A national survey was conducted among members of the Neurohospitalist Society (NHS) to characterize updated practice patterns, perspectives, compensation, and career satisfaction of neurohospitalists in the U.S. Between March and May 2024, a 96-question online survey was distributed. Findings were compared to those from a similar NHS survey conducted in 2016. 143 neurohospitalists completed the survey (response rate 19%). 80% had some fellowship training, with vascular neurology (35%) and neurohospitalist (17%) being most common. 60% were ≤10 years out of completion of training, and 68% had ≤10 years of practice experience. 76% worked in academic medical centers and 24% in community hospitals. Cerebrovascular disease, seizure, and delirium/encephalopathy were the 3 most common neurological diagnoses seen in the inpatient setting. The most frequent work schedule (≥44%) was 7 days on/7 days off. The most common compensation model was base salary plus incentives (≥58%). For neurohospitalists working for academic medical centers, median total annual compensation was $299 250 (IQR $100 000). For neurohospitalists with non-academic employers, median total compensation was $367 000 (IQR $98 000). 70% of academic neurohospitalists and 53% of community neurohospitalists reported working with non-physician practitioners (NPPs). Although 64% of neurohospitalists reported having experienced burnout, 91% agreed that they find their clinical work personally rewarding. This survey, the largest to date among neurohospitalists, confirms that neurohospitalists remain a diverse, relatively young workforce with national presence. When compared to the 2016 survey, neurohospitalist compensation has increased.
The IRRAflow device combines intracerebroventricular (ICV) medication infusion, cerebrospinal fluid (CSF) irrigation, and continuous intracranial pressure (ICP) monitoring. While ICV milrinone is conventionally given as a bolus to manage vasospasm after aneurysmal subarachnoid hemorrhage (aSAH), this case presents the use of continuous ICV milrinone infusion via IRRAflow in a patient with refractory cerebral vasospasm following aSAH. A 47-year-old female with aSAH (Hunt Hess 2, Modified Fisher Grade 4) underwent coil embolization for a ruptured left PICA aneurysm. Despite standard management, severe vasospasm was detected in the bilateral middle cerebral arteries and basilar arteries on hospital day 6. Following initiation of systemic milrinone IV and intra-arterial verapamil treatment, subsequent transcranial Doppler (TCD) and CT angiogram revealed persistent vasospasm. Elevated ICPs precluded further angiography or ICV bolus therapy through the existing external ventricular drain (EVD). An IRRAflow catheter was inserted intraventricularly for continuous CSF drainage and ICV milrinone administration (2.6 mg/kg/day). Over days 8 to 12, vasospasm improved significantly, ICP normalized, and neurologic examination permitted extubation. Continuous ICV milrinone therapy was tapered over 5 days, and the IRRAflow system removed on day 14 without complications, leading to discharge for acute rehabilitation. Patient consent for case publication was documented per institutional protocol. Continuous intrathecal milrinone infusion via IRRAflow may be a feasible adjunct for treating refractory vasospasm after aSAH. After the combined use of ICV milrinone via the IRRAflow catheter with standard-of-care therapies for severe vasospasm, the patient demonstrated favorable clinical and radiographic improvement without complications.
Background and Purpose: Telestroke improves access to acute ischemic stroke (AIS) expertise, aids in decision-making, and reduces interhospital transfers. Few studies have examined subacute inpatient telestroke services, which focus on inpatient stroke workup and management. Methods: In this retrospective cohort study of patients with emergency department (ED) diagnosis of AIS from 10/2021-6/2024, we sought to determine the impact of a novel subacute telestroke program on ED transfer rates at participating hospitals. For intervention sites (that implemented the subacute teleconsult program), the period prior to subacute consult "go-live" date was considered the pre-implementation period. Control sites (without the subacute program) were considered preimplementation prior to 5/22/2023 (when approximately half of intervention sites had initiated the subacute program). Logistic regression with generalized estimating equations evaluated the association between implementation time-period and odds of ED transfer in intervention and control sites, adjusting for age, NIHSS, sex, race, and an intervention by time-period interaction term. Results: 1266 patients met eligibility criteria (N = 544 patients from 11 control sites and N = 722 patients from 11 intervention sites). The ED transfer rate was lower within intervention sites post-implementation (pre: 25.7% to post: 22.5%) and higher in control sites (pre: 25.9% to post: 27.1%). These comparisons were statistically nonsignificant in the multivariable analysis. Conclusions: There was a reduction in interhospital transfers after implementation of a subacute telestroke consultation service, but results were nonsignificant in adjusted models. Future analyses should evaluate whether receipt of a subacute telestroke encounter at the patient-level is associated with reduced interhospital transfer for stroke.
