Pearson Syndrome and Neuropathy, Ataxia and Retinitis Pigmentosa (NARP)

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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.

Pattern of mandibular third molar impaction: A cross‑sectional study in northeast of Iran

Objectives: Impacted teeth, if left untreated, have a potential to induce various complications. The aim of the current study was to determine the prevalence and pattern of impacted mandibular third molar in the Iranian population.Study Design: This cross‑sectional study was performed in patients who were referred to the Department of Oral Radiology between July 2009 and October 2010 to obtain an orthopantomogram (OPG). Data were collected regarding age and gender, prevalence of impacted mandibular third molars, angulation of impacted teeth (Winter’s classification), level of impaction (Pell and Gregory classification), and relationship of the mandibular third molar with the ramus (Pell and Gregory classification). The collected data were analyzed using SPSS software version 11.0 with a confidence interval of 95%.Results: Among the 1433 patients included in the study, 489 (34.12%) patients were male and 944 (65.88%) were female. Of the total OPGs performed, 871 (60.78%) OPGs demonstrated at least one impacted mandibular third molar. In addition, of the 2866 mandibular third molars investigated, 1397 (48.74%) were found to be impacted. A significant association was observed between gender and the number of impacted teeth or the presence of impaction of any mandibular tooth (or teeth) (P < 0.05). The most common type of tooth angulation was mesioangular (48.67%). In addition, the most prevalent type of impaction level and ramus relationship was level B (63.85%) and Class II (48.46%), respectively. There were no significant differences between the two sides of the mandible for the prevalence of impacted third molar (P > 0.05).Conclusion: The pattern of mandibular third molars in the Northeast region of Iran revealed a high prevalence of impaction, which was mostly mesioangular, level B, and Class II with a gender predilection for females.Keywords: Impaction, Iran, mandibular third molar, orthopantomogram, patter

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

Spatially homogeneous black hole solutions in

In this paper, we present two new families of spatially homogeneous black hole solution for $$z=4$$ Hořava–Lifshitz Gravity equations in $$(4+1)$$ dimensions with general coupling constant $$\lambda$$ and the especial case $$\lambda =1$$, considering $$\beta =-1/3$$. The three-dimensional horizons are considered to have Bianchi types II and III symmetries, and hence the horizons are modeled on two types of Thurston 3-geometries, namely the Nil geometry and $$H^2\times R$$. Being foliated by compact 3-manifolds, the horizons are neither spherical, hyperbolic, nor toroidal, and therefore are not of the previously studied topological black hole solutions in Hořava–Lifshitz gravity. Using the Hamiltonian formalism, we establish the conventional thermodynamics of the solutions defining the mass and entropy of the black hole solutions for several classes of solutions. It turned out that for both horizon geometries the area term in the entropy receives two non-logarithmic negative corrections proportional to Hořava–Lifshitz parameters. Also, we show that choosing some proper set of parameters the solutions can exhibit locally stable or unstable behavior

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

Complications of Bilateral Sagittal Split Osteotomy in Patients with Mandibular Prognathism

Introduction: Bilateral sagittal split osteotomy (BSSO) of mandible is vastly used in treatment of mandibular deficiencies and discrepancies. Since this method could affect esthetic as well as function, evaluating these effects from various aspects is crucial. This study assessed the effects of this technique on the function of masseter muscle, jaw movements, and sensory changes along with failures in screws used for fixation. Methods: 48 patients with mandibular prognathism participated. Electromyography (EMG) of the masseter muscle; limits of jaw movements including maximum opening (MIO), protrusive (PM), lateral movements (LLE and LRE); presences of sensory changes and two point discrimination test; and number of removed screws were recorded at the baseline, 3 months, and 6 months after surgery. Results: EMG activity of masseter decreased significantly 3 months after the surgery. However, after 6 months the masseter activity revealed no statistically significant difference with baseline activity. There was a significant decrease in MIO and PM after 3 months. The 6 month measurement of MIO and PM was also lower than baseline. However, no difference was observed between LRE and LLE in both follow up sessions. Among 46 patients, 27 patients developed lip paresthesia 3 months after surgery. After 6 month, lip paresthesia remained in 11 patients. Among 276 screws used for fixation 3 screws removed due to exposure to oral cavity and 2 due to patient discomfort. Conclusion: As BSSO in patients with mandibular prognathism revealed temporary functional and sensory changes, it is a safe and appropriate method in orthognathic surgery