Myoclonic Status Research Articles

Can we predict neurological outcome following out-of-hospital cardiac arrest (OHCA) and therapeutic hypothermia?

Can we predict neurological outcome following out-of-hospital cardiac arrest (OHCA) and therapeutic hypothermia?

Transient myoclonic state with asterixis presenting as persistent hyperperfusion on single‐photon emission computed tomography: A case report

AbstractTransient myoclonic state with asterixis is characterized by sudden onset generalized myoclonus and asterixis without consciousness impairment. Electrophysiological studies have shown that myoclonus correlates with cortical hyperexcitability localized in the primary motor cortex during the symptomatic period. However, it is unclear whether this excitability remains in the asymptomatic period. Here, we report a 79‐year‐old Japanese man who presented with transient myoclonic state with asterixis. He had been diagnosed with progressive non‐fluent aphasia and he suffered from rhythmic myoclonus; predominantly in the neck, shoulders and upper extremities. Asterixis was observed in the forearms. Brain 123I‐iodoamphetamine single‐photon emission computed tomography showed focal hyperperfusion in the bilateral precentral gyri even at 3 months after remission of the myoclonus. These data suggest that cortical hyperactivity was persistent without symptoms and led to transient myoclonic state with asterixis, which could have been due to the underlying neurodegenerative disorder of progressive non‐fluent aphasia.

Abstract 257: Combining the Initial Four B Score and Continuous EEG to Predict Survival After Cardiac Arrest

Background: Outcome prediction after cardiac arrest is a challenging problem and multiple tests should be performed to predict outcome accurately. We tested whether the combination of initial brainstem reflex examination (FOUR_B score) and continuous EEG were superior to either test alone for predicting survival after cardiac arrest. Methods: Review of consecutive patients receiving continuous EEG monitoring between April 2010 and June 2013. Patients were divided into three groups according to initial FOUR_B score; FOUR_B = 0-1, FOUR_B = 2 and FOUR_B = 4. A blinded rater dichotomized continuous EEG patterns during the first 48 hours into malignant patterns (non-convulsive status epilepticus, convulsive status epilepticus, myoclonic status epilepticus and generalized periodic epileptiform discharges). The primary outcome was survival to hospital discharge. Results: Of 380 subjects, 45 were excluded (44 missing initial FOUR_B score, 1 with age of 16 years). Mean (SD) age was 58 (17) and 208 (62.1%) subjects were male. Ventricular fibrillation was initial rhythm for 95 (28.4%) subjects. Survival to hospital discharge rate was 7.8% for FOUR_B 0-1, 32.0% for FOUR_B 2 and 51.9% for FOUR_B 4, respectively. Among subjects with malignant cEEG, survival to hospital discharge rate was 0% for FOUR_B 0-1, 8.1% for FOUR_B 2 and 12.5% for FOUR_B 4, respectively. All 42 subjects with no pupillary light reflex, no corneal reflex and malignant cEEG, had poor outcome (false positive rate 0; 95% CI, 0-7%). In the multivariate analysis, survival was independently associated with shockable rhythm (OR 5.76; 95% C.I. 2.88-11.54), FOUR_B 2 (OR 7.36; 95% C.I. 2.83-19.90), FOUR_B 4 (OR 8.99; 95% C.I. 3.58-22.56) and non-malignant cEEG (OR 19.91; 95% C.I. 6.44-61.51). Conclusion: Initial FOUR_B score and malignant cEEG pattern were independently associated with survival to hospital discharge. All subjects who have no pupillary light reflex, no corneal reflex and malignant cEEG did not survive to hospital discharge.

Prognostication in comatose survivors of cardiac arrest: an advisory statement from the European Resuscitation Council and the European Society of Intensive Care Medicine.

