Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies

Shinichi Wada; Mayu Kamei; Naoko Uehara; Koji Tsuzaki; Toshiaki Hamano

doi:10.2169/internalmedicine.5934-20

Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies

Intern Med. 2021 May 15;60(10):1607-1610. doi: 10.2169/internalmedicine.5934-20. Epub 2020 Dec 15.

Authors

Shinichi Wada¹, Mayu Kamei¹, Naoko Uehara^{1

2}, Koji Tsuzaki^{1

2}, Toshiaki Hamano^{1

2}

Affiliations

¹ Department of Neurology, Kansai Electric Power Hospital, Japan.
² Division of Clinical Neurology, Kansai Electric Power Medical Research Institute, Japan.

Abstract

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.

Keywords: Lambert-Eaton myasthenic syndrome; SOX-1 antibodies; autopsy; paraneoplastic cerebellar degeneration; small cell lung carcinoma.

Publication types

Case Reports

MeSH terms

Aged
Autoantibodies
Humans
Lambert-Eaton Myasthenic Syndrome* / complications
Lambert-Eaton Myasthenic Syndrome* / diagnosis
Lung Neoplasms* / complications
Lung Neoplasms* / diagnosis
Male
Paraneoplastic Cerebellar Degeneration* / diagnosis
Small Cell Lung Carcinoma* / complications
Small Cell Lung Carcinoma* / diagnosis

Substances

Autoantibodies