Convulsions Research Articles

Status Epilepticus in an Internal Medicine Ward: Different Patients Therefore Distinct Approaches.

Background Status epilepticus (SE) is a medical condition that bestows substantial morbidity and mortality. Literature is scarce regarding SE in elderly patients, particularly in the context of internal medicine wards. Aim To characterize SE patients admitted to an internal medicine ward, identify potential outcome predictors and differences between young and elderly, as well asconvulsive (CSE) and non-convulsive SE (NCSE) patients. Methods We enrolled 135 consecutive patients in an observational, retrospective cohort study. We established elderly patients as more than 64 years old and defined worse prognosis as a modified Rankin Scale (mRS)>4. Results The SE population was 73% elderly, and 75% presented with NCSE, mainly metabolic, idiopathic, or vascular SE. The intra-hospital mortality was 51%, and 62% had an mRS>4 at discharge. NCSE and electroencephalogram (EEG) with paroxysmal activity at discharge were predictive of a worse prognosis. Elderly patients had increased disability at admission, most had NCSE (81%), and the SE etiology differed with more idiopathic and vascular causes. In the elderly, mortality was increased, as was the number of patients with mRS>4 at discharge. NCSE patients had the more neurodegenerative disease (30%) and presented predominantly with vascular and anoxic causes. Morbidity and mortality were also increased in the NCSE group. There was no difference in the antiepileptic drugs used or in the percentage of patients achieving an EEG with no paroxysmal activity between the subpopulations. Conclusion SE in elderly patients should be addressed distinctly. Current approaches based on the strategies used for standard CSE have shown little or no efficacy overall.

Структура судомного синдрому в пацієнтів із черепно-мозковою травмою в гострому періоді

Актуальність. Вивчення структури епілептичних нападів, часу їх появи, зв’язку з характером черепно­мозкової травми дозволяє виявити групу потерпілих, які потребуватимуть невідкладної хірургічної корекції їх стану, профілактичного призначення протиепілептичних препаратів, а також уточнити вплив епілептичних нападів на наслідки травми. Мета: вивчити структуру епілептичних нападів у гострому періоді черепно­мозкової травми для оптимізації діагностичних і лікувальних заходів у відповідної категорії пацієнтів. Матеріали та методи. Проведено клінічне, інструментальне обстеження й проаналізовано результати лікування 779 хворих із черепно­мозковою травмою, у яких при надходженні та/або перебуванні в стаціонарі мали місце епілептичні напади. Середній вік пацієнтів становив 35,5 ± 7,8 року. Більшість спостережень — кримінальна травма — 320 хворих (41,1 %), у 272 осіб травма була наслідком дорожньо­транспортної пригоди (34,9 %), 109 хворих отримали травму при падінні з висоти власного росту (13,9 %), у 78 хворих обставини травми були невідомі (10,0 %). Результати. Епілептичні напади виникають у 8,4 % хворих у гострому періоді черепно­мозкової травми. У 81,4 % мали місце негайні епілептичні напади, у 15,7 % — ранні епілептичні напади, у 2,9 % — пізні епілептичні напади. Для негайних і ранніх епілептичних нападів характерними були парціальні епілептичні судоми, у структурі пізніх епілептичних нападів у гострому періоді черепно­мозкової травми переважали генералізовані епілептичні напади. 422 хворих (54,2 %) прооперовано в ургентному порядку. Хірургічне лікування після попередньої консервативної терапії виконано у 232 хворих (29,8 %). Консервативна терапія проведена в 125 пацієнтів (16,0 %). Висновки. Неврологічне обстеження хворих з епілептичними нападами в гострому періоді черепно­мозкової травми обов’язково повинне доповнюватися проведенням комп’ютерної томографії головного мозку, незалежно від рівня порушення свідомості пацієнта. Екстрене хірургічне втручання в таких хворих, спрямоване на усунення прогресуючого дислокаційного синдрому, часто є основним методом лікування. Терапія протиепілептичними препаратами повинна проводитись після появи одиничного епілептичного нападу протягом короткого періоду часу, за наявності неспровокованих епілептичних нападів і їх повторюваності прийом протиепілептичних препаратів повинен бути тривалим.

