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[Wallenberg syndrome: a review of 25 cases].

INTRODUCTION: Wallenberg's syndrome is one of the most common clinically recognized conditions due to brain stem infarct, which can nowadays be identified thanks to modern neuro-imaging techniques. We present a retrospective study of 25 cases admitted to our hospital in the past eight years, to evaluate epidemiological aspects and clinical findings and to correlate these with magnetic resonance topography. MATERIAL AND METHODS: Patients diagnosed in the Neurology Section of Hospital Xeral-Calde in Lugo between January 1989 and December 1997 as having Wallenberg's syndrome. RESULTS: There was a predominance of middle aged men presenting at 55.06 years of age (range 30- 78). Arterial hypertension was the main risk factor (52%). There was a progressive form of onset in most cases. The commonest symptom was dysphonia followed by dysphagia. The commonest finding on physical examination was ataxic gait (24 patients) MR was positive in 22 of the 23 cases in which this was done. There were different clinical findings depending on the site of the lesion, whether rostral, caudal or medial. This is considered in the discussion. In most cases the prognosis was good. The commonest sequel was ataxia. CONCLUSIONS: The results are similar to those in the literature. We emphasize the excellent correlation of clinical data and neuroimaging findings.

Adult↗

Basilar artery vasospasm and delayed posterior circulation ischemia after aneurysmal subarachnoid hemorrhage.

BACKGROUND AND PURPOSE: The clinical and hemodynamic impacts of basilar artery (BA) vasospasm (VS) after aneurysmal subarachnoid hemorrhage (SAH) are ill-defined. The purpose of the present study was to evaluate the relationship between BA-VS and regional cerebral blood flow (rCBF) with posterior circulation after aneurysmal SAH. METHODS: Daily transcranial Doppler (TCD) measurements of posterior and anterior circulation arteries were conducted in 162 patients with aneurysmal SAH. rCBF to the brain stem (BS) and other brain territories was assessed by multiple single-photon emission computed tomography with (99m)Tc ethyl cysteinate dimer single-photon emission computed tomography (ECD-SPECT) imaging during the course of VS. RESULTS: SPECT imaging showed delayed BS hypoperfusion in 29 patients (17.9%). Of them, 23 patients (79.3%) were found to have BA-VS. Patients with very high BA flow velocities (FVs; >115 cm/s) had a 50% chance of developing delayed BS ischemia. BA-VS was found at a higher rate in patients who experienced reduced rCBF in the cerebellum (56.3%), thalamic nuclei (68.4%), and occipital lobe (81.8%). Although patients with delayed BS hypoperfusion did not present with a higher clinical grade, their clinical outcome was significantly worse (Glasgow Outcome Score after 30 days 2.48+/-1.16 versus 3.3+/-1.27; P=0.001). CONCLUSIONS: These findings suggest for the first time that BA-VS after aneurysmal SAH is associated with hypoperfusion to BS and other posterior circulation territories. The risk for delayed BS ischemia increased significantly when TCD BA-FVs were >115 cm/s.

Adult↗

Brain stem venous congestion due to dural arteriovenous fistulas of the cavernous sinus.

BACKGROUND: Venous congestion of the brain stem due to dural arteriovenous fistulas (DAVFs) in the cavernous sinus is rare and presents therapeutic challenges. To assess the prognosis of patients with symptomatic DAVFs and brain stem dysfunction, we evaluated the degree of venous ischemia by examining pre- and post-treatment magnetic resonance images (MRI) in 2 patients presenting with venous congestion of the brain stem. METHODS: A 56-year-old woman with left hemiparesis and a 70-year-old woman with gait disturbance attributable to right cavernous sinus DAVFs were referred to our hospital. In both cases, T2-weighted magnetic resonance imaging (MRI) disclosed a hyperintensity lesion in the brainstem due to venous congestion. FINDINGS: Both patients underwent open surgery for direct embolization of the cavernous sinus because there were no approach routes for transvenous embolization. The patient whose pretreatment MRI demonstrated Gd enhancement continued to manifest neurological deficits and persistence of the abnormal hyperintensity on post-treatment T2-weighted MRI. In the other patient whose pretreatment MRI showed no Gd enhancement, treatment produced a complete response of her neurological deficit and disappearance of the abnormal hyperintensity area. CONCLUSIONS: We tentatively conclude that lesions corresponding to hyperintensity areas on non-Gd-enhanced, T2-weighted MRI may reflect a reversible condition whereas lesions identified as hyperintense areas on GD-enhanced T2-weighted MRI may be indicative of irreversibility.

