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Intracranial dural arteriovenous fistula with venous reflux to the brainstem and spinal cord mimicking brainstem infarction--case report.

A 73-year-old man presented with a rare transverse sinus dural arteriovenous fistula (dAVF) with venous reflux to the brainstem and medulla manifesting as brainstem and spinal cord edema mimicking brainstem infarction. Complete occlusion of the fistula was achieved by transvenous embolization, resulting in angiographic cure of the fistula and progressive improvement of the symptoms. Intracranial dAVFs with perimedullary venous drainage, type V according to the Cognard classification, are rare lesions with distinctive clinical, radiological, and therapeutic aspects. This case demonstrates that the symptoms of dAVF with perimedullary venous reflux are variable, so dAVF should be considered in patients with clinical and radiological findings suggestive of congestion in the brainstem and spinal cord. Dysfunction of the medulla and spinal cord caused by venous hypertension is the most probable cause of the neurological symptoms in such cases. Interventional therapy can lead to angiographic cure and resolution of the symptoms.

Aged↗

[Dide-Botcazo syndrome associated with Anton's syndrome after a cardioembolic infarction in the distribution of the bilateral posterior cerebral arteries].

Dide-Botcazo syndrome (Rev Neurol, 1902) is a unique neuropsychological syndrome, characterized by combinations of cortical blindness, amnesia, and topographical disorientation. We report 82-year-old right-handed man manifesting such syndrome associated with Anton's syndrome after a cardioembolic infarction in the distribution of the bilateral posterior cerebral arteries. The MRI study demonstrated recent extensive infarctions bilaterally in the occipital lobes and the medial temporal lobes, and thalamus. Following the resolution of unconsciousness and tetraparesis, the patient persistently presented with denial of cortical blindness (Anton's syndrome), profound anterograde amnesia and retrograde amnesia of about 50 years, severe topographical disorientation, and partial impairment of the tactile and auditory naming for objects. The bilateral extensive damages to the visual area, the memory area, and the connecting areas including the occipital lobe of the non-dominant hemisphere possibly responsible for topographical disorientation, may account for producing Dide-Botcazo syndrome. The syndrome may clinically occur following the "top of the basilar" syndrome.

Aged↗

[Cerebrovascular accidents with familial antithrombin III deficiency--gene analysis study].

We report two cases of the cerebral infarction with familial antithrombin III type I deficiency. Case 1 is a 47-year-old woman presenting deep cerebral vein thrombosis. Case 2 is a 20-year-old man presenting the top of the basilar syndrome. Beside them, substantial number of members had cerebral infarction in this pedigree. We measured AT III activity and quantity for 18 persons in this pedigree. As a consequence, 6 of 18 persons in this pedigree had AT III type I deficiency. Analysis of PCR products of AT III gene derived from the case 1's leukocyte DNA revealed a novel frame shift insertion was demonstrated as mutant alleles with 4-base CTTT in nt 2,420 or nt 2,424 in exon 2 domain on the AT III gene. AT III type I deficiency caused by frame shift insertion has been reported only twelve pedigrees. This is the first case report of AT III type I deficiency caused by frame shift insertion in Japan.

Adult↗

Recovery in a patient with locked-in syndrome.

BACKGROUND AND PURPOSE: Sildenafil citrate has been shown to enhance neurogenesis, angiogenesis, synaptogenesis, and neurological outcome by augmentation of cyclic guanosine monophosphate (cGMP) levels in animal models of ischemic stroke. Whether sildenafil citrate may be helpful for recovery in human stroke is unknown at this time. METHODS: A 41-year-old woman with locked-in syndrome due to pontine infarction began receiving 150 mg of oral sildenafil citrate daily on a compassionate use basis in August 2003 and continues treatment at this time. Magneto-encephalography (MEG) was performed at 12 and 17 months after stroke. RESULTS: No serious adverse events have occurred. Significant milestone recoveries including standing, use of both arms, talking, and full return of swallowing have occurred, particularly after nine months of treatment. The MEG showed a significantly increased amplitude in the somatosensory cortex. CONCLUSION: Daily use of high dose sildenafil citrate appears to be safe in this patient with stroke resulting in locked-in syndrome. Further studies will be required to establish safety and efficacy.

