Hitoshi Tsugu

Brain abscess caused by streptococcus intermedius: two case reports

BACKGROUND Although Streptococcus intermedius has been recognized as an important pathogen for abscess formation outside the central nervous System, the Streptococcus milleri group, of which it is a member, has not been recognized as a specific pathogen for brain abscess, often thought to be caused by Streptococcus viridans, which includes other streptococcal species. CASE DESCRIPTION Two cases of brain abscess in the left frontal lobe caused by S. intermedius, which responded well to antimicrobial treatment combined with needle aspiration, are presented. In the first patient, the predisposing disease was paranasal sinusitis of the frontal and ethmoid sinuses. In the second patient, the source of the pathogen was not detected despite extensive examination. The patients underwent aspiration of pus under ultrasound guidance in the first patient, and via a computed tomography-guided stereotactic procedure in the second patient. They subsequently received appropriate antimicrobial therapy against S. intermedius isolated from the pus culture. Both patients were discharged without any neurological deficits. CONCLUSION It is important to recognize S. intermedius as a pathogen of brain abscess, and to be aware of its predisposing factors, i.e., mucosal disturbance and liver abscess.

Expression and cellular localization of low-density lipoprotein receptor-related protein/α2-macroglobulin receptor in human glioblastoma in vivoreceptor in human glioblastoma in vivo

Low-density lipoprotein receptor-related protein/α2-macroglobulin receptor (LRP) has been proposed to mediate the cellular uptake and clearance of inactivated protease-inhibitor complexes in regulating proteinase activity at the cell surface, which is necessary for cellular migration and invasive processes. In this study, we investigated the presence of both LRP and urokinase-type plasminogen activator receptor (uPAR) in glioblastoma by reverse transcriptase-polymerase chain reaction (RT-PCR), and the cellular localization of LRP in glioblastoma tissues by immunohistochemical analysis. LRP mRNA was frequently expressed in glioblastomas and anaplastic astrocytomas compared with low-grade astrocytomas by RT-PCR analysis, and was well correlated with uPAR expression. The immunohistochemistry of LRP on sequential frozen sections showed that neoplastic glial cells and endothelial cells of glioblastomas exhibited intense LRP immunoreactivity, whereas LRP was almost undetectable in low-grade astrocytomas or in normal glial cells and endothelial cells of normal brain tissue. Glioblastomas from 11 patients in which the expression of LRP mRNA was observed by PCR displayed strong to moderate LRP immunoreactivity, with predominantly diffuse cytoplasmic and cell-surface localization. In normal brain tissues, LRP immunoreactivity was identified in the pyramidal neurons of the cerebral cortex. These results indicate that LRP is present both in the cellular cytoplasm and on the cell surface of glioblastomas with an increased expression of uPAR. Altered LRP expression might contribute to the stimulation of cell-surface proteolytic activity that in turn facilitates the invasiveness of glioblastoma in vivo.

Maffucci's syndrome associated with intracranial chordoma: case report.

This report discusses Maffuccis syndrome associated with intracranial chordoma. Of the 170 reported cases of this syndrome, only 27 have been associated with intracranial lesions and none was involved with intracranial chordoma. Chordomas are occasionally difficult to differentiate from chondroma and chondrosarcoma. An immunohistochemical analysis was able to prove, however, that the chordoma of this case significantly differed from chondroma and chondrosarcoma.

Survival and late effects on development of patients with infantile brain tumor

Background:  Most infants with brain tumor may have a poor prognosis. The aim of the present study was to retrospectively analyze the survival and outcome with regard to mental and physical development in 11 subjects with brain tumor; these tumors were diagnosed when the patients were under 1 year of age.

Hypothalamic Hamartoma Associated with Multiple Congenital Abnormalities

We report 2 patients with hypothalamic hamartoma associated with multiple congenital abnormalities and analyze 42 (including our own) reported cases, including our 2 cases, of hypothalamic hamartoma or hypothalamic hamartoblastoma with multiple congenital abnormalities, to understand the timing of their occurrence and clarify the prognosis. To this end, we classified them into lethal and nonlethal cases. We found poly- and syndactyly, cleft or high-arched palate and nose abnormalities to be important manifestations of this syndrome. Major organ abnormalities and CNS and endocrine abnormalities occurred frequently among the lethal cases, very likely indicative of a disturbance of embryogenesis between gestational days 34–37 and thus implicated in a negative prognosis.

A Case Report of Caudal Regression Syndrome Associated with an Intraspinal Arachnoid Cyst

We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.

