[Conference at the Salpêtrière. March 1989. Rapid paraplegia, chronic arachnoiditis and hemispheric contrast enhancement in the x-ray scanner].
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Biomedical subjects
Publications and source records attributed to M B Delisle.
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Gliomatosis cerebri is a glial neoplastic process. It is diffusely distributed through brain structures whose anatomical configuration remains intact. Clinical diagnosis of this rare disease is difficult. The symptoms are varied and biological and radiological data are not specific. The case reported shows the characteristic features of the disease. Diagnosis was possible only on autopsy, in spite of several previous clinical and biological investigations and nuclear magnetic resonance study.
The authors report a case of malignant pheochromocytoma. They explain the difficulties of the diagnostic of malignancy which can only be confirmed by metastases. Radioisotope scanning with I131 metaiodobenzyl guanidine is the best available method to locate the primary tumor and the metastases. The results of radio isotope scanning are compared to the ultrastructural study.
The clinicopathological findings in a 49-year-old man who presented multisystemic neurological degenerative disease are reported. The patient presented, at the age of 36, distal upper limb amyotrophy and 8 years later pyramidal signs with fasciculations. In his last year of life, he suffered extrapyramidal hypertonus and mental deterioration. Neuropathological examination showed anterior spinal root and cerebral atrophy. Myelin pallor was mild and predominated on posterior cords. Anterior horn neuronal loss was noted in the spinal cord as well as Alzheimer-type changes in the brain. Inclusion bodies consistent with Lewy bodies were diffusely apparent. The peculiar clinical progression in this case and the extension of neuropathological lesions with inclusion bodies mainly in the substantia nigra and cerebral cortex are an interesting subject of discussion.
Over the last few decades, a new pathology has appeared, directly related to the modified immune status of the hosts. It presents several distinctive points. The central nervous system is particularly affected. The opportunistic pathogenic agents do not usually injure the brain parenchyma and are not known for their aggressiveness in normal adults (papovavirus). Diagnosis of these different diseases is often difficult, some biological tests being irrelevant because of alterations of the immune system (toxoplasmosis). The lesions may be exclusively located in the brain (tuberculosis, lymphomas) which is not usually affected by these agents. Response to therapy is frequently poor, the clinical course being rapid and fatal. However, therapy may be successful in some cases, justifying the use of somewhat aggressive procedures (biopsy) in order to obtain an accurate diagnosis. It is important to be aware of these data, and an understanding of them may help in managing these already difficult patients. They also make possible some interesting pathogenic hypotheses.
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Leigh's disease is a subacute metabolic encephalopathy characterized by bilateral and symmetrical lesions of basal ganglia, mid-brain and pons. Lesions are related to an abnormal pyruvate metabolism which mechanisms remain still uncertain. This disease is rare and the authors present a case with complete anatomic study.
A series of 22 cases of amyotrophic lateral sclerosis (ALS) and 22 controls have been assessed for the presence of neurofilamentous accumulations in axons and perikarya. Large axonal swellings were seen in the spinal cord of 12 controls and of 13 ALS cases. When they were present in ALS cases they tended to be much more numerous than in controls, and when they were not present in ALS cases there tended to be severe neuronal loss in the cord. Axonal swelling on lower motor neurons appears to be a significant feature of the pathology of ALS. Its implications in terms of etiology are unknown.
The case of a 33-year old woman with recurrent cerebral and peripheral ischaemic attacks is reported and the difficult nosological problem of juvenile ischaemia is discussed. Thromboangiitis obliterans (Buerger's disease) was excluded by morphological studies of finger tips, temporal artery and dermis, which demonstrated arteriolar dysplasia and premature aging of the connective tissue. A diagnosis of "juvenile atheroma" may be considered in view of the dyslipidaemia and increase in skin cholesterol and apoprotein B concentrations observed. A review of the literature, together with the biochemical and histological findings in this patient, suggests that ischaemic attacks, dysplasia of small vessels and serum dyslipidaemia reflected in the dermis are all manifestations of early atheroma.
The clinical and pathologic findings of a case of amyloidogenic heredo familial neuropathy type I is presented. The patient is a man, died at 46 years. The examination of a nerve biopsy, the clinical evolution and the family history are representative of the condition. The study of autopsy material revealed amyloïd deposits within vessels walls, autonomic nerves of almost all viscera but liver, muscle and skin, around myofibers in the heart. Kidney glomeruli were markedly involved and numerous obsolescent glomeruli were seen. Lesions of central nervous system were of interest: enlargement of meningeal vessels walls due to amyloïd deposits as subpial deposition band were observed. The pattern of amyloïd deposits within peripherical nerves was quite different according to their proximal or distal ends. While distal amyloïd deposits were scant, nerve damages were striking. In proximal portions, amyloïdosis could be found in all layers of nerve structures including the walls of endoneural small vessels. Semi-thin sections allowed to observe rare myelinated fibers entrapped by aggregates of a microfibrillary material. While there was absence of a patent axonal lesion, clusters of regenerating fibers were not infrequent. Ultrastructural study confirmed the close relationship between amyloïd fibrils, Schwann cell basal laminae and collagen fibers. An immunopathological study using immunserums raised against the AA composant of amyloïdosis, K and lambda light chains, and prealbumin were negative. Our results are in agreement with descriptions already reported in the medical literature. Therefore, they allow to discuss already known pathogenic mechanisms for this rare affection transmitted by a dominant autosomic gene.