Second-generation androgen receptor inhibitors in therapy of non-metastatic castration resistant prostate cancer: role of safety profile and effect on quality of life

The article discusses the role of second-generation androgen receptor inhibitors (ARi) in therapy of non-metastatic castration resistant prostate cancer (nmCRPC). The phase 3 trials PROSPER (enzalutamide), SPARTAN (apalutamide) and ARAMIS (darolutamide) showed that these medicines if used in combination with androgen deprivation therapy (ADT) make possible to significantly improve, as compared with placebo, metastasis-free survival and overall survival of patients with nmCRPC. An important role in choosing a certain medicine of the group is played by its safety profile, including its drug-drug interaction potential and effect on patient’s quality of life. In this respect, darolutamide has a number of advantages over apalutamide or enzlutamide. The frequency of CNS (cognitive, psychiatric, convulsive) and other adverse drug reactions that significantly worsen the patients’ quality of life (falls, fractures, hypertension, etc.) in therapy with darolutamide does not differ from that with placebo. Moreover, this medicine retains its antagonistic effects to all clinically significant androgen receptor mutations that develop resistance to enzalutamide and apalutamide.

Sudden convulsion with multiple pulmonary nodules in a girl aged 15 years

A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.

Sudden unexpected postnatal collapse in a neonate

A healthy full-term female neonate, aged 3 days and born by vaginal delivery (with a 1-minute Apgar score of 10 and a 5-minute Apgar score of 10), had unexpected cardiac and respiratory arrests in the early morning on day 3 after birth and recovered to spontaneous breathing and heartbeat after a 10-minute resuscitation. The child had poor response and convulsion after resuscitation. Blood gas analysis showed metabolic acidosis, and amplitude-integrated EEG showed a burst-suppression pattern. She was diagnosed with sudden unexpected postnatal collapse but improved after hypothermia and symptomatic/supportive treatment. This article reports the first case of sudden unexpected postnatal collapse in China and summarizes related risk factors, pathophysiological mechanisms, and preventive and treatment measures of this disorder.

Predictors of Intensive Care Unit Admission among Hospitalized COVID-19 Patients in a Large University Hospital in Tehran, Iran.

Background: The rapid increase in the spread of COVID-19 and the numbers of infected patients worldwide has highlighted the need for intensive care unit (ICU) beds and more advanced therapy. This need is more urgent in resource-constrained settings. The present study aimed to identify the predictors of ICU admission among hospitalized COVID-19 patients. Study design: The current study was conducted based on a retrospective cohort design. Methods: The participants included 665 definite cases of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) hospitalized in Imam Hossein Hospital from February 20 to May 14, 2020. The baseline characteristics of patients were assessed, and multivariate logistic regression analysis was utilized to determine the significant odds ratio (OR) for ICU admission. Results: Participants were aged 59.52±16.72 years, and the majority (55.6%) of them were male. Compared to non-ICU patients (n=547), the ICU patients (n=118) were older, had more baseline comorbidities, and presented more often with dyspnea, convulsion, loss of consciousness, tachycardia, tachypnea, and hypoxia, and less often with myalgia. Significant OR (95% CI) of ICU admission was observed for the 60-80 age group (2.42, 95%CI: 1.01; 5.79), ≥80 age group (3.73, 95%CI: 1.44; 9.42), ≥3 comorbidities (2.07, 95%CI: 1.31; 3.80), loss of consciousness (6.70, 95%CI: 2.94, 15.24), tachypnea (1.79, 95%CI: 1.03, 3.11), and SpO2<90 (5.83, 95%CI: 2.74; 12.4). Abnormal laboratory results were more common among ICU-admitted patients; in this regard, leukocytosis (4.45, 95%CI: 1.49, 13.31), lymphopenia (2.39, 95%CI: 1.30; 4.39), elevated creatine phosphokinase (CPK) (1.99, 95%CI: 1.04; 3.83), and increased aspartate aminotransferase (AST) (2.25, 95%CI: 1.18-4.30) had a significant OR of ICU admission. Chest computer tomography (CT) revealed that consolidation (1.82, 95%CI: 1.02, 3.24), pleural effusion (3.19, 95%CI: 1.71, 5.95), and crazy paving pattern (8.36, 95%CI: 1.92, 36.48) had a significant OR of ICU admission. Conclusion: As evidenced by the obtained results, the predictors of ICU admission were identified among epidemiological characteristics, presenting symptoms and signs, laboratory tests, and chest CT findings.

