Evaluation of ubiquitin C-terminal hydrolase-L1 enzyme levels in patients with epilepsy / Avaliação dos níveis de enzima ubiquitina C-terminal hidrolase-L1 em pacientes com epilepsia

ABSTRACT Objective: Ubiquitin C-terminal Hydrolase-L1 (UCH-L1) enzyme levels were investigated in patients with epilepsy, epileptic seizure, remission period, and healthy individuals. Methods: Three main groups were evaluated, including epileptic seizure, patients with epilepsy in the non-seizure period, and healthy volunteers. The patients having a seizure in the Emergency department or brought by a postictal confusion were included in the epileptic attack group. The patients having a seizure attack or presenting to the Neurology outpatient department for follow up were included in the non-seizure (remission period) group. Results: The UCH-L1 enzyme levels of 160 patients with epilepsy (80 patients with epileptic attack and 80 patients with epilepsy in the non-seizure period) and 100 healthy volunteers were compared. Whereas the UCH-L1 enzyme levels were 8.30 (IQR=6.57‒11.40) ng/mL in all patients with epilepsy, they were detected as 3.90 (IQR=3.31‒7.22) ng/mL in healthy volunteers, and significantly increased in numbers for those with epilepsy (p<0.001). However, whereas the UCH-L1 levels were 8.50 (IQR=6.93‒11.16) ng/mL in the patients with epileptic seizures, they were 8.10 (IQR=6.22‒11.93) ng/mL in the non-seizure period, and no significant difference was detected (p=0.6123). When the UCH-L1 cut-off value was taken as 4.34 mg/mL in Receiver Operating Characteristic (ROC) Curve analysis, the sensitivity and specificity detected were 93.75 and 66.00%, respectively (AUG=0.801; p<0.0001; 95%CI 0.747‒0.848) for patients with epilepsy. Conclusion: Even though UCH-L1 levels significantly increased more in patients with epilepsy than in healthy individuals, there was no difference between epileptic seizure and non-seizure periods.

RESUMO Objetivo: Níveis da enzima ubiquitina C-terminal hidrolase-L1 (UCH-L1) foram investigados em pacientes com epilepsia, crise epiléptica, período de remissão e indivíduos saudáveis. Método: Foram avaliados três grupos principais, incluindo crise epiléptica, epilepsia no período não convulsivo e voluntários saudáveis. Pacientes com convulsão no departamento de emergência ou trazidos por confusão pós-ictal foram incluídos no grupo de crise epiléptica. Os pacientes que tiveram crise epiléptica ou foram ao ambulatório de Neurologia para acompanhamento foram incluídos no grupo não convulsivo (período de remissão). Resultados: Os níveis da enzima UCH-L1 de 160 pacientes com epilepsia (80 pacientes com crise epiléptica e 80 pacientes com epilepsia no período não convulsivo) e 100 voluntários saudáveis foram comparados. Enquanto os níveis da enzima UCH-L1 foram 8,30 (IQR=6,57‒11,40) ng/mL em todos os pacientes com epilepsia, os níveis detectados foram de 3,90 (IQR=3,31‒7,22) ng/mL em voluntários saudáveis e aumentaram significativamente na epilepsia (p<0,001). No entanto, ao passo que os níveis de UCH-L1 foram 8,50 (IQR=6,93‒11,16) ng/mL nos pacientes com crise epiléptica, foram 8,10 (IQR=6,22‒11,93) ng/mL no período não convulsivo, e nenhuma diferença significativa foi detectada (p=0,6123). Quando o valor de corte de UCH-L1 foi considerado 4,34 mg/mL com base na análise da curva ROC, sensibilidade e especificidade foram detectadas como 93,75 e 66,00%, respectivamente (AUG=0,801; p<0,0001; IC95% 0,747‒0,848) para os pacientes com epilepsia. Conclusão: Embora os níveis de UCH-L1 tenham aumentado significativamente nos pacientes com epilepsia em relação aos indivíduos saudáveis, não foi observada diferença entre crise epiléptica e períodos não convulsivos.

