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A direct immunofluorescence study was performed on cerbro-spinal fluid (CSF) cells of 24 patients with multiple sclerosis (MS) and 26 control patients with other neurological disorders. A significant increase (p less than 0.01) of the percentage of lymphoid cells with intracytoplasmic IgG (IgGLC) was found in a group of 20 IgG tested MS cases as compared to a control group of 22 IgG tested patients. However, the statistical significance was lost when the same MS group was compared to a group of 10 neurological diseases with supposed immunopathological mechanisms. An increase of the percentage of the IgA-containing cells was also found in some cases of MS. A good relationship appeared between the increase of IgGLC cells and the presumed intrathecal secretion of immunoglobulins as assessed on the protein composition of the CSF. This technique might be of some value in the diagnosis of MS.

Post-mortem study of every patient who died from AIDS in Pitié-Salpêtrire Hospital from June 1984 to November 1985 was performed without regard to the presence of neurological signs and symptoms. Autopsy were performed in 31/48 cases. Patients had been hospitalized in the Departments of Parasitology-Infectious Disease (24 cases) Internal Medicine (4 cases) and Neurology (3 cases). In every case, formalin-fixed material from the brain and the spinal cord were embedded in paraffin (20 samples), stained with hematoxylin-eosin, PAS, Alcian blue, Giemsa, Grocott and Ziehl techniques and Bodian's silver impregnation along with Luxol fast blue, and, in celloïdin (8 samples), stained with hematoxylin-eosin and Loyez' impregnation. There were 30 men (27 caucasian, 1 egyptian, 1 haïtian, 1 senegalese) and one woman (congolese). Twenty eight (28) patients were homosexuals. AIDS was transfusion-associated in two cases. Neurologic complications revealed the disease in 2 cases. Eighteen (18) patients had neurological signs or symptoms before death. Age range at death was 22-58 (mean 38). Brain weight in AIDS (from 1150 gms to 1750 gms-mean 1428 gms) was not statistically different from the mean weight of 100 male patients in the same age range autopsied in the same laboratory during the identical period (mean 1427 gms, standard deviation: 23). Microscopic abnormalities were present in every brain examined. These included non-Hodgkin lymphoma (3 cases), opportunistic infections (21 cases: 13 toxoplasmosis, 4 cytomegalovirus encephalitis, 3 cryptococcal meningitis, 1 infection by mycobacterium avium-intracellulare), and subacute encephalitis (17 cases, 9 isolated, 8 associated with other disorders). The characteristic changes consisted of lympho-monocytic focal infiltrates (so-called microglial nodules) and mild lympho-monocytic perivascular cuffs in 10 cases. Typical giant cells were seen only in one case. Mild demyelinating changes were also seen in only one case. No spinal cord spongiosis, nor Progressive Multifocal Leukoencephalopathy was found. HIV localization was performed on frozen sections utilizing in situ hybridization techniques (2 cases) and immunohistologic techniques (5 cases). HIV, RNA and proteins, was detected in 2 cases with subacute encephalitis. Infected cells were labeled with macrophage markers, and rarely with T4 lymphocyte markers. Infected astrocytes (identified by anti-GFAP serum) or neurons (identified by anti-NSE serum) were never observed. No giant cells were seen in these two cases.

Dolichoectatic intracranial arteries (DIA) have been given several names, e.g. fusiform aneurysms, dolichomega-arteries. It is not an uncommon condition and it raises interesting points: 1) symptoms and signs result from compression (cranial nerve palsies; hydrocephalus) from ischemic or hemorrhagic episodes; 2) the diagnosis is made easier with CT since the arterial wall is often calcified; 3) the pathophysiology has long been assumed to be atherosclerotic in nature, but controversial assumptions are discussed; 4) associated arterial abnormalities are common: one of our cases and approximately half of the post mortem reported DIA were associated with aneurysm of the abdominal aorta. Two cases are reported here: 1) A 56 y/o male presented with progressive gait disorder, deafness and ischemic episodes. CT showed DIA and hydrocephalus. DIA involved the basilar artery, both intracranial carotid arteries, the abdominal aorta and probably one renal artery. A ventriculo-atrial shunt was performed unsuccessfully. Post mortem findings are reported; 2) A 52 y/o male had suffered from trigeminal neuralgia for about one year and from trigeminal permanent pain for two weeks. He had developed left hemiparesis and dysarthria. DIA involved the basilar artery and the left intracranial internal carotid artery. The right internal carotid artery and the right vertebral artery were hypoplastic. Clinical, radiographical and pathophysiological features are reviewed.

