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Parkinsonism Relat Disord 2022 Jun 3. Epub 2022 Jun 3.

University of Florida, Department of Neurology, Norman Fixel Institute for Neurological Diseases, 3009 SW Williston Rd, Gainesville, FL, USA.

Transl Cancer Res 2022 May;11(5):1434-1439

Breast Disease Center, Peking University First Hospital, Beijing, China.

Background: Paraneoplastic cerebellar degeneration (PCD) is a relatively rare complication among patients with cancers with nonmetastatic tumor manifestation, including breast cancer. A breast cancer diagnosis is usually made several months (or even years) after the onset of neurological symptoms.

Case Description: In this study, we describe the early diagnosis and treatment of PCD in one patient with breast cancer. Read More

Cerebellum 2022 May 26. Epub 2022 May 26.

Department of Neurology, Peking Union Medical College Hospital, Beijing, China.

Autoimmune cerebellar ataxia (ACA) is an important and potentially treatable cause of sporadic cerebellar syndrome, but studies with large sample size are limited. This study reported a large ACA series in China and described its etiology and clinical characteristics. We reviewed all ACA patients from our hospital (2013-2021) and analyzed their clinical and paraclinical features, treatment, and outcome. Read More

J Clin Neuromuscul Dis 2022 Jun;23(4):189-200

Departments of Neurology and.

Abstract: This update covers a number of treatment topics starting with Fc receptor inhibitors and the Federal Drug Administration approval of efgartigimod. Some uncertainties regarding the use of corticosteroids are addressed, namely the risk of exacerbation with initiation of treatment and how to taper. The presence and potential importance of antibody overshoot following plasmapheresis is noted and the evolving increase in usefulness of acetylcholine receptor antibodies in diagnosing ocular myasthenia. Read More

BMC Neurol 2022 May 2;22(1):165. Epub 2022 May 2.

Department of Internal Medicine, Division of Neurology, St Marianna University School of Medicine: Sei Marianna Ika Daigaku, Kawasaki, Kanagawa, Japan.

Background: As a debilitating syndrome, paraneoplastic cerebellar degeneration (PCD) remains challenging to treat. Further, anti-Yo antibody (directed against human cerebellar degeneration-related protein 2) detection in patients with PCD is associated with unsatisfactory responses to existing therapies. Here, we present the case of a 60-year-old woman who developed PCD with anti-Yo antibodies and a submandibular gland tumor. Read More

Neurol Sci 2022 Jun 7;43(6):3979-3982. Epub 2022 Apr 7.

Neurology Unit, Azienda Ospedaliero-Universitaria Di Modena, Modena, Italy.

Background And Aims: Antibodies against SOX1 (or anti-glial nuclear antibody, AGNA) are partially characterized onconeural antibodies, firstly described in association with small cell lung cancer (SCLC). Lambert-Eaton myasthenic syndrome is the most frequent paraneoplastic syndrome (PNS) found in patients with anti-SOX1-antibody positivity. Other associations are chronic axonal polyneuropathy, paraneoplastic limbic encephalitis, and paraneoplastic cerebellar degeneration. Read More

Arch Soc Esp Oftalmol (Engl Ed) 2022 Jun 12;97(6):350-357. Epub 2022 Mar 12.

Institut Clínic d'Oftalmologia (ICOF), Hospital Clínic, Barcelona, Spain; August Pi i Sunyer Biomedical Research Institute (IDIBAPS), Barcelona, Spain.

Paraneoplastic syndromes consist on systemic manifestations associated with certain cancers which are not a direct consequence of tumor invasion or its metastases. It is known that autoimmunity and autoantibody synthesis play an important role in its pathophysiology due to a process of molecular mimicry. Paraneoplastic syndromes in ophthalmology are rare, but it is important to recognize them clinically because in some cases symptoms preceded the diagnosis of an underlying neoplasia. Read More

Front Immunol 2022 17;13:813926. Epub 2022 Feb 17.

Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.

Background: Immune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbs-associated CAs. Read More

J Spinal Cord Med 2022 Mar 2:1-5. Epub 2022 Mar 2.

Department of Neurology, Nimes University Hospital, Nimes, France.

Context: Posterior spinal cord lesions are found in patients with ganglionopathy. These are normally found in later stages of the neuronopathy as a consequence of dorsal root ganglia degeneration. Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS) is an emerging neurological disorder. Read More

Brain Sci 2022 Feb 12;12(2). Epub 2022 Feb 12.

Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, MMC 76, 420 Delaware St. SE, Minneapolis, MN 55455, USA.

Autoimmune-mediated ataxia has been associated with paraneoplastic disease, gluten enteropathy, Hashimoto thyroiditis as well as autoimmune disorders without a known associated disease. There have been relatively few reports describing the neuropathology of these conditions. This review is an attempt to consolidate those reports and determine the ways in which autoimmune ataxias can be neuropathologically differentiated from hereditary or other sporadic ataxias. Read More

Cureus 2022 Jan 11;14(1):e21106. Epub 2022 Jan 11.

Neurology, Cheikh Khalifa Ibn Zayed Hospital, Faculty of Medicine, Mohamed VI University of Health Sciences (UM6SS), Casablanca, MAR.

