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August 2019 - July 1959

Decade

Year

Issue

April 2012, Vol 69, No. 4, Pages 426-543

Editorial

Antibodies to Low Density Lipoprotein Receptor-Related Protein 4 in Seronegative Myasthenia Gravis

Abstract Full Text
Arch Neurol. 2012;69(4):434-435. doi:10.1001/archneurol.2011.2855
Original Contributions

Autoantibodies to Lipoprotein-Related Protein 4 in Patients With Double-Seronegative Myasthenia Gravis

Abstract Full Text
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Arch Neurol. 2012;69(4):445-451. doi:10.1001/archneurol.2011.2393

To determine whether patients with myasthenia gravis (MG) have serum antibodies to lipoprotein-related protein 4 (LRP4), Zhang and colleagues tested serum samples from 217 patients with MG with known status of serum antibodies to the acetylcholine receptor and muscle-specific kinase and samples from 121 control subjects (healthy individuals and those with other diseases) for antibodies to LRP4.

Acute Severe Animal Model of Anti–Muscle-Specific Kinase Myasthenia: Combined Postsynaptic and Presynaptic Changes

Abstract Full Text
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Arch Neurol. 2012;69(4):453-460. doi:10.1001/archneurol.2011.2200

Richman et al determine the pathogenesis of anti–muscle-specific kinase (MuSK) myasthenia, a newly described severe form of myasthenia gravis associated with MuSK antibodies characterized by focal muscle weakness and wasting and absence of acetylcholine receptor antibodies, and determine whether antibodies to MuSK can induce disease in the mature neuromuscular junction.

Autoimmune Autonomic Ganglionopathy With Reversible Cognitive Impairment

Abstract Full Text
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Arch Neurol. 2012;69(4):461-466. doi:10.1001/archneurol.2011.2372

Gibbons et al investigate the relationship between orthostatic hypotension, antibody titers, and cognitive impairment in patients with autoimmune autonomic ganglionopathy.

Corticomotoneuronal Integrity and Adaptation in Spinal Muscular Atrophy

Abstract Full Text
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Arch Neurol. 2012;69(4):467-473. doi:10.1001/archneurol.2011.1697

Farrar and coauthors used a prospective, double-center study design to gain further insight into the disease pathophysiologic process and potential adaptations by investigating whether cortical dysfunction or plasticity is a feature of spinal muscle atrophy (SMA).

Familial Adult Myoclonic Epilepsy: Recognition of Mild Phenotypes and Refinement of the 2q Locus

Abstract Full Text
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Arch Neurol. 2012;69(4):474-481. doi:10.1001/archneurol.2011.584

Crompton et al expands the phenotypic spectrum of familial adult myoclonic epilepsy, highlights diagnostic pointers to this underrecognized disorder, and refines the FAME2 genetic locus.

Original Contribution

Age-Dependent Structural Connectivity Effects in Fragile X Premutation

Abstract Full Text
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Arch Neurol. 2012;69(4):482-489. doi:10.1001/archneurol.2011.2023

Wang et al examine the effects of premutation alleles on major brain fiber tracts in males, who are at risk of developing fragile X-associated tremor/ataxia syndrome and may manifest subtle cognitive, social, and emotional disturbances before clinical involvement.

Loss of Myelin-Associated Glycoprotein in Kearns-Sayre Syndrome

Abstract Full Text
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Arch Neurol. 2012;69(4):490-499. doi:10.1001/archneurol.2011.2167

Lax and colleagues explore myelin components and mitochondrial changes within the central nervous system in patients with well-characterized mitochondrial disorders due to nuclear DNA or mitochondrial DNA mutations.

Factors Influencing Disease Progression in Autosomal Dominant Cerebellar Ataxia and Spastic Paraplegia

Abstract Full Text
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Arch Neurol. 2012;69(4):500-508. doi:10.1001/archneurol.2011.2713

In a prospective cohort study, Tezenas du Montcel et al evaluate disease progression in autosomal dominant cerebellar ataxia and spastic paraplegia and determine validity of clinical tools for therapeutic trials.