Aged↗

Intracranial dissecting and saccular aneurysms in polycystic kidney disease.

A young man with polycystic kidney disease was seen initially with an brain-stem infarction. Postmortem examination disclosed a dissecting aneurysm of the basilar artery and a saccular aneurysm of the right vertebral artery. Dissecting intracranial aneurysms rarely are associated with saccular aneurysms and, to our knowledge, have not been reported in association with polycystic kidney disease.

Adult↗

Early brain-stem auditory evoked responses in vertebrobasilar transient ischemic attacks.

Brain-stem auditory evoked response (BAER) studies were performed one to 16 days after a vertebrobasilar transient ischemic attack (VB TIA) in eight patients and repeated two to 16 days later in six of them. Initially, all showed absence of waveforms, prolonged interpeak latencies, and/or amplitude reduction. Five of six patients showed reversal of BAER changes to normal; the remaining patient returned to near normal. Normalization occurred six to 24 days after the VB TIA. These results are different from those reported in other studies. Early sequential BAER studies may be helpful in differentiating VB TIA from brain-stem infarction and syndromes that mimic VB TIA.

Aged↗

Stenting for vertebrobasilar artery stenosis.

We report our experience with stenting for symptomatic vertebrobasilar artery stenosis. One hundred and sixteen patients with vertebrobasilar artery stenosis (101 vertebral ostial stenosis, 15 intracranial vertebrobasilar artery stenosis) were treated with stenting. Indication criteria of treatment were 1) symptomatic lesion, 2) angiographical stenosis more than 60%. Under local anesthesia, pre-dilatation was first performed, then stents were placed to the lesion. Successful dilatation was obtained in 115 cases. The stenosis rate reduced to 2% post-stenting in ostial lesions and 16% in intracranial lesions. Transient neurological complications developed in 2 patients. Follow-up angiographies more than 6 months after stenting were performed in 94 patients with ostial lesions and all patients with intracranial lesions. Of these, 8 patients (9.5%) with ostial lesions and 4 patients (27%) with intracranial lesions developed restenosis. All patients with restenosis were treated successfully with PTA (percutaneous transluminal angioplasty). During the follow-up period, 3 patients developed recurrence of VBI (vertebro-basilar insufficiency) symptoms due to restenosis. One patient developed brain stem infarction due to in-stent occlusion 8 months after stenting. Conclusion. Stenting for vertebrobasilar artery stenoses is feasible and safe. Prevention of restenosis, especially in intracranial arteries, is the next problem to be solved.

Aged↗

Palatal myoclonus associated with extremity tremor.

Palatal myoclonus associated with extremity movements such as myoclonus or tremor is uncommon and reports are rare. Five patients with palatal myoclonus and a rest tremor are presented. In four patients, a slow rest tremor (3 Hz or less) was present. The tremor persisted on sustained posture and finger-to-nose maneuvers and was usually not synchronous with the palatal movements. It was not associated with clinical manifestations of Parkinson's disease and occurred in conjunction with brain-stem infarction in three patients.

Adult↗

Duplicated origin of right vertebral artery with rudimentary and accessory left vertebral arteries.

