Clinical and biological progress over 50 years in Rett syndrome

H Leonard, S Cobb, J Downs - Nature Reviews Neurology, 2017 - nature.com
In the 50 years since Andreas Rett first described the syndrome that came to bear his name,
and is now known to be caused by a mutation in the methyl-CpG-binding protein 2 (MECP2) …

The utility of DXA assessment at the forearm, proximal femur, and lateral distal femur, and vertebral fracture assessment in the pediatric population: 2019 ISCD official …

DR Weber, A Boyce, C Gordon, W Högler… - Journal of Clinical …, 2019 - Elsevier
Dual-energy X-ray absorptiometry (DXA) is widely used in the evaluation of bone fragility in
children. Previous recommendations emphasized total body less head and lumbar spine …

[HTML][HTML] Consensus guidelines on managing Rett syndrome across the lifespan

C Fu, D Armstrong, E Marsh, D Lieberman… - BMJ paediatrics …, 2020 - ncbi.nlm.nih.gov
Background Rett syndrome (RTT) is a severe neurodevelopmental disorder with complex
medical comorbidities extending beyond the nervous system requiring the attention of health …

Gastrointestinal and nutritional problems occur frequently throughout life in girls and women with Rett syndrome

KJ Motil, E Caeg, JO Barrish, S Geerts… - Journal of pediatric …, 2012 - journals.lww.com
Objective: We conducted a nationwide survey to determine the prevalence of common
gastrointestinal and nutritional disorders in Rett syndrome (RTT) based on parental …

Exclusive expression of MeCP2 in the nervous system distinguishes between brain and peripheral Rett syndrome-like phenotypes

PD Ross, J Guy, J Selfridge, B Kamal… - Human molecular …, 2016 - academic.oup.com
Rett syndrome (RTT) is a severe genetic disorder resulting from mutations in the X-linked
MECP2 gene. MeCP2 protein is highly expressed in the nervous system and deficiency in …

The trajectories of sleep disturbances in Rett syndrome

K Wong, H Leonard, P Jacoby… - Journal of sleep …, 2015 - Wiley Online Library
Rett syndrome is a rare neurodevelopmental disorder usually affecting females, and is
associated with a mutation in the MECP 2 gene. Sleep problems occur commonly and we …

Rett syndrome and epilepsy: an update for child neurologists

A Dolce, B Ben-Zeev, S Naidu, EH Kossoff - Pediatric neurology, 2013 - Elsevier
Rett syndrome, a neurogenetic disorder predominantly affecting females, has many
characteristic features including psychomotor retardation, impaired language development …

The natural history of scoliosis in females with Rett syndrome

J Downs, I Torode, K Wong, C Ellaway, EJ Elliott… - Spine, 2016 - journals.lww.com
Study Design. Population-based longitudinal observational study. Objective. To describe the
prevalence of scoliosis in Rett syndrome, structural characteristics and progression, taking …

Linking MECP2 and pain sensitivity: The example of Rett syndrome

J Downs, SM Géranton, A Bebbington… - American journal of …, 2010 - Wiley Online Library
Recent animal studies suggest links between MeCP2 function and sensitivity to pain. This
study investigated the nature and prevalence of atypical pain responses in Rett syndrome …

Methodological standards and interpretation of video‐electroencephalography in adult control rodents. A TASK 1‐WG 1 report of the AES/ILAE Translational Task …

SD Kadam, R D'Ambrosio, V Duveau, C Roucard… - …, 2017 - Wiley Online Library
In vivo electrophysiological recordings are widely used in neuroscience research, and video‐
electroencephalography (vEEG) has become a mainstay of preclinical neuroscience …