The Massachusetts General Hospital Handbook of Neurology

Authors: Flaherty, Alice W.; Rost, Natalia S.

Title: Massachusetts General Hospital Handbook of Neurology, The, 2nd Edition

Copyright 2007 Lippincott Williams & Wilkins

> Table of Contents > Adult Neurology > Paraneoplastic Syndromes

Paraneoplastic Syndromes

A. Incidence

Although rare (1% of cancer pts.), paraneoplastic syndromes precede the diagnosis of cancer in about 60% of cases.

Table 22. Paraneoplastic syndromes.

Syndrome Clinical Features Antibody Typical Cancer
Brainstem encephalitis Ataxia, cranial n. or motor dysfunction, abnl CSF Ma1, Ma2 Small-cell lung cancer (SCLC), testicular
Limbic encephalitis Depression, confusion, abnl CSF Hu, others SCLC
Subacute cerebellar degen. Ataxia, dysarthria, nystagmus, nl CSF Yo (AKA Purkinje cell) Breast, ovary; SCLC
Opsoclonus, myoclonus Jerky eye and limb movement Ri Lung, breast
Isolated CNS angiitis     Hodgkin's
Retinal degeneration Loss of vision Retinal SCLC, melanoma
Spinal Cord Sx      
Necrotizing myelopathy Weakness, sensory loss ? SCLC
Motor neuron disease Weakness, fasciculations ? Various lymphomas and carcinomas
Stiff person syndrome Painful spasms Amphiphysin, GAD Lung, lymphoma, breast, thymoma
Peripheral Nerve Sx      
Subacute sensory neurop. Sensory ataxia, gait instability, dysesthesias Hu, others SCLC, plasma cell dyscrasias
Gammopathy-assoc. neurop. Sensory loss, weakness, areflexia Monoclonal Ig Myeloma, Waldenstrom's, lymphoma
Guillain-Barr (AIDP) Rapid weakness, areflexia ? Lymphoma
Neuromuscular Junction Sx      
Lambert-Eaton Proximal weakness, areflexia, eyes spared Calcium channel SCLC (1%-3% of pts.)
Myasthenia gravis Weakness, areflexia, eyes often involved ACh receptor Thymoma (15% of pts.)
Muscle Sx      
Dermato- or polymyositis Weakness, high CPK Muscle Breast, lung, GI, uterine, ovarian, Hodgkin's

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