Vitamin D deficiency rickets in infants presenting with hypocalcaemic convulsions / Raquitismo por deficiencia de vitamina D en lactantes que presentan convulsiones por hipocalcemia

AIM: Hypocalcaemia evaluation of the clinical, biochemical and radiological features of 91 infants with rickets who presented as hypocalcaemic convulsions. SUBJECTS AND METHODS: Ninety-one hypocalcaemic infants who were brought to hospital with convulsion and diag-nosed with rickets related to vitamin D deficiency according to their clinical, biochemical and radio-logical features were retrospectively reviewed. RESULTS: Mean values of the laboratory data were as follows: calcium 5.55 ± 0.79 mg/dL, phosphorus 4.77 ± 1.66 mg/dL, alkaline phosphatase 1525.5 ± 925.4 U/L and parathormone 256.8 ± 158.3 pg/mL. Serum 25-OH vitamin D levels were below normal (< 20 ng/mL) in 37 infants. CONCLUSION: Vitamin D deficiency should be considered in infants presenting with hypocalcaemia. To avoid complications such as convulsions, clinicians should give vitamin D supplementation to such infants.

OBJETIVO: Evaluación hipocalcémica de los aspectos clínicos, bioquímicos y radiológicos de 91 lactantes con raquitismo, que presentaron convulsiones por hipocalcemia. PACIENTES Y MÉTODOS: Noventa y un lactantes hipocalcémicos llevados al hospital con convulsiones y a quienes se les diagnosticó raquitismo asociado a la deficiencia de vitamina D de acuerdo con sus características, bioquímicas y radiológicas, fueron revisados retrospectivamente. RESULTADOS: Los valores medios de los datos de laboratorio fueron los siguientes: calcio 5.55 ± 0.79 mg/dL, fósforo 4.77 ± 1.66 mg/dL, fosfatasa alcalina 1525.5 ± 925.4 U/L, y paratohormona 256.8± 158.3 pg/mL. Los niveles séricos de la vitamina 25 (OH) D estuvieron por debajo de lo normal en 37 lactantes (< 20 ng/mL). CONCLUSIÓN: La deficiencia de vitamina D debe considerarse en los infantes que se presentan con hipocalcemia. A fin de evitar complicaciones tales como convulsiones, se les debe dar suplementos de vitamina D.

Comparison of Serum Zinc Levels Measured by Inductively Coupled Plasma Mass Spectrometry in Preschool Children with Febrile and Afebrile Seizures

BACKGROUND: Changes in levels of trace elements have been proposed to underlie febrile seizures. Particularly, low zinc levels have been proposed as related factor of febrile seizure. In this study, we investigated whether mean serum zinc levels differed between children with febrile seizure and afebrile seizure. METHODS: Using inductively coupled plasma mass spectrometry, serum zinc levels were measured in 288 children who had been diagnosed with febrile seizures (N=248) and afebrile seizures (N=40). Mean serum zinc levels were compared between the 2 groups. RESULTS: Mean serum zinc level was 60.5+/-12.7 microg/dL in the febrile seizure group and 68.9 +/-14.5 microg/dL in the afebrile seizure group. A significant difference in serum zinc levels was observed between the febrile and afebrile seizure groups (P<0.001). CONCLUSIONS: Zinc levels in children with febrile seizure were significantly lower than those in children with afebrile seizure.

Seizures in hyperglycemic patients.

