[A Surgical Case of Chiari Malformation Type I in Elderly Patient].

Chiari malformation type I (CM-1) is a disease in which part of the cerebellum and brainstem invades into the spinal canal beyond the foramen magnum. Patients with CM-1 can present with various symptoms; however, most cases of CM-1 are asymptomatic. Symptomatic cases are distributed bimodally in children and middle-aged adults, but occur very rarely in elderly individuals. We experienced a case of CM-1 onset after the age of 60 years that followed a favorable postoperative course. We report the potential mechanism of asymptomatic CM-1 in elderly patients along with a review of the literature.

Clinical evaluations of pituitary apoplexy in incidental nonfunctional pituitary adenomas.

Pituitary apoplexy is an uncommon syndrome that often results in spontaneous hemorrhage or infarction of pituitary tumors or glands. We previously reported pituitary apoplexy occurred most frequently in nonfunctional pituitary adenomas among all types of pituitary incidentalomas. In the present study, we aimed to investigate the characteristics of pituitary apoplexy in patients with incidental nonfunctional pituitary adenomas. 65 patients with pituitary incidentaloma were enrolled. All patients underwent clinical/endocrinological/pathological investigations. As a result, 33 patients were diagnosed with nonfunctional pituitary adenomas. Of these, 12.1% of patients had pituitary apoplexy. There was no difference in tumor diameter, age, or sex between the apoplexy and the non-apoplexy groups. However, the liver enzymes aspartate transaminase and alanine aminotransferase were significantly higher, and plasma sodium and chloride levels were significantly lower in the apoplexy group than in the non-apoplexy group (each P < .05). In addition, low-density lipoprotein-cholesterol was significantly higher in the apoplexy group than in the non-apoplexy group (P < .05). Besides, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone, and prolactin deficiencies were significantly more frequent in the apoplexy group than in the non-apoplexy group (each P < .05), and growth hormone and adrenocorticotropic hormone deficiencies were more frequent in the apoplexy group than in the non-apoplexy group (P = .09 and.08, respectively). Furthermore, tumor diameter was not associated with pituitary apoplexy, whereas thyroid-stimulating hormone, luteinizing hormone, and follicle-stimulating hormone deficiencies were significantly associated with the apoplexy group (each P < .05). Hence, the present study indicated that pituitary apoplexy could not be related to tumor diameter. Moreover, hormonal deficiencies, hepatic dysfunction, hyponatremia or hypochloremia, and dyslipidemia might be indicators of pituitary apoplexy. There could be the possibility the treatment for dyslipidemia prevents pituitary apoplexy.

Changes in vascular supply pattern associated with growth of nonfunctioning pituitary adenomas.

Background: The vascular supply to nonfunctioning pituitary adenomas (NFPAs) differs compared with that of the anterior lobe of the normal pituitary gland. In this study, we aimed to identify feeding arteries and flow dynamics using 3.0 T magnetic resonance imaging (MRI) in NFPAs. Methods: We divided 77 cases of NFPA into three groups according to the time-intensity curve (TIC) pattern by dynamic MRI. We also investigated the presence of feeder arteries as a flow void signal on T2-weighted imaging (T2WI). Results: According to the TIC, 39 cases demonstrated an ascending pattern, 10 cases demonstrated a descending pattern, and 28 cases demonstrated a monophasic pattern. Tumor size in the ascending group was larger compared with the descending group (P = 0.0036). Flow void signals were identified in 51 of 77 cases (66.2%) on T2WI. Tumor size was larger in tumors with a flow void signal compared with those without (P < 0.0001). Flow void signals were more frequently observed in the group of ascending pattern compared with the group of monophasic and descending pattern (P = 0.032 and P = 0.003, respectively). Particularly on the caudal side, the difference between the ascending group and the monophasic and descending groups was remarkable (P = 0.0035 and P < 0.0001, respectively). Conclusion: We successfully evaluated the blood supply pattern by the TIC analysis and identified flow voids using 3.0 T MRI. Blood supply pattern was significantly associated with NFPA size. These results suggested that NFPA hemodynamics changes during tumor growth.

