[Vertebrobasilar manifestations revealing dissecting aneurysm of the aorta].
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Biomedical subjects
Publications and source records attributed to P Trouillas.
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Early CT scan showed a large hypodensity throughout midbrain. Brainstem auditory evoked potential showed initially an abolition of III and V pikes suggesting brainstem injury. Two days later neurologic examination and brain stem auditory evoked potential returned to normal. CT scan performed three weeks after the onset was normal. These finding suggest a vasospasm; in this case betasympathomimetic agents given two weeks before the onset of toxemia for preterm labor could lead to the vasospasm.
Vertebrobasilar insufficiency is a well-known syndrome, but no corresponding hemodynamic deficit has yet been established. We propose to define nuclear hemodynamic vertebrobasilar insufficiency on the basis of an oligemia lower than 35 mL/100 g per minute in the brain stem-cerebellar region with use of the xenon Xe 133 inhalation method. Fifteen patients fulfilling this criterion underwent four-vessel angiography, computed tomography, and a standardized neurologic examination. An acetazolamide test showed poor reactivity in more than half of the patients, sometimes specifically in the vertebrobasilar area. With use of single-photon emission computed tomography and intravenous technetium Tc 99m-labeled hexamethylpropyleneamineoxime in two cases, the considerable decrease of regional cerebral blood flow in the brain stem-cerebellar region was confirmed. An excellent correlation was observed between the existence of nuclear hemodynamic vertebrobasilar insufficiency and angiographically proved arterial occlusions. The dominant nuclear oligemic zone was regularly on the side of the anatomic arterial chief lesion. Clinical manifestations included rare transient ischemic attacks (in one of 15 patients), intermittent basilar symptoms (in 15 of 15 patients), and a subacute vertebrobasilar "threatening" syndrome. Thus, imaging of a nuclear hemodynamic vertebrobasilar deficit provides an objective basis to the diagnosis of vertebrobasilar insufficiency and useful objective data for revascularization surgery.
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.
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.
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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.
The authors report the case of a 57-year-old, right-handed female who experienced a sudden acute headache, followed by a period of confusion and fever. Over the next week she developed language disturbances. No diagnosis was established. Three weeks later she was referred to the neurologist, complaining of a right frontal headache. The C.T. scans with and without contrast enhancement were suggestive of an aneurysm in each sylvian fissure. The angiography disclosed the presence of four aneurysms: a large left M.C.A. aneurysm, a larger right M.C.A. aneurysm, an aneurysm on the anterior communicating aneurysm and a small right M.C.A. aneurysm located more distally. The left M.C.A. aneurysm was presumably ruptured and responsible for the initial symptoms. The right M.C.A. aneurysms was apparently unruptured, but accounted for the persistent right headache. The other two aneurysms were thought to be asymptomatic. The left M.C.A. was operated first. Blood in the sylvian fissure confirmed the earlier rupture. Two weeks later, the three other aneurysms were surgically treated, via the same right pterional approach. No sign of hemorrhage was found on the right side. The post-operative angiogram demonstrated the disappearance of all four aneurysms. Three months later, the patient was able to resume her previous occupations and clinically, she demonstrated no sign of psychological or intellectual disturbances. The management of multiple and bilateral cerebral aneurysms is discussed, as well as the management of asymptomatic aneurysms.
A 76 year-old hypertensive man developed an acute inability to stand due to a right cerebellar ataxia. Somatosensory performances were normal, but a transient and mild weakness of the right arm and leg with Babinski's sign was observed. There was a prominent asterixis of the right hand. CT scan showed a hemorrhage of the thalamus with surrounding edema of the adjacent internal capsule. Initial median nerve somatosensory evoked potentials showed a mild reduction of left parietal responses with absent left frontal SEPs (P22 and N30). Fourty days later the cerebellar ataxia was persisting while asterixis had disappeared. A second recording of SEPs showed a complete recovery of all cortical components. MRI performed at the same time showed a left postero lateral thalamic lesion. CT, MRI and SEPs findings suggested that asterixis could result from interruption of somatosensory fibres projecting to the motor cortex.
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.
A linkage analysis with chromosome 9 markers was performed in 33 families with Friedreich ataxia (FA). Linkage with D9S15, previously established by S. Chamberlain et al. (1988, Nature London 334:248-249) was confirmed in our sample (z(theta) = 6.82 at theta = 0.02) while INFB (interferon-beta gene) shows looser linkage. An additional marker, D9S5, was also shown to be closely linked to FA (z(theta) = 5.77 at theta = 0.00).
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.
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.
Three groups of 10 patients each with multiple sclerosis (MS) in a progressive phase were openly matched on the basis of age and invalidity (DSS Kurtzke). Variance analysis showed no significant difference between them for the main MS features. Group 1 received cyclophosphamide for 3 weeks (mean total dose: 152 mg/kg) with methylprednisolone (mean total dose: 2.77 g). Group 2 had a mean number of 9 plasma exchanges prior to a cyclophosphamide-methylprednisolone regimen similar to Group 1 (mean total dose of cyclophosphamide: 160 mg/kg and of methylprednisolone: 3.16 g). Group 3 was made up of controls. At three years, the proportion of stabilized and improved cases was 6/10 in group 1, 9/10 in Group 2 (statistically significant when compared with Group 1), and 0/10 in Group 3. The study of the variations of invalidity (DSS gains) showed a clear significant benefit in the treated groups when compared to controls, but no difference between the treated groups. Longitudinal studies showed that the mean therapeutic benefit was about 2.5 years. The role of cyclophosphamide and of plasma exchanges in these results is discussed.