ObjectivesTo review and update the evidence on predictors of poor outcome (death, persistent vegetative state or severe neurological disability) in adult comatose survivors of cardiac arrest, either treated or not treated with controlled temperature, to identify knowledge gaps and to suggest a reliable prognostication strategy.MethodsGRADE-based systematic review followed by expert consensus achieved using Web-based Delphi methodology, conference calls and face-to-face meetings. Predictors based on clinical examination, electrophysiology, biomarkers and imaging were included.Results and conclusionsEvidence from a total of 73 studies was reviewed. The quality of evidence was low or very low for almost all studies. In patients who are comatose with absent or extensor motor response at ≥72 h from arrest, either treated or not treated with controlled temperature, bilateral absence of either pupillary and corneal reflexes or N20 wave of short-latency somatosensory evoked potentials were identified as the most robust predictors. Early status myoclonus, elevated values of neuron-specific enolase at 48–72 h from arrest, unreactive malignant EEG patterns after rewarming, and presence of diffuse signs of postanoxic injury on either computed tomography or magnetic resonance imaging were identified as useful but less robust predictors. Prolonged observation and repeated assessments should be considered when results of initial assessment are inconclusive. Although no specific combination of predictors is sufficiently supported by available evidence, a multimodal prognostication approach is recommended in all patients.Electronic supplementary materialThe online version of this article (doi:10.1007/s00134-014-3470-x) contains supplementary material, which is available to authorized users.

Prognostication in comatose survivors of cardiac arrest: An advisory statement from the European Resuscitation Council and the European Society of Intensive Care Medicine

ObjectivesTo review and update the evidence on predictors of poor outcome (death, persistent vegetative state or severe neurological disability) in adult comatose survivors of cardiac arrest, either treated or not treated with controlled temperature, to identify knowledge gaps and to suggest a reliable prognostication strategy. MethodsGRADE-based systematic review followed by expert consensus achieved using Web-based Delphi methodology, conference calls and face-to-face meetings. Predictors based on clinical examination, electrophysiology, biomarkers and imaging were included. Results and conclusionsEvidence from a total of 73 studies was reviewed. The quality of evidence was low or very low for almost all studies. In patients who are comatose with absent or extensor motor response at ≥72h from arrest, either treated or not treated with controlled temperature, bilateral absence of either pupillary and corneal reflexes or N20 wave of short-latency somatosensory evoked potentials were identified as the most robust predictors. Early status myoclonus, elevated values of neuron specific enolase at 48–72h from arrest, unreactive malignant EEG patterns after rewarming, and presence of diffuse signs of postanoxic injury on either computed tomography or magnetic resonance imaging were identified as useful but less robust predictors. Prolonged observation and repeated assessments should be considered when results of initial assessment are inconclusive. Although no specific combination of predictors is sufficiently supported by available evidence, a multimodal prognostication approach is recommended in all patients.

Retrospective clinical study of 8 cases with Angelman syndrome

Objective To explore the clinical and electroencephalogram(EEG) characteristics, genetic data and the effect of ketogenic diet(KD) for children with Angelman syndrome(AS). Methods The clinical and EEG characteristics of 8 patients with AS at the Department of Neurology, Children's Hospital of Fudan University from Jan.2012 to Dec.2013 were analyzed retrospectively.The treatment and prognosis were also followed up. Results Of 8 patients, there were 4 male and 4 female.All patients(100%) had apparently happy demeanor or smile, general developmental delay and movement disorder, and head circumference of 4 patients was smaller or microcephaly diagnosed.Epilepsy was present in 7 cases(88%) with onset age from 6 to 33 months.Most frequent types were myoclonic seizures, atypical absences, atonic seizures and generalized tonic-clonic seizures.Myoclonic status epilepticus was present in 4 cases(57%). Rhythmic delta and theta in the anterior, the posterior and the generalized areas mixing with spikes and ware waves were the characteristic EEG patterns.Genetic testing by methylation specificity multiple link probe amplification techniques(MS-MLPA)was done for all patients, 6 cases(75%) were confirmed with a maternal deletion involving the chromosome 15q11PWS/AS critical region.Seven patients were treated with multiple antiepileptic drugs, while 6 patients(86%) belonged to refractory epilepsy.Three cases were treated with KD, 1 month after KD, seizure improved effectively in 2 cases, but 1 case had no improvement, 3 months after KD, 1 case got seizure free, and 1 case had seizure reduced effectively, while 1 case with seizure had no improvement. Conclusions There are relative and characteristic EEG manifestations clinically in AS and the diagnosis can be assisted by genetic test.Epilepsy is common and myoclo-nic status epilepticus is also frequent in AS.Epilepsy in AS is often hard to control but KD can be used as a treatment option for AS with refractory epilepsy. Key words: Angelman syndrome; Epilepsy; Electroencephalogram; Ketogenic diet

