7 research outputs found
Leukodystrophies with Intracranial Calcifications
How to Cite This Article: Mahvelati Shamsabadi F. Leukodystrophies with Intracranial Calcifications. Iran J Child Neurol. Autumn 2014;8;4(Suppl.1):16-17.Pls see pdf
Assessment of Cognitive and Motor Development in 150 Children with Refractory Epilepsy
ObjectiveNeuropsychological impairment is an important co-morbidity of chronic epilepsy. The aim of this study was to determine the state of the cognitive and motor development of patients with refractory epilepsy.Materials & Methods We studied 150 consecutive children with epilepsy who were referred to Mofid Children Hospital, a third level public referral University Hospital in Tehran, Iran, from October 2007 to October 2008. Refractory epilepsy was defined as therapeutic failure of three antiepileptic drugs which were used appropriately.Data regarding sex, age, age at which the first seizure occurred, microcphaly, muscle tonicity, EEG findings, kind of treatment for controlling seizures and cognitive and motor development delay were collected from medical records.Development delay was defined as delay in acquiring cognitive ability and motor skills for age according to the Denver Scale II.Results Of 150 patients 72% were younger than 2 years old and 56.7% were male. About 35.3% were microcephalic while 76% had normal muscular tonicity.Only 2.7% had normal EEGs. About 37.3% showed a good response to anticonvulsive drugs and became seizure free, 13.3% showed a relative response to anticonvulsants but 49.3% did not respond. In the present study, 68% had cognitive developmental delay and 60.7% suffering motor delay. There was a significant difference in response to treatment between patients with cognitive and motor development delay.Conclusion Cognitive developmental delay was more frequent in patients with refractory epilepsy, suggesting that early cognitive screening and introduvtion of rehabilitation programs are necessary for patients with refractory epilepsy.
Effect of growth hormone on muscle strength, tone and mobility of children with Prader-Willi syndrome
ObjectivePrader-Willi Syndrome (PWS) is a genetic syndrome presenting with severe hypotonia and decreased agility. Growth Hormone (GH), which is often used in these patients to treat short stature and obesity, seems improve hypotonia, physical strength, activity, and locomotor developmental ability. The aim ofthis study was to find the effects of growth hormone on agility and strength of these patients.Material & MethodsIn a prospective randomized controlled clinical trial in an out-patient pediatric endocrine clinic in Tehran, 21 PWS children (12 boys and 9 girls, 4 to 9 years old) were divided into either GH-treated or control groups and followed for two years. Agility run, sit ups, weight lifting, and inspiratory and expiratory strength were considered as the main outcome measures.ResultsAll the outcome measures of the GH treated group showed a significant improvement compared to the control group.ConclusionGH causes a significant improvement in agility and strength of PWS children.Key words: Preder-willi syndrome; PWS; growth hormone;agility; strengt
Hearing Evaluation in Children with Meningitis by Auditory Brainstem Response and Otoacoustic Emissions
Objective: Meningitis is one of the most important causes of
sensorineural hearing loss in childhood. Because of the critical
situation of patients, proper attention is not usually paid to hearing
assessment in meningitis. By early detection and medical intervention
at proper time, the retadation of lingual development can be avoided.
Material & Methods: The hearing function of 40 children with
meningitis was evaluated at 24-72 hours after diagnosis (acute period)
and 24 hours before discharging from hospital (recovery period) with
auditory brainstem responses (ABR). All patients 7-14 days after
discharge underwent thorough investigation by detecting otoacoustic
emissions test (OAE). Findings: Using the ABR test, during the acute
period, 35 (87.5%) patients had normal hearing and in 5 (12.5%)
patients severe to profound sensorineural hearing loss was detected.
The same results were obtained during recovery period. OAE test showed
normal hearing in 37 (92.5%) patients and impaired cochlear function in
3 (7.5%) patients. Conclusion: Early detection of hearing loss in the
acute and recovery period of meningitis can be made by ABR and OAE
tests
Acute Inflammatory Demyelinating Polyneuropathy in Children; Clinical and Electrophysiologic Findings
Objective: The aim of this study was to evaluate the
electrophysiologic findings of Guillain Barre Syndrome (GBS) in
children and their relation with clinical progress of the disease.
Methods: Twenty-three children with GBS were evaluated between 2005
and 2007. Electrophysiologic evaluations were performed at admission
and one month later. Findings: Five patients needed respirator, 15
were bedridden, 1 developed recurrence 6 months later, and 2
experienced chronic GBS. The most common findings included: decreased
amplitude of muscle action potential (CMAP) (96%), increased distal
latency (74%), increased F wave latency (69%), and decreased nerve
conduction velocity (NCV) (61%). Sensory nerve conduction (evaluating
sural nerve) was normal in 78% of the cases. These measures did not
significantly change after 1 month. Conclusion: Electrodiagnostic
evaluations are helpful at the primary stages of GBS for diagnosis.
Fibrillation potentials and positive sharp waves showing denervation
and axonal injury are presentative of longer duration of the disease
and a worse prognosis
A Comparison of Buccal Midazolam and Intravenous Diazepam for the Acute Treatment of Seizures in Children
Objective: The purpose of the present study is to compare efficacy and
safety of buccal midazolam with intravenous diazepam in control of
seizures in Iranian children. Methods: This is a randomized clinical
trial. 92 patients with acute seizures, ranging from 6 months to 14
years were randomly assigned to receive either buccal midazolam (32
cases) or intravenous diazepam (60 cases) at the emergency department
of a children's hospital. The primary outcome of this study was
cessation of visible seizure activity within 5 minutes from
administration of the first dosage. The second dosage was used in case
the seizure remained uncontrolled 5 minutes after the first one.
Findings: In the midazolam group, 22 (68.8%) patients were relieved
from seizures in 10 minutes. Meanwhile, diazepam controlled the
episodes of 42 (70%) patients within 10 minutes. The difference was,
however, not statistically significant (P=0.9). The mean time required
to control the convulsive episodes after administration of medications
was not statistically significant (P=0.09). No significant side effects
were observed in either group. Nevertheless, the risk of respiratory
failure in intravenous diazepam is greater than in buccal midazolam.
Conclusion: Buccal midazolam is as effective as and safer than
intravenous diazepam in control of seizures