Differences in clinical characteristics between Tourette syndrome patients with and without 'generalized tics' or coprolalia

Abstract  The purpose of this study is to examine whether there are differences in clinical characteristics between Tourette syndrome (TS) patients with and without 'generalized tics' (GT) which involve the entire body, and/or coprolalia. Subjects were 64 patients (55 males and 9 females, mean age, 17.4 ± 7.2 years) who visited Tokyo University's outpatient clinic of neuropsychiatry from 1974 to 1993 and who met criteria for Tourette's disorder of DSM-III-R. Data on clinical characteristics, including tic symptoms and courses of their development, complications and developmental histories, treatment and severity, were collected by systematic chart review of all subjects. Tourette syndrome patients with 'generalized tics' tended to show multiple complex vocal tics more frequently than TS patients without GT. Tourette syndrome patients with coprolalia tended to show significantly higher rates of copropraxia, echolalia, and 'cleaning/washing' compulsion than did the TS patients without coprolalia. Tourette syndrome patients with both GT and coprolalia were classified as the severest group in terms of tic symptoms and social impairment. Tourette syndrome patients who had neither of these morbidities were classified into the mildest group in all aspects. Generalized tics and coprolalia seemed to indicate the severest end of the TS spectrum and seemed to be related with a need of intensive treatment.     

Direct Drug Transport from the Rat Nasal Cavity to the Cerebrospinal Fluid: the Relation to the Molecular Weight of Drugs

To clarify the relationship between the direct transport from the rat nasal cavity to the cerebrospinal fluid (CSF) and the molecular weight of the drug, the transport of fluorescein isothiocyanate-labelled dextran (FD) with various molecular weights was investigated. FDs (average molecular weights 4400 (FD4); 9400 (FD10); 18 900 (FD20); 40 500 Da (FD40)) were administered nasally or intravenously to rats, and the concentrations in the plasma and the CSF were measured and compared. None of the FDs were detected in the CSF after intravenous administration. However, FD4, FD10 and FD20 were observed to appear in the CSF after nasal administration, whereas the concentration in the plasma was much lower than that after intravenous administration. FD40 was not detected even after nasal administration. In addition, the concentration of these FDs in the CSF decreased with the increase in the molecular weight of FDs. These findings show that drugs with a molecular weight up to at least 20 000 Da can be directly transported from the nasal cavity to the CSF and that the transport of FDs to the CSF is dependent on their molecular weights.     

Lennox-Gastaut syndrome: A new vista

Abstract Lennox-Gastaut syndrome (LGS) is regarded as a model of the epileptic syndrome because of its specific clinicoelectrical manifestation. However, a close investigation reveals that its outline is somewhat vague, having the borderland around it. Precise diagnosis in an individual case is not always easy. In this paper, the diagnostic criteria of LGS are described. According to these criteria, cases with LGS were subclassified into the typical and the atypical cases, and also cases in the borderland of LGS were reviewed. On the other hand, our prospective long-term follow-up study revealed that cortical mechanisms played an important role in the pathophysiology, clinical features and refractoriness of LGS. Secondary bilateral synchrony (SBS) is supposed to be a mode of expression of cortical mechanisms of LGS. A newly developed method with coherence and phase analysis demonstrated that the pathophysiology was based on SBS in 33% of the typical LGS cases. This finding is not only crucial for the choice of rational treatment including epilepsy surgery, such as callosotomy, but also contributes to a more refined subclassification of LGS.     

PREVALENCE OF RAYNAUD'S PHENOMENON AND SPECIFIC CLINICAL SIGNS RELATED TO PROGRESSIVE SYSTEMIC SCLEROSIS IN THE GENERAL POPULATION OF JAPAN

Background. In order to evaluate the prevalence rates of Raynaud's phenomenon (RP) and specific clinical signs related to progressive systemic sclerosis (PSS) in the general population of Japan, inquiries were made concerning RP in the hands and dermatologic examinations were also conducted. Methods. One thousand and sixty-three subjects (332 men and 731 women) over 30 years of age who underwent inhabitants' health examinations in 1990 were considered for this study. Results. The prevalence of RP was 3.0% in men and 3.4% in women. In 8 men and 17 women with RP who received the blood tests, the positive rates of antinuclear antibody (ANA) were 12.5% and 35.3% in men and women, respectively. The prevalence rates of all five specific clinical signs related to PSS, sclerodactyly, pitting scars of the fingers, brown pigmentation of the body, shortened frenulum of the tongue, and flexion contracture of fingers, were under 2% in men and 3% in women. In women with RP the prevalence rates of sclerodactyly, pitting scars of the fingertips, brown pigmentation of the body, and shortened frenulum of the tongue were 16.0, 4.0, 4.0, and 16.0%, respectively. These values were significantly higher than those of persons without RP. Conclusions. Because some persons with primary RP may become typical cases of PSS within several years, a follow-up study, particulary for women who have positive titers of ANA with RP, should be carried out to find out whether the persons suffer from PSS or not.     

