Steerable microcatheter for distal access of a giant cavernous carotid artery aneurysm during treatment with Pipeline Embolization Device: A case report and review of the literature.

Background: In the treatment of giant cerebral aneurysms with flow-diverting stents, access to the distal parent artery is critical but occasionally challenging. This article provides our experience with a novel steerable microcatheter in such a situation, as well as a review of the literature. Case Description: A 73-year-old woman presented with right ptosis and external ophthalmoplegia. Magnetic resonance angiography revealed a giant right cavernous internal carotid artery aneurysm. Endovascular treatment was planned with flow diversion, but distal access was not possible using the standard technique. A 2.4-Fr steerable microcatheter, Leonis Mova Selective, was implemented, and by bending the catheter tip toward the distal parent artery, a guidewire could be guided distally. After the catheter exchange, two flow-diverting stents were deployed successfully. Conclusion: Steerable microcatheters may provide an option in treatment with flow-diverting stents for giant cerebral aneurysms where access to the distal parent artery is compromised.

Rupture Risk Factors and Strategies for Unruptured Distal Anterior Cerebral Artery Aneurysms.

BACKGROUND: Distal anterior cerebral artery (dACA) aneurysms are rare. Ruptured dACA aneurysms typically present with subarachnoid hemorrhage in conjunction with intracerebral hematoma and cause neurological deterioration. This study aimed to determine their risk of rupture and examine associated factors. METHODS: We retrospectively analyzed patients with dACA aneurysms to compare patient and aneurysm characteristics between ruptured and unruptured aneurysms. Clinical outcome was used the modified Rankin scale. Univariate analyses were performed to identify rupture risk factors. RESULTS: One hundred three patients with dACA aneurysms were examined (51 ruptured and 52 unruptured). The median aspect ratio of ruptured and unruptured aneurysms was 1.69 and 1.22, respectively (P < 0.01). The median maximum diameter of ruptured and unruptured aneurysms was 5.2 and 3.1 mm, respectively (P < 0.01). The median size ratio of ruptured and unruptured aneurysms was 3.32 and 2.17, respectively (P < 0.01). Maximum diameter was <5 mm in 45.2% of ruptured dACA aneurysms. dACA aneurysm, showing size ratio >2.4 and aspect ratio >1.4, had ruptured in 71.4% and 78.6%, respectively. We suggested that these are the threshold of size ratio and aspect ratio for rupture of dACA aneurysms. A total percentatge of 78.1% of aneurysms with aspect ratio >1.4 and size ratio >2.4 had ruptured. CONCLUSIONS: Distal anterior cerebral artery (dACA) aneurysms may rupture, even when small. We found a significant difference between ruptured and unruptured aneurysms with respect to maximum diameter, aspect ratio, and size ratio. Treatment for small aneurysms should be considered based on size ratio and aspect ratio, not just size.

Mechanical thrombectomy for acute paradoxical cerebral embolism due to pulmonary arteriovenous malformation: A case report and review of literature.

Background: We report a case of acute occlusion of the middle cerebral artery (MCA) due to pulmonary arteriovenous malformation (PAVM), for which mechanical thrombectomy was performed and a good outcome was achieved. Case Description: A 59-year-old woman presented with severe right hemiplegia and dysarthria, and a National Institutes of Health Stroke Scale (NIHSS) score of 10. Magnetic resonance imaging (MRI) diffusion-weighted imaging revealed high-signal areas in the left lenticular nucleus to the corona radiata, and right temporal lobe. Magnetic resonance angiography revealed the left MCA occlusion. Chest plain computed tomography (CT) revealed PAVM in the lower lobe of the left lung. Transvenous thrombolytic therapy was started 2 h after onset, and mechanical cerebral thrombectomy was performed with an aspiration catheter and stent retriever. A red thrombus was retrieved in one pass and complete recanalization was achieved. After treatment, her symptoms improved markedly, but a symptomatic intracerebral hematoma was observed in the contralateral right temporal lobe. The patient underwent embolization for PAVM on day 24 and was transferred for rehabilitation (NIHSS score = 2). Conclusion: Although rare, PAVM is a treatable cause of stroke in relatively young adults; thus, it is important to keep in mind the possibility of its presence.

