All living creatures are subject to aging, but our understanding of what governs aging is limited. In the course of a lifetime, with the constant renewal of the organic substance of living creatures errors arise, e.g. in the formation, disposal, and reproduction of DNA, proteins and lipids or in the constant substitution of aging cells in the organs. These errors are recognized and generally counterbalanced by appropriate repair mechanisms. This process is obviously determined partly by environmental influences (e.g. UV radiation, oxidizing influences, thermal shock) and genetic factors (such as the significance of so-called survival genes and gene mutations). In this paper the authors both explain and test the hypothesis that the aging of organs and organisms is the consequence of and not the reason for a progressive weakening of the repair mechanisms throughout life.

Toxocariasis is a worldwide-occurring parasitic infection leading to tissue damage in various organs due to wandering Toxocara larvae (visceral larva migrans). More than 40 cases of CNS involvement in children and immunocompetent adults have been documented in detail to date. Here, we present evidence of eosinophilic meningomyelitis in an adult without known risk factors and with positive Toxocara antibody response in CSF, but not in blood. Toxocariasis has to remain among the differential diagnosis in patients with eosinophilic CNS infection even if serological tests in blood are negative. Adult cases seem to be more frequent than previously thought (about 60%).

Both Marfan's syndrome and neurofibromatosis type 1 are hereditary, autosomal dominant conditions. Here, we report the rare case of a patient fulfilling the clinical criteria for both diseases. In the absence of a family history of either of the two conditions, two independent de novo mutations are the most likely cause.

Patients with Hashimoto’s encephalopathy (HE), a steroid-responsive disorder, associated with Hashimoto’s disease and high levels of thyroid-related autoantibodies usually present with a subacute onset of confusion, focal or generalized seizures. Frequent EEG abnormalities include generalized, rhythmic bifrontal or temporal slowing. Elevated protein levels or an intrathecal IgG synthesis may be present in cerebrospinal fluid (CSF). A 39-year-old woman underwent a relapsing course of myocloni and generalized seizures. Initially, thyroid function, thyroid-related autoantibody screening and cerebral MRI were unrevealing. CSF showed oligoclonal bands. Short-term treatment with high doses of prednisolone resolved the myocloni. During the 5th episode of myocloni, signs of hyperthyroidism and elevation of thyroid microsomal antibody titer developed. Hashimoto’s thyroiditis and HE were diagnosed. After subtotal thyroidectomy the patient remained asymptomatic.

OBJECTIVES:Anticipation has been linked to unstable trinucleotide repeats in many neurological disorders. We examined the hypothesis of genetic anticipation in familial cavernous angioma (FCA) of the central nervous system.MATERIAL AND METHODS:The mean ASO of affected individuals was compared between successive generations in 55 families. Intergenerational pair-wise comparisons were employed to avoid several ascertainment biases. Regarding severity of disease both type of manifestation and number of cavernous angiomas were compared between generations.RESULTS:The mean ASO decreased significantly both from the first to the second generation (31.6 vs 17.8 years; P = 0.000) and from the second to the third generation (17.8 vs 6.7 years; P = 0.002). The pair-wise comparisons also showed significantly earlier ASO. No clear evidence for anticipation with regard to severity of disease was found.CONCLUSIONS:Molecular genetic studies will determine whether trinucleotide repeats are the underlying mechanism for our observation of anticipation in FCA.

Conventional vestibular examination tests the function of the pair of horizontal semicircular canals and ignores the two pairs of vertical semicircular canals and otoliths. For complete vestibular testing, a four-axis gimbal system was constructed with two motor-driven axes and two axes which are manually adjustable. This apparatus allows stimulation of all semicircular canals and also-by rotation with respect to the gravity vector of the otoliths. Positioning the subject in a light-proof sphere prevents visual orientation in space. Projected visual stimuli (light spot and full-field random dot pattern) complete the possibilities for oculomotor testing. Largely avoiding the use of metal offers the possibility of three-dimensional eye movement recording with the search coil method.