Certain type of chronic lung disease of newborns is associated with Ureaplasma urealyticum infection in utero

BACKGROUND: Recent studies of chronic lung disease (CLD) of newborns emphasize the contribution of antenatal infection. However, the association of Ureaplasma urealyticum infection and CLD has been controversial. The purpose of the present paper was to determine whether U. urealyticum is associated with chorioamnionitis (CAM) and a certain type of CLD. METHODS: One hundred and five infants <32 weeks of gestation who were admitted to the neonatal intensive care unit at Jichi Medical School Hospital, who underwent both histological and microbiological examinations and who survived to discharge were included. CAM was determined by histological examination. Placenta, gastric and tracheal aspirates, and nasopharyngeal swabs were cultured for Mycoplasma and other microorganisms. CLD was defined as oxygen needed at 28 days of age with symptoms of persistent respiratory distress and hazy or emphysematous and fibrous appearance upon X-ray. CLD was further divided into two subtypes according to the presence of antenatal infection. RESULTS: CAM was associated with premature rupture of membrane (odds ratio [OR], 10.19; 95% confidence interval [CI]: 3.10-33.56), placental colonization of U. urealyticum (OR 6.73, 95%CI: 1.89-23.91), neonatal colonization of other microorganisms (OR 7.33, 95%CI: 1.22-44.13) and level of IgM (OR 1.06, 95%CI: 1.01-1.11). Comparisons between CLD and non-CLD patients showed that gestational age (OR 0.43, 95%CI: 0.30-0.61) and white blood cell count (WBC) at birth (OR 1.06, 95%CI: 1.01-1.11) were risk factors for CLD, while gestational age (OR 0.38, 95%CI: 0.23-0.64), neonatal colonization of U. urealyticum (OR 5.98, 95%CI: 1.17-30.6) and WBC (OR 1.08, 95%CI: 1.01-1.15) were independent risk factors for infection-related CLD compared with non-CLD. Within CLD, infection-related CLD was associated with neonatal colonization of U. urealyticum (OR 43.7, 95%CI: 2.84-673.8) and WBC (OR 1.27, 95%CI: 1.07-1.50). CONCLUSIONS: Placental colonization of U. urealyticum was significantly related to CAM; and neonatal colonization of U. urealyticum and leukocytosis at birth were risk factors for infection-related CLD.     

Alternating Non-Cross Resistant Chemotherapy for Small Cell Lung Cancer

After stratification for the extent of disease, previously untreatedpatients .with small cell lung cancer randomized to receivetherapy with the four-drug combination of cyclophosphamide,oncovin, nimustine hydrochloride (ACNU), and procarbazine (CONP)every four weeks (continuous regimen) or to receive CONP alternatingwith the three-drug combination of etoposide (VP-16), adriamycinand cisplatin (VAD) at four-week intervals (alternating regimen).Sixty-nine patients were entered in the study. Of 34 evaluablepatients receiving the continuous regimen, six (17.6%) achievedcomplete response (CR) and 16 (47.1%) achieved partial response(PR). Of 31 evaluable patients receiving the alternating regimen,10 (32.3%) achieved CR, and 16 (51.6%) achieved PR. There wasa tendency in favor of the alternating regimen in CR and overall response rates (0.05 < p < 0.1). There were no significantdifferences be tween the regimens in response duration or survival.The projected median survival times were 9.2 months and 9.4months for the continuous and alternating regimens, respectively.One patient receiving the continuous regimen and three receivingthe alternating regimen have been living for more than two years.The major toxicity was myelosuppression in both regimens. Onepatient died of hemorrhage due to thrombocytopenia during inductionwith CONP, and one patient died of cisplatin-induced renal failure.We conclude that alternating non-cross resistant chemotherapyleads to improved CR and response rates, but does not improvesurvival.     

