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Unter Dystonie versteht man langsame, unwillkurliche Kontraktionen vorwie gend der proximalen Muskulatur, die oft zu rotierenden Bewegungen und bizarren Gelenkstellungen sowie Korperpositionen fuhren. Die Dystonien werden nach Marsden (1982) unterteilt in: 1. fokale Dystonien, falls nur eine einzelne Region betroffen ist (z. B. Nacken oder Gesicht), 2. segmentale Dystonien, wenn ein Segment bilateral (z. B. beide Arme) oder wenn zwei oder drei benachbarte Korperabschnitte betroffen sind (z. B. Gesicht und Nacken) und 3. generalisierte Dystonien, falls ein Arm und Bein der jeweils anderen Seite oder mehr als drei Segmente betroffen sind (Fahn 1983). Aufgrund der symptomatischen Dystonien beim M. Wilson und Befunden bei perinatalen Schadigungen mit Dystonie wird eine Lasion der Basalganglien angenommen.

135 out of 168 patients with Hodgkin's disease in complete remission who had received radiotherapy with tumor doses between 3000 and 5000 rad in the years 1948–1974 were investigated with regard to long-term side effects. The results obtained so far are as follows: 1.1. Catamnestic results: Deteriorated general condition and reduced performance in 23% and 28%, spontaneous restriction of the daily routine in 45% and increase in susceptibility to infections (common cold) in 15% of the patients 2.2. Hematological investigations, (a) Peripheral blood: No indication of severe anemia. Granulocytopenia with less than 3000 cells/mm 3 in 18.5%, lymphocytopenia with less than 2000 cells/mm 3 in 37%, no incidence of thrombocytopenia with less than 100,000 platelets/mm 3 or leukemia. (b) Sternal marrow cytology (locally irradiated): Aplasia in 76%, hypoplasia in 18%. No signs of leukemia. (c) Iliac crest cytology (not directly irradiated): Slight hypoplasia in 38%, severe hypoplasia in 8%, no signs of leukemia. 3.3. Pulmonary investigations: Slight paramediastinal fibrosis in 44%, distinct in 36% and severe in 9%. Restrictive ventilation disorder in 84%, increase in residual volume in 61%, O 2 -diffusion disorder in 18% and obstructive ventilation disorder in 7% of the patients. 4.4. Cardiological investigations: Under stress pulmonary hypertension in 18%, right ventricular function disorder in 9%, left ventricular function disorder in 9%, cardiac insufficiency in 2%, restriction of the maximum ergometer cycling exercise capacity in 53% of the patients. 5.5. Thyroid function investigations: Hypothyroidal or latent hypothyroidal thyroid insufficiency in 16.5%. 6.6. Neurological symptomatology (excluding herpes zoster): Slight to moderately severe double-sided cross section symptomatology in 3 of 122 patients (2.5%), bilateral or unilateral plexus paresis in 4 of 122 patients (3.5%). 7.7. Genito-urinary tract (male) investigations: Infertility (azoospermia, asemia, akinesis) following infradiaphragmatic irradiation using the inverted Y-method in 70%, restricted fertility (oligo-, astheno-, teratospermia) in 26% of the cases (in spite of the constant usage of a testicular capsule). 8.8. Dermatological investigations: According to the number of patients, teleangiectasis, induration of the irradiated fields, and medium to severe irradiation discomfort occured in 22, 15.6 and 8% respectively.

A new rating scheme (score) is presented for ocular symptomatology in myasthenia gravis. The score (0-10) combines separate ratings upper eyelid weakness (ptosis) and paralysis of the outer ocular muscles (double vision). It is relatively insensitive to subjective influences and to the rater's experience. It yields replicable values which are especially important for longitudinal studies, for testing new therapeutic strategies and for their statistical validation.

During the flight of Spacelab 1 the crew performed a number of experiments to explore changes in vestibular function and visual-vestibular interactions on exposure to microgravity. Measurements were made on the threshold for detection of linear oscillation, vestibulo-ocular reflexes elicited by angular and linear movements, oculomotor and posture responses to optokinetic stimulations, and responses to caloric stimulation. Tests were also conducted on the ground, during the 4 months before and on days 1 to 6 after flight. The most significant result was that caloric mystagmus of the same direction as on the earth could also be evoked in the weightless environment.

