Intrathecal synthesis of virus-specific oligoclonal antibodies in patients with enterovirus infection of the central nervous system.

Cerebrospinal fluid (CSF) and serum samples from six patients with enterovirus infections were investigated by isoelectric focusing (IEF) and affinity-mediated immunoblot AMI) for the clonal distribution of entervirus-specific antibodies. In two patients with either acute meningitis or encephalitis and in one patient with a relapse of multiple sclerosis, oligoclonal IgG bands specific for enteroviruses were found predominantly in the CSF, revealing intrathecal synthesis of these antibodies. In three other patients with neurological symptoms probably unrelated to a current enterovirus infection, IEF and AMI disclosed nearly identical patterns of coxsackievirus-B-specific oligoclonal bands in the CSF and serum, indicating diffusion of these antibodies from the serum into the CSF. Although the number of patients in this study is small, the results suggest that intrathecally synthesized enterovirus-specific antibodies may be used as a means of identifying an enterovirus infection of the CNS.

Double-blind comparison of diclofensine with nomifensine in outpatients with dysphoric mood.

Depressed outpatients (n = 107, age 26-75 years) were treated with either a 50 mg single morning dose of diclofensine (n = 54) or 75-100 mg nomifensine given in two divided doses (n = 53) over a period of three weeks. The baseline mean values of the Depression Status Inventory (DSI index) of Zung corresponded to those of a mildly depressed population, as given by Zung. At the end of the treatment the mean DSI and Anxiety Status Inventory (ASI-index) values of both groups dropped to the levels of a normal population. The side-effect profile of the two treatments was similar. There were no side-effects indicating sedation. Adverse effects of the anticholinergic type were rare. It can be concluded that both diclofensine and nomifensine are beneficial for the treatment of depressed outpatients and that in a dose relation of 2:3 (diclofensine:nomifensine) they lead to a similar improvement in depressive outpatients.

Clinical and experimental investigations of vasopressin secretion in acute porphyrias.

In 41 patients suffering from acute hepatic porphyrias the arginin-vasopressin (AVP) levels in their urine were measured by RIA. In 6 patients, AVP secretion was normal; in 8 cases AVP levels were significantly elevated, while 27 cases showed decreased levels of AVP (p less than 0.001). A linear correlation between AVP secretion and urine volume was not found. In animal experiments, 20 rats were treated with delta-aminolevulinic acid (ALA), (1.5 mmol/kg/24 h and 1.5 mmol/kg/48 h) for 4 weeks. Afterwards vasopressin production in the hypothalamo-hypophyseal system was analysed by the immunoperoxidase technique and a microdensitometric method. In ALA-treated animals, AVP positive neurones showed coarse-grained granules of different intensity and a distinct increase of peroxidase positive granules in the zona interna of the eminentia mediana. Furthermore, in comparison with the control group in ALA-treated animals the mean diameter of nuclei in AVP positive neurons was greater. While animals treated daily showed an increase of transmission in the pituitary, microdensitometric findings in animals treated at 48 hourly intervals showed an equal transmission in AVP producing nuclei compared to the control group. Our results seem to point to a toxic effect of porphyrin precursors on the CNS, which may also induce via hypothalamus lesion either diabetes insipidus or a SIADH-syndrome.

[Cardiovascular disorders in acute intermittent porphyria (AIP) (author's transl)]. / Kardiovaskuläre Störungen bei der akuten intermittierenden Porphyrie (AIP).

The acute porphyric crisis is a characteristic clinical feature common to all hereditary hepatic porphyrias (acute intermittent porphyria [AIP], porphyria variegata and hereditary coproporphyria). The crisis is marked by an acute disorder of the central, peripheral and autonomic nervous system. Autonomic disorders may play a major part and may provoke severe cardiovascular symptoms. According to the literature our findings support data describing supraventricular tachycardia as the most important sign, followed by hypertension--or, rarely, hypotension--cardioarrhythmias and cardiomyopathy. While tachycardia, blood pressure disturbances and cardioarrhythmia indicate sympathetic overactivity, cardiomyopathic alterations suggest functional or structural coronary dysfunction. The existence of a specific "angiopathia porphyrica"--based on functional, angiospastic or secondary hypertensive disorders--has been discussed for a long time. Recent results concerning a 20-year follow-up study of AIP patients revealed chronic hypertension as being the most significant disorder and seem to support a hypertensive aetiology.

