Plott syndrome
| Classification according to ICD-10 | |
|---|---|
| J38.0 | Paralysis of the vocal folds and the larynx |
| ICD-10 online (WHO version 2019) | |
The Plott syndrome is a very rare congenital disease with the main features of paralysis of the muscle cricoarytaenoideus posterior with closing the glottis with intellectual disabilities .
Synonyms are: abductor paralysis, laryngeal - mental retardation; English Vocal Cord Dysfunction, Familia; Laryngeal abductor paralysis
The name refers to the first author of the first description from 1964 by the American neurologist Dwight Plott .
distribution
The frequency is given as less than 1 in 1,000,000, the inheritance is probably X-linked - recessive , the cause is not yet known.
Clinical manifestations
Clinical criteria are:
- Congenital permanent paralysis of the larynx abductor muscle with inspiratory stridor , cyanosis , swallowing disorders , speech disorders
- mental disability
Individual evidence
- ↑ a b c Abductor paralysis, laryngeal - mental retardation. In: Orphanet (Rare Disease Database).
- ↑ D. PLOTT: Congenital laryngeal-abductor paralysis due to nucleus ambiguus dysgenesis in three brothers. In: The New England Journal of Medicine . Vol. 271, September 1964, pp. 593-597, doi: 10.1056 / NEJM196409172711203 , PMID 14172969 .