Management of Refractory Status Epilepticus in an Actively Dying Patient

ABSTRACTNo consensus guidelines exist for the treatment of refractory myoclonic status epilepticus or refractory myoclonus in the palliative care setting. Evidence-based guidelines for the general medical population are often neither practical nor applicable at the end of life. Many challenges, including medication availability, route of administration, monitoring, and work-up are all unique to the palliative care setting. Two patients with refractory myoclonus versus refractory myoclonic status epilepticus are described here, illustrating the challenges involved in treatment as well the need for further research for therapy in the palliative care setting.

Early infantile epileptic encephalopathies

Epileptiform abnormalities contribute to progressive deterioration of cerebral function. Considered: Ohtahara Syndrome; Early myoclonic epileptic encephalopathy; West Syndrome; Dravet Syndrome; Myoclonic status in not progressive encephalopathies; CDKL5 encephalopaty. Ohtahara syndrome (OS) early infantile encephalopathy (EIEE). Most cases linked to cerebral malformations or very occasionally to metabolic disorders. Main seizures: tonic spasms, tonico- clonic, myoclonic and atonic and partial spasms. Treatment Vigabatrin, Topamax, Zonegran, Steroids, ACTH and Ketogenic diet, vagal stimulation and more invasive surgery. Mutations of the gene STBX1 (Sintaxin binding protein) Mutations of SPTA gene and GC1 have been reported. Prognosis is poor. Early Myoclonic Encephalopathy (EME). Onset: neonatal period or first months of life. Seizures mainly erratic, myoclonic or partial, tonic spasms. EEG burst suppression. Etiology mostly unknown. Inborn errors of metabolism as nonketotic hyperglycinemia, or propionic acidemia reported . Prognosis poor. Therapy similar to that for OS. West Syndrome (WS) aka as infantile spasms with hypsarrhythmia, EEG abnormality with asynchronous very high amplitude, irregular, continuous multifocal spike an slow wave discharges. In 80% of cases a cause can be found or suspected (symptomatic or criptogenethic)In about 20% of patients no cause can be found (idiopathic). 1:3200 / 1:3500 newborns. Causes: infections, cerebral malformations, hypoxic ischemic injuries, metabolic and genetic disorders, tuberosclerosis. Corticotropin considered effective. This finding later confirmed. No definite therapeutic scheme. High dose and long term treatment associated with hypertension, cuschingoid features and ipokalemia. Vigabatrin and Topiramate useful. Zonisamide, Lamotrigine and Levetiracetam can be used. Prognosis much better in idiopathic cases when treatment is started within the first month from the appearance of the symptoms. Myoclonic status in nonprogressive encephalopathies. Rare, onset in the first years of life. Partial motor seizures, myoclonic absences or massive myoclonias sometimes with startles. Interictal EEG epileptiform discharges and background slowing. Described in genetic conditions like Angelman and 4p – syndromes. Prognosis poor. Dravet syndrome (Severe myoclonic infantile epilepsy or SMEI). 1/500 cases of infantile epilepsy. Febrile convulsions in the first year. Subsequently myoclonic seizure with fever. Photosensitivity. In 70% of cases mutations of SCN1A gene. Difficult to treat. Topiramate, Clobazam, Stiripentol. CDKL5 Encephalopathy. Mutations in the human X linked cyclin–dipendent kinase like 5 (CDKL5), infantile spasms and Rett (RTT) like phenotype. Initially normal EEG and severe hypotonia. Aquired microcephaly and hand stereotypes. Identified with early infantile epileptic encephalopathy 2. Prognosis severe.

