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Rett Syndrome (Rett's Disorder)

An inherited neurological developmental disorder that is associated with X-LINKED INHERITANCE and may be lethal in utero to hemizygous males. The affected female is normal until the age of 6-25 months when progressive loss of voluntary control of hand movements and communication skills; ATAXIA; SEIZURES; autistic behavior; intermittent HYPERVENTILATION; and HYPERAMMONEMIA appear. (From Menkes, Textbook of Child Neurology, 5th ed, p199)
Also Known As:
Rett's Disorder; Syndrome, Rett; Rett's Syndrome; Autism Dementia Ataxia Loss of Purposeful Hand Use Syndrome; Cerebroatrophic Hyperammonemia; Autism, Dementia, Ataxia, and Loss of Purposeful Hand Use; Rett Disorder; Cerebroatrophic Hyperammonemias; Hyperammonemia, Cerebroatrophic; Hyperammonemias, Cerebroatrophic; Retts Syndrome; Syndrome, Rett's; Autism-Dementia-Ataxia-Loss of Purposeful Hand Use Syndrome
Networked: 1475 relevant articles (40 outcomes, 123 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neurodevelopmental Disorders
2. Epilepsy (Aura)
3. Tuberous Sclerosis (Bourneville's Disease)
4. Seizures (Absence Seizure)
5. Autistic Disorder (Autism)

Experts

1. Hayek, Joussef: 41 articles (01/2022 - 08/2009)
2. Neul, Jeffrey L: 33 articles (01/2022 - 04/2004)
3. De Felice, Claudio: 32 articles (01/2022 - 08/2009)
4. Leoncini, Silvia: 32 articles (01/2022 - 08/2009)
5. Signorini, Cinzia: 31 articles (01/2022 - 08/2009)
6. Zoghbi, Huda Y: 31 articles (01/2021 - 01/2002)
7. Ciccoli, Lucia: 29 articles (01/2022 - 08/2009)
8. Valacchi, Giuseppe: 28 articles (01/2022 - 08/2009)
9. Pecorelli, Alessandra: 27 articles (01/2022 - 07/2011)
10. Percy, Alan K: 27 articles (01/2022 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Rett Syndrome:
1. Brain-Derived Neurotrophic Factor (BDNF)IBA
2. Methyl-CpG-Binding Protein 2IBA
3. trofinetideIBA
4. beta-Endorphin (Endorphin, beta)IBA
5. Naltrexone (ReVia)FDA LinkGeneric
6. N- dimethyl- 2,2- diphenyl- 3- furanmethanamine hydrochloride tetrahydro-NIBA
7. mecasermin (Increlex)FDA Link
8. Biogenic Amines (Biogenic Amine)IBA
9. Insulin-Like PeptidesIBA
10. Anticonvulsants (Antiepileptic Drugs)IBA

Therapies and Procedures

1. Therapeutics
2. Deep Brain Stimulation
3. Gastrostomy
4. Splints (Splint)
5. Spinal Fusion (Spondylosyndesis)