Spontaneous subarachnoid hemorrhage first from an intracranial and then from a spinal arteriovenous malformation. Case report

A patient presented with spontaneous subarachnoid hemorrhage (SAH) from a cerebral arteriovenous malformation (AVM) which was later totally removed at surgery. The patient presented again with a new SAH from a spinal AVM that was also totally removed at surgery. Coexistence of spinal and cerebral arteriovenous malformations are exceedingly rare and hemorrhage from each is not previously reported. This case emphasizes the importance of investigating the spinal canal in otherwise unexplained spontaneous SAH.     

Evolving magnetic resonance spinal cord trauma in child: from hemorrhage to intradural arachnoid cyst

Acquired arachnoid cysts of the spinal cord are uncommon causes of spinal cord compression in the pediatric group. Meningitis, trauma and hemorrhage are considered to be causative or contributing factors. Interestingly, no spinal arachnoid cysts have been reported in patients after subarachnoid hemorrhage or meningitis, conditions expected to cause arachnoid scarring. We describe a child of 1 year and 10 months with thoracic spine trauma with crural paraplegia and anesthesia at level T5 submitted to serial magnetic resonance imagery at 5 days and 18 months, after trauma showing evolution from subarachnoid hemorrhage and adhesions of the arachnoid space to a posterior hypertensive thoracic intradural arachnoid cyst.     

Histological findings of gallbladder mucosa in 95 control subjects and 80 patients with asymptomatic gallstones

BACKGROUND AND PURPOSE: Decisions on invasive arteriovenous malformation (AVM) treatment are currently based on natural-course risk estimates of AVM bleeding and assumptions on morbidity from cerebral hemorrhage in general. However, morbidity of AVM hemorrhage has rarely been reported. We sought to assess the morbidity of intracranial hemorrhage in patients with cerebral AVMs. METHODS: From a prospective AVM database, 119 patients were analyzed: 115 had a hemorrhage as the diagnostic event, and 27 of them suffered a second hemorrhage during follow-up; an additional 4 patients had other diagnostic symptoms but bled during follow-up. The type (parenchymal, subarachnoid, intraventricular) and location of AVM hemorrhage were determined by CT/MR brain imaging. Disability and neurological impairment were assessed with the Barthel Index, the Rankin Scale, and the National Institutes of Health Stroke Scale, with a mean follow-up time of 16.2 months. RESULTS: Of the 115 incident hemorrhages, 34 (30%) were subarachnoid, 27 (23%) parenchymal, 18 (16%) intraventricular, and 36 (31%) in combined locations. In 54 patients (47%; 95% confidence interval [CI], 38% to 56%) the incident hemorrhage resulted in no neurological deficit, and an additional 43 patients (37%; 95% CI, 28% to 46%) were independent in their daily activities (Rankin 1). Fifteen patients (13%; 95% CI, 7% to 19%) were moderately disabled (Rankin 2 or 3), and 3 (3%; 95% CI, 0% to 6%) were severely disabled (Rankin > or =4). Parenchymal hemorrhages were most likely to result in a neurological deficit (52%). Type and morbidity of hemorrhage during follow-up were similar to incident events. Twenty (74%) of 27 patients with both incident and follow-up hemorrhages were normal or independent (Rankin 0 or 1). None of the patients with a hemorrhage during follow-up died during the observation period. CONCLUSIONS: Hemorrhage from cerebral AVMs appears to have a lower morbidity than currently assumed. This finding encourages a reevaluation of the risks and benefits of invasive AVM treatment.     

Transluminal angioplasty and intra-arterial papaverine for the treatment of cerebral vasospasm after ruptured arteriovenous malformations

BACKGROUND: This is the first report on the use of intra-arterial papaverine and percutaneous transluminal angioplasty in two patients with severe, symptomatic cerebral vasospasm who suffered ruptured arteriovenous malformations (AVMs). CASE DESCRIPTIONS: The source of hemorrhage was a venous aneurysm in the first case and a pedicular aneurysm of the distal posterior inferior cerebellar artery in the second case. In both cases, the AVMs were located in the superior vermis and there was minimal subarachnoid hemorrhage. The first patient underwent removal of the AVM before the period of cerebral vasospasm and the second patient underwent removal of the AVM after the cerebral vasospasm had resolved. The outcome was excellent in the first patient and poor in the second patient. CONCLUSION: Arteriovenous malformation with ruptured aneurysms may be at high risk for cerebral vasospasm even when there is minimal subarachnoid hemorrhage. We recommend early treatment of AVMs with ruptured pedicular, intranidal, or venous aneurysms to avoid rebleeding and to allow for aggressive treatment of cerebral vasospasm. The management of cerebral vasospasm after AVM rupture is discussed.     

