neurologic syndromes (PNS) are a group of disorders characterized by an autoimmune response against the nervous system due to cross-reactivity between malignant and normal neural tissue. The most commonly associated malignancies include small cell lung cancer, ovarian cancer, breast cancer, and lymphoma. Multiple PNS have been reported including paraneoplastic cerebellar degeneration, retinopathy, sensorimotor peripheral neuropathy, encephalopathy, opsoclonus-myoclonus syndrome, and stiff-person syndrome. Read More

Neurol Sci 2021 Jun;42(6):2599

Department of Neurology, BLK Super Speciality Hospital, New Delhi, India.

J Breast Cancer 2021 Feb;24(1):106-116

Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, USA.

Paraneoplastic neurologic diseases (PND) are rare but can occur in patients with common malignancies including breast cancer. In patients with hormone receptor (HR)-negative human epidermal growth factor receptor 2 (HER2)-positive breast cancer, PND have been reported in association with anti-Yo antibodies and with clinical presentation of paraneoplastic cerebellar degeneration. We describe the case of a woman with progressively altered mental status and seizures, ultimately requiring admission. Read More

Clin Nucl Med 2021 06;46(6):e327-e328

Department of Health Science, University of Genoa, Genoa, Italy.

Abstract: In paraneoplastic cerebellar degeneration (PCD), the standard diagnostic workup might be inconclusive, especially in seronegative subtypes. Brain 18F-FDG PET is an accurate supportive diagnostic tool in immune-mediated disorders, but findings in PCD are controversial. Semiquantitative analysis of 18F-FDG PET can meaningfully assist visual assessment in different neurological conditions and has been mainly applied to disclose regional hypometabolism. Read More

Breast J 2021 May 21;27(5):484-485. Epub 2021 Feb 21.

Department of Nuclear Medicine, Marmara University Pendik Training and Research Hospital, Istanbul, Turkey.

J Int Med Res 2021 Feb;49(2):300060521992231

Department of Neurology, Sixth Medical Center of Chinese PLA General Hospital, Beijing, China.

Paraneoplastic cerebellar degeneration (PCD) is a neurological syndrome that is likely caused by tumor-induced autoimmunity against the cerebellum. Neuroendocrine carcinoma (NEC) is a type of neoplasm with high-grade malignant histology and biological behavior. The prognosis for both PCD and NEC is typically poor. Read More

Neurol Sci 2021 Jun 8;42(6):2523-2525. Epub 2021 Feb 8.

Department of Neurology, BLK Super Speciality Hospital, New Delhi, India.

Background: Paraneoplastic Cerebellar degeneration (PCD) is one of the classical paraneoplastic syndromes (PNS) which is characterised by subacute onset, progressive cerebellar ataxia and is usually associated with small cell lung carcinoma, adeno carcinoma of breast and ovary followed by Hodgkin's lymphoma.

Objective: We herein report a case of subacute onset, progressive cerebellar ataxia in a 37-year-old female, who on evaluation was found to have non-Hodgkin's lymphoma and experienced good clinical response to treatment.

Discussion: As compared to solid tumours, chances of association of PNS with Lymphomas is quite low and there are only few case reports in the literature showing association of PCD with non-Hodgkin's lymphoma. Read More

Neurol Neuroimmunol Neuroinflamm 2021 03 2;8(2). Epub 2021 Feb 2.

From the Department of Neurology (I.H., M.H., C.T., C.V.), Haukeland University Hospital; Department of Clinical Medicine (I.H., K.M., C.V.), University of Bergen; and Departments of Neurology and Clinical Medicine (I.H., C.T., C.V.), Neuro-SysMed-Centre of Excellence for Experimental Therapy in Neurology, Bergen, Norway.

Objective: Investigate the value of including cerebellar degeneration-related protein 2-like (CDR2L) as a marker in commercial diagnostic tests for anti-Yo-associated paraneoplastic cerebellar degeneration (PCD).

Methods: We included sera and CSF samples from 24 patients with suspected PCD (6 of whom had PCD with underlying gynecologic or breast cancer), who were positive for Yo antibodies using the commercially available, paraneoplastic neurologic syndromes (PNS) 14 Line Assay from Ravo Diagnostika. The samples were further evaluated using the EUROLINE PNS 12 Ag Line Assay and a cell-based assay (CBA) from Euroimmun. Read More

Gynecol Oncol Rep 2021 Feb 31;35:100695. Epub 2020 Dec 31.

