Management for a patient of moyamoya disease presenting with ischemic stroke in the first trimester of pregnancy.

We report an extremely rare case of a 27-year-old woman presenting with ischemic stroke as an initial manifestation of moyamoya disease in the first trimester of pregnancy. We conducted an artificial abortion when her neurological symptoms rapidly became refractory to optimal antithrombotic treatments. The progression of neurologic deficits stopped immediately after abortion, resulting in recovery to independence, with slight motor aphasia and right hemiparesis due to improved cerebral flow. We highlight rapid artificial abortion combined with antithrombotic treatment for patients of moyamoya disease with pregnancy-associated ischemic stroke as an appropriate treatment to correct hemodynamic instability and suppress the progression of neurological symptoms.

[A Case of Right Frontal Dermoid Cyst with Temporal Imaging Changes in the Desease Course].

Dermoid cysts are rare benign intracranial neoplasms derived from embryonal remnant tissues. Here, we describe a case of dermoid cyst located in the right frontal lobe, which showed repeated changes on CT. An 11-year-old girl was referred to our hospital to treat a brain neoplasm. Brain CT and MRI revealed a cystic tumor in the right frontal lobe. Incidentally, brain CT had been performed 6, 2, and 1 year before the presentation, which demonstrated repeated changes in the tumor over the clinical course. Gross total resection of the tumor was achieved through right frontal craniotomy. Histological findings revealed keratin flakes, mature bones, cholesterol crystals, and granulation with macrophages. The cyst wall was composed of squamous epithelium with adnexal structures, such as hair follicles and sebaceous glands. Therefore, we diagnosed the tumor as a dermoid cyst. The postoperative course was uneventful, and she was discharged on postoperative day 10 without neurological deficits. Dermoid cysts are difficult to be diagnosed on routine neuroimaging. An accurate diagnosis requires details of the clinical course and analysis of both imaging and pathological studies.

[Postoperative Neurological Deficit due to Transient Hypoperfusion in the Pretreated Contralateral Hemisphere after Bypass Surgery in an Adult Patient with Moyamoya Disease:A Case Report].

Postoperative neurological deficits frequently occur in adult moyamoya disease. In this case report, we describe the time course and disease state of a patient with adult moyamoya disease, who experienced a postoperative neurological deficit due to transient hypoperfusion in the pretreated contralateral hemisphere. A 68-year-old female presented with a sudden onset of left hemianopia due to ischemic moyamoya disease. She had severely low cerebral blood flow(CBF)in the right hemisphere and deterioration of cerebrovascular reactivity in the left hemisphere. First, right combined bypass surgery was performed; subsequently, left combined bypass surgery was performed. Two days after left hemisphere surgery, left hemiparesis gradually appeared. Hypoperfusion of the right hemisphere and hyperperfusion of the left hemisphere were revealed by SPECT and CT perfusion imaging. Blood pressure was controlled to normal levels, and an antiepileptic drug, antiplatelet drug, and edaravone were administered. The patient gradually recovered 2 weeks later, and was able to resume her normal daily life. During the clinical course, laterality of CBF was improved following improvement of clinical symptoms. Abnormal postoperative disproportion of the CBF may occur after revascularization surgery for adult moyamoya disease. Prompt assessment of CBF and proper treatment are needed.

[A Case of Ruptured Distal Anterior Choroidal Artery Aneurysm Associated with a Twig-Like Middle Cerebral Artery, Treated with Single-Stage Aneurysm Clipping and STA-MCA Double Anastomoses in the Acute Phase].

