Guillain-Barre Syndrome Presenting With Bilateral Facial Nerve Palsy

How to Cite This Article: Inaloo S, Katibeh P. Guillain-Barre Syndrome Presenting With Bilateral Facial Nerve Palsy. Iran J Child Neurol. 2014 Winter;8(1):69-71.ObjectiveThis case study is about an 11-year-old girl with bilateral facial weakness, abnormal taste sensation, and deep tendon reflexes of both knees and ankles were absent. However, the muscle power of the lower and upper extremities across all muscle groups was normal. After 2 days, she developed paresthesia and numbness in the lower extremities. Other neurologic examinations, such as fundoscopic evaluation of the retina were normal with the muscle power of both upper- and lower-extremities intact. A lumbar puncture revealed albumincytological dissociation. EMG and NCV were in favor of Guillain-Barre syndrome, for which IVIG was prescribed and the abnormal sensations in the lower limbs rapidly improved. Bilateral facial diplegia without weakness and paresthesia is a variant of Guillain-Barre syndrome that mostly presents withacute onset, rapid progression with or without limb weakness, paresthesia, and decreased or absent DTR and albumin-cytological dissociation.References:Barbi F, Ariatti A, Funakoshi K, Meacci M, Odaka M, Galassi G. Parvovirus B19 infection antedating Guillain-Barre’ syndrome variant with prominent facial diplegia. J Neurol 2011 Aug; 258(8):1551-2. doi: 10.1007/s00415-011-5949-5. Epub 2011 Feb 15.Yardimci N, Avci AY, Kayhan E, Benli S. Bilateral facial nerve enhancement demonstrated by magnetic resonance imaging in Guillain-Barré syndrome. Neurol Sci 2009 Oct; 30(5):431-3. doi:10.1007/s10072-009-0120-0.Lim TC, Yeo WS, Loke KY, Quek SC. Bilateral facial nerve palsy in Kawasaki disease. Ann Acad Med Singapore 2009; 38(8):737-8.Quintas E, Silva A, Sarmento A. Bilateral facial palsy in a young patient after meningococcal meningitis, associated to herpetic infection. Arq Neuro-Psiquiatr 2009; 67(3a): 712-14.Jain V, Deshmukh A, Gollomp S. Bilateral facial paralysis: case presentation and discussion of differential diagnosis. J Gen Intern Med 2006; 21(7):C7-10.Kamaratos A, Kokkoris S, Protopsaltis J, Agorgianitis D, Koumpoulis H, Lentzas J et al. Simultaneous Bilateral Facial Palsy in a Diabetic Patient. Diabetes Care 2004; 27 (2): 623-24.Magliocca KR, Leung EM, Desmond JS. Parotid swelling and facial nerve palsy: an uncommon presentation of sarcoidosis. Gen Dent 2009; 57(2):180-2.Atsumi M, Kitaguchi M, Nishikawa S, Susuki K. A variant of Guillain-Barré syndrome with prominent bilateral peripheral facial nerve palsy-facial diplegia and paresthesias. Rinsho Shinkeigaku 2004 Aug; 44(8):549-52.Narayanan RP, James N, Ramachandran K, Jaramillo MJ. Guillain-Barré Syndrome presenting with bilateral facial nerve paralysis: a case report. Cases J 2008 Dec 8;1(1):379.Azarisman SMS, Shahrin TCA, Marzuki AO, Fatnoon NNA, Rathor MY. Bilateral facial nerve palsy secondary to an atypical presentation of Guillain-Barré syndrome. IMJ 2009; 8(1):41-4.Sethi NK, Torgovnick J, Arsura E, Johnston A, Buescher E. Facial diplegia with hyperreflexia--a mild Guillain-Barre Syndrome variant, to treat or not to treat? J Brachial Plex Peripher Nerve Inj 2007; 10(2):9.Burina A, Sinanović O, Smajlović D, Vidovic. Bilateral Oculomotor Nerve Palsy in Guillain-barre Syndrome. Med Arh 2008; 62 (2):119-120.Verma R, Chaudhari TS, Giri P. Unilateral facial palsy in Guillain-Barre syndrome (GBS): a rare occurrence. BMJ Case Rep 2012 Oct 19; 2012. pii: bcr2012007077. doi: 10.1136/bcr-2012-007077.Susuki K, Koga M, Hirata K, Isogai E, Yuki N.A Guillain-Barré syndrome variant with prominent facial diplegia. J Neurol 2009; 256(11): 1899-1905

