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Whether aggressive treatment or no treatment is the optimal management for low-grade gliomas is controversial. However, symptomatic low-grade gliomas require prompt therapeutic intervention because of neurological impairment, uncontrolled seizures, and deterioration of life quality.We report the long-term follow-up, 71 months, of seven patients treated by procarbazine, lomustine and vincristine (PCV) therapy for a symptomatic low-grade oligodendrogliomatous tumor. The mean age at diagnosis was 47 years, the mean time from first symptoms to initiation of PCV therapy was 62 months (range 15-147).All patients initially responded favorably, with improvement of the neurological symptoms and radiological response. Chemotherapy was clinically well tolerated, the main side effect being low hematological toxicity. During the follow-up, no progression was observed in two patients. For the five remaining patients, the time to progression after the PCV induction was 56+/-12 months (range 38 to 73). Four of these patients showed favorable response to a second line of treatment.PCV therapy is an interesting therapeutic option for progressively symptomatic low-grade gliomas, even in cases with large tumoral volume. This treatment, of moderate toxicity, improves the quality of life and can result in long-term tumor stabilization.

The onset of post-anoxic encephalopathies can be delayed after the acute hypoxic injury.We present the case of a 45-year-old woman who achieved complete recovery from an episode of hypoxia related to a suicide attempt (ingestion of benzodiazepine). Three weeks later she developed a confusional state with akinetic mutism and parkinsonism. Brain CT-scan showed bilateral hemispheric white matter hypodensities. MRI showed extensive bilateral hyperintensities on T2-weighted and Flair sequences within the hemispheric white matter and the globus pallidus. EEG showed diffuse slow activity. All investigations for leukodystrophies were negative. Brain biopsy showed normal cortex and widespread demyelination with axonal sparing in the underlying white matter. The patient experienced a partial clinical recovery.The clinical course and the results of paraclinic investigations were consistent with the diagnosis of delayed post-anoxic leukoencephalopathy.

Headache of subacute onset, associated with other signs of increased intracranial pressure, represents the most frequent symptom of cerebral thrombophlebitis. We report a case of subarachnoid hemorrhage as a rare presentation of cerebral thrombophlebitis. Unfractionated intravenous heparin treatment resulted in resolution of subarachnoid hemorrhage on control brain CT scan and quick clinical recovery.

We describe a case of a 70-Year-old man who presented subacute pseudo-dementia due to a dural fistula. Neurological assessment and the reversibility of the symptoms after embolization support the originality of this observation.

Admission delay remains the main cause for stroke patient exclusion from urgent therapeutic protocols. Public lack of knowledge about stroke symptoms may result in delay in seeking medical care and late presentation at hospital. Lack of knowledge of risk factors for stroke may also hamper compliance with stroke prevention practices. The aim of this prospective study using a standardized questionnaire was to evaluate the stroke awareness of acute stroke patients in France. From July 2, 1998 to July 2, 1999, 166 consecutive stroke patients were admitted at our stroke unit. Among the 91 patients who were able to answer the questionnaire during the first 48 hours, only 19 patients (21 p.cent) thought they were having a stroke before their arrival at the hospital, 38 patients (42 p.cent) did not know a single sign of stroke and 33 patients (36 p.cent) did not know a single risk factor of stroke. The most common risk factors named by the patients were smoking and hypercholesterolemia (named by 31 patients (34 p.cent) and 19 patients (21 p.cent), respectively). The most common warning signs named by the patients were paralysis of one side of body or one limb and speech disturbance (named by 40 patients (44 p.cent) and 15 patients (16 p.cent), respectively). Female sex and "knowing somebody who had a stroke" were significantly associated with awareness of signs of stroke in multivariate analysis. Educational public programs regarding stroke awareness are needed in France. Educational campaigns must stress the risk factors and symptoms of stroke and the appropriate response in the hopes of reducing admission delay and improving stroke prevention.

