[A case of hypertrophic cranial pachymeningitis developed skull lesion].

We report a case of hypertrophic cranial pachymeningitis (HCP) developed skull lesion. A 70-year-old male presented with the symptom of left hemiconvulsion. MRI revealed that the enhanced intraosseous mass infiltrated into the the dura and brain parenchyma under the parasagittal region of the right parietal bone. Histological examination revealed chronic inflammation with lymphoplasmacytic infiltrate and fibrosis of both intraosseous mass and dural invasive lesion. Steroid therapy resulted in improvement of clinical symptoms and enhanced lesion of MRI. Three years later, the patient presented with generalized convulsion and weakness of right upper and lower limbs. MRI revealed dural thickening with gadolinium enhancement in the bilateral parasagittal region and falx. Angiography showed occlusion of the superior sagittal sinus. The cause of relapsing symptoms in this patient may have been related to the occlusion of the superior sagittal sinus, due to HCP. We considered that the incipient intraosseous mass resulted from a response of the marrow by destructive progression of chronic inflammation passed through the fracture crack or the cavity of arachnoid granulation.

[Treatment of spontaneous intracranial hypotension with continuous epidural saline infusion: a case report].

We describe that a 27-year-old woman with a severe postural headache for a month due to spontaneous intracranial hypotension was successfully treated with a continuous epidural saline infusion (CESI). She presented with sudden postural headache and back pain. On admission, CT scans of the head and thoracic spine were normal. Spontaneous intracranial hypotension (SIH) was suspected from her symptoms that her headache was worsen by the erect position and relieved by the supine position. She was treated with intravenous infusion (1000 ml/day), but her headache did not resolve. Three weeks later, MR images showed descent of the brain stem and cerebellar tonsil, effacement of the sulci and cisterns, subdural fluid collections and diffuse meningeal enhancement. A lumbar puncture was performed and yielded an opening CSF pressure of 75 mmH2O in the horizontal position. Radionucleotide cisternography showed early accumulation of nucleotide in the bladder, slow ascent along the spinal axis, less than expected activity over the cerebral convexities and early disappearance of radioactivity. As for the treatment, an epidural catheter was placed at the L 1-2 level and CESI of 15 ml/hour was started. Immediately the postural headache resolved and the next day she could walk. CESI was continued for 5 days. MR images after CESI showed normalization of the sulci and cisterns including spinal subarachnoid space, reelevation of the brain stem and cerebellar tonsil and improvement of meningeal enhancement. She remained headache free during 7 months of follow-up period. We propose this method as a safe and effective treatment for SIH.

[Subependymal giant cell astrocytoma associated with tuberous sclerosis exhibiting rapid regrowth after 17 years: a case report].

A 17-year-follow-up case of subependymal giant cell astrocytoma (SGCA) is reported. In 1979, when aged 28 years, the patient first presented obstructive hydrocephalus caused by a tumor in the right lateral ventricle close to the foramen of Monro. It was partially removed by a transcallosal approach. Pathological examinations showed gemistocytic astrocytoma or SGCA associated with tuberous sclerosis. A ventriculo-peritoneal shunt was carried out and 36Gy of radiation therapy was administered. Eight months later, the patient suffered from an intraventricular hemorrhage originating from SGCA, but he responded to conservative therapy. He was followed-up by CT scans over 17 years. In 1996, because of rapid regrowth of the tumor, total removal was performed by a transcortical approach via the right frontal horn. The pathological diagnosis was SGCA. The greater part of the recurrent tumor was composed of blood vessels. The tumor cells were grouped into two morphological types, large cells and spindle cells. We compared the tumor in 1996 with that in 1979, each revealing immunohistochemical stainability for glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE) and S-100 protein (S-100). The large cells in 1979 were negative for GFAP, NSE and S-100, but were positive for NSE and S-100 in 1996. The spindle cells in 1979 were positive for GFAP, NSE and S-100, but were negative for GFAP in 1996. The pathological origin of SGCA remains a subject of controversy. These results suggest that the origin of SGCA could be variably differentiated cells like the germinal matrix cells.

[Isolated angiitis of the CNS associated with Hashimoto's disease].

