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Erdheim‐Chester disease is a non‐Langerhans cell histiocytosis syndrome characterised by histiocytic infiltration of different organs and systems in the body. Erdheim‐Chester disease with isolated central nervous system (CNS) involvement causes diagnostic difficulties due to the absence of systemic findings and may result in misdiagnosis and inaccurate treatment choices. The case discussed in this report exemplifies how challenging it is to diagnose Erdheim‐Chester disease with isolated CNS involvement. This case, which presented with progressive pyramidocerebellar syndrome, was clinically and radiologically resistant to all immunosuppressive and immunomodulatory treatments administered. The presence of false negative results in repeated histopathological investigations and the absence of evidence for systemic disease hindered the diagnosis and treatment work‐up. In this study, we reviewed and discussed the prominent features of the presented case in light of the relevant literature. [ABSTRACT FROM AUTHOR]

Background: Patients with multiple sclerosis (MS) who discontinue fingolimod might present with rebound activity. The reasons for the development of rebound have been identified, but there are limited data on the long‐term clinical outcomes of these patients. This study aimed to compare the long‐term outcomes of patients with MS with and without rebound activity after fingolimod discontinuation. Methods: A total of 31 patients who discontinued fingolimod for various reasons with a minimum follow‐up of 5 years were included in the study. Of these, 10 were assigned to the rebound group and 21 to the non‐rebound group. Clinical and demographic data and 5‐year clinical outcomes of both groups were prospectively examined. Results: At fingolimod initiation, there were no significant differences in age, disease duration, and Expanded Disability Status Scale (EDSS) score. The annualized relapse rate (ARR) was significantly higher in the rebound group than in the non‐rebound group before the fingolimod treatment (p = 0.005). In the rebound group, EDSS scores 2 months after rebound treatment and at the 5‐year follow‐up were not significantly different than before fingolimod initiation (p = 0.14 and p = 0.46, respectively). The last recorded EDSS was significantly higher in the non‐rebound group than in the rebound group (3.6 ± 2.3 vs. 2.15 ± 1.4, p = 0.045). At the last follow‐up, one patient was diagnosed with secondary progressive multiple sclerosis in the rebound group (10%), and 11 patients were in the non‐rebound group (52.4%, p = 0.05). Conclusion: When rebound activity is well‐monitored and treated after fingolimod discontinuation, no overall EDSS change is expected in the long‐term follow‐up. [ABSTRACT FROM AUTHOR]

Alemtuzumab is one of the most effective agents in the treatment of multiple sclerosis (MS). On the other hand, the spectrum of possible side effects is wide and carries the potential to cause a variety of inflammatory and thrombotic complications. In this report firstly, a case who developed a systemic thrombotic and inflammatory response after alemtuzumab administration and then presented with pretibial edema in a period of 1 month is discussed. Then, based on the findings detected via this case, the possible side effects of alemtuzumab, both during drug infusion and in the early and late periods after administration, were classified. This classification was created by compiling the reported adverse effects in the literature related to the use of alemtuzumab for the treatment of multiple sclerosis. This presented case shows that prothrombotic predisposition after alemtuzumab administration may result in peripheral microvascular circulatory disorders even in the long term and one of them is pretibial edema. According to the current literature data, side effects of alemtuzumab treatment which is used for multiple sclerosis are categorized as follows: (i) infusion‐related reactions, (ii) acute/subacute inflammatory and pro‐thrombotic effects, (iii) immunosuppression‐related opportunistic infections, (iv) immune reconstitution‐related secondary autoimmune disorders. Increasing awareness of the possible side effects of alemtuzumab treatment used in the course of multiple sclerosis will enable both the development of possible prophylactic treatment options and the early control of emerging complications. [ABSTRACT FROM AUTHOR]

Background and purpose: COVID‐19 is a novel infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) in which neurological complications have been increasingly recognized. Acute symptomatic epileptic seizures and status epilepticus are frequently reported neurological complications associated with this infection. The nervous system damage caused by SARS‐CoV‐2 may be mediated by the immune system. Interleukin 6 (IL‐6), an important component of the cytokine storm, is directly correlated with the severity of symptoms. Tocilizumab is an inhibitor of IL‐6 receptors, which blocks IL‐6‐mediated signal transduction and is used in the treatment of COVID‐19 and status epilepticus. Case report: A patient with the Unverricht–Lundborg disease is presented who had developed refractory recurrent status epilepticus during COVID‐19 infection, which was finally controlled by treatment with tocilizumab. Discussion Tocilizumab, an IL‐6 inhibitor, may be considered as a treatment option in patients with status epilepticus and refractory seizures. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and distinctive imaging features of myelopathy associated with Behçet's disease (BD).Methods: We evaluated the records of patients meeting the following criteria: (1) fulfillment of the International Study Group criteria for BD; (2) clinically suggestive of myelopathy; (3) simultaneous spinal cord and brain magnetic resonance images (MRIs) within 1 month of acute worsening of myelopathy; and (4) follow-up duration ≥ 1 year after initial MRI evaluation. Patients not fulfilling all inclusion criteria and having MRIs with poor quality or missing sequences were excluded.Results: In 11 patients (9 men, 2 women), we studied 14 MRIs during distinct myelopathy episodes and nine follow-up MRIs. Two distinct MRI patterns of spinal cord involvement were described according to T2-weighted (T2W) axial images: (1) "Bagel Sign" pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and (2) "Motor Neuron" pattern: a symmetric involvement of the anterior horn cells. Bagel Sign was present in 13 of 14 myelopathy episodes whereas Motor Neuron pattern was observed in 1 of 14 MRIs. Of the 13 MRIs with Bagel Sign long myelopathy (n = 9), both long and short myelopathy (n = 2) and short myelopathy (n = 2) was observed. All patterns cleared with some residual lesions after steroid use and immunomodulation with associated clinical recovery in patients.Interpretation: The signal characteristics of the Bagel Sign potentially represent venous engorgement and/or acute blood products within the spinal cord. To our knowledge, Bagel Sign has not been observed in other forms of longitudinal myelopathy outside of BD, including neuromyelitis optica. Ann Neurol 2017;82:288-298. [ABSTRACT FROM AUTHOR]