Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia.

Context: Sickle cell anemia (SCA) is a chronic illness causing progressive deterioration in quality of life. Brain dysfunction may be the most important and least studied problem affecting individuals with this disease.

Objective: To measure neurocognitive dysfunction in neurologically asymptomatic adults with SCA vs healthy control individuals.

Gray matter perfusion correlates with disease severity in ALS.

Objective: The goal of this study is to determine if regional brain perfusion, as measured by arterial spin labeling (ASL) MRI, is correlated with clinical measures of amyotrophic lateral sclerosis (ALS) disease severity. The presence of such a relationship would indicate a possible role for ASL perfusion as a marker of disease severity and upper motor neuron involvement in ALS.

Methods: Disease severity was assessed in 16 subjects with ALS (age 54 +/- 11) using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) and the pulmonary function measure, forced vital capacity (FVC).

Orbitofrontal cortex and dynamic filtering of emotional stimuli.

Event-related potentials (ER) were recorded in response to mildly aversive somatosensory and auditory stimuli. Patients with orbitofrontal lesions exhited enhanced ERPs (i.e.