An epidemiologic study of 389 children with epilepsy in southern Iran

How to Cite this Article: Inaloo S, Katibeh P. An epidemiologic study of 389 children with epilepsy in southern Iran.Iranian Journal of Child Neurology2011;5(4):15-20Objective Approximately 4% of the world's population experience one or more febrile seizures during their lifetime, and 0.5-1% of the population has active epilepsy.Less than one-third of the reported seizures are categorized as epilepsy. The cause of established epilepsy is important in determining the treatment and prognosis.Materials & Methods We studied 389 cases of documented epilepsy in children aged 2 months to 18 years who visited the hospital for neurologic examination during 2005-2010.Chi-square test or Fisher's exact test was performed for categorical variables.Results The most common age for the first seizure was below 2 years, and the most common type of epilepsy was generalized tonic-clonic seizure. Electroencephalography (EEG) showed an epileptic pattern in 60%, 29.8%, and 51% of the patients with idiopathic, symptomatic, and cryptogenic epilepsy, respectively. This pattern was significantly different among these 3 categories of epilepsy.Conclusion The most common type of seizure was cryptogenic; however, in most industrialized countries, idiopathic epilepsies were more frequent. With respect to the age and sex of patients, the prevalence of epilepsy in southern Iran is not so much different from that of patients in other parts of the world. As to generalized or partial epilepsy, there are different reports from different part ofthe world; however, generalized tonic-clonic seizures were more common in our area.References Hauser, Hesdorffer DC. Epilepsy, frequency, causes and consequences. New WA York, NY: Demos Publications;1990.P.1–51.Jallon P. Epilepsy in developing countries. Epilepsia 1997; 38: 1143-51.King M, Newton M, Jackson G, Fitt G, Mitchell L, Silvapulle M, Berkovic S. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. The Lancet 1998; 352: 1007-1011.Sridharan S. Epidemiology of epilepsy. Current Science 2002;82:664-70Martin JB, Jacqueline AF. Management of epilepsy in adolescents and adults. Lanset 2000; 356: 323-29.Chang BS, Lowenstein DH. Epilepsy. N Engl J Med 2003; 349: 1257-66.Guerrini R. Epilepsy in children. Lancet 2006; 367:499- 524.Mohammadi MR, Ghanizadeh A, Davidian H, Mohammadi M, Norouzian M. Prevalence of epilepsy and comorbidity of psychiatric disorders in Iran. Seizure 2006;15(7):476-82.Luengo A, Parra J, Colas J, Ramos F, Carreras T, Fernández-Pozos MJ. Prevalence of Epilepsy in Northeast Madrid. J Neurol 2001; 248: 762-767.Sridharan R. Epidemiology of epilepsy.Curr Sci 2002; 82(6):664-70.Najib Kh, Fallahzadeh E, Fallahzadeh MH. Disease spectrum and mortality in hospitalized children of southern Iran. Iran J Pediatr 2007; 17(3):359-363.Commission on Classification and Terminology of the International League against Epilepsy. Proposal for revised clinical and electrographic classification of epileptic seizures. Epilepsia 1981; 22: 489–501.Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30: 389–99.Kramer U, Nevo Y, Neufeld MY, Fatal A, Leitner Y, Harel S. Epidemiology of epilepsy in childhood: A cohort of 440 consecutive patients. Pediat Neurol 1998; 18 :46-5.Olafsson E, Ludvigsson P, Gudmundsson G, Hesdorffer D, Kjartansson O, Hauser WA. Incidence of unprovoked seizures and epilepsy in Iceland and assessment of the epilepsy syndrome classification: a prospective study. Lancet Neurol 2005;4:627-34.Hauser, W. A. The Prevalence and Incidence of Convulsive Disorders in Children. Epilepsia 1994; 35: S1-S6.Kochen S, Melcon MO. Prognosis of epilepsy in a community based study: 8 years of follow up in an Argentine community. Acta Neurologica Scandinavica 2005; 112: 370-374.Hauser WA, Kurland LT. epidemiology of epilepsy in Rochester, Minnesota 1936 through 1967.Epilepsia 1983;24:502-14. Preux PM, Druet-Cabanac M. Epidemiology and etiology of epilepsy in sub-Saharan Africa. The Lancet Neurol 2005; 4: 21-31.Olafsson E, Hauser W A, Ludvigsson P , Gudmundsson G. Incidence of Epilepsy in Rural Iceland: A Population- Based Study. Epilepsia 1996; 37: 951–955.Joensen P. Prevalence, incidence, and classification of epilepsy in the Faroes. Acta Neurologica Scandinavica1986;74:150–155.Granieri E, Rosati G, Tola R, Pavoni M, Paolino E, Pinna L, Monetti V C. A Descriptive Study of Epilepsy in the District of Copparo, Italy, 1964-1978. Epilepsia 1983; 24:502–514.Nash TE, Del Brutto, Butman JA Corona T, Delgado- Escueta A, Duron RM ,et al. Calcific neurocysticercosis and epileptogenesis. Neurology 2004; 62: 1934-38. Sillanpaa M, Jalava M, Kaleva O, Shinnar S. Long-term prognosis of seizures with onset in childhood. N Engl J Med 1998; 338:1715-22.O’Dell C, Shinnar S. Initiation and discontinuation of antiepileptic drugs. Neurol Clin 2001 ;19(2):289-311.Medina MT, Durón RM, Martínez L, Osorio JR, Estrada AL, Zúniga C, et al. Prevalence, incidence, and etiology of epilepsies in rural Honduras: the SalamáStudy. Epilepsia 2005 Jan;46(1):124-31.Sander JWAS. Some aspects of prognosis in the epilepsies:a review. Epilepsia1993;34:1007-16.Berg AT, Shinnar S, Levy SR, Testa FM, Smith-Rapaport S, Beckerman B. Defining early seizure outcomes inpediatric epilepsy: the good, the bad and the in-between.Epilepsy Res 2001 Jan;43(1):75-84. King M, Newton M, Jackson G, Fitt G, Mitchell L, Silvapulle M, et al. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. The Lancet 1998; 352: 1007-1011.Duncan JS. Imaging and Epilepsy. Brain 1997; 120: 339- 77.Lhatoo S D,Sander JWAS. The Epidemiology of Epilepsy and Learning Disability. Epilepsia 2001; 42: 6–9.Aicardi J. Epileptic syndromes in childhood. Epilepsia 1988; 29(suppl 3):551 - 5.Stafstrom CE, Patxot CE, Gilmore HE, et al. Seizures in children with Down’s syndrome: etiology, characteristics and outcome. Dev Med Child Neurol 1991; 33:191 – 200

