A follow-up angiography study must be performed during the early stage (within approximately 3 weeks after onset of symptoms) to confirm the formation or enlargement of an aneurysm, because such conditions may be amenable to surgical treatment. Unruptured VA dissection could otherwise be treated and followed conservatively. Although the majority of dissected lesions seem likely to stabilize within a few months, as evidenced on angiography, in some cases a longer observation period is required.

A 34-year-old man with a 1-week history of diplopia was referred to the authors' hospital. Neurological examination revealed left abducens nerve palsy. Computed tomography showed a lesion in the left sphenoid sinus involving the medial wall of the left internal carotid artery (ICA) and osteolytic change at the clivus bordering the lesion. Magnetic resonance imaging demonstrated an extensive soft-tissue mass occupying the left sphenoid sinus. Surgical intervention by the endoscopic transnasal method allowed most of the lesion to be removed. Only the portion attached to the medial wall of the ICA was not removed. Postoperatively, the lesion was diagnosed as a giant cell tumor (GCT) and the patient received 120 mg of subcutaneous denosumab every 4 weeks, with additional doses on Days 8 and 15 during the first month of therapy. MRI a week after starting denosumab revealed shrinkage of the initially fast-growing residual tumor. The patient was discharged upon completion of the third denosumab administration. GCT is an aggressive stromal tumor developing mainly in young adults. Complete resection is recommended for GCT in the literature. However, size and location of the CGT often limit this approach. Various adjuvant treatments for skull base GCTs have been reported, including radiation and chemotherapy. However, the roles of adjuvant therapies have yet to be clearly defined. Denosumab, a monoclonal antibody, was recently approved for GCT in several countries. Denosumab may permit less invasive treatments for patients with GCTs while avoiding deleterious outcomes, and may also limit disease progression and recurrence.

A 67-year-old female presented with a rare metastatic skull tumor from cholangiocarcinoma (CCC) manifesting as a progressive painful subcutaneous tumor. Computed tomography and magnetic resonance (MR) imaging revealed an osteolytic tumor attached to the sigmoid sinus and mastoid sinus. Mass reduction of the tumor was performed and radiotherapy applied to the postoperative cavity. The symptoms resolved following the surgical intervention. The skull metastasis from CCC appeared as heterogeneous intensity on MR imaging reflecting the honeycomb structure. Surgery should be considered to relieve symptoms and improve the patient's quality of life, if there is a low risk of morbidity or mortality. The present case indicates another metastatic pathway through the vertebral plexus in the clinical course of CCC.

BackgroundIsolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH).Case presentationA 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly.ConclusionIsolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.

The improvement of rCMRO(2) correlated with the response to VPS. Significant changes in rOEF might predict poor response to VPS.

Fluorine-18-fluorodeoxyglucose positron emission tomography ([ 18 F]FDG PET) was assessed as a method for providing information about the malignancy of orbital tumors. Twelve patients with 13 orbital tumors underwent [ 18 F]FDG PET followed by biopsy or tumor removal via a transcranial approach. The accumulation ratio between the tumor and the contralateral normal tissue (T/N ratio) was calculated for 10 of the 13 lesions. The T/N ratio in benign lesions was compared with that in malignant tumors. Histological examination identified 7 lesions as malignant: anaplastic astrocytoma of the optic nerve in 1 patient, which recurred as glioblastoma of the optic nerve, malignant lymphoma of mucosaassociated lymphoid tissue type in 1 patient, malignant melanoma in 1 patient, adenoid cystic carcinoma in 2 patients, and adenocarcinoma (unknown origin) in 1 patient. The T/N ratio was 1.06 ± 0.03 (mean ± standard deviation) in benign tumors, and significantly higher at 1.81 ± 0.27 in malignant tumors (p ＝ 0.0027). Both patterns of high and iso uptake of [ 18 F]FDG were found in orbital pseudotumor. [ 18 F]FDG PET can determine the malignancy of orbital tumors, but cannot distinguish malignant tumor from inflammatory disease such as pseudotumor.

A 1-year 9-month-old girl presented with achondroplasia. Serial magnetic resonance (MR) imaging demonstrated mild compression of the medulla oblongata by the occipital bone, macrocrania, and progressive hydrocephalus. Cerebrospinal fluid (CSF) flow study using MR imaging clearly demonstrated CSF flow disturbance at the cervicospinal junction. Foramen magnum decompression was performed for her hydrocephalus and compressed medulla. Postoperative CSF flow study demonstrated improvement of CSF flow at the craniocervical junction. The patient has remained in a stable condition for 7 months postoperatively. Achondroplasia represents hydrocephalus or medullary compression caused by narrowed foramen magnum, which can result in sudden death in some infants. Surgical indications and methods for hydrocephalus combined with achondroplasia remain controversial because the natural history of the hydrocephalus has remained unclear. CSF flow study using MR imaging can provide useful information regarding the surgical indication and methods for the treatment of hydrocephalus combined with achondroplasia.

A 14-year-old girl was referred to our department because of headache and visual impairment following the resection of recurrent cardiac myxoma. Head magnetic resonance imaging (MRI) scan detected an intra-and supra-sellar tumor. Moreover, the patient showed the presence of spotty skin pigmentations on her cheeks and lower lip. Blood examination revealed hypothyrotropinemia, and ultrasonography results revealed multiple thyroid nodules. She was diagnosed with Carney complex (CNC). Her pituitary tumor was suspected as growth hormone (GH)-secreting adenoma, because overgrowth was observed in the patient. However, biochemical examinations, including oral glucose tolerance test, failed to show the characteristic findings of GH-secreting adenoma. In contrast, insulin tolerance test showed GH deficiency. Her visual impairment improved without performing decompression surgery, and the tumor size decreased, as per the MRI findings. Based on clinical course, the patient was diagnosed with pituitary apoplexy in pituitary adenoma, following which she was discharged. At 3 months after discharge, thyrotropin-releasing hormone loading test performed revealed low thyrotropin-stimulating hormone and thyroid hormone levels, and the patient was in a depressed mood. Therefore, l-T4 replacement was initiated, following which her GH secretory capacity gradually improved. Here, we report, to the best of our knowledge, the first case of a patient with pituitary apoplexy in CNC. Such condition must be identified in young patients with recurrent cardiac myxoma, and examinations, such as head MRI, must be performed.