In 1987, a 69-year-old female suffering from epilepsy and right hemiparesis was admitted to the hospital and given conservative therapy. During the next three weeks she became a stuporous state. Steroids were then prescribed, and she made a complete recovery. Following hormonal and immunological investigations, a diagnosis of Hashimoto's disease with hypothyroidism was made. Following her discharge, thyroxin and anticonvulsant treatments continued. On May 30, 1989, at the age of seventy-one, the patient was found lying unconscious at home, and taken to our hospital. She had signs of a confused state, and seizures of her right arm on admission. Cranial computed tomography (CT) revealed a low density area in the left parieto-occipital lobe, which was heterogeneously enhanced by contrast medium. Magnetic resonance imaging (MRI) showed a lesion of low signal intensity in T1-weighted images and high signal intensity in T2-weighted images, which was homogeneously enhanced by Gd DTPA. Cerebral angiography disclosed no abnormality. Two weeks later, follow-up CT showed that the lesion had become larger and markedly enhanced. A brain tumor, probably malignant lymphoma, was suspected. On June 14, stereotaxic biopsy of the enhanced lesion was performed. Histopathological examination disclosed dense infiltration of the entire walls of many small parenchymal vessels, both arterioles and venules, by lymphocytes. Treatment for angiitis (betamethasone 16 mg daily) was started on June 20, then gradually tapered, and the lesion diminished on CT. Our case has some features of both isolated angiitis of the central nervous system and encephalopathy in compensated Hashimoto's disease. It is suggested that some common base of the autoimmune diseases exists in this case.

[Diffuse cerebrospinal gliomatosis. Case report].

A 65-year-old male was admitted with memory and gait disturbance. A computed tomography (CT) scan showed bilateral, diffuse, low-density areas with two round, slightly enhanced masses. T1-weighted magnetic resonance image revealed a low-intensity area in the left paraventricular region, which converted to increased signal intensity, extending to the right paraventricular region through the splenium, on T2-weighted images. The tumor was diagnosed as glioblastoma multiforme after needle biopsy and treated by irradiation and chemotherapy. Seven months after admission, a CT scan revealed subependymal infiltration of the tumor with spotty calcification. He died of respiratory complications 11 months after the onset of symptoms. The autopsy showed brain swelling with flattened gyri. Horizontal sections of the brain showed diffuse enlargement of the white matter and basal ganglia with scattered hemorrhage and necrosis. Microscopically, the lesion was far more extensive and diffuse than was suspected from gross examination. Wide glial tumor cell infiltration was observed in the cerebral hemispheres, basal ganglia, brainstem, cerebellum, and even the cervical spinal cord with minimum destruction of the pre-existing architecture. Calcification was found around the thrombosed vessels and necrotic lesions. The clinical diagnosis and histological features of gliomatosis cerebri are discussed with reference to reported cases.

Identification of CSF fistulas by radionuclide counting.

A radionuclide counting method, performed with the patient prone and the neck flexed, was used successfully to diagnose CSF rhinorrhea in two patients. A normal radionuclide ratio (radionuclide counts in pledget/radionuclide counts in 1-ml blood sample) was obtained in 11 normal control subjects. Significance was determined to be a ratio greater than 0.37. Use of radionuclide counting method of determining CSF rhinorrhea is recommended when other methods have failed to locate a site of leakage or when posttraumatic meningitis suggests subclinical CSF rhinorrhea.

[Primitive neuroectodermal tumor with peritoneal metastasis through a ventriculoperitoneal shunt. Case report].

A 13-month-old boy admitted with lethargy and hydrocephalus was found to have a right thalamic mass. Ventricular drainage was instituted, and the tumor mass was reduced by partial resection and local irradiation. A ventriculoperitoneal shunt was then placed. However, the tumor recurred 16 months later, with extensive ventricular seeding and peritoneal metastasis through the shunt tube. The child died 22 months after onset. Histological study of surgical specimens of the primary tumor and autopsy specimens of the brain and peritoneal metastatic tumors revealed poorly differentiated, small, round cells with numerous mitotic figures. In addition, autopsy specimens of the brain tumor contained areas of ependymal, oligodendroblastic, and spongioblastic differentiation. On immunohistochemical study, the tumor cells of each specimen were positive for anti-neuron specific enolase and anti-neurofilament antibodies, but negative for anti-glial fibrillary acidic protein antibodies. Electron microscopy revealed some zonulae adherens. These findings strongly suggest that the tumor originated from primitive multipotential cells capable of differentiating into ependymal, glial, and neuronal lines.

[Enlarging skull fracture treated by early surgery. Case report].

