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Background: Current treatments for myasthenia gravis, including immunomodulatory therapies, are associated with significant comorbid events. Method: Retrospective chart review of all adults diagnosed with generalized myasthenia gravis in our clinic over 5 years to evaluate potential associations between treatment regimens and emergent comorbid events according to system organ class. Comorbid events were categorized by affected system organ class as endocrine, neuropsychiatric, musculoskeletal, gastrointestinal, pulmonary, cardiovascular, urologic, infectious, and hematologic. MG treatment regimens at the latest available date during the 1-year follow-up were categorized by corticosteroid use and further stratified by medication class and combination therapy. Result: A total of 68 patients were included in the analysis (corticosteroid group, n = 43; non-corticosteroid group, n = 25). We found no significant differences in the frequencies of comorbid events between patients whose regimens included corticosteroids and patients with corticosteroid-free regimens. Patients who received pyridostigmine, high-dose corticosteroids, and corticosteroid-sparing agents in combination experienced higher incidences of emergent endocrine and neuropsychiatric events than those who received other corticosteroid-containing treatments. Conclusion: Polypharmacy that included high-dose corticosteroids was associated with a greater incidence of new endocrine and neuropsychiatric comorbid events within 1 year of diagnosis

Background Despite the availability of several immunomodulatory therapies, about 20% of myasthenia gravis (MG) patients remain refractory to conventional treatments. There is limited evidence to support the use of maintenance therapeutic plasma exchange (TPE) therapy for refractory generalized MG. Methods Retrospective chart review of 14 patients with refractory generalized MG treated for 12 months with maintenance TPE therapy. Outcome measures were myasthenia gravis composite (MGC) score, myasthenia gravis activities of daily living (MG-ADL), number of acute exacerbations, medication changes, and adverse events. Data were collected at 3 monthly intervals for 12 months before and after initiation of TPE therapy. Results Clinically meaningful reductions in mean MG-ADL (>2 points) (mean MG-ADL score: 9.9 ± 0.5; 12-month pre-TPE to 5.2 ± 0.9; 12-month post-TPE) and MGC (>3 points) (mean MGC score: 25.2 ± 1.6; 12-month pre-TPE to 11.7 ± 1.4; 12-month post-TPE) were observed at 3 months following initiation of TPE and were maintained up to 12 months in all patients. After 12 months of TPE therapy, all patients had a significant reduction in daily prednisone and pyridostigmine use. Patients previously on IVIG or rituximab therapy were successfully weaned off both treatments. There was a significant reduction in acute MG exacerbations; 7.8 ± 1.1 mean exacerbations/patient (12-month pre-TPE) to 2 ± 1.1 mean exacerbations/patient (12-month post-TPE). Conclusion Over a period of 12 months, maintenance TPE therapy improved MG-ADL, and MGC with decreased immunosuppressant requirement, while being well-tolerated.

Context: Current guidelines recommend bed rest for 24 hours after receiving intravenous alteplase which may not be necessary and delay rehabilitation in a large proportion of acute ischemic stroke patients. Objective: To determine the feasibility of “early mobilization protocol” within the intensive care unit (ICU) in patients with acute ischemic stroke who received intravenous alteplase. Methods: All consecutive patients were admitted to the ICU with an acute ischemic stroke who received intravenous alteplase from October 2019-June 2021 were considered for “early mobilization protocol”. Patients≥18 years of age with minor, moderate or severe ischemic stroke (NIHSS score ≤22) were eligible and mobilization was initiated within 13-24 hours after intravenous alteplase administration. Exclusion criteria included hemodynamic instability, on mechanical ventilation, unstable neurological examination or progressive symptoms, or presence of external ventricular drain for hemorrhagic transformation within 24 hours of intravenous alteplase. Results: A total of 137 patients (56.8%) patients were eligible among 241 patients who were admitted to our institution with acute ischemic stroke after receiving intravenous alteplase. Mean age (±SD) was 63.4±16.6 and 66.2±17.0 years for the patients included and excluded in the early mobilization protocol, respectively. The mean NIHSS score (±SD) at baseline was 5.3±4.4 and 9.9±7.3 for the patients included and excluded in the early mobilization protocol, respectively. Early mobilization protocol was initiated at 18.3±3.6 hours in eligible patients compared with routine mobilization initiated at 41.6±19.8 hours in excluded patients (p Conclusion: Early mobilization protocol in patients post intravenous alteplase treatment can be initiated in the ICU in appropriately selected patients without any adverse effects on neurological and functional outcomes.

