[A Case of Dural Arteriovenous Fistula that Developed 21 Months after Cerebral Venous Sinus Thrombosis].

A 56-year-old man experienced a sudden onset of left hemiparesis. The computed tomography(CT)scan revealed a lobar hemorrhage in the right fronto-parietal lobe. After his admission, deep vein thrombosis was detected in his left lower limb, and angiograms taken on the 36th day of hospitalization revealed cerebral venous sinus thrombosis. Anticoagulant treatment was induced. After 21 months, he experienced a sudden onset of left hemiparesis again. The CT scan revealed a new lobar hemorrhage in the right frontal lobe, and angiograms revealed that two dural arteriovenous fistulas(dAVF)developed in the superior sagittal sinus(SSS)and the left transverse-sigmoid sinus. The one in the SSS had retrograde drainage from the bilateral middle meningeal artery, and we performed transarterial embolization with 50% n-butyl-cyanoacrylate. Postoperative course was uneventful and no further stroke occurred. Intracranial dAVF is known to be an acquired disease caused by venous hypertension, but we rarely find new development of dAVFs after venous diseases. This patient's case will help to elucidate the pathophysiology of dAVF.

[A case of moyamoya disease with a subarachnoid hemorrhage treated with endovascular technique].

We report a case of a moyamoya disease presenting with subarachonoid hemorrhage (SAH) due to a ruptured aneurysm. A 40-year-old woman presented with sudden onset of headache and vomiting. Computed tomography (CT) showed diffuse thick SAH localized around basal cistern. 3D-CT Angiography (3D-CTA) and digital subtraction angiography (DSA) demonstrated a saccular aneurysm at the bifurcation of the left superior cerebellar artery and basilar artery. In addition, the both carotid arteries were occluded at the terminal portion and the territory of both middle cerebral arteries were perfused by abnormal moyamoya vessels. The aneurysm was completely embolized by endovascular embolization. The SAH due to a ruptured aneurysm associated with moyamoya disease is rare. We think endovascular therapy is safe and effective. However, a vasospasm of the catheter technique occurred during the operation. This fact is very important to consider when we treat diseases such as this in the future.

Cerebral hemorrhage and cerebral infarction in 30 cases of adult moyamoya disease: comparison between conservative therapy and superficial temporal artery-middle cerebral artery anastomosis.

To clarify the effect of surgery on the prevention cerebral hemorrhage in adult moyamoya disease, we compared postoperative courses between superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and conservative therapy. The study subjects were 30 adults with moyamoya disease. Unilateral STA-MCA anastomosis was conducted in 7 of these 30 cases, and bilateral STA-MCA anastomosis was conducted in 8 of these 30 cases. Therefore, anastomosis was performed in a total of 23 sides. The postoperative clinical course was observed for more than 5 years after the STA-MCA anastomosis. Cerebral hemorrhage occurred after operation in 2 sides (8.7%) among the 23 sides that received STA-MCA anastomosis. On the contrary, hemorrhage occurred during conservative therapy in 5 sides (13.5%) among 37 non-operation sides (no significance in chi2 test). Cerebral infarction occurred in 3 sides (13%) among 23 sides treated with STA-MCA anastomosis. However, the infarction occurred in 2 sides (5.4%) among the 37 non-operation sides (no significance in chi2 test). Cerebral hemorrhage tended to occur less frequently after STA-MCA anastomosis, and bypass surgery was suggested to have some beneficial effect in preventing cerebral hemorrhage in adult moyamoya disease. However, it was revealed that STA-MCA anastomosis exacerbated the brain ischemia. Therefore, strict management is mandatory in the perioperative period.

Atherothrombotic lesion of the middle cerebral artery: report of 21 cases with stenotic and obstructive lesions.

