Clinical, neurophysiological and serological clues for the diagnosis of neuromyotonia and distinction from cramp-fasciculation syndrome.

Neuromyotonia and cramp-fasciculation syndrome diagnosis currently relies on neurophysiological examination. In this study we investigated the clinical features and neural antibody profile of patients with neuromyotonia and cramp-fasciculation syndrome to assess the diagnostic value of serological testing. Available sera from adult patients with electromyography-defined neuromyotonia and cramp-fasciculation syndrome were tested for neural antibodies by indirect immunofluorescence on mouse brain sections and live cell-based assays. Forty patients were included, 14 with neuromyotonia and 26 with cramp-fasciculation syndrome. Neural antibodies were detected in 10/10 neuromyotonia sera, most commonly against contactin-associated protein 2 (7/10, 70%), and in 1/20 (5%) cramp-fasciculation syndrome sera. Clinical myokymia, hyperhidrosis, and paresthesia or neuropathic pain were more common in neuromyotonia and mostly associated with contactin-associated protein 2 antibodies. Central nervous system involvement was present in 4/14 (29%) neuromyotonia patients. A tumor was detected in 13/14 (93%) neuromyotonia patients (thymoma, 13), and in 4/26 (15%) with cramp-fasciculation syndrome (thymoma, 1; other neoplasms, 3). Twenty-one/27 (78%) patients achieved a significant improvement or complete remission. Our findings highlight clinical, neurophysiological and serological clues that can be useful in the diagnosis of neuromyotonia and cramp-fasciculation syndrome. Antibody testing is valuable for neuromyotonia diagnosis, while its usefulness in cramp-fasciculation syndrome confirmation is limited.

Clot evaluation and distal embolization risk during mechanical thrombectomy in anterior circulation stroke.

INTRODUCTION: Clot features along with patients' clinical features may influence thrombus compactness predisposing at distal embolization during thrombectomy. The aim of this study was to evaluate thrombus features assessed by radiological and histopathological analysis along with patient-related features to predict distal embolization during thrombectomy. METHODS: We performed a retrospective analysis of a prospectively maintained dataset of a tertiary stroke center inclusive of all cases of endovascular treatment for acute ischemic stroke involving anterior circulation occlusion. All patients underwent head and neck CT-angiography (CTA) at baseline. Patients were enrolled if thrombus material was suitable for histopathologic analyses. RESULTS: A total of 327 patients underwent mechanical thrombectomy between March 2017 and May 2020. Among them, 133 (40.7%) had thrombus material suitable for histopathological analysis but 11 patients were excluded due to posterior circulation occlusion. A total of 122 patients were included in the analysis. A distal embolism was documented in 27 patients (28.4%). Multivariable analysis with distal embolism as dependent variable showed an adjusted OR of 2.64 (95%CI: 0.9-7.73; p-value: 0.08) for anticoagulant therapy, an adjusted OR of 1.38 (95%CI: 1.01-1.91; p-value 0.05) each 5-mm increasing of thrombus length at CTA. No association was found with age, sex, thrombolysis and first thrombectomy technique used. CONCLUSION: The combined effect of anticoagulant therapy and thrombus length may have a potentially harmful effect on reperfusion during mechanical recanalization, causing distal embolization and this aspect should be taken into account in patient's risk assessment and when planning treatment strategy.

Systemic mastocytosis: The roles of histamine and its receptors in the central nervous system disorders.

Mastocytosis is a rare disease of clonal hematological disorders characterized by a pathological accumulation of Mast Cells (MCs) in different tissues, with variable symptomatology and prognosis. Signs and symptoms of Systemic Mastocytosis (SM) are due to pathological infiltration of MCs and to the release of chemical mediators, mainly histamine. Patients with SM may also present with neurological symptoms or complications. The pathophysiology of these neurological disorders remains uncertain to this day, but it can be associated with the infiltration of tissue mastocytes, release of mastocytes' mediators or both. Moreover, there is a lot to understand about the role of neurological symptoms in SM and knowing, for example, what is the real frequency of neurological disorders in SM and if is present a relation between other SM subtypes, because it has been noted that the alteration of the histamine expression may be an initiating factor for susceptibility, gravity and progression of the epigenetic disease. In this review we explain the possible pathophysiological mechanism about neurological symptomatology found in some patients affected by SM, describing the role of histamine and its receptors in the nervous system and, in light of the results, what the future prospects may be for a more specific course of treatment.

