Iranian Journal of Child Neurology (IJCN)
Volume:6 Issue: 4, Autumn 2012

Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of neurological manifestations in children with rheumatologic disorders is limited. This review describes the neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally.

Breath holding spells (BHS) are common paroxysmal non-epileptic eventsin the pediatric population which are very stressfull despite their harmlessnature. There has been no specific treatment found for the spells yet. The aimof this study was to evaluate the efficacy of piracetam (2-oxo-l-pyrrolidine)on these children.In this randomized double blind clinical trial study, 150 children with severe BHS referred to our pediatric outpatient service were enrolled from August2011 to July 2012. The patients were randomized into two equal groups.One received 40mg/kg/day piracetam and the other group received placebo,twice daily. Patients were followed monthly for three months. The numberof attacks/month before and after treatment were documented.Of the enrolled patients, 86 were boys. The mean age of the patients was17 months (range, 6 to 24 months). In the piracetam group, 1 month after treatment an 81% response to treatment was found. In the placebo group,none of the patients had complete remission and 7% of the cases had partialremission. Overall, control of breath-holding spells was observed in 91%of the patients in the group taking piracetam as compared with 16% in the group taking placebo at the end of the study. There wasd nosignificant difference detected between the groups regarding the prevalenceof drug side effects.A significant difference was detected between piracetam and placebo in prevention and controlling BHS. Piracetam (40mg/kg/day) had a good effecton our patients.

Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy that occurs mostly after prior infection. The diagnosis of this syndrome is dependent heavily on the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually confirm the diagnosis. This is a retrospective study which was performed to investigate the atypical features of GBS.Thirty three patients (21/63.6% males and 12/36.4% females) with GBS were retrospectively studied and prospectively evaluated at the Child Neurology institute of Mofid Children Hospital of Shahid Beheshti University of Mediccal Sciences between May 2011 and September 2012.The mean age was 5.4 years (range, 1.5-10.5).Twenty one patients (87.9 %) had previous history of infections. Eight patients (24.2%) admitted with atypical symptoms like upper limb weakness (3%), ptosis (3%), neck stiffness (3%), inability to stand (proximal weakness) (9.1%), headache (3%) and dysphagia (3%).According to disease process, weakness was ascending in 26 (78.8%), descending in 5 (15.2%) and static in 2 (6.1%) patients. Cranial nerve involvement was found in 8(24.3%) children, most commonly as facial palsy in 3 (9.1%).In this study, 24.3% of our patients presented with atypical symptoms of GBS as upper limb weakness, ptosis, neck stiffness, inability to stand (proximal weakness), headache and dysphagia

Hypothyroidism may be an exacerbating factor for primary headaches andmigraine is one of the most common primary headaches in childhood. Thepurpose of this study was to evaluate the effect of treatment of subclinicalhypothyroidism on children with migraine headache. In a quasi-experimental study, the severity and monthly frequency ofheadache of 25 migraineur children with subclinical hypothyroidism whowere referred to the pediatric neurology clinic of Shahid Sadoughi Universityof Medical Sciences,Yazd, Iran between January 2010 and February 2011and were treated with levothyroxine for two months were evaluated.Thirteen girls (52%) and 12 boys (48%) with the mean age of 10.2 ± 2.76years were evaluated.In children with hypothyroidism, the monthly frequency of headache (mean± SD: 17.64 ± 9.49 times vs. 1.2 ± 1.1 times) and the severity of headache(mean± SD: 6.24±1.8 scores vs. 1.33 ± 0.87 scores) were significantlydecreased by treatment.Based on the results of this study, treatment of subclinical hypothyroidismwas effective in reducing migraine headaches. Therefore, it is logical tocheck thyroid function tests in migraineur children.

