Masayuki Ezura

Clinical and angiographic follow-up of childhood-onset moyamoya disease

To clarify the differences between childhood-onset moyamoya disease and that with onset in adulthood, we studied the clinical course and angiographic findings of adult patients (over 20 years of age) with moyamoya disease of childhood onset (up to 15 years of age). The clinical course in 25 patients could be assessed. The follow-up period was 5–27 years. Neurological deficits were noted in 11/23 and mental disorders in 9/21. In all except one, the illness had started before the age of 7 years. Neither neurological nor mental condition changed during or after adolescence (15–20 years of age). Two patients died of intracranial hemorrhage. The disease progressed in angiographic stage until adolescence, but had stabilized or almost stabilized by the age of 20 years. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiographic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.

Selective and superselective infusion of urokinase for embolic stroke

Intraarterial administration of urokinase using Tracker microcatheter was performed in 11 patients with acute cerebral infarction caused by embolic occlusion of the internal carotid or the middle cerebral artery. Recanalization was observed in seven cases (64%) following the fibrinolytic therapy, and the time until recanalization from the start of the treatment was on the average 2.8 hours. Recanalization was seen in five out of six cases that received superselective infusion of urokinase, while it was seen in two out of five cases that received selective infusion. This study suggests that superselective infusion of urokinase is an excellent therapeutic method for embolic occlusion of the cerebral artery.

Determinants of Poor Outcome After Aneurysmal Subarachnoid Hemorrhage when both Clipping and Coiling Are Available: Prospective Registry of Subarachnoid Aneurysms Treatment (PRESAT) in Japan

OBJECTIVE To examine current determinants of poor outcome after aneurysmal subarachnoid hemorrhage (SAH) when ruptured aneurysms are treated with either microsurgery (clipping) or endovascular treatment (coiling) depending on each patients characteristics. METHODS Between March 2006 and February 2007, 534 patients with SAH were enrolled in the Prospective Registry of Subarachnoid Aneurysms Treatment (PRESAT) project. Patients were treated according to the preference of investigators who were experienced in performing both clipping and coiling. Factors influencing poor outcome (12-month modified Rankin Scale [mRS], 3-6) were determined using multivariate logistic regression analyses. RESULTS In this cohort, 32.4% of patients were World Federation of Neurosurgical Societies (WFNS) grade IV-V, and 28.1% had a poor outcome. Clipping was preferably performed for small aneurysms with a wide neck and for middle cerebral artery (MCA) aneurysms, whereas coiling was preferred for larger, internal carotid artery (ICA) and posterior circulation aneurysms. In addition to increasing age, admission WFNS grade IV-V, preadmission aneurysmal rerupture, vasospasm-induced cerebral infarct, pneumonia, sepsis, shunt-dependent hydrocephalus and seizure, postclipping hemorrhagic complications (odds ratio 4.8, 95% confidence interval 1.5-15.3, P < 0.01), and postcoiling ischemic complications (odds ratio 4.4, 95% confidence interval 1.3-15.2, P < 0.05) significantly caused poor outcomes, although the complications did not affect mortality. Type of treatment modality and size and location of aneurysms did not influence outcome. CONCLUSIONS Introducing an endovascular treatment option has made aneurysm characteristics less important to outcome, but procedural complications are problematic and should be reduced to improve outcome.

Dissociation of letter and picture naming resulting from callosal disconnection

Background: Detailed mapping of the corpus callosum for functional fractionation in humans remains incomplete. Objective: To examine separable interhemispheric transfer of visual information by callosal fibers, especially in the splenium. Methods: We examined callosal disconnection signs in a 14-year-old boy with a lesion confined to the posterior part of the splenium and reviewed reported cases with callosal lesions. Results and Conclusion: The patient presented with left hemialexia as the only manifestation of callosal disconnection syndrome. The only difficulty demonstrated was in reading aloud or copying letters, which were presented tachistoscopically to the left visual field, with his right hand. He could copy letters presented to his left visual field with his left hand, however. Therefore, left hemialexia was not due to hemiamblyopia or hemineglect. There was no anomia for pictures and colors in the left visual field. MRI revealed that the lesion was limited to the ventroposterior end of the splenium. Review of 40 reported patients with callosal lesions suggests that the anterior to middle part of the splenium is involved in transferring picture information from the language-nondominant hemisphere to the language-dominant hemisphere and that the ventroposterior part is involved in transferring letter information.

Spontaneous disappearance of a huge cerebral arteriovenous malformation : Case report

A case of spontaneous disappearance of a cerebral arteriovenous malformation (AVM) is reported. A 59-year-old woman, who had been diagnosed as having a huge AVM in the left occipital lobe 6 years before and who was monitored without treatment, complained of a sudden headache and vomiting. Computed tomography revealed an acute subdural hematoma, intracerebral hematoma, and subarachnoid hemorrhage, for which a craniotomy was performed. Cerebral angiograms performed 9 days after the operation demonstrated a decrease in the size of the AVM. Repeated cerebral angiograms performed a month later demonstrated complete disappearance of the AVM. Follow-up angiograms performed 19 months after hemorrhage confirmed complete disappearance of the AVM. Spontaneous disappearance is known to occur occasionally in small AVMs but rarely in huge ones such as the one presented here. Several possible mechanisms for spontaneous disappearance of AVMs are discussed.