Effect of the COVID-19 pandemic and the lockdown measures on the local stroke network.

INTRODUCTION: The COVID-19 outbreak highly impacted the acute ischemic stroke care management. The primary end point of the study was to evaluate the impact of the COVID-19 outbreak and the following lockdown measures on our hub-and-spoke network; the secondary end point was to evaluate if the impact of the COVID-19 outbreak was different in hub-and-spoke centers. METHODS: This was a retrospective multicenter observational study conducted at the Stroke Units of Policlinico Gemelli, Ospedale San Filippo Neri, Ospedale di Belcolle, and Ospedale San Camillo de Lellis. We collected clinical reports of all consecutive patients admitted with diagnosis of acute ischemic stroke or transient ischemic attack (TIA) during the phase 1 of the lockdown period (11 March 2020-4 May 2020). As controls, we used all consecutive patients admitted for acute ischemic stroke or TIA in the same period of the previous year. RESULTS: A total of 156 and 142 clinical reports were collected in 2019 and 2020, respectively. During the COVID-19 outbreak, we observed a reduction of number of thrombolysis, a reduction of the length of hospitalization, and an increase of pneumonia. Regarding performance indicators, we observed an increase in onset-to-door time and in door-to-groin time. We did not observe any statistically significant interaction between year (2019 vs 2020) and facility of admission (hub vs spoke) on all variables analyzed. DISCUSSION: Our observational study, involving hub-and-spoke stroke network of a wide regional area, indicates that the COVID-19 outbreak impacted on the acute stroke management. This impact was equally observed in hub as well as in spoke centers.

Multidimensional assessment of cervical spondylotic myelopathy patients. Usefulness of a comprehensive score system.

OBJECTIVE: Cervical spondylotic myelopathy (CSM) is caused by cervical spine degeneration and surgery may be beneficial, but selection for surgery might be challenging. We performed a multimodal analysis to assess predicting factors that may be useful to help surgeons in this choice. PATIENTS AND METHODS: We retrospectively evaluated clinical, motor evoked potentials (MEP), and MRI data of patients who undergone surgery for CSM. Seventy-six consecutive patients (46 males) were enrolled. The median age was 65.5 [IQR: 57-71] years, and the duration of symptoms was 11 [8-13] months. A multivariate analysis in order to assess predictors of outcome and ROC curve analysis were performed. RESULTS: Thirty patients (M:18, 39.5%) gained 6 or more points on mJOA and they were collected in good recovery group, whereas 46 patients (60.5%, M:28) showed a fair recovery. We developed a comprehensive score system (CSS) taking into account clinical, neurophysiological, and neuroradiological data. ROC curve analysis was performed to determine the discriminative power of four models derived from the multivariate logistic regression analysis for predictors of good outcome considering only clinical variables, MRI variables, and MEP variables or considering the comprehensive model, demonstrating a good accuracy of CSS model to predict outcome. CONCLUSION: This study demonstrates that CSS model taking into consideration functional assessment by mJOA score, neurologic evaluation, cervical MRI, and MEP may be a feasible method to predict outcome in patients candidate to surgery, supporting surgeon's decisions both for those patients candidate to surgery and for patients in whom a "wait and see" approach could be proposed.

Predicting Factors of Functional Outcome in Patients with Acute Ischemic Stroke Admitted to Neuro-Intensive Care Unit-A Prospective Cohort Study.