Recent data favor the hypothesis of a genetically-induced overproduction of amyloid protein as the cause of Alzheimer's disease. This could be responsible for the occurrence of senile changes (senile plaques and neurofibrillary tangles) which are both characteristic of so-called normal aging of the brain and of Alzheimer's disease and lead to neuronal death. However, as shown by studies of twins, epigenetic factors must be very important in the triggering and/or acceleration of this process since the concordance between monozygotes does not reach higher levels than 41. It is argued that brain microglia, and/or astrocytes are the most likely candidates to the regulatory function of amyloid deposit in the brain. These cells could act by modulation of either the hydrolysis of the precursor protein during amyloidogenesis or the resorption of amyloid fibrils, which could occur in Alzheimer's disease as suggested by recent data. Further research on this topic could have therapeutical repercussions.

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.

We report a neuropathological study of cerebellar infarctions involving the territory of the posterior inferior cerebellar artery (PICA) in 28 cases. Fifteen cases involved the PICA territory only. In 13 cases infarctions in the anterior inferior cerebellar artery (AICA) territory and/or in the superior cerebellar artery (SCA) territory were also present. A thorough post-mortem study of the arterial supply of the brain from the heart up to the cerebellar arteries, including the cervical spine segment of the vertebral arteries was performed in 27 cases. The territory of the cerebellar infarcts has been ascertained. In 15/28 cases (54 percent), infarction involved the PICA territory only (17 infarcts). All of these cases had a benign outcome and death was due to another cause. Six of these were recent infarctions. None had evidence of swelling and tonsillar herniation. Infarcts were generally of small size and involved the entire PICA territory in only 2 cases. Most of these cases were unexpected discovered at autopsy. Cerebellar infarction in the territory of the medial branch of the PICA (9/17 infarcts) drew grossly a set square with a dorsal base and a ventral top headed for the IVth ventricle. Five out of these cases were associated with infarction in the dorsal and lateral medullary territories. Retrospective clinical study showed that they had been unnoticed or overshadowed by other neurological disorders (4 cases), or presented as Wallenberg's syndromes (4 cases), or as a pure vestibular syndrome (due to an infarction involving only the cerebellum) mimicking an acute labyrinthine disorder (1 case). Infarctions in the territory of the lateral branch of the PICA (5/17 infarcts) always occurred without medullary involvement. All of them were unexpectedly discovered at autopsy, and were unnoticed during the life (3 infarcts) or were overshadowed by other neurological disorders (2 infarcts). That was also the case in 2 cases of infarction in the whole PICA territory (3/17 infarcts). Thus infarctions strictly localized to the entire PICA territory only were rare. Thirteen/28 cases (46 p. 100) of infarction in the whole PICA territory were associated with infarction in the AICA and/or the SCA territories. This resulted from an association with other infarctions and not from an abnormally large territory of the PICA. Cerebellar swelling with brain stem compression and tonsillar herniation occurred 8/13 cases (62 p. 100). There were other massive median and paramedian brain stem infarctions involving midbrain, pons or medulla in 55 p. 100 of 13 cases.(ABSTRACT TRUNCATED AT 400 WORDS)

A 56-year-old man had radiation necrosis of the optic pathways, hypothalamus and brainstem following irradiation of a pituitary adenoma at a conventional dose. Factors which predispose to this complication are discussed. Vascular risk factors appear to facilitate radiation induced necrosis, and a reduction of doses is suggested in these cases.

The myelinotoxicity of sera from multiple sclerosis (MS) patients was determined by the assessment of visible myelin damage in guinea-pig spinal cord-spinal ganglia and in rat cerebella cultivated on collagen-coated coverslips in Leighton tubes. No change was seen in 22 cases. These data show that serum myelinotoxicity is low in MS - as compared with that of Experimental Allergic Encephalomyelitis. It appears a little specific phenomenon, the mechanism of which remains unclear. It can be easily assessed only in very sensitive culture techniques and is best measured by biochemical methods. This does not preclude the pathophysiological significance of the serum myelinotoxicity in MS. Supernatants of cerebro-spinal fluid (CSF) cell cultures in 10 MS patients caused no demyelination. Pooled concentrated supernatants of CSF cell cultures from 13 to 20 MS patients--containing from 7.3 to 11.7 micrograms IgG/ml gave no patent in vitro demyelination in 3 different experiments. The released products of CSF cell cultures in MS are not very toxic for myelin. However these experiments have to be repeated with more sensitive culture techniques, biochemical assay of myelinotoxicity and more concentrated CSF cultures supernatants.

Increasing evidence of damage to the Blood-Brain Barrier (BBB) in Multiple Sclerosis (MS) has been reported. However, little is known of the significance of this damage. Is it relevant to the pathogenesis of the disease ? Or is it, on the contrary, a secondary phenomenon ?

In 70 pathologically proven MS cases, we found 16 cases with onset after 45. Sex ratio (3/1) was the same in the late onset cases and early onset control group. There was no positive family history of MS. The diagnosis was more frequently never considered in late onset cases (3/16) than in control cases (3/54). Mean duration of the disease was 8.6 +/- 6 years (8.1 +/- 6 in female patients). This was significantly shorter (P less than 0.05) than in the control group. In addition, in remittent-progressive courses and in progressive courses, it appeared that the duration of the disease was shorter in late onset MS - although this was not statistically significant. Progressive courses (8 cases) and acute courses (4 cases) were more frequent (P less than 0.05) than in control cases. Initial signs and symptoms were not significantly different from those of control cases. The length of the evolution of the disease was higher in cases with vestibular-cerebellar onset. New signs and symptoms occurred at the same rate in the late onset cases as in the control cases. Although motor, sensory and bladder disturbances were more frequent in the late onset cases than in the control cases, this was not statistically significant. Amyotrophy was more frequent in late onset MS; on the contrary optic neuritis, vestibulo-cerebellar disturbances and nystagmus were less frequent in late onset MS than in the control group (P less than 0.05).

The Normarski interference-contrast microscope is well suited to observations of transverse thick sections of epon-embedded peripheral nerves. The quantitative study of myelinated fibers is possible on photomicrographs with a final magnification of X 500. The induced distorsion is very small as compared with the results obtained by conventional bright field and positive phase contrast optics. The comparative study of the same field with the three devices allows a better interpretation of marginal pictures.

Nerve and muscle biopsies were performed on 20 patients with HIV infection and peripheral neuropathy. Nine patients had distal symmetrical peripheral neuropathy (DSPN) (six ARC and three AIDS), six had inflammatory demyelinating polyneuropathy (IDP) (three ARC, one AIDS, and two otherwise asymptomatic patients), one had mononeuropathy multiplex (MM) (AIDS), 1 had mononeuropathy (ARC), one had meningoradiculitis (AIDS), and two had areflexia-associated lymphocytic meningitides (ARC), DSPN exhibited axonal degeneration in four of nine cases and was associated with segmental demyelination in five of nine cases. IDP exhibited segmental demyelination associated with axonal degeneration in four of six cases. Demyelination was more frequent in asymptomatic patients (2 of 2 cases) and in ARC (7 of 12 cases), whereas axonal degeneration was predominant in AIDS (6 of 6 cases). Mononuclear cell infiltration was seen in 1 of 2 asymptomatic patients and in 11 of 12 ARC patients but was exceptionally found in AIDS (1 of 6 cases). Involvement of the walls of small vessels, mostly venules ("subacute microvasculitis"), was found in 1 of 2 asymptomatic patients, in 8 of 12 ARC patients, and never in AIDS. The polyclonal mononuclear cell population was composed mainly of Leu 2 (T8) positive cells in seven cases of ARC. No virions were seen in electron microscopy. HIV was isolated in two cases from the CSF or the nerve biopsy.

Three cases of simple ameloblastoma were studied by electron microscopy. The classical ultrastructural features of these tumors are analysed and compared with reports in the literature and the development of the enamel organ. The authors stress the presence of two cell types (light and dark) probably corresponding to different degrees of maturity. One case of cystic ameloblastoma and one of granular cell ameloblastoma are then reported. The nature of the inclusions in this latter tumour is probably mixed: lysomal and secretory, perhaps ameloblastic. Finally, a malignant ameloblastoma is described under electron microscopy, for the first time. Closely related to simple ameloblastomas, it may be distinguished only by the presence of signs of cellular degeneration and numerous mitoses, sometimes abnormal.

