Ming-Liang Chou

Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy

Aim: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. Methods: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. Results: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. Conclusions: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.

Tuberous Sclerosis Complex With Multiple Intracranial Aneurysms in an Infant

Tuberous sclerosis complex is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of signs and clinical features. Occlusive vascular disease and aneurysms of the cerebral and visceral arteries were described in these patients. We report on an 8-month-old boy who manifested tuberous sclerosis complex with multiple intracranial aneurysms involving the internal carotid artery and middle cerebral artery. The unusual features of our case favor the concepts of the presence of an arterial dysplasia in tuberous sclerosis complex, and of developmental defects as the cause of intracranial aneurysms.

Late-Onset Pyridoxine-Dependent Seizures: Report of Two Cases

We report two cases of late-onset pyridoxine-dependent seizures with age onset at 14 and 9 months, respectively. Their intractable seizures were refractory to diazepam, phenobarbital, phenytoin, carbamazepine, valproic acid, and adrenocorticotropic hormone, but stopped soon after intravenous administration of pyridoxine. The psychomotor development was retarded in both cases. Pyridoxine dependency is one of the few treatable intractable seizures in infancy. It should always be kept in mind, even in patients with intractable seizures beyond the neonatal period, regardless of previous patterns of electroencephalography and seizure types and responsiveness to the conventional anticonvulsants.

Acute disseminated encephalomyelitis in children: a single institution experience of 28 patients.

The aim of this study is to describe the clinical, laboratory, and neuroimaging features, treatment and outcome of acute disseminated encephalomyelitis (ADEM) in Taiwanese children to compare with two series from United States of America and Japan. We retrospectively reviewed the medical records and magnetic resonance images of 28 children, 23 boys and 5 girls, with ADEM between January 2001 and December 2009. Their mean age at disease onset was 6 years 9 months. Twenty four children experienced a prodromal illness. There was no special seasonal distribution in our patients. They presented mostly with impaired consciousness and headache. Cerebrospinal fluid samples of 21 patients were analyzed and none showed intrathecal oligoclonal bands. Magnetic resonance imaging showed variable findings: lesions with abnormal signal changes frequently found in the subcortical white matter of frontal and parietal lobes. No patient showed cortical gray matter involvement. We also found a high rate of deep gray matter involvement including thalami and basal ganglia. Treating with steroids was usually associated with a rapid recovery and both intravenous high dose methylprednisolone and dexamethasone had the same effect. All patients survived. Twenty three patients recovered completely with only mild sequelae in the remaining five children.

Risk Factors of Respiratory Failure in Children with Guillain-Barré Syndrome

BACKGROUND Respiratory failure is rarely associated with Guillain-Barré syndrome (GBS) in children. The aim of the study was to determine the risk factors of respiratory failure in children with GBS to advance management. METHODS In this retrospective study, the variables that lead to respiratory failure were investigated in 40 children. The risk factors were compared for 4 children with intubation and 36 without. We also analyzed the specific treatments, including corticosteroids, intravenous immunoglobulin, plasmapheresis, and clinical status at discharge. RESULTS Four (10.0%) of the 40 children with GBS required mechanical ventilation. The need for mechanical ventilation was significantly related to the Hughes score at nadir (p<0.001), respiratory distress (p<0.001), and hypotension (p<0.001). Atypical presentation of symptoms such as croup, hoarseness, vomiting, ataxia, consciousness disturbance, and previous event of diarrhea were more predominant in patients younger than 6 years. Disability grades >3 at discharge were found in 15 patients (37.5%), and there was no mortality in the present case series. CONCLUSIONS Respiratory failure in childhood GBS was related to the Hughes score at nadir, respiratory distress, and hypotension. Atypical presentations of symptoms were more predominant in patients younger than 6 years. The prognosis in our series was good and not related to previous events. Understanding the risk factors of severe GBS will provide better treatment strategies and improve the outcomes.

Rectal Diazepam Solution Is as Good as Rectal Administration of Intravenous Diazepam in the First-aid Cessation of Seizures in Children With Intractable Epilepsy

