Focal Status Epilepticus and Extreme Delta Brush Associated With Thrombotic Thrombocytopenic Purpura.

Acute focal neurological deficits demand immediate evaluation. In this report, we present the case of a woman 20-some years of age with a history of hemolytic anemia and thrombocytopenia who presented with altered mental status and focal neurological deficits including aphasia, acute left gaze preference, right homonymous hemianopsia, right lower facial weakness, and right arm and leg weakness. Extensive neurological and hematological workup revealed that the patient suffered from focal status epilepticus associated with an extreme delta brush patten on electroencephalogram, likely secondary to thrombotic thrombocytopenic purpura. This case underscores the connection between hematological disorders and the neurological axis, emphasizing the critical role of integrating the neurological examination and neuroimaging findings to formulate an effective management plan.

Cervical Radiculopathy Presenting as Ischemic Stroke After Carotid Artery Stent Placement.

Abrupt loss of focal brain function is the main characteristic of the beginning of ischemic stroke. However, individuals with disorders other than stroke can also present with similar features. These conditions include seizure disorders, migraine, central nervous system abscess or tumor, head trauma, subdural hematoma, cerebral venous thrombosis, viral encephalitis, conversion reaction, hypertensive encephalopathy, multiple sclerosis, and spinal cord disorder. An 82-year-old man presented with a sudden onset of numbness in his left forearm in the distribution of C6 and C7 spinal nerves, 2 days after undergoing endarterectomy and stent placement in his right carotid artery because of stenosis. He was diagnosed with hypo-pharyngeal squamous cell carcinoma (T1, L0, M0) 17 years earlier (2006) which was treated with 70 Gy intensity-modulated radiotherapy (IMRT). The patient underwent stent insertion into his left carotid artery 3.5 years earlier because of 80% carotid artery stenosis. He was initially suspected to have an ischemic stroke. However, computed tomography angiography of the head and neck did not show stenosis or occlusion of the major intracranial arteries and no aneurysms were identified. It showed interval stenting of the cervical portion of the right carotid artery and stable appearance of left carotid artery stent. Both carotid artery stents and the vertebral arteries were patent. The cervical spine showed bilateral moderate to severe foramen stenosis in C3-C4 and C5-C6, and moderate to severe stenosis in the right C2-C3 and left C4-C5. His symptoms subsided after performing neck extension exercises. This is the first report of a patient whose cervical radiculopathy symptoms were suspected to be caused by ischemic stroke. The recent angioplasty and stent placement in the right carotid artery made the association more likely and had to be excluded. Clinicians should be aware that cervical radiculopathy could present as ischemic stroke. It is therefore important that disorders that cause symptoms similar to ischemic stroke are also considered in these individuals.

Emerging Subspecialties in Neurology: Interventional Neurology: A Starter Guide for Neurology Residents.

Interventional neurology is a subspecialty at the cross-section of neurology, neurosurgery, and neuroradiology that uses image-guided endovascular catheter techniques to diagnose and treat neurovascular disorders. Although interventional neurology techniques have existed for decades, recent landmark trials in stroke thrombectomy have catalyzed dramatic changes to the interventional neurology field, and there is rising neurology interest and representation. Unlike other neurology subspecialties, however, interventional neurology training and fellowship applications are not standardized. Thus, it can be difficult for neurology trainees to navigate this rapidly changing and multidisciplinary field. This article seeks to provide neurology residents and residency program directors with a brief overview of the modern interventional neurology field, the current fellowship application process, and tips on how neurologists can best prepare for a career in interventional neurology. To this end, we conducted a nationwide survey of interventional neurology fellowship program directors regarding training neurologists. We distill survey responses into concrete, actionable items for neurology trainees.

Correction of carotid artery stenosis by stent placement ameliorated paroxysmal hypertension after radiation treatment of hypopharyngeal carcinoma: a case report.