During the past 15 years, increasing studies have been devoted to glial cells, i.e. oligodendrocytes, astrocytes and microglial cells of the central nervous system. Although most of their functions remain mysterious, the concept of intracellular interactions between neurons and neuroglia becomes more and more substantiated. This review describes some aspects of phylogeny of glia, recent technical approaches to the study of these cells, their major morphological and biochemical characteristics, their maturation as well as their pathology.

A prospective longitudinal study was undertaken in a geriatric hospital on women over 75 years of age, clinically diagnosed as either intellectually normal or having senile dementia of the Alzheimer type (SDAT) of varying degrees of severity. Mental impairment was assessed prospectively. Fifteen brains from this population were studied to evaluate quantitatively the distribution of senile plaques (SP) in relation to cortical lamination. SP density in four neocortical areas (first temporal gyrus; supramarginal gyrus calcarine area; precentral gyrus) was significantly correlated with the degree of mental impairment. SP distribution in the cortical layers was evaluated by an indirect method and appeared to be fairly constant from one case to another. Significantly higher SP densities were observed in layers II and III of the temporal and occipital samples, while minimal values were noted in layer I. Lower densities of SP were found in layers V and IV of the occipital and temporal lobes. These data suggest a selective vulnerability of some areas of cortical projections in SDAT.

The authors report a case of amiodaron-induced neuropathy in a seventy one years old man. First signs appeared seventeen months after the treatment was started with 400 mg/day for one year and continued with 200 mg/day. Examination on the 29th month disclosed a severe sensory and motor deficit of the limbs with distal predominancy. Motor nerve conduction velocity was strongly impaired without modification of distal latencies. Fundi were normal. The patient improved quickly after drug withdrawal. The authors review the rare similar cases reported in the literature and attempt to describe the clinical caracteristics of amiodaron neuropathy. Qualitative and quantitative light and electron microscopical studies of nerve, muscle and skin biopsies, including teased fibers preparations were performed and they disclosed a marked reduction of the number of myelinated fibers. Wallerian degeneration predominated (31 p. 100) other segmental demyalination (25 p. 100). Numerous polymorphous lipid-laden lysosomes were present in Schwann cells, fibrocytes, pericytes, endothelial and muscle cells. These previously undescribed morphological findings are similar to those present in perhexiline maleate intoxications. We believe amiodaron neuropathy is a new neuropathy with drug-induced lipidosis.

The association of Adie's syndrome and of neuropathy of chronic evolution is not a frequent occurrence; first made known by Togi in 1967, four cases have been described ever since. The authors report two new observations: one concerns a man showing a unilateral tonic pupil associated to sensory neuropathy, raised C.S.F. protein, rarefaction of myelinated fibres with schwannian proliferation, without amyloid deposit. The other is that of a woman affected with bilateral pupillotonia, motor neuropathy of the four limbs and generalized amylosis. If the first observation is comparable to the five cases reported, the second is more questionable: the absence of amyloid deposit on three nerve biopsies and at the ciliary ganglion level does not absolutely exclude the possibility of amyloid neuropathy.

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.

The pathological findings in four nerves and muscles and in one skin biopsies from four patients treated with perhexiline maleate for angina pectoris are reported. In every case, a muscular denervation atrophy and a decrease in the large diameter myelinated fibers were observed. Only one case showed a decrease of the total number of myelinated fibers, on quantitative studies. The electron microscopic study of each nerve displayed findings consistent with a predominant schwannian degeneration, associated with a few onion bulbs formations and, in two cases, with a mild wallerian degeneration. The most striking finding consisted in the presence of polymorphous membrane-bound inclusions reminding the morphology of lysosomal complex lipids. These structures were very abundant in Schwann cells, but they were seen also in fibrocytes, endothelial and pericytic cells. Similar inclusions were present in the single muscle and skin biopsies studied by electron microscopy. In the muscle, they were seen in muscular cells as well as in endothelial and pericytic cells. In the skin, similar inclusions were observed in endothelial, smooth muscle and sweat gland cells. These inclusions were difficult to identify in one micron thick sections, emphazing the need of ultrastructural study for diagnostic purposes.

