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This Month in Archives of Neurology
July 2011

This Month in Archives of Neurology

Arch Neurol. 2011;68(7):841-842. doi:10.1001/archneurol.2011.136

GoldmanArticle points out that the childhood leukodystrophies are characterized by neonatal or childhood deficiencies in myelin production or maintenance; these may be due to hereditary defects in genes for myelin maintenance, as in Pelizaeus-Merzbacher disease, or to enzymatic deficiencies resulting in substrate misaccumulation or misprocessing, as in the lysosomal storage disorders. Regardless of their respective etiologies, these disorders are essentially all manifested by a profound deterioration in neurological function with age. As a result, glial progenitor cells (GPCs), which can give rise to new myelinogenic oligodendrocytes, have become of great interest as potential vectors for the restoration of myelin to the dysmyelinated brain and spinal cord. In addition, by distributing throughout the neuraxis after perinatal graft, and giving rise to astrocytes as well as oligodendrocytes, GPCs may be of great utility in rectifying the dysmyelination-associated enzymatic deficiencies of the lysosomal storage disorders. Goldman reviews his own exciting research and the overall achievements made recently in this field.

Although traditionally regarded as spared, a range of oculomotor dysfunction has been recorded in patients with amyotrophic lateral sclerosis. Most frequent is ophthalmoparesis, particularly in patients with prolonged survival; however, pursuit, nystagmus, and saccadic impairments have also been reported as reviewed here by Sharma and colleaguesArticle.

Lin et alArticle investigate the immediate and longitudinal mechanisms of action of intravenous immunoglobulin (IVIg) on axonal function in chronic inflammatory demyelinating polyneuropathy (CIDP). Their findings from the present series establish a modulatory effect of IVIg on axonal function in CIDP patients, suggesting that IVIg immunotherapy stabilizes axonal membrane potential and thereby axonal recovery.Editorial perspective is provided by Richard A. C. Hughes, MD, FRCP, and Kerry Mills, PhDArticle.

Plasma exchange (PLEX) is a beneficial rescue therapy for acute, steroid-refractory central nervous system inflammatory demyelinating disease (CNS-IDD). Despite the approximately 45% PLEX response rate reported among patients with CNS-IDD, determinants of interindividual differences in PLEX response are not well characterized. Magaña and colleaguesArticle perform an exploratory analysis of clinical, radiographic, and serological features associated with beneficial PLEX response. They have identified clinical and radiographic features that may aid in identifying patients with fulminant, steroid-refractory CNS-IDD attacks who are more likely to respond to PLEX.

Frequency of presenting symptoms at the index attack (A) and plasma exchange response rate by presenting symptom among all patients with that symptom (B). Most patients had polysymptomatic attacks and thus could have more than 1 presenting symptom.

Frequency of presenting symptoms at the index attack (A) and plasma exchange response rate by presenting symptom among all patients with that symptom (B). Most patients had polysymptomatic attacks and thus could have more than 1 presenting symptom.

Gilli et alArticle have identified a gene signature for multiple sclerosis (MS), which reverted back to normal during pregnancy. Reversion was particularly evident for 7 genes: SOCS2, TNFAIP3, NR4A2, CXCR4, POLR2J, FAM49B, and STAG3L1, most of which encode negative regulators of inflammation. They corroborate dysregulation of transcripts, evaluate the prognostic value of genes, and study modulation of genes during different treatments. They demonstrate that there is a new molecular pathogenic mechanism that underlies the initiation and progression of MS. Defects in negative feedback loops of inflammation lead to an overactivation of the immune system so as to predispose the brain to inflammation-sensitive MS.

Landt and colleaguesArticle investigate the safety, acceptability, and feasibility of positron emission tomography (PET) using carbon 11–labeled Pittsburgh Compound B ([11C]PiB) to measure cerebral β-amyloid in adults with Down syndrome (DS) and to explore if the technique differentiates between participants with and without Alzheimer disease. They report that dynamic [11C]PiB PET can be used successfully to measure cerebral β-amyloid deposition in DS.

Weintraub et alArticle examine the frequency and characteristics, including changes over time, of antipsychotic (AP) use in a large cohort of patients with Parkinson disease (PD). They report that half of the patients with PD and psychosis receive APs, not uncommonly high-potency agents associated with worsening parkinsonism, and frequency of use is unchanged since the “black box” warning for AP use in patients with dementia was issued. Recent trends are a shift to quetiapine use and the common use of aripiprazole.

Villemagne and colleaguesArticle assess the diagnostic potential of imaging striatal monoaminergic terminal integrity with the vesicular monoamine transporter type 2 radioligand 18F 9-fluropropyl-(+)-dihydrotetrabenazine ([18F]AV-133) and positron emission tomography to distinguish dementia with Lewy bodies (DLB) from Alzheimer disease (AD). They note that [18F]AV-133 allows assessment of nigrostriatal degeneration in Lewy body diseases. [18F]AV-133 can robustly detect reductions of dopaminergic nigrostriatal afferents in DLB patients and assist in the differential diagnosis from AD.

Uzawa et alArticle evaluate the degree of blood-brain barrier disruption by analyzing the levels of soluble intercellular adhesion molecule 1 and soluble vascular cell adhesion molecule 1 in patients with neuromyelitis optica. They conclude that measuring adhesion molecules is useful to evaluate blood-brain barrier disruption in patients with neuromyelitis optica.

Collongues and colleaguesArticle identify neuromyelitis optica with a good outcome among a large population of patients and describe demographic and clinical variables associated with neuromyelitis optica with a good outcome vs standard neuromyelitis optica and benign multiple sclerosis. Among patients in metropolitan France, neuromyelitis optica with a good outcome occurs rarely. However, because a disabling attack may occur after a long follow-up period, a benign form of neuromyelitis optica cannot be defined.

Godau et alArticle note that the level of serum insulinlike growth factor 1 (IGF-1) is increased in idiopathic Parkinson disease (PD). They assess whether (1) IGF-1 level is increased in patients with PD at the time of diagnosis, (2) increased IGF-1 level is related to impaired motor function in healthy individuals, and (3) detection of increased IGF-1 level will help to identify persons at risk for PD. They find that the IGF-1 level was higher in patients with PD compared with healthy participants (P = .004) and inversely correlated with the Unified Parkinson's Disease Rating Scale, Part III (UPDRS-III) score (ρ = −0.77). The IGF-1 level was not related to motor function in the healthy group. However, there was no significant difference between the IGF-1 level in the at-risk subgroup vs the PD patients (corrected P = .15), and the IGF-1 level was positively correlated with the UPDRS-III score (ρ = 0.80). Thus, serum IGF-1 monitoring may be valuable in the diagnosis of PD and for the identification of individuals with a putatively increased risk for PD.

Berg and colleaguesArticle evaluate whether substantia nigra hyperechogenicity (SN+) may be associated with an increased risk for Parkinson disease (PD) in a healthy elderly population. In their prospective study, they demonstrate for the first time a highly increased risk for PD in elderly subjects with SN+. Transcranial ultrasonography of the midbrain may therefore be a promising primary screening procedure to define a risk population for imminent PD.