PulseRider "jack-up" technique for wide-necked basilar tip aneurysms that incorporate parent arteries: A technical note.

Background: Treatment of intracranial wide-necked bifurcation aneurysms remains challenging. We report a novel PulseRider "jack-up" technique for the treatment of wide-necked basilar tip aneurysms. Case Description: We performed coil embolization in three patients with an unruptured wide-necked basilar tip aneurysm. Aneurysm neck diameters ranged between 7.6 and 11 mm and dome-to-neck ratios ranged from 1.0 to 1.15. All three aneurysms were very shallow with aspect ratios of 0.51-0.69 and incorporated both posterior cerebral arteries into the aneurysm wall. Because each was difficult to embolize using the standard PulseRider procedure, the PulseRider was deployed more proximally than usual and a coil frame constructed above it. Then, the coil was pushed or "jacked up" into the aneurysm with the PulseRider. Adequate embolization was achieved in all three patients. Conclusion: Coil embolization using the PulseRider jack-up technique is effective for treatment of wide-necked basilar tip aneurysms that incorporate parent arteries.

Ruptured Distal Middle Cerebral Artery Aneurysms-Characteristics and Strategy.

BACKGROUND: Distal middle cerebral artery (dMCA) aneurysms are very rare. Most ruptured dMCA aneurysms lead to subarachnoid hemorrhage (SAH) coexisting with intracerebral hematoma (ICH), resulting in a deteriorating state. The risk factors of rupture of dMCA aneurysms remain unknown. To elucidate the risk of rupture, we studied differences between 4 ruptured and eleven unruptured dMCA aneurysms. METHODS: We retrospectively analyzed patients diagnosed with a dMCA aneurysm according to the maximum size of the aneurysm, aspect ratio, and aneurysm size to vessel size (size ratio). RESULTS: Four patients presented with SAH coexisting with ICH, resulting in a midline shift, caused by rupture of the dMCA aneurysm. The median aspect ratio of ruptured and unruptured dMCA aneurysms was 2.38 and 1.65, respectively, with no significant difference. The median maximum diameter of ruptured and unruptured dMCA aneurysms was 2.98 and 3.78 mm, respectively, with nonsignificant difference. The median size ratio of ruptured and unruptured dMCA aneurysms was 3.13 and 1.84, respectively, being significant difference (P < 0.02). The outcomes of 4 patients having ruptured dMCA aneurysm were as follows: one patient died due to the initial attack, and the others were severely disabled due to cerebral infarction caused by vasospasm. CONCLUSIONS: Distal MCA aneurysms, even if they are small, may be likely to rupture leading to SAH complicated by ICH and result in a poor neurological state and unfavorable outcomes. We recommend aggressive treatment for dMCA aneurysms considering morphological factors such as the size ratio even if they are small and unruptured.

Pseudo-continuous arterial spin labeling with short post-labeling delay time sensitively reflects the hemodynamics of symptomatic patients with permanent large vessel occlusion before and after revascularization.

BACKGROUND AND PURPOSE: This study evaluated the feasibility of arterial spin labeling (ASL) for diagnosing hemodynamic stroke due to permanent anterior circulation large vessel occlusion. METHODS: Three-dimensional pseudo-continuous ASL data at two post-labeling delay (PLD) times (1.5 and 2.5 s) in patients with unilateral permanent middle cerebral artery (MCA) segment 1 (M1) or internal cerebral artery (ICA) occlusion were acquired during routine magnetic resonance angiography. Sixty-one patients with symptomatic occlusion (M1, 24; ICA, 37) and 69 patients with asymptomatic occlusion (M1, 21; ICA, 48) were enrolled. Regions of interest were automatically placed in the MCA region using a template. The respective scans were compared with asymptomatic M1 or ICA occlusion scans. The ratio of signal intensity (occlusion side/non-occlusion side) in the perfusion area of MCA (asymmetry index [AI]) was compared between both groups. RESULTS: In both PLD groups, AI was significantly lower in symptomatic patients than in asymptomatic patients. The receiver operating characteristic curve showed moderate capacity for the prediction of symptomatic AI in both groups (area under the curve, 0.739 and 0.712, respectively). As a result of extracranial-intracranial bypass operation in 28 symptomatic (M1, eight; ICA, 20) patients, AI was significantly higher postoperatively in the PLD 1.5 s group than in the PLD 2.5 s group. CONCLUSION: In symptomatic patients with permanent large vessel occlusion, the signal intensity ratio of pseudo-continuous ASL with short PLD sensitively reflects the hemodynamics before and after revascularization; therefore, this technique may be an alternative method in situations where PET or SPECT cannot be performed.