Although thrombectomy is beneficial for most stroke patients with large vessel occlusion (LVO), it has added new issues in acute management due to intensive care support. In this prospective cohort study, we described the patients admitted to our neuro-intensive care unit (NICU) after thrombectomy in order to assess factors linked to functional outcomes. The outcome was independency assessed for stroke patients consecutively admitted to NICU for an ischemic stroke due to LVO of the anterior cerebral circulation that underwent intra-arterial mechanical thrombectomy (IAMT), either in combination with intravenous thrombolysis (IVT) in eligible patients or alone in patients with contraindications for IVT. Overall, 158 patients were enrolled. IVT (odds ratio (OR), 3.78; 95% confidence interval (CI), 1.20-11.90; p = 0.023) and early naso-gastric tube removal (OR, 3.32; 95% CI, 1.04-10.59 p = 0.042) were associated with good outcomes, whereas a high baseline National Institutes of Health Stroke Scale (NIHSS) score (OR, 0.72 for each point of increase; 95% CI, 0.61-0.85; p < 0.001) was a predictor of poor outcomes at 3 months. Older age (OR, 0.95 for each year of increase; 95% CI, 0.92-0.99; p = 0.020) and hemorrhagic transformation (OR, 0.31; 95% CI, 0.11-0.84; p = 0.022) were predictors of poor outcomes after IAMT, whereas a modified Treatment in Cerebral Infarction (mTICI) score of 2b/3 was a predictor of good outcomes (OR, 7.86; 95% CI, 1.65-37.39; p = 0.010) at 6 months. Our results show that acute stroke patients with LVO who require NICU management soon after IAMT may show specific clinical factors influencing short- and long-term neurologic independency.

The management of carotid restenosis: a comprehensive review.

Carotid artery stenosis (CS) is a major medical problem affecting approximately 10% of the general population 80 years or older and causes stroke in approximately 10% of all ischemic events. In patients with symptomatic, moderate-to-severe CS, carotid endarterectomy (CEA) and carotid angioplasty and stenting (CAS), has been used to lower the risk of stroke. In primary CS, CEA was found to be superior to best medical therapy (BMT) according to 3 large randomized controlled trials (RCT). Following CEA and CAS, restenosis remains an unsolved problem involving a large number of patients as the current treatment recommendations are not as clear as those for primary stenosis. Several studies have evaluated the risk of restenosis, reporting an incidence ranging from 5% to 22% after CEA and an in-stent restenosis (ISR) rate ranging from 2.7% to 33%. Treatment and optimal management of this disease process, however, is a matter of ongoing debate, and, given the dearth of level 1evidence for the management of these conditions, the relevant guidelines lack clarity. Moreover, the incidence rates of stroke and complications in patients with carotid stenosis are derived from studies that did not use contemporary techniques and materials. Rapidly changing guidelines, updated techniques, and materials, and modern medical treatments make actual incidence rates barely comparable to previous ones. For these reasons, RCTs are critical for determining whether these patients should be treated with more aggressive treatments additional to BMT and identifying those patients indicated for surgical or endovascular treatments. This review summarizes the current evidence and controversies concerning the risks, causes, current treatment options, and prognoses in patients with restenosis after CEA or CAS.

May endovascular thrombectomy without CT perfusion improve clinical outcome?

BACKGROUND: DAWN and DEFUSE-3 trials demonstrated the benefit of endovascular thrombectomy in late-presenting acute ischemic strokes due to anterior circulation large vessel occlusion. The aim of our study is to evaluate results of endovascular thrombectomy in large intracranial vessel occlusion without perfusion CT patient selection. METHODS: we reviewed our prospectively collected endovascular databases for patients with an acute stroke from March 2016 to October 2018, treated after 6 h from stroke onset, without perfusion CT selection. Baseline characteristics, procedural data, and outcomes were evaluated. A good outcome was defined as a 90-day modified Rankin Scale score of 0-2. The association between clinical and procedural parameters and functional outcome was assessed. RESULTS: out of 212 patients 55 were treated after 6 h from stroke onset, 49 of which for an anterior circulation occlusion. 18/49 were functional independent at 90 days (mRS 0-2), Successful recanalization (mTICI 2b to 3) was achieved in 38/49 patients (77 %). Multivariate logistic regression indicated that a low baseline NIHSS was associated with favorable outcome (OR 0.66, 95 % CI 0.52-0.83, p-value 0.001). CONCLUSIONS: in our retrospective analysis, baseline NIHSS is the only parameter that can predict good outcome (90-days mRS 0-2). We confirm data from recent papers assessing that perfusion CT can provide a better patients' selection compared to mCTA for large vessels occlusion treated beyond six hours from symptom onset.

