Cause Of Encephalopathy Research Articles

Diagnostic Approach to the Patient with Altered Mental Status.

Acute encephalopathy is a common presenting symptom in the emergency room and complicates many hospital and intensive care unit admissions. The evaluation of patients with encephalopathy poses several challenges: limited history and examination due to the patient's mental status, broad differential diagnosis of systemic and neurologic etiologies, low yield of neurodiagnostic testing due to the high base rate of systemic causes, and the importance of identifying less common neurologic causes of encephalopathy that can be life-threatening if not identified and treated. This article discusses the differential diagnosis of acute encephalopathy, presents an approach to the history and examination in a patient with encephalopathy, reviews the literature on the yield of neurodiagnostic testing in this population, and provides a diagnostic framework for the evaluation of patients with altered mental status.

Genetic Comparison of Human Parainfluenza Virus Type 3 Detected in Respiratory Samples from Patients with Encephalopathy and Airway Inflammation in Aichi Prefecture, Japan.

Human parainfluenza virus type 3 (HPIV-3, human respirovirus 3) is the second most frequently detected virus in lower respiratory tract infections in children after human respiratory syncytial virus (HRSV). HPIV-3, similar to related respiratory viruses such as HRSV and influenza virus, may cause encephalopathy; however, the relevance of HPIV-3 as a pathogenic factor in encephalopathy is unknown. We attempted to detect HPIV-1, HPIV-2, HPIV-3, HPIV-4, HRSV, and human metapneumovirus (HMPV) in 136 patients with encephalitis/encephalopathy or suspected encephalitis/encephalopathy during a 6-year period from 2014 to 2019. HPIV-3 was detected in 6 patients, followed by HRSV in 3 patients. The HPIV-3 strains detected were closely related to those detected in a patient with respiratory disease during the same period. Although HPIV-3 is less widely recognized than HRSV as a triggering virus of encephalopathy, our results suggest that HPIV-3 is as important as HRSV. Surveillance of the causative viruses of encephalopathy, including HPIV-3, would help clarify the causes of encephalopathy in Japan, as the cause is currently reported in less than half of cases in Japan.

Treatment refractory acute necrotizing myelitis after COVID-19 vaccine injection: a case report

Introduction and importance: Post-vaccination myelitis is a rare and debilitating clinical situation. There are few reports of post-COVID-19 infection and vaccination neurological sequela. Case presentation: A 69-year-old lady was admitted to the emergency department due to weakness and hypoesthesia in her hands 1 week after the Sinopharm vaccine injection. MRI showed a cervicothoracic cord haemorrhagic lesion that deteriorated within 48 h. The clinical course was refractory to conservative treatments. She underwent an emergency cervical laminectomy as a salvage treatment. Intraoperative samples were in favour of acute necrotizing myelitis. Discussion: In the review of the literature, the inflammatory storm, vasculitis, and many unknown etiologies are deemed to be the possible causes of encephalopathy and myelitis after COVID-19 infection and vaccination. There are few cases of post-COVID-19 myelitis and hematomyelia, but this case was the first report of post-vaccination necrotizing myelitis. Conclusion: Post-vaccination necrotizing myelitis is a lethal medical situation requiring intensive and emergent neurosurgical vigilance. Early clinical diagnosis in the beginning and full neurosurgical-neurological treatment armamentarium options are cornerstones of treatment paradigms. Salvage treatment options such as extensive laminectomy may play a life-saving role in treatment refractory cases of acute necrotizing myelitis.

