A 62-year-old man presented with a 3-month history of progressive weakness in his limbs, anorexia, and weight loss. He reported smoking cigarettes and experiencing chronic lumbar pain and right leg paresis because of a past stroke. Neurological examination showed right facial droop involving the forehead, absent gag reflex, right VI cranial nerve paresis, paraparesis with hyperreflexia, and bilateral Babinski sign without sensory alteration or a sensory level. A cranial and spinal cord magnetic resonance imaging (MRI) with gadolinium revealed T2-weighted and fluid-attenuated inversion recovery (FLAIR) hyperintensities in the right frontoparietal white matter and centrum semiovale (Figure 1A). Abnormal hyperintensity was also present in the middle third of the corpus callosum, and it extended along the right pyramidal tract to the spinal cord, mainly at cervical and upper dorsal level (Figure 1B and Figure 2). Mild contrast enhancements were present in cranial and spinal cord lesions. A right pulmonary hilum tumor, paratracheal and precarinal mediastinal adenopathy, and several costal and pleural metastases were also identified by MRI. The biopsy results were compatible with small cell lung cancer. Examination of the cerebrospinal fluid (CSF) revealed elevated protein content of 0.13 g/dL (to convert to grams per liter, multiply by 10.0), mild pleocytosis (15 leukocytes/mm3, of which most were lymphocytes), and a normal glucose level. Glutamic acid decarboxylase 65 (GAD65) antibodies were detected in serum (1342 U/mL) and CSF (28 870 U/mL). The remaining onconeuronal antibodies (anti-Hu, anti-Yo, anti-Ri, anti-CV2, antibodies against paraneoplastic antigen Ma2 [anti-Ma2, also known as anti-Ta], amphiphysin, recoverin, Sry-like high mobility group box [anti-SOX1], titin, anti-Zic4, anti-Tr [target of Delta/notch-like epidermal growth factor-related receptor (DNER)]) were not present. An extensive CSF workup, including cytological testing and testing for oligoclonal IgG bands and anti–neuromyelitis optica antibodies, had negative results. The patient was diagnosed with definite paraneoplastic neurological syndrome based on the presence of paraneoplastic encephalomyelitis (PEM) and small cell lung cancer along with serum and CSF GAD65 antibodies. He received a 1-g bolus of methylprednisolone daily for 5 days, with partial neurological improvement. Because of the advanced stage of his neoplasia, 2 cycles of carboplatin-etoposide were performed as palliative chemotherapy. He died 4 months after diagnosis.
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Miralles O, Caballol N, Velasco R. Paraneoplastic Encephalomyelitis With Glutamic Acid Decarboxylase Antibodies Presenting as Longitudinal Pyramidal Tract Hyperintensity. JAMA Neurol. 2020;77(7):899–900. doi:10.1001/jamaneurol.2020.0940
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