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Showing 8 results for Encephalopathy
The Susac's syndrome: A case report from neurology ward of Imam Khomeiny Hospital, 1999
Ghlich Nia Omrani H, Ghaffar Poor M,
Volume 59, Issue 2 (5-2001)
Abstract
This syndrome is a very rare neurological presentation which was first reported by Susac in 1975, and usually involves the young women with triad of: (1) Visual loss due to occlusion of retinal artery branches. (2) Subacute encephalopathy with psychic manifestation, personality disorders, involvement of the cortico-spianal tracts, pseudo-bulbar, focal or generalized seizures and myoclonic jerks. (3) Bilateral sensory neural hearing loss with tinnitus. All of the symptoms and signs are pathologically due to microinfarcts. It's pathogenesis is unknown. Brain biopsy shows multiple microinfarctions. Diagnosis of this syndrome is based on the clinical tiard, retinal angiography and neuroimaging studies. In this report, a young lady of 25 years old was referred to neurology ward of Imam Khomeini Hospital with above mentioned symptoms an signes and was recognized as a case of this syndrome.
The Susac's syndrome: A case report from neurology ward of Imam Khomeiny Hospital, 1999
Ghlich Nia Omrani H, Ghaffar Poor M,
Volume 59, Issue 3 (6-2001)
Abstract
This syndrome is a very rare neurological presentation which was first reported by Susac in 1975, and usually involves the young women with triad of: (1) Visual loss due to occlusion of retinal artery branches. (2) Subacute encephalopathy with psychic manifestation, personality disorders, involvement of the cortico-spianal tracts, pseudo-bulbar, focal or generalized seizures and myoclonic jerks. (3) Bilateral sensory neural hearing loss with tinnitus. All of the symptoms and signs are pathologically due to microinfarcts. It's pathogenesis is unknown. Brain biopsy shows multiple microinfarctions. Diagnosis of this syndrome is based on the clinical tiard, retinal angiography and neuroimaging studies. In this report, a young lady of 25 years old was referred to neurology ward of Imam Khomeini Hospital with above mentioned symptoms an signes and was recognized as a case of this syndrome.
Ekiri syndrome: a report of 13 cases
Rahbarimanesh Aa, Zandkarimi Mr, Naderi F, Salamati P,
Volume 66, Issue 11 (2-2009)
Abstract

Background: Ekiri syndrome or lethal toxic encephalopathy is a complication of shigellosis with dysentery, hyperpyrexia, seizures, headache and altered level of consiousness, which rapidly progresses to death. These children die at the beginning of the disease (8-48 hours from the beginning of symptoms), from brain edema. However they had no symptoms or signs of sepsis, dehydration, DIC or Hemolytic Uremic Syndrome (HUS).

Methods: This survey is a case series study of children with Ekiri syndrome in Bahrami hospital from October 1998-2008 presented with loss of consciousness, colitis and high fever shortly after admission. Information about the patients was gathered from the documents according to physical signs and symptoms, lab data of those whom Ekiri syndrome had been diagnosed for them. Studied variables in this assessment were age, sex, fever, convulsions and loss of consciousness. Headache, encephalopathy, dehydration, elevated ICP, colitis, underline disease, stool, blood and CSF cultures.

Results: The subjects contain 13 cases (10 male, 3 female), averaged 30/5 months of age. All had seizure, elevated ICP, encephalopathy and coma. All of the patients had fever between 39 and 40, averaged 39.5 degree of centigrade. Seven patients had headache and three ones was dehydrated. The first presentation symptom in three patients was gastroenteritis, in 9 was siezure and in 1 patient was headache. Stool culture in all patients was positive, but blood culture was positive in only one of them. CSF culture was negative in all of the patients. Mortality was 100%.

Conclusion: Symptoms, signs and presentation of Ekiri syndrome, a rare complication of infection with shigella, in the patients in Bahrami hospital was similar with the other studies beforehand in other countries. In this study, all the patients were died and supportive treatments were ineffective.


Determining the specific activity of thymidine phosphorylase in leukocytes of patients with MNGIE and the plasma thymidine level by RP-HPLC
Rezaei Sh, Salehipour M, Golestani A, Vardasbi Joybary I S, Nafisi Sh, Doosti M, Golmohammadi T,
Volume 69, Issue 3 (6-2011)
Abstract

Background: Thymidine phosphorylase (TP) catalyses the conversion of thymidine into thymine. Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disease which is caused by mutations in the nuclear gene encoding TP, bringing about severe impairment of TP-enzyme specific activity and accumulation of thymidine in plasma. The clinical manifestations of MNGIE are recognizable and homogenous, but not in the early stages of the disease. In patients who are suspected of having MNGIE, determination of TP-specific activity in leukocytes and thymidine levels in plasma are diagnostic. The methods that are usually used for the measurement of TP activity and plasma thymidine are not rapid or accurate enough and lack sensitivity.