AIM OF STUDY: To study the pattern of seizures in hyperglycemic patients and its correlation with serum osmolality, blood sugar and serum sodium. MATERIALS AND METHODS: (Study period June 1999-June 2000) Forty patients who presented with first time seizures and who were detected to be diabetic were included in the study. All patients had detailed history, clinical examination blood sugar, BUN, serum electrolytes, serum osmolality, serum calcium estimation. EEG and CT head scan was also done. The seizures were classified as per ILAE classification (1981). The study group was further divided into Group A--those with normal CT (n 24) and Group B-- those showing infarcts in CT scan (n 16). The control group (n 40) were matched for age and had first occurrence of seizures and CT showed infarct and were euglycemic. All the investigations were done for the control group as well. Statistical analysis was done using ANOVA. RESULTS: None of the patient had diabetic ketoacidosis. Patients in study group had statistically significant incidence of (1) Focal seizures with or without generalisation (100%); (2) Visual hallucination (25%); (3) Epilepsia partialis continua (20%). Comparing the subgroups (A and B) complex partial seizures and visual hallucination was significant at 5% level in the group A (NKH with normal CT). Ninety percent in the study group had increased serum osmolality (Vs 22.5% in the control group). Duration (eight days Vs 1) and frequency (15 per day vs 1.45) were positively correlated with RBS > S.osmolality > S.sodium. CONCLUSION: 1. Long duration and frequent focal motor seizures with or without secondary generalisation occurs in NKH which at times may be the first manifestation of DM. 2. Complex partial seizures, visual hallucination and epilepsia partialis continua are more common in NKH patients than in euglycemic patients. 3. There is positive correlation of blood sugar, S.osmolality and S.sodium with frequency and duration of seizures. RBS > S.osmolality > S.Na. 4. Correction of hyperglycemia is the main stay of management of seizures. 5. Movement induced seizures which is specific for NKH is a rarity.

Independent and combined effects of L-arginine and diazepam on ammonium chloride-induced convulsions in rats.

The independent and combined effects of L-arginine (840 mg/kg) and diazepam (0.75 mg/kg) pretreatment (30 min) were tested on ammonium chloride (400 mg/kg)-induced convulsions in rats. Ammonia concentrations were determined in blood and brain regions (cerebral cortex, brain stem and cerebellum) 30 min after L-arginine or diazepam treatment. Ammonia concentrations were measured at the time of induction of convulsions by ammonium chloride in L-arginine, diazepam or saline pretreated animals. L-arginine and not diazepam decreased ammonia concentrations in control as well as in ammonium chloride-treated animals. However, both the compounds suppressed convulsions elicited by ammonium chloride. Protection produced concurrently by these agents was much greater than that produced by them independently. It is concluded that convulsions caused by hyperammonemic condition can be suppressed either by preventing a rise in brain ammonia to toxic level or by anticonvulsant agents having a GABA potentiating action. A much greater protection can be achieved if agents having these properties are administered concurrently.

Clinical management and outcome of eclampsia at Rajavithi Hospital.

The purpose of this clinical study was to review experience in the management, and outcome of eclamptic patients at Rajavithi Hospital. Standardized treatment for all cases of eclampsia has consisted of magnesium sulfate intravenously and intramuscularly to control convulsions by means of Chesley and Tepper's regimen, intravenous hydralazine intermittently to lower diastolic blood pressure when it exceeds 110 mmHg, and initiation of delivery as soon as the patient has regained consciousness and is stable. During a ten-year period there were 167,200 deliveries and 90 eclamptic patients, yielding an incidence of eclampsia of 1 in 1,857 deliveries. There were three maternal deaths (3.3%) due to intracerebral hemorrhage. Serious adverse maternal outcomes were more frequent in women whose convulsions occurred before delivery. Excluding postpartum cases, perinatal mortality of fetuses weighing 1,000 g or more was 11.7 per cent. Magnesium sulfate is the drug of choice for treatment of eclamptic convulsions. In most situations, clinical assessment of deep tendon reflexes, respirations, and urine output is adequate to monitor maternal magnesium toxicity without the need to determine actual maternal serum magnesium levels.

Biochemical abnormalities in neonatal seizures.