Over the last decades, clinical evidence has accumulated indicating that the immune system may play an important role in some central nervous system diseases usually regarded as degenerative. The best known example is paraneoplastic cerebellar ataxia (PCA), which is thought to involve autoimmune cross-reaction between tumor and nervous system antigens. In the past twenty years, several antibodies directed against neuronal and tumor antigens have been described in association with PCA, leading to the description of different subtypes of PCA depending on the associated antibodies, the clinical course and the type of tumor. In some types, cerebellar ataxia occurs alone, whereas, in others, it is only one clinical feature in the setting of extensive nervous system disease. Circulating antibodies have also been described in patients with non-paraneoplastic cerebellar ataxia (N-PCA), suggesting that the immune system may be involved in certain cases of sporadic cerebellar ataxia. In this review, the clinical presentation of the different subtypes of potentially immune-mediated PCA and N-PCA will be described, and the experimental approaches developed in order to understand the pathogenic importance of the immune system in these ataxias will be discussed.

This report describes the observations of two patients with a several years' history of multiple sclerosis who presented sudden neurologic impairment. The symptomatology was suggestive of a non-convulsive partial status epilepsy. The clinical presentation was a paroxysmal dysphasic phenomenon in the first case without any consciousness impairment, associated with slight right hemiparesis. Electroencephalographic investigations revealed asymmetrical patterns, left-sided slow waves and periodic lateralized epileptiform discharges (PLEDs). Antiepileptic treatments were partially effective and intravenous steroids were needed for complete recovery. For the second patient, clinical presentation was acute psychiatric symptoms with disorientation, alternating manic symptomatology and mutism. Electroencephalography showed left fronto-central rhythmic continuous slow wave and spike wave activity. Intravenous antiepileptic treatment quickly improved the symptomatology. These observations draw attention to the fact that an epileptic cause should not be ruled out when a patient with multiple sclerosis presents sudden neurologic or psychiatric impairment. An early diagnosis allows immediate antiepileptic treatment. Intravenous steroids can be added to stop seizures.

A 78-year-old right handed man with a past history of atrial fibrillation developed in November 1994, a slight right hemiparesis with aphasia which cleared over one month. Head CT scan showed a left middle cerebral artery infarct involving the posterior part of the temporal lobe. In September 1995, a second stroke occurred. Head CT scan revealed a recent right middle cerebral artery infarct within the posterior part of temporal cortex. Auditory agnosia was diagnosed. Auditory evoked potentials recording showed bilateral dysfunction of central auditory pathways mainly over the right hemisphere. Clinical data and evoked potentials suggest that auditory agnosia might be related to the right temporal lobe damage. This later is involved in linguistic processes as suggested by positron emission tomography studies.

A 31 year old right handed woman presented with acute onset of aphasia which cleared over two days. CT-scan showed a left middle cerebral artery infarct within Wernicke area. Initial transesophageal two-dimensional echocardiography disclosed a mitral valve lesion suggesting a thrombus. She was discharged on oral anticoagulant treatment. A second stroke occurred ten months later involving left lenticulo-striate arteries area. Echocardiography remain unchanged. Subsequently, giant Lambl's excrescences of mitral valve was confirmed by operation and pathologic examination. The majority of patients with Lambl's excrescences are asymptomatic. However surface thrombus is common with this tumors which reposant a potential us for cerebral embolization. These tumors should be operated since complete excision is the only definitive means of eliminating the source of recurrent embolization.

Brainstem auditory evoked potentials (BAEPs) and middle-latency auditory evoked potentials (MLAEPs) have been recorded in 67 patients who had a stroke in well-defined territories of the vertebral and basilar arteries. Either CT scan or MRI have been performed in all cases. BAEPs were abnormal in 41/67 patients and MLAEPs were abnormal in 25/39 patients. BAEPs abnormalities were either bilateral (29/41 cases) or unilateral (12/41 cases). All components of BAEPS were unilaterally absent in four cases and bilaterally in one case. Pa component of MLAEPs was unilaterally delayed or reduced in five cases and bilaterally in 20 cases. Considering the topography of the infarct as shown by CT scan or MRI: medulla oblongata (13 cases): BAEPSs were normal in nine cases; pons (24 cases): BAEPs were abnormal in 16 cases; MLAEPs were abnormal in ten of 15 patients whose BAEPs were abnormal as well; mesencephalon (seven cases): BAEPs were abnormal in only two cases, and MLAEPs were abnormal in two cases one of which BAEPs were normal; in patients with diffuse infarctions either BAEPs or MLAEPs or both were abnormal in all cases. Stimulation of the ear ipsilateral to the lesion disclosed more BAEPs or MLAEPs abnormalities than stimulation of the contralateral ear.