A Case of Intracranial Vertebral Artery Dissection Undetected by CT, MRI, and MRA at the Onset of Headache That Caused Subarachnoid Hemorrhage Seven Days Later.

Objective: We report a patient with normal imaging findings at the onset of preceding headache who developed subarachnoid hemorrhage (SAH) due to intracranial vertebral artery dissection 7 days later. Case Presentation: A 51-year-old woman with a history of chronic headache visited our emergency outpatient department with a complaint of mild to moderate right nuchal pain. CT, MRA, and MRI (diffusion-weighted image, T2-weighted image, FLAIR, MR cisternography, and basi-parallel anatomical scanning) were normal. Seven days later, she was admitted to our hospital with sudden disturbance of consciousness. CT revealed SAH and CTA demonstrated dilatation of the right vertebral artery (VA). The dilated lesion with an intimal flap on the right VA proximal to the posterior inferior cerebellar artery was confirmed on DSA. The dilated lesion and the proximal VA were occluded endovascularly using coils. The condition of the patient improved gradually, and she was transferred to the rehabilitation hospital on day 45 with a modified Rankin Scale score of 2. Conclusion: The clinical course of the presented case, although rare, should be kept in mind in daily clinical practice.

Non-Sinus-Type Laterocavernous Sinus Dural Arteriovenous Fistula Treated by Transarterial Venous Coil Embolization: A Case Report.

Objective: Laterocavernous sinus dural arteriovenous fistulas (DAVFs) are rare and not always accessible transvenously due to their angioarchitecture. We report a case of non-sinus-type laterocavernous sinus DAVF treated by endovascular transarterial venous coil embolization. Case Presentation: A 78-year-old woman was admitted to our hospital with loss of consciousness, right hemiparesis, and motor aphasia. CT demonstrated intracerebral hematoma in the left frontal lobe and subarachnoid hemorrhage. On CTA and MRA, a DAVF was found in the left laterocavernous sinus region associated with the accessory meningeal artery (AMA) and draining directly into the superficial middle cerebral vein. The diagnosis was confirmed by DSA, which revealed a DAVF fed by the large and straight AMA and the internal carotid artery's meningohypophyseal trunk. Endovascular transarterial venous coil embolization was performed through the AMA. A microcatheter was advanced beyond the shunt point into the origin of the draining vein, and coils were placed in the venous and arterial sides of the fistula. The fistula was completely occluded, and 15-month follow-up angiography demonstrated stable obliteration of the fistula. Conclusion: Transarterial venous coil embolization may be a treatment option for non-sinus-type laterocavernous sinus DAVF with a large fistula size and a large and straight feeding artery.

Endovascular Treatment for Vertebrobasilar Artery Tandem Occlusion Beyond 24 Hours From Onset of Stroke-Case Report.

We report a case in which endovascular treatment (EVT) was performed for vertebral and basilar artery (VA and BA) tandem occlusion beyond 24 hours from onset of stroke. A 78-year-old man was admitted to our institution with dysarthria and disturbance of gait. MRI revealed occlusion of the BA with acute ischemic change in bilateral cerebellum and brain stem. At 36 hours after onset and 30 hours after administration, EVT was performed because of deteriorating neurological symptom. Successful revascularization was achieved with percutaneous transluminal angioplasty for VA and thrombectomy for BA occlusion. The neurological symptoms were improved in postoperative course.

Outcome and effect of endovascular treatment in stroke associated with acute extracranial internal carotid artery occlusion: Single-center experience in Japan.

