[Brain infarction and cerebral venous thrombosis in paroxysmal nocturnal hemoglobinuria: case report].

A 65-year-old woman with a six-year history of paroxysmal nocturnal hemoglobinuria (PNH) was admitted due to weakness in the right leg following a seven-day history of fever and upper respiratory infection. MRI revealed several high-intensity areas in bilateral frontal lobe cortices and the left cerebellum on diffusion-weighted imaging, and signal hypointensity along the course of the cortical vein in the left frontal lobe on T2*-weighted imaging. We diagnosed cerebral venous thrombosis and brain infarction, and commenced heparin infusion. She developed right-sided dens hemiparesis on hospital day 6, when brain CT showed subcortical hemorrhage in the left frontal lobe. Despite eculizumab administration and decompressive craniectomy for hematoma, she died on hospital day 26. Thrombosis in PNH has been recognized as a life-threating complication, and intensive treatment including emergent administration of eculizumab is warranted if this situation arises.

Periprocedural Outcomes of Carotid Artery Stenting in Elderly Patients.

BACKGROUND: Carotid artery stenting has emerged as an alternative to carotid endarterectomy especially in patients with high risk of carotid endarterectomy. Older age (≥80 years old) was recognized as one of the high risk factors of carotid endarterectomy. However, the association between older age and increased risk of adverse events for carotid artery stenting has been reported. The purpose of this study was to evaluate the association between age and periprocedural outcomes after carotid artery stenting. METHODS: A total of 126 symptomatic and asymptomatic cases of carotid artery stenosis were treated with tailored carotid artery stenting. The type of stents and embolic protection devices were chosen according to clinical and morphologic characteristics of the patients. Procedural, imaging, and clinical outcomes were retrospectively assessed and compared between the elderly patients group (≥80 years old) and the non-elderly patients group (<80 years old). RESULTS: Clinical and morphologic characteristics except for dyslipidemia were not significantly different between the 2 groups. Periprocedural neurologic complications were not significantly different between the 2 groups (P = .095). Minor stroke occurred more frequently in the elderly patients group (P = .021). However, the frequency of major stroke was not significantly different between the 2 groups (P = 1). Presence of new ischemic lesions on postprocedural examination was not significantly different between the 2 groups (P = .84). Myocardial infarction and death did not occur in either group. CONCLUSIONS: Carotid artery stenting can be performed safely in elderly patients, comparable with non-elderly patients.

Complications and Predictors of Hypotension Requiring Vasopressor after Carotid Artery Stenting.

A significant controversy exists regarding the clinical impact of hemodynamic depression on major adverse events after carotid artery stenting (CAS). The purpose of this study was to evaluate the incidence, predictors, and clinical significance of hypotension after CAS. A total of 118 cases of carotid artery stenosis were treated with CAS. Hypotension was defined as sustained systolic blood pressure <80 mmHg and requiring intravenous administration of vasopressor to maintain adequate systolic blood pressure after the procedure. Baseline characteristics, procedural characteristics, and periprocedural major adverse events were retrospectively compared between postprocedural hypotension group and non-hypotension group. Morphological and procedural characteristics were not significantly different between the two groups. Periprocedural major adverse events, presence of new ischemic lesions, and number of new ischemic lesions were not significantly different between the two groups (P = 1, P = 0.36, P = 0.68). Hypertension was an independent protective factor (P = 0.037), and use of proximal protection and the distance from carotid bifurcation to maximum stenotic lesion ≤ 10 mm were independent risk factors for developing hypotension after CAS (P = 0.034, P = 0.027). There was no significant relationship between hypotension after CAS and major adverse events in this study. Maintenance of periprocedural adequate cerebral perfusion is thought to be important to prevent ischemic complications due to hypotension after CAS, especially in these cases.

Factors for Achieving Safe and Complete Treatment for Unruptured Saccular Aneurysm Smaller Than 10 mm by Simple Clipping or Simple Coil Embolization.

OBJECTIVE: Reducing complications from unruptured aneurysms (UAs) treatment is important. We clarify the criteria for achieving safe and complete treatment for UAs ≤10 mm by clipping or coil embolization. METHODS: This study included 59 newly treated UAs in the past 2 years. We prospectively decided on criteria to recommend active treatment. UAs ≤10 mm and in ≤75 year-olds, located at in the internal carotid artery at the paraclinoid portion and the posterior circulation aneurysms except for a vertebral artery-inferior posterior cerebellar artery aneurysm were mainly treated by coil embolization, and those in the internal carotid artery except at the paraclinoid portion, in the anterior or middle cerebral artery, and in the vertebral artery-inferior posterior cerebellar artery were treated preferably by clipping. UAs with a height/neck ratio or a dome/neck ratio ≤1.4 were treated preferentially by clipping. Specific preoperative imaging and careful manipulation were adopted for clipping. RESULTS: Fifty-seven (96.6%) achieved modified Rankin scale (mRS) 0-1, 2 (3.4%) mRS 2-5, and 0 had mRS 6. Fifty-three UAs (89.8%) achieved complete occlusion (CO) and 7 (10.1%) had neck remnants (NR). Forty-one UAs (100%) within the criteria achieved mRS 0-1, 40 (98%) achieved CO, and 1 (2%) NR. The odds ratio of NR for those outside the criteria was 18.5 (95% confidence interval, 1.83-186.6) (P < 0.05). CO treated within the criteria was 39 and NR was 1. CO treated outside the criteria was 14 and NR was 5 (P < 0.05). The mRS 0-1 with age ≤75 years was 55 and the mRS 2-6 was 0. The mRS 0-1 with age ≥76 years was 2 and the mRS 2-6 was 2 (P < 0.01). CONCLUSIONS: The treatment for UAs within the criteria, with the most recent points of concern, can lead to safe and complete results.