Utility of the American Diabetes Association risk test in a community screening program

In many patients with superficial siderosis of the central nervous system (CNS) no source of bleeding can be established, despite extensive examinations. The authors report a patient with superficial siderosis and a spinal arteriovenous malformation (AVM) that was not visible on magnetic resonance (MR) imaging or myelography but was identified on angiographic studies. This 71-year-old man presented with a 2-year history of progressive gait difficulties and hearing loss. Examination showed ataxia, hearing loss, and quadriparesis. On MR imaging superficial siderosis of the brain and spinal cord as seen; however, MR imaging of the CNS, as well as cerebral angiography and myelography studies, did not reveal the source of hemorrhage. Spinal angiography revealed a small slow-flow pial AVM at the C-5 level originating from the anterior spinal artery. A C-5 corpectomy was performed and the AVM was obliterated. The patient did well and reported no further progression of his symptoms during 3 months of follow up. Spinal angiography is indicated to complete the evaluation of patients with superficial siderosis, even if results of spinal MR imaging and myelography studies are normal. Obliteration of spinal AVMs may successfully prevent the progression of superficial siderosis.     

Nucleotide substitutions at the -6 position in the promoter region of the factor IX gene result in different severity of hemophilia B Leyden: consequences for genetic counseling

A case of vessel perforation by a guide wire during an interventional neuroradiological procedure is reported. The patient was a 59-year-old woman with a left frontal basal arteriovenous malformation (AVM) fed by the left anterior cerebral artery. Transarterial embolization of the AVM was attempted. During the procedure, vessel perforation by the guide wire occurred at the left A1-A2 junction and resulted in subarachnoid hemorrhage, which stopped spontaneously. The patient developed progressive obstructive hydrocephalus, and surgical treatment was performed. The AVM was totally removed after ventricular drainage, and the arterial perforation site was explored. When clot around the left A1-A2 junction was removed, hemorrhage recurred. This hemorrhage was similar to what has been observed when a small perforating artery was avulsed. The hemorrhage site was coagulated under temporary occlusion of both A1 segments. Surgical intervention was probably not necessary for this type of bleeding if it had stopped spontaneously, because the rebleeding from the small pinhole would be unlikely, and the operation was more hazardous than the usual aneurysmal surgery.     

Use of polymerase chain reaction in clinical diagnostic laboratories in viral hepatitis B and C]

alpha 1-Antitrypsin deficiency has been associated with a variety of vascular disorders including arterial aneurysms, spontaneous extracranial arterial dissections, and arterial fibromuscular dysplasia. We determined the distribution of alpha 1-antitrypsin phenotypes in patients with intracranial arterial dissections, a rare cause of subarachnoid hemorrhage. The study population consisted of 4 consecutive patients with subarachnoid hemorrhage due to spontaneous intracranial arterial dissections. The vertebral artery was involved in 3 patients and the posterior inferior cerebellar artery in 1 patient. Three of these 4 patients were found to have a heterozygous alpha 1-antitrypsin deficiency (PiMZ or PiMS phenotypes). These data support previous studies suggesting that patients with alpha 1-antitrypsin deficiency may be at an increased risk of developing spontaneous arterial dissections.     

Successful surgical removal of a large arteriovenous malformation in a patient with hemophilia: case report

OBJECTIVE AND IMPORTANCE: This is the first reported case of the successful surgical removal of a large arteriovenous malformation (AVM) in a patient with hemophilia A. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our department with intracranial hemorrhage. He had previously been diagnosed with hemophilia A and a cerebral AVM. Carotid angiography revealed a large AVM in the right temporal and parietal lobes. The neurological and neuroradiological findings, especially those of single photon emission computed tomography, identified an area of devitalization around the lesion, which was thought to reduce the risk of new deficits resulting from surgical manipulation. INTERVENTION: We resected the AVM in conjunction with supplemental infusions of Factor VIII before, during, and after the operation. A slight cerebral hemorrhage on the 7th postoperative day was observed despite control with Factor VIII, but the patient was discharged without any new deficits. CONCLUSION: We evaluated and managed all problems of a patient with multiple complications and achieved a medical cure.     

