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Johnston and Goldstein (p. 1009) compared findings in 569 patients who had carotid imaging with angiography vs a smaller number of imaged patients with ultrasound, CTA, and MRA at both an academic and community hospital. Using angiography as the gold standard, misclassification using ultrasound alone occurred in 28% but was reduced by MRA to 8%with similar results in both hospitals.
The accompanying editorial by Norris and Rothwell (p. 990) considers this and other evidence that indicates the need for caution in making treatment decisions without angiography.
Congenital muscular dystrophy
A small proportion of children weak from birth prove to have muscular dystrophy, and subgroups of these also have CNS malformations. Cormand et al. (p. 1059) studied 21 families with 29 affected subjects defining two separate phenotypes that correlated well with genetic linkage: muscleeyebrain disease on chromosome 1p and the WalkerWarburg syndrome that is unlinked to chromosome 1p.
The accompanying editorial by Mendell (p. 993) traces the development of the concept of congenital muscular dystrophy and summarizes the six to eight different types that have been defined.
Migraine as a channelopathy: Evidence from genetic linkage and from EMG
Mutations in the P/Q type calcium channel
-subunit on chromosome 19p13 cause familial hemiplegic migraine. Terwindt et al. (p. 1028) studied families with typical migraine with and without aura for sharing of alleles on chromosome 19p13 and found that sibs with migraine with aura shared alleles significantly.
Ambrosini et al. (p. 1038) found single fiber EMG abnormalities in patients with migraine with aura, suggesting that muscle fibers could also express abnormalities in P/Q type calcium channels.
Oxandrolone in Duchenne dystrophy (DD)
Prednisone increases strength in DD but is associated with side effects in many patients. Fenichel et al. (p. 1075) studied oxandrolone vs placebo in 51 patients with DD and found that it improved strength as measured by quantitative muscle testing. It had no detected side effects. The improvement with oxandrolone was less than that with prednisone.
CJD after a new brand of dural graft
Previous cases of dural graft iatrogenic CJD came from a single brand source. Hannah et al. (p. 1080) document CJD from a new brand source. The patient who served as donor had a CJD-like illness.
Does cognitive impairment worsen driving skills in MS?
Schultheis et al. (p. 1089) tested MS patients with cognitive impairment vs those without and vs normals with two computerized driving tests. Many MS patients with impaired cognition were at high risk of accident. The study did not establish that such patients actually have more accidents.
Educating neurologists to prevent mistakes: Malpractice claims vs medical error?
Glick (p. 1099) proposes that malpractice claims may suggest targets for educating clinicians, examining malpractice claims made against neurologists. He reviewed 253 claims against neurologists: half were for misdiagnosis, one third for mistreatment, others for lapses in professional behavior (10%).
The accompanying editorial by Holloway and Panzer (p. 991) notes the innovativeness and interest of Glicks approach. However, they point out that finding ways of identifying the far more frequent medical errors that escape detection and do not cause injury will provide better targets for educating clinicians (and for making changes in systems). Protection of health care providers from the risks of reporting error is a far bigger and more important legal challenge.
Rectal diazepam: How high a dose is safe?
Brown et al. (p. 1112) report that much larger than recommended doses (188 to 256% higher than the established maximum dose recommended) were given in the study that found it a successful treatment for acute repetitive seizures. No serious effects were notedparticularly no respiratory depression.
Compulsive toasting
The Hahm et al. patient with pathological collecting behavior of toy bullets (2001;56:398400) prompted neurologist Oliver Sacks correspondence on toasting (p. 1118).
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