We report the case of a patient who has progressed well over 5 years following single-stage aneurysm clipping and superficial temporal artery-middle cerebral artery(STA-MCA)double anastomoses in the acute phase, for a ruptured distal anterior choroidal artery(AChA)aneurysm accompanied by a twig-like MCA. The patient was a 49-year-old female who developed a sudden severe headache and disturbance of consciousness due to subarachnoid hemorrhage and intraventricular hemorrhage(IVH). Cerebral angiography showed a right twig-like MCA associated with an abnormal vascular network and a ruptured aneurysm in the distal AChA. A day after emergency ventricular drainage for acute hydrocephalus, right frontotemporal craniotomy enabled distal AChA aneurysm clipping, together with removal of the IVH via transchoroidal fissure approach, in addition to STA-MCA double anastomoses to prevent recurrence of hemorrhage. The IVH resolved after surgery and no new infarct area was observed. Cerebral angiography revealed the disappearance of the aneurysm, good patency of the double bypass, and reduction of the abnormal vascular network. The patient gradually recovered without any neurological deficits, except for mild memory disturbance. Five years after the surgery, the patient has experienced no recurrence. The single-stage operation of aneurysm clipping and STA-MCA double anastomoses was made possible by devising an approach for a ruptured cerebral aneurysm, even in the acute stage. The successful improvement of cerebral circulation and prevention of cerebral hemorrhage from an early stage could serve as a reference for the treatment of similar hemorrhagic cases.

[Ruptured C2 Aneurysm of the Internal Carotid Artery with Ipsilateral Unruptured Giant Internal Carotid Artery Aneurysm:A Case Report].

A giant intracranial internal carotid artery(ICA)aneurysm is difficult to treat. We encountered a case with an unruptured giant intracranial ICA aneurysm with a ruptured C2 aneurysm of the ipsilateral ICA. In this case report, we describe how the treatment and review the literature. A 59-year-old man presented with sudden-onset headache and vomiting. He was diagnosed at a local neurosurgical unit as having a subarachnoid hemorrhage and admitted to our hospital. Cerebral angiogram revealed an unruptured giant ICA C3-4 aneurysm with a ruptured aneurysm of the ipsilateral ICA C2. The ruptured aneurysm had a wide neck and was located in the medial wall of the ICA C2;it was difficult to perform neck clipping or coil embolization. We performed a high-flow bypass using a radial artery graft followed by trapping of the left ICA. Postoperative imaging showed no new ischemic damage, and the patency of bypass flow was good. The left ophthalmic artery was perfused from the external carotid artery. Although the patient experienced incomplete left oculomotor nerve palsy and left lower quadrantanopsia, his neurological function had recovered within a year, and he was able to resume his previous work. High-flow bypass with ICA trapping is effective for ICA giant aneurysm. Attention to postoperative ophthalmic function and oculomotor symptoms is important.

Efficacy of Early Superficial Temporal Artery-Middle Cerebral Artery Double Anastomoses for Atherosclerotic Occlusion in Patients with Progressing Stroke.

BACKGROUND: We investigated the efficacy of early superficial temporal artery-middle cerebral artery (STA-MCA) double anastomoses for patients with progressing stroke due to atherosclerotic occlusion. MATERIALS AND METHODS: Nine consecutive patients who underwent early STA-MCA double anastomoses were enrolled. All patients presented with progressing stroke despite maximal medical treatment. Cerebral blood flow in 7 patients was analyzed by single-photon emission tomography. Clinical outcomes were investigated postoperatively, and we evaluated the utility of early STA-MCA double anastomoses. RESULTS: Nine patients in the present study included those with middle cerebral artery occlusion (n = 6) and internal carotid artery occlusion (n = 3). The mean age was 58.4 years. Subjects comprised 1 female (11.1%) and 8 males (88.9%). The cause was low perfusion ischemia due to atherosclerotic occlusion with a small infarct. The mean regional cerebral blood flow (rCBF) ratio in the middle cerebral artery territory compared to the normal side was 69.6 ± 5.3%. The duration from onset to surgery was 1-8 days (median, 3.11 days). All patients underwent early STA-MCA double anastomoses, and no reperfusion-induced hemorrhage occurred. All of them slowly achieved obvious remission compared to symptoms on admission and achieved a good functional outcome. CONCLUSIONS: Early STA-MCA double anastomoses were safe and effective, and early revascularization resulted in rapid neurological improvement. We recommend this procedure for patients with progressive ischemia due to main trunk artery occlusion, when the rCBF flow ratio with the normal side was 70 ± 10%, even at the subacute stage.

