Background/Purpose:Downbeat nystagmus (DBN) is an uncommon finding in comatose patients, especially as a manifestation of epileptiform activity. We report a 59-year-old man who developed DBN in the context of myoclonic status epilepticus following anoxic brain injury secondary to cardiac arrest. The DBN was phase-locked with generalized periodic epileptiform discharges (GPDs) on electroencephalography (EEG) and resolved with pharmacologic burst suppression. Conclusion:This case suggests a potential link between DBN and cortical epileptiform activity, which we hypothesize may be due to bilateral cortical hyperexcitability and cerebellar disinhibition. The presence of DBN in this setting may indicate a poor prognosis.
In 2021 and 2023, the American Medical Association (AMA) revised evaluation and management (E/M) service reporting. Clinicians now determine most service levels by medical decision making or total time spent on the date of service. Trainees often receive little formal education about how documentation and coding affect clinical practice. Clinicians may also receive limited structured feedback on their documentation and coding. This review discusses inpatient pediatric neurology notes, critical care reporting, electroencephalography (EEG) interpretation, and attestation by the teaching physician. It also considers improvement models such as pathway-linked templates and department billing and coding review programs.
The effect of induced hypertension (iHTN) on functional outcomes in noncardioembolic acute ischemic stroke (AIS) with early neurological deterioration remains unclear. We performed a systematic review and meta-analysis evaluating the safety and efficacy of iHTN vs medical management. A literature search of PubMed/MEDLINE, Web of Science, Ovid Embase, and Scopus from inception through November 6th, 2025. The protocol was registered in PROSPERO (CRD420261282211). Studies evaluating iHTN within 72 h of AIS onset and reporting 90-day functional independence (modified Rankin Scale [mRS] 0-2) were included. Safety outcome was hemorrhagic transformation. Pooled risk ratios (RRs) with 95% confidence intervals (CIs) were estimated using random-effects model based on Restricted Maximum-Likelihood (REML) methods. Three studies were included, comprising 366 patients (iHTN, n = 180; control, n = 186). Mean age was 65.8 ± 11.8 years, 60.9% were men, and the mean initial NIHSS was 5.4 ± 3.5. Target systolic blood pressure (SBP) was a 15-25% increase from baseline SBP using phenylephrine. Functional independence at 90-days occurred more often in the iHTN group than in controls (68.8% vs 50.5%; RR = 1.37; 95% CI, 1.09-1.73; P = 0.007). Hemorrhagic transformation was uncommon and did not differ significantly between groups (3.3% vs 0.5%; RR = 2.08; 95% CI, 0.05-88.07; P = 0.70). In our study, phenylephrine-induced hypertension was associated with greater 90-day functional independence in selected AIS subtypes,without a significant increase in hemorrhagic transformation. However, the limited studies, predominance of retrospective designs, exclusively Asian cohorts, and heterogeneity in treatment protocols and populations warrant cautious interpretation and underscore the need for larger multicenter RCTs.
Background:Thrombotic thrombocytopenic purpura (TTP) is a hematologic disorder characterized by a severe deficiency or absence of the disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 enzyme (ADAMTS13), which is a protease essential for cleaving von Willebrand factor. This deficiency promotes microvascular thrombosis and can lead to ischemic strokes. Case Presentation:We present the case of a 29-year-old female with a history of rheumatological disease controlled with chronic low-dose prednisone who presented with an acute ischemic stroke due to a large vessel occlusion (LVO). She underwent mechanical thrombectomy, during which extensive recurrent clot formation occurred with recurrent intracranial occlusions. Further evaluation revealed nonbacterial thrombotic endocarditis. Interestingly, her initial hematological workup revealed only fluctuating thrombocytopenia, and her remaining hematologic tests were largely unremarkable. ADAMTS13 level was markedly reduced, and she was ultimately treated with plasma exchange, high-dose corticosteroids, and rituximab, with a good response and no recurrent ischemic events during follow-up. Conclusions:In this case, we highlight the importance of including hematological disorders like TTP in the differential diagnosis of cryptogenic stroke and particularly in the presence of autoimmune conditions or subtle hematologic abnormalities.