The results of a systematic neurological and electro-encephalographical follow-up study in 344 head injured adults and 116 children were compared. The results show that posttraumatic EEG abnormalities lasting more than 24 hours, are equally frequent in children (53%) and in adults (49%). Mostly short lasting neurological signs of focal tissue damage are however significantly more frequent in children (30% vs. 13% and EEG-abnormalities (foci and general slowing) are on the average more pronounced. The recovery of EEG abnormalities follows the same pattern as in adults, occurs however faster among children. In both groups the normalization of general slowing precedes that of focal abnormalities only in 25% of the cases. Amnesias on the average last shorter in infants. Their duration seems of lesser significance for the judgement of the severity of the trauma in children than in adults. 6 months after the trauma children complain less frequently about headache, dizziness, or impaired memory and diminished ability to concentrate; emotional lability and impairement of drive, however, are more frequent than in adults. The results indicate an equal diagnostic and prognostic importance of systematic EEG studies (within 2 days, after 1 week and 6 month after the trauma) in both groups.

Doppler sonography of the extracranial arteries was done in 556 unselected patients (greater than 40 years) in a prophylactic study. 226 high risk patients with arterial occlusive leg disease (AOD) served as control group. Doppler sonography revealed stenoses or occlusions of the carotid, vertebral and (or) subclavian arteries in 9.17% of normal persons and in 38.05% of AOD patients. Medical treatment is available only for stenoses (not occlusions) of the carotid arteries. Thus the frequency of asymptomatic internal carotid stenoses--only stenoses of more than 50% lumen diminution can be detected--of 2.33% in the group of normal probands and of 17.2% in the patient group is the reference value and justification for use of Doppler sonography for prophylactic assessment. The atraumatic and safe method with a success rate of 2.33% is suitable particularly in increased risk factors (age, males, smoking, hypertension, diabetes). As a prophylactic investigation in patients with arterial occlusive disease Doppler sonography is mandatory before prospective operations.

Tetrabenazine (50-150 mg/day) was a moderate to excellent efficacy in each of six patients with severe spontaneous oral dyskinesia. Slight akinesia, rigidity, and transient disorientation were occasional side effects that could easily be controlled and were tolerated by the patient in view of the lasting amelioration of this debilitating symptom. When tetrabenazine was administered for blepharospasm, only two of six cases showed partial improvement with more severe side effects.

Physicians experienced in the treatment of migraine and other forms of headache are nowadays well aware that the daily intake of antipyretic or anti-inflammatory analgesics or combinations with ergotamine, sedatives, or hypnotics may result in chronic daily headache. Conversely, if a patient complains of daily headache and takes “pain killers” daily, this headache is most likely to be caused and sustained by the medication and will vanish with abstinence. Chronic headache was originally attributed to the excessive use of ergotamine preparations (Lippman 1955; Horton and Peters 1963; Lucas and Falkowski 1973; Rowsell et al. 1973; Wainscott et al. 1974; Andersson 1975; Dige-Petersen et al. 1977; Hokkanen et al. 1978; Ala-Hurula et al. 1982; Pradalier et al. 1984) and was therefore named “ergotamine headache.” A number of subsequent reports from different countries in Western Europe and the United States, however, indicated that analgesics may induce chronic headache as well (Worz et al. 1975; Medina and Diamond 1977; Tfelt-Hansen and Krabbe 1981; Kudrow 1982; Isler 1982; Worz 1983; Dichgans et al. 1984; Henry et al. 1985; Rapaport et al. 1985). We, therefore, propose to add to the original terms “ergotamine headache” and “analgesic headache” a new entity and to include both under the term “drug-induced headache.”

The correlation between the severity of symptoms in myasthenia gravis (score) and the level of acetylcholine receptor-antibodies (ACHR-Ab) was studied in 24 patients treated with immunosuppressive therapy. This correlation was significant in every case regardless of whether or not thymectomy was performed in addition. It was demonstrated by means of a long-term follow up study, that a change in ACHR-Ab preceeds the corresponding change in score by 2-4 months in 80% of our patients. Measurements of ACHR-Ab ease the management of the therapy with immunosuppressants and make it safer. This is particularly true prior to and after termination of the treatment. A change in ACHR-Ab level by more than 20% indicates a change in score.

