Ramon Syndrome
Overview
A rare genetic condition characterized by cherubism, enlarged gums, epilepsy, mental deficiency and excessive body hair (hypertrichosis).
Symptoms
- Seizures
- Mental deficiency
- Gingival fibromatosis
- Cherubism
- Fibrous dysplasia of upper jaw bone
- Stunted growth
- Growth of excess hair
Causes
birth trauma (inadequate oxygen supply to the brain, blood incompatibility, or hemorrhage),perinatal infection,anoxia (after respiratory or cardiac arrest)
Treatment
Generally, treatment of epilepsy consists of anticonvulsant therapy to reduce the number of future seizures. The most commonly prescribed drugs include phenytoin, carbamazepine, phenobarbital, gabapentin, or primidone administered individually for generalized tonic-clonic seizures and complex partial seizures. Valproic acid, clonazepam, and ethosuximide are commonly prescribed for absence seizures. Gabapentin and felbamate are also anticonvulsant drugs. A patient taking anticonvulsant medications requires monitoring for toxic signs: nystagmus, ataxia, lethargy, dizziness, drowsiness, slurred speech, irritability, nausea, and vomiting.