Usefulness of cervical computed tomography and magnetic resonance imaging for rapid diagnosis of crowned dens syndrome: A case report and review of the literature.

INTRODUCTION: Crowned dens syndrome is a rare disease entity which radiologically shows calcification of the cruciform ligament around the odontoid process. We report a patient with crowned dens syndrome who improved dramatically in 5days following treatment with oral nonsteroidal anti-inflammatory medication. PRESENTATION OF CASE: A 61-year-old man was admitted to our hospital with a severe occipital headache and sudden onset of neck stiffness. Neurological examination on admission revealed a high fever and cervical rigidity. Laboratory examination revealed a markedly elevated white blood cell count and C-reactive protein level, but cerebrospinal fluid studies revealed only a slight abnormality. A cervical computed tomography scan and its three-dimensional reconstruction detected a remarkable crown-like calcification surrounding the odontoid process. Cervical magnetic resonance imaging did not demonstrate strong direct compression of the cervical cord; however, the soft tissue surrounding the odontoid process was hyperintense on T2-weighted imaging with fat suppression. Based on the radiological findings, the patient was diagnosed with crowned dens syndrome and was immediately treated with non-steroidal anti-inflammatory drugs. The patient's condition drastically improved within 5days. DISCUSSION: It was very interesting that the soft tissue surrounding the odontoid process was hyperintense on magnetic resonance T2-weighted imaging with fat suppression, and the signal change disappeared 2 weeks after the administration of oral non-steroidal anti-inflammatory drugs. We think that magnetic resonance imaging is useful for proving inflammation in patients with crowned dens syndrome. CONCLUSION: This is the first report making reference to the magnetic resonance imaging findings of crowned dens syndrome.

[Long-term Outcome over Five Years after Surgical Revascularization in Adult Moyamoya Disease].

BACKGROUND AND PURPOSE: The long-term outcome of patients with adult moyamoya disease following revascularization surgery remains unclear. Here, we investigated these outcomes more than five years after revascularization surgery. PATIENTS AND METHODS: This study included 48 cerebral hemispheres from 26 patients who underwent revascularization surgery for adult moyamoya disease. The mean follow-up duration was 14.3(5.5-25.6)years. The risk factors of late-onset hemorrhage after revascularization surgery were compared between the bleeding and non-bleeding groups. We also estimated cumulative bleeding-free survival rates using Kaplan-Meier curves. RESULTS: Four patients experienced late-onset bleeding during the follow-up period. Bleeding occurred after an average of 11(8.5-16.4)years following revascularization surgery. The annual hemorrhagic rate was 1.14%. In the bleeding group, higher Suzuki stage(p=0.02), posterior cerebral artery(PCA)involvement(p<0.01), cerebral aneurysm(p=0.04), microbleeds(p=0.03), and post-operative periventricular anastomosis, especially in the thalamus and the choroidal artery(p<0.01 and p=0.01 respectively), were significantly different from those in the non-bleeding group. Kaplan-Meier analysis showed that the probability of late-onset bleeding was higher with postoperative periventricular anastomosis(p<0.01 for thalamic type, p=0.01 for choroidal type), higher Suzuki stage(p<0.01), PCA involvement(p<0.01), and cerebral aneurysm(p=0.02). CONCLUSIONS: In spite of good perfusion and reduction in moyamoya vessels after revascularization surgery, the risk of bleeding persisted. Periventricular anastomosis, especially in the thalamus and choroidal artery, after surgery, higher Suzuki stage, presence of obstructive lesions in the PCA, and cerebral aneurysm may be associated with late-onset bleeding after surgery. More studies are needed to identify the risk factors for late-onset bleeding after revascularization surgery for moyamoya disease.

