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We report a case of persistent anterograde amnesia secondary to an anterior thalamic infarct. A 49-year-old right-handed man is referred for acute anterograde amnesia. Diffusion-weighted imaging performed at 24 hours shows an acute punctiform infarct of the left anterior thalamus, while T2-weighted imaging reveals a contralateral and symmetrical ischemic sequelae in the right anterior thalamus. The two lesions are isolated and remarkably centered with the mamillothalamic tract. We suggest the symptoms are caused by the addition of the two lesions interrupting the mamillothalamic tracts. This is the second clinico-pathological observation of a persistent amnestic syndrome secondary to a bilateral lesion of the mamillothalamic tract.

Vertebrovertebral Arteriovenous Fistula (V.V.A.V.F.) is a relatively rare entity. It may be an incidental finding or be detected in patients presenting with pulsatile tinnitus, cervical bruit, or vertebro-basilar insufficiency. It can be spontaneous but it most frequently is post-traumatic in etiology. The authors report 8 patients, 4 women and 4 men aged between 20 to 77 years, with 4 post-traumatic V.V.A.V.F. and 4 spontaneous V.V.A.V.F. that were seen over a 15 year period. Imaging work-up included Doppler US (n=4), MRI 9n=3) and angiography (n=8). Seven of 8 patients were treated successfully using an endovascular technique (5 with balloon occlusion, 1 with coil embolization and 1 using a mechanical maneuver), without complication or recurrence, except in one case. We compare our results with published reports from the literature and review the underlying pathology and management strategies of V.V.A.V.F.

Purpose: To report four cases of patient with an acute ischemic event as a presenting symptom of a berry aneurysm., Patients and Methods: One male and three female (aged range 38 to 65 years) patients were admitted for acute stroke. The neuroradiologic finding disclosed aneurysm thrombosis, inferior to twenty five millimetres in three cases. Lumbar puncture was done in one case and showed subarachnoid haemorrage., Discussion: We will discuss the hypothesis leading to the mechanism of aneurysm thrombosis. Two theories will be presented: "hemodynamic" and "parietal" modifications. We will propose a management protocol for these patients with atypical presentation of intracranial aneurysms given the potential risk of rupture., Conclusion: The natural history of intracranial aneurysms is still not fully understood. Nevertheless, aneurym thrombosis may occur and lead to ischemic stroke.

Introduction: mycotic intracranial aneurysms are a rare complication of infectious endocarditis. We report four cases of patients with endocarditis, complicated by an acute stroke, revealing a mycotic intracranial aneurysm., Patients and Methods: four men (aged range 24 to 63 year old) were admitted for endocarditis, complicated by ischemic stroke in two cases and hemorrhagic stroke in the two other cases, including one with subarachnoid hemorrhage. Neuroimaging disclosed a mycotic cerebral aneurysm in all four cases., Discussion: we will discuss the natural history and the management of mycotic intracranial aneurysm based on a review of the literature and our experience. Three therapeutic options are possible: medical treatment, surgery and endovascular embolisation., Conclusion: management of mycotic intracranial aneurysms is still controversial, frequently requiring a multidisciplinary strategy with priority given to endovascular interventions.

Magnetic resonance angiography (MRA) is a very valuable tool in the routine evaluation of patients with stroke syndrome. It provides powerful noninvasive imaging of the cervical and intracranial vessels allowing the detection and the diagnosis of vascular anomalies. MRA usefully supplements, during the same examination, the analysis by MRI of the cerebral parenchyma. We will describe the indications of the various techniques (MRA with and without injection of contrast media) and show the value, artifacts and limitations of MRA in atherosclerotic stenosis or occlusive disease and in arterial dissections. This noninvasive vascular assessment will depend on the initial therapeutic orientation. Within the framework of the hemorrhagic stroke, we will discuss the role and the interest of dynamic MR angiography in the tracking and control of intracranial aneurysms and also the contribution of this newer sequences with gadolinium injection in the detection of cerebral vascular malformations.

