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 :
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.
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.
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