Iranian Journal of Child Neurology
Year:2017 | Volume:11 | Issue:2|Papers Count:13

Moyamoya is a rare chronic progressive occlusive cerebrovascular disease. Itsmanifestation varies from stroke, progressive learning impairment and transientischemic attack to headache and seizure. There is no accepted medical treatmentand surgery usually, is needed. We report here a case of 8 yr old boy referredto psychiatrist outpatient. An eight yr old boy with intermittent hemiplegiawas brought to Imam Ali Clinic, Yazd, Iran in 2015 because his headache andmedical problem began from 6 yr old. Stress and excitement exacerbated hiscondition. His first attack was at the age of 6 yr old. During attack, he hadincontinence, severe headache, alogia, pallor, claudication and left hemiplegia(Left lower limb). Magnetic resonance angiography (MRA) was done and ourdiagnosis was moyamoya disease. Moyamoya is a mysterious disease andpsychiatrists should consider it in differential diagnosis of alogia and plegia.Acute management of this disease is mainly symptomatic. Nowadays, surgeryis a good choice and early diagnosis of this disease can change our patient’slife.

One of the rare complications of brucellosis is neurobrucellosis. There havebeen numerous reports showing clinical forms of brucellosis affecting CNS, such as cranial nerve involvement, myelitis, vascular disease, radiculoneuritis, meningitis, meningoencephalitis, and demyelinating disease. In this casereport, we introduce a 2.5 yr old girl with unilateral abducens nerve palsyreferred to Baghiyatallah Hospital Outpatient Clinic, Tehran, Iran in June 2015.

Objective Herpes Simplex virus (HSV) is one of the most common sexually transmitted diseases in the world. This study aimed to determine the prevalence of herpes simplex virus in pregnant women in Iran.Materials & Methods A systematic literature review was conducted to study the HSV subtypes in Persian and English papers through several databases. We searched Pub Med, Scopus, Ovid, Science Direct and national databases as Magiran, Iranmedex and Science Information Database (SID) up to October 2015. Random-effects model were applied to calculate the pooled prevalence of HSV subtypes.Results Five eligible studies were identified, including 1140 participants. The pooled prevalence of HSV infection in pregnant women was 0.64% (95% CI: 0.10- 1.18) in Iran. The pooled prevalence of studies on both HSV-1 and HSV-2 was 0.91% (CI: 0.81-1.02) and studies on only HSV-2 was 0.23% (CI: -0.61-0.63), respectively.Conclusion The prevalence of HSV infection in pregnant women in Iran was higher. HSV infection of the central nervous system, especially with HSV-2, can also cause recurrent aseptic meningitis and monophasic, as well as radiuculitis or myelitis.The performance of screening to detect infection in pregnant women can play an important role in the prevention and treatment of patients and help to prevent the transmission of HSV infection to infants in Iran.

ObjectiveInfantile spasms is diagnosed late even by expert pediatricians. Late diagnosis(later than 3 weeks) can have a negative effect on the long-term prognosis. Weaimed to investigate infantile spasms treated with intravenous methylprednisolonepulse.Materials & MethodsIn this case series study, 20 infants with infantile spasms in 17-Shahrivar Hospital, Rasht, Iran were enrolled. Drugs were administered based on Mytinger protocolthat included 3 days of methylprednisolone pulse and 56 days of oral prednisolone.The control of spasms and the omission of hypsarrhythmia in infants followupwere the primary and secondary outcomes, respectively. Remission wasindicated if the caregivers mentioned no spasms or>50% decrease regardingdrug initiation for at least 5 consecutive days and the electroencephalographyduring sleep period noted the omission of hypsarrhythmia.ResultsEleven female (55%) and 9 male (45%) patients with the mean age of 4.95±1.39months were enrolled. Mean rapid remission was noted as 4.41±1.50 days.Twelve patients (60%) noted early remission. seizure was controlled in 3 (15%)patients completely after 24 months. Five (25%) occasional seizures were notedcontrolled by routine anticonvulsant drugs after 24 months and 12 (60%) noresponse was mentioned. Most of the patients (65%) had cryptogenic etiologyfor infantile spasms. Uncontrolled seizure was mentioned after initial remission.ConclusionMethyl prednisolone is an appropriate drug based on easy administering, lowcost, and its accessibility.

