Cerebral Phaeohyphomycosis: A Rare Cause of Brain Abscess

Cerebral phaeohyphomycosis (CP) is a very rare but serious form of central nervous system fungal infection that is caused by dematiaceous fungi. It is commonly associated with poor prognosis irrespective of the immune status of the patient. In this study, the authors describe the first case of CP in Korea that occurred in a 75-year-old man without immunodeficiency and showed favorable outcome after surgical excision and antifungal therapy. In addition, the authors herein review the literature regarding characteristics of this rare clinical entity with previously reported cases.

Rupturing Anterior Communicating Artery Aneurysm during Computed Tomography Angiography: Three-Dimensional Visualization of Bleeding into the Septum Pellucidum and the Lateral Ventricle

Computed tomography angiography (CTA) is commonly used in setting of subarachnoid hemorrhage, but imaging features of aneurysm rupturing taking place at the time of scanning has rarely been described. The author reports a case of actively rebleeding aneurysm of the anterior communicating artery with intraventricular extravasation on the hyperacute CTA imaging. The rebleeding route, not into the third ventricle but into the lateral ventricles, can be visualized by real-time three-dimensional CT pictures. The hemorrhage broke the septum pellucidum and the lamina rostralis rather than the lamina terminalis.

Aberrant CpG Islands Hypermethylation Profiles in Malignant Gliomas

BACKGROUND: The authors analyzed whether the promoter hypermethylation of cancer-related genes was involved in the tumorigenesis of malignant gliomas. METHODS: A total of 29 patients received surgery and histologically confirmed to have malignant gliomas from January 2000 to December 2006. The promoter methylation status of several genes, which were reported to be frequently methylated in malignant gliomas, was investigated using methylation-specific polymerase chain reaction. RESULTS: All cases of malignant gliomas represented the promoter hypermethylation in at least 2 or more genes tested. Of 29 tumors, 28 (96.55%) showed concurrent hypermethylation of 3 or more genes. Ras association domain family member 1, epithelial cadherin, O-6 methyl guanine DNA methyltransferase, thrombospondin 1, p14 and adenomatous polyposis coli were frequently methylated in high grade gliomas including glioblastomas, anaplastic astrocytomas, and anaplastic oligodendrogliomas. CONCLUSION: Aberrant hypermethylation profile was closely related with malignant gliomas suggesting that epigenetic change may play a role in the development of malignant gliomas. Two or three target genes may provide useful clues to the development of the useful prognostic as well as diagnostic assays for malignant gliomas.

A Case of Rathke Cleft Cyst Apoplexy / 계명의대학술지

Rathke cleft cysts (RCCs) are benign epithelial lesions of the sellar and suprasellar region. Most RCCs remain clinically silent throughout an individual's life. Symptomatic patients with RCCs manifest headaches, endocrinopathies, and visual disturbances secondary to parasellar extension. Rarely, RCCs can present in a manner similar to pituitary tumor apoplexy. One such case is reported herein. This 53-year-old man presented with acute headache, vomiting, and partial hypopituitarism. Surgical drainage of the cyst contents via a transnasal route confirmed the diagnosis of RCC and pituitary infarction.

Temozolomide Salvage Chemotherapy for Recurrent Anaplastic Oligodendroglioma and Oligo-Astrocytoma

