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NEUROLOGY 2006;66:464-465
© 2006 American Academy of Neurology

February 28 Highlights

Ventricular garlands and spinal cord lesions on MRI in Alexander disease

The MRI phenotypic variation of Alexander disease continues to broaden. van der Knaap et al. describe seven patients with a DNA-confirmed diagnosis, who display medulla atrophy, a spinal cord lesion, or ventricular garlands on MRI, whereas cerebral white matter abnormalities were mild or virtually absent.

see page 494

The editorial by Barkovich and Messing notes that Alexander disease has usually been characterized by macrocephaly, delayed development, and MRI evidence of leukodystrophy affecting the frontal lobes. Diagnosis previously required brain biopsy or autopsy and demonstration of protein aggregates known as Rosenthal fibers with the cytoplasm of astrocytes. Now molecular diagnosis has expanded the clinical phenotype of juvenile and adult forms of Alexander disease to include children without obvious leukodystrophy but possessing a newly recognized and peculiar anomaly in the walls of the lateral ventricles termed garlands. From a genetic standpoint Alexander disease is a remarkably homogeneous disorder, with >95% of patients accounted for by mutations in GFAP; despite this, the MRI picture is becoming more variable.

see page 468

Rehabilitation trial for walking after acute SCI

Nonambulatory patients with incomplete SCI were randomized to 12 weeks of equal intensity of treadmill training with weight support vs conventional therapy on admission for rehabilitation. At 6 months, Dobkin et al. found that 90% of subjects with initial leg movement in each group walked independently at 1.1 m/sec.

see page 484

Treadmill training after spinal cord injury: Good but not better

The editorial by Jonathan R. Wolpaw notes that although this study lowers the expectations for body weight supported treadmill training (BWSTT), it provides unexpected encouragement. The benefit of BWSTT and the control experience regimen were both much better than expected. This implies that various rehabilitation regimens can achieve good results if focused on a defined objective and vigorously pursued. It also shows that course of disability after spinal cord injury remains poorly defined.

see page 466

{gamma}-Secretase inhibition in Alzheimer patients

Using plasma Aß as a biomarker, Siemers et al. show inhibition of {gamma}-secretase in a study of 70 patients with AD treated for 6 weeks with LY450139. The agent was well-tolerated.

see page 602

Quality of life following epilepsy surgery in children

Using the Quality of Life in Childhood Questionnaire (QOLCE), Sabaz et al. found that epilepsy surgery improves quality of life in those children who become seizure-free.

see page 557

White matter integrity and cognition in childhood and old age

Deary et al. report an association between white matter integrity and cognition in nondemented octogenarians. The association was mediated by reaction time, which measures speed of information processing. The authors also found that the white matter integrity in old age is associated with childhood IQ.

see page 505

The editorial by Hill and Mitchell notes that this study takes advantage of psychometric data from the Scottish Mental Survey of 1932. Forty nondemented patients underwent repeat cognitive testing and MRI 70 years later. Fractional anisotropy measurements (a measure of white matter integrity) in the centrum semiovale correlated with current cognitive function (at a mean age of 83). While these results need confirmation, mounting evidence that white matter disease (or leukoaraiosis) is predictive of dementing illness suggests a need for randomized trials in which the primary outcome is cognitive function. Blood pressure control is the most likely intervention.

see page 470

Preimplantation exclusion of familial Creutzfeldt–Jakob (fCJD)

Meiner et al. report the performance of successful exclusion preimplantation genetic diagnosis for fCJD. By implementing this method, at-risk families may prevent the transmission of fCJD and avoid ethical dilemmas concerning pregnancy termination and predictive testing.

see page 607

Migraine and obesity

Migraine and obesity are both frequent disorders in the population. Furthermore, obesity is a risk factor for chronic daily headaches. Bigal et al. found that obesity is not comorbid to migraine. However, migraine sufferers who are obese have more frequent and severe attacks, as well as more associated symptoms.

see page 545

Posttraumatic stress symptoms after stroke

Bruggimann et al. studied 49 patients 1 year after a nonsevere stroke. Symptoms of posttraumatic stress disorder were found in 31% of the patients. Symptoms of intrusion (distressing recollections or dreams) were increased after basal ganglia strokes, suggesting that the phenomenon of re-experiencing may be modulated by frontosubcortical pathways.

A Patient Page on this topic is available at www.neurology.org.

see page 513


Figure 12
Figure. Axial T2-weighted images of 15-year-old Patient 1. Signal abnormalities in the medulla (A), hilus of the dentate nucleus (B), and a thin periventricular rim (C). Remaining white matter is normal. On the ventricular wall there are structures that resemble garlands (D).

FLAIR image showing rim of periventricular signal change and the garlands attached.


Figure 22


Figure 32


Related Articles

Treadmill training after spinal cord injury: Good but not better
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Neurology 2006 66: 466-467. [Full Text] [PDF]

Alexander disease: Not just a leukodystrophy anymore
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Neurology 2006 66: 468-469. [Full Text] [PDF]

White matter lesions and cognition: It’s time for randomized trials to preserve intelligence
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Neurology 2006 66: 470-471. [Full Text] [PDF]

Weight-supported treadmill vs over-ground training for walking after acute incomplete SCI
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Alexander disease: Ventricular garlands and abnormalities of the medulla and spinal cord
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