[Morphological signs of mitochondrial cytopathy in skeletal muscles and micro-vessel walls in a patient with cerebral artery dissection associated with MELAS syndrome].

Skin and muscles biopsy specimens of a patient harboring A3243G mutation in mitochondrial DNA, with dissection of internal carotid and vertebral arteries, associated with MELAS were studied using histochemical and electron-microscopy techniques. Ragged red fibers, regional variability of SDH histochemical reaction, two types of morphologically atypical mitochondria and their aggregation were found in muscle. There was correlation between SDH histochemical staining and number of mitochondria revealed by electron microscopy in muscle tissue. Similar mitochondrial abnormality, their distribution and cell lesions followed by extra-cellular matrix mineralization were found in the blood vessel walls. In line with generalization of cytopathy process caused by gene mutation it can be supposed that changes found in skin and muscle microvessels also exist in large cerebral vessels causing the vessel wall "weakness", predisposing them to dissection.

[Ultrastructural changes of skin arteries in patients with spontaneous cerebral artery dissection].

Spontaneous cerebral artery dissection is a common cause of ischemic stroke in young adults and children. Dissection is often related to arterial wall weakness the cause of which is unknown. An aim of the present paper was to carry out an electron microscopic study of skin arteries and arterioles in patients with ischemic stroke caused by cerebral artery dissection. Skin biopsy samples from 3 patients (2 men and one women, 15, 25 and 43 years of age, respectively) were studied. Electron microscopy revealed changes of endothelial, smooth muscle cells and fibroblasts in the skin microvessels. These changes included the decrease in the number of mitochondria and their alterations (vacuolization, destruction of the cristae, the presence of the needle-shaped crystals and crystal-like inclusions) and swelling of the endoplasmic reticulum. Some of these changes were characteristic of mitochondrial diseases. The changes in the extracellular matrix (thickening of the subendothelial layer and deposition of microcalcificats) were also detected. It is assumed that the mitochondrial cytopathy found in the skin microvessels may be also present in large cerebral arteries. This could underlie dysplastic changes in the cerebral artery wall and predispose to its dissection.

[Mitochondrial arteriopathy as a cause of spontaneous dissection of cerebral arteries].

The vascular wall weakness caused by dysplastic alterations predisposes to the spontaneous dissection of cerebral arteries. The authors hypothesized for the first time that dysplasia might be the result of mitochondrial cytopathy. To test this hypothesis, the muscle biopsy was conducted in 3 male patients, aged 30-38 years, with the spontaneous dissection of the internal carotid (2) and posterior cerebral (1) arteries. Clinically dissections manifested by ischemic stroke (2) or the peripheral paresis of the hypoglossal nerve (1). The morphological study of fresh frozen sections of muscle by modified Gomori trichrome method revealed ragged-red fibers The histochemical study showed the severe decrease of the stain on succinate dehydrogenase and cytochrome-c-oxidase as well as the focal intensive staining of peripheral regions of muscle fibers. The complex of found changes is characteristic for a mitochondrial pathology. No patients had A3243G tRNA gene mutation, the most common mutation for MELAS. The serum lactate level was elevated only in one patient. We suggest that the mitochondrial disorder occurs not only in muscle, but also in cerebral artery wall--mitochondrial arteriopathy, which predisposes to spontaneous cerebral artery dissection.

[Recurrent brain infarctions in atherosclerosis and arterial hypertension]. / Povtornye infarkty golovnogo mozga pri ateroskleroze i arterial'noi gipertonii.

In 100 cases of brain infarctions of different size and time caused by atherosclerosis and arterial hypertension, the infarctions were located in the territory of carotid arteries (44 cases), of vertebro-basilar system arteries (18 cases) and of both the systems (38 cases). The infarctions were repeated, multiple in 82 cases, single--in 18 cases. Atherothrombosis and atherostenosis, thromboembolism of the brain arteries from the heart are main pathogenetic factors. The phenomenon of "infarction in the infarction" is observed in the limits of one arterial pool.

[Pathology of the brain in Creutzfeldt-Jakob disease]. / Patologiia golovnogo mozga pri bolezni Kreittsfel'dta-Iakoba.

The autopsy cases of Creitsfeldt-Jacob disease (a sporadic form) are reported which were diagnosed clinically and supported by the data of biopsy and autopsy of the brain (classic triad: death of neurons, astrogliosis and spongiform degeneration of the gray substance of the cortex of brain hemispheres, preferentially), followed by clinical morphologic comparisons. The focal character of the disease was observed on the early stages of the disease, while diffuse alterations were found on the late stages.

[In vivo diagnosis of Creutzfeldt-Jakob disease]. / Prizhiznennaia diagnostika bolezni Kreitzfel'dta-Iakoba.

The paper presents the data concerning usage of some original method of vital laboratory diagnostics of Creutzfeldt-Jacob disease that belongs to the group of prionic diseases. The method consisted in the inoculation of inoculative culture of rat Gasser ganglion's neurinoma by biologic materials investigated (serum and clot of blood) with the following passivation and investigation of the contaminated culture by means of both morphologic and electron microscopic methods. As an example of vital verificated case the wide pathomorphologic analysis of the biopsy sample of brain was presented. Besides, the efficiency of the investigation of cognitive evoked potentials (P300) together with EEG was also demonstrated as the method of objectification of the development of dementia in this disease.

[Lifetime diagnosis of prion transmissive encephalopathies]. / Prizhiznennaia diagnostika prionovykh transmissivnykh éntsefalopatii.

An original method for life-time diagnosis of Greutzfeldt-Jakob's disease, a neurodegenerative disease belonging to transmissive spongiform encephalopathies, is proposed. It consists in inoculation of the examined biological materials (blood serum and clot) into a continuous culture of the rat Gasser's node neurinoma, followed by passages and study of the inoculated culture by morphological methods and electron microscopy.

[Hypertensive angio-encephalopathy and vascular dementia]. / Gipertonicheskaia angioéntsefalopatiia i sosudistaia dementsiia.

A clinicomorphological analysis of 60 postmortem cases with brain circulation disturbances against the background of arterial hypertension (AH) is performed. Lacunar infarcts, areas of incomplete necrosis and perivascular encephalolysis, small hemorrhages, criblures, persistent oedema and widespread spongiosis are found in the white matter (WM) of the brain in cases of AH of long duration with dementia. An important role in the WM lesions belongs to its ischemia due to hypertension angiopathy and disturbances of hemodynamics and liquor circulation. Complex of these lesions is called "hypertension leucoencephalopathy".

[Hypersomnic syndrome in circulatory disorders of the oral portions of the brain stem (clinico-morphologic observations)]. / Gipersomincheskii sindrom pri narushenii krovoobrashcheniia v oral'nykh otdelakh stovola mozga (Kliniko-morfologicheskie nabliudeniia).

Hypersomnia is described in 11 patients who have had a cerebral circulation episode with prevalent localization of the lesion in the oral divisions of the brain trunk. Three morphological observations are presented: these were cases of bilateral affection of certain meso- and diencephalic structures, e.g. medial divisions of the thalami and the diencephalon cover with involvement of the grey matter of the Sylvian aqueduct into the process. The observations presented confirm the opinion that the nonspecific structures of the meso- and diencephalic region are a part of the complex cortico-subcortical system participating in maintaining the state of wakefulness.