Patients with spontaneous intracranial hemorrhage (ICH) who present to non-tertiary care centers are often transferred to a facility with neurosurgical expertise without consideration of the likelihood of surgical intervention. At our Comprehensive Stroke Center (CSC), a minority of patients transferred for neurosurgical evaluation undergo interventions putting a strain on hospital resources. This study aimed to (1) quantify the frequency of neurosurgical intervention in ICH patients transferred to our hospital and, (2) to develop a tool to aid in transfer decisions. Using an IRB-approved retrospective cohort study design, we identified all spontaneous ICH patients transferred to our CSC between January 1, 2016, and May 31, 2023. All patients were reviewed to ensure a primary diagnosis of non-traumatic supratentorial ICH. Odds ratios were calculated using a logistic regression model to identify factors predictive of neurosurgery which were weighted by strength of association. Internal validation was then performed. Of the 496 participants included in the final dataset, 78 (15.7%) underwent neurosurgical intervention. Age, Glasgow Coma Scale, ICH volume, and intraventricular extension were the greatest predictors of neurosurgery. These factors were used to create the Likelihood of Neurosurgery Score (LoNS), a weighted score used to inform transfer decisions. The score performed well on calibration and discrimination tests. The LoNS is a new tool to identify ICH patients unlikely to be neurosurgical candidates who could be safely managed at the local level rather than urgently transferred to a tertiary care center. Prospective validation is needed.
Telestroke emergency department (ED) workflow emulates that of in-person care, yet limitations of the virtual interaction may require accommodations. The optimal sequence of non-contrast head CT, cerebral CT angiogram, and decision to infuse IV thrombolytic is not defined for telestroke. In this twenty-two-hospital multicenter retrospective study between 1/1/2024-12/31/2024, we characterized timing of ED CT angiograms, either bundled with the non-contrast head CT or non-bundled and performed after the IV-thrombolytic decision. The primary outcome was effect of bundling on door to needle time (DTN) when directed by telestroke. Timing of CT scans was determined by time stamps of scout images. In total, 5704 patients were included, with a median age of 65 years (Interquartile range, IQR 58-77) and NIHSS 8 (IQR 6-13). There were 4518 patients in the bundled group (n = 12 hospitals), with time from non-contrast CT to CTA of median 2 min (IQR 0-6 min; vs non-bundled (n = 10 hospitals): 7, 4-15 min; P = .0001). DTN was not statistically different between the groups, bundled vs not: median 47 min (IQR, 35-62 min) vs 54 min (IQR, 41-67 min, P = .21). Door in door out (DIDO) times also were unchanged (169, 117-254 vs 175, 123-247 min; P = .56). These findings, although showing no statistical difference, may have significance for clinical workflows and were consistent with the results of validation analyses conducted on individual patients. Bundling non-contrast head CT with the CT angiogram before thrombolytic decision did not delay DTN times, trended to clinically improve times, and may be relevant for telestroke ED workflows.
Spontaneous intracranial hypotension (SIH) results from cerebrospinal fluid (CSF) leakage due to spinal dural tears or CSF-venous fistulas. Orthostatic headache is the hallmark presentation, though severe downward displacement of the brainstem may lead to altered consciousness or coma. Definitive treatments include targeted epidural blood patches, venous embolization, or surgical repair. This article reviews the role of the Trendelenburg position as a temporizing measure in the acute management of SIH. We describe the correct technique, physiologic rationale, and practical considerations for its application, with attention to both therapeutic and diagnostic utility. Positioning the patient with the feet elevated above the head can reduce brain sag and provide short-term symptomatic relief while awaiting definitive treatment. Although the Trendelenburg position is widely used in practice, supporting evidence remains limited, and clinicians must be aware of its benefits, indications, and inherent limitations. Its appropriate application may assist in stabilizing patients with acute or severe SIH, particularly those with impaired consciousness, until more definitive interventions are pursued.
