Bilateral hearing loss preceding rhomboencephalitis - a hint for Kelch-like 11 syndrome.

BACKGROUND: Paraneoplastic neurological syndromes have diverse clinical presentations and offer an opportunity for early diagnosis of malignancy and treatment. Recently, a new paraneoplastic syndrome associated with seminoma was described, consisting of rhombencephalitis with antibodies targeting the Kelch-like protein 11 (KLHL11). Questions were raised as to the spectrum of clinical symptoms and strength of association to seminoma. METHODS: We present a 45-year-old man with bilateral sensorineural hearing loss, vertigo, and progressive ataxia. An extensive diagnostic workup led to the diagnosis of anti-KLHL11 paraneoplastic syndrome based on an immunofluorescence assay showing a typical pattern and a confirmatory serological assay. As a result, the patient underwent a meticulous search for an underlying seminoma. RESULTS: Although initially, all images were interpreted as negative, a revision of the positron emission tomography-CT (PET-CT) examination identified a small mediastinal suspicious mass. The mass was resected, and pathological examination confirmed it to be an extra-testicular seminoma. CONCLUSIONS: Patients presenting with progressive sensorineural hearing loss, vertigo, and ataxia should be evaluated for KLHL11 paraneoplastic syndrome. Furthermore, we support a strong association between anti-KLH11 rhombencephalitis and an underlying seminoma and recommend a thorough search for an undiagnosed germ cell tumor in these patients.

Erenumab-Induced Severe Nausea Leading to Smoking Cessation: A Retrospective Case Series.

OBJECTIVE: Erenumab is a novel treatment modality with a relatively benign and safe side effect profile, currently approved for the prevention of migraine headache. We present 3 cases with chronic migraine who are cigarette smokers were prescribed erenumab, and developed an intense smoking-induced nausea which eventually led to smoking cessation. METHODS: A multicenter retrospective review of 3 cases with cigarette smoking, one of whom was also smoking marijuana, suffering from chronic migraine resistant to multiple preventive therapies was studied. All were prescribed monthly injections of erenumab 70 mg. Response in terms of headache frequency and intensity and smoking habits was obtained through medical record review. RESULTS: Out of 3 patients, 2 reported reduced headache frequency and intensity. All patients developed severe nausea while smoking cigarettes after their first dose of erenumab, leading to smoking cessation. One patient co-smoked marijuana, which did not result in nausea after being treated. CONCLUSION: To the best of our knowledge, this is the first report of severe nausea secondary to erenumab administration and smoking cigarettes, which finally resulted in complete cigarette smoking cessation. As such, further study is indicated on the benefit of erenumab and other calcitonin gene-related peptide antagonists in migraineurs who smoke to promote smoking cessation.