BACKGROUND: Positive data from several randomized controlled trials (RCTs) of endovascular treatment (EVT) for acute ischemic stroke (AIS) patients with anterior circulation large vessel occlusion (ACLVO) have emerged. However, little evidence exists on EVT for acute extracranial internal carotid artery (EC-ICA) occlusion. We therefore analysed the outcome and effect of EVT on AIS due to ACLVO caused by EC-ICA occlusion, including tandem occlusion compared with that caused by pure intracranial artery occlusion. METHODS: A total of 135 consecutive AIS patients with ACLVO between July 2014 and December 2017 were identified. We retrospectively analysed the efficacy of EVT for ACLVO after introducing a stent retriever (SR). We classified ACLVO into the following categories: group A, intracranial artery occlusion without EC-ICA occlusion (pure intracranial artery occlusion), and group B, ipsilateral EC-ICA occlusion with/without intracranial artery occlusion. RESULTS: In total, 65 patients were enrolled. Group A comprised 71% (46/65) of all cases. No difference was observed in terms of age, National Institute of Health Stroke Scale (NIHSS) score, Alberta Stroke Program Early Computed Tomography Score-Diffusion Weighted imaging (ASPECTS-DWI), several clinical time intervals, rate of successful revascularization (74% versus 84%), and rate of functional independence (42% versus 39%) between groups A and B. In all patients, an ASPECTS-DWI ≥6 and an onset-to-door time ≤6 h were associated with good outcome, whereas intracranial artery occlusion without EC-ICA occlusion (pure intracranial artery occlusion) was not. CONCLUSIONS: The outcomes support the efficacy of EVT in stroke associated with acute EC-ICA occlusion. In the EVT of AIS due to ACLVO, there was no significant difference in the results between ipsilateral EC-ICA occlusion with/without intracranial artery occlusion and intracranial artery occlusion without EC-ICA occlusion (pure intracranial artery occlusion).

Periventricular small cystic lesions in a patient with Coffin-Lowry syndrome who exhibited a novel mutation in the RPS6KA3 gene.

BACKGROUND: Coffin-Lowry syndrome is a rare X-linked disease, caused by loss-of-function mutations in the RPS6KA3 gene. Patients exhibit severe intellectual disability with characteristic dysmorphism. As there are no specific laboratory findings to support the diagnosis of Coffin-Lowry syndrome, it may be difficult to diagnose-especially in young children, where the characteristic craniofacial features are less discernible. CASE: Here we report on a 2-year-old boy with Coffin-Lowry syndrome with a novel missense mutation in the RPS6KA3 gene. On magnetic resonance imaging, his brain exhibited periventricular signal abnormalities with multiple small cystic lesions. These findings may aid in diagnosis of Coffin-Lowry syndrome.

[Successful Treatment of Repeated Bilateral Middle Cerebral Artery Occlusion by Performing Mechanical Thrombectomy in a Patient with Trousseau Syndrome].

We report a patient with Trousseau syndrome who presented with repeated acute middle cerebral artery (MCA) occlusion, which was successfully treated by performing mechanical thrombectomy. A 66-year-old man with a lung lesion and abdominal lymph node swelling experienced a sudden onset of left hemiparesis. Magnetic resonance angiography (MRA) revealed a right MCA occlusion. Perfusion-weighted imaging revealed reduced cerebral blood flow in the right MCA territory. Complete recanalization of the occluded vessel was achieved by performing mechanical thrombectomy, and his symptoms remarkably disappeared. Twenty days after the procedure, he experienced right hemiparesis and total aphasia. MRA revealed a left MCA occlusion, which was located on the contralateral side of the first occlusion. The second mechanical thrombectomy was successfully performed, and complete recanalization was successfully achieved. Right hemiparesis improved after the procedure. Histological examination of the retrieved clots revealed coagulated fibrin and platelets. Cytodiagnosis of pleural effusion revealed adenocarcinoma, and he was diagnosed with lung adenocarcinoma and cancerous pleurisy. Trousseau syndrome was the presumed primary cause of the patient's tendency for thrombogenesis. To the best of our knowledge, there were no reports regarding the repeated use of mechanical thrombectomy for patients with bilateral MCA occlusion caused by Trousseau syndrome. Neuroendovascular therapy can offer good neurological outcomes even in patients with malignant lesions.

Rituximab ameliorates anti-N-methyl-D-aspartate receptor encephalitis by removal of short-lived plasmablasts.

