Dupilumab-related type 1 diabetes in a patient with atopic dermatitis: a case report.

Dupilumab, a humanized monoclonal antibody that inhibits both interleukin (IL)-4 and IL-13 signals, is used as a treatment for a variety of allergic diseases including atopic dermatitis. We experienced a case of dupilumab-related type 1 diabetes in a patient with atopic dermatitis. An 18-year-old female presented with thirst and polydipsia seven months after initiating dupilumab therapy for atopic dermatitis and was found to have marked hyperglycemia with ketosis. She was positive for anti-glutamic acid decarboxylase antibody, leading to the diagnosis of type 1 diabetes. She carried human leukocyte antigen (HLA) genes associated with type 1 diabetes. Most type 1 diabetes is considered a T-helper (Th) 1 type autoimmune disease, whereas IL-4 and IL-13, which are Th2 cytokines, play inhibitory roles in the pathogenesis of type 1 diabetes. This case implies that dupilumab might contribute to the development of type 1 diabetes in individuals with a genetic background of type 1 diabetes via relative Th1 dominance. To our knowledge, this is the first case of the development of type 1 diabetes during dupilumab therapy. As dupilumab therapy might accelerate the development of type 1 diabetes, it is important to note cases like this case to clarify the pathogenic mechanisms underlying dupilumab-related type 1 diabetes.

Ruptured dissecting aneurysms of the A1 segment of the anterior cerebral artery: three case reports and a review of the literature.

Dissecting aneurysms in the anterior cerebral artery (ACA), although rare, can cause ischemic and/or hemorrhagic stroke. Hemorrhagic dissecting aneurysms in the A1 portion of the ACA are often associated with a poor prognosis. We retrospectively investigated three rare cases of hemorrhagic dissecting aneurysms in the A1 portion. Dissecting aneurysms were diagnosed by carotid angiography or computed tomography angiography to visualize morphological changes in the vessel. All patients presented with diffuse subarachnoid hemorrhage. In one case, computed tomography angiography performed at the onset of the subarachnoid hemorrhage revealed fusiform dilatation at the right ACA (A1), which did not appear on a magnetic resonance angiogram obtained 1 year prior to the onset of the subarachnoid hemorrhage. In the other two cases, A1 dissecting aneurysms were diagnosed from a growing aneurysmal bulge revealed at a non-bifurcated site via repeated carotid angiography. Two patients underwent surgical intervention (trapping or clipping), and their outcome was favorable, whereas the third patient died of delayed rebleeding before receiving surgical treatment. Hemorrhagic dissecting aneurysms in the A1 portion cause severe subarachnoid hemorrhage. Surgical treatments that include revascularization are necessary to prevent rebleeding, and direct surgery is recommended, particularly at the A1 portion.

[A Case of Cervical Spinal Dural Arteriovenous Fistula with Extradural Drainage Presenting with Subarachnoid Hemorrhage due to a Ruptured Anterior Spinal Artery Aneurysm].

We report a rare case of a cervical spinal dural arteriovenous fistula(AVF)at the C2 level presenting with subarachnoid hemorrhage(SAH)due to a ruptured anterior spinal artery aneurysm. A 61-year-old man presented with sudden onset headache. Initial computed tomography revealed SAH around the brainstem. Digital subtraction angiography(DSA)demonstrated a cervical dural AVF that was fed by the left C1 radicular, left C2 radicular, and anterior spinal arteries, and drained into the epidural plexus. An aneurysm in the branch of the cervical anterior spinal artery was considered the bleeding point. A left lateral suboccipital craniotomy and C1 hemilaminectomy were performed on day 43. The feeding arteries were clipped, followed by coagulation of the draining veins. However, the aneurysm was not clipped because we deemed that obliteration of the aneurysm would be difficult without disrupting the blood flow of the parent artery. The patient showed no neurological deterioration after the operation. Postoperative DSA revealed residual dural AVF. Therefore, a second surgery was performed. After the second open surgery, DSA showed that the dural AVF and aneurysm disappeared. The patient also showed no neurological deterioration after the second surgery.

A case of transient hypothermia after trans-lamina terminalis and third ventricle clipping of an extremely high-position basilar tip aneurysm.

