ABSTRACT
Childhood epilepsy remains a challenge to treat. Despite the availability of antiepileptic drugs (AEDs), >25% of children with childhood epilepsy continue to have seizures. Conventional AEDs have been the mainstay of therapy for many years but are often poorly tolerated and have a tendency to interact with other drugs. Current American Academy of Neurology guidelines support the use of four of the newer AEDs (gabapentin, lamotrigine, topiramate, and oxcarbazepine) as adjunctive treatment of refractory partial seizures in children, based on class I evidence. This paper includes a summary of the results from a recent randomized, double-blind, placebo-controlled study, which shows that levetiracetam is also effective and well tolerated in this pediatric population, and provides evidence supporting its use in refractory partial seizures in children.
Subject(s)
Anticonvulsants/therapeutic use , Epilepsies, Partial/drug therapy , Amines/adverse effects , Amines/therapeutic use , Anticonvulsants/adverse effects , Carbamazepine/adverse effects , Carbamazepine/analogs & derivatives , Carbamazepine/therapeutic use , Child , Cross-Sectional Studies , Cyclohexanecarboxylic Acids/adverse effects , Cyclohexanecarboxylic Acids/therapeutic use , Double-Blind Method , Drug Therapy, Combination , Epilepsies, Partial/epidemiology , Fructose/adverse effects , Fructose/analogs & derivatives , Fructose/therapeutic use , Gabapentin , Humans , Lamotrigine , Oxcarbazepine , Practice Guidelines as Topic , Randomized Controlled Trials as Topic , Topiramate , Treatment Outcome , Triazines/adverse effects , Triazines/therapeutic use , United States , gamma-Aminobutyric Acid/adverse effects , gamma-Aminobutyric Acid/therapeutic useABSTRACT
The role of the Na(+)-Ca2+ exchanger was examined in isolated rat dorsal root ganglion (DRG) neurons. Neurons were dialyzed with the Ca2+ indicator Indo-1. Ca2+ transients were elicited by depolarizing the cells from -80 to 0 mV for 100 ms under voltage clamp conditions. In most cells (45 of 67), the decay of intracellular Ca2+ concentration ([Ca2+]i) could be fitted with a single exponential with a time constant of 2.43 s. In the remaining 22 cells, the decay of [Ca2+]i could be described with a double exponential with time constants of 0.76 and 11.84 s. In cells that displayed a biphasic [Ca2+]i relaxation, Na(+)-free medium caused resting [Ca2+]i to increase from 116 to 186 nM; the slow component of recovery to basal [Ca2+]i was nearly abolished in Na(+)-free medium or by application of 5 mM Ni2+. In 35 of 45 cells displaying a monophasic [Ca2+]i decay, omitting external Na+ increased the time constant of [Ca2+]i decay from 2.02 to 3.63 s. In the remaining 10 cells, Na(+)-free solution did not affect Ca2+ handling. The time constant of [Ca2+]i relaxation was voltage dependent. These findings demonstrate the important role of the Na(+)-Ca2+ exchanger in DRG neurons. Its presence was further confirmed both at the mRNA and the protein level.
Subject(s)
Carrier Proteins/metabolism , Ganglia, Spinal/metabolism , Neurons/metabolism , Animals , Calcium/metabolism , Calcium/physiology , Carrier Proteins/genetics , Electrophoresis, Polyacrylamide Gel , Electrophysiology , Ganglia, Spinal/cytology , Immunohistochemistry , In Vitro Techniques , Nickel/pharmacology , Polymerase Chain Reaction , Rats , Sodium/metabolism , Sodium/physiology , Sodium-Calcium ExchangerABSTRACT
We studied 200 female patients with multiple sclerosis (MS) to investigate whether pregnancy after the onset of disease influences long term disability. As an index of progression, we used the time between disease onset and wheelchair dependence. Patients who had at least one pregnancy after onset were wheelchair dependent after 18.6 years, versus 12.5 years for the other women (P < 0.0001). This difference remains statistically significant after correction for age at onset of disease.
Subject(s)
Disability Evaluation , Multiple Sclerosis/diagnosis , Pregnancy Complications/diagnosis , Activities of Daily Living/classification , Adult , Female , Follow-Up Studies , Humans , Middle Aged , Pregnancy , WheelchairsSubject(s)
Fasciculation/physiopathology , Muscle Cramp/physiopathology , Action Potentials , Humans , SyndromeSubject(s)
Dementia/diagnosis , Leukodystrophy, Globoid Cell/diagnosis , Adult , Diagnosis, Differential , HumansABSTRACT
We present a patient with adult-onset globoid cell leukodystrophy (GBL) who had almost complete deficiency of galactosylceramide beta-galactosidase. A brother of the index patient deteriorated neurologically and died at the age of 4, probably from the late-infantile form of the disease. In this family, two clinical types of GBL are probably different expressions of an identical genotype.
Subject(s)
Galactosylceramidase/deficiency , Leukodystrophy, Globoid Cell/genetics , Adult , Dura Mater/pathology , Female , Humans , Leukodystrophy, Globoid Cell/diagnosis , Leukodystrophy, Globoid Cell/enzymology , Magnetic Resonance ImagingABSTRACT
Computerized tomography (CT) and magnetic resonance imaging (RMI) showed cortico-subcortical atrophy as well as calcification of the basal ganglia and the cerebellar dentate nuclei in a patient presenting with cerebellar syndrome. RMI was particularly useful in imaging the demyelination of the periventricular white matter and the subcortical U fibres. A diagnosis of Cockayne's syndrome was made. This is an extremely rare hereditary disease of unknown pathogenesis. Defective recovery of DNA synthesis has been suggested.
Subject(s)
Cockayne Syndrome/diagnosis , Magnetic Resonance Imaging , Adult , Brain/diagnostic imaging , Brain/pathology , Cockayne Syndrome/diagnostic imaging , Humans , Male , Tomography, X-Ray ComputedABSTRACT
The MR findings in an adult patient with globoid cell leucodystrophy (GLD) or Krabbe's disease are presented. MRI showed a bilateral periventricular hyperintensity of the parieto-occipital white matter on the T2-weighted images. A hyperintense signal was seen bilaterally along the corticospinal tract. There was no immediate nor delayed contrast enhancement. The MR findings in this case of GLD are rather atypical.
