The study of epidemiology of hereditary neurological diseases in Mongolia began a few years ago. It covered a few aimags (provinces) (D.Baasanjav, 2000-2005). Previous studies have shown, that hereditary neurological diseases are not rare in Mongolia and structure and prevalence of these diseases vary by aimags. Mongolia has 21 aimags (administrative provinces), each with a population from 45000 till 110440. In some “isolated” populations specific neurological hereditary diseases can be marked as “indigenous”. So the epidemiological study of hereditary neurological 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 in families 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 from 47959 (Dundgobi) to 110440 (Uvurkhangai). Diseases were diagnosed by neurologists using clinical and genealogical methods.

Results: 

The study revealed 77 patients in 40 families in the studied population with variety of neurological hereditary diseases. The overall prevalence of hereditary neurological diseases for 100,000 population is 12,26. The prevalence varies by aimags from 2,08 (Dundgobi) to 32,50 (Gobi-Altai) per 100.000 population. The prevalence and their range by nosological structure per 100000 population in aimags are: myotonic dystrophy 5.41 widely varying by aimags: from 4.66 in Bayankhongor to 27.09 in Gobi-Altai; Charcot-Marie-Tooth syndrome 1.59 and ranging from 2.08 in Dundgobi to 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; Strumpell familial spastic paraplegia -0.95 for total pop and ranging from 2.71 in Uvurkhangai to 5.41 in Bayankhongor; familial paroxysmal 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 myodistrophy and 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 from 29 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 priority problem of the national health care.

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