Nach einer Schwellkörperautoinjektion von Papaverin und Phentolamin zur Behandlung einer erektilen Dysfunktion kam es bei dem hier vorgestellten 48jährigen Patienten zu einer prolongierten Erektion. Die Behandlung dieser Komplikation mit intrakavernöser Applikation des potenten Alphamimetikums Metaraminol führte zu einem krisenhaften Blutdruckanstieg und in dessen Folge zu einer Tachyarrhythmia absoluta und einer intrazerebralen Massenblutung. Der Fall demonstriert, daß die Behandlung der prolongierten Erektion als Folge der Schwellkörperautoinjektionstherapie komplikationsträchtig sein kann.

The tremorlytic activity of the novel antiparkinson agent budipine was quantified in an open trial. Eleven patients with Parkinson's disease (PD) were treated with individual doses of budipine added to stable conventional antiparkinsonian medication. Tremor activity was measured using long-term electromyogram (EMG) recordings. Tremor intensity was reduced by 25%, tremor occurrence by 34%, and conventional “Unified Parkinson's Disease Rating Scale” (UPDRS) scores improved by 20% with this medication. There were two dropouts because of side effects. One dropout appeared not to be related to budipine. Apart from those, the drug was well tolerated by all patients. We conclude that budipine is an effective and well-tolerated tremorlytic drug and that the method of long-term EMG recording is suitable for tremor quantification in clinical studies.

The tremorlytic activity of the novel antiparkinson agent budipine was quantified in an open trial. Eleven patients with Parkinson's disease (PD) were treated with individual doses of budipine added to stable conventional antiparkinsonian medication. Tremor activity was measured using long-term electromyogram (EMG) recordings. Tremor intensity was reduced by 25%, tremor occurrence by 34%, and conventional "Unified Parkinson's Disease Rating Scale" (UPDRS) scores improved by 20% with this medication. There were two dropouts because of side effects. One dropout appeared not to be related to budipine. Apart from those, the drug was well tolerated by all patients. We conclude that budipine is an effective and well-tolerated tremorlytic drug and that the method of long-term EMG recording is suitable for tremor quantification in clinical studies.

We have recently developed a method of long-term EMG recording (Bacher et al., 1989), which has proven suitable for the quantification of pathological tremor. In the present paper we show that (1) the principal parameters of the method (tremor occurrence, tremor intensity, tremor frequency) are highly reproducible with intraclass correlation coefficients in the order of 0.9, (2) that the method has both a high specificity (87.5%) and sensitivity (96%) for detection of pathological tremor, and (3) that it can be useful for examining agonist-antagonist muscle activation under everyday conditions.

Matters arisingshift from ocular to generalised myasthenia gravis was a more common feature of anti-AChR antibodies-positive myasthenia gravis (14 of 28 patients, 50%) than that of seronegative myasthenia gravis (one of five patients, 20%).Subsequent to a follow-up period of more than one year, the group of anti-AChR antibodies-negative patients consisted of four (3%) ocular cases and 10 (7%) generalised cases.Consequently, I am unable to confirm Toyka's observation of only 45% anti- AChR antibodies-positive cases in long- standing ocular myasthenia gravis.Toyka also suggests that cases with questionable myasthenia gravis may have been included in the analysis of generalised myasthenia gravis' resulting in lower estimates of the sensitivity relating to such cases.This is a very unlikely explanation in view of the scrutiny of all cases including clinical assessment by an experienced examiner, all of which is thoroughly expounded in my article' and also in my epidemiological study.

IgG and IgM concentrations in tears of multiple sclerosis patients (n = 38) are increased compared to normal controls (n = 23). The occurrence of oligoclonal tear IgG bands (7.9%) - as determined by immunoblotting - did not differ between groups. Our findings suggest an altered reactivity of the secretory immune system in MS patients, but did not differ from findings in patients with eye affections or wearing hard contact lenses.