We report a case with focal neurological deficits suggesting vertebro-basilar system ischemia, in the course of pre-eclampsia. An early CT scan showed a large hypodensity throughout the midbrain. Brainstem auditory evoked potentials initially showed an abolition of III and V pikes suggesting brainstem injury. Two days later both neurological examination and brain stem auditory evoked potentials returned to normal. A CT scan performed three weeks after the onset was normal. These findings suggest a vasospasm which may have been due to sympathomimetic agents given two weeks before the onset of toxemia for preterm labor.

A 78-year-old man had a midbrain hemorrhage, documented by CT scan and MRI, resulting in a mild cerebellar syndrome predominant on the left upper limb, lateropulsion, and a complex ocular motor syndrome. The latter was associated with right nuclear oculomotor nerve palsy, gaze paresis to the left for voluntary saccades, and a tonic ocular tilt reaction. Pathophysiological mechanisms are discussed.

A 59-year old man developed subacute tetraparesis following severe sudden neck pain. MRI showed a subdural cervical hematoma. Prothrombin complex activity was low. An unusual coagulopathy after rodenticides exposure was found. Diphenacoum, an effective antagonist of vitamin K1, was present in the patients plasma. Specific medical management led to a complete recovery. Follow-up MRI seventy days later confirmed the complete disappearance of the hematoma.

Two patients with autosomal dominant pure cortical cerebellar atrophy, belonging to the same family, exhibited imitation synkineses of hands and feet when the contralateral extremity was moved. The phenomenon was observed particularly when alternate movements of one hand were performed, but it also existed when flexion-extension movements of one foot took place. The induced synkinetic movements were mainly observed on the right side in one patient and exclusively observed on the right side in the other one. At electromyography, the imitation synkinesis took place about 200 milliseconds after the first inducing movement, but tended to be simultaneous with the following ones. Imitation synkinesis appeared to be shared by other cerebellar conditions: 8 cases of sporadic pure cerebellar atrophy and 2 cases of post-surgical injury of the anterior lobe vermis. In the 2 genetic cases, there was no pyramidal sign nor sensitive disturbance, the somesthesic evoked potentials being normal. Thus, the imitation synkinesis was considered as having a cerebellar origin. The cerebellar imitation synkinesis might be provoked by the lack of a physiologic cerebellar inhibition located in the paleo- and/or neo-cerebellum. The predominance of imitation synkineses on the right side suggests that cerebellar inhibition is stronger for the dominant side, in order to liberate it from archaic synkineses.

A retrospective study was conducted on sixteen cases of encephalitis or acute amnesic encephalopathies, only one of which was confirmed by pathological examination, seen over the last fifteen years. The amnesic syndrome was always associated with a very marked anterograde deficiency and the absence of severe disturbances of other higher functions. Its onset was always acute, was secondary to disturbances of consciousness or associated with epilepsy, and in half of the cases presented as a severe infections disorder with high fever. The mood and behavioural disorders, and the temporal epilepsy, were evidenced clinically as a pure or predominant hippocampic lesion. The diversity of the progressive nature of the disorders, and the etiological environmental factors, enable divison into three groups : a group of six cases of postencephalitic Korsakoff's syndrome, probably of herpetic origin, and associated with a stable amnesic syndrome which remained as a sequela ; a group of 5 patients with non-herpetic but probable viral "curable amnesic encephalitis" ; another group of 5 patients with "acute amnesic encephalopathy" with various etiologies and with obvious encephalitic lesions.

