Gelb et alArticle have developed explicit diagnostic criteria for Parkinson disease. Three levels of diagnostic confidence are differentiated. This will be a widely discussed and quoted article and is clearly user friendly for the clinician.
Storey et alArticle describe patients with spinocerebellar ataxia type 2 with CAG repeat expansions in which frontal and executive impairment was documented. The fronto-ponto-cerebellar circuit may be important for normal cognitive function. These findings are put into critical perspective in an insightful editorial by GeschwindArticle.
Kropp et alArticle report cases of Creutzfeldt-Jakob disease (CJD) with blurred vision, visual field restriction, metamorphopsia, or cortical blindness having the Heidenhain variant. The neuropathological manifestation is unique, and Western blots show prion proteinCJD type 1 in all cases. The clinical, neuropathological, and molecular genetic features of this unique form of CJD are reviewed.
Yasuda et alArticle report a missense mutation in a region not conserved between presenilin 1 and presenilin 2 that can cause Alzheimer disease. Previously, all known mutations were in nucleotides of the presenilin 1 or presenilin 2 genes that code for amino acids that are conserved between the 2 presenilins. The molecular genetics of presenilin mutations are expanded by this important report.
Bongioanni and colleaguesArticle report that increased tumor necrosis factor β binding might be linked to lymphocyte activation. Their data demonstrate that a major effect of interferon beta-1b treatment is decreased T-cell activation, which may be one of the basic mechanisms of response to therapy.
Schneider et alArticle have studied patients with HIV-associated myositis and polyneuropathies. In none of the HIV-associated disorders studied were substantial proportions of apoptotic T cells observed, as assessed by nuclear morphological testing and in situ labeling techniques. This study provides clear quantitative and qualitative information about basic immunological functions in HIV-related neuromuscular diseases.
Tagliati and colleaguesArticle have studied a large number of HIV-infected individuals in electrophysiological and laboratory studies. A series of interacting factors including age, immunosuppression, nutritional status, and chronic disease all contribute to distal peripheral nerve dysfunction in HIV infection, as reported in this thorough analysis.
The role of toxin exposure and the development of multiple system atrophy (MSA) is studied intensively by Hanna and colleaguesArticle. Exposure of patients with MSA to malathion, diazinon, formaldehyde, n-hexane, benzene, methyl-isobutyl-ketone, and pesticides is reported. This work raises the ominous specter that MSA, a sporadic disease, may indeed be of environmental toxic origin.
Winikates and JankovicArticle have attempted to differentiate Parkinson disease from vascular parkinsonism. They describe specific neurological features that in their view separate these 2 diseases.
Arteriovenous malformations are more likely to result in developmental learning disorders than are tumors or aneurysms. Lazar et alArticle suggest that these data support the notion that disorders of behavioral and intellectual function are sensitive markers of early cerebral status.
This Month in Archives of Neurology. Arch Neurol. 1999;56(1):15. doi:10.1001/archneur.56.1.15