Reports on the trans-lamina terminalis and trans-third ventricular approach are rare. The risk associated with this approach is unknown. After an unsuccessful endovascular surgery, we performed direct surgical clipping via the third ventricle on a 78-year-old woman presenting with an extremely high-positioned, ruptured basilar tip aneurysm. She experienced transient hypothermia for 5 days, and it was considered that this was due to hypothalamic dysfunction. It is necessary to recognize that there is the potential for hypothermia after surgery via the lamina terminalis and third ventricle, even though the mechanisms of hypothalamic thermoregulation are still unclear.

[A case of cavernous angioma at the convexity in the dura mater: characteristics of images in the literature].

We describe a rare case of cavernous angioma in the dura mater and discuss published MRI findings on similar cases. The patient was a 78-year-old woman who was referred to Shimane Prefectural Central Hospital with complaints of headaches. We were subsequently able to identify a tumor at the convexity in the dura mater. The tumor showed a high intensity on T2-weighted images and was heterogeneously enhanced on contrast-enhanced T1-weighted images. The maximum size of the tumor was 35 mm. Moreover, preoperative angiography showed a slight vascularity in the tumor. We performed surgery with the expectation of finding a meningioma, metastatic brain tumor, or another mesenchymal tumor. The tumor was dark and red, attached to the dura mater, and adhered to the arachnoid. However, we were able to peel the tumor away from the meninges and achieved a total removal of the mass, successfully cutting a fine feeding cortical artery. The patient was discharged without neurological defects 9 days after surgery;the pathological diagnosis was cavernous angioma. In conclusion, it is difficult to discern between meningioma and cavernous angioma in the dura mater. However, the specificity of high intensity on T2-weighted images is relatively high, and preoperative diagnosis can be determined by MRI and angiography findings.

Treatment of ruptured vertebral artery dissecting aneurysms. A short report.

We evaluated the outcomes of endovascular or surgical treatment of ruptured vertebral artery dissecting aneurysms (VADAs), and investigated the relations between treatment complications and the development and location of the posterior inferior cerebellar artery (PICA). We treated 14 patients (12 men, two women; mean age, 56.2 years) with ruptured VADAs between March 1999 and June 2012 at our hospital. Six and eight patients had Hunt and Hess grades 1-3 and 4-5, respectively. Twelve patients underwent internal endovascular trapping, one underwent proximal endovascular occlusion alone, and one underwent proximal endovascular occlusion in the acute stage and occipital artery (OA)-PICA anastomosis and surgical trapping in the chronic stage. The types of VADA based on their location relative to the ipsilateral PICA were distal, PICA-involved, and non-PICA in nine, two, and three patients, respectively. The types of PICA based on their development and location were bilateral anterior inferior cerebellar artery (AICA)-PICA, ipsilateral AICA-PICA, extradural, and intradural type in one, two, two, and nine patients, respectively. Two patients with high anatomical risk developed medullary infarction, but their midterm outcomes were better than in previous reports. The modified Rankin scale indicated grades 0-2, 3-5, and 6 in eight, three, and three patients, respectively. A good outcome is often obtained in the treatment of ruptured VADA using internal endovascular trapping, except in the PICA-involved type, even with high-grade subarachnoid hemorrhage. Treatment of the PICA-involved type is controversial. The anatomical location and development of PICA may be predicted by complications with postoperative medullary infarction.

Extracranial internal carotid artery pseudoaneurysm associated with neurofibromatosis type 1 treated with endovascular stenting and coil embolization.

An internal carotid artery (ICA) pseudoaneurysm associated with neurofibromatosis type 1 (NF-1) is rare. We report the first case of unruptured extracranial pseudoaneurysm of the ICA in a patient with NF-1 successfully treated with endovascular stenting and coil embolization.A 66-year-old woman diagnosed with NF-1 had sudden left neck pain and massive swelling 3 years earlier. Radiological examination showed a ruptured pseudoaneurysm of the left internal thoracic artery (ITA). The posttreatment computed tomography (CT) scan revealed complete obliteration of the aneurysm of the left ITA and an unruptured pseudoaneurysm of the right ICA. After 3 years of follow-up, a CT scan revealed the enlargement of the pseudoaneurysm of the right extracranial ICA. Endovascular stenting and coil embolization were performed to prevent rupture, and the lesion was completely obliterated. Follow-up angiography at 6 months revealed good flow of the ICA through the stent without any filling of the aneurysm.

[A case of tumor progression after gamma knife radiosurgery for brain metastasis of thyroid papillary carcinoma in deep subcortical white matter].