Psychiatric Manifestations Caused by Mycoplasma pneumoniae Encephalitis Mimicking Autoimmune Encephalitis

AbstractA significant etiological factor for upper respiratory tract infections and community-acquired pneumonia is Mycoplasma pneumoniae. The incidence of extrapulmonary neurological problems in infected patients has been shown to range from 0.1 to 7%, often manifesting within a timeframe of 2 to 14 days following the onset of respiratory symptoms. Acute disseminated encephalomyelitis, Guillain–Barré syndrome, and transverse myelitis are among the immune-mediated illnesses encompassed under the syndrome. A 3-year-old male child exhibited symptoms of acute encephalopathy and behavioral disruption subsequent to an infection caused by M. pneumoniae. He presented with irritability, sleep disturbance, slurred speech, increased appetite, episodes of unresponsiveness, moving in circles, staring, and laughing episodes lasting for up to 15 to 30 minutes over a week. He lost his previous toilet training. Abnormal jerks were noted while awake and asleep. Symptoms were preceded by exposure to vague febrile illness 3 weeks prior to presentation. The patient's brain magnetic resonance imaging was normal. Electroencephalography showed a slow background with no epileptiform discharges. Cerebrospinal fluid analysis and polymerase chain reaction for viruses were negative. The workup for autoimmune encephalitis was negative. Mycoplasma serology IgM was detected. Marked improvement was noted after methylprednisolone pulse therapy, intravenous immunoglobulin, valproic acid, and azithromycin. In conclusion, our report serves as a reminder that M. pneumoniae infection is a possible cause of encephalopathy and behavioral disturbance in children. Early recognition and promotion of immunomodulatory and antimicrobial treatment can prevent the affected child from experiencing different levels of long-lasting impairments in cognitive, physical, or visual abilities.

SAT160 Alcoholic Ketoacidosis : An Endocrine Emergency

Abstract Disclosure: L.E. Tiu: None. T.D. Crisostomo: None. Background: Alcoholic Ketoacidosis is a complex metabolic condition frequently confused with Diabetic Ketoacidosis. Currently, there remains an undefined consensus guideline in the diagnosis and management of this disorder that leads to unreported cases. Clinical Case: A 47-year-old male presented with 2-day history of poor dietary intake due to abdominal pain and vomiting after an alcoholic binge drinking. He is a chronic alcoholic beverage drinker (2 liters of beer per day for 10 years). He has family history of Type 2 Diabetes Mellitus but has no known co-morbidities. Upon admission, vital signs were normal, but he was drowsy and incoherent with signs of dehydration and epigastric tenderness. Initial capillary blood glucose was 52 mg/dL. D50W 50ml intravenously was given with repeat CBG of 222 mg/dL. Arterial blood gas (ABG) showed high anion gap metabolic acidosis (pH 7.0 and anion gap of 42) with undetectable bicarbonate and elevated lactate at 17mmol/L. Serum ketones were elevated at 6mmol/L but Hba1c was normal at 4.6 %. Thus, Alcoholic Ketoacidosis was highly considered. Intravenous hydration with saline solution was instituted then shifted to D10W 125mL/hour to allow insulin infusion therapy at initial rate of 2units/hour. Sodium bicarbonate 100 mEqs was given intravenously with recovery of sensorium thereafter. Oral Vitamin B supplement was started, and two doses of Thiamine 100 mg/IV were given. Other tests showed hyperkalemia (5.28meq/L), hypochloridemia (89.7meq/L), hyperuricemia (12.74mg/dL), and hyperphosphatemia (5.8mg/dL). Serial monitoring and correction of electrolytes were done. Moreover, management integrated simultaneous referrals to other subspecialties to rule out other causes of encephalopathy, to evaluate and manage Hypertriglyceridemic Pancreatitis and Alcoholic Liver Disease, and to facilitate nutritional, psychosocial support and rehabilitation. On 2nd hour, repeat ABG showed improvement of pH to 7.3 and HCO3 to 9.80mmol/L. Lactate decreased to 12.70mmol/L. On the 17th hour, ABG showed metabolic alkalosis with resolution of ketosis hence insulin was discontinued. CBG levels for the next 24-48 hours were within normal limits. Oral feeding was supplemented with parenteral nutrition due to decreased appetite. On 48th hour, he displayed combative behavior with visual hallucinations, but there was no consent for psychiatric referral. On 72nd hour, there was spontaneous marked improvement in his behavior. He was discharged clinically stable. Conclusion: Early recognition of Alcoholic Ketoacidosis is imperative. This is an unrecognized endocrine emergency that portends good outcome with timely diagnosis and management. Treatment includes 1. adequate fluid resuscitation 2. thiamine 3. judicious use of insulin and bicarbonate 4. correction of electrolytes 5. multidisciplinary team management. Presentation: Saturday, June 17, 2023

Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), known as Hashimoto's encephalopathy (HE), represents a heterogeneous group of neurological and neuropsychiatric symptoms associated with a presence of antithyroid antibodies in case of other causes of encephalopathy were excluded. Clinical symptoms most commonly includes acute onset of encephalopathy, behaviour changes and cognitive dysfunction, epileptic seizures as well as cerebellar and extrapyramidal symptoms. Corticoids provides rapid and sustained therapeutic benefit in most patients and only a few patients require other immunosuppressive therapy such as plasmapheresis, intravenous immunoglobulins, or others. We present the cases of two patients with acute onset of encephalopathy, status epilepticus based on SREAT, with rapid improvement after steroid treatment.

Value of brain injury-related indicators based on neural network in the diagnosis of neonatal hypoxic-ischemic encephalopathy.

Neonatal hypoxic ischemic encephalopathy is a common disease, which is caused by fetal hypoxia, asphyxia, and other reasons. It may cause sequelae of the nervous system and even death in children. Computer tomography examination can clarify the scope and location of the disease and provide the basis for clinical treatment and prognosis. Relevant personnel analyzed the symptoms of ischemic hypoxia and found that ischemia and hypoxia were the main causes of encephalopathy. Neonatal ischemia and hypoxia are easy to cause serious damage. At present, with the development of medicine, the function of the human brain is the most important issue in natural science. The law of neural activity and the role of molecular cells, organs, and systems have fundamental construction significance for the prevention and treatment of nerve and mental diseases. By analyzing the value of the diagnosis of neonatal hypoxic-ischemic encephalopathy in the analysis of experimental data, by setting the newborns in the controlled group and the trial group as experimental subjects, this paper analyzed the situation of newborns in terms of body temperature, body weight, and respiratory rate, and used Apgar score to score these standards. It was found that the score of the controlled group was 7 and above, and the score of the trial group was below 7. It was found that the Apgar scoring method was more simple. Then, the newborns were analyzed by cord blood gas analysis. It was found that most of the data in the control group were between 7.8 and 8.4, and the data in the trial group were between 5.8 and 7.1. The umbilical blood gas analysis score of the experimental group was lower than that of the control group. By comparing the satisfaction of cord blood gas analysis and the Apgar score, it was found that the satisfaction of cord blood gas analysis was 24.06% higher than that of the Apgar score.

Refractory Seizures with Encephalopathy May be Due to Hashimoto Encephalopathy

Background: Hashimoto's encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis, is a relatively uncommon neurological disorder, mostly in adults, with an extremely rare occurrence in children. Clinical Description: A 9-year-old girl presented with unprovoked seizures and encephalopathy. She had been diagnosed with hypothyroidism, at around 6 ½ years of age, though was noncompliant to therapy with levothyroxine. Management: The child was managed with intravenous (IV) antiepileptics and other supportive care. Seizures were refractory and with worsening sensorium, she required mechanical ventilation. The magnetic resonance imaging of the brain was unremarkable, as also the cerebrospinal analysis. Autoimmune encephalitis panel was negative. The electroencephalogram showed background activity of 10–11 Hz and few areas with generalized slow waves. As she was a known case of hypothyroidism without taking thyroid supplements for the past few months, thyroid profile was sent. Reports showed FT3-1.1 pg/dL, FT4-0.515 ng/dL, and thyroid-stimulating hormone (TSH) - 107.3 μIU/mL. Thyroid antibody profile revealed antithyroid peroxidase antibody >500 IU/mL and antithyroglobulin antibody >1000 IU/mL. Following this, an ultrasound of the neck was done which showed features of thyroiditis. Considering the possibility that the underlying thyroiditis could explain the obtunded state and refractory seizures, the patient was started on levothyroxine and IV methylprednisolone. The sensorium of the child improved dramatically, and she got extubated. There were no further seizures and her TSH level reduced gradually and eventually, she was discharged. Conclusions: In a child with altered sensorium and refractory seizures, Hashimoto encephalopathy is a diagnosis of exclusion and may be considered when most of the common causes of encephalopathy and seizures have been ruled out, especially in the setting of some clinical clues of hypothyroidism. The condition is often reversible with good response to steroid therapy.