Predicting Neurologic Outcome After Targeted Temperature Management for Cardiac Arrest

Targeted temperature management improves survival and neurologic outcomes for adult out-of-hospital cardiac arrest survivors but may alter the accuracy of tests for predicting neurologic outcome after cardiac arrest. We systematically searched Medline, Embase, CINAHL, and CENTRAL from database inception to September 2012. Citations were screened for studies that examined diagnostic tests to predict poor neurologic outcome or death following targeted temperature management in adult cardiac arrest survivors. Data on study outcomes and quality were abstracted in duplicate. We constructed contingency tables for each diagnostic test and calculated sensitivity, specificity, and positive and negative likelihood ratios. Of 2,737 citations, 20 studies (n = 1,845) met inclusion criteria. Meta-analysis showed that three tests accurately predicted poor neurologic outcome with low false-positive rates: bilateral absence of pupillary reflexes more than 24 hours after a return of spontaneous circulation (false-positive rate, 0.02; 95% CI, 0.01-0.06; summary positive likelihood ratio, 10.45; 95% CI, 3.37-32.43), bilateral absence of corneal reflexes more than 24 hours (false-positive rate, 0.04; 95% CI, 0.01-0.09; positive likelihood ratio, 6.8; 95% CI, 2.52-18.38), and bilateral absence of somatosensory-evoked potentials between days 1 and 7 (false-positive rate, 0.03; 95% CI, 0.01-0.07; positive likelihood ratio, 12.79; 95% CI, 5.35-30.62). False-positive rates were higher for a Glasgow Coma Scale motor score showing extensor posturing or worse (false-positive rate, 0.09; 95% CI, 0.06-0.13; positive likelihood ratio, 7.11; 95% CI, 5.01-10.08), unfavorable electroencephalogram patterns (false-positive rate, 0.07; 95% CI, 0.04-0.12; positive likelihood ratio, 8.85; 95% CI, 4.87-16.08), myoclonic status epilepticus (false-positive rate, 0.05; 95% CI, 0.02-0.11; positive likelihood ratio, 5.58; 95% CI, 2.56-12.16), and elevated neuron-specific enolase (false-positive rate, 0.12; 95% CI, 0.06-0.23; positive likelihood ratio, 4.14; 95% CI, 1.82-9.42). The specificity of available tests improved when these were performed beyond 72 hours. Data on neuroimaging, biomarkers, or combination testing were limited and inconclusive. Simple bedside tests and somatosensory-evoked potentials predict poor neurologic outcome for survivors of cardiac arrest treated with targeted temperature management, and specificity improves when performed beyond 72 hours. Clinicians should use caution with these predictors as they carry the inherent risk of becoming self-fulfilling.

Meropenem-induced myoclonus: A case report

A 61-year-old woman was seen at the intensive care unit because of myoclonic status. Her medical history included arterial hypertension, dyslipidemia and diabetes. In the previous year she had been hospitalized because of dilated cardiomyopathy (ejection fraction 21%) and non-sustained ventricular tachycardia. At that time she was managed with an implantable cardiac defibrillator. On this admission she complained of precordial pain and had heart failure with a cold and dry profile, for which she was given vasoactive drugs and an intra-aortic balloon. Her coronary angiography was unremarkable, but a lower limb echo-Doppler revealed a high-flow arteriovenous fistula between the right superficial femoral artery and vein. The fistula was not managed surgically because of the patient’s clinical status. In the following days her dyspnea worsened, requiring non-invasive ventilation, and her central catheter became colonized. She developed oliguria, vomiting, hypoglycemia, syncope and cardiac arrest, which was successfully managed with cardiopulmonary resuscitation (CPR). A urinary infection ensued 5 days later and as her renal function had decreased, intravenous meropenem 1000 mg/day was started (dosing for CrCl of 10–25 mL/min: 500–1500 mg/day). Soon after

Generalized Myoclonus and Spasticity Induced by Lamotrigine Toxicity

Lamotrigine (LTG) is a well-tolerated broad-spectrum antiepileptic drug, which is chemically unrelated to other existing antiepileptic medications. The drug has also some mood-stabilizing properties and, according to some studies, modest antidepressant effects. The exact mechanism of action is unknown, but some animal studies suggest the inhibition of neuronal glutamate release. Despite being relatively safe, LTG has been demonstrated to have proconvulsant effect especially in certain types of epilepsies like myoclonic status epilepticus. Myoclonic status epilepticus and its variations including generalized myoclonic status epilepticus, status myoclonus, and prolonged myoclonus describe a variety of clinical states, which have continuous, unremitting seizures lasting longer than 5 minutes. It is not a commonly reported treatment-emergent neurological complication, but the treatment is always a medical emergency. We report a case of a 46-year-old man who developed generalized myoclonus status epilepticus a few hours after suicidal ingestion of LTG. He remained hemodynamically stable throughout hospitalization and started to recover and achieved complete recovery 3 days later. This is the first reported case of this de novo complication induced by LTG toxicity. We proposed a subcortical mechanism for this complication induced by the toxic doses of LTG.