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The third recurrence of a primary mucinous adenocarcinoma of the right parietal scalp was studied by histochemistry and electron microscopy. Histochemically the mucin produced by this neoplasm was of the sialomucine type. Electron microscopy revealed a high differentiated tumour whose cells looked like eccrine mucinous ones. The natural history of this carcinoma is similar to that of 33 ones already published. The tumours, round and elevated, grow in 75 p. 100 of the cases in patients over 50 years of age. They are located on the face or the scalp and more rarely on the trunk. Such tumours are frequently misdiagnosed as carcinomas. But the knowledge of thier morphological and histochemical features should lead to the right diagnosis all the more since the various biological and radiological investigations do not discover other primary neoplasm. Besides if these neoplasms are not metastases they are quite different from the other sweat gland carcinomas. They have a better prognosis than these tumours. However they give local recurrences, lymph node or subcutaneous metastases and a fatal issue in 6 p. 100 of the cases.
Progressive lumbar kyphosis (camptocormia), a rare, usually familial disease in elderly patients, is characterized by inability to immobilize the lumbar spine in relation to the pelvis. CT scan reveals selective involvement of the spinal muscles with a heterogeneous appearance and is in favour of a primary disorder of these muscles. Our aim was to define the muscular lesions and clarify their nature in this particular disorder. Biopsies of the paravertebral muscles of 14 patients with lumbar kyphosis and of 20 operated on for disc herniation or narrowed lumbar canal, were studied by light microscopy, histochemistry and electron microscopy. In both groups, type 1 fibre predominance and atrophy of type 2 fibres were observed. Ragged-red fibres with abnormal mitochondria also occurred. The differential feature was increased frequency of extensive diffuse or lobulated fibrosis in camptocormia. Other features related to pathogenesis could not be determined.
PURPOSE: The purpose of this work was to describe the CT and MR findings in three patients with cavernous hemangioma (CH) of the intracranial optic pathways. METHOD: CT and MR studies of three patients with CH of the optic chiasm were reviewed. All patients underwent MRI of the chiasmal area, with coronal T2- and T1-weighted studies as well as gadolinium-enhanced coronal and sagittal T1-weighted studies. RESULTS: The patients (mean age, 40 years) presented with chiasmal apoplexy (two cases) and progressive decrease of visual acuity (one case). In all cases, MRI showed regular enlargement of the optic chiasm, with extension to the optic nerve in one case and to the left optic tract in one case. The chiasmatic dimension was 2.5-3 cm in two cases and 1-1.5 cm in the other case. In all cases, MRI revealed an acute (isointense signal on T1-weighted and hypointense signal on T2-weighted sequences) or subacute (hyperintense signal on T1 - and T2-weighted sequences) hemorrhage with, adjacent to it, an area with signals of blood of different ages, highly suggestive of CH. CT showed, in chiasmatic CHs, a suprasellar mass spontaneously denser than adjacent brain parenchyma. In two cases, microcalcifications were associated. In two cases, CT and MRI revealed slight heterogeneous enhancement after contrast agent administration. In one case, no enhancement was observed. Two patients underwent surgery by frontopterional craniotomy. The optic chiasms were swollen with an intrinsic bluish mass. The cerebrospinal fluid was not xanthochromic. Microscope examination confirmed the diagnosis of CH. After 12 months, the operated patients had improved visual acuity and visual field but did not completely recover. The nonoperated patient (because of spontaneous rapid recovery of visual acuity) was followed clinically and on MRI over 18 months. CONCLUSION: CH in the optic chiasm must be suspected in the presence of an acute chiasmatic syndrome. MRI is the best imaging modality, showing either an acute or a subacute chiasmatic hemorrhage or the typical pattern of CH with heterogeneous alternation of foci of blood of different ages, with a central focus of methemoglobin, a peripheral rim of hemosiderin, adjacent foci of acute or subacute hemorrhage, and slight or no enhancement after gadolinium administration.
A patient operated upon 20 years ago for myocardial revascularization with two Vineberg procedures and one coronary artery bypass graft (CABG) on the right coronary artery had a recurrence of unstable angina due to the thrombosis of the left internal mammary artery (IMA) and the right CABG. The right IMA was patent but stenosed distally. Reoperation was performed with a direct end to side anastomosis of the patent right IMA onto the left anterior descending artery and a saphenous vein graft as a CABG on the marginal branch. We comment on the choice of this "second hand" IMA graft and the pathological appearances of the patent IMA.
To elucidate whether mild rejection requires treatment, we retrospectively examined the spontaneous natural history of this histologic feature without an increase of immunosuppression. During a 4-year period, 55 heart transplantations were performed in 54 patients on whom 958 endomyocardial biopsies were performed. Among these biopsies, 162 specimens showed features of mild rejection. We studied the results of subsequent biopsies performed 7 to 10 days later, without any change in immunosuppression. These revealed regression of lesions to minimal rejection in 51 cases (31%), the same histologic feature in 82 cases (51%), or progression to moderate or severe rejection in 29 cases (18%). In 82% of these cases, therefore, no aggravation of histologic feature was observed. We separated the cases in which current-study biopsies showed mild rejection into three groups according to the result of the most recent biopsy, that is, minimal, mild, or moderate-severe rejection. The percentage of good outcome was not modified by the nature of the previous biopsy specimen: 84%, if minimal rejection was preceding the study biopsy; 82%, in the cases of mild rejection; and 77%, for moderate or severe rejection. We did not find significant differences in this evolution between patients with fewer or more than two moderate or severe acute rejections in the first 4-month period after heart transplantation (respectively, 15% or 24% progression to moderate or severe acute rejection after nontreated mild rejection).(ABSTRACT TRUNCATED AT 250 WORDS)
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