Pre-hospital management of childhood convulsions in Ilesa, South-west, Nigeria

Convulsion evokes a lot of fear in mothers and caregivers whenever it occurs. They even think that their child is dying. Many interventions are often carried out because of anxiety and limited knowledge. The aim of this study is to examine the interventions instituted by caregivers for children with convulsions before presentation in the hospital for treatment. We prospectively studied all the 276 children that presented to the emergency room with convulsion over a period of seven months and the care administered at home before presenting in the hospital was noted. One hundred and nine (39.5%) of the children had water poured on them during convulsion. Substances administered orally were; salt 32 (11.6%), onions 14 (5.1%), palm oil 3 (1.1%) and herbal concoctions 13 (4.7%), of which two contained cow urine. Metal spoons were used as mouth gag in 41 (14.9%) of subjects while 29 (10.5%) children had intramuscular (IM) injections given by health workers at home (eight) in various health centres (15) and hospitals (6). Other interventions were scarifications and drinking of their mothers’ urine. While 40 (14.5%) children had more than one treatment given to them before their arrival, 99 (35.9%) had no treatment given before their arrival. Caregivers continue to carry out various interventions at home for convulsions in children. Majority of the interventions are not beneficial while some are harmful. There is, therefore, need to continue to educate caregivers on the appropriate prehospital interventions of convulsions in children before presenting in the hospital. Key words: Convulsion, pre-hospital interventions.

Assessing the effect of water deprivation on the efficacy of on-farm euthanasia methods for broiler chickens

ABSTRACT 1. Moribund or diseased poultry requiring euthanasia are often dehydrated. To understand how dehydration influences the efficacy of various killing methods, this experiment investigated the effect of water deprivation (WD) on times to unconsciousness and death. 2. Broiler chickens (n = 179) were water-deprived for 0, 24, 48 or 72 hours to mimic dehydration, then killed via manual cervical dislocation, mechanical cervical dislocation (Koechner Euthanising Device (KED)), or non-penetrating captive bolt (Zephyr-EXL), at 8, 22, 36 or 50 d of age. Degree of WD was confirmed by skin turgor, packed cell volume and body weight loss. Method efficacy was evaluated by the time to unconsciousness and death using pupillary light (PUP), palpebral blink (PAL) and nictitating membrane (NIC) reflexes, feather erection (FE), cloacal winking (CW) and convulsions (CN). The extent of damage caused by each method was examined via radiography, gross pathology and histopathology. The main effects of WD time and euthanasia method were analysed by two-way analyses of variance (CRD, PROC MIXED, SAS 9.4) with a-priori contrasts to compare water-deprived versus non-water-deprived (NON) birds. 3. Skin turgor, packed cell volume and body weight loss had a quadratic relationship with WD, with highest values for those birds which were water-deprived for 72 h. WD level did not affect time to unconsciousness. Time to death was longer for WD birds than NON, with longer latencies to FE, CW and CN for water-deprived birds. WD only affected radiography or gross pathology scores on d 8, with the extent of subcutaneous haemorrhage within the neck decreasing as WD increased. 4. The shortest latency to PUP loss, at all ages, and to PAL and NIC loss, at 22 d, was with the Zephyr-EXL. KED had the longest time to unconsciousness (PUP, PAL and NIC), at all ages, and to death, at 36 and 50 d. 5. Overall, WD increased time to death, but did not affect the onset of unconsciousness, with no interaction between methods and WD level.

Therapeutic management of hypovitaminosis A and zinc deficiency in a lactating cow from a small dairy herd in muranga county, Kenya