We observed a rare cerebrovascular anomaly in a patient with brain-stem infarction. Two right vertebral arteries arose from the subclavian artery and communicated directly with each other under the transverse foramen of the fourth cervical vertebra. The left vertebral artery consisted of a rudimentary artery that arose from the left subclavian artery, ran through the transverse foramen of the sixth cervical vertebra and then tapered down to disappear at the fourth/fifth cervical vertebrae, plus a second, accessory artery that arose from a branch of the left thyrocervical trunk, ran through the transverse foramen of the fifth cervical vertebra and tapered off to disappear at the first/second cervical vertebrae.

Adult↗

Adverse events with continuous doxapram infusion against late postoperative hypoxaemia.

OBJECTIVE: A randomized double-blind controlled trial of doxapram versus placebo against late postoperative hypoxaemia was planned to include 40 patients (2 x 20). RESULTS: After inclusion of 18 patients a serious adverse event was encountered with development of a brain stem infarction in a 90-year-old woman receiving doxapram. At this point the randomization code was broken and we decided to terminate the trial. Three of nine patients receiving doxapram had had an adverse event whereas none of the patients receiving placebo had adverse events (P = 0.2). In the 18 patients studied, there was an insignificant trend towards higher mean oxygen saturation in the doxapram group, and a significantly higher minimum oxygen saturation and reduced number of hypoxaemic events on the first postoperative night. CONCLUSION: Although these preliminary data on the effect of doxapram on postoperative hypoxaemia seem promising, further studies on the effect of continuous nocturnal postoperative doxapram infusion on levels of arterial oxygen saturation should be postponed until more knowledge about the pharmacokinetics of doxapram in this particular clinical situation has been gathered.

Adult↗

Spontaneous dissection of the anterior inferior cerebellar artery.

A patient presented with the clinical features of a brain stem infarct. MRI confirmed changes of infarction but also revealed an unusual lesion in the left cerebellopontine angle cistern. The evolution of this lesion on MRI, together with angiographic changes, indicated a dissecting aneurysm of the left anterior inferior cerebellar artery, a previously unreported phenomenon. The presentation, causes and diagnosis of intracranial dissection are discussed.

Aortic Dissection↗

Preoperative embolization of cervical spine tumors.

PURPOSE: To assess the technical success rate, complications, and effect on intraoperative blood loss of preoperative transarterial embolization of cervical spine tumors. METHODS: A retrospective analysis was performed on 38 patients with tumors of the cervical spine; 69 vertebrae were affected. Polyvinyl alcohol particles, coils, gelfoam particles, either alone or in combination, were used for preoperative tumor embolization. After embolization a total of 57 corporectomies with titanium basket implantation were performed. RESULTS: In 36 of 38 patients, complete (n = 27) or partial (n = 9) embolization was achieved. In 23 patients one vertebral artery was completely occluded by coil placement, and in one patient the ipsilateral internal and external carotid arteries were occluded in addition. No neurological complications could be directly related to the embolization, but two postoperative brain stem infarctions occurred. The mean intraoperative blood loss was 2.4 L. CONCLUSION: Transarterial embolization of cervical spine tumors is a safe and effective procedure to facilitate extensive surgery.

Blood Loss, Surgical↗

Postpartum cerebellar infarction and haemolysis, elevated liver enzymes, low platelet (HELLP) syndrome.

Pregnancy is considered to be a hypercoagulable state per se with an increased risk for cerebrovascular events, however cerebellar infarction has been rarely described in pregnant women. A nulliparous pre-eclamptic woman at 25 weeks' gestation was submitted to an echocardiographic exam that showed an impaired cardiac structure and function. After 2 h, the patient underwent caesarean section for diagnosis of haemolysis, elevated liver enzymes, low platelet (HELLP) syndrome. Afterwards her platelet count raised, and eight days later she developed nystagmus, ataxia, dysmetria and motor deficit in the right limbs and sensory impairment in the right side of the face and in the left limbs. Cerebral magnetic resonance imaging (MRI) demonstrated a right cerebellar and median posterior bulbar infarction. Colour-coded sonography of cerebral vessels showed an occlusion of the right vertebral artery. Coagulation pattern analysis evidenced double heterozygosis of the methylenetetrahydrofolate reductase (MTHFR) gene and single mutation of the prothrombin gene. This case report gives evidence of the importance of considering the different risk factors involved in stroke occurrence during pregnancy.

