Posterior callosal infarction. Clinicopathological correlations.

Clinicoanatomical observations on a patient with partial interhemispheric disconnection associated with complete ischaemic destruction of the splenium and of the posterior part of the body of the corpus callosum are presented. Neuropathological examination of the areas containing degenerated white matter indicated that the lesions affected the transcallosal fibres that link the cortex of the occipital lobes and the superior parietal lobules (SPL). The white matter situated in the temporal lobes was intact. This suggests that in man, and contrary to what has been described in the monkey, the callosal pathway followed by the temporal fibres is rostral to the one followed by the parietal or at least by the SPL fibres. The most prominent disconnection syndrome elements were left tactile anomia (in spite of a rather good tactile-motor integration between the hemispheres), left visual anomia, agraphia of the left hand and 'diagnostic' apraxia. The fact that the anterior part of the corpus callosum was intact accounts for the preservation of interhemispheric transfer of somatic sensory information and for the absence of left extinction during the dichotic listening test. An attempt is made to give a more detailed explanation of the results obtained during the different tests.

Fibromuscular dysplasia of cervico-cephalic arteries with multiple dissections and a carotid-cavernous fistula. A pathological study.

A rare case of widespread fibromuscular dysplasia (F.M.D.) is reported, involving the cervico-cephalic arteries associated with multiple dissections, saccular aneurysms and a carotid-cavernous fistula. A detailed post-mortem examination revealed FMD involvement of the intracranial vessels, not demonstrated by arteriography.

[Sarcoidosis of the central and peripheral nervous system]. / Sarcoïdose du système nerveux central et périphérique.

The authors report two cases of Besnier -Boeck- Schaumann sarcoidosis with a neurological presentation. The first case was a 25 year old woman with a 5 year history of polyneuritis affecting the cranial and spinal nerves with very low nerve conduction times and without any detectable visceral disease. The diagnosis was made by distal bronchial biopsy. The second case was of an elderly man with tremor, difficulty in keeping his balance and deafness with chronic meningitis. The diagnosis was made by proximal bronchial biopsy, chest X-Ray showing a benign hilar lymphoma. In the first case, therapy comprised two three months courses of high dose intravenous steroids followed by oral corticosteroid therapy tailing off over three weeks. In the second case, the same intravenous steroid therapy was relayed by continuous oral steroids. The therapeutic effect was spectacular in the first case, but incomplete in the second, the intravenous steroids seeming to be more effective than the oral steroids.

[Treatment of malignant glioma. Apropos of 39 cases]. / Traitement des gliomes malins. A propos de trente-neuf cas.

Thirty-nine patients with malignant gliomas (36 glioblastomas, 3 astrocytomas grade III; age median 52.5) were treated after surgery by combination chemotherapy followed by irradiation. The median survival on the actuarial curve is 7 months. An attempt is made to evaluate each therapeutic modality by analysis of clinical and CT parameters.

[Thalamic dementia. Expansive lacunae of the thalamo-paramedian mesencephalic area. Hydrocephalus caused by stenosis of the aqueduct of Sylvius]. / Démence thalamique. Lacunes expansives du territoire thalamo-mésencéphalique paramédian. Hydrocéphalie par sténose de l'aqueduc de Sylvius.

The case reported here is that of a woman, without any significant pathological antecedent. At age 54, she developed signs of thalamic dementia and died 5 years later. The prominent symptoms were massive anterograde amnesia, apathia, apragmatism, ataxia and a frontal syndrome. She never showed aphasia, apraxia, agnosia or disorders of ocular motility. The C. T. Scan showed lacunar low densities in the mesencephalon and both thalami, mainly on the left side, as well as hydrocephalus caused by a stenosis of the aqueduct as shown by other neuroradiological procedures. The neuropathological examination showed space-occupying lacunae, bulging in the third ventricle, squeezing the aqueduct and protruding into the fourth ventricle. These lacunae were situated in the territory of the paramedian mesencephalo-thalamic arterial pedicle. They were perivascular spaces distensions, probably caused by disorder of the permeability of the arterial wall. This hypothesis is supported by the presence of severe lesions of segmental necrotizing angeitis on a paramedian mesencephalic artery. The etiology of this angeitis is unknown. As far as we know, such neuropathological lesions have never been reported previously. Therefore the pathology and etiopathogeny of cerebral lacunae should be reconsidered.

[Recovery from chronic polyradiculoneuritis after high-dose intravenous corticoid therapy]. / Guérison d'une polyradiculonévrite chronique après corticothérapie intraveineuse à fortes doses.

A chronic relapsing polyneuropathy did not improve with oral prednisone therapy. After 7 years, 3 intravenous infusions of 1,5 g of methylprednisolone led to complete recovery which is present after 4 years.

[Oligodendrogliomas. Clinical and therapeutic study (author's transl)]. / Contribution à l'étude clinique et thérapeutique des oligodendrogliomes. Etude préliminaire è propos de trente-quatre observations.

Preliminary study concerning oligodendrogliomas : 21 cases treated only by neurosurgery ; 10 more recent cases by neurosurgery associated with chemotherapy. Discussion of the various factor : clinical symptomatology ; scintigraphic and tomodensitometric data ; time of evolution ; histological type. Favourable impression of chemotherapy effects. Necessity of a multicentric projective study on this subject.

[Amnesia from bilateral ischemic lesions of the thalamus (author's transl)]. / Amnésie par lésion ischémique bi-thalamique.