This was a retrospective analysis of 12 consecutive cases of middle cerebral artery stenosis and 9 consecutive cases of middle cerebral artery occlusion that presented to our hospital with acute cerebral ischemia. The degree and area of the cerebral infarctions were assessed with the Alberta Stroke Program Early CT Score (ASPECTS) and ASPECTS-DWI (APSECTS with assessment of white matter lesion using diffusion-weighted image). As for cerebral infarctions in the region of the perforating artery, lesions that were more than 20 mm long in the caudal-cranial direction were diagnosed as branch atheromatous disease (BAD). Activities of daily living (ADL) were poorer in the cases with lower ASPECTS and ASPECTS-DWI. ADL tended to be worse in cases with BAD than in those without. The prognosis was significantly poorer in the group with ASPECTS< or =7 points. ASPECTS tended to be lower in cases with BAD than in those without. ADL, ASPECTS and the presence of BAD were not significantly different between the stenosis and obstruction groups. In summary, the neurological prognosis was dependent on the extent of the cerebral infarction and was related to BAD to some extent. These findings will be important when considering medical treatment at the outpatient clinic setting.

[Factors which affect the neurological condition in subarachnoid hemorrhage].

The factors which were related to the neurological condition were analyzed in 233 cases of subarachnoid hemorrhage. Bivariate analysis and multiple(binomial)logistic regression analysis were performed as for Hunt & Kosnik grade, modified Rankin Scale at discharge and modified Rankin Scale in the out-patient department to detect the factors which were related to the neurological condition. Hematoma-filled intraventricular hemorrhage, intracerebral hemorrhage with midline shift, acute subdural hematoma and aneurysm of the vertebrobasilar system were the representative factors which caused poor neurological condition. Hunt & Kosnik grade was poor when rebleeding occurred or hematoma was formed in the sylvian fissure. Hunt & Kosnik grade and modified Rankin Scale at discharge tended to be poor in the cases with acute hydrocephalus. The elevation of intracranial pressure was the major factor in neurological deterioration.

[Subarachnoid hemorrhage caused by dissection of the basilar artery: a case report].

We report a case of subarachnoid hemorrhage, which was caused by dissection of the basilar artery during treatment for diabetes mellitus. The patient was a 60-year-old male who consulted our hospital complaining of sudden-onset of a severe headache. Head CT scan showed subarachnoid hemorrhage around the basilar artery, but 3D-CT angiography revealed no abnormality. The basilar artery was shown to be normal during cerebral angiography on Day 1. However, in retrospect pseudolumen of the basilar artery was suspected in the proximal portion of the branching point of the anterior inferior cerebellar artery. During a second cerebral angiography on Day 17, blood pressure elevated to 185/83mmHg and 30 minutes later this patient's consciousness deteriorated to 10 points of the GCS(E4, V2, M4). The double lumen in the basilar artery was identified around the branching point of the anterior inferior cerebellar artery. Head MRI on the same day showed intramural hemorrhage of the basilar artery as a high intensity lesion. Head MRI on Day 18 revealed multiple cerebellar infarctions in the region of the bilateral anterior inferior cerebellar arteries. His consciousness recovered to almost normal by reducing the blood pressure. Transient gait disturbance also recovered thereafter. Head MRI on Day 90 indicated disappearance of the intramural hemorrhage in the basilar artery. It was important to reduce the blood pressure to prevent recurrence of the arterial dissection.

[A case with Stanford type A acute aortic dissection that presented with consciousness disturbance and hemiparesis].

The patient was a 63-year-old female who had a past history of hypertension. She suddenly complained of agonizing pain and became comatose soon thereafter. Upon admission, she was in a state of shock, with upper airway obstruction and a coma. The pupils were dilated on both sides. The laboratory data showed that D-dimer was >80µg/mL. Brain CT scan and diffusion weighted MRI of the brain showed no abnormality. On brain 2D-CT angiography, the visualization of the right internal carotid artery and the right vertebral artery was poor. She eventually was able to nod her head in response to verbal commands, but her left extremities were completely hemiplegic. Cerebral angiography showed occlusion of the right vertebral artery at the branching point from the brachiocephalic artery, and was visualized in a retrograde fashion through the left vertebral artery. The brachiocephalic artery was severely stenotic in aortography. During angiography, she became able to speak and complained of back pain. Chest CT just after angiography showed a dissection in the aortic arch. Therefore, she was directed to the cardiovascular surgeon for immediate consultation. During the operation, the aortic dissection was revealed to be Stanford type A. Laceration of the intima was found in the ascending aorta and cardiac tamponade was also found. Total arch replacement was performed. The pathological examination showed that the arterial dissection occurred in the layer of elastic fiber, and the minimum arterial thickness of the medial layer was 0.2mm. The patient improved after the operation and her neurological deficits disappeared completely 13 days after operation. Brain and spinal MRI 15 days after the operation showed no abnormality.

