A case of craniocervical junction pial arteriovenous fistula causing postoperative medullary and spinal cord edema.

OBJECTIVES: Pial arteriovenous fistulas (pAVFs) are direct connections between the pial artery and vein without an intervening nidus. We report a rare case of craniocervical junction (CCJ) pAVF causing medullary and spinal cord edema resulting from surgical removal of the varix with remnant shunt after coil embolization. CASE DESCRIPTION: A 16-year-old man presented with subarachnoid hemorrhage. Digital subtraction angiography revealed a CCJ pAVF with multiple fistulas at the 2 varices (varix A and varix B), which was fed by the bilateral lateral spinal arteries and anterior spinal artery (ASA), and drained into the median posterior vermian vein with varix (varix C) and anterior spinal vein (ASV). Varices A and B were embolized using coils, but the shunts remained in varix C. Then, varix C was surgically removed. After this operation, medullary and spinal cord edema occurred. Digital subtraction angiography showed the ASV drainage responsible for edema. Finally, surgical removal of varices A and B was performed. However, arteriovenous shunts, supplied by the ASA and drained into the ASV via the intrinsic vein, were found in the medulla oblongata and coagulated, resulting in disappearance of edema. CONCLUSIONS: Edema was probably caused by concentration of drainage from the arteriovenous shunt in the medulla oblongata into the ASV by surgical removal of varix C acting as another draining route. High flow AVF can induce angiogenesis and secondary arteriovenous shunt. Precise analysis of the angioarchitecture is important to treat such cases without complications.

Recurrent Lobar Hemorrhages and Multiple Cortical Superficial Siderosis in a Patient of Alzheimer's Disease With Homozygous APOE ε2 Allele Presenting Hypobetalipoproteinemia and Pathological Findings of 18F-THK5351 Positron Emission Tomography: A Case Report.

In Alzheimer's disease, the apolipoprotein E gene (APOE) ε2 allele is a protective genetic factor, whereas the APOE ε4 allele is a genetic risk factor. However, both the APOE ε2 and the APOE ε4 alleles are genetic risk factors for lobar intracerebral hemorrhage. The reasons for the high prevalence of lobar intracerebral hemorrhage and the low prevalence of Alzheimer's disease with the APOE ε2 allele remains unknown. Here, we describe the case of a 79-year-old Japanese female with Alzheimer's disease, homozygous for the APOE ε2 allele. This patient presented with recurrent lobar hemorrhages and multiple cortical superficial siderosis. The findings on the 11C-labeled Pittsburgh Compound B-positron emission tomography (PET) were characteristic of Alzheimer's disease. 18F-THK5351 PET revealed that the accumulation of 18F-THK 5351 in the right pyramidal tract at the pontine level, the cerebral peduncle of the midbrain, and the internal capsule, reflecting the lesions of the previous lobar intracerebral hemorrhage in the right frontal lobe. Moreover, 18F-THK5351 accumulated in the bilateral globus pallidum, amygdala, caudate nuclei, and the substantia nigra of the midbrain, which were probably off-target reaction, by binding to monoamine oxidase B (MAO-B). 18F-THK5351 were also detected in the periphery of prior lobar hemorrhages and a cortical subarachnoid hemorrhage, as well as in some, but not all, areas affected by cortical siderosis. Besides, 18F-THK5351 retentions were observed in the bilateral medial temporal cortices and several cortical areas without cerebral amyloid angiopathy or prior hemorrhages, possibly where tau might accumulate. This is the first report of a patient with Alzheimer's disease, carrying homozygous APOE ε2 allele and presenting with recurrent lobar hemorrhages, multiple cortical superficial siderosis, and immunohistochemically vascular amyloid ß. The 18F-THK5351 PET findings suggested MAO-B concentrated regions, astroglial activation, Waller degeneration of the pyramidal tract, neuroinflammation due to CAA related hemorrhages, and possible tau accumulation.

Successful basilar artery dilatation in pure bilateral cerebral peduncular infarctions using balloon angioplasty.