Importance of perioperative management for emergency carotid artery stenting within 24h after intravenous thrombolysis for acute ischemic stroke: Case report.

INTRODUCTION: We report a patient treated successfully via endovascular surgery within 24h after intravenous thrombolysis using recombinant tissue plasminogen activator for acute cervical internal carotid artery occlusion. PRESENTATION OF CASE: A 68-year-old man was admitted to our hospital. Neurological examination revealed severe left-sided motor weakness. Magnetic resonance imaging showed no cerebral infarction, but magnetic resonance angiography revealed complete occlusion of the right internal carotid artery. Systemic intravenous injection of recombinant tissue plasminogen activator was performed within 4h after the onset. But, magnetic resonance angiography still revealed complete occlusion. Revascularization of the right cervical internal carotid artery was performed via endovascular surgery. The occluded artery was successfully recanalized using the Penumbra System(®) and stent placement at the origin of the internal carotid artery. Immediately after surgery, dual antiplatelet therapy (aspirin and clopidogrel) was initiated, and then cilostazol was added on the following day. Carotid ultrasonography and three-dimensional computed tomographic angiography at 14days revealed no further obstruction to flow. DISCUSSION: When trying to perform emergency carotid artery stenting within 24h after intravenous recombinant tissue plasminogen activator administration, several issues require attention, such as the decisions regarding the type of stent and embolic protection device, the selection of antiplatelet therapy and the methods of preventing hyperperfusion syndrome. CONCLUSION: Emergency carotid artery stenting for the acute internal carotid artery occlusion may be considered a safe procedure in preventing early stroke recurrence in selected patients.

[A Case of ECA-MCA Double Anastomoses for Hemorrhagic Type of Twig-Like MCA].

Herein, we describe the case of a superficial temporal artery(STA)- and occipital artery (OA)-middle cerebral artery (MCA) double anastomoses that we performed to treat a hemorrhagic twig-like MCA. A 55-year-old man presented to our hospital for investigation of an incidentally identified left MCA occlusion. Left cerebral angiography revealed a twig-like MCA. The (123)I-IMP-single photon emission computed tomography( SPECT) demonstrated no reduction in the cerebral blood flow (CBF), so the patient was initially observed with no treatment. Three months later, he was readmitted with a disturbance of consciousness. The cranial computed tomography revealed a subcortical hemorrhage in the left frontotemporal region and a subdural hematoma. The hematoma was removed via emergency craniotomy. The abnormal vessels were identified and resected, but the parietal branch of the STA was damaged during the skin incision. The histological examination did not reveal the marked fibrous thickening of the intima or wavy internal elastic lamina typically seen with Moyamoya disease. Six months after the initial surgery, a STA-MCA bypass surgery was planned to prevent a future hemorrhage by reducing the hemodynamic overload of the twig-like MCA and improving the cerebral ischemia in the MCA territory that was detected on the preoperative SPECT. However, the parietal branch of the left STA had been injured during the first operation, so we performed a double anastomoses to the MCA using the frontal branch of the STA and the OA. The patient's postoperative course was uneventful and he was discharged 2 weeks after surgery. The follow-up study performed 1 year postoperatively demonstrated no evidence of cerebral infarction and revealed an improvement in the resting CBF and vascular reactivity in the left cerebral hemisphere. No subsequent cerebrovascular events have occurred in this patient during the 7 years since the double anastomoses surgery. Direct bypass for flow conversion from the internal carotid artery to the external carotid artery can be an indispensable treatment for patients with a hemorrhagic twig-like MCA.

[A Case of Ruptured Peripheral Cerebral Aneurysm at Abnormal Vessels Associated with Middle Cerebral Artery Stenosis:Similarity to Moyamoya Disease].

