Navigation Links
Fragile period of childhood brain development could underlie epilepsy
Date:8/23/2009

BOSTON A form of partial epilepsy associated with auditory and other sensory hallucinations has been linked to the disruption of brain development during early childhood, according to a study led by researchers at Beth Israel Deaconess Medical Center (BIDMC).

Described in today's Advance On-line issue of Nature Medicine, these new findings provide the first genetic link between childhood brain development and a seizure disorder that lasts throughout adulthood, and also identify a new pathway that controls how neuron circuits are "pruned" and matured.

"During early childhood roughly between the ages of one and five the brain undergoes a period of major circuit remodeling," explains senior author Matthew Anderson, MD, PhD, a principal investigator in the Departments of Neurology and Pathology at BIDMC. "Our discovery that a familial form of temporal lobe epilepsy can develop at this point demonstrates the fragility of the brain during this critical period."

The new findings focus on the development of synapses, the connections between brain cells.

"At birth, the brain is loaded with excitatory synapses which help make nerve cells 'fire,'" explains Anderson, who is also an Assistant Professor of Neurology and Pathology at Harvard Medical School. "However, if these excess synapses are not adequately 'pruned,' they can overgrow, leading to excessive transmission of excitatory signals and the development of pathological conditions, including learning disabilities and autism in addition to epilepsy."

Using a genetically engineered mouse model created in his laboratory, together with brain slice patch-clamp electrophysiology techniques, Anderson and his scientific team found that a mutant form of the LGI1 (leucine-rich glioma-inactivated 1) gene was preventing the normal brain development.

"The first clue was our discovery that LGI1 is not expressed until the exact time when excitatory synapses are matured," said Anderson. "We subsequently learned that the mLGI1 gene was indeed prohibiting excitatory synapses from being adequately pruned, leading to an increased excitability of circuits in the brain which left it prone to excessive synchronous discharges that are characteristic of epilepsy."

Autosomal dominant lateral temporal lobe epilepsy (ADLTE) is characterized by frequent partial seizures (two to five per month) that are associated with auditory or other sensory auras. Tonic-clonic seizures also occur in the majority of ADLTE patients, but are infrequent, developing only about once a year.

"These partial seizures can have a significant impact on a patient's quality of life," notes Anderson. "Because patients can be disoriented and excessively tired following a seizure event, their day-to-day lives can sometimes be seriously disrupted. And when it comes to driving and other activities, there is still a real danger associated with this condition.

"One important reason to identify genetic causes of epilepsy is the hope that these discoveries will eventually lead to new therapies," he adds. "By identifying this new pathway, we may have found a new target for future drug development."


'/>"/>

Contact: Bonnie Prescott
bprescot@bidmc.harvard.edu
617-667-7306
Beth Israel Deaconess Medical Center
Source:Eurekalert

Related medicine news :

1. $21.8 Million NIH Grant for Fragile X Research
2. Stem cell research produces a key discovery for Fragile X Syndrome
3. Brain Defect Helps Drive Fragile X Syndrome
4. Fragile X Foundations Sign Merger Agreement
5. Fragile X Syndrome Corrected in Mice
6. Drosophila drug screen for fragile X syndrome finds promising compounds and potential drug targets
7. The Area's Most Fragile Babies Have a New Home
8. The Most Fragile Babies in the Nations Capital Have a New Home
9. Earlier Testing Needed for Fragile X Syndrome
10. NIH Plan Charts Course to Better Treatments and Cure for Most Common Known Genetic Cause of Autism: Fragile X
11. Microtek Medical Receives Early Termination of Hart-Scott-Rodino Act Waiting Period for Its Proposed Acquisition by Ecolab
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/13/2016)... (PRWEB) , ... February 13, 2016 , ... ... School of Nursing with an in-kind gift of a VeinViewer® Vision vein ... as they learn how to start an IV and draw blood, combining technology ...
(Date:2/13/2016)... ... February 13, 2016 , ... DDi ... Management Solution Providers list for its expertise in eClinical Solutions. DDi has built ... serve the technology needs of global clients. DDi provides smarter technology for Clinical ...
(Date:2/12/2016)... ... February 12, 2016 , ... The law firm of Morrow, Morrow, Ryan & ... purpose of these scholarships is to encourage applicants to pursue a degree in their ... these two parishes. , “We have available jobs in St. Landry and Evangeline ...
(Date:2/12/2016)... ... February 12, 2016 , ... CDRH Enforcement Trends: , Back to the Future , ... , As Winston Churchill said, “Those who don’t learn from history are doomed to ... expect when they come knocking this year. But that takes time. , Take a ...
(Date:2/12/2016)... , ... February 12, 2016 , ... As a former ... schedule, a demanding job, and no time to decompress, Rabinowitz found herself drawn to ... to meditation for its impact on her life, implementing a 20-minute-per-day meditation practice with ...
Breaking Medicine News(10 mins):
(Date:2/11/2016)... 2016 The primary goal of this research ... on the usage of liquid biopsy. Key information the ... - Timeframe of liquid biopsy adoption amidst future users ... organization type - Sample inflow to conduct liquid biopsy ... serum, and so on. - Correlation analysis of sample ...
(Date:2/11/2016)... (NasdaqCM: PDEX) today announced financial results for its fiscal 2016 ... its Quarterly Report on Form 10-Q for the second quarter ... today. --> --> Quarter ... --> Net sales for the three months ended December ... from $2.8 million for the three months ended December 31, ...
(Date:2/11/2016)...  Kindred Biosciences, Inc. (NASDAQ: KIN ), a ... of pets, today announced the submission to FDA of ... Application (NADA) for Zimeta™ (dipyrone injection, KIND-012).  Positive topline ... for the control of pyrexia (fever) in horses were ... --> The Chemistry, Manufacturing, and Controls ...
Breaking Medicine Technology: