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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↗
PubMed1984

[Cavitary orthochromatic leukodystrophy in the adult. Oligodendroglial proliferation and inclusions].

A 41 year-old woman presented with two Grand Mal fits followed by progressive spastic paraparesis and intellectual impairment. After 18 months there was a remission which lasted for 4 years but thereafter inexorable progression of the disease and many fits led to a state of akinetic mutism. Death occurred nine and a half years after the onset of the illness. There was no family history. The neuropathological studies included light and electron microscopy of a cerebral biopsy and a complete post mortem examination. Myelin loss and cavitation of the white matter was associated with the presence of sudanophilic lipid and abundant cells at the periphery of the lesions. These cells had the characteristics of oligodendrocytes. The combination of sudanophilic leucodystrophy, cavitation of white matter and oligodendrocyte proliferation has previously been observed only in childhood.

Adult↗
PubMed1984

[Baló's concentric sclerosis].

A clinico-pathological case of concentric sclerosis (Baló type) is reported. A 30-year-old man experienced dizziness, nausea and vomiting. Twelve days later he developed gait disturbances. Neurological examination showed broad based gait, brisk tendon reflexes, bilateral extensor plantar responses, right hemihypoesthesia, cerebellar dysmetria, and a left lateral gaze palsy. CSF examination showed, 520 mg p. 100 ml protein, 7500 red blood cells, 31 lymphocytes and 9 polymorphonuclear leukocytes/mm3, 18 p. 100 gammaglobulin. Three CT scans were performed and showed a round hypodensity in the parieto-occipital white matter with contrast enhancement on one occasion, and several other hypodensities in the contralateral parieto-occipital white matter and in both frontal lobes. 23 days after the onset of the disease, the patient became comatose. A cerebral biopsy was obtained from one of the frontal lesions. He died from aspiration bronchopneumonia 2 months after the first signs. Neuropathological examination showed numerous concentric zones of demyelination which involved the white matter of both hemispheres, brain stem, and cerebellum. On light microscopy sudanophilic myelin breakdown products were numerous in the bands of demyelinisation. Astrocytic proliferation was marked, with frequent Rosenthal fibers. Edema was noted in some lesions. Myelin-axonal dissociation was obvious, but some axonal swelling were observed. Electron microscopy demonstrated the integrity of oligodendrocytes and of blood vessels and confirmed the prominent alterations of the astrocytes. Fifteen similar cases of the literature have been reviewed. The present case seems to be the first one with CT scan examination and electron microscopic study of a brain biopsy. The nosological situation of Baló's disease among the inflammatory demyelinating diseases of the group of MS is discussed.

Adult↗
PubMed1983

Status spongiosus in the course of treatment of malignant gliomas.

Two cases of fatal encephalopathy which appeared in the course of treatment of malignant gliomas are described. CT scan showed diffuse, low density, non-enhanced lesions of the white matter. Pathological findings showed that the CT scan aspects corresponded to status spongiosus without demyelination. We were unable to find similar reports in the literature.

Brain↗
PubMed1983

[Acquired cerebral toxoplasmosis in adults. Clinical and neuropathologic study].

Two cases of acquired cerebral toxoplasmosis in adults are reported. In the first case no subjacent disease was found, and the neurological signs were of a meningo-encephalitic type with disorders of consciousness and a C.S.F. cellular reaction. A second case was observed in the course of the treatment of Waldenström disease by chloraminophene. The neurological signs were bifocal, and the C.S.F. showed a moderate increase of protein. In both cases C.T. scan showed contrast enhanced and hypodense areas. In the first case the enhancement was heterogenous and the hypodense area occupied the whole right hemisphere. The second case showed two ring enhancements in the left frontal and the right occipital regions. A cerebral biopsy was performed in both cases but no parasite was found. The diagnosis was presumed to be malignant lymphoma or encephalitis in the first case, glioblastoma in the second. No specific treatment was administered and the patients died within a few weeks. The complete pathological study by light and electron microscopy showed free and cystic forms of Toxoplasma Gondii. The results of the neuropathological examination and the thorough study of the biopsy specimens were compared to the pathological findings in the literature. The particular character of the necrosis and the inflammatory encephalitic reaction in the absence of the parasite may suggest the diagnosis when the clinical, biological and radiological data are compared.

Aged↗
PubMed1983

[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases].