ObjectiveThere have been contradictory findings on the relationship betweenSocioeconomic Status (SES) and child development although SES is associatedwith child development outcomes. The present study intended to define therelationship between SES and child development in Tehran kindergartens, Iran.Materials & MethodsThis cross-sectional survey studied 1036 children aged 36-60 month, indifferent kindergartens in Tehran City, Iran, in 2014-2015.The principal factor analysis (PFA) model was employed to construct SESindices. The constructed SES variable was employed as an independentvariable in logistic regression model to evaluate its role in developmentaldelay as a dependent variable.ResultsThe relationship between SES and developmental delay was significant atP=0.003. SES proved to have a significant (P<0.05) impact on developmentaldelay, both as an independent variable and after controlling risk factors.ConclusionThere should be more emphasis on developmental monitoring and appropriateintervention programs for children to give them higher chance of having a moreproductive life.

ObjectiveAnticonvulsant drugs can cause various forms of skin drug reactions, rangingfrom exanthema to severe blistering reactions. An association betweenHLA-B*1502 allele and severe skin reactions have been reported.Materials & MethodsFifteen patients with severe skin reactions following treatment withanticonvulsant drugs (Carbamazepine, lamotrigine, phenobarbital, primidone)and 15 controls (age-matched epileptic patients taking similar anticonvulsantswithout drug eruption) were included. They were referred to Mofid Children’sHospital in Tehran, Iran, between Jan 2012 to Jan 2014. Genomic DNA wasextracted from peripheral blood of all patients and HLA- B*1502 genotype wasdetected by real-time PCR.ResultsNone of the patients was positive for HLA- B*1502, but two patients in controlgroup had positive HLA- B*1502.ConclusionThe HLA- B*1502 is not correlated with severe anticonvulsant drugs -inducedskin reactions in Iranian children.

ObjectiveIntractable epilepsy is a serious neurologic problem with different etiologies.Decreased levels of pyridoxal phosphate in cerebral spinal fluid of patientswith intractable epilepsy due to pyridoxine dependency epilepsy are reported.The aim of this study was to compare plasma pyridoxal 5’-phosphate level inpatients with intractable and controlled epilepsy.Materials & MethodsThis cross- sectional analytic study included 66 epileptic children, 33 patientswith controlled and 33 patients with intractable epilepsy, after neonatal periodup to 15 yr old of age. Thirty-three patients with intractable epilepsy (10-162 months) and 33 patients with controlled epilepsy (14-173 months) wereenrolled. The study was conducted in Pediatric Neurology Clinic of MofidChildren Hospital, Tehran, Iran from January 2010 to December 2010.Patients’ clinical manifestations, laboratory and neuroimaging findings werecollected. Non-fasting plasma 5’- pyridoxal phosphate levels of these subjectswere assessed by high-pressure liquid chromatography.ResultsMean plasma 5’- pyridoxal phosphate level (PLP) in patients with controlledepilepsy was 76.78±37.24 (nmol/l) (15.5-232.4). In patients with intractableepilepsy, mean plasma 5´- pyridoxal phosphate was 98.67 ± 80.58 (25.5-393) nmol/l. There was no statistically significant difference between plasmapyridoxal phosphate levels of these two groups (P═0.430).ConclusionPyridoxine dependent epilepsy is under diagnosed because it is manifestedby various types of seizures. Plasma pyridoxal phosphate levels did not differin our patients with intractable or controlled epilepsy. If PDE is suspectedon clinical basis, molecular investigation of ALDH7A1 mutations, as feasibletest, until PDE biomarkers becomes available is recommended.

ObjectiveOverall, 2%-5% of patients with multiple sclerosis (MS) experiencedthe first episode of disease before the age 18 years old. Since the age ofonset among children is not similar to that in general population, cliniciansoften fail to early diagnose the disease. This study aimed to determine theepidemiological and clinical patterns of MS among Iranian children.Materials & MethodsIn this cross-sectional study carried out in Iran in 2014-2015, informationwas collected using a checklist with approved reliability and validity. Methodsampling was consensus. Data were analyzed using frequency, mean andstandard deviation indices by means of SPSS ver.20 software.ResultsTotally, 177 MS children were investigated.75.7% of them were female.Mean (SD), minimum and maximum age of subjects were 15.9 (2), 7 and 18yr, respectively. The most reported symptoms were sensory (28.2%), motor(29.4%), diplopia (20.3%) and visual (32.8%). Primary MRI results showed91.5% and 53.1% periventricular and spinal cord lesions, respectively.ConclusionMS is significantly more common among women. The most common age ofonset is during the second decades. Sensory and motor problems are the mostsymptoms, while, periventricular and spinal cord lesions are the most MRIresults.

