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The etiology of solid retroperitoneal mass may be autoimmune or neoplastic and should be investigated by imaging and histology. The spectrum of differential diagnoses includes retroperitoneal fibrosis and retroperitoneal tumors. As treatment for these entities differs substantially, early and accurate diagnosis is essential. We present a case of a 54-year-old woman admitted to our hospital with stroke-like symptoms. Suspecting vasculitis, magnetic resonance imaging of the head and abdomen was performed, which revealed circular enhancement of the internal carotid artery as well as retroperitoneal and periaortic masses. In light of the radiographic findings, an autoimmune process, such as retroperitoneal fibrosis, was hypothesized. Steroid treatment was initiated but did not lead to significant remission. Re-evaluation of the mass with fine-needle aspiration did not show malignant cells while diagnostic surgery and histological assessment revealed neoplastic lymphoproliferation. The final diagnosis was a non-Hodgkin B-cell lymphoma. Chemo- and immunotherapy were initiated. Follow-up abdominal computed tomography revealed significant remission of the retroperitoneal mass. Initially, the retroperitoneal mass was highly suspicious for RF. While imaging can be useful, obtaining histology should always be considered when there is an uncertain clinical presentation. Without histology, we would have missed a non-Hodgkin B-cell lymphoma in this case. Minimally invasive techniques such as fine-needle aspiration may be practical but can give false-negative results.

Background Tocilizumab (TCZ) has recently been approved for treatment of giant cell arteritis (GCA). Despite clinical, radiologic and serologic improvement in the majority of patients, it remains unclear whether inflammation of the vascular wall in patients with large vessel vasculitis may persist. Objectives To report 2 patients who were treated with TCZ for large vessel vasculitis involving the ascending aorta. The patients underwent aortic valve replacement in both cases and partial replacement of the ascending aortic arch in one case. Methods Following therapy with glucocorticosteroids (GC) and TCZ, replacement of the aortic valve and of the partial ascending aorta were performed, followed by a histopathologic examination of the surgical specimen. Results We report two female patients, aged 40 and 67 years, both diagnosed with large vessel vasculitis involving the aortic arch. Aortitis of the ascending aorta and significant aortic insufficiency were demonstrated in both patients by PET-CT, MR angiography and transesophageal cardiac ultrasound. In Pat 1, PET CT and MR angiography revealed vascular inflammation. Intravenous pulse GC was initiated, followed by oral prednisone with stepwise tapering and TCZ iv 8mg/kg/month. Two weeks after the 2nd infusion of TCZ, MR angiography showed significant reduction of gadolinium enhancement in the wall of the ascending aorta and the patient subsequently underwent valve replacement. Pat 2 was diagnosed with vasculitis of the aortic arch by MR angiography. Therapy with oral GC (1mg prednisone/kg) and methotrexate was initiated. After 12 months, the treatment was switched to TCZ 162mg sc once weekly because of inadequate control of disease. The patient was monitored clinically and by PET CT and GCA was considered well controlled over 4 years. Due to continuous enlargement of the ascending aorta resulting in significant aortic insufficiency, the patient underwent replacement of the aortic valve and the ascending aorta. Both patients showed normalization of CRP and ESR during TCZ treatment. In summary, despite PET-CT and MR angiography showing reduction in vascular wall inflammation before surgery and CRP and ESR normalized, both patients showed histologically persistent lymphoplasmacellular infiltrates (predominantly CD3+CD4+ T cells in patient 1) and, in addition, giant cells in pat 2. Conclusion Despite adequate control of large vessel vasculitis involving the ascending aorta clinically as well as by imaging, the 2 patients reported here had to undergo valve replacement which showed persistent inflammation of the aortic wall despite treatment with TCZ. Disclosure of interests Andrea Rubbert-Roth Consultant for: Chugai, Eli Lilly, Roche, and Sanofi, Speakers bureau: abbVie, Bristol-Myers Squibb, Chugai, Hexal/Novartis, Janssen, Eli Lilly, Merck Sharp & Dohme, Pfizer, Roche, and Sanofi, Thomas Langenegger: None declared, Peter Karl Bode: None declared, Claudia Pfofe: None declared, Olaf Chan-Hi Kim: None declared, Johannes von Kempis: None declared