Neurological paraneoplastic syndromes are a rare entity that affects patients with cancer. Anti-Ri antibodies affect the brain stem and produce a heterogeneous rapidly progressive subacute syndrome depending on the involvement of the different regions concerned. The most common clinical presentation is opsoclonus-myoclonus syndrome and paraneoplastic cerebellar degeneration. Read More

Case Rep Oncol 2021 Sep-Dec;14(3):1799-1805. Epub 2021 Dec 20.

Oncology Department, Hospital Español, Mexico City, Mexico.

Paraneoplastic neurological syndromes (PNS) are rare presentations of an underlying oncological disease and more unusual during an oncological disease. They most likely present in small-cell lung carcinomas and thymomas, but present in <1% of the gynecological neoplasms. Acknowledging the pathophysiology is essential for management, explaining its clinical presentation, and future research. Read More

Neurologist 2022 Jan 20. Epub 2022 Jan 20.

Clinical Neurology Research Center Department of Pathology Hematology Research Center, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: Hypertrophic olivary degeneration (HOD) is a unique form of trans-synaptic neuronal degeneration within the dentato-rubro-olivary pathway which is manifested by the enlargement and hyperintensities of the inferior olivary nucleus in the brain magnetic resonance imaging.

Case Report: We report a 53-year-old man admitted to our emergency department with a history of progressive ataxia and vertigo for 6 months before admission. Neurological examination revealed cerebellar dysfunction, and the brain magnetic resonance imaging showed bilateral HOD without an identifiable causative lesion within the brain or abnormal meningeal enhancement. Read More

Clin Nucl Med 2022 Feb;47(2):130-132

Department of Neurology, Flevoziekenhuis, Almere, the Netherlands.

Abstract: A 47-year-old woman presented with an acute cerebellar syndrome. Neither cerebellar atrophy nor an infarction or tumor was shown on MRI. A diagnostic CT demonstrated enlarged axillary lymph nodes, but no primary tumor. Read More

Cureus 2021 Dec 6;13(12):e20203. Epub 2021 Dec 6.

Hematology/Oncology, Houston Methodist Hospital, Houston, USA.

Anti-Yo-associated paraneoplastic cerebellar degeneration (PCD) syndrome is a very rare condition that is most commonly associated with breast and gynecologic cancers. Those cases associated with breast cancer tend to be human epidermal growth factor receptor 2 (HER2)-positive, though the reason for this correlation is unknown. Most commonly, the neurologic symptoms of the PCD syndrome predate the patient's cancer diagnosis. Read More

Int J Gynecol Cancer 2022 01;32(1):101-104

Department of Medical Oncology, Centre Eugène Marquis, Rennes, France.

Brain Sci 2021 Oct 26;11(11). Epub 2021 Oct 26.

Department of Neurology, Philipps-University Marburg, 35043 Marburg, Germany.

To provide an update on paraneoplastic cerebellar degeneration (PCD), the involved antibodies and tumors, as well as management strategies. PCD represents the second most common presentation of the recently established class of immune mediated cerebellar ataxias (IMCAs). Although rare in general, PCD is one of the most frequent paraneoplastic presentations and characterized clinically by a rapidly progressive cerebellar syndrome. Read More

J Neuroimmunol 2022 01 11;362:577766. Epub 2021 Nov 11.

Department of Neurology, Haukeland University Hospital, Pb 1400, 5021 Bergen, Norway; Department of Clinical Medicine, University of Bergen, Pb 7804, 5020 Bergen, Norway; Neuro-SysMed - Centre of Excellence for Experimental Therapy in Neurology, Departments of Neurology and Clinical Medicine, Pb 1400, 5021 Bergen, Norway. Electronic address:

Patients with ovarian cancer and paraneoplastic cerebellar degeneration, a cancer-related immune disorder, often have anti-Yo antibody. Here we studied the distributions of anti-Yo antigens CDR2L and CDR2 in rat and human brain using immunohistochemistry and western blot. CDR2L localized mainly to the Purkinje cells and large neurons scattered in the brain stem. Read More

Cerebellum 2021 Nov 24. Epub 2021 Nov 24.

Neuroimmunology Group, Hospital das Clínicas da Universidade de São Paulo, Avenida Dr Eneas de Carvalho Aguiar, 255-CEP, São Paulo, 05403-000, Brazil.

Rapidly progressive cerebellar ataxia is a classical paraneoplastic neurological syndrome associated with different autoantibodies and typical demographic characteristics, extracerebellar signs, tumor association, and prognosis. Anti-Tr/anti-Delta/Notch-like epidermal growth factor-related receptor (DNER) antibody is one of the associated antibodies. Given the rarity of this condition, our current knowledge is based on case reports and small case series. Read More

Clin Lymphoma Myeloma Leuk 2022 02 20;22(2):e124-e127. Epub 2021 Oct 20.

Department of Molecular Medicine, University of Pavia, Pavia, Italy; Division of Hematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Front Neurol 2021 22;12:728700. Epub 2021 Oct 22.

Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, United States.