A Patient with a Type I Split Cord Malformation and an Open Myelomeningocele without Advanced Lower Limb Paresis: A Case Report and a Review of the Literature.

We report a rare case of a split cord malformation (SCM) combined with an open myelomeningocele (MMC) on the right hemicord. The patient was a male neonate, who exhibited an MMC in the lumbosacral region at birth. Both of his lower limbs moved with slight spasticity, but no atrophic changes or clubfoot deformities were seen. Three-dimensional computed tomography (CT) demonstrated a bony septum, and the patient was diagnosed with a type I SCM. Magnetic resonance imaging (MRI) showed an MMC on the right hemicord (a hemimyelomeningocele). The repair of the open MMC and the removal of the septum were performed immediately to prevent infection and neurological deterioration. Intraoperatively, the right hemicord was thinner than the left hemicord. No additional neurological deficits or complications appeared during treatment. Our findings suggest that when a minor hemicord is affected by both an SCM and an open MMC, good functional outcomes of the lower limbs can be achieved.

[Multiloculated Hydrocephalus Complicated by Meningitis and Ventriculitis Treated with Staged Fenestration:A Case Report].

Multiloculated hydrocephalus following severe meningitis with ventriculitis is often therapeutically challenging. Neonatal meningitis is commonly associated with ventricular inflammation, and approximately 30% of patients show septum formation. Although placement of a single ventriculoperitoneal shunt system could serve as optimal treatment for a multiloculated cerebrospinal cavity that is converted into a single chamber, multiple devices are often required for disease stability. We report a case of multiloculated hydrocephalus that occurred after meningitis in a patient who was successfully treated with a single shunt system using staged multimodality treatments.

[Seven Patients with Pituitary Metastases Treated at Our Department:Case Reports].

Pituitary metastases(PM)are rare and show a poor prognosis. However, recent advances in diagnostic imaging could increase the chances of PM being diagnosed without a history of cancer. Furthermore, it was unclear whether adjuvant therapy could increase the survival of patients with PM or not. To clarify the clinical course of patients with PM, we report seven cases of PM with a literature review. Most patients showed symptomatic adenohypophyseal dysfunction(AD)and diabetes insipidus(DI)as initial symptoms. All patients underwent radiotherapy for PM and showed good local tumor control. However, except for one patient with improved DI, neither AD nor DI improved with radiotherapy. As for the prognosis, three patients with PM without a history of cancer survived longer than those with a history of cancer(20.3 vs. 11.7 months, respectively). In summary, early diagnosis and appropriate hormone replacement therapies are important in PM. Improvement of the general condition enables adjuvant therapy to prolong patient survival.

[A Case of Unruptured Internal Carotid Artery Aneurysm with Improved Endocrinological Function after Treatment].

We experienced a case of unruptured internal carotid artery aneurysm improved endocrinological function after the treatment. A 68-year-old woman was admitted to our hospital complaining of general fatigue, dizziness, and decreased visual acuity. Radiological examination revealed unruptured large aneurysm at the right anterior carotid artery compressing on the pituitary gland. We underwent right STA-MCA bypass and trapping of right internal carotid artery. Post-operative course was uneventful. Although visual function was not improved, her endocrinological function was improved 8 months after surgery by thrombosed and shrunken aneurysm. The mechanism of panhypopituitarism due to aneurysm has been suggested to involve mechanical compression on the pituitary gland, pituitary stalk, or hypophyseal artery. Although it was unclear about the improvement of endocrine function after the treatment of aneurysm, some cases could recover the hypopituitarism after enough follow-up period.

Clinical investigation of pituitary incidentalomas: A two-center study.