The authors report a case in which an enlarging skull fracture was surgically repaired on the fourth day after the initial injury. A 5-month-old boy fell from his father's arms and was hospitalized with a large, irregular protrusion in the left parieto-occipital region and right hemiparesis. Plain skull films showed a diastatic linear fracture with a maximum width of 8 mm, which expanded to 11 mm by the fourth day. Computed tomography (CT) scans revealed a cerebral contusion just beneath the fracture as well as a left subdural effusion. At operation, the contused brain tissue was found to have herniated over the bone defect. Craniotomy revealed a large dural defect, which was repaired with lyophilized dura. The patient was discharged with no neurological deficit. On the basis of a review of 58 recently reported cases of skull fracture, the authors conclude that the following conditions warrant consideration of early surgery: 1) a diastatic skull fracture with a width of at least 4 mm; 2) CT demonstration of a cerebral contusion beneath the fracture; 3) overlying scalp swelling; and 4) a neurological abnormality contralateral to the fracture. Performing surgery before the fracture gap becomes scalloped will result in more rapid neurological recovery and reduce the likelihood of enlargement of the fracture.

[Interaction between neurogenic and metabolic factors upon deterioration in cerebrovascular tonus--experimental study on the etiology of cerebral vasoparesis].

It is widely accepted that a tremendous increase in cerebral blood volume (CBV) due to progressive cerebral vasoparesis is an essential to the development of acute brain swelling. This study was designed to determine whether neurogenic and/or metabolic factors are predominant and how these interact with each other in producing cerebral vasoparesis. Fifty-one awake cats immobilized with pancuronium bromide were divided into 4 groups: group I, control; group II, normocapnic hypoxia (PaO2 = 50 mmHg); group III, normoxic hypercapnia (Pa-CO2 = 70 mmHg), and group IV, increased intracranial pressure (ICP = 40 mmHg) by brain compression. Systemic arterial pressure (BP), CBV (photoelectric method), and ICP (epidural pressure) were continuously recorded. The dorsomedial hypothalamic nucleus (DM) and the reticular formation of the midbrain (MB-RF) were bilaterally coagulated by a stereotaxic technique (3mA, 1 min). Therefore alterations in cerebrovascular tonus created by destruction of the cerebral vasomotor centers were examined in the animals with metabolically induced cerebral vasodilatation to various degree's. In group I, vasomotor center destruction resulted in an immediate and transient decrease in BP (DM; -14.1 +/- 6.7 mmHg, MB-RF; -10.2 +/- 4.8 mmHg) and simultaneous increase in CBV and ICP (DM; 7.6 +/- 7.0 mmHg, MB-RF; 6.0 +/- 5.6 mmHg) for 3 to 4 minutes. Increase in ICP by destruction of vasomotor centers reduced significantly in group II (DM; 2.3 +/- 2.6 mmHg, MB-RF; 1.6 +/- 1.2 mmHg) and reduced slightly in group IV (DM; 7.5 +/- 4.0 mmHg, MB-RF; 4.8 +/- 3.2 mmHg). In these 3 groups, autoregulation of cerebral blood flow and CO2 vasoreactivity were not changed by destruction of vasomotor centers.(ABSTRACT TRUNCATED AT 250 WORDS)

Craniopharyngioma in the third ventricle: necropsy findings and histogenesis.

A case of craniopharyngioma confined within the third ventricle with necropsy is reported. A stalk-like structure in this tumour was present in the wall of the third ventricle at its base. It is suggested that this tumour might have arisen from the remnants of Rathke's pouch persisting in the tuber cinereum.

[A case of pituitary hyperplasia associated with primary hypothyroidism].

A thirteen-year-old girl was admitted complaining of short stature and anemia. The low titers of her serum T3 and T4 and the abnormally high TSH level represented primary hypothyroidism. Although she had normal sellar size, CT demonstrated an intra- and suprasellar round mass with homogeneous enhancement. With thyroid replacement therapy the enhanced mass diminished on CT within 5 months, and her symptoms regressed. Twelve cases with radiological diminution of pituitary mass or visual field improvement after thyroid replacement therapy are reviewed. They were considered to be pituitary hyperplasia, rather than pituitary adenoma, caused by long-standing untreated hypothyroidism. In four of them, the pituitary mass on CT was diminished after the therapy. Characteristic CT findings of pituitary hyperplasia, including our case, was a round isodensity mass with homogeneous enhancement in the midline of the pituitary region. In the experimental studies, pituitary hyperplasia is based on the feedback mechanism of hypothalamic-pituitary-thyroid axis, and ultimately autonomous pituitary adenoma may occur. Pituitary mass with hypothyroidism, visual field defect, amenorrhea or galactorrhea tend to be mistaken for prolactinoma or non-functioning adenoma with pituitary hypothyroidism. Thorough endocrinological examination must be carried out. The first choice of treatment for this type of pituitary mass should be thyroid replacement therapy. If there is no improvement of visual field, no regression of pituitary mass on CT, or continuing high TSH levels, then pituitary surgery must be considered.

