[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].

Abstract

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.

• Authors: Shigemi Nagayama, Fumihiko Koike, Tetsuo Sakai, Yasunobu Antoku, Motohiro Yukitake, Yasuo Kuroda
• Journal: Brain and nerve = Shinkei kenkyu no shinpo (Brain Nerve) Vol. 60 Issue 12 Pg. 1470-4 (Dec 2008) ISSN: 1881-6096 [Print] Japan
• PMID: 19110759 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Autoantibodies
• Biomarkers, Tumor
• Calcium Channels, P-Type
• Calcium Channels, Q-Type
• Peptide Fragments
• Recombinant Proteins
• pro-gastrin-releasing peptide (31-98)

Topics

• Autoantibodies (blood)
• Biomarkers, Tumor (blood)
• Brain Stem
• Calcium Channels, P-Type (immunology)
• Calcium Channels, Q-Type (immunology)
• Combined Modality Therapy
• Encephalitis (diagnosis, etiology, therapy)
• Humans
• Lambert-Eaton Myasthenic Syndrome (diagnosis, etiology, therapy)
• Lung Neoplasms (complications, diagnosis, immunology, therapy)
• Male
• Middle Aged
• Paraneoplastic Cerebellar Degeneration (diagnosis, etiology, therapy)
• Peptide Fragments (blood)
• Positron-Emission Tomography
• Recombinant Proteins (blood)
• Small Cell Lung Carcinoma (complications, diagnosis, immunology, therapy)
• Treatment Outcome