Pediatric prolonged seizures and status epilepticus are medical emergencies necessitating immediate life-support and seizure-control measures. A systematic review of published data on the management of prolonged seizures and status epilepticus showed that buccal midazolam was significantly more effective than rectal diazepam, reaching a seizure-control rate of 70% and recurrence rate of 8%. Intranasal lorazepam was as effective as intramuscular paraldehyde in a cost-restrained setting. In refractory status epilepticus, both intravenous midazolam and valproate were equally effective to intravenous diazepam, with valproate exhibiting significantly faster seizure cessation and safer profile than diazepam, even in infancy. In conclusion, buccal midazolam is efficacious and safe thanks to its convenient route of administration, which may serve as first-line in the treatment of prolonged seizures. Intranasal lorazepam is an effective, easy-to-use, and safe drug for prolonged seizures. Intravenous valproate exhibits favorable efficacy and safety profile as third-line in status epilepticus, refractory to diazepam and phenytoin.

No abstract

Glial fibrillary acidic protein (GFAP) is the major structural protein of the intermediate filaments found in glial cells. Increased levels in the cerebrospinal fluid (CSF) have been found to indicate gliosis. Neurofilament (NFL) is a structural element of neurons, mainly found in large myelinated axons. Its presence in the CSF has been suggested to reflect destruction of axons. The aim of this study was to see if GFAP and NFL in the CSF of children with neurological disabilities and an abnormal signal on magnetic resonance imaging (MRI) of the cerebral white matter could be used to clarify the underlying neuropathology. The potential of GFAP and NFL to differentiate a progressive disease from a stationary disorder was investigated, as was the correlation with disability and clinical findings. CSF from 26 children, eleven with progressive and 15 with non-progressive disorders, was analysed. GFAP was increased in all, interpreted to reflect gliosis. NFL was elevated in seven and considered to indicate ongoing neuroaxonal damage as all but one patient were found to have a progressive disease. GFAP did not differentiate between progressive and non-progressive disorders, although low levels were found in stationary and high levels in progressive disorders. The severity of the disability correlated with the NFL levels, but not with the concentration of GFAP.

No abstract

Disorders of the cerebral white matter in children constitute a heterogeneous group and the diagnostic work is often complicated. Clinical and radiological characteristics can provide diagnostic clues but there is a need for further diagnostic methods. This study focused on assessing neurochemical "markers" in the cerebrospinal fluid considered to reflect damage to white matter components such as myelin and glial cells as well as neurones with their axons and synapses. The aim was to evaluate whether they contributed to the elucidation of pathogenic processes and the direction of further diagnostic efforts. Seventeen of the 26 cases had increased levels of the glial cell marker ganglioside GD3, indicating gliosis, or of the CNS myelin marker sulfatide, indicating myelin disturbance. As signs of disturbed maturation or sustenance, the nerve cell markers GD1 b, GT1 b and total gangliosides were reduced, as was the synapse marker GD1a. Increased 5-HIAA indicated increased serotonergic turnover. Children with an increased level of the axonal marker Tau protein had a progressive disease whereas GD1a was reduced in the progressive group (n = 11). In contrast, GD3 and HVA were increased in the non-progressive group (n = 15). The chemical profiles were found to be useful, in combination with clinical and radiological findings, when investigating children with white matter abnormalities.