Occurrence of severe vasospasm following intraventricular hemorrhage from an arteriovenous malformation. Report of two cases

The authors present two rare cases of severe cerebral vasospasm following the rupture of arteriovenous malformations (AVMs). Computerized tomography revealed intracerebral hemorrhage in the thalamus in one case and in the putamen in the other, both accompanied by cast formation of intraventricular clots without radiological evidence of subarachnoid hemorrhage. Initial angiograms showed arterial narrowing of the bilateral internal carotid arteries in the supraclinoid portion but failed to demonstrate an arteriovenous shunt. Subsequent angiograms clearly demonstrated the existence of an AVM. Radiological features and possible mechanisms are discussed.     

Intraoperative spinal sonography in the cervical anterior approach

Intraoperative spinal sonography was used during cervical anterior approach procedures for cervical discectomy and osteophytectomy to demonstrate spinal pulsation, the protruded disc or osteophyte, the anterior subarachnoid space, and the spinal cord. Spinal pulsation was recognized in some cases before removal of the disc but the anterior subarachnoid space and spinal cord could not be observed. However, the latter were more clearly observed during removal of the disc and could be seen after total removal of the disc and osteophyte. This method allows confirmation of decompression and pulsation of the spinal cord without cutting and removal of the posterior longitudinal ligament.     

Efficacy and pharmacokinetics of a new intrarectal quinine formulation in children with Plasmodium falciparum malaria

The O-2A progenitor cell, which serves as a stem cell for the myelinating oligodendrocyte, has been implicated as a major target for radiation-induced spinal cord injury. In an attempt to increase the number of O-2A cells in the spinal cord, we applied an ex vivo gene therapy procedure for delivering platelet derived growth factor (PDGF). Recombinant fibroblasts expressing PDGF A chain were injected into the cisterna magna of adult rats, which resulted in cell seeding of the subarachnoid space of the cervical spinal cord. The number of O-2A progenitors in the cervical spinal cord was then assessed with an in vitro clonogenic assay. O-2A cells were found to be increased 8 days after recombinant cell injection, and they remained elevated up to at least 14 days. Analysis of O-2A colonies indicated that the implantation of PDGF-expressing cells increased the number of O-2A progenitors without affecting their in vitro proliferation potential or differentiation capacity. These data suggest that implantation of PDGF-expressing cells in the subarachnoid space of the cervical spinal cord may influence a stem cell population critical to the repair of demyelinated lesions.     

A biomathematical model of intracranial arteriovenous malformations based on electrical network analysis: theory and hemodynamics

