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  1. N. Stepanenko1,
  2. E. Fedorov1,
  3. S. Salugina1,
  4. S. Feoktistova2
  1. 1V.A.Nasonova Research Institute of Rheumatology, Pediatric, Moscow, Russian Federation
  2. 2Russian New University (RosNOU), Psychology and Pedagogics, Moscow, Russian Federation


Background: Monogenic auto-inflammatory diseases (mAID) are a group of severe chronic multisystemic diseases with recurring episodes of fever and other manifestations that significantly affect the patients’ life quality. Moreover, the hyper expression of pro-inflammatory cytokines (IL1β, etc.) observed in these patients may have a negative effect on the central nervous system.

Objectives: to study the state of the cognitive and emotional spheres in children suffering from monogenic auto-inflammatory diseases.

Methods: there were examined 22 children at the age of 7 to 17 years old diagnosed with CAPS-9, TRAPS-8, FMF-5. Among them there were 12 boys and 10 girls. The diagnosis in all the patients was confirmed through detection of pathogenic mutations in the NLRP3, TNFRSF1A and MEFV genes. The following methods were used: a clinical conversation; memory diagnostics (learning by heart of 10 words, a pictogram using cues taking into account the patients’ age); attention diagnostics (Schulte tables); thinking diagnostics (establishing a sequence of events, “four is a crwod”, simple analogies, interpretation of proverbs); emotional and communicative fields (the Eight-Color Luscher Test; CMAS (adaptation by A. Prikhozhan); STAI test, a drawing called “an animal that does not exist” and “a house-a tree-a man”).

Results: The memory study revealed in all patients with TRAPS and FMF high and medium values of short-term and long-term memory, in patients with CAPS - a low level of short-term auditory-speech memory, information storage and indirect memorization in 1/3 of patients. In 100% of the examined patients with TRAPS, a significant decrease in all processes of attention and distribution of attention. In 1/3 of patients with CAPS, an increased exhaustion of attention was registered and in 11% - a decrease in its stability. In patients with FMF, attention disorders were not detected. In 44% of patients with CAPS, a decrease in the level of generalization and difficulties in establishing causal relationships were registered. In 25% of patients with TRAPS a decrease in the level of generalization, in 12.5%- difficulties in establishing cause-effect relationships, inertia of thinking in 37.5%. In 60% of patients with FMF: a decrease in the level of generalization, in 80%: difficulties in establishing cause-effect relationships, inertia of thinking in 20%. In the emotional sphere, patients with CAPS, TRAPS, and FMF demonstrated signs of aggression (11.1%, 20% and 20% of patients, respectively), communicative disorders (77.8% -80% - 80%), and reduced social adaptation (55.5% - 80% - 80%), a tendency to form neurotic fears (22% - 40% - 40%). A high level of personal anxiety was noted in 1/3 of patients with CAPS and 40% of patients with FMF.

Conclusion: various psychological disorders in the cognitive and emotional fields were noted in the majority of the examined patients with monogenic auto-inflammatory diseases. In patients with TRAPS, attention processes are most significantly affected; in patients with CAPS, memory is more often affected. In patients with FMF, disorders in thinking processes are revealed more often. In the emotional sphere, most patients with all the three forms of AID note communicative disorders and social adaptation.

Disclosure of Interests: None declared

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