BACKGROUND The tolerability of high-dose oral corticosteroids in patients with generalized myasthenia gravis (gMG) has not been systematically assessed. We evaluated adverse side effects (ASEs) of corticosteroid treatment in patients with gMG. MATERIAL AND METHODS Retrospective analysis was conducted of ASEs reported as being related to corticosteroid treatment in 39 patients with gMG who were treated with oral corticosteroids for ≥1 year. RESULTS Median (interquartile range [IQR]) age was 60 (21) years, 53.8% of patients were women, and 66.7% were aged ≤65 years. Median (IQR) prednisone treatment duration was 14 (2) months; median (IQR) daily dose was 40 (15) mg. The median number of ASEs reported as corticosteroid-related was 2/patient (IQR, 1). Pre-diabetes and weight gain were most common (each 43.6% of patients). Bruising, insomnia, and osteoporosis were more prevalent in patients aged >65 years, while irritability, osteopenia, and pre-diabetes were more common in patients aged £65 years, although differences were not statistically significant. Irritability and weight gain were more prevalent in women (P=0.010 for irritability); osteoporosis and pre-diabetes more common in men (P=0.015 for osteoporosis). ASEs were generally more common in the high-dose prednisone group (>30 mg/day), but were only statistically significant for irritability (P=0.001). CONCLUSIONS Corticosteroid-related ASEs were common in patients with gMG. Some of these ASEs can have serious medical consequences, and certain ASEs appeared to be associated with specific patient characteristics. Demographics and comorbidities of patients with gMG must be carefully considered before corticosteroid initiation. Potential ASEs, such as unanticipated osteoporosis in men, require extra vigilance.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting both the central and peripheral nervous system. The median survival rate for ALS patients after symptom onset is 2.5 to 3.5 years and after diagnosis of ALS is about 1.5 to 2.5 years. Patients with ALS can have a wide spectrum of sleep disorders including but not limited to insomnia, sleep related breathing disorders, parasomnias, obstructive sleep apnea (OSA) and nocturnal hypoventilation (NH). Sleep-related breathing disorders substantially increase both morbidity and mortality in ALS patients. In this review, we have discussed the ALS motor symptoms, sleep-related breathing disorders, behavioral abnormalities and sleep disturbing factors which impair the health-related quality of life.

Introduction: Diagnosis of Guillain Barre syndrome (GBS) is often made clinically. Certain patient and disease characteristics can cause delays in diagnosis and management.Methods: Observational retrospective study of forty-four patients diagnosed with GBS either clinically, cerebrospinal fluid analysis, and/or by electro-diagnostic criteria at a teaching hospital (University of Missouri Hospital) in Columbia, Mid-Missouri between 2011 and 2017.Results: Patients with coexisting neurological conditions had statistically significant delay in diagnosis of GBS [Mean (SD); 13 ± 5 vs. 9.39 ± 4.7; p = 0.03]. Patients presenting with motor + symptoms (sensory and/or autonomic, in addition to motor), compared to those with only motor symptoms had statistically significant delay in diagnosis of GBS [Mean (SD); 11.90 ± 5 vs. 8.58 ± 4; p = 0.04].Discussion: Presence of co-existing neurological conditions, and motor + symptoms can delay timely diagnosis and management of GBS.