Hemodynamics play a significant role in the propensity of intracranial arteriovenous malformations (AVMs) to hemorrhage and in influencing both therapeutic strategies and their complications. AVM hemodynamics are difficult to quantitate, particularly within or in close proximity to the nidus. Biomathematical models represent a theoretical method of investigating AVM hemodynamics but currently provide limited information because of the simplicity of simulated anatomic and physiological characteristics in available models. Our purpose was to develop a new detailed biomathematical model in which the morphological, biophysical, and hemodynamic characteristics of an intracranial AVM are replicated more faithfully. The technique of electrical network analysis was used to construct the biomathematical AVM model to provide an accurate rendering of transnidal and intranidal hemodynamics. The model represented a complex, noncompartmentalized AVM with 4 arterial feeders (with simulated pial and transdural supply), 2 draining veins, and a nidus consisting of 28 interconnecting plexiform and fistulous components. Simulated vessel radii were defined as observed in human AVMs. Common values were assigned for normal systemic arterial pressure, arterial feeder pressures, draining vein pressures, and central venous pressure. Using an electrical analogy of Ohm's law, flow was determined based on Poiseuille's law given the aforementioned pressures and resistances of each nidus vessel. Circuit analysis of the AVM vasculature based on the conservation of flow and voltage revealed the flow rate through each vessel in the AVM network. Once the flow rate was established, the velocity, the intravascular pressure gradient, and the wall shear stress were determined. Total volumetric flow through the AVM was 814 ml/min. Hemodynamic analysis of the AVM showed increased flow rate, flow velocity, and wall shear stress through the fistulous component. The intranidal flow rate varied from 5.5 to 57.0 ml/min with and average of 31.3 ml/min for the plexiform vessels and from 595.1 to 640.1 ml/min with an average of 617.6 ml/min for the fistulous component. The blood flow velocity through the AVM nidus ranged from 11.7 to 121.1 cm/s with an average of 66.4 cm/s for the plexiform vessels and from 446.9 to 480 dyne/cm2 with an average of 463.5 dyne/cm2 for the fistulous component. The wall shear stress ranged in magnitude from 33.2 to 342.1 dyne/cm2 with an average of 187.7 dyne/cm2 for the plexiform vessels and from 315.9 to 339.7 cm/s with an average of 327.8 cm/s for the fistulous component. The described novel biomathematical model characterizes the transnidal and intranidal hemodynamics of an intracranial AVM more accurately than was possible previously. This model should serve as a useful research tool for further theoretical investigations of intracranial AVMs and their hemodynamic sequelae.     

Relationships between delayed ischemic dysfunctions and intracranial hemodynamics following subarachnoid hemorrhage

Delayed ischemic dysfunctions (DID) are one of the main complications following subarachnoid hemorrhage. It was the aim of our study to analyse the possible prognostic value of different transcranial Doppler ultrasonography parameters and to elucidate the risk of developing DID with particular reference to the intracranial pressure. The relative change of mean blood flow velocity and of corresponding cerebral circulatory resistance index as well as the intracranial pressure were determined in 44 patients with spontaneous subarachnoid hemorrhage. No relationship was found between the occurrence and extent of DID and the relative change of mean flow velocity during the clinical course. In contrast, there was a significant correlation between the relative change of the cerebral circulatory resistance index and the occurrence of DID. While patients without or with reversible DID showed a decreased resistance index compared to the initial value (without DID: -17% +/- 15%; reversible DID: -3% +/- 14%), patients with irreversible DID had a significant increase of the resistance index (+14% +/- 9%). Accordingly, patients with irreversible DID showed a significant increase of intracranial pressure compared to the patients with reversible DID. We conclude that the evaluation of relative changes of the cerebral circulatory resistance index by bedside monitoring is a useful tool to predict the occurrence of subarachnoid hemorrhage-associated DID and has therapeutic impact.     

Subarachnoid hemorrhage as a form of onset of systemic lupus erythematosus

INTRODUCTION: The most frequent cause of spontaneous subarachnoid hemorrhage (SAH) is rupture of intracranial arterial aneurysms (> 70%). The remainder are due to many different aetiologies. Although SAH is a relatively common neuropathological finding in systemic lupus erythematosus (SLE), it is normally due to the extent of the intercerebral hemorrhage and not to its isolated presentation. CLINICAL CASE: We report the case of a 34 year old woman who presented with non-traumatic SAH at the onset of her lupus disorder. The patient was attended for SAH and at the same time a multisystemic disorder and severe thrombocytopenia were found, leading to a diagnosis of SLE. The neuroimaging techniques, selective cerebral arteriography, cerebral and spinal magnetic resonance, and magnetic resonance angiography did not show any vascular malformations. The patient was treated with immunosuppressive therapy, nimodipine, and following angiographic tests, with antiaggregants and anticoagulants. CONCLUSIONS: The greater frequency of SAH in patients with lupus, as compared to the general population, has been attributed to the presence of intracranial vasculitis. However, neuropathological studies have shown that true vasculitis is very infrequent in the central nervous system of SLE patients. In the case we describe, the first in which SAH appeared at the onset of the disease, we consider that the origin of the hemorrhage was her high arterial blood pressure and thrombopenia.     

