Brain microstructural changes and cognitive function in non-demented essential tremor patients: a diffusion tensor imaging study.

BACKGROUND: Essential tremor (ET) is disease with both motor and non-motor features. Notable among the non-motor features is cognitive impairment. While this impairment has been attributed to cortico-thalamo-cerebellar pathway pathology, it is likely that a more complicated involvement of brain structures underlies cognitive function in ET. OBJECTIVE: To evaluate the brain microstructural changes of both white matter and grey matter in ET using region of interest based diffusion tensor imaging (DTI), and to correlate these changes with cognitive function assessed during detailed neuropsychological testing. METHOD: Thirty-five non-demented ET patients with a range of cognitive function (Clinical Dementia Rating = 0-0.5, mean age = 57.5 ± 16.7 years, age range = 23-76 years) underwent a comprehensive neuropsychological evaluation and brain magnetic resonance imaging, including DTI. DTI findings were reported as fractional anisotropy, average diffusion coefficient, these values were evaluated for 32 ROIs. Cognitive domains included attention, visuospatial functions, executive function, verbal memory, visual memory, and language. Domain Z-scores were calculated each cognitive domain and compared for each brain region. RESULTS: Microstructural changes in prefrontal cortical areas (dorsolateral, ventrolateral), paralimbic and limbic structures (posterior cingulate cortex, precuneus, hippocampus), basal ganglia (substantia nigra, putamen, caudate nucleus) and white matter bundles (corpus callosum, anterior thalamic radiation, longitudinal fasciculus, frontooccipital fasciculus, etc.) correlated with specific domains of cognitive function in ET patients. CONCLUSION: These data suggest that not only the cerebello thalamocortical pathway, but numerous other brain structures are related to level of cognitive performance and possibly underlie cognitive dysfunction in ET.

Evaluation of cardiac arrhythmias by electrocardiographic markers in pediatric patients who have tuberous sclerosis without cardiac rhabdomyoma.

BACKGROUND: Tuberous sclerosis (TS) is an autosomal dominant and hereditary disorder. Cardiac rhabdomyoma and arrhythmias are the most deleterious risk factors linked to TS. Although arrhythmias in pediatric patients with TS who have cardiac rhabdomyoma have been frequently reported, arrhythmia in patients who have TS without rhabdomyoma is rarely reported in the literature. The study aimed to assess the susceptibility of pediatric patients who have TS without cardiac rhabdomyoma to cardiac arrhythmia using electrocardiographic (ECG) markers. METHODS: This prospective study included 10 patients who had TS without cardiac rhabdomyoma. The control group was made up of 30 healthy children of the same age and sex as the patient group. P wave, P wave dispersion, QT dispersion, QTc dispersion, TP-e interval, and TP-e interval dispersion were calculated on 12-lead surface ECGs for each patient in both groups and compared. RESULTS: P wave, P wave dispersion, QT dispersion, and QTc dispersion were found to be significantly higher in the patient group (P<0.001). Furthermore, patients had a greater Tp-e interval and Tp-e interval dispersion than healthy children (P<0.001). CONCLUSION: Pediatric patients with TS without cardiac rhabdomyoma might be prone to atrial and ventricular arrhythmias according to their prolonged ECG markers. Our findings suggest that patients with TS without cardiac rhabdomyoma need close monitoring for atrial and ventricular arrhythmias.