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In the study by de Seze et al., the prevalence of SS was assessed in 60 patients with PPMS. Ten patients (16.6%) met diagnostic criteria for SS. The authors propose that SS should be systematically sought in patients with PPMS. Whether SS actually causes a disease that is difficult to distinguish from PPMS or whether the two diseases are associated is not clear. It is also unclear whether the 10% incidence of SS in PPMS studied here occurs only in this form of MS.
see page 1359
The accompanying editorial by Giovannoni and Thorpe emphasizes that despite including MRI in the new diagnostic criteria for MS it remains a clinical diagnosis. Both MRI and electrophysiologic criteria are nonspecific. Many inflammatory/vascular disorders can produce the dissemination in time and space that help to define MS.
see page 1357
Comparing the efficacy of different triptans in migraine
Adelman et al. applied stringent outcome criteria currently being advocated in migraine research to data from triptan trials. These criteria specify pain- and symptom-free at 2 hours and 24 hours sustained pain free. They report that in studies where rizatriptan was directly compared with sumatriptan, zolmitriptan, or naratriptan for the acute treatment of a migraine, rizatriptan was the most effective treatment as judged by these criteria.
see page 1377
Tics in school children: A marker for learning difficulties
Kurlan et al. examined 1,596 school children and found that 27% of those in special education had tics vs 19.7% in regular classes. The authors conclude that tics are common in school children and are associated with school dysfunction. Tics may reflect an underlying brain developmental disturbance that contributes to learning difficulties.
see page 1383
Sleep disorders in PD
Ondo et al. had 320 patients with PD complete the Epworth Sleepiness Scale (ESS), questions assessing the presence of restless legs syndrome (RLS) and falling asleep while driving, and a modified National Sleep Foundation survey. In 50.2% of patients the ESS score was increased (>10). Sleepiness correlated with longer duration and more advanced PD and with the use of any dopamine agonist. Falling asleep while driving was reported by 22.6% of currently driving patients.
see page 1392
Vigabatrin for infantile spasms
Elterman et al. compared low-dose with high-dose vigabatrin in patients <2 years of age with newly diagnosed infantile spasms. In the initial 2-week single blind phase of the study, high-dose patients responded significantly better than low-dose patients. Response rate (defined as seizure-free) increased after 2 weeks of treatment and was higher in those with tuberous sclerosis. Vigabatrin was well-tolerated in this 3-year study.
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see page 1416
Controlled trial of albuterol in fascioscapulohumeral dystrophy (FSHD)
Kissel et al. treated three groups of 30 patients with FSHD with albuterol (16 vs 32 mg/d) or placebo for 1 year. Although the studys primary endpoint (global strength) did not improve significantly, grip strength and muscle mass did improve. Albuterol was well-tolerated.
see page 1434
Head circumference and incident AD
Borenstein Graves et al. followed 1,869 nondemented individuals over age 65 for a mean of 3.8 years. The combination of small head circumference with APOE
4 strongly predicted earlier onset of AD with a hazard ratio of 14.1 (p= 0.0007) compared with persons without an
4 allele and with larger head circumference.
see page 1453
APOE genotype: Regionally specific effects
Hashimoto et al. found that hippocampal and amygdalar atrophy was exaggerated but whole brain atrophy was reduced with increasing number of APOE
4 alleles in patients with AD. The effect of APOE
4 allele on the neuronal changes was region-specific.
see page 1461
Nursing home placement in patients with dementia
Smith et al. examined predictors of time to nursing home placement in a community-based dementia sample. Cognitive and functional impairment were predictive of placement time. Median time from diagnosis to placement was 5.6 years. Nursing home placement time is a valid outcome in dementia clinical trials.
see page 1467
APOE genotype in neurodegenerative diseases: A unifying hypothesis
The Chapman et al. Medical Hypothesis seeks to explain the observations that APOE
4 advances the age of AD onset but does not have a major effect on progression rate, vs MS and ALS, where the
4 allele is associated with faster progression, but not with earlier onset. APOE genotype effects on neurologic diseases is explained in terms of ubiquitous cellular mechanisms. In diseases that are subject to diagnosis early in their course (e.g., MS), the phenotypic effect of the APOE genotype is mainly on the rate of progression. In those in which diagnosis is made after years of cumulative damage (e.g., AD), the predominant effect is on the age at onset.
see page 1482
Spontaneous resolution of syringomyelia
Kastrup et al. report a 61-year-old woman with typical symptoms and signs of syringomyelia whose cystic enlargement of the entire spinal cord collapsed spontaneously without surgery. Clinical findings did not change. Such spontaneous collapse has also occurred in syrinx associated with Chiari malformations.
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see page 1519
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