Stiff-person syndrome: an atypical presentation and a review of the literature.

Introduction: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.Case report; In this report, we present a rare case of a 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS. Extensive laboratory and CSF studies were unrevealing. Electromyography showed significant peroneal motor neuropathy and complex repetitive discharges in the left tibialis anterior muscle. Antibodies to glutamic acid decarboxylase were significantly elevated at 124 units/mL. She was subsequently started on oral diazepam with significant improvement in her symptoms.Conclusion: The presentation of SPS can vary based on epidemiologic factors, clinical symptoms, and associated disorders. These forms can have overlapping features which may make the categorization of patients into one of these forms challenging.

Froin Syndrome, a Rare Complication of Multiple Myeloma.

INTRODUCTION: Froin syndrome is a rare condition that is defined as marked coagulability, elevated protein level, and xanthochromia of the cerebrospinal fluid (CSF). Froin syndrome more commonly occurs because of obstruction of CSF flow due to underlying inflammatory or neoplastic conditions. CASE REPORT: We present a case of a 38-year-old man who was found to have Froin syndrome a year after he was diagnosed with multiple myeloma (MM). CSF analysis, laboratory work-up, and magnetic resonance imaging of the neuroaxis supported the diagnosis of Froin syndrome related to leptomeningeal myelomatosis. To our knowledge, this is the first reported case of FS due to MM. CONCLUSION: Leptomeningeal myelomatosis associated with MM can cause blockage of CSF circulation leading to Froin syndrome. Inflammatory or neoplastic conditions should be considered as an underlying etiology.

Reversible Cerebral Vasoconstriction Syndrome Following Carotid Endarterectomy: A Case Report.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal intracranial arterial constriction and dilation that occurs spontaneously or as a result of a stimulant. The authors present a case of RCVS in a patient who presented with a new-onset thunderclap headache a day after carotid endarterectomy (CEA). RCVS has been rarely reported after CEA. CASE REPORT: A 65-year-old woman was evaluated for a new-onset thunderclap headache a day after left-sided CEA. Computed tomography (CT) of the head revealed left frontal and parietal subarachnoid hemorrhage (SAH). CT angiography did not show any saccular aneurysms or vessel stenosis. The initial impression was SAH related to reperfusion injury after carotid revascularization. Seven days postoperatively, the patient returned to the hospital with a persistent headache. CT revealed SAH in the vertex of the frontal region bilaterally. Magnetic resonance angiogram (MRA) of the head revealed multifocal stenosis of the intracranial circulation bilaterally. A follow-up MRA 9 weeks postoperatively showed interval improvement of the caliber of the circle of Willis branches and significant improvement of the multifocal stenosis. The patient was diagnosed with RCVS as a result of CEA. CONCLUSION: The authors advise clinicians to consider RCVS as a cause of thunderclap headache or recurrence of a severe headache shortly after CEA-particularly with the presence of a nonaneurysmal convexity SAH.

Safety and Efficacy of Flow Reversal in Acute and Elective Carotid Angioplasty and Stenting Using the Mo.Ma Device with Short-Term Follow-Up.

OBJECTIVE: To determine the safety and efficacy of flow reversal following proximal flow arrest as an embolic protection strategy for carotid angioplasty and stenting (CAS) with short-term follow-up. METHOD: We performed a retrospective review of our CAS database for patients who underwent stent-supported carotid revascularization in the setting of acute/subacute stroke or TIA. We reviewed clinical and radiographic data during a 36-month period. Primary outcome was clinical evidence of ipsilateral stroke in the first 30 days. Secondary outcomes include clinical outcomes and sonographic and/or angiographic follow-up over 6 months, 6-month functional scale, and all-cause mortality. RESULTS: Fifty-five patients underwent CAS using flow reversal: 26 females and 29 males with a mean age of 69.7 years. Median time to treatment from index event was 3 days. 11% underwent stenting as part of hyperacute stroke therapy. Average luminal stenosis was 86%. The 9-Fr Mo.Ma device was used in combination with Penumbra aspiration in all cases. There were no ipsilateral strokes. Incidence of any ischemic event was 3.64%, but only 1 (1.82%) patient had a postoperative stroke. Clinical follow-up was available for 94.5%, while lesion follow-up was available for 73% of patients. Three patients had evidence of restenosis, but none were symptomatic. Luminal restenosis was ≤30% in all three. Median pre- and post-NIHSS were 1 and 1, respectively. CONCLUSION: Flow reversal using the Mo.Ma device is a safe and effective strategy in preventing distal embolization during carotid artery revascularization.

Late-Onset Encephalopathy Associated With SARS-CoV-2 Infection.

Several typical and atypical neurological manifestations of viral pandemics have been reported. Neurological manifestations of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have recently been reported. In this case report, we present a patient with encephalopathy as a late neurologic manifestation of SARS-CoV-2 infection. The patient initially tested positive for the novel coronavirus after presenting with fever, cough, and altered mental status. The symptoms resolved within 5 - 7 days and the patient was discharged home. He subsequently developed worsening encephalopathy in the absence of respiratory symptoms, required hospitalization, and tested positive for SARS-CoV-2. Complete workup was unrevealing otherwise. We advise clinicians to be aware of late neurological manifestations of coronavirus disease 2019 (COVID-19) including encephalopathy.

Transient Capgras Syndrome Secondary to Bilateral Ischemic Stroke: A Case Report.

