Possible Role of Streptococcus Pyogenes in Mucocutaneous Lymph Node Syndrome. XII. Variable Responses of Platelets in MCLS Seem to Be Explainable by Streptococcal Pyrogenic Exotoxin

As a model system for mucocutaneous lymph node syndrome (MCLS), we have advocated and used mice which had been rendered tolerant to Streptococcus pyogenes-associated antigens by neonatal infection with group A fteta-hemolytic streptococci, because these mice have shown a variety of peculiar bioimmunological characteristics bearing a striking resemblance to those of MCLS patients. The results of our current investigations reaffirmed the reliability of the animal model by indicating that mice subjected to neonatal infection with 5. pyogenes , or inoculation with streptococcal pyrogenic exotoxin (SPE) in Freund's adjuvant, were perfect counterparts of patients with MCLS on account of their platelet activation and hyperaggregability in response to provocative treatment, which are familiar findings in this disease.     

A newly designed attachment device of multipurpose frame for neuronavigator

Summary A newly designed attachment device of the multipurpose head frame (Sugita) for Neuronavigator (Watanabe) is presented with an illustrative case of glioblastoma in an eloquent area. This has extended the usefulness of the neuronavigator for those who prefer and use the multipurpose head frame, while the requirements for keeping a stereotactic combination and the original concept of the multipurpose head frame, as well as that of the neuronavigator have been kept undisturbed.     

Detection limit in low-amplitude EEG measurement.

Electrocerebral inactivity for the determination of cerebral death is defined as no findings of EEG greater than the amplifier's inherent internal noise level when recording at increased sensitivity. A surface biopotential electrode contains two interfaces composed of skin gel (electrolyte) and gel electrode (metal), each forming a noise source. The power spectral density, S(f), of extremely low noise signals was obtained by means of autocorrelation and fast Fourier transformation. Interelectrode resistance, R(f), was measured with synchronous rectification. The formula of equivalent noise resistance R(n) = S(f)/4kT, where k is the Boltzmann constant and T is room temperature in Kelvin, gives a resistance that generates the thermal noise corresponding to the measured S(f). Rn/R is a parameter derived from normalization by R. When Rn/R = 1, measured noise contains thermal noise only. Meanwhile, Rn/R > 1 indicates presence of excess noise, such as 1/f, and tissue noise in addition to the thermal noise. Mean square root (Rn/R) of the scalp noise was 10.8 at 10 Hz, showing existence of excess noise. The study results suggest that it is necessary to take excess noise into consideration in the measurement of low-amplitude EEG for the determination of cerebral death.     

T Cell Receptor Vβ Repertoires of Angioimmunoblastic Lymphadenopathy-like T Cell Lymphoma

To elucidate the pathogenesis of angioimmunoblastic lymphadenopathy-like T cell lymphoma (AILD-T) we investigated the T cell receptor Vβ gene repertoires of four AILD-Ts and compared them with those of other histological types of lymphomas and three cases with reactive disorders. All lymphoma patients had rearrangement bands detected by Southern blot analysis. Only 1 of the 4 cases of AILD-T showed a single predominant usage of Vβ 20 gene by PCR with 20 different Vβ specific primers and the others had repertoires somewhat restricted but similar to reactive lesions. Subsequent sequencing of this PCR product revealed that only 2 of 7 clones were identical. These results suggest the monoclonal malignant cells in AILD-T are scant and that the infiltrating T cells show a reactive pattern. In the only AILD-T case with a single dominant Vβ usage, the relationships of this repertoire and lymphoma cells seems to be of some consequence.     

Late Effects of Childhood Acute Leukemia and Its Treatment

Late effects of childhood acute leukemia and its treatment were studied in 766 patients (684 ALL, 73 ANLL, and 9 others) in Japan who had remained in remission for more than 1 year after their first complete remission. Delayed adverse sequelae involve a wide variety of organs and their functions. Short stature was present in 2.61%, obesity in 3.79%, abnormalities of growth hormone secretion in 1.5%, delayed secondary sex characteristics in 1.5% of males and 0.6% of females, motor disturbances in 1.17%, sensory disturbances in 0.91%, intellectual and learning disabilities in 2.48%, abnormal findings in routine neurologic examinations in 1.31%, EEG abnormalities in 4.30%, brain CT abnormalities in 5.09% and cardiac dysfunction in 1.07%. Various other disorders were seen in 20 patients. Many of these delayed adverse sequelae are caused by or related to central nervous system prophylaxis and systemic combination chemotherapy. The results suggest that it is needed to improve therapeutic methods through the stratification of patients by risk factors and detailed analysis of prognostic factors. Moreover it is important to render medical and psychosocial support to long-term survivors of childhood leukemia through interactions between the patient, parents and medical staff.     

