Iranian Journal of Child Neurology

Acute transverse myelitis (ATM) is a rare inflammatory and demyelinating disorder of spinal cord with an immune – mediated basis (1). It is potentially a devastating disorder with variable outcomes that can present with subacute onset of motor, sensory and autonomic dysfunction.(2) TM can occur as an isolated condition, usually as a postinfectious disorder or may be the first presentation of relapsing acquired demyelinating syndromes (ADS) that include acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica (NMO). ATM also, must be distinguished from compressive and noninflamatory myelopathies. Diagnostic evaluation, prognosis, recurrence risk, and treatment plans are different among these entities

Background and Objective: There are frequent anti-epileptic drugs which are used in management of epilepsy. Anti-epileptic drugs may have some complications on bone and vitamin-D metabolism. In this study we aimed to Comparison of Bone metabolism disorder in epileptic children with health child in zahedan

Methods: This case-control study was performed on bone metabolism disorder in epileptic children. Forty epileptic children was enetered to the study based on accessability scheme and 40 participants randomly selected for control group from those who were referred to the centers for rutine check up. Blood samples were collected from all participants to assess serum calcium, phosphorus, PTH, magnesium, vitamin D, calcium urine, serum albumin, creatinine random urine. Statistical analyses were performed using SPSS software.

Results: Of 40 epileptic children 23 (57.5%) and 17 (42.5%) were male and female respectively. The prevalence of low vitamin D was 37.5% for patients compared to 12.5% for controls (chi-square = 6.667 and p= 0.010).  Of 80 participants, 15 individuals had abnormal PTH level which is distributed of 2 and 13 for patients and control groups respectively (chi-square = 9.928 and p= 0.002).   In the cases of Ca and Mg their levels were similar in both patients and controls.  The status of the parameters in the classification of normal and abnormal assessed based on number of medications intake resulted that number of medications intake  had not effect on the parameters status.

Conclusion: our study, like previous studies showed that in epileptic children, frequency of hyperparathyroidy and vitamin D deficiency increased.

Objective: To compare prevalence of 25-hydroxyvitamin D deficiency in cerebral palsied (CP) with healthy control children and to evaluate possible correlations between 25-hydroxyvitamin D and severity of CP and motor function.

Materials & Methods: In this case control study, serum levels of 25-hydroxyvitamin D were evaluated in 65 children with CP and compared with 65 healthy children. Blood samples were taken to measure levels of 25-hydroxyvitamin D, calcium, phosphorus and alkaline phosphatase. Regarding 25-hydroxyvitamin D levels, patients were classified as sufficient (≥30 ng/ml), insufficient (20-30 ng/ml) and deficient (<20 ng/ml).

Results: Mean 25-hydroxyvitamin D levels were 28.03±24.2 ng/ml in patients and 30±1.94 ng/ml in control group. 25-hydroxyvitamin D deficiency was seen in 44.6% of CP and 18.5% of healthy children. There was no significant difference in 25-hydroxyvitamin D levels between boys and girls, CP types and use of antiepileptics in case group. There was significant negative correlation between age and 25-hydroxyvitamin D levels. The correlation between 25-hydroxyvitamin D and Gross Motor Function Classification System was not significant.   

Conclusion: 25-hydroxyvitamin D deficiency is common in children with CP in comparison with healthy children. There was significant negative correlation between age and 25-hydroxyvitamin D levels .Routine measurement of 25-hydroxyvitamin D levels and its proper treatment is recommended to prevent its deficiency and subsequent consequences.

Objective: Use of narcotics to relieve pain in labor affects neurobehavioral and nutritional conditions of newborns after birth. However, there are inadequate data on the effects of drugs currently used in labor. This study was performed to examine the association between newborns' breastfeeding behaviors in the first two hours after birth and drugs used for their mothers in labor.