We describe what, to our knowledge, is the third reported fatal outcome in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy secondary to cerebral edema and brainstem herniation. A 42-year-old woman presented with neck and back pain with tremors, progressing to confusion and memory impairment. Neuroimaging revealed encephalomyelitis. CSF revealed antibodies to GFAP. After an initial response to corticosteroids, she had recurrence of encephalopathy. Following a repeat lumbar puncture, she developed diffuse cerebral edema with herniation and brain death. Diffuse cerebral edema should be considered a rare but potentially fatal presentation of GFAP astrocytopathy. Clinicians involved in the care of patients diagnosed with or suspected to have GFAP astrocytopathy should remain vigilant about the development of cerebral edema throughout the diagnostic and treatment process and evaluate the patient with neuroimaging before initial and subsequent lumbar punctures.
Flecainide is class Ic antiarrhythmic drug with a narrow therapeutic range. Adverse drug reactions during flecainide therapy are predominately cardiac in nature. Severe neurological adverse drug reactions have rarely been described. We describe the case of a 71-year-old female who developed myoclonus and severe asthenia requiring intubation and management in the neuroscience intensive care unit. Immediate discontinuation of flecainide and supportive care to allow for drug elimination was the primary treatment. Resolution of flecainide neurotoxicity resolved after 5 days of stopping flecainide. Clinicians should be cognizant of potential neurologic toxicity of flecainide.
Leucine-rich glioma-inactivated-1 (LGI1) IgG-associated autoimmune encephalitis typically presents with faciobrachial dystonic seizures, hyponatremia, and temporal lobe MRI abnormalities. However, the clinical spectrum of LGI1 IgG-associated AE extends beyond these classical features, with reports of diverse seizure semiologies and atypical presentations challenging traditional diagnostic paradigms. This study reports a male in his late 60s presenting with recurrent episodes of right foot cramping and electric shock-like pain, progressing to generalized tonic-clonic seizures. Initial workup suggested chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but LGI1 IgG antibodies were subsequently detected. Despite treatment with intravenous immunoglobulin (IVIG), the seizures persisted. This case highlights the heterogeneity in LGI1 encephalitis presentations. The patient’s proprioceptive-induced reflex seizures represent a rare manifestation of the disease. Treatment with rituximab, a B-cell depleting therapy, resulted in significant improvement, suggesting its potential effectiveness in LGI1 encephalitis with a resistant seizure course. The case also emphasizes the intricate interplay between the mesial temporal lobe, basal ganglia, and frontal regions in LGI1 encephalitis pathophysiology.
Background:Acute disseminated encephalomyelitis (ADEM) is a rare, rapidly progressive, immune-mediated demyelinating disorder with limited contemporary data describing its epidemiology in adults and real-world uptake of myelin oligodendrocyte glycoprotein (MOG) autoantibody testing. Methods:We used the TriNetX global collaborative network (112 contributing healthcare organizations) to estimate ADEM prevalence and incidence rates from 2000 to 2025, both overall and stratified by age of onset, sex, race, and ethnicity. We also assessed uptake of MOG autoantibody testing among people with an ADEM diagnosis code. Results:We identified 4911 ADEM cases among 153,342,461 individuals from 2000 to 2025. Overall period prevalence was 3.26 (95% CI [confidence interval] 3.17-3.35) per 100,000 persons and overall incidence rate was 0.63 per 100,000 person-years (95% CI: 0.61-0.65). ADEM diagnosis codes were most frequently recorded in those ≤14 years old. Estimated were similar between sexes, lower among Black compared to White individuals, but similar across other racial groups and ethnicities. Only 11% of ADEM-coded individuals had documented MOG autoantibody testing. Conclusion:In a large, real-world, electronic health record network, ADEM diagnosis codes were most frequently recorded in children, though cases were observed across the adult age range. MOG autoantibody testing was uncommon.
