Marchiafava-Bignami Disease:Clinical and MRI Findings in Two Patients

We'herein report two alcoholic patients with highly suspicious Marchiafava- Bignami disease(MBD) who developed acute neurologic dysfunction and showed characteristic abnormalities in the corpus callosum on the brain MRIs: focal low signal lesion (s) in the genu or splenium of the corpus callosum, and diffuse callosal atrophy on the sagittal Tl-weighted images

A Study on Selective Cerebellar Degeneration Following ,β-Fluorethyl Acetate Poisoning

This is to report clinical and radiological findings in 7 patients with selective cerebellar degeneration following ,B-fluoroethyl acetate intoxication. ,B-Fluoroethyl acetate, an ethyl ester of fluoroacetate which is a metabolic inhibitor of Krebs cycle is used as a rodenticide. Following initial stage of coma from intoxication, the patients woke up to show selective cerebellar dysfunction, often so severe as not to be able to sit or stand unsupported. They improved gradually over several months to years with variable degrees of residual cerebellar dysfunction. Gait disturbance and dysarthria were the most prominent and persistent factors of the cerebellar dysfunction, whereas mild nystagmus was rarely seen. Cognitive function was not impaired. Cerebellar atrophy became noticeable on CT and MRI 4 weeks after poisoning, and progressed over time even with clinical improvement. Cerebellar degeneration contrasts with pallidal degeneration following carbon monoxide poisoning. ,B-Fluoroethyl acetate may be selectively toxic to the cerebellum

Identification of PSEN1 and APP Gene Mutations in Korean Patients with Early-Onset Alzheimer's Disease

Although mutations in three genes, amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2), have been identified as genetic causes of early-onset Alzheimer's disease (EOAD), there has been a single report on a PSEN1 mutation in Koreans. In the present study, we performed a genetic analysis of six Korean patients with EOAD. Direct sequencing analysis of the APP, PSEN1 and PSEN2 genes revealed two different mutations of the PSEN1 gene (G206S and M233T) and one mutation of the APP gene (V715M) in three patients with age-at-onset of 34, 35, and 42 yr, respectively. In addition, two patients with age-at-onset of 55 and 62 yr, respectively, were homozygous for APOE ε4 allele. One woman had no genetic alterations. These findings suggest that PSEN1 and APP gene mutations may not be uncommon in Korean patients with EOAD and that genetic analysis should be provided to EOAD patients not only for the identification of their genetic causes but also for the appropriate genetic counseling

The effects of galantamine treatment on attention and its relationship with cognition and activities of daily living in patients with mild to moderate Alzheimer's disease

Background and Purpose The positive effects of galantamine on cognition and activities of daily living (ADL) in Alzheimer's disease (AD) are thought to be mediated via improvements in attention. The purpose of this study was to determine the effect of galantamine on attention in AD patients using a computerized attention test and to elucidate the relationship between improvements in attention and change in cognition and ADL. Methods In this multicenter, open-label, prospective study, patients with mild to moderate AD received galantamine and then submitted to computerized attention tests, the Alzheimer's Disease Assessment Scale-cognitive subscale, and instrumental ADL (IADL) at baseline, 4 weeks, and 12 weeks. The differences in reaction time on computerized tests were explored relative to the changes in cognition and IADL. Results After 12 weeks of taking the trial medication there was a significant reduction from baseline levels in the choice reaction time (baseline, 5,216 +/- 3,650 sec; 12 weeks, 4,139 +/- 2,920 sec; p<0.01) and the simple reaction time (baseline, 1,089 +/- 782 sec; 12 weeks, 908 +/- 606 sec; p<0.01). Correlation analyses of changes in choice or simple reaction times relative to cognition and ADL measures yielded no significant associations. The improvement in attention observed at 4 weeks of galantamine treatment was not associated with any significant changes in outcome measures at the end of trial. Conclusions This study found no significant association between the improvement in attention after treatment with galantamaine and changes in cognition and ADL in patients with mild to moderate AD, despite the significant improvement in attention over the course of the treatment.N