Background: Hypovitaminosis A and zinc deficiency were diagnosed in a 7-year-old Friesian cow from a small milking herd comprising of thirty Friesian cows in a zero grazing unit in Muranga County in Kenya, on Friday, November 15, 2019. The cow was weighing approximately 500 kilograms and had calved four months ago. The daily production of milk was tweny five litres. The cow had a history of sudden inappetance, reduced weight, sternal recumbency, convulsions and heavy lacrymation. Clinical examination revealed slight clouding of the cornea, and dilated pupils which did not respond to light. The menace response was almost absent but palperal and corneal reflexes were present. The cow was not apparently blind. Methods: The clinical examination of the skin revealed a fungal infection. Skin scrapings and hair from the lesions were examined for fungal species by direct microscopy in 10% KOH and lactophenol. Collected samples were inoculated on mycobiotic agar. The inoculum was incubated at 28°C for two to six weeks and examined for colony formation. Culture examination revealed Trichophyton verrucosum as the cause of dermatophytosis. Blood sample was taken from the cow into a vial containing heparin for spectrophotometric estimation of vitamin A and serum zinc concentrations. Results: Vitamin A and serum zinc concentrations were 5.12 μg/dl and 3.24 μg/L, respectively. The reference serum values for vitamin A are in the range of 15.4 to 32.3 μg/dl, while reference serum zinc levels are in the range of 6-12 μg/L for optimum physiologic functioning in dairy cattle. On the basis of history, clinical examination and significantly low serum vitamin A and zinc levels, the lactating friesian cow was diagnosed to be suffering from combined hypovitaminosis A and zinc deficiency. The dairy cow was therapeutically managed through administration of zinc sulphate orally, at a dosage rate of 1 gram per week for six weeks, administration of vitamin A at a dosage rate of 30,000 international units (IU), deep intramuscularly, once daily for seven days, and intramuscular administration of 3 mls of BelamylR, once daily for seven days. The body parts with lesions caused by T. verrucosum were treated through topical administration of zinc oxide ointment once daily for twenty one days. Following treatment of the lactating cow, significant improvement was observed in terms of disappearance of lachrymation, corneal clouding, sternal recumbency, skin lesions and convulsions. The appetite also returned to normal. All the other cows in the farm were supplemented with vitamin A and zinc. The client was also advised to include fresh napier and bermuda grass in the cattle diet, since they have been shown to contain adequate levels of beta carotene and zinc. Conclusion: In conclusion, zinc and vitamin A supplementation may be of benefit for recovery of cows from sternal recumbency and dermatophytosis.

Nitrate-nitrite toxicosis associated with duckweed (Portulaca Oleracea L.) (Portulacaceae) consumption in a herd of sheep in Kenya

Background: Nitrate-nitrite toxicosis associated with the consumption of duckweed (Portulaca oleracea L.) (Portulacaceae) was diagnosed in a small herd comprising of 11 sheep in Korayo Sub Location, Rangwe Sub County of Homabay County in Kenya. Methods: The clinical signs were dyspnea, froth from the mouth, labored breathing, incoordination, tachycardia, increased urination, neck, fore and hind limb distension, aggressive movements, bloat, convulsions, and coma, before death. Results: All the 11 sheep died of intoxication before institution of therapy. Brownish mucous membranes and chocolate colored and poorly clotted blood were the significant necropsy observations. Mild pulmonary edema was characterized by the presence of limited froth in the trachea and bronchioles. At postmortem, the liver of the sheep was grossly congested, and the cardiac pericardium was inflamed. There was gastritis, enlarged, and congested kidneys with pin-point hemorrhages. Diphenylamine tests carried out on the fed duckweed and on ruminal duckweed contents were positive for nitrates. Duckweed (Portulaca oleracea) fed on sheep had 7.4% nitrate on dry matter basis (DM), whereas ruminal contents had 5.38% nitrate on DM basis. Chocolate-colored blood collected during postmortem had a methemoglobin fraction of 86%. Nitrate levels in pastures of 0.5% and above have been shown to be potentially dangerous, with acute poisoning likely to occur at times when the levels are above 1%. Death in sheep can occur when blood methemoglobin levels are in the range of 67%–90%. Conclusion: The observations from the present study are indicative of death in a herd of sheep due to nitrate-nitrite poisoning associated with the consumption of duckweed (Portulaca oleracea).

Comparison of ketofol (ketamine and propofol) and etomidate in electro convulsive therapy: A double-blinded randomized controlled trial

Background and Aims: Electroconvulsive therapy (ECT) is the most effective treatment modality for severe and medication-resistant psychiatric disorders. ECT provokes generalized tonic-clonic seizures. Induction of optimal seizures (duration of motor seizures >15 s) is considered as an important goal during the ECT procedure. We compared ketofol and etomidate as induction agents in ECT with respect to the seizure duration and seizure threshold as there is less information available in the literature. Methods: One hundred and twenty patients posted for ECT were randomized into two groups. Group A received ketofol 1:1 (ketamine 0.5 mg/kg + propofol 0.5 mg/kg), while as Group B received etomidate 0.2 mg/kg. After administration of the muscle relaxant (succinylcholine 0.5 mg/kg) ECT was delivered. The primary outcome was seizure duration, and secondary outcomes such as seizure threshold, hemodynamic parameters, recovery profile, and any complications were noted. Motor seizure duration was recorded as the time interval between starting of the seizure episode until the cessation of tonic-clonic motor activity in the isolated upper limb. Data were entered in MS-Excel and analyzed in SPSS V22. Descriptive statistics were represented with percentages, and parametric data were represented with mean with standard deviation. The statistical analysis was carried out using the Chi-square test, independent t-test. A Probability (P) value Results: There was a statistically significant difference in seizure duration between two groups with Group A having less meantime 38 ± 14.9 s compared to Group B 45.3 ± 17.5 s with P = 0.014. Seizure threshold, hemodynamic parameters, recovery profile, and complications except myoclonus were not statistically significant between the two groups. Conclusion: Etomidate has the definite advantage of longer seizure duration compared to ketofol, and hence, etomidate is a better induction agent in patients with psychiatric disorders undergoing ECT.