Stroke priorities during COVID-19 outbreak: acting both fast and safe.

While the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) spreads all over the world, the healthcare systems are facing the dramatic challenge of simultaneously fight against the outbreak and life-threating emergencies. In this biological setting, emergency departments and neurovascular teams are exposed to high risk of infection and should therefore be prepared to deal with neurological emergencies safely. The purpose of this article is to analyze the current evidence on COVID-19 in the context of acute ischemic stroke and to describe the model of behavior we are putting into action to maintain the stroke pathway both rapid for the patient and safe for the healthcare professionals. We reserve a specific focus on personal protection equipment, dress code and healthcare professional behavior.

Reversible Cerebral Vasoconstriction Syndrome: A Severe Neurological Complication in Postpartum Period.

A 38-year-old woman 12 days after delivery of her second pregnancy was admitted to emergency room for a severe occipital headache started 3 days before, associated with confusion, nausea, vomiting and walking impairment. Neurological examination showed left hemiparesis, hypoesthesia in left arm and leg. Brain computer tomography images showed a large intraparenchymal hematoma in the right frontoparietal lobes with mass effect on adjacent subarachnoid spaces and on lateral ventricle. The third day during hospitalization, the patient experienced a sudden worsening of the neurological symptoms and a severe headache peaking within 1 minute (min); a new brain computed tomography and brain magnetic revealed another small intraparenchymal hematoma in the left parietal lobe with increase of the amount of subarachnoid hemorrhage. Digital subtraction angiography discloses multifocal narrowing of the middle and small arteries in both anterior and posterior circulation with a relative spare of large vessels. Postpartum reversible cerebral vasoconstriction syndrome with intracranial hemorrhage is a rare clinical condition that can be misdiagnosed with other dramatic neurological diseases such as eclamptic encephalopathy, cortical venous thrombosis, primary angiitis of the central nervous system or posterior reversible encephalopathy syndrome with whom may share predisposing factors and neurological presentation but clinical course, treatment and prognosis is quite different and emergency physicians and neurologists should consider this diagnosis in postpartum patients with hemorrhage.

Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome: Clinical and Radiological Considerations.

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are relatively uncommon neurological disorders, but their detection has been increasing mainly due to clinical awareness and spreading of magnetic resonance imaging (MRI). Because these syndromes share some common clinical and radiologic features and occasionally occur in the same patient, misdiagnosis may occur. PRES is characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficit, confusion, disorders of consciousness, seizures, and motor deficits often associated to peculiar neuroradiological pattern even if uncommon localization and ischemic or hemorrhagic lesions were described. RCVS is a group of diseases typically associated with severe headaches and reversible segmental vasoconstriction of cerebral arteries, often complicated by ischemic or hemorrhagic stroke. Pathophysiological basis of PRES and RCVS are still debated but, because they share some risk factors and clinical features, a possible common origin has been supposed. Clinical course is usually self-limiting, but prognosis may fluctuate from complete recovery to death due to complications of ischemic stroke or intracranial hemorrhage. Neuroradiological techniques such as digital angiography and MRI are helpful in the diagnostic pathway and a possible prognostic role of MRI has been suggested. This review will serve to summarize clinical, neuroradiological features and controversies underlying both syndromes that may mislead the diagnostic pathway and their possible relationship with pathophysiology, clinical course, and prognosis.

