Kushak Suchdev

Fulminant Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is increasingly being recognized as a diagnosis in patients presenting with thunderclap headaches. In the vast majority of the cases, the syndrome follows a benign course and patients recover completely without any significant therapeutic intervention. In the rarest of cases, RCVS follows a monophasic course with rapid worsening, severe neurological deficits, and poor outcomes. We present the case of a 25-year-old female who presented with headaches which were worsening over 1 week. She was admitted to the hospital and rapidly worsened to develop severe neurological deficits over the next day. Initial computed tomography scan showed areas of hemorrhage and multiple ischemic strokes. Computed tomography angiogram and a conventional cerebral angiogram both revealed multifocal vasoconstriction, highly suggestive of RCVS. Despite aggressive medical and surgical management, the patient continued to worsen and eventually died. Autopsy findings did not show evidence of vasculopathy or any other underlying disorder, further supporting the diagnosis of RCVS. The RCVS is usually a benign self-remitting condition which commonly affects young females and presents with an insidious onset of headaches. Rarely, it can have a fulminant course with devastating outcomes. This case illustrates an exceptionally uncommon clinical course of RCVS and the challenges in its treatment.

Updates in Refractory Status Epilepticus

Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status.

Late onset neuromyelitis optica mimicking an acute stroke in an elderly patient

Neuromyelitis optica spectrum disorder (NMOSD) was recently proposed as a unifying term for Neuromyelitis optica (NMO) and related syndromes to incorporate patients with NMO antibody but without the full clinical spectrum. NMO is a rare, demyelinating condition which predominantly affects females with a peak incidence in the third and fourth decade of life. We report a case of NMOSD in an elderly patient with extremely late onset (>80years) of disease. The patient presented with findings of sudden onset unilateral symptoms, which is extremely unusual for NMOSD. She had frequent relapses which were treated with high dose steroids and plasmapheresis but has not had any relapse since being started on immunosuppressive therapy and continues to do well. Our case also highlights the varied clinical presentations of NMOSD and we believe that the diagnosis of NMOSD should be considered in elderly patients in the appropriate clinical setting despite the presence of unconventional symptoms.

Alpha 3 ganglionic acetylcholine receptor antibody associated refractory status epilepticus

Autoimmune encephalitides are a group of syndromes that may present with subacute onset of disorientation, personality changes, memory loss and most prominently seizures. Earlier reports of this entity were from patients with paraneoplastic limbic encephalitis, however autoimmune etiologies have also been described in patients with non-paraneoplastic encephalitis [1]. The Alpha 3 ganglionic acetylcholine receptor autoantibody (a3-AChR Ab) mainly causes autoimmune dysautonomia that is either subacute or insidious in onset. A direct relationship betweenantibody titer and severity of dysautonomia occurs in both experimental animals and patients [2]. Most of these cases have been paraneoplastic (mainly adenocarcinomas) however a few cases have been linked to other autoimmune disorders such as systemic lupus erythematosus (SLE) and Sjogren’s syndrome. Patients who are seropositive for a3-AChR Ab may present with less common neurological manifestations such as peripheral neuropathies, encephalopathy and subacute neuropsychiatric presentations. This report presents a unique case of refractory new-onset status epilepticus associated with a3-AChR antibody which responded to immunotherapy.

New Developments in Hypertensive Encephalopathy.

Purpose of ReviewThis review summarizes the latest science on hypertensive encephalopathy and posterior reversible encephalopathy syndrome (PRES). We review the epidemiology and pathophysiology of these overlapping syndromes and discuss best practices for diagnosis and management.Recent FindingsDiagnosis of hypertensive encephalopathy largely relies on exclusion of other neurological emergencies. We review the extensive causes of PRES and its imaging characteristics. Management strategies have not changed substantially in the past decade, though newer calcium channel blockers simplify the approach to blood pressure reduction. While this alone may be sufficient for treatment of hypertensive encephalopathy in most cases, management of PRES also depends on modification of other precipitating factors.SummaryHypertensive encephalopathy and PRES are overlapping disorders for which intensive blood pressure lowering is critical. Further research is indicated to both in diagnosis and additional management strategies for these critical conditions.

Cerebral Abscess following Mechanical Thrombectomy for Ischemic Stroke: Report of a Case and Review of Literature

Cerebral infections have been reported after endovascular interventions such as embolization and coiling. Such complications are extremely rare and only one other case has been reported in a patient who underwent an endovascular therapy for ischemic stroke. We report a 32-year-old woman, who presented to our hospital with headaches lasting four weeks after an endovascular intervention for ischemic stroke via mechanical thrombectomy. Further investigations revealed a cerebral abscess in the area of the infarct. She was effectively treated with antibiotics in combination with stereotactic drainage and was discharged after she made a good recovery. A review of literature on cerebral abscesses after minimally invasive procedures such as endovascular intervention was also done and is being presented in this paper. A cerebral abscess can occur rarely after endovascular interventions. A high degree of suspicion is important in identifying patients with an abscess and appropriate treatment can prevent significant morbidity or even death.

Apixaban for the treatment of cerebral venous thrombosis: A case series

BACKGROUND Venous thrombosis affecting cerebral veins and sinuses (CVT) is an uncommon neurological condition. Traditionally patients are treated with intravenous heparin followed by an oral vitamin K antagonist like warfarin. Direct oral anticoagulants (DOACs) may offer advantages over warfarin. There is evidence to demonstrate the effectiveness of both dabigatran and rivaroxaban. No data, however, has been published describing the use of apixaban in patients with CVT. METHODS Report of three cases of CVT and review literature on available treatment options; efficacy and safety of novel oral anticoagulants in patients with systemic thrombosis. RESULTS All patients presented with typical features of CVT. After confirming the diagnosis, they were acutely treated with heparin and later discharged on apixaban. During follow up visits, they tolerated apixaban well and did not have any bleeding complications. Follow up scans showed resolution of the thrombus and recanalization. CONCLUSION CVT is an uncommon neurological condition and is often complicated by associated intraparenchymal hemorrhage. Although not recommended in current guidelines, apixaban may be a safe and effective option for the treatment of CVT.