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Biomedical subjects

R Escourolle

Publications and source records attributed to R Escourolle.

At least 91 records · Page 5Linked to original sources

[Neuropathologic and toxicologic study of 12 cases of bismuth encephalopathy].

Examination of twelve cases of bismuth encephalopathy showed a constantand marked elevation of the bismuth levels in autopsy cerebral tissue (ranging from 2-8 mg/kg to 25 mg/kg). These values were found to be much higher than the last-measured Bismuth venous blood values. In every case, periveinular lymphocytic infiltration and abundant intra-cytoplasmic lipofuscin were seen. The microanalysis with Castaing's electrode revealed the presence of focal areas of bismuth in the leptomeningeal spaces. The significance of the lesions is not fully established and it will undergo further investigations.

Adult↗

[Presence of aluminum and magnesium in the cerebral arteries and parenchyma of patients with striatonigral syndrome: study by Castaing's microprobe].

Electron probe microanalysis demonstrates the presence of aluminium, magnesium, iron, calcium, phosphorus in and around blood vessels in the pallidum (vascular siderosis) and in the putaminal parenchyma in five out of six cases of striatonigral degeneration, associated with orthostatic hypotension in two of these cases. These results suggest that striatonigral degeneration could be the result of a vascular disease, the result of an elemental intoxication of unknown cause.

Aluminum↗

Bulbar infarcts. Systematic study of lesion topography in 49 cases.

A pathological study has been carried out of 49 examples of medullary infarction in 33 patients. The medial infarcts, very seldom single (2 /12), spared the subolivary levels. The pontomedullary junction was affected in 3 /9 cases studied. The lesions were more frequent in the ventral part of the arterial zonal pattern. They more often extended to its dorsal part at the level of the middle and upper one-third of the olive. The lateral infarcts, seldom single (6/30), extended to the subolivary levels in 6/22 cases studied and to the pontomedullary junction in 9/24 cases. The structures involved in each case and the area of the lesions at each cranio-caudal level have been studied. None of the 7 dorsal infarcts was single; in 6 of these cases there was also a cerebellar infarct involving the area of supply of the posterior inferior cerebellar artery. The subolivary levels (1/6) and ponto-medullary junctions (1/6) were seldom affected. The extent of the lesions in these uncommon infarcts has been described in detail.

Brain Stem↗

Bulbar infarcts. Vascular lesions in 26 observations.

The pattern of arterial occlusion and related lesions have been studied in 26 cases with 38 medullary infarcts. In 10 of these cases, only the intracranial vessels were examined. In the 16 other cases, the entire cerebral arterial supply was dissected. Medial infarcts were more often related to occlusion of the vertebral artery involving the termination of the artery (7/10 cases). In 6 cases a thrombosis on an atheromatous plaque and, once, an embolus of cardiac origin, were found. Lateral infarcts were usually due either to occlusion of the vertebral artery (14/23 cases), reaching, in 10 cases, the mouth of the posterior inferior cerebellar artery (PICA) or to an isolated occlusion of the latter vessel (3 cases). A thrombosis on an atheromatous plaque was found in 15 cases, and emboli from the heart or from the origin of the vertebral artery in the others. The dorsal infarcts were related, in 4/5 cases, to an isolated occlusion of the stem of the PICA and, in the other case, to one of the vertebral artery involving the ostium of the PICA. A thrombosis due to atheromatous stenosis was found in 4 cases, an embolus of cardiac origin in the other one.

Basilar Artery↗

["Astasia-abasia," unilateral left-sided apraxia, and touch disorders in an astrocytoma of the corpus callosum. A clinico-pathological report].

The authors report the case of a 55 year old woman suffering from an astrocytoma of the corpus callosum, probably originating in the middle part of the commissure and subsequently involving the whole structure, beyond which it extended very little. They describe the symptoms and signs, consisting initially of a progressive isolated astasia-abasia, in the absence of any psychological disturbance, and subsequently consisting of an apraxic disorganisation involving particularly gestural activity of the left upper limb. They discuss the significance of the psychological disturbances and problems of stasis and walking classically described in relation to tumoural pathology of the corpus callosum, generally attributed to an associated pre-frontal involvement. They review the principal features the "callosal disconnection syndrome" of which their patient constituted an example.

Apraxias↗

[Huntington's chorea and cerebellar atrophy. Apropos of amanatomo-clinical case].

The authors report the clinical and pathological findings in a 46 years old woman with Huntington's chorea and cerebello-olivar atrophy. Eight previously reported cerebellar atrophies in Huntington's chorea with pathological examination are reviewed. The primary or secondary nature of the cerebellar lesions is discussed.

Adult↗

[Granular cell pituicytoma (choristoma) of the pituitary stalk].

A case of granular cell tumour in the pituitary stalk is described marked by the sudden loss of visual acuity in the left eye, followed by the right years later. A comparison with 17 cases previously published and with cases of asymptomatic granular cell nodules leads the authors to suggest the term granular cell pituicytoma. They discuss the place of this neoformation among other systemic granular cell tumours (granular cell myoblastoma). Fifteen patients were operated on, complete excision being carried out in only three (including the case published). Whether complementary cobalt therapy is useful remains problematical since very few cases have been published because of the slow development of the neoplasm.

Choristoma↗

[Giant cell granulomatous angiitis of the central nervous system].

The case is described of a coloured woman of 58, previously affected by filaria, who presented with meningo-encephalitis which proved fatal within a month. Pathological anatomy showed granylomatous angiitis with giant cells scattered throughout the brain and the meninges but confined exclusively to the nervous system. Biological and anatomopathological tests were unable to connect this condition with any precise aetiology. The aetiology of granulomatous angiitis is discussed in this connection and the clinical and anatomopathological charateristics peculiar to giant cell granulomatous angiitis of the nervous system are recalled.

Arteritis↗

[Catecholaminergic axons in the human cerebral cortex. Observation by histofluorescence of cerebral biopsies in 2 cases of Alzheimer's disease].

Catecholamine axons have been visualized in human cerebral cortex obtained during routine neurosurgical operations. The fluorescence histochemical method of Lindvall et al. was used, slightly modified (calcium-deprived buffer, glyoxylic acid fixation followed by formaldehyde vapours exposition). The frontal cortex was more richely provided with catecholamine terminals than the parietal cortex. Two general types of axon morphology are evident. The most frequent is thin and sinous, sometimes forming clews, or loose basket-like arrangement around presumed nerve cells. The other one is moniliform and demonstrates spherical evenly-spaced varicosities. They look like, respectively, the well characterized dopaminergic and noradrenergic axons of the rat cerebral cortex. In two cases of Alzheimer's disease, noradrenergic-like fibers were missing and voluminous green-fluorescent varicosities, sometimes in obvious connection with typical axons, were observed in the proximity of senile plaques.

Alzheimer Disease↗

[Familial olivo-ponto-cerebellar atrophy with myoclonus. Limits of cerebellar myoclonic dyssynergia (Ramsay-Hunt syndrome)].

The case is described of a woman of 26 suffering (like her mother, a brother and a sister) from a progressively degenerating cerebellar syndrome, at first considered to be hereditary cerebellar ataxia, but which, after action myoclonus appeared, was diagnosed as dyssynergia cerebellaris myoclonica (D.C.M.). Anatomical verification however revealed a syndrome of olivo-ponto-cerebellar atrophy comprising massive demyelinisation of the white matter of the cerebellum and of the cerebellopontine fibres; atrophy of the pontine nuclei; the cerebellar cortex itself was severely affected; moderate nigral lesions; marked spinal lesions of the cerebellospinal fasciculi and of the posterior columns; lesions of the anterior horns and of the bulbar nuclei; cortical chromatolysis. The fact that the dentate system remained unaffected, also noted in some cases of olivo-ponto-cerebellar atrophy with myoclonus, whilst in a number of other cases the lesion remains clinically silent, emphasises the difficulty in establishing an accurate correlation between myoclonus and dentate nucleus. Discussion of the nosological limits of D.C.M.: confirmed cases generally displayed lesions of the dentate system and hereditary degenerative spino-cerebellar lesions. The same clinical symptoms can be observed in cases which do not come under this classification--or even under that of degenerative conditions of the cerebellar system--and D.C.M. appears to be only a syndrome, the Ramsay-Hunt syndrome.

Adult↗

Ultrastructural observations on human cerebral capillaries in organ culture.

The use of an organotypic-in the strictly literal meaning of the word, nervous tissue culture device has allowed the identification and ultrastructural study of various types of developing capillaries in human cerebellum and olfactory bulb in vitro. Most capillaries were similar to those already described by other authors or by us, in human or animal embryos and fetuses. Large Type I Capillaries. Their luminal diameters were greater than 8 microns. The basement membranes were thin and discontinous. Numerous interendothelial junctions were either plate-like attachments or contained pentalaminar zones. Type II Capillaries. Their lumina were between 2 and 8 microns in diameter. The basement membranes were wider than those of type I capillaries and were sometimes continuous. The interendothelial junctional complexes of type II capillaries included pentalaminar portions. Many simple or complex vascular sprouts (type IV and V capillaries) had small or non-patent lumina. Their basement membranes were absent or very thin and discontinuous. Their interendothelial junctions were similar to those of type I capillaries. Some of the less frequently encountered capillary types seen in developing human nervous tissue were absent in culture. Some pathological features were seen-especially in long-term cultures-in type I and II capillaries containing degenerating blood cells or processes sometimes obviously related to histiocytic cells. They consisted mainly of an accumulation of microfilaments and modifications of the rough endoplasmic reticulum in the endothelial cells. These pathological changes did not modify the main characteristics of the capillaries. The origin of the vascular sprouts, the exact nature of the interendothelial junctions and the significance of the pathological changes are discussed. This model may prove useful for the study of cerebral vasculogenesis, the development of the blood-brain barrier and the physiological or pathological properties of the human brain capillaries in tissue culture.

Basement Membrane↗

Metachromatic leukodystrophy. Ultrastructural and enzymatic study of a case of variant O form.

A variant of metachromatic leukodystrophy (MLD), Austin disease, is characterized by a multiple isozyme deficiency of arylsulfatase. A 3 1/2-year-old girl with progressive mental and physical deterioration had decreased activities of arylsulfatases A and B in the leukocytes, shown by acylamide gel electrophoresis. Under the electron microscope, biopsy specimens of the brain and the peripheral nerve showed lamellar structures with socalled zebra bodies in the cytoplasmic processes of glial cells, granulo-membranous inclusions with fingerprint configurations in neurons, and myelinlike material in Schwann cells. Results from our study suggest an intricate nature of this dysmetabolic disorder, which shows ultrastructural changes usually seen in classic MLD, a deficiency of arylsulfatase A only, concomitant with those seen in mucopolysaccharidoses such as Hurler and Sanfilippo syndromes.

Cerebroside-Sulfatase↗

Electron microscopic study of the developing capillaries of human brain.

The ulstrastructural characteristics of the cerebellum and olfactory bulb capillaries were studied in 12 human specimen measuring 25-200 mm. Type I capillaries, whose lumina were wider than 8 mu, contained no pores of fenestrations. The basement membrane was sometimes discontinous. In that case, junctions without quintuple-layered zones could be observed. Such capillaries may correspond to primary vessels in which the sinusoid character has disappeared. Elsewhere, the basement membrane was continuous and the interendothelial junctions always contained quintuple-layered zones. Type II capillaries, whose lumina measured between 2 and 8 mu had basement membranes that were either discontinuous or continuous. They were identical to the immature capillaries described previously in numerous species including man. Type III caillaries, rarely observed, were characterized by the presence of several endothelial cells with abundant cytoplasm, which limited the mina from 0.5 --3 mu in diameters. They had continuous basement membranes. Type IV and V capillaries has small or non-patent lumina. The basement membrane was absent or rudimentary. These capillaries appeared to correspond to simple and complexendothelial sprouts. In Types II, III, V and most Type IV capillaries, the interendothelial junctions contained quintuple-layered zones. No mature capillaries were observed. The hypothetical pathways of development of cerebral capillaries and the degree of permeability of immature interendothelial junctions are discussed. The long duration of maturation in man probably accounts for the diversity of capillary formations observed as compared with animals. The co-existence over along period of time of several capillary types with probably varying permeability may play a significant role in the maturation of the blood-brain barrier.

Basement Membrane↗

[Akinetic mutism and bicingular softening. 3 anatomo-clinical cases].

The authors describe three pathological cases of akinetic mutism with, as a common basic lesion, bilateral infarction of the cingulate gyrus secondary to aneurysm of the anterior communicating artery (case n degrees 1), to a huge olfactory meningioma (case n degrees 2), both operated on, and to atheromatous occlusion of the anterior cerebral arterial system (case n degrees 3). These three cases enable a variety of "anterior and waking" akinetic mutism to be described which is unusual enough to be compared with other mesencephalic and diencephalic aspects of this syndrome. It is in fact an akinetic mutism characterized by: a certain dissociation in its non-response to various stimuli, a particularly marked appearance of wakefulness when day-time alertness is considered, conservation of the waking-sleeping rhythm, perception and reaction unpredictable and paradoxical in both degree and quality, complete absence of any spontaneous verbal communication in contrast to relative break-down of solicited communication which is infrequent, uncertain and unresponsive to the usual methods of stimulation, without any possibility of a code. In addition, there is a remarkable mimic and segmental general akinesia, resistant to the usual nociceptive stimuli, but sensitive to slight excitation of the manual and oral zones. Besides this special akinetic mutism, there are variously systematised signs, mostly asymmetrical, indicating lesion of the cortico-sub-cortical frontal structures bordering on the gyrus cinguli. This unusual behaviour pattern corresponds in these three cases to extensive anterior bilateral ischemic lesions of the cingulate gyrus regularly associated with bilateral infarctions confined to the medial aspect of F1 in the superficial territory of the two anterior cerebral arteries, to possible neurosurgical changes (ablation of the right frontal pole) and to compressive or ischaemic lesions of the gyrus rectus. These exclusively cortico-sub-cortical associated lesions are in contrast with the remarkably intact caudate nuclei, the pallidal, thalamic, hypothalamic and septal formations and the anterior pillars of the fornix. These findings compared with the results of experimental research carried out by M. Kennard, help, if help is needed, to resolve the apparent contradictions between the effects of therapeutic cingulectomies or cingulotomies and the scanty pathological data already available in cerebral vascular pathology.

Adult↗

[Methyl bromide poisoning. 3 cases, 1 fatal. Neruopathological study of one case of coma with myoclonus followed for 5 years].

Three new cases of methyl bromide poisoning (leakage from a fire extinguisher) are reported. Two of these were characterized, after an initial period of coma and myoclonic status, by an action myoclonus. Electrical data are presented. A follow up of 4 and 8 years allowed to study the effect of drugs, two of which (clonazepam and baclofene) being pecularly contributive. The third patient outlived for 5 years in a stuporous state with myoclonus. The anatomical findings consisted mainly of a necrosis of both inferior colliculi with gliosis in the upper brain stem reticular formation and moderate changes in the dentate and pontine nuclei. Their significance is discussed in view of physiological and experimental knowledge.

Adolescent↗