[Internal carotid artery dissection as a cause of severe ischemic stroke with lethal outcome]. / Dissektsiya vnutrennei sonnoi arterii kak prichina tyazhelogo ishemicheskogo insul'ta s letal'nym iskhodom.

We present a medical history of a 30-year old male patient with fatal ischemic stroke, resulting from the right internal carotid artery (ICA) dissection provoked by repeated head tilts and verified by magnetic resonance imaging and pathomorphological examination. At admission, the high level of creatine phosphokinase (5284 un/ml, normal level<171) in the blood was found, the coagulation parameters were normal. Autopsy revealed intramural hematoma (IMH), which was located between the media and adventitia of the arterial wall, began at 3 cm above the common carotid artery bifurcation and extended to the base of the skull. The lumen of the ICA at the level of the IMG and intracranial parts as well as of the middle cerebral artery was occluded by the thrombus. The histological examination of the right ICA wall found splitting, thinning, fragmentation, disrupters of internal elastic membrane, severe media fibrosis, myocyte necrosis at the site of the dissection with the surrounding leukocyte infiltration, as well as lymphocytic infiltrates, clusters of eosinophils in adventitia. Similar changes, except myocyte necrosis, were also found in intact (non-dissected) brain supplying arteries. In general, they were similar to those in fibromuscular dysplasia (FMD). Histochemical and electron microscopic studies of skeletal muscles showed signs of mitochondrial cytopathy. The authors discuss the relationship between the dissection, FMD and mitochondrial pathology.

[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.

[Virus-like inclusions in the myocytes of the skeletal muscle in lateral amyotrophic sclerosis]. / Virusopodobnye vkliucheniia v miotsitah skeletnoy myshtsy pri bokovom amiotroficheskom sklerose.

Microscopic examination of musculus gastrocnemius biopsies was made in four cases of sporadic lateral amyotrophic sclerosis (LAS). The validity of the clinical diagnosis was confirmed by detected neurotrophic atrophy of the muscular fibers typical for LAS. Electron microscopic study revealed virus-like inclusions 200-450 nm in size in sarcoplasm of myocytes of all the patients. The inclusions consist of lined cells of hexagonal shape at the distance of 37-41 nm from each other. The inclusions resemble enteroviruses but are not identical to them both by size and structure of their elements. There were also specific ultrastructural changes of myocytes corresponding to viral infection. The above virus-like inclusions should be considered as specific structures formed as a result of metabolic shifts caused by productive action on the cell of infective or unknown factor.

[Morphology of oculomotor muscles and their nervous apparatus in lateral amyotrophic sclerosis in conditions during long-term artificial lung ventilation]. / Morfologiia glazodvigatel'nykh myshts i ikh nervnogo apparata pri bokovom amiotroficheskom skleroze, protekavshem v usloviiakh dlitel'noi iskusstvennoi ventiliatsii legkikh.

Light and electron microscopy, histochemical study of acetylcholinesterase (ACE) were used in examination of the state and innervation of ocular muscles in autopsy material from a patient who died of amyotrophic sclerosis (ATS). The patient had lived 14 years under artificial lung ventilation, ATS was diagnosed 22 years before the death. Light microscopy demonstrated the intactness of the muscle fibers and the presence of three types characteristic of the extraocular muscles: thin, granular and rough. Besides typical differences structural changes were observed in some muscle fibers of the myopathic character and inclusions not limited by membrane having filiform or granular structure. Ocular muscles had an intensive innervation. Nervous fiber terminals revealed by a reaction for ACE were represented by single motor plaques and multiple cluster-like endings. Ultrastructurally, nervous endings of two types differed by terminals and fold expression on the postsynaptic membrane. There were no pathological changes in axons and myelin. Thus, ocular muscles were not affected as well as their nervous apparatus at completion of AMS, this indicating the noninvolvement of this muscular allotype in a specific degenerative process.

[The ultrastructural localization of NO-synthase NADPH diaphorase in a peripheral nerve and its change in diphtheritic polyneuropathy]. / Ul'trastrukturnaia lokalizatsiia NO-sintaznoi NADPH-diaforazy v perifericheskom nerve i ee izmenenie pri difteriinoi polineiropatii.

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. It is suggested that there is an association of the synthesis of nitric oxide with the myelin-producing function of Schwann's cells.

[Expression of NADPH-diaphorase in the peripheral nerve and its changes at different stages of diphtheritic polyneuropathy]. / Ekspressiia NADPH-diaforazy v perifericheskom nerve i ee izmenenie na raznykh stadiiakh difteriinoi polineiropatii.

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. A decrease of enzyme reactivity in SC cytoplasm of the damaged nerve fibers and rising enzyme reactivity in the cytoplasm of activated SC were observed in DP. High reactivity in SC of small fibers was found at earlier stages and that of thick fibers at later stages. This distinction reflected, apparently, sequence of entering at first thin, then thick fibres in the reparative process. Under the electron microscope, the reaction product was deposited on membranes of endoplasmic reticulum, nuclear membrane and Golgi complex. The enzyme was also located in nucleus of activated SC. Ultrastructural location and the fact that the highest intensity of reaction is present in SC of nervous fibres with morphological signs of remyelination suggest link of this enzyme with the reparative process. This study provides the first evidence of NADPH-d reactivity in SC and shows that NADPH-d histochemistry is a useful tool for peripheral nerve biopsies study.

[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.

[Diphtheritic polyneuropathy: clinico-morphologic study]. / Difteriinaia polineiropatiia: kliniko-morfologicheskoe issledovanie.

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. The basis of DP is toxic myelopathy with paranodal demyelination mainly in the large myelinated neural fibers and a segmentary one in the smaller neural fibers. Axonal degeneration was observed in the gravest cases of DP and was secondary being the result of axon squeezing by a folded myelin and voluminous Schwann cell cytoplasm invaginated into the axon. In no case of DP inflammatory changes and(or) involvement of the immunocompetent cells were found. There was pronounced proliferation and activation of Schwann cells due to intensive utilization of the degradation products of myelin and remyelinization. Morphological signs of remyelinization were observed on the 35th day of DP in the presence of enhancing neurological symptoms. But remyelinization was not complete even on the 69th day of DP.

[The Guillain-Barré syndrome]. / Sindrom Giiena--Barre.

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. Macrophages always took part in demyelination sometimes followed by lymphocytic infiltration. Ultrastructural investigation of nerve biopsies from 6 patients with GBS showed macrophage-associated demyelination with little or no lymphocytic infiltration. It is likely that axonal damage revealed in the biopsies and at the autopsies occurs as secondary consequence of demyelination.

[Inclusion body myositis: its clinico-electrophysiological and morphological diagnosis]. / Miozit s vkliucheniiami: kliniko-élektrofiologicheskaia i morfologicheskaia diagnostika.

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. The neuritic component proved aggravating in the course of the disease. Diagnostic myographic and morphological criteria are analyzed which can distinguish myositis with inclusions from other muscular inflammatory disease.

[The astrocyte reaction to an experimental herpes infection]. / Reaktsiia astrotsitov na gerpeticheskuiu infektsiiu v ékperimente.

The experimental research carried out on rabbits was aimed at determination of astrocytes reaction during brain inflammation caused by Herpes simplex virus. The study was made with the help of glia marker. Immunocytochemical findings showed that the earliest structural brain responses to the infection were changes in astrocyte glia. This was evident from the swelling and hypertrophy of vessel crus and from increased number of astrocytes around the vessels. Glia complexes (astrocyte agglomeration) were formed in the affected sites. During the exudative reaction the hyperplastic alterations of astrocytes were not observed. Later the injured spots of brain tissue were replaced by proliferating astrocytes. The conducted investigation has shown the astrocyte glia to be different during different stages of the infectious process. One may suppose that different subtypes of astrocytes response has place during each stage which should be further confirmed. One may also suppose that a similar alterations of astrocytes coincide with interferon and interleukin secretion by these cells. This is suggested to be a structural base of immune response of the brain.

[Morphologic changes in the skin and superficial temporal arteries in Sneddon's syndrome]. / Morfologicheskie izmeneniia v kozhe i poverkhnostnykh visochnykh arteriiakh pri sindrome Sneddona.

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. "Arteriopathy" is the most appropriate term for such lesions. Focal and diffuse fibro-muscular elastic hyperplasia of the intima and muscular layer fibrosis in the wall of superficial temporal arteries may be considered as age-associated lesions. Ultrastructurally, a selective damage of the non-adrenergic part of the nervous apparatus of the dermal arteries and superficial temporal arteries were observed; this suggests the participation of the damaged vascular neurogenic regulation in the formation of organic vascular changes.

[Natural autoantibodies of neonatal rats detectable by a hybridoma technic]. / Estestvennye autoantitela neonatal'nykh krys, vyiavliaemye gibridomnoi tekhnikoi.

Neonatal rat hybridomas were tested for natural autoantibodies (NAA) production, using different screening procedures. NAA were discovered in 35% of immunoglobulins producing hybridomas. Radioimmunoassay (RIA) on brain and liver homogenates and immunocytochemistry on brain sections are the procedures of choice revealing the major part of the identified NAA. On the contrary, only a small portion of NAA could be detected with indirect immunofluorescence on fixed fibroblasts and with RIA on individual autoantigens. All the NAA revealed proved to be of the IgM type and almost all of them possessed neither organ nor species-specificity. In spite of that, most of the NAA reacted with definite cell populations of nervous tissue such as glia, neurons, ependyma or brain vessel cells. The studied panel of NAA from neonatal rats has common features with similar panels from newborn and old mice, though some species-specific characteristics do exist.

[Virus-like inclusions in the cells of the central nervous system in amyotrophic lateral sclerosis]. / Virusopodobnye vkliucheniia v kletkakh tsentral'noi nervnoi sistemy pri bokovoi amiotroficheskom skleroze.

Electron microscopic examination of the frontal cortex of a patient with amyotrophic lateral sclerosis (ALS) with 14-year prolongation of life by artificial ventilation of the lungs revealed heretofore undescribed cytoplasmic virus-like inclusions in neurons and glial cells as well as changes in the granular endoplasmic reticulum in astrocytes consisting of large accumulations of ribosomes in which regular arrays were frequently observed. The discovered inclusions were not similar to any identified viral inclusions but were similar to virus-like particles found in the muscle in ALS and viral inclusions in experimental poliomyelitis. The role of the observed inclusions and their etiological importance remain obscure.