Turcot's syndrome presenting with medulloblastoma and familiar adenomatous polyposis: a case report and review of the literature.
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Biomedical subjects
Publications and source records attributed to T Akai.
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BACKGROUND: Recurrence of chronic subdural hematoma after surgery has not been eliminated. We sought to determine whether irrigation influences recurrence rate. METHODS: Patients who had undergone surgery for chronic subdural hematoma were reviewed retrospectively. RESULTS: Between 1986 and 1993, 186 cases of chronic subdural hematoma were treated surgically at Mito National Hospital. Recurrence was limited to six cases (3.2%). A closed drainage system without irrigation has been used since 1988 in 119 patients. Before 1988, 67 cases were treated with single burr hole irrigation and drainage. The rate of recurrence with the closed drainage system was 3.4% (four cases), compared with 3.0% (two cases) for irrigation and drainage. CONCLUSION: Compared with irrigation and drainage, the closed drainage system without irrigation was safe and effective, and recurrence of chronic subdural hematoma is not influenced by irrigation.
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A 36-year-old female with a history of recurrent pregnancy loss experienced sudden onset of disturbance in consciousness, with right hemiparesis and total aphasia. Computed tomography revealed a massive hemorrhage in the left frontal lobe, and angiography showed occlusion of the anterior two-thirds of the superior sagittal sinus. Laboratory investigations detected the presence of lupus anticoagulant, elevation of the anticardiolipin beta 2-glycoprotein I complex antibody level, and a decreased protein S activity level. There were no underlying conditions, such as connective tissue disorders, malignancies, infectious diseases, and drug-induced disorders, so the diagnosis was primary antiphospholipid syndrome. Primary antiphospholipid syndrome should be considered in the evaluation of patients with "idiopathic" or "primary" sinus and cerebral venous thrombosis.
A 41-year-old female underwent complete resection of a left petroclival meningioma via an anterior transpetrosal approach, during which the greater superficial petrosal nerve was divided. On the 14th day after the operation, she first noticed leakage of clear fluid from her right nostril whenever the ambient room temperature rose. This pseudo-cerebrospinal fluid rhinorrhea may have developed because of parasympathetic hypersensitivity due to division of the greater superficial petrosal nerve.
A 63-year-old male presented with an unusual case of nontraumatic intradiploic arachnoid cyst in the frontotemporal convexity area. Skull radiography showed a circular osteolytic lesion of the right parietal bone. Computed tomography revealed a defect of the inner table, widened diploic space, and thinned outer table. Magnetic resonance imaging showed a cyst containing cerebrospinal fluid. Bone scintigraphy showed no abnormal uptakes. Intraoperative observation confirmed the neuroimaging findings. Histological examination found no abnormal findings in specimens of skull bone or arachnoid membrane. Intradiploic arachnoid cyst is characterized by parasagittal, multiple, well-demarcated osteolytic lesions on radiographs in the elderly. However, the clinical features of this disease remain unclear and diagnosis without an exploratory surgical procedure may not be possible.
A 4-year-old boy presented with a rare case of an epidural tumor causing compression of the thoracic spinal cord manifesting as rapid worsening of gait disturbance. The tumor was grossly totally resected, and radiotherapy and chemotherapy were subsequently administered. The histological diagnosis was primitive neuroectodermal tumor. He has been free of recurrence for more than 6 years. Early tumor resection followed by chemotherapy and irradiation is recommended for patients with primitive neuroectodermal tumor and unstable symptoms.
A 14-year-old boy presented with an extraneural metastasis of germinoma with syncytiotrophoblastic giant cells (STGC). The tumor was located in the pineal area, suprasellar cistern, and frontal base, and associated with the slight elevation of the beta-human chorionic gonadotropin (HCG) serum level. Following radiation therapy, the tumor completely disappeared, and the beta-HCG level decreased to normal. Two years after the initial treatment, metastasis of the tumor to the left femur was observed without recurrence of the primary lesion. The biopsy specimen revealed germinoma, the diagnosis was germinoma with STGC because of the associated elevation of HCG level. The metastatic tumor was treated by chemotherapy with cisplatin and etoposide, and the tumor completely disappeared. With adjuvant chemotherapy, he has been recurrence-free for 3 years. Extraneural metastasis occurs more frequently from germinoma with STGC than germinoma. Aggressive chemotherapy combined with radiation therapy in the initial treatment might have to be tried to avoid metastasis from germinoma with STGC.