Objective: To report the case of a 35-year-old woman with treatment-resistant aquaporin-4 (AQP-4) immunoglobulin G (IgG) seronegative neuromyelitis optica spectrum disorder (NMOSD) successfully treated with eculizumab (a terminal complement inhibitor).Methods: The investigational procedures and treatment regimens the patient received were documented over 8 years [2012 (first presentation) to 2020].Results: The patient presented with subacute onset of lower-limb weakness and numbness, gait imbalance, and urinary incontinence. Magnetic resonance imaging (MRI) showed abnormalities in the thoracic spine from T7 to T10, but brain and cervical spine scans, visual evoked potential latencies, and IgG index were normal; cerebrospinal fluid pleocytosis and oligoclonal bands were both present. After treatment with intravenous methylprednisolone 1 g/day for 5 days, the patient was discharged without medication to acute rehabilitation but experienced relapses from 2012 to 2014. She was treated with oral prednisone (initiated at 40 mg/day in 2014; the dose was halved in 2015 due to weight gain) and mycophenolate mofetil (MMF) 1 g twice daily (from June 2015), but between 2014 and 2019 experienced 4–5 relapses/year, requiring treatment with intravenous methylprednisolone, with added maintenance plasma exchange from 2018 onwards. Although the patient tested negative for antibodies to AQP-4 and myelin oligodendrocyte glycoprotein, she was diagnosed with NMOSD in February 2017, based on recurrent episodes of longitudinal extensive transverse myelitis, MRI changes, and area postrema syndrome. By 2018 the patient needed a cane to walk. Prednisone and MMF were discontinued mid-2018, and rituximab was prescribed from July 2018 (maintenance regimen two 1 g doses 2 weeks apart every 6 months) but discontinued in July 2019 owing to lack of significant improvement. From July 2019 eculizumab was prescribed for 6 months (900 mg weekly for the first four doses, then 1200 mg every 2 weeks). The patient had no relapses or adverse events during and after eculizumab treatment (as of August 2020) and was able to walk unaided; her Expanded Disability Status Scale score improved from 4–5 during 2015–2018 to 2 in 2020 following eculizumab treatment.Conclusion: Eculizumab shows promise as a treatment for AQP-4 IgG-seronegative NMOSD and further studies are warranted.

Background: Although established therapies are effective in most patients with generalized myasthenia gravis (gMG), some patients do not respond or they experience intolerable adverse events, highlighting the need for better tolerated, targeted therapies for treatment-refractory gMG. Objective: To describe real-world experience with eculizumab in patients with treatment-refractory acetylcholine receptor antibody-positive (AChR+) gMG. Methods: Retrospective chart review of 15 patients with treatment-refractory AChR+ gMG treated for 12 months with eculizumab (900 mg/week for 4 weeks then 1200 mg every 2 weeks). Outcome measures were Myasthenia Gravis–Activities of Daily Living (MG-ADL) scores, number of exacerbations, single-breath count test (SBCT) score, medication changes, selected Quantitative Myasthenia Gravis (QMG) evaluations, and adverse events. Data collected at 3-monthly intervals for 12 months before and after eculizumab initiation were analyzed. Results: Clinically meaningful reductions in total MG-ADL scores were observed at 3 months following eculizumab initiation and maintained up to 12 months in all patients. After 12 months’ eculizumab treatment, there was a significant reduction in the number of acute exacerbations; mean (SD) SBCT score improved from 28.13 (0.33) to 50.26 (2.86); all patients achieved a ‘none’ or ‘mild’ rating for QMG evaluations; all patients reduced their daily prednisone dose; and nine patients had discontinued pyridostigmine. At the end of treatment, intravenous immunoglobulin was discontinued in all six patients receiving this therapy at eculizumab initiation. Eculizumab was well tolerated. Conclusions: This real-world study demonstrated improvement in outcome measures and decreased concomitant drug requirement within 12 months of eculizumab initiation in patients with treatment-refractory AChR+ gMG.

Highlights • Neurological Symptoms are being reported commonly in COVID-19 patients. • Mechanism of Seizure manifestation is still Unclear. • It is important to maintain control of epilepsy with AEDs in Patients with Epilepsy (PWE) infected with COVID-19 as mortality associated with epilepsy is higher in patients with uncontrollable seizures. • Consider possible interactions between epilepsy medications and the agents currently being used for symptomatic management in patients with COVID-19., Since the emergence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in Wuhan, China, this highly transmissible virus has since spread rapidly around the world. Though respiratory complication is the primarily reported manifestation though rare, yet serious neurological complications are being frequently reported in the literature. In selected coronavirus disease-2019 (COVID-19) cases neurologic complications may manifest as seizures. In this paper, we have reviewed current literature on seizures linked with SARS- COV 2 infection including published or pre-print original articles, review articles, and case reports. We have discussed the electroencephalogram (EEG), imaging, and Cerebrospinal fluid (CSF) findings in COVID-19 patients presenting with seizure. We will be concluding the paper by briefly discussing the three possible seizure development mechanisms in patients infected with SARS- COV 2, which includes - (a) Direct Mechanism (b) Indirect Mechanism and (c) Exacerbation of Seizure in Patients with Epilepsy (PWE). Our aim is to update the physicians working with COVID-19 patients about this potential complication and hope that understanding of these proposed mechanisms can provide an opportunity for the physicians for early diagnosis or even better, help prevent this complication.