Inflammatory index and treatment of brain abscess.

This study retrospectively analyzed 12 patients with brain abscesses. Half of the patients were diagnosed inaccurately in the initial stage, and 7.2 days were required to achieve the final diagnosis of brain abscess. The patients presented only with a moderately elevated leukocyte count, serum CRP levels, or body temperatures during the initial stage. These markers changed, first with an increase in the leukocyte count, followed by the CRP and body temperature. The degree of elevation tended to be less prominent, and the time for each inflammatory index to reach its maximum value tended to be longer in the patients without ventriculitis than in those with it. The causative organisms of a brain abscess were detected in 10 cases. The primary causative organisms from dental caries were Streptococcus viridians or milleri, and Fusobacterium nucleatum. Nocardia sp. or farcinica were common when the abscess was found in other regions. The primary causative organisms of unrecognized sources of infection were Streptococcus milleri and Prolionibacterium sp. Nocardia is resistant to many antibiotics. However, carbapenem, tetracycline and quinolone were effective for Nocardia as well as many other kinds of bacteria. In summary, the brain abscesses presented with only mildly elevated inflammatory markers of body temperature, leukocyte and CRP. These inflammatory markers were less obvious in the patients without ventriculitis and/or meningitis. The source of infection tended to suggest some specific primary causative organism. It was reasonable to initiate therapy with carbapenem.

Treatment of a cerebral dissecting aneurysm in anterior circulation: report of 11 subarachnoid hemorrhage cases.

This report presents 8 cases of internal carotid artery aneurysms, 1 case of a middle cerebral artery aneurysm, and 2 cases of anterior cerebral artery aneurysms, together with a discussion of the treatment of aneurysms in anterior circulation. All cases showed subarachnoid hemorrhage. Two of the 8 internal carotid artery aneurysms were trapped with a low-flow bypass; however, both patients died of an immediate hemodynamic infarction or vasospasm-induced infarction. Five of the 8 internal carotid artery aneurysms were trapped after revascularization with high flow bypass. Four of those patients were self-supporting at discharge, but one patient was discharged in a vegetative state due to the sacrifice of arterial branches which were included in the dissecting portion. One case of the dissecting aneurysm in the M2 portion of the middle cerebral artery was trapped after low-flow bypass. This patient was self-supporting at discharge. In 2 cases of anterior cerebral artery aneurysms, the lesions were first wrapped with Bemsheets, and then the aneurysmal clip was applied on the wrapped dome. Trapping following high-flow bypass is the best method for treating a dissecting aneurysm in the internal carotid artery. Trapping also can be used to treat a dissecting aneurysm of the middle cerebral artery, after low-flow bypass. Clipping on the wrapped aneurysm can also be performed successfully in the anterior cerebral artery aneurysm.

Air vent of vein graft in extracranial-intracranial bypass surgery.

Revascularization with a vein graft is a mandatory method for treatment of dissecting or pseudoaneurysms of the internal carotid artery. We report the necessity for an air vent from the vein graft and explain its use in our two cases. In Case 1, we searched for a great saphenous vein around its junction with a femoral vein during the harvest of vein graft. An accessory branch of that great saphenous vein was also found around the junctional region with a femoral vein, and was temporarily ligated. At first, anastomosis was completed on both the distal and proximal sides. After the proximal side of a vein graft was opened and the ligation of the branch was loosened, an air vent could be made through the branch of the vein graft. Multiple air bubbles and a large quantity of white microemboli were discharged through this branch. The postoperative course was uneventful. In Case 2, the air vent was omitted to shorten ischemia. During the opening of the vein graft, the migrated air was observed to move to the middle cerebral artery. A computed tomography scan demonstrated that brain infarction and dysarthria occurred postoperatively. The air vent of the vein graft is essential in extracranial-intracranial bypass surgery, because the air bubbles or microthrombi are easily trapped around the valve and cause cerebral infarction. An air vent can be easily made if the branch of a vein graft exists in the outflow pathway, because intraluminal air or thrombus can be washed out through the branch at the final stage of surgery.

