Abnormal brain MRI in a case of acute ataxia as the only sign of abdominal neuroblastoma

Ataxia is a movement disorder that may manifest an acute, intermittent, non progressive or chronic progressive course. Ataxia alone is rare as a paraneoplastic sign, especially if it is due to neuroblastoma [abdominal or chest]. We report an abdominal neuroblastoma in a two-year-old girl presenting with only acute ataxia and abnormal neuroimaging. Brain MRI showed abnormal signal finding in the medulla, pons, corticospinal tract and the periventricular space. In the abdominal CT, a mass was detected in the right adrenal gland with calcification and the histopathologic examination re-vealed neuroblastoma. We suggest in children with acute ataxia, with or without opsoclonus-myoclonus, neuroblastoma should be considered

Intractable seizure disorders: efficacy of the classic ketogenic diet

The ketogenic diet is a high-fat, low carbohydrate, adequate protein diet, developed in the 1920s for the management of intractable seizure disorders in children. To evaluate efficacy and tolerability of the classic ketogenic diet, we analyzed records of the children started on the diet from 1999 to 2006 at the Mofid children's hospital. The subjects were 87 children, mean age 55 months. Before initiation of the diet, 55% of the patients had seizures, at least 1-4 times per day, 36%-5 or more per day and 9%-2 to 4 times per week. Mean number of Anti Epileptic Drugs [AEDs] tried for them was 8 and 67% were receiving three or more drugs. The ketogenic diet showed drastic improvement, with at least 50% reduction in seizure frequency in 87% of our patients, 39% of whom showed complete seizure control in the third month. After one year, in 80% of the patients who returned, improvement continued, with 26% of them being seizure free; besides, 23% had one AED decreased, 36% had two or three AEDs decreased, and 25% [one child] had all AEDs discontinued. Of the 30 improved cases, 20%, at the end of the first year, had improved behavior as well, and 23% of them had become more alert. The medium diet duration of the improved group was 15 months. The improvement in our patients, low side effects, and the duration of diet by families reveal that the ketognic diet can still be a very useful alternative therapy in certain epileptic children

Comparative effects of diazepam infusion and divided doses of diazepam on the treatment of absence status epilepticus

Absence status epilepticus [ASE] is a common form of nonconvulsive status epilepcticus. It is characterized by loss of consciousness with spike and wave discharges in EEG simultaneously. The most effective treatment of ASE is diazepam, either infusion or in divided doses; the former is more expensive since patients must be admitted in an Intensive Care Unit. The aim of this study was to evaluate and compare the efficacy of diazepam infusion and parenteral diazepam in divided doses in the treatment of ASE. This randomized controlled clinical trial, enrolled 20 patients with absence status epilepticus. Diagnosis was made based on the clinical manifestations and electroencephalogram [EEG]. Prior to treatment, all patients underwent EEG and imaging. Patients were randomized to receive 0.2 mg/kg/h diazepam infusion or 0.2 mg/kg in six daily doses. Clinical and EEG improvements were considered to be optimal responses. Of the 20 patients studied, 13 [65%] were boys and the remaining 7[35%] were girls. There were no differences between the two groups regarding age and sex [non-significant]. Following treatments after 48 hours, 1 week and 1 month respectively, clinical improvement in previous problems [loss of consciousness, ataxia, behavior and speech problems] and EEGs was similar in both groups [p=1]. There were controlled seizures in 18 [90%], abnormal CT scans in 5 [25%], abnormal EEGs after treatments in 6 [30%] cases; however no significant differences were seen between the two groups. This study demonstrates that there are no significant differences between treatments of ASE with diazepam infusion and parenteral diazepam in divided doses. Treatment of ASE, with divided doses of diazepam is easier, less expensive and patients do not require to be hospitalized in an Intensive Care unit

