Miller and Hou describe patients with developing dementia in whom visual artistic creativity emerges. Art in the context of dementia provides a unique window into cognitive processes and the altered and compensatory behavioral syndromes that appear.
Orlacchio and colleagues provide an elegant clinical assessment, linkage analysis, haplotype study, and expression of mutant spastin protein in cultured cells. A founder SPG4 mutation, N386S, was identified. Their study demonstrates evidence for further genetic heterogeneity in autosomal dominant hereditary spastic paraplegia. Editorial comment is provided by John K. Fink, MD, and Shirley Rainier, PhD.
Gordon et al show a significant difference in mean scores in patients with Parkinson disease receiving embryonic cell implants and those having sham surgery. Reaction time and movement time were improved in those receiving implants compared with control patients who underwent sham operation. Changes correlated with Unified Parkinson's Disease Rating Scale off scores at 4 and 12 months in patients younger than 60 years. Editorial perspective is provided by Roger N. Rosenberg, MD.
Patients with Parkinson disease (PD) may have elevated plasma homocysteine (Hcy) levels in part because levodopa metabolism produces Hcy. O'Suilleabhain and colleagues found that patients with PD and elevated plasma Hcy levels were more likely to be depressed and to perform worse on neuropsychometric tests as compared with patients who had PD and normal levels of Hcy. Elevated plasma Hcy levels may be an important risk factor for cognitive slowing in PD.
Smith and colleagues convincingly show that most individuals find neurogenetic testing to be beneficial, regardless of the result. Anxiety or depression may persist in some persons with either positive or negative results. Testing can have a demonstrable effect on family planning and interpersonal relationships.
Currie et al demonstrate that estrogen in postmenopausal women is associated with a reduced risk of developing Parkinson disease.
Statin therapy using atorvastatin causes a marked decrease in blood coenzyme Q10 concentration as reported by Rundek and colleagues. Widespread inhibition of coenzyme Q10 synthesis could explain the most commonly reported adverse effects of statins, especially exercise intolerance, myalgia, and myoglobinuria Figure 1.
Alfadhli and colleagues found a significantly lower number of CTG repeat expansions in a Kuwaiti population compared with Europeans, which offers a good explanation for the rare occurrence of myotonic dystrophy in Kuwait.
Rakocevic et al correlated titers of anti–glutamic acid decarboxylase antibodies in the serum and cerebrospinal fluid of patients with stiff-person syndrome with the degree of clinical severity. They found that antibody titers did not correlate with disease severity or duration. Thus, a positive antibody test result is a reliable marker of disease but is not useful to predict disease severity or duration.
Primary intracerebral hemorrhage (PICH) without microbleeds was shown by Jeong et al more common in younger patients, whereas PICH with microbleeds was more common in elderly patients with prominent ischemic change and frequent use of antithrombotics or anticoagulants.
Amaximum of four 5% lidocaine patches for up to 18 hours daily are well tolerated in patients with painful diabetic polyneuropathy, as reported by Barbano et al.
Significant reductions in 123I-labelled 2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane labeled with iodine I123 binding as a measure of the dopamine transporter was found by O'Brien and colleagues in the caudate and anterior and posterior putamen in patients who had dementia with Lewy bodies (DLB) compared with those with Alzheimer disease (AD) and healthy control subjects. Measuring dopamine transporter function in this way can be of considerable clinical utility to differentiate DLB from AD.
Patients with migraine were studied by Jen et al for mutations in the CACNA1A and ATP1A2 genes. They report that these 2 genes are not associated with more common migraine syndromes and are not the most common hemiplegic migraine genes.
Kieseier and colleagues show that interferon beta exhibits inhibitory effects at the posttranslational level of matrix metalloproteinase (MMP) activity, whereas simvastatin augments the proteolytic activity of MMP-2 and MMP-9, suggesting that statins exert not only anti-inflammatory but also proinflammatory effects. This dual mechanism of action should be considered given the recent interest in developing drugs for the treatment of multiple sclerosis.
Honjo et al found that a decrease in regional cerebral blood flow in the cerebellum of patients with spinocerebellar ataxia type 6 was associated with duration of illness, dysarthria and ataxia, and cerebellar atrophy. No remote effect of cerebellar hypoperfusion was found in these patients.
This Month in Archives of Neurology. Arch Neurol. 2004;61(6):828-829. doi:10.1001/archneur.61.6.828