Introduction While coronavirus disease 2019 (COVID-19) mostly causes respiratory illnesses, emerging evidence has shown that patients with severe COVID-19 can develop complications like venous thromboembolism (VTE) and arterial thrombosis as well. The incidence of thrombosis among critically ill patients in the literature has been highly variable, ranging from 25 to 69%. Similarly, reported mortality among critically ill patients has been highly variable too, and it has ranged from 30 to 97%. In this study, we analyzed data from a large database to address the incidence, the risk factors leading to thrombotic complications, and mortality rates among COVID-19 patients. Material and methods Data were obtained from TriNetX (TriNetX, Inc., Cambridge, MA), a multinational clinical research platform that collects medical records from 42 healthcare organizations (HCOs). All nominal data were compared using the chi-squared test. Alpha of

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) cases were first reported in Wuhan, Hubei province of China in December, 2019. SARS- COV-2 primarily affects the cardio-respiratory system. Over the last few months, several studies have described various neurological sequelae of SARS-COV-2 infection. Neurological complications are more frequent in patients with severe respiratory infections. In this review, we have analyzed the current literature on neuromuscular complications associated with SARS-COV-2 and highlighted possible mechanisms of neuromuscular invasion. We reviewed 11 studies describing 11 cases of Guillain Barre syndrome (GBS), and 1 case each of Miller Fisher syndrome, Polyneuritis Cranialis, Acute myelitis, Oculomotor paralysis and Bell's Palsy associated with SARS-COV-2 infection. Mean age of patients with GBS was 61.54 years, with standard deviation (SD) 14.18 years. Majority patients had fever and cough as the first symptom of SARS COV-2 infection. Mean time for onset of neurological symptoms from initial symptoms in 11 patients was 8.18 days, with SD of 2.86 days. Mean time to performing electrodiagnostic study from onset of neurological symptom was 6 days with standard deviation of 3.25. Six patients had demyelinating pattern, three had acute sensory motor axonal neuropathy, and one had acute motor axonal neuropathy on electrodiagnostic studies.

Central venous catheter (CVC) insertion is extensively utilized in Intensive Care Units for evaluation of hemodynamic status, administration of intravenous drugs, and for providing nutritional support in critically ill patients. Unfortunately, CVC use is associated with complications including lung injury, bleeding, infection, and thrombosis. We present a patient with an acute ischemic stroke from an inadvertently placed CVC into the right common carotid artery. A 57-year-old male presented to our institution for left hemiplegia and seizures 2 days after a CVC was placed. He was found to have a right frontal ischemic stroke on computed tomography (CT). CT angiography noted that the catheter was arterial and had a thrombosis around it. He was started on a low-dose heparin infusion. A combination of cardiothoracic surgery and interventional cardiology was required to safely remove the catheter. Central arterial catheterization is an unusual cause for acute ischemic stroke and presents management challenges.

Background.Continuous electroencephalography (CEEG) monitoring is used for detection of convulsive and nonconvulsive seizures and assessing the degree of encephalopathy in critically ill patients. A commonly seen encephalopathic pattern on CEEG is generalized periodic discharges with triphasic wave (TW) morphology. The underlying role and prognostic significance of TW in relationship to intracranial pressure (ICP) remain unknown. We present a case highlighting the relationship of TW with ICP in a case with severe traumatic brain injury (TBI).Method.Case report.Results.A patient with severe TBI and no underlying metabolic abnormalities was admitted to the neurocritical care unit. TW were seen on CEEG. The TW diminished during episodes of intracranial hypertension but reappeared with reduction of the intracranial pressure.Conclusion.This study highlights a possible favorable prognostic marker of finding TW in a patient with intracranial hypertension. We have proposed a preliminary understanding of the relationship between TW and intracranial hypertension, which may be helpful in formulating future studies involving larger cohorts.

Malignant Hyperthermia (MH) is a life-threatening pharmacogenetic disorder which results from exposure to volatile anesthetic agents and depolarizing muscle relaxants. It manifests as a hypermetabolic response resulting in tachycardia, tachypnea, hyperthermia, hypercapnia, acidosis, muscle rigidity and rhabdomyolysis. An increase in the end-tidal carbon dioxide is one of the earliest diagnostic signs. Dantrolene sodium is effective in the management of MH, and should be available whenever general anesthesia is administered. This review also aims to highlight the genetics and pathology of MH, along with its association with various inherited myopathy syndromes like central core disease, multi-mini core disease, Native-American myopathy, and King-Denborough syndrome.