A 73 year-old man experienced left monocular blindness and transient right hand clumsiness. A left carotid arteriogram was performed 4 days after admission. Immediately following arteriography, there was a right hemiparesia and dysphasia. After 24 hours, the abnormalities resolved. The patient was treated with heparin. During the following weeks, he became gradually drowsy and confused. Pseudo-bulbar palsy and astasia appeared after a fluctuating but progressive neurological course. The combination of systemic symptoms, high sedimentation rate, renal failure, livedo reticularis and purple toes suggested necrotizing angiitis. With corticosteroid treatment, there was a slight improvement of systemic symptoms. Cholesterol emboli were seen in both fundi. Cholesterol embolization was proved by identifying the biconcave cholesterol crystal clefts in muscle and skin biopsies. The subsequent course was marqued by continuous neurological deterioration. The patient became stuporous and died 7 months after admission. Despite the lack of central nervous system pathological study, the clinical picture was highly suggestive of cerebral cholesterol embolism. A few cases have been reported, with only eight well-documented clinical descriptions. Clinical signs and symptoms were closely similar to those of the present case. Anticoagulant therapy of cholesterol emboli has been unsuccessful. In the present case, the onset of embolization was temporally related to anticoagulation.

A simple and reliable method of sampling explants from the nucleus of the Locus Coeruleus of newborn mice and their cultivation in Leighton tubes is described. This technique reliably provides cultures available for experiments lasting, at least, ten days. The cells of the nucleus of the Locus Coeruleus and newly formed norepinephrine-containing fibers are demonstrated by fluorescence histochemistry together with pharmacological tests. Their morphological characteristics before and after fixation and staining are described. Electron microscopy confirmed the healthy condition of these cultures.

Various questions are asked to the neuropathologist when the diagnosis of malignant glial tumor is suspected: is there a tumor on the sample? is it a glial tumor? What is the degree of malignancy and the prognosis? Answers to be given are easier if the biopsy has been performed before any treatment than in case of suspicion of tumor recurrence. The limits of the neuropathological methods, as well classical as more up to date ones such as electron microscopy, immunohistochemistry, image analysis or tissue culture and those of other research methods such as biochemistry, are emphasized.

Cytosolic and/or nuclear 3H-R 5020 binding sites were detected in 6/6 samples of normal leptomeninges obtained at the time of operation in human adults. The levels of binding sites were higher in leptomeninges samples (range of values: 140-1780 fmol/gT) than in the corresponding intracranial tumors (one meningioma, two neurinomas, one metastasis) in 3 out of the 4 cases that were simultaneously studied. The 3H-R 5020 binding systems were characterized in 2 groups, A and B, of pooled samples representing respectively 4 and 17 samples of normal leptomeninges. The study of A (cytosolic and nuclear binding sites) and B (cytosolic binding sites) groups showed that binding systems had a limited capacity and a high affinity (Kd were respectively 2 X 10(-9) and 5.3 10(-10) M) as previously noted for the progestin receptor in human meningioma. Biochemical data and ultrastructural pictures obtained in the present study as well as in previous works, allow to consider the presence of the progestin receptor in normal leptomeninges.

Report of a case of Incontinentia pigmenti (IP) of the Bloch-Sulzberger type, with neurological features, in a 3 month-old deceased infant. The neuropathological examination revealed signs of a destructive process which had occurred in the perinatal period: ulegyria, cavities of white matter and patchy scar softening of the cerebellar cortex. In addition, there was a diffuse inflammatory process involving the pia-arachnoid and brain tissue: perivascular cuffs of lymphocytes, histiocytes and eosinophilic polymorphs, and mononuclear nodules. No prenatal malformations or vascular lesions were found. These findings are discussed in the light of both the relevant literature on neurological abnormalities in IP and the only already reported neuropathological examination by O'Doherty and Norman (1968). Two main hypotheses might account for the frequency of neurological disturbances in IP, 1. a peculiar sensitivity of the brain to anoxia and/or ischemia: 2. its involvement by the specific inflammatory process which occurs both in the skin as other tissues in this genetically determined disease (Perié et al., 1976).

The signs, symptoms and courses of 64 cases of "primary" haematoma of the basal ganglia have been retrospectively studied, and the main clinical data have been analyzed in relation to the location and extent of the bleeding. Onset with hemiplegia, accompanied or not by impaired consciousness, was more frequent with intermediate and lateral haematomas than with medial haematomas (p less than 0.001). Among patients with initial hemiplegia, impaired consciousness was more frequent with intermediate and medial haematomas than with lateral haematomas (p less than 0.005). At a later stage, patients with intermediate and lateral haematomas more often went into deep coma than those with medial haematomas (p less than 0.01). There were no statistically significant differences between the three anatomical groups with regard to frequency of other signs and symptoms or duration of the disease before death occurred.

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.