An Autopsy Case of Tracheal Agenesis with Broncho-esophageal Fistula

Abstract True incidence of this malformation is probably greater than that reported since the definitive diagnosis has been made at autopsy in most cases. Various hypotheses on the pathogenesis of tracheal agenesis have been proposed but they are still controversial.
In this report, we present a case of tracheal agenesis with a broncho-esophageal fistula and discuss the formal genesis.     

Rupture of an isolated internal iliac artery aneurysm into the rectum: Report of a case

Aneurysmal rupture into the intestinal tract is a rare but disastrous complication of an internal iliac artery aneurysm. We report herein the successful surgical repair of a fistula between a huge aneurysm of the right internal iliac artery and the rectum in an 81-year-old man. After a femoro-femoral cross-over bypass had been performed, the aneurysm was opened and its patent arterial branches were ligated with sutures. The fistula was then intra-aneurysmally sutured and covered with an omental flap. The diagnostic and therapeutic approaches to this severe complication are discussed with a review of the literature following the presentation of this case.     

Clinical usefulness of the frontal assessment battery (FAB) for elderly in memory clinic]

To investigate the usefulness of the frontal assessment battery (FAB) as a brief assessment for diagnosis in patients with dementia, we examined cognitive function using the FAB, Mini-mental state examination (MMSE), Kana-hiroi test, and Raven's colored progressive matrices (RCPM). Subjects were 82 senile patients having amnesia as a complaint including 28 Alzheimer's disease (AD), 14 frontotemporal dementia (FTD), 17 vascular dementia (VD), 13 mild cognitive impairment (MCI), and 10 healthy persons. We discussed the difference of scores in different types of dementia. In the FAB, the scores of healthy persons and MCI were much higher compared with the scores of all types of dementia. There were high correlation between FAB and MMSE, Kana-hiroi test, and RCPM. Although some subjects showed normal score in MMSE and/or RCPM, they showed low score in FAB. Based on this, FAB is efficient for the differential diagnosis of dementia as brief screening tool.     

Clinical Course and Autopsy Findings of a Patient with Clival Chordoma Who Underwent Multiple Surgeries and Radiation during a 10-Year Period.

The management of clival chordoma remains problematic. We present the case of a 48-year-old woman with clival chordoma who underwent multiple surgeries and radiation therapy, including gamma knife stereotactic radiosurgery (GK-SRS), during a 10-year clinical course. The tumor was initially removed by gross total resection via the trans-sphenoidal approach, followed by external linac radiation therapy. The tumor recurred at the clivus 5 years after the initial operation. After repeated trans-sphenoidal removal of recurrent tumors, she twice underwent GK-SRS for a tumor remnant adjacent to the brainstem. Although this part of the tumor was controlled by GK-SRS, there was further tumor extension toward the sphenoid and maxillary sinuses. Ultimately, lower cranial nerve dysfunction developed due to tumor extension into the lower part of the clivus and the patient died of respiratory failure. Autopsy revealed the tumor to extend from the lower clivus to the bilateral middle fossae. The lower part of the tumor extended to the nasal cavity and to the posterior wall of the pharynx, resulting in compression of the upper pharyngeal region. The tumor around the jugular foramen compressed the lower cranial nerves bilaterally. Tumor cells did not, however, invade the intradural space microscopically. Although chordoma is not biologically malignant, this tumor can show massive extension with destruction of bony structures and extracranial invasion of connective tissues. Therefore, the optimal treatment strategy is to remove the tumor mass as extensively as possible, including normal bony structures and connective tissues surrounding the tumor, using skull base surgical techniques.