Cerebral vasospasm and delayed cerebral ischemia are well-known complications of an aneurysmal subarachnoid hemorrhage (aSAH), generally occurring days to weeks after hemorrhagic ictus. Management strategies for these complications are controversial and vary in efficacy. There is a growing interest in supporting the use of intravenous (IV) milrinone to manage vasospasm. A 31-year-old male presented to the hospital after being found down outside his home. Computed tomography (CT) of the head and subsequent CT angiogram revealed a Fisher Grade 4 aneurysmal subarachnoid hemorrhage (aSAH). Six hours after admission, he became hypotensive and his neurological examination declined. A repeat CT head showed a new, left frontoparietal intracerebral hemorrhage (ICH) along with increasing SAH. He was stabilized with vasopressors and underwent emergent decompressive hemicraniectomy with subsequent clipping of the aneurysm. Approximately one week later, transcranial Doppler (TCD) showed increasing mean flow velocities in the bilateral anterior and middle cerebral arteries consistent with cerebral vasospasm. He was treated with intravenous milrinone. Repeat TCD 6.5 hours after the initial TCD showed improved mean flow velocities. His cardiac function by echocardiogram assessment was normal. The decrease in TCD velocity following treatment with milrinone indicates an improvement in the cerebral vasospasm regardless of cardiac output in a patient with subarachnoid hemorrhage. This case suggests that augmenting cardiac output may not be the only mechanism for the therapeutic benefit of milrinone.

Thromboelastography with platelet mapping (TEG-PM) is a modality to measure platelet function, especially in patients taking antiplatelet medications. It consists of two components: arachidonic acid (AA), which is sensitive to aspirin, and adenosine diphosphate (ADP), which is sensitive to clopidogrel. In patients with spontaneous intracerebral hemorrhages (sICH), the clinical interpretation of platelet mapping is unclear. The objective of this study was to evaluate TEG-PM in patients with sICH on aspirin and/or clopidogrel who receive platelet transfusions. This study was an IRB-approved, retrospective case-control study over three years at an academic medical center. Adult patients with sICH were included if they had an admission computed tomography head (CTH) and platelet mapping followed by a repeat platelet mapping and CTH post platelet transfusion. A threshold of 50% inhibition was used as the benchmark for both ADP and AA inhibition. Around 248 subjects with sICH were identified, and 107 were excluded for incomplete documentation, leaving 141 for analysis. Of these, nine met our inclusion criteria. No statistical significance was found on the antithrombotic effects of aspirin or clopidogrel on TEG-PM (p=1.00 for both). Sensitivity and specificity of TEG-PM for clopidogrel was 100% and 42.9%, respectively, and 80% and 0%, respectively, for aspirin. Platelet transfusion did not significantly change AA or ADP inhibition (p=1.00). Hemorrhagic expansion on CTH was not associated with a decrease AA or ADP inhibition (p=1.00). TEG-PM is not an effective measure of platelet inhibition in sICH patients who were on antiplatelet medications and is not a reliable measurement following platelet transfusion.

Delayed post-hypoxic leukoencephalopathy (DPHL) is a unique clinical entity that presents with cognitive impairment days to weeks after an episode of acute hypoxic brain injury. Frequently hypoxia is unrecognized as a mechanism for clinical decline and extensive workup ensues. We present two cases of DPHL highlighting the neuroimaging findings. In both patients, a cerebral hypoxic event was followed by a recovery phase with subsequent delayed clinical decline. Patient 1 suffered hypoxia from drug-induced respiratory depression and lack of post-operative positive airway pressure (PAP) support. Her neurological exam on follow-up revealed progressive cognitive decline. Magnetic resonance imaging (MRI) brain showed bilateral white matter changes involving the centrum semiovale. Patient 2 developed a generalized tonic-clonic seizure during an endobronchial biopsy procedure and was found to have multiple air emboli on computed tomography (CT) head scan. She was initially in a drug-induced coma for her seizures. Electroencephalography (EEG) on day 14 of admission showed changes consistent with diffuse encephalopathy. MRI brain showed bilateral white matter changes particularly at the watershed zones and in the centrum semiovale. DPHL is a rare and under-recognized clinical entity that requires clinical suspicion and detailed evaluation for diagnosis. Neuroimaging studies can provide prognostic information regarding the extent of neurological injury.

