bilateraal anterieur operculair syndroom (aandoening) | | bilateraal anterieur operculair syndroom | | Foix-Chavany-Marie-syndroom
| | Foix Chavany Marie syndrome | | Bilateral anterior opercular syndrome Facio-pharyngo-glosso-masticatory diplegia
| | Acortico-subcortical suprabulbar or pseudobulbar palsy of the lower cranial nerves, with characteristics of severe dysarthria and dysphagia associated with bilateral central facio-pharyngo-glosso-masticatory paralysis, with prominent automatic-voluntary dissociation in which involuntary movements of the affected muscles are preserved. Less than 150 cases have been described in the literature so far. Can occur at any age. Patients have severe speech disturbances and most are mute.Chewing and swallowing are severely impaired. Caused by developmental or acquired bilateral lesions of the anterior opercula. In children, it presents congenitally (bilateral opercular polymicrogyria) or as an acquired disorder due to encephalitis, epilepsy and neurodegenerative disorders. The syndrome is generally sporadic but some familial cases have been described. |
| Id | 720956003 | Status | Primitive |
DHD Diagnosis thesaurus reference set |
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | Target | G52.7 | Term | Aandoeningen van multipele hersenzenuwen |
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SNOMED CT to Orphanet simple map | 2048 |
SNOMED CT to ICD-10 extended map | Target | G52.7 | Rule | TRUE | Advice | ALWAYS G52.7 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | Correlation | SNOMED CT source code to target map code correlation not specified |
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