Late-Onset Epilepsy with Status Myoclonicus: A Diagnostic and Management Dilemma

ABSTRACT.Status myoclonicus (myoclonic status epilepticus) has been described in generalized epilepsy syndromes, neurodegenerative disease, infectious or inflammatory neurologic disease, toxic-metabolic states, and following anoxic brain injury. It is extremely uncommon in elderly people. Hence, status myoclonicus can be a challenge to diagnose and manage especially if it is cryptogenic epilepsy (unknown cause). We describe the case of a 77-year-old female who had new-onset uncontrolled seizures despite three antiepileptic drugs. Following concern about nonepileptic events, she was eventually diagnosed by epilepsy monitoring to have status myoclonicus. Her seizures were then controlled with appropriate antiepileptic drug changes.

Neurological consequences of cardiac arrest: Where do we stand?

With increasing public education in basic life support and with the widespread use of automated defibrillators, post-cardiac arrest comatose patients represent a growing part of ICU admissions. However the prognosis remains very poor and only a very low proportion of these resuscitated patients will recover and will leave the hospital without major neurological impairments. Neurological dysfunction predominantly includes disorders of consciousness, and may also include other manifestations such as seizures, myoclonus status epilepticus and other forms of movement disorders including post-anoxic myoclonus. In the most severe cases, coma may be irreversible or evolve towards a minimally conscious state, a vegetative state or even brain death. These severe conditions represent by far the leading cause of mortality and disability in such patients. Currently, early use of mild therapeutic hypothermia is the only treatment that demonstrated its ability to decrease neurological consequences and to improve the prognosis. Prognostication outcome is still mainly based on a rigorous clinical evaluation coupled with neuro-physiological investigations, but brain functional imaging could become a valuable tool in the near future. Clinical research focusing on survivors should be strongly encouraged in order to assess the mid- and long-terms outcome of survivors and to evaluate the impact of new treatments or strategies.

Usefulness of bilateral bispectral index (BIS) monitoring in a comatose patient with myoclonic status epilepticus secondary to cefepime

Status epilepticus, particularly non-convulsive status epilepticus (NCSE), is a frequent complication in patients with altered renal function receiving treatment with intravenous cefepime. To the best of our knowledge, we report the first case, illustrated by video-EEG, of a critically ill patient receiving treatment with cefepime who developed an episode of confirmed symptomatic myoclonic status epilepticus (MSE). Case report and video-EEG. A 60-year-old man, who had received a liver transplant due to alcoholic cirrhosis one year ago, was admitted to our intensive care unit due to septic shock. Computed tomography revealed a prostatic abscess as cause of his sepsis. On Day 27, a respiratory infection due to Pseudomona aeruginosa was diagnosed, and treatment with intravenous cefepime (2 g/8 hours) was initiated. On Day 32, his mental status deteriorated and he developed inattention, a reduced level of consciousness, and multifocal and generalised continuous myoclonic jerks. A video-EEG study was compatible with the diagnosis of symptomatic MSE. On Day 35, cefepime was stopped and general anaesthesia with midazolam was started in order to achieve a faster clinical improvement. We used the BIS-Vista™ monitor to guide general anaesthesia and detect potential episodes of NCSE. On Day 40, an EEG confirmed the existence of moderate diffuse encephalopathy. Finally, the patient died as a consequence of severe heart failure. Cefepime may be a cause of MSE in non-anoxic comatose patients. Clinicians should be aware of this possibility when evaluating comatose patients on cephalosporin therapy in order to establish a correct diagnostic approach and accurate prognosis. [Published with video sequences].