Adult↗

Detection of acute brainstem infarction by using DWI/MRI.

Even using diffusion-weighted images (DWI) detection of acute brainstem infarction (BI) is still a challenge. To evaluate the clinical efficacy of a DWI protocol with improved spatial resolution all images of 44 patients with clinically possible BI on admission (24 patients with definite BI and 20 patients with other etiologies) and first DWI within 24 h after symptom onset were blindly reanalyzed for visibility and detection of BI on the first DWI by reviewers with different expertise levels. Neuroradiologists identified definite BI in 21 out of 24 patients (sensitivity 90%, specificity 100%); neurologists and junior house officers achieved similar results (sensitivity 86 and 83%, specificity 98 and 97%). The use of DWI allows a definite diagnosis of BI, even if raters have limited experience.

Adult↗

Peri-operative brainstem infarct in a patient with antiphospholipid antibody (APLA) syndrome.

Antiphospholipid antibody (APLA) syndrome is a major risk factor for arterial and venous thrombosis. Surgical risks in patients known to suffer from APLA syndrome are usually related to haemorrhage or to thrombo-embolic events, such as deep venous thrombosis and pulmonary emboli. The rare published reports of patients with APLA syndrome undergoing neurosurgical procedures relate to haemostatic complication, with none alerting to the peri-operative risk of stroke in these patients. We present a case of a peri-operative stroke in a patient undergoing resection of a foramen magnum meningioma. We discuss the association of peri-operative stroke and APLA syndrome and emphasize the high risk it imposes for neurosurgical procedures in these patients. In addition, we suggest an anticoagulation treatment algorithm for APLA syndrome patients undergoing craniotomies.

Antiphospholipid Syndrome↗

[Correlation of clinical and magnetic resonance imaging findings in patients with brainstem infarction].

The aim of this study was the comparison of clinical and neurological findings in 30 patients presenting with ischemic brainstem lesions. These were localized in the midbrain in 4 cases, in the medulla in 12 cases and in the pons in 11 cases, while the remaining three patients demonstrated combined lesions. Symptoms were lesions of the pyramidal tract in 77% of cases, vertigo in 57% of cases, speech disturbances in 40% of cases and gait ataxia in 37% of cases. Cranial nerve lesions were evident in 87% of patients, while 33% of patients demonstrated a Horner syndrome. Brainstem lesions were diagnosed in 22 (73%) of patients on magnetic resonance imaging, while all 30 patients had clinical signs suggestive of brainstem lesions. We conclude that neuroradiological studies can provide helpful information regarding patients with brainstem lesions, but by no means replace exact neurological examination.

Adult↗

[Diagnostic angiography using multi-slice spiral CT in acute obstruction of the basilar artery].

OBJECTIVE: To evaluate the diagnostic efficiency of CT angiography in case of clinical signs of acute brainstem infarction for the therapeutic management of catheter-based local thrombolysis. MATERIAL AND METHODS: 3 patients (2 males, 1 female) suffering from an acute onset of brainstem symptoms and being suspicious of an occluded basilar artery were included into this report. 1 patient underwent selective vertebral arteriography. 2 patients were initially examined with CT angiography using a 4-row scanner and 100 ml intravenous contrast agent. RESULTS: In one patient, an occlusion of the basilar artery was excluded with catheter-based angiography. Subsequently, the patient was treated with systemic thrombolysis using r-tPA because of a thalamus infarction seen in MRI. 2 patients who have been initially examined with CT angiography presented with complete occlusions of the basilar arteries. These patients underwent r-tPA thrombolysis by means of superselective micro-catheter approaches of the vertebrobasilar vessels. CT angiography was very useful for determinating the occlusion length of the basilar artery pre-therapeutically, and in 1 case for ruling out an occluded vertrebral artery for catheterization. All patients recovered well under thrombolytic therapy applied systemically or selectively. CONCLUSION: Cerebral multi-slice CT angiography is a fast and save technique for detecting or ruling out an acute basilar artery occlusion. Thus, in cases of equivocal clinical signs CT angiography is recommended before the decision of thrombolytic therapy is made.