Purpose: to describe the magnetic resonance imaging features of hypertrophic olivary degeneration (HOD) subsequent to lesion in the "Guillain-Mollaret triangle"., Materials and Methods: 15 cases of HOD were diagnosed at MR imaging in 12 patients (4 women and 8 men) with posterior fossa lesion. The time interval from the beginning of the disease to the MR examination was 3 weeks to 8 (1/2) years. Evaluation of the changes in signal intensity over time of the lesions was available in 6 patients., Results: Nine cases of HOD were unilateral and three were bilateral. HOD was associated to a lesion of the contralateral dentate nucleus in 8 cases, to a lesion of the ipsilateral central tegmental tract in 5 cases and to a lesion of the contralateral superior cerebellar peduncle in 2 cases. Hyperintensity and hypertrophy of the olive on proton density and T2-weighted images appeared as early as 3 weeks after the ictus. A curved central hyperintensity (CCH) could be observed 7 months after the initial presentation. Decrease in the signal intensity was observed after 3 (1/2) years and abnormal signal intensity could persist for up to 13 years. Palatal myoclonus at 5 months was associated with HOD in one case., Conclusion: HOD is considered a trans-synaptic degeneration subsequent to lesions in the Guillain-Mollaret triangle. Hyperintensity and hypertrophy of the olive are observed, sometimes with palatal myoclonus.

Over the last 25 years, advances in neuroimaging have significantly changed the evaluation and management of acute stroke syndromes. In the seventies, computed tomography (CT) could differentiate between ischemic and hemorrhagic stroke. Magnetic resonance imaging (MRI) is nowadays the imaging modality of choice in the initial assessment of acute stroke. MRI images can better discriminate acute, subacute and chronic infarcts, differentiate venous from arterial infarcts, detect arterial dissection, stenosis or occlusion. Diffusion-weighted images are highly sensitive and specific to acute infarction and the combination with perfusion technique is suitable to define potentially reversible ischemia (area of cerebral "mismatch" which is thought to represent the so-called ischemic penumbra). This penumbra is a potential therapeutic target of valuable interest for the treating physician.

Purpose: To point out the importance of both MRI and MR angiography for the diagnosis and follow-up of moyamoya disease., Materials and Methods: We report seven cases. MRI was performed in the initial phase in three cases. MR angiography without conventional angiography led to the diagnosis in one case. Conventional angiography was performed in six cases. Follow-up MRI and MR angiography were obtained in two., Results: The initial MRI revealed ischemic lesions in the sylvian territory in two cases, and a cerebral malformation in one, after-effect lesions in two, and cerebral atrophy and ventricular dilatation in one. Conventional angiography provided the diagnosis of moyamoya disease in all cases, associated with cerebral arteriovenous malformation in two., Conclusion: MRI and MRA should be performed for the diagnosis and follow-up of moyamoya disease.

Material-Methods: Thirty three patients (16 men, 17 women), mean age 45 years underwent an intravascular carotid balloon occlusion for intracavernous aneurysms, carotid-cavernous fistulas or cervical carotid injuries by Fogarty balloons (5 patients before 1982) or detachable balloons (28 patients). Transient balloon test occlusion consisted in a clinical examination, an angiographic study for 32 patients with an electroencephalogram for 16 patients., Results: Early complications occurred for four patients by veineous rupture (1 patient) or ischemic injury (3 patients). Late complications occurred for two patients by ischemic injuries, one asymptomatic., Discussion: These results are similar to the literature's results and the transient test occlusion consisting in clinical examination, angiographic study and EEG seems to be reliable compared to cerebral blood flow determination., Conclusion: Even if its indications have decreased because of technic improvements permitting carotid flow preservation, carotid endovascular balloon occlusion stays a reliable, cheap technic in carotid injuries' treatment.

Objective: Describe the MRI findings in capillary telangiectasias., Materials and Methods: Between 1996 and 1999, we observed 9 cases of capillary telangiectasia in 7 patients explored 5 times for posterior fossa symptoms. In two cases capillary telangiectasia was a fortuitous discovery. All patients were explored by MRI with T1 sequences with and without gadolinium injections, turbo spin echo T2 coupled in 5 cases with a double echo gradient echo T2 sequence (TR: 970 ms, TE: 15 and 35 ms). Two patients also underwent vertebral angiography., Results: The telangiectasia gave a low intensity signal on T1 sequences in 2 of the 9 cases and a discretely high intensity signal on T2 sequences in all cases. After gadolinium injection, 9 telangiectasias showed homogeneous or speckled enhancement. The echo-gradient T2 images showed a very low intensity signal in 7 out of 7 cases on the second echo. At the first echo, 4 capillary telangiectasias were undetectable. The two vertebral angiographies were normal and the follow-up MRI in 5 patients showed lesion stability., Conclusion: Pontile lesions with no mass effect showing enhancement after gadolinium injection and with or without a discrete T2 high intensity signal but with a frank echo-gradient T2 signal strongly suggest capillary telangiectasia.

