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Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe thunderclap headaches and evidence of multifocal, segmental, reversible vasoconstrictions of the cerebral arteries. Several precipitating factors have been identified and reported, including the use of recreational substances or sympathomimetic drugs and the postpartum state. Case description: Here we present the case of a woman who developed RCVS after the administration of adrenaline (epinephrine) in the setting of an anaphylactic reaction during antibiotic allergy testing. Discussion: To our knowledge, this is the first reported case of RCVS following the administration of exogenous adrenaline. This case contributes to the understanding of the physiopathological mechanisms underlying reversible cerebral vasoconstriction.

Background: Tumefactive demyelinating lesions (TDL) share similar clinical features and magnetic resonance imaging (MRI) characteristics with high grade glioma (HGG). This study develops an approach to navigating this diagnostic dilemma, with significant treatment implications as the management of both entities is drastically different. Methods: A retrospective analysis of 41 TDLs and 91 HGG with respect to demographics, presentation and classical MRI characteristics was performed. A diagnostic pathway was then developed to help diagnose TDLs based on whole neuraxis MRI and cerebrospinal fluid (CSF) examination. Results: The diagnosis of TDL is more likely than HGG in younger females who present with subacute or chronic symptoms. MRI characteristics favoring TDL over HGG include smaller size, open rim enhancement, little or no associated edema or mass effect and the presence of a T2 hypointense rim. MRI of the whole neuraxis for detection of other lesions typical of multiple sclerosis (MS), in combination with a lumbar puncture (LP) showing positive CSF-specific oligoclonal bands (OCB), was positive in 90% of the TDL cohort. Conclusion: The diagnostic pathway, proposed on the basis of specific clinicoradiological features, should be followed in patients with suspected TDL. If MRI demonstrates other lesions typical of MS and LP demonstrates positive CSF-specific OCBs, then patients should undergo a short course of IV steroids to look for clinical improvement. Patients, who continue to deteriorate, do not demonstrate other lesions on MRI or where the LP is negative for CSF-specific OCB, should be considered for biopsy if safe to do so. This pathway will give the patients the best chance at neurological preservation.

ObjectivesTumefactive demyelinating lesions (TDLs) occur spontaneously during the course of MS or as the first manifestation of demyelination and may be precipitated by drug withdrawal, vaccination, viral infection or fingolimod. The objective of this study was to identify the frequency, clinical context and radiological features of TDLs in a hospital based cohort.MethodsThis is a retrospective analysis of 515 MS patients from the MS clinic at Royal North Shore Hospital. Lesions were regarded as tumefactive when larger than 2 cm in diameter. Clinical, laboratory and radiological data were collected. The lesions were characterised according to the radiological features and topography and correlated with the clinical presentation.ResultsForty-five (8%) patients had TDLs. More than half of TDLs exhibited ‘ring-like’ enhancement on MRI (58%). A ‘Balo-like’ pattern occurred in 24%. In 71% (32) of patients TDLs developed without a precipitant during the course of MS, and in 27% of patients (12) TDLs appeared in the context of a precipitating factor (prior vaccination, viral infection, treatment with fingolimod or cessation of medication due to pregnancy). In 20 (44%) patients the lesions were exclusive to the WM. The most frequent location of TDLs were the frontal lobes (56%), followed by parietal (53%) and temporal lobes (46%). In six patients TDLs in the cerebellum or brainstem were identified. Patients with multifocal, non-precipitated TDL’s exhibited the highest EDSS (mean 2.44).ConclusionsTumefactive MS is predominantly a white matter disease and lesions are frequently in the frontal lobes and exhibit ring-like enhancement. Curiously, TDLs occur most frequently during the course of otherwise prototypic MS and in this scenario may cause significant disability.

IntroductionNeurosarcoidosis is a rare disease with variable clinical and radiological manifestations. We report a case of longitudinally extensive transverse myelitis mimicking NMO that was pathologically confirmed to be sarcoidosis.Case a 50 year old Caucasian man presented with left arm paraesthesia, sensory and gait disturbance. Spinal MRI showed a swollen longitudinally extensive cord lesion (6 spinal segments) with peripheral enhancement. Brain MRI was normal. The CSF protein was 0.51 g/L and oligoclonal bands were restricted to the CSF. AQP-4 and MOG antibodies were negative. Serum ACE, vasculitis, and infectious screens were negative. The patient was administered a four day course of 1 g of iv methylprednisolone once daily, and improved clinically and radiologically. However, subsequent deterioration prompted treatment with plasma exchange (6 courses) and rituximab, which was ineffective. The peripheral nature of the enhancement pattern and steroid responsiveness prompted a whole body PET, showing FDG avid mediastinal lymph nodes, and a biopsy demonstrated non-necrotizing granulomas. Mycobacteria were absent. The diagnosis of sarcoidosis was made and the patient was initiated on intravenous infliximab (500 mg at 0, 2, 6 and 8 weeks). The patients gait and sensation improved.ConclusionNeurosarcoidosis should be included in the differential of longitudinally extensive transverse myelitis, particularly in AQP4-IgG seronegative patients.References1. Tavee JO, Stern BJ. Neurosarcoidosis. Continuum (Minneap Minn)2014Jun;20(3 Neurology of Systemic Disease):545–59.2. Sohn M, Culver DA, Judson MA, Scott TF, Tavee J, Nozaki K. Spinal cord neurosarcoidosis. Am J Med Sci2014Mar;347(3):195–8.3. Flanagan EP, Kaufmann TJ, Krecke KN, Aksamit AJ, Pittock SJ, Keegan BM, Giannini C, Weinshenker BG. Discriminating long myelitis of neuromyelitis optica from sarcoidosis. Ann Neurol2016Mar;79(3):437–47.