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This Month in Archives of Neurology
June 2005

This Month in

Arch Neurol. 2005;62(6):853-854. doi:10.1001/archneur.62.6.853

Stem Cell Transplantation for Multiple Sclerosis

Burt and colleagues review results with more than 200 patients with multiple sclerosis worldwide who have undergone hematopoietic stem cell transplantation. They discuss 3 variables that are important in predicting benefit and minimizing toxicity, including selecting patients who are still in its inflammatory phase, providing treatment early in its course before the onset of significant irreversibly progressive disability, and using an intense immune suppressive but nonmyeloablative conditioning regimen.

Standard of Cerebrospinal Fluid Analysis in the Diagnosis of Multiple Sclerosis

Freedman et al, representing an international panel of experts in multiple sclerosis and cerebrospinal fluid diagnostic techniques, developed a consensus recommendation for the evaluation of cerebrospinal fluid in patients who have multiple sclerosis. These new recommendations should greatly complement the clinical assessment in ensuring an accurate diagnosis of multiple sclerosis.

Organ Donor Transmission of Rabies Encephalitis  

Burton and colleagues describe the transmission of rabies virus from an organ donor to 4 recipients who subsequently developed rabies encephalitis. The clinical, neuroradiologic, neurovirologic, and neuropathologic studies in these organ recipients are meticulously documented, and the authors point out the challenges of identifying and preventing the passage of rabies virus by organ transplantation. Editorial perspective is provided by Karen L. Roos, MD.

Glatiramer Therapy in Multiple Sclerosis

Allie and colleagues studied glatiramer acetate to determine its mechanism of action in patients with relapsing-remitting multiple sclerosis. In an elegant immunologic study, they find that glatiramer suppresses autoreactive CD4+ effector memory T cells and enhances CD8+ regulatory responses and further indicate that bystander modulation of chemokine receptor expression may occur in the periphery. Editorial perspective on these important observations is provided by Nitin J. Karandikar, PhD, and Michael K. Racke, MD.

Axonal Injury in Multiple Sclerosis

Rovaris et al report that the amount of whole-brain N-acetylaspartate and normalized brain volumes were significantly lower in patients with primary progressive multiple sclerosis than in healthy control subjects. Axonal/neuronal damage in the brains of patients with primary progressive multiple sclerosis occurs independently of the burden of lesions visible with magnetic resonance imaging.

Intermittent vs Continuous Levodopa Therapy in Parkinson Disease

Stocchi and colleagues show that continuous levodopa infusion is associated with reduced motor complications in comparison with the standard oral formulation of the drug for patients with advanced Parkinson disease. They propose that if levodopa/carbidopa could be administered orally to mirror the pharmacokinetic pattern of the infusion, it might reduce in a similar manner the degree of motor complications.

Expression Profiling of Substantia Nigra in Parkinson Disease, Supranuclear Palsy, and Frontotemporal Dementia

Hauser et al report the microarray expression analysis of postmortem substantia nigra tissue. Identification of gene expression in these disorders was studied using Affymetrix U133A microarrays. Hierarchical clustering analysis provided separation of 4 of 5 Parkinson disease cases from supranuclear and frontotemporal dementia cases. Expression microarray gene analyses have potential in the molecular diagnosis of parkinsonism.

Frontotemporal Lobar Degeneration: Characteristics of 353 Patients

Johnson and colleagues compared diagnostic category, age at onset, sex, Mini-Mental State Examination score at first visit, education, andneuropathological diagnoses in a large sample of patients with frontotemporal lobar degeneration (FTLD). The FTLD cases were divided into 3 subgroups: frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia. Using new research criteria for FTLD, cohorts of patients can be combined and demonstrate striking demographic differences among FTLD subgroups. Cases of FTLD with ubiquitin-positive inclusions accounted for one half of all neuropathologic diagnoses.

Posterior or Middle Cerebral Artery Infarct

Maulaz et al studied the frequency of posterior cerebral artery infarction that mimics middle cerebral artery infarction and which portion of the posterior cerebral artery is most commonly responsible for simulation. They find that posterior cerebral artery infarct simulating middle cerebral artery infarct is more frequent than commonly thought, and early recognition may allow specific management decisions.

Serotonin 1A Receptor Binding in Juvenile Myoclonic Epilepsy

Meschaks and colleagues report that the serotonin 1A receptor binding potential is reduced in juvenile myoclonic epilepsy. Their data provide support for the view that not all brain regions are homogenously involved in juvenile myoclonic epilepsy and bring into question the current classification of primary generalized epilepsy.

Sex, Apolipoprotein E ε4 Status, and Hippocampal Volume in Mild Cognitive Impairment

Fleisher et al examined the relationship between the apolipoprotein E ε4 genotype and hippocampal volume in men and women with mild cognitive impairment. They report that apolipoprotein E ε4 genotype status appears to have a greater deleterious effect on gross hippocampal pathology and memory performance in women than in men.

Fragile X–Associated Tremor/Ataxia Is Rare in Multiple System Atrophy

Biancalana et al determined whether fragile X–associated tremor/ataxia syndrome contributes to the syndrome of multiple system atrophy. They measured the fragile X mental retardation 1 gene (FMR1) repeats in 77 patients diagnosed with multiple system atrophy. Two patients carried FMR1 premutations of 110 and 135 repeats. Nine patients carried alleles in the intermediate size range, from 41 to 53 repeats.

Brain γ-Aminobutyric Acid Changes in Stiff-Person Syndrome

Levy and colleagues report a prominent and significant decrease in γ-aminobutyric acid levels in stiff-person syndrome in the sensorimotor cortex and a smaller decrease in the posterior occipital cortex.

Predictive Utility of Apolipoprotein E in Mild Cognitive Impairment

Devanand et al report that apolipoprotein E ε4 homozygosity was associated with increased conversion to Alzheimer disease from mild cognitive impairment.

Clinical and Magnetic Resonance Imaging Features of Sporadic Cerebellar Ataxia

Bürk et al studied whether patients with multiple system atrophy of the cerebellar type (MSA-C) represent a separate and distinct disorder from patients who have cerebellar ataxia with extracerebellar features. Patients with MSA-C showed a higher frequency and severity of magnetic resonance imaging abnormalities of the middle cerebellar peduncles and pons. These magnetic resonance imaging features point to the diagnosis of MSA-C and help differentiate MSA-C from other types of sporadic cerebellar ataxia.

Cognitive Dysfunction in Late-Onset GM2 Gangliosidosis

Frey et al examined the natural history of cognitive dysfunction in patients with late-onset GM2 gangliosidosis. They find cognitive dysfunction is a frequent manifestation of late-onset GM2 gangliosidosis, and those patients who experience cognitive dysfunction are more likely to have a greater number of other neurologic manifestations of the disease. It may take the form of static encephalopathy, but progressive dementia was more frequently documented.