We report a case of venous angioma revealed by seizure after a non hemorrhagic venous infarct. The initial neuroradiological work-up with CT scan, angiography and brain MRI evidenced the venous infarction with abnormal contrast uptake. The control MRI 3 months later showed clear involution of the venous infarct. Non-hemorrhagic venous infarction on venous angiomas are exceptional. Only 2 cases have been reported in the literature. Impaired venous drainage could be implicated.

Blood flow via an aberrant internal carotid artery within the tympanic cavity is a rare pathogenic embryonic variation causing pulsatile tintinus and a vascularized tympanum. CT-scan provides the diagnosis. MRI and 3D time-of-flight MRA appear ideal for exploring aberrant flow both for diagnostic purposes and to establish the vascular morphology.

Materials and Methods: From 1977 to 1996, 49 direct carotido-cavernous fistulae were studied among the sixty some cases diagnosed over these 20 years. Five were caused by spontaneous rupture of an intracavernous aneurysm and the others were caused by trauma., Results: The clinical presentation in 37 patients was exophthalmia with pulsating conjunctival hyperhemia and vascular murmur. Some cases had a neurological syndrome suggesting cavernous involvement. A bilateral presentation was observed in 2 cases. One patient had no ophthalmologic syndrome but had a vascular murmur. Prior to 1982, all patients were treated and cured by occlusion of the internal carotid after direct access via the neck using a 3 F Fogarty catheter. Since 1982, patients have been treated with the detachable balloon technique. The carotid was preserved in 16 cases. In one case, secondary thrombosis occurred due to major dissection. In one case, the size of the breach was too small for the balloon so a coil was used. In one other case, insertion of the guide wire and catheter was sufficient to occlude the fistula. There was one death during treatment due to fistula rupture and one partially regressive right hemiplegia which could not be explained. This patient also developed left hemiplegia two years later, again with no explaining cause. Cure was achieved in the other patients without sequellae., Discussion: Direct carotido-cavernous fistulae due to rupture of an aneurysm or trauma are uncommon. When flow through the breach is minimal, vascular treatment may not be necessary unless clinical signs appear since this type of fistula heals spontaneously. In other cases, an endovascular balloon procedure is indicated. There are few complications. Embolization with coils or other devices should only be used in selected cases when the breach is too small for the balloon.

In 37 patients suspected of having a stroke 71 carotid bifurcations were explored by MR-angiography and by digital angiography the reference technique. A 3D sequence was acquired with the time-of-flight technique, using a transmitter-receiver cranial coil, followed by a strictly receiver Helmoltz coil on a 1 Tesla magnet. Two examiners evaluated the carotid bifurcations and measured the degree of stenosis in terms of diameters, according to the north american symptomatic carotid endarterectomy trial (NASCET). Five classes were established: class 1: normal; class 2: 1 to 29%; class 3: 30 to 69%; class 4: 70 to 99% and class 5: thrombosis. The results obtained in the determination of classes were identical with both coils: the coefficient of correlation with straight angiography were 0.973 with the cranial coil and 0.966 with the Helmoltz coil. Five stenoses were overestimated and classified as Class 3 instead of Class 2. The five stenoses greater than 70% (Class 4) showed a signal-void area at their level, due to severe dephasing induced by turbulences. Finally, there was a false-negative image of occlusion: the high-intensity signal of the thrombus was mistaken for one of flow. The data of our study were in accordance with the excellent results obtained by several authors in the literature, which makes it possible for us to propose this type of examination as a novel mean of investigating bifurcations of carotid arteries. Provided a strict technique is applied, and in addition to carotid bifurcation the Willis' circle and the cerebral parenchyma are explored, MR-angiography can complete the results of Doppler-echo. Standard arteriography could then be reserved to surgical patients and to those with discordant results of MR-arteriography and Doppler echo systems.

Thirteen patients with dural fistula of the cavernous sinus were studied by angiography. Nine of them presented with ophthalmic symptoms (chemosis and oculomotor disorders caused by the fistula). In one patient the lesion was discovered by chance, and 2 other patients had consulted for a disabling tinnitus. Eight patients accepted to the treated by the endovascular route for embolization of the maxillary artery, using particles. Embolization was unilateral in 4 cases and bilateral in 4 other cases. All embolizations were followed by serial control angiography immediately performed. All subjects were seen again as out-patients at 3 month, and 5 of them accepted a control angiography. Three patients were then regarded as clinically and anatomically cured. Two patients with incomplete clinical and angiographic results had a second embolization which resulted in clinical and anatomical cure at a 4-month control examination. These 8 patients were re-examined clinically after one month of treatment and found to be symptomless. Only one complication (transient oedema of the face) was noted. Dural fistulae are lesions that are most probably acquired by alteration of the physiological dural arteriovenous shunts occurring soon after venous thrombosis. Their course is capricious, and they sometimes heal spontaneously. However, the cavernous sinus location with its repercussion on the eye usually requires treatment. This treatment is initially endovascular; surgery and multifascicular irradiation being reserved for failures. Particle embolization of maxillary arteries is a simple and efficient procedure which must be used initially. If it proves insufficient, embolization of other arterial feeders (but it is often more dangerous) or the venous route can be tried.

