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January 2011, Vol 68, No. 1, Pages 11-138

Editorial

Should We Target Insulin Resistance to Prevent Dementia Due to Alzheimer Disease?

Abstract Full Text
Arch Neurol. 2011;68(1):17-18. doi:10.1001/archneurol.2010.339

New Lessons From the Alzheimer's Disease Neuroimaging Initiative

Abstract Full Text
Arch Neurol. 2011;68(1):19-21. doi:10.1001/archneurol.2010.344
Original Contribution

Insulin Resistance and Alzheimer-like Reductions in Regional Cerebral Glucose Metabolism for Cognitively Normal Adults With Prediabetes or Early Type 2 Diabetes

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Arch Neurol. 2011;68(1):51-57. doi:10.1001/archneurol.2010.225
BackgroundInsulin resistance is a causal factor in prediabetes (PD) and type 2 diabetes (T2D) and increases the risk of developing Alzheimer disease (AD). Reductions in cerebral glucose metabolic rate (CMRglu) as measured by fludeoxyglucose F 18–positron emission tomography (FDG-PET) in parietotemporal, frontal, and cingulate cortices are associated with increased AD risk and can be observed years before dementia onset.ObjectivesTo examine whether greater homeostasis model assessment insulin resistance (HOMA-IR) is associated with reduced resting CMRglu in areas vulnerable in AD in cognitively normal adults with newly diagnosed PD or T2D (PD/T2D), and to determine whether adults with PD/T2D have abnormal patterns of CMRglu during a memory encoding task.DesignRandomized crossover design of resting and activation FDG-PET.SettingUniversity imaging center and Veterans Affairs clinical research unit.ParticipantsTwenty-three older adults (mean [SEM] age, 74.4 [1.4] years) with no prior diagnosis of diabetes but who met American Diabetes Association glycemic criteria for PD (n = 11) or diabetes (n = 12) based on fasting or 2-hour oral glucose tolerance test (OGTT) glucose values and 6 adults (mean [SEM] age, 74.3 [2.8] years) with normal fasting glucose values and glucose tolerance. No participant met Petersen criteria for mild cognitive impairment.InterventionsFasting participants underwent resting and cognitive activation FDG-PET imaging on separate days. Following a 30-minute transmission scan, subjects received an intravenous injection of 5 mCi of FDG, and the emission scan commenced 40 minutes after injection. In the activation condition, a 35-minute memory encoding task was initiated at the time of tracer injection. Subjects were instructed to remember a repeating list of 20 words randomly presented in series through earphones. Delayed free recall was assessed once the emission scan was complete.Main Outcome MeasuresThe HOMA-IR value was calculated using fasting glucose and insulin values obtained during OGTT screening and then correlated with CMRglu values obtained during the resting scan. Resting CMRglu values were also subtracted from CMRglu values obtained during the memory encoding activation scan to examine task-related patterns of CMRglu.ResultsGreater insulin resistance was associated with an AD-like pattern of reduced CMRglu in frontal, parietotemporal, and cingulate regions in adults with PD/T2D. The relationship between CMRglu and HOMA-IR was independent of age, 2-hour OGTT glucose concentration, or apolipoprotein E ε4 allele carriage. During the memory encoding task, healthy adults showed activation in right anterior and inferior prefrontal cortices, right inferior temporal cortex, and medial and posterior cingulate regions. Adults with PD/T2D showed a qualitatively different pattern during the memory encoding task, characterized by more diffuse and extensive activation, and recalled fewer items on the delayed memory test.ConclusionsInsulin resistance may be a marker of AD risk that is associated with reduced CMRglu and subtle cognitive impairments at the earliest stage of disease, even before the onset of mild cognitive impairment.

