Clinical features:
(1) The onset may be abrupt and rapidly progressive, or subacute and slowly progressive.
(2) After a period of time the clinical features may stabilize.
(3) The underlying malignancy may not be clinically evident for months or years.
(4) The findings may not improve with treatment of the underlying neoplasms, but rare patients reverse spontaneously.
Clinical signs and symptoms:
(1) truncal and limb ataxia
(2) gait ataxia
(3) titubation (stumbling or staggering gait with shaking of the head and trunk)
(4) dysarthria
(5) ocular findings: diplopia, oscillopsia, downbeating nystagmus, ocular dysmetria, opsoclonus
(6) vertigo
(7) occasionally myoclonus (with anti-Ri)
(8) severe disability, with an inability to stand, walk or sit
(9) cerebellar atrophy on imaging studies
Rare patients may have a pure autoimmune disorder and develop all of the signs and symptoms without evidence of neoplasia. An occult small cell carcinoma of the lung needs to be excluded before this diagnosis can be made.
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Autoantibody
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Associated Neoplasm
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anti-Hu (ANNA-1)
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small cell carcinoma of the lung, neuroblastoma, prostate cancer
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anti-Yo (PCA-1)
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cancers of ovary, breast, lung, esophagus
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anti-Tr
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Hodgkin's disease
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anti-CRMP5 (anti-CV2)
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small cell carcinoma of the lung, thymoma
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anti-PCA-2
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small cell carcinoma of the lung
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anti-Ma1
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lung cancer, other
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anti-mGluR1
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Hodgkin's disease
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anti-VGCC
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small cell lung cancer, GI tumors, thymoma, others
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anti-Ri (ANNA-2)
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cancer of breast, lung, urinary bladder, gynecologic tract
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where:
• Several antibodies (anti-Yo, anti-Tr, anti-PCA-2, anti-mGluR1) target the cytoplasm of Purkinje cells.