Primary central nervous system lymphoma

Primary central nervous system lymphoma [PCNSL] is an extremely rare condition in childhood. We report the first case of PCNSL in a child in Iran. A nine-year-old boy was referred to Mofid Hospital with the history of headache of four months and seizure of 2 months duration. Magnetic resonance imaging of the brain revealed a hyper-intense lesion in left fronto-parietal area with secondary satellite lesions. Biopsy of the brain mass was performed. Pathologic findings showed brain lymphoma and immunohistochemistry confirmed this diagnosis. The treatment started with intrathecal and systemic chemotherapy in combination with radiotherapy

Medical therapy in childhood psychocognitive problems

Neurodevelopmental disability is one of the most common problems of children referred to Pediatric Neurology Clinics. These children may suffer from speech delay, intellectual deficiency and behavioral disorder. Some patients with neurodevelopomental delay, especially those with intellectual disability and speech problems, have abnormal electroencephalograms, without clinical seizure. It seems that treating these patients with antiepileptic drugs normalizes the EEG, thereby preventing the electrical paroxysmal discharges that could be harmful for the developing brain. Several studies reported the use of Valproate, Lamotrigine and Corticosteroid in suppression of subclinical epileptiform discharges and improvement of developmental behavioral conditions. In our experiences, oral Na-Valproate was effective in developmental behavioral condition of children with neurodevelopmental delay and elimination of subclinical EEG discharges after 18 months of treatment. Also we used high dose intravenous methyl - prednisolone in a group of children with neurodevelopmental delay and electrical status epilepticus during slow - wave sleep without clinical seizure. In these children results of the appropriate neuro-metabolic tests and magnetic resonance imaging of the brain revealed no abnormality. Because no underlying etiology could be determined, isolated non convulsive status epilepticus was established. After treatment no significant response was observed in these group of children. In another study we used Lamotrigine in children with neurodevelopmental delay, abnormal epileptiform discharges but without clinical seizures. Our results revealed Lamotrigine provides effective control of both subclinical epileptiform discharges and behavioral disorder, without improvement in their cognition. Further studies are needed to investigate and confirm the cognitive and behavioral effects of Lamotrigine in children with psychomotor retardation

Acute combined demyelination of CNS and PNS: a case report

Acute disseminated encephalomyelitis [ADEM] and Guillain-Barre syndrome [GBS] are both para infectious demyelinating disorders. While ADEM almost always affects the CNS, GBS affects the PNS. The combined demyelinating process - demyelination of both upper motor neuron [UMN] and lower motor neuron [LMN] - occurs very rarely. Here we report a case of severe combined peripheral and central demyelination, in which the former disorder was preceded by the latter

High and low dose IVIG therapy in guillainbarre-syndrome children: a comparison

Acute inflammatory demyelinating peripheral neuropathy [Guillain-Barre-Syndrome] is by far the most common cause of immune-medicated peripheral nervous system disease in children; with the near disappearance of poliomyelitis, GBS is responsible for the great majority of cases of acute flaccid paralysis. So far, in several controlled studies, corticosteroids, plasmapheresis and IVIG have been utilized in pediatric patients, afflicted with GBS. Regarding IVIG therapy, two methods have been used; the high dose [1 gr/kg/day for 2 days], and the low dose [400mg/kg/day for 5 days]. Review of literature shows that a faster rate of recovery can be accomplished in patients who receive total dose of IVIG in 2 days as compared to the dose being given over 5 days. In this study we have compared these two types of treatment in an investigation, conducted in the Mofid Children Hospital on pediatric patients who had sudden onset of acute flaccid paralysis, and were diagnosed as having GBS. Based on histories, physical examination and electrodiagnosis, subjects were divided in two groups, the high dose IVIG treatment, 1gr/kg/day for 2 days [experimental group], and the low dose IVIG treatment, 400 mg/kg/day for 5 days [control group]. Statistical analyses were then carried out using the appropriate software. Result of this study showed a faster rate of recovery for patients in the high dose IVIG group; in this group duration of weakness of limbs was shorter and returning of DTR was faster than in controls. In fact, in this type of treatment, the relationship between high dose IVIG therapy and drug side effects was not significant. Base upon the finding in the present study, we conclude that the high dose IVIG therapy is superior to low dose, in view of faster duration of recovery and shorter hospital stay. Also we may infer that shorter hospital stay could be a factor in reducing of more nasocomial infection. In conclusion, we suggest using high dose IVIG treatment of choice in GBS

