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Paraneoplastic cerebellar degeneration (PCD) is a rare immune mediated phenomenon often associated with cancer of the ovarian. Hitherto, tumor dissection is the mainstay in therapy while immunomodulatory treatment regimes often fail. Here we report on an 86 year old female patient who developed a severe pancerebellar syndrome. Clinical course, onconeural (anti-Yo) antibodies and detection of ovarian cancer suggest the assumption of PCD as the most probable diagnosis. We initiated a high-dose course of corticosteroids followed by a single dose of cyclophosphamide (600 mg/day). Surprisingly patient's condition improved and stabilized within days subsequent to cyclophosphamide application. This case demonstrates the benefit of immunosuppressive therapy in an anti-Yo positive patient with severe PCD secondary to an ovarian cancer.

Paraneoplastic cerebellar degeneration (PCD) is the most frequent paraneoplastic syndrome affecting the brain. Until now, anti-Tr associated PCD was only seen in patients with Hodgkin's disease. We report a male patient who presented with a progressive ataxia, affecting predominantly the lower limbs and a cerebellar dysarthria. Extensive diagnostic approach initially showed no evidence of tumor. The patient was found to have anti-Tr antibodies in his serum. Fourteen months after onset of symptoms a whole body PET-scan showed a pathological focus at the right hilus of the lungs. A mediastinoscopy was performed and peribronchial node sampling was done. The anatomopathological analysis revealed a non-well differentiated squamous cell carcinoma. This is the first report about the association between an anti-Tr associated PCD and squamous cell carcinoma.

We report the case of a patient with subacute cerebellar degeneration presenting as paraneoplastic syndrome, preceding the final diagnosis of breast cancer. The tumor had high HER2 overexpression, so a weekly regimen of paclitaxel/trastuzumab was started after surgery of the primary breast tumor. Negativity of specific antineuronal antibodies and clinical stabilization of the patient was achieved after 12 cycles of treatment. The interest of this case is the unusual presentation and the targeted therapeutic approach that has been used.

A 56 year old woman had a 19 month history of a severe subacute progressive cerebellar degeneration, peripheral sensory neuropathy, and urinary incontinence. She was confined to a wheelchair, needed assistance with eating, and her speech was almost unintelligible. No underlying cancer was found despite repeated investigations, and no autoantibodies were demonstrated. She received a 3-month course of intensive immunosuppressant therapy with intravenous immunoglobulin 400 mg/kg per day for 5 days every month, oral cyclophosphamide 50 mg twice or three times a day to maintain the total lymphocyte count between 500 and 750/mm(3), and prednisone 60 mg per day. She experienced dramatic subjective and objective improvement. The dysarthria and the upper extremity dysmetria disappeared, and she regained the ability to write and cook. The lower extremity ataxia improved and she became able to walk with a cane. Urinary incontinence disappeared. A trial of intensive immunosuppressant treatment is worth considering in a patient with a clinical syndrome resembling paraneoplastic disorders, even if an underlying neoplasm and autoantibodies are not demonstrated.