Rett Syndrome
Rett Syndrome is a progressive neurological disorder estimated to affect 1:10,000- 1:15,000 live female births.
The disorder is generally characterized by:
- Apparently normal psychomotor development during the first six months of life;
- Progressive developmental stagnation, altered communicative ability, loss of play interest, and partial or complete loss of acquired purposeful movement between 6 and 18 months of age;
- Autistic behaviors such as hand wringing/squeezing, clapping/tapping, and mouthing appear after purposeful behaviors are lost; and
- The appearance of gait apraxia and truncal ataxia between ages 1 and 4 years.
Two common complications of Rett Syndrome are seizures and swallowing dysfunction. Both of which are associated with a high risk of aspiration and consequent pulmonary infection.
Immobility, or limited mobility exhibited as part of Rett Syndrome impedes normal airway clearance and compromises the ability to clear aspirated material.
Scoliosis, commonly seen in Rett Syndrome, can further impair airway clearance in that it restricts the movement of the thorax and limits the ability to take a deep breath. The cough reflex is therefore compromised.
To clear excessive secretions, and minimize the complications of the disease, people with Rett Syndrome often require frequent Airway Clearance Therapy.
Sources
Armstrong DD. Review of Rett Syndrome. Journal of Neuropathology and Experimental Neurology. 1997; 56: 843.
Hagberg BA. Rett syndrome: clinical peculiarities, diagnostic approach and possible cause. Pediatr Neurol. 1989; 5: 75–83.
Ribeiro RA, Romano AR, Birman EG, Alves Mayer MP. Oral manifestations in Rett syndrome: a study of 17 cases. Pediatric Dentistry. 1997; 19: 349.
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