Structure and prevalence of hereditary neurological diseases in population of some provinces (aimags) of Mongolia / Монголын Анагаах Ухаан

ABSTRACT

BACKGROUND: The study of epidemiology of hereditary neurological diseases in Mongolia began a few years ago. Itcovered a few aimags (provinces) (D.Baasanjav, 2000-2005). Previous studies have shown, that hereditary neurologicaldiseases are not rare in Mongolia and structure and prevalence of these diseases vary by aimags. Mo n g o l i ahas 21 aimags (administrative provinces), each with a population from 45000 till 110440. In some “isolated” populationsspecific neurological hereditary diseases can be marked as “indigenous”. So the epidemiological study of hereditaryneurological diseases in Mongolia is one of the urgent medical shortcoming in Mongolia.MATERIAL AND METHOD: We used descriptive epidemiological method for revealing hereditary neurological diseases infamilies in population of 8 provinces (aimags) of Mongolia, Bayan-Ulgii, Bayankhongor, Govi-Altai, Zavkhan, Dundgobi,Khentii, Orkhon, Uvurkhangai, total of 627,000 population. The population of these aimags fluctuated in the range from47959 (Dundgobi) to 110440 (Uvurkhangai). Diseases were diagnosed by neurologists using clinical and genealogicalmethods.RESULTS: The study revealed 77 patients in 40 families in the studied population with variety of neurological hereditarydiseases. The overall prevalence of hereditary neurological diseases for 100,000 population is 12,26. The prevalencevaries by aimags from 2,08 (Dundgobi) to 32,50 (Gobi-Altai) per 100.000 population. The prevalence and their rangeby nosological structure per 100000 population in aimags are myotonic dystrophy 5.41 widely varying by aimags from4.66 in Bayankhongor to 27.09 in Gobi-Altai; Charcot-Marie-Tooth syndrome 1.59 and ranging from 2.08 in Dundgobito 4.66 in Bayankhongor; Duchenne muscular dystrophy -0.79 and ranging from 0.90 in Uvurkhangai to 3.19 in Bayan-Ulgii; the limb girdle muscular dystrophy -0.95 and ranging from 1.81 in Uvurkhangai to 4.66 in Bayan-Ulgii; Strumpellfamilial spastic paraplegia -0.95 for total pop and ranging from 2.71 in Uvurkhangai to 5.41 in Bayankhongor; familialparoxysmal myoplegia -0.79 for total pop. and range from 2.22 in Orkhon to 5.41 in Gobi-Altai; spinocerebellar ataxia -0.47 for total pop. (3.91-in Zavkhan); bulbo-spinal amyotrophy -0.32(only in Bayankhongor); spinal amyotrophy of adults-0.16(only in Uvurkhangai); arthrogryposis with myodystrophy -0.63 (only in Khentii); kyphoscoliosis with myodistrophyand nanism -0.16 (1.06 in Bayan-Ulgii)A comparatively high prevalence of all neurological hereditary diseases per 100.000 pop. was established in Gobi-Altai(32.50), Orkhon-26.56, Bayankhongor-15.16, Bayan-Ulgii -11.70 rates; the low prevalence - in Dundgobi-2.08 and-Zavkhan-3.91.The hereditary neuromuscular diseases among all hereditary neurological diseases taken up 71.9% i.e 55 patients from29 families. The myotonic dystrophy is tops the list of neuromuscular diseases (61.8%)CONCLUSION: Hereditary neurological diseases have a relatively high prevalence among the population of Mongolia,specially the neuromuscular diseases; so the control of these diseases in the country is one of the special priorityproblem of the national health care.