This Month in Archives of Neurology

Pandolfo describes his research and related molecular research on the cause of Friedreich ataxia. Yeast that is deficient in the frataxin homologue accumulates iron in mitochondria and shows increased sensitivity to oxidative stress. His review brilliantly describes what is currently known about the dysregulation of iron metabolism. Article

Garbern and colleagues review the mutations in the proteolipid gene responsible for Pelizaeus-Merzbacher disease. Information provided here is relevant not only for Pelizaeus-Merzbacher disease but also for the molecular pathogenesis of other demyelinating diseases. Article

DeBiasi and Tyler show how important polymerase chain reaction has become in detecting minute amounts of DNA or RNA in tissues or fluids to establish the diagnosis of neurological infectious diseases, including human immunodeficiency virus infection, herpes simplex virus infection, rabies, varicella-zoster virus infection, and bacterial, mycobacterial, fungal, rickettsial, and protozoan diseases. Polymerase chain reaction has become an indispensable tool for the clinician, and this well-focused review is a very handy resource.Article

Bergsneider and colleagues describe new and significant findings in the use of subatmospheric siphoning techniques for the treatment of shunt-dependent hydrocephalus. This is an important advance with clear clinical implications. Pleasure highlights the clinical importance of these findings in a well-focused editorial. ArticleArticle

Johnson et al provide clinical and pathologic data describing the frontal lobe variant of Alzheimer disease. Their findings provide distinctive clinical and pathologic features of this clinical entity that will be of considerable value in patient evaluation.Article

Tumani et al describe glutamine synthetase levels in the lumbar cerebrospinal fluid of patients with Alzheimer disease as a new and potentially useful diagnostic marker for Alzheimer disease. Article

Sharma et al describe autonomic system functions in patients with Huntington disease and extend our knowledge concerning the pathophysiological characteristics of this genetic disorder. Article

Di Patre et al describe the quantitative progression of Alzheimer disease in patients evaluated with both biopsy and subsequent autopsy and provide new, compelling information on the natural history of this dementing illness.Article

Mohr et al have measured the levels of depression in patients with multiple sclerosis prior to, during, and after the administration of interferon beta-1a therapy. Levels of depression that were significant before therapy often showed significant reduction at initiation of therapy and subsequently returned to initial levels.Article

Lopez and colleagues examined behavioral symptoms and the use of psychiatric medication in patients with Alzheimer disease and used these data as a means to predict the subsequent progression of dementia. Their studies show that the use of antipsychotic and sedative agents can affect the natural course of the disease.Article

Wilson and colleagues have examined changes in the cognitive function of older persons in a community population and the relationship of these changes to age and Alzheimer disease. In those patients free of Alzheimer disease, they found little evidence of cognitive decline. Advancing age alone does not necessarily yield cognitive decline. Article

De Jonghe et al describe a family with a new autosomal dominant motor-sensory neuropathy that does not map to established Charcot-Marie-Tooth genetic loci. The molecular-clinical relationships in these different genetic diseases with similar phenotypes are reviewed in a fine editorial by Pleasure. ArticleArticle

Jacob et al describe a 27-year-old patient with the onset of Pick disease confirmed by cerebral biopsy findings showing Pick cells and tau-positive Pick bodies. The early occurrence of Pick disease is uncommon, but it is necessary to consider this possibility in a patient with a dementing disease in early adulthood.Article