Psychomotor delay, a possible rare presentation of moyamoya disease

Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemic strokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily, an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoya refers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentation of moyamoya disease. She was diagnosed by magnetic resonance imaging [MRI] and magnetic resonance angiography [MRA]

Use of a-interferon, amantadin and isoprinosine in subacute sclerosing panencephalitis [SSPE]: comparing the effectiveness

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

Neuronal migration disorder; a case report of subcortical band heterotopia associated with corpus callosum agenesis

Subcortical band heterotopia [SBH] or 'double cortex' is a congenital brain abnormality that results from aberrant migration of neurons during development of the cortex. MRI shows a continuous band of heterotopic gray matter located between the cortex and ventricular walls, separated from them by a thin layer of white matter. The condition is quite rare, found predominantly in females, and is occasionally familial with an X-linked dominant inheritance. Corpus callosum agenesis is another brain abnormality, more common than SBH, diagnosed during neurological examinations for developmental delay. We report a 6-month-old boy with SBH and corpus callosum agenesis associated with uncommon clinical and radiological findings such as polymicrogyria and periventricular cystic area

Breath-holding spells: an analysis of 43 cases

Breath holding spells, very often misinterpreted as epileptic seizures, are most common in children aged 6 months to 6 years of age. In this investigation, we sought to prospectively document the natural history of breath holding spells [BHS] among children with cyanotic, pallid and mixed type BHS referred for neurological consultation. This was a cross-sectional study in which a total of 43 children [23 boys, 20 girls] with BHS, admitted to the out patient clinic of the Children's Hospital Medical Center, between Sept 1998 and June 1999, were enrolled. A structured interview was under taken at the time of initial consultation to confirm BHS and its type, associated phenomenon, family history, sex and age at initiation of spells. Laboratory, electroencephalographic and electrocardiographic tests were done. Patients were between 1.7 and 42.8 months [mean age 18.4 months]. In 76.8% of cases, BHS began during the first 12 months of age. Anger and pain were the most common triggering factors [65.1%].A positive family history of BHS was identified in 51% and parental consanguinity was found in 30% of cases. The spells were cyanotic in 79.1% [34 children]. 78% of cases were iron deficient and 53% of cases had iron deficiency anemia. The results of this study emphasize the role of genetic factors in BHS; measurement of hemoglobin and serum ferritin is recommended all such cases

Blood pressure distribution among healthy schoolchildren aged 6-13 years in Tehran

To obtain blood pressure distribution for Iranian children, we assessed 10,288 students aged 6-13 years [4871 boys and 5417 girls] in Tehran. Mean systolic and diastolic blood pressure showed incremental increases with age, weight and height in both sexes. Mean increases in systolic blood pressure for boys and girls were 1.7 and 0.8 mmHg per year respectively and for diastolic blood pressure were 0.7 and 0.9 mmHg respectively. According to Second Task Force [STF] criteria, 4.9% of boys and 3.5% of girls had significant systolic hypertension and 10.1% of boys and 3.3% of girls had significant diastolic hypertension. Mean systolic and diastolic blood pressures were higher than STF reports, especially among boys

[Myoclonic encephalopathy]

The dancing eye syndrome or Opsoclonusmyoclonus syndrome is so called because of the bizarre eye movements which are almost invariably present in this rare condition. They are jerky and correspond with jerky movements of the limbs particularly the arms. The cause of entity is not known with certainty but it is thought to be an autoimmune condition. In approximately 10 percent of cases the condition develops as complication of neuroblastoma. In this paper we introduce an 18-month old girl. A previously well child becomes irritable and unsteady, had coordinated jerks of arms and legs, and abnormal eye movements. Urine VMA, CSF analysis and EEG were normal. The final diagnosis was Opsoclonus-myoclonus syndrome and the patient was treated with prednisolon and IVIg

[ prevalence of hypoparathyroidism among patients with major thalassemia aged above 10 years]

Major Thalassemia is a chronic hemolytic anemia. It is the most common hemoglobinophathy disorder in Khuzestan province. Hypoparathyroidism is one of its endocrinologic complications due to Iron deposition especially in the second decade of life. Early diagnosis of this complication could prevent other sever disorders such as seizures, osteopenia, and osteoporsis. In a cross sectional study, 96 patients with major thalassemia at the age above ten years referred to Shafa Thalassemia Center, Ahwaz, in addition information about age, sex, weight, height, duration and route of desferral injection, duration of transfusion therapy, vitamin D and calcium consumption, social and educational level of parents, history of splenectomy, any history of seizures, tingling, chvostek, trousseau, carpopedal signs, plasma level of calcium, phosphorus, alkaline phpsphatase, PTH, BUN, Cr, ferritin, and total protein were obtained using questionnaires. In ninety'six patients with major thalassemia the prevalence of hypoparathyroidism was%27/1 in 11-39 yearolds[average 19.3 years]. All patients had low serum calcium, PTH, high serum phosphorus and low to normal alkaline phosphatase. In 26 patients with hypoparathyraidism, 46% had tingling, 38.4% positive chvostek, 30.7% positive trousseau and 11.5% carpopedal spasm. 69.2% of the patients had only sign, whereas 30.8% showed clinically no signs. The average level of serum ferritin in thalassemia patients without hypoparathyroidism was 2770 ng/ml, in hypoparathyroid patients 5020 ng/ml. From 10 patients with irregular desferral consumption 7 patients had overt hypoparthyroidism. Significant correlation was seen between irregular desferral consumption, high ferritin level and hypoparathyroidism. Screening for hypoparathyroidism is recommended in children with major thalassemia aged above 15 years once every 6 months or yearly with measurement of plasma level of PTH, Ca and alkaline phosphatase

Auditory Brainstem Responses [ABR] in hyperbilirubinemic newborns

To evaluate the changes of ABR values in hyperbilirubinemic newborns, 85 cases with severe jaundice [total bilirubin levels over 16 mg/dL and direct bilirubin less than 2 mg/dL] were selected from those admitted to Children's Medical Center by simple sampling method. These infants had no other problem except jaundice. ABR was taken before treatment in all cases and in some [10 cases] after exchange transfusion or phototherapy as treatment modalities. 41 neonates [48%] had abnormal ABR values, such as increased hearing threshold, prolonged latencies of waves I, III and V [p- value<0.05], prolonged interpeak latencies of I- V and III-V [p-value<0.05], absence of waves and abnormal wave morphology. These findings could be indicative of both peripheral [8th nerve] and brainstem disturbances. After treatment ABRs showed improvement in some aspects, such as latencies of waves I, ITI and V [p value<0.05], but not in interpeak latencies [I-III, III-V and I-V] and hearing thresholds. In accordance with previous studies, there was no association between serum total bilirubin concentration and ABR test results. This could be interpreted as low significance of serum total bilirubin as a criterion for early prediction of bilirubin induced encephalopathy