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Introduction: We compared the sensitivity of intermittent electroencephalography (iEEG) and continuous electroencephalography (cEEG) in malignant pattern (MP) detection in post-cardiac arrest patients, and described the delay in time to detection. Methods: IRB approved, retrospective cohort study of post-cardiac arrest subjects treated with therapeutic hypothermia (TH) and monitored with cEEG from 4/1/2010 to 6/4/2013. We extracted demographic and EEG data from subjects who demonstrated MP, defined as generalized periodic epileptiform discharges (GPEDs), myoclonic status epilepticus (MSE) and nonconvulsive status epilepticus (NCSE), during the first 48 hours after application of EEG. Post cardiac arrest category (PCAC) was determined on hospital arrival. The results were compared with simulated iEEG sampling intervals of 6, 8, and 12 hours post EEG initiation and twice daily at 9 am and 5pm, with each spot check lasting 20 minutes. The primary outcome was sensitivity of MP detection. The secondary outcome was time delay in MP detection. Data were analyzed with ANOVA and Mann-Whitney U test. Results: In 380 subjects included, mean age was 57 (SD 17), most were male (N=236, 62%) and experienced out-of-hospital cardiac arrest (N=289, 76%). The predominant primary rhythm was ventricular fibrillation (N=116, 30%), and PCAC IV (N=203, 61%). A total of 110 (29%) subjects survived in the cohort, with 7 (6%) surviving after MP development. MPs were detected in 111 (30%) subjects with 62 (56%) developing GPEDs, 67 (60%) developing MSE, and 28 (25%) developing NCSE. Median duration of MP was 26 hours (IQR 13, 39; range 1–48). MP detection sensitivity varied with iEEG sampling intervals of 6, 8, and 12 hours and twice daily at 9am and 5pm, with corresponding overall detection sensitivities of 94.3% (151/160), 90.6% (145/160), 90.6% (145/160), and 83.1% (133/160) [p=0.0068]. Similarly, average delay in detection varied by sampling interval (1.2 hours (IQR 0, 2.5), 1.7 hours (IQR 0, 3), 2.6 hours (IQR 0, 4.8) and 6.2 hours (IQR 2.4, 8.8), p=0.0001). Conclusions: MP detection sensitivity decreased and delay to MP recognition increased with prolonged iEEGs sampling interval. Survivorship following development of malignant EEG patterns remains low.

Cefepime neurotoxicity in the intensive care unit: a cause of severe, underappreciated encephalopathy

IntroductionCefepime, a broad spectrum antibiotic, is commonly prescribed in intensive care units (ICU) and may be an overlooked cause of neurologic symptoms such as encephalopathy, myoclonus, seizures, and coma. We aimed to characterize cefepime neurotoxicity in the ICU.MethodsWe performed a retrospective study of adult ICU patients treated with intravenous cefepime for at least 3 days between January 1, 2009 and December 31, 2011. The primary outcome was the development of cefepime neurotoxicity, with the likelihood of causality ascribed via a modified Delphi method.ResultsThis study included 100 patients. The mean age was 65.8 years (± 12.7 years). The median daily average dose of cefepime was 2.5 (IQR 2.0 to 3.5) grams. The median treatment duration was 6 (IQR 4 to 10) days. Renal failure in any form was present in 84 patients. Chronic kidney disease affected 40 patients, and 77 had acute kidney injury. Cefepime neurotoxicity occurred in 15 patients. Of these, seven were considered definite cases, three probable, and five possible. Neurotoxic symptoms included impaired consciousness (n = 13), myoclonus (n = 11), disorientation (n = 6), and nonconvulsive status epilepticus (n = 1). The dose of cefepime was appropriately adjusted for renal clearance in 64 patients (75.3%) without cefepime neurotoxicity and four patients (28.6%) with neurotoxicity (P = 0.001). Chronic kidney disease was present in 30 patients (35.3%) without neurotoxicity and in 10 (66.7%) of those with neurotoxicity (P = 0.04).ConclusionsCritically ill patients with chronic kidney disease are particularly susceptible to cefepime neurotoxicity. Myoclonus and impaired consciousness are the predominant clinical manifestations. Neurotoxic symptoms occur more often when the cefepime dose is not adjusted for renal function, but can still occur despite those modifications.

SODIUM OXYBATE AS A TREATMENT FOR DBS–RESISTANT, ALCOHOL–RESPONSIVE POST–ANOXIC MYOCLONUS

Myoclonic jerks are sudden shock–like involuntary movements, either manifesting as a brief contraction of a group of muscles (positive) or cessation of muscle activity (negative), driven by aberrant activity in...