Abstract #1377584: Brain on Fire: A Case of Hashimoto's Encephalitis in a 29-Year-Old Female

A 29-year-old female presents to the hospital for insomnia, hallucination, and bizarre behavior. The patient had been feeling well until approximately three days early when she became psychotic after fasting from food for three days. She began having auditory and visual hallucinations and then started having seizures. Initially, the patient was admitted to a hospital in another city where she was diagnosed with Autoimmune Encephalitis for which she received IVIg. Brain MRI showed questionable T2 changes around the fourth ventricle on the left during hospital admission. The patient was discharged home after her symptoms improved. However, she was brought to our hospital for psychiatric consultation as her condition deteriorated. Upon arrival at Larkin Community Hospital, the patient was hemodynamically stable, breathing room air, and continued to complain of having delusions and hallucinations. Initial Vital Signs: BP: 122/74 mmHg, HR: 70 bpm, RR: 18, T: 98°F. weight 56.2 kg, BMI 24.21. She was in nonacute distress, although anxious with pressured speech. She was cooperative and able to follow commands and track the examiner. Her NIH Stroke Scale/Score (NIHSS) was 0 and Glasgow Coma Scale (GCS) was 15. She denied using illicit drugs, smoking, and alcohol. Neurology decided to repeat LP CSF r/o NMDA Antibody encephalitis and started empirically a 5-day course of azithromycin to cover for streptococcal infections while awaiting further results. Thyroglobulin antibodies were ordered to rule out Steroids Responsive Encephalopathy and it came back elevated (91.2). She was started on Solu-Medrol 500mg IV daily for 3 days which was increased to 1000mg IV daily to complete 5 days. Even though, she developed tachycardia in the 120s; she started feeling much better as well as improved her symptoms. “Steroid-responsive encephalopathy associated with autoimmune thyroiditis” (SREAT) is an uncommon condition that is associated with Hashimoto thyroiditis. It is also called Hashimoto encephalopathy (HE). This condition was originally described in 1966 by Brain et al1. HE is a rare disorder with an approximate prevalence of 2/100,0002. It affects women more often than men. This diagnosis is typically made when a patient has encephalopathy associated with antithyroid (anti-thyroid peroxidase [TPO]) and/or anti-thyroglobulin (TG) antibodies, when other causes have been excluded. The symptoms typically seen include subacute onset of confusion with altered level of consciousness, seizures, and myoclonus. Based on the literature review of 251 cases of SREAT by Laurent et al3, SREAT has various presentations including coma to isolated psychiatric disorder. This disorder is thought to be immune mediated as opposed to being the result of an altered thyroid state on the nervous system. In most of the reported cases of SREAT, the patients are euthyroid during the presentation of symptoms. However, the exact mechanism of SREAT is still unknown. The diagnosis is made after excluding other toxic, infectious, and metabolic causes of encephalopathy with examination of cerebrospinal fluid and neuroimaging.

