Theodor Heller first described a severe regression of adaptive function in normally developing children, something he termed dementia infantilis, over one 100 years ago. Dementia infantilis is most closely related to the modern diagnosis, childhood disintegrative disorder. We translate Heller's paper, Über Dementia Infantilis, and discuss similarities in presentation between Heller's cases, and a group of children with childhood disintegrative disorder. In particular we discuss a prodromal period of affective dysregulation described by Heller, and also evident in our sample, but not previously described in any detail since the publication of Über Dementia Infantilis.
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http://dx.doi.org/10.1007/s10803-012-1559-z | DOI Listing |
Front Neurol
June 2023
Laboratorio de Diagnóstico Genómico, Instituto Nacional de Medicina Genómica, Mexico City, Mexico.
Objectives: To report the first Mexican case with two novel mutations causing primary ovarian failure, uterus , and early-onset dementia secondary to leukoencephalopathy.
Methods: Detailed clinical, clinimetric, neuroimaging features, muscle biopsy with biochemical assays of the main oxidative phosphorylation complexes activities, and molecular studies were performed on samples from a Mexican female.
Results: We present a 41-year-old female patient with learning difficulties since childhood and primary amenorrhea who developed severe cognitive, motor, and behavioral impairment in early adulthood.
BMC Psychiatry
October 2015
CESP, INSERM U1178, Univ., Paris-Descartes, USPC, Paris, 75014, France.
Background: Deletions and mutations involving the SHANK3 gene lead to a nonspecific clinical presentation with moderate to profound intellectual disability, severely delayed or absent speech, and autism spectrum disorders (ASD). Better knowledge of the clinical spectrum of SHANK3 haploinsufficiency is useful to facilitate clinical care monitoring and to guide molecular diagnosis, essential for genetic counselling.
Case Presentation: Here, we report a detailed clinical description of a 10-year-old girl carrying a pathogenic interstitial 22q13.
J Autism Dev Disord
February 2013
Yale Child Study Center, New Haven, CT 06519, USA.
Theodor Heller first described a severe regression of adaptive function in normally developing children, something he termed dementia infantilis, over one 100 years ago. Dementia infantilis is most closely related to the modern diagnosis, childhood disintegrative disorder. We translate Heller's paper, Über Dementia Infantilis, and discuss similarities in presentation between Heller's cases, and a group of children with childhood disintegrative disorder.
View Article and Find Full Text PDFBrain Dev
June 2003
Department of Child and Adolescent Psychiatry, Bispebjerg Hospital, DK-2400, Copenhagen, Denmark.
In 1908 a Viennese remedial educator Theodor Heller described six children under the name of dementia infantilis who had insidiously developed a severe mental regression between the 3rd and 4th years of life after normal mental development. Neuropathological and other medical conditions are sometimes associated with this disorder, but contrary to earlier belief this is not typical. Interest in childhood disintegrative disorder has increased markedly in recent years and in this review attention is given to more recently published cases based on ICD-9, ICD-10 and DSM-IV diagnostic systems.
View Article and Find Full Text PDFClin Psychol Rev
January 2000
University of Georgia, Department of Educational Psychology, Athens 30602-7143, USA.
First termed Dementia Infantilis by Theodore Heller in 1908, Childhood Disintegrative Disorder (CDD) has had a history longer than that of Autistic Disorder. Presently, CDD is classified as a Pervasive Developmental Disorder in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders. The characteristics most often cited as distinguishing CDD from Autistic Disorder, another one of the Pervasive Developmental Disorders, is the age of onset and evidence of normal development prior to the presence of symptomatology.
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