Cerebral Palsy in 1-12 Year Old Children in Southern Iran

How to Cite This Article: Inaloo S, Katibeh P, Ghasemof M. Cerebral Palsy in 1-12 Year Old Children in Southern Iran. Iran J Child Neurol. Winter 2016; 10(1):35-41.AbstractObjectiveCerebral palsy (CP) is a non-progressive CNS disorder due to an insult to the growing brain, usually occurring in the first two years of life. During the recent years, its etiology has been changed; perinatal and postnatal insults are not considered as its main causes in developed countries any more. The aim of this study was to evaluate the causes of CP in children in southern Iran.Materials & MethodsOverall, 200 children with CP aged 1-12 yr old referring to Pediatric Neurology Clinic affiliated to Shiraz University of Medical Sciences, Shiraz, Iran between 2012 and 2013 were enrolled. In addition, 200 healthy age and sex-matched children were considered as the control group. Exclusion criteria were isolated movement disorders with no other evidence of CP, progressive neurologic disorders, metabolic disorders, and incomplete or uncertain past history. After collecting the data on pregnancy period, prenatal history and past medical problems, they were analyzed with appropriate statistical methods.ResultsMaternal age, medical problems during pregnancy period, route of delivery, head circumference at birth, neonatal admission, neonatal jaundice, and prematurity were the main risk factors for CP.DiscussionThe distribution of risk factors of CP is different from that of developed countries in our region. Pre- and peri-natal etiologies are still among the common causes of CP in Iran

Evaluation of the quality of life in epileptic children of Shiraz, Southern Iran

Introduction: People  suffer chronic disease like epilepsy are highly prone to debilitating changes in factors that affect the quality of life such as physical capacity, self-esteem, relationships with others and fulfillment of their daily life activities.  In this study, we decided to evaluate the quality of life in children with epilepsy in Shiraz, South Iran. Methods: Epileptic patients  referred to epilepsy clinic of Shiraz University of Medical Sciences and had no first time episode of seizures in the previous 6 months and no febrile-seizure were included in the study. Patients were evaluated using the standard KIDSCREEN-27 questionnaire. Data were analyzed using the statistical software SPSS 21, Man Whitney and Chi-square tests and reported in terms of descriptive statistics. The  significance level was considered less than 0.05. Results: In this case-control study, 229 children with epilepsy were compared with a control group of 400 normal individuals. The mean age  was 12.44±3.16 and 12.10±2.69 years. The tonic-clonic seizure had the highest prevalence . Being a boy, older age and having more seizures per year were associated with lower quality of life; in general, epileptic children had significantly lower QOL compared to normal cases. Conclusion: In general, epileptic children had an overall lower QOL while factors such as older age, male gender, and higher number of seizures over the years reduced the quality of life of these patients. 