Saccade Abnormalities in Autopsy-Confirmed Frontotemporal Lobar Degeneration and Alzheimer Disease

Abstract Full Text
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Arch Neurol. 2012;69(4):509-517. doi:10.1001/archneurol.2011.1021

Boxer and coauthors determine the saccade abnormalities associated with autopsy-defined cases of frontotemporal lobar degeneration and of Alzheimer disease, because clinical frontotemporal dementia (FTD) syndromes can correspond to a number of different underlying neuropathologic FTD and non-FTD diagnoses.

Clinical Pathologic Conference

Progressive Weakness With Respiratory Failure in a Patient With Sarcoidosis

Abstract Full Text
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Arch Neurol. 2012;69(4):534-537. doi:10.1001/archneurol.2011.2572

Chaudhry et al describe a patient who presented with slowly progressive diffuse weakness, length-dependent sensory changes, and respiratory failure.

Images in Neurology

What Lies Beyond Plain Sight

Abstract Full Text
Arch Neurol. 2012;69(4):538-540. doi:10.1001/archneurol.2011.1850

Cranial Neuropathies in Multiple Sclerosis Defined by Magnetic Resonance Imaging and Fludeoxyglucose F 18 Positron Emission Tomography

Abstract Full Text
Arch Neurol. 2012;69(4):541-542. doi:10.1001/archneurol.2011.1936

Cerebral and Spinal Cord Subdural Hematomas

Abstract Full Text
Arch Neurol. 2012;69(4):543. doi:10.1001/archneurol.2011.2220
Observation

Addressing Diffuse Glioma as a Systemic Brain Disease With Single-Cell Analysis

Abstract Full Text
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Arch Neurol. 2012;69(4):523-526. doi:10.1001/archneurol.2011.2910

Using immunohistochemical analysis, Sahm et al analyze infiltration patterns of IDH1 mutant diffuse gliomas into the brain by identification of single tumor cells applying an antibody specific to mutant IDH1 R132H protein.

Use of Acetazolamide in Sulfonamide-Allergic Patients With Neurologic Channelopathies

Abstract Full Text
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Arch Neurol. 2012;69(4):527-529. doi:10.1001/archneurol.2011.2723

Platt and Griggs report the safe and successful use of the carbonic anhydrase inhibitor acetazolamide for treatment of patients with episodic ataxia and periodic paralysis who had been denied treatment because of a history of severe allergic reactions to antibiotic sulfonamides.

Blocked Muscle Fat Oxidation During Exercise in Neutral Lipid Storage Disease

Abstract Full Text
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Arch Neurol. 2012;69(4):530-533. doi:10.1001/archneurol.2011.631

Laforêt and colleagues studied a 37-year-old woman with neutral lipid storage disease (NLSD) with myopathy, who cycled while lipid oxidation was assessed using U-13C palmitate tracer dilution technique. The goal was to determine whether impaired exercise capacity in NLSD with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role.

This Month in Archives of Neurology

This Month in Archives of Neurology

Abstract Full Text
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Arch Neurol. 2012;69(4):427-428. doi:10.1001/archneurol.2011.1449
Neurological Review

Molecular Pathophysiology and Disease-Modifying Therapies for Spinal and Bulbar Muscular Atrophy

Abstract Full Text
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Arch Neurol. 2012;69(4):436-440. doi:10.1001/archneurol.2011.2308

Katsuno et al outline the molecular pathophysiology, summarize the therapeutic strategies currently being developed, and discuss the future direction of translational research of spinal and bulbar muscular atrophy.

From JAMA

Bypassing Trouble

Abstract Full Text
Arch Neurol. 2012;69(4):518-520. doi:10.1001/archneurol.2011.3486

Treatment of Arteriovenous Malformations: A Meta-analysis

Abstract Full Text
Arch Neurol. 2012;69(4):521-522. doi:10.1001/archneurol.2011.3514
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