Atypical teratoid/rhabdoid tumors (AT/RT) are rare, aggressive, embryonal brain tumors that occur most frequently in very young children; they are characterized by rhabdoid cells and loss of INI1 protein nuclear expression. Here, we report the case of a 24-year-old man with a left frontal lobe tumor that was composed mainly of rhabdoid cells showing loss of INI1 nuclear reactivity and polyphenotypic immunohistochemical expression, with a small INI1-positive component of ependymoma. Array comparative genomic hybridization separately conducted for each histologically distinct component revealed 22 shared identical copy number alterations, including loss of heterozygosity of chromosome 22q containing the INI1 locus. Furthermore, we found the C11orf95-RELA fusion gene, the genetic hallmark of supratentorial ependymomas, not only in the ependymoma component but also in the AT/RT component by fluorescence in situ hybridization analysis, suggesting that the AT/RT cells secondarily progressed from the preexisting ependymoma cells. A second genetic inactivating event in the INI1 gene was not detected in the AT/RT component. There are several reported cases of AT/RT (or INI1-negative rhabdoid tumors) arising in the setting of other primary brain tumors (gangliogliomas, pleomorphic xanthoastrocytomas, and high-grade gliomas), but the present case
For not only young but also elder patients with a treated aneurysm (from the fifth decade to the sixth), especially for women, late angiography or alternative modalities of less-invasive examination should be considered. To detect de novo intracranial aneurysms before rupture, the search for a de novo aneurysm should be performed within 6.39 years after a previous examination that shows an aneurysm to be nonexistent, in view of the 95% confidence interval of the mean time to de novo aneurysmal rupture (6.39-15.1 years). If applied this survey, 75% (8 cases of 12 cases) of our de novo aneurysms would be detected before rupture.
We present a rare case of complete human Kluver-Bucy syndrome (KBS) following recovery from transtentorial herniation caused by acute subdural haematoma (ASDH). A 17-year-old right-handed high school boy got into stupor within five minutes after 3-rounds of sparing at boxing. Emergency computed tomographic (CT) scan showed right cerebral hemispheric ASDH, which was evacuated following intentional decompressive craniectomy. After recovery of consciousness, he developed emotional changes (placidity with loss of normal fear and anger), psychic blindness, aberrant sexual behaviour, excessive oral tendencies, increased appetite, and hypermetamorphosis in order of mention, which were observed with waxing and waning from 17th to 28th hospital day. Peri-operative CT scaning and magnetic resonance imaging showed lesions of the right temporal lobe and right-dominant orbitofrontal regions including bilateral rectal and medial orbital gyri, and the intact left temporal lobe. Two pathogeneses can be thought of and the whole picture of KBS following ASDH can arise even though one (left in this case) temporal lobe is preserved, 1) in which associated orbitofrontal lesions of the frontal lobes may correlate with occurrence of KBS, or 2) cerebral blood hypoperfusion of both temporal lobes due to increased intracranial pressure and/or compression of both posterior cerebral arteries at the edge of the tentorium cerebelli occurs.
Exertional vertebrobasilar insufficiency (VBI) secondary to the non-atherosclerotic cause is uncommon. We herein report the case of a patient who developed exertional VBI long after extracranial right vertebral artery (VA) dissection. At the time of dissection, the right VA was completely occluded near its origin, but the distal flow was compensated by the collateral flow from the right deep cervical artery (DCA). After conservative management, the patient was discharged without neurologic deficit. Six years later, he developed recurrent VBI in association with the exertion of his right shoulder. A vascular evaluation revealed that the right proximal VA was still occluded, and there was no evidence of right subclavian artery lesions. The intracranial right VA flow was markedly reduced during the period, while branches of the right DCA were given off to the muscles of the right shoulder and neck. Then, occipital artery (OA)-posterior inferior cerebellar artery (PICA) anastomosis was performed. Intraoperative indocyanine green videoangiography (ICG) confirmed that the flow of the right PICA was predominantly supplied from the compensatory flow from the contralateral VA, and the antegrade flow in the right VA was clearly delayed in comparison to that of the left VA while there were prominent branches providing the blood flow to the medulla oblongata. After the anastomosis, these medullary branches provided the blood flow to the medulla oblongata more quickly and extensively than before. Postoperatively, VBI no longer occurred even after exertion. Surgical revascularization can be a viable option in the treatment of refractory VBI of the non-atherosclerotic cause.
venous plexus in epidural space, descent of the cerebellar tonsil, a flattened brain stem, and pituitary swelling are also valuable for the diagnosis of this condition. CSF leakage from the spinal dural sac can be detected by certain imaging modalities. In radioisotope (RI) cisternography, abnormal RI accumulation in the spinal epidural space, increased clearance of the tracer from the CSF space, or early bladder filling (EBF) of RI are regarded as suggestive of CSF leakage. MRI or MRI/computed tomography (CT) myelography can also identify CSF in the spinal epidural space. 2)Although these are valuable modalities to find CSF leakages, it is difficult to detect the leakage point of CSF in patients with spontaneous intracranial hypotension.In this article, we report a patient with cerebrospinal fluid (CSF) hypovolemia, the leakage of which was not detected by either RI cisternography or MRI, but detected by threedimensional computed tomography (3D-CT) performed just after the epidural injection of the patient's own blood, containing a contrast medium. This finding suggests that 3D-CT epidurography may be a useful tool to detect the fistula of CSF leakage, even in patients in whom other modalities including MRI, CT, or RI cisternography cannot specify the leakage point. Case ReportA 31-year-old female high school teacher with no particular past history was involved in two rear-end collisions 5 years previously. Four years later, she sometimes had headache, these rapidly worsened several weeks before admission. The headache was intractable and uncontrolled by commercially available painkillers, which resulted in her visiting and being admitted to our hospital. She had no specific neurological deficits and no abnormal findings on cranial CT.In spite of bed rest and sufficient hydration, her headache got worse and was enhanced by the orthostatic stress. MRI examined 24 days after admission showed a thickened dura mater, which was diffusely enhanced by intravenous gadolinium injection, narrowing of the prepontine cistern, a swelling of the pituitary, and dilated intracranial venous sinuses (Fig. 1). It also revealed thin chronic subdural hematoma in her frontal region. Spinal MRI of lumbar level showed a narrowing of the intra dural space and epidural fluid collection (Fig. 2). On a cervical level, abnormalities were not shown (Fig. 3). In RI cisternography, the tracer injected intrathecally via lumbar puncture filled the bladder after only 20 minutes. The tracer did not reach the basal Spontaneous intracranial hypotension presents with many symptoms including orthostatic headache, dizziness, and nausea due to cerebrospinal-fluid (CSF) leakage from the spinal dural sac. Although CSF leakage can be estimated by radioisotope (RI) cisternography or computed tomography/magnetic resonance imaging myelography, it is not easy to detect the leakage point using these modalities. Here, we describe a patient with spontaneous intracranial hypotension in whom threedimensional computed tomography (3D-CT) performed just after an epid...
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