A Novel Mutation in Aspartoacylase Gene; Canavan Disease

How to Cite This Article: Ashrafi MR, Tavasoli AR, Katibeh P, Aryani O, Vafaee-Shahi M. A Novel Mutation In Aspartoacylase Gene; Canavan Disease. Iran J Child Neurol. Autumn 2015; 9(4): 54-57.AbstractObjectiveCanavan disease (CD) is a type of vacuolating leukodystrophy with autosomal recessive inheritance. Aspartoacylase deficiency results in decrease of myelin biosynthesis, dysmyelination and brain edema. Although CD is a very common in Ashkenazi Jews patients, several cases have been reported from non- Jewish population. This report is based on a homozygous C.202G>A mutation in the ASPA gene identified from an Iranian patient. To our knowledge, this type of mutation has not been reported in non-Jewish population in the literature

The Relationship between Tuberculin Response, BCG Vaccine Scar and Asthma

Recent studies have proposed that a decline in bacterial infections such as tuberculosis is a factor underlying the rising prevalence and severity of atopic disorder in developed countries. There are conflicting ideas about the inverse relationship between BCG (Bacillus Calmette-Guerin) vaccination and asthma. Stronger response to tuberculin test as an indicator of more potent TH1 response is supposed to influence TH2 modulated allergic reactions. BCG scar considered as an indicator of TH1 - immunoresponse has been proposed to be smaller in asthmatic children in some studies. In a case-control study, 97 asthmatic and 97 control children younger than 5 years of age and BCG vaccinated at birth were tested with 5 units of tuberculin intradermaly. After 48-72 hours, the indurated area was measured in two diameters. Mean while, the scar of BCG in both groups was measured. Severity of asthma in the case group was recorded and categorized into mild, moderate and severe groups. The case group consisted of 63% boys and 37% girls and their tuberculin response was significantly smaller than that of the control (p=0.000), but no data supported the inverse relationship between the tuberculin response and severity of asthma (p=0.113). The scar of BCG was not significantly different in the asthmatic children with variable severity of asthma and control group (p=0.864). Children with definite asthma had a significant weaker response to tuberculin. This might be an indication of less potent TH1-reponse in allergic patients, but it was not associated with severity of asthma. No significant relationship between the size of BCG scar and asthma or its severity was found so perhaps BCG scar is not a sensitive indicator for development of asthma in future

Laryngeal Mask Airway Prevented Pulmonary Aspiration in an Obese Patient with Massive Gastric Regurgitation

Laryngeal Mask Airway is used for airway management in anaesthesia and in emergency medicine. Aspiration of gastric contents with LMA occurs in 2 per 10000 patients. It is almost similar to tracheal intubation in elective patients with the incidence of 1.25 per 10000 patient

Evaluation of the the metabolic syndrome criteria and body composition in ambulatory children with Epilepsy in southern Iran: a case – control study

Objectives:  Previous studies in adults with epilepsy revealed a higher prevalence of metabolic syndrome, which resulted in cerebrovascular and cardiovascular events, however, there is insufficient data about body composition and metabolic syndrome in children, especially in Middle Eastern region. We aim to Investigate metabolic syndrome criteria and body composition in ambulatory children with Epilepsy in Southern Iran. Material and Methods: 90 children aged 11.4 ± 3.2 years with epilepsy and their age- gender-matched controls were included in this study. Anthropometric data, lipid profile, blood sugar and blood pressure were checked. Body composition was evaluated using Hologic system dual – energy X-ray absorptiometry. Results: Prevalence of metabolic syndrome in patients (6.7%) was more than the controls (1.1%) (p-value = 0.043). Patients’ fat mass index was more than the controls (P = 0.012), and lean mass+ Bone Mineral Content (BMC) index lower than the controls (P = 0.017). Serum triglycerides in patients using carbamazepine was higher than the others (P = 0.019, Beta = 0.379). Blood pressure was higher in patients using carbamazepine (p = 0.016, Beta = -0.26). Fat mass index was higher in patients using sodium valproate (p = 0.031, Beta = 0.238).  Conclusion: Our study revealed that children with epilepsy are more prone to metabolic syndrome and higher body fat mass. Therefore, early diagnosis and prevention of metabolic syndrome criteria in patients with epilepsy, performing regular exercise and having a healthy diet should be encouraged in these children

Case Report of RANBP2 Mutation and Familial Acute Necrotizing Encephalopathy

Introduction. Acute necrotizing encephalopathy (ANE), a rare entity with unique clinical presentation, can be associated significant morbidity and mortality. The majority of ANE reported cases are sporadic. However, reports of extremely rare familial cases are scarce. Case Presentation. We described three cases, two siblings and their cousin, affected by ANE, all of them exhibiting RAN-binding protein 2 (RANBP2) gene mutation. They all presented with seizure and decreased level of consciousness. Unlike the siblings, the cousin eventually expired mainly due to the delay in diagnosis, resulting from late presentation of typical brain involvements of ANE in magnetic resonance imaging (MRI). Conclusion. The presented cases are the first reports of familial ANE in Iran. Attempt was made to raise awareness on this disease, because high clinical suspicion plays an important role in the early diagnosis and proper management of these patients