Iranian Journal of Child Neurology

Minor trauma to the head is common in childhood and does not require any medical or surgical treatment. Nevertheless, head injury in infancy and childhood is the single most common cause of death and permanent disability. Measurable deficits occur even after mild to moderate head injury but are markedly greater after severe injury. They include impaired cognition, motor impairments, disruption of attention and information processing, and psychiatric disturbances. Despite the frequency of the sequelae of head injury in childhood, there is relatively little information about the structural basis of the clinical deficits. Classical literature suggests that the immature brain and its coverings, at a time when it is rapidly acquiring new information, respond differently from the adult brain when subjected to an equivalent amount of mechanical force, whether mediated by contact or inertial loading. Identification of different patterns of injury in different age groups has resonance in clinical practice and now provides a reference point for future clinical and neuropathological studies. This work not only provides the basis for the future management of patients, but also serves to remind us of the continuing value of the autopsy and the proper examination of retained organs using modernstandardized techniques.

Objective

 The aim of this study was to evaluate and compare the effects of Na Valproate and Phenobarbital on changes in the weight of epileptic patients following treatment for their condition using the drugs mentioned.

Materials and methods

Sixty epileptics were assigned into two groups of 30 patients each, the case and controls. The diagnosis was made on the basis of the International League Against Epilepsy (ILAE) characteristics. BMI was defined. In the case group, the patients received 20mg/kg/day of Na Valproate, while the 30 controls received 5mg/kg/day of Phenobarbital for 6 months. Using the Mc Nemar and Chi-2 tests, BMI changes were compared after 6 months between the groups. Fisher's exact test was used to evaluate the role of age, sex, and primary weight on the weight increase due to Na Valproate usage.

Results

There were no specific changes in age, sex, primary BMI and fatness between the 2 groups; in the case group, 20 patients(66.7%) and in the controls 4(13.3%) gained weight (P

Conclusion

The results indicate that epileptic children, aged over 10 years, and those who are overweight have more chances of gaining weight or becoming fatter, following treatment with Na Valproate. Further studies investigating the issue are warranted.

Objective

To study the clinical presentation, hospital course and outcomes of patients admitted with Guillain-Barre Syndrome (GBS) to three tertiary care hospitals in Mashhad, Iran.

Materials and Methods

The records of all patients admitted with flaccid paralysis between April 1999 and January 2005 were reviewed and those with the diagnosis of GBS were included in the study. Standard questionnaires were used to record clinical data on was recorded on a standardized questionnaire, which included patients' age, sex, antecedent infectious history, neurological signs and symptoms and ventilation requirements. The hospital course, including therapy given and the functional status of patients, was analyzed, including therapy given and the functional status of patients.

Results

Ninety-one cases of acute flaccid paralysis were admitted to the hospitals during the study period. Eighty-three cases, age range 10 months to 11 years, were later diagnosed as GBS afterwards, with an age range of 10 months to 11 years. The mean age for disease onset was 4.2 years; there were 47 boys and 34 girls, male to female ratio 1:0.7. Upper respiratory tract infection (62.6%) was the most common antecedent event, followed by gastrointestinal infections (19%), urinary tract infection (1.2%) and chicken pox (2.4%), while the remaining cases (14.8%) had no other cases (14.8%) did not have any reliable history of any preceding antecedent infections. Most patients developed GBS within one month of the preceding infection. Cranial nerve abnormalities (19.3%), autonomic dysfunction (7.2%) and respiratory failure requiring intubation (10.8%) were also common. The in-patient mortality was 2.4% (2 of 83).

Conclusion

GBS was found to occur slightly more often in male patients, majority of whom had histories of previous infection. Despite persistent disability, in-hospital mortality was low.

Objective

Neonates, for many reasons, are at particular risk for the development of seizures, which are a strong predictor of later morbidity and mortality in infants.

We undertook this study to determine the incidence, etiologic distribution and neonatal seizure type in neonates with hospital admission over a period of 4 years.

Materials and Methods

This, a retrospective study of newborns admitted in hospital with a diagnosis of neonatal seizures, was conducted over a 4 year period between March 2001 and March 2005.

Data were obtained from hospital records was analyzed using the Chi-square test.

Results

Of 4541 newborns, admitted to hospital, during the study period, seizures occurred in 110 neonates. The incidence of neonatal seizures was 2.4%; the causes of neonatal seizure were Hypoxic-Ischemic Encephalopathy (HIE) - 36.4%, infections -19.1%, metabolic abnormalities - 7.3%, Intra Cranial Hemorrhage (ICH) - 2.7%, structural disorders - 1.8% and in 32.7% of cases, the cause was unknown.

Subtle seizures (39.1%) were the most common type of seizures; and the other types were myoclonic (17.3%), clonic (10.0%), Tonic (7.3%), Generalized Tonic Clonic Seizures (GTCS) (12.7%) and in 13.6% of cases the type of seizure was not mentioned. Mortality rate was 13.6%.

Conclusion

Health care workers and parents need to be made aware of subtle seizures and the importance of timely and appropriate treatment to decrease any further complications.

Objective

Sub acute Sclerosing Pan Encephalitis (SSPE), a progressive neurological disorder characterized by inflammation of the brain (encephalitis), is the result of an inappropriate immune response to the measles virus or measles vaccination.

SSPE usually develops 2 to 10 years after the original viral attack. Some of the major signs and symptoms are mental deterioration, jerky movements, and seizures specially myoclonic type, involuntary movements, and/or behavioral changes, difficulty in walking, speech, and loss of cognition, respiratory distress and death.