Postoperative recovery from unilateral blindness caused by tuberculum sellae meningioma.

A 47-year-old female had noticed diminished visual acuity in both eyes 2 months previously. The patient had vision loss (no light perception) in her right eye on admission. Her left visual acuity was 1.2 (naked vision) and an upper temporal quadrant hemianopsia was revealed in her left eye. Optic disc atrophy was also found bilaterally during a fundus examination. The tumor was located at the tuberculum sella. The first operation was performed using a right pterional approach. The right optic nerve was thin and atrophic and was severely encased by the tumor. Considering the deterioration of her visual evoked potential, the operation was terminated in the remaining major part of the tumor. Postoperatively, the patient suffered visual loss in her right eye (no light perception), decreased visual acuity (naked: 0.6 (corrected: 1.0)), and deteriorated visual field defects (upper temporal quadrant hemianopsia) in her left eye. The tumor remnant was resected again 2 weeks later using the right frontobasal and pterional approaches. The tumor around the bilateral internal carotid arteries and optic nerves was not resected. Light perception in the right eye appeared 2 weeks after the operation. Although an opthalmological examination revealed right optic atrophy, finger counting was possible in the upper nasal visual field of the right eye three months after the second operation. Her visual acuity was 0.7 (1.0), and the upper temporal quadrant hemianopia of the left eye improved in comparison with the preoperative one. Our case demonstrated the possibility of a recovery from blindness.

Repair of carotid blow-out using a carotid sheath in a patient with recurrent thyroid cancer.

The patient had thyroid cancer and underwent subtotal thyroidectomy. Local recurrence occurred on both sides 5 years and 6 months later. The sterno-hyoid muscle and sterno-thyroid muscle were severed and the tumor around the cricoid cartilage was removed. The tumor extended into the space between the right common carotid artery and internal jugular vein and was located under the right common carotid artery and vagus nerve on the lateral side. The carotid sheath was peeled off of the carotid artery quite easily. The right common carotid artery ruptured abruptly at the distal side during this procedure. The right common carotid artery had two layers, which were very fragile, so the direct suture or repair with a graft was impossible. The carotid artery could not be trapped with ligation because the cerebral vascular supply was not examined preoperatively. This portion was repaired using the remaining carotid sheath. However, re-bleeding occurred at the proximal portion of the previous laceration spontaneously. Fibrin glue with oxidized cellulose was initially used to seal the second small hole in this lesion. The second ruptured section was repaired using the remaining sterno-thyroid and sterno-hyoid muscles. The proximal portion of the right common carotid artery was reinforced with the harvested external jugular vein. These procedures resulted in hemostasis. Three-dimensional CT angiography showed irregular stenosis just after the operation, but it recovered 11 days later. No cerebral infarction occurred after the operation and the patient's general condition was good.

Schwannoma originating from lower cranial nerves: report of 4 cases.

Four cases of schwannoma originating from the lower cranial nerves are presented. Case 1 is a schwannoma of the vagus nerve in the parapharyngeal space. The operation was performed by the transcervical approach. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia happened transiently after the operation. Case 2 is a schwannoma in the jugular foramen. The operation was performed by the infralabyrinthine approach. Although only the intracapsular tumor was enucleated, facial palsy, hoarseness, dysphagia and paresis of the deltoid muscle occurred transiently after the operation. The patient's hearing had also slightly deteriorated. Case 3 is a dumbbell-typed schwannoma originating from the hypoglossal nerve. The hypoglossal canal was markedly enlarged by the tumor. As the hypoglossal nerves were embedded in the tumor, the tumor around the hypoglossal nerves was not resected. The tumor was significantly enlarged for a while after stereotactic irradiation. Case 4 is an intracranial cystic schwannoma originating from the IXth or Xth cranial nerves. The tumor was resected through the cerebello-medullary fissure. The tumor capsule attached to the brain stem was not removed. Hoarseness and dysphagia happened transiently after the operation. Cranial nerve palsy readily occurs after the removal of the schwannoma originating from the lower cranial nerves. Mechanical injury caused by retraction, extension and compression of the nerve and heat injury during the drilling of the petrous bone should be cautiously avoided.

