Two cases with megalencephalic leukoencephalopathy with subcortical cysts and MLC1 mutations in the Turkish population.
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Megalencephalic leukoencephalopathy with subcortical cysts is a rare leukodystrophy that is characterized by macrocephaly and a slowly progressive clinical course. It is one of the most commonly reported leukoencephalopathies in Turkey. Mutations in the MLC1 gene are the main cause of the disease. We report two patients with megalencephalic leukoencephalopathy with subcortical cysts with confirmed mutations in the MLC1 gene. The mutation in the second patient was novel. We also review identified mutations in the Turkish population. (+info)
Large hypothalamic hamartoma with calcification and cystic components in an adult--case report.
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A 24-year-old female presented with an unusual case of hypothalamic hamartoma manifesting as seizure. Neuroimaging findings were atypical, showing the large tumor (maximum diameter, 50 mm) with a cystic component and calcification. Surgery was performed and histological examination demonstrated heterotopia. Hamartoma should be considered in the differential diagnosis of a suprasellar, non-enhanced mass attached to the hypothalamus. Excessive unnecessary surgery should be avoided, and intraoperative pathological examination may lead to enhanced assessment and better outcomes. (+info)
Clinical features of nonpituitary sellar lesions in a large surgical series.
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Intraorbital encephalocele in an adult patient presenting with pulsatile exophthalmos. Case report.
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A 25-year-old man presented with an intraorbital encephalocele manifesting as progressive left pulsatile exophthalmos. He had a history of frontal lobe contusion from a motorbike accident 10 years before the onset of the symptom. Computed tomography and magnetic resonance imaging revealed an oval-shaped defect in the left orbital roof with an underlying intracranial cystic lesion, herniated into the orbit. Intraoperative findings included disruption of the dura mater around the bony defect. The loculated arachnoid membrane and protruding brain tissue were excised with primary dural closure and reconstructive cranioplasty with a titanium mesh. The postoperative course was uneventful, and the pulsatile proptosis disappeared immediately after the procedure. Intracranial cyst may be important in the development of progressive pulsatile exophthalmos and intraorbital encephalocele. (+info)
Indocyanine green videoangiography of optic cavernous angioma - case report - .
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The intraoperative findings of an indocyanine green videoangiography (ICG-VAG) study of a cavernous angioma located in the optic chiasm are reported. A 23-year-old Japanese man suddenly developed visual field loss, and magnetic resonance imaging suggested the presence of a suprasellar tumor in contact with the optic chiasm. Preoperative angiography did not clearly show any tumor shadow. Right fronto-temporal craniotomy was performed, and an aggregation of blood vessels was seen on the right surface of the optic chiasm. Cavernous angioma was suspected. ICG-VAG was begun 22 seconds after the beginning of contrast agent infusion via a peripheral blood vessel. The lesion remained unstained, although the brain surface, an artery superior to the optic nerve, and veins were visualized. The cavernous angioma was resected following surface coagulation. ICG-VAG is currently being evaluated for future application in the differential diagnosis based on imaging findings, and the present case provides an important example of intraoperative ICG-VAG imaging of an unoperated cavernous angioma. (+info)