We report a case of a brain metastasis of thyroid papillary carcinoma. A 67-year-old man had undergone a radical operation for thyroid papillary carcinoma 6 years before. He had no neurological deficit, but CT showed an enhanced lesion in the left frontal lobe. He underwent gamma knife radiosurgery. Four months later, the lesion with massive peritumoral edema was identified on magnetic resonance imaging (MRI). The edema was treated medically, however, recovery was seen neither on MRI nor clinically. Then left craniotomy was performed, with total resection of the tumor. During the operation Komai's stereotactic instruments were used for CT guided stereotactic surgery. Histopathological analysis of the surgical specimen confirmed mixed necrosis and papillary carcinoma of the thyroid gland. The patient was discharged without neurological deficit and is now kept under observation as an outpatient. Brain metastases from thyroid cancer are rare and we report a case of resection of brain metastasis of a thyroid papillary carcinoma after gamma knife radiosurgery.

[Six cases of organized chronic subdural hematoma].

Surgical procedures for chronic subdural hematoma (CSDH) are performed using various methods on the basis of burr hole irrigation and drainage, but treatment for organized CSDH is rarely required. Primary operation for CSDH was performed in our hospital for 535 patients (391 men, 144 woman; age, 8 months to 104 years) between December 1991 and March 2007. Of these, 6 patients diagnosed with organized CSDH were reviewed. Five patients had a history of burr hole surgery. Computed tomography showed membranous structure and heterogenous distribution of air after burr hole surgery had perforated the subdural space. As for treatment, craniotomy was performed in all cases (small craniotomy, n=4; enlarged craniotomy, n=2), and additional treatment was required in 2 patients. Diagnosis of organized CSDH is not easy before a primary operation, but removal of both organized CSDH and the outer membrane by craniotomy in proportion to hematoma expansion is important once the presence of organized CSDH has been determined.

[Evaluation of ruptured cerebral aneurysms under 5 mm in size on 3D-rotation angiography].

According to the recent guideline of the Japanese Society for Detection of Asymptomatic Brain Diseases, it should be considered that the operative indication for unruptured cerebral aneurysms is larger than 5 mm, but we have often encountered patients with subarachnoid hemorrhage (SAH) caused by small ruptured cerebral aneurysms. The aim of our study was to evaluate retrospectively the characteristics of ruptured cerebral aneurysms under 5 mm in size on 3-dimensional digital rotation angiography (3D-RA). Eighty patients who presented with acute SAH caused by ruptured aneurysms were admitted in our hospital between January 2003 and September 2007. All patients were examined with 3D-RA and divided into two groups by aneurysmal size; group A was under 5 mm (N = 18), group B was larger than 5 mm (N = 62). Of aneurysms under 5 mm, 45% were located in the anterior communicating artery or anterior cerebral artery, 78% were female and 78% were treated with clipping. Clips of mini and/or the slim type were often applied for aneurysmal clipping. 3D-RA images were useful not only in identification of smaller aneurysms, but also in assessing aneurysmal morphology and relationships to neighboring vessels. However, in the cases of small aneurysms, it is necessary to remember that aneurysms become blood blister-shaped or thrombosed. The clipping for the aneurysm should be performed with consideration of choice for clips consisting of various types according to aneurysmal morphology.

[Microvascular decompression for glossopharyngeal neuralgia: case report].

We report the case of a glossopharyngeal neuralgia that was successfully treated using microvascular decompression (MVD). A 61-year-old female reported intermittent piercing pain from tongue to pinna on the left side. Although she had been prescribed carbamazepine and has undergone attempted nerve block on several occasions, no pain relief has been achieved. MVD was thus attempted using a lateral suboccipital approach. The offending vessel, which was PICA, had adhered to the glossopharyngeal nerve and was repositioned laterally away from the nerve by interposition of a felt cushion. Pain disappeared immediately after surgery and has not recurred. In the literature, MVD for glossopharyngeal neuralgia has been performed using a transcondylar approach to achieve minimally invasive surgery. However, the sensory distributions for the floor of the oral cavity and tongue involve 4 overlapping nerves: the trigeminal nerve, sensory components of the facial and vagal nerves, and the glossopharyngeal nerve. In typical cases, it seems that the transcondylar fossa approach is appropriate for glossopharyngeal neuralgia. If the pain occurs in the place involving an overlapping nerve, the lateral suboccipital approach might be necessary.