This Month in Archives of Neurology

THIS MONTH IN ARCHIVES OF NEUROLOGY

great variety of human tissues following hematopoietic Oculomotility Disorders and Brainstem stem cell transplantation. It is an important therapeutic Motor Neuron Development advance for patients with adrenoleukodystrophy. Edi- torial perspective is provided by Hugo W. Moser, MD, and ngle (page 633) reviews the congenital cranial Asif Mahmood, MD, MPH. dysinnervation disorders. These disorders ap- E pear to result from mutations in genes that are essential to the normal development or connectivity of cranial motoneurons. These genetic defects lead to dis- Hereditary Spastic Paraplegia 3A ruption in early motoneuron development, aberrant axo- Associated With Axonal Neuropathy nal targeting onto motoneurons, and aberrant axonal targeting onto extraocular muscles. vanova et al (page 706) studied the frequency and distribution of mutations in SPG3A in a cohort of I 182 families with pure complex hereditary spastic Contribution of Intermediate Progenitor paraplegia phenotypes that were negative for mutations Cells to Cortical Histogenesis in SPG4. In 12 probands (6.6%), they identified 12 dif- ferent SPG3A mutations. Mutations in SPG3A represent n this elegant review, Noctor and colleagues an important cause of patients in the overall hereditary (page 639) describe recent advances in molecular spastic paraplegia population. SPG3A is more often as- and cell biology that have made possible the study sociated with a neuropathy than previously assumed. I Thus, patients with a bipyramidal syndrome and a neu- of specific cell populations and 2 cortical progenitor cell types, radial glial cells in the ventricular zone and inter- ropathy should be screened for mutations in SPG3A. mediate progenitor cells in the subvectricular zone, that have been shown to generate neurons in the embryonic cerebral cortex. These findings have refined our understanding of cortical neurogenesis with implications for Transdermal Rotigotine understanding the etiology of neurodevelopmental dis- in Parkinson Disease orders and their potential treatment. ankovic et al (page 676) report that the transdermal system for rotigotine, a nonergolinic dopa- Genetic Models of Migraine J mine agonist, consistently demonstrated statistically significant and clinically relevant effi- his review by van de Ven et al (page 643) indi- cacy over placebo (Figure) for patients with early- cates that identification of “threshold genes” stage Parkinson disease and was well tolerated. T and deciphering their function will help un- ravel the triggering mechanisms for migraine attacks. Familial hemiplegic migraine (FHM) is a rare mono- 60 genic subtype of migraine with aura. Three genes have Placebo Group been identified for familial hemiplegic migraine. Re- 50 Rotigotine Group cently, knock-in mice have been generated carrying hu- 40 man pathogenic FHM1 mutations. This review empha- 30 sizes the importance that genetic migraine models will 20 have in unraveling the triggering mechanisms of mi- 10

graine attacks and identifying novel migraine prophy- 0

lactic targets and therapies. –10 % Change From Basaeline –20 ∗ Genotype and Protein Expression –30 –40 After Bone Marrow Transplantation 3 4 5 6 7 8 9 10 11 for Adrenoleukodystrophy Visit

cho¨nberger and colleagues (page 651) report evi- Figure. Percentage change from baseline in Unified Parkinson Disease dence for the stable development of a wild-type Rating Scale subtotal (parts II [activities of daily living] and III [motor function]) score during the 24-week maintenance phase in the rotigotine X-linked adrenoleukodystrophy genotype and per- and placebo groups (PϽ.001 vs placebo). Visits 3 and 4, titration phase; S Ͻ oxisomal adrenoleukodystrophy protein expression in a visits 5 to 11, maintenance phase. *P .001.

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©2007 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 rum creatinine level greater than 1.08 mg/dL (Ͼ95.5 µmol/ Magnetic Resonance Imaging Detection L). They concluded that urinalysis may have utility in of Lesion Progression in Adult Patients the early identification of cardioembolic stroke subtype With X-linked Adrenoleukodystrophy in patients with acute ischemic stroke.

ichler et al (page 659) found that brain magnetic resonance imaging abnormalities in adult Atherosclerotic Burden and Early Mortality E patients with adrenoleukodystrophy progress in Acute Ischemic Stroke slower than reported in childhood. The involvement of the corticospinal tracts is prominent and may at times oquer and colleagues (page 699) studied the in- represent a variant course of progressive inflammatory fluence that previous clinical expressions of sys- demyelination. R temic atherosclerosis may have on evolution and early mortality in patients with acute ischemic stroke. They Testosterone Treatment in Multiple Sclerosis found that previous symptomatic atherosclerotic disease evaluated by a simple clinical score is an indepen- dent predictor of early mortality in patients with first- icotte and colleagues (page 683) found that in ever ischemic stroke. male patients with relapsing-remitting multiple S sclerosis, testosterone gel treatment was associated with improvements in spatial and working memory performance and a slowing of brain atrophy. These find- Evaluation of Cognitive Impairment ings suggest that testosterone treatment is safe and well- in Older Adults tolerated and has potential neuroprotective effects in men with relapsing-remitting multiple sclerosis. alvin and colleagues (page 718) used the AD8, a screening tool for dementia, combined with Word List Recall and showed that this ap- Aggressive Therapy for Neurosarcoidosis G proach improved the ability to detect the presence of dementia. The AD8 can be administered to an informant n treating 48 patients with neurosarcoidosis, Scott and, when combined with Word List Recall, is a power- et al (page 691) report that among patients treated ful yet brief method of detecting cognitive impairment. I with immunosuppressive therapies, 18 (69%) improved, 4 (15%) remained stable, and 4 (15%) worsened. Of patients treated with corticosteroids alone, 7 (35%) improved, 11 (55%) remained stable, and 2 (10%) Patient’s Rating of Cognitive Ability: worsened. Most of these high-risk patients benefited from Using the AD8 this therapeutic regimen. alvin et al (page 725) tested the ability of patients to rate their own cognitive ability using Utility of Urinalysis in Discriminating the AD8 compared with informant and clini- Cardioembolic Stroke Mechanism G cian ratings of cognitive status. They found the AD8 is a brief measure that, when completed by an informant, dif- iehman and colleagues (page 667) found in con- ferentiates nondemented from demented individuals. They ducting urinalysis that the best single predictor also demonstrated that a self-completed AD8 differenti- V for cardioembolic stroke subtype was a white ates nondemented from demented individuals. In the ab- blood cell count of greater than 14.5/µL followed by a sence of reliable informants, the AD8 may provide an un- red blood cell count of greater than 41.67/µL and a se- derstanding of patients’ perception of their cognitive status.

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