Duration of apnoea in anaesthetized children required for desaturation of haemoglobin to 95%: comparison of three different breathing gases

In this study, we compared three gas compositions to determine if the duration of apnoea for Spo₂ to decrease is proportionate to the oxygen fraction of the gas prior to apnoea. Twenty-five patients ASA physical status 1–2 aged two months to 12 years were included in the study. Anaesthesia was induced via a mask with 5% sevoflurane and 66% N₂O in oxygen. After paralysis with vecuronium (0.12 mg·kg^?1, i.v.) the trachea was intubated and anaesthesia was maintained with sevoflurane and N₂O in oxygen. When cardiovascular stability was obtained, the patient was randomly set to breathe one of three gas compositions: 1. oxygen (Fio₂ 1.0), 2. N₂O/O₂ (Fio₂ 0.4), and 3. air/O₂ (Fio₂ 0.4). All three gas compositions included 2–4% of sevoflurane to maintain anaesthesia. After more than eight min of each gas breathing, apnoea was begun by disconnecting the breathing circuit from the tracheal tube. The time from the start of apnoea (Spo₂ 100%) to Spo₂ of 95% (T₉₅) was measured. T₉₅ measured after breathing N₂O/O₂ and air/O₂ were 34.6 ± 5.7 and 28.8 ± 4.7% of that measured after oxygen breathing (P < 0.001 vs oxygen breathing, P < 0.001 vs oxygen and N₂O/O₂ breathing), respectively. Preoxygenation before intubation was validated to delay the haemoglobin desaturation brought about by apnoea. An induction technique using a low Fio₂ will allow rapid haemoglobin desaturation.     

Anti-DNA antibody derived from a systemic lupus erythematosus (SLE) patient forms histone–DNA–anti-DNA complexes that bind to rat glomeruli in vivo

Histone can mediate the binding of both free DNA and DNA complexed to anti-DNA antibody to the glomerular capillary wall. We tested whether preformed histone–DNA–anti-DNA immune complexes (IC) could bind to the glomerular capillary wall. The immune complex, generated with anti-DNA antibody derived from an SLE patient and excess of ¹²⁵I-DNA followed by digestion with DNase, was mixed with histones. The complex containing 4 μg DNA was injected via the aorta into the left kidney of rats. At 15 min, 1.3% of the histone–DNA–anti-DNA antibody complex bound (measured as ¹²⁵I-DNA), when histone was omitted less than 0.1% of the DNA–anti-DNA antibody complex bound. By immunofluorescence human immunoglobulins and histones, representing the IC, could be observed in a capillary pattern; but no complement deposition was detected. Electron microscopy revealed discrete, electron dense deposits in a subendothelial, subepithelial and mesangial localization at 15 min. These results provide direct evidence that antibodies from serum of SLE patients can form soluble histone–DNA–anti-DNA immune complexes that bind to the glomerular capillary wall in vivo     

Juvenile Secretory Carcinoma and Juvenile Papillomatosis

Two cases of juvenile secretory carcinoma associated with juvenilepapillomatosis are reported. One patient's disease was initiallydiagnosed at the age of 13 and she died 12 years later withextensive metastases. The contralateral breast at autopsy showedfindings indicative of juvenile papillomatosis. The second caseis that of an 18-year-old girl who had multiple juvenile secretorycarcinomas in the left breast associated with juvenile papillomatosis.     

Umbilical cord blood as a rich source of immature hematopoietic stem cells

To investigate immaturity of hematopoietic progenitor cells in umbilical cord blood mononuclear cells (CB-MNC), the formation of macroscopic colonies and mixed-cell colonies was assayed by methylcellulose culture with various combinations of cytokines (stem cell factor [SCF], interleukin [IL]-3, IL-6, granulocyte-colony stimulating factor [G-CSF], erythropoietin [EPO]) and compared with bone marrow (BM)-MNC. Moreover, distribution of the subpopulations divided by CD34, CD38, HLA-DR and CD33 was compared by flow-cytometry. Colonies derived from CB-MNC were so large that they could be observed with the naked eye and consisted of a variety of types of hematopoietic cells. Mixed-cell colonies were formed to a much greater extent in CB-MNC than in BM-MNC. Addition of EPO, IL-3, and SCF had rapid effects on the growth of mixed-cell colonies. The subpopulations of immature hematopoietic progenitor cells (CD34⁺, CD38⁻, HLA-DR⁻), which are supposed to be able to differentiate into hematopoietic precursors and stromal cells, were significantly higher in CB-MNC (8.7±6.6%) than in BM-MNC (0.0±0.1%; P < 0.001). These results suggest that CB is a rich source of immature hematopoietic progenitor cells compared to BM.