Surgical results of parasagittal and falx meningioma.

Sixteen operative cases of parasagittal and falx meningioma were analyzed retrospectively. Parasagittal meningioma totaled 12 cases and falx meningioma numbered 4 cases. Preoperative symptoms were paresis of a lower extremity in 7 cases and disturbed consciousness or mentality in 6 cases. Paresis and/or consciousness deteriorated just after the operation in 11 cases. The deterioration was identified in paresis (6 cases), consciousness (3 cases), paresis and consciousness (2 cases). Motor function further deteriorated postoperatively when the patients had shown preoperative paresis. The cause of postoperative deterioration of motor function and/or consciousness level was intracerebral hematoma in 1 case, and newly-developed brain edema in 1 case. There was no obvious explanation for the symptomatic exacerbation in the other 9 cases. At discharge, 5 cases showed deterioration of motor function in comparison to their preoperative condition, and 3 cases showed an improvement. Eleven cases showed no change of consciousness in comparison to the preoperative condition, and 5 cases showed improvement at discharge. Surgical result was good for consciousness or mentality, but was relatively poor for motor function. It was considered that surgery should be performed carefully in patients with preoperative paresis.

[Recovery of consciousness by electrical dorsal column stimulation in brain injury patients].

Treatment with electrical dorsal column stimulation was performed in 7 cases of diffuse axonal injury, 2 cases of brain contusion and 1 case of hypoxic diffuse brain damage. After inadequate response to various treatment modalities, each patient was implanted with a spinal cord stimulation system. The effectiveness was assessed using a standard scoring system which consisted of state scale and reaction scale (the society for treatment of coma). Both state scale and reaction scale were considered to improve in 4 patients after dorsal column stimulation. In 5 patients, the effectiveness of dorsal column stimulation could not be distinguished from natural improvement. One patient of hypoxic brain damage showed slight deterioration after the dorsal column stimulation. Among the state scale, significant improvement was found in spontaneous movement of the oral cavity and pharynx, spontaneous changes of expression muscles, concern about circumstances, voluntary purposeful movement, and coherent verbalization 2 weeks after the operation. As dorsal column stimulation can cause consciousness recovery from the semicomatose state, it should be considered as the treatment choice for the consciousness disturbance.

[Epidermoid tumor originating from the petrous bone: report of two cases].

In case 1, the tumor was incidentally found in the right petrous bone, middle cranial fossa and cerebello-pontine angle. T1 weighted MRI demonstrated a low intense mass and T2 weighted imaging demonstrated hyperintensity. Through the subtemporal extradural approach, an epidermoid in the middle cranial fossa was partially removed. Postoperative course was uneventfull but an episode of rhinorrhea occurred 15 months later. Bone-window CT scan disclosed air cells of the petrous bone were exposed to the previous surgical cavity. Using the same approach, an epidermoid was totally removed. With the sealing of the tumor cavity with the vascularized muscle flap, the patient became free from rhinorrhea. Case 2 had complainted of sensory impairment in the left trigeminal nerve distribution, atrophy of the left temporal and masseter muscle, and diplopia. T1 weighted imaging of MRI demonstrated a low-intense mass in the left petrous bone, middle cranial fossa, temporal lobe, and cerebello-pontine angle, and T2 weighted imaging demonstrated hyperintensity. The intradural tumor under the temporal lobe was removed at another hospital. As the diplopia deteriorated 5 years later, the patient was re-introduced to our hospital. At first, the tumor in the cerebello-pontine angle was removed using the left retromastoid lateral suboccipital approach. Later, the tumor in the petrous bone and middle cranial fossa was removed through the left subtemporal extradural approach. With the sealing of the tumor cavity with the vascularized muscle flap, postoperative cerebrospinal fluid leakage was prevented. The epidermoid tumor in the petrous apex is a congenital and rare disease. The obstruction of the petrous air cell and dural defect using the vascularized flap is most important to prevent postoperative cerebrospinal fluid leakage.

[Intracranial metastasis of thymoma: report of three cases].