A 43-year-old woman developed during post-partum an axial amnesia of ischemic origin. Stereotactic correlations were obtained by matching CT scan images with the true reference plane of the examination, according to an original method. This led to the diagnosis of infarction in the territory of a paramedian thalamomesencephalic artery. The amnesia probably resulted from bilateral lesions of Vicq d'Azyr's bundle. Atypical features in this case were the absence of disorder of vigilance and oculomotor disorders. This striking fact in this patient was recovery of some recognition capability contrasting with the persistence of a major evocation disorder.

[Myopathy, osteomalacia, and congenital hypoalbuminemia (author's transl)]. / Myopathie. Ostéomalacie et hypoalbuminémie congénitale.

A myopathy revealed the presence of a vitamin-sensitive osteomalacia in a patient with congenital hypoalbuminemia. The severe myopathy and osteomalacia recovered after treatment with 25-hydroxycholecalciferol. Hypoalbuminemia was detected in two brothers of the patient; parents and grandparents were consanguineous. Various tests demonstrated that the patient's hypoalbuminemia was due to faulty synthesis. Albumin reactions to several drugs were normal, but those normally transported by albumin have to be administered cautiously because of the low plasma concentrations. The possible relationship between hypovitaminosis D and congenital hypoalbuminemia, an association considered to be fortuitous by Montgomery, remains debatable.

[Dysautonomia and behavioural disorders from periventricular metastasis (author's transl)]. / Dysautonomie et troubles du comportement par métastase périventriculaire.

In a patient presenting dysautonomia a severe orthostatic hypotension revealed the presence of a subependymal metastatic infiltration from a large cell anaplastic bronchial carcinoma. Clinical pharmacological studies were conducted. A paradoxical sinus bradycardia during orthostatism, and an absence of bradycardia after clonidine injection, were suggestive of a central origin of the dysautonomia. At post-mortem, metastatic infiltration involved the walls of the lateral ventricles, and the floor of the IIIrd and IVth ventricles, from where it invaded structures responsible for cardiovascular control. Correlations could be established between the results of the clinical pharmacological tests and the pathological findings. Also present were a) anterograde amnesia dating from the onset of the orthostatic hypotension, probably due to compression of the anterior pillars of the trigone by tumour invasion of the septum; b) behavioural disorders with absence of spontaneous movement and speech, indifference and docility, probably arising from destruction of the septum.

[Probable cerebral tuberculoma in an apparently healthy Caucasian: normal computed tomography recordings after two months of treatment (author's transl)]. / Tuberculome cérébral probable chez un métropolitain floride: normalisation tomodensitométrique en deux mois de traitement.

A probable case of cerebral tuberculoma is described. An atypical feature was the occurence of the lesion in an apparently healthy Caucasian. Computed tomography examinations enabled the course of the disease to be followed-up during treatment.

[Acute neuromyelitis optica and acute pulmonary tuberculosis (author's transl)]. / Neuromyélite optique aiguë associée à une tuberculose pulmonaire aiguë.

A case of neuromyelitis optica and acute pulmonary tuberculosis in a 22 year old moroccan girl is reported. Regression of the medullary lesion and high level gamma globulin inflammatory CSF bring this case close to multiple sclerosis. The association with acute pulmonary tuberculosis suggest an acute demyelinisation caused by an immunologic reaction.

Asterixis in focal brain lesions.

Asterixis was observed in 20 cases of focal brain lesions. Metabolic or toxic factors were excluded. An electromyogram study of asterixis was carried out in nine cases to establish the diagnosis. The site of the focal lesion was either parietal or mesencephalic and was always contralateral to the asterixis. "Focal asterixis" could result from a dysfunction of the sensorimotor integration in the parietal lobe and the midbrain.

[Interhemispheric disconnection syndrome with diagonistic dyspraxia in the course of Marchiafava-Bignami disease]. / Syndrome de dysconnection inter-hémisphérique avec dyspraxie diagnostique au cours d'une maladie de Marchiafava-Bignami.

The probable diagnosis of Marchiafava-Bignami disease was made in a chronic alcoholic patient because of the rapid onset of signs of inter-hemisphere disconnection. These inclused left visual and tactile anomia, difficulty of a limb to imitate postures presented in the contralateral visual half-field, constructional apraxia of the right hand, agraphia of the left hand, left auditive extinction in the dichotic test, difficulty in responses of the left limbs to verbal orders, which the authors propose to name left "aphasic apraxia" and a bimanual asynergia, sometimes presenting an aspect of diagonistic dyspraxia. These symptoms clearly show the competence of the left hemisphere for verbal tasks, or more generally, those of an imaginary or conceptual nature, and that of the right hemisphere for visiopatial tasks, or more generally, those required by concrete situations.

[Clinical anatomical study of a case of amnesic encephalitis of herpetic origin (author's transl)]. / Etude anatomo-clinique d'un cas d'encéphalite amnésiante d'origine herpétique.

A report on a case of herpes encephalitis in a patient of particularly high intellectual level who was kept under observation for a period of 13 years. Anatomical examination of the brain demonstrated massive bilateral destruction of the limbic system. Having studied the higher functions of this patient for many months it was possible to establish interesting correlations relating to the role of the limbic system. It appears to be indispensable for the utilization of verbal, praxognosic, and conceptual information. Though this is still retained in the intact neocortex, mobilization of the information is possible only when stimuli arise from the exterior and for as long as they last. Spontaneous evocation of this data is, in fact, impossible, as is the stocking of new information. The limbic system, therefore, while not being the site of retention of memorised informations which is the role of the neocortex, appears to be a logic system. Which is indispensable for proper functioning of the memory machine.