Adult↗

Treatment of calcific aortic stenosis by balloon valvuloplasty.

Recent reports have established the feasibility of using balloon valvuloplasty to reduce left ventricular outflow tract obstruction due to a calcified aortic valve. The present study summarizes experiences with this technique in 9 patients (7 women, 2 men, mean age 78 years) in whom balloon valvuloplasty was used to treat calcific aortic stenosis. Peak aortic valve gradient (mm Hg) decreased from 68 +/- 8 (mean +/- standard error of the mean) before valvuloplasty to 35 +/- 5 after valvuloplasty (p = 0.003). Mean aortic valve gradient decreased from 57 +/- 7 before valvuloplasty to 30 +/- 5 after valvuloplasty (p = 0.006). Calculated aortic valve area increased from 0.42 +/- 0.04 to 0.81 +/- 0.06 cm2 (p = 0.005). Balloon valvuloplasty failed to diminish aortic valve obstruction in only 1 patient who, at subsequent surgery, had a congenitally bicuspid aortic valve. Significant aortic regurgitation was not observed in any of the 9 patients after valvuloplasty. One patient did have a highly focal, presumably embolic, brain stem infarct during the procedure. Femoral arterial blood loss, related to wire-guided exchange of balloon catheters too large for a 12Fr introducer sheath, was minimized by direct arterial exposure in 8 of the 9 patients. Thus, these findings confirm the efficacy of balloon valvuloplasty for the treatment of calcific aortic stenosis. The procedure, however, is not without hazard.

Aged↗

Proximal clipping and bypass between bilateral vertebral arteries using a radial arterial graft for the treatment of a dissecting aneurysm of the vertebral artery.

Proximal clipping has been performed recently as the main surgical treatment for a dissecting aneurysm of the vertebral artery. When there is a contralateral vertebral artery of a smaller size with arteriosclerotic changes, some form of bypass to prevent cerebellar and brain stem infarction is needed in addition to proximal clipping. We treated a 50-year-old man with a ruptured dissecting aneurysm of the left vertebral artery and stenosis at the V3 segment of the right vertebral artery. The caliber of the right vertebral artery was smaller than the left. After an anastomosis between bilateral vertebral arteries using a radial arterial graft, dissecting aneurysm was clipped at the proximal portion of the aneurysm.

Aortic Dissection↗

Increased angiotensin-converting enzyme activity in a patient with severe ovarian hyperstimulation syndrome.

OBJECTIVE: To assess plasma angiotensin-converting enzyme (ACE) activity in a patient with severe ovarian hyperstimulation syndrome (OHSS). DESIGN: Case report. SETTING: Private, university-affiliated infertility practice. PATIENT(S): A 35-year-old woman with OHSS. INTERVENTION(S): Clomiphene citrate induction of ovulation. MAIN OUTCOME MEASURE(S): Plasma ACE activity. RESULT(S): The patient had a brain stem infarction as a result of thrombosis caused by severe OHSS. Plasma ACE activity was significantly elevated and persisted long after resolution of the OHSS. CONCLUSION(S): Elevated ACE activity appears to have been associated with the development of OHSS in this patient. Further study of the ovarian renin-angiotensin system in the development of OHSS is warranted.

Adult↗

Classification and treatment of vertebral dissecting aneurysm.