Six clinico-pathological cases (4 males and 2 females) with a Parkinson-like syndrome which lasted an average of 5 1/2 years are reported. The average age at death was 73 years. Neuropathological features were similar to those described in post encephalitic Parkinson's syndrome and in Steele-Richardson-Olszewski disease. However, neurofibrillary tangles were less numerous; lesions in tectal, periaqueductal and reticular structures were less severe. Furthermore, the lesions seemed more pronounced in the substantia nigra, the globus pallidus and the nucleus subthalamicus, giving the appearance of a systematic pallido-luyso-nigral atrophy. None of these patients had a history of encephalitis. Clinical examination did not reveal the dystonic rigidity in extension of the neck nor the characteristic ophthalmoplegia of progressive supranuclear palsy. These 6 cases are compared to 10 cases of progressive supranuclear palsy typical both on clinical and pathological grounds. They could be considered as a particular form of Steele-Richardson-Olszewski disease without ophthalmoplegia.

Aged↗
PubMed1983

[Crossed cerebellar atrophy following hemispheric lesions occurring in adulthood].

Crossed cerebellar atrophy generally results from a large contralateral hemispheric lesion involving the cortico-spinal tract in neonates or infants. Two cases of crossed cerebellar atrophy which were particular from two points of view are reported: 1) the cerebellar alterations resulted from cerebral lesions which had occurred in adult life, after complete maturation of the central nervous system. One was observed in a 78 years old hypertensive woman who had presented 24 years before a right lenticular haemorrage, the other in a 73 years old woman who when aged 18 had presented a left cortical frontal traumatic lesion. The clinical and pathological data are compared to 13 similar cases reported in the literature. The retrograde or anterograde route of this transneuronal degeneration is discussed. The pathological features of those 2 cases lead the authors to favor the anterograde route; 2) the cerebral lesion was small and strictly limited to F2 in 1 case, the secondary lesions involving then, only the medial pontine nuclei and the quadrangular cerebellar lobule. This allows a clear demonstration, in man, of a systematisation of the cortico-pontine and ponto-cerebellar fibers similar to what has been described in the animal (Brodal, 1980).

Age Factors↗
PubMed1982

[Trichinosis of the central nervous system. One case (author's transl)].

A case of trichinosis involving the cental nervous system is presented. The neurological symptoms developed 20 days after ingestion of the larvae and 3 days after the onset of facial oedema. They consisted of behavioural disorders, tetraparesis, incontinence and oculomotor paralysis. All symptoms progressively regressed. Computed tomography showed transient low-density areas in the white matter. The main manifestations and the physiopathological mechanisms (transport of the parasite through the bloodstream, immuno-allergic reaction) of cerebral trichinosis are discussed.

Adult↗
PubMed1982

Parinaud's syndrome: electro-oculographic and anatomical analyses of six vascular cases with deductions about vertical gaze organization in the premotor structures.

Six cases of Parinaud's syndrome, with downward (Cases 1, 2), upward (Cases 3, 4) and both downward and upward gaze paralysis (cases 5, 6) are reported. Four cases (Cases 1, 2, 3, 5) were studied anatomically using serial sections of the brain and 3 cases (Cases, 1, 4, 6) analysed electro-oculographically. In all the cases there were rather small vascular lesions in the mesodiencephalic region, sparing the oculomotor nuclei. Since the rostral interstitial nuclei of the medial longitudinal fasciculus (riMLF), located above the oculomotor nuclei, contain the final relays producing all vertical saccades, it is suggested that the different aspects of Parinaud's syndrome may result from damage to their cells or to their excitatory efferent tracts, or even to their afferent pathways. Downgaze paralysis results from bilateral lesions involving the regions located just caudal, medial and dorsal to the upper poles of the red nuclei. The critical area is probably related to the mediocaudal part of the riMLF, the lateral portion of which appears to be spared. These anatomical data, combined with the clinical observation that most downward eye movements (except slow reflex movements) are affected in the case with such paralysis, lead us to propose that it is the riMLF efferent tracts mediating downgaze and projecting on to the oculomotor nuclei that are principally damaged by the lesions. Upgaze paralysis results from unilateral lesions in or near the posterior commissure. The clinical data allow us to propose that it is also the riMLF efferent tracts, mediating upgaze, that are damaged in such cases. consequently these tracts, probably originating from the dorsolateral part of the riMLF, would decussate through the posterior commissure before they reach the oculomotor nuclei. Combined downgaze and upgaze paralysis results from bilateral lesions involving the region related to the whole riMLF on both sides. The principal conclusion is that the riMLF efferent tracts mediating upward and downward gaze have clearly separate courses in the immediate premotor structures.

Adult↗