Early diagnosis and appropriate treatment of biochemical abnormalities accompanying neonatal seizures is important for effective seizure control and to avoid further brain damage. The present study was carried out on 35 neonates to determine the frequency of various biochemical abnormalities in neonatal seizures. Diagnostic evaluation included estimation of levels of serum calcium, phosphorus, magnesium, sodium, potassium, zinc, and blood glucose. Two-thirds of the neonates with seizures had biochemical disturbances in their sera. A variety of abnormalities occurred in asphyxiated infants, including hyponatremia, hypoglycemia, hypocalcemia, and hypomagnesemia. Primary metabolic disorders accounted for one-forth of the cases of neonatal seizures, the most common being hypoglycemia, hypoglycemia/hypocalcemia, and hypocalcemia/hyperphosphatemia. Inappropriate intrauterine growth, inadequate feeding, and feeding with cow's milk were the main risk factors for primary metabolic seizures. Hyponatremia was a frequent finding in seizures resulting from brain damage like birth asphyxia, meningitis, and intracranial hemorrhage. No infant had hypernatremia, hyperkalemia, hypokalemia, or low serum zinc.

Prolactin and cortisol levels in seizure disorders.

Levels of prolactin (PRL) and cortisol were estimated to find out the acute effects of generalised tonic clonic seizures (GTCS), partial seizures and pseudoseizures in 60, 18 and 9 patients respectively. Prolactin levels were estimated at 20, 60 and 120 minutes whereas, cortisol was estimated at 20, 60, and 120 minutes postictally. Cortisol and PRL estimation was also done in 10 healthy controls and 11 patients of epilepsy during interictal phase. Serum PRL levels were elevated (> 25 ng/ml) in 68.33% of GTCS and 11.11% of partial seizure cases. The peak levels were achieved in first 30 minutes after the seizures with a gradual return to base line during subsequent one hour. None of the patients with pseudoseizure showed any rise in serum PRL levels. The interictal PRL levels were normal in all the epileptics. Plasma cortisol levels were elevated during 60 to 120 minute postictal period in 45% of GTCS, 55.55% of partial seizures and 66.66% of pseudoseizure patients. Cortisol appears to be non-selectively triggered by all stressful events but postictal PRL estimation can help in differentiating pseudoseizures from GTCS. While an elevated PRL indicates the occurrence of grandmal seizure, a normal postictal PRL level does not always exclude epileptic seizure, specially a partial seizure.

Plasma prolactin in epilepsy and pseudoseizures.

Postictal values of plasma prolactin levels were measured in 15 children with generalized seizures, 8 children with pseudoseizures and 6 control subjects. In patients with generalized seizure, the mean plasma prolactin level was 28.6 +/- 2.3 ng/ml, whereas in patients with pseudoseizures and in controls, its mean values were 10.4 +/- 3.8 ng/ml and 9.8 +/- 2.6 ng/ml, respectively. Thus, prolactin levels were significantly (p < 0.001) elevated following generalized seizures but were almost normal following pseudoseizures. Plasma prolactin levels may, therefore, be helpful in differentiating between generalized seizures and pseudoseizures.

Serum prolactin and cortisol in children with some paroxysmal disorders.

Postictal serum prolactin and cortisol levels were estimated in 73 children having either epilepsy, febrile seizures, breath-holding spells, or fever without other manifestation and in 20 normal controls. Mean serum prolactin levels (28.6 +/- 2.3 ng/ml) were significantly higher (p < 0.001) in the epileptic group than in the group with febrile seizures (12.7 +/- 2.8 ng/ml), non-specific febrile illness (12.2 +/- 2.4 ng/ml), breath-holding spells (8.8 +/- 1.1 ng/ml) and normal controls (9.8 +/- 2.6 ng/ml). Mean serum cortisol levels were non-specifically elevated in children with epilepsy (32.8 +/- 2.2 ug/dl), febrile convulsion (34.2 +/- 4.1 ug/dl) and non-specific febril illness (30.6 +/- 2.4 ug/dl). Our observations suggest that elevated prolactin levels associated with afebrile epileptic seizures may help in differentiating epilepsy from febrile seizures and breath-holding spells. Cortisol levels appear to be non-specifically elevated in all stressful conditions.

Níveis plasmáticos do fenobarbital em crianças convulsivas

Verificou-se a obediência `a prescriçäo médica, através da dosagem do fenobarbital plasmático, em 70 crianças que, segundo a informaçäo dos genitores, vinham usando regularmente esse medicamento. Em apenas 32,8% dos casos, o nível sérico do fenobarbital estava nos limites terapêuticos