We report a case of ruptured peripheral cerebral aneurysm at abnormal vessels associated with severe stenosis at the middle cerebral artery (MCA). A 66-year-old woman was admitted at our hospital with headache on foot. Computed tomography (CT) showed intracerebral hemorrhage in the left fronto-basal area. Three-dimensional-CT and conventional angiogram revealed abnormal vessels, which were similar to those seen in moyamoya disease, with a small enhancement close to the hematoma. On day 11, subsequent cerebral angiogram demonstrated an aneurysm at the peripheral portion of an abnormal vessel arising from the left A2. On day 17, soon after the diagnosis of the ruptured aneurysm was made (while still at the subacute stage), we operated on the aneurysm. Superficial temporal artery (STA)-MCA anastomosis was also performed to preserve cerebral blood flow and reduce hemodynamic stress. Several days after the operation, she had transient aphasia due to hyperperfusion of the MCA territory, but eventually recovered with no neurological deficit at discharge. Follow-up study revealed revascularization from the branches of the external carotid artery as well as the STA. On admission, we initially thought that this patient had abnormal vessels associated with arteriosclerotic MCA stenosis. However, the postoperative clinical course as well as the histopathological specimens of both the abnormal artery with the aneurysm and the STA revealed similar findings to those of moyamoya disease. Although this case did not satisfy the criteria for moyamoya disease, it is conceivable that a single arterial occlusive lesion associated with moyamoya-like vessels might develop in the same mechanism with that of moyamoya disease.

[Hemorrhagic Adult Unilateral Moyamoya Disease with Multiple Unruptured Intracranial Aneurysms: A Case Report].

Adult unilateral moyamoya disease with intracranial aneurysm is frequently reported in the literature, but there is much variation in its treatment. In this case report, we describe the time course and treatment regimen of a patient with moyamoya disease and review the literature regarding moyamoya disease with intracranial aneurysm. A 64-year-old man had untreated intracranial aneurysm and unilateral moyamoya disease for 10 years. He presented with sudden-onset right hemiparesis and aphasia due to a subcortical hemorrhage. He was admitted to the local neurosurgical unit, and upon resolution of symptoms, he was admitted to our hospital. A cerebral angiogram revealed the champagne bottleneck sign of the left carotid artery and obliteration of the top of the left intracranial carotid artery with a moyamoya phenomenon. Two unruptured intracranial aneurysms were identified in the anterior communicating artery(Acom A) and the right intracranial carotid artery(C3). We performed superficial temporal artery-middle cerebral artery anastomosis followed by aneurysmal neck clipping of the Acom A aneurysm. Postoperative imaging showed no new ischemic damage and improved cerebral blood flow. Although the patient experienced temporal worsening of aphasia, his function recovered a few months later and he was able to resume his normal daily life activities. The combination of direct bypass surgery and aneurysmal neck clipping might be a therapeutic option for hemorrhagic unilateral moyamoya disease with unruptured intracranial aneurysm.

[Ruptured giant fusiform anterior cerebral artery aneurysm treated by proximal clipping with a3-a3 bypass:case report].

Giant fusiform aneurysm of the anterior cerebral artery (ACA) involving the anterior communicating artery (AcomA) is difficult to treat, and its postoperative course is not well known. We treated a patient with subarachnoid hemorrhage (SAH) due to rupture of a giant fusiform aneurysm of the ACA involving the AcomA. Here we describe its treatment and postoperative course, and review the literature regarding ACA involving an AcomA aneurysm that was treated with bypass surgery. A 65-year-old man presented with sudden onset of headache and vomiting. Computed tomography (CT) revealed SAH due to rupture of a giant fusiform ACA involving an AcomA aneurysm. He was admitted to the local neurosurgical unit 5 days after symptom onset. After waiting for vasospasm to resolve, he was transferred to our hospital. Three-dimensional CT demonstrated giant fusiform dilatation (25mm in diameter) of the left ACA (A1-A2), and the AcomA was involved. The perforating branches around the aneurysm were not identified. We performed proximal clipping with A3-A3 bypass for protection of reversal flow to the perforating branches of the left ACA. Postoperative magnetic resonance imaging showed a small infarction in part of the left caudate nucleus and the fornix. Cerebral angiogram revealed complete obliteration of the aneurysm. The patient did not experience palsy or aphasia, but he suffered from transient disturbance of frontal lobe function. A few months later, his cognitive function had recovered, and he was able to resume his normal daily life. Although clinicians should monitor for the development of postoperative neurological symptoms, including cognitive dysfunction proximal clipping with A3-A3 bypass was an effective and reliable treatment for ruptured giant fusiform ACA involving an AcomA aneurysm.