Materials and Methods: In this descriptive-analytical study, 300 women were selected according to convenience sampling and assigned to four groups. Data were collected by a demographic questionnaire and the Newborn Breastfeeding behaviors Tool completed by the researcher as she observed the newborns during breastfeeding after birth. The data were analyzed by one-way ANOVA, chi-square test and Kruskal-Wallis test in SPSS 20.

Results: There is no significant difference between demographic characteristics of the studied groups (p>0.05). In addition, there is significant difference in breastfeeding behaviors between groups. More clearly, breastfeeding ability was higher in the infants of the women administered with no drug than those of the women in the group (p=0.000).

Conclusion: Regarding the findings of the present study, physicians, nurses, and midwives can be informed about the side effects of the drugs used in labor on the newborns' breastfeeding, and improve their breastfeeding outcomes by decreasing the dose of used drug and the duration of the women's treatment with these drugs.

Key words: oxytocin, hyoscine, pethidine, labor, newborns' breastfeeding behaviors

Objective

Hearing loss can affect the perception of emotional reaction to the music.
The present study investigated whether the students with congenital hearing
loss exposed to the deaf culture, percept the same emotion from the music
as students with acquired hearing loss.
Materials & Methods
Participants were divided into two groups; 30 students with bilaterally
congenital moderate to severe hearing loss that were selected from deaf
schools located in Tehran, Iran and 30 students with an acquired hearing
loss with the same degree of hearing loss selected from Amiralam Hospital,
Tehran, Iran and compared with the group of 30 age and gender-matched
normal hearing subjects served our control in 2012. The musical stimuli
consisted of three different sequences of music, (sadness, happiness, and
fear) each with the duration of 60 sec. The students were asked to point to
the lists of words that best matched with their emotions.
Results
Emotional perception of sadness, happiness, and fear in congenital hearing
loss children was significantly poorly than acquired hearing loss and
normal hearing group (P<0.001). There was no significant difference in
the emotional perception of sadness, happiness, and fear among the group
of acquired hearing loss and normal hearing group (P=0.75), (P=1) and
(P=0.16) respectively.
Conclusion
Neural plasticity induced by hearing assistant devises may be affected
by the time when a hearing aid was first fitted and how the auditory
system responds to the reintroduction of certain sounds via amplification.
Therefore, children who experienced auditory input of different sound
patterns in their early childhood will show more perceptual flexibility in
different situations than the children with congenital hearing loss and Deaf
culture

 

Background: Considering the fact that there are no reports about the clinical presentations and outcome of Guillain-Barré syndrome (GBS) in our region, we aimed to report some mentioned findings in children diagnosed with GBS in Isfahan, Iran. Materials and Methods: In this retrospective study, pediatric patients diagnosed with GBS were enrolled.The following information were extracted from the medical files of patients; age, gender, early signs and symptoms of GBS, neurological features, sensory and motor and autonomic involvements, sphincter dysfunction, bulbar muscle involvement, respiratory failure, cranial nerve paralysis, delay time from onset to definite diagnosis and management of GBS and the outcome. Results: During this study, 57 children with GBS aged 1-13 years were evaluated. Frequency of GBS was significantly higher in boys than in girls (38.6% vs. 61.4%, P=0.01, OR=0.39). The most common clinical presentations were distal lower limb weakness (92.11%), reduced deep tendon reflex (DTR ) (82.46%) and neuropathic pain (75.44%). 92.9% of patients had complete recovery. Conclusion: The findings of this study indicated that distal lower limb weakness, reduced deep tendon reflex and neuropathic pain are the main clinical presentation in children with GBS but in some patients DTR may be normal or even exaggerated in early stage of disease. This finding suggests that revising the diagnostic criteria for GBS may be necessary. Most of our patients had complete recovery. The only death was due to autonomic involvement.

Abstract

Objective: Cochlear microphonic (CM) is a cochlear AC electric field, recorded within, around, and remote from its sources. Nowadays it can contribute to the differential diagnosis of different auditory pathologies such as auditory neuropathy spectrum disorder (ANSD). The aim of this study was to compare CM waveforms (CMWs) and amplitudes with broad and narrow band stimuli in 25 healthy male young adult Wistar rats.

