Headache as initial symptom of schizophrenic disease]

Initial schizophrenia was observed in 15 out of 93 out-patients being treated for cephalea in a Regional Neuropsychiatrical Department. Certain abnormal phenomena in the field of consciousness and body sensations are typical of coenasthetic schizophrenia, with vital asthenia and vegetative symptoms. Cephaleas and head dysaesthesias are reported. There is no doubt that coenaesthetic schizophrenia has many points in common with latent schizophrenia; on the other hand it is also closely linked to hypochondriac depression, the syndrome of endogenous juvenile failure, certain latent depressions, hypochondriac euphoria, vegetative and endoreactive dysthmia and pseudoneurotic schizophrenia. Personal studies of stress responses in schizophrenia, and pneumoencephalographic examinations and EEG data in the active stage suggest diencephalic alteration. For diagnostic and initially therapeutic purposes, every patient with cephalea should be examined thoroughly by the psychiatrist; in this way the number of schizophrenias identified and treated will be considerably increased.     

Arthritis as first symptom of leukemia in children

Three children out of 30 (10%) referred for juvenile chronic arthritis had leukaemia. The patients had complained of intermittent musculoskeletal pain and painful joint swelling for three weeks, nine months and eighteen months prior to admission. On admission two of the patients had active arthritis with soft tissue swelling in one and three joints respectively. The third patient had only arthralgias and no joint swelling. All patients had slight anaemia, normal to slightly reduced thrombocyte count, slight neutropenia and absence of blasts in the peripheral blood. The correct diagnosis was made by bone marrow aspiration. Two children had acute lymphoblastic leukaemia and the third acute myeloblastic leukaemia. Leukaemia thus remains an important differential diagnosis in children presenting with musculoskeletal pain and/or arthritis.     

Antibody diagnosis iin sprue/celiac disease]

The clinical appearance of celiac disease is diverse, often times uncharacteristic and may therefore be very difficult to diagnose. Furthermore, severe inflammation of the small bowel can be present without gastrointestinal symptoms. It is estimated that most subjects with celiac disease are not diagnosed. Yet, early diagnose is desirable, since celiac disease causes growth retardation in untreated children and is potentially cancer disposing. Thus, all patients should adhere to a strict gluten-free diet. The role of antibody testing and small bowel biopsy in celiac disease will be discussed. In the future, the development of new tests may allow screening of large populations.     

Identification of tissue transglutaminase as the autoantigen of celiac disease

Celiac disease is characterized by small intestinal damage with loss of absorptive villi and hyperplasia of the crypts, typically leading to malabsorption. In addition to nutrient deficiencies, prolonged celiac disease is associated with an increased risk for malignancy, especially intestinal T-cell lymphoma. Celiac disease is precipitated by ingestion of the protein gliadin, a component of wheat gluten, and usually resolves on its withdrawal. Gliadin initiates mucosal damage which involves an immunological process in individuals with a genetic predisposition. However, the mechanism responsible for the small intestinal damage characteristic of celiac disease is still under debate. Small intestinal biopsy with the demonstration of a flat mucosa which is reversed on a gluten-free diet is considered the main approach for diagnosis of classical celiac disease. In addition, IgA antibodies against gliadin and endomysium, a structure of the smooth muscle connective tissue, are valuable tools for the detection of patients with celiac disease and for therapy control. Incidence rates of childhood celiac disease range from 1:300 in Western Ireland to 1:4700 in other European countries, and subclinical cases detected by serological screening revealed prevalences of 3.3 and 4 per 1000 in Italy and the USA, respectively. IgA antibodies to endomysium are particularly specific indicators of celiac disease, suggesting that this structure contains one or more target autoantigens that play a role in the pathogenesis of the disease. However, the identification of the endomysial autoantigen(s) has remained elusive. We identified tissue transglutaminase as the unknown endomysial autoantigen. Interestingly, gliadin is a preferred substrate for this enzyme, giving rise to novel antigenic epitopes.     

Unilateral neonatal cystic disease of the kidney as first manifestation of tuberous sclerosis]

Traumatic optic neuropathy (TON) causes blindness of varied severity and occurs infrequently as a complication of closed head injury. A case is presented of TON that occurred in a patient who suffered complete T4 paraplegia from a motorcycle accident but in whom no severe head injury took place. In this case, high-dose intravenous methylprednisolone was begun for the spinal cord injury and repeated 24 hours later for the TON. Vision improved from near total blindness to 20/400 in the left eye (OS) and 20/130 in the right eye (OD). Two weeks later, however, the patient's vision suddenly worsened. Magnetic resonance imaging (MRI) using fat suppression confirmed a lesion along the optic nerve consistent with TON. A third course of methylprednisolone again led to improved vision. The steroids were then tapered orally over 2 weeks and the patient had no further relapses. Moderate to severely impaired vision of 20/ 400 OS and 20/130 OD continues to interfere with the patient's function and spinal cord rehabilitation program. It was concluded that a steroid taper was important in maintaining initial visual gains in this case. Awareness of TON and careful attention to the patient's clinical course can minimize deficit and maximize functional outcomes.     

