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.     

Serum levels of IL-10, IL-15 and soluble tumour necrosis factor-alpha (TNF-α) receptors in type C chronic liver disease

We previously reported that the number of TNF-α-producing cells was increased in the liver of patients with type C chronic liver disease. To understand further the pathophysiology of this change, we examined serum levels of two soluble TNF receptors, TNF-αRI (p55) and -αRII (p75), and IL-10, all of which act as TNF-α buffer, and IL-15, a novel cytokine sharing many immunological activities with IL-2, using ELISA methods. We studied control individuals and patients with type C chronic liver disease, including asymptomatic hepatitis C virus (HCV) carriers with persistently normal serum ALT values, and those with chronic hepatitis (CH), liver cirrhosis (LC) and hepatocellular carcinoma (HCC). Both types of sTNF-αR closely correlated with disease progression. Patients with LC and HCC had significantly elevated levels for sTNF-αRII compared with the other patient groups and controls. Serum IL-10 levels were significantly greater in all chronic liver disease groups than in controls. With respect to IL-15, the values were high in CH, LC and HCC compared with those of controls. Notably, HCC patients showed highest values for both IL-10 and IL-15, with significant differences from the other patient groups. Serial determinations revealed that interferon (IFN) treatment for CH patients resulted in the suppression of circulating IL-10 and IL-15 levels along with decrease in serum aminotransferase values. Both cytokines remained at decreased levels after cessation of therapy in patients who went into clinical and virological remission. On the other hand, treatment did not affect serum levels of sTNF-αRs. These findings indicate that serum levels of these molecules correlated with disease progress in chronic HCV infection, and that IL-10 and IL-15 may reflect the degree of inflammation in the liver. It is also suggested that both cytokines may be related to the development of HCC.     

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.     

A 56 Year-Old Female with Congenital Hepatic Fibrosis Diagnosed by Laparoscopy

Abstract: We describe a 56-year-old woman with congenital hepatic fibrosis. Blood tests and liver scanning with Tc-99m-labelled galactosyl human serum albumin revealed mild liver dysfunction. Per-rectal portal scintigraphy with iodine-123 iodoamphetamine showed severe abnormalities in the portal circulation, and the portal pressure measured during percutaneous transhepatic portography was high (350 mmH₂O). Idiopathic portal hypertension was suspected. Laparoscopy disclosed diffuse, intense dendritic white markings around the liver. Congenital hepatic fibrosis was confirmed on histologic examination of a biopsy specimen obtained during laparoscopy. In summary, we report a rare and relatively elderly case of CHF, in which laparoscopy was useful in the diagnosis. (Dig Endosc 1999; 11: 174–178)     

Clonal and non-clonal karyotypically abnormal cells in haemophagocytic lymphohistiocytosis

We studied chromosomes in bone marrow (BM) or peripheral blood cells of nine patients with haemophagocytic lymphohistiocytosis (HLH); three of them had a family history of HLH and four others underwent concurrent Epstein-Barr virus (EBV) infection. In addition to a large population of normal mitotic cells, karyotypically abnormal clonal cells were found in two patients, abnormal clonal cells and a nonclonal (single) abnormal cell in one, and nonclonal abnormal cells in three. All the six patients with chromosome abnormalities died of progressive disease; one of them also had EBV infection and EBV-associated clonal proliferation. Two of three patients with EBV infection and only normal mitotic cells in BM completely recovered from the disease.
Although HLH did not show histological and/or haema-tological evidence of a neoplastic disease, clonal chromosome abnormalities and the fatal clinical outcome found in some of the patients suggest that the disease may be heterogenous and include malignancy. HLH patients with karyotypically abnormal clonal cells in BM should warrant more intensive chemotherapy than that presently being applied to them and should be considered as candidates for BM transplantation.     

Congenital leukaemia with a mixed phenotype of megakaryoblasts and erythroblasts: a case report and characterization of the blasts

We present a congenital leukaemia with a mixed phenotype of megakaryoblasts and erythroblasts. A newborn male with exopthalmus and multiple skin nodules, had bone marrow blasts which expressed CD41b, CD42b, glycophorin-A and haemoglobin, but monocyte or lymphoid markers were negative. The patient achieved a complete remission with chemotherapy. Blasts cultured for a few months expressed erythroid markers but lost the megakaryocytic phenotype, although addition of phorbol ester induced the latter phenotype. Spontaneous colony formation was observed in semi-solid culture and the number of colonies was increased by erythropoietin. Detailed studies further indicated the heterogeneity of congenital leukaemia.     

One-year prognosis of depressive illness in the elderly population in Japan

A 1-year prospective study of 43 elderly depressed residents (13 men and 30 women) in Nagai City in Japan is described. An initial survey was carried out in 1993 to find depressed residents. The subjects of the survey were 2056 residents of 65 years of age and older. The Japanese version of the Geriatric Depression Scale (GDS) was employed as a screening tool in the first phase of the survey. In the second phase, screened subjects and control subjects were interviewed by psychiatrists using the Structured Interview for DSM-III-R (SCID). The diagnosis of depression was made by the psychiatrists on the basis of the results of the SCID. Forty-three persons were judged to be depressed. At follow-up, 10 were still depressed and 15 were well. Four were demented. Fourteen dropped out due to death, hospitalization, absence from home or refusal. The results showed that approximately half of the elderly depressed persons seemed to recover by the time of the 1-year follow-up. One-year prognosis of dysthymia was the worst. Some types of depression seemed to be a precursor for dementia. The concern is with how the findings may be used as an aid in understanding and planning community mental health services strategies. The results indicate that it is very important to pay close attention to patients with depressive illness who do not meet the criteria for major depression.