Methods: Using an extratympanic technique in ECochG (Electrocochleography) recording, CMWs in response to click and tonal stimuli with different octave frequencies were recorded at a high intensity level in subjects. The CMW amplitudes were calculated by a graphical user interface (GUI) designed in MATLAB. The data was analyzed by One-way ANOVA test.

Results: The CMW magnitude increased upon an increase in band width stimulation. Across tonal stimuli, the CMW amplitudes at lower frequency tones were larger than those at higher frequency tones. Those findings were statistically significant (P< 0.001).

Conclusion: This study found that CMW most likely is a reflection of spatial summation of voltage drops generated by hair cell groups in response to acoustic stimulation. In order to production nature of CM potentials as well as their very small magnitudes especially with tonal stimuli, thus, we recommend using click stimulation for CM potential recording especially in patient with ANSD that CM plays an important role in its differential diagnosis and follow up

Aim: To investigate blood lead level (BLL) in children with neurologic disorders of unknown causes and compare with normal children.

Methods: In this prospective case control study, 68 patients aged 1 to 18 years with neurologic disorders of unknown causes, were selected during a 12 month period from Sep. 2013. They were compared with 1:1 ratio, age and sex matched healthy children. BLL was checked from all participants using 3 cc heparinized venous blood sample. Level of ≥5 mcg/dl was considered toxic dose.

Results: Totally, 136 children (68 cases and 68 controls) with mean ages of 5.20±4.12 and 4.18±3.86, respectively, entered the study. Mean BLL was higher in case group than in controls but the difference was not significant (P=0.84), though they were less than toxic levels in both. Also, the difference in mean BLLs were not significant in terms of living place, sex and age.  Totally, 17.7% 0f the study sample had BLL ≥5 mcg/dl. The frequency of BLL ≥5 mcg/dl was significantly higher in case group (P=0.024) with and odds ratio 2.9 times higher (95% CI: 1.066-7.60).

Conclusions: Strategies in public health must focus on practicing primary and secondary preventions of lead exposure in children.

Abstract

Objectives: Children's emotional-behavioral problems will have a huge impact on their future. It seems such problems are more seen in the siblings of children with special needs. The present study sought to compare emotional-behavioral problems in the healthy siblings of autistic children with the healthy siblings of children with Down syndrome in order to identify such children in Iran.

Materials & Methods: This descriptive study was carried out on 174 healthy children aged between 3 and 9 years old among whom 58 cases had autistic siblings, 58 cases had siblings with Down syndrome, and 58 cases had typically development siblings. The participants were selected using convenience sampling technique. All volunteer parents filled in SDQ Questionnaire. The study results were calculated using independent sample t-test, two-way ANOVA, and Tukey post hoc test by SPSS Software V.21.

Results: The mean overall score of Strengths and Difficulties questionnaire was reported as 17.98±6.19 in the autism group, 11.01±6.56 in the Down syndrome group and 4.43±4.34 in the healthy group so that there was a significant difference among autism, Down syndrome and healthy groups. In the group of siblings with Down syndrome, the problems were significantly more in the age range of 3 to 7 years old (p <0.05). However, no significant difference was observed in the scores of males and females (p> 0.05).

Conclusions: Siblings with autism or Down syndrome can have especial psychological effects on healthy children in families in the way that such effect will be more with autistic children. Therefore, formulating beneficial strategies for their parents is useful to prevent emotional- behavioral problems.

 

 

Introduction: Type 1 diabetes mellitus(T1DM) is a chronic immune-mediated disease. Diabetic peripheral neuropathy (DPN) is an important micro vascular complication of T1DM. One of the most important risk factors for the development of DPN is poor glycemic control. The aim of this study was to evaluate the prevalence of DPN among T1DM patients and to determine the association between DPN and glycated hemoglobin(HbA1c) level.