Methamphetamine-induced encephalopathy in the absence of hyperammonemia

BackgroundMethamphetamine is an addictive drug with various effects on the neurotransmitters in the central nervous system. Methamphetamine-induced encephalopathy in the absence of hyperammonemia presents a unique challenge in a clinical setting. Previously published cases of methamphetamine-induced encephalopathy suggested that methamphetamine-induced hepatotoxicity and subsequent hyperammonemia may be the cause of encephalopathy. However, the literature is limited on methamphetamine-induced encephalopathy without hyperammonemia.CaseThis case presents a disoriented patient with methamphetamine use disorder in acute toxicity, unable to ambulate independently, and poorly responsive to verbal stimuli. The patient was found to have normal ammonia levels.DiscussionThis patient’s presentation and laboratory findings, namely normal ammonia levels, suggest a different pathophysiological pathway for methamphetamine-induced encephalopathy. One potential pathway is through the direct action of methamphetamine on the central nervous system through acute disruption of neurotransmitter signaling and disruption of the blood-brain barrier.ConclusionFurther research should be conducted into the prevalence and pathophysiology of methamphetamine-induced encephalopathy in the absence of hyperammonemia.Key PointsMethamphetamine-induced encephalopathy (MIE) in the absence of hyperammonemia presents a unique challenge in a clinical setting.Previously published cases of MIE suggest that methamphetamine-induced hepatotoxicity and subsequent hyperammonemia may be the cause of encephalopathy.Further research should be conducted into the prevalence and pathophysiology of MIE in the absence of hyperammonemia.

Perinatal antecedents of moderate and severe neonatal hypoxic ischaemic encephalopathy: An Australian birth cohort study.

Neonatal hypoxic ischaemic encephalopathy (HIE) is the most common cause of encephalopathy in the neonatal period and carries a high risk of mortality and long-term morbidity. The aim of this study was to investigate key antecedents of moderate and severe HIE in a large contemporary birth cohort. A retrospective cohort study of births meeting criteria was conducted between 2016 and 2020 at the Mater Mothers' Hospital, Brisbane, Australia. This is a quaternary perinatal centre and Australia's largest maternity hospital. Univariate and multivariate Firth logistic regression were used to account for imbalanced frequency classes between non-HIE and HIE groups. Maternal variables and intrapartum factors were investigated for associations with neonatal moderate and severe HIE. Overall, 133 of 46 041 (0.29%) infants were diagnosed with HIE: 77 (0.17%) with mild HIE and 56 (0.12%) with moderate/severe HIE. Nulliparity, type 1 diabetes mellitus and maternal intensive care unit admission were associated with increased odds of moderate/severe HIE. Intrapartum risk factors included emergency caesarean birth, emergency caesarean for non-reassuring fetal status or failure to process, intrapartum haemorrhage and an intrapartum sentinel event (shoulder dystocia, cord prolapse, uterine rupture and placental abruption). Neonatal risk factors included male sex, late preterm gestation (35+0 -36+6 weeks), Apgar score less than four at 5min, severe respiratory distress requiring ventilatory support and severe acidosis at birth. This cohort study identified a series of potentially modifiable maternal and obstetric risk factors for HIE. Risk factors for HIE do not appear to have changed significantly with evolution in modern obstetric care.

Hashimoto's Encephalopathy: Case Series and Literature Review.

To describe the clinical manifestations of Hashimoto's encephalopathy (HE) and discuss its pathogenesis in light of recent research. The pathogenesis of HE is uncertain. Available evidences point towards an autoimmune etiology due to vasculitis or other inflammatory process. Detection of thyroid antibodies - antithyroid peroxidase and anti-thyroglobulin are essential for diagnosis. Autoimmune encephalitis including Anti-IgLON5 disease needs to be excluded in suspected cases with appropriate tests for neuronal surface antibodies. Detection of thyroid autoantibodies is nonspecific, as these can be detected in some normal individuals and in other autoimmune diseases. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels in younger males with very high levels of thyroid antibodies. The role of the thyroid autoantibodies in the central nervous system (CNS) tissue damage remains unclear and these can act only as markers for diagnosis. Conversely, they have a role to play in determining the thyroid pathology - more glandular fibrosis associated with thyro-peroxidase antibody than with the thyroglobulin antibody. HE is a syndrome characterized by altered mental status, confusion, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually responsive to glucocorticoid therapy. Diagnosis requires the exclusion of other causes of encephalopathies and encephalitis including autoimmune encephalitis associated with neuronal surface antibodies and paraneoplastic ones. Diagnosis also is dependent on the demonstration of thyroid autoantibodies in serum. Since there is no direct pathophysiologic link between antithyroid antibodies, Hashimoto thyroiditis and the cerebral syndrome, the nomenclature HE could be misleading. The response to steroids led to a renaming of the syndrome to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), though some cases do not respond to steroids. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels (IgG4-related disease). This is characterized by a higher incidence in men (5:1) than in women, onset at a younger age, more intense thyroid inflammation and higher antithyroid antibody titters. Such patients have excessive production of IgG4 + plasmacytes, which infiltrate various organs leading to their fibrosis and sclerosis, sometimes resulting in inflammatory tumors. HE is treated with corticosteroids along with treatment of the dysthyroid condition, if any. There are yet no guidelines regarding steroid dose and/or duration.