BACKGROUND Acute seizures are readily recognizable episodes requiring urgent treatment. This study was conducted to compare the efficacy and safety of suppository use of rectal diazepam solution [Stesolid rectal tube (SRT), Alpharma, Inc., Lierskogen, Norway] with those of intravenous diazepam (IVD), Li Ta Pharma Co, Ltd., Taichung, Taiwan for control of acute seizures in children with intractable epilepsy. METHODS Subjects were patients, aged 1-18 years, with intractable epilepsy under at least three kinds of antiepileptic treatments. Caregivers were trained to rectally administer SRT or IVD (dosage varying from 0.2 to 0.5mg per kilogram of body weight) and to monitor respiration condition, seizure severity, and adverse drug effects. RESULTS Among the 24 subjects, 9 males and 15 females, treated for a period of 3 months, the ages ranged from 2 to 18 years, with a mean of 9.1 years. Seizure types were generalized tonic and/or clonic. Seizure frequency varied from once per week to 20 times per day. Twenty-one (87.5%) of them had mental retardation and/or developmental delay, and 103 of the 127 (81.1%) IVD administrations and 90 of the 103 (87.3%) SRT administrations resulted in rapid cessation of seizures within 10 minutes. Each first dose failed to control seizures in 24 and 13 episodes, respectively. A second dose of IVD achieved cessation of seizure in 21 of the 24 episodes and a second dose of SRT in 12 of the 13 episodes within another 10 minutes. Four episodes (3 with rectal IVD and 1 with SRT) of prolonged seizure beyond 20 minutes needed IVD injection at our emergency room. Sedation occurred in 17% of patients, which was attributed to IVD in 8% and SRT in 9% of patients. No respiratory depression was attributable to IVD or SRT. There was no significant statistical difference in efficacy and safety between these two forms of diazepam. CONCLUSION Rectal diazepam solution, administered by capable caregivers, is as effective and safe as rectal administration of IVD for children with intractable epilepsy.

Immunologic analysis and serum heavy metal levels in exacerbated Tourette syndrome

Hsieh M‐Y, Lee W‐I, Lin K‐L, Hung P‐C, Chou M‐L, Chang M‐Y, Huang J‐L, Wang H‐S. Immunologic analysis and serum heavy metal levels in exacerbated Tourette syndrome. 
Pediatr Allergy Immunol 2010: 21: e764–e771.
© 2010 John Wiley & Sons A/S

Transcranial ultrasound diagnosis of intracranial lesions in children with headaches

The diagnostic value of transcranial ultrasound for intracranial lesions in children with headaches was evaluated. From January 1995 to December 1998, 444 children with headaches for more than 2 weeks visited our Pediatric Neurologic and Neurosurgical Outpatient Clinics. A 2-MHz-sector sonographic transducer was used to perform transcranial ultrasound. Nineteen patients were lost to follow-up. Two patients, both 15 years of age, failed to have a successful examination. Thirteen of the enrolled 423 patients presented with headaches and other symptoms and positive neurologic signs and had positive magnetic resonance imaging findings of intracranial lesions correlating with their transcranial ultrasound findings. Twelve of the remaining 410 children complaining of isolated headache had abnormal transcranial ultrasound findings. Nine of the 12 patients were confirmed to have intracranial lesions by magnetic resonance imaging studies. Among the nine patients, five had brain tumors, two patients had hydrocephalus, one patient had intracerebral hematoma, and one patient had temporal arachnoid cyst. The other three patients were misinterpreted to have a suprasellar lesion by transcranial ultrasound. These three patients and the remaining 398 children were regularly followed for at least 12 months. All revealed no evidence of an intracranial lesion. The sensitivity and specificity of transcranial ultrasound to detect intracranial lesions for children with isolated headaches were 75% and 99.7%, respectively. The average saving from utilizing this technique was

Risk Factors for Delayed Neuropsychiatric Sequelae in Carbon Monoxide Poisoning: Ten Years' Experience in a Pediatric Emergency Department

222 U.S. dollars per person. We suggest that transcranial ultrasound study is a reliable, convenient, time-saving, and economic diagnostic tool in detecting intracranial lesions in children with headaches.

Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics

Objective: The occurrence of Delayed Neuropsychiatric Sequelae (DNS) after Carbon Monoxide (CO) poisoning is uncommon in children. Early identification of risk factors for the development of DNS in pediatric emergency departments is important. The objective of this study was to analyze risk factors for DNS after CO poisoning. Methods: We retrospectively analyzed children with CO poisoning admitted to the pediatric emergency department from 2001 to 2010. Clinical, demographic, and laboratory data were collected; chi-square and univariate analyses were performed to assess risk factors for DNS. Results: Among 68 children with CO poisoning, seven (10.3%) developed DNS. Clinical parameters such as Glasgow Coma Scales (GCS), Methemohemoglobin (MetHb), troponin-I, CPK level, myocardial injury, and neuroimaging abnormalities, were important associated risk factors for DNS. Decreased GCS level (OR = 0.701) and Methemohemoglobin (MetHb) ≥ 0.8% (OR = 19.54) are associated with DNS. Carboxyhemoglobin, and inadequate HBO therapy were not risk factor. Conclusions: Prompt treatment of CO poisoning and identify risk factor for DNS is still challenge in PED. Our data demonstrate that decreased GCS level and increased MetHb level were independent risk factors associated with DNS. Early recognition and prompt treatment is important to prevent further neurological damage.