A 48-year-old man presented with impaired joint movement and buccofacial apraxia. The disease progressed for six years associating an akineto-hypertonic syndrome, marked anarthria, saliva incontinence, bi-opercular syndrome, bucco-facial apraxia, severe global gestual apraxia and a frontal syndrom. Oculo-motricity and gait were normal. Magnetic resonance imaging of the brain demonstrated fronto-parietal atrophy and HMPAO Tc99 tomography revealed hypoperfusion of the cortex clearly predominating in the left parietal region. These particular findings with predominantly intense joint involvement is similar to the clinical picture in corticobasal degeneration--subcortical signs (progressively uncontrollable hypertonia) together with cortical signs (severe gestual apraxia). The neuroradiological imaging and functional results also suggest a degenerative process.

This study concerned a series of 12 patients, 4 of whom had Von Hippel-Lindau disease. Six of these patients were explored by myelography, 6 by spinal cord angiography, 8 by CT scan with contrast injection and 12 by MRI, with gadolinium injection in 8. MRI proved to be the choice examination for the diagnosis of spinal cord tumor, but gadolinium injection was necessary since it made it possible to detect the tumoral bud and its intense enhancement. The absence of gadolinium injection led us to an erroneous initial diagnosis of syringomyelia in two patients and glioma in one. Sagittal sections made it easier to evaluate the tumoral extension in patients with evidence or suspicion of Von Hippel-Lindau disease. Arteriography was indicated, as it provided a preoperative map and diagnosed punctiform lesions.

The purpose of this study was to evaluate MR-Angiography (MRA) in comparison with digital angiography in the diagnosis, therapeutic indication and therapeutic follow-up of cerebral aneurysms. Out of 27 patients explored by angiography and MRA, 17 had aneurysm, 5 had an aneurysm excluded by a detachable balloon, and 5 without aneurysm were used as controls. MRA, performed by the paradoxical enhancement technique, required a 3 DFT acquisition with short TR and TE (40-8 ms) in gradient echo, with a 15 degrees flip angle. Analysis of angiograms and partitions was compared with that of angiography. Sixteen of the 17 aneurysms were found by a study of both angiograms and partitions. The result was doubtful in 1 patient. No false-negative result was recorded. Spasm was always detected, but the neck of the aneurysm was never sufficiently well defined. The quality of angiogram depends on the type of balloon used in the patients treated: only the balloon without metallic index did not induce an artefact. Thus, MRA seems to be useful in the detection of cerebral aneurysms, and the absence of artefact induced by detachable balloons without metallic index could allow a therapeutic follow-up. But for the moment, arteriography remains the reference for pretherapeutic evaluation.

Hemangioblastoma may arise in isolation ("sporadic" cases) or as a major manifestation of von Hippel-Lindau (VHL) disease, an autosomal dominant disorder with a prevalence of at least 1/36,000. In addition of central nervous system hemangioblastomas (cerebellum, spinal cord and retina), affected patients may develop renal cysts or carcinomas, pheochromocytomas and pancreatic cysts. A multidisciplinary group including neurosurgeons, geneticists, pathologists and clinicians from all involved specialities has been organized to develop a national registration of all hemangioblastoma and VHL patients. The findings of a preliminary 10-year study (1983-1993) conducted in France are presented. Two hundred thirteen cases of hemangioblastoma were reviewed for their location and genetic features. The majority (77%) of the tumors were located in the cerebellum whereas 23% were located inside the spinal canal. By thorough clinical examination of the patients and systematic genetic inquiry of their family background, it was found that 34.3% of the total (58.7% before age 30) were afflicted with VHL disease. Spinal hemangioblastomas were more often related to VHL disease than infra-tentorial locations (50% versus 36.6%). In addition, mean age at diagnosis in VHL disease was significantly younger than in sporadic cases (33.5 +/- 10 versus 43.6 +/- 15 years). Recent progress in VHL molecular genetics led to the identification of the mutated gene to the distal part of the short arm of chromosome 3 (3p25-3p26), paving the way to presymptomatic diagnosis and, hopefully, to elucidation of pathogenesis, which may offer a further glimpse into tumorigenesis in general. Because of the usually early adulthood onset, accurate presymptomatic diagnosis of affected members would be of great benefit to VHL families. However, the fact that very few mutations in the VHL gene are identified precludes molecular diagnosis of "sporadic" hemangioblastomas. In summary, this study reveals that VHL-related hemangioblastoma is a more common clinical problem that it was previously reported. Thus, all patients with an apparently isolated central nervous system hemangioblastoma should be investigated for evidence of VHL disease.