Background: Patients with intracerebral hemorrhages (ICHs) have higher incidence of seizures. Previous studies have suggested that location and size of hemorrhage may increase epileptogenicity. We aim to evaluate seizure development risk factors from clinical examination, imaging, and continuous electroencephalography (cEEG) in critically ill patients with ICH. Methods: We reviewed 57 consecutive patients with ICH admitted to a neurocritical intensive care unit over a 24-month period who were monitored on cEEG. Their demographic and examination data, ICH score, Glasgow Coma Scale (GCS), location of bleed, cEEG patterns, and discharge status were analyzed. Results: Sixteen (28%) patients from our study cohort had seizures at a mean duration of 7.46 h from cEEG hookup. Fifteen (93%) of those patients had only electrographic seizures. The finding of lateralized periodic discharges (LPDs) was significantly (P = 0.019) associated with seizures. Other variables, such as ICH score, size and location of hemorrhage, GCS, mental status, and other cEEG patterns, were not significantly associated with seizures. Conclusion: We found that LPDs were predictive of seizures in ICH patients. cEEG for longer than 24 h is preferred for detection of seizures as they occurred at a mean later than 7 h and most were without clinical signs.

Somatosensory evoked potentials (SSEPs) are a sensitive, minimally invasive technique used to localize dysfunction of myelinated peripheral and central axons in the nervous system. The utility of SSEPs in acutely assessing central nervous system function in brainstem herniation in the neuroscience intensive care unit (NICU) has not been well established. We discuss a case of an 18-year-old, postpartum female who presented with intermittent headache, diplopia, nausea/vomiting and cachexia following delivery two months prior. Shortly after arrival to the emergency department, she developed flaccid quadriparesis and complete ophthalmoplegia. Computed tomography (CT) of the head showed effacement of the basal cisterns along with 2 cm cerebellar tonsillar herniation into the foramen magnum concerning for intracranial hypotension. Raising the head of bed caused hemodynamic instability necessitating prolonged Trendelenburg positioning. The patient was evaluated with serial SSEPs which initially showed a bilateral low amplitude N20 response and normal N13 response. Subsequent SSEP testing showed increased N20 amplitude which correlated with clinical improvement in the patient. SSEP is a minimally invasive and sensitive method used to assess the integrity of the somatosensory nervous system pathway; SSEPs may be a useful monitoring adjunct to assess the evolution of posterior fossa lesions leading to brainstem compression.

Herpes simplex-1 virus encephalitis (HSE) is the most commonly recognized cause of sporadic encephalitis in the United States. Historically HSE has been considered extremely detrimental given the associated relentless neurological deterioration secondary to cerebral edema and status epilepticus. With recent advances in antiviral therapeutics in past decades, the majority of complications can be managed effectively although the associated morbidity and mortality still remains high. The key modifiable factor determining recovery is the rapid initiation of antiviral therapy. We discuss the case of a 19-year-old female with HSE who received standard acyclovir therapy. Despite using recommended dosage and duration of acyclovir, her clinical condition worsened significantly and subsequently required multiple antiviral therapeutics and steroid therapy.

Background Seizures are a considerable complication in critically ill patients. Their incidence is significantly high in neurosciences intensive care unit patients. Seizure prophylaxis with anti-epileptic drugs is a common practice in neurosciences intensive care unit. However, its utility in patients without clinical seizure, with an underlying neurological injury, is somewhat controversial. Body In this article, we have reviewed the evidence for seizure prophylaxis in commonly encountered neurological conditions in neurosciences intensive care unit and discussed the possible prognostic role of continuous electroencephalography monitoring in detecting early seizures in critically ill patients. Conclusion Based on the current evidence and guidelines, we have proposed a presumptive protocol for seizure prophylaxis in neurosciences intensive care unit. Patients with severe traumatic brain injury and possible subarachnoid hemorrhage seem to benefit with a short course of anti-epileptic drug. In patients with other neurological illnesses, the use of continuous electroencephalography would make sense rather than indiscriminately administering anti-epileptic drug.