The mental status of 15 women over 75 years, either intellectually normal of affected by senile dementia of the Alzheimer type at various degrees of severity, was assessed prospectively by Blessed’s test score. The pattern and density of tau immunoreactivity were studied in temporal neocortex area 22, and compared with those of PHF immunoreactivity and with the density of amyloid revealed by thio-flavin S.

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.

Four cases of chloroquine neuropathy presented two points of particular interest: 1) these were all young women with strictly prophylactic doses of chloroquine (100 mg/24 h); two patients showed pronounced loss of weight; 2) signs of myogenic lesions were apparent in only one patient whereas clinical and electric signs of neurogenic involvement were observed in all 4 cases. Biopsy in 3 patients (muscle in one and muscle and nerve in two) failed to show characteristic vacuoles; in one case mild segmental demyelinization was present. A favorable course was obtained in all cases after discontinuation of chloroquine, with dramatic recovery from the motor deficit. Pharmacokinetic studies in 1 patient showed abnormally high chloroquine levels at initial assay with slow elimination after its discontinuation.

A clinico-pathological case of C. J. disease which occured in a 26 year old patient and evolved over 34 months is reported. Published cases of patients dead before 30 y.o. are briefly reviewed. The average length of evolution was 28 months. Concerning the french cases it appears that this average length was significantly longer when C. J. disease had started before 50 y.o., than when it had begun after 75 y.o. However the general survey of the published cases does not show any correlation between the starting age of the disease and the duration of evolution.

The nervous system may be affected at any stage in the course of HIV-1 infection. Acute or subacute inflammatory demyelinating polyradiculoneuropathies occur often early and improve spontaneously. Distal symmetrical axonal, predominantly sensory, painful polyneuropathies occur in the late stages of the disease. Microvasculitis is frequent in the early neuropathies. Spastic and ataxic paraparesis associated with vacuolar myelopathy are rare and probably not related only to HIV-1. Aseptic lymphocytic meningitis may occur as the presenting or sole manifestation of HIV-1 infection. The more frequent subacute encephalitis, probably directly related to HIV-1, is late in the course of the disease. Microscopic changes are suggestive but non-specific (microglial nodules, multinucleated giant cells). According to some authors, HIV-1 infection of the central nervous system macrophages may be early and latent until associated pathologies (opportunistic infections, lymphoma) trigger the replication of HIV-1 by infected macrophages.

An unexplained peripheral neuropathy was observed in five patients with positive serology for LAV/HTLV III. Three of them presented with polyneuropathy, one with chronic meningitis and oculomotor palsies, and one with a mononeuropathy. CSF was abnormal in 5/5, with elevated protein content (0.4-4 g/l) and abnormal cell count (29-65/mm3). Intrathecal production of LAV-specific IgG was demonstrated in 3/4 cases. Electromyographic examination showed reduced nerve conduction velocity in 4/5. Neuromuscular biopsy revealed microvasculitis with mononuclear cell infiltrates in 3/4 cases; characterization of these cells showed that they were predominantly non monoclonal T8 lymphocytes. Other symptoms of "AIDS-related complex" were present in all five patients. None had other causes of peripheral neuropathy. Thus, peripheral neuropathy can be the initial manifestation of LAV/HTLV III infection. Isolation of the virus from the nerve in one published case, and arguments for intrathecal synthesis of LAV-specific IgG suggest the direct role of this agent; however, the lymphocytic infiltration seen in three of our cases favours an indirect immune mechanism, as in other organs, such as lungs and lymph nodes.

Sixteen cases of anoxic encephalopathy in adult patients admitted to the intensive Care Department of Raymond Poincaré Hospital (Garches) have been selected on the following criteria: resuscitation by external cardiac massage for cardio-respiratory failure, without any previous history of anoxia and with detailed data on the subsequent evolution of neurological signs and symptoms. Main clinical features were compared to the results of the neuropathological examination. The pattern and the distribution of changes are detailed. The cerebral cortex was always involved by diffuse ischemic cell changes or by laminar necrosis. The Sommer's sector of Ammon's horn was affected in 15 cases, the basal ganglia in 14, the cerebellum in 11, the brain stem in 6. The very rarely mentionned necrotic brain stem changes, which involved inferior colliculi, periaqueductal gray matter and substantia nigra were sometimes associated with lesions of the floor of the IVth Ventricle and of the mamillary bodies. They were different from Wernicke's encephalopathy with could be dismissed on clinical and pathological grounds. No clinical signs or symptoms specific for this distribution of changes could be found by the retrospective study of the clinical files.