Methods: The subjects were recruited prospectively upon initial evaluation at a tertiary hospital. Patients with T1DM were selected based on the inclusion criteria (i.e., age of 6≤years and absence of other co-morbidities). DPN was assessed through electro diagnostic studies and neurological examinations, while diabetes control was evaluated by measuring the HbA1c level.

Results: In total, 50 patients with T1DM were enrolled in this study. The mean diabetes duration of patients was 8.38±3.79 years (mean age16.68±6.68 years). The mean HbA1c level was 8.6±2.1% in patients without DPN and 10.5±3 in those with DPN (P=0.016). Overall, 24% of the subjects were presented with DPN according to nerve conduction velocity(NCV) findings. A positive correlation was found between NCV and clinical symptoms with signs (P<0.001, r=0.45 and P<0.001, r=0.644, respectively). Sensitivity and specificity of neurological examination for DPN diagnosis were 91.7% and 63.2%, respectively. Our findings revealed that, poor diabetes control is associated with DPN. Also, HbA1c level was used as an index for glycemic control over the past six months.

Conclusion: It seems that rigid blood glucose control and periodic neurological examinations were the best strategies to prevent DPN. 

Introduction: Type 1 diabetes mellitus(T1DM) is a chronic immune-mediated disease. Diabetic peripheral neuropathy (DPN) is an important micro vascular complication of T1DM. One of the most important risk factors for the development of DPN is poor glycemic control. The aim of this study was to evaluate the prevalence of DPN among T1DM patients and to determine the association between DPN and glycated hemoglobin(HbA1c) level.

Methods: The subjects were recruited prospectively upon initial evaluation at a tertiary hospital. Patients with T1DM were selected based on the inclusion criteria (i.e., age of 6≤years and absence of other co-morbidities). DPN was assessed through electro diagnostic studies and neurological examinations, while diabetes control was evaluated by measuring the HbA1c level.

Results: In total, 50 patients with T1DM were enrolled in this study. The mean diabetes duration of patients was 8.38±3.79 years (mean age16.68±6.68 years). The mean HbA1c level was 8.6±2.1% in patients without DPN and 10.5±3 in those with DPN (P=0.016). Overall, 24% of the subjects were presented with DPN according to nerve conduction velocity(NCV) findings. A positive correlation was found between NCV and clinical symptoms with signs (P<0.001, r=0.45 and P<0.001, r=0.644, respectively). Sensitivity and specificity of neurological examination for DPN diagnosis were 91.7% and 63.2%, respectively. Our findings revealed that, poor diabetes control is associated with DPN. Also, HbA1c level was used as an index for glycemic control over the past six months.

Conclusion: It seems that rigid blood glucose control and periodic neurological examinations were the best strategies to prevent DPN. 

Objective: To evaluate the distribution of the Bayley screening test scores by age, in Persian children and compare developmental risk categories distributions between Persian and reference norms.

Materials & Methods: A representative sample of 417, 1 to 42-months-old, typically developing children by consecutive sampling from health -care centers recruited, during the 2014 to 2015 in Tehran city.  The Bayley measures children’s cognitive, receptive and expressive communication, fine and gross motor skills. For determining cut points for the subtest scores, two cuts points were determined for each age group, dividing scores into the three bands that identify the at risk, emerging (between the 2^nd and 25^th percentiles), and competent categories. We estimated the agreement between the distributions of the risk categories between the two samples using weighted kappa statistics.

Results: The comparison of neurodevelopmental classification of children based on two norms determined that; approximately 70-80% of all tests administered to the participating children were classified as normal by both norms. Weighted kappa coefficients for the five subtests ranged from 0.56 to 0.89 suggesting moderate agreement between the classification using the reference and Persian norms. Expressive and receptive communication subtests had the lowest kappa scores (0.56 and 0.59, respectively), and classification of gross motor demonstrated the highest level of agreement (0.89).

