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Paraneoplastic Neurological and Muscular Syndromes Paraneoplastic neurological and muscular syndromes Short compendium Version 4.5, April 2016 By Finn E. Somnier, M.D., D.Sc. (Med.), copyright ® Department of Autoimmunology and Biomarkers, Statens Serum Institut, Copenhagen, Denmark 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) Table of contents PARANEOPLASTIC NEUROLOGICAL SYNDROMES .................................................... 4 DEFINITION, SPECIAL FEATURES, IMMUNE MECHANISMS ................................................................ 4 SHORT INTRODUCTION TO THE IMMUNE SYSTEM .................................................. 7 DIAGNOSTIC STRATEGY ..................................................................................................... 12 THERAPEUTIC CONSIDERATIONS .................................................................................. 18 SYNDROMES OF THE CENTRAL NERVOUS SYSTEM ................................................ 22 MORVAN’S FIBRILLARY CHOREA ................................................................................................ 22 PARANEOPLASTIC CEREBELLAR DEGENERATION (PCD) ...................................................... 24 Anti-Hu syndrome .................................................................................................................. 25 Anti-Yo syndrome ................................................................................................................... 26 Anti-CV2 / CRMP5 syndrome ............................................................................................. 26 Anti-Ma1 syndrome ............................................................................................................... 27 Anti-PCA2 syndrome ............................................................................................................. 28 Anti-Tr (PCA-Tr) syndrome ................................................................................................. 28 Anti-mGluR1 syndrome ........................................................................................................ 29 Anti-CARP8 syndrome .......................................................................................................... 29 Anti-GAD syndrome ............................................................................................................... 29 Anti-ZIC4-syndrome ............................................................................................................. 29 LEMS-associated ..................................................................................................................... 30 Anti-ARHGAP26 (GRAF) ....................................................................................................... 30 Anti-PKC gamma .................................................................................................................... 30 PARANEOPLASTIC CHOREO-ATHETOSIS / STRIATAL ENCEPHALITIS ................................. 31 PARANEOPLASTIC MORE CLASSICAL CNS DISORDERS ......................................................... 33 Paraneoplastic encephalomyelitis (PEM) ....................................................................... 34 Paraneoplastic limbic encephalitis (PLE),may include other structures ............ 35 A variety of associated autoatibodies ...................................................................................................... 36 Paraneoplastic brainstem encephalitis ........................................................................... 37 Anti-Ta (Ma2) syndrome .......................................................................................................................... 38 Paraneoplastic myelitis / myelopathy ............................................................................. 38 PARANEOPLASTIC MOTOR NEURON DISEASE? ........................................................................ 40 PARANEOPLASTIC OPSOCLONUS / MYOCLONUS (POM) ..................................................... 42 POM in children ....................................................................................................................... 42 POM in adults ........................................................................................................................... 43 Anti-Hu, Yo, Ta (Ma2) syndromes ............................................................................................................ 43 Anti-Ri syndrome...................................................................................................................................... 43 PARANEOPLASTIC OPTIC NEURITIS ........................................................................................... 45 PARANEOPLASTIC RETINOPATHY (CAR, MAR) .................................................................... 47 STIFF-PERSON SPECTRUM OF SYMPTOMS (SPS AND PERM) ............................................ 51 SPS variants ............................................................................................................................. 53 SYNDROMES OF THE PERIPHERAL NERVOUS SYSTEM ........................................ 56 PARANEOPLASTIC AUTONOMIC NEUROPATHY ......................................................................... 56 CHRONIC GASTROINTESTINAL PSEUDOOBSTRUCTION .......................................................... 56 PARANEOPLASTIC MOTOR NEUROPATHY .................................................................................. 58 PARANEOPLASTIC SENSORY-MOTOR NEUROPATHY ............................................................... 58 PARANEOPLASTIC SENSORY NEURONOPATHY (SSN, PSN) ............................................... 60 SYNDROMES OF THE NEUROMUSCULAR JUNCTION .............................................. 63 LAMBERT-EATON MYASTHENIC SYNDROME ............................................................................. 64 ACQUIRED NEUROMYOTONIA, ISAACS’ SYNDROME ............................................................... 66 MORVAN’S FIBRILLARY CHOREA ................................................................................................ 66 1 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) PARANEOPLASTIC SEROPOSITIVE MYASTHENIA GRAVIS WITH THYMOMA ....................... 70 PARANEOPLASTIC MYOPATHIES .................................................................................... 75 POLYMYOSITIS, DERMATOMYOSITIS ......................................................................................... 75 NECROTIZING MYOPATHY ............................................................................................................ 77 SPORADIC INCLUSION BODY MOSITIS ...................................................................................... 78 MYASTHENIA GRAVIS-ASSOCIATED MYOPATHY ..................................................................... 80 RIPPLING MUSCLE SYNDROME – ACQUIRED SPORADIC, AUTOIMMUNE ............................ 82 SYMPTOMATIC OVERVIEW ................................................................................................ 86 PARANEOPLASTIC ATAXIA ........................................................................................................... 86 PARANEOPLASTIC EPILEPSY ........................................................................................................ 86 PARANEOPLASTIC EXTRAPYRAMIDAL DISORDERS ................................................................. 88 PARANEOPLASTIC PAIN ............................................................................................................... 88 MATERNAL AUTOANTIBODIES AND PASSIVE TRANSFER IN HUMANS ........ 89 AUTOANTIBODIES ASSOCIATED WITH PNS ............................................................. 94 PARANEOPLASTIC ANTIBODIES TARGETING THE NERVOUS SYSTEM OR STRIATED MUSCLES VERSUS NEOPLASMS ................................................................ 97 DIAGNOSTIC CRITERIA - OVERVIEW ......................................................................... 106 OVERVIEW OF MANAGEMENT ........................................................................................ 107 LISTING OF SOME BOOKS AND REVIEWS ................................................................ 108 SUBJECT INDEX ..................................................................................................................... 109 AUTOIMMUNE ENCEPHALITIS, PLEASE SEE SEPARATE COMPENDIUM .... 112 CHANNELOPATHIES, RECEPTOR AND SOLUTE CARRIERS DISORDERS IN NEUROLOGY, PLEASE SEE SEPARATE COMPENDIUM ......................................... 113 Throughout this book, text is colour coded as follows: Clinical features printed in blue Treatment printed in green Underlined text is hyperlinked If you want to purchase an electronic version, please contact the author 2 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) Figure 1: Immunoreactivity of some of the most common oncological anti-neuronal antibodies Top left: Anti-Hu staining of neuronal nuclei (cerebellar granule & Purkinje cells) Top right: Anti-Yo staining of Purkinje cell cytoplasm; the red nuclei in the granular layer stained by ethidium bromide Second level left & right: Anti-Ri immunoreactivity is identical to that of anti-Hu on neurons of the central nervous system. Unlike anti-Hu however, anti-Ri antibodies do not immunoreact with neurons of the peripheral nervous system. Third level left: Immunoreactivity of anti-amphiphysin (protein of nerve terminals) detected by immunofluorescence on cerebellum. Notice that the cytoplasm of Purkinje cells is negative.
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