Background and Purpose:Substance use is rising among young adults with stroke. We aimed to assess the prevalence of substance use, and the use of toxicology screening in the diagnostic evaluation, for young individuals with acute ischemic stroke (AIS) at a single comprehensive stroke center. Methods:This retrospective study was conducted at a single comprehensive stroke center in individuals ages 18 to 55 years old with AIS from 2020-24. Cases of AIS and recent substance use, or the use of cocaine, methamphetamine, marijuana, opioids, or other illicit substances within 30 days of admission, were compared to those with AIS and no substance use. Descriptive statistics and least absolute shrinkage and selection operator (LASSO) modeling were completed to evaluate predictors of toxicology screening. Results:Of 425 encounters screened, 307 were eligible for inclusion. Recent substance use was documented in 21.5% of encounters. Individuals with recent substance use were younger (median age 46 vs 51 years old in controls). Toxicology screens were ordered for 29.3% of all encounters, and in 27.9% of cryptogenic strokes. Models did not identify a variable that predicted the performance of toxicology screening. Conclusions:Recent substance use was documented in about 1 in 5 young individuals with AIS. Further research is needed to understand the relationship between AIS and substance use, and the appropriate use of toxicology screening in stroke care.
A previously healthy 35-year-old woman in her third trimester of pregnancy presented with 2 weeks of progressive cognitive dysfunction, behavioral changes, and headache. Neurological examination was nonfocal, without evidence of meningeal irritation. Brain MRI showed multifocal punctate diffusion-restricting lesions in the subcortical white matter. The etiology was initially unclear despite extensive testing, Several days later, the patient presented again with worsening symptoms and interval radiographic progression. Ultimately, the constellation of clinical features and diagnostic findings suggested a unifying diagnosis. We discuss her clinical course and highlight the diagnostic reasoning. This case emphasizes the importance of maintaining a broad differential diagnosis and integrating evolving clinical and radiographic features, while balancing pregnancy-specific diagnostic and therapeutic considerations, in the evaluation of subacute encephalopathy.
Background and Purpose:Understanding trends in hospital length of stay (LOS) and costs for ischemic stroke is essential for improving care delivery and resource allocation. We evaluated national trends from 2016 to 2022, with a focus on the COVID-19 pandemic. Methods:We performed a retrospective analysis using the National Inpatient Sample including adults (≥18 years) with a primary diagnosis of ischemic stroke (ICD-10-CM I63.x). Hospital charges were converted to estimated costs using cost-to-charge ratios and adjusted to 2022 U.S. dollars. Trends were compared between pre-COVID (2016-2019) and COVID-era (2020-2022) periods. Illness severity was stratified by the APR-DRG index, and Pearson correlation assessed the relationship between annual mean LOS and costs. Results:Among 953 325 ischemic stroke hospitalizations, mean LOS rose from 6.4 days in 2016 to 7.8 days in 2022. Mean hospital costs increased from $19,014 to $28,733 over the same period. COVID-era hospitalizations had significantly higher LOS (7.44 vs 6.47 days; P < 0.001) and costs ($27,006 vs $20,568; P < 0.001) compared to pre-COVID admissions. Annual mean LOS and costs were strongly correlated (r = 0.974, P < 0.001), and greater illness severity was associated with longer LOS and higher costs. Conclusions:Hospital LOS and costs for ischemic stroke increased markedly from 2016 to 2022, with a sharp rise during the COVID-19 period. While LOS and costs were strongly correlated, additional factors-including illness severity, complication rates, and treatment complexity-likely contribute to rising expenditures. These findings underscore economic burden of stroke care and need for efficient inpatient care models.
A 53-year-old man who had defaulted on treatment for clinically diagnosed leprosy presented with four-month history of low-grade fever and progressive cognitive impairment. He started having left focal motor seizures with progressive left hemiparesis, followed by right focal seizures and right hemiparesis prior to hospital admission. Examination revealed axillary lymphadenopathy along with asymmetric (Left > Right) quadriparesis. Cerebrospinal fluid (CSF) evaluation did not reveal pleocytosis or hypoglycorrhachia with negative infective and inflammatory workup. Magnetic Resonance Imaging (MRI) was suggestive of abnormal signals in the cerebral cortices with swelling and a paraspinal abscess (Figure). Paraspinal tap was Culture negative with Cartridge based nucleic Acid Amplification test (CBNAAT) Positive for Mycobacterium Tuberculosis. Axillary lymph node biopsy was suggestive of caseous necrosis. He was started on Anti-Tubercular Therapy (ATT) with Multi Drug Therapy (MDT) for Leprosy and steroids along with anti-seizure medications (ASM) with significant clinical and radiological improvement in follow up. During his follow up he was ambulatory and independent in the intermediate term. Unfortunately, he contracted pneumonia in follow up and succumbed to sepsis related complications. Parenchymal involvement in the form of cortical encephalitis/cerebritis is an extremely rare presentation of CNS tuberculosis, characterised by T2/FLAIR hyperintense lesions with patchy or gyriform enhancement.