Obtaining neuroimaging in patients on cardiopulmonary support devices such as extracorporeal membrane oxygenation (ECMO) can be challenging, given the complexities in monitoring, instrumentation, and associated hemodynamic lability. Transcranial Doppler (TCD) is used as an ancillary test for the assessment of cerebral circulatory arrest, but its use in non-pulsatile blood flow in venoarterial (VA) ECMO is not well described. We report the use of TCD in a patient on VA ECMO post-cardiac arrest for evaluation of death by neurological criteria. A 72-year-old female was admitted for elective trans-catheter aortic valve replacement. Her postoperative course was complicated by hemo-pericardium evolving into pulseless electrical activity causing cardiac arrest. She was resuscitated with return of spontaneous circulation and initiated on VA ECMO and intra-aortic balloon pump for cardiogenic shock. Over the next few days, serial evaluations persistently showed a poor neurological examination. She was too unstable to transport for neuroimaging. Evaluation for death by neurological criteria was performed with a clinical examination, apnea testing, and TCD as an ancillary study. TCD showed systolic spikes supporting an impression of cerebral circulatory arrest consistent with an irreversible brain injury.

Internal carotid artery dissection (ICAD) accounts for 25% of cerebrovascular accidents in young and middle-aged patients. Dissection occurs when the intimal wall of an artery is damaged as a result of trauma or defect. ICAD development after dental work is a relatively uncommon phenomenon. Our study highlights a rare presentation of ICAD that resulted from a direct lidocaine nerve block injection in a patient undergoing pulpotomy for a right maxillary second premolar tooth. We have described the case and reviewed the literature on this rare but potentially life-threatening phenomenon.

Respiratory failure can be the result of impairment in the central control of respiration, neuromuscular disorders or due to intrinsic lung problems. In the neuromuscular causes of respiratory failure, several factors like upper airway problems, inspiratory/expiratory muscle weakness, low tidal volumes, etc. can be contributory. Features like rapid shallow breathing, use of accessory muscles of respiration, cough after swallowing, and paradoxical breathing point towards the possibility of impending respiratory failure. For physicians treating neuromuscular disorders, identification of early signs of impending respiratory failure is crucial for better patient outcomes. In this chapter, we review the physiology and pathology of respiration in the setting of neuromuscular disorders along with objective assessment of respiratory failure.

Although a neurological examination is fundamental to the evaluation of comatose patients, it is less reliable in a medically induced coma. A commonly misinterpreted finding in patients in a pentobarbital coma is altered pupillary reactivity secondary to an exaggerated ciliospinal reflex. Recognizing an exaggerated ciliospinal reflex in patients in a pentobarbital coma is important and may prevent unnecessary intervention. We present a patient induced in a pentobarbital coma for the treatment of status epilepticus who exhibited a nonreactive pupil secondary to an exaggerated ciliospinal reflex confirmed by pupillometry. We also discuss the anatomy of the ciliospinal reflex and literature regarding its clinical relevance.

Cerebrovascular autoregulation may be dysfunctional after acute intracerebral hemorrhage (ICH). This disruption in autoregulation can potentially result in secondary neurological damage that may present as an intracranial hemorrhage at locations distant from the primary site of hemorrhage. We discuss a case of 68-year-old female who presented with acute left hemiparesis from a spontaneous right frontal ICH. Magnetic resonance imaging (MRI) was negative for any other blooming artifact. Her weakness was improving, but after 72 hours from admission, she had an acute change in her mental status and was found to have a new left frontal ICH distant from the primary hemorrhage. Cerebral dysregulation following spontaneous ICH may predispose patients with risk factors, such as chronic hypertension, to a secondary spontaneous ICH distant from initial ICH. Recognizing this phenomenon can guide the management of acute ICH.

Pure ocular presentation of Lambert-Eaton syndrome is not a common phenomenon. Such presentation poses significant diagnostic challenges and requires conscientious evaluation. In this review, we have described a case of a patient with pure ocular weakness, initially diagnosed as seronegative ocular myasthenia which on further evaluation was found to have ocular Lambert-Eaton myasthenic syndrome (LEMS).

Amyotrophic lateral sclerosis (ALS) is a clinically progressive neurodegenerative syndrome predominantly affecting motor neurons and their associated tracts. Riluzole and edaravone are the only FDA certified drugs for treating ALS. Over the past two decades, almost all clinical trials aiming to develop a successful therapeutic strategy for this disease have failed. Genetic complexity, inadequate animal models, poor clinical trial design, lack of sensitive biomarkers, and diagnostic delays are some of the potential reasons limiting any significant development in ALS clinical trials. In this review, we have outlined the possible reasons for failure of ALS clinical trials, addressed the factors limiting timely diagnosis, and suggested possible solutions for future considerations for each of the shortcomings.