We investigated the alteration of heparan sulfate proteoglycans induced by thrombin in cultured vascular endothelial cells. Heparan sulfate proteoglycans, which were metabolically labeled with [3H] glucosamine and [35S] sulfate, were isolated by DEAE-Sephacel ion-exchange chromatography and characterized by molecular sieve gel filtration. Core proteins were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis of [35S] amino acids-labeled heparan sulfate proteoglycans after digestion with hepartinase. It was revealed that the high molecular weight subclass of heparan sulfate proteoglycans in the cell layer was markedly decreased by thrombin without changes of the hydrodynamic size of the molecules and the molecular weight of heparan sulfate chains. In addition, thrombin decreased the amount of large heparan sulfate proteoglycan core protein with a molecular weight of approximately 400 kDa, probably perlecan core, in the cell layer and the conditioned medium. The present data suggest that thrombin-induced decrease in the amount of heparan sulfate in vascular endothelial cell layer includes a reduction of the number of large heparan sulfate proteoglycan perlecan molecules through a suppression of the core protein synthesis.
A 36-year-old female was admitted with leptomeningeal melanoma associated with straight sinus thrombosis manifesting as headache and vomiting. Computed tomography and magnetic resonance imaging showed the subarachnoid space was diffusely enhanced. Her consciousness rapidly deteriorated to a coma. Angiography demonstrated straight sinus thrombosis. Thrombolysis by superselective catheterization and infusion of urokinase was successfully performed. She recovered consciousness, but developed paraparesis 2 weeks later. Malignant melanoma with meningeal dissemination was diagnosed by an open biopsy of the lumbar lesion. Angiitis induced by the infiltration of tumor cells and activation of the blood coagulation cascade was probably the causative mechanism of the sinus thrombosis.
The outcome of cerebrovascular disease in the elderly, defined as persons 70 years of age and over, and in the non-elderly, those under 70 years, was compared. Fifty-one consecutive elderly and 156 non-elderly patients were studied. The patients in each group were classified according to type of cerebrovascular event: intracerebral hemorrhage, cerebral infarction, or subarachnoid hemorrhage. Patients were evaluated by using the Glasgow Outcome Scale both at the time discharge and 6 months later. The elderly patients were more severely disabled at the time of discharge than the non-elderly patients. The number of patients evaluated as "good recovery" or "moderate disability" had increased at the 6-month follow-up examination in the non-elderly patients, whereas the condition of many of the elderly patients had declined. The death rate of the elderly at the time of discharge and 6 months later was 17.6% and 52.0%, respectively, as opposed to 13.5% and 18.8% in the non-elderly group. The outcome of cerebrovascular disease was much worse in the elderly patients than in the non-elderly patients. Patient age and status at discharge were important predictors of the clinical course.
A case of skull base Aspergillus granuloma originating from the sphenoid sinus was reported. This 64-year-old man complained of unilateral visual loss and serious facial pain, and was admitted to our department. He had been treated by transnasal sphenoidectomy because of sphenoid sinus aspergillosis. MRI showed a mass lesion located in the sphenoid sinus, cavernous sinus, ethmoid sinus and intraorbit. Angiography showed severe stenosis of the left internal carotid artery at the cavernous portion. As the symptoms gradually aggravated, we felt compelled to perform intracranial surgical treatment using the extradural approach to the skull base lesions. As the fibrous lesion was very hard and strongly adherent to the surrounding tissues, only partial removal of the lesion and resection of the trigeninal nerve were carried out. After surgery, the patient was released from severe facial pain and had an uneventful course. But, two months later, he died due to Aspergillus meningoencephalitis. Aspergillosis originating from the sphenoid sinus is rare, but it is known that some lesions show abrupt invasive extension to the skull base and the prognosis is bad. Early diagnosis and suitable treatment for the skull base or intracranial lesions is required.