OBJECTIVE: To evaluate the efficacy of temozolomide (TMZ) chemotherapy for recurrent anaplastic oligodendroglioma (AO) and anaplastic oligoastrocytoma (AOA). METHODS: A multi-center retrospective trial enrolled seventy-two patients with histologically proven AO/AOA who underwent TMZ chemotherapy for their recurrent tumors from 2006 to 2010. TMZ was administered orally (150 to 200 mg/m2/day) for 5 days per 28 days until unacceptable toxicity occurred or tumor progression was observed. RESULTS: TMZ chemotherapy cycles administered was median 5.3 (range, 1-41). The objective response rate was 24% including 8 cases (11%) of complete response and another 23 patients (32%) were remained as stable disease. Severe side effects (> or =grade 3) occurred only in 9 patients (13%). Progression-free survival (PFS) of all patients was a median 8.0 months (95% confidence interval, 6.0-10.0). The time to recurrence of a year or after was a favorable prognostic factor for PFS (p<0.05). Overall survival (OS) was apparently differed by the patient's histology, as AOA patients survived a median OS of 18.0 months while AO patients did not reach median OS at median follow-up of 11.5 months (range 2.7-65 months). Good performance status of Eastern Cooperative Oncology Group 0 and 1 showed prolonged OS (p<0.01). CONCLUSION: For recurrent AO/AOA after surgery followed by radiation therapy, TMZ could be recommended as a salvage therapy at the estimated efficacy equal to procarbazine, lomustine, and vincristine (PCV) chemotherapy at first relapse. For patients previously treated with PCV, TMZ is a favorable therapeutic option as 2nd line salvage chemotherapy with an acceptable toxicity rate.

Isolated Oculomotor Nerve Palsy Following Minor Head Trauma : Case Illustration and Literature Review

Isolated oculomotor nerve palsy (ONP) attributable to mild closed head trauma is a distinct rarity. Its diagnosis places high demands on the radiologist and the clinician. The authors describe this condition in a 36-year-old woman who slipped while walking and struck her face. Initial computed tomography did not reveal any causative cerebral and vascular lesions or orbital and cranial fractures. Enhancement and swelling of the cisternal segment of the oculomotor nerve was seen during the subacute phase on thin-sectioned contrast-enhanced magnetic resonance images. The current case received corticosteroid therapy, and then recovered fully in 13 months after injury. Possible mechanism of ONP from minor head injury is proposed and previous reports in the literature are reviewed.

A Textiloma on the Pterion : A Rarely Occurred Craniotomy Complication

Textiloma is an inflammatory mass containing surgical sponges that are unintentionally left behind in a surgical wound. This complication has been most commonly described by abdominal and gynecologic surgeons. However, the occurrence of textiloma after intracranial procedures especially under the temporalis muscle has not been documented. The author reports a rare case of textiloma of the pterion in a patient who presented with a subcutaneous tumor developed eight years after frontotemporal craniotomy for aneurysm clipping.

Imaging Findings of Brain Death on 3-Tesla MRI

OBJECTIVE: To demonstrate the usefulness of 3-tesla (3T) magnetic resonance imaging (MRI) including T2-weighted imaging (T2WI), diffusion weighted imaging (DWI), time-of-flight (TOF) magnetic resonance angiography (MRA), T2*-weighted gradient recalled echo (GRE), and susceptibility weighted imaging (SWI) in diagnosing brain death. MATERIALS AND METHODS: Magnetic resonance imaging findings for 10 patients with clinically verified brain death (group I) and seven patients with comatose or stuporous mentality who did not meet the clinical criteria of brain death (group II) were retrospectively reviewed. RESULTS: Tonsilar herniation and loss of intraarterial flow signal voids (LIFSV) on T2WI were highly sensitive and specific findings for the diagnosis of brain death (p < 0.001 and < 0.001, respectively). DWI, TOF-MRA, and GRE findings were statistically different between the two groups (p = 0.015, 0.029, and 0.003, respectively). However, cortical high signal intensities in T2WI and SWI findings were not statistically different between the two group (p = 0.412 and 1.0, respectively). CONCLUSION: T2-weighted imaging, DWI, and MRA using 3T MRI may be useful for diagnosing brain death. However, SWI findings are not specific due to high false positive findings.

A Rathke's Cleft Cyst Presenting with Apoplexy

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.

