Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma.

TY - JOUR

T1 - Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma.

AU - Johnson, Vijay

AU - Friedman, Norman

AU - Haller, Nairmeen Awad

AU - Hagel, Christian

PY - 2008

Y1 - 2008

N2 - OBJECTIVE: To describe a case of cerebellar ataxia associated with renal cell carcinoma. CASE REPORT: A 53-year-old Caucasian male with a history of Schizophrenia presented with generalized weakness, nausea, vomiting, severe weight loss, and progressively worsening gait difficulty associated with multiple falls. Physical examination revealed profound ataxia with inability to ambulate despite normal strength. The patient also appeared cachectic. A contrast-enhanced CT of the abdomen/pelvis showed a 10.4 x 8.3 cm(2) left renal mass. Histopathology of the tissue revealed morphology consistent with renal cell carcinoma. Paraneoplastic antibodies including anti-Hu, anti-Ma, anti-Ri, and anti-Yo were negative, however, biotinylated serum analysis was positive for hippocampal and cerebellar Purkinje cells. DISCUSSION: The pathogenesis of paraneoplastic neurological syndromes is believed to be associated with antibody and T-cell mediated response to antigens shared between the tumor and neural tissue. Though serum from this patient was negative for well-characterized antibodies, further testing revealed the presence of proteins binding to the hippocampal region of the midbrain and the cerebellum, and to components of the extracellular matrix of the tumor which may suggest partially characterized or as yet uncharacterized antibodies directed against renal cell tumor tissue and the nervous system. This possibility is supported by the observed symptom resolution upon tumor resection. The described case introduces a possible association between an antibody, which may be specific to paraneoplastic neurological syndromes, and renal cell carcinoma.

AB - OBJECTIVE: To describe a case of cerebellar ataxia associated with renal cell carcinoma. CASE REPORT: A 53-year-old Caucasian male with a history of Schizophrenia presented with generalized weakness, nausea, vomiting, severe weight loss, and progressively worsening gait difficulty associated with multiple falls. Physical examination revealed profound ataxia with inability to ambulate despite normal strength. The patient also appeared cachectic. A contrast-enhanced CT of the abdomen/pelvis showed a 10.4 x 8.3 cm(2) left renal mass. Histopathology of the tissue revealed morphology consistent with renal cell carcinoma. Paraneoplastic antibodies including anti-Hu, anti-Ma, anti-Ri, and anti-Yo were negative, however, biotinylated serum analysis was positive for hippocampal and cerebellar Purkinje cells. DISCUSSION: The pathogenesis of paraneoplastic neurological syndromes is believed to be associated with antibody and T-cell mediated response to antigens shared between the tumor and neural tissue. Though serum from this patient was negative for well-characterized antibodies, further testing revealed the presence of proteins binding to the hippocampal region of the midbrain and the cerebellum, and to components of the extracellular matrix of the tumor which may suggest partially characterized or as yet uncharacterized antibodies directed against renal cell tumor tissue and the nervous system. This possibility is supported by the observed symptom resolution upon tumor resection. The described case introduces a possible association between an antibody, which may be specific to paraneoplastic neurological syndromes, and renal cell carcinoma.

M3 - SCORING: Zeitschriftenaufsatz

VL - 90

SP - 279

EP - 281

JO - J NEURO-ONCOL

JF - J NEURO-ONCOL

SN - 0167-594X

IS - 3

M1 - 3

ER -