Epilepsy with Eyelid Myoclonia Complicated by Polycystic Ovary Syndrome: Valproate Withdrawal, Vagus Nerve Stimulation, and Seizure Reduction.

Epilepsy with eyelid myoclonia is a childhood-onset generalized epilepsy, which is more common in women. Over 90% of the patients continue antiseizure medications, especially valproate, and more than 60% of cases are refractory. The efficacy of vagus nerve stimulation in treating eyelid myoclonia is still unknown. Polycystic ovary syndrome is highly prevalent in women with epilepsy receiving valproate; nevertheless, no reports on the complication of polycystic ovary syndrome in women with epilepsy with eyelid myoclonia were found. In this report, a case of a woman with epilepsy with eyelid myoclonia who developed polycystic ovary syndrome while receiving valproate and underwent vagus nerve stimulation is described. A 26-year-old female patient has been administered valproate since the occurrence of generalized seizures at the age of 12 years and then developed polycystic ovary syndrome. When the dose of valproate was reduced as an adult, her epilepsy became intractable. Information from her mother led to a video electroencephalography re-evaluation, and she was finally diagnosed 15 years after onset. The patient underwent vagus nerve stimulation. In a short-term follow-up, she achieved >50% seizure reduction at low output currents of <1.00 mA. Polycystic ovary syndrome was cured 15 months after valproate withdrawal. There are three key points presented in this case: Vagus nerve stimulation therapy was useful for treating epilepsy with eyelid myoclonia with absence. Women with epilepsy with eyelid myoclonia taking valproate must be aware of the risk of polycystic ovary syndrome and monitor their menstrual cycles. Information from the family, such as home videos, helped with the diagnosis.

A case of an atypical teratoid/rhabdoid tumor with distinctive histology in the pineal region in an adult patient.

A 31-year-old man suffered from headaches and presented at a hospital after the symptom worsened. Obstructive hydrocephalus and a pineal tumor were identified, and he was transferred to our hospital for further investigation and treatment. Cranial computed tomography revealed a hypodense mass lesion on the right of the pineal region, and calcifications and enlargement of the lateral and third cerebral ventricles were also evident. Blood tests were negative for all tumor markers. Laparoscopic biopsy and third-ventricle fenestration were performed that day as an emergency surgery to treat the obstructive hydrocephalus. Postoperative cranial magnetic resonance imaging revealed a solid tumor that was hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging, and heterogeneously enhanced by Gd. Subsequently, the tumor increased in size, and craniotomy and tumorectomy were performed. Histologically, the tumor proliferated as round or short spindle-shaped cells in a myxoid matrix, forming arrays that surrounded the blood vessels. As a few cells with eosinophilic cytoplasm were also present and immunostaining for INI-1 was negative, the patient was diagnosed with atypical teratoid/rhabdoid tumor (AT/RT). AT/RT of the pineal region in adults is rare, and herein, we report the morphological characteristics of this case and reviewed the relevant literature.

Pedicle Galeo-pericranial Flap Augmentation in Salvage Frontotemporal Cranioplasty: Additional 'Neurosurgeon-friendly' Reconstruction Technique of Aesthetic Neurosurgery in Superficial Temporal Artery Branch Compromised Host.

This technical note aims to demonstrate the usefulness, indications and its limitations of augmentation technique by bipedicle galeo-pericranial rotation flap and by monopedicle galeo-pericranial flap, both in STA (superficial temporal artery) branch compromised hosts in salvage frontotemporal cranioplasty. Although these flaps are not always idealistically vascularized owing to accidental injuries to the STA branches during previous surgeries, they are properly augmenting after salvage frontotemporal craniotomy when infection is not active. The procedure is indicated for salvage frontotemporal craniotomy when vasculature is needed at the surgical site, such as beneath the skin incision line in a thin injured scalp, onto the titanium plates or beneath the fragile fibrous scar. We do not apply this technique by neurosurgeons alone where infection is active or if the host is irradiated. This technique is recommended as a reconstructive aesthetic neurosurgical procedure. It is a 'neurosurgeon-friendly' simple procedure, as it does not require any special tools or complicated techniques.

[A Case of Multiple Atherosclerotic Fusiform Cerebral Aneurysms with Repeated Subarachnoid Hemorrhage].