BACKGROUND: Paroxysmal hypertension can be associated with failure of the carotid artery baroreceptors due to past exposure to radiation treatment. This report describes a patient whose repeated paroxysmal hypertensive episodes were ameliorated following placement of a carotid artery stent for the treatment of carotid artery stenosis. CASE REPORT: A 79-year-old caucasian male was diagnosed with hypopharyngeal squamous cell carcinoma (T1, L0, M0) in 2006, and received 70 Gy intensity-modulated radiotherapy in 2006 and underwent a total laryngectomy in 2008. He experienced paroxysmal hypertensive episodes since 2010 that exacerbated in frequency in 2019. Eighty percent left internal carotid artery stenosis was demonstrated by ultrasound and arteriography. Angioplasty and stenting of the left carotid artery was performed. A Doppler ultrasound study performed 5 months after the stent placement did not reveal any hemodynamic stenosis in the left carotid artery. The patient experienced postprandial hypotension and had experienced only three episodes of paroxysmal hypertension in the following 24 months. He was able to abort paroxysmal hypertensive episodes by eating warm food. DISCUSSION: This is the first report of a patient whose paroxysmal hypertensive episodes that occurred following radiation of the neck subsided after placement of a stent in a stenotic carotid artery. The exact mechanism leading to this phenomena is unknown but may be due to several factors. The reversal of the carotid artery stent and improvement in blood flow to the carotid artery baroceptors may play a role in this phenomenon. CONCLUSION: The ability to ameliorate paroxysmal hypertensive episodes in a patient with carotid artery stenosis by stent placement may be a promising therapeutic intervention for paroxysmal hypertension.

Healthcare Worker With Large Vessel Acute Ischemic Stroke Likely Related to Mild SARS-CoV-2 Infection.

We report the case of a healthcare worker who presented with a large vessel acute ischemic stroke in setting of a mild SARS-CoV-2 infection and provide a review of the emerging literature on COVID-related stroke. A 43-year-old female presented with right-sided hemiparesis, aphasia and dysarthria. She had a nonproductive of cough for 1 week without fever, fatigue or dyspnea. A CT Head, CT angiography and CT perfusion imaging revealed a M1 segment occlusion of the left middle cerebral artery requiring transfer from a primary to a comprehensive stroke center. A nasopharyngeal swab confirmed SARS-CoV-2 infection prior to arrival at the accepting center. During the thrombectomy a 3 cm thrombus was removed. Thrombus was also evident in the 8 French short sheath during closure device placement so a hypercoagulable state was suspected. Stroke work-up revealed a glycosylated hemoglobin of 8.7%, elevation of inflammatory markers and an indeterminate level of lupus anticoagulant IgM. On discharge home, she had near complete neurological recovery. This case highlights suspected mechanisms of hypercoagulability in SARS-CoV-2 infection and the importance of optimizing stroke care systems during the COVID-19 pandemic.

A Case of Neurosarcoid Presenting as Multiple Intraparenchymal Hemorrhages.

This report explores the case of a 49-year-old African American male with a six-month history of multifocal neurological deficits who presented to an outside hospital after a generalized seizure. Patient was transferred to our tertiary medical center after brain imaging showed multiple bilateral supratentorial intraparenchymal hemorrhages (IPH). A brain biopsy confirmed parenchymal and perivascular non-caseating granulomas with vasculitis. The patient was definitively diagnosed with neurosarcoidosis (NS) and his condition improved with high dose corticosteroids and additional immunosuppressive therapies. Intracranial hemorrhage in the setting of NS is extremely rare, with fewer than thirty documented cases; however, this is likely an underestimation of its true prevalence. This case illustrates the difficulty in diagnosis as many other etiologies of IPH must be considered. Additionally, the clinical course and manifestations of NS is often quite variable. The uniqueness of this case lies in the rapid progression from seemingly incidental microhemorrhages to multiple large IPHs over two months. While the cause of this progression is not immediately apparent, a possible cause may be inadequate initial treatment due to delayed diagnosis. Our case demonstrates the importance of early recognition and initiation of immunosuppressive therapy, potentially leading to dramatic clinical improvement, as seen in this patient.

Arteriovenous Malformations Treated With Frameless Robotic Radiosurgery Using Non-Invasive Angiography: Long-Term Outcomes of a Single Center Pilot Study.

