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Testosterone and Cognition
Lu and colleagues evaluated testosterone therapy on cognition, neuropsychiatric symptoms, and quality of life in male patients with mild Alzheimer disease and healthy elderly men. For patients with Alzheimer disease, importantly, the testosterone-treated group had significantly greater improvements in the scores on the caregiver version of the quality-of-life scale.
Apolipoprotein E and Dementia in Parkinson Disease
Huang et al report after a meta-analysis that the apolipoprotein E ε4 allele is associated with a higher prevalence of dementia associated with Parkinson disease.
Steroid-Responsive Encephalopathy With Autoimmune Thyroiditis
Castillo and colleagues characterize the clinical, laboratory, and radiologic findings in patients with steroid-responsive encephalopathy with autoimmune thyroiditis and report that the findings are more varied than previously appreciated. Misdiagnosis at presentation is common. This treatable syndrome should be considered even if the thyroid-stimulating hormone levels and erythrocyte sedimentation rate are normal. Editorial perspective is provided by J. Y. Chong, MD, and Lewis P. Rowland, MD.
Levodopa-Induced Dyskinesia in Parkinson Disease
Van Gerpen et al report that levodopa dyskinesias can be expected to develop in nearly 60% of patients after 10 years, but these will be severe enough to require medication adjustments in only 43% of patients (Figure).
Kaplan-Meier estimate and 95% confidence interval for the probability that a patient with Parkinson disease who is taking levodopa will be free of dyskinesias of any severity.
Trial of Etilevodopa in Parkinson Disease
The Parkinson Study Group found that despite the theoretical pharmacokinetic advantage of etilevodopa, there was no improvement in total daily time to “on,” number of response failures, or “off” time compared with levodopa.
Nondegenerative and Nonvascular Dementia
Knopman and colleagues show that nondegenerative and nonvascular dementia causes in their cohort of patients were more common than expected in patients with younger onset of dementia. None of the patients with dementia reverted to normal with treatment of the putative reversible cause.
Synuclein Abnormalities and Clinical Outcome
Holtzer et al found that there is no clear relation between quantitative neuropathologic indices of Lewy body abnormalities and clinical outcome. The variability of clinical features in Alzheimer disease was not related to the presence or degree of Lewy body abnormalities.
Vertebral Artery Compression of the Medulla
Savitz et al present the largest collection of patients with medullary vascular compression. Further studies are needed to estimate its frequency, natural course, and preferred management.
White Matter Hyperintensity Volume and Decreased Cognitive Functioning
Au and colleagues report in their cohort of patients that nondemented individuals with large white matter hyperintensity, as compared with those with less or no white matter hyperintensity, performed significantly worse in cognitive domains generally associated with the frontal lobe and, to a lesser extent, the medial temporal area.
Brain Changes With Congenital Muscular Dystrophy
Mercuri et al show magnetic resonance imaging changes in brain in patients with congenital muscular dystrophy and mutations in the fukutin-related protein (FKRP) gene. The severity of brain involvement broadly reflected the severity of the disruption of the α-dystroglycan glycosylation. This study further highlights the central role that dystroglycan has in neuronal migration.
G93C Mutation in Superoxide Dismutase 1
Régal and colleagues found that the G93C mutation in the superoxide dismutase 1 (SOD1) gene was associated with a purely lower motor neuron phenotype without bulbar involvement and with longer survival compared with other subgroups of amyotrophic lateral sclerosis. Their findings demonstrate the independent prognostic value of the G93C mutation.
Absence of Rapid Eye Movement Sleep Behavior Disorder in a Familial Tauopathy
Boeve et al provide convincing evidence against nigral degeneration being the primary cause of a rapid eye movement behavior disorder in a familial form of a tauopathy. The difference in frequencies of a rapid eye movement behavior disorder associated with the synucleinopathies compared with the tauopathies suggests differences in the selective vulnerability of brainstem circuits between the synucleinopathies and tauopathies.
Phenotype With Compound Heterozygous Parkin Gene Mutations
Deng and colleagues report on the variable phenotypes in patients with mutations in the parkin (PRKN) gene causing autosomal recessive early-onset Parkinson disease. Their findings support the view that complex gene-environment interactions play a role in the pathogenesis of PRKN early-onset Parkinson disease.
Phenotypes With Sjögren-Larsson Syndrome
Lossos et al report significant clinical variability and the nonprogressive neurological course of Sjögren-Larsson syndrome in adult siblings with the same ALDH3A2 genotype.
This Month in The Archives of Neurology. Arch Neurol. 2006;63(2):171–172. doi:10.1001/archneur.63.2.171
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