[Case of anti P/Q type VGCC antibody positive small lung cell carcinoma that occured with subacute cerebellar degeneration, Lambert-Eaton myasthenic syndrome, and brainstem encephalitis].
A 62-year-old man was admitted to our hospital because of rapidly progressive dysarthria, truncal ataxia, and gait disturbance. High titers of the ProGRP and anti-P/Q-type VGCC antibody were detected in the serum. High accumulation of [18F] was detected at the hilus of the left lung on [18F]-FDG-PET scan. A high-frequency repetitive stimulation test of the median nerve yielded an incremental response. On the basis of these findings, a diagnosis of paraneoplastic cerebellar degeneration (PCD) and Lambert-Eaton myasthenic syndrome (LEMS) associated with small cell lung carcinoma (SCLC) was diagnosed. After intravenous immunoglobulin therapy (IVIg), methylprednisolone (m-PSL) pulse therapy, and other multidisciplinary concurrent treatments, a partial regression of the SCLC and a significant improvement in neurological symptoms were observed. However, ataxia relapsed and brainstem encephalitis developed 6 months later. A marginal improvement in neurological symptoms was observed with IVIg, m-PSL pulse therapy, and intravenous cyclophosphamide pulse therapy (IVCY). SCLC also recurred later. We hypothesized that VGCC of the brainstem was damaged by anti-P/Q-type VGCC antibody.
Nagayama
Shigemi
S
Division of Neurology, Yanagawa Rehabilitation Hospital, Japan.
Koike
Fumihiko
F
Sakai
Tetsuo
T
Antoku
Yasunobu
Y
Yukitake
Motohiro
M
Kuroda
Yasuo
Y
D001323:Autoantibodies / Q000097:blood*; D014408:Biomarkers, Tumor / Q000097:blood*; D001933:Brain Stem; D020865:Calcium Channels, P-Type / Q000276:immunology*; D020907:Calcium Channels, Q-Type / Q000276:immunology*; D003131:Combined Modality Therapy; D004660:Encephalitis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D020362:Paraneoplastic Cerebellar Degeneration / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D010446:Peptide Fragments / Q000097:blood; D049268:Positron-Emission Tomography; D011994:Recombinant Proteins / Q000097:blood; D055752:Small Cell Lung Carcinoma / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D016896:Treatment Outcome
D001323:Autoantibodies; D014408:Biomarkers, Tumor; D020865:Calcium Channels, P-Type; D020907:Calcium Channels, Q-Type; D010446:Peptide Fragments; D011994:Recombinant Proteins; C087079:pro-gastrin-releasing peptide (31-98)