Background:Giant cell arteritis (GCA) may affect the aorta and the large aortic branches and lead to dissections and aortic aneurysms. Tocilizumab (TCZ) treatment has the capacity to control aortic inflammation as has been demonstrated by CRP normalization and imaging data. However, limited data are available on the histopathological findings obtained from patients who underwent surgery because of aortic complications during TCZ treatment.Objectives:We report on 5 patients with aortitis who were treated with TCZ and developed aortic complications.Methods:We describe a retrospective case series of patients with GCA treated with TCZ, who presented in our clinic between 2011 and 2019. Three patients underwent surgery. Histopathologic examination was performed in specimen from all of them.Results:Five female patients were diagnosed with GCA (4/5) or Takaysu arteritis (1/5) involving the aorta, all them diagnosed by MR angiography and/or FDG PET CT scan. Three patients (one with aortic aneurysm, one with dissection) underwent surgery after having been treated with TCZ for seven weeks, nine months and four years, respectively. Imaging before surgery showed remission on MRI and/or PET-CT in all cases. At the time of surgery, all patients showed normalized CRP and ESR values. Histopathological evaluation of the aortic wall revealed infiltrates, consisting predominantly of CD3+CD4+ T cells. Enlargement of pre-existing aneuryms was observed in the other two patients 10 weeks and 4 months after discontinuation of TCZ, respectively. Both patients were not eligible for surgical intervention and died during follow-up.Conclusion:Our case series suggests that during treatment with TCZ, regular imaging is necessary in this patient population to detect development of structural changes such as aneurysms or dissections. Despite treatment, residual inflammation might persist which could contribute to eventual aortic complications.Disclosure of Interests:Andrea Rubbert-Roth Consultant of: Abbvie, BMS, Chugai, Pfizer, Roche, Janssen, Lilly, Sanofi, Amgen, Novartis, Peter Karl Bode: None declared, Thomas Langenegger: None declared, Claudia Pfofe: None declared, Thomas Neumann: None declared, Olaf Chan-Hi Kim: None declared, Johannes von Kempis Consultant of: Roche

Tularemia, a zoonotic disease caused by Francisella tularensis, can cause a broad spectrum of disease in humans including six major clinical presentations: the ulceroglandular, glandular, oculoglandular, oropharyngeal, typhoidal and pneumonic form. The epidemiology and ecology and thus transmission of tularemia are complex, depending on conditions unique to specific locations. Thirteen cases with different forms of the disease and one very rare case of a myocarditis are reported, discussed, and reviewed within the scope of current literature. Tularemia is a rare, but emerging disease in Central Europe with glandular and ulceroglandular disease as its predominant forms. Transmission is mainly caused by contact with lagomorphs, rodents and tick bites. However, domestic cats may play an important role in transmission too. Myocarditis is probably a worldwide, but very rare manifestation of tularemia.

Background Sudden onset of aphasia is usually due to stroke. Rapid diagnostic workup is necessary if reperfusion therapy is considered. Ictal aphasia is a rare condition but has to be excluded. Perfusion imaging may differentiate acute ischemia from other causes. In dubious cases, EEG is required but is time-consuming and laborious. We report a case where we considered de novo status epilepticus as a cause of aphasia without any lesion even at follow-up. A 62-year-old right-handed woman presented to the emergency department after nurses found her aphasic. She had undergone operative treatment of varicosis 3 days earlier. Apart from hypertension and obesity, no cardiovascular risk factors and no intake of medication other than paracetamol were reported. Neurological examination revealed global aphasia and right pronation in the upper extremity position test. Computed tomography with angiography and perfusion showed no abnormalities. Electroencephalogram performed after the CT scan showed left-sided slowing with high-voltage rhythmic 2/s delta waves but no clear ictal pattern. Intravenous lorazepam did improve EEG slightly, while aphasia did not change. Lumbar puncture was performed which likely excluded encephalitis. Magnetic resonance imaging showed cortical pathological diffusion imaging (restriction) and cortical hyperperfusion in the left parietal region. Intravenous anticonvulsant therapy under continuous EEG resolved neurological symptoms. The patient was kept on anticonvulsant therapy. Magnetic resonance imaging after 6 months showed no abnormalities along with no clinical abnormalities. Conclusions Magnetic resonance imaging findings were only subtle, and EEG was without clear ictal pattern, so the diagnosis of aphasic status remains with some uncertainty. However, status epilepticus can mimic stroke symptoms and has to be considered in patients with aphasia even when no previous stroke or structural lesions are detectable and EEG shows no epileptic discharges. Epileptic origin is favored when CT or MR imaging reveal no hypoperfusion. In this case, MRI was superior to CT in detecting hyperperfusion. This article is part of a Special Issue entitled “Status Epilepticus”.

Myokymia of the tongue is a very rare clinical condition and is much less common than facial or focal myokymia of the limbs. Radiation-induced delayed nerve damage is a well-known cause of myokymia, but other etiologies i.e. tumor recurrence should be considered as a differential diagnosis. We describe a case series of neurophysiologically proven unilateral tongue myokymia, which arose in two patients after radiotherapy of the neck/head and in one patient due to a space occupying meningioma of the cerebrospinal passage affecting the hypoglossal nerve. With this case series and a review of the literature we aim to raise clinical suspicion of tongue myokymia and highlight the clinical and electromyographic impact of myokymia in the diagnosis of malignancies and treatment-associated lesions of the hypoglossal nerve.

A 40-year-old asymptomatic female was referred for investigation of a systolic murmur. Transthoracic echocardiography revealed dilated right-sided cardiac chambers ( Panel A ). Transoesopageal echocardiography ruled out an atrial septal defect but demonstrated pulmonary venous flow …