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Paraneoplastic cerebellar degeneration (PCD) has an association with tumors of the ♻️ovary and breast, small cell lung cancer and Hodgkin’s lymphoma, but has also been reported in association with many other types of tumors.
Paraneoplastic cerebellar degeneration is seen in about 20% of paraneoplastic neurological syndromes and typically
affects♻️ more females than males.The disease usually has an acute or subacute onset, ♻️with vertigo,dizziness, vomiting, and nausea, and evolves rapidly, over weeks to months, to gait ataxia, truncal and limb ataxia, dysarthria and nystagmus. Also, PCD usually reaches its peak and then stabilizes. Other signs and symptoms may be present, including♻️ dysphagia, brisk reflexes, hearing loss, extrapyramidal signs, peripheral neuropathy and cognitive impairment.
✔In patients with ♻️rapid onset ataxia (acute or subacute), clinicians should ♻️suspect a paraneoplastic disorder. Ataxia symptoms may be part of opsoclonus-myoclonus syndrome. ♻️In this syndrome, opsoclonus is associated with truncal ataxia,
diffuse or focal myoclonus, vertigo, dysarthria and encephalopathy.
In children, it may be associated with neuroblastoma or ganglioneuroma, and in adults with lung cancer or
ovarian teratoma. The disorder is paraneoplastic in about 30–40% of children♻️.
Brain MRI is usually normal,
particularly in the early course of the disease. Later, during the disease progression, diffuse cerebellar atrophy may occur. ♻️CSF usually contains a lymphocytic pleocytosis.
✔🔰Treatment is commonly unsuccessful, although an improvement or stabilization of neurological symptoms after surgical resection of the tumor may occur. Other treatment options are immunosuppressive therapies with corticosteroids, intravenous immunoglobulin, cyclophosphamide, tacrolimus, mycophenolate and, more recently, rituximab (anti-CD20).
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