Posttraumatic cerebral infarction due to progressive occlusion of the internal carotid artery after minor head injury in childhood: a case report.

PURPOSE: Although minor head injury in childhood is a common occurrence and usually no complications, posttraumatic cerebral infarction has rarely been reported. Such infarction is characterized by occlusion of the lateral lenticulostriate artery. The authors report an atypical case of posttraumatic occlusion of the internal carotid artery (ICA) after minor head injury in childhood. CASE REPORT: A healthy 16-year-old boy was hit on the head by a pitch while playing baseball. He developed a transient ischemic attack involving the left extremities 15 min after the accident. Initial magnetic resonance imaging revealed neither hemorrhage nor infarction, and MR angiography demonstrated mild stenosis of the right carotid fork. Conservative therapy was started. However, 24 h after the accident, he suddenly developed left hemiparesis. Emergent neuroimaging demonstrated progressive occlusion of the supraclinoid portion of the right ICA and cerebral infarction of the deep white matter in the right frontal lobe. The hemiparesis deteriorated and the infarction area continued to expand on a daily. The patient underwent emergent superficial temporally artery-middle cerebral artery (STA-MCA) bypass. Intraoperative observation demonstrated that the supraclinoid portion of the right ICA was not thrombosed but pale with low tension and did not appear dissected. He fully recovered by 2 weeks after the operation. Postoperative investigations showed gradual improvement of the ICA occlusion. DISCUSSION: Minor head injury can cause cerebral infarction in childhood, although this is rare. If conservative therapy cannot prevent progressive cerebral infarction, STA-MCA bypass should be considered in case of the ICA occlusion.

[Two cases of cervical carotid artery stenosis with high risk post-operative hyperperfusion treated with dexmedetomidine after carotid endarterectomy].

Dexmedetomidine is a central alpha2 adrenoceptor agonist recently shown to be a safe and acceptable sedative agent for patients requiring sedation after brain surgery. We report two patients successfully treated by carotid endarterectomy (CEA) with postoperative management under dexmedetomidine anesthesia for transient ischemic attack (TIA) resulting from severe stenosis of the internal carotid artery (ICA). Case 1: A 75-year-old man was admitted to our hospital with aphasia and weakness of the right side of his body. Although no evidence of acute cerebral infarction was obtained on magnetic resonance imaging (MRI)/diffusion-weighted image (DWI), MR angiography (MRA) revealed severe stenosis of the left cervical ICA. (123)I-IMP-single photon emission tomography (SPECT) and transcranial Doppler (TCD) revealed marked reduction of cerebral blood flow in the left cerebral hemisphere. Although CEA induced hyperperfusion, aggressive control of blood pressure under dexmedetomidine anesthesia enabled effective management of the resulting hyperperfusion syndrome. The patient was discharged without neurological deficits. Case 2: A 68-year-old man was admitted to our hospital with amaurosis fugax and numbness of the right side of his body. Although no evidence of acute cerebral infarction was obtained on MRI/DWI, MRA disclosed severe stenosis of the left cervical ICA. (123)I-IMP-SPECT revealed extremely low perfusion and disturbance of vascular reactivity in the territory of the left ICA. Although conservative therapy was performed, crescendo TIA was noted. Revascularization using CEA was therefore performed. After surgery, hyperperfusion was observed in the same fashion as in case 1, and again aggressive control of blood pressure under dexmedetomidine anesthesia enabled effective management of the resulting hyperperfusion syndrome. The patient was discharged 1 month postoperatively without neurological deficits. Dexmedetomidine is a safe and acceptable sedative drugs preventing hyperperfusion syndrome after CEA.