Capgras syndrome is one of a variety of delusional misidentification syndromes that can be associated with acute ischemic stroke, neurodegenerative disease, or metabolic conditions. Most cases reported in the literature are associated with frontal and/or parietal lobe involvement. Transient Capgras syndrome is rare but has been reported. We present a case of transient Capgras syndrome following bilateral cerebral ischemic infarcts in the frontal, parietal, and temporal regions, and involving the right prefrontal cortex. To our knowledge, transient Capgras syndrome with rapid resolution over a period of days is rare.

Patent Foramen Ovale Closure in the Setting of Cryptogenic Stroke: A Meta-Analysis of Five Randomized Trials.

BACKGROUND: The clinical benefit of patent foramen ovale (PFO) closure after cryptogenic stroke has been a topic of debate for decades. Recently, 3 randomized controlled trials of PFO closure in patients with cryptogenic stroke demonstrated a significantly reduced risk of recurrent stroke compared with standard medical therapy alone. This meta-analysis was performed to clarify the efficacy of PFO closure for future stroke prevention in this population. METHODS: A systematic literature search was undertaken. Published pooled data from 5 large randomized clinical trials (CLOSE, RESPECT, Gore REDUCE, CLOSURE I, and PC) were combined and then subsequently analyzed. Enrolled patients with cryptogenic stroke were assigned to receive standard medical care or to undergo endovascular PFO closure, with a primary outcome of reduction in stroke recurrence rate. Secondary outcomes included rates of transient ischemic attack (TIA), composite outcome of stroke, TIA, and death from all causes, and rates of atrial fibrillation events. RESULTS: We analyzed data for 3412 patients. Transcatheter PFO closure resulted in a statistically significant reduced rate of recurrent stroke, compared with medication alone. Patients undergoing closure were 58% less likely to have another stroke. The number needed to treat with PFO closure to reduce recurrent stroke for 1 patient was 40. CONCLUSIONS: Endovascular PFO closure was associated with a reduced risk of recurrent stroke in patients with a prior cryptogenic cerebral infarct. Although the absolute stroke reduction was small, these findings are clinically significant, given the young age of this patient population and the patients' lifetime risk of recurrent stroke.

Central Hypoventilation: A Rare Complication of Wallenberg Syndrome.

Central alveolar hypoventilation disorders denote conditions resulting from underlying neurologic disorders affecting the sensors, the central controller, or the integration of those signals leading to insufficient ventilation and reduction in partial pressures of oxygen. We report a patient who presented with a left lateral medullary ischemic stroke after aneurysm repair who subsequently developed a rare complication of CAH. Increased awareness of this condition's clinical manifestations is crucial to make an accurate diagnosis and understand its complications and prognosis.

Intracranial hemorrhage in the setting of posterior reversible encephalopathy syndrome: two case reports and a review.

Posterior reversible encephalopathy syndrome (PRES) is clinically characterized by seizures, changes in vision, altered mental status, and headache, with associated radiologic changes on brain imaging. Intraparenchymal hemorrhage is a rare complication of PRES and an atypical initial presentation of this condition. In this report, we discuss two patients who presented with multifocal cerebral hemorrhages that were later attributed to PRES. We further expand on the pathophysiology, management, and prognosis on patients with hemorrhagic PRES. Increased awareness of this complication of PRES is important in guiding prognostication and treatment.

A novel approach to the management of carotid blowout syndrome: the use of thrombin in a case of failed covered stenting.

Acute hemorrhage relating to an expanding pseudoaneurysm of the carotid artery is referred to as carotid blowout syndrome (CBS). CBS is associated with a high morbidity and mortality. We describe the case of a patient who presented with dysphagia and a pulsatile mass in the neck. Imaging revealed a pseudoaneurysm originating from the bifurcation of the distal right common carotid artery. On neuroangiography the patient lacked sufficient collaterals to allow for vessel sacrifice. A decision was made to use covered stents to prevent flow into the pseudoaneurysm while maintaining vessel patency. Despite placement of multiple covered stents there was residual slow filling of the pseudoaneurysm. We augmented this therapy with direct percutaneous thrombin injection into the pseudoaneurysm. This resulted in complete thrombosis of the pseudoaneurysm. For recalcitrant lesions in which the usual methods of stopping blood flow to the pseudoaneurysmal sac fail, an adjuvant approach with thrombin should be considered.

A novel approach to the management of carotid blowout syndrome: the use of thrombin in a case of failed covered stenting.

Acute hemorrhage relating to an expanding pseudoaneurysm of the carotid artery is referred to as carotid blowout syndrome (CBS). CBS is associated with a high morbidity and mortality. We describe the case of a patient who presented with dysphagia and a pulsatile mass in the neck. Imaging revealed a pseudoaneurysm originating from the bifurcation of the distal right common carotid artery. On neuroangiography the patient lacked sufficient collaterals to allow for vessel sacrifice. A decision was made to use covered stents to prevent flow into the pseudoaneurysm while maintaining vessel patency. Despite placement of multiple covered stents there was residual slow filling of the pseudoaneurysm. We augmented this therapy with direct percutaneous thrombin injection into the pseudoaneurysm. This resulted in complete thrombosis of the pseudoaneurysm. For recalcitrant lesions in which the usual methods of stopping blood flow to the pseudoaneurysmal sac fail, an adjuvant approach with thrombin should be considered.