Febrile seizure is one of the most common neurological conditions of childhood. Several theories, such as iron deficiency anemia have been proposed as the pathogenesis of this condition. The aim of this study was to find the association between iron deficiency anemia and febrile seizures in children aged 6 months to 3 years admitted in Valie Asr hospital in Zanjan.Hemoglobin (Hb), mean corpuscular volume (MCV), serum iron (SI), total iron binding capacity (TIBC) and SI/TIBC ratio were assessed in one hundred children with febrile seizures and compared to the values of one hundred healthy children presenting in a heath care center in the same period as the control group.A total of 6% of cases had iron deficiency anemia which was similar to the control group. In the case group SI/TIBC ratio below 12% was seen in 58% of children which was significantly higher than that of the control group (29%).The results of this study suggest that although anemia was not common among febrile seizure patients, iron deficiency was more frequent in these patients.

Because of geographical and periodical variation, we prompted to determine the demographic features and causative factors for febrile seizure in Rasht. In this cross-sectional study, all 6–month- to 6-year-old children with the diagnosis of febrile seizure admitted to 17 Shahrivar hospital in Rasht, from August, 2009 to August, 2010 were studied. Age, sex, family history of the disease, seizure types, body temperature upon admission and infectious causes of the fever were recorded. All statistical analysis was performed with SPSS software, version 16. Of the 214 children (mean age, 25.24±15.40 months), 124 were boys and 109 had a positive family history. Complex seizures were seen in 39 cases. In patients with a complex febrile seizure, 59% had the repetitive type, 20.5% had the focal type and 20.5% had more than 15 minutes duration of seizures. Most of the repetitive seizures (78.3%) occurred in patients under 2 years old; the difference between under and over 2-year-old patients was statistically significant (P=0.02). Study results did not show significant differences between the two genders for simple or complex seizures. The mean body temperature upon admission was 38.2±1.32◦C (38.31±0.82 degrees in boys and 38.04±1.78 in girls). Upper respiratory infections were seen in most patients (74.29%). All cases of lower respiratory infections were boys. There was a statistically significant difference between boys and girls in causes of fever. Most of the children had a positive family history and the most common causative factor was upper respiratory infection.

Hospitalization of a child can cause severe anxiety and stress in the parents, especially for the mother. This stress consequently affects the treatment course of the child. Hereby, we investigate the impact of different stressors in mothers of hospitalized children. In this cross-sectional study, 225 mothers of hospitalized children in the pediatric ward of Besat hospital were randomly selected and studied. Data collection tool was a two-part questionnaire gathered by interviewing the mother. The first part included demographic information of the patients. The second part included questions regarding stressors in four different categories; child-related factors, environmental factors, socioeconomic factors and health professional factors. SPSS 16.5 was used for statistical analysis and data were analyzed by one way ANOVA and T test.In the child-related factor category, fear of child death (84%); in the socioeconomic factor category, fear of disease in the other siblings (84%); in the environmental factor category, unpleasant odors in the ward (56%); and in the health professional category, not enough explanation about inserting IV lines, (54.2%) constituted the most important factors.There was a meaningful correlation between the stressors and the mothers’ age and occupation, child age, days of hospitalization, types of admission and health insurance coverage, but there was no meaningful correlation between stressors and other factors.Professional and in depth training programs should be provided for health care providers and nursing staff regarding dealing with mothers of hospitalized children.

Schizencephaly is a rare central nervous system disorder with variable presentations. Here we report a patient with a huge bilateral schizencephaly and septo-optic dysplasia presenting with anterior encephalocele.

Several syndromes have been recognized with digital abnormality and CNS involvement such as oculodentodigital dysplasia (ODDD), Mohr syndrome and Joubert syndrome. We report a patient who was referred to us because of the neurological signs suspicious of metabolic disorders. This case was a 22-year-old woman whose problems began 4 years ago with shortening of memory, ataxia, abnormal gait and diplopia which progressed slowly. She consulted many neurologists and was on treatment with the suspicion of vasculitis, but no response was detected. She had severe short stature, hypoplasia of the middle and distal phalanges of the first, second and third fingers, clinodactyly, abnormal toes, abnormal enamel and missing teeth. She had no characteristic faces of ODDD and ophthalmological abnormality. Our patient might be a variant of ODDD or a new syndrome with somatic and neurologic signs.