Dengue virus infection is increasingly recognized as a cause of neurological complications, though severe parenchymal involvement such as acute necrotizing encephalitis remains exceptionally rare in adults. We report a 35-year-old woman presenting with fever, seizures, and altered consciousness. Laboratory tests confirmed dengue virus infection, while cerebrospinal fluid analysis revealed normal protein and glucose, no pleocytosis, and positive dengue IgM. Brain MRI demonstrated bilateral thalamic hyperintensities with central hypointense cores, the characteristic "double-doughnut" sign, along with additional involvement of the brainstem, cerebellum, and occipital lobe, consistent with ANE. Despite prompt initiation of high-dose intravenous methylprednisolone, the patient developed persistent akinetic mutism, highlighting the poor prognosis in adult-onset cases. This case underscores the dissociation between severe radiological findings and relatively unremarkable CSF, emphasizes the diagnostic utility of MRI, and illustrates the therapeutic challenges in resource-limited settings. Early recognition and context-appropriate management are critical to improving outcomes.
Data analyzing usage patterns, efficacy, tolerability, and long-term continuation of brivaracetam (BRV) when initiated in the acute, inpatient setting is lacking. Retrospective chart review of adult patients who initiated BRV in the emergency or inpatient setting at Yale New Haven Hospital over 5-year span. Of 133 patients, BRV was used for status epilepticus (SE) in 37% (n = 49), seizures in 38% (n = 51), and rhythmic/periodic patterns (RPP) in 21% (n = 28). Eighty-six (65%) were in an ICU and 33 (25%) were in a non-ICU setting. BRV was the first anti-seizure medication (ASM) tried in 9 (7%), while 124 (93%) tried other ASMs first (mean ASMs = 2; SD = 1). Initial dosing ranged from 50-400 mg (median = 200 mg, IQR = 200-300 mg), then median maintenance dosing of 200 mg/day (IQR = 200-300 mg/day). Of patients with RPP, seizures, or SE on EEG (n = 115, 86%), 46% (n = 53) had electrographic and/or clinical improvement, including 23 (20%) with complete resolution. In patients with clinical seizures (n = 10) or SE (n = 2) not on EEG, BRV was effective in 10 patients. BRV was discontinued in 49 (36%) patients, typically for inefficacy. Of 88 survivors to discharge, 86 (98%) were discharged on ASMs, including 62% (n = 54) discharged on BRV. Follow-up data were available for 53 (60%) patients - 51 (96%) remained on ASMs, with 33 (65%) remaining on BRV (median follow-up = 30 d, IQR = 18-65 d). BRV appears safe, well-tolerated, and efficacious for acute, inpatient management of RPP, seizures, and SE. Prospective studies validating these findings and directly comparing BRV with other ASMs are warranted.
Background: Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease that may affect any organ of the body. The American College of Rheumatology (ACR) defines 19 neuropsychiatric syndromes that can be directly attributed to SLE, including psychosis and movement disorder, which is most commonly chorea. Parkinsonism is exceedingly rare as a manifestation of neuropsychiatric SLE. The infrequent occurrence of parkinsonism in SLE and the lack of specific confirmatory tests can pose significant diagnostic challenges. Purpose: To describe an elderly female who presented with psychosis and acute-onset parkinsonism as the only initial clinical features of SLE. Research Design: Case report. Study Sample: A 65-year-old woman without prior autoimmune disease who presented with new-onset psychosis and rapidly progressive parkinsonism. Data Collection and/or Analysis: Clinical presentation, laboratory testing, neuroimaging, treatment course and response were reviewed and summarized. Results: The patient developed new-onset psychosis and parkinsonism. Initial imaging and CSF studies were non-diagnostic, but she later demonstrated positive anti-nuclear antibody (ANA), anti-Sm and low complement levels. She was successfully treated with plasmapheresis and immunosuppressants with complete symptom resolution. Conclusions: Psychosis and parkinsonism may be early or isolated manifestations of SLE. It is important to consider SLE and autoimmune conditions as part of the differential diagnosis in select patients presenting with psychosis and acute-onset parkinsonism as early recognition and timely immunomodulatory therapy can result in complete clinical recovery.
