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This Month in Archives of Neurology
July 2005

This Month in Archives of Neurology

Arch Neurol. 2005;62(7):1036-1037. doi:10.1001/archneur.62.7.1036
Statins and the Risk of Dementia

Rea and colleagues in the Cardiovascular Health Cognition Study studied community-dwelling adults aged 65 years and older for the risk of incident all-cause and type-specific dementia associated with time-dependent statin therapy compared with never use of lipid-lowering agents. They report that in this cohort study, statin therapy was not associated with a decreased risk of dementia. Statin therapy for prevention of dementia is not supported by this comprehensive study.

Transthyretin-Related Familial Amyloidotic Polyneuropathy

Ando et al review current clinicopathological, biochemical, molecular genetic, and epidemiological aspects of transthyretin-related familial amyloidotic polyneuropathy. They review an up-to-date description of the molecular genetic mutations and the diagnostic value of using them for obtaining an accurate and rapid diagnosis. Liver transplantation is an effective form of therapy.

Figure.
Clinical symptoms in patients with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP), shown as the total numbers of amyloid-associated tissues, from previous reports. The polyneuropathic form is often accompanied by cardiac amyloid deposition.

Clinical symptoms in patients with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP), shown as the total numbers of amyloid-associated tissues, from previous reports. The polyneuropathic form is often accompanied by cardiac amyloid deposition.

Insulin Resistance in Cognitive Impairment: The InCHIANTI Study

Geroldi and colleagues studied the association between cognitive impairment, with and without subcortical features, and insulin resistance in an elderly community-dwelling population. They report that cognitive impairment with subcortical features but not cognitive impairment without subcortical features is associated with biochemical and clinical features of insulin resistance syndrome. In epidemiological populations, insulin resistance might contribute to cognitive impairment through a vascular mechanism. Editorial perspective is provided by Suzanne Craft, PhD.

Follow-up of 89 Asymptomatic Patients With Adrenoleukodystrophy Treated With Lorenzo's Oil

Moser and colleagues assessed the effect of 4:1 glyceryl trioleate–glyceryl trierucate (Lorenzo’s oil) on disease progression in asymptomatic boys with X-linked adrenoleukodystrophy who have normal brain magnetic resonance images. Of note, they report that plasma C26:0 fatty acid reduction by Lorenzo’s oil was associated with reduced risk of developing magnetic resonance image abnormalities. Therapy with Lorenzo’s oil is recommended in asymptomatic boys with X-linked adrenoleukodystrophy who have normal brain magnetic resonance images. Editorial perspective is provided by Raymond Ferri, MD, PhD and Phillip F. Chance, MD.

Insular Cortex Infarction in Acute Middle Cerebral Artery Territory Stroke

Fink et al studied insular involvement in middle cerebral artery territory infarction and its relationship with infarcts in the remainder of the middle cerebral artery territory, stroke severity, and clinically relevant cardiovascular conditions. They report that the insular cortex is involved in almost one half of patients with nonlacunar ischemic middle cerebral artery territory strokes.

Spectrum of Mutations in Biopsy-Proven CADASIL

Peters and colleagues studied the spectrum of NOTCH3 mutations in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and detected 54 distinct mutations in 120 (96%) of 125 patients. Sixty percent of the mutations were located in exon 4 and 86% in exons 2 through 6. There were 117 missense mutations and 3 in-frame deletions.

Sporadic and Familial Dementia With Ubiquitin-Positive Tau-Negative Inclusions

Godbolt et al studied the clinical features of 11 familial (autosomal dominant) and 18 sporadic cases of frontotemporal lobar degeneration. Patients had neuropathologic confirmation of frontotemporal dementia with ubiquitin-positive tau-negative inclusions. Within the behavioral group, clinical features were indistinguishable in sporadic and familial cases. Semantic dementia was seen only in sporadic cases. An accurate diagnosis of the specific type of frontotemporal lobar degeneration requires neuropathologic confirmation because of the range of clinical phenotypes in frontotemporal lobar degeneration.

Different Patterns of Magnetic Resonance Imaging Atrophy for Frontotemporal Lobar Degeneration Syndromes

Short and colleagues evaluated the clinical presentations and magnetic resonance imaging patterns of atrophy in 59 patients with frontotemporal lobar degeneration and 26 patients with probable Alzheimer disease. Patients with frontotemporal lobar degeneration who had altered personal conduct had significant bifrontal atrophy whereas patients with semantic dementia had significant left temporal atrophy and bifrontal atrophy compared with other groups. Disinhibited behavior and hyperphagia correlated with right frontal atrophy and fluent, anomic aphasia correlated with left temporal atrophy.

Myopathic Changes With Severe Acute Respiratory Syndrome

Leung et al examined patients with severe acute respiratory syndrome for muscle pathologic abnormalities to correlate with clinical weakness and increased creatine kinase levels. Postmortem skeletal muscles from 8 patients who died from severe acute respiratory syndrome were studied by light and electron microscopy and immunohistochemistry. They report a spectrum of myopathic changes, including focal myofiber necrosis.

Hereditary Spastic Paraplegia With SPG4 and SPG3A Mutational Analysis in Korean Patients

Park et al studied 18 patients with hereditary spastic paraplegia (11 autosomal dominant and 7 sporadic) for mutations in the SPG4 and SPG3A genes. They report 8 different SPG4 mutations, 7 of which have not been reported elsewhere. No mutation was found in the SPG3A gene. In this population, a high frequency of SPG4 gene mutations in autosomal dominant hereditary spastic paraplegia was found (7 [64%] of 11 patients), so it is important to screen this gene in Korean patients with autosomal dominant hereditary spastic paraplegia.

Initial Glasgow Coma Scale Score Predicts Outcome After Thrombolysis for Posterior Circulation Stroke

Tsao and colleagues measured thrombolytic treatment times for posterior circulation stroke and factors that might predict clinical outcome. They report that thrombolytic therapy for posterior circulation stroke is beneficial even when instituted 8 hours after symptom onset. Level of consciousness, as measured by the Glasgow Coma Scale score, is a more important predictor of outcome than the initial National Institutes of Health Stroke Scale score.

Serum S100B as a Marker for Long-term Outcome and Infarct Volume in Middle Cerebral Artery Infarction

Foerch et al studied the value of S100B serum levels to predict long-term outcome and infarct size in acute middle cerebral artery infarctions. They report that single S100B values obtained at 48 and 72 hours after stroke onset provide the highest predictive values related to functional outcome and infarct size in nonlacunar middle cerebral artery infarction.

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