ObjectiveThe aim of our study was to find the relationship of MPV (Mean PlateletVolume) levels and platelet counts as markers of inflammation between simpleand complex febrile seizures.Materials & MethodsIn this retrospective comparative study, we investigated the recordingsof 356 children between 5 months and 6 yr with diagnosis of simple andcomplex febrile seizure (SFS& CFS) in Amircola’s Children’s Hospital, BabolUniversity of Medical Sciences, Babol, Iran between Mar 2011 and Dec 2015.ResultsMean age was similar in two groups. The MPV of the CFS group (8.32±0.48fl)was lower than that of the SFS group (8.58±0.34fl) but this difference wasnot significant statistically. The platelet count of the CFS group (315.03×103±117.17×103) was higher than that of SFS group (291.82×103 ± 87.49×103) butthere was no significant statistical difference.ConclusionWe did not find significant differences between two groups. Therefore, furtherstudies about this idea should be performed.

Objective Epilepsy is a chronic neurological disorder requiring long-term therapy using antiepileptic medications. Reports have incriminated long-term antiepileptic drugs use in deficiency of vitamin D and bone diseases in all age groups. We aimed to investigate the association between serum 25-hydroxyvitamin D levels and pediatric epilepsy in Indian patients.Materials & Methods We prospectively recruited 100 pediatric epilepsy patients, on monotherapy for minimum one-year duration, and 50 age and sex matched controls. This study was carried out at Yashoda Hospital, India from 2011-2014. All cases and controls underwent tests for serum 25-hydroxyvitamin D, alkaline phosphatase, serum calcium and phosphorus levels.Results Patients with 25-hydroxyvitamin D deficiency were significantly higher among cases (45%) than controls (24%). Mean alkaline phosphatase was significantly higher in cases and mean serum calcium was significantly lower (8.3±1.5) in cases. Amongst antiepileptic drugs, carbamazepine and sodium valproate were significantly associated with 25-hydroxyvitamin D deficiency. Risk of vitamin D deficiency was highest with sodium valproate usage (odds: 4.0; 95%CI 1.4-11.6) followed by carbamazepine use (odds: 2.7; 95%CI 1.0-6.8). After adjustment using multiple logistic regression, antiepileptic drugs showed independent association with 25-hydroxyvitamin D deficiency (odds: 2.2; 95%CI 0.9-4.5).Conclusion 25-hydroxyvitamin D deficiency was significantly associated with use of carbamazepine and sodium valproate in pediatric epilepsy.

ObjectiveEthylmalonic encephalopathy (EE) is a severe mitochondrial disease ofearly infancy clinically characterized by a combination of developmentaldelay, progressive pyramidal signs, and vascular lesions including petechialpurpura, orthostatic acrocyanosis, and chronic hemorrhagic diarrhea.Biochemical hallmarks of the disease are persistently high level of lactate, and C4–C5-acylcarnitines in blood, markedly elevated urinary excretion ofmethylsuccinic and ethylmalonic (EMA) acids. Here we report two patientswith EE as a 16-months-old male infant and a 2-yr-old boy referred toPediatric Neurology Clinic in Children’s Medical Center, Tehran-Iran that inone patient genetic analysis revealed a homozygous mutation of the ETHE1gene in favor of ethylmalonic academia.

A tumefactive lesion of central nervous system (CNS) is defined as a mass-like lesion with a size greater than 2 cm in brain detected by magnetic resonance imaging (MRI). Neuroimaging may help to distinguish the nature of a tumefactive lesion and therefore, can prevent an unnecessary brain biopsy. Here we emphasized on determining the nature of a CNS tumefactive lesions with the help of MRI and more explanations about demyelinating lesions with focus on Schilder and Balo diseases as two multiple sclerosis variants. We have reported here two boys of 10 and 8 years of age respectively of multiple sclerosis (MS) variants who presented with acute neurologic complications to our hospital as one of the two referral children hospital in Tehran, Iran. Tumefactive demyelinating lesions can be considered a separate entity that itself can contain Schilder disease, Balo disease, some cases of acute disseminated encephalomyelitis (ADEM) or classic MS. MRI can help to establish a diagnosis of a tumefactive lesion and to differentiate among different underlying etiologies.

Myelodysplasia includes a group of developmental anomalies resulting fromdefects that occur during neural tube closure. Urological morbidity in patientswith myelodysplasia is significant and if not treated appropriately in a timelymanner can potentially lead to progressive renal failure, requiring dialysis ortransplantation. We report the case of a 13-year old girl with neurogenic bladderwho presented chronic renal failure secondary to lipomyelomeningocele withretethering of cord. She was managed with urinary indwelling catheterizationuntil optimization of renal function and then underwent detethering of cord withexcision and repair of residual lipomeningomyelocele. Her renal parametersimproved gradually over weeks and then were managed on self clean intermittentcatheterization. The case emphasizes the need for considering retethering ofspinal cord in children with myelodysplasia where symptoms of neurogenicbladder and recurrent urinary tract infections occur.