UTA, UT Southwestern researchers’ findings challenge current approach to rare pediatric neurological disorder

The University of Texas at Arlington News Releases



UTA’s Jianzhong Su, professor and chair of mathematics

Researchers at The University of Texas at Arlington and the University of Texas Southwestern Medical Center have discovered that diet changes and early diagnosis could help outcomes for patients with Glucose Transporter Type 1 Deficiency, a rare pediatric neurological disorder that can cause motor developmental problems and trigger seizures and epilepsy.
“We set out to clarify issues surrounding the diagnosis and treatment of the disease through the creation of the first registry to collect detailed medical and treatment history from a broad range of patients, including phenotypes or traits, treatment history and genetic information,” said Dr. Juan Pascual, a neurologist and director of the Rare Brain Disorders Program at UT Southwestern’s O’Donnell Brain Institute.
“Our data suggests that the single most important factor determining positive patient outcome is an early age of diagnosis, and also demonstrates that alternative dietary therapies can radically improve patient health over the long-term.”
The results were published today in JAMA Neurology in an article titled: “Clinical Aspects of Glucose Transporter Type 1 Deficiency: Information from a Global Registry.”  UTA’s Jianzhong Su, professor and chair of mathematics, and Dr. Jian Hao, a member of Dr. Su’s research team, were co-authors on the paper.
The research results reflected data from a registry of 181 patients located worldwide, for the period from Dec. 1, 2013 to Dec. 1, 2016.
“We used several statistical methods tailored to address the age of onset of various forms of the disease, associated manifestations, natural history, treatment efficacy and diagnostic procedures,” Dr. Su said.
“These factors were correlated in a predictive mathematical model designed to guide prognosis on the basis of clinical features at diagnosis,” he added.
The three main novel results are:
The effectiveness of alternative dietary therapies, such as the “modified” Atkins diet. The term “modified” describes the lower carbohydrate limit compared to Atkins recommendations ...

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