[Sneddon's syndrome and systemic lupus erythematosus with cerebrovascular disturbances and widespread livedo].

A comparative clinical and instrumental analysis of 97 patients with Sneddon's syndrome (SS), a combination of cerebrovascular ischemic disturbances with widespread livedo, and 12 patients with systemic lupus erythematosus (SLE) with the same combination, has been conducted. Despite the presence of similar features related to antiphospholipid syndrome (APS)--cerebrovascular disturbances, livedo, fetal loss, peripheral venous thrombosis, thrombocytopenia, antibodies to phospholipids, etc--there were distinct differences between SS and SLE. In SS, no skin lesions ("butterfly", discoid lupus, photosensibilization) typical for SLE as well as sores of mucous oral cavity, polyarthritis, serosity, diagnostically significant titers of antinuclear factor and antibodies to DNA were observed.

[Pathology of the brain in Creutzfeldt-Jakob disease].

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.

[The ultrastructural localization of NO-synthase NADPH diaphorase in a peripheral nerve and its change in diphtheritic polyneuropathy].

Light and electron microscopy was used to study the distribution and changes of NADPH-diaphorase in the cutaneous nerve biopsy specimens in different periods of diphtheritic polyneuropathy (DP). there was a reduction in the reaction rate of the enzyme in Schwann's cells of the destructively changed nerve fibers and an increase in the remyelinated nerve fibers. The enzyme is located on the nuclear and endoplasmic reticulum membranes and ribosomes.

[Expression of NADPH-diaphorase in the peripheral nerve and its changes at different stages of diphtheritic polyneuropathy].

Distribution and intensity of NADPH-d reactivity, a marker for enzyme of the nitric oxide synthesis, in nervus suralis biopsies in severe DP were studied at light and electron microscopic levels. The study of control specimens has shown that NADPH-d reactivity was permanently present in Schwann cells (SC) and was distributed in all parts of their cytoplasm. Axon and myelin were devoid of NADPH-d reactivity.

[In vivo diagnosis of Creutzfeldt-Jakob disease].

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.

[Diphtheritic polyneuropathy: clinico-morphologic study].

18 patients with grave DP treated with a long-term artificial pulmonary ventilation and feeding through a naso-gastric probe were studied. Biopsies of n. suralis taken at different periods after the appearance of the first signs of DP (from the 19th to the 69th day) were studied at light and electronic microscopy.

Clinical and molecular analysis of a large family with three distinct phenotypes of progressive muscular dystrophy.

We describe a unique six-generation, highly consanguineous family originating from an isolated mountainous village in the Russian province of Daghestan. Three separate clinical phenotypes of progressive muscular dystrophy were identified in this large family. Seven patients developed a classical limb-girdle variant of muscular dystrophy (LGMD), with disease onset at 15-30 years and loss of ambulation within a 25-year course.

[The Guillain-Barré syndrome].

A clinicomorphological study was conducted in 11 patients with severe forms of Guillain-Barré syndrome (GBS) at different periods of the disease. Five postmortem cases of GBS were investigated. In all the cases there was a multifocal loss of myelin in the peripheral nervous system with axon degeneration of various degree.

[Hypertensive angio-encephalopathy and vascular dementia].

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.

[Inclusion body myositis: its clinico-electrophysiological and morphological diagnosis].

The paper reports three cases of myositis. The findings at detailed electroneuromyographic, morphologic and ultrastructural tests were indicative of characteristic vacuole inclusions in the muscular fibers. Two patients had associated neuritic disorders diagnosed neurophysiologically and morphohistochemically.

[Morphologic changes in the skin and superficial temporal arteries in Sneddon's syndrome].

Skin biopsies from livedo's areas of 25 patients and fragments of superficial temporal arteries of 10 patients with Sneddon's syndrome were examined. Pathological changes in the dermis arteries of small and medium calibers were found in the form of the intima hyperplasia, proliferation of vascular wall cell elements (80%), arterial thrombosis (with diameter of 60-200 microns). These changes were found in 68% of observations when clinical and morphological signs of vasculitis were lacking.

The role of short-term hypobaric hypoxia in prevention of disorders of the cerebral circulation in rats during acoustic stress.