Opercular myoclonic‐anarthric status epilepticus due to glutamic acid decarboxylase antibody‐associated encephalitis

We report a patient who was diagnosed with opercular myoclonic-anarthric status epilepticus and found to have glutamic acid decarboxylase antibody (GADA)-associated encephalitis, a previously unrecognised aetiology of this condition. The patient was a 23-year-old female admitted for investigation of focal myoclonic status epilepticus in the right side of the face and glossopharyngeal area. Intravenous corticosteroid was administered and improvement was observed in seizure activity and overall general health. A video sequence of opercular myoclonia is included. Due to the presence of inflammatory elements based on brain MRI and CSF studies, a decision to investigate autoimmune encephalitis was undertaken. Anti-GAD65 radioimmunoassay was markedly positive. This case study highlights the need for awareness of the clinical presentation of GADA-associated encephalitis. [Published with video sequences].

Clinical examination for prognostication in comatose cardiac arrest patients

ObjectiveTo build new algorithms for prognostication of comatose cardiac arrest patients using clinical examination, and investigate whether therapeutic hypothermia influences the value of the clinical examination. MethodsFrom 2000 to 2007, 500 consecutive patients in non-traumatic coma were prospectively enrolled, 200 of whom were post-cardiac arrest. Outcome was determined by modified Rankin Scale (mRS) score at 6 months, with mRS≤3 indicating good outcome. The clinical examination was performed on days 0, 1, 3 and 7 post-arrest, and clinical variables analyzed for importance in prognostication of outcome. A classification and regression tree analysis (CART) was used to develop a predictive algorithm. ResultsGood outcome was achieved in 9.9% of patients. In CART analysis, motor response was often chosen as a root node, and spontaneous eye movements, pupillary reflexes, eye opening and corneal reflexes were often chosen as splitting nodes. Over 8% of patients with absent or extensor motor response on day 3 achieved a good outcome, as did 2 patients with myoclonic status epilepticus. The odds of achieving a good outcome were lower in patients who suffered asystole (OR 0.187, 95% CI: 0.039–0.875, p=0.033) compared with ventricular fibrillation or non-perfusing ventricular tachycardia, but some still achieved good outcome. The absence of pupillary and corneal reflexes on day 3 remained highly reliable for predicting poor outcome, regardless of therapeutic hypothermia utilization. ConclusionThe clinical examination remains central to prognostication in comatose cardiac arrest patients in the modern area. Future studies should incorporate the clinical examination along with modern technology for accurate prognostication.

Predicting Outcome after Cardiopulmonary Arrest in Therapeutic Hypothermia Patients: Clinical, Electrophysiological and Imaging Prognosticators

Predicting outcome in comatose survivors of cardiac arrest is based on data validated by guidelines that were established before the era of therapeutic hypothermia. We sought to evaluate the predictive value of clinical, electrophysiological and imaging data on patients submitted to therapeutic hypothermia. A retrospective analysis of consecutive patients receiving therapeutic hypothermia during years 2010 and 2011 was made. Neurological examination, somatosensory evoked potentials, auditory evoked potentials, electroencephalography and brain magnetic resonance imaging were obtained during the first 72 hours. Glasgow Outcome Scale at 6 months, dichotomized into bad outcome (grades 1 and 2) and good outcome (grades 3, 4 and 5), was defined as the primary outcome. A total of 26 patients were studied. Absent pupillary light reflex, absent corneal and oculocephalic reflexes, absent N20 responses on evoked potentials and myoclonic status epilepticus showed no false-positives in predicting bad outcome. A malignant electroencephalographic pattern was also associated with a bad outcome (p = 0.05), with no false-positives. Two patients with a good outcome showed motor responses no better than extension (false-positive rate of 25%, p = 0.008) within 72 hours, both of them requiring prolonged sedation. Imaging findings of brain ischemia did not correlate with outcome. Absent pupillary, corneal and oculocephalic reflexes, absent N20 responses and a malignant electroencephalographic pattern all remain accurate predictors of poor outcome in cardiac arrest patients submitted to therapeutic hypothermia. Prolonged sedation beyond the hypothermia period may confound prediction strength of motor responses.