The authors report a case of sacral pseudomeningocele discovered by chance in a 29-year old male patient presenting with Marfan's disease. In this disease, such abnormalities are common and due to excessive fragility. of the dura mater. CT and MRI provide an accurate morphological analysis and a complete evaluation of lumbosacral osteomeningeal abnormalities.

In a 39-year old woman hospitalized for intracranial hypertension MRI revealed the presence of abnormal areas in the cerebellar cortex. The negative results of investigations for possible causes, as well as the appearance of radiological abnormalities and their persistence over a 6-month period incited the authors to make a tentative diagnosis of Lhermitte-Duclos disease, despite the lack of anatomicopathological evidence.

A case of late radiation lesions of the brain was discovered 32 years after conventional X Ray therapy for pituitary adenoma. The major clinical picture was made of dementia and slight hypothalamic and pituitary dysfunction. Extensive bone-necrosis of the skull was observed. A defect of the sella led to recurrent meningitis. Segmental atheromatous lesions of cortical arteries are supposed to be induced by the photonic radiations. Intra parenchymatous lesions were slightly of the necrotic type and mainly of the degenerative one. Severe hyalin and calcic degeneration of small vessels was observed as well as extensive demyelination. The role of vascular and metabolic effects in the genesis of these late radiation lesions is discussed.

A new method is proposed for calculating the enlargement factor in order to measure left ventricular volume. It is based on the grid method of Kasser and Kennedy, modified and simplified by making use of the technical possibilities of their apparatus. The enlargement factor is measured from a graduated scale and reference to a pre-established graph.

Ischemia of the motoneurons in the anterior horn is a well known pathological entity. Their clinical signs and symptoms are similar to those of amyotrophic lateral sclerosis. Evidence by selective angiography of angiomas of the spinal cord or compression or deviation of Adamkiewicz artery may be suggestive of an initial vascular lesion. Various data (knowledge of development or lesions during experimental ischemia, selective electrophysiologic analysis of anterior horn neurons, evidence of precise circumstances of spinal vascular disorder or spinal arteriography) suggest that anterior horn ischemia is a multiple aspect phenomenon. Our 4 cases illustrate this hypothesis and demonstrate under confirmed vascular circumstances the different clinical aspects of anterior horn ischemic lesions. In addition to typical amyotrophic paralysis unusual or misleading symptoms may occur such as claudication, paroxysmal contractures or progressive spastic paraparesis. Investigations required and possible treatment of the lesions are simplified by awareness of these various clinical aspects.

A patient with Paget's disease developed an osteogenic sarcoma of the cranium. This emphasizes the need to conduct full neuroradiological investigations, including a CT scan and even selective arteriography as well as standard radiographs, whenever the possible existence of such a lesion is suspected, to determine therapeutic strategy.

The authors report one case of paralysis of the peroneal nerve due to an intraneural synovial cyst, in connection with the superior tibio-fibular joint. After an anatomic work and a complete revue of the literature, etiopathogenic and diagnostic problems seem to be resolved, and this justifies their terminologic choice.

Five patients were treated after a period varying between 6 weeks and 18 months following original injury. The five lesions were similar in that they could not be reduced by conventional orthopedic measures and that they were fixed during dynamic review examinations using a brilliance amplifier.

Based on their personal experience of the use of arteriography in 11 patients, the authors describe the angiographic appearances of both benign and malignant peripheral nerve tumours. They emphasise the reasons for errors, and the criteria enabling the precise nature of the lesion to be determined pre-operatively in the majority of cases. Other contributions supplied by angiography are discussed.

A peculiar case of neuro-sarcoidosis is reported. Clinical symptoms associated ocular dysfunction, hypopituitarism due to hypothalamic disorders, hypersomnia and alveolar hypoventilation. Dynamic tests have proved hypothalamic involvement. The patient died swiftly and suddenly. Neuropathological examination showed typical granuloma consistent with sarcoidosis mainly distributed to the area of the third ventricle and spread hyperplastic gliosis of pons and medulla.