A quantitative evaluation of cerebellar ataxia, with an ataxia score (total, static, kinetic) and the measurement of objective values related to the major symptoms, is proposed. 21 patients with heredo-ataxias were treated for 12 months with high doses (16 mg/kg/day) of D-L-5-HTP, L-5-HTP or the association D-L-5-HTP (16 mg/kg/day)-Benserazide(6 mg/kg/day). A computerized processing of the data obtained by regular examination was performed. The ataxia showed a significant regression at the 12th month, mainly in the static performances and in the speed of speech. L-5-HTP appeared to be more active than D-L-5-HTP. The regression of the cerebellar ataxia was also observed in non degenerative conditions such as multiple sclerosis and surgical injury of the anterior lobe vermis, showing that 5-HTP was active on the cerebellar syndrome in general. The regression of the cerebellar ataxia was very slow in inherited diseases and continued for 2 or 4 months after the treatment stopped. A serotoninergic cerebellar control of motricity is discussed.

Imitation synkineses had not been described in cerebellar ataxia and were not known in cerebellar experimental neurophysiology. We describe here imitation synkineses when alternate movements of foot or hand were performed, in 2 cases of pure genetic cerebellar ataxia. The phenomenon was observed in other types of cerebellar ataxia (8 cases of anterior lobe cortical atrophy, 2 cases of surgical injury of the vermis). More imitation synkineses occurred on the right side in right-handed subjects, suggesting that the cerebellum physiologically inhibits a contralateral transfer of the copy of the movement. This inhibition would be particularly effective on the dominant side.

18 patients with degenerative cerebellar ataxia were given for 12 months, high doses of D-L-5 Hydroxytryptophan (16 mg/kg/day) with a peripheral inhibitor of 5-HTP Decarboxylase. The ataxia was regularly evaluated with a semi-quantitative score and quantitative measurements, allowing a computerized processing of the data. A highly significant improvement of the static ataxia was observed at the 12th month, while adiadocokinesia, hypermetria and dysarthria were also influenced. These results could correspond to the presence of serotoninergic nerve terminals in the cerebellar cortex, the cerebellar deep nuclei and the inferior olivary complex. Moreover, the improvement of the ataxia continued for 3 months after treatment stopped, suggesting an effect distinct from the classical neuromediation phenomenon.

The authors present a retrospective study of 670 histologically verified tumours of the neuraxis collected over 90 months in the Neurological Hospital, Lyons. The main facts concerning histological type, topography of the tumour, date of diagnosis, address, profession, O.B.O. and Rhesus groups, age and sex were codified for feeding into a computer and processed by sophisticated statistical methods. This analysis shows the homogeneity of topographical distribution in the brain of the different types of glioma, the special genetic code of meningiomas (predominance of A and B genes of the A.B.O. group), absence of the same factors for glioblastoma. Sex ratio analysis shows the contrast between definitely masculine tumours--glioblastomas and astrocytomas--and meningiomas and spongioblastomas. Analysis of the "age specific rate" separates tumours prevalent in the young, where incidence is a decreasing function of age (spongioblastoma, medulloblastoma, cerebellar astrocytoma) and tumours prevalent in adults which are mainly meningiomas and glioblastomas whose incidence is an increasing exponential function of age. Epidemiological analysis shows the difference in geographical distribution of glioblastomas and meningiomas. As far as glioblastomas are concerned, it would appear that on to the general rule of constant tumourincidence over the area as a whole, is superimposed another of high incidence in particular districts of the Beaujolais and Maconnais regions. The aetiological implications of these various findings are discussed.

A case of pure graphic disorder without any disorder of speech, reading or praxis is described. The conditions under which it occurred after surgery for frontal oligodendroglioma would seem to confirm that a single frontal lesion is involve affecting in particular the foot of F2. This case is compared with similar ones which have appeared in the literature and are classified within the wider category of kinesthetic disorders caused by frontal lobe lesion.

