Vol 2, No 4 (2008)

The authors present a 5-year experience of register of parkinsonism in the Krasnoyarsk region. The register comprises 826 patients with different forms of parkinsonism. The great majority of the revealed cases (79,1%) falls into Parkinson’s disease (PD), more rare are vascular (11,3%) and neuroleptic parkinsonism (2,1%), as well as neurodegenerative syndromes from the group of «parkinsonism-plus» (2,9% in total). The prevalence of PD in the Krasnoyarsk city is 62.72 per 100 000 in general population and 261.25 per 100 000 in the population above 65 years of age, that corresponds to the data in other populations; at the same time, lower figures in the Krasnoyarsk region (22.57 per 100 000 in general population and 100.32 per 100 000 in the population above 65 years of age) suggest, most probably, low detectability of this condition in primary care hospitals of the region. We detected earlier debut of PD in patients working in chemical industry (62,27±5,69 years), as well as in veterinaries and cattlemen (57,54±4,91), which may serve as confirmation of the discussed in the literature connection between PD and some neurotoxins used in the agriculture. High prevalence of cognitive disturbances of various severity in PD was registered (79,2% patients in total), and higher frequency and severity of cognitive decline was seen in an akineticrigid form of the disease.

Examination of 102 patients with acute ischemic stroke and diabetes mellitus of type II was carried out. Cerebral ischemia developed on the background of concomitant risk factors: hypertension, coronary disease, and dislipidemia. Ischemic stroke in diabetes mellitus type II has several characteristic features in the acute phase: it occurs frequently in the period of patient’s daily activity, clinical presentations are more severe and include severe disturbances of consciousness and marked focal neurological symptoms. Repeated strokes occur in 33% of cases. According to pathogenetic subtype, atherothrombotic stroke is prevalent in diabetes mellitus of type II. Computed tomography in the majority of patients showed multiple small cysts, leucoaraosis and atrophic changes, that suggests the presence  of long-term chronic insufficiency of cerebral blood flow.

Naminopathies belong to a wide allelic series of diseases caused by mutations of one gene, LMNA, encoding for protein lamin A/C. Different mutations in the LMNA gene cause autosomal dominant and autosomal recessive Emery–Dreifuss muscular dystrophy, dilated cardiomyopathy 1A, familial partial lipodystrophy, atypical Werner’s syndrome, Hutchinson–Gilford progeria and motor-sensory neuropathy type 2B1. In the review, the lamin structure and functions, clinical characteristics of hereditary  laminopathies, their etiology, pathogenesis and molecular bases are discussed.

Rasmussen’s encephalitis (RE) is a rare inflammatory brain disease characterized by severe intractable epilepsy and unilateral progressive motor defect associated with contralateral hemispheric atrophy. The authors present clinical features, EEG and MRI data and results of effective treatment of patient with adult onset RE. Despite the predominance of RE in children, stressed is the necessity of taking into consideration this condition in adults as well in differential diagnostics of intractable epilepsies.