Semptomatik Hipokalsemi ve Nütrisyonel Rikets Nedenli Hastane Başvuruları: Göçmen Suriye’li Süt Çocuklarının Predominansı

Amac: Nutrisyonel rikets, buyume cagindaki cocuklarda D vitamini ve kalsiyum eksikligine bagli olarak gelisen epifizyal kikirdagin defektif mineralizasyonudur. Nutrisyonel riketsin onlenmesi icin D vitamini profilaksisi ulkemizde sut cocuklugu doneminde rutin olarak uygulanmaktadir. Ancak halen semptomatik hipokalsemi ve D vitamini eksikligi nedeniyle hastane basvurularinin olmasi dikkat cekicidir. Calismamizda bu olgularin ozelliklerinin ortaya konulmasi amaclanmistir. Gerec ve Yontem: Ocak 2018-Mayis 2018 tarihleri arasinda nutrisyonel rikets tanisi ile yatirilarak izlenmis olan 28 hastanin (9 kiz, 19 erkek) verisi retrospektif olarak incelendi. Olgularin basvuru anindaki antropometrik ve laboratuvar ozellikleri kaydedildi. Bulgular: Olgularin ortanca basvuru yasi 8,8 (1-35) ay idi. Olgularin %67,9’u Suriye gocmeni idi. Hastalarin %57,1’i hic D vitamini profilaksisi almamis, %42,9’u ise duzensiz almisti. Basvuru yakinmasi olgularin %50’sinde konvulsiyon gecirme, %28,6’sinda rikets bulgulari iken %21,4’unde ise ates, enfeksiyon vb. nedenlerden dolayi hastaneye basvuran hastalarda rastlantisal olarak hipokalsemi tespit edilmis olmasi idi. Ilk basvuruda ortanca kalsiyum 6,6 mg/dL; iyonize kalsiyum 0,85 mmol/L; fosfor 3,5 mg/dL; alkalen fosfataz 787 U/L; parathormon 241 pg/mL ve 25-hidroksi vitamin D 3,4 ng/mL saptanmisti. Olgularin %60,7’sinde hipofosfatemi, %3,6’sinda hiperfosfatemi mevcuttu. Tum olgular hastanede yatirilarak tedavi edilmis ve D vitamini eksikligine bagli nutrisyonel rikets tanisi almisti. Sonuc: Ulkemizde D vitamini profilaksisine ragmen halen nutrisyonel rikets gorulebilmekte ve kis-ilkbahar aylarinda hipokalsemik konvulsiyon nedeniyle hastane basvurularina yol acabilmektedir. Ozellikle gocmen Suriyeli sut cocuklarinda D vitamini destek programina uyumun artirilmasi nutrisyonel riketsin onlenmesi acisindan onemlidir.

Short Communication on Epilepsy

Epilepsy is a neurological disorder characterized by seizures. Short bursts of intense electrical energy in the brain cause seizures. When these bursts occur in the brain, it is known as a partial seizure. When they occur throughout the brain, it is known as a generalized seizure. These seizures cause symptoms in the entire body. This type of epilepsy was generally known as generalized convulsive epilepsy. Generalized seizures follow a basic pattern. First, your muscles stiffen and become rigid. Then, you experience violent muscle contractions in which the muscles move in quick, random spasms. You lose consciousness, or black out, so that you are no longer aware of what’s happen ing.