Treatment With Cholinesterase Inhibitors and Memantine of Patients in the Alzheimer's Disease Neuroimaging Initiative

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Arch Neurol. 2011;68(1):58-66. doi:10.1001/archneurol.2010.343

Defective Mitochondrial Adenosine Triphosphate Production in Skeletal Muscle From Patients With Dominant Optic Atrophy Due to OPA1 Mutations

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Arch Neurol. 2011;68(1):67-73. doi:10.1001/archneurol.2010.228
ObjectiveTo assess whether impaired energy metabolism in skeletal muscle is a hallmark feature of patients with dominant optic atrophy due to several different mutations in the OPA1 gene.DesignWe used phosphorus 31 magnetic resonance spectroscopy to assess calf muscle oxidative metabolism in subjects with molecularly defined dominant optic atrophy carrying different mutations in the OPA1 gene. In a subset of patients, we also evaluated serum lactate levels after exercise and muscle biopsy results for histology and mitochondrial DNA analysis.SettingUniversity neuromuscular and neurogenetics and magnetic resonance imaging units.PatientsEighteen patients with dominant optic atrophy were enrolled from 8 unrelated families, 7 of which carried an OPA1 mutation predicted to induce haploinsufficiency and 1 with a missense mutation in exon 27. Fifteen patients had documented optic atrophy.Main Outcome MeasuresPresence of skeletal muscle mitochondrial oxidative phosphorylation dysfunction as assessed by phosphorus 31 magnetic resonance spectroscopy, serum lactate levels, and histological and mitochondrial DNA analysis.ResultsPhosphorus 31 magnetic resonance spectroscopy showed reduced phosphorylation potential in the calf muscle at rest in patients with an OPA1 mutation (−24% from normal mean; P = .003) as well as a reduced maximum rate of mitochondrial adenosine triphosphate synthesis (−36%; P < .001; ranging from −28% to −49% in association with different mutations). In 4 of 10 patients (40%), the serum lactate level after exercise was elevated. Only 2 of 5 muscle biopsies, from the 2 patients with a missense mutation, showed slight myopathic changes. Low levels of mitochondrial DNA multiple deletions were found in all muscle biopsies.ConclusionsDefective oxidative phosphorylation in skeletal muscle is a subclinical feature of patients with OPA1 -related dominant optic atrophy, indicating a systemic expression of the OPA1 defect, similar to that previously reported for Leber hereditary optic neuropathy due to complex I dysfunction. This defect of oxidative phosphorylation does not appear to depend on the low amounts of mitochondrial DNA multiple deletions detected in muscle biopsies.

Deficits in Functional Connectivity of Hippocampal and Frontal Lobe Circuits After Traumatic Axonal Injury

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Arch Neurol. 2011;68(1):74-84. doi:10.1001/archneurol.2010.342

Perfusion Computed Tomography in Transient Ischemic Attack

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Arch Neurol. 2011;68(1):85-89. doi:10.1001/archneurol.2010.320

Familial Aggregation of Dementia With Lewy Bodies

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Arch Neurol. 2011;68(1):90-93. doi:10.1001/archneurol.2010.319

Bilateral Deep Brain Stimulation of the Pallidum for Myoclonus-Dystonia Due to ε-Sarcoglycan Mutations: A Pilot Study

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Arch Neurol. 2011;68(1):94-98. doi:10.1001/archneurol.2010.338

Meta-analysis of the Association Between Variants in SORL1 and Alzheimer Disease

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Arch Neurol. 2011;68(1):99-106. doi:10.1001/archneurol.2010.346

Association of Antiepileptic Drugs With Nontraumatic Fractures: A Population-Based Analysis

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Arch Neurol. 2011;68(1):107-112. doi:10.1001/archneurol.2010.341

Cerebrospinal Fluid Profiles and Prospective Course and Outcome in Patients With Amnestic Mild Cognitive Impairment

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Arch Neurol. 2011;68(1):113-119. doi:10.1001/archneurol.2010.334
Images in Neurology

Unilateral White Matter Involvement in Krabbe Disease

Abstract Full Text
Arch Neurol. 2011;68(1):130-131. doi:10.1001/archneurol.2010.331

