Neurological paraneoplastic syndromes

Common, occurring in up to 7% of cancer patients. Most common syndromes are:

peripheral neuropathy

♦ proximal myopathy

Other major syndromes are relatively rare. Can affect any part of the neurological system and most are thought to be secondary to autoimmune mechanisms via production of anti-tumour antibodies that cross-react with nervous tissue e.g. anti-Hu, anti-Yo, anti-Ri. Treatment is based upon treatment of the cancer and decreasing antibody production by immune system suppression e.g. corticosteroids, IV immunoglobulin, and plasma exchange. In contrast to endocrine syndromes, response to treatment is often poor (except for Lambert-Eaton myasthenic syndrome (LEMS)).

Peripheral neuropathy (PN)

Asymptomatic PN is common; symptomatic PN less so. Usually occurs after diagnosis of cancer has been made and caused by axonal degeneration or demyelination. Many types of PN e.g. motor, sensory, autonomic, sensorimotor.

Cancer types SCLC, myeloma, HD, breast, GI cancers Presentation Depends upon type and site.

Diagnosis Exclude non-paraneoplastic causes; nerve conduction studies, nerve biopsy—look for inflammatory infiltrates; serum anti-Hu antibodies in some cases. Treatment Corticosteroids; treat underlying cancer.

Encephalomyelopathies

Perivascular inflammation and selective neuronal degeneration at several levels of the nervous system. Can affect the limbic system, brainstem, and spinal cord.

Cancer types SCLC (75% of cases), breast, ovary, NHL. Presentation Slow, subacute onset; progressive

Diagnosis CSF—raised protein/IgG level, pleocytosis; serum— anti-Hu antibody; MRI. Treatment Anti-tumour therapy.

Paraneoplastic cerebellar degeneration (PCD)

Cancer types Breast, SCLC, ovary, Hodgkin's disease (HD). Presentation Rapid onset and progression; usually prior to cancer diagnosis; bilateral cerebellar signs; late diplopia and dementia. Diagnosis CT—cerebellar atrophy (late); serum auto-antibodies— anti-Yo, -Tr, and -Hu.

Treatment Response to anti-tumour treatment, steroids, plasma-pheresis.

Cancer-associated retinopathy (CAR)

Cancer types SCLC, breast, melanoma.

Presentation Visual defects i.e. blurred vision, episodic visual loss, impaired colour vision; leads to progressive painless visual loss; usually precedes cancer diagnosis.

Diagnosis Loss of acuity; scotomata; abnormal electroretinogram; anti-retinal ab's. Treatment Corticosteroids.

Lambert-Eaton myasthenic syndrome (LEMS)

Disorder of the neuro-muscular junction; reduced pre-synaptic calcium-dependent acetylcholine release. About 60% of patients with LEMS have underlying cancer.

Cancer types SCLC (60-70%), breast, thymus, GIT cancers. Presentation Proximal muscle weakness.

Diagnosis EMG—normal conduction velocity with low-amplitude compound muscle action potential that enhance to near normal following exercise.

Treatment Cancer treatment, corticosteroids, plasma exchange (high response rate).

Dermatomyositis/polymyositis

Inflammatory myopathies, often present prior to cancer diagnosis. Cancer types NSCLC, SCLC, breast, ovary, GIT cancers Presentation Proximal myopathy, skin changes, other systemic features; cardiopulmonary conditions, arthralgias, retinopathy. Diagnosis Serum—high CK, LDH, aldolase; muscle biopsy—myositis; EMG-fibrillation, insertion irritability, short polyphasic motor units.

Treatment Search for and treat tumour; corticosteroids, azathioprine.

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