HEALTH RELATED QUALITY OF LIFE OF THE PATIENT WITH IMMUNOGLOBULIN G4-RELATED CRANIAL HYPERTROPHIC PACHYMENINGITIS (IGG-HP) CAUSING DISTURBANCES OF COGNITIVE CONTROL TREATED WITH NEUROFEEDBACK

Abstract

Health related quality of life (HRQoL) is the most desired patient centered outcome of medical care (Leplége et al. 1997). In patients with long term illness, such as Hypertrophic Pachymeningitis (HP) still under diagnosis, therefore no possible to properly cure, it might be the only outcome achievable (Netuveli et al. 2005; Trystuła 2017). The problem becomes even more serious when occurs in young person: starting a family and caring for children becomes hard or even impossible, because of his/her physical and psychical conditions. Most clinicians are aware of the importance for quality of life of this functional limitation, but there are no articles describing this problem in the literature. We aimed to fill this gap in knowledge. 29 year old patient, a car mechanic, married, with 4-year-old son, with the long his- tory of the illnes, and especially persistent, diffuse, non-specific headaches, frequent seizures and cognitive deterioration which have been particularly troublesome recently. He was finalny diagnosed with Hypertrophic Pachymeningitis (HP) associated with Immunoglobulin G4-related (IgG4) with the use of specific diagnostic criteria for HP associated with IgG4-RD (IgG4-HP), which rely on histopathologic analysis (Lindstrom et al. 2010; Lu et al 2014). Computed tomography (CT) of the head showed massive calcifications visible along the cerebellar tentorium on the right side, along the cerebral falx, as well as on dura mater on the cranial vault of both cerebral hemispheres. The patient was diagnosed with common variable immunodeficiency (D 83), thrombocytopenia, chronic EBV hepatitis and epilepsy. IgG4-HP was confirmed by CT, MRI and biopsy (IGg4-RHP antibody was detected). The treatment with steroids, and immunosuppressive therapy (RTX) was introduced (as it was suggested by Levraut et al. (2019). It should be stressed that he had a significantly reduced health-related quality of life (HRQoL), mainly because of long-lasting illness, diverse symptoms, often hospitalization, complex differential diagnosis, and especially biopsy which requires neurosurgical intervention, which affect his physical and mental well-being, especially cognitive control, and not possibility to take care of his family. To help the patient we introduce HBI methodology (Kropotov 2016), that is an evaluation of working brain in milliseconds. Quantitative electroencephalography (qEEG), event-related potentials (ERPs) and low-resolution sLORETA tomography were performed. We did not found any paroxysm of 3 Hz rhythm in Eyes Closed (EO) and in Eyes Open (EO) conditions, however the ERPs deviations from the reference indicate deficit of cognitive control (decrease of P3 NOGO wave in comparison to 100 persons from the normative data base from the Human Brain Index in Chur, Switzerland). Therefore, the patient was offered Transcranial Direct Current Stimulation (atDCS) combined with goal-oriented psychotherapy program. It was found that after 40 days of therapy, cognitive control returned, which was translated into a better quality of life related to the patient's health, measure in the 36-Item Short Form Survey (SF-36). The patient returned to his previous job as a head of car mechanic service. Final diagnosis of IgG4-HP and subsequently, proper farmacotherapy, and introduction of HBI methodology allowing for the selection of an adequate method of neurotherapy, for our patient the transcranial direct current stimulation (atDCS) combined with goal- oriented psychotherapy, was helpful in the improvement of his quality of life.