Iranian Journal of Child Neurology

Abstract:

Herpes Simplex encephalitis (HSE) is a life threatening outcome of Herpes simplex virus (HSV) infection of the central nervous system (CNS). HSV

accounts for 2-5 percent of all cases of encephalitis. One third of cases occur in those younger than 20 years old and one half in those older than 50 years old.

Clinical diagnosis is recommended in the encephalopathic, febrile patients with focal neurological signs. However, the clinical findings are not pathogonomic because numerous other diseases of CNS can mimic HSE. Diagnosis should be confirmed based on medical history, analysis of cerebrospinal fluid (CSF) for protein and glucose contents, the cellular analysis and identifying the pathogens by serology and Polymerase Chain Reaction (PCR) amplification .

The diagnostic gold standard is the detection of HSV DNA in the cerebrospinal fluid by PCR. But negative results need to be interpreted regarding the

patients clinical signs and symptoms and the time of CSF sampling. Spike and slow wave patterns is observed in Electroencephalogram (EEG).

Neuroimaging, especially Magnetic Resonance Imaging (MRI) is essential for evaluating the patients, which shows temporal lobe edema or hemorrhage.

All patients with HSE should be treated by intravenous Acyclovir (10mg/kg q8hr for 14-21 days). After completing therapy, PCR of the CSF can confirm

the elimination of replicating virus, assisting further management of the patient.

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Objective

Neuromuscular disorders (diseases of the motor unit), can cause respiratory problems such as impaired cough reflex, chest deformity, recurrent pneumonia and acute respiratory failure; these are the worst most common complications of these diseases and the leading cause of death in such patients (1, 2). Their management hence, very often, entails admission to the Pediatric Intensive Care Unit (PICU) (3,4) and during this phase, endotracheal intubation is almost always necessary, to maintain the patency of airways and to apply Positive Pressure Ventilation (PPV). However, endotracheal intubation is always temporary, and its success or failure depends on the timely decision of its termination to restore the normal respiration or to avoid the risk of recurring respiratory failure (5, 6). We designed this study to evaluate the role of neuromuscular disorders in causing extubation failure as compared to that of other risk factors.

Materials & Methods

In an analytical cross-sectional study, the risk factors of reintubation and duration of mechanical ventilation in two groups of 30 patients each, was compared, the first successful extubation and the second with extubation failure.

Results

Neuromuscular disorders (including Spinal Muscular Atrophy, Guillain- Barre' Syndrome, Congenital Myopathies and Muscular Dystrophies) were the main underlying diseases in extubation-failure group (P= 0.0002). Hypercapnia (PaCO2>50mmHg) was shown to be the most common cause of both the first intubation (P=0.001) and reintubation (P=0.004) in the group of patients who failed extubation. The mean duration of intubation and mechanical ventilation was longer in patients with neuromuscular disorders who had extubation failure (P= 0.01).

Conclusion

This study showed that, as underlying problems, neuromuscular disorders are the most common causes of prolonged intubation which defeat weaning from the ventilator and result in reintubation by inducing hypercapnia. Therefore the weaning process needs to be done gradually in these patients, and in conjunction with supportive measures, such as close observation for at least for 72 hours following extubation to monitor any possibility of recurrence of hypercapnic respiratory failure.

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Objective

Most children brought to the emergency department (ED) for evaluation of seizures undergo an extensive laboratory workup. Since results are usually negative, the value of such routine laboratory workups has been questioned. A group of children with unprovoked seizures was prospectively studied to determine the diagnostic values of routine serum chemistries and to identify risk factors predictive of abnormal findings.

Materials & Methods

All patients evaluated at the ED of the Ghaem hospital during a consecutive 12 months period between Jan 2004 through Jan 2005 were studied. We collected data for patient's demographics, details of the history of present illness (including vomiting, diarrhea, apnea), medication use, past history of seizures, family history of seizures, metabolic disorders or other chronic medical illnesses, neonatal history and neurological examination as well as nutritional status, type and time of seizure. The role of abnormal serum chemistries as a seizure trigger factor was assessed in patients with a history of seizure.

Results

A total of 210 patients (mean age 19.2 months) with unprovoked seizures were evaluated. Twenty- three serum abnormalities were noted in the patients (12 cases of hyponatremia, 7 of hypoglycemia, 4 of hypokalemia, 4 of uremia). The incidence of abnormal serum biochemical values was higher in patients with a first seizure, younger patients, and those with gastrointestinal symptoms.

Conclusion

According to the present study, one can conclude that in children younger than 2 years and having no structural CNS abnormality, electrolyte and glucose screening is recommended only for a first unprovoked seizure, when gastrointestinal symptoms or symptoms suggesting electrolyte disturbances are present.

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Objective

This study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine factors that help the clinician in his/her diagnosis and management.

Materials & Methods

A retrospective review was performed on all pediatric patients suffering from myasthenia gravis (M.G) admitted in the department of pediatric neurology of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and 2002.

Results

Of the thirty-two children with M.G. enrolled in our study, seven were suffering from the congenital type while the remaining (25 cases) had the juvenile M.G. Initial symptoms of congenital M.G were ptosis (7/7), limitation of eye movement (2/7) and mild generalized weakness (6/7). Although the Tensilon test was positive in 85% of congenital M.G cases, no myasthenia crisis or spontaneous remission was observed in any of them. In children with juvenile M.G, the age of presentation was 1.2 to 12.5 years, mean age 5.7+4.2 years (15 girls and 10 boys). The most common presenting symptoms in juvenile group were ptosis in 96% and generalized weakness in 76%. Eight of them (32%) had had at least one myasthenia crisis. EMG was diagnostic in 83% and one case the tensilon test was positive in 84%. One patient had hyperthyroidism and had already been diagnosed with hypothyroidism; two of them were epileptics. Eight patients underwent thymectomy microscopically; in specimens examined, five (62%) showed thymic follicular hyperplasia while in remaining three results were normal. One patient (12.5%) recovered completely after thymectomy with no need for medication during the follow up. Four patients (50%) showed relative  improvement and in three cases (37%) improvement was negligible.

Conclusion

The results showed a female to male ratio of 1.5/1 which was correlated to adult M.G. The most common presenting symptoms consisted of ophtalmoplegia, with bilateral ptosis being the most significant. Although this study revealed that thymectomy lacks any remarkable prognostic influence, all patients had thymectomy after two years of disease onset. Some reports have indicated positive results if surgery was performed within two years of onset of disease.

Key words: Myasthenia gravis, Children, Thymectomy, Congenital

Objective

Whatever the health field, compliance with the recommended practice guidelines or parameters for diagnosis by specialists and expert health professionals benefits the patients. This study was conducted to determine the whether or not these guidelines or parameters are applied to the evaluation of children with first simple febrile convulsion (SFC) in a regional teaching hospital.

Materials & Methods

In a prospective study conducted on children with SFC, admitted in the Pediatric ward of Imam hospital, Oromieh, records of investigations ordered between Jan 2003 and Dec 2004 for children diagnosed with SFC were collected. Practice parameters of the American Academy of Pediatrics (AAP) were employed as diagnostic standards. Applied practices were compared with AAP recommended practice parameters. Investigations performed included lumbar puncture, complete blood count, CRP, ESR, blood glucose, serum calcium, electrolytes, renal function tests, urinalysis, urine culture, and blood culture, chest X-ray, EEG and CT scan.

Results

Two hundred and fifty-one consecutive cases, aged 6-60 months, were evaluated. Complete blood count, blood glucose, serum calcium, serum electrolytes, renal function tests, urinalysis, urine culture, and blood culture were tested in all cases (100%). Lumbar puncture, chest X-ray, EEG and CT brain scan had been performed in 10%, 24%, 1.4% and 0.65% of cases, respectively. The mean number of routine investigations was twelve.

Conclusion

Compared to recommended practice guidelines the results of this study highlighted that children with first SFC had more often than necessary investigation. These, not only resulted in significant expense, they proved to be of little diagnostic value. Compliance with a centrally organized national program would significantly help to improve SFC evaluation.

Keywords: Febrile convulsion, Simple fébrile seizure, Lumbar puncture, Convulsion, Fever

Objective

Different medical and rehabilitation interventions have been used for treatment of cerebral palsy (CP). In addition to conventional methods, complementary medicine such as homeopathy has been used in treatment of neurodevelopmental disorders. This study has been done to determine what effect homeopathic treatment would have on motor development (MD) of children with spastic CP, when added to rehabilitation normally used for such children.

Materials & Methods

This 2004 study was a double blind clinical trial, conducted on twentyfour subjects recruited from a developmental disorders clinic in Tehran. Using the minimization technique, subjects were divided to the case and control groups. Routine rehabilitation techniques were carried out for 4 months on both groups. In addition the cases were given homeopathy drugs, while the controls received placebos. Levels of gross and fine motor development were assessed with the Denver Developmental Screening Test II (DDST II). Data was collected by assessment forms, direct observations and examinations. Dependant variables in the two groups were compared at the beginning and at the end of the study.

Results

The average ages of the case and control groups were 28 and 28.4 months respectively. Gross and fine motor development and motor developmental quotient in the case group, compared to the controls showed no statistically significant differences.

Conclusion

Based on the results of this study adding homeopathy to rehabilitation had no significant effect on motor development of CP children. Considering the documented effects of homeopathy on the physical status of children with CP it would be better not to reject the possibility of effects of homeopathy on motor development of children with CP. As homeopathy is young in Iran, it is recommended to conduct further more extensive research on the effects of homeopathy on neurodevelopmental diseases.

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Objective

Febrile convulsion is the most common benign convulsive disorder in children. Meningitis is one of the most important causes of fever and convulsions, diagnosed by lumbar puncture (LP), a painful and invasive procedure much debated  regarding its necessity. This study evaluates the frequency of abnormal LP findings in a group of patients, to determine whether or not unnecessary LP can be prevented without missing patients with serious problems such as meningitis.

Materials& Methods

The study was a descriptive, cross sectional study, conducted on 200 children suffering from fever and convulsions. Medical files of patients were taken from the hospital records and relevant data were collected to complete the appropriate forms.

Results

Of 200 patients included in the study, 116 (58%) children were male, and 84 (42%) were female. 47 cases (23.5%) underwent LP, of whom just one (0.5%) had abnormal LP and meningitis.

Conclusion

Regarding Considering the low prevalence of meningitis in children with convulsion and fever, we conclude that by means of precise clinical examination and monitoring, it is possible to prevent unnecessary LP in these patients.

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Abstract:

Griscelli syndrome (GS) is a rare disease first described in 1978. It is inherited in autosomal recessive pattern. This disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement & uncontrolled phases of macrophage & lymphocyte activation.

We report a 5 months Old Iranian girl presenting with silver-gray hair,eyelashes and eyebrows, hepatosplenomegaly, pancytopenia, hemophagocytosis and progressive neurologic deterioration. Griscelli syndrome can be suggested according to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure.

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