Adult↗

Clinical characteristics of patients with brainstem strokes admitted to a rehabilitation unit.

OBJECTIVE: To examine the clinical characteristics of patients with brainstem strokes admitted to a rehabilitation unit. DESIGN: Retrospective cohort. SETTING: Inpatient rehabilitation unit. PARTICIPANTS: Eighty-five consecutive admissions (56 men, 29 women; mean age, 61.9+/-14.4y; range, 18-85y) with radiologically confirmed focal evidence of specific lesions within the pons, midbrain, cerebellum, and medulla. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Various clinical characteristics, including stroke-related deficits and stroke risk factors, were identified and compared between brainstem subgroups. The incidence of complications, including pneumonia, deep vein thrombosis, and seizure disorder, was also reported. RESULTS: Seventy (82%) of the strokes resulted from infarctions and 15 (18%) were caused by hemorrhages. The functional deficits of hemiparesis, ataxia, and diplopia were present in 41 (48%), 73 (86%), and 32 (38%) patients, respectively. Dysarthria was reported in 42 patients (49%) and dysphagia in 40 (47%). Pneumonia during hospitalization was a complication in 9 (11%) of the patients with brainstem stroke. The risk factors of diabetes and hypertension were present in 22 (26%) and 47 (55%) patients, respectively. Fourteen (17%) of these patients had suffered a previous stroke. CONCLUSIONS: Rehabilitation patients experience a variety of functional impairments as a consequence of brainstem stroke. These include hemiparesis, dysarthria and dysphagia, diplopia, and ataxia. A significant number of patients had pneumonia as a complication. The characteristics and impairment profiles of patients within the subgroups were similar, with the exception of the incidence of ataxia and hemiparesis.

Adolescent↗

Giant vertebrobasilar aneurysms: endovascular treatment and long-term follow-up.

OBJECTIVE: To report long-term imaging follow-up and clinical outcome of 13 patients with a giant vertebrobasilar aneurysm treated by parent artery occlusion (PAO). METHODS: From 1994 to 2000, 13 consecutive patients with a giant vertebrobasilar aneurysm were treated by PAO. Symptoms were related to mass effect in nine patients and to a subarachnoid hemorrhage in four. Endovascular treatment consisted of aneurysm trapping in nine patients and occlusion of one or both vertebral arteries in four. We assessed the clinical outcome and imaging findings in all patients during a 28-month period. RESULTS: Endovascular treatment resulted in clinical improvements in eight patients, worsening of symptoms in four, and death in one. One woman with a ruptured vertebral aneurysm died from a rebleeding after PAO without trapping. One man developed a brainstem infarction after lower basilar artery occlusion and incurred hemiparesis. In three patients, symptoms of mass effect increased after the procedure. Long-term follow-up revealed good or excellent clinical outcome in all patients and a sharp decrease in size of the thrombosed aneurysm in nine patients. One basilar aneurysm recanalized despite selective coiling and subsequent bilateral vertebral artery occlusion; one vertebral aneurysm and one basilar aneurysm did not decrease in size despite complete occlusion. CONCLUSION: Giant vertebrobasilar aneurysms are rare and challenging lesions for both neurosurgeons and neurointerventionalists. Their treatment by endovascular PAO remains safe and effective. Early clinical worsening may be observed, but long-term follow-up shows good or excellent results in most patients. This treatment can be carried out with minimal morbidity and mortality using clinical and angiographic monitoring.

Adolescent↗

[Cerebral dissecting aneurysms].

