[Cerebral angiitis caused by periarteritis nodosa]. / Périartérite noueuse révélée par une artérite cérébrale.

INTRODUCTION: We report a case of periarteritis nodosa revealed by a cerebral angiitis which recovered under treatment. OBSERVATION: A 52-year-old patient suddenly presented with a left sensory syndrome and a fluctuating aphasia due to ischemia involving both parietal lobes. The diagnosis of periarteritis nodosa was based on the following criteria: severe loss of weight, renal insufficiency, hypertension, angiography suggesting an arteritis. Instead of an ileo-cecal perforation, the patient recovered under corticosteroid and immunosuppressive therapy. CONCLUSIONS: Stroke in periarteritis nodosa may occur early be and associated with a good outcome.

[Lateral bulbar infarction due to intracranial dissection and subarachnoid haemorrhage]. / Infarctus latéro-bulbaire sur dissection intracrânienne suivi d'une hémorragie sous-arachnoïdienne.

INTRODUCTION: Intracranial vertebral artery dissecting aneurysms are a recognized cause of subarachnoid hemorrhage and the hemorrhagic recurrence risk after a first rupture of the dissecting aneurysm is high and of poor prognosis. However, when the dissection is discovered in a patient with vertebrobasilar territory ischemia, little is known about the risk of hemorrhagic rupture risk and there is no consensus on management. OBSERVATION: We report the case of a 49-year-old man who developed subarachnoid hemorrhage 48 hours after the occurrence of a latero-bulbar syndrome caused by a spontaneous dissection with occlusion of the right vertebral artery. The subsequent angiography showed a V4 dissecting aneurysm of the right vertebral artery which was treated by stenting and coiling without any complications. CONCLUSION: This case underlines the hemorrhagic risk of an intradural vertebral artery dissection and its possible progression to aneurysm. Subsequent angiographic imaging must be carefully examined to search for aneurysms which may require early specific treatment because of the high risk of recurrent bleeding.

[Spontaneous thrombosis of lesion-free carotid arteries: a retrospective analysis of eight patients]. / Thrombus endoluminal de l'artère carotide sans lésion sous-jacente. Etude rétrospective de 8 patients.

Strokes are rarely secondary to spontaneous carotid artery thrombosis. The objectives of this retrospective analysis were to define characteristic features and the clinical course. The study population included eight patients (6 females/2 males) seen at six university neurological centers. Age of onset was 46.5 years (range 38-52). Half of the patients had no vascular risk factor. Symptoms were TIA (n=1), strokes (n=7). Echotomography revealed intraluminal thrombus, with occlusion in 2 cases. Thrombi were found in common carotid artery (n=3), carotid bifurcation (n=2) and internal carotid artery (n=3). The thrombus was mobile in 4 cases. Seven patients were treated by anticoagulation therapy, one by surgery because of recurrent TIA. Further echotomographic exams revealed total resolution (3 cases) or decrease of the thrombus (3 cases). Occlusion was definitive in one patient. A cause was identified in six patients: acute leukemia (n=1), thrombocytopenic purpura (n=1), iron deficiency anemia (n=4).

[Pitfalls during ultrasonography investigation of the carotid arteries (author's transl)]. / Les pièges du Doppler carotidien.

The authors describe the principal pitfalls and sources of error encountered during the application and interpretation of continuous ultrasonography examinations of the carotid artery. These include poor examination technique, limitations of the ultrasonography examination, and incorrect interpretation of results. The effects of these errors in relation to each segment of the carotid axis are discussed, and the importance of comparing the results obtained with those of palpation and auscultation of the cervical region is emphasized. The greatest difficulty is the certain recognition of a pathological signal from the internal carotid artery: acceleration, turbulence, absence of signal. This latter anomaly may be present both when there exists an occlusion of the internal carotid artery and a pseudo-occlusive stenosis of this artery. The difference is fundamental from the therapeutic point of view. Ultrasonography does not therefore replace arteriography but can assist the decision to undertake urgent or delayed operative therapy.

[Simultaneous use of ultrasound and pulsed Doppler with spectrum analysis of frequency in diseases of the cervical and cerebral vessels. Initial results]. / Apport simultané de l'échotomographie et du Doppler pulsé avec analyse spectrale de fréquence dans la pathologie des vaisseaux cervicaux et cérébraux. Premiers résultats.