A 72-year-old female with intradiploic haemangioma associated with epidural haematoma is reported. MRI finding of the lesion is described. The cause of the epidural haematoma is speculated due to fragile vessels attached directly to dura mater through the pores of the inner table of the diploë.
A 10-year-old boy with progressive left visual disturbance associated with craniobasal fibrous dysplasia underwent left frontotemporal craniotomy. Dysplastic lesions of the sphenoid ridge, orbital roof, anterior clinoid, and ethmoid sinus were removed through an extradural pterional approach and the optic nerve was completely decompressed. His vision was markedly improved postoperatively. Consecutive follow-up studies for 3 years have shown no deterioration of his visual acuity. Early optic nerve decompression is highly recommended to preserve visual function in patients with craniofacial fibrous dysplasia causing visual disturbance.
To clarify the roles of dopamine D1 and D2 receptors in behavioral symptoms of Parkinson's disease, antiparkinsonian effects of various dopamine agonists in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-lesioned parkinsonian monkeys were investigated with regard to induction of hyperactivity such as excitability, irritability and aggressiveness. The non-selective dopamine agonist apomorphine ameliorated the parkinsonism, but induced marked hyperactivity dose-dependently. Pretreatment with either the dopamine D1 antagonist SCH 23390 or the dopamine D2 antagonist sulpiride markedly suppressed the apomorphine-induced hyperactivity with slight attenuation of the antiparkinsonian effects. Both the dopamine D2-receptor agonist quinpirole and the dopamine D1-receptor agonist SKF 82958 ameliorated the parkinsonism in a dose-dependent manner with a slight induction of hyperactivity. Combination treatment of a threshold dose of quinpirole with that of SKF 82958 augmented the antiparkinsonian effects without a marked induction of hyperactivity. However, the combination treatment at higher doses induced marked hyperactivity accompanied by augmented antiparkinsonian effects. These results suggest that stimulation of either central dopamine D1 or D2 receptors is requisite for the antiparkinsonian effects and concurrent strong stimulation of both central dopamine D1 and D2 receptors causes marked hyperactivity which may be predictive of dopaminergic psychiatric side effects.
A 69-year-old male presented with sudden onset of headache. Computed tomography of the head demonstrated diffuse subarachnoid hemorrhage. Angiography showed a saccular basilar bifurcation aneurysm. Endovascular coil occlusion of the aneurysm on the same day enabled complete obliteration of the aneurysm. He did not develop vasospasm and returned home 4 months later. Early endovascular occlusion of the aneurysmal sac is an alternative method for the management of ruptured basilar bifurcation aneurysms.
A 31-year-old male developed intramedullary tumors in the medulla oblongata and the upper cervical spinal cord. He was first admitted with tetraparesis. Magnetic resonance (MR) imaging revealed a low intensity mass lesion in the medulla oblongata. The tumor was removed and diagnosed as a pilocytic astrocytoma. Nine years later, he was readmitted with motor weakness and dysesthesia in the right arm. MR imaging revealed a mass lesion in the cervical cord. This tumor was removed and diagnosed histologically as ependymoma. We suggest that the displacement of primitive spongioblasts with subsequent differentiation resulted in an astrocytoma and an ependymoma in adjacent areas.
A sixty-two-year-old woman was diagnosed as having the mitral valve insufficiency seven months prior to admission. The patient was admitted to the hospital with complaints of right hemiparesis and aphasia. CT scan revealed an intracerebral hematoma in the left front-parietal region. Cerebral angiography disclosed an aneurysm at the distal portion of left middle cerebral artery. An increase in the amount of C reactive protein and leukocytosis indicated the presence of inflammatory lesions. Antibiotics were administered because a mycotic aneurysm was suspected. White blood cell count and C reactive protein returned to normal after treatment with antibiotics for one months. The aneurysm had disappeared on the second angiography performed after treatment. Strategy for mycotic aneurysm is still controversial. 49 reported cases in the literature since 1975 were reviewed and the efficiency of antibiotic therapy was discussed.