Mutations in aminoacyl-tRNA synthetase genes: an analysis of 10 cases

To study the clinical features of the diseases associated with aminoacyl-tRNA synthetases (ARS) deficiency. A retrospective analysis was performed of the clinical and gene mutation data of 10 children who were diagnosed with ARS gene mutations, based on next-generation sequencing from January 2016 to October 2019. The age of onset ranged from 0 to 9 years among the 10 children. Convulsion was the most common initial symptom (7 children). Clinical manifestations included ataxia and normal or mildly retarded intellectual development (with or without epilepsy; n=4) and onset of epilepsy in childhood with developmental regression later (n=2). Some children experienced disease onset in the neonatal period and had severe epileptic encephalopathy, with myoclonus, generalized tonic-clonic seizure, and convulsive seizure (n=4); 3 had severe delayed development, 2 had feeding difficulty, and 1 had hearing impairment. Mutations were found in five genes: 3 had novel mutations in the AARS2 gene (c.331G>C, c.2682+5G>A, c.2164C>T, and c.761G>A), 2 had known mutations in the DARS2 gene (c.228-16C>A and c.536G>A), 1 had novel mutations in the CARS2 gene (c.1036C>T and c.323T>G), 1 had novel mutations in the RARS2 gene (c.1210A>G and c.622C>T), and 3 had novel mutations in the AARS gene (c.1901T>A, c.229C>T, c.244C>T, c.961G>C, c.2248C>T, and Chr16:70298860-70316687del). A high heterogeneity is observed in the clinical phenotypes of the diseases associated with the ARS deficiency. A total of 14 novel mutations in 5 genes are reported in this study, which enriches the clinical phenotypes and genotypes of the diseases associated with ARS deficiency.

Vasovagal syncope or postural orthostatic tachycardia syndrome in children with neurological symptoms at disease onset: a clinical analysis of 88 cases

To study the clinical features of vasovagal syncope (VVS) and postural orthostatic tachycardia syndrome (POTS) in children with neurological symptoms at disease onset. A retrospective analysis was performed on the medical data of 88 children with the initial symptoms of the nervous system, such as transient loss of consciousness, dizziness, headache, and convulsion, who were finally diagnosed with VVS or POTS. Of the 88 children, there were 35 boys (40%) and 53 girls (60%), with an age of 4-15 years. The peak age of onset was between 10 and 13 years. All the children had the initial symptoms of transient loss of consciousness, dizziness, headache, and convulsion. Nervous system diseases were excluded by electroencephalography, cerebrospinal fluid examination, and cranial MRI. Of the 88 children, 53 (60%) were confirmed with VVS, and 35 (40%) with POTS, according to the results of head-up tilt test (HUTT). Five children with the initial symptom of transient loss of consciousness were misdiagnosed with epilepsy. Predisposing factors were determined for 59 children (67%), and prolonged standing was the most common factor, followed by change in body position and strenuous exercise. Premonitory symptoms were observed in 66 children (75%), among which chest discomfort was the most common symptom, followed by gastrointestinal symptoms (nausea, vomiting, and abdominal pain) and pale complexion. All 88 children received health education and exercise for autonomic nerve function, among whom 53 children with VVS were given oral rehydration salts and 35 children with POTS were given oral rehydration salts and metoprolol. All 88 children were followed up for 18 months, and the response rates to the above treatment at 3, 6, 12, and 18 months of follow-up were 87%, 93%, 93%, and 90% respectively. In addition to nervous system diseases, functional cardiovascular diseases including VVS and POTS should be considered for children with the initial symptoms of transient loss of consciousness, dizziness, headache, and convulsion. HUTT can be used to make a confirmed diagnosis, and the early treatment can achieve a good outcome.

Clinical features and SLC6A8 gene mutations of cerebral creatine deficiency syndrome I: an analysis of two families

This article reports the clinical and genetic features of two cases of cerebral creatine deficiency syndrome I (CCDSI) caused by SLC6A8 gene mutations. Both children were boys. Boy 1 (aged 2 years and 10 months) and Boy 2 (aged 8 years and 11 months) had the clinical manifestations of delayed mental and motor development, and convulsion. Their older brothers had the same symptoms. The mother of the boy 1 had mild intellectual disability. The genetic analysis showed two novel homozygous mutations, c.200G>A(p.Gly67Asp) and c.626_627delCT(p.Pro209Argfs*87), in the SLC6A8 gene on the X chromosome, both of which came from their mothers. These two novel mutations were rated as possible pathogenic mutations and were not reported in the literature before. This study expands the mutation spectrum of the SLC6A8 gene and has great significance in the diagnosis of boys with delayed development, and epilepsy.