 

Conclusion: Our findings demonstrate that reliance on reference -based norms for the Bayley test in Persian children results in misclassification of developmental delay.

objective

Gaucher disease is the most common of the inborn error of metabolic disorder that  known as lipid storage disorders. This disorder  is caused by a deficiency of the enzyme glucocerebrosidase. .There for , glucocerebroside accumulate in multiple organs. Symptoms may begin at any age ( in early life until the adulthood )and include skeletal disorders, organomegally , and cytopenia. Gaucher disease is classified into three types. type 1 is the most common form and named non neuropatic form . Type 2 is named acute infantile neuropathic form,because  severe neurological symptoms  are apparent by 3 months of age. Type 3  is named chronic neuropathic form. Videlicet,this disorder  is classified on the presence or absence of neurological symptoms .

Material and methods

In this study , We assesed neurological symptoms in  our GD1patients and compared them with  GD3 patients.

 Three patients with GD3 and eight patients with GD1 entered in this study.

Result

We found in this study that patients with GD1  had  neurological  problems  including: Cognitive impairment,developmental disability ,behavioral disorder, microcephaly ,increased DTR,too.

Though,some symptoms including :seizure,supranuclear gaze palsy, cerebellar sign and ataxia only excist in GD3 patients .

conclusion

In previous studies has  mentioned  that distinction between type 1 and 3 is by  neurological symptoms  but we found  some neurological signs  are seen in Type 1,too. Although, the neurological symptoms are  most severe in type 3 and some symtoms only detected in GD3 . So,  generally neurological symptoms alone can not  differentiate the types of gauche disease.

Molybdenum cofactor deficiency is a rare metabolic disorder with neonatal onset seizures, developmental delay, microcephaly and spasticity. In this report, we describe a three-month-old infant presented with neonatal onset, poorly controlled seizures, developmental delay, microcephaly, spastic quadriparesis and visual insufficiency. Magnetic resonance imaging of brain had shown cystic encephalomalacia involving bilateral parieto-occipital lobe, and elevated lactate in magnetic resonance spectroscopy.  Restricted diffusion noted along the corticospinal tract in our case is a novel imaging finding in molybdenum cofactor deficiency.  Low serum uric acid and elevated urine sulphite excretion were observed. A novel homozygous mutation was detected in exon 4 of MOCS2 gene.

Early infantile or neonatal onset seizures, developmental delay, microcephaly and cystic encephalomalacia in neuroimaging mimicking hypoxic ischaemic encephalopathy should raise the suspect for molybdenum cofactor deficiency. Screening of all neonates for urinary sulphite metabolites helps in early diagnosis and management. Early diagnosis and treatment with cyclic pyranopterin monophosphate could arrest the progression of this disease. More research is needed to explore further treatment options in this otherwise lethal disorder.

Wilson’s disease (WD) is a rare autosomal recessive disease due to copper metabolism disturbance. The clinical presentation spectrum of Wilson’s disease is wide and initial findings of the disease depend on the organ involved. Neurologic disorders can develop insidiously or precipitously with intention tremor, dysarthria, rigid dystonia, parkinsonism, deterioration in school performance or behavioral changes. The aim of this article is presenting an 11-year old boy with chief complaint of falling and upper limb spasm referred to our center. His symptoms began from 6 month earlier as mood instability (prolonged spontaneous crying). He was also suffering from occasionally tremor and micrographia. Initial work ups were normal and with diagnosis of depression and psychiatric problems he was undergone treatment of fluoxetine and risperidone. Finally, concluded that Wilson’s disease should be considered in the diagnosis of all children with psychiatric and musculoskeletal symptoms.

Gate disturbance is a common presentation of neurologic disease

in children.Limping is a kind of gate dysfunction that occurs due to

neurologic &skeletal disease. Diskitis is one of the cause of limping specially

in child less than 3years. We aim to report a case of diskitis and limping as the significant manifestation of Gate disturbance in 17 Shahrivar hospital in Rasht, IRAN.