The 2 h influence of the 'altitude' of 5000 m on the genetically epilepsy-prone rats of the KM line reduces the death rate and the extent of neurological changes (the frequency and severity of motion disorders and the development of intracranial haemorrhages) under the conditions of acoustic stress.

[Effect of short-term adaptation to hypoxia on the development of acute cerebral circulatory disorders in rats genetically disposed to epilepsy].

The exposure of KM rats genetically predisposed to autogenic convulsive fits, to hypobaric hypoxia had a protective effect on the extension of cerebrovascular disorders in conditions of acoustic stress, reducing the severity of motor disorders and the degree of intracranial hemorrhage (subdural, subarachnoidal, intraventricular).

[Hypertensive angiopathy of the brain].

Cerebral vessels were studied by light microscopy in 20 autopsies after hemorrhagic stroke from arterial hypertension. Primary (acute), secondary (reparative) changes, as well as changes reflecting compensatory-adaptive processes, were found in the intracerebral and superficial arteries of the brain. The whole complex of these vascular changes was defined as hypertonic angiopathy.

[Experimental arterial microanastomoses].

The morphological changes in the walls of 40 arterial microanastomoses were studied in experiments. The initial signs of endothelization appear in 3 days and the process ends by the 9th-12th day. The tissue reaction to the different suture material is of the same type and its degree is not linked with the type of synthetic thread used.

[Hypertonic angioencephalopathy in its pathomorphological aspect].

This is a report on 20 autopsies of patients with arterial hypertension and atherosclerosis who died of a hemorrhagic stroke. Morphological changes characteristic of hypertonic angioencephalopathy were detected in the intracerebral vessels and cerebral substance. They included plasmorrhagia with arterial stenosis and necrosis, miliary aneurysms, isolated necrosis of the pia mater attended with vascular deformation, complete and incomplete necrosis of the cerebral substance, gliomesodermal cicatrices, perivascular hemorrhages, lacunar infarctions at various stages of their progression, etc.

[Morphology of primary and secondary hemorrhages into the brain stem].

On the basis of morphological examinations of 5 cases of primary and 15 cases of secondary hemorrhages to the brain stem histological characteristics of those hemorrhages are presented. A high frequency of preceding disorders of the brain stem circulation in the forms of infarctions at various stages of development, perivascular hemorrhages and cysts due to changes (mostly of hypertensive origin) in the cerebral arterie is noted. These changes are regarded as factors promoting the spreading, and, possibly, the development of primary hemorrhages to the stem, as well as aggravating the degree of the circulatory impairment when the pathological focus develops in the supratentorial space of the brain.

[Morphology of the Shy-Drager syndrome].

The changes found in the brain and spinal cord and vegetative ganglias in one observation of nerogenic orthostatic hypotension or Shy-Drager syndrome are described. The morphological picture corresponds to a variant of Shy-Drager syndrome with a wide involvement of the nervous system at its various levels. Its is concluded that a thorough examination of the nervous system (including the spinal cord and vegetative ganglia) should be done in all cases of Shy-Drager syndrome and clinically similar diseases accompanied by vegetative disorders, for instance in shaking palsy.

[Myelinolysis of the pons Varolli].

A case of myelinolysis of pons varolli which was diagnosed only after a microscopic examination of the brain is described. Certain morphological features of the focus in pons varolli were observed. It is assumed that in this observation the development of central pontine myelinosis was influenced by the abuse of alcohol as well as by exacerbation of pulmonary tuberculosis.

[Atherosclerosis of the vessels of Willis' circle in certain variants in its structure].

Random studies of the base of the brain vessels in 278 men who had died at the age of 50--64 years were carried out; 29 variants of the structure of the circle of Willis were established; the most common were threadlike connective arteries and posterior trifurcations of the inner carotid arteries. The investigation of atherosclerotic changes in every vessel of the base of the brain showed that in the so-called posterior trifurcations there was a statistically reliable growth of the average degree of atherosclerosis in the intracranial region of the inner carotid artery ensuring in these cases the blood supply for a considerably greater region of the brain. In posterior connective arteries atherosclerotic changes were revealed only when their diametre exceeded 0.