نتایج جستجو برای: hypotonia

تعداد نتایج: 1818  

Journal: :Paediatrics & Child Health 2005

Journal: :Journal of Orthopaedics and Trauma 2011

Journal: :Neurology 2001
C K Kong C H Ko S F Tong C W Lam

Dopa-responsive dystonia (DRD) is an autosomal dominant disorder typically presenting as dystonia with diurnal variability. Described is an 8-year-old boy who had had waddling gait, generalized hypotonia, and proximal weakness since early childhood. He responded well to low-dose L-dopa. He had a point mutation of the GTP cyclohydrolase I gene. The patient's father and sister had the same mutati...

Journal: :American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics 2010
Beata A Nowakowska Ewa Obersztyn Krystyna Szymańska Monika Bekiesińska-Figatowska Zhilian Xia Christian B Ricks Ewa Bocian David W Stockton Krzysztof Szczałuba Magdalena Nawara Ankita Patel Daryl A Scott Sau Wai Cheung Timothy P Bohan Paweł Stankiewicz

We present four patients, in whom we identified overlapping deletions in 5q14.3 involving MEF2C using a clinical oligonucleotide array comparative genomic hybridization (CGH) chromosomal microarray analysis (CMA). In case 1, CMA revealed an approximately 140 kb deletion encompassing the first three exons of MEF2C in a 3-year-old patient with severe psychomotor retardation, periodic tremor, and ...

Journal: :Archives of Disease in Childhood 1955

Journal: :Developmental Medicine & Child Neurology 2007

Journal: :Movement disorders : official journal of the Movement Disorder Society 2006
Emmanuel Roze Marie Vidailhet Nenad Blau Lisbeth Birk Moller Diane Doummar Thierry Billette de Villemeur Anne Roubergue

Little information is available on the long-term course and adult outcome of patients with 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency. We describe the course of a 32-year-old woman with hypotonia, dystonia, choreoathetosis, mental retardation, behavioral disturbances, and incomplete puberty due to PTPS deficiency. From the age of 6 months she developed progressive hypotonia and chor...

2012
Joon Woo Choi Eun-Ju Kim Byung Woo Min Jong Seouk Ban Sang Gon Lee Ji-Hyang Lee

Prader-Willi syndrome is characterized by infantile hypotonia, childhood-onset obesity, short stature, mental retardation, hyperphagia, hypogonadism. After infantile hypotonia phase, patient is prone to morbid obesity due to hyperphagia. Complications associated with morbid obesity are recognized as the main risk factors for death the lifespan of patients with Prader-Willi syndrome. We experien...

2004
R van Toorn

The pre-, periand postnatal history is important. Enquire about the quality and quantity of fetal movements, breech presentation and the presence of either polyor oligohydramnios. The incidence of breech presentation is higher in fetuses with neuromuscular disorders as turning requires adequate fetal mobility. Documentation of birth trauma, birth anoxia, delivery complications, low cord pH and ...

2012
Su Bin Son Jung Mi Chun Kyung Ah Kim Sun Young Ko Yeon Kyung Lee Son Moon Shin

Congenital myotonic dystrophy type 1 (DM1) presents severe generalized weakness, hypotonia, and respiratory compromise after delivery with high mortality and poor prognosis. We presented a congenital DM1 of premature twins in the 30th week of gestation. These twins were conceived by in vitro fertilization (IVF). Both babies presented apnea and hypotonia and had characteristic facial appearance....

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