Iatrogenic Brain and Cervical Cord Magnetic Resonance Imaging Susceptibility Artifacts From Metallic Microemboli

Abstract Full Text
Arch Neurol. 2011;68(1):132-133. doi:10.1001/archneurol.2010.336
Observation

Suspecting Optic Neuritis, Diagnosing Bartonella Cat Scratch Disease

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Arch Neurol. 2011;68(1):122-126. doi:10.1001/archneurol.2010.345

Stridor as a Neonatal Presentation of Skeletal Muscle Sodium Channelopathy

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Arch Neurol. 2011;68(1):127-129. doi:10.1001/archneurol.2010.347
Book Reviews

Harrison’s Neurology in Clinical Medicine, 2nd ed

Abstract Full Text
Arch Neurol. 2011;68(1):134. doi:10.1001/archneurol.2010.327
Annual Reviewers List

Reviewers Who Completed a Review During 2010

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Arch Neurol. 2011;68(1):12-13. doi:10.1001/archneurol.2010.348
This Month in Archives of Neurology

This Month in Archives of Neurology

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Arch Neurol. 2011;68(1):15-16. doi:10.1001/archneurol.2010.335
Neurological Review

Preclinical Biomarkers of Parkinson Disease

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Arch Neurol. 2011;68(1):22-30. doi:10.1001/archneurol.2010.321

Spreading Depolarization: A Possible New Culprit in the Delayed Cerebral Ischemia of Subarachnoid Hemorrhage

Abstract Full Text
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Arch Neurol. 2011;68(1):31-36. doi:10.1001/archneurol.2010.226

Aneurysmal subarachnoid hemorrhage (SAH) is a devastating disease with a high mortality and morbidity rate. Gradual improvements have been made in the reduction of mortality rates associated with the disease during the last 30 years. However, delayed cerebral ischemia (DCI), the major delayed complication of SAH, remains a significant contributor to mortality and morbidity despite substantial research and clinical efforts. During the last several years, the predominant role of cerebral vasospasm, the long-accepted etiologic factor behind DCI, has been questioned. It is now becoming increasingly clear that the pathophysiology underlying DCI is multifactorial. Cortical spreading depression is emerging as a likely factor in this complex web of pathologic changes after SAH. Understanding its role after SAH and its relationship with the other pathologic processes such as vasospasm, microcirculatory dysfunction, and microemboli will be vital to the development of new therapeutic approaches to reduce DCI and improve the clinical outcome of the disease.

Clinical Trials

Open-Label Trial of Recombinant Human Insulin-like Growth Factor 1/Recombinant Human Insulin-like Growth Factor Binding Protein 3 in Myotonic Dystrophy Type 1

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Arch Neurol. 2011;68(1):37-44. doi:10.1001/archneurol.2010.227
ObjectiveTo evaluate the safety and tolerability of recombinant human insulin-like growth factor 1 (rhIGF-1) complexed with IGF binding protein 3 (rhIGF-1/rhIGFBP-3) in patients with myotonic dystrophy type 1 (DM1).DesignOpen-label dose-escalation clinical trial.SettingUniversity medical center.ParticipantsFifteen moderately affected ambulatory participants with genetically proven myotonic dystrophy type 1.InterventionParticipants received escalating dosages of subcutaneous rhIGF-1/rhIGFBP-3 for 24 weeks followed by a 16-week washout period.Main Outcome MeasuresSerial assessments of safety, muscle mass, muscle function, and metabolic state were performed. The primary outcome variable was the ability of participants to complete 24 weeks receiving rhIGF-1/ rhIGFBP-3 treatment.ResultsAll participants tolerated rhIGF-1/rhIGFBP-3. There were no significant changes in muscle strength or functional outcomes measures. Lean body muscle mass measured by dual-energy x-ray absorptiometry increased by 1.95 kg (P < .001) after treatment. Participants also experienced a mean reduction in triglyceride levels of 47 mg/dL (P = .002), a mean increase in HDL levels of 5.0 mg/dL (P = .03), a mean reduction in hemoglobin A1c levels of 0.15% (P = .03), and a mean increase in testosterone level (in men) of 203 ng/dL (P = .002) while taking rhIGF-1/rhIGFBP-3. Mild reactions at the injection site occurred (9 participants), as did mild transient hypoglycemia (3), lightheadedness (2), and transient papilledema (1).ConclusionsTreatment with rhIGF-1/rhIGFBP-3 was generally well tolerated in patients with myotonic dystrophy type 1. Treatment with rhIGF-1/rhIGFBP-3 was associated with increased lean body mass and improvement in metabolism but not increased muscle strength or function. Larger randomized controlled trials would be needed to further evaluate the efficacy and safety of this medication in patients with neuromuscular disease.Trial Registrationclinicaltrials.gov Identifier: NCT00233519