OBJECTIVE: CT-guided, frameless robotic radiosurgery is a novel radiotherapy technique for the treatment of intracranial arteriovenous malformations (AVMs) that serves as an alternative to traditional catheter-angiography targeted, frame-based methods. METHODS: Patients diagnosed with AVMs who completed single fraction frameless robotic radiosurgery at Medstar Georgetown University Hospital between July 20, 2006 - March 11, 2013 were included in the present study. All patients received pre-treatment planning with CT angiogram (CTA) and MRI, and were treated using the CyberKnife radiosurgery platform. Patients were followed for at least four years or until radiographic obliteration of the AVM was observed. RESULTS: Twenty patients were included in the present study. The majority of patients were diagnosed with Spetzler Martin Grade II (35%) or III (35%) AVMs. The AVM median nidus diameter and nidal volume was 1.8 cm and 4.38 cc, respectively. Median stereotactic radiosurgery dose was 1,800 cGy. After a median follow-up of 42 months, the majority of patients (81.3%) had complete obliteration of their AVM. All patients who were treated to a total dose of 1800 cGy demonstrated complete obliteration. One patient treated at a dose of 2,200 cGy developed temporary treatment-related toxicity, and one patient developed post-treatment hemorrhage. CONCLUSIONS: Frameless robotic radiosurgery with non-invasive CTA and MRI radiography appears to be a safe and effective radiation modality and serves as a novel alternative to traditional invasive catheter-angiography, frame-based methods for the treatment of intracranial AVMs. Adequate obliteration can be achieved utilizing 1,800 cGy in a single fraction, and minimizes treatment-related side effects.

Endovascular Management of Stroke Patients with Large Vessel Occlusion and Minor Stroke Symptoms.

Endovascular mechanical thrombectomy for stroke patients with large vessel occlusion (LVO) in the anterior circulation has become the standard of care based on several major randomized clinical trials. The successful result reported by these trials constitutes what may be the largest achievement in the history of neurological sciences. However, most of these mechanical thrombectomy trials (except for the multicenter randomized clinical trial of endovascular treatment for acute ischemic stroke in the Netherlands, i.e., MR CLEAN and Extending the Time for Thrombolysis in Emergency Neurological Deficits-Intra-Arterial, i.e., EXTEND-IA) excluded stroke patients with minor to mild stroke symptoms with National Institutes of Health Stroke Scale (NIHSS) scores of six to eight or lower. The median NIHSS score for patients who underwent acute endovascular thrombectomy was approximately 15 to 17 in all trials. To date, the evidence is lacking to support the mechanical thrombectomy in patients with acute stroke and LVO with minor to mild severity on NIHSS score. The purpose of this review was to assess the current data, safety and clinical outcomes in stroke patients with minor to mild symptoms who were treated with endovascular thrombectomy.

Venous sinus compromise after pre-sigmoid, transpetrosal approach for skull base tumors: A study on the asymptomatic incidence and report of a rare dural arteriovenous fistula as symptomatic manifestation.

The sigmoid sinus is routinely exposed and manipulated during pre-sigmoid, transpetrosal approaches to the skull base, but there is scant data available on the incidence of venous sinus compromise after surgery. We encountered a dural arteriovenous fistula as a result of sigmoid sinus occlusion and examined the incidence of venous sinus thrombosis or narrowing after transpetrosal surgeries. We performed a retrospective analysis of a series of patients treated by the senior surgeons (WCJ, MH, HJK), who underwent either a posterior petrosectomy or translabyrinthine approach for various skull base tumors. All available clinical and radiographic data were thoroughly examined in each patient to determine the post-operative fate of the venous sinuses. Of the 52 available patients, five patients were discovered post-operatively to have a narrowed or constricted sigmoid sinus ipsilateral to the surgery, whereas another five patients were diagnosed with asymptomatic sinus thrombosis either in the transverse or sigmoid or both. None of these patients experienced symptoms, nor were there any instance of ischemic or hemorrhagic complications. However, there was one additional patient who presented with pulsatile tinnitus 2years after surgery. His angiogram showed an occlusion of the ipsilateral sigmoid sinus and a posterior fossa dural arteriovenous fistula. A two-stage transvenous and transarterial embolization was successful in eliminating the fistula. Technical considerations to avoid sinus injuries during pre-sigmoid, transpetrosal surgery are discussed.

Bilateral Subaxial Rotational Vertebral Artery Occlusion in a Setting of a Prior Cervical Construct.

