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This Month in Archives of Neurology
September 2007

This Month in Archives of Neurology

Arch Neurol. 2007;64(9):1231-1232. doi:10.1001/archneur.64.9.1231
Gene Therapy for Duchenne Muscular Dystrophy

Rodino-Klapac and colleagues are leaders in gene therapy for Duchenne muscular dystrophy (DMD), and their review indicates that this approach has promise for treating DMD. They conducted the very first viral-mediated gene transfer for any muscle disease at Columbus Children's Research Institute and the Ohio State University for limb girdle muscular dystrophy type 2D. They are now conducting the first viral-mediated trial of gene transfer for DMD. Thus, they are in a prime position to tell us of the challenges and potential problems as the field advances this treatment modality to clinical reality.

Aging and Parkinson Disease

Levy emphasizes that age influences the clinical progression of Parkinson disease (PD). Advancing age is associated with faster rate of motor progression, decreased levodopa responsiveness, more severe gait and postural impairment, more severe cognitive impairment, and the development of dementia in patients with PD. A model for the relation of PD with aging is proposed.

Magnetic Resonance Spectroscopy and Human Immunodeficiency Virus–Induced Inflammation

Roc and colleagues examined whether lactate, a marker of inflammation and anaerobic glycolysis, and lipid, an indictor of cell membrane turnover resulting from oxidative stress, could serve as surrogate biomarkers within the lenticular nucleus of patients with human immunodeficiency virus (HIV) infection with different degrees of neurocognitive impairment. They report that as seen by 2-dimensional chemical shift magnetic resonance spectroscopy, HIV induces inflammation and oxidative stress in patients with HIV infection despite highly active antiretroviral therapy. Editorial perspective is provided by Martin G. Pomper, MD, and Ned Sacktor, MD.

Deep Brain Stimulation in Primary Dystonia: Functional Mapping of the Pallidum

Houeto and colleagues investigated the functional map of the globus pallidus in patients with primary generalized dystonia. They found that ventral pallidal stimulation, mainly internal globus pallidus and medullar lamina, is the optimal target for the treatment of dystonia (Figure).

Figure.
Effects of ventral contact vs dorsal contact stimulation on dystonia motor disability. A, Global effect. B, Comparative effects in 3 subgroups of patients with marked improvement (> 50%), moderate improvement (50%-25%), and slight or no improvement (< 25%). In each subgroup, the percentage improvement in the Burke-Fahn-Marsden Dystonia Rating Scale score is shown.

Effects of ventral contact vs dorsal contact stimulation on dystonia motor disability. A, Global effect. B, Comparative effects in 3 subgroups of patients with marked improvement (> 50%), moderate improvement (50%-25%), and slight or no improvement (< 25%). In each subgroup, the percentage improvement in the Burke-Fahn-Marsden Dystonia Rating Scale score is shown.

Platelet Decline and Dementia Associated With the Human Immunodeficiency Virus

Wachtman et al studied the association between platelet decline from baseline and human immunodeficiency virus (HIV)–dementia. They report that patients with declining platelet counts are at greater risk for HIV-dementia and that the dynamics of circulating platelet numbers vary with respect to the temporal progression of HIV-dementia. These interesting studies highlight an avenue to be explored in the understanding of pathogenic mechanisms common to the central nervous system and the periphery.

Putamen Hypertrophy in Patients With Human Immunodeficiency Virus Infection and Cognitive Compromise

Castelo et al examined subcortical volumes in nondemented patients with human immunodeficiency virus (HIV) infection to investigate relationships between cognitive function, immune health, and subcortical volumes. They report that basal ganglia hypertrophy accompanies HIV-related mild cognitive impairment. These findings may represent a structural imaging parallel to functional imaging studies demonstrating basal ganglia hypermetabolism in patients who are HIV positive with mild cognitive compromise and early HIV-associated brain disease.

Association of Higher Serum Calcium Levels With Smaller Infarct Size in Stroke

Buck and colleagues assessed the relation between admission serum calcium levels and initial diffusion-weighted magnetic resonance imaging lesion volumes among patients with acute ischemic stroke. Of note, higher levels of admission serum calcium are associated with smaller cerebral infarct volumes among patients with acute ischemic stroke. These results suggest that serum calcium level may serve as a clinical prognosticator following stroke and may be a potential therapeutic target for improving stroke outcome.

Magnetic Resonance Imaging Effects of Interferon-Beta1b

Barkof et al examined detailed magnetic resonance imaging (MRI) findings from the first 2 years of the BENEFIT study. In this study, interferon beta-1b delayed conversion to multiple sclerosis in patients with a first clinical event and at least 2 clinically silent brain MRI lesions. Of significance, receiving 250 μg of interferon beta-1b subcutaneously every other day had a robust effect on MRI measures, supporting its value as an early intervention in this patient group.

Cervical Cord Gray Matter Lesions in Multiple Sclerosis

Agosta et al report that cervical cord gray matter is not spared by multiple sclerosis pathology, and such damage is an additional factor contributing to the disability of these patients.

Cognitive Decline in Healthy Apolipoprotein E ε4 Homozygotes

Caselli and colleagues report that apolipoprotein E ε4 homozygotes in their 60s have higher rates of cognitive decline than apolipoprotein E ε4 heterozygotes or noncarriers before the diagnosis of mild cognitive impairment (MCI) and Alzheimer disease, thus confirming and characterizing the existence of a pre-MCI state in this genetic subset.

Cholinergic Axon Density in Mild Cognitive Impairment and Early Alzheimer Disease

Ikonomovic et al found a lack of increase in cholinergic axonal innervation of the superior frontal cortex in mild cognitive impairment, suggesting that structural reorganization of cholinergic profiles is not the mechanism underlying the transient cholinergic plasticity reported in this region. Rather, they state that the stability of cholinergic enzyme activity in mild to moderate Alzheimer disease is likely the result of a biochemical up-regulation of choline acetyltransferase protein or enzyme activity levels in the superior frontal cortex, compensating for reduced regional cholinergic fibers and axon varicosities.

Whole-Body Tremulousness: Isolated Generalized Polymyoclonus

McKeon and colleagues report that isolated whole-body tremulousness should raise suspicion of generalized polymyoclonus, which is easily confirmed using routine surface electromyographic studies. Recognition is important because the differential diagnosis includes autoimmunity (paraneoplastic or idiopathic) and drug-induced myoclonus.

A Neuropsychological Test Battery for Use in Alzheimer Disease Clinical Trials

Harrison et al evaluated a neuropsychological test battery (NTB) for measuring drug efficacy in Alzheimer disease clinical trials. The NTB exhibited excellent psychometric properties, and it appears to be a reliable and sensitive measure of cognitive changes in patients with mild to moderate Alzheimer disease.

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