[Psychotic disturbance and epilepsy as principal signs of arteriovenous angioma of the brain--described on case history basis (author's transl)]. / Psychotische Störung und Epilepsie als Leitsymptom arteriovenöser Angiome des Gehirns--dargestellt anhand eines kasuistischen Beitrages.

The article describes the case of a 36-year old female patient with left temporal arteriovenous angioma suffering from psychomotoric epilepsy followed five years later by a symptomatic psychosis (paraphrenia). Basing on the case history of this patient, the phenomenological, neurophysiologico-biochemical and cerebrolocalisatory common features of psychotic disturbance and psychomotoric epilepsy are discussed; the etiologically underlying lesion of the limbic system is described. As far as clinical practice is concerned, the author raises the demand that, to say the least, orientating neurological diagnosis should be included at any early stage into differential diagnostic considerations.

[The idiopathic paroxysmal myoglobinuric myopathy (Meyer-Betz) (author's transl)]. / Die idiopathische paroxysmale myoglobinurische Myopathie (Meyer Betz).

The idiopathic paroxysmal myoglobinuric myopathy (IPMM) as a genuine disease can be differentiated from other myoglobinurias by clinical criterias. Concerning the course of the disease two different types of IPMM are to be seen, sporadic cases are observed as well as familiar, autosomal recessive inherited ones. Regarding the pathogeny of IPMM several metabolic disorders are discussed: Provocation of the disease by physical exertion and glucose deficiency point to a disturbance of carbohydrate metabolism. Disorders of lipid metabolism are reported, too. So we tried to analyse the results of a case study in relation to the presently discussed - still hypothetical - pathogenetic ideas. Our laboratory, histological and electromyographic findings suggest a primary myopathy, which might be based on a peripheral glucose utilisation disorder (ATP-deficiency). Under the condition of exertion this disorder leads to a decompensation of the muscular functional and structural metabolism manifested by an excessive efflux of myoglobin and enzymes.

[Diagnosis of acute intermittent porphyria. Results of neurological, biochemical and genetic studies]. / Zur Diagnostik der akuten intermittierenden Porphyrie: Ergebnisse neurologischer, biochemischer und genetischer Untersuchungen.

Neurological and biochemical studies have been performed on four AIP families with 21 members. Five patients suffered from manifested AIP (Uroporphyrinogen Synthase defect and characteristic urine findings); among their relatives five persons with latent AIP were detected and eight carriers of the genetic-enzymic defect (Uroporphyrinogen Synthase defect). Internal and neurological symptoms could be interpreted as a panneuropathy. Acute and chronical polyneuropathies could be observed as well as myelopathies and cerebral co-reactions. A frequent symptom dominating the crisis and the latent state of AIP were etiologically abscure 'myalgias.' The character of the course of AIP is various and dubious: beyond the 'classical' courses with its intermittent porphyric crises we observed one case which was characterized by a permanent crisis and a second case marked by a chronical, slow progredient course without any porphyric attacks.

[Schizophrenia and neuro-endocrine functional disorders--a case study on the problem of paranoid psychosis]. / Schizophrenie und neuro-endokrine Funktionsstörung- eine Fallstudie zum Problem der paranoiden Psychose

Schizophrenia and neuro-endocrine functional defects - a case report concerning the problem of paranoid psychosis: The case of a female patient with paranoic psychosis and endocrine defects has been reported. Under the present pathophysiological concepts the possibility of a combination of schizophrenic and neuro-endocrine symptomatic is suggested. The results of more recent investigations are discussed in respect of organic origin of schizophrenia that is based on potential reduction in the region of the limbic-hypothalamic nervous system.

[Quantitative determination of glia-specific proteins in the cerebrospinal fluid of patients with MS (author's transl)]. / Nachweis Glia-spezifischen Proteins im Liquor bei multipler Sklerose

150 cerebrospinal fluids from MS patients (85 cases) and patients with different neurological diseases (65 cases) were investigated for their glia-specific content. The demonstration was made quantitatively by means of modified passive hemagglutination tests. The brain-specific glycoprotein was examined for its possible endogenous antigen and/or antibody properties in the cerebrospinal fluid (csf). It could only be demonstrated in the CSF as antigen. CSF with a quantitatively detectable glia-specific protein content- recognizable by a significant increase in titer - were set in relation to other CSF parameters such as cell count, total protein and globulin ratio, and investigated for possible relationships to the clinical syndromes mentioned and their development. A firm correlation was found between the glia-specific protein content and the total protein content of the CSF with retained equivalence.