We measured anti-N-methyl-D-aspartate receptor (NMDAR) autoantibody levels and assessed B cell subsets using multicolor flow cytometry of peripheral blood mononuclear cells (PBMCs) from a recurrent anti-NMDAR encephalitis case to evaluate the effectiveness of rituximab treatment. Rituximab depleted CD20(+) fractions of naïve and memory B cell subsets and reduced the number of CD20(-) plasmablasts. This study suggests that short-lived plasmablasts are removed by rituximab-induced depletion of the CD20(+) B cell population. Increased numbers of plasmablasts in PBMCs may be a candidate predictive factor for unfavorable prognosis of anti-NMDAR encephalitis and an indication of when to commence second-line immunotherapy.

Autoimmune neurological disorders associated with group-A beta-hemolytic streptococcal infection.

Although central nervous system (CNS) disorders associated with group-A beta-hemolytic streptococcal (GABHS) infection occur only rarely, Sydenham's chorea is a well-recognized disease that can arise following infection. Children may develop a tic, obsessive compulsive disorder (OCD), and extrapyramidal movement subsequent to GABHS infection. These disorders have been termed pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS). Herein we report one case each of acute disseminated encephalomyelitis (ADEM), PANDAS and subacute encephalitis associated with GABHS infection. To evaluate the pathogenesis of the CNS disorders associated with GABHS infection, we measured levels of neurotransmitters, cytokines, anti-neuronal autoantibodies, and performed immunohistochemistry using patient sera to stain human brain sections. All three cases showed psychiatric behavioral disorders. Immunotherapy was effective, and homovanillic acid levels in the cerebrospinal fluid (CSF) were elevated at the acute stage in all three cases. In each case of ADEM and PANDAS, immunohistochemistry demonstrated neuronal impairment in the basal ganglia during the acute stage. Neuronal immunoreactivity was visualized in the cerebral cortex at the acute stage in the case of subacute encephalitis. There was no direct correlation between immunoreactivity of patient sera on the brain sections and positivity of anti-neuronal autoantibodies or CSF biomarkers. The results suggest that autoimmune responses may modulate neurotransmission, and the use of patient serum for immunohistochemistry is a sensitive screening method for the detection of anti-neuronal autoantibodies in CNS disorders associated with GABHS infection.

[Two cases of inoperable advanced bileduct cancer treated effectively with a gemcitabine/cisplatin combination].

We report two cases of inoperable advanced bileduct cancer that responded well to combined gemcitabine (GEM)/cisplatin (CDDP) therapy. A 70-year-old woman was admitted to the hospital because of jaundice; PTCD was performed, followed by laparotomy, however, the tumor was found to be inoperable because of extensive direct invasion of the surrounding organs. Then, after insertion of a stent, the patient was started on combined chemotherapy with GEM 1,000 mg/m2 on days 1, 8 and CDDP 70 mg/m2 on day 1 administered once every 3 weeks. After 4 courses, thrombocytopenia appeared. Therefore, chemotherapy was continued for another three courses with GEM 800 mg/m2 on days 1, 15 and CDDP 50 mg/m2 on day 1 every 4 weeks. After 7 courses the serum CEA level returned to normal range, and marked reduction of the tumor size was observed on CT. The patient has been able to maintain a good quality of life without occurrence of any severe adverse effects. A 61-year-old woman was admitted to the hospital because of icterus, and was diagnosed to have lower bile duct cancer with liver metastasis. After insertion of a stent, she was started on combined chemotherapy with GEM 1,000 mg/m2 on days 1, 8 and CDDP 70 mg/m2 on day 1 administered once every 3 weeks. After completion of 4 courses, the patient developed thrombocytopenia and a feel- ing of dullness. Therefore, chemotherapy was continued for the next three courses with GEM 800 mg/m2 on days 1, 15 and CDDP 50 mg/m2 on day 1, administered every 4 weeks. This treatment was associated with a fall of the serum CA19-9 level to within normal range, and marked improvement of the liver metastasis was also noted. Combined GEM/ CDDP therapy improves the QOL of inoperable bile duct cancer patients and offers promise as pre-operative chemotherapy.

Regeneration of peritoneum using amniotic membrane to prevent postoperative adhesions.