20 day-old rat thoracic dorsal root ganglia were grown for 48 hrs. in Iscove's medium supplemented with 8% fetal calf serum and 600 mg/100 ml glucose. Naftidrofuryl was added at 10(-6), 10(-7), 10(-8) and 10(-9) M concentrations to the culture medium. The 10(-7) and 10(-8) M concentrations induced a statistically significant increase of the number (30 to 40%; p = .0054 and .0016, respectively) and length (20 to 30%; p = .0012 and .001, respectively) of neurites of the outgrowth measured after Bodian's protargol impregnation. The width of the cell spread in the outgrowth was also enlarged at the 10(-7) and 10(-8) M concentrations (18 to 26%; p = .0012; p less than .0001, respectively).

A 45-year-old male received split course multiple daily fractionated radiotherapy for an anaplastic left parieto-temporal astrocytoma. A total dose of 60 grays was delivered by 15 fractions of 2 grays given in 5 days and repeated after a rest period of 15 days. Forty grays were delivered to the whole brain and 20 grays to the tumor area. No surgery or radiosensitizer or chemotherapy were used at any time. The patient noted progressive loss of vision in both eyes and became blind 9 months after irradiation. Post-mortem study revealed 2 areas of radionecrosis in the chiasma and corpus callosum, corresponding to the dose of 57-60 grays. The necrotic area were at a distance of the tumor remnants. The high Daily Dose Multifractionation schedule may be responsible for the radionecrosis.

The authors present the therapeutical results of two clinical and histological homogeneous series of malignant gliomas. The first group includes 46 patients operated upon and treated successively by chemotherapy associating VM26 and CCNU, a conventional delayed radiotherapy 6 months after surgery and again chemotherapy. In this group, the mean survival is 17 months and 46% of patients were surviving at 18 months. The second group includes 28 patients operated upon and treated with the same associated chemotherapy without radiation therapy. The mean survival was 11 months and 24% of patients were surviving at 18 months. The comparison of results shows that after surgery, the association of a chemotherapy and delayed radiotherapy is more efficient than chemotherapy alone. However, the systematic study of nervous system tolerance to irradiation post-chemotherapy suggests that, in some cases, the chemotherapy is able of increasing the pathogenic effect of irradiation on the brain.

Two anatomo-clinical cases of a necrotic form of demyelinating disease are reported. The disease occurred in two women, had a late onset (patient were about 50 years old) and had a relapsing-remitting course during more than 10 years. The CSF displayed a high protein level over 125 mg/100 ml whereas the gamma-globulin level was normal. The anatomical study found symmetrical cavitations involving both hemispheres and optic tracts with clear-cut limits. Axons and myelin were both destroyed, only the vascular network being partially spared. At the lesion's border-line mononuclear cell infiltrates as well as some phagocytes with sudanophilic inclusions were found. The scarcity of the compound granular corpuscules suggest an old pathological process. A narrow zone of myelin-axonal dissociation was also observed. Astrocytic proliferations was unimportant. Blood vessels were normal. In one case plaques of multiple sclerosis were found in the spinal cord. Those two cases are unusual forms of a diffuse disseminated sclerosis: multiple sclerosis and Schilder's disease are considered as two anatomo-clinical variants of the same pathological process. The observed necrotic lesions are different from the acute necrotic forms of multiple sclerosis as the latter have rapidly developed. The long lasting course of the disease, over 10 years, allowed a complete resolution of the lesions explaining the cavitations. The late onset of the disease and the CSF high protein level are pointed out. The significant of the high protein level and normal gammaglobulin level in the CSF is discussed.

A 54 year old man without pathologic past history but mild hypertension, obesity and gastric ulcer, presented with a syndrome of Wallenberg. He had complained for five days of progressive and diffuse headache. The neurological condition improved initially, but the patient died suddenly two weeks later. Pathological examination showed no significant alteration except for left ventricular enlargement and mild arteriosclerosis. There was a hemodissection (dissecting aneurysm) of the left vertebral artery next to the inferior oliva. It induced a lateral infarct and a limited dorsal infarct at the middle third level of medulla oblongata. Although the location of the arterial changes is usual, their nature is exceptional. The cause of the arterial hemodissection could not be ascertained: fibrous arterial dysplasia, atherosclerosis or congenital abnormalities of internal elastic layer may be discussed. But no definite conclusion can be reached.

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.

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)