Proposed criteria for diagnosis of celiac disease in children]

At a seminar arranged in September 1997 by the Swedish Paediatric Working Group for Coeliac Disease, a diagnostic protocol proposed by the working group was approved by a majority of the paediatricians present, representing almost all paediatric units in Sweden. Briefly, a small bowel biopsy is called for in all children, both at presentation and as a control during gluten-free dieting. Subsequent gluten challenge and biopsy are mandatory only in cases of atypical presentation or if the diagnosis is questioned at some future date. Serum antigliadin and anti-endomysial antibody tests are complementary tools. Agreement was also reached regarding the institution of a national coeliac disease registry.     

Autoantibodies to tissue transglutaminase as predictors of celiac disease

Our aim was to study the prognostic value of growth hormone (GH) -stimulated insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 (IGFBP-3) generation in patients with compensated [group 1 (N = 8) with a Child-Pugh (CP) score of 5-8] and decompensated postnecrotic liver cirrhosis [group 2 (N = 7) with a CP score of 9-12]. Serum levels of IGF-I, GH-binding protein (GHBP), and IGFBP-3 were measured before and 24 hr after a single subcutaneous injection of recombinant human GH (rhGH, 0.14 units/kg). Patients (mean age 56 years) were followed prospectively for three years. Six patients (40%) died during the follow-up period, of whom half had a CP score <9. Mean serum IGF-I levels 24 hr after rhGH injection (group 1 vs group 2, 17.4 +/- 6.8 vs 7.4 +/- 0.7 nmol/liter) predicted survival with 93% accuracy. Levels <10 nmol/liter portended a poor prognosis, with 15% survival at one year, whereas levels >10 nmol/liter had a 100% survival rate at one and two years, respectively. Baseline IGF-I (9.98 +/- 2.0 vs 6.38 +/- 0.8 nmol/liter), GHBP (9.2 +/- 3 vs 5.7 +/- 0.8%/50 microl), and IGFBP-3 serum levels at baseline (1.7 +/- 0.3 vs 0.86 +/- 0.2 mg/liter) and at 24 hr (2.04 +/- 0.38 vs 0.99 +/- 0.3 mg/liter) did not add to the predictive value of stimulated IGF-I levels at 24 hr and were less accurate in predicting the outcome in comparison to CP score (80%). We conclude that stimulated IGF-1 <10 nmol/liter may be a true predictor of a negative prognosis in patients with liver cirrhosis.     

Demonstration of celiac disease in Cuba

A study performed in 50 children carriers of celiac disease with ages between 8 months and 14 years of age, is presented; diagnosis was proved based on clinical picture, histological changes of jejunal mucosa, intestinal absorption tests and response to the suppression of gluten from the diet, with clinical, histological and biochemical control according to international criteria. It has been insisted on the familial study and the gluten overload test and its incidency in the black race. The development of public health has made it possible to demostrate the existence of this disease in our country.     

Erectile insufficiency as first symptom of HTLV I/II associated myelopathy. Case report

People infected with Trypanosoma cruzi remain so for life, yet only 30-40% of these individuals develop characteristic chagasic cardiomyopathies. Similarly, when infected with the Brazilian strain of T. cruzi, DBA/2 mice develop severe cardiac damage while B10.D2 mice do not. To better understand the immunological parameters that may be involved in the disease process, we have used this murine model (DBA/2 vs B10.D2) and compared the changes in cytokine production during the course of infection with T cruzi. Concanavalin A (Con A) stimulation of spleen cells harvested during the acute phase (day 30) resulted in similarly high levels of IFN-gamma in both mouse strains. However, the amount of IFN-gamma in supernatants from cultures of B10.D2 spleen cells initiated during the chronic phase (day 72) was at subacute levels, whereas secretion by chronic DBA/2 spleen cells remained high. In addition, Con A-stimulated spleen cells from acute DBA/2 mice produced approximately twice as much IL-10 and significantly more IL-4 than cells from B10.D2 mice. IL-4 secretion remained low by cells from chronic B10.D2 mice, but when using cells from chronic DBA/2 mice, levels continued to increase beyond the already high levels secreted by cells harvested during the acute phase. Proliferative responses to Con A stimulation by spleen cells from DBA/2 mice were significantly higher than those from B10.D2 mice in both the acute and chronic phases. These data suggest that enhanced responses in DBA/2 mice, which may be related to a higher parasite burden, a lack of down-regulation, and/or the onset of autoimmune phenomena, correlate with the more severe cardiomyopathy seen in pathopermissive mice.