BACKGROUND/AIMS: Adhesions following intraperitoneal surgery are frequent causes of small bowel obstruction. Attempts to prevent postoperative adhesions have mostly proven disappointing clinically. Currently used by ophthalmologists in ocular surface disorders, amniotic membrane transplantation can reduce inflammation and promote re-epithelization. We used amniotic membrane for facilitating peritoneal regeneration and prevention of adhesions with surgical trauma. METHODOLOGY: 20 rats were randomized in equal number into treatment or control groups. Seven days after operation, the incidence and severity of adhesions were evaluated. Histologic and immunohistochemical analyses were examined at 1, 4, 10 weeks after operation. RESULTS: While severe adhesions were observed after 1 week between the cecum and surrounding organs in the control group, adhesion formation was significantly reduced in the amniotic membrane group. Histologic examination demonstrated that free-floating myofibroblasts in the peritoneal cavity attached to surfaces of amniotic membrane grafts to form a layered structure. Free-floating mesothelial cells were incorporated into the regenerating mesothelium on the myofibroblast layer in 4 weeks, while implanted amniotic membrane grafts were absorbed by 10 weeks. In the amniotic membrane group the cecum appeared nearly normal. CONCLUSIONS: Amniotic membrane grafts reduced intraperitoneal adhesions after surgical trauma, were well absorbed, and served as a substrate for regenerating mesothelium.

[Effects of piracetam therapy in a case of Lance-Adams syndrome].

We report a 17-year-old female patient with Lance-Adams syndrome caused by anoxic encephalopathy during a severe attack of bronchial asthma. She had difficulty in writing because of action myoclonus in her arms. She also exhibited freezing gait and was unable to walk without cane. Although her gait disturbance resembled those seen in patients with parkinsonism secondary to anoxic encephalopathy, surface electromyography revealed that it was caused by action myoclonus in her legs. The presence of giant somatosensory evoked potentials and enhanced cortical reflexes in response to the electrical stimulation to her posterior tibial nerves supported our diagnosis. A combined therapy with valproate sodium, clonazepam and piracetam (15 g/day) was not effective. However, her freezing gait remarkably improved and she was able to walk without help, after the treatment with sufficient dose of piracetam (21 g/day). Cortical hyperexcitability as revealed by electrophysiological examination also improved. We concluded that the combined therapy with antiepileptic drugs and piracetam was effective in the treatment for action myoclonus. However, because the effects seemed dose-related, the dosage of piracetam needed to be increased until the optimum effects were obtained.

Hemorrhage due to tracheoarterial fistula with severe motor and intellectual disability.

BACKGROUND: Tracheoarterial fistula (TAF) is an unusual but highly lethal complication of tracheostomy, and successful surgical intervention for TAF has been reported. Few investigations are available for TAF in severe motor and intellectual disability (SMID). The aim of the present paper was to analyzed TAF in SMID to clarify which clinical variables might predict the occurrence of TAF, and adequate management for lifesaving. METHODS: Medical records at Metropolitan Fuchu Medical Center were retrospectively investigated for SMID between 1970 and 2000, and 10 TAF patients verified on operation or autopsy were identified. Details were reviewed including clinical status, emergency treatment at the occurrence of TAF, and operation and/or autopsy recordings. RESULTS: Four of 10 patients underwent successful operation and survived, while the other six died from hemorrhagic shock. Eight patients had tracheoinnominate artery fistula, the others had tracheocarotid artery fistula. Characteristic features as SMID such as etiology of brain disease, muscle tonus and convulsion were no apparent relevance to occurrence of TAF. All patients suffered from endotracheal granuloma extending to the arterial walls. Seven of 10 patients had re-bleeding after stabilization of the first massive hemorrhage, especially fiber bronchoscopy to confirm the diagnosis of TAF precipitated to fatal re-bleeding. One patient underwent interruption of the artery at relapse of TAF, the other three underwent suturing and had good outcome. CONCLUSIONS: There were no apparent predictors of TAF in SMID. Tracheal granuloma was recognized and consequent on formation of TAF, so control of granuloma may prevent TAF. Fiber bronchoscopy for suspected TAF is not recommended because it precipitates fatal bleeding.