CTP has increasingly been incorporated into the evaluation of all patients with suspected acute ischemic stroke (AIS), including those with minor symptoms. We aimed to assess the frequency with which CTP is performed in patients with possible AIS based on NIHSS as well as the role of CTP in acute treatment decision-making among patients with low NIHSS. We performed a retrospective cohort study of all patients who underwent CTP upon presentation to the ED at 3 academic, urban hospitals in Philadelphia, PA between January 1, 2022 and December 31, 2022. We collected data on initial NIHSS score, AIS treatment decisions, subsequent neuroimaging, and final diagnosis. The study was deemed exempt by the Hospital of the University of Pennsylvania IRB. There were 530 patients with a median age of 65 years (IQR 54-73) and 56% were women. The frequency of CTP by NIHSS is displayed in the figure. A total of 89 CTP studies (16.8%) were performed in patients with very low NIHSS (defined as NIHSS ≤ 2). Of these, just 2 (2.2%) received thrombolysis and 0 (0%) received thrombectomy. CTP did not influence the treatment decision in either case. CTP is frequently performed in patients with low NIHSS. It had limited impact on acute treatment decisions, notably none among those with NIHSS ≤ 2, suggesting that CTP may be over-utilized in this subset of patients with AIS.
Neurenteric cysts are rare congenital lesions of endodermal origin that typically present with slowly progressive myelopathy. Accounting for less than 2% of all spinal tumors, they most often occur in the cervical and upper thoracic spine. Acute neurological deterioration due to a neurenteric cyst is uncommon and represents a clinical emergency. We describe an 18-year-old woman with no prior medical history who developed sudden-onset neck pain, rapidly progressive quadriparesis, and respiratory compromise. Neurological examination revealed upper motor neuron signs with a C4 sensory level. Cervical magnetic resonance imaging showed a ventral intradural extramedullary cystic lesion at the C2-C3 level, compressing the spinal cord and producing cord edema. Emergent posterior C2-C3 laminectomy was performed, and a tense mucin-filled cyst was completely excised. Histopathology demonstrated a columnar mucinous epithelium with goblet cells, confirming the diagnosis of a neurenteric cyst. Postoperatively, the patient exhibited substantial neurological recovery, regaining independent ambulation within three months. Follow-up imaging at six months showed no recurrence. This case underscores two important lessons. First, although neurenteric cysts are classically indolent, they may present with abrupt, life-threatening neurological decline, even in the absence of associated congenital vertebral anomalies. Second, prompt surgical decompression with gross total resection remains the cornerstone of management, offering excellent potential for recovery. Given the risk of recurrence, radiological surveillance is warranted. This report adds to the limited literature on cervical neurenteric cysts with hyperacute presentations and emphasizes the need for early recognition and timely intervention to optimize outcomes.
Baclofen, a GABA agonist, is used for the management of spasticity. Intrathecal route is indicated in cases of severe spasticity associated with spinal cord injury. Baclofen withdrawal symptoms can happen after IT pump removal which can present as anxiety, paranoia, psychosis, seizures, hallucinations, severe spasticity and dyskinesia. Cardiac arrest after baclofen withdrawal is rare with only 2 cases in literature so far however in both cases IT-pump was replaced. We present a case of PEA arrest after baclofen IT-pump removal which was managed without replacing. 33 year-old male with history of paraplegia from cervical spinal cord injury (C-6) level, chronic spasticity, fibular osteomyelitis and chronic sacral ulcers presented with wound dehiscence and exposed baclofen pump which was replaced 4 weeks ago due to the end of the pump's life. After 24-48 h of removal of the baclofen pump, cardiovascular instability in the form of supra ventricular tachycardia with hypotension and altered mentation was noted. It evolved to progressive clinical worsening with stiffness of body, loss of responsiveness, and up-rolling of eyes. Patient underwent PEA, and ROSC after 3 rounds of CPR. He was subsequently intubated, mechanically ventilated and sedated with midazolam intravenously. Baclofen and clonazepam were started through the feeding tube. Over a period of 10 days, midazolam was weaned, and baseline clinical stability was achieved without IT-baclofen pump. Though rare, cardiac arrest can happen after baclofen withdrawal. High index of clinical suspicion is advised for long term baclofen pump patients after pump explant to prevent withdrawal symptoms and complications.
Navigating health-related decisions after severe acute brain injury (SABI) can be challenging, especially when the patient's preferences and the prognosis remain unclear. This uncertainty adds a layer of complexity for surrogates and medical teams striving to make treatment choices. To address these challenges, this article presents an interview study examining decisions that were retrospectively relevant for a surrogate decision-maker. Key moments for shared decision-making and advance care planning were identified and compared to a theoretical decision model, providing valuable insights for decision-making in the context of SABI given time-pressure, prognostic uncertainties, and the patient's neurological impairment. A semi-structured interview was conducted with the 31-year-old daughter of a 53-year-old woman who had experienced an aneurysmal subarachnoid hemorrhage. The interview was thematically analyzed, and eight preference-sensitive decision moments were identified and visualized within a timeline: bleeding event, emergency treatment, intensive care unit treatment (general), severe complication, long-term life-sustaining surgical interventions, admission to rehabilitation, further severe complication, and palliation. In conclusion, this case study supports an iterative evaluation of treatment preferences and suggests well-suited moments for reevaluation of medical treatment goals and shared decision-making within a timeline. This framework may serve to facilitate shared decision-making by identifying key preference-sensitive junctures and providing a basis for designing tools that incorporate deliberate timing.