Progress in the treatment of unruptured aneurysms.

Recent technological progress has reduced the complication rate of unruptured aneurysm. We treated 128 unruptured aneurysms between April 2006 and March 2012. Seventy-six aneurysms (59 %) were clipped and 52 (41 %) were coil embolized. After 2010, we applied new instruments, i.e., near-infrared indocyanine-green videoangiography (ICG), an intraoperative endoscope, preoperative detailed MRI, and a stent-assisted coil embolization. In the results: (1) In 60 aneurysms treated before 2009, three patients showed a deterioration of more than two points in mRS (5 %). In 68 aneurysms treated after 2010, no patients showed deterioration (0 %) (p: n.s.). (2) No patients died and 126 patients (98 %) were discharged to home directly. (3) No patients showed rupture after treatment. In conclusion, the appropriate selection of treatment and recent technological progress have facilitated sophisticated treatment of unruptured aneurysms. Recently, the complication rate in surgery and endovascular surgery for unruptured aneurysms has become acceptably low.

Mucinous adenocarcinoma arising from supratentorial intramedullary neuroenteric cyst with broncho-pulmonary differentiation.

Neuroenteric cysts are benign intradural endoderm cysts lined by gastrointestinal (GI) or tracheobronchial epithelial cells. Their malignant transformation is extremely rare and only six cases have been reported. In these cases, tissue lineage of the cystic endoderm cells giving rise to carcinoma was not clearly identified either as respiratory or as GI type. Herein, we report a case of mucinous adenocarcinoma arising from the neuroenteric cyst with broncho-pulmonary differentiation in the right cerebral hemisphere of a Japanese woman in her late 50s. The cyst wall was entirely lined by the following respiratory epithelial components: stratified bronchial ciliated columnar epithelium with basal cells positive for CK5 and p63, terminal bronchiolar Clara cells positive for thyroid transcription factor (TTF)-1, surfactant B and negative for surfactant C, type I pneumocytes positive for TTF-1, negative for surfactant B and C, and type II pneumocytes positive for TTF-1 and surfactant B and C. In addition, nests of hyperplastic single layered mucinous epithelial cells with bronchial goblet cell phenotype were also demonstrated, and histologic features were almost the same as the pulmonary type I congenital adenomatoid malformation (CCAM) with mucinous epithelial proliferation. The mucinous epithelial nests of type I CCAM are liable to develop mucinous adenocarcinoma and frequently accompany K-ras mutation and expression of p16. However, K-ras mutation and p-16 expression were not detected in this case.

Relationship between preoperative magnetic resonance imaging and surgical findings: aneurysm wall thickness on high-resolution T1-weighted imaging and contact with surrounding tissue on steady-state free precession imaging.

This study evaluated the aneurysm wall thickness by high-resolution T1-weighted imaging and the contact between the aneurysm and surrounding tissue by steady-state free precession (SSFP) imaging. The surgical findings were prospectively compared with these preoperative magnetic resonance (MR) imaging findings in 35 consecutive patients with 37 unruptured cerebral aneurysms (UCAs). The aneurysm wall was not visible in 13 UCAs, but was visible in 23. Subarachnoid space between the aneurysm and surrounding tissue was visible in 16 UCAs, a visible layer of cerebrospinal fluid (CSF) between the aneurysm and surrounding tissue in 12, and no visible layer in 7. MR imaging predicted the surgical findings in 29 UCAs (78%), showed different findings in six UCAs (16%), and two (5%) could not be evaluated due to insufficient quality of preoperative MR images. Among the UCAs with different findings, five UCAs had a partially thin wall even though high-resolution T1-weighted imaging had shown a visible wall, and one UCA showed less contact with the surrounding tissue even though the SSFP imaging had shown no visible CSF layer. In conclusion, high-resolution T1-weighted imaging and SSFP imaging provided significant additional preoperative information regarding UCAs and the surrounding tissue.

Trigonal cavernous malformation with intraventricular hemorrhage: a case report and literature review.