Viral, Bacterial, Metabolic, and Autoimmune Causes of Severe Acute Encephalopathy in Sub-Saharan Africa: A Multicenter Cohort Study

To assess whether viral, bacterial, metabolic, and autoimmune diseases are missed by conventional diagnostics among children with severe acute encephalopathy in sub-Saharan Africa. One hundred thirty-four children (6 months to 18years) presenting with nontraumatic coma or convulsive status epilepticus to 1 of 4 medical referral centers in Uganda, Malawi, and Rwanda were enrolled between 2015 and 2016. Locally available diagnostic tests could be supplemented in 117 patients by viral, bacterial, and 16s quantitative polymerase chain reaction testing, metagenomics, untargeted metabolomics, and autoimmune immunohistochemistry screening. Fourteen (12%) cases of viral encephalopathies, 8 (7%) cases of bacterial central nervous system (CNS) infections, and 4 (4%) cases of inherited metabolic disorders (IMDs) were newly identified by additional diagnostic testing as the most likely cause of encephalopathy. No confirmed cases of autoimmune encephalitis were found. Patients for whom additional diagnostic testing aided causal evaluation (aOR 3.59, 90% CI 1.57-8.36), patients with a viral CNS infection (aOR 7.91, 90% CI 2.49-30.07), and patients with an IMD (aOR 9.10, 90% CI 1.37-110.45) were at increased risk for poor outcome of disease. Viral and bacterial CNS infections and IMDs are prevalent causes of severe acute encephalopathy in children in Uganda, Malawi, and Rwanda that are missed by conventional diagnostics and are associated with poor outcome of disease. Improved diagnostic capacity may increase diagnostic yield and might improve outcome of disease.

Status Epilepticus in Older Adults: Diagnostic and Treatment Considerations.

Status epilepticus (SE) is one of the leading life-threatening neurological emergencies in the elderly population, with significant morbidity and mortality. SE presents unique diagnostic and therapeutic challenges in the older population given overlap with other causes of encephalopathy, complicating diagnosis, and the common occurrence of multiple comorbid diseases complicates treatment. First-line therapy involves the use of rescue benzodiazepine in the form of intravenous lorazepam or diazepam, intramuscular or intranasal midazolam and rectal diazepam. Second-line therapies include parenteral levetiracetam, fosphenytoin, valproate and lacosamide, and underlying comorbidities guide the choice of appropriate medication, while third-line therapies may be influenced by the patient's code status as well as the cause and type of SE. The standard of care for convulsive SE is treatment with an intravenous anesthetic, including midazolam, propofol, ketamine and pentobarbital. There is currently limited evidence guiding appropriate therapy in patients failing third-line therapies. Adjunctive strategies may include immunomodulatory treatments, non-pharmacological strategies such as ketogenic diet, neuromodulation therapies and surgery in select cases. Surrogate decision makers should be updated early and often in refractory episodes of SE and informed of the high morbidity and mortality associated with the disease as well as the high probability of subsequent epilepsy among survivors.