Pure fusiform aneurysms without dissection are considered to have a low risk of rupture. Furthermore, aneurysm wall with atherosclerotic change tends to be less susceptible to rupture. We present a rare case of multiple atherosclerotic fusiform aneurysms associated with repeated subarachnoid hemorrhage(SAH)during a 9-year observation period. A 34-year-old woman was admitted to our hospital because of SAH. SAH with a temporal hematoma due to a fusiform aneurysm of the right middle cerebral artery(MCA)was detected using computed tomography(CT). Associated fusiform aneurysms were observed in the left posterior cerebral artery(PCA)and the left MCA. The right MCA aneurysm was occluded with angioplastic clipping. Eight years after the first onset, angioplastic clipping was performed on the other two fusiform aneurysms because of their growth. Intraoperative findings showed atherosclerotic change in the parent artery wall, similar to the right MCA aneurysms. The right MCA aneurysm ruptured again 1 year later. The aneurysm was treated with proximal occlusion combined with a bypass from the occipital artery to the distal MCA. The patient was in a stable state, although, she was disabled because of SAH damage. The effects of atherosclerotic fusiform aneurysms, which are rarely encountered, are not well known. Some studies have reported the risk of hemorrhage from these aneurysms. This case suggests the necessity of long-term follow-up for the prediction of aneurysm growth and bleeding. Moreover, proximal occlusion combined with an external bypass is better for the treatment of this type of aneurysm because angioplastic clipping is not curative.

Outflow occlusion with A3-A3 anastomosis for a doughnut-shaped partially thrombosed giant A2 aneurysm.

BACKGROUND: A doughnut-shaped aneurysm, which is defined as a round-shaped aneurysm composed of an intraluminar thrombus and marginal parent artery, is an extremely uncommon subtype of partially thrombosed giant aneurysms. Surgical treatment of this characteristic aneurysm is technically challenging. CASE DESCRIPTION: We report a rare case of a 79-year-old man with a symptomatic doughnut-shaped giant aneurysm at the A2 portion, which was successfully treated by outflow occlusion with an A3-A3 side-to-side anastomosis. Postoperative angiograms demonstrated no filling of the doughnut-shaped aneurysm and perfusion in the distal right anterior cerebral artery territory via the anastomosis. Follow-up magnetic resonance imaging 1 year after the surgery demonstrated significant diminution of the aneurysm. CONCLUSIONS: Outflow occlusion with distal revascularization could be an effective surgical option for such a unique aneurysm. To the best of our knowledge, this is the first report of outflow occlusion as a therapy for doughnut-shaped aneurysms.

[Dural cavernous angioma:a case report and review of the literature].

Here, we report a case of dural cavernous angioma. A 54-year-old man presented with headache in his right frontal area for the previous few weeks. Computed tomography (CT) and magnetic resonance imaging revealed a well-demarcated extramedullary mass, 3 x 2.5cm in size, within the subdural space at the right frontal region. The mass was not enhanced on a contrast-enhanced CT scan, and heterogeneously enhanced after administration of Gd-DTPA on magnetic resonance images. Cerebral angiography revealed an avascular mass. Right frontal craniotomy was performed. On surgery, the mass was found to be mainly extramedullar, and partially intramedullar. No adhesion between the mass and the overlying dura was observed. It was removed in an en bloc fashion. The pathological diagnosis was cavernous angioma. The patient's headache was resolved soon after surgery. Cavernous angiomas are usually intramedullar in the subcortical white matter of the cerebral hemispheres;extramedullary cavernous angiomas are rare. Extramedullary dural cavernous angiomas located in the subdural space at the cerebral convexity are extremely rare. They usually present with headache or mass signs, and resemble meningioma radiologically. During surgery they are easily resected with minimal blood loss. Dural cavernous angiomas should be considered in the differential diagnosis of intradural extramedullary mass at the cerebral convexity.

Supratentorial high convexity intradural extramedullary cavernous angioma: case report.

A 59-year-old man presented with a 2-month history of numbness in the lower left side of the face and upper left extremity. Axial T(1)-weighted magnetic resonance imaging showed a wedge-shaped mass measuring 3 x 2.5 cm in the right frontoparietal high convexity area that was heterogeneously enhanced after administration of gadolinium-diethylenetriaminepenta-acetic acid. Right frontoparietal craniotomy was performed and a bluish soft mass was found under the arachnoid membrane. The mass could be dissected free from the arachnoid membrane and the brain surface. Histological examination revealed the typical findings of cavernous angioma. Cavernous angioma should be considered in the differential diagnosis of supratentorial high convexity intradural extramedullary tumor, especially appearing as a heterogeneously enhanced mass adjacent to the brain parenchyma causing mass effect.