The effect of refractive error and luminance on circularvection, the illusory sensation of self-motion resulting from rotation of the visual field, was determined. Neither reduction of luminance to levels near absolute scotopic threshold nor induced refractive errors of more than 16 diopters abolished circularvection or influenced any of its latency measures. The results are discussed in terms of the role of the peripheral visual field and the nature of the visual stimulus in ego orientation.

In humans interaction of a prior vestibular stimulus (chair acceleration for 10 s) with an immediately following optokinetic stimulus (full field stimulation) was quantitatively studied to determine the change of slow phase velocity (SPV) of nystagmus elicited by the addition of the optokinetic stimulus. This change was compared to the SPV during pure optokinetic stimulation on the basis of the magnitude of the optokinetic input (retinal image motion) at the onset of the optokinetic stimulus. When measured shortly (0.7s) after optokinetic stimulus onset visual vestibular interaction is in fact linear i.e. the optokinetic reflexes are as effective in changing SPV during vestibular nystagmus as with pure optokinetic stimulation. As the optokinetic stimulus affects the charge in the visual vestibular integrator interaction cannot be expected to be linear after longer intervals of optokinetic stimulation.

Fifty-two patients, most of whom had had daily headaches for years, were examined and treated. Among them there were 40 who originally had migraine, the others had vasomotor or post-contusional headaches. Average duration of the migraine was 21 years, of chronic headache 7.6 years. All patients had been taking analgesics of a mixed type regularly and for a long time, on average 35.6 tablets or suppositories weekly. All patients had taken more than three different drugs. After an observation period of 3-6 months for grading the headaches and registering the amount of drug intake, all patients were admitted to hospital when all analgesics were at once discontinued. Changing degrees of withdrawal symptoms were the rule: increased headaches, nausea, vomiting, tachycardia, sweating, sleep disorders, and in some also hallucinations and cerebral episodes. At the end of the hospital stay chronic headache had completely disappeared or markedly improved in 77% of patients. Even after an average of 16 months of subsequent observation, chronic headache continued to be significantly improved in 70% of patients. There was a significant reduction in frequency and intensity of attacks in the patients with originally typical migraine. Regular intake of analgesics of the mixed type induces chronic headaches. These are most commonly caused by ergotamine tartrate and aminophenol derivatives, while psychological and physical dependence on anti-migraine drugs is initiated and maintained by additional barbiturates, caffeine and codeine.

This study reports the case of a 19-year-old Turkish woman who was suffering from a cerebral abscess and granuloma formation caused by Candida albicans. The diagnosis of her illness was established by cytologic analysis of the cerebrospinal fluid. Combined application of amphotericin B and 5-fluorocytosine cured the patient's disorder. Its remission was documented over a period of 2 years by the use of computed tomography.

Posturography is a very useful clinical instrument to quantify, analyze, and document postural sway and to differentiate patterns of instability that are typical for the different types of lesions encountered within the cerebellum and to differentiate cerebellar ataxia from spinal ataxia. An objective method of measurement like this is most valuable for follow-up studies and the surveillance of therapeutic efficacy. The method of recording short, medium, and long latency reflexes in leg muscles after a sudden tilt of a movable platform toe-up is not only totally atraumatic and fast, but more importantly it gives significant hints towards the possible site of a central lesion in the sensory-motor system.

Postural stabilization in altered visual and vestibular conditions was investigated in humans subjected to fast transient disturbances and during sinusoidal movement of the standing support. Visual inputs were varied by applying stroboscopic illumination, stabilizing the visual surround in respect to head movements, inducing apparent body movement in pitch by continuously moving stripe patterns up or down and by eye closure. Static vestibular input was modified by bending the head forwards or backwards, or to the right or left shoulder (eyes closed). Neither biomechanical parameters of standing nor EMG responses of the anterior tibial and triceps surae muscles were modified by the different visual and vestibular conditions during fast transient (80 degrees/s) platform movements 4 degrees toe-up. Continuous regulation of upright stance during sinusoidal movements (1 Hz, 0.3 Hz), however, clearly depended on the different modifications of visual and vestibular inputs. Fast transient disturbances are easily compensated in a reflex-like manner independent of visual and vestibular feedback. Continuous regulation of upright posture during slow disturbances, however, clearly depends on the evaluation of afferent information from the visual, vestibular, and proprioceptive systems.