BACKGROUND: Rotational vertebral artery occlusion, or bow hunter's syndrome, most commonly affects the C1-2 level because of its importance in regulating rotational movement. CASE DESCRIPTION: A 50-year-old man with increasing neck pain and severe symptoms of vertebrobasilar insufficiency with bidirectional head rotation had undergone several prior subaxial cervical spine operations. Dynamic cerebral angiography demonstrated complete occlusion of the left vertebral artery during head rotation to the right and complete occlusion of the right vertebral artery during head rotation to the left. Occlusions occurred at the level of and rostral to his prior construct, with immediate recurrence of debilitating vertigo and near syncope. Successful radiographic and clinical resolution of symptoms was achieved by posterior instrumentation and fusion from C2, connecting to his prior hardware. CONCLUSIONS: A brief literature review and treatment options are discussed for this unusual presentation of a rare clinical entity.

Intermittent balloon occlusion to favor nidal penetration during embolization of arteriovenous malformations: a technique modification.

Endovascular embolization of brain arteriovenous malformations (AVMs) has improved with liquid occlusive agents, but flow-related and anatomic restrictions limit endovascular capabilities. AVM compartments supplied by leptomeningeal networks and feeding arteries too small and/or tortuous for safe catheterization are rarely penetrated by liquid occlusive agents. A case with both impediments prompted a novel solution. A balloon was inflated across the supply to a lenticulostriate feeder, thereby favoring penetration of the liquid occlusive agent, injected from a different feeding territory, back into the AVM compartment supplied by the temporarily occluded feeder. This technique may reduce the number of embolization stages in large high-flow AVMs and increase the likelihood of achieving complete occlusion. This technique is highly complex and requires meticulous monitoring of multiple events.

Pipeline embolization device: a new source for embolic retinal vascular occlusion.

A 57-year-old woman underwent treatment of a left internal carotid artery aneurysm with a Pipeline embolization device. She subsequently experienced multiple branch retinal artery occlusions in her left eye. Although rare, ophthalmic complications may follow this new technique in the treatment of intracranial aneurysms.

Intermittent balloon occlusion to favor nidal penetration during embolization of arteriovenous malformations: a technique modification.

Endovascular embolization of brain arteriovenous malformations (AVMs) has improved with liquid occlusive agents, but flow-related and anatomic restrictions limit endovascular capabilities. AVM compartments supplied by leptomeningeal networks and feeding arteries too small and/or tortuous for safe catheterization are rarely penetrated by liquid occlusive agents. A case with both impediments prompted a novel solution. A balloon was inflated across the supply to a lenticulostriate feeder, thereby favoring penetration of the liquid occlusive agent, injected from a different feeding territory, back into the AVM compartment supplied by the temporarily occluded feeder. This technique may reduce the number of embolization stages in large high-flow AVMs and increase the likelihood of achieving complete occlusion. This technique is highly complex and requires meticulous monitoring of multiple events.

Neurological sequelae from brachiocephalic vein stenosis.

Stenosis of central veins (brachiocephalic vein [BCV] and superior vena cava) occurs in 30% of hemodialysis patients, rarely producing intracranial pathology. The authors present the first cases of BCV stenosis causing perimesencephalic subarachnoid hemorrhage and myoclonic epilepsy. In the first case, a 73-year-old man on hemodialysis presented with headache and blurry vision, and was admitted with presumed idiopathic intracranial hypertension after negative CT studies and confirmatory lumbar puncture. The patient mildly improved until hospital Day 3, when he experienced a seizure; emergency CT scans showed perimesencephalic subarachnoid hemorrhage. Cerebral angiography failed to find any vascular abnormality, but demonstrated venous congestion. A fistulogram found left BCV occlusion with jugular reflux. The occlusion could not be reopened percutaneously and required open fistula ligation. Postoperatively, symptoms resolved and the patient remained intact at 7-month follow-up. In the second case, a 67-year-old woman on hemodialysis presented with right arm weakness and myoclonic jerks. Admission MRI revealed subcortical edema and a possible dural arteriovenous fistula. Cerebral angiography showed venous engorgement, but no vascular malformation. A fistulogram found left BCV stenosis with jugular reflux, which was immediately reversed with angioplasty and stent placement. Postprocedure the patient was seizure free, and her strength improved. Seven months later the patient presented in myoclonic status epilepticus, and a fistulogram revealed stent occlusion. Angioplasty successfully reopened the stent and she returned to baseline; she was seizure free at 4-month follow-up. Central venous stenosis is common with hemodialysis, but rarely presents with neurological findings. Prompt recognition and endovascular intervention can restore normal venous drainage and resolve symptoms.