Intracranial ectopic pancreatic tissue.

A young Japanese female was in 2007 reported to suffer from a congenital brain malformation with a non-functioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue.1 Ectopic pancreas is normally confined to other endodermally derived organs and not previously reported to be found in the brain. Therefore, we sought to better understand the true pancreatic nature of the tissue and to further understand the mechanism by which ectopic pancreas could appear in the brain. A detailed immunohistochemical analysis for pancreatic hormones, transcription factors, ductal/exocrine markers and stem cell markers on sections from the resected tumor tissue was performed. All five endocrine cell types are observed but pancreatic polypeptide cells are quite rare and ghrelin and glucagon cells are more numerous than in normal human pancreas. Insulin immunoreactive cells stain for c-peptide. The β-cell specific transcription factor, Nkx6.1, is expressed only in the insulin immunoreactive cells while Neither Ptf1a or PDX-1 immunoreactive cells can not be observed. Duct like structures stain strongly for pan-cytokeratin, E-cahderin. The exocrine like tissue stains strongly for pancreatic amylase, lipase and chymotrypsin. Ngn-3 cells were very rare and not in the pancreatic area. Examining for endodermal markers we observed Sox17 had a weak staining in some areas of the pancreatic tissue but was much less widely expressed than FoxA2. The tumor tissue did not stain for the stem cell markers, Oct-4 and Sox2. It is speculated that the ectopic pancreas domain may arise from misexpression of homeodomain transcription factors related to Pdx1 within a domain of Ptf1a expression.

A case of a heavily pigmented orbital melanocytoma

We present an extremely rare case of an orbital melanocytoma that occurred in a 51-year-old man. The patient suffered from diplopia and mild exophthalmos of the right eye for 2 months. Brain magnetic resonance imaging showed a well-demarcated round mass 3.5 cm in diameter in the right orbit. We performed total resection of this tumor. Histological findings revealed a proliferation of large polygonal cells with fine pigment granules in the cytoplasm and prominent nucleoli. Although these tumor cells revealed immunohistochemical reactivity in HMB-1, there was no S-100 or Melan A antibody reactivity. Also, there were no malignant findings of nuclear polymorphism, mitoses, or necrosis. The brown pigments were confirmed to be melanin by bleaching and the Fontana-Masson silver stain method. The MIB-1 labeling index was less than 1%. This tumor also consisted of 50% melanophages, which revealed immunohistochemical reactivity in CD68, CD163, and in (1-AT antibodies. These histological findings led us to diagnose an orbital melanocytoma with partial tumor regression.

Neurohypophyseal Granulomatous Germinoma Invading the Right Cavernous Sinus: Case Report and Review of the Literature

We encountered a rare case of neurohypophyseal germinoma with a prominent granulomatous reaction, which invaded the right cavernous sinus. The neuroimaging and histopathology features in this case were unique, distinguishing it from other types of suprasellar lesions. A 13-year-old boy presented with loss of appetite and polyuria; both symptoms were present for 1 year, and headache, general fatigue and blurred vision present for the prior 2 months. On admission, neurological examination indicated bitemporal hemianopsia and optic atrophy. Endocrinological exam showed panhypopituitarism. Tumor markers such as α-fetoprotein, human growth hormone, carcinoembryonic antigen, and placental alkaline phosphatase were negative. Brain CT revealed a suprasellar tumor with calcification. MR T1-weighted and T2-weighted images showed the tumor to be isointense to normal brain parenchyma and to be enhanced densely. The tumor also involved the right cavernous sinus, so that a biopsy was performed by the transsphenoidal approach. On pathologic examination of the specimen, typical large tumor cells with lymphocytic cell infiltration and prominent granulomatous reaction were observed. Neurohypophyseal granulomatous germinoma was diagnosed. Radiotherapy was performed with a total dose of 51 Gy and the tumor shrank remarkably. The patient returned to school under hormone replacement therapy.

Infant botulism with Down syndrome.

A 4‐month‐old boy with Down syndrome and infant botulism is reported. He was admitted to our hospital with stridor and developed respiratory failure and generalized paralysis. Laboratory examination revealed botulinal toxin type A in his blood and feces. Clostridium botulinum organisms were found in the feces and in the honey which he had been fed. Anti‐botulinal toxin was administered on the 10th day of illness. Artifical ventilation was given for 87 days and he survived. This is the 11th known case of infant botulism in Japan. It is possible that other cases remain undiagnosed.