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    ABSTRACT: Paraneoplastic cerebellar degeneration (PCD) is characterized by a subacute, severe pancerebellar syndrome, which is related to an underlying tumor. The presence of CSF or serum onconeural antibodies confirms the diagnosis and indicates the underlying tumor type. However, the association between PCD and extragonadal germ cell tumors with the absence of an onconeural antibody has rarely been described. We present a 55-year-old man who developed a pancerebellar syndrome, which made him unable to walk alone after 10months. Routine blood analysis, brain MRI and CSF examination were normal. Despite onconeural antibodies were negative, a whole body PET-CT scan showed a hypermetabolic nodule in the thymus, which was removed, and a hypometabolic presacral mass. The pathologic study revealed a germinoma surrounded by a pronounced inflammatory infiltrate. Shortly after, the patient clearly improved his symptoms, before receiving chemotherapy and prednisone. The cerebellar ataxia worsened when steroids were reduced below 30mg/day. Testicular sonography showed a suspicious lesion in one testicle, but no malignancy was found after orchiectomy. The presacral mass was removed after chemotherapy disclosing a mature teratoma. Our patient emphasizes that despite the absence of onconeural antibodies, studies to rule out an underlying tumor are mandatory in patients with subacute cerebellar ataxia.
    Journal of the neurological sciences 07/2012; 320(1-2):153-5. DOI:10.1016/j.jns.2012.06.021 · 2.47 Impact Factor
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    ABSTRACT: Paraneoplastic neurological syndromes (PNS) are rare remote effect of cancer. The antibodies and tumors associated with PNS have been well described, but there are still many clinically suspected cases in which no tumor or antibody can be identified. This is the first report of PNS showing hot cross-bun sign and caused by exceptionally rare underlying malignancy, such as burned-out testicular tumor. A 42-year-old man presented subacute progression of hearing loss and cerebellar ataxia. Cerebrospinal fluid showed continuous inflammation and magnetic resonance imaging (MRI) revealed cerebellar atrophy and hot cross-bun sign. Resection of tumors improved both laboratory findings and neurological signs and their pathology was seminoma. Seminoma can cause PNS showing 8th cranial nerve palsy, cerebellar, and brainstem atrophy with hot cross-bun sign on MRI study. Extensive screening for onconeural antibodies was negative and thereby suggested that unknown antibodies worked for both antitumor immunity and induction of PNS. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
    Acta Neurologica Scandinavica 08/2015; DOI:10.1111/ane.12469 · 2.40 Impact Factor

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