Frontotemporal Degeneration in a Child - 06/10/17

Doi : 10.1016/j.pediatrneurol.2017.04.001

Tyler Terrill, MD ^a, Juan M. Pascual, MD, PhD ^a,^b,^c,^d,^⁎
^a Rare Brain Disorders Program, Department of Neurology and Neurotherapeutics, The University of Texas Southwestern Medical Center, Dallas, Texas
^b Department of Physiology, The University of Texas Southwestern Medical Center, Dallas, Texas
^c Department of Pediatrics, The University of Texas Southwestern Medical Center, Dallas, Texas
^d Eugene McDermott Center for Human Growth & Development/Center for Human Genetics, The University of Texas Southwestern Medical Center, Dallas, Texas

^∗Communications should be addressed to: Dr. Pascual, The Once Upon a Time Foundation Professorship in Pediatric Neurologic Diseases, Director; Rare Brain Disorders Program; UT Southwestern Medical Center; 5323 Harry Hines Blvd.; Mail code 8813; Dallas, TX 75390-8813.Rare Brain Disorders ProgramUT Southwestern Medical Center5323 Harry Hines Blvd.Mail code 8813DallasTX75390-8813

Abstract

Background

There is a predilection for the frontal and temporal lobes in certain cases of dementia in the adult, leading to the syndrome of frontotemporal dementia. However, this syndrome has seemed to elude the developing brain until now.

Methods and results

We describe an example of apparently selective neurodegeneration of the frontal and temporal regions during development associated with some of the clinical, magnetic resonance imaging, and fludeoxyglucose positron emission tomography (FDG PET) scan features of canonical frontotemporal dementia in the adult. This patient does not have any of the common frontotemporal dementia-causing mutations or known progressive brain disorders of children.

Conclusion

This patient illustrates that symptomatic, selective, and progressive vulnerability of the frontal and temporal lobes is not restricted to adulthood, expanding the phenotype of frontotemporal degeneration.

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Keywords : Pick disease, atrophy, apraxia, neurodegeneration, MRI

Plan

Introduction

Patient, methods, and results

Discussion

	Author contributions: J.M.P. contributed to conception and design, obtaining funding, and supervision of the study. T.T. and J.M.P. contributed to acquisition, analysis, and interpretation of data; drafting of the manuscript; and critical revision of the manuscript for important intellectual content.
	Competing interests: None.

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Vol 72

P. 62-64 - juillet 2017 Retour au numéro

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Frontotemporal Degeneration in a Child - 06/10/17

Abstract

Background

Methods and results

Conclusion

Plan

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