Materials and Methods

During the ten years, from July 1991 to July 2001, we admitted 45 cases of (SSPE), at different stages of the disorder. Regardless of their stage of disease, for intervention, randomly, we used one of three drugs; Amantadin, Interferon alfa and Isoprinosine, administered to the patients, for between one month to one year. Fourteen cases received Amantadin, 15 Alfa interferon, and 16 were given Isoprinosine.

Results

While the results show all three drugs to be relatively effective, Isoprinosine showed four times more effectiveness than Amantadin and twice as much as Interferon.

Conclusion

The results showed Isoprinosine to be much more effective than Amantadin and Alfa interferon in treating the condition.

Objective

Cerebral palsy (CP), a common static motor neurological disorder of childhood with wide spectrum of underlying etiologies, can be demonstrated with different neuro imaging techniques. We undertook this study to investigate the diagnosis of intracranial lesions in children with CP and its correlation between clinical deficits and neuroradiological findings.

Materials and methods

In this prospective hospital-based study, the data of 120 patients with CP, aged below 18 years, referring to the neurology clinic of the Ali Asghar Pediatric hospital in Tehran was studied; data on their cranial neuroimaging findings was analyzed any possible association(s) between the gestational ages, prenatal history and neurological deficits were investigated.

Results

Of the 120 patients, 72 (60%) were male; 75% were aged below 7 years. Common predisposing factors were prenatal asphyxia, LBW, prematurity and toxemia of pregnancy. Of the 120 cases, 90%(107) had spastic CP, with the quadriplegic type being the most common (54%), followed by spastic paraplegia (21%); twenty-four patients (20%) had significant Preventricular Leucomalacia (PVL), a finding more common among those born pre-term.

Sixteen patients had hemiplegic CP, 14 of whom showed unilateral lesions on brain MRI imaging. Ten (8%) had extra pyramidal CP, a condition more common among term born infants, while six of the 10(72%) showed significant abnormalities on the basal ganglia. Cerebral atrophy was seen in 60 (50%) of patients and PVL in 20%; encephalomalacia, gliosis, middle cerebral artery infarcts, PVL and gliosis indicated hypoxia as a risk factor for CP. Extent of MRI lesions correlated with the severity of neurological deficits in CP lesions, which were more extensive in Quadriplegics and double hemiplegics rather than paraplegics, and among those delivered preterm as compared to those born at term.

Conclusion

Radiological findings were found to be closely related to the type of CP and the neurological deficits and gestational ages of patients with brain insult; we believe that MRI is helpful in delineating the underlying etiology, extent, severity and timing of insult to the developing brain and prognostication in CP patients.

Objective

The aim of the present study was to evaluate the etiology and frequency of neonatal seizure in hospitalized neonates.

Materials and Methods

In this descriptive, cross-sectional study, we evaluated 1295 neonates with seizures admitted to neonatal and NICU wards in our center. Data was collected on age, sex, birth weight, serum levels of calcium, glucose, and sodium, CT scan findings, history of maternal opium abuse, blood and cerebrospinal fluid culture, and analyzed using SPSS 13.

Results

Of a total of 1295 patients, 34 (2.62%) had seizure. Mean age was 14.03 ± 10.05 days (range, 1 to 29 days); twenty-five (73.5%) neonates were boys and 9 (26.5%) were girls. Of 34 neonates with neonatal seizures, 12 (35.3%), 11 (32.4%), 9 (26.5%), 7 (20.6%), and 3 (8.8%) had hypocalcemia, asphyxia, hypoglycemia, intracranial hemorrhage, and hypernatremia, respectively.

Maternal addiction, meningitis, and sepsis were found in 3 (8.8%), 1 (2.9%) and 1 (2.9%) of neonates, respectively.

Conclusion

The incidence rate of neonatal seizure in the neonates in our NICU and neonatal ward was 2.62%. Common causes of seizure in this study included hypocalcemia, asphyxia, hypoglycemia, intracranial hemorrhage, and hypernatremia. Maternal ddiction, meningitis and sepsis had the lowest prevalence.

Objective

Botulism is the acute, descending, flaccid paralysis that results when the neurotoxin of Clostridium botulinum blocks neuromuscular transmission. C botulinum toxin is the most poisonous substance that blocks neuromuscular transmission and causes death through airway and respiratory muscle paralysis; all forms of botulism manifest neurologically as asymmetric, descending, flaccid paralysis beginning with the cranial nerve musculature. Food-borne botulism results from the ingestion of food in which C botulinum has multiplied and produced its toxin.

Patient

We report a new case of food-borne botulism in a 7 year old boy with recurrent episodes of weakness, difficulty in wallowing and speech; bilateral ptosis and mydriasis. He had positive history of the same symptoms, documented twice before. The patient's samples were sent for detection of toxin of Clostridium botulinum, and toxin of C. botulinum, type A was found in his stool sample, confirming our diagnosis. This case was unusual report because our patient has not history of canned food ingestion and also because recurrent episodes of paralysis in this case are unusual findings in botulism.

Conclusion

In this report we want to emphasize that canned-food ingestion is not necessary for diagnosis of food-borne botulism and because delayed treatment leads to increase mortality and morbidity, treatment should be initiated promptly on the basis of clinical suspicion.

Mitochondrial myopathy, lactic acidosis, and sideroblastic anemia (MLASA) syndrome is a rare autosomal recessive disorder of oxidative phosphorylation and iron metabolism. The association between myopathy and sideroblastic anemia was initially reported in 1974. Here we report an 8.5 year old boy with normal cognitive function, suffering from chronic progressive weakness in his lower extremities, inability to walk and palor. Microcytic sideroblastic anemia, mild lactic acidosis and inflammatory myopathy (myositis) in muscle biopsy was detected and treated; the response to corticosteroid therapy and rehabilitation was excellent and the patient was ambulatory after four months.