Abstract: Myocardial 123I-Meta-iodobenzylguanidine uptake was markedly reduced in a patient with postural tachycardia syndrome (POTS). This finding suggests that loss of sympathetic autonomic neurons in the heart may play a role in the etiology of POTS.
Abstract: Symptoms related to alterations of the autonomic nervous system are frequent in patients with multiple sclerosis (MS). Bladder or bowel dysfunction or impairment of sexual performance is highly distressing for most MS patients,whereas the clinical relevance of other autonomic symptoms is less clear. Cardiovascular autonomic alterations might relate to clinical signs such as orthostatic intolerance; a relationship with fatigue is uncertain. However, the frequency of abnormal findings in tests for the cardiovascular autonomic system varies due to the lack of standardized test performance or differentially used cut-off values. The incidence of additional symptoms such as pupillomotor or sweating alterations and especially their relationship to overall autonomic nervous system abnormalities is not well known. Although their impact on daily life is low, they can at least serve as diagnostic tools. Beside these clinical aspects, alterations of the autonomic nervous system have also been reported to alter immunological cascades in experimental conditions. However, corresponding results have not been confirmed in clinical trials yet.
Abstract: Multiple sclerosis (MS) is the most frequent chronic neurological disease affecting young persons in developed countries. MS is, however, considered as a secondary cause, of central origin, for autonomic dysfunction. The most common autonomic symptoms in MS are disorders of micturation, impotence, sudomotor and gastrointestinal disturbances, orthostatic intolerance as well as sleep disorders. The majority of the patients suffer at some period of the disease from lower urinary tract symptoms and sexual dysfunction. Awareness and treatment of these conditions is vital to improving health and quality of life in patients with MS. The increased understanding of the pathophysiological mechanisms in autonomic dysfunction in MS, along with technological and pharmaceutical developments has advanced our ability to treat the multiple aspects complicating autonomic failure in MS.
Abstract: Extrasystoles occur in normal subjects but are significant more frequently (16.25% vs. 55%; chi(2) = 19.3; P < 0.001) seen in Parkinson's disease (PD) patients. The extrasystolic decreases in stroke volume and systolic pressure activate sympathetic vasomotor innervation and lead to a blood pressure increase for a few heartbeats. The purpose of this study was to prove whether the short time analysis of this blood pressure regulation allows the assessment of sympathetic neurocirculatory function. Records of noninvasive blood pressure monitoring were reviewed from 40 PD patients and 80 controls. A battery of cardiovascular autonomic tests, including Valsalva maneuver, tilt-table testing, echocardiography, and cardiac scintigraphy with [(123)I]meta-iodobenzylguanidine were performed. Fifty-five percent of the PD patients had at least one premature ventricular contraction (PVC) in 10 minutes lying supine at rest. After every PVC (13 PVCs) recorded from normal subjects, we found an increase in systolic blood pressure above base line with a maximum at the seventh heart beat. In all of the 22 PD patients, the systolic blood pressure was significantly decreased less than baseline in every PVC from the second to the ninth postextrasystolic beat (P < 0.001). In both groups, the extrasystolic fall in blood pressure was on average approximately 22%. The postextrasystolic potentiation did not differ (5.3% vs. 4.4%, not significant). If a PVC occurs, the analysis of short-time blood pressure regulation is a sensitive tool for baroreceptor reflex function. The advantage of this method results from the independence of patients cooperation and the high sensitivity to prove a sympathetic neurocirculatory failure within 10 heart beats.
Abstract: PURPOSE: Valproic acid (VPA) is an antiepileptic drug (AED) commonly used for generalized and focal epilepsies. We provide an update on hepatotoxic side effects in Germany between 1994 and 2003. METHODS: We mailed a questionnaire to all members of the German Section of the International League Against Epilepsy, asking for VPA-induced side effects, especially severe side effects such as hepatopathy. RESULTS: As a result of our questionnaire, we found 31 cases of reversible hepatotoxicity and nine cases of lethal hepatopathies in Germany from 1994 to 2003. CONCLUSIONS: The outcome of patients with severe hepatotoxicity is better than that in the past. The risk of a VPA-induced hepatopathy is not limited to patients younger than 2 years, receiving polytherapy, or patients with congenital or acquired metabolic diseases.