The development of autoimmune antibody panels has improved the diagnosis of paraneoplastic neurological disorders (PNDs) of the brain and spinal cord. Here, we present a case of a woman with a history of breast cancer who presented with a subacute sensory ataxia that progressed over 18 months. Her examination and diagnostic studies were consistent with a myelopathy. Read More

Thorac Surg Clin 2021 Nov;31(4):519-537

Hospital of the University of Pennsylvania, Ravdin 6, 3400 Spruce Street, Philadelphia, PA 19104, USA.

Paraneoplastic syndromes are clinical entities associated with cancers and often overlap with metabolic and endocrine syndromes. The cell types of lung cancer involved are frequently small cell, squamous cell, adenocarcinoma, large cell, and carcinoid tumor. A number of neurologic paraneoplastic syndromes have been described for which the tumor product remains unknown. Read More

Brain Commun 2021 26;3(3):fcab185. Epub 2021 Aug 26.

French Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, 69677 Bron, France.

In this study, we report the clinical features of Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome, design and validate a clinical score to facilitate the identification of patients that should be tested for Kelch-like protein 11 antibodies, and examine in detail the nature of the immune response in both the brain and the tumour samples for a better characterization of the immunopathogenesis of this condition. The presence of Kelch-like protein 11 antibodies was retrospectively assessed in patients referred to the French Reference Center for paraneoplastic neurological syndrome and autoimmune encephalitis with (i) antibody-negative paraneoplastic neurological syndrome [limbic encephalitis ( = 105), cerebellar degeneration ( = 33)] and (ii) antibody-positive paraneoplastic neurological syndrome [Ma2-Ab encephalitis ( = 34), antibodies targeting N-methyl-D-aspartate receptor encephalitis with teratoma ( = 49)]. Additionally, since 1 January 2020, patients were prospectively screened for Kelch-like protein 11 antibodies as new usual clinical practice. Read More

Acta Neurol Taiwan 2021 Jun;30(2):74-77

Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.

Purpose: Paraneoplastic neurological disorders associated with autoantibodies are rare diseases, causing abnormal manifestations in the central or peripheral nervous system separately or simultaneously. Early recognizing the occurrence of paraneoplastic syndrome can lead to prompt and effective management.

Case Report: We presented a patient of subacute cerebellar degeneration with cachectic and bed-ridden status, who was proven to have positive SOX1 antibody. Read More

Neurol India 2021 Jul-Aug;69(4):1099-1100

Department of Medicine, DY Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra, India.

Front Neurol 2021 18;12:683048. Epub 2021 Aug 18.

Department of Neurology, University of Colorado, Aurora, CO, United States.

Movement disorders are a common feature of many antibody-associated neurological disorders. In fact, cerebellar ataxia is one of the most common manifestations of autoimmune neurological diseases. Some of the first autoantibodies identified against antigen targets include anti-neuronal nuclear antibody type 1 (ANNA-1 or anti-Hu) and Purkinje cell cytoplasmic antibody (PCA-1) also known as anti-Yo have been identified in paraneoplastic cerebellar degeneration. Read More

Clin Nucl Med 2022 Feb;47(2):e125-e128

Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.

Abstract: Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological disease characterized by adaptive immune response against onconeural antigens physiologically expressed in the cerebellum. It is characterized by presence of highly specific onconeural autoantibodies such as anti-Yo, anti-Hu, anti-Ri, and anti-Ma2 in the serum and cerebrospinal fluid as diagnostic biomarkers. Antiamphiphysin autoantibody-related paraneoplastic encephalitis is a less commonly seen autoimmune neurological disorder usually presenting as stiff person syndrome. Read More

Indian J Nucl Med 2021 Apr-Jun;36(2):220-222. Epub 2021 Jun 21.

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

The case of a 39-year-old woman presented with symptoms of progressive cerebellar degeneration for few months preceding by the diagnosis and treatment of breast cancer. The causative association was revealed with the strong positivity of antineuronal antibody. Because of the multidisciplinary team approach with surgery and adjuvant endocrine treatment, the patient did improve symptomatically and she is alive without any evidence of disease after 22 months following the initial diagnosis of the neurological disorder. Read More

Neuropathol Appl Neurobiol 2022 02 8;48(2):e12755. Epub 2021 Aug 8.

Clinical Neurosciences, Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, UK.

We report a rapidly progressive and fatal CD8 T-cell-mediated cerebellitis after ipilimumab (cytotoxic T-lymphocyte-associated protein 4 inhibitor) for small cell lung cancer. Clinical features and histopathology were consistent with an accelerated form of paraneoplastic cerebellar degeneration. A patchy CD8 T-cell infiltrate spatially corresponded to areas of Purkinje cell loss, with occasional CD8 polarisation towards Purkinje cells. Read More

Neurooncol Pract 2021 Aug 24;8(4):362-374. Epub 2021 Feb 24.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Paraneoplastic neurological syndrome (PNS) comprises a group of neurological disorders that result from a misguided immune response to the nervous system triggered by a distant tumor. These disorders frequently manifest before the diagnosis of the underlying neoplasm. Since the first reported case in 1888 by Oppenheim, the knowledge in this area has evolved rapidly. Read More