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Ambient echolalia is a rare condition with few reported cases. We report the case of a 20-year-old man with a germinoma around the bilateral ventriculus lateralis who exhibited ambient echolalia. Clinical features included instinctive grasp reaction and compulsive manipulation of tools in his right hand. Speech or mental deterioration has been cited as a cause of ambient echolalia, but neither dementia nor aphasia was present. We propose that ambient echolalia in our case could be interpreted as a disinhibition of pre-existing essentially intact motor subroutines due to damage of the medial frontal lobe.

Ten primary central nervous system lymphomas (PCNSL, brain lymphomas) were examined for p14 gene exon 1beta deletion, mutation and methylation by Southern blot analysis, nucleotide analysis of polymerase chain reaction clones and Southern blot-based methylation assay. In Southern blot analysis, from the signal densities of the hybridized bands and their similarities to those of exons 2 and 3 in our previous quantitative study, we found that exon 1beta was homozygously deleted in four cases, hemizygously deleted in five cases and not deleted in one case. Thus, the same deletion patterns covered the entire p14 gene for all cases except for one case, which suggested the hemizygous deletion of exons 1beta and 2 and homozygous deletion of exon 3. In addition, although exon 1beta mutation is rare in various tumors, we detected a missense mutation (L50R) in one case with a hemizygous deletion. Methylation of the 5'CpG island of the p14 gene was not suggested for any case without homozygous deletion. Our observation of frequent p14 gene abnormalities (90%) and inactivation (40-60%) was in striking contrast to the same pathological subtype of systemic lymphoma in which p14 gene abnormalities and inactivation were infrequent, suggesting a difference in carcinogenesis between PCNSL and systemic lymphoma.

Using reverse transcription-polymerase chain reaction (RT-PCR), six primary brain lymphomas, pathologically diagnosed as diffuse large B-cell lymphoma, were examined for rearranged VH-D-JH sequences of the immunoglobulin heavy chain gene, focusing on somatic mutations and intraclonal heterogeneity. The reliability of the isolated PCR clones was confirmed by in situ hybridization (ISH) with complementarity-determining region (CDR) 3 oligonucleotide probes. Sequence analysis of the PCR clones revealed a high frequency of somatic mutation, ranging from 8.8 to 27.3% (mean 18.2%) in the VH gene segments in all the lymphomas. A significantly lower frequency of replacement (R) mutations than expected was also seen in their frameworks (FRs) in all cases. These findings suggested that the precursor cells were germinal center (GC)-related cells in these lymphomas. However, despite extensive cloning experiments, intraclonal heterogeneity was not detected in any case except for one in which it could not be ruled out. Thus, it seemed likely that all of our brain lymphomas were derived from GC-related cells and that at least most of them were from post-GC cells.

With the use of RT-PCR (reverse transcriptase-polymerase chain reaction), Northern blot analysis, and Western blot analysis, seven primary brain lymphomas were examined for the state of the MMACI tumor suppressor gene. Nucleotide analysis of RT-PCR clones revealed no abnormality in the MMAC1 coding sequence in each case. Although Northern blot revealed variation among cases in the signal intensities for MMAC1 mRNA, Western blot revealed a distinct MMAC1 protein band in all cases, suggesting that the actual MMAC1 expressions were similar. In Western blot analysis of phosphorylated Akt (p-Akt), which is regulated positively by PI3K (phosphoinositide-3 kinase) and negatively by MMAC1, all the lymphomas revealed an Akt band but not a p-Akt band, suggesting that the MMAC1 phosphatase activity was maintained in each case. These findings suggest that the MMAC1 gene is normal in its coding sequence, gene expression, and phosphatase activity in the lymphomas. Thus, unlike the p16 and p15 tumor suppressor genes, which are frequently deleted and inactivated in brain lymphoma and represent a striking contrast to systemic lymphoma, MMAC1 may not play an important role in carcinogenesis in this tumor, as in the systemic counterpart.

Subpopulations of infiltrating lymphocytes were studied by immunohistological method using monoclonal antibodies in gliomas and metastatic brain tumors. Thirteen specimens from 8 glioma patients, and 7 specimens from 3 metastatic brain tumor patients were used. No special therapy for brain tumor had been performed in these cases, but 3 glioma patients and all metastatic brain tumor patients had received steroid hormone. Frontal lobe obtained from the autopsy case of chest trauma was served as a normal control. Frozen sections were stained with avidin-biotin peroxidase complex method using Leu-series monoclonal antibodies for pan T-cells (Leu-1), cytotoxic/suppressor T-cells (Leu-2 a), helper/inducer T-cells (Leu-3 a) and B-cells (Leu-12). Lymphocyte infiltrates were quantitated by counting positively stained cells in 13 glioma and 7 metastatic brain tumor specimens. In normal frontal lobe, only a few T-cells infiltrated around several blood vessels in the parenchyma and subarachnoid space. But in the cases of glioma, many perivascular lymphocytic infiltrates were found and in the cases of metastatic brain tumor, many lymphocytes were found diffusely in the interstitial area between nests of tumor cells. Most of these lymphocytes were T-cells and B-cells were scarce, and Leu-2 a and Leu-3 a positive cells intermingled with each other. Len-3 a/2 a ratio ranged from 0.2 to 0.9 in the half of gliomas and 1.5 to 3.6 in another half of gliomas, three of which were treated with steroid hormone.(ABSTRACT TRUNCATED AT 250 WORDS)

Subpopulations of tumor-infiltrating lymphocytes (TIL's) and the major histocompatibility complex (MHC) antigens of neoplastic cells were examined in three intracranial germinomas by an immunohistochemical method using monoclonal antibodies. About 70% to 80% of TIL's were T lymphocytes which were either infiltrating diffusely or in clusters, whereas 20% to 30% of TIL's were B lymphocytes which tend to cluster in tumor tissues. Examination of T lymphocyte phenotypes revealed both the cytotoxic/suppressor and helper/inducer T lymphocytes, as in other tumors. However, the existence of a considerable number of B lymphocytes in the TIL population was uncommon and seemed to be a characteristic feature of the intracranial germinoma, which might suggest a difference of host immune response to this neoplasm as compared to other tumors. On examination of the MHC antigens, no MHC class I or II antigens in the neoplastic cells were stained, while positive staining for both antigens was seen in the TIL and stroma tissues. From these findings, it was suggested that the degree of TIL infiltration might not be correlated with the expression of MHC antigens in neoplastic cells in cases of primary intracranial germinoma.