Rigi-Fede Syndrome

Rigi-Fede syndrome

Rigi-Fede syndrome is a rare disease characterized by periodic fever, convulsions and epileptiform seizures.

This syndrome was first described by Italian doctors Augusto Riga and Federico Fede at the end of the 19th century. The disease usually appears between the ages of 3 months and 3 years. The main symptoms are attacks of fever lasting from 2 to 4 days, followed by an increase in body temperature to 39-41°C. The attack is accompanied by convulsions, vomiting, and headache.

The causes of Rigi-Fede syndrome are not fully understood. It is believed that it may be associated with mutations in the SCN1A, ATP1A2 or PCDH19 genes. Diagnosis is based on the clinical picture and exclusion of other diseases.

Treatment is mainly symptomatic - anticonvulsants, antipyretics. The prognosis is usually favorable, in most cases the symptoms disappear by 6-7 years. However, in some patients, attacks persist into older age.



Rigi-Fede syndrome is a rare disease that was first described by Italian doctors in 1881. This is a neurological disease that often occurs in newborns and infants.

The name of the syndrome comes from the names of Italian doctors - Riga and Fede. Antonio Riga was a 19th century physician who first described this pathology in his patient. Francesco Fede was an Italian pediatrician who also contributed to the study of this disease.

The main symptom of Rigi-Fede syndrome is a painful swelling that occurs on the tongue of newborns and infants. The tumor usually appears yellowish or white in color and can reach sizes of up to several centimeters.

In most cases, the tumor is not cancerous and disappears on its own within a few weeks or months. However, in some cases, the tumor may continue to grow and cause discomfort when eating and swallowing.

In addition to the swelling on the tongue, people with Rigi-Fede syndrome may also have swollen lymph nodes in the neck and may have trouble breathing and swallowing.

Conflicting data about the causes of this syndrome are still unclear, but some researchers associate it with dysfunction of the immune system, as well as certain environmental factors.

Treatment for Rigi-Fede syndrome is usually not required, as the tumor disappears on its own. However, if the tumor causes discomfort or makes digestion and breathing difficult, surgery may be required.

In conclusion, Rigi-Fede syndrome is a rare condition that is characterized by the appearance of a painful swelling on the tongue in newborns and infants. The tumor usually goes away on its own, but in some cases surgery may be required. Despite the fact that the causes of this syndrome are still unclear, its diagnosis and treatment are at a fairly high level.