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Objective: Erdheim-Chester disease(ECD)is a rare type of non-Langerhans histiocytosis. We report a surgical case of ECD with multiple lesions at the falx cerebri, tentorium cerebelli, and in the suprasellar region, with a literature review., Case Report: A 70-year-old woman presented with frequent falling and difficulty in standing. Her past medical history revealed ovarian cystectomy, transient thrombocytopenia, hypertension, left lower leg pain, and overactive bladder. Her head CT and MRI findings revealed well-defined mass lesions, suspected of meningioma, at the falx cerebri and tentorium cerebelli. Craniotomy and near total resection of the tumor at the falx cerebri was performed, leaving a hard portion of the tumor on the right falx. Intraoperative findings showed a solid and hard tumor, which was extremely difficult to decompress. Although the histopathological diagnosis was originally a metaplastic meningioma, considering her complaints of lower leg pain, we suspected ECD and performed a right tibial biopsy. The right tibial biopsy revealed ECD. Twenty-two months after the operation, the patient exhibited a marked enlargement of the tentorium lesion and a new lesion in the suprasellar region. Resection of the tentorial lesion was performed. The second intraoperative findings were similar to those of the first. The histopathological diagnosis of the tentorial lesion was ECD. After the surgeries, steroid therapy and radiation therapy were performed, but only with temporary improvement., Conclusion: ECD is a rare disease; therefore, accumulation of clinical data to establish its treatment is necessary.

Microvascular decompression is now a standard surgical technique for the treatment of trigeminal neuralgia. However, it is occasionally difficult to expose the trigeminal nerves because of the high anatomical variety of vascular or bony structures in the posterior fossa. We reported the case of a 59-year-old woman with trigeminal neuralgia whose site of neurovascular compression could not be observed in microvascular decompression. On approaching the trigeminal nerve, the suprameatal tubercle was so prominent that it prevented adequate visualization of the nerve tract. After drilling out the tubercle concealing the trigeminal nerve behind it, we exposed the nerve entirely and subsequently decompressed it from the superior cerebellar artery. Retrospectively, the suprameatal tubercle was found 3mm high above the posterior surface of the petrous bone. Then, we analyzed the height of suprameatal tubercles in 106 patients who underwent three-dimensional CT of the skull. Mean values of the suprameatal tubercles were 1.4-1.7mm in height, and 5.2% of them were higher than 3mm. The result suggested the high morphological variety of the petrous bone. We emphasize the importance of presurgical evaluation of the petrous bone in trigeminal neuralgia, because the neurovascular compression site may not be exposed sufficiently by the suprameatal tubercle in approximately 5% of the patients.

A 58-year-old male was admitted to our hospital because of repeated transient ischemic attack of right hemiparesis and speech disturbance. A CT scan did not demonstrate any remarkable findings. No significant cerebral arterial stenosis or occlusion was found on angiography. However, dural arteriovenous fistula fed by the radiculo-meningeal artery was found at the cranio-cervical junction on left vertebral angiography. The shunt flow from the arteriovenous fistula drained into the superior petrosal sinus and sigmoid sinus in a retrograde fashion. On the angiographic findings when his transient ischemic attack (TIA) had disappeared, the venous drainage had returned to its normal fashion. Venous hypertension around the brain stem was supposed to have caused the transient ischemic attack in this case. We performed coagulation of the draining vein and fistula surgically. After surgery, the patient's TIA completely disappeared. We report the first case of dural arteriovenous fistula at the cranio-cervical junction presenting transient ischemic attack.

A computer-assisted battery for neuropsychological tests (CNT) has been designed to screen adults for cognitive impairment. The aim of this study was to gather evidence for the construct validity of CNT and also investigate the relationship between CNT and conventional neuropsychological tests. Subjects were 45 healthy adults (21 men and 24 women), who ranged in age from 20 to 70 years (mean = 33.5, SD = 1.9) with no history of substance abuse, or of psychotic or neurological disorders. The CNT in our study consists of six subtests designed to assess various components of driving, such as digit span, visual scanning, visual and verbal memory, complex reaction time, and vigilance. Mini-mental state test, Kana-hiroi test, word fluency, the auditory-verbal learning test and Raven's colored progressive matrices were also performed as conventional neuropsychological tests. Results showed there were high correlations between each CNT subtests and conventional neuropsychological tests. A factor analysis (with varimax rotation) identified 4 factors with eigen values greater than 1, which accounted for over 70% of the variance. CNT was able to estimate each factor related to cognitive function such as learning and memory, attention, judgment, and visual scanning selectively. CNT may thus be a useful tool for detection of cognitive impairment, although this test has important limitations. Broader applications of these tests will require extensive population-based validation.

A case of agraphia due to cerebral infarction in the left parietal lobe was reported. A 63-year-old right-handed man was admitted to our hospital with writing disturbance. His spontaneous speech was fluent, and object naming, word fluency, repetition, verbal comprehension, and reading were fully preserved. However, his writing was slow and required effort. He showed hesitation in spontaneous writing and dictation. His power to copy was better than his power to write spontaneously or to take dictation, but he had some difficulty in copying letters and complex figures. The patient showed abnormal sequences of strokes and completed his strokes by piecing out of several fragments. CT scan and MRI showed a cerebral infarction in the left parietal lobe which included the superior parietal lobule. The amytal (Wada) test, which was performed via the left internal carotid artery, revealed that the left hemisphere was dominant for language. The characteristics of his agraphia much more closely resembled "apractic agraphia", as reported by Alexander et al (1992), than spatial agraphia or pure agraphia. Agraphia in this patient might result partially from the loss or unavailability of the memory of motor patterns necessary for writing letters.