Abstract: Valproic acid (VPA) is a broad-spectrum antiepileptic drug and is usually well-tolerated. Rare serious complications may occur in some patients, including haemorrhagic pancreatitis, bone marrow suppression, VPA-induced hepatotoxicity and VPA-induced encephalopathy. The typical signs of VPA-induced encephalopathy are impaired consciousness, sometimes marked EEG background slowing, increased seizure frequency, with or without hyperammonemia. There is still no proof of causative effect of VPA in patients with encephalopathy, but only of an association with an assumed causal relation. We report 19 patients with VPA-associated encephalopathy in Germany from the years 1994 to 2003, none of whom had been published previously.
Abstract: We present a questionnaire-based survey on methods adopted in European autonomic nervous system (ANS) laboratories in the year 2002. Seventy-nine laboratories from 16 countries in Western and Eastern Europe provided information on test methods, equipment, staff, educational programme and research activities. The results confirmed the anticipated heterogeneity of techniques used to investigate ANS disorders. However, cardiovascular (84.8%) and sudomotor (43.0%) tests were the most common. There were no standards regarding equipment, but devices used varied widely, in part commercially available, in part self-developed. Some ANS laboratories performed cardiovascular tests using standard EMG devices with implemented routines to measure heart rate variability. Many investigators used published normative data (75.4%). Most of the responders were involved in scientific work, half were active in education of trainees. A wide variation was found in number and profession of staff members. As more than 60% of questionnaires were returned by neurologists our results may be limited to neurological ANS laboratories. Given the wide variation of methods and equipment used in European ANS laboratories, minimal standards for routine ANS testing are desirable. Standards may help to compare results of diagnostic tests, evaluate equipment and test laboratory methods and commercial devices and set-up educational programmes.
Abstract: The relationship of disturbed autonomic skin innervation to altered transdermal absorption in polyneuropathy is not yet clear. In this study we measured serum estradiol in 29 normals and 10 polyneuropathy patients to assess potential differences of estradiol uptake from different skin sites.
Abstract: A severe course of alcohol withdrawal has been observed in 28% of patients in a neurological intensive care unit due to complicating central nerve system (CNS) diseases. In any atypical alcoholic delirium, especially with focal neurological signs, partial seizures, or decreased level of consciousness, CNS diseases like meningoencephalitis, intracranial hemorrhage, or central pontine myelinolysis must be diagnosed by computed tomography (CT) scan and cerebral spinal fluid (CSF) tap. The diagnostic and prognostic value of CT scan and CSF analysis was examined in 32 persons with alcohol withdrawal syndrome or delirium tremens. Neurological complications and cerebral convulsions at the beginning of delirium tremens appear to predispose the patient to a protracted clinical course and necessary mechanical ventilation. Blood-CSF barrier permeability is increased in 70% of alcohol withdrawal patients and that also seems to be a marker of a prolonged clinical course. Cerebral atrophy as shown in CT scan does not play a role in predicting clinical course. In our experience, CT examination or lumbar puncture is not necessarily recommended if clinical signs are typical for alcohol delirium.
Abstract: Severe hyponatraemia may be cause unconsciousness, vomiting, seizures or exogenous psychosis and is associated with a high mortality. We report on a 44-year-old woman who presented with somnolence and psychomotor unrest. After rousing stimuli she showed no verbal response and did not follow any instructions. For three days she suffered from nausea and vomiting. Laboratory values included a natrium serum level of 97 mmol/l. CT scan demonstrated no abnormal findings. Because of severe arterial hypertonia she received for 12 days intensive diuretic therapy with 50 mg hydrochlorothiazide and 100 mg triamterene. Retrospectively, we proved that as a result of saluretic therapy, chronic hyponatremia had already existed before admission. Serum sodium was corrected slowly (< 12 mmol/l) with fluid restriction and normal saline solution. This is considered to be the first case report of a complete restitution after hyponatremia less than 100 mmol/l. We suggest that the preexisting chronic hyponatremia and the slow correction of serum sodium level are responsible for the favorable outcome of this case of severe hyponatremia.
