Pellinen Supervision and revision of manuscript: T
Pellinen Supervision and revision of manuscript: T. some cases have reported antibodies to -amino-3-hydroxy-5-methylisoxazole-4-proprionic acid receptor, leucine-rich glioma inactivated 1 protein (LGI1), contactin associated protein 2 (Caspr2), and glutamic acid decarboxylase (GAD). A new entity has been explained in a few cases of paraneoplastic encephalitis associated with thymomas, with lesions extending beyond the mesial temporal lobe structures. CASE Statement A 35-year-old man presented with a 2-week history of seizures, poor appetite, generalized headaches and nausea. Neurologic assessment revealed poor attention, orientation and memory loss, without clinical seizures. Computed tomography head showed multiple cortical hypodensities, prompting an magnetic resonance imaging (MRI) brain, which revealed multiple foci of cortical hyperintensity involving the medial left frontal cortex, the right dorsal aspect of the insula and adjacent right temporal cortex as well as the posterior left temporal cortical medullary junction and the medial posterior left temporal cortex and lateral cortex [Physique 1]. Electroencephalogram revealed hyperexcitability over the right temporal region and frequent focal seizures and he was empirically treated with IV acyclovir, lacosamide and levetiracetam. Cerebral spinal fluid (CSF) analysis revealed protein of 0.51 g/L, glucose of 3.5 mmol/L, 3 10?6/L reddish blood cells and 9 10?6/L white blood cells (76% lymphocytes, 18% monocytes, 6% neutrophils). CSF and serum studies were also positive for neuronal antibodies to GAD [3.93 nmol/L in the CSF (normal 0.02 nmol/L), 250.0 AZD4547 kIU/L in the serum (reference range 0-5 kIU/L)], VGKC-complex antibodies [159 pmol/L in the CSF (reference range 0-31 pmol/L), 98 pmol/L in the serum (reference range 0-31 pmol/L)], CRMP5 (reflex titer 1:240; positive western blot), and AchR [binding antibody 17.5 nmol/L in the CSF (normal 0.02 nmol/L), and 142.8 nmol/L in the serum (reference range 0.0-0.4 nmol/L)]. Additionally, serum studies revealed presence of systemic antibodies to thyroperoxidase (37.8 kIU/L, reference range 0-5.5 kIU/L), thyroglobulin (372.2 kIU/L, reference range 0-5 kIU/L), Ro/SSA (6.8 AI, reference range 1 AI), and an ANA titer of 80 (reference range 0-40) with a nucleolar pattern. A paraneoplastic syndrome was suspected, and full body imaging revealed a large multilobulated right anterior mediastinal mass, interfacing multiple right-sided mediastinal structures and invading the bilateral lung apices. This mass was later confirmed to be a metastatic type B2, stage III thymoma via biopsy and after resection [Figure 2]. The patient was treated with intravenous methylprednisolone (1 g/day for 10 days) followed by 0.4 g/kg daily immunoglobulin (IVIG) for total of 4 days. He had significant improvement in his mental status with the first dose of steroids. Repeat imaging 2 weeks after initial presentation showed improvement in the cortical lesions, specifically, decrease in the abnormal fluid-attenuated inversion recovery (FLAIR) hyperintensities [Figure 1]. Further treatment included chemotherapy with cisplatin, doxorubicin, and cyclophosphamide given the invasive nature of the thymoma. Prior to thymomectomy, his course was complicated by gastrointestinal AZD4547 dysmotility syndrome thought to be a result of his paraneoplastic syndrome. He underwent a successful thymomectomy with complete resection approximately 3 months after initial presentation, and continued to improve thereafter. Open in a separate window Figure 1 Initial and follow-up magnetic resonance imaging brain scans showing multiple Rabbit Polyclonal to BORG3 cortically-based signal abnormalities. Images ACC are axial fluid-attenuated inversion recovery images showing multifocal elevated cortical T2/FLAIR signal intensity with associated swelling with involvement of the adjacent subcortical white matter from patients initial presentation. There AZD4547 was no contrast enhancement, and there was no definite involvement of the deep gray structures, brainstem, or cerebellum. Images DCF are follow up images approximately two weeks later that reveal overall improvement, for instance, previously seen abnormality along the medial left frontal lobe, and in the posterior right insular lesion improved Open in a separate window Figure 2 Computed tomography chest showing large right mediastinal mass. Contrast-enhanced computed tomography images (A: axial; B: coronal), showing a large multilobulated right anterior mediastinal mass measuring 6.0 cm 4.7 cm in transverse dimension by approximately 13 cm in craniocaudal dimension. There was found to be mass effect with mild compression of the right atrium and superior vena cava (though patent). Thymoma was suspected, and proven later on biopsy DISCUSSION Based on literature review, our patient is one of the very few reported cases of thymoma-associated panencephalitis (TAPE). These cases AZD4547 highlight the similar neurological symptoms at presentation.