A 53-year-old woman experienced a right retrobulbar pain followed by ipsilateral extraocular palsies in all directions without dilated pupils or ptosis. Because a plain head computed tomography (CT) scan obtained on her initial visit showed no abnormal findings, such as subarachnoid hemorrhage or a giant cavernous aneurysm, her condition was provisionally diagnosed as Tolosa–Hunt syndrome and elective magnetic resonance (MR) imaging was scheduled. The day after her initial visit, however, she suddenly developed complete ptosis and a dilated pupil on the right side. Emergency MR imaging and angiography revealed a clover leaf-shaped aneurysm projecting to the cavernous sinus at the junction of the internal carotid artery and the posterior communicating artery. Her condition was diagnosed as impending rupture of the aneurysm, and she underwent emergency open surgery. Her symptoms completely resolved within the following 2 weeks. Our case demonstrated that a medium-sized internal carotid artery–posterior communicating artery aneurysm can cause simultaneous oculomotor and abducens nerve palsies with retrobulbar pain if the shape of the aneurysm is complicated. Although these symptoms are very similar to those of Tolosa–Hunt syndrome, we believe that prompt radiological examinations such as MR or 3D CT angiography should be performed to prevent subsequent rupture of the aneurysm.
Cases involving intracranial dural arteriovenous fistulas (AVFs) with spinal perimedullary venous drainage exhibit variable presentations, which results in delayed diagnoses. We describe a case of a 66-year-old female with a transverse-sigmoid sinus dural AVF with spinal perimedullary venous drainage who developed dysesthesia and hypalgesia that ascended from the peripheral lower extremities. Sixty cases of intracranial dural AVFs resulting in myelopathy have been reported, and an absence of brainstem signs significantly correlated with a delay in diagnosis (positive group: 3.4 months vs. negative group: 9.6 months, P < 0.05). Intracranial dural AVFs with brainstem signs should be diagnosed without delay because the myelopathy and bulbar symptoms could progress aggressively without alternative drainage routes besides the perimedullary veins. We emphasize that intracranial dural AVFs should be considered as a differential diagnosis in case presenting with symptoms, such as atypical dysesthesia and hypalgesia ascending from the toes, without brainstem signs. Moreover, we should perform cerebral angiography as early as possible because dural AVFs with slow-flow venous drainage can produce false negatives on magnetic resonance angiography.
Bow Hunter’s syndrome is an unusual symptomatic vertebrobasilar insufficiency resulting from intermittent mechanical compression of the vertebral artery, and is rarely a trigger for cerebral infarction following thrombus formation on the damaged endothelial vessels (Bow Hunter’s stroke). The authors present an extremely rare case of a 45-year-old man showing Bow Hunter’s stroke due to congenital vertebral artery fenestration stretching and sliding between C1 and C2 after head rotation to the right. Congenital vertebral artery anomaly rarely causes cerebral infarction, but could cause embolic strokes by mechanical stretching without bony abnormalities.
A 31-year-old woman with pure red cell aplasia presented with motor aphasia and right homonymous hemianopia due to a left temporal and parietal lobe infarction. Magnetic resonance angiography revealed an occlusion of the left anterior and middle cerebral artery, with the development of moyamoya vessels. She was diagnosed with quasi-moyamoya disease and subsequently underwent direct and indirect anastomosis surgery, while continuing steroid and immunosuppressant therapy for pure red cell aplasia. The postoperative course was uneventful, and follow-up cerebral angiography 6 months after the surgery revealed the development of neovascularization through an indirect anastomosis. Neovascularization can be induced while the patient is receiving steroid and immunosuppressant therapy in quasi-moyamoya disease.
We present a case of a 63-year-old male presenting with right hemiparesis and diagnosed as cerebral infarction. He had a previous history of left pharyngeal carcinoma, which was treated by radial dissection and radiation therapy 10 years before. Magnetic resonance imaging (MRI) showed multiple cerebral infarction in the bilateral cerebral hemisphere, predominantly on the left. Cerebral angiography showed occlusion of bilateral internal carotid arteries and severe stenosis at the orifice of the left external carotid artery (ECA). Single photon emission tomography (SPECT) showed reduced cerebral blood flow and decreased cerebral vascular reserve in the bilateral anterior cerebral artery and left middle cerebral artery territories. He was successfully treated by a combination of carotid stenting for the left ECA and left superficial temporal artery–middle cerebral artery (STA-MCA) anastomosis. He did not have further ischemic symptom for more than 2 years. Radiation-induced carotid vasculopathy often involves multiple arteries and treatment is often complex and difficult. Safer and less invasive treatment strategy should be considered using both endovascular and direct surgery.