Acute embolization of ruptured brain arteriovenous malformations.

BACKGROUND: Ruptured brain arteriovenous malformations (bAVMs) are at increased risk of re-hemorrhage but management has historically been conservative. This is because: (1) ruptured bAVMs have not been considered as catastrophic as ruptured cerebral aneurysms, (2) surgical resection is aided by waiting for brain edema to resolve and clot to liquefy and (3) fear exists that partially treated bAVMs may be more dangerous than those untreated. The purpose of this study was to determine the feasibility and safety of acute embolization in patients with ruptured bAVMs. METHODS: 21 consecutive patients who underwent acute embolization of ruptured bAVMs from 2007 to 2011 were retrospectively reviewed. All treatments consisted of embolization exclusively using Onyx liquid embolic agent. Neurological outcomes were assessed using the Glasgow Outcome Scale (GOS) at hospital discharge following initial treatment. RESULTS: Nine patients (43%) were male, mean age was 38 years (range 8-75) and initial embolization was performed at a median of 4 days after ictus (mean 5.8, range 0-19). Spetzler-Martin grades ranged from I to VI (mean 3, median 3). In seven patients (33%) the AVM was completely occluded with a single treatment. With subsequent embolizations, complete occlusion occurred in four additional patients (19%). Six patients (29%) had post-embolization AVM resection and one patient (5%) had post-embolization gamma knife irradiation. None has suffered subsequent hemorrhages (mean follow-up 7.5 months). The mean discharge GOS of all patients was 4.4 (range 1-5). CONCLUSION: Treatment of ruptured bAVMs is often delayed but our experience with Onyx suggests that acute embolization is safe and feasible.

Transcranial laser therapy for acute ischemic stroke: a pooled analysis of NEST-1 and NEST-2.

BACKGROUND: NeuroThera Effectiveness and Safety Trials (NEST) 1 and 2 have demonstrated safety of transcranial laser therapy (TLT) for human treatment in acute ischemic stroke. NEST 1 study suggested efficacy of TLT but the following NEST 2, despite strong signals, missed reaching significance on its primary efficacy endpoint. In order to assess efficacy in a larger cohort, a pooled analysis was therefore performed. METHODS: The two studies were first compared for heterogeneity, and then a pooled analysis was performed to assess overall safety and efficacy, and examined particular subgroups. The primary endpoint for the pooled analysis was dichotomized modified Rankin scale (mRS) 0-2 at 90 days. RESULTS: Efficacy analysis for the intention-to-treat population was based on a total of 778 patients. Baseline characteristics and prognostic factors were balanced between the two groups. The TLT group (n = 410) success rate measured by the dichotomized 90-day mRS was significantly higher compared with the sham group (n = 368) (P = 0·003, OR: 1·67, 95% CI: 1·19-2·35). The distribution of scores on the 90-day mRS was significantly different in TLT compared with sham (P = 0·0005 Cochran-Mantel-Haenszel). Subgroup analysis identified moderate strokes as a predictor of better treatment response. CONCLUSIONS: This pooled analysis support the likelihood that transcranial laser therapy is effective for the treatment of acute ischemic stroke when initiated within 24 h of stroke onset. If ultimately confirmed, transcranial laser therapy will change management and improve outcomes of far more patients with acute ischemic stroke.

Assessment of long-term outcomes for the STRokE DOC telemedicine trial.