Preliminary results of the use of the ultrasound Duplex Scanner are reported and correlated with angiographic findings. The main interest of this examination resides in its application in cases of atheromatous obstruction of cervical vessels to the brain: definition of type of obstruction: occlusion or stenosis; assessment of hemodynamic alterations: modification of frequency spectrum, of pulsed Doppler signal to 5 degrees as defined by Strandness as a function of extent of narrowing of arterial lumen. Pre-occlusive lesions may be difficult to distinguish from an occlusion. In contrast, minor anomalies: plaque producing little or no stenosis, calcifications, appear to be simpler to detect than by angiography. In intracranial vascular malformations: arteriovenous fistulas and particularly intracranial angiomas of a certain size, this examination provides data on the increase in diameter and acceleration of flow in vessels supplying the malformation. In cases of brain death, the ultrasound Duplex Scanner demonstrates absence or marked reduction in internal carotid artery flow, whereas flow is normal in the external carotid arteries in the absence of collapse. Confirmation of reliability of this non-invasive exploratory procedure requires further evidence of correlations.

[Transcranial Doppler ultrasonic examination in vertebro-basilar circulatory pathology]. / Le Doppler transcrânien en pathologie circulatoire vertébro-basilaire.

Vertebrobasilar ischemic disease cannot be adequately explored by echo-Doppler ultrasonic investigating procedures, since they do not permit the study of the intracranial arteries. The transcranial pulsed Doppler (2 MHZ) velocity detection method developed by R. Aaslid now offers such possibilities, although it has not yet been validated in this field. The authors relate their experience and methodological criteria (patient's position, deepness of access, flow direction, response to compression tests). They conducted 2 studies, one bearing on the detection of 14 cases of 75% stenosis of the superior segment, or of arteriographically-confirmed endocranial vertebral/basilar artery occlusion; the other one was concerned with assessing the basilar hemodynamic consequences of vertebral-subclavian steal syndromes. Out of 17 steal syndrome cases, of which 5 were symptomatic (4 pre-steal cases, 7 permanent, 6 intermittent), basilar flow determined spontaneously or following postischemic hyperemic test of the upper limb, was moderately changed in 30% of cases. Transcranial pulsed Doppler ultrasonic examination could be recommended in addition to the regular echo-Doppler velocity detection test, and thus contribute to exploring and monitoring transient or full-blown ischemic accidents of the vertebrobasilar circulation, in expectation of medical, surgical or angioplastic management.

[Long term survival after basilar artery occlusion. 4 cases]. / Survie prolongée après occlusion du tronc basilaire. 4 cas.

Four cases of basilar artery occlusion with a follow-up from 7 to 12 years are reported. The first patient, a 60 year old woman, had a proximal occlusion which was revealed by an acute brain stem ischemia. The second case was a 63 year old man with an aortic aneurysm who had a single episode of vertebro-basilar insufficiency. Cerebral angiography demonstrated a lower basilar artery occlusion. The third patient, a 60 year-old woman, had been operated from right carotid artery and left vertebral artery stenosis; 8 years later, without clinical manifestations, a left carotid artery stenosis and an occlusion of the lower part of the basilar artery were discovered. The evolution was eventless after a left carotid endarterectomy. The last case was a 60 year old man who had a lower basilar artery occlusion associated with a left internal carotid occlusion. There was a full recovery after a hemiplegic stroke. From our personal cases and the review of the literature, long term survival after basilar artery occlusion may occur in occlusions restricted to the lower or middle part of the basilar artery and with a good collateral supply from carotid and cerebellar arteries.

[POEMS syndrome disclosed by ophthalmological manifestation]. / Syndrome POEMS à révélation ophtalmologique.

POEMS syndrome is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. Papilledema is a common finding but ophthalmological complaints are rare. We report a case of POEMS syndrome in a 42-year old man which was revealed by oscillopsia and blurred vision. An osteosclerotic myeloma of the fifth lef rib was found, but there was no improvement after removal of the rib. The patient died two months later from pulmonary embolism.

[Benign acute ataxia in an adult with VZV infection]. / Ataxie aiguë bénigne au cours d'une varicelle de l'adulte.

In adults, neurological complications of VZV virus usually occur after herpes zoster infection in patients with AIDS. We report a case of acute and benign cerebellar ataxia after chickenpox in a patient without immunodeficiency.

[Benign multiple sclerosis with childhood onset]. / Sclérose en plaques bénigne à début infantile.

