Neoplasm of endolymphatic sac origin: clinical, radiological and pathological features.

This paper reports on a 55-year-old female who had undergone middle ear surgery 12 years previously and was admitted with a 6-months history of unilateral hearing loss and facial weakness. MRI and CT demonstrated a space-occupying lesion arising from the temporal bone and extending into the posterior fossa. Treatment consisted in complete tumour removal. Temporal and mastoid bone destruction associated with typical histological features led to the diagnosis of neoplasm of endolymphatic sac origin. Clinical, histological, radiological and intra-operative features of these rare tumours are described and discussed. The pertinent literature is reviewed.

Spongy encephalopathy in ketotic hyperglycinemia.

Three cases of ketotic hyperglycinemia are described. Spongy encephalopathy was present in white as well as gray matter. The cell type that predominantly exhibited swelling was the astrocyte. Glycine binding is required for activation of the NMDA receptor. By constant excitation a surplus of glycine could disturb the ion balance. This might provide the pathogenetic principle of seizures and cytotoxic edema in hyperglycinemia.

Bombesin receptor gene expression in rat pancreatic acinar AR42J cells: transcriptional regulation by glucocorticoids.

BACKGROUND/AIMS: This study investigated the correlation between glucocorticoid-regulated gene expression of the bombesin receptor (BR) and cellular sensitivity to bombesin stimulation in the rat pancreatic acinar cell line AR42J. METHODS: BR gene expression was assessed using a cloned complementary DNA probe and radioligand binding assays. Intracellular Ca2+ mobilization was assessed by dual wavelength spectrophotometry using fura-2 in single cells. RESULTS: Dexamethasone resulted in a rapid dose- and time-dependent decrease of BR messenger RNA levels with maximal inhibition to 25% +/- 2% of controls (n = 4) after 6 hours of hormone treatment. BR messenger RNA half-life was approximately 120 minutes and was not affected by dexamethasone pretreatment; nuclear run-on analysis showed a decreased transcription rate of the BR to approximately 25% of control after hormonal treatment. Radioligand binding studies showed a time-dependent decrease of specific bombesin binding to 25% +/- 8% of control after 48 hours of hormone treatment. Down-regulation of BR gene expression by dexamethasone resulted in a time- and dose-dependent decrease of intracellular Ca2+ mobilization after bombesin stimulation compared with untreated controls. CONCLUSIONS: Glucocorticoids decrease BR gene transcription. The subsequent decrease in cellular BR number renders AR42J cells less sensitive for bombesin-stimulated intracellular Ca2+ mobilization.

Ultrastructural morphology of the aged pineal.

Thirty-four pineals of 18-month-old rats were studied, of which 12 belonged to animals exposed to 7% and 13 to a group exposed to 10% oxygen in breathing gas. Nine pineals were from controls kept under normoxic conditions. For comparison 2 pineals of 6-week-old rats were also examined. No influence of hypoxia on histology of the pineal gland can be stated. In this the pineal behaves like other parts of the rat brain, which in previous experiments has been shown to adapt well to low-oxygen concentrations. Also no important difference between pineals of young and advanced aged rats were found by us. In 8 of 34 pineals striated muscle cells were present. We consider this an important finding, as pineal cells have a unique differentiation potential in cell culture systems and in tumorous lesions of the pineal. As rats exposed to severe hypoxia exhibit body organ changes indicating stress, it would be interesting to measure melatonin secretion and pineal melatonin content under prolonged and increasing hypoxic conditions.

[Intraspinal neurinoma in the thoracolumbar junction presenting unusual symptoms. The differential diagnosis of lumbago]. / Intraspinales Neurinom im thorako-lumbalen Ubergang mit ungewöhnlicher Symptomatik. Ein Beitrag zur Differentialdiagnose von Kreuzschmerzen.

