NERVOUS SYSTEM LESION IN ANKYLOSING SPONDYLITIS, IN THE DISEASE BEGINNING IN СHILDHOOD AND ADULTHOOD

Summary. the incidence of ankylosing spondylitis (as) in population amounts to 0.3 %, which is significantly more likely to develop at the age of 20–30 years. there are two forms of as – juvenile and adults, depending on the age of the disease debut. the problem of juvenile as (Jas) diagnostics is one of the most relevant in pediatric rheumatology, and the evolution of this disease remains un-explored in adulthood. The aim of the study – to learn the frequency and nature of separate clinical signs of Cns and pns lesions in patients with as, and to evaluate their special features in the disease beginning in childhood and adulthood. Materials and Methods. 217 patients with as (193 males and 24 females) with an average age of 38 years were examined. the rapidly progression course is detected in 21 % of patients, moderate and high activity rate – in 79 %, stage II–III – in 82 %, polyarthritis – in 65 %. Jas was noted in 16 % of cases (all boys), in which stage III took place twice as often than other patients. Results and Discussion. Changes in pns are observed in 4.9 times more often among patients with Jas, and Cns is 2 times less often than in cases of the disease debut in adulthood, moreover among the patients of the 1 st group, the severity of Cns disturbance is associated with the involvement of the cervical spine and the prevalence of spondylopathy, pns is associated with the availability of tendovaginitis, arthritis of the "root joints" (shoulder, hip) and changes in the thoracic spine, while in the 2 nd group it is associated with the parameters of the integral index of arthritis activity and the x-ray stage of the disease, with disturbance of "root" and sacroiliac joints, wherein the age of the disease onset affects the development of asthenic vegetative and corticonuclear syndrome, the emergence of radiculopathy, cervicocranialgia and metacarpal canal syndrome, and in the pathogenetic architectonics of the Cns pathology the level of immunoglobulin-a plays a greater role, and in pns the serum interleukin 1-β contents, which, in addition, in cases of JaC determine the occurrence of cervicocranialgia, and in the remaining observations of as – the morton's metatarsalgia. Conclusions. as with different age of the disease debut is a risk factor for the development of certain symptoms of the Cns and pns disturbances, which in these groups have their pathogenetic features.

INTRODUCTION the prevalence of ankylosing spondylitis (as) reaches 0.3 % among the population [1,2], and one of the most common manifestations of the disease is the central and peripheral nervous system lesions (Cns, pns) [3,4], which is primarily caused by the pathology of the spine [5]. the main causes of severe neuropathy in patients with as are subluxation and micro fracture of the vertebrae [6,7,8], as well as demyelinating pathology [9].It should be noted that for such patients there are violations of the nervous system vegetative link [10], which, first of all, affect its parasympathetic component [11].almost always as is accompanied by psychosomatic disorders [12,13], which, eventually, further exacerbates the poor quality of patients' life [14,15].two forms of as are identified -juvenile onset (JaC) and adult onset (aaC), depending on the age of the disease debut [16].r.Conway and F. d. o'shea [17] consider that these variants of the disease differ "like apples from oranges".an increasing research interest in the disease over recent years has led to a number of significant insights in as. the differences between juvenile and adult-onset as represents an ongoing controversy.It is still uncertain whether these 2 entities are distinct disease processes or different manifestations of the same disease modulated by age of onset.a number of groups have looked at this aspect of the disease using a variety of methodologies and publishing a number of contrasting findings [18].
The aim of the study -to learn the frequency and nature of separate clinical signs of Cns and pns lesions in patients with as, to assess their special features in disease that began in childhood and adulthood.
MATERIALS AND METHODS 217 as patients aged 16 to 57 years old (an average of (37.7±0.64 years) were under observation, 88.9 % of men and 11.1 % of women among them.the disease duration averaged (10.5±0.39)years.I degree of as activity was established in 21.2 % of cases, II -in 56.7 % and III -in 22.1 %, I stage was noted in 18.4 % of the surveyed patients', II -in 50.7% and III -in 30.9 %. slowly progressive disease course occurred in 79.3 % of cases, fast-progressing -in 20.7 %. the so-called "central form" of as was diagnosed in 33.6 % of cases, "rhizomelic" -in 2.8 %, "peripheral" -in 5.1 %, "undifferentiated" -58.5 %. ophthalmopathy (uveitis) and visceral changes are established in ¾ of the patients' number.
all patients were divided into two groups: the first (main) amounted to 35 (16.1 %) patients with Jas (all males with onset of the disease under the age of 18), and the others 182 (83.9 %), with aas were included in the 2nd (control) group.the age of the main group representatives in the disease debut was (14.3±0.52)years, and at the time of the examination -(24.9±0.83)years, while in the control group it was (29.6±0.45) years and (40.2±0.58) years respectively.the duration of the disease in the 1st and 2nd groups did not differ.both groups were approximately equal in the disease degree activity, but rapid progressive course of the disease in aas was 2.7 times more common.