Oncology Institute "Ion Chiricuta", Medical Oncology Department, Cluj Napoca, Romania.

Paraneoplastic neurologic syndromes (PNS) are a rare heterogeneous group of disorders associated with malignancy that can result in significant functional impairment. One syndrome in particular, paraneoplastic cerebellar degeneration (PCD), may be severely disabling. PCD is a rare neurological syndrome, associated with active or subclinical cancer, characterized by acute or subacute onset cerebellar ataxia due to tumor-induced autoimmunity against cerebellar antigens. Read More

Tunis Med 2020 Nov;98(11):869-872

Neurological presentation of paraneoplastic syndromes is rare. They are often difficult to diagnose, especially when they precede the diagnosis of cancer. Hodgkin's lymphoma is associated with several paraneoplastic neurological syndromes such as cerebellar degeneration and dermato/polymyositis. Read More

Curr Oncol 2021 Jan 19;28(1):560-564. Epub 2021 Jan 19.

Eskisehir City Hospital, Department of Neurology, Eskisehir 26080, Turkey.

Paraneoplastic cerebellar degeneration (PCD) is a rare neuroimmunological disease that may accompany tumors. In this article, we present a patient with progressive gait difficulty who was diagnosed with PCD and, in a rare comorbidity, with papillary thyroid carcinoma (PTC) following malignancy screening. A 46-year-old male patient reported having experienced poor balance for 2 years. Read More

J Mov Disord 2021 Jan 12;14(1):10-28. Epub 2021 Jan 12.

Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield, UK.

Since the first description of immune-mediated cerebellar ataxias (IMCAs) by Charcot in 1868, several milestones have been reached in our understanding of this group of neurological disorders. IMCAs have diverse etiologies, such as gluten ataxia, postinfectious cerebellitis, paraneoplastic cerebellar degeneration, opsoclonus myoclonus syndrome, anti-GAD ataxia, and primary autoimmune cerebellar ataxia. The cerebellum, a vulnerable autoimmune target of the nervous system, has remarkable capacities (collectively known as the cerebellar reserve, closely linked to plasticity) to compensate and restore function following various pathological insults. Read More

Clin Neurol Neurosurg 2021 01 5;200:106407. Epub 2020 Dec 5.

Research Center of Neurology, Volokolamskoe Shosse 80, Moscow, 125367, Russia.

Intern Med 2021 May 15;60(10):1607-1610. Epub 2020 Dec 15.

Department of Neurology, Kansai Electric Power Hospital, Japan.

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. Read More

J Neuroimmunol 2020 Sep 29;350:577408. Epub 2020 Sep 29.

Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China. Electronic address:

Anti-protein kinase Cgamma (anti-PKCγ) antibodies are rare onconeural antibodies associated with paraneoplastic cerebellar degeneration (PCD). To date, only two patients with PCD and anti-PKCγ antibodies have been reported. Here, we report a Chinese patient with PCD and anti-PKCγ antibodies. Read More

Mov Disord Clin Pract 2020 Nov 14;7(8):904-909. Epub 2020 Sep 14.

Department of Neurology Mayo Clinic Rochester Minnesota USA.

Background: To date, 10 patients with GTPase Regulator Associated with Focal Adhesion Kinase 1/Rho GTPase Activating Protein 26-Immunoglobulin (GRAF1/ARHGAP26-IgG) associated neurological disorders have been described, most with ataxia.

Objective: To report the clinical, oncological, and radiological associations of GRAF1 autoantibodies.

Methods: We identified 17 patients whose serum and/or cerebrospinal fluid IgG was confirmed to target GRAF1/ARHGAP26-IgG by both tissue-based immunofluorescence and transfected cell-based assay. Read More

AJNR Am J Neuroradiol 2020 12 22;41(12):2176-2187. Epub 2020 Oct 22.

From the Division of Neuroradiology, Department of Radiology (A.A.M., C.M.C., P.P.M., A.L.K., C.H.H., L.J.E., E.P.L., F.E.D.).

Paraneoplastic syndromes are systemic reactions to neoplasms mediated by immunologic or hormonal mechanisms. The most well-recognized paraneoplastic neurologic syndrome, both clinically and on imaging, is limbic encephalitis. However, numerous additional clinically described syndromes affect the brain, spinal cord, and peripheral nerves. Read More

Indian J Cancer 2020 Oct-Dec;57(4):487-488

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