Katsutoshi Kitamura

Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children

The prognosis of 27 patients with moyamoya disease was studied. The ages at onset ranged from 11 months to 4-11/12 years. Follow-up study was performed within 4 years from the onset in 13, 5 to 9 years in 5, and 10 to 15 years in 9. Transient ischemic attacks (TIA) occurred most often during the first four years and decreased thereafter. Intellectual deterioration and neurologic deficits increased with time. Outcome included no sequelae in five (19%), occasional TIA or headache alone in nine (33%), mild intellectual and/or motor impairment in seven (26%), requirement for special school or care by parents or institutions after reaching the teen years in three (11%), continuous 24-hour care in two (7%), and death in one (3%). Poor prognosis was correlated with an early age at onset and hypertension.

Endoscopic biopsy has limited accuracy in diagnosis of ampullary tumors

Endoscopic biopsy specimens and surgically resected specimens in a collective series of 78 Japanese patients with ampullary tumor were retrospectively reviewed to investigate the clinical implications of endoscopic biopsy. Endoscopic biopsy specimens were classified into five groups based on the degree of epithelial atypia: group 1 (no atypia), group 2 (mild atypia), group 3 (moderate atypia or adenoma), group 4 (severe atypia or carcinoma in situ), and group 5 (invasive carcinoma). Final diagnosis of the 78 resected ampullary tumors was adenoma in five cases, carcinoma in 27 cases, and both adenoma and carcinoma in 46 cases. Biopsy accuracy of carcinoma (group 4 or 5) was 70% (51 of 73) overall in 73 carcinoma cases. Biopsy accuracy was 50% (7 of 14) in the intramural protruding type, 64% (21 of 33) in the exposed protruding type, and 88% (23 of 26) in the ulcerating type. The diagnostic accuracy of adenoma (group 3) was 80% (4 of 5) in five cases of ampullary adenoma. In 18 (25%) of the 73 carcinoma cases, biopsy diagnosis was adenoma (group 3), whereas carcinoma was found in the deeper layers of surgically resected specimens. Biopsy diagnosis of adenoma does not rule out the possibility of deeper carcinoma in ampullary tumors.

Glial fibrillary acidic protein in medulloblastoma

SummaryTwenty-four cases of classical medulloblastoma and one case of desmoplastic medulloblastoma were examined for glial fibrillary acidic protein (GFAP) using the immunoperoxidase method to assess astrocytic differentiation. In 16 cases of classical medullablastoma GFAP-positive cells were present in variable numbers.These cells were classified as three different types according to size and shape. The type 1 cell was morphologically identical to the ordinary tumor cell, with a hyperchromatic nucleus and a scanty cytoplasm. The type 2 cell had a fairly rich cytoplasm with short cytoplasmic processes. The type 3 cell was characterized by a relatively large nucleus with sparse chromatin and well-developed cytoplasmic processes, and was considered a reactive astrocyte. The type 1 and some of the type 2 cells seemed to be neoplastic, displaying astrocytic differentiation. The remaining type 2 cells may have been reactive astrocytes.In one case of desmoplastic medulloblastoma, the majority of GFAP-positive cells were arranged in “islands”, and had delicate fibrillated processes. GFAP-positive cells were also observed outside these “islands”, though they were less numerous. Most of them were regarded as type 3 cells, but some were type 2. This may be interpreted as meaning that the glial character of the tumor was expressed more within than outside these “islands”.

Surgical treatment for paediatric patients with moyamoya disease by indirect revascularization procedures (EDAS, EMS, EMAS).

SummarySurgical results of paediatric patients with Moyamoya disease who were treated by indirect revascularization procedures are reported. Encephalo-duro-arterio-synangiosis (EDAS), encephalomyo-arterio-synangiosis (EMAS), and/or encephalo-myo-synangiosis (EMS) were performed on 47 sides of 29 children with Moyamoya disease. The results of those non-anastomotic EC-IC bypass procedures were evaluated clinically, angiographically, and by computed tomography (CT). Postoperative external carotid angiograms showed a good collateral formation through EDAS, EMAS, or EMS in about 70–80 percent of all surgically treated sides. The symptoms such as TIA, RIND, and/or involuntary movements disappeared in the cases with a good collateral formation but not in those with insufficient development of the collateral circulation. The indirect EC-IC bypass surgery seems effective for most of the children with Moyamoya disease who present with ischaemic signs.

Intracranial venous angiomas.

Publications in the scientific literature are controversial in regard to the clinical significance of intracerebral venous angiomas. The present study of 11 patients with venous angiomas underscores the clinical importance of these lesions as potential causes of cerebral hemorrhage and obstructive hydrocephalus. The clinical and radiographic manifestations in 9 of these 11 patients correlated well. In cases of venous angiomas, the venous phase of angiography must be carefully scrutinized, particularly in patients with subarachnoid or intracerebral hemorrhage. Some diagnostic problems related to the angiography of venous angiomas are discussed.