according to wilcoxon-rao's multivariate dispersion analysis, the age of patients in the disease debut is influenced on integral signs of Cns changes.we selected those signs of as clinical course that simultaneously had brown-Forsythe dispersion relations and Kendall's correlation with the severity of the nervous system lesion.It turned out to have existed a direct relationship between the severity of the cervical spine lesion and the prevalence of spondylopathy in cases of Jas, and with the das parameter and the involvement of the "root" (shoulder, hip) joints in cases of aas.
according to wilcoxon-rao analysis, the age of as patients at the onset of the disease affects the integral clinical signs of pns.In the main group, there is a direct correlation between the severity of changes in pns with tendovaginitis and involvement the thoracic spine, and in the control group -with the as stage, the presence of the coxitis and the severity of sacroileitis.
the age of the disease debut in the main group correlates inversely with the severity of the dyscirculatory encephalopathy, asthenic vegetative and epileptiform syndrome, and in the control one -directly correlates with the corticonuclear syndrome and has negative correlation relations with the epileptiform syndrome and cerebellar ataxia, as shown by the Kendall and pearson correlation analyzes (Fig. 3-6).
both in the first and in the second patients' groups with as there are direct correlations the severity of radiculopathy with the age of patients, and the inverse ones -with cervicocranialgia.In addition, there are multi-directional reliable relationships in Jas patients with the facial nerve neuritis (direct relation) and metacarpal canal syndrome (inverse relation).based on the survey findings, we made conclusions with a definite practical orientation: the debut of the disease under the age of 11 (<m-sd in the patients of the main group) is a risk factor for the development of asthenic vegetative syndrome, cervicocranialgia and metacarpal canal syndrome, and the onset of the disease is above 35 years of age (> m + sd in the control group) -a prognostic negative sign in the occurrence of corticonuclear syndrome and radiculopathy.
as shown by brown-Forsyth's dispersion analysis, in the case of Jas, the beginning of asthenic vegetative syndrome is influenced by the das parameters, the pyramidal syndrome -by the prevalence of spondylopathy, cerebellar ataxia -by the stage of the pathological process, peripheral polyneuropathy -by the presence of tendovaginitis in patients, cervicocranialgia -by the cervical spine lesion, metacarpal canal syndrome -by the degree of disease activity.In cases of aas, the development of dyscirculatory encephalopathy is closely related to enthesitis, pseudobulbar syndrome -with the "root" joints lesion, pyramidal syndrome -with changes in the cervical spine, cortical ataxia and metacarpal canal syndrome -with das indices, radiculopathy -with as stage.
regardless of the as variant and the nature of the nervous system lesion, there is a dispersion relation between the severity of the Cns, pns pathology and Iga, CIC levels in blood.In addition, the severity of the central nervous system lesion in the patients of the main and control groups is closely related to aCCp serum level, and in the case of aas, as is also to fibrinogenemia level.the severity of Cns lesion in patients with Jas is directly correlated with level of Iga and tnFα in blood, and pns -with Il1β.In cases of aas, there are inverse correlations between the severity of Cns changes and immunoglobulinemia, CIC and Il1β parameters, and it is observed a direct correlation with tnFα values.It should be emphasized that the severity of pns lesion in patients with aas is inverse correlated with the concentration of CIC in blood and directly from Il1β.
taking into account the performed statistical data results of the survey, the prognostic positive sign in regard to the severity of the Cns damage is the level of Iga <2 mmol / l the next stage of our research was an assessment of the immune disorders role in the pathogenetic constructions of Cns and pns lesion in patients with Jas and aas.For this purpose, we selected those indicators that simultaneously had significant brown-Forsythe dispersion relations and Kendall correlation.It turned out that the development of dyscirculatory encephalopathy directly depends on the level of CIC, pyramidal syndrome -on Crp, peripheral polyneuropathy -on Il1β, cervicocranialgia -on Iga, the metacarpal syndrome -on tnFα, morton's metatarsalgia -on Fg. there were no such dependencies in patients with aas, but there were completely different ones.thus, corticonuclear syndrome and the development of mono-and polyneuropathy were associated with the level of CIC, pseudobulbar syndrome -with Igm, the severity of radiculopathy -with Crp, morton's metatarsalgia -with Il1β.these data once again point to the completely polysemantic manifestations of the as with the debut of the disease in childhood and adulthood.CONCLUSIONS Changes in pns are observed 4.9 times more often in patients with Jas, and Cns lesion is 2 times less likely than in cases of the disease debut in adulthood, while in patients with Jas the severity of Cns damage is associated with the involvement of the cervical spine and the abundance of spondylopathy, pns -with the presence of tendovaginitis, arthritis of the "root joints" (shoulder, pelvic) and changes in the thoracic spine, whereas in the case of aas, pns lesion depends on das parameters, the x-ray stage of the disease, the involvement of the sacroiliac joints, and the age of the disease onset affects the development of asthenic vegetative and corticonuclear syndromes, the occurrence of radiculopathy, cervicocranialgia and metacarpal canal syndrome.and Iga level is more involved in the pathogenetic constructions of the Cns pathology, and the level of Il1β in blood -in pns, moreover, Il1β and Iga levels determine the occurrence of cervicocranialgia and morton's metatarsalgia in other observations of the as.
Conflict of interest the authors declare that there is no conflict of interest, while the authors did not receive from the individuals and organizations financial support for research, fees, and other forms of rewards.