•An extremely rare case of bilateral cerebral peduncular infarctions (BCPI) is reported.•The detection of the pure Mickey Mouse ears sign on MRI is an indicator of a need for reperfusion therapy.•Severe stenosis of the basilar artery (BA) and a poor collateral supply from both posterior cerebral arteries were seen.•Balloon angioplasty for the BA stenosis ameliorated the stenosis and produced a favorable outcome.

Spinal epidural arteriovenous fistulas with unusual manifestation of sudden onset of severe neurological deficits: case report.

Spinal epidural arteriovenous fistulas with perimedullary venous drainage cause venous hypertension, and usually manifest as slowly progressive myelopathy. We treated two patients presenting with sudden onset of severe neurological deficits. Moreover, in Case 1, the venous drainage was exclusively epidural and no perimedullary venous drainage was present. Angiographic findings of this patient were characterized by a slow-flow fistula with marked retention of the epidural venous drainage. Rapidly progressing thrombosis of the epidural venous plexus may have caused the sudden onset of the symptoms. In Case 2, hematomyelia may also be possibly associated with the sudden onset of the symptoms. Early diagnosis and treatment are essential to achieve favorable outcome in such cases because venous congestion results in irreversible venous infarction within a short period.

Clinical and angiographic characteristics of cavernous sinus dural arteriovenous fistulas manifesting as venous infarction and/or intracranial hemorrhage.

INTRODUCTION: Cavernous sinus (CS) dural arteriovenous fistulas (DAVFs) rarely cause venous infarction (VI) and/or intracranial hemorrhage (ICH) despite the presence of cortical venous drainage (CVD). The present study investigated the characteristics of CS DAVFs manifesting as VI/ICH. MATERIALS AND METHODS: Fifty-four patients treated for CS DAVFs were retrospectively studied. RESULTS: Six patients presented with VI/ICH. Two of the three patients presenting with ICH had CVD only to the superficial sylvian vein (SSV) or the deep sylvian vein (DSV). Three patients presenting with VI had multiple drainages, and angiography of these patients showed a varix on the SSV, drainage into the DSV with agenesis of the second and third segment of basal vein of Rosenthal, and thrombosis of the distal petrosal vein. CS DAVF with CVD only carries higher risk of VI/ICH than multiple drainages. Many CS DAVFs presenting with VI, especially those with drainage into the petrosal vein, have multiple drainages in the early stage. Thrombosis of the inferior and superior petrosal sinuses and superior orbital vein gradually increases pressure of the CVD, and then, VI may occur. In contrast, CS DAVFs with CVD only from the beginning, common in the patients with drainage into the SSVs and DSVs, are likely to cause ICH. CONCLUSION: Angiographic risk factors causing VI/ICH are CVD only, varix formation, agenesis of the second and third segment of basal vein of Rosenthal, and thrombosis of the superior orbital vein, lateral half of the superior petrosal sinus, and distal CVD.

Urgent stenting for patients with acute stroke due to atherosclerotic occlusive lesions of the cervical internal carotid artery.

Acute symptomatic occlusion of the cervical internal carotid artery (ICA) can be treated by intravenous administration of tissue plasminogen activator, percutaneous transluminal angioplasty, and carotid endarterectomy. Carotid artery stenting (CAS) is now indicated for cervical ICA stenosis, but the safety and the efficacy of urgent CAS have not been established. We retrospectively reviewed 10 patients treated by urgent CAS for atherosclerotic occlusive lesions of cervical ICA with acute stroke. Five patients had complete occlusions and five had near total occlusions. Five of the 10 patients had intracranial tandem occlusions. Indication for urgent CAS was determined by mismatch of diffusion-weighted and perfusion-weighted magnetic resonance imaging findings. Stents were successfully deployed in all lesions. Three of five patients with concomitant intracranial tandem occlusions were treated by additional intraarterial fibrinolysis after the CAS. Intracranial artery occlusions were completely recanalized in one patient, and partially recanalized in two by fibrinolysis. Hyperperfusion syndrome did not occur in any of the patients. A favorable outcome (modified Rankin Scale < or =1) was obtained in all of the five patients with isolated cervical ICA occlusion and one of the five patients with intracranial tandem occlusions. Urgent CAS is a safe and effective treatment in patients with isolated cervical ICA occlusion. Treatment of intracranial tandem occlusions is an issue that must be resolved.