Jensen and St Louis present in this salient review key decision points in the management of patients with acute cerebellar stroke, with particular emphasis on clinical and imaging issues.
Glatzel and colleagues provide new clinical and basic science insights into the pathogenesis of human prion diseases. Their review discusses current concepts and controversies surrounding the basic biology of prions.
Anderson and colleagues report in a comprehensive study that stimulation of either the globus pallidus internalis or the subthalamic nucleus improves many features of advanced Parkinson disease. It is premature to exclude the globus pallidus internalis as an appropriate target for deep brain stimulation in patients with advanced disease. Editorial perspective is provided by Michael S. Okun, MD, and Kelly D. Foote, MD.
Mean ± SEM Unified Parkinson’s Disease Rating Scale motor score off (circles) and on (triangles) levodopa therapy and on globus pallidus internalis (filled, n = 10) or subthalamic nucleus (open, n = 10) stimulation.
Biernacki and colleagues find that interferon beta mediates production of nerve growth factor at the level of the blood-brain barrier, acting as an immunomodulator or directly on neural cells. It is another potential mechanism contributing to the magnetic resonance imaging–defined effect of interferon beta on brain atrophy when given early in the course of multiple sclerosis.
Sastre-Garriga and colleagues report that metabolite changes, which differ in cortical gray matter and in normal-appearing white matter, occur in early primary progressive multiple sclerosis and are linked with disability.
Villar and colleagues describe results using a new oligoclonal IgG band detection method to show intrathecal IgG synthesis that is more sensitive and easier to interpret than previously described methods. The accuracy of the oligoclonal IgG band method reinforces the value of cerebrospinal fluid studies in the early differential diagnosis of multiple sclerosis.
Oreja-Guevara and colleagues find diffusion tensor magnetic resonance imaging shows progressive microstructural changes in the normal-appearing gray matter of patients with untreated relapsing-remitting multiple sclerosis. Their data directly confirm the importance of gray-matter pathologic features in multiple sclerosis.
Carmosino and colleagues report that a significant percentage of patients referred to a university multiple sclerosis center have a low possibility of actually having multiple sclerosis and have undiagnosed, untreated psychiatric illness or other common conditions with abnormal brain magnetic resonance imaging scan results.
Desautels and colleagues in a clinical molecular genetic study report the presence of a major restless legs syndrome susceptibility locus on chromosome 12q, which is designated RLS1, and also suggest that at least 1 additional locus may be involved in the etiology of this prevalent condition.
Chen and colleagues report different missense mutations in exon 1 of the myofibrillogenesis regulator 1 gene that cosegregate with disease in 2 families with paroxysmal nonkinesigenic dyskinesia.
Zappia and colleagues report that risk factors for peak-dose dyskinesias, both clinical and genetic, act in different ways for men and for women. Genetic factors related to the dopamine receptor D2 gene polymorphic status have a protective effect on peak-dose dyskinesias development in men but not in women.
Lossos and colleagues describe genotype-phenotype correlations in patients with choreoacanthocytosis. Awareness of the early manifestations may prevent misdiagnosis and enable appropriate genetic counseling.
Matthews and colleagues identified 11 patients with central nervous system Whipple disease, and cerebellar ataxia was present in 5 cases (45%). Cerebellar ataxia is a common feature of Whipple disease.
Hart and colleagues report that treatment with coenzyme Q10 and vitamin E in 10 patients with Friedreich ataxia resulted in significant improvement in cardiac and skeletal muscle bioenergetics, which was maintained throughout the 47 months of therapy. However, posture and gait scores and hand dexterity scores continued to deteriorate.
Yeh and colleagues document the finding that 67% of patients with Machado-Joseph disease had at least 3 symptoms related to autonomic dysfunction. Thus, autonomic dysfunction is a common feature in this cohort of patients with Machado-Joseph disease and appears to be related to clinical progression.
Mittal and colleagues find overrepresentation of the A-C-A haplotype in the large normal alleles in India as compared with the Portuguese population, suggesting that the expansion-prone large normal alleles with A-C-A haplotype might have been introduced in the Portuguese population through admixture with South Asian populations.
Morris and colleagues report that high intake of folate may be associated with cognitive decline in older persons. These unexpected findings call for further study of the cognitive implications of high levels of dietary folate in older populations.
This Month in Archives of Neurology. Arch Neurol. 2005;62(4):531-532. doi:10.1001/archneur.62.4.531