Bei 35 nicht verwandten Patienten mit okularer Myasthenia gravis, bei denen sich auch nach zwei Jahren keine Anzeichen einer Generalisation fanden und eine medikamentose Induktion ausgeschlossen war, wurden HLA-Antigene und die Titer der Antikorper gegen Acetylcholin-Rezeptoren (6) untersucht. Die Ergebnisse wurden mit den Befunden bei 47 Patienten mit einer generalisierten Myasthenia gravis und mit der Verteilung der HLA-Marker der Normalbevolkerung (1) verglichen.

Correlation studies on patients with myasthenia gravis are reported in which clinical assessment of fatigue and neurophysiological findings are compared to blood levels of pyridostigmine. Measurements using a high-pressure liquid chromatography method (HPLC), give reproducible results. The levels of pyridostigmine in the serum or plasma of healthy controls and of patients show no essential differences. Components of coffee, tea, chocolate and cigarettes can markedly disturb the chromatography by adding additional peaks, so that interpretation becomes difficult or impossible. Blood levels can be measured approximately one hour after oral intake of 60 mg pyridostigmine. Concentrations rise for two to four hours and then decline exponentially. The half-life of pyridostigmine was between 156 and 210 minutes. Despite identical oral dosages, the concentration differed intraindividually and interindividually among patients. While the blood level does not reach its maximum value for 1-1 1/2 to 3 hours, the maximum clinical and neurophysiological effect of pyridostigmine appears 30-60 minutes after ingestion. Variable distribution of cholinesterase inhibitors over the different compartments (blood, synaptic region) is assumed to cause this temporal lag. If the total amount of pyridostigmine is divided into 4-5 doses, the concentration profiles over the course of a day are relatively stable. There is no significant correlation between the variations in blood level throughout one day, and changes in myasthenic symptomatology. Effects of pyridostigmine can be measured at levels as low as 5 ng/ml; at levels above 40 ng/ml further improvement can be detected only rarely. Blood levels were lower if corticosteroids were administered simultaneously; azathioprine had no influence on blood levels. Blood levels assays allow better differentiation of cholinergic and myasthenic crises and the identification of disturbed absorption and interactions with other medications.

The present study used recordings of visual potentials evoked by pattern reversal (VEPs) to investigate the central effects of three drugs used in migraine prophylaxis: the calcium channel blocker nifedipine, the beta-1-selective blocker metoprolol, and the nonselective beta adrenoreceptor blocker propranolol. The study involved 58 patients with common or classical migraine who were treated in a double-blind randomized study over a period of 7 months, while the effectiveness of prophylactic treatment was recorded in headache diaries that were subjected to time series analysis. VEPs were recorded at the beginning of a 2-month baseline period without treatment, after 4 months of treatment, and at the end of a 3-month washout period. At baseline, migraine patients had significantly higher VEP amplitudes and longer latencies than did a group of 87 healthy control subjects. Patients were separated by statistical analysis into responders and nonresponders to each prophylactic treatment. Nifedipine had no effects on the frequency, intensity, and duration of migraine attacks, nor on amplitude and latency of the VEPs. In contrast, the use of beta blockers resulted in a significant decrease in VEP amplitude, both in responders and nonresponders, whereas VEP latency remained unchanged. VEP amplitudes returned to the initial values at follow-up in the nonresponders, but stayed at lower levels in responders. Beta blockers thus appear to have a significant effect on the increased excitability of the visual system in patients with migraine, although their action is not directly related to their reduction of migraine frequency.