Acute headaches are in most cases significant symptoms or premonitary signs of a neurological condition. From a semiological point of view, they may be: (i) isolated, (ii) associated with neurological symptoms (ophtalmoplegia, hemiplegia, hemianesthesia...). From an etiological point of view, the haemorragic conditions are predominant (30%): encephalic vascular malformation with or without subarachnoidal haemorragia (21%), subarachnoidal haemorragia without malformation (6%) and subdural haematoma (3%). Two types of conditions are also frequently observed: ischemic attacks (22,3%) and inflammatory meningeal syndromes (12%). Rare cases with hypophyseal adenomas, ischemic attacks under oestro-progestative treatment, accidents of mono-amine-oxydase inhibitors and multiple sclerosis are observed. 23,8% of the cases remained without any precise diagnosis. One of the interesting points in the acute headache issue is the possibility of discovering an encephalic vascular malformation without any important bleeding and, therefore, good conditions for surgery.

Twelve consecutive patients with a progressive form of multiple sclerosis were submitted to 6 sessions of plasma exchange as single therapy. Results were evaluated by Mac Alpine's, Kurtzke's, a simplified semi-quantitative neurologic validity scale and by determination of 4 totally objective quantitative parameters. A decrease in the disability score, according to Kurtzke, was noted in 4 patients, and comparison of scores of neurologic validity before and after plasma exchange showed a highly significant difference (p less than 0.0001). A significant effect was noted also for two of the quantitative parameters. The effect was predominant on pyramidal and cerebellar disorders. Improvement was rapid but transient and did not persist after 2 months. Theoretically, this improvement after plasma exchange implies the existence of circulating neuro-inhibitory factors and that multiple sclerosis is a disease not limited to the central nervous system. Indications for plasma exchange in the progressive form of multiple sclerosis are discussed.

Two patients with severe, repeated confusional episodes of apparent fluctuating dementia type, and electroencephalographic alterations (permanent, diffuse slow waves) on recordings, were found to have elevated parathormonemia and slightly raised or normal levels of calcemia. The only other sign of hyperparathyroidism was intermittent hypophosphoremia. Exploratory cervicotomy identified a parathyroid microadenoma in one case and a parathyroid adenoma in the other. Recovery of intellectual functions was slow but complete. The possible direct role of parathormone in the genesis of this parathyrotoxic encephalopathy is discussed.

We propose in the paper of the concept of nuclear hemodynamic vertebrobasilar insufficiency, defined as an oligemic blood flow lower than 35 ml/100 g/min. in the brain stem-cerebellum zone, when using the method of inhalation of 133Xe. In 15 patients, the neurophysiologic manifestations included intermittent symptoms. We describe here permanent neurophysiologic motor disturbances: extrapyramidal Dopa sensitive syndrome (2/15), chronic cerebellar ataxia (12/15), often associated with cerebellar atrophy (8/12). The concept of chronic oligemic cerebellar ataxia, corresponding to selective neuronal death and/or neurochemical failure, is proposed.

The action of VM 26 on cerebral glioblastomas was suggested by studies of phase II or protocols in which the drug was used in association. The randomised protocol presented here, involving 10 treated subjects and 11 controls, showed that VM 26 was active in terms of the duration of survival of patients undergoing surgery for a glioblastoma. The mean survival was 16.4 months as against 9.6 months in the controls (statistically significant difference at p less than 0.05). This activity would tend to characterise the drug as one of the essential factors in the chemotherapy of all malignant gliomas, in particular since it is itself free of all haematological complications, even at high doses, and causes scarcely any allergic problems.

Twenty-five cases of amyotrophic lateral sclerosis occurring before the age of 40, collected between 1963 and 1973 in the Neurological Hospital, Lyons, have prompted the authors to make the following observations. -The incidence of disease seems to have been on the increase during this period both in absolute terms and relatively speaking (relative to the total number of patients hospitalized and to the total number of patients hospitalized for amyotrophic lateral sclerosis after the age of forty). -The 25 cases involved mostly women--the opposite of what was found in cases of amyotrophic lateral sclerosis occurring after forty. -Only one was possibly familial. -The onset is mostly with purely pyramidal features or of the topographically circumscribed peripheral type. Development appears to take longer than the average for amyotrophic lateral sclerosis. It would be worth while confirming these findings by analysis of other similar groups.