Anti-aquaporin-4 antibodies (AQP4-Ab) are highly specific for neuromyelitis optica spectrum disorder (NMOSD), but their prevalence in patients with non-inflammatory diseases, including malignant tumors, is unclear. A 54-year-old man presented with progressive weakness and dysesthesia of the right upper limb over 2 months. Cervical magnetic resonance imaging (MRI) revealed T2 hyperintense lesions in the area postrema and lower cervical/upper thoracic spinal cord, the latter of which demonstrated ring enhancement. Serum AQP4-Ab was positive on cell-based assay. He was initially diagnosed with NMOSD and treated with immunotherapy, but his neurological deficits progressed, and follow-up MRI demonstrated lesion expansion. Given the atypical course, a spinal cord biopsy was performed, and he was diagnosed with glioma with H3K27 M mutation; tumor cells were also AQP4-positive. Subsequent cancer-directed treatment led to improvement of spinal cord swelling and lesion enhancement. This case demonstrates that gliomas can present with both AQP4-Ab positivity and imaging features mimicking NMOSD, highlighting that the diagnosis of NMOSD should be based on comprehensive evaluation of antibody results, imaging findings, and the clinical course.
We present the case of a 32-year-old woman who developed recurrent thunderclap headaches, initially misattributed to migraine, ultimately diagnosed as reversible cerebral vasoconstriction syndrome (RCVS) based on multifocal arterial narrowing and clinical presentation. Her course was complicated by right occipital intracerebral hemorrhage (ICH) and later by formation of a cerebral abscess at the prior hemorrhagic site. Cultures revealed methicillin-sensitive Staphylococcus aureus without a clear systemic infectious source; dermatologic seeding was suspected. This case highlights two uncommon features: an RCVS-related ICH complicated by abscess formation and the absence of typical infectious risk factors. The pathophysiology was hypothesized to involve hematogenous spread in the context of transient blood-brain barrier disruption following hemorrhage. While RCVS typically has favorable outcomes, this case underscores the importance of maintaining a broad differential diagnosis in patients with evolving symptoms and neuroimaging findings. To our knowledge, this represents a rare instance of spontaneous abscess development at the site of RCVS-related hemorrhage in an immunocompetent individual.
Background and Purpose:Status epilepticus (SE) is a life-threatening emergency associated with high morbidity and mortality. Limited guidance exists on optimal therapy for refractory SE (RSE), which may include administration of repeat second-line IV-antiseizure medications (IV-ASMs) or escalation to IV-anesthesia (IVA). This study examined real-world treatment dynamics and outcomes of hospitalized patients with RSE in the United States. Methods:A retrospective, cross-sectional analysis of hospitalized patients with SE between 2018-2022 was performed using PINC AI™ Healthcare Database. Patient encounters for RSE were categorized into RSE-no IVA (≥2 IV-ASMs without IVA) and RSE-IVA (≥1 IV-ASMs with IVA and concomitant mechanical ventilation). RSE-no IVA episodes were further stratified by exposure to 2 IV-ASMs and ≥3 IV-ASMs. Results:Across 140 538 SE episodes in 113 229 unique patients, 44% were RSE. IV-ASM and IVA exposure, as well as time to escalation varied widely across SE episodes. Compared to RSE-no IVA episodes (59%), RSE-IVA episodes (41%) were associated with increased ICU length of stay (LOS; 5 vs 3 days), hospital LOS (9 vs 6 days), and in-hospital mortality (25% vs 12%). Compared to patients with RSE-no IVA administered 2 IV-ASMs, those administered ≥3 IV-ASMs had increased ICU admission (73% vs 62%), longer ICU LOS (4 vs 2 days), and hospital LOS (9 vs 5 days). Conclusions:Heterogeneity in RSE treatments management is prominent, with variation in IV-ASM treatment sequencing and escalation timing. Both increased IV-ASM utilization and IVA exposure were associated with similarly worse outcomes and healthcare utilization. Rapidly effective anti-SE treatments remain an urgent unmet need in this patient population.