Publisher Summary In this chapter, single unit activity is recorded from the peripheral vestibular nerve of unrelaxed and unanaesthetized goldfish, and rabbits. Eye movements are also registered. Nearly 10 % of the neurones show a frequency modulation starting before the beginning of each spontaneous saccadic eye movement and rapid phase of optokinetic and vestibular nystagmus. Bidirectionally activated (type-a) and bidirectionally inhibited (type-i) neurones can be established. In the goldfish, a few direction-specific neurones with inhibition or activation depending upon the direction of horizontal saccadic eyemovements (type-d) are found. The presaccadic initiated phasic discharge-modulation is supposed to originate from supranuclear optomotor centers. In addition to these phasically modulated neurons, found in both the goldfish and rabbit, the goldfish shows neurones whose discharge-frequency depends upon eye position (type-p). These neurones may be influenced by proprioceptive afferents from the eye muscle. Mainly in the goldfish, most of the neurones are identified as being efferent ones. In the rabbit, afferent bidirectionally activated neurones of type-a are verified. The results demonstrate the existence of a vestibular control by oculomotor impulses mediated by efferent fibers to the vestibular receptor level in teleosts and lower mammals. The possible functional significance is discussed in this chapter.

The studies of Steinhausen [10] and electrophysiolog ical work on fishes [7] and frogs [5, 8, 9] supported the concept of the cupuI~ endolymph system as an overdamped torsion pendulum which responds to changes in angular velocities. Within a limited frequency range it integrates acceleration over time, thus measuring velocities of angular head movements. The system, however, is deficient as a speedometer since it lacks information about constant velocities. The lack of steady state discrimination results in the system's failure to discriminate between decelerati3n from constant velocity, and acceleration in the opposite direction from zero velocity. For motion control therefore, the vestibular system is deficient, and supplementary sensory information is required, The experiments described sngges~ that in the goldfish this supplementary information about constant velocity movement of the body is provided by the visual sense. Only the interaction of visual and vestibular information during combined stimulation of both sensory organs results in accurate velocity information. Methods Single unit activity was recorded with tungsten micro-electrodes from the vestibular nuclei of the goldfish (Carassius auratus). The floor of the fourth ventricle was exposed, and the microeteetrode was inserted under visual control with ~he aid of a mierescop~. The correct position of the electrode was verified by histology. Experiments were carried out with an enc@hale isol6 preparation, relaxed with Flaxedil, but without general anaesthesia. Animals were placed on a turn-table (TSnnies), The two sensory inputs were separately tested by either rotating the animal in the dark (exclusive vestibular stimulation) or rotating the visual surround with the animal stationary (visual stimulation). This was provided by projecting a pattern of black and white stripes onto the inner walls of a cylinder that surrounded the turn-table. Movement of the projected visual scene was opposite to the direction of body movement as it would be for normal rotation. The interaction of the two sensory inputs was tested when the animal was rotated in the light with a stationary visual environment.

Impulses of single fibres of the vestibular nerve in the goldfish were recorded either from the rostral portion of the left vestibular nerve, or from the left Ramus ampullaris lateralis, or from the proximal stump of the severed nerve.

Saccade characteristics have been studied during coordinated eye-head movements in monkeys. Amplitude, duration, and peak velocity of saccades with head turning were compared with saccades executed while the head was artificially restrained. The results indicate that the saccade characteristics are modulated as a function of head movement, hence the gaze movement (eye+head) exactly matches saccades with head fixed. Saccade modulation is achieved by way of negative vestibulo-ocular feedback. The neck proprioceptors, because of their longer latency, are effective only if the head starts moving prior to the onset of saccade. It is concluded that saccades make with head turning are not 'ballistic' movements because their trajectory is not entirely predetermined by a central command.

A series of experiments /1/ were performed in the Spacelab-1 mission on November/December, 1983, pre-, in-, and postflight. These experiments covered various aspects of the functions of the vestibular system, the inflight tests comprising threshold measurements for linear movements in three orthogonal axes, optokinetic stimulation, vestibulo-ocular reflexes under linear and angular accelerations, caloric stimulation with and without linear accelerations; pre- and postflight tests repeated the inflight protocol with the addition of subjective vertical and eye counter-rotation measurements using a tilt table. One of the most surprising and significant results was the caloric test: strong caloric nystagmus on the two subjects tested was recorded inflight; this was contrary to what was expected from Barany's convection hypothesis for caloric nystagmus.