We read with great interest the case report by Murray et al describing transverse myelitis associated with helminthic infection. While intriguing, several aspects of the diagnostic workup and clinical interpretation warrant further clarification. Pain, treatment response, and cerebrospinal fluid (CSF) and radiological follow-up findings suggest that a demyelinating centrale nervous system (CNS) disorder, such as MOG antibody-associated disease, multiple sclerosis, or neuromyelitis optica spectrum disorders (NMOSD), could represent a plausible alternative explanation. Additional investigations, including antibody testing and serial magnetic resonance imaging (MRI), would have strengthened the diagnostic conclusions. We argue that a more comprehensive assessment is essential to avoid premature attribution of the clinical picture solely to helminthic infection.
End-of-life (EOL) care in acute stroke is complex. Understanding family experiences is essential to improve care quality and promote a dignified death. This study aimed to assess family members perceptions of EOL care in patients who died after stroke and to identify factors associated with perceived dignified death. A cross-sectional survey of relatives of deceased stroke patients during 1 year was conducted. Of 111 eligible cases, 68 questionnaires were returned (61.3%). Following the descriptive analysis, we compared relatives who perceived the death as dignified with those who did not. Survey responses were then integrated with clinical data regarding treatment history and place of death. Most relatives (82.4%) considered that the patient died with dignity. Perceived dignified death was significantly associated with adequate and comprehensible prognostic information, respectful communication, respect for patient and family preferences, adequate symptom control, being accompanied at the time of death, and empathetic staff attitudes (all P < 0.05). No significant associations were observed with room type, religious variables, clerical visits, expectedness of death, continuous sedation, IV fluids, antibiotics, enteral nutrition, or location of death; symptom control ratings did not differ between sedated and non-sedated patients. Most relatives perceived the death as dignified and they were generally satisfied with EOL care. Dignity was primarily linked to communication, respect for preferences, emotional support, and humane care rather than specific medical interventions.
Introduction: New Onset Refractory Status Epilepticus (NORSE) occurs without an acute structural, toxic, or metabolic cause in individuals without known epilepsy or a related neurological disease. In about 50% of cases, NORSE is attributed to autoimmune or viral encephalitis; in the rest, it remains cryptogenic, posing significant treatment challenges and high risks of mortality and long-term neurological issues. Standard management often involves multiple antiseizure medications, and immunosuppressive therapies used even when an autoimmune cause is unproven. Case Description: We report a 23-year-old woman with cryptogenic NORSE resistant to multiple antiseizure medications, intravenous anesthetics, and immunosuppression, requiring a 5-month barbiturate-induced coma. Attempts to reduce anesthetics triggered recurrent super-refractory status epilepticus. Extensive working up including neuroimaging, cerebrospinal fluid, and autoimmune testing revealed no clear etiology. High-dose steroids, IVIG, plasmapheresis, rituximab, tocilizumab, and anakinra were ineffective. An FDA authorization for emergency single-patient IND (eIND) approval allowed treatment with IV ganaxolone, a GABAA receptor modulator, which was used alongside electroconvulsive therapy. Nine days after initiation of ganaxolone and 13 days after ECT was started, pentobarbital was successfully tapered, and seizures ceased. Consciousness and near-normal language function returned gradually, with residual cognitive deficits. After an 8-month hospitalization, she was discharged to inpatient rehabilitation and subsequently home. At 6 months post-discharge, her Glasgow Outcome Scale-Extended Score was 7.