Recent advances in imaging technology resulted in an increase in pituitary incidentalomas (PIs) detection. PIs were reported to be present in 1.6% persons with magnetic resonance imaging of the brain. Whereas, there were few studies about PIs with detailed investigation. We aimed to investigate the clinical and endocrinological characteristics of PIs. We evaluated 65 patients diagnosed with PIs who underwent detailed clinical and endocrinological evaluations. Of the 65 patients, 33 (50.8%) had non-functional pituitary adenomas (NFPAs), 11 (16.9%) had Rathke's cleft cysts (RCCs), 7 (10.8%) had functional pituitary adenomas (FPAs), 6 (9.2%) had benign extra-pituitary tumors (BEPTs), and 8 (12.3%) had malignant tumors (MTs). Compared with patients with NFPAs, those with MTs were significantly younger and had a significantly lower body mass index, lower prevalence of hypertension, and lower prevalence of dyslipidemia. Patients with MTs had significantly higher prevalence of central diabetes insipidus than those with NFPAs. In addition, patients with NFPAs had significantly higher prevalence of pituitary apoplexy than those with FPAs, BEPTs, and MTs. In conclusion, our study demonstrated clinical and endocrinological characteristics of PIs. Highly detailed clinical and endocrinological investigations should be performed for PIs. In addition, MTs should be considered in the differential diagnosis for young and lean patients with central diabetes insipidus.

[Treatment of Rathke's Cleft Cyst:Technical Note for Preservation of Pituitary Function].

Pituitary dysfunction, such as panhypopituitarism or diabetes insipidus(DI), is often found in patients with Rathke's cleft cyst. Patients were treated with transsphenoidal microscopic surgery; however, pituitary dysfunction did not usually recover. Recently, endoscopic transsphenoidal surgery(eTSS)has enabled minimally invasive surgery for patients with Rathke's cleft cyst. In this study, we analyzed 22 consecutive patients with Rathke's cleft cyst who underwent eTSS to determine if pituitary dysfunction recovered. The follow-up period ranged from 3 months to 19.25 years(mean, 4.75 years). Preoperative endocrinological evaluation showed impaired secretion of adrenocorticotropic hormone(ACTH)in 4 cases(18.2%), thyroid-stimulating hormone(TSH)in 2 cases(9.1%), hyperprolactinemia in 5 cases(22.7%), growth hormone(GH)in 9 cases(40.9%), and luteinizing hormone(LH)/follicle-stimulating hormone(FSH)in 11 cases(50%). In addition, preoperative DI was found in 2 cases(9.1%). We planned the site of fenestration for the cyst wall using preoperative sagittal magnetic resonance imaging. As a result, the recovery rate for ACTH, GH, and TSH secretion was 25%, 33.3%, and 50%, respectively. On the other hand, two patients with DI and other hormonal deficiencies did not recover pituitary function because of severe inflammation. Pituitary function might be preserved with minimally invasive surgery for Rathke's cleft cyst with mild inflammation.

The Likelihood of Remnant Nonfunctioning Pituitary Adenomas Shrinking Is Associated with the Lesion's Blood Supply Pattern.

OBJECTIVE: Nonfunctioning pituitary adenomas (NFPA) often shrink after transsphenoidal surgery. However, little is known about the predictors of spontaneous NFPA regression. The aim of this study was to determine whether the blood supply pattern of remnant NFPA lesions was associated with the likelihood of such lesions shrinking. METHODS: A total of 37 remnant tumors in 31 patients who were treated at the Department of Neurosurgery, Yamagata University Hospital, were included in this study. All patients underwent preoperative dynamic 3.0T magnetic resonance imaging (MRI) to evaluate their tumors' arterial blood supplies, followed by endoscopic transsphenoidal surgery and intraoperative 1.5T MRI. Follow-up MRI scans were obtained at 1-2 weeks and 3-6 postoperative months. RESULTS: We detected tumor shrinkage in 15 of 37 (40.5%) remnant tumors on follow-up MRI scans obtained at 3-6 postoperative months. Remnant tumors were found in rostral and caudal locations in 21 and 16 cases, respectively. Rostral remnant tumors were significantly more likely to shrink (P < 0.0001). The tumors were classified into 3 groups according to their blood supply patterns (23 ascending, 6 descending, and 2 monophasic). The ascending blood supply pattern was found to be a positive predictor of tumor shrinkage (P = 0.002). Furthermore, no remnant tumors with rich blood supplies underwent spontaneous regression (P < 0.0001). CONCLUSIONS: Evaluations of the blood supplies of remnant NFPA via preoperative dynamic MRI and the locations of the remnant tumors could be useful for predicting postoperative tumor shrinkage.

[A Case of Lymphocytic Adenohypophysitis Presenting Visual Disturbance in the Third Trimester of Pregnancy].

Lymphocytic hypophysitis(LH)has first been described as an autoimmune endocrinopathy by Goudie in 1962. In particular, lymphocytic adenohypophysitis(LAH)is usually associated with pregnancy and hypopituitarism due to insufficient endocrine of ACTH. However, several cases of LAH in pregnant patients showing only visual disturbances have recently been documented. We treated a patient with LAH presenting only the chiasma syndrome in the third trimester without hypopituitarism. A 27-year-old woman unexpectedly experienced visual disturbance starting in the 28th week of pregnancy. Her symptoms progressed rapidly. MRI revealed a pituitary mass lesion compressing the optic chiasma. In addition, ophthalmological examination revealed bitemporal hemianopsia. The patient underwent endoscopic transsphenoidal surgery(eTSS)during the 30th week of pregnancy. LH was diagnosed histologically during surgery. We performed decompression of optic chiasma. After surgery, the patient's visual field markedly widened and the pituitary mass regressed along with replacement of corticosteroids. In the 37th week of pregnancy, she delivered a healthy baby. We speculate that the reason for the absence of hypopituitarism during pregnancy in patients with LH, especially in the third trimester, might be that the placental endocrine system masks pituitary endocrinopathy. In summary, we report a case of LAH that did not present with hypopituitarism, and eTSS could be performed safely during pregnancy.

[A case of calcified chronic subdural hematoma wherein MRI was useful for decision of the treatment strategy].

Calcified chronic subdural hematoma is a rare condition, representing 0.4-2.6% of all chronic subdural hematomas. It is often difficult to remove the hematoma without damaging the brain, owing to the adhesion between hematoma capsule and brain. Therefore, surgical intervention in managing calcified chronic subdural hematoma cases is still considered controversial. We report a case of calcified chronic subdural hematoma, which was successfully performed under microscopic guidance. A 72-year-old man complained of progressive left hemiparesis of 6-month duration. A CT scan revealed a calcified chronic subdural hematoma, 10 cm long and 4.5 cm thick, in the right convexity. On MRI, T2-weighted images showed a thin layer of cerebrospinal fluid intensity between the hematoma capsule and brain. There was no brain edema adjacent to the chronic subdural hematoma. Based on these MRI observations, we believed that adhesion between the hematoma capsule and brain would be mild. We then planned and succeeded in total removal of the calcified chronic subdural hematoma mass under microscopic guidance. The left hemiparesis disappeared after surgery. The patient was discharged without any neurological deficit. While considering surgical management in calcified chronic subdural hematoma cases, it should be important to evaluate adhesion between the hematoma capsule and brain with MRI.

[A case of Churg-Strauss syndrome with subarachnoid hemorrhage].

Churg-Strauss syndrome (CSS) is a vasculitis syndromes and is only rarely complicated by subarachnoid hemorrhage. In the current report, we describe a case of CSS with subarachnoid hemorrhage, which showed a favorable outcome following conservative treatment. A 68-year-old man with CSS on maintenance steroid therapy underwent MRI/A during tinnitus aggravation, and showed dilation of the left middle cerebral artery and stenosis of the peripheral area of the right vertebral artery. After 2 months, he presented sudden pain in the occipitocervical area, and CT revealed subarachnoid hemorrhage. Intracranial 3D CT-A and MRI/A showed the development of a protrusion at the base of the left anterior cerebral artery. Although both findings suggested cerebral artery dissection, the source of hemorrhage could not be identified. The 2009 Japanese Guidelines for the Management of Stroke recommends early diagnosis and treatment of hemorrhagic cerebral artery dissection because of the high risk of re-bleeding. However, considering the risks of vasculitis aggravation, development of systemic complications, and recurrence, conservative treatment was selected. In addition, owing to the risk of complications associated with the frequent use of iodinated contrast agents and angiography procedures, patient was followed up using MRI. His course was favorable, and he was discharged despite mild right abducens paralysis. When patients with hemorrhagic cerebral artery dissection have a history of allergic diseases, CCS should be considered; conservative treatment consisting of rest, strict blood pressure control, and steroid therapy may be the most appropriate option for certain patients.

Bilateral carotid and vertebral rete mirabile presenting with subarachnoid hemorrhage caused by the rupture of spinal artery aneurysm.

Rete mirabile (or carotid rete) is a normal structure that plays physiological roles in the lower mammals. However, the rete does not exist in the normal carotid circulation of humans. Carotid rete mirabile (CRM) is a rare condition compensating for congenital dysplastic internal carotid artery. Arterial plexus at the cavernous region, which supplies intradural internal carotid artery instead of the aplastic cavernous portion of internal carotid artery, looks like the "rete mirabile" seen in the lower mammals, and is a characteristic angiographical finding of CRM. In addition to the CRM, existence of segmental occlusion and tortuous collaterals of vertebral artery, so-called carotid and vertebral rete mirabile (CVRM), is a very rare condition. We report a 70-year-old female patient with bilateral CVRM presenting with subarachnoid hemorrhage (SAH) caused by the rupture of a cervical spinal artery aneurysm. Our patient is the oldest, compared with the previously reported four patients with CVRM. Moreover, this is the first report of ruptured spinal artery aneurysm as a cause of SAH associated with CRM/CVRM. To avoid rebleeding in the patient, we successfully treated the patients by performing coil embolization of the remaining spinal aneurysms. In patients with CVRM, aneurysm formation of the cervical spinal artery may be a reasonable consequence because of the hemodynamic stress on the spinal artery as a collateral pathway. Detailed evaluation of the cervical spinal arteries should be performed to detect or to rule out ruptured aneurysm in patients with SAH associated with CVRM.

[Spontaneous regression of primary intracranial germinoma: a case report].

Here we report a case in which an intracranial germinoma displayed spontaneous regression. An 11-year-old boy presented with polyuria and headache. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed tumors in the suprasellar and pineal regions, and obstructive hydrocephalus. As repeat MRI demonstrated shrinkage of these tumors, resection was deferred. The patient was discharged and followed up with serial MRI. The tumor continued to regress for three weeks; however, the patient was readmitted due to tumor regrowth. We performed endoscopic biopsy, and histopathologic diagnosis was germinoma. The patient underwent three courses of combined chemotherapy and radiotherapy, and complete response was achieved. Although the precise cause of the transient regression is unknown, racranial germinoma may occasionally unde rgo spontane ous regression.

[Sacrococcygeal dermal sinus presented bacterial meningitis: a case report].

A rare case of sacrococcygeal dermal sinus suffering from relapsing meningitis is presented. In addition, deference of clinical figure and pathology between sacrococcygeal region and other regions are discussed. A 10 -month-old boy was suffering from relapsing meningitis. Spinal MRI was performed to find occult spinal dysraphism because he had a pit on his hip. The MRI revealed sacrococcygeal dermal sinus. He received surgical resection of dermal sinus based on this findings. Interestingly, different from other dermal sinuses, the dermal sinus runs to caudal direction, and was fused filum terminale with dermoid cyst. This interesting pathological feature of sacrococcygeal dermal sinus seems to come from developmental difference between sacrococcygeal dermal sinus and the other dermal sinuses. Only sacrococcygeal dermal sinus is related with caudal cell mass on its pathogenesis. Because filum terminale is also developed from caudal cell mass, sacrococcygeal dermal sinus seems to be related with filum terminale. Most important differential diagnosis is coccygeal pit that is found in 1 -4% of newborn baby. The clinical feature and pathology got from our case must be very helpful to distinguish sacrococcygeal dermal sinus from coccygeal pit.