We report a case of multiple sclerosis which began at the age of 12 years. Clinical symptoms at onset were acute, regressive cerebellovestibular ataxia and optic neuritis. Twenty-four years later vertigo, motor and sensory deficit of the right lower limb and grand mal seizures developed. CSF and MRI were suggestive of multiple sclerosis. The patient is now free of neurological symptoms with an 8 years' follow-up.

[Recurrent acute polyneuropathy and cutaneous vasculitis]. / Polyneuropathie aiguë récidivante et vasculite cutanée.

A patient with relapsing cutaneous vasculitis presented 3 times in 10 years with an acute extensive neuropathy and respiratory failure which fully recovered. The relationships between Guillain-Barré syndrome and vasculitis are discussed.

[Cerebrovascular complications and hereditary protein S deficiency: 2 cases]. / Accidents vasculaires cérébraux et déficit héréditaire en protéine S: 2 cas.

Hereditary protein S deficiency (HSPD) is a predisposing factor to recurrent venous thrombosis but is not currently associated with stroke. We report two cases of HSPD revealed by stroke in young adults. The first one was a 36-year-old patient whith a pure motor hemiplegia, who gradually recovered without sequelae. Total and free protein S was decreased (55 and 10%). One of his brothers died from pulmonary embolism at 20 years and a sister had low protein S level without clinical signs. The second case was a 26-year-old patient who had a right hemiplegia with aphasia due to an infarction in middle cerebral artery area. He partially recovered, but the course of the illness was complicated by deep venous thrombosis of the lower limbs and pulmonary embolism. Total and free serum protein S level was severely decreased (25 and 0%). The patient's mother and one of his sisters also had low protein S but never had clinical complications. In both case, dupplex scanning, transcranial doppler, echocardiography, serum antithrombin III and protein C were normal. Cigarette smoking was the only risk factor for arterial disease. These two cases suggest that HSPD must be investigated in young patients with stroke, even in cases of lacunar stroke.

[Emergency disobliteration of the carotid prepolygonal segment. Personal experience of 76 cases]. / La désobstruction d'urgence de l'axe carotidien dans son segment pré-polygonal. Expérience personnelle à partir de 76 observations.

The authors report 76 cases of carotid obstruction, mainly localized at the origin of the carotid artery, that have been operated upon in emergency. Two types of lesions are considered: occlusion and very tight stenosis. The principle of early surgery in cases of acute occlusion is very controverse. According to the facts reported (38 cases) results are rewarding when operative decision is taken under precise conditions judged as favorable: short lapse of time since occlusive onset, absence of intracranial hypertension, absence of comatose state and no sign of brain oedema. Some patients under go surgery in the priviledged condition of being already in a medico-surgical unit (post operative, post angiographic, embolic occlusions). Majority present the most common hemiplegic attack. However difficulty resides in the inefficient mode of transport to hospital and lack of highly specialized units on admission. A very tight stenosis (at extreme a pseudo-occlusive stenosis) with clinical recurrent deficit, modified Loppler's recording and hemodynamic repercussion at angiography is an operative emergency. When results of early surgery on 38 cases of stenosis are compared to those of late operation in 14 similar cases early decision becomes compulsory once tight stenosis of the carotid artery is identified and operative criteria respected. In the light of this report that "wait and see attitude" needs be somewhat revised when confronted to the dramatic ictal hemiplegia.

[Intracranial haemorrhage associated with phaeochromocytoma]. / Hémorragie intracrânienne et phéochromocytome.

INTRODUCTION: Pheochromocytoma is rarely disclosed by intracranial hemorrhage. We report two cases. OBSERVATION: The first 26-year-old patient developed subarachnoid hemorrhage due to a ruptured aneurysm of the middle cerebral artery. The second patient, aged 44 years, had a temporal hematoma. Diagnosis was suggested in both patients by hypertension and elevated urinary catecholamines and confirmed by imaging and MIBG scintigraphy. Adrenal gland tumors, on both glands in the first patient and on the right gland in the second were successfully removed; cranial hypertension totally regressed. Von Hippel Lindau disease was diagnosed by molecular genetics in the first patient. Paroxysmal hypertension could explain the brain hemorrhage in the first patient and may have favored aneurysmal rupture in the second. CONCLUSION: The relationships between pheochromocytoma and cerebral aneurysm are discussed.

[Facial palsy and central nervous system infection with varicella virus following adult chickenpox]. / Paralysie faciale et infection du système nerveux par le virus VZV après une varicelle de l'adulte.