100 children with acute ataxia; a survey in Mofid children's hospital

The term "Ataxia" refers to disturbances of body posture and movement that are normally controlled by the cerebellum, frontal lobes and the posterior columns of the spinal cord. The primary symptom and the most prominent feature of ataxia is abnormal gait which is characterized by lurching and wide base walking. Ataxia was considered acute, if it had occurred within the two preceding weeks. Knowing how frightening acute-onset Ataxia is for the family is not surprising that the condition prompts an immediate visit to the physician. In view of the lack of information in our country, on the etiology of sudden-onset Ataxia, the authors enrolled 100 children with the chief complaint of acute loss of equilibrium, who came to the attention of the Pediatric Neurology Department over a two year duration [Sept.2001-Sept 2003]; they were admitted to the Mofid Children's' Hospital and all necessary investigations were carried out. The results revealed that Acute Cerebellar Ataxia was the most common cause of the problem, the second most frequent being drug intoxication, which most commonly occurred in patients, 2-4years old. The remaining causative factors in order of descending frequency consisted of infectious polyneuropathy, migraine, opsoclonus-myoclonus, brain tumor, acute disseminated encephalomyelitis, multiple sclerosis, and epilepsy

Moyamoya: report of a pediatric case

Moyamoya [a Japanese term, meaning 'hazy things'] was first described by Takeuchi In 1963. Two forms of this disease have been distinguished: 1-Primary moyamoya, or moyamoya disease, with a strong hereditary predisposition and girls are more frequently affected. 2-Secondary moyamoya, or moyamoya syndrome, which is caused by a variety of underlying diseases. The Japanese scientists have classified moyamoya into four types: hemorrhagic, epileptic, infarct, and transient ischemic attack. Herein, we introduce an 8-years-old girl with the chief complaint of speech disorder. In her physical examination, we detected expressive aphasia. and right-sided central facial palsy. After a few days, right hemiplegia and cortical blindness appeared as well. Gradually she was totally unable to move and was transferred to the ICU because of loss of consciousness. MRI showed diffuse hyper signal lesions in the left temporoparietal and bilateral occipital area. MRA showed narrowing of the internal carotid artery and abnormal collaterals [moyamoya vessels]. After indirect bypass surgery [EDAS], she Is now able to sit, walk, run and speak. There are rare angiographically proven moyamoya cases. To our knowledge this was the first EDAS in Iran and a rare case of moyamoya with a dramatic response to operation

Developmental age of children in Tehran first year of life in tehran

The differentiation of human being in the life that leads to somatic, mental and social maturation is called development. The rate of development in childhood is more rapid than other stages of life, particularly during the first year, which is the fastest. We decided to evaluate the developmental quotient in 6500 infants, because to the best of our recollection, there has not been a longitudinal study accessing this subject in the first year of life in a large group of children in our country. This shortcoming prompted us to undertake this investigation. We studied the developmental age in respect, of grasping, crawling, sitting, walking and social age. The cases that have been studied were consisted of the healthy children who were referred to Tehran health and vaccination centers. Those who had a definite organic diseases or a clear cut neurodevelopment delay were excluded. Therefore, the infants who were considered completely healthy children were enrolled in our study. To evaluate the developmental age of different skills, we used "Munich functional diagnostic" as our assessment test. The result showed that developmental age had relationship to the following factors: maternal educational level, socioeconomic status of the family, the environment which the child was taken care there, and supplementation of iron, vitamins and additional food. At the conclusion of our study we found that the age of development in Iranian children was earlier than Germans infants during the first year of life, as median of developmental quotient was 115.41. Also it was true about the median age of acquiring different skills as follow: Grasping, crawling, sitting, walking, perception, speech reception, speech expression, and social interaction