INTRODUCTION: Blood stream penetrates through the lacerations of the arterial layers determining dissecting aneurysms. Each intramural clot localization determines different clinical form. OBJECTIVE: We present the clinical observations carried out in two patients with vertebrobasilar dissecting aneurysms and we review the state of the art of this process to remark the early identification of these unusual lesions. CASE REPORTS: Two cases of vertebrobasilar dissecting aneurysms were described. They were young patients that debuted, one as posteroinferior artery brain steam infarction and the other one as a subarachnoid hemorrhage caused by a right posterior cerebral artery dissecting aneurysms. Lesions were confirmed by helicoidal cerebral angiotomographic and angiograms. This patient was operated on and we clipped the posterior communicating that irrigated dissecting segment. We carry out an anatomical and hemodynamic analysis of that lesion. Both patients had a satisfactory clinical evolution. CONCLUSIONS: Dissecting intramural hematomas of the vertebrobasilar territory are frequent cause of young people ictus. It has been suggested that the ictus may happen after minor head trauma and pain, main symptom, always precedes neurological dysfunction signs. Hemorrhagic presentation has a high incidence of rebleeding and elevated mortality. It always requires, as soon as possible, direct microsurgical treatment or endovascular methods (coils). Most useful microsurgical procedure, until today, is parent artery clipping. This technique has a low index of isquemic and neural postoperative damage.

Adult↗

Ictal paralysis with tonic eye gazing mimicking a pontine infarction.

PURPOSE: Concomitant positive and negative motor phenomena in a single seizure have not been reported before. METHOD: We used an extensive history review, neurological examination, EEG, MRI and SPECT study to demonstrate a rare combination of motor presentations as an ictal phenomenon. RESULT: A 64-year-old male was brought to the emergency room with dizziness, progressive drowsiness and left hemiparesis. A spontaneous eye deviation to the left side with nystagmus was observed. A right pontine lesion was tentatively diagnosed. However, a focal motor seizure of the patient's left face and limbs occurred 3.5h later. A brain MRI revealed a high signal in the right amygdala, hippocampus and thalamus, instead of the pons. An EEG showed periodic epileptic discharges in the right posterior temporal parietal region. Regional hyperperfusion was found by brain SPECT. The level of consciousness improved dramatically after adequate phenytoin treatment. CONCLUSION: A posterior temporal-parietal seizure can present with a prolonged ictal paralysis, a positive ocular nystagmoid deviation and an altered level of consciousness. The EEG is essential for a correct diagnosis, especially with a negative or an unexplainable MRI study. The SPECT has an additional role for the differential diagnosis.

Amygdala↗

Somatotopic organization of the corticospinal tract in the human brainstem: a MRI-based mapping analysis.

To investigate the incompletely understood somatotopical organization of the corticospinal tract in the human brainstem, we performed a voxel-based statistical analysis of standardized magnetic resonance scans of 41 prospectively recruited patients with pyramidal tract dysfunction caused by acute brainstem infarction. Motor hemiparesis was rated clinically and by the investigation of motor evoked potentials to arms and legs. Infarction affected the pons in 85% of cases. We found the greatest level of significance of affected brainstem areas between the pontomesencephalic junction and the mid pons. Lesion location was significantly more dorsal in patients with hemiparesis affecting more proximal muscles and was significantly more ventral in patients with predominantly distal limb paresis. Comparison of magnetic resonance lesion from patients with paresis predominantly affecting arm or leg did not show significant topographical differences. We conclude that a topographical arm/leg distribution of corticospinal fibers is abruptly broken down as the descending corticospinal tract traverses the pons. Corticospinal fibers, however, follow a somatotopical order in the pons with fibers controlling proximal muscles being located close to the reticular formation in the dorsal pontine base, and thus more dorsal than the fibers controlling further distal muscle groups.

Adult↗

Babinski-Nageotte's syndrome and Hemimedullary (Reinhold's) syndrome are clinically and morphologically distinct conditions.