We reported a case of trigonal cavernous malformation (CM) with intraventricular hemorrhage. This 67-year-old woman experienced sudden onset of loss of consciousness and her Glasgow Coma Scale (GCS) was 5 points (E1V1M3) on admission. CT scan demonstrated intraventricular hemorrhage and acute hydrocephalus. Angiography did not demonstrate any vascular abnormality. Ventricular drainage was performed for acute hydrocephalus and the postoperative course was good. CT showed a hyperdense lesion in the left trigone, which was contrast-enhanced on T1-weighted MR. Removal of CM was performed via the left middle temporal sulcus. We conducted a Pub Med search for trigonal CM and found 17 cases. Herein we discuss the symptoms, CT and MR findings and treatment.

Rare variant of persistent primitive hypoglossal artery, arising from the external carotid artery.

A 63-year-old man presented with an extremely rare variant of persistent primitive hypoglossal artery (PHA), which was found incidentally during examination for a contralateral asymptomatic internal carotid artery (ICA) stenosis. This anastomotic vessel arose from the external carotid artery, not the ICA, and joined the vertebrobasilar artery through the hypoglossal canal. Persistent PHA is rare and the reported incidence is 0.027-0.26%. Recognition of the existence of this variant vessel and preservation during neuroradiologic intervention or surgery is important to prevent possible ischemic complications.

[Two cases of spontaneous intracranial hypotension with bilateral chronic subdural hematomas].

Spontaneous intracranial hypotension (SIH) is characterized by postural headaches without obvious cause and is occasionally related to chronic subdural hematomas (CSHs). Brain sag due to decrease in cerebrospinal fluid, and growing CSHs may occur secondary to a decrease in intracranial pressure. Therapy for this condition differs from that for mere traumatic CSHs and is controversial. We report 2 cases of CSH related to SIH. One patient required drainage of the CSHs and an epidural self-blood patch. Furthermore, this patient had to undergo reoperation for drainage of the CSHs. The other patient was cured after the first time the CSHs were drained.

Treatment selection for ruptured aneurysm and outcomes: clipping or coil embolization.

The International Subarachnoid Aneurysm Trial has shown that coil embolization achieves a better outcome for aneurysms treatable by either clipping or coil embolization. However, many ruptured aneurysms are hardly treatable by either clipping or coil embolization. Selection of either clipping or coil embolization will affect the treatment outcome for ruptured aneurysms. The relationship between patient selection and treatment outcome in a so-called "regional center hospital" in Japan must be clarified. This study included 113 patients with ruptured intracranial saccular aneurysms measuring less than 10 mm. Selection criteria for coil embolization were principally paraclinoid or posterior circulation aneurysm, Hunt and Hess grade IV or over, and patient age 75 years or older. Other aneurysms were principally treated by clipping. Aneurysms with a dome/neck ratio of less than 1.5, distorted aneurysms, Hunt and Hess grades I-III, patient age 74 years or younger, and middle cerebral artery aneurysm were actively treated by clipping. A few exceptional indications were considered in detail. Low invasiveness coil embolization is better than clipping to obtain good neurological outcome for patients with perforators difficult to dissect, aneurysms difficult to dissect due to previous open surgery, and aneurysms requiring bilateral open surgery, despite the slightly higher rebleeding rate in coil embolization. Overall outcomes were modified Rankin Scale (mRS) 0-2 in 82 of 113 patients (73%) and mRS 3-6 in 31 (27%). Appropriate selection of clipping or coil embolization can achieve acceptable treatment outcomes for ruptured aneurysm.

Effects and complications of stereotactic aspiration for spontaneous intracerebral hemorrhage.

Stereotactic aspiration of intracerebral hemorrhage (ICH) improves the general condition of patients, promotes improvement of consciousness, and decreases the incidence of pneumonia, but may induce rebleeding. The present study investigated the effects of stereotactic aspiration and factors that inhibit rebleeding in 70 consecutive patients who underwent stereotactic aspiration for ICH. Consciousness was significantly improved after surgery. Of patients who underwent surgery on day 0 or 1, 5 patients developed pneumonia and 29 patients did not. Of patients who underwent surgery on day 2 or later, 14 patients developed pneumonia and 22 patients did not. Early surgery within 2 days significantly reduced the rate of aspiration pneumonia. Patients with rebleeding tended to have liver dysfunction and hemorrhagic tendency. Early stereotactic aspiration of ICH facilitates better patient management than conservative treatment in patients with moderate consciousness disturbance. Patients with liver dysfunction and hemorrhagic tendency should be identified.

Percutaneous vertebroplasty under three-dimensional radiography guidance. Technical note.

An accurate, safe, and convenient method for performing percutaneous vertebroplasty (PVP) under three-dimensional (3D) radiography guidance has been developed. Six PVP procedures were performed in 5 elderly stroke patients complaining of back pain caused by compressive fracture which interfered with further rehabilitation. T(1)-weighted magnetic resonance imaging showed low intensity and T(2)-weighted imaging showed high intensity in the fractured vertebral body. The most suitable trajectory for passing the lateral mass of the lamina and pedicle, and reaching the vertebral body was identified on the axial view of 3D radiography. A 13-gauge needle was advanced appropriately in all patients, and improvement of back pain was obtained after injection of polymethylmethacrylate. No major complications occurred in any patient. PVP under 3D radiography guidance is an accurate, safe, and convenient method.