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This Month in Archives of Neurology
October 2007

This Month in Archives of Neurology

Arch Neurol. 2007;64(10):1383-1384. doi:10.1001/archneur.64.10.1383
TDP-43 and Neurodegenerative Disease

To facilitate understanding of a group of disorders collectively known as TDP-43 proteinopathies, Neumann and colleagues review advances made since it was shown that TAR-DNA binding protein 43 (TDP-43) is the major disease protein that links frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without motor neuron disease to amyotrophic lateral sclerosis. It is an important new insight into the molecular basis of these disorders and offers new insights into disease causation.

The Minimally Conscious State

Fins et al propose 2 models characterizing minimal conscious state (MCS) epidemiology. A severity model integrates diagnostic and severity of injury codes and a venue model that tracks patient migration after hospital discharge. The epidemiology of MCS is reviewed.

DNA Gene Vaccination for Multiple Sclerosis

A new DNA gene vaccine encoded for full-length human myelin basic protein was studied by Bar-Or and colleagues. They assessed the safety and immune modulations of the vaccine in patients with multiple sclerosis. They found that it is safe and well tolerated and provided favorable trends on brain magnetic resonance imaging and produced beneficial antigen-specific immune changes. These immune changes consisted of a marked decrease in proliferation of interferon γ–producing myelin-reactive CD4+ T cells and a reduction in titers of myelin-specific autoantibodies from cerebral spinal fluid. Editorial perspective is provided by Olaf Stüve, MD, PhD; Todd N. Eagar, PhD; Elliot M. Frohman, MD, PhD; and Petra D. Cravens, PhD.

Fulminant Demyelinating Disease

In a retrospective case study, de Seze and colleagues analyzed 60 patients with acute demyelinating encephalomyelitis (ADEM) for risk of evolution to multiple sclerosis (MS). They concluded that the presence of any 2 of 3 criteria could be used to differentiate ADEM from MS: atypical clinical symptoms for MS, absence of oligoclonal bands, and gray matter involvement.

Cortical Inflammatory Lesions in Multiple Sclerosis

Calabrese et al used double inversion recovery sequence magnetic resonance imaging to determine frequency, time of appearance, and clinical relevance of intracortical lesions (ICLs) in patients with multiple sclerosis (MS). They indicate that ICLs assist in defining MS clinical heterogeneity and prognosis (Figure).

Figure
Mean numbers of intracortical lesions (ICLs) in all patients included in the study. First, second (median), and third quartiles are used for the box construction, whereas 1.5 × (quartile 3−quartile 1) is used for the whiskers. Points outside the whiskers are considered outliers (cases with values between 1.5 and 3 box lengths from the upper or lower edge of the box). Asterisks indicate extreme cases (cases with values more than 3 box lengths from the upper or lower edge of the box). CIS indicates clinically isolated syndrome; RRMS, relapsing-remitting multiple sclerosis; and SPMS, secondary progressive multiple sclerosis.

Mean numbers of intracortical lesions (ICLs) in all patients included in the study. First, second (median), and third quartiles are used for the box construction, whereas 1.5 × (quartile 3−quartile 1) is used for the whiskers. Points outside the whiskers are considered outliers (cases with values between 1.5 and 3 box lengths from the upper or lower edge of the box). Asterisks indicate extreme cases (cases with values more than 3 box lengths from the upper or lower edge of the box). CIS indicates clinically isolated syndrome; RRMS, relapsing-remitting multiple sclerosis; and SPMS, secondary progressive multiple sclerosis.

Progranulin in a Family and Diagnoses of Alzheimer and Parkinson Disease

Brouwers et al assessed whether genetic variability in progranulin (PGRN) associated with frontotemporal lobar degeneration also contributed to other common degenerative brain disease, such as Alzheimer disease (AD) or Parkinson disease (PD). They report that their mutation data indicated that null mutations are very rare in patients with AD and PD and likely result from etiological heterogeneity rather than PGRN haploinsufficiency per se.

Risk of Cognitive Impairment or Dementia in Relatives of Patients With Parkinson Disease

Rocca et al found that cognitive impairment or dementia may share familial susceptibility factors with Parkinson disease.

Imaging the Aβ-Related Neurotoxicity of Alzheimer Disease

Moreno and colleagues show that the neurotoxicity related to Aβ peptide can be captured in vivo by functional imaging and suggest hippocampal subregions most vulnerable to its toxic effects.

Three-Dimensional Gray Matter Atrophy Mapping in Mild Cognitive Impairment and Mild Alzheimer Disease

With magnetic resonance imaging and an advanced 3-dimensional cortical mapping technique, Apostolova and colleagues show striking cortical differences between mild Alzheimer disease and amnestic mild cognitive impairment.

Pharyngeal-Cervical-Brachia Variant of Guillain-Barré Syndrome

Nagashima and colleagues describe the clinical features of pharyngeal-cervical-brachial weakness.

Real-World Replication of Randomized Controlled Trial Results for Carotid Endarterectomy

Feasby et al report that randomized clinical trials for carotid endarterectomy can be achieved in the “real world.” However, some hospitals exceed the maximum suggested adverse event rates, which highlights the need for continuous outcome monitoring and associate quality improvement efforts to ensure that all providers and institutions involved are achieving desired outcomes.

TDP-43 Pathology and Frontotemporal Lobar Degeneration

Grossman and colleagues studied demographic, clinical, and neuropsychological features associated with subtypes of frontotemporal lobar degeneration (FTLD-U) with TDP-43 inclusions. They found that there are significantly different clinical and neuropsychological patterns of impairment in FTLD-U subtypes. Distinct TDP-43 profiles impact clinical phenotypes differentially in patients with FTLD-U pathology.

Asian Origin for the Worldwide-Spread Mutational Event in Machado-Joseph Disease

Martins et al found that the worldwide-spread mutation of Machado-Joseph disease may have occurred in Asia, being later diffused throughout Europe with a founder effect accounting for its high prevalence in Portugal.

Importance of Low-Range CAG Expansion and CAA Interruption in SCA2 Parkinsonism

Kim et al conclude that SCA2 is one of the causes of Parkinson disease and the Parkinson variant of multiple system atrophy. CAG repeats were in the low-range expansion and interrupted by CAA in all patients.

Reduced Hippocampal Functional Connectivity in Alzheimer Disease

Allen et al determined whether functional connectivity of the hippocampus is reduced in Alzheimer disease (AD). Functional connectivity magnetic resonance imaging was used to investigate coherence in the magnetic resonance signal between hippocampus and all other regions of the brain. Their findings suggest a functional disconnection between hippocampus and other brain regions in AD.

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