Analysis of adverse reactions of Xianling Gubao preparation based on real world SRS data

From January 1, 2004 to July 21, 2016 a total of 2 796 cases of adverse drug reaction/adverse event(ADR/AE) after the use of Xianling Gubao Capsules/Tablets were reported by National Adverse Drug Reaction Monitoring Center. The following results were obtained by analyzing the reports of 2 796 cases of adverse drug reactions/adverse drug events after the use of Xianling Gubao Capsules/Tablets. A total of 75 patients, accounting for 2.68% of the total ADR/AE time, had severe ADR/AE events. Among them, 30 patients were aged 65 and above, accounting for 40.00% of the total number of severe ADR/AE patients. All the patients with ADR/AE were aged 45-64 years, which totaled 1 346 cases and took up 48.14% of the total patients with ADR/AE. All of ADR/AE cases and severe ADR/AE cases were orally given Xianling Gubao Capsules/Tablets. Females accounted for 52.50% and 76.00%, respectively, and the proportion of females was significantly higher than that of males. Among patients with a medical history of ADR/AE, severe ADR/AE was higher than the average, accounting for about 1.33%. The proportion of cases orally given 1-3 tablets of Xianling Gubao Capsules/Tablets in all ADR/AE cases and severe ADR/AE cases was 95.32% and 96.00%, which conformed to the usage in the package insert. All ADR/AE cases and severe ADR/AE cases orally given Xianling Gubao Capsules/Tablets twice daily occupied the highest proportions, or 77.00% and 61.00%, respectively. The proportion of severe ADR/AE cases orally given Xianling Gubao Capsules/Tablets was slightly higher than that of all ADR/AE cases in the medication frequency, which didn't conform to the usage in the package insert. All the symptoms of ADR/AE orally given Xianling Gubao Capsules showed many manifestations, and the top 10 symptoms were nausea, rash, itching, stomach dysfunction, vomiting, abdominal pain, dizziness, diarrhea, anaphylaxis, and reflux heartburn. The symptoms of severe ADR/AE after oral administration of Xianling Gubao Capsules were varied, and the top 10 symptoms were abnormal liver function, rash, suffocation, itching, dizziness, vomiting, anaphylaxis, abdominal pain, weakness, and convulsions. Abnormal liver function accounted for 44.12%. All of ADR/AE cases occurred within 2 days after oral administration of Xianling Gubao Capsules/Tablets, accounting for 54.26%. Severe ADR/AE occurred within 2 days after the use of Xianling Gubao Capsules/Tablets, accounting for 25.34%. The proportion of ADR/AE cases occurring within 15 days after oral administration of Xianling Gubao Capsules/Tablets increased again(57.33%). The overall trend contained two peaks.

Clinical features of central nervous system demyelinating diseases with myelin-oligodendrocyte glycoprotein antibody positive in children

Objective To investigate the clinical features and treatment effect of children with central nervous system demyelinating diseases and seropositivity to myelin-oligodendrocyte glycoprotein (MOG) antibody. Methods The clinical characteristics of 28 had seropositivity to MOG among 115 children with central nervous system demyelinating diseases and who were hospitalized at Department of Neurology, Children′s Hospital of Zhejiang University School of Medicine from March 2017 to February 2019 were retrospectively analyzed. Results Twenty-eight patients were included in this study, including 10 males and 18 females, with the ratio of male/female of 1.00∶1.80, and the median age of 7 years and 9 months.The clinical manifestations were diverse, including encephalopathy symptoms such as hea-dache, vomiting, and drowsiness (13/28 cases), vision loss (7/28 cases), spinal symptoms (6/28 cases), cerebellar symptoms such as ataxia, slurred speech (4/28 cases), convulsions (2/28 cases), and cranial nerve symptoms (1/28 cases). Among 24 cases who underwent CSF detection, 10 patients (41.7%) had slightly increased white blood cells, 2 patients (8.3%) had elevated protein, 6 patients (25.0%) had positive MOG antibody, and CSF-restricted oligoclonal band was negative in all 24 patients.Twenty-five cases (89.3%) showed brain magnetic resonance imaging (MRI) abnormalities, including cerebral white matter (20/28 cases), cerebellum (10/28 cases), cerebral gray matter (9/28 cases), thalamus/basal ganglia (6/28 cases), brainstem (6/28 cases), optic nerve (5/28 cases), and corpus callosum (4/28 cases). Of the 28 cases, 13 patients had spinal cord involvement, involving cervical spinal cord in 10 cases, thoracic cord in 9 cases and lumbar spinal cord in 5 cases; besides, 8 cases of them had long segmental spinal cord lesions with ≥ 3 segments.Fourteen patients received the visual evoked potentials′ examination, and the subclinical visual impairment was found in 2 of them with unobstructed clinical performance.All patients underwent high-dose Methylprednisolone therapy.The clinical symptoms of 16 patients who were treated with Gamma globulin were relieved in the acute phase.Seven patients had recurrence during the follow-up period, with the recurrence rate of 25.0%.Relapsed patients re-treated with high-dose Methylprednisolone therapy combined with Gamma globulin, clinical symptoms could be alleviated. Conclusion The main clinical phenotype of children with central nervous system demyelinating diseases and seropositivity to MOG is acute disseminated encephalomyelitis.The spinal cord lesions are mainly involving cervical and thoracic segments.The current treatments of this disease include glucocorticoid and Gamma globulin, which have significant effect, but the disease is easy to relapse.The re-use of glucocorticoid and Gamma globulin after relapse is still effective. Key words: Myelin-oligodendrocyte glycoprotein antibody; Child; Central nervous system demyelinating disease