A Randomized Pilot Clinical Trial of the Safety of Pioglitazone in Treatment of Patients With Alzheimer Disease

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Arch Neurol. 2011;68(1):45-50. doi:10.1001/archneurol.2010.229
ObjectivesTo evaluate the safety of the peroxisome proliferator–activated receptor gamma agonist pioglitazone in nondiabetic patients with Alzheimer disease (AD) and to explore treatment effect sizes on clinical outcomes.DesignDouble-blind, placebo-controlled randomized controlled trial of 18-month duration.SettingTwo academic medical center outpatient clinics.PatientsNondiabetic patients meeting research criteria for probable AD were enrolled. Twenty-five of 29 subjects completed the study; no withdrawals were attributable to adverse effects.InterventionSubjects received pioglitazone (Actos), titrated to 45 mg daily, or matching placebo, and 200 IU of vitamin E daily. Patients maintained treatment with cholinesterase inhibitors and could begin memantine therapy when it became available during the study.Main Outcome MeasuresThe primary outcome was frequency of reported adverse effects (AEs). Secondary outcomes were measures of cognition, activities of daily living, neuropsychiatric symptoms, and global function.ResultsPeripheral edema was the principal AE occurring more frequently in subjects taking pioglitazone than placebo (28.6% vs 0%). This is consistent with the known AE profile of pioglitazone. No group differences in laboratory measures were identified. No significant treatment effect was observed on exploratory analysis of clinical efficacy.ConclusionsPioglitazone was generally well tolerated in this pilot study. There were no serious or unanticipated adverse events or clinical laboratory changes attributable to pioglitazone over a long-term exposure in nondiabetic patients with AD. The tolerability of pioglitazone in this population and peroxisome proliferator–activated receptor gamma effects in laboratory models of AD support further study of this drug class in earlier disease stages.Trial Registrationclinicaltrials.gov Identifier: NCT00982202.
From the Archives

Is Intranasal Midazolam Better Than Rectal Diazepam for Home Management of Acute Seizures?

Abstract Full Text
Arch Neurol. 2011;68(1):120-121. doi:10.1001/archneurol.2010.337
Correspondence

Warfarin Therapy Does Not Increase Risk of Symptomatic Intracerebral Hemorrhage in Eligible Patients After Intravenous Thrombolysis

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.323

Warfarin Therapy Does Not Increase Risk of Symptomatic Intracerebral Hemorrhage in Eligible Patients After Intravenous Thrombolysis—Reply

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.324

Uveitis and Pediatric Sarcoidosis: Does IgG Really Matter?

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.328

Uveitis and Pediatric Sarcoidosis: Does IgG Really Matter?—Reply

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.329

Prospective Research on Parkinson Nonmotor Symptoms

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.332

Prospective Research on Parkinson Nonmotor Symptoms—Reply

Abstract Full Text
Arch Neurol. 2011;68(1):135-138. doi:10.1001/archneurol.2010.333
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