Intracerebral schwannoma: Case report.

A case of intraparenchymal schwannoma is presented. The neuroradiological findings of reported intraparenchymal schwannomas, including the case reported here, are discussed. The specific tumor characteristics are emphasized. This report appears to be the only documentation as a result of magnetic resonance imaging of this specific tumor.

Evaluation of regional cerebral blood flow using single photon emission tomography for the selection of patients for local fibrinolytic therapy of acute cerebral embolism

To clarify the indications for local fibrinolytic therapy for acute cerebral embolism, correlation among initial regional cerebral blood flow (rCBF) abnormalities, recanalization timing, and computed tomographic scan (CT) findings two days later were evaluated in 17 cases. All cases included had embolic occlusion of middle cerebral or internal carotid arteries which showed no abnormal findings on initial CT corresponding to the acute events but did show abnormal CBF reduction on initial single photon emission computed tomography (SPECT). Relative rCBF was evaluated as the percentage radioisotope counts in the region of interest (ROI) of the affected side against the corresponding ROI in the unaffected contralateral side. Within 6 hours from onset, there was a tendency towards reversed time dependent tolerance of cortical infarction with residual relative rCBE. Hemorrhagic transformation was observed in one case whose relative rCBF was 35% or less. In 10 patients whose occluded arteries were not recanalized within 6 hours, cortical areas with residual relative rCBF of 70% or more did not develop infarction. In conclusions, a pre-therapeutic rCBF study using SPECT is considered to be mandatory: cases with moderate ischemia involving the cortex with residual relative rCBF of between 35% and 70% may be good candidates for local fibrinolytic therapy.

Combined intravascular parent artery and ophthalmic artery occlusion for giant aneurysms of the supraclinoid internal carotid artery.

BACKGROUND Parent artery occlusion is an effective method to treat internal carotid giant aneurysms. However, incomplete parent artery occlusion may cause revascularization. Here, a modification of the parent artery occlusion procedure for giant aneurysms of the internal carotid artery is described. METHODS Three patients with giant aneurysms of the internal carotid artery at the supraclinoid portion between the origin of the ophthalmic artery and the posterior communicating artery were treated by a combination of parent artery occlusion and occlusion of the origin of the ophthalmic artery. All patients had a giant aneurysm that manifested as cranial nerve palsy due to mass effect. RESULTS A balloon occlusion test of the ipsilateral internal carotid artery together with the ophthalmic artery showed that permanent occlusion was tolerable. It was confirmed that the ipsilateral eye was supplied by maxilloophthalmic anastomosis instead of the ophthalmic artery. Cranial nerve palsy and visual acuity were markedly improved in two cases after the therapy. Follow-up selective internal and external carotid angiography did not demonstrate the aneurysm. CONCLUSIONS Combined parent artery and ophthalmic artery occlusion is an effective treatment for a giant aneurysm located between the ophthalmic artery and the posterior communicating artery.

Fusiform aneurysm of the vertebral artery presenting hemifacial spasm treated by intravascular embolization: Case report

BACKGROUND A rare case of fusiform vertebral artery aneurysm causing hemifacial spasm was successfully treated by intravascular embolization. CASE DESCRIPTION A 53-year-old man presented with left hemifacial spasm persisting for 2 years. No other clinical symptoms were observed. Vertebral angiography revealed a fusiform aneurysm of the left vertebral artery, and thin-slice spoiled gradient-recalled acquisition magnetic resonance imaging demonstrated the aneurysm compressing the root exit zone of the left facial nerve. The parent artery was occluded together with the aneurysm by intravascular embolization with Guglielmi detachable coils (GDCs). The patient is free of left hemifacial spasm without any complication. CONCLUSION Hemifacial spasm caused by aneurysms, especially fusiform aneurysms, is quite rare but can be treated by parent artery occlusion and coiling the aneurysm with GDCs.

Intra-aneurysmal GDC embolization followed by intrathecal tPA administration for poor-grade basilar tip aneurysm.

BACKGROUND This 42-year-old male presented with subarachnoid hemorrhage of Hunt and Kosnik Grade IV, complicated by neurogenic pulmonary edema, prolongation of the electrocardiographic Q-Q interval, and acute renal failure. METHODS Surgical clipping was not indicated, so intra-aneurysmal embolization using Guglielmi detachable coils (GDCs) was performed followed by intrathecal infusion of tissue-type plasminogen activator (tPA) via spinal drainage. RESULTS The patient made a complete recovery 2 1/2 months later except for partial third cranial nerve palsy. CONCLUSIONS Intra-aneurysmal GDC embolization followed by intrathecal tPA via spinal drainage is an excellent method for treating aneurysms that are difficult to treat surgically.