Eagle syndrome is a set of symptoms associated with an elongated styloid process. Although it is an important cause of cerebrovascular complications such as carotid artery dissection (CAD) or thromboembolism, the condition may be underdiagnosed. We treated three patients with CAD caused by an elongated styloid process within a year. The first patient was a 55-year-old man who developed recurrent thromboembolism despite anticoagulation therapy. Computed tomography (CT) angiography showed bilateral CAD with tips of styloid processes attached to the dissected lesions. He underwent surgical resection of the styloid process followed by carotid artery stenting. The second patient was also a 55-year-old man who developed acute stroke due to carotid artery occlusion, and underwent thrombectomy and carotid artery stenting. Both these patients experienced resolution of their neurological symptoms and had no recurrence of stroke. The third patient was an 80-year-old man with an asymptomatic dissecting aneurysm of the cervical internal carotid artery. He had a history of odynophagia and underwent surgical resection of the styloid process, with resolution of his symptoms. These cases, taken together with recent evidences showing that CAD was associated with the styloid process length, suggest that Eagle syndrome may not be an uncommon cause of CAD. Examination by CT angiography is important to avoid misdiagnosis. A literature review indicates that some cases were refractory to anticoagulation and surgical resection of the elongated styloid process or carotid artery stenting could be a treatment option to prevent further stroke.
Thrombus formation in a pulmonary vein stump after pulmonary lobectomy is extremely rare, but can trigger cerebral embolism of unknown cause. We encountered a case of cerebral embolism in a 58-year-old man 2 days after left upper lobectomy. Since intravenous administration of recombinant tissue plasminogen activator was contraindicated, thrombus removal by endovascular reperfusion therapy was performed. Cerebral angiography showed left internal carotid artery occlusion. Thrombus removal using a retrieval device was performed and complete recanalization of the left internal carotid artery was obtained. Although blood abnormalities or arrhythmia such as atrial fibrillation were not observed, thrombus in the left upper pulmonary vein stump was detected with contrast-enhanced computed tomography of the body trunk, which was therefore considered as the source of cerebral embolism. The patient is continuing on anticoagulant therapy to prevent embolism recurrence caused by thrombus formation in the pulmonary vein resection stump. To the best of our knowledge, this is the first report of thrombus removal by acute-phase endovascular reperfusion therapy to treat cerebral embolism likely caused by thrombus formation in the pulmonary vein stump after left upper lobectomy. When cerebral embolism of unknown cause develops after left upper lobectomy, thrombus formation in the pulmonary vein stump should be considered among the differential diagnoses. For acute-phase onset of cerebral embolism after pulmonary lobectomy, thrombus removal by endovascular reperfusion therapy may be considered as one of the therapies.
Alveolar soft part sarcoma (ASPS), a rarely observed tumor, is a soft tissue sarcoma with an unidentified cell origin. It constitutes 0.5–1.0% of all soft tissue sarcomas. It may appear in various parts of the body, but mostly observed in the trunk and the extremities. It has a high metastasis potential. To the best of our knowledge, only three cases of primary intracranial ASPS without a demonstrable lesion elsewhere is encountered. An 11-year-old girl was operated because of fronto-parietal mass lesion by craniotomy. Pathological examination revealed ASPS and no primary focus was detected. In spite of radiotherapy and chemotherapy as an adjuvant therapy, after 45 months she had a second operation for recurrence of the tumor. Since it is possible to observe metastases in late phases, up to 30 years, the patients must be followed up for a long period. Although radiotherapy and chemotherapy followed by surgery is the most accepted treatment strategy, the prognosis is still poor.
Implantation of carmustine-impregnated wafers (Gliadel®) into the tumor resection cavity has demonstrated a survival benefit for patients with malignant glioma. However, some precautions should be taken regarding Gliadel implantation. We report a case of a 63-year-old man with glioblastoma who was implanted with Gliadel after a left temporal lobe tumor had been removed, and who later developed vasospasm of the lenticulostriate artery close to the implanted Gliadel, leading to serious cerebral infarction. Therefore, the implantation of Gliadel in cases where important vessels run close to the resection cavity should be considered with great caution.
Although direct Gliadel® wafer implantation into the resection cavity has been shown to significantly improve survival in patients with high-grade gliomas, several complications have been associated with the implantations of Gliadel wafers, including brain edema, healing delay, cerebral spinal fluid leak, intracranial infections, and cyst formation. The brain edema that is associated with Gliadel wafer implantation might result in neurological deficits and significant morbidities and mortalities. In particular, it is not clear if they should be placed in the eloquent areas, such as language areas, motor areas, and areas related to cognitive function, even if these areas contain a remnant tumor. Here, we present a case of profound brain edema along the pyramidal tract due to Gliadel wafer implantation, which resulted in severe neurological deficits. This treatment represents a double-edged sword due to the possibility of severe symptomatic brain edema along the eloquent area, even though Gliadel wafers might be effective in controlling local tumor growth. We should keep in mind that Gliadel wafer placement in eloquent areas may result in severe disadvantages to patients and a loss of their quality of life.