Recurrent encephalopathy associated with pegylated beta-interferon treatment

A 62-year-old woman with a history of multiple sclerosis (MS) presented with recurrent episodes of confusion, dysarthria and gait difficulties. These episodes occurred about 3 days after administration of pegylated interferon-beta-1a (Plegridy®) and resolved spontaneously in around 4 days. The brain MRI scan, laboratory findings and cerebrospinal fluid analysis during these episodes were negative for other causes of encephalopathy. She discontinued treatment with interferon and was started on teriflunomide, experiencing no recurrence of symptoms. We believe that interferon was responsible for this patient's recurrent encephalopathic syndrome, possibly due to its effects on inflammatory cytokines and endothelial dysfunction.

Tuberous Sclerosis Complex Presenting as Afebrile Encephalopathy

Background: Tuberous sclerosis complex (TSC) is a multisystem genetic disorder with a range of physical manifestations that require regular evaluation, surveillance, and management. Central nervous system manifestations are the major cause of morbidity and mortality in TSC patients. Encephalopathy, which may be due to multiple etiologies, maybe seen sometimes in these patients. Clinical Description: We are sharing a series of three cases, presenting to us within a 6-month period with encephalopathy, who were admitted and treated by us. Etiologies were identified in all three cases with variable spectrum from nonconvulsive status epilepticus to autistic regression to valproate-associated hyperammonemia. Management: All three patients admitted with us were treated as per clinical guidelines for respective etiologies. All patients respond well to treatment and were discharged and are under regular follow-up. Conclusion: There are various etiologies for encephalopathy in a child with TSC. Therefore, thorough history, examination, and investigations should be carried out in every child with TSC to find out the likely cause of encephalopathy, and treatment should be initiated according to the underlying cause.

Clinical Spectrum of Neonatal Encephalopathy and the Role of CK-MB Assay in Transient Myocardial Ischaemia in Neonates with Hypoxic Ischaemic Encephalopathy: A Prospective Cohort Study

Introduction: Significant advancements have been made in the field of perinatal medicine through the use of newer monitoring technologies. However, birth asphyxia continues to cause prolonged hospitalisation due to multiple organ dysfunction and unfortunately, sometimes results in death. An elevation of the serum Creatine Kinase Myocardial Bound (CK-MB) fraction in myocardial ischaemia could indicate exposure to asphyxia and shock in neonates. Aim: The aim of this study was to examine the clinical spectrum of Neonatal Encephalopathy (NE) and investigate Transient Myocardial Ischaemia (TMI) in neonates with Hypoxic Ischaemic Encephalopathy (HIE) using CK-MB assay at birth, 24 hours, and 72 hours. Materials and Methods: A prospective clinical study was conducted in the Neonatal Intensive Care Unit (NICU) at a Tertiary Care Hospital in Tamil Nadu, India. The study duration was eight months, from January 2014 to August 2014. A total of 70 term neonates admitted with NE symptoms were included. Among them, 65 babies with evidence of asphyxia, such as an Appearance, Pulse, Grimace,Activity and respiration (APGAR) score less than 3 at five minutes, heart rate less than 60 Beats Per Minute (bpm), meconium-stained amniotic fluid, or the need for positive pressure ventilation for more than one minute, were evaluated for TMI. Myocardial involvement was assessed through clinical evaluation, chest X-ray (CXR), Electrocardiogram (ECG), Echocardiography (ECHO), and CK-MB assay at birth, 24 hours, and 72 hours. Statistical analysis was performed using Epi Info software version 7.0. Results: The mean Gestational Age (GA) of the study participants was 38.97±1.31 weeks. During the study period, 70 babies were admitted with NE symptoms, and HIE was the cause of encephalopathy in 65 babies. Other causes included hypoglycaemia, intraventricular haemorrhage, and bilirubin encephalopathy. Meconium staining of amniotic fluid was the most common intrapartum event in HIE babies. The survival rate in encephalopathy cases was 28.57%. Shock and respiratory failure were common complications in HIE, representing 46.15% and 44.61% respectively. Cardiomegaly in CXR, ST depression in ECG, Tricuspid Regurgitation (TR) in ECHO, and elevated CK-MB were commonly detected in babies with TMI associated with HIE. Conclusion: NE caused by HIE has a high mortality and morbidity rate. 83.3% of asphyxiated babies with shock had elevated CK-MB levels at birth, but the CK-MB values at 24 hours and 72 hours were not significantly elevated.