After more than a year of persistent lumbosacral pain a 53-year-old woman suddenly developed unilateral monoradicular pain over the S1 dermatome. Neurological, general medical and biochemical examinations were unremarkable, but myelography and magnetic resonance imaging revealed a cystic intraspinal space-occupying lesion at the level of L1, which was completely excised surgically. It proved to be a neurinoma, 5 x 3 x 3 cm, completely filling the spinal canal at the junction between the conus medullaris and the cauda equinus. The patient was without symptoms and neurological deficit after the operation. The tumour having been completely removed, the prognosis is good.

[Supratentorial glioma: analysis of clinical and temporal parameters in 163 cases]. / Supratentorielle Gliome: Analyse klinischer und zeitlicher Parameter in 163 Fällen.

163 cases of supratentorial astrocytomas and glioblastomas were evaluated retrospectively after close-meshed observation and treatment. We attached the greatest importance to the reevaluation of already known prognostic parameters and to the temporal analysis of the course of gliomas. We could confirm the influence of the histologic grade on the survival time. Histologic grading by means of immunohistochemistry proved to be more precise than grading only by means of HE staining. Furthermore, the patient's age was one of the most important prognostic variables for survival time after operation. Other factors were the first preoperative Karnofsky rating, the preoperative diameter of the tumour, the duration of preoperative symptoms and the interval between operation and diagnosis of tumour recurrence as well as between tumour recurrence and reoperation. Epileptic seizures as preoperative symptoms were found to be far less prognostic for survival time. Localisation of the tumour, other preoperative symptoms, Karnofsky rating before reoperation and the extent of tumour resection proved to be of no importance for survival time.

Chromosomal changes and correspondingly altered proto-oncogene expression in human gliomas. Value of combined cytogenetic and molecular genetic analysis.

A combined cytogenetic and molecular genetic study was performed to analyze seven primary brain tumors: one oligoastrocytoma WHO-grade II, one anaplastic astrocytoma grade III, one anaplastic astrocytoma grade III/IV and four glioblastomas by G-banding and RNA dot blotting. A normal karyotype was found in the oligoastrocytoma. One of the two anaplastic astrocytomas (male) contained cells with a normal karyotype and cells with a Y-chromosomal loss, and the other one showed structural abnormalities too complex for complete analysis in mostly polyploid cells. Two of the four glioblastomas had few and the other two multiple chromosomal changes such as +7, +20, -8, -9 del(9)(p21), -10, del(10)(q24), -13,14, del(17)(p21), -22, add(3)(q13), double minutes and marker chromosomes. Compared to normal brain, all tumors had an increased EGFr and both anaplastic astrocytomas as well as three glioblastomas a decreased H-ras expression. The two glioblastomas with multiple chromosomal changes showed increased EGFr, Ki-ras, myb, mos and myc, decreased H-ras and N-ras and unchanged levels of abl, src and sis. Both the cytogenetic and molecular genetic findings are compatible even in the case of chromosomal losses, where the genes on the remaining allele may be responsible for dominant gene regulation mechanisms which result in a protooncogene overexpression. Our findings indicate that, apart from proto-oncogene overexpression, other mechanisms, e.g. tumor suppressor gene inactivation, are important for glial tumorigenesis. Karyotypic analysis makes it possible to search specifically for genetic events still unknown but arising from particular chromosomal changes.

In contrast to EGFr gene overexpression, H-ras gene expression decreases in human gliomas.

We have examined the expression of the epidermal growth factor receptor (EGFr) gene and 14 other oncogenes in several human gliomas. EGFr overexpression was apparent in 17 of 18 tumors. Nevertheless, remarkable amounts of aberrantly-sized EGFr mRNAs were not found in the 11 tumors examined. Among the 14 other oncogene probes employed, only the H-ras probe revealed changes in the expression level. The expression of this oncogene was 2-3-fold reduced in 13 of 18 tumors. In neural tissue the H-ras gene expression can be correlated with cellular differentiation. A correlation between H-ras protein expression and a favourable clinical prognosis was found in neuroblastomas. The decreased amounts of H-ras transcripts in gliomas may be a sign of a lower state of differentiation of the cells.