Rozanolixizumab is a neonatal Fc receptor (FcRn) blocker recently approved for treating generalized myasthenia gravis (gMG), particularly in patients who are anti-AChR or anti-MuSK antibody positive. Although clinical trials have demonstrated a favorable safety profile, rare adverse neurological effects remain underreported. A 36-year-old woman with refractory anti-MuSK-positive generalized myasthenia gravis received an infusion of rozanolixizumab after failing to respond to corticosteroids and azathioprine. 5 days post-infusion, she presented with an acute-onset headache, nausea, vomiting, photophobia, phonophobia, and neck stiffness. Cerebrospinal fluid (CSF) analysis revealed a white blood cell count of 150 cells/μL with 90% neutrophils, normal protein, and glucose levels. The infectious meningitis and encephalitis panel was negative. She was managed supportively, and her symptoms resolved over the following week without antimicrobial therapy. This case suggests a possible link between Rozanolixizumab and aseptic meningitis. Clinicians should be aware of this potential adverse effect and consider drug-induced etiologies in similar clinical scenarios. Early recognition may prevent unnecessary treatment and hospitalizations.
Insulin Resistance (IR) impact on Acute Ischemic Stroke (AIS) outcomes has been challenging to assess due to the lack of a readily available IR biomarker in acute settings. The Triglyceride-Glucose (TyG) index is a novel surrogate marker for IR that may help bridge this gap. We aimed to evaluate the relationship between IR and clinical outcomes after AIS in patients with large vessel occlusion (LVO). This single-center retrospective cohort study included patients presenting between 2010 and 2022 with internal carotid artery (ICA) or middle cerebral artery (M1) occlusion who underwent endovascular treatment (EVT). Eligibility criteria were: pre-stroke modified Rankin Scale (mRS) score ≤2 and available fasting triglyceride and glucose levels. The TyG index was calculated as Ln [triglyceride (mg/dL) × glucose (mg/dL)]/2. Multivariable ordinal logistic regression was performed to assess the association between the TyG index and other demographic variables on 90-day mRS. Among 155 patients, 53% were males, and 47% were females. TyG index was significantly associated association with 90-day mRS (OR = 2.04, P < 0.01). Other associated variables included age (OR = 1.05, P < 0.01) and smoking status, with former smokers having lower odds of worse outcomes (OR = 0.44, P = 0.03). Lower TyG index values, indicative of reduced IR, were associated with better functional outcomes following EVT in AIS patients with LVO. Future studies should establish optimal TyG index cut-off values to refine vascular risk management and improve stroke outcomes.
A 64-year-old female with a history of Subarachnoid Hemorrhage (SAH) of non-aneurysmal origin underwent 4 cerebral Digital Subtraction Angiography (cDSA) studies to investigate the cause of the SAH. All angiograms were unrevealing. Two years and 3 months following her SAH, she presented to the emergency department with ataxia and aphasia. Magnetic Resonance Imaging (MRI) of the brain without contrast showed a right posterior parietal lobe stroke with a watershed appearance and a chronic right cerebellar infarct with encephalomalacia. Repeat cDSA showed stenosis in the right A3 of the Anterior Cerebral Artery (ACA) and right M2 of the Middle Cerebral Artery (MCA) suggesting CNS vasculitis. Known reversible and secondary causes of CNS vasculitis were tested and found to be negative. However, Indirect Immunofluorescence Assay (IFA) testing revealed a high titer of IgG phase II Coxiella indicating acute Q fever. CDC lab findings from 10 years earlier also showed a high titer IgG phase II Coxiella, indicating untreated Q fever prior to the SAH event. A whole-body Positron Emission Tomography (PET) scan was negative for systemic or CNS vasculitis. The patient was treated for Q fever and CNS vasculitis with 1 year of doxycycline and hydroxychloroquine. There was no recurrence of symptoms 15 months after treatment. We conclude that Q fever can present with SAH in addition to CNS vasculitis and stroke.
New-onset refractory status epilepticus (NORSE) is one of the most severe neurological manifestations of status epilepticus. The etiology is unknown in about 50% cases, as so-called cryptogenic NORSE (C-NORSE). We report a case with emergence of atypical lymphocytes just before C-NORSE. A 25-year-old woman with no significant medical history presented to our department with a 4-day history of headache and fever. Neurological examination and cerebrospinal fluid tests yielded normal results, but atypical lymphocytes were observed in the serum. Two days later, she was admitted to our emergency department in a comatose state, and subsequently developed convulsive refractory status epilepticus. The etiology remained unknown despite an extensive diagnostic work-up, so C-NORSE was diagnosed. Seizures were controlled with immunotherapies including corticosteroids, intravenous immunoglobulins, rituximab, tocilizumab, and administration of 4 antiseizure medications. The etiology of atypical lymphocytes involves hypercytokinemia similar to NORSE, and clarification of the emergence of atypical lymphocytes just before the onset of NORSE could contribute to the understanding of the pathophysiology of NORSE.