Acute radiological pattern and outcome in posterior reversible encephalopathy syndrome patients.

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of acute or subacute onset characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficits, confusion, disorders of consciousness, seizures, and motor neurological deficits. Even if recognition of severe forms of PRES has improved, mainly due to magnetic resonance imaging, pathogenesis is still unclear and management of these patients remains challenging. Moreover, prognosis is unpredictable varying from complete recovery to death and factors related to prognosis are still lacking. We studied early magnetic resonance imaging characteristics and their relationships with prognosis. PATIENTS AND METHODS: We performed a retrospective analysis in patients with clinical and neuroradiological charateristics of PRES performing magnetic resonance of the brain within 2 days of symptoms onset. RESULTS: After reviewing site database of magnetic resonance imaging and clinical records compatible with PRES, 157 patients were selected. After imaging reviewing, 25 patients with clinical and neuroradiological diagnosis of PRES were enrolled, 22 (88%) females. Mean age of enrolled patients at presentation was 44.4+18.4 years (range, 21-84 years). Patients were classified according to neuroradiological characteristics such as ischemic lesions, distribution and severity of edema, hemorrhage and contrast enhancement. In our group 23 patients (92%) showed an almost complete recovery but 2 patients (8%) died during hospitalization. Outcome was significantly related with hypointensity on ADC (p = 0.002) and CE (p < 0.001). CONCLUSION: Early MR features may be helpful in suggesting prognosis. Moreover, neuroimaging at the early stage of PRES may give new insights in pathophysiological mechanisms underlying brain damage and neurological impairment.

A Puzzling Case of Cryptogenic Stroke.

BACKGROUND: Stroke is a common neurological complication of infective endocarditis (IE) and it is associated with increased morbidity and mortality but infective endocarditis in acute stroke setting is hard to discover. MATERIAL AND METHODS: A 75-year-old man referred to hospital for the onset of left hemiparesis and dysarthria. His past medical history included hypertension. He had 3 months history of fatigue, fever, and weight loss. Neurological examination revealed left hemiparesis and dysarthria. FINDINGS: Brain CT and CT angiography revealed a right M1 segment occlusion. Thrombolysis was delivered followed by mechanical thrombectomy by clot aspiration and recanalization was achieved. Anatomopathological analysis of the clot showed necrotic material and bacterial colonies consistent with septic emboli. The day after he developed fever and brain CT revealed a right parieto-occipital intraparenchymal and subarachnoid hemorrhage. Blood cultures demonstrated growth of Enterococcus faecalis. Treatment with vancomycin and ampicillin was started. CONCLUSION: Management of acute ischemic stroke related to IE is difficult. The great clinical challenge for the physician is recognizing the signs suggestive of IE in the acute stroke setting. Anatomo-pathological and bacteriological analysis of the clot in patients eligible to mechanical thrombectomy can provide the remarkable advantage to analyse directly the extracted material, allowing an early diagnosis and appropriate antibiotic therapies and treatments.

Pulmonary embolism in a stroke patient after systemic thrombolysis: clinical decisions and literature review.

Pulmonary embolism can be a catastrophic event that can result in early death or serious hemodynamic dysfunction. The dehydration, immobility, and infections occurring in acute stroke patients puts these patients at risk of developing deep vein thrombosis and pulmonary embolism. Recombinant tissue-type plasminogen activator (rt-PA) is the established therapy for acute ischemic stroke, and its prompt administration results in a better outcome in stroke patients. We describe a 73-year-old man who arrived at the emergency room within 2 hours of acute onset of left hemiparesis who was treated with rt-PA and suffered a pulmonary embolism 3 days after acute stroke therapy. rt-PA is also a current therapy for pulmonary embolism, but an ischemic stroke in the previous 3 months is an absolute contraindication to thrombolysis because of the high risk of intracranial hemorrhage. We discuss clinical and therapeutic decisions and review the current literature.