Takayasu Arteritis Mistaken for Epilepsy: A Case Presenting With Convulsive Syncope

Takayasu arteritis (TA) is a chronic inflammatory disease characterized by granulomatous vasculitis that predominantly manifests as panaortitis. This occlusive thromboaortopathy lacks pathognomonic features often resulting in a diagnostic dilemma leading to its under-recognition, misdiagnosis and delayed management. Although neurological manifestations are not uncommon in TA, convulsive syncope as an initial clinical presentation is extremely rare. We report a case of convulsive syncope as a manifesting symptom of TA. A 17-year-old male patient of African origin was referred to us from an upcountry regional hospital with a diagnosis of medically intractable epilepsy for cardiovascular review. He presented with a 28-week history of generalized tonic-clonic seizures followed by loss of consciousness. He denied history of recurrent headaches, fever, visual disturbances, arthralgias, claudication or unintentional weight loss. Physical examination revealed feeble left-sided brachial and radial pulses, elevated blood pressure, differences in blood pressure between arms and left-sided carotid and vertebral bruits. Computed tomography angiogram of his thoracic and abdominal aorta revealed changes suggestive of a diffuse arteritis. Additionally, magnetic resonance angiogram of the brain revealed total occlusion of the left common carotid, left internal carotid, left external carotid and left vertebral arteries. Based on the physical examination and radiological findings, we reached a diagnosis of TA. He was prescribed dexamethasone, methotrexate, acetylsalicylic acid and amlodipine. He had a remarkable recovery and was seizure-free for the last 5 months after discharge. TA may manifest with convulsive syncope mimicking epilepsy. Despite its rarity, presentations of this nature continue to challenge clinicians resulting in delayed diagnosis with irreversible life-threatening consequences to patients. In view of this, physicians should strive to take detailed history and perform thorough physical examination so as to timely pick the characteristic signs of TA especially in patients presenting with unanticipated symptoms. J Med Cases. 2020;11(2):37-40 doi: https://doi.org/10.14740/jmc3424

The effect of ketamine versus thiopental sodium on hemodynamic profile and seizure duration during electroconvulsive therapy in patients with major depressive disorder: A randomized cross-over clinical trial.

Background: Electroconvulsive therapy (ECT) is an effective treatment in major depressive disorder (MDD). Earlier studies suggest that ketamine has antidepressive effects and prolongs seizure duration in favor of therapeutic efficacy of ECT. A great concern with the use of ketamine is its possible adverse hemodynamic effects during ECT. This study was conducted to compare the hemodynamic effect and seizure duration between ketamine and sodium thiopental, a common anesthetic in ECT. Methods: In a cross-over clinical trial, 26 patients of either sex with MDD who were candidates for ECT therapy in a university hospital were enrolled. A total of 13 patients received induction with ketamine in the first session and thiopental for the second. The sequence of treatments was vice versa in the other group. The followings were measured in all patients: blood pressure, heart rate, and oxygen saturation before induction, immediately after induction, at 1, 2, 4, and 10 minutes postinduction, and after recovery from anesthesia. Also, seizure duration, recovery time, and complications of treatments were measured. Chi square test and student t test were used for categorical data and numerical data, respectively. P values < 0.05 were considered statistically significant. Analyses were performed with SPSS software version 21.0. Results: Heart rate, systolic and diastolic blood pressure, oxygen saturation, and recovery time were comparable between groups. Mean duration of convulsion in patients who received ketamine was significantly more than those who received STP (36±11 vs 28±9 s, p=0.001). Myalgia was less common in patients who received ketamine rather than sodium thiopental (11.5 vs 46.2%, p=0.014). Conclusion: Ketamine prolongs seizure duration with no deleterious effects on hemodynamic parameters. Considering the antidepressant effects of ketamine, it may be a better choice, compared to thiopental sodium, for MDD patients.