This Month in Archives of Neurology FREE

Schenk and colleaguesArticle describe their recent research using the 42-amino-acid form of the β-amyloid peptide to immunize transgenic mice carrying the human amyloid precursor protein codon 717 mutation. Amyloid-containing plaques of Alzheimer type are dramatically reduced in the immunized transgenic mouse brain. This research sets the stage for similar immunization approaches in patients with Alzheimer disease.

Kokontis and GutmannArticle bring us up to date with current therapies of the major neuromuscular diseases. This review is highly informative and of great value for the clinician.

Wong and SternbergArticle describe recent advances in neuroimmune interactions as they relate directly to the practice of neurology and understanding neurological disease. Immunological assays currently employed to diagnose and monitor neurological disease are included. They provide us with insights and explanations of these clinically important diagnostic procedures and their clinical relevance.

Vorgerd and colleaguesArticle describe a double-blind, placebo-controlled, crossover study using oral creatine to treat patients with McArdle disease. This is the first controlled study indicating that creatine supplementation does improve skeletal muscle function in McArdle disease. This important observation is highlighted and put into proper perspective in a most thoughtful editorial by Ronald G. HallerArticle.

Wiszniewska et alArticle describe the clinical risk factors and imaging characteristics of leukoaraiosis. Their correlative findings suggest that leukoaraiosis is primarily related to small-vessel disease.

Achiron and BarakArticle have studied the rate of progression from probable to clinically definite multiple sclerosis, and they define rapid progression as occurrence of a second attack within one year following the relapse. Their clinical and magnetic resonance imaging observations and correlations are of considerable importance and are discussed in a highly focused and compelling manner in an editorial by Elliot Frohman et alArticle.

Molinuevo and colleaguesArticle describe a series of patients with Parkinson disease treated by subthalamic nucleus stimulation and the subsequent need for levodopa. Bilateral subthalamic nucleus stimulation improved parkinsonian symptoms and decreased the need for levodopa in this group. Details of their approach and conclusions will be of great interest to the clinician in treating these patients with advanced Parkinson disease.

Lee and colleaguesArticle describe the usefulness of triphasic perfusion computed tomography in diagnosing acute middle cerebral artery occlusion and defining the degree of ischemia and infarction in a rapid and noninvasive manner. These findings provided the means to pursue their subsequent therapy study, presented in the following article.

Lee and colleaguesArticle, in a follow-up study, demonstrate in a series of patients that triphasic perfusion computed tomography is of considerable value in monitoring and treating acute middle cerebral artery territory stroke with recombinant tissue-type plasminogen activator (rtPA). Rapid identification and treatment of acute stroke may be advanced by this approach.

Oliveira-Filho et alArticle show that diffusion-weighted magnetic resonance imaging is well suited for the detection of acute small infarcts as compared with other imaging modalities. Acute and chronic lesions due to small-penetrator vessel infarction can be readily detected and differentiated.

Filippi and colleaguesArticle demonstrate that diffusion-weighted magnetic resonance imaging scans are able to identify multiple sclerosis lesions readily. Their study also shows that widespread increased water diffusion can be measured in normal-appearing white matter from patients with multiple sclerosis and that such changes are partially independent of larger abnormalities. This study extends the level of sensitivity in appreciating the variety and type of imaging defects present in acute multiple sclerosis lesions.

Schifitto and colleaguesArticle have studied the products of immune activation, including cytokines and lipid membrane derivatives, in the pathogenesis of neurological features, including autonomic dysfunction, associated with human immunodeficiency virus type 1 (HIV-1) infection. Their findings provide immunological information that can be used to measure HIV-1 disease progression and autonomic nervous system performance.

Benatar and EastmanArticle describe a series of patients with human immunodeficiency virus (HIV)–related polyradiculopathy manifesting as isolated severe motor symptoms confined to the legs. It remains unclear whether this clinical picture should be regarded as a variant of the Guillain-Barre syndrome or whether it represents a unique disorder associated with early HIV infection.

Kirkwood et alArticle compare presymptomatic HD gene carriers with nongene carriers at risk for HD. They found subtle changes in motor function, speed of movement, and reaction time in HD gene carriers who did not have sufficient signs to make a clinical diagnosis of HD. The spectrum of minor preclinical features of HD is provided, and this has a practical value.

Verhagen and collegesArticle describe a patient with autosomal dominant vestibulocochlear dysfunction caused by a unique COCH mutation. The phenotypic variability of this syndrome is defined.

Mitoma and colleaguesArticle have described carefully the gait characteristics in patients with pontine medial tegmental lesions. According to their data, impairment of the spinal-cerebellar circuit may be contributing to the underlying gait disturbance.

Collins and colleaguesArticle describe an 82-year-old woman with pathologically confirmed Creutzfeldt-Jakob disease who had a novel mutation in the prion protein gene. This patient had no family history of dementia, and unexpectedly, full sequencing of the prion protein gene open reading frame revealed a single novel mutation. This case highlights the problematic issues in human transmissible spongiform encephalopathies, as illustrated by disease penetrance and age of onset.

Isojärvi and TapanainenArticle describe 3 patients who developed a reproductive endocrine disorder while taking valproate. The evaluation of ovarian structure and function should be considered in women of reproductive age taking valproate for epilepsy, especially if they develop menstrual cycle disturbances during treatment.