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Naoki Kageyama

✓ Five cases of ectopic pinealoma in the chiasmal region are reported and the clinical characteristics emphasized. These include diabetes insipidus, hypopituitarism, visual defects, plus normal skull films and intracranial pressure. Microscopic studies of autopsy and surgical specimens show that visual disturbance is caused by destruction of the optic nerve or chiasm by tumor infiltration. Four patients received cobalt-60 radiotherapy and are healthy 6 to 10 years later.

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Isao Yamamoto and Naoki Kageyama

✓ Thirty cadaver brains were examined under × 6 to 16 magnification in order to define the microsurgical anatomy of the pineal region, particularly the relationship of the pineal body, posterior cerebral artery, superior cerebellar artery, vein of Galen, basal vein of Rosenthal, internal cerebral vein, straight sinus, bridging vein, the size of the tentorial notch, and the third and the fourth cranial nerves. The infratentorial and supratentorial approaches to the pineal region are compared from the viewpoint of microsurgical anatomy.

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Hiroshi Yamada, Shigetoshi Nakamura and Naoki Kageyama

✓ The first reported instance of identical twins with moyamoya disease is presented. The involved portions of the main intracranial arteries were similar, but the formation of the moyamoya vessels was somewhat different. The cause of obstructive changes in large intracranial arteries remains obscure, but the frequent familial occurrence suggests that the initial stages of this disease and the formation of the moyamoya vessels occur in the prenatal period.

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Hiroshi Yamada, Shigetoshi Nakamura, Masataka Tajima and Naoki Kageyama

✓ The neurological and neuroradiological manifestations of pediatric achondroplasia are analyzed on the basis of 10 cases. In addition to the classical symptomatology of an enlarging head, with or without increased intracranial pressure, several patients presented symptoms related to a small foramen magnum. Respiratory problems and quadriparesis were also observed in these patients. Six patients who were treated by foramen magnum decompression showed remarkable improvement. Neuroradiological evaluation revealed a significant number of patients with dilated cortical sulci and basal cisterns, and mild dilatation of the ventricles on computerized tomography (CT) and/or ventriculography. Two patients showed signs of obstruction at the fourth ventricle outlets. Some exhibited anomalous dilatation of cerebral veins and dural sinuses, narrowing of the sinuses at the jugular foramen, and enlarged emissary veins. Ventriculoperitoneal or ventriculoatrial shunts were placed in three patients. In the other children with a large head and dilated ventricles, head growth curves paralleled the normal slope, and there was no significant clinical evidence of increased intracranial pressure or enlarging ventricles on follow-up CT scan.

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Tatsuya Kobayashi, Naoki Kageyama and Ken Ohara

✓ The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography.

Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately.

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Symptomatic Rathke's cleft cyst

Morphological study with light and electron microscopy and tissue culture

Jun Yoshida, Tatsuya Kobayashi, Naoki Kageyama and Masaki Kanzaki

✓ A rare case is reported in which a symptomatic Rathke's cleft cyst was studied by light and electron microscopy and tissue culture. The findings are compared with those of a craniopharyngioma studied in the same way. The patient was a 26-year-old woman presenting with headache, chiasmatic syndrome, and hypopituitarism. A cyst containing a mural nodule was partially removed and an Ommaya reservoir placed in the operative site for further treatment. The cyst wall was composed of connective tissue and three kinds of epithelial cells: non-ciliated squamous, ciliated columnar, and mucous-secreting cells. The morphology of these cells in vitro was similar to prickle cells seen in craniopharyngioma and the epidermis. It is concluded that both Rathke's left cyst and craniopharyngioma originate in remnants of Rathke's pouch, but at times may show some histological differences.

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Tatsuya Kobayashi, Naoki Kageyama, Yoshihisa Kida, Jun Yoshida, Naoki Shibuya and Kazuhiko Okamura

✓ Clinical characteristics of six cases of germinoma involving a unilateral basal ganglion and thalamus are summarized. The incidence was estimated as 10% of all intracranial germinomas. The average age at the onset was 10.5 years. The sex incidence showed a male dominance. The clinical course was slowly progressive, and the average duration between onset and diagnosis was 2 years 5 months. Common symptoms and signs were hemiparesis in all cases, fever of unknown origin and eye symptoms in most, mental deterioration and psychiatric signs in three, and convulsions, pubertas praecox, and diabetes insipidus in two. Signs of increased intracranial pressure were found in only two cases in the later state of the disease. Early diagnosis is difficult because of nonspecific symptomatology and slow progression. Carotid angiography and pneumoencephalography showed abnormal findings compatible with basal ganglia and thalamic tumors, but not specific to germinoma. Ipsilateral cortical atrophy and ventricular dilatation might be significant findings. Radioisotope scanning was useful. Computerized tomography scans were the best method of detecting the location and nature of this tumor, and repeat scans showed response to radiation therapy.

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Yoshihisa Kida, Tatsuya Kobayashi, Jun Yoshida, Kyouzou Kato and Naoki Kageyama

✓ Therapeutic results in 17 cases of alpha-fetoprotein (AFP)-secreting tumors of the central nervous system are reported. Twelve of the patients were male and five female. The patients' ages ranged from 5 years to 25 years (mean 13.6 years). The tumors originated in the pineal region in 10 cases, in the suprasellar region in four cases, and in both regions in one case; of the other two tumors, one originated in the basal ganglia and one in the sacrococcygeal region. Extraneural metastases or subarachnoid dissemination occurred in seven cases. Increased AFP titer in the serum or cerebrospinal fluid was verified in 13 cases. The patients were divided into two groups: those treated before (Group A) and those treated after (Group B) the introduction in 1980 of cisplatin as a chemotherapeutic agent. All seven Group A patients died, even after extensive therapy consisting of surgery, radiotherapy, and chemotherapy (ACNU or adriamycin) in some of the cases. The mean survival time following diagnosis for Group A was 23.7 months. Six of the 10 Group B patients died an average of 25.8 months after diagnosis; however, the other four were still alive and well an average of 25.3 months after diagnosis. The tumors responded well to chemotherapy consisting of cisplatin either alone or combined with bleomycin and vinblastine. The initial response rate to treatment in Group B was 100% and five cases had a complete remission, verified by computerized tomography or the serum AFP level. Once the tumor recurred, the response to cisplatin was markedly decreased. It is suggested that an effective therapy must still be sought for recurrent tumors.

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Osamu Sato, Makoto Hara, Takehiko Asai, Ryuichi Tsugane and Naoki Kageyama

✓ The effect of intravenous dexamethasone on cerebrospinal fluid (CSF) production was studied in dogs by a method of caudocephalad perfusion of the spinal subarachnoid space with an inulin-containing buffer. The CSF production rate began to reduce immediately after the injection of 0.15 mg/kg and attained a maximal reduction of 50% in 50 minutes.

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Kenichiro Sugita, Toshiyuki Hirota, Ikuzo Iguchi, Naoki Kageyama and Akikazu Ito

✓ The authors report a unique case of transient amaurosis occurring every time the patient flew in a jet plane and frequently when he drove up a mountain. X-ray examination showed dysplasia of the sphenoidal sinus and optic canals. The pathogenesis and the treatment of the amaurosis is discussed.