INTRODUCTION: VZV virus-related peripheral neuropathies usually occur after shingles in adults and more rarely after chickenpox in childhood. CASE REPORT: A 54-year-old patient presented with a right VIIth nerve palsy following a chickenpox rash and recovered after antiviral treatment. CSF analysis revealed lymphocytic meningitis and the virus was identified by PCR. CONCLUSIONS: Although previous chickenpox was not found in the patient's past history, the probability of reinfection is likely. The virus can be assumed to affect the nervous system directly; the axonal or demyelinating mechanism of the neuropathy may be discussed.

[Ataxic sensory neuropathy and Lyme disease]. / Neuropathie sensitive ataxiante et maladie de Lyme.

INTRODUCTION: The clinical spectrum of peripheral neuropathies in Lyme disease is very wide. We report a case which was revealed by an ataxic sensory neuropathy. OBSERVATION: A 77-year-old patient presented with a subacute ataxic sensory neuropathy which occurred 2 weeks after a skin lesion involving the right lower limb. He fully recovered after specific antibiotic treatment. EMG was suggestive of a predominantly axonal neuropathy. Diagnosis of Lyme disease was assessed by progressive elevation of serum antibodies, demonstration of a lymphocytic meningitis and intrathecal synthesis of antibodies. CONCLUSION: Lyme disease may be added to the list of diseases which may induce subacute sensory neuropathies.

[Infarction of the pons, neurosyphilis and HIV infection]. / Infarctus pontique, syphilis nerveuse et infection par le VIH.

A 31-year old patient presented a right hemiparesis related to a left basis pontis infarction. Laboratory investigations revealed both neurosyphilis and HIV infection. The patient was treated with two courses of penicillin and recovered from hemiparesis. The occurrence of strokes due to syphilitic arteritis in AIDS is emphasized.

[Thalamo-peduncular germinoma]. / Germinome thalamo-pédonculaire.

A 24 year-old patient presented with chronic intracranial hypertension, palsy of both abducens nerves and upward gaze, and right hypoesthesia. CT scan and MRI showed a left thalamic tumor infiltrating the left cerebral peduncle. A stereotactic biopsy revealed a germ cell tumor. Following radiotherapy the patient recovered except for ophthalmoplegia. The tumor totally disappeared on CT scan and MRI.

[Vertebro-basilar aneurysms. 23 cases observed in a neurology department]. / Anévrysmes vertébro-basilaires. 23 cas observés en milieu neurologique.

Twenty-three cases of vertebro-basilar aneurysms observed in a neurology department are reported. Ten were located on the basilar artery (7 at the basilar bifurcation), 5 on the postero-inferior cerebellar arteries, 4 on the superior cerebellar arteries, 3 on the vertebro-basilar junction and 1 on the anteroinferior cerebellar artery. There were 6 fusiform and giant aneurysms, others were sacciform. In 5 cases, there was no clear relationship between the clinical data and the aneurysm, which was discovered by chance at angiography or MRI. One giant aneurysm of the basilar artery was mimicking a tumor of the posterior cerebral fossa. Two other cases were revealed by brain stem ischaemia. Aneurysmal rupture was present in 15 cases: there were signs of meningeal haemorrhage in all cases and clinical signs of posterior cerebral fossa involvement in 7 cases. CT scan, performed in 8 cases, showed in all of them a meningeal haemorrhage, with a brain stem haematoma in 3 cases. All the aneurysms were shown by angiography; 7 were associated with a vasospasm. Five patients were not operated upon; all died. In one case, clipping the aneurysm was impossible, but there was a good recovery with a 12 year follow-up. Nine patients were operated, 3 recovered completely, 2 had neurological sequelae, 1 a late worsening; 3 patients died of brain stem ischaemia.

[Acute polyradiculoneuritis after intravenous injection of heroin, HIV seropositivity disclosed 3 years later]. / Polyradiculonévrite aiguë après injection intra-veineuse d'héroine, séropositivite HIV constatée trois ans plus tard.

An acute polyradiculoneuritis with tetraplegia and respiratory failure was observed in a 27 year-old heroin addict 3 hours after an intravenous injection of heroin. Full recovery was obtained after respiratory assistance and plasma exchanges. The biological and morphological changes (sural nerve biopsy) suggested an immune mediated mechanism. Three years after the recovery, the patient was tested for HIV antibody and was seropositive. The role of HIV infection and of the injection of heroin are discussed.