Indications for one-stage extensive indirect vascular reconstructive surgery for pediatric moyamoya disease: 4 case reports.

BACKGROUND: Many modified surgical techniques for moyamoya disease have been developed, and each of which has some advantages and disadvantages. We report the indication for one-stage extensive indirect vascular reconstructive surgery that is our original technique for pediatric moyamoya disease. CASE DESCRIPTION: Case 1 was a 3-year-old boy who had TIAs involving the bilateral extremities and provoked by intense crying. Case 2 was a 5-year-old girl who had TIAs involving the right extremities and provoked by intense crying. Case 3 was an 8-year-old girl who had frequent TIAs involving the bilateral extremities. Case 4 was a 3-year-old boy who had mild tetraplegia due to bilateral cerebral infarction. In all cases but case 4, preoperative investigations demonstrated same-stage moyamoya disease. In case 4, preoperative investigations demonstrated different-stage moyamoya disease in which acute progression of bilateral major arterial stenosis occurred. Hence, all patients underwent one-stage extensive indirect vascular reconstructive surgery of the bilateral cerebral hemispheres. Postoperative investigations revealed the development of rich neovascularization, disappearance of clinical symptoms in all cases. CONCLUSIONS: We recommend one-stage extensive indirect vascular reconstructive surgery for pediatric moyamoya disease, particularly for patients younger than 5 years, when preoperative investigations reveal a same-stage case or bilateral acute progressive case.

Bilateral vertebral artery dissecting aneurysm with subarachnoid hemorrhage treated with staged bilateral vertebral artery coil occlusion: a case report.

BACKGROUND: Vertebral artery dissecting aneurysm is now increasingly recognized as a cause of posterior circulation stroke in young adults. Here, we report a case of bilateral VADA with SAH, treated by bilateral coil occlusion using GDCs. CASE DESCRIPTION: A 64-year-old woman was admitted to our hospital with consciousness disturbance (Hunt and Kosnik: grade 4). Computed tomography showed diffuse SAH with a thick hematoma in the left C-P angle. Magnetic resonance angiography, 3D-CTA, and cerebral angiography revealed bilateral VADAs. First, the ruptured left VADA involving the PICA and a perforating branch was treated by occluding the lower half of the VADA and the proximal VA with GDCs in the acute stage. Thereafter, the residual VADA and contralateral VADA exhibited enlargement on cerebral angiography and MRA for 2 months after the initial surgery; thus, the right VADA was occluded by GDCs just proximal to the right PICA after confirming BTO tolerance. CONCLUSION: Staged bilateral VA coil occlusions combined with BTO may be one of the treatment strategies for bilateral VADA with SAH in cases presenting surgical difficulty due to anatomical factors or severe grade of SAH.

Postoperative temporary neurological deficits in adults with moyamoya disease.

