Reproducibility of semi-quantitative assessment of aortic valve calcification and valve motion on echocardiography: a small-scale study.

BACKGROUND: Aortic stenosis (AS) is the most common degenerative valve disease in high income countries. While hemodynamic metrics are commonly used to assess severity of stenosis, they are impacted by loading conditions and stroke volume and are often discordant. Anatomic valve assessments such as aortic valve calcification (AVC) and valve motion (VM) during transthoracic echocardiography (TTE) can offer clues to disease severity. The reliability of these semi-quantitatively assessed anatomic imaging parameters is unknown. METHODS: This is a retrospective study of semi-quantitative assessment of AVC and valve VM on TTE. TTEs representing a range of AS severities were identified. The degree of calcification of the aortic valve and the degree of restricted VM were assessed in standard fashion. AVC scores and valve motion were assessed by readers with varied training levels blinded to the severity of AS. Correlation and inter-reader reliability between readers were assessed. RESULTS: 420 assessments (210 each for AVC and VM) were collected for 35 TTEs. Correlation of AVC for imaging trainees (fellows and students, respectively), ranged from 0.49 (95% CI 0.18-0.70) to 0.62 (95% CI 0.36-0.79) and 0.58 (95% CI 0.30-0.76) to 0.54 (95% CI 0.25-0.74) for VM. Correlation of anatomic assessments between echocardiographer-assigned AVC grades was r = 0.76 (95% CI 0.57-0.87)). The correlation between echocardiographer-assigned assessment of VM was r = 0.73 (95% CI 0.53-0.86), p < 0.00001 for both. For echocardiographer AVC assessment, weighted kappa was 0.52 (0.32-0.72), valve motion weighted kappa was 0.60 (0.42-0.78). CONCLUSION: There was good inter-reader correlation between TTE-based semi-quantitative assessment of AVC and VM when assessed by board certified echocardiographers. There was modest inter-reader reliability of semi-quantitative assessments of AVC and VM between board certified echocardiographers. Inter-reader correlation and reliability between imaging trainees was lower. More reliable methods to assess TTE based anatomic assessments are needed in order to accurately track disease progression. CLINICAL TRIAL NUMBER: STUDY00003100.

The frontal arousal rhythm.

This study involved 50 patients with the frontal arousal rhythm (FAR), a rare pattern (0.22%) seen in children with an average age of 4 yrs who show long bursts of bifrontal sharp waves at a theta frequency upon arousal from stage II sleep. The pattern is seen mainly in males and children with histories of seizures (70%), usually with generalized attacks. Although cognitive/behavior disorders were seen in an overlapping 56%, this incidence was not different from a control group from the same clinic with normal EEGs.

The "Mozart effect" on epileptiform activity.

The "Mozart Effect," using the Piano Sonata in D Major (K.448), was examined in patients with seizures. In 23 of 29 instances significant decreases in epileptiform activity were noted from patients even in coma, with status epilepticus or with periodic lateralized epileptiform discharges (PLEDs). The effect may be immediate or require 40-300 sec to manifest itself. The change in the amount of ictal activity in one patient in coma was from 62% before the music to 21% during Mozart. Amplitudes of these discharges also have often decreased. Examples of PLEDs on both temporal areas are shown in which the effect was only on the left temporal area but in other patients only on the right temporal area. Brain maps during the music showed theta and alpha activity decreased on the central areas, while delta waves increased on the frontal midline area. The basis of this effect is likely that the superorganization of the cerebral cortex with its highly structured radial columns seen throughout both hemispheres may resonate with the superior architecture of Mozart's music.

Brain SPECT imaging and treatment with IVIg in acute post-infectious cerebellar ataxia: case report.

A 19 year-old man presented with pharyngitis and cervical lymphadenopathy, followed by vomiting and acute cerebellar ataxia. Serologic studies were consistent with a recent Epstein-Barr virus infection. Although contrast-enhanced brain computed tomography and MRI scans were normal, brain perfusion single photon emission tomography (SPECT) examination using 99mTc-HMPAO, performed on the 15th day of illness, showed marked cerebellar hyperperfusion, suggesting a diagnosis of acute post-infectious cerebellitis. After treatment with intravenous human immunoglobulin (IVIg, 2 g kg-1 over three days), progressive neurologic improvement occurred over two weeks. A brain SPECT study repeated after two additional weeks demonstrated a normal perfusion pattern. We conclude that brain perfusion SPECT examination is useful in identifying post-infectious cerebellitis and in monitoring its clinical course. In addition, IVIg may be helpful in treating this condition.

Long term changes in patients with hypsarrhythmia-infantile spasms: 505 patients, up to 43 years follow-up.

This study involved 505 patients with the EEG pattern of hypsarrhythmia (H) and clinical attacks of infantile spasms (IS) studied over a 51 year period from 1945 until 1996. The total number of EEGs was 1300 and changes in the EEG and type of clinical seizure were followed for up to 43 yrs in a given patient. Although H and IS usually occur together in a given patient, 15% showed a disparity between the electrographic and clinical pattern, usually within a 6-12 mo period. The duration of H was usually < 1 mo but lasted as long as 7 yrs. The duration was dependent in part on the onset age and a very early onset of < 2 mo was associated with a short-lasting H and generally a good prognosis, as was an onset of 8-12 mo. Slightly more than half of these patients had an onset age of > 1 yr, likely in part related to a relatively large number studied before the introduction of the measles, mumps, and rubella vaccine. The next pattern after H was usually around 3 yrs of age, often focal discharges on the occipital or temporal areas or bilateral spike and wave complexes. The major changes in the EEG over time were the progressive increase in bilateral spike and wave complexes and temporal lobe discharges, in addition to an increase in other focal discharges at 6-9 yrs of age. Slow waves on the temporal areas and diffuse slowing became prominent in adulthood. The types of clinical attacks that became prominent in adulthood were generalized tonic-clonic and complex partial seizures. Other types of seizures most often occurred between the late teens and early 20's with absence attacks seen especially at 8-10 yrs of age.