Vinorelbine and ifosfamide for unresectable non-small cell lung cancer

OBJECTIVE: The diagnostic value of cine magnetic resonance imaging (CMRI) that visualizes fluid and tissue movement was evaluated in patients with spinal intradural arachnoid cysts, a rare but increasingly detected cause of spinal cord dysfunction. METHODS: Four patients with thoracic spinal intradural arachnoid cysts were investigated with conventional T1- and T2-weighted and cardiac-gated CMRI. Four normal volunteers also underwent CMRI for comparison. RESULTS: Sagittal T1- and T2-weighted imaging showed lesions as an abnormal widening of the posterior spinal subarachnoid space, but mixed high- and low-signal intensities on T2-weighted imaging suggested cystic lesions. CMRI, using 16 to 20 sagittal gradient echo images during the cardiac cycle of normal volunteers, indicated synchronous signal changes along the subarachnoid space, suggesting a smooth cerebrospinal fluid flow. CMRI of patients detected that the caudal or cranial direction of the high-signal propagation suddenly reversed at some locations (as if rebounding) in an asynchronous fashion along the lesion (asynchronous rebound phenomenon), which was well demonstrated by the closed-loop video mode. Cystectomy revealed that the cysts consisted of multiple lobules and that the asynchronous rebound phenomenon corresponded with some boundaries of cyst lobules. CMRI also visualized dynamic spinal cord compression by the cyst. CONCLUSION: CMRI can demonstrate abnormal fluid flow and spinal cord compression caused by a spinal intradural arachnoid cyst.     

A new perspective on the relationship between communication impairment and disempowerment following head injury in information exchanges

Transcranial doppler sonography is a diagnostic tool that allows the noninvasive study of intracranial circulation. In patients with aneurysmal subarachnoid hemorrhage, cerebral vasospasm is promptly diagnosed, even before clinical related symptoms. Feeding arteries of an arteriovenous cerebral malformation can be detected and their variations can be correlated to the treatment (neurosurgical and/or neuroradiological). When raised intracranial pressure (namely in head trauma), transcranial doppler sonography depicts vasospasm and/or typical variations of the waveforms, suggesting intracranial circulatory arrest.     

Spontaneous thoracic spinal cord herniation through an anterior dural defect

A 44-year-old woman was examined for progressive left lower extremity weakness and spasticity. Thoracic spine MR imaging and CT myelography showed a ventral dural defect at T7-T8 with an extradural subarachnoid fluid collection and extradural herniation of the spinal cord. Intraoperative sonography confirmed the appropriate level for dural entry and the finding of spinal cord herniation. After reduction of the herniated spinal cord, the patient experienced gradual improvement in neurologic function.     

Vascular malformations of the spinal cord. Diagnosis and therapy

A review is given on the current diagnosis and treatment of spinal vascular malformations with special emphasis on the use of selective spinal angiography. Spinal vascular malformations are mostly encountered within the thoraco-lumbar segment of the spinal cord. Typical clinical symptoms are due to either progressive myelopathy or hemorrhage (subarachnoidal hemorrhage). The prognosis of untreated spinal angiomas is poor. Therefore, therapeutic measures are aiming to prevent bleeding of angiomas resulting in subarachnoidal hemorrhages or hematomyelia or to prevent spinal cord ischemia due to compression. Using selective spinal angiography, spinal angiomas can now be treated successfully by complete surgical extirpation or embolization procedures. The evaluation of radicular myelopathies and subarachnoidal hemorrhages of unknown origin has also considerably benefited from this technique. It is concluded that early diagnosis of spinal vascular malformations is of crucial importance for good therapeutical results.     

An evidence-based approach to management of increased intracranial pressure

This article reviews the current understanding of genetic factors involved in the development of intracranial aneurysms. Of the large number of heritable connective tissue disorders that have been associated with intracranial aneurysms, three are discussed in detail: autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome type IV, and alpha 1-antitrypsin deficiency. The author reviews the familial occurrence of intracranial aneurysms, which is found in about one-fifth of patients with aneurysm subarachnoid hemorrhage. Guidelines are provided for the evaluation of patients with familial intracranial aneurysms and screening of their relatives.     

Oncotic aneurysm. Case report

The author describes a case of intracerebral and subarachnoid hemorrhage associated with an intracranial aneurysm caused by a metastatic tumor. The aneurysm formation is explained as being result of tumor cells invading the vessel wall while still preserving the arterial circulation.