BACKGROUND: Several authors have reported temporary neurologic deterioration after revascularization surgery in some patients with moyamoya disease. The present study examined the incidence and mechanisms of PONDs in adult patients with moyamoya disease. METHODS: Postoperative neurological deficits were retrospectively evaluated 1 month or less postoperatively on 32 hemispheric sides of 17 symptomatic adult patients with moyamoya disease treated surgically with direct and/or indirect revascularization. RESULTS: Various PONDs were observed in 9 sides (28%) from 7 patients 1 month or less after surgery. Symptoms were recognized in 7 (39%) of 18 sides with ischemic onset, and 2 (14%) of 14 sides with hemorrhagic onset. Postoperative neurological deficits were usually observed 1 week or less after surgery, and resolved within 2 weeks. Postoperative neurological deficits were divided into 3 groups based on duration of symptoms: single transient neurologic deficits in 3 sides; repeated transient neurologic deficits in 3 sides; and continuous neurologic deficits in 3 sides. Radiologic examinations demonstrated no ischemic changes in any patients, and subsequent focal hyperemia after surgery on 3 sides. Postoperative neurological deficits occurred more frequently in younger patients or those with poor vascular response before surgery. CONCLUSIONS: Postoperative neurological deficits frequently occur in patients with moyamoya disease, but are temporary. These deficits appear to result from focal hyperperfusion after surgery, rather than from ischemic changes.

[Two cases of main trunk artery occlusion associated with multiple cerebral aneurysms].

We report 2 cases of multiple aneurysms (AN) associated with main trunk artery occlusion. CASE 1: A 52-year-old male was admitted to our hospital with dysarthria and weakness of the right side of the body. Computed tomography (CT) showed cerebral infarction in the left corona radiata. MR angiography and conventional angiography showed occlusion of the left middle cerebral artery (MCA) and saccular aneurysms (ANs) at the origin of the anterior communicating artery (A-com) and bifurcation of the right MCA. Subsequent 123I-IMP-single photon emission tomography (SPECT) revealed marked reduction of cerebral blood flow and disturbed reactivity to acetazolamide in the left cerebral hemisphere. Superficial temporal artery (STA)-MCA anastomosis was performed to improve cerebral blood flow and reduce hemodynamic stress for AN of the A-com and right MCA. At 5 months after the first operation, neck clipping was performed successfully for the non-ruptured A-com AN and right MCA AN. CASE 2: A 65-year-old male was admitted to our hospital. CT revealed subarachnoid hemorrhage (SAH), and 3D-computed tomographic angiography (CTA) and cerebral angiography showed basilar top AN, A-com AN and right MCA AN associated with right internal carotid artery occlusion. Right ACA and MCA territories were visualized from the A-com artery and posterior cerebral artery. STA-MCA anastomosis was performed to improve cerebral blood flow and reduce hemodynamic stress for ANs. In the same operation, successful neck clipping was performed for BA top AN and right MCA AN. In such cases as these, particularly in ischemic cases associated with main trunk artery occlusion, it was important to consider surgery for AN after STA-MCA anastomosis in anticipation of improved cerebral blood flow and reduce hemodynamic stress for AN.

[A case of rheumatoid factor-positive hypertrophic spinal pachymeningitis].

A 54-year-old female was admitted to our hospital with the complaint of progressive gait disturbance, starting with left abdominal pain 1 month previously. She developed acute paraparesis and stocking-anesthesia type sensory disturbance within a few days. MR imagings on admission, revealed an encircled subdural mass at the Th1-9 vertebral level, and her spinal cord was compressed at several places at the Th5-6. The subdural mass showed low intensity on T1 and T2 weighted image, and was homogenously enhanced after Gd-DTPA administration. Laboratory data revealed elevation of rheumatoid factor, CH50, C3, C4, and C-reactive protein. An emergency operation was performed by left hemilaminectomy from Th4 to Th6. The subdural mass was resected as far as possible, followed by dural plasty and initiation of steroid intraoperatively. Histopathologic examination of the resected lesion documented a hypertrophic pathymeningitis. Postoperatively, her neurological findings were improved immediately, followed by adjuvant therapy. Recurrence was not present on radiographic images 3 years after surgery. Laboratory data normalized within 4 months. Hypertrophic pachymeningitis is a rare case, and it is difficult to diagnose preoperatively. If there are subdural mass lesions at the multiple vertebral levels, we should suspect this disease. Treatment by immediate decompressive surgery and continuous adjuvant therapy was effective in our case.