Histopathologic investigation of a case of meningioangiomatosis not associated with von Recklinghausen's disease.

A case of meningioangiomatosis not associated with von Recklinghausen's disease is reported. Microscopically, irregularly branched blood vessels extending into the gray matter from the meningeal surface are surrounded by a concentric arrangement of proliferating spindle-formed cells. Ultrastructurally these proliferating cells are composed of elongated heterochromatin-rich nuclei and slender cytoplasm-containing microfilaments, occasionally associated with desmosomal junctions and basal laminalike structures. Judging from these findings, together with a negative immune reaction for S-100 protein, the histogenesis of these proliferating cells is most probably meningothelial in origin.

Agenesis of the left internal carotid artery, common carotid artery, and main trunk of the external carotid artery associated with multiple cerebral aneurysms.

A case of agenesis of the left internal carotid artery, common carotid artery, and the main trunk of the external carotid artery with multiple cerebral aneurysms is presented. This case was diagnosed by angiography and computed tomography scanning and confirmed by operation. Correlation between the anomaly of the circle of Willis based on the absence of the internal carotid artery and the development of cerebral aneurysm is discussed on the basis of the reported cases.

[Application of the RI-counting method in CSF rhinorrhea].

Some diagnostic procedures for CSF rhinorrhea have been reported, but they can not always identify and localize CSF leakage. This method, using intranasal cotton pledgets after the intrathecal injection of radioisotope (111In-DTPA) is thought to be reliable, informative and innocuous for localizing the sites of CSF leakage. This technique was named RI-counting method, and applied in two cases that were difficult to diagnose especially with positional-loading. The patients were first in the supine position, then in the sitting position, after the intrathecal injection of radioisotope and the intranasal insertion of cotton pledgets. After exchanging these pledgets, the patients were set in the prone position. After measuring the radioactivity of these pledgets and of 1 ml of blood, the ratios of the radioactivity of the pledgets to that of the blood were calculated. In case 1 the ratios of the left sphenoethmoid recess and the left middle meatus were significantly higher when the patient took the prone position, but in the supine or sitting position the ratios were in the normal range. In case 2 the ratio of right olfactory cleft was significantly higher in the sitting position or neck flexion. Both cases were operated and demonstrated that the location of the CSF leakage was presumable by using RI-counting method, regarding the anatomical relationship between the intranasal sites of pledgets and the opening of the paranasal sinuses. Positional-loading seemed to be essential. We obtained 72 pledgets of patients without CSF rhinorrhea, and the ratios of radioactivity of these pledgets had a mean value (M) of 0.156 and a standard deviation (SD) of 0.107.(ABSTRACT TRUNCATED AT 250 WORDS)

[A case of convexity cavernous hemangioma associated with meningioma].

A case of convexity cavernous hemangioma associated with sellar meningioma with parasellar extension is presented. A 61-year-old female who had complained of left blepharoptosis and diplopia was admitted to our hospital. On admission she showed left oculomotor nerve palsy. Plain CT revealed an isodense mass in the sellar and parasellar region. Computed angiotomography demonstrated that this mass was enhanced heterogeneously and filled the sellar turcica and extended superiorly. And homogeneously enhanced mass in the convexity without mass effect was observed. Angiogram revealed no tumor stain in any phase. With these findings, this case was diagnosed as the multiple meningiomas preoperatively. Left frontotemporal craniotomy was carried out, and subtotal removal of sellar and parasellar tumor and total removal of convexity tumor were performed. Pathologically the sellar and parasellar tumor was diagnosed as meningioma, convexity tumor as cavernous hemangioma derived from dura mater. Convexity cavernous hemangioma derived from dura mater is very rare, only one case was reported previously. Only 2 cases of cavernous hemangioma associated with meningioma have been reported in the literature. Correlation between cavernous hemangioma and meningioma is discussed. It is possible to owe this complication to the developmental abnormality concerning with inner factor like genetic factor and so on, because both tumors are mesodermal origin. However it is proper to regard that this complication occurred by chance.