A hemimedullary infarction, in which medial and lateral medullary lesions occur simultaneously, is a rare cerebrovascular disease. It has been suggested that the Babinski-Nageotte's syndrome is the classical brainstem syndrome that corresponds to hemimedullary lesion. In this study we compare clinical symptoms and magnetic resonance imaging (MRI) data of two patients exhibiting classical Babinski-Nageotte's syndrome according to the original description with symptoms and MRI data of a patient with clinically complete hemimedullary lesion. Our study shows that Babinski-Nageotte's syndrome is neither clinically nor on MRI identical with hemimedullary lesion. Hypoglossal palsy, an invariable symptom of hemimedullary syndrome, is not part of the Babinski-Nageotte's syndrome according to the original description. Consistent with the original historical report, Babinski- Nageotte's syndrome is a lateral "Wallenbergian" medullary lesion with a spreading of the lesion to the more basal localised pyramidal tract. The clinical features of the hemimedullary syndrome, described in 1894 by Reinhold, and the MRI appearances in our patient with this syndrome are clearly different from other classical brainstem syndromes and should be called Reinhold's syndrome.

Adult↗

Vestibular evoked myogenic potentials in brainstem stroke.

OBJECTIVES/HYPOTHESIS: Despite its widespread application in the posterior fossa tumor, the study of vestibular evoked myogenic potential (VEMP) in cases of posterior fossa stroke remains scarce. The purpose of the study was to establish the role of VEMP in patients with brainstem stroke. STUDY DESIGN: Retrospective study. METHODS: Patients with acute vertigo were admitted and underwent a battery of auditory vestibular tests including caloric and VEMP tests; then they were surveyed by magnetic resonance imaging scan. Seven patients (two men and five women) were demonstrated as having brainstem stroke, including infarction in five patients and hemorrhage in two. RESULTS: Clinical manifestations consisted of dizziness/vertigo in all patients, spontaneous nystagmus in five (71%), and ataxia in three. No patients had experienced conscious change or conventional neurological deficits (eg, long tract signs). Electronystagmography revealed abnormal findings on eye tracking test in 100% of patients, on optokinetic nystagmus test in 71% of the patients, and on caloric testing in 10 ears (71%), including absent ice-water caloric test response in 7 ears and canal paresis in 3 ears. Vestibular evoked myogenic potential testing displayed normal response in 3 ears and abnormal response in 11 ears (79%), including absent vestibular evoked myogenic potentials in 8 ears and delayed vestibular evoked myogenic potentials in 3 ears. When results of both caloric testing and VEMP test were combined, the abnormal rate increased to 93% (13 of 14 patients). CONCLUSIONS: Caloric testing assesses the vestibulo-ocular reflex, which passes upward through the upper brainstem, whereas VEMP testing evaluates the sacculocollic reflex, which travels downward through the lower brainstem. Hence, in evaluating the extension of brainstem stroke, both caloric and VEMP testing should be performed.

Adult↗

Lateral and medial medullary infarction: a comparative analysis of 214 patients.

BACKGROUND AND PURPOSE: No large-scale study has ever compared the clinical and radiological features of lateral medullary infarction (LMI) and medial medullary infarction (MMI). The aim of this study was to investigate them through the use of cooperatively collected cases. METHODS: Medical information on all patients from 1996 to 2000 with medullary infarction (MI) proven by brain MR images at 35 stroke centers in the Tohoku district, Japan, was collected, and their clinical and radiological features were analyzed. RESULTS: A total of 214 cases of MI were registered. They included 167 cases (78%) of LMI, 41 (19%) of MMI, and 6 (3%) of LMI plus MMI. The mean age of onset and the male-to-female ratio were 60.7 years and 2.7:1 in LMI and 65.0 years and 3.6:1 in MMI, respectively. The middle medulla was most frequently affected in LMI, and the upper medulla was most frequently affected in MMI. Dissection of the vertebral artery was observed in 29% of LMI and 21% of MMI. Prognosis, assessed by the Barthel Index, was favorable in both LMI and MMI. Diabetes mellitus was more frequently associated with MMI than with LMI. CONCLUSIONS: The present study surveyed a large number of MI cases and revealed that (1) the mean age of onset of MMI is higher than that of LMI, (2) the dissection of the vertebral artery is an important cause not only of LMI but also of MMI, and (3) diabetes mellitus is frequently associated with MMI.

