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Holoprosencephaly in neurosurgical practice

Kunihiko Osaka and Satoshi Matsumoto

✓ The clinical, angiographic, and pneumoencephalographic features of seven cases with holoprosencephaly are reported. Three of the alobar type in this series revealed the facial dysmorphia characteristic of the anomaly. In the other four cases (one alobar, one semilobar, and two lobar), such facial anomaly was absent, and the diagnosis was achieved only after contrast studies.

An azygous anterior cerebral artery running over the cerebral surface due to a defective interhemispheric fissure is pathognomonic for alobar and semilobar holoprosencephaly. Dysgenesis of the deep venous system was found in alobar, semilobar, and lobar holoprosencephaly, a finding helpful in diagnosis, especially of the lobar type. Air study and computerized tomography scan revealed incomplete separation of the ventricular system. The basic nature of holoprosencephalization and its differentiation from non-holoprosencephalic malformation are discussed. Six of the seven patients reported had hydrocephalus.

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Statistical Reevaluation of Pinealoma and Related Tumors in Japan

Chisato Araki and Satoshi Matsumoto

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A Simplified Technique for Performing the Ventriculo-Peritoneal Shunt

Technical Note

Anthony J. Raimondi and Satoshi Matsumoto

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Myelomeningocele before birth

Kunihiko Osaka, Takashi Tanimura, Akihiko Hirayama, and Satoshi Matsumoto

✓ The authors report a study of 92 human embryos and four fetuses with myeloschisis. The characteristics of embryonic myeloschisis compared with spina bifida cystica in infants are: 1) the lesion is often more diffuse, involving the whole spinal cord (12 embryos); 2) the cervical cord is more frequently affected (23 of the remaining 80 embryos); 3) holoprosencephaly is frequently associated (18 embryos); 4) meningocele is not found; and 5) hydrocephalus and Arnold-Chiari malformation are not yet developed. Hydrocephalus and Arnold-Chiari malformation are found in myeloschistic fetuses. Almost all embryos with diffuse and cervical myeloschisis or with holoprosencephaly are extruded before birth by spontaneous abortion. Absence of meningocele in the embryonic period implies that its appearance is deferred to the fetal period. The development of hydrocephalus and Arnold-Chiari malformation also seems to be delayed until the fetal period. Our observation implies that myelomeningocele is induced by non-closure of the neural tube, not by rupture once it was closed. “Neural overgrowth” and disturbed “recanalization process” are discussed in relation to the pathogenesis of myelomeningocele.

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Intracranial granuloma as an unusual complication of subdural peritoneal shunt

Case report

Kazuyoshi Korosue, Norihiko Tamaki, Satoshi Matsumoto, and Yoshiyuki Ohi

✓ The authors report a rare case of intracranial granuloma as a complication of subdural-peritoneal shunting for the treatment of subdural effusion. The necessity of the removal of the entire shunt system as soon as the subdural effusion has cleared is emphasized.

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Veins draining the pineal body

An anatomical and neuroradiological study of “pineal veins”

Norihiko Tamaki, Kiyoshi Fujiwara, Satoshi Matsumoto, and Hajime Takeda

✓ The authors present a detailed anatomical and neuroradiological study of the veins draining the pineal body proper, which they designate “pineal veins.” They describe three variations of the pineal veins. Since each has a characteristic angiographic relationship to the pineal body, the results of the study may permit early diagnosis of a pinealoma and distinguish the nature of the tumors of the posterior third ventricle.

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Characterization of periventricular edema in normal-pressure hydrocephalus by measurement of water proton relaxation times

Norihiko Tamaki, Takayuki Shirakuni, Kazumasa Ehara, and Satoshi Matsumoto

✓ The magnetic resonance longitudinal relaxation time (T1) and transverse relaxation time (T2) of the water proton of the periventricular white and cortical gray matter were measured for 17 control patients and 21 patients with suspected normal-pressure hydrocephalus (NPH). Of the latter group, 14 showed good response to shunting (true-NPH group) and seven showed no response (false-NPH group). In the true-NPH group, both the T1 and the T2 of the periventricular white matter were significantly prolonged compared to the control values, and slowly shortened after cerebrospinal fluid (CSF) shunting. The true-NPH group showed significantly longer T1 and T2 of the white matter than did the false-NPH group. The T1 and T2 of the white matter were longer than those of the gray matter in this group, which was the reverse of the relationship observed in the control patients. In the white matter of the false-NPH group, there was a significant prolongation of T1 only; no difference was seen in the T2 compared to control values. There was no change in either T1 or T2 of this region after CSF shunting. The false-NPH group showed no significant difference in either T1 or T2 between the white and the gray matter. There was no difference in either T1 or T2 of the gray matter between the false-NPH and control groups or between preshunt and postshunt measurements in each patient group. It is suggested that a distinction between true- and false-NPH, which cannot be made from the radiographic appearance alone, may be possible from measurement of relaxation times. The mechanism of varied relaxation behavior between two entities may be explained by a difference in properties of the biological water and its environment.

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Antigen related to cell proliferation in malignant gliomas recognized by a human monoclonal antibody

Takashi Kokunai, Norihiko Tamaki, and Satoshi Matsumoto

✓ A human monoclonal antibody (CLN-IgG) was produced from a human-human hybridoma derived from lymphocytes of a patient with cervical carcinoma. The reactivities of this antibody with various human glioma tissues and cultured glioma cells and the characterization of the antigen recognized by CLN-IgG on malignant glioma cells were analyzed and reported. CLN-IgG reacted with various human glioma cells and glioma tissues, especially glioblastoma, but did not react with normal brain tissues or fetal brain tissues. A large amount of antigen recognized by CLN-IgG was expressed on cell membranes of undifferentiated glioma cells and of glioma cells at the G2/M tumor growth phase in cycling cells. Antigen recognized by CLN-IgG was detected in only one of seven samples of cyst fluid, and was not detected in 27 serum samples or 18 samples of cerebrospinal fluid from glioma patients. CLN-IgG exhibited antibody-dependent cell cytotoxicity against U-251MG glioma cells and primary cultured cells of glioblastomas and anaplastic astrocytomas. These data suggest that the antigen recognized by CLN-IgG might be related to cell proliferation in malignant gliomas. Thus, CLN-IgG might be useful for immunotherapy or immunoimaging of malignant gliomas.

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ACNU-resistant mutants of 9L rat glioma cell line

Isolation and preliminary characterization of these subclones

Takashi Kokunai, Norihiko Tamaki, and Satoshi Matsumoto

✓ Three ACNU-resistant subclones were isolated and characterized from a wild-typed 9L rat glioma cell line in culture. At an early stage after cloning, these ACNU-resistant subclones showed a high frequency of chromosomal aberrations compared with nonresistant 9L cells. These ACNU-resistant subclones revealed a cross resistance to BCNU, CCNU, methyl CCNU, nitrogen mustard, cyclophosphamide, and cis-platinum, which are alkylating agents. Further studies are necessary to clarify the mechanisms of ACNU-resistance from the aspect of repair of DNA alkylation damage.

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Magnetic resonance images of brain-stem encephalitis

Case report

Kohkichi Hosoda, Norihiko Tamaki, Michio Masumura, and Satoshi Matsumoto

✓ The clinical and radiological findings in a case of brain-stem encephalitis are described with special emphasis on the serial magnetic resonance imaging. This pathological condition should be differentiated from brain-stem tumors, which may present with similar symptoms.