Methods: The specific activity of TP was measured by RP-HPLC in leukocytes of both the controls and the patients exhibiting clinical features suggestive of MNGIE. Moreover, plasma thymidine was assessed by the same method.

Results: The patients had detectable plasma thymidine (>3 µmol/L) but it was undetectable in the healthy controls. The patients' TP-specific activity decreased to less than 5% relative to the controls (14±4 nmol/h/mg vs. 525±165 nmol/h/mg, P<0.05). A diagnostic algorithm for the definitive diagnosis of MNGIE is suggestible based on the results of this study which relies on the measurement of plasma thymidine, TP-specific activity in leukocytes, or both.

Conclusion: In this study, we set up a sensitive and rapid assay for the evaluation of TP-specific activity by using RP-HPLC in Iran. In addition, we established reference values for TP-specific activity and plasma thymidine in the Iranian patients.


Wernicke’s encephalopathy following hyperemesis gravidarum
Leila Pourali , Sedighe Ayati , Atieh Vatanchi , Parvaneh Layegh , Nasrin Pourmoghadam ,
Volume 74, Issue 3 (6-2016)
Abstract

Background: ″Wernicke’s Korsakoff″ syndrome is the most important complication of severe thiamine deficiency. The term refers to two different syndromes, each representing a different stage of the disease. Wernicke’s encephalopathy (WE) is an acute syndrome requiring emergent treatment to prevent death and neurologic morbidity. Korsakoff syndrome (KS) refers to a chronic neurologic condition that usually occurs as a consequence of WE. It is a rare complication of hyperemesis gravidarum that confusion, ocular signs, and gait ataxia are the most prevalent symptoms, respectively. Typical brain lesions of wernicke’s encephalopathy (WE) are observed at autopsy in 0.4 to 2.8 percent of the general population in the western world and the majority of affected patients are alcoholic. The prevalence of wernicke’s encephalopathy lesions seen on autopsy was 12.5% of alcohol abusers in one report. Among those who with alcohol-related death, it has been reported to be even higher, 29 to 59%. The aim of this study was to report a case of wernicke’s encephalopathy following hyperemesis gravidarum.

Case Presentation: A 28-year-old-pregnant woman in 19th weeks of gestation referred to the hospital with hyperemesis, gait ataxia, and dysarthria before that she had hyperemesis gravidarum with weight loss and unresponsive to outpatient and inpatient medical therapy. MRI showed hyperdense lesion around thalamus which was characteristic of wernicke’s encephalopathy. Rapid improvement in patient’s condition occurred after high dose thiamine infusion.

Conclusion: In hyperemesis gravidarum, presence of either symptoms of ocular or mental disorder or ataxia must be considered to rule out and appropriate treatment of Wernicke’s syndrome which can cause maternal and fetal death.


Evaluation of the role of TLR4 in endotoxin-induced hepatic encephalopathy in rats with biliary cirrhosis
Razieh Mohammad Jafari , Farahnaz Jazaeri ,
Volume 78, Issue 2 (5-2020)
Abstract
Background: Hepatic encephalopathy is defined as a neuropsychiatric brain dysfunction in acute or chronic liver failure. Infection and inflammation have crucial role in its pathophysiology. The purpose of our study was to demonstrate the relationship between toll-like receptor 4 (TLR4) expression and the encephalopathy induced with endotoxin in biliary cirrhotic rats.
Methods: The present study was conducted experimentally on male adult Wistar albino rats from May to January 2018 at the Pharmacology Department of Medical Faculty, Tehran University of Medical Sciences, Tehran, Iran. The animals were divided into two groups, cirrhotic vs sham-operated. Cirrhosis was induced by surgical ligation of the bile duct in male Wistar rats but in sham group the bile duct was not ligated during abdominal surgery (control). The animals in each group were divided to two subgroups that half of them were given intraperitoneally saline or low dose of endotoxin (0.1 mg/kg) on day 29. Then animal behavior study was done on the clinical sign of hepatic encephalopathy. Also, the histopathology of brain cortex and the expression of brain cortex toll-like receptor four protein were evaluated.
Results: Our results showed that endotoxin treatment decreased the patient's level of alertness and produced hepatic encephalopathy signs in cirrhotic rats and control groups. Cirrhosis increases toll-like receptor four expression in brain and acute endotoxin treatment increases toll-like receptor four expression in this group still more. It means that acute endotoxin treatment-induced clinical signs of acute encephalopathy in sham and cirrhotic rats and significant toll-like receptor four overexpression in cirrhotic animals. The histopathological assessment did not show a significant difference between the groups and did not show any changes after induction of cirrhosis and/or acute endotoxin treatment.
Conclusion: Cirrhosis and acute low dose endotoxin injection in cirrhotic rats induced hepatic encephalopathy signs that parallels with significant increased toll-like receptor 4 protein expression in brain cortex. Since the severity of the signs of encephalopathy was the same as intact animals, based on the definitions in pharmacology, in the cells of cirrhotic groups endotoxin tolerance has developed.

Posterior reversible Leukoencephalopathy following thrombotic thrombocytopenic purpura at postpartum period: case report
Leila Pourali, Atiyeh Vatanchi, Negar Ramezanpour, Sedigheh Ayati, Farzaneh Ramezani,
Volume 78, Issue 11 (2-2021)
Abstract
Background: Posterior reversible leukoencephalopathy syndrome (PRLS) is a clinical and radiological syndrome of heterogeneous etiologies that are grouped because of the similar findings on neuroimaging studies which may occur at any age. The pathogenesis of RPLS remains unclear, but it appears to be related to disordered cerebral autoregulation and endothelial dysfunction. The syndrome is more commonly seen in women, RPLS has been described in several medical conditions, with hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressant drugs being the most common conditions. Prompt recognition and treatment are important in preventing the permanent damage that can occur in this otherwise typically reversible condition. The pathogenesis of RPLS remains unclear, but it appears to be related to disordered cerebral autoregulation and endothelial dysfunction. This report aimed to introduce a case of reversible posterior leukoencephalopathy following postpartum thrombotic thrombocytopenic purpura (TTP).
Case presentation: The patient was a 30-year-old primigravid woman at 33 weeks of pregnancy who was referred to the Ghaem hospital, Mashhad University of Medical Sciences in Mashhad in July 2017 due to high blood pressure, blurred vision, headache and generalized tonic colonic seizure. She did not report in previous history of high blood pressure or seizure before pregnancy. With a diagnosis of eclampsia remote from delivery, she underwent a cesarean section. After delivery, generalized tonic colonic seizure repeated several times and a significant reduction in consciousness level happened. Renal failure also occurred, so she underwent daily plasmapheresis with a diagnosis of TTP. After 35 days, she was discharged with a good general condition.
Conclusion: In predisposing conditions, such as high blood pressure in pregnancy, in the case of clinical suspicion of posterior leukoencephalopathy, any attempt for early diagnosis and appropriate treatment are important factors in reducing the rate of morbidity and mortality.

Evaluation of severity, duration and complications of jaundice in infants of both sexes at Ghaem Hospital in Mashhad from 2014 to 2021
Hassan Boskabadi , Mahdie Mir, Maryam Zakerihamidi,
Volume 80, Issue 6 (9-2022)
Abstract
Background: The gender of the baby is one of the risk factors for neonatal jaundice, but the difference in the severity of jaundice and its prognosis between the two sexes is not clear. Therefore, in this study, we investigated the severity, duration and prognosis of jaundice in both sexes.
Methods: The present study is a cross-sectional study that was performed on 2847 icteric neonates over 35 weeks admitted to Ghaem Hospital in Mashhad from May 2014 to May 2021. This study is done by available sampling. After confirmation of jaundice in infants, data were recorded using a researcher-made checklist including maternal demographic information (maternal age, mode of delivery), complete infant characteristics (age, birth weight, age at onset of jaundice, jaundice recovery age) and laboratory findings (Bilirubin, Indirect Coombs, direct Coombs, G6PD), and neonatal development up to two years of age was performed based on the Denver 2 test. Then data analysis was performed by SPSS software using Chi-square test and Student’s t-test.
Results: 1642 infants (57.7%) were boys and 1205 infants (42.3%) were girls. Mean and standard deviation of bilirubin in values less than 20 mg/dl in boys was 17.20±2.48 mg/dl and in girls was 16.54±2.80 mg/dl (P=0.000), birth weight was 3.16±0.49 (kg) for boys and 3.07±0.45 for girls (P=0.000). In two groups of male and female infants, age (P=0.004), direct bilirubin (P=0.001), direct and indirect Coombs (P=0.000), and G6PD enzyme deficiency (P=0.000) had a significant difference. Acute kernicterus was reported in 25 (2.03%) boys and 4 (0.46%) girls (P<0.001). In the two-year follow-up, 23 boys (1.9%) and 11 girls (1.28%) had developmental delay (P<0.05).
Conclusion: The incidence of jaundice in male infants was higher than female infants, which is probably due to a higher prevalence of G6PD deficiency in boys. The severity of jaundice was higher in boys less than 20 mg/dl. Jaundice has a worse prognosis in male infants.

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