Encephalo-duro-arterio-synangiosis in children with Moyamoya disease

SummaryEncephalo-duro-arterio-synangiosis (EDAS) was done in 16 Japanese children with Moyamoya disease on 22 sides. The results were evaluated clinically, angiographically, and by positron emission computed tomography (PET). Postoperative external carotid angiograms showed a good collateral circulation through EDAS in 72 percent of the treated sides. Two-thirds of the sides examined by PET showed improvement in cerebral blood circulation, particularly at the surgically-treated cortex. Postoperatively the symptoms disappeared in those with good new collateral formation. TIA, RIND, and/or involuntary movement disappaered in 31 percent and partially so in 44 percent 6 months after EDAS. The TIA in the lower limb and/ or involuntary movement persisted in some children. This surgical approach seems applicable particularly for children with the ischaemic type of Moyamoya disease, however, the procedure also has drawbacks. Development of collateral circulation was insufficient in some cases, and the territories of the anterior cerebral artery (ACA) or posterior cerebral artery (PCA) were often not covered, even in those with a good new collateral formation in the middle cerebral arterial (MCA) area.

Microvascular abnormalities in ethylnitrosourea (ENU)-induced rat brain tumors: structural basis for altered blood-brain barrier function.

SummaryThe fine structure, histometric characteristics, and permeability of microvessels were studied by electron microscopy in normal and in ethylnitrosourea (ENU)-induced glioma tissue from rats, using horseradish peroxidase (HRP) as a tracer. The tumor vessels were classified into (1) capillary buds (Type I); (2) round small to large capillaries (Type II); (3) sinusoidal or venule-like microvessels (Type III), and (4) abnormal arteriole-like microvessels (Type IV). All endothelial cells, basement membranes and periendothelial cells in the tumor tissue demonstrated changes in structure. The most striking alterations occurred in the endothelial cells; there were abnormal endothelial tight junctions, altered pinocytotic activity, and thickening. In the tracer study, the reaction product of HRP was present around some sinusoidal or venule-like microvessels (Type III) and extended to the widened extracellular spaces around the microvessels. The endothelial cells of Type III microvessels showed decreased nuclear and mitochondrial fractions, and increased euchromatin content and a rough endoplasmic reticulum fraction. The pinocytotic vesicles with the HRP reaction product in the endothelial cells were not increased in number. Fenestrations and gaps of the endothelial cells were observed. These alterations of the endothelial cells of sinusoidal or venule-like microvessels (Type III) are considered to be the main cause of breakdown of the blood-brain barrier in this tumor.

Prolonged injection angiography for diagnosing intracranial cavernous hemangiomas.

A case of intracranial cavernous hemangioma displaying feeding arteries, tumor stains, and venous pools is presented. Tumors of this type may simulate avascular masses on angiography; however, when surgically excised, they bleed profusely. A correct preoperative diagnosis can help avoid unnecessary incisions. Prolonged injection angiography clearly depicts the tumors and their venous pools, which may be pathognomonic of cavernous hemangiomas.

Angiographic manifestations of intracerebral cavernous hemangioma.

SummaryA case of intraventricular cavernous hemangioma is reported, with a review of the literature, especially that dealing with angiography of intracerebral lesions. Dense venous poolings and a localized area of venous stain were observed in this case.

Proliferative activity of meningiomas as evaluated by bromodeoxyuridine uptake examination.

SummaryProliferative activity of meningiomas was examined in 12 consecutive cases by administering bromodeoxyuridine (BrdU) before surgical removal and by immunohistochemical staining of the removed tumours using anti-BrdU monoclonal antibody (anti-BrdU MAb) to detect BrdU-labelled tumour cells. The 12 cases consisted of 6 with a primary tumour and 6 with a recurrent tumour. All of the tumours contained labelled cells and the labelling index (LI) was obtained in each tumour. The highest average LI was 13.6% and the second highest was 9.0% both in recurrent cases of histologically malignant meningothelial meningioma. The high LIs of both cases were thought to correspond well with the rapid recurrence of the tumour. The average LI of a case of recurrent haemangiopericytic tumour was 2.0%, and that of a case of meningioma associated with von Recklinghausen disease was 1.5%. The other 8 meningiomas showed the average LIs ranging from 0.1 to 0.9%, which were considered to be the LIs of usual benign meningiomas.The results of the BrdU uptake examination was considered to reflect well the clinical behaviour of meningiomas. The usefulness of the BrdU uptake examination in brain tumours, which can be employed in the clinical practice without any serious side-effects, is stressed.