title: A clinicopathological study of a patient with anti-Hu-associated paraneoplastic sensory neuronopathy with multiple cranial nerve palsies.
abstract: Only a few cases of paraneoplastic neurologic syndrome with multiple cranial palsies have been reported. This is the case report of a patient with small-cell lung cancer and a high titer of anti-Hu antibodies who developed a tonic left pupil and multiple cranial nerve palsies, including palsies of the left fifth through tenth nerves and both twelfth nerves, as in Garcin syndrome showing at least more than seven ipsilateral cranial nerve palsies, in the course of paraneoplastic sensory neuronopathy (PSN). Pathologic examination revealed no metastasis or direct invasion of malignancy with gliosis and perivascular inflammation throughout the brainstem, indicating paraneoplastic encephalomyelitis (PEM). The numbers of EBM11+ cells (probably reactive microglia), CD8+ cells, and CD4+ cells increased. Intracellular adhesion molecule-1 and lymphocyte function associated molecule-1 were expressed intensely on the endothelia of microvessels and were found to have infiltrated mononuclear cells around microvessels in the brainstem. Multiple cranial nerve palsies and their effects including the tonic pupil are likely due to the paraneoplastic effect of the primary systemic malignancy.
authors.affiliation: Third Department of Internal Medicine, Oita Medical University, Hasama 1-1, Oita 879-5593, Japan.
authors.forename: Shin
authors.initials: S
authors.lastname: Fujimoto
authors.forename: Toshihide
authors.initials: T
authors.lastname: Kumamoto
authors.forename: Tomoko
authors.initials: T
authors.lastname: Ito
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Sannomiya
authors.forename: Takashi
authors.initials: T
authors.lastname: Inuzuka
authors.forename: Tomiyasu
authors.initials: T
authors.lastname: Tsuda
mesh_terms: D000368:Aged; D018288:Carcinoma, Small Cell / Q000150:complications*; D003389:Cranial Nerve Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D051959:ELAV Proteins; D004679:Encephalomyelitis / Q000209:etiology / Q000473:pathology; D006801:Humans; D007249:Inflammation; D007963:Leukocytes, Mononuclear; D008175:Lung Neoplasms / Q000150:complications*; D008297:Male; D009419:Nerve Tissue Proteins / Q000032:analysis*; D020364:Paraneoplastic Polyneuropathy / Q000473:pathology*; D016601:RNA-Binding Proteins / Q000032:analysis*
chemical_list: D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins