Proton MR Spectroscopy in The Diagnostic Evaluation of Suspected Mitochondrial Disease in Iran

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Improving Cognitive Regulation in Patients with Bipolar Disorder Using Cyberspace-Based and Family-Centered Intervention

Introduction:Bipolar Affective Disorder is cyclic swinging of mood between mania or hypomania and depression. The present study aimed to examine cognitive regulation in patients with bipolar disorder using the cyberspace-based family-centered intervention.Method:This study was a quasi-experimental research with a pretest-posttest design and a follow-up period. The statistical population of the study included patients with bipolar disorder and a family member. 30 patients with a family member were selected by convenient sampling method and randomly assigned to experimental and control groups. Garnefski Cognitive Emotion Regulation Questionnaire (CERQ) was the research tool. After administration of the questionnaires, the Miklowitz family-focused therapy was performed for the experimental group using cyberspace. At the end of the course, participants in both groups were evaluated using the research tools. Data were analyzed using SPSS22 software and mixed analysis of variance with the repeated measures.Result:The results of analysis of variance with repeated measures showed that the Miklowitz model led to a significant difference in the score of emotion regulation with an effect size of 45% in the experimental and control groups (P<0.05). In other words, the cognitive emotion regulation and its subscales have been evident in the experimental group. Conclusion:As a result, it can be said that the use of the Miklowitz model through cyberspace affects cognitive emotion regulation and subscales of self-blame, acceptance, rumination, positive refocusing, refocusing on planning, positive reappraisal, putting into perspective, catastrophizing, and other-blame.Declaration of Interest: Non

Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Dysembryoplastic neuroepithelial tumor (DNT) considered as a benign cortical Glioneuronal neoplasm of children or young adults, typically present with drug-resistant focal epilepsy. DNTs are usually located in the temporal lobe but can found in any part of the supratentorial brain cortex. Multifocal DNTs have rarely reported. Here we present an eight years old boy with two years follow up, having a somewhat stable diffuse multinodular DNT of the most significant spatial extent that may have reported, involving cortical and subcortical left temporo-occipital lobe, bilateral basal ganglia and thalamus, presenting with headache, short stature, and behavioral disorder

Comparative evaluation between diameter difference of thumb and asymmetry of lateral cerebral ventricles in child with developmental delay; a new finding

How to Cite This Article: Keihanidoust Z, Shariat M, Rahimian E, Tehrani F, Saddighi Gh. Comparative Evaluation between Diameter Difference of the Thumb and Asymmetry of Lateral Cerebral Ventricles in Children with Developmental Delay: A  New Finding. Iran J Child Neurol. Summer 2015;9(3):9-12.Abstract Objective Anthropometry (measurement of body dimensions) has been used for clinical diagnosis of growth and developmental disorders during pregnancy and after birth. Different brain volumes have also been shown in abnormal developmental disorders. This study compares the different horizontal diameters of the left- and right-hand thumbnails and asymmetry of lateral cerebral ventricles in children with developmental delays. Materials & Methods This retrospective case control study was carried out in the Pediatric Neurologic Outpatient of a university hospital in Tehran, Iran (2009–2011). Twenty-eight patients with motor developmental disorders (case) and 28 healthy individuals (control) had brain MRIs and volume of lateral cerebral ventricles size had been studied. The maximum horizontal diameters of the left and right thumbnails were measured by calipers during physical and neurological exams by a pediatric neurologist. Finally, we compared and analyzed different horizontal diameters of the left and right hand thumbnails and asymmetry of lateral cerebral ventricles. Results There was a significant correlation between asymmetry of brain lateral ventricles size and mean difference of horizontal diameter of thumb nails (P = 0.0001). A meaningful relation between brain hemispheres asymmetry and developmental delay (P = 0.04) was seen. Conclusion The asymmetry of thumbnails can be a marker for asymmetry of lateral ventricles and child developmental delays

Long term video-EEG monitoring findings in children and adolescents with intractable epilepsy

Introduction:   In spite of appropriate management, seizures are not controlled in10 to 20 percent of epileptic children. If we can find the epileptogenic focus and resect it by surgery, patient may be cured from refractory seizures. Long Term Video-EEG Monitoring (LTM) may give us important information in the preoperative assessment of these patients. We performed this study for the first time in pediatric age group in Iran.Materials and Methods: In this cross-sectional study, 43 children between 4 to 18 years, with intractable epilepsy who were Referred to Shefa Neuroscience Research Center between 1386_1391, were enrolled to study in order to evaluate their long-term video EEG findings.Results: Forty-three children were enrolled to this study with mean age of 10.07 years, that 24(65.9%) were boys and 19(44.1%) were girls.Seven patients with definite epileptogenic zone were advised to perform lesionectomy surgery, in 2 patients there was not any seizure onset focus but corpus callosotomy was advised to control their frequent falling. Eight cases were recommended to perform Electrocorticography or invasive EEG monitoring. Twenty-six cases were recommended to adjust medical treatment. In 3 cases there was not any electrical seizure activity during clinical attacks, so discontinuing anti-epileptic drugs were recommended with diagnosis of conditions that mimic epilepsy.Conclusions:   It is necessary to perform LTM in patients with refractory epilepsy in order to determine their treatment strategy. If there is any doubt about pseudoseizure LTM can help to differentiate epilepsy from conditions that mimic epilepsy

The clinical features and diagnosis of Metachromatic leukodystrophy: A case series of Iranian Pediatric patients

How to Cite This Article: Jabbehdari S, Rahimian E, Jafari N, Sanii S, Khayatzadeh Kakhki S, Nejad Biglari H. The Clinical Features and Diagnosis of Metachromatic Leukodystrophy: A Case Series of Iranian Pediatric Patients. Iran J Child Neurol. Summer 2015;9(3):57-61.AbstractObjectiveMetachromatic leukodystrophy disorder (MLD) is one of the rare neurometabolicdiseases caused due to lack of saposin B and arylsulfatase A enzyme deficiency.Materials & MethodsEighteen patients diagnosed as metachromatic leukodystrophy in the NeurologyDepartment of Mofid Children’s Hospital in Tehran, Iran between 2010 and2014 were included in our study. The disorder was confirmed by clinical,EMG-NCV, arylsulfatase A enzyme checking and neuroimaging findings alongwith neurometabolic and genetic assessment from reference laboratory in Iran.We assessed age, gender, past medical history, developmental status, clinicalmanifestations, and neuroimaging findings of 18 patients with metachromaticleukodystrophy.ResultsFrom 18 patients, 80% were offspring from consanguineous marriages. A familyhistory of metachromatic leukodystrophy disease was positive for four patients.Twelve patients had late infantile form of this disorder and six patients had juvenile form. A history of tonic type seizure was positive in 20% of the patients and tonic spasm was confirmed with clinical information. Electromyographgraphy (EMG) in 96% of patients was abnormal with demyelinating sensorimotor neuropathy pattern. MRI in all patients showed the leukodystrophic pattern as arcuate fibers sparing and subcortical rim in white matter and periventricular involvement. Our diagnosis was confirmed by EMG-NCV findings with sensorimotor neuropathy pattern and the assessment of arylsulfatase A enzyme function. ConclusionMLD is an inheritance metabolic disorder, which was confirmed by theassessment of arylsulfatase A enzyme function, peripheral blood leukocyte thatassessed in a referral laboratory in Iran

Neuroimaging Findings of the High-risk Neonates and Infants Referred to Mofid Children’s Hospital

Abstract Objectives Neuroimaging in high-risk neonates and infants is done to help child neurologists predict the future neurodevelopmental outcome of these children. In this study, we assessed high-risk neonates and infants admitted to the NICU or neonatal wards of Mofid children’s Hospital, especially regarding clinical development and brain imaging Materials & Methods This cross-sectional study was conducted on 170 patients admitted to the neonatal and NICU ward of Mofid children’s Hospital.Considering the inclusion criteria, 112 patients were included in this project. Brain ultrasonography was performed on almost all of these babies by a single radiologist. Some patients underwent a brain CT scan, and brain MRI without contrast was done on the others. These images were interpreted and compared by a single pediatric neuroradiologist blinded to clinical data. All of these babies were followed up until 18 months of age. Results In this study, 57.1% of the patients were male and 42.9% were female. Of 44 patients who obtained Electroencephalogram (EEG) during the hospitalization period with probable seizure, 25 (56.8%) had normal EEGs. Of 89 babies who were examined by ultrasound, 19 (21.3%) had abnormal findings; ventriculomegaly and then germinal matrix hemorrhage (GMH) were the most common abnormalities.Also, 27 cases (71.1%) of 38 patients undergoing a CT scan had abnormal findings. The most common findings were a hypodense area in the white matter and ventriculomegaly. Of 41 patients who underwent MRI between 1 and 27 months, 34 cases (82.9%) had an abnormal MRI. The most common findings were periventricular hyperintensities in 17 cases (41.5%), mildly delayed myelination in 15 cases (36.6%), and severe brain atrophy or thinning of corpus callosum or white matter volume loss in seven cases (17.1%). During the follow-up period, which was 18.55 ± 6.56 months, 79 (70.5%) of the children had normal development and 33 (29.5%) were suffering from a global neurodevelopmental delay. More precisely, 49 (43.7%) and 35 (31.2%) patients had motor development delay and delayed verbal development, respectively. The abnormal findings of brain imaging in the ultrasound, CT scan, and MRI were all significantly associated with an adverse neurodevelopmental outcome (P <0.001, P = 0.02, and P <0.001, respectively). ConclusionIn this study, we showed that at any time before six months or afterone year of age, the result of brain MRI was a strong predictor of thepatient’s outcome

Volumetric Assessment of Extratemporal Structures in Patients With Temporal Lobe Epilepsy

Background: We assessed the presence of brain volume loss in the extratemporal structures in patients with temporal lobe epilepsy (TLE). The associations between brain volume loss in these structures and epilepsy duration, magnetic resonance imaging (MRI) findings, and occurrence of focal to bilateral tonic-clonic seizures (TCS) were assessed. Methods: In this cross-sectional study, all adult patients with drug-resistant TLE, who were admitted to the epilepsy monitoring unit at Loghman-Hakim Hospital, Tehran, Iran, during 2016-2020, were included. For all the participants, brain MRI was performed and patients with TLE were divided into two subgroups of those with hippocampal sclerosis (TLE-HS) and patients with normal-appearing brain MRI findings (TLE-no). Independent sample t test was applied to compare quantitative variables in the study groups. Pearson correlation test examined the correlation between the clinical and volumetric features. Results: 203 participants (81 patients with TLE and 122 healthy controls) were studied. Compared with healthy controls, patients with TLE showed a decrease in their midbrain (P = 0.02) and thalamus (P = 0.01) volume. The degree of thalamic atrophy was more significant in TLE-HS (P = 0.03). Moreover, the degree of midbrain volume loss was more significant (P = 0.07) in patients who had TCS in the past two years (N = 31) compared with those who did not (N = 50). The volume of the thalamus (r: -0.252, P = 0.02) and pallidum (r: -0.255, P = 0.02) had inverse correlations with the epilepsy duration. Conclusion: Patients with TLE have lower midbrain and thalamus volume compared with the healthy controls, which may be attributed to the seizure-induced injury. Midbrain atrophy may theoretically increase the risk of sudden unexpected death in epilepsy (SUDEP) because of the enhanced autonomic dysfunction

Health-related Quality of Life Assessment in People with M.S City of Iranshahr using the Second Version of the Questionnaire Sf- 36

One of the diseases this century. Unfortunately most young people, especially women to become infected patients with multiple sclerosis (M. S) is a world specialist physicians still cause is unknown. Loss or inability to work, in people with this disease the huge economic burden to society. Today, self-assessed health status using a standard set of epidemiological research-based society. Given the importance of quality of life relative improvement of life study, Reporting and Assessment of quality of life using the second version of the questionnaire 36sf in the life of these patients. This cross - sectional study that randomly among 70 patients with multiple sclerosis. in Iranshahr  area of Iran a questionnaire on items Physical function, physical problems, emotional problems, vitality, mental health, social functioning, bodily pain, general health was conducted. The obtained data were anlyazed by using statistical software spss 16, excel 2010. The findings showed that the mean scores of physical components (pcs) 5.7857 and total score that each component in M.S patients (67/0 = r, 01/0 = p) is positive and significant relationship. The results showed the importance of quality of life and social protection, which can be life satisfaction and hope in patients with multiple sclerosis . However, though the quality of life in various forms including financial, cultural, educational and supportive services can have on the well-being of patients with M.S contribute, However, due to the lack of definitive therapy for the treatment of this disease so overall quality of life can also have a significant relationship with partial recovery of patients. Design and implementation of intervention programs to enhance people's understanding of MS, Ways to prevent it and promoting protective behaviors is essential at the community level.

The Clinical Features and Diagnosis of Canavan’s Disease: A Case Series of Iranian Patients

How to Cite This Article: Karimzadeh P, Jafari N, Nejad Biglari H, Rahimian E, Ahmadabadi F, Nemati H, Nasehi MM, Ghofrani M, Mollamohammadi M. The Clinical Features and Diagnosis of Canavan’s Disease: A Case Series of Iranian Patients. Iran J Child Neurol. 2014 Autumn;8(3): 66-71.AbstractObjectiveCanavan’s disease is a lethal illness caused by a single gene mutation that is inherited as an autosomal recessive pattern. It has many different clinical features especially in the non-Ashkenazi Jewish population.Material & Methods45 patients were referred to the Pediatric Neurology Department of Mofid Children’s Hospital in Tehran-Iran from 2010–2014 with a chief complaint of neuro developmental delays, seizures, and neuroimaging findings of leukodystrophy were included in this study. Magnetic Resonance Spectrometry (MRS) and neuro metabolic assessment from a referral laboratory in Germany confirmed that 17 patients had Canavan’s disease.ResultsVisual impairment, seizure, hypotonia, neuro developmental arrest, and macrocephaly were the most consistent findings in the patients in this study. Assessments of neuro developmental status revealed that 13 (76%) patients had neuro developmental delays and 4 (24%) patients had normal neuro development until 18 months of age and then their neuro developmental milestones regressed.  In this study, 100% of cases had macrocephalia and 76% of these patients had visual impairment. A history of seizures was positive in 8 (47%) patients and began around 3 months of age with the most common type of seizure was tonic spasm. EEGs were abnormal in all epileptic patients. In ten of the infantile group, we did not detect elevated level of N-acetylaspartic acid (NAA) in serum and urine. However, the MRS showed typical findings for Canavan’s disease (peaks of N-acetylaspartic acid).ConclusionWe suggest using MRS to detect N-acetylaspartic acid as an acceptable method for the diagnosis of Canavan’s disease in infants even with normal serum and urine N-acetylaspartic acid levels. ReferencesAdornato BT, O’Brien JS, Lampert PW, Roe TF, Neustein HB. Cerebral spongy degeneration of infancy. A biochemical and ultrastructural study of affected twins. Neurology 1972;22(2):202-10.Banker BQ, Robertson JT, Victor M. Spongy Degeneration of the Central Nervous System in Infancy. Neurology 1964; 14:981-1001.Chou SM, Waisman HA. Spongy Degeneration of the Central Nervous System: Case of Homocystinuria. Arch Pathol 1965; 79:357-63.Divry P, Vianey-Liaud C, Gay C, Macabeo V, Rapin F, Echenne B. N-acetylaspartic aciduria: report of three new cases in children with a neurological syndrome associating macrocephaly and leukodystrophy. 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