Thymoma with metastasis to the central nervous system is extremely rare. Herein, three rare cases of thymoma with intracranial metastasis are reported. Case 1 (thymoma, B3 in the WHO classification); cranial magnetic resonance imaging (MRI) revealed a mass lesion with a ring enhancement effect in the right temporal lobe. The cystic and hemorrhagic lesion was subtotally removed and radiation therapy of 40 Gy was administered to the tumor cavity containing the remains of the tumor around the ependyma. The patient received combination chemotherapy with carboplatin and gemcitabine hydrochloride, and he was discharged 3 months after the operation. Case 2 [(thymoma, C (thymic carcinoma)]; multiple cerebral metastases of 8 lesions were found and whole brain irradiation of 40 Gy was performed. Three months later, rapid development of 2 metastatic lesions resulted in the patient's death. At autopsy, neoplastic lesions were found in the neck lymph node, right shoulder, chest frame, pleural cavity, diaphragm, lung, periaortic lymph node, liver and pancreas. Case 3 (thymoma, B2); computed tomography (CT) scan and MRI showed a tumorous lesion over the cerebellar hemisphere. At operation, a vascular, elastic-soft and grey tumor was found to originate from the dura mater and had invaded the occipital bone. The tumor had also invaded the arachnoid membrane and sinus wall but the pia mater was free from invasion. Thereafter local irradiation of 40 Gy was performed to the tumor cavity containing the remains of the tumor around the sinus. However local recurrence reoccured 3 years later. After stereotactic irradiation of 40 Gy to the recurrent tumor, the tumor diminished. The patient is wheelchair-bound 3 years and 4 months after the operation. Most cerebral metastases are extremely rare. Outcome remains poor and life expectancy is very short when brain metastasis is present. Treatment for thymoma is multimodal, including surgery, irradiation and chemotherapy.

Visual disturbance following shunt malfunction in a patient with congenital hydrocephalus.

A 25-year-old woman presented with complaints of nausea and headache. She had been treated with a ventriculoperitoneal shunt for hydrocephalus when she was 7 months old. Her bilateral optic discs showed moderate atrophy. Right visual acuity allowed only perception of hand movement and left visual acuity was 0.02 (1.2). Computed tomography (CT) showed mild ventricular dilation but no periventricular lucency. Intracranial pressure (ICP) was not high when the shunt valve was punctured. Her visual acuity deteriorated 5 days after the consultation. She was referred again 8 days after the first consultation. The bilateral optic discs were completely pale. Both pupils were dilated on admission, and the bilateral direct light reflexes were absent. The patient could slightly detect only green light stimulus. CT showed moderate enlargement of the ventricle. ICP was 47 cmH(2)O when the shunt valve was punctured. Shuntgraphy showed obstruction of the shunt at the distal end of peritoneal catheter. Emergent total shunt revision was performed. She could detect dark stimulus and the still-dilated left pupil had recovered direct light reflex on the next day. The visual acuity was 0.01 (0.7) on the left 6 months after the operation, although she was blind in the right eye and the bilateral optic discs were completely pale. Visual loss associated with shunt failure remains a major morbidity in shunted congenital hydrocephalus. Early diagnosis and shunt revision may allow visual recovery.

Neuromyelitis optica spectrum disorder: 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography findings--case report.

A 51-year-old female with a history of rheumatoid arthritis rapidly developed anterior neck pain and paresis in the left upper and lower extremities and right lower extremity, sensory disturbance in the left upper and lower extremities, and bladder and rectal disorder. Adduction of the left eye and abduction of the right eye were also disturbed. Spinal magnetic resonance imaging demonstrated severe edema in the C1-T5 levels, which then deteriorated rapidly over 3 days, and lesions enhanced with gadolinium in the C1-C3 and C5-T3 levels. 2-Deoxy-2-[18F]fluoro-D-glucose positron emission tomography study demonstrated the inflammatory sites as segmental enhanced accumulation in the C1-C3, C5-C6, and T1 levels. The serum anti-aquaporin 4 antibody level was positive and she was diagnosed with neuromyelitis optica spectrum disorder. Marked improvement in the neurological conditions, concomitant with reduced spinal cord edema, was obtained by steroid pulse therapy.