BACKGROUND: For many years, dissecting aneurysms of the intracranial vertebral artery were believed to be quite rare. In recent years, because vascular disorders have been studied more thoroughly by three dimensional-computed tomography (3D-CT), angiographically and pathologically, these aneurysms are being reported with more frequency. METHODS: Among the 45 patients diagnosed to have aneurysms arising from the vertebral artery or its branches over a 20-year period, 16 had dissecting aneurysms. The authors present their therapeutic strategy for these patients. Surgery was performed in the 16 patients, the most common technique being clip-occlusion or trapping of the parent artery wherever feasible, in an attempt to optimize cerebral blood flow. The dissecting aneurysms of the vertebral artery were classified into two groups for the purpose of determining a therapeutic approach, namely unilateral and circumferential groups. In the unilateral group, the dissection seemed to involve only on one side of the vessel according to the conventional cerebral angiogram. These patients underwent surgical reconstruction of the vertebral artery by direct clipping. In the circumferential group, the dissection was all around the artery. Proximal clipping or trapping was performed in this group. RESULTS: In six out of eight patients with unilateral dissecting aneurysms, vascular reconstruction was possible by direct clipping. Of these six patients, the surgical outcome was considered excellent in four, fair in one, and one patient died of cardiac failure after 12 days as his preoperative morbid condition remained the same after surgery. Two other patients with unilateral dissecting aneurysms were treated with trapping technique and the surgical outcome was excellent in one patient and good in the other patient. Both patients resumed a normal social life. In five out of eight patients with circumferential dissecting aneurysms, trapping or proximal clipping was performed and the surgical outcome was excellent in two patients, good in one and fair in one patient. One patient with preoperative brain stem infarction died of aspiration pneumonitis after 8 months. Two patients who were noted to have an increase in the size of aneurysm during follow-up angiography underwent a craniotomy with clipping and wrapping of the aneurysm. There was a favorable surgical outcome in both patients. The remaining three patients had Grade IV subarachnoid hemorrhage (SAH) prior to surgery and at autopsy a disturbed vascular wall was detected. CONCLUSION: The authors' experience suggests that when surgically feasible, direct clipping is an effective alternative approach in the treatment of dissecting aneurysms of the vertebral artery in which blood flow in the parent artery is to be preserved.

Adult↗

Intracranial aneurysms with superior division paresis of the oculomotor nerve.

Five patients with intracranial aneurysms had unilateral ptosis, limitation of supraduction, and normal pupillary reactivity. The most common aneurysm location for superior division paresis of the oculomotor nerve was the superior cerebellar-posterior cerebral artery junction, where aneurysms (3) of the basilar artery compressed and flattened the interpeduncular oculomotor nerve from below. A superior cerebellar artery aneurysm and brain stem infarction affected the intra-axial course of the oculomotor nerve producing superior division paresis. Only an aneurysm of the intracavernous carotid artery resulted in superior division paresis at the expected anatomic site of bifurcation of the oculomotor nerve into its superior and inferior divisions. Because the superior division supplies innervation exclusively to the superior rectus muscle and levator palpebrae, an aneurysm involving this branch does not affect the pupillo-constrictor fibers. Consequently, cerebral angiography should be considered in the initial evaluation of this ocular motor deficit.

Adult↗

Basilar artery occlusion due to mucormycotic emboli, preceded by acute hydrocephalus.

A rare case of brain stem infarction caused by mucormycotic emboli, preceded by acute hydrocephalus, is reported. The patient, who had suffered from leukemia and had undergone bone marrow transplantation several months before, presented initially with seizure and persistent disturbance of consciousness. A head CT scan revealed marked ventricular dilation and diagnosed as acute hydrocephalus. The patient received emergent ventricular drainage. Despite the aggressive treatment, the patient did not survive. Autopsy revealed systemic mucormycosis occluding and invading various arteries including basilar artery and its branches, causing fatal brainstem infarction. Although early diagnosis remains difficult in the cases of systemic mucormycosis, prompt initiation of treatment is mandatory; one must have in mind the possibility of presence of fungal infection when treating patients with acute neurological deterioration who have underlying debilitating diseases, even though fungi themselves are hard to detect in most cases.

Acute Disease↗