Paraneoplastic cerebellar degeneration


Paraneoplastic cerebellar degeneration is a rare disease which is mainly linked to cancers of the ovary, uterus, or adnexa, cancer of the breast or small-cell carcinoma of the lung.

The onset of symptoms is generally very rapid with limb and truncal ataxia, lack of coordination, dysarthria, and nystagmus. The patient is rapidly reduced to a severely disabled state.

Although cancer may respond to treatment, the neurological alterations in patients remain severe: the patient is unable to walk alone or even to sit. Some spontaneous remissions have been described.

This disease is related to auto-antibodies. Two major patterns have been described :

Anti-Yo antibodies

Found in women with cerebellar degeneration accompanying gynaecologic and breast malignancies, these antibodies recognise cytoplasmic proteins of Purkinje cells and can be found in serum and cerebrospinal fluid.

Anti-Hu antibodies

They are found in many tumours : small-cell lung cancers, neuroblastomas, sarcomas and prostate carcinoma. The anti-Hu antibodies react with proteins present in nuclei and cytoplasm of virtually all neurons.

References:

Darnell RB: The importance of defining the paraneoplastic neurologic disorders. N Engl J Med 1999 Jun 10; 340(23): 1831-3

See also: E-medicine

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