A Case of Ectopic Rathke's Cleft Cyst in the Prepontine Cistern

A Rathke's cleft cyst (RCC) is a benign pituitary cyst derived from the remnant of Rathke's pouch, and usually presents as an intrasellar lesion with varying degrees of suprasellar extension. However, to date, a description of a primary prepontine RCC with no intrasellar component has not been reported. The author describes an exceptional case of a symptomatic RCC located behind the sella turcica in a 41-year-old woman who presented with severe headache. The author also provides an embryological hypothesis of the development of an ectopic RCC, with a special emphasis on radiologic characteristics.

Accuracy and Safety of Bedside External Ventricular Drain Placement at Two Different Cranial Sites : Kocher's Point versus Forehead

OBJECTIVE: External ventricular drain (EVD) is commonly performed with a freehand technique using surface anatomical landmarks at two different cranial sites, Kocher's point and the forehead. The aim of this study was to evaluate and compare the accuracy and safety of these percutaneous ventriculostomies. METHODS: A retrospectively review of medical records and head computed tomography scans were examined in 227 patients who underwent 250 freehand pass ventriculostomy catheter placements using two different methods at two institutions, between 2003 and 2009. Eighty-one patients underwent 101 ventriculostomies using Kocher's point (group 1), whereas 146 patients underwent 149 forehead ventriculostomies (group 2). RESULTS: In group 1, the catheter tip was optimally placed in either the ipsilateral frontal horn or the third ventricle, through the foramen of Monro (grade 1) in 82 (81.1%) procedures, in the contralateral lateral ventricle (grade 2) in 4 (3.9%), and into eloquent structures or non-target cerebrospinal space (grade 3) in 15 (14.8%). Intracerebral hemorrhage (ICH) >1 mL developed in 5 (5.0%) procedures. Significantly higher incidences of optimal catheter placements were observed in group 2. ICH>1 mL developed in 11 (7.4%) procedures in group 2, showing no significant difference between groups. In addition, the mean interval from the EVD to ventriculoperitoneal shunt was shorter in group 2 than in group 1, and the incidence of EVD-related infection was decreased in group 2. CONCLUSION: Accurate and safe ventriculostomies were achieved using both cranial sites, Kocher's point and the forehead. However, the forehead ventriculostomies provided more accurate ventricular punctures.

The Effect of Octreotide LAR on GH and TSH Co-Secreting Pituitary Adenoma

Growth hormone (GH) and thyroid stimulating hormone (TSH)-secreting pituitary adenomas are very rare and they account for only 0.5% for all pituitary adenomas. These adenomas are usually treated with surgery, but this surgery is not easy because the tumor is usually huge and invasive. We reported here on a case of a GH-TSH-secreting adenoma in a 23-year-old male patient who was initially treated with octreotide LAR. He presented with symptoms of headache, palpitation and a visual defect that he had for the 3 months. He had hypertrophy of the frontal bone and enlargement of both the hands and feet. The visual field test showed bitemporal hemianopsia. The laboratory examinations showed high serum levels of free T4, TSH and free alpha-subunit. Additionally, the serum levels of GH and insulin-like growth factor-I (IGF-I) were increased. GH was not suppressed below 1microg/L by an oral 75g glucose loading test, and TSH was not stimulated by thyrotropin-releasing hormone (TRH). Because sellar MRI showed invasive macroadenoma encasing the vessels, we initially tried octreotide LAR for treatment. A year later, the IGF-I and thyroid function tests were normalized and the size of the tumor was reduced with cystic change. The symptoms of palpitation and headache were improved without a change of the visual field defect.

Fibrous Dysplasia of the Clivus

Fibrous dysplasia (FD) of craniofacial structures is well documented, however, its involvement of the clivus is seldom described. We report a case of clival FD in a young man who presented with headache localized to the occipital area. The radiological studies revealed a monostotic disease confined to the clivus, with typical findings of hypointensity on magnetic resonance images and ground-glass density on computed tomography. The diagnosis of FD was confirmed on pathological examination of specimens taken through transsphenoidal surgery. The patient showed reduction of symptoms and no change of residual lesion on follow-up imaging taken 2.5 years later after surgery. This study includes clinical aspect, radiographic appearance, differential diagnosis and treatment strategy of this rare skull base lesion.

A case of TSH-secreting pituitary adenoma with acromegaly / 대한내과학회지

Growth hormone (GH) and thyrotropin (TSH)-secreting pituitary adenomas are very rare and account for only 0.5% of all pituitary adenomas. We report a case of a GH/TSH-secreting pituitary adenoma in a 53-year-old male patient. He presented with symptoms of thyrotoxicosis, clinical features of acromegaly, and diabetes mellitus. The laboratory examinations showed high serum levels of free T4, TSH, and free alpha-subunit. Additionally, serum levels of GH and insulin-like growth factor (IGF-1) wereincreased. GH was not suppressed below 1 microgram/L by an oral 75 g glucose loading test, and TSH was not stimulated by thyrotropin- releasing hormone. A sellar MRI showed a large lobulated mass on the pituitary gland, so transcranial surgery was performed. Immunohistochemical staining showed anti-GH and anti-TSH positive tumor cells in the cytoplasm. Serum GH, IGF-1, free T4, and TSH levels normalized after surgery.

Nonaneurysmal Subarachnoid Hemorrhage : Rare Complication of Vertebroplasty

On rare occasions, percutaneous vertebroplasty (PV) may be associated with adverse spinal and extraspinal events. Subarachnoid hemorrhage (SAH) has not been reported complication following a PV. This is a report of two elderly women with spine compressions who developed idiopathic SAH after injecting polymethylmethacrylate into the thoracolumbar region transcutaneously. PV was performed as an usual manner on prone position under local anesthesia for these patients. During the interventions, two patients complained of a bursting nature of headache and their arterial blood pressure was jumped up. Computed tomography scans revealed symmetric SAH on the both hemispheres and moderate degree of hydrocephalus. Any intracranial vascular abnormalities for their SAH were not evident on modern neuroangiography modalities. One patient received a ventricular shunt surgery, but both fully recovered from the procedure-related SAH. The pathophysiologic mechanism that induce SAH will be discussed, with suggesting the manner that prevent and minimize this rare intracranial complication after PV.

Spontaneous Intracranial Hypotension: Clinical Presentation, Imaging Features and Treatment

OBJECTIVE: In the present study, the authors investigated the clinical and imaging features as well as the therapeutic outcomes of SIH (spontaneous intracranial hypotension) patients. METHODS: A retrospective review of 12 SIH patients was carried out. The diagnostic work-up included lumbar tapping and measurement of CSF opening pressure, radioisotope cisternography, brain and spinal magnetic resonance imaging (MRI), and computed tomography (CT) myelography. Autologous epidural blood patching was performed in patients who did not respond to conservative therapies, including analgesics, steroids, hydration and rest. RESULTS: Typical postural headache was found in 11 (91%) patients. Nine (75%) patients showed pachymeningeal enhancement on their initial T1-weighted MR images. The CSF opening pressure was less than 60 mmH2O in 9 of 11 patients. Autologous epidural blood patching was performed in 7 patients, and all of them showed good responses. CONCLUSION: SIH can present with various clinical presentations and neuroimaging findings. Autologous epidural blood patching is thought to be the treatment of choice for patients with SIH.

Silent Microbleeds and Old Hematomas in Spontaneous Cerebral Hemorrhages

OBJECTIVE: The authors studied the risk factors of silent cerebral microbleeds (MBs) and old hematomas (OHs) and their association with concurrent magnetic resonance (MR) imaging findings in the patients of intracerebral hemorrhages (ICHs). METHODS: From April 2002 to June 2007, we retrospectively studied 234 patients of primary hemorrhagic stroke. All patients were evaluated with computed tomography (CT) and 3.0-tesla MR imaging studies within the first week of admission. MBs and OHs were assessed by using T2*-weighted gradient-echo (GRE) MR imaging. The patients were divided into 2 groups, depending on whether or not they had two GRE lesions of chronic hemorrhages. A correlation between MBs and OHs lesions were also statistically tested. Lacunes and white matter and periventricular hyperintensities (WMHs, PVHs) were checked by T1- and T2-weighted spin-echo and fluid attenuated inversion recovery sequences. Variables on the clinical and laboratory data and MR imaging abnormalities were compared between both groups with or without MBs and OHs. RESULTS: MBs were observed in 186 (79.5%) patients and a total of 46 OHs were detected in 45 (19.2%) patients. MBs (39.6%), OHs (80.4%), and ICHs (69.7%) were most commonly located in the ganglionic/thalamic region. Both MBs and OHs groups were more frequently related to chronic hypertension and advanced WMHs and PVHs. The prevalence and number of MBs were more closely associated with OHs groups than non-OH patients. CONCLUSION: This study clearly demonstrated the presence of MBs and OHs and their correlation with hypertension and cerebral white matter microangiopathy in the ICHs patients. Topographic correlation between the three lesions (MBs, OHs, and ICHs) was also noted in the deep thalamo-basal location.

Cerebellar Ganglioglioma

The location of ganglioglioma (GG) within the infratentorial compartment is unusual. The authors report a rare case of GG in the cerebellar hemisphere. A 12-year-old boy suffered from headache and gait disturbance. Neuroimaging studies demonstrated a large enhancing cerebellar mass with cystic components compressing the forth ventricle. After complete resection of the tumor, the patient became symptom free. Histological examination on the tumor disclosed glial cells and dysplastic ganglion cells. Although it is a rare tumor, in the appropriate clinical setting, a GG should be considered in the presence of a cerebellar mass with both solid and cystic components on magnetic resonance images in children.

Spinal Epidural Cavernous Angioma: A Case Report

Spinal extradural cavernous angiomas(CAs) are apparently rare lesions. The author report a 67 year old man with an epidural CA at T9-10 space presenting with progressive leg weakness. Magnetic resonance imaging showed a thoracic epidural lesion appearing as isointense on T1-weighted with strong contrast enhancement and hyperintense on T2-weighted images. A moderate hyperdensity and foraminal widening was found on computed tomography. Hemilaminectomy was performed to relieve the spinal cord compression. A highly vascular tumor without bony involvement was observed in the epidural space. Histopathology of the lesion revealed a typical CA. The patient's symptoms resolved after treatment. Clinical aspect, radiological features, and surgical treatment of this rare entity will be reviewed.

Intracranial Fusiform Aneurysms: It's Pathogenesis, Clinical Characteristics and Managements

OBJECTIVE: The objective of this study is to investigate clinical characteristics, management methods and possible causes of intracranial fusiform aneurysm. METHODS: Out of a series of 2,458 intracranial aneurysms treated surgically or endovascularly, 22 patients were identified who had discrete fusiform aneurysms. Clinical presentations, locations, treatment methods and possible causes of these aneurysms were analyzed. RESULTS: Ten patients of fusiform aneurysm were presented with hemorrhage, 5 patients with dizziness with/without headache, 4 with ischemic neurologic deficit, and 1 with 6th nerve palsy from mass effect of aneurysm. Two aneurysms were discovered incidentally. Seventeen aneurysms were located in the anterior circulation, other five in the posterior circulation. The most frequent site of fusiform aneurysm was a middle cerebral artery. The aneurysms were treated with clip, and/or wrapping in 7, resection with/without extracranial-intracranial (EC-IC) bypass in 6, proximal occlusion with coils with/without EC-IC bypass in 5, EC-IC bypass only in 1 and conservative treatment in 3 patient. We obtained good outcome in 20 out of 22 patients. The possible causes of fusiform aneurysms were regard as dissection in 16, atherosclerosis in 4 and collagen disease or uncertain in 2 cases. CONCLUSION: There is a subset of cerebral aneurysms with discrete fusiform morphology. Although the dissection or injury of internal elastic lamina of the cerebral vessel is proposed as the underlying cause for most of fusiform aneurysm, more study about pathogenesis of these lesions is required.