A case report of Wernicke Korsakoff syndrome in a patient with cholangiocellular carcinoma: An underestimated cause of encephalopathy in cancer patients

Wernicke's encephalopathy, a disorder caused by thiamin deficiency, is characterized by a classical triad of encephalopathy, ataxia, and ophthalmoplegia. Although alcoholism is the most common predisposing factor, it can also be associated with nonalcoholic states (hyperemesis gravidarum, intestinal obstruction, bariatric surgery, and others). This work presents a case of nonalcoholic Wernicke-Korsakoff syndrome diagnosed in a cholangiocellular carcinoma patient and literature review. A 65-year-old male patient with a history of cholangiocellular carcinoma (Klatskin tumor) was treated with radiotherapy at the operation site after Roux-en-Y hepaticojejunostomy. During follow-up, the patient developed gastric outlet obstruction and was diagnosed with peritoneal carcinomatosis after a palliative gastrojejunostomy. As the patient could not tolerate oral nutrition during hospitalization, total parenteral nutrition was administered. After 10 days of admission, the patient showed decreased response to verbal stimuli as well as bilateral horizontal nystagmus, lethargy, and disorientation. Furthermore, the patient displayed confabulation. Clinical and imaging findings were consistent with Wernicke's encephalopathy. Therefore, treatment with intravenous thiamin replacement was initiated. The patient's encephalopathy regressed on the second day after treatment, and he recovered the place-person-time orientation. In the following month, the abnormal imaging findings were almost entirely resolved. In order to prevent irreversible brain damage induced by chronic thiamin deficiency, thiamin replacement therapy with parenteral nutrition solutions should be included as a treatment for hospitalized cancer patients unable to receive enteral nutrition for a long time.

Transient disruption of functional connectivity and depression of neural fluctuations in a mouse model of acute septic encephalopathy.

Septic encephalopathy leads to major and costly burdens for a large percentage of admitted hospital patients. Elderly patients are at an increased risk, especially those with dementia. Current treatments are aimed at sedation to combat mental status changes and are not aimed at the underlying cause of encephalopathy. Indeed, the underlying pathology linking together peripheral infection and altered neural function has not been established, largely because good, acutely accessible readouts of encephalopathy in animal models do not exist. Behavioral testing in animals lasts multiple days, outlasting the time frame of acute encephalopathy. Here, we propose optical fluorescent imaging of neural functional connectivity (FC) as a readout of encephalopathy in a mouse model of acute sepsis. Imaging and basic behavioral assessment were performed at baseline, Hr8, Hr24, and Hr72 following injection of either lipopolysaccharide or phosphate buffered saline. Neural FC strength decreased at Hr8 and returned to baseline by Hr72 in motor, somatosensory, parietal, and visual cortical regions. Additionally, neural fluctuations transiently declined at Hr8 and returned to baseline by Hr72. Both FC strength and fluctuation tone correlated with neuroscore indicating this imaging methodology is a sensitive and acute readout of encephalopathy.

Identifying encephalopathies from acute metabolic derangements.

Metabolic derangements, when acute and severe, affect brain function. This presents mostly with a marked decline in the level of consciousness, resulting in impaired responsiveness, abnormal receptivity, impaired content, and loss of memory retention. The term metabolic encephalopathy has been used but is conjecture that can be challenged in the age of modern neuroimaging. We now recognize that many metabolic encephalopathies may involve structural lesions and at an early stage. Common clinical conundrums are the evaluation of the degree of brain injury and its recoverability. This review discusses the appropriate terminology for these conditions, the diagnostic approach, therapy recommendations, and prediction of recovery potential. In evaluating a presumed metabolic cause for encephalopathy, we must (1) search for and rule out structural injury to the brain despite an obvious explanatory metabolic derangement, (2) recognize that several confounding conditions often co-exist, and (3) acknowledge that resolution of brain dysfunction may be protracted despite normalization of laboratory values.