Paraneoplastic Syndromes
Definition: disorders caused by indirect damage from systemic cancer to central or peripheral nervous system; not due to metastasis.
Epidemiology: rare. Any cancer may be associated with paraneoplastic syndrome; most syndromes associated with specific cancer or particular group of cancer. Symptoms often appear before cancer is manifest.
Pathogenesis: presumably autoimmune; antibodies against tumor cross-react with neural components. Tumor-secreted hormones sometimes responsible.
Clinical Syndromes
Paraneoplastic cerebellar degeneration: ataxia of gait and limbs, dysarthria, nystagmus, oscillopsia. Antibodies:
anti-Yo (ovarian cancer), anti-Hu (small-cell lung cancer [SCLC]), anti-Tr or anti-GluR1 (Hodgkin disease), anti-Ri (breast cancer). Pathology: Purkinje cell loss.
Sensory neuronopathy: painful paresthesias, CSF pleocytosis, loss of sensory evoked potentials; normal motor function. Anti-Hu antibody (SCLC).
Limbic encephalitis: personality and mood changes, delirium, dementia; CSF pleocytosis; enhancing signal sometimes present on MRI. Anti-Ma2 (testicular cancer), anti-Hu antibody (SCLC).
Brainstem encephalitis: cranial nerve, basal ganglia dysfunction; spreads to diffuse brain disease.
Opsoclonus-myoclonus: constant irregular rapid motion of eyes in random direction, myoclonus; mostly children with neuroblastoma.
Myelitis: spinal cord symptoms evolve in days to weeks. CSF: pleocytosis, high protein content, normal sugar, oligoclonal bands sometimes.Related posts:
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