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ObjectiveTo illustrate a case of a woman with rhombencephalitis with antibodies to Kelch-like protein-11 (KLHL11) and a metastatic carcinoma.BackgroundKLHL11 encephalitis is an autoimmune paraneoplastic syndrome first described in 2019. The clinical presentation consists of a brainstem cerebellar syndrome with symptoms of hearing loss, diplopia, vertigo and ataxia. This entity has been mostly described in male patients with associated testicular seminomas. Few cases have been described in women. This is a case of a woman with a history of hysterectomy and oophorectomy with KLHL11 encephalitis and an associated aortocaval tumor.Design/MethodsNA.ResultsThe patient was a 62-year-old woman that presented to clinic with a 9-month history of vertigo, progressive bilateral sensorineural hearing loss, diplopia, oscillopsia, ataxia and bilateral tremor. Her MRI brain obtained 8 months after symptom onset showed T2 hyperintense lesions in the inferior cerebellar hemispheres and right medial hippocampus with mild contrast enhancement in these areas. Cerebral spinal fluid analysis showed a lymphocytic pleocytosis, elevated protein, and negative infectious work-up. She completed 5 days of intravenous methylprednisolone and continued a steroid taper. She noted mild to moderate improvement in tremors, gait, and diplopia after steroids. Her symptoms, however worsened as she tapered her steroid dose. Serum KLHL11 antibody levels were positive at a titer of 1:7680. Computed tomography of the chest, abdomen and pelvis did not reveal any evidence of malignancy. However, whole body proton emission tomography/computed tomography (PET CT) revealed a large hypermetabolic aortocaval mass soft tissue mass. A biopsy of the mass showed pathology consistent with a metastatic carcinoma of gynecologic origin for which the patient is undergoing chemotherapy with plans for possible tumor debulking.ConclusionsThis case highlights the importance of considering KLHL-11 encephalitis in female patients presenting with rhombencephalitis, and the need for adequate malignancy evaluation in this disorder.

ObjectiveTo determine if initial treatment with corticosteroids plus steroid-sparing immunosuppressive therapy (IST) results in faster gadolinium contrast-enhancement resolution in neurosarcoid myelitis (NSM) than corticosteroid monotherapy (CSM).BackgroundNeurosarcoidosis is a rare cause of myelitis, treated initially with corticosteroids. Whether combination therapy of corticosteroids plus IST is more effective than CSM at disease onset is unknown.Design/MethodsWe retrospectively reviewed cases of definite or probable NSM, defined by Neurosarcoidosis Consortium Consensus Group, in adults treated at six United States centers. We characterized clinicoradiographic features and treatment outcomes after NSM diagnosis. Treatment groups were defined as CSM, corticosteroids plus intermediate oral IST (methotrexate or mycophenolate mofetil), corticosteroids plus highly effective IST (cyclophosphamide/TNF-alpha inhibitors), or corticosteroids plus other. We hypothesized that initial treatment with corticosteroids and IST would result in faster gadolinium contrast-enhancement resolution (primary end point).Results63 patients with NSM (32 female, 30 definite, median age 48) were identified. 86% had spinal cord enhancement on post-gadolinium T1 sequences (8% without enhancement, 6% without data). Time from symptom onset to treatment initiation varied from 11 days to 10 years (median 4 months). All but one patient received corticosteroids initially. 16/63 received corticosteroids alone. 29/63 received corticosteroids and subsequently IST (10 TNF-alpha inhibitor/cyclophosphamide, 10 mycophenolate/methotrexate, 9 other). Median time to IST initiation was 9.1 months (range 1–132). 16/63 were treated with initial IST (4 TNF-alpha/cyclophosphamide, 8 mycophenolate/methotrexate, 4 other). There was no significant difference in time to contrast-enhancement resolution between corticosteroids with/without subsequent IST versus corticosteroids + initial IST (Wilcoxon-rank sum test 0.93) or CSM versus corticosteroids + initial IST (Wilcoxon-rank sum test 0.97). Limitations are small sample size, variations in initiation of treatment and doses used, and intervals of clinical/radiographic follow-up.ConclusionsStandardization of reporting and monitoring of treatment and outcomes is needed for patients with NSM to better assess optimal initial therapy plans.