Neurology
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Randomized Controlled Trial Multicenter Study
Silent cerebral infarct definitions and full-scale IQ loss in children with sickle cell anemia.
To evaluate whether application of the adult definition of silent cerebral infarct (SCI) (T2-weighted hyperintensity ≥5 mm with corresponding T1-weighted hypointensity on MRI) is associated with full-scale IQ (FSIQ) loss in children with sickle cell anemia (SCA), and if so, whether this loss is greater than that of the reference pediatric definition of SCI (T2-weighted hyperintensity ≥3 mm in children on MRI; change in FSIQ -5.2 points; p = 0.017; 95% confidence interval [CI] -9.48 to -0.93). ⋯ The adult definition of SCI may be too restrictive and was not associated with significant FSIQ decline in children with SCA. Based on these findings, we find no utility in applying the adult definition of SCI to children with SCA and recommend maintaining the current pediatric definition of SCI in this population.
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To elaborate the risk of headache among adolescent survivors exposed to terror. ⋯ Exposure to terror increases risk of persistent weekly and daily migraine and TTH in adolescent survivors, above expected levels. The terrors of other violence may similarly increase the risk of frequent headaches. After severe psychological traumas, interventions may need to address survivors' pain to hinder chronification.
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A rare cause of familial frontotemporal dementia (FTD) is a mutation in the CHMP2B gene on chromosome 3 (FTD-3), described in a Danish family. Here we examine whether CSF biomarkers change in the preclinical phase of the disease. ⋯ Increased NfL levels in presymptomatic individuals and in symptomatic patients with FTD-3 indicate a continuous process of neurodegeneration from the presymptomatic to symptomatic state. Although not specific for FTD-3 pathology, our data suggest that CSF NfL could serve as a valuable biomarker to detect onset of neurodegeneration in FTD-3 mutation carriers.
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Multicenter Study
Multicenter evaluation of neurofilaments in early symptom onset amyotrophic lateral sclerosis.
To examine neurofilament (Nf) concentrations according to symptom onset and clinical diagnostic certainty categories of amyotrophic lateral sclerosis (ALS). ⋯ This study provides Class II evidence that CSF and serum Nf concentrations discriminate ALS with early symptom onset from other neurologic diseases.
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To assess longitudinal trends in shortages of generic drugs used for neurologic conditions over a 15-year period in the United States. ⋯ Continued drug shortages may compromise the care of patients with neurologic conditions. Manufacturers, together with professional organizations, patient advocacy groups, and the government, need to continue to address this issue, which may escalate with a growing burden of neurologic disease.