Background: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. Purpose:To address these challenges, this article presents an interview study examining decisions that were retrospectively relevant for a surrogate decision-maker. Study Design: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. Study Sample: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. Analysis and Results: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. Conclusion: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.
Background:Optimal timing of pharmacologic venous thromboembolism (VTE) prophylaxis after spontaneous intracerebral hemorrhage (ICH) remains uncertain due to concerns regarding hematoma expansion. Prior studies have primarily relied on arbitrary time thresholds rather than physiological markers of hemorrhage stability. Objectives:To summarize the literature on the safety and efficacy of pharmacologic VTE prophylaxis initiated after radiographic confirmation of hematoma stability in patients with spontaneous ICH. Methods:We conducted a systematic review and meta-analysis of studies assessing heparinoids prophylaxis following repeat neuroimaging demonstrating hemorrhage stability. Three databases were searched: PubMed, Embase, Cochrane Central. The meta-analysis was registered in PROSPERO (CRD420261282903). The primary outcome was new or worsening intracranial hemorrhage, including hematoma expansion or new bleeding. Secondary outcomes included deep vein thrombosis and pulmonary embolism. Random-effects models were applied. P value was set at 0.05. Results:Four observational studies comprising 935 patients met inclusion criteria. Comparative meta-analysis demonstrated no significant association between pharmacologic prophylaxis after radiographic stability and intracranial bleeding risk (OR: 1.24, 95% CI: 0.80-1.94, P = 0.33), with negligible heterogeneity (I2 = 0%). Sensitivity analysis restricted to parenchymal hematoma expansion showed similar results (OR 2.37, 95% CI: 0.42-13.43, P = 0.33). Thromboembolic outcomes trended in favor of pharmacological prophylaxis without reaching statistical significance (OR: 0.63, 95% CI: 0.33-1.22, P = 0.17). Conclusions:Pharmacologic VTE prophylaxis initiated after radiographic confirmation of hematoma stability does not increase intracranial hemorrhage risk and may reduce thromboembolic complications. These findings support further investigation into imaging-guided, individualized strategies for pharmacologic VTE prophylaxis after ICH.
We describe a unique case of combined baclofen and tizanidine overdose followed by dual withdrawal syndromes, highlighting overlapping and contrasting clinical features that may complicate diagnosis and management. A 26-year-old man with paraplegia from cervical spinal cord injury, prescribed baclofen and tizanidine for spasticity, was found unresponsive at home next to partially empty medication bottles. Clinical evaluation included laboratory testing, neuroimaging, continuous monitoring, and serial neurologic and cardiopulmonary assessments. On admission, the patient exhibited CNS depression, hypotension, bradycardia, hypothermia, and subsequent episodes of severe psychomotor agitation. Supportive care with fluids, vasopressors, and sedation was required, including 2 subsequent intubations for airway protection. Within 24 hours, fever, tachycardia, and worsening agitation developed, consistent with withdrawal. Reintroduction of home baclofen and tizanidine alongside benzodiazepines and dexmedetomidine led to gradual resolution of symptoms, and the patient was discharged at neurologic baseline with psychiatric follow-up. This case underscores the diagnostic and therapeutic challenges of overlapping baclofen and tizanidine toxicity and withdrawal. Recognizing key bedside features-such as hypotonia vs tremor and mydriasis vs miosis-is essential. Sedation and bradycardia may be more severe in combined overdose than in overdose of either agent alone. Clinicians should anticipate withdrawal even in the setting of persistent toxicity. Early recognition and tailored supportive care are the cornerstone of management.
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.
