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:5/23/2016)... ... May 23, 2016 , ... ... and sexual objectification, according to Georgia State University researchers. , In ... in the fifth, sixth and seventh grades shared their experiences with strained ...
(Date:5/23/2016)... ... May 23, 2016 , ... Cosmetic Town, an ... about cosmetic procedures, began updating their library of over 300 encyclopedic medical articles. ... articles in order to feature the latest breakthroughs in plastic surgery procedures since ...
(Date:5/23/2016)... WASHINGTON, D.C. (PRWEB) , ... May 23, 2016 , ... ... Phyllis Greenberger, MSW, has decided to move on from that role. , “No one ... years as did Phyllis Greenberger,” said SWHR Founder Florence Haseltine, MD, PhD. “We offer ...
(Date:5/23/2016)... ... 2016 , ... In Pursuit of Balance (IPOB) co-founders Jasmine Hirsch and Rajat ... the end of 2016. , “We created IPOB to change the dialogue around ... back on what we have achieved, we’re gratified by the response of wine producers, ...
(Date:5/23/2016)... ... May 23, 2016 , ... Four Circles Recovery Center, a ... is proud to announce the launch of its new extended care program, Fifth Circle. ... treatment at Four Circles or another primary treatment program, Fifth Circle is designed to ...
Breaking Medicine News(10 mins):
(Date:5/19/2016)... SAN FRANCISCO, Calif. , May 19, 2016 /PRNewswire/ ... a biopharmaceutical company developing novel therapeutics for the treatment ... announced that new GBT440 data will be presented in ... 21 st Congress in Copenhagen ... ongoing Phase 1/2 GBT440-001 study in sickle cell disease ...
(Date:5/19/2016)... 2016 BioLineRx Ltd. (NASDAQ/TASE: BLRX) ... treatment of multiple cancer and hematological indications, will be ... abstract titled " Clinical response in relapsed/refractory AML patients ... BL-8040, a potent CXCR4 antagonist; results of a Phase ... at the European Hematology Association 21st Congress, to be ...
(Date:5/19/2016)... CAMBRIDGE, Massachusetts , May 19, 2016 ... pharmaceuticals and diagnostics based on the gut microbiome, is pleased ... Cambridge, MA to support its operations ... office is located at Kendall Square, the heart of ... global hub for transforming scientific insights into new drugs. The ...
Breaking Medicine Technology: