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R Escourolle

Publications and source records attributed to R Escourolle.

At least 19 recordsLinked to original sources
PubMed1986

[Myoclonic cerebellar dyssynergia (Ramsay-Hunt syndrome) and cerebellar telangiectasia].

A 8 year-old girl presented with generalized epileptic seizures followed by the progressive onset of myoclonic jerks, sometimes associated with willed movements, and a static and kinetic cerebellar syndrome without conspicuous intellectual impairment. Death occurred 10 years after the onset of the disorders. There was no family history. Neuropathological studies showed lesions confined to the cerebellum. Diffuse and bilateral telangiectases were present in the cerebellar white matter. They were associated with patchy cortical alterations of the distal parts of some folia involving mainly the granule-cells ans sparing the Purkinje cells. No Lafora bodies and no abnormal lipofuscin storage were observed. The dentate nuclei, superior cerebellar peduncles and red nuclei were normal as were the inferior olives and inferior cerebellar peduncles. The spino-cerebellar tracts were unaffected. This case confirms the hypothesis that dyssynergia cerebellaris myoclonica corresponds only to a clinical entity. It may be encountered in various degenerative or metabolic disorders involving the cerebellum and/or its pathways. To our knowledge the association of a Ramsay Hunt syndrome with a vascular malformation has not been previously reported.

Adolescent↗
PubMed1986

Postmortem studies on posthypoxic and post-methyl bromide intoxication: case reports.

In two cases of action myoclonus following hypoxic or shock encephalopathy, neuropathological examination disclosed mild or moderate scattered changes involving thalamus, griseum centrale mesencephali, and nucleus centralis superior. Other areas were affected only in one of these cases (striatum, nucleus subthalamicus or hippocampus, nuclei pontis, and cerebellar cortex). In another case (an alcoholic patient), the changes, which involved only corpus mamillare and thalamus, were those of Wernicke-Korsakoff encephalopathy. In one case of oscillatory myoclonus following septic shock, there were marked cerebellar changes involving deep nuclei and mild abnormalities in the thalamus and inferior olive. The last case of action myoclonus following acute methyl bromide intoxication was characterized by marked changes in the inferior colliculi and moderate or mild abnormalities of thalamus, griseum centrale mesencephali, nucleus centralis superior, nucleus reticularis tegmenti pontis, nuclei pontis, and dentatus. The findings are compared with the data of seven previously reported neuropathological examinations in action myoclonus following hypoxic encephalopathy.

Adult↗
PubMed1985

Value of multiple sclerosis diagnostic criteria. 70 autopsy-confirmed cases.

We have evaluated the sensitivity of the most recent and most frequently used criteria for the diagnosis of definite multiple sclerosis by the retrospective study of the clinical files of 70 pathologically confirmed cases. For each case, the date of diagnosis was determined separately using different sets of criteria. The delay of diagnosis was then calculated. The diagnosis was made significantly earlier when certain criteria were used. This was more marked in the earlier years of the disease. We also found that cases of multiple sclerosis with progressive courses were diagnosed later than cases with other courses, whatever the criteria used. This was statistically significant only for a single criterion.

Autopsy↗
PubMed1985

Leukoencephalopathy in diffuse hemorrhagic cerebral amyloid angiopathy.

We have studied 12 patients with diffuse hemorrhagic cerebral amyloid angiopathy clinically and at postmortem examination. The brains in 8 patients had diffuse bilateral loss of myelin in the hemispheric white matter sparing the U fibers, corpus callosum, and internal capsules. The periventricular areas were predominantly affected. Microscopic examination of the white matter showed an association with subacute or chronic edematous lesions: spongiosis, swollen oligodendroglia, widening of the perivascular spaces with edema fluid or siderophages, hyalinization of the blood vessel walls, incomplete myelin loss, and astrocytic gliosis. Three of 8 autopsied patients had undergone computed tomographic examination, which showed bilateral hypodensity of the hemispheric white matter. The brains of 4 patients with illnesses of shorter duration showed only discrete but similar lesions in the centrum semiovale. These white matter changes are similar to those observed in Binswanger's subcortical encephalopathy. We suggest that a common mechanism of hypoperfusion of the distal white matter causes the leukoencephalopathy.

Aged↗
PubMed1985

Cortical atrophy in senile dementia of the Alzheimer type is mainly due to a decrease in cortical length.

A prospective study was undertaken to select mentally normal old subjects and patients with senile dementia of the Alzheimer type (SDAT). The test score of Blessed et al. (1968) (BTS) was used to determine the severity of mental impairment. A pathologic study confirmed the diagnosis of either SDAT or normal brain aging at postmortem examination in 12 cases. The cortical area and the cortical perimeter of the different cerebral lobes were measured on 1-cm-thick coronal sections using a semiautomatic image analyzer. Cortical length and thickness were calculated using perimeter and area values. BTS was significantly correlated with both the area (r = 0.7695, P = 0.003) and the length (r = 0.7421, P = 0.006) of the temporal cortex. There was no significant correlation between BTS and thickness of the temporal cortex (r = 0.559, P = 0.059). These results show that reduction of length is one of the major determinants of cortical atrophy. Although this has to be confirmed by histological study, they favor the hypothesis of a column-selective atrophy in SDAT which should be considered in the interpretation of the microscopic data.

Aged↗
PubMed1985

[Arteriopathic leukoencephalopathy (17 anatomo-clinical cases)].

Seventeen clinico-pathological cases of leukoencephalopathy due to vascular diseases are reported. All of them had diffuse, often spotty demyelination sparing the U fibers, the corpus callosum, the internal capsule and the optic radiations. Microscopic examination of the white matter showed the association of the following lesions: oedema, swollen oligodendroglia, spongiosis, incomplete loss of myelin, astrocytic gliosis with Rosenthal's fibers, widening of the perivascular spaces with oedema fluid or hemosiderin laden macrophages, thickening and hyalinization of the blood vessels walls. Among those cases, 8 were consistent with Binswanger's subcortical encephalopathy (5 males, 3 females, mean age at death: 64 years, mean illness duration: 6, 7 years); all of them were hypertensive and had lacunae in the basal ganglia, hemispheric white matter and pons. Another case could be considered as a cortico-subcortical chronic hypertensive encephalopathy (77 y.o., female, illness duration: 4 years); she was hypertensive and had small cortical infarcts and perivascular bleedings, lobar and cerebellar hematomas, but no amyloid deposits in the blood vessels walls. Eight patients (5 males, 3 females mean age at death: 72 years, mean illness duration: 5, 4 years, 6 normotensive, 2 hypertensive) had diffuse meningocortical amyloid angiopathy with multiple small cortical infarcts, small cortical perivascular bleedings, slit haemorrhages and one or more lobar hematomas. Four of them had numerous senile plaques and neurofibrillary tangles. To our knowledge such a leukoencephalopathy in cerebral amyloid angiopathy has not been yet pointed out. It was present in 8 out of 12 cases of diffuse haemorrhagic form of cerebral amyloid angiopathy observed in the Charles Foix Laboratory of la Salpêtrière, during the last 10 years. A common mechanism with hypoperfusion of the distal white matter and alteration of the blood brain-barrier is suggested for this leukoencephalopathy.

Aged↗
PubMed1985

[Nerve and muscle microvasculitis: 50 cases].

Fifty consecutive cases of nerve and muscle microvasculitis (MV) seen on nerve and muscle biopsies were studied. These were observed in a 5 years period, among 1076 nerve and/or muscle biopsies performed in adult patients in the Laboratoire de Neuropathologie Charles Foix. The systemic necrotizing vasculitides, in which the arteries of diameter greater than 70 microns are involved, acute polymyositis, sarcoidosis and acute polyneuritis were not considered in this study. Mononuclear cell infiltration was the rule. It was associated to leukocytoclasis in 2 cases. No fibrinoid necrosis was seen. These changes were highly diagnostic when seen in the nerve or the connective tissue of the epi or perimysium. The etiology of these microvasculitides was mainly connective tissue diseases (42 p. 100) and, overall, panarteritis nodosa (16 p. 100), or malignancies (28 p. 100) which comprise 7 solid tumors and 4 lymphomas. Other cases were related either to systemic diseases (thromboangiitis obliterans, monoclonal dysglobulinemia, cholesterol embolus) or to local trauma. The relationship between MV and peripheral neuropathy was less obvious in 3 cases of mononeuritis multiplex associated with diabetes mellitus and in 3 cases of acute idiopathic and regressive mononeuritis multiplex. In 5 cases, no cause was found.

Adult↗
PubMed1985

[Clinical analysis of 70 neuropathologic cases of multiple sclerosis].

A retrospective study of clinical files of 70 pathologically confirmed cases of Multiple Sclerosis (MS) (53 women and 17 men), selected from the records of the Laboratoire de Neuropathologie Charles Foix (Hôpital de la Salpêtrière) was performed. The following data were recorded and analysed by a computer program (HP 85): sex, age of onset of disease, clinical course (classified into Remittent, Remittent-Progressive, Progressive and Acute) and the date of each new neurological symptom or sign. The mean age of onset was 36.8 +/- 12. In women, the disease began earlier (34.6 +/- 12) and the duration was longer (17.4 +/- 12). In men the age onset was 40.6 +/- 11 and the duration was 12.5 +/- 6. In remittent courses, the mean age of onset was 30.8 +/- 13 and the duration was 21 +/- 10. In progressive courses, the age at onset was 45 +/- 10 and the duration was 2. In women, progressive courses began significantly later (42.3 +/- 9.2) and were shorter (15 +/- 8) than remittent courses which began at 26.8 +/- 8.2 and lasted 23 +/- 10. The histogram of the duration of clinical courses showed three groups: acute courses (8 cases less than 5 years long), intermediate courses (41 cases, between 5 and 20 years long), and long courses (21 cases longer than 20 years). Women were more often affected with acute (7/8 cases) or long courses (20/21 cases). The mean duration of the disease was the same when the symptoms and signs at onset were motor weakness, sensory disturbances, optic neuritis or diplopia.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1979

Moyamoya disease and intracerebral hematoma. Clinical pathological report.

This report concerns a hypertensive woman who suffered a stroke at the age of 51 and recovered from left hemiparesis after 3 to 4 months. During the subsequent 24 years she had four seizures which involved the left arm and face and became generalized, ending with left hemiparesis, from which she recovered after 4 to 5 days. Carotid angiography was performed in 1967, 1973 and 1974 and the characteristic picture of moyamoya disease was demonstrated. She died at the age of 77 with extensive vascular disease. The literature concerning 215 cases of moyamoya disease, in which there were 14 intracerebral hematomas, is reviewed and discussed.

Adult↗
PubMed1979

[Diagnosis of cerebral hemorrhages. A report of 247 anatomo-pathological cases (author's transl)].

A series of 247 cases with simple and apparently primitive cerebral hemorrhages selected from the pathological records of Charles Foix laboratory from 1962 to 1977 is reviewed with regard to the accuracy of the premortem diagnosis as a function of the size, the location and the age of the bleeding, the patient's age, the practices of angiography or lumbar puncture and the considered period of death. The clinical diagnosis of cerebral vascular disease was made in 75% of the cases from that serie but the hemorrhagic mechanism was identified only in 50,6% of cases. The diagnosis accuracy was enhanced in large and recent hemorrhages occuring in patients under 70 years old. It seemed better in cerebellar and intermediate locations. It was enhanced in more recent observations (collected since 1974). The practice of cerebral angiography and lumbar puncture rose up (in a somewhat equivalent way) the proportion of accurate diagnoses. The lumbar puncture practice was not associated with a higher rate of cerebral herniae.

Adult↗
PubMed1979

[Quantitative and electron microscope study of the nerve in seven cases of sporadic idiopathic sensory neuropathy (author's transl)].

An electron microscope study and quantitation of myelinated and unmyelinated fibers of seven nerve biopsies performed in sporadic cases of idiopathic sensory neuropathy is reported. The number of myelinated fibers is markedly decreased or absent in every case. On the contrary, the unmyelinated fiber numbers are normal or increased. In most cases, the small diameter myelinated and unmyelinated fibers proportions are higher than those of control biopsies. The electron microscope study discloses evidence of degeneration of Wallerian tye and regeneration is also indicated by quantitative studies. Regenerative phenomena seen more obvious in sporadic cases than in previously reported studies of familial cases.

Biopsy↗
PubMed1979

[Progressive dialytic encephalopathy. Role of the aluminium and neurological study. One case (author's transl)].

Report a typical case of dialytic dementia in a patient treated with aluminium gels. The course was fatal in fifteen months duration. Before interruption of aluminium gel intake, the aluminium blood level measured by atomic absorption spectrography was at 1300 microgram/l (normal less than 40 microgram/l). Cerebral aluminium was studied by the method of Le Gendre and Alfrey. On the three studied specimens of gray matter including, the parieto rolandic cortex, the thalamus, the cerebellar cortex, the mean aluminium concentration was seven times higher than the witness. The optic and electronic microscopy study showed important accumulation of lipofuscin. No neurofibrillary degeneration was observed. In contrast to the intensity of the clinical signs and the fatal course the cerebral lesions were slight.

Aluminum↗
PubMed1979

[Electron microscopic and neurochemical study of Alexander's disease (author's transl)].

The authors report the results of a cerebral and of a neuromuscular biopsies and of the autopsy findings in another infantile case of Alexander's disease in a girl. They review the 17 previously reported cases of this disease and the various etiopathogenic hypotheses mentioned. The presence of numerous, sometimes abnormal enlarged mitochondria and of abundant membranous cytoplasmic bodies in the astrocytic cytoplasm seems to be unreported elsewhere. Peripheral nerve changes are mentioned for the first time.

Astrocytes↗
PubMed1979

Analysis of the major lipid classes in human peripheral nerve biopsies. Age group differences and abnormalities of ganglioside level in perhexiline maleate therapy.

We report here the results of a simple and reproducible technique which can be used in semi-routine analysis of peripheral nerve biopsy specimens, so as to have a quantitative analysis of the major lipid classes, i.e. cholesterol, cerebrosides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine + phosphatidylinositol, sphingomyelin and gangliosides. Glycolipid hexoses, cholesterol and total phospholipids have been compared in different age groups. Although all lipid classes increased from the younger to the older age group, the molar ratio of cholesterol to phospholipid differed less than the glycolipid to phospholipid ratio. Both increased significantly, even between age group 10--16 and older patients (36, 54, 61, 68, 72 and 73 years old). Although individual variations in lipid content are noteworthy, it must be emphasized that evolution with age of the lipid composition must be taken into account. Furthermore, this study confirms and extends earlier findings of increased ganglioside levels in some cases of peripheral neuropathies observed during perhexiline maleate therapy where characteristic lipid-like polymorphous inclusions have been demonstrated.

Adolescent↗
PubMed1979

[Chronic inflammatory neuromyopathies in adults treated for gluten-sensitive enteropathy. A report on three cases with microvascular nerve and muscle lesions (author's transl)].

Neuromyopathies developed in three patients with gluten-sensitive enteropathy, a long time after they had been cured of their digestive disease by following a gluten-free diet. These cases differed radically from typical deficiency neuropathies by the presence of microvascular inflammatory lesions in nerves and muscles. The semiological findings were similar in all 3 cases, and were distinguished by the association of signs eveking lesions of the largest myelinated nerves fibers to the posterior rami with lesions in the muscles. Corticotherapy improved the condition but did not affect its chronic course. Nerve and muscle biopsies revealed the presence of segmentary microrascularitis, mainly lymphohistiocytic. The probable mechanism of these histological changes is alterations in the circulating immune-complexes, usually found in gluten-sensitive enteropathy, producing various types of associated disorders. Some of these immune-complexes would not be related straight to digestive intolerance to gluten, but would persist during the gluten-free diet period, and could be responsible for the micro-angiitis.

Adult↗