Research Awards
Solomon L. Moshé, M.D.
Albert Einstein College of Medicine
"Clinical investigation of autonomic dysfunction in Rett children under evaluation for epilepsy"
1-Year Award: $49,173
Research Sponsor: Julian Robertson
Final Report (November 2005)
Rett Syndrome (RTT) results in abnormal regulation of vital functions such as breathing, heart rate and blood pressure. These functions are regulated automatically by a specialized circuits and brain regions known collectively as the autonomic nervous system. RTT is also often associated with seizures and epilepsy. Even in the absence of RTT, seizures can impact on the control of the autonomic nervous system. The autonomic manifestations of seizures in RTT have not been clearly delineated. This question is important because clinical observations suggest that autonomic dysfunction in RTT is associated with disturbances of the cardiac cycle and respiration that increase the risk of sudden death. We suspect that the underlying abnormality of autonomic function associated with RTTS results an increased susceptibility to seizure-induced disruptions of vital functions, and increased the risk of death or impairment in children with RTT.
We studied the impact of seizures on the autonomic nervous system in children with RTT and in relatively homogeneous group of patients without RTT, those temporal lobe epilepsy, with a particular emphasis on the regulation of the heart rate. Patients were recruited from those admitted to an epilepsy monitoring unit for evaluation and management of their seizures.
Our preliminary results suggest that in patients without RTT, seizures can result in transiently increased activity of the autonomic nervous system followed by a more prolonged decrease in autonomic activity that characteristically outlasts the seizure. We have as of yet monitored too few patients with RTT to compare their seizures to patients with temporal lobe epilepsy. We plan to continue monitoring epilepsy patients with and without RTT to better characterize seizure-related autonomic changes, their potential to precipitate hemodynamically significant arrhythmias, and their impact upon respiratory patterns.
Solomon L. Moshé, M.D.Albert Einstein College of Medicine
"Clinical investigation of autonomic dysfunction in Rett children under evaluation for epilepsy"
1-Year Award: $49,173
Research Sponsor: Julian Robertson
Final Report (November 2005)
Rett Syndrome (RTT) results in abnormal regulation of vital functions such as breathing, heart rate and blood pressure. These functions are regulated automatically by a specialized circuits and brain regions known collectively as the autonomic nervous system. RTT is also often associated with seizures and epilepsy. Even in the absence of RTT, seizures can impact on the control of the autonomic nervous system. The autonomic manifestations of seizures in RTT have not been clearly delineated. This question is important because clinical observations suggest that autonomic dysfunction in RTT is associated with disturbances of the cardiac cycle and respiration that increase the risk of sudden death. We suspect that the underlying abnormality of autonomic function associated with RTTS results an increased susceptibility to seizure-induced disruptions of vital functions, and increased the risk of death or impairment in children with RTT.
We studied the impact of seizures on the autonomic nervous system in children with RTT and in relatively homogeneous group of patients without RTT, those temporal lobe epilepsy, with a particular emphasis on the regulation of the heart rate. Patients were recruited from those admitted to an epilepsy monitoring unit for evaluation and management of their seizures.
Our preliminary results suggest that in patients without RTT, seizures can result in transiently increased activity of the autonomic nervous system followed by a more prolonged decrease in autonomic activity that characteristically outlasts the seizure. We have as of yet monitored too few patients with RTT to compare their seizures to patients with temporal lobe epilepsy. We plan to continue monitoring epilepsy patients with and without RTT to better characterize seizure-related autonomic changes, their potential to precipitate hemodynamically significant arrhythmias, and their impact upon respiratory patterns.