Telemedicine can provide stroke evaluations in locations with limited available expertise. The reliability of telestroke has been established. Decision making efficacy has been shown in the National Institutes of Health's STRokE DOC trial. No prospective trial has assessed long-term telestroke outcomes, however. In an institutional review board-approved trial (NCT00936455), we contacted patients originally enrolled in the STRokE DOC trial. A telephone script was used to verify consent. Patients were asked standardized questions regarding disposition, modified Rankin Scale (mRS) score, mortality, and recurrent stroke for 2 retrospective time points (6 and 12 months postevent) and one current time point. Blind was maintained. Primary outcome measures of mortality and percent mRS score of 0-1 [%mRS(0-1)] at 6 months are reported. Wilcoxon's rank-sum test was used for continuous variables, and Fisher's exact was used for categorical variables. Of the original 222 participants, 75 patients or surrogates could be contacted. Mean time from enrollment was 3.96 ± 1.0 years (range, 2.33-5.45 years). Mean National Institutes of Health Stroke Scale (NIHSS) score was 8 ± 7 (5 ± 8 for telephone; 12 ± 8 for telemedicine; P = .002). The rate of intravenous recombinant tissue plasminogen activator (rt-PA) use was 31%. Six-month %mRS(0-1) outcome was not different, at 42%. Mortality after imputation to the entire study sample also was not different, at 18%. There was no difference in the rate of recurrent stroke (P = .61). Some 85% of patients were home at 6 months. This study reports a good 6-month outcome for stroke patients evaluated by telemedicine or telephone. This design is limited by the time since original enrollment and resultant inability to contact participants. Although these findings can add to the limited data on telemedicine outcomes, a prospective trial is needed.

Alberta Stroke Program Early CT Score (ASPECTS) in patients with wake-up stroke.

One-quarter of ischemic strokes occur during sleep, and affected patients are excluded from thrombolytic therapy because of an unknown time of stroke onset. It has been suggested that early ischemic changes detected on computed tomography (CT) are similar in patients with acute stroke and patients who recently awoke with stroke. We compared head CT scans using the Alberta Stroke Program Early CT Score (ASPECTS) in patients who were likely to suffer their stroke during sleep (awoke group) and a control group of patients with stroke of known onset time. Patients were recruited from a prospectively collected acute stroke database. The awoke group was defined as all ischemic stroke patients who were "last seen normal" more than 4 hours ago, arrived between 4 a.m. and 10 a.m., and underwent head CT within 15 hours of the time last seen normal. The control group was randomly selected from patients who underwent head CT within 4 hours of stroke onset. The ASPECTS evaluations were performed by investigators blinded to patient group and time of onset. A modified Rankin Scale (mRS) score was available in 15 awoke patients and 46 control patients at 90 days after stroke. Twenty-eight awoke patients and 68 control patients had suitable imaging for the ASPECTS. Baseline demographic characteristics and risk factors were similar in the 2 groups. The dichotomized ASPECTS analysis (≤7 vs 8-10) showed no significant differences between the groups. ASPECTS was 8-10 in 89.3% the awoke group and 95.6% in the control group (P=.353). There was a trend toward higher 90-day mRS score (0-1) in the awoke group versus controls (73% vs 45%; P=.079). Initial ASPECTS was similar in patients with wake-up stroke and those with 4 hours of symptoms. This suggests that a subset of wake-up stroke patients might be suitable for thrombolytic therapy.

Prevalence and risk factors for multiple simultaneous intracerebral hemorrhages.

BACKGROUND: Multiple simultaneous intracerebral hemorrhages (MSICH) are rare. We aimed to describe the frequency and potential risk factors for MSICH. METHODS: We reviewed 522 consecutive patients admitted to our institution with spontaneous ICH between August 1, 2006 and November 30, 2009. Patients with traumatic hemorrhages, predominantly subarachnoid or intraventricular hemorrhages, hemorrhagic transformation of ischemic infarcts, and hemorrhages due to cerebral venous thrombosis were excluded. We defined MSICH as spontaneous hemorrhages in 2 discrete areas with similar density profiles on initial CT imaging. We then classified MSICH as primary (spontaneous) or secondary (due to an underlying condition). In a 1:5 ratio case-control design study, we assessed risk factors for primary MSICH compared to solitary ICH using logistic regression. Values of p < 0.05 were considered significant in the final analyses. RESULTS: Of 522 ICH patients, 29 met the criteria for MSICH (5.6%) with a mean age of 59 years. The mean systolic blood pressure at presentation was 176 mm Hg. Fifteen cases had secondary causes while 14 were considered as having primary MSICH. In a multivariable model comparing primary MSICH cases to 70 solitary ICH controls, the presence of >5 chronic microhemorrhages on gradient echo imaging (adjusted OR 9.8, 95% CI 2.0-49.3, p = 0.006) was the only independent predictor of primary MSICH. CONCLUSIONS: In a single-center experience, we found that MSICH account for 5.6% of all spontaneous ICH. Prior microhemorrhage burden, perhaps due to severity of hypertension or cerebral amyloid angiopathy, may mark those at risk for primary MSICH occurrence.