Age Factors↗

Management of acute cerebellar infarction: one institution's experience.

OBJECTIVE: The management of cerebellar infarctions is controversial. The aim of this study was to determine which patients require surgical treatment and which surgical procedure should be performed when a patient with a cerebellar infarction exhibits progressive neurological deterioration. METHODS: A total of 44 patients (24 male and 20 female patients; average age, 56 yr) were treated at our institution for cerebellar infarctions in the past 8 years. Twenty-five patients received conservative treatment; two patients who were deeply comatose received no treatment. The remaining 17 patients underwent emergency surgery. Of those 17 patients, 8 underwent external ventricular drainage alone, 5 underwent external ventricular drainage as the first treatment plus secondary suboccipital craniectomy, and 4 underwent suboccipital craniectomy, with removal of necrotic tissue, as the first treatment. RESULTS: Of the 25 conservatively treated patients, 20 experienced good outcomes, 4 experienced moderate outcomes, and 1 died as a result of pulmonary embolism. Of the 17 surgically treated patients, 10 experienced good functional recoveries (7 treated with external ventricular drainage only and 3 treated with drainage followed by suboccipital craniectomy) and 3 survived with mild neurological deficits (one patient underwent ventriculostomy, one suboccipital craniectomy plus external ventricular drainage, and one suboccipital craniectomy only). The overall mortality rate was 13.6% (6 of 44 patients). CONCLUSION: For patients with worsening levels of consciousness and radiologically evident ventricular enlargement, we recommend external ventricular drainage. We reserve surgical resection of necrotic tissue for patients whose clinical status worsens despite ventriculostomy, those for whom worsening is accompanied by signs of brainstem compression, and those with tight posterior fossae.

Adolescent↗

Delirium in the first days of acute stroke.

BACKGROUND AND PURPOSE: Delirium is an acute, transient disorder of cognition and consciousness with fluctuating intensity. The aim of this study was to investigate the presence and the risk factors for delirium in the first days after stroke onset. PATIENTS AND METHODS: We assessed delirium prospectively in a sample of 218 consecutive patients (mean age 57 years) with an acute (</= 4 days) stroke (28 subarachnoid haemorrhages, 48 intracerebral haemorrhages, 142 cerebral infarcts) and in a control group of 50 patients with acute coronary syndromes with the Delirium Rating Scale (DRS) (cut-off score >/= 10). RESULTS: 29 (13%) acute stroke patients (mean DRS score = 13.2, SD = 2.3) and only one (2 %) acute coronary patient had delirium (chi(2) = 5.2, p = 0.02). In nine patients delirium was secondary to stroke without any additional cause, in 10 patients there were also medical complications and in the remaining 10 there were multiple potential causes for delirium. Delirium was more frequent after hemispherical than after brainstem/cerebellum strokes (p = 0.02). No other statistically significant associations with stroke locations were found. Medical complications (OR = 4.3; 95% CI = 1.8 to 10.2), neglect (OR = 3.5; 95% CI = 1.3 to 9.2), intracerebral haemorrhage (OR = 3.1; 95% CI = 1.3 to 7.5) and age >/= 65 (OR = 2.4; 95% CI = 1.0 to 5.8) were independent factors to the development of delirium in stroke patients. CONCLUSION: Delirium was more frequent in stroke than in coronary acute patients. Among stroke patients, delirium was most frequent in older patients, in those with neglect, with medical complications and with intracerebral haemorrhages. These findings indicated that delirium in acute stroke patients 1) is not a non-specific consequence of acute disease and hospitalisation and 2) is secondary to hemisphere brain damage and to metabolic disturbances due to medical complications.

Acute Disease↗