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Im Jahre 1958 wurde am Inselspital in Bern eine Neurologische Abteilung im Rahmen der Medizinischen Klinik gegründet. Acht Jahre später wurde sie zur selbstständigen Klinik. Aus bescheidenen Anfängen hat sich die Neurologie im Rahmen der zunehmenden Bedeutung der Neurowissenschaften zu einer bedeutenden und renommierten Institution entwickelt. Von 1958 bis zu seinem frühzeitigen Tod 1960 lag die Leitung in den Händen von Rolf Magun. Von 1962 bis 1990 wurde sie von Marco Mumenthaler und seither von Christian W. Hess geleitet.

A rare case of Wernicke-Korsakoff syndrome due to malnutrition (in a 35-year-old woman) is reported. It was probably the result of a psychiatric condition progressing to severe inanition. In the course of the malnutrition typical Wernicke-Korsakoff syndrome developed. Vitamin B1 deficiency was confirmed by determining erythrocyte transketolase. At the same time there was hypomagnesiaemia; substitution treatment led to complete recovery.

Headache is very frequent and it has many different causes. It is caused by a severe organic disease only in a small number of patients. In most cases a clinical examination, with special emphasis on the history, is sufficient for a correct evaluation. Only in selected cases additional investigations are needed. Headaches which necessitate an urgent examination are rather infrequent. In most cases the natural course may be observed for a certain period of time without the risk of missing important therapeutic measures.

In 1958 a neurological section of the internal medicine was founded. Eight years late it became an independent department. After a very modest start neurology developped to an important and renowned institution. This development was favoured by the increasing importance of the neurosciences. From 1958 until his premature death in 1960 Bernese neurology was directed by Rolf Magun. From 1962 to 1990 it was headed by Marco Mumenthaler and since then by Christian W. Hess.

Fluctuations (wearing-off, on off phenomenon, dyskinesias) are a big challenge in the long term treatment of parkinsonian patients. We describe the current strategies for prevention and treatment of fluctuations. Special emphasis is put on two new methods which will soon be available. The new dopamine agonist Rotigotine (Neupro) can be transdermally applied. This is a rather simple way to achieve a continuous dopaminergic stimulation. The same goal is achieved by direct application of levodopa-/carbidopa-gelsuspension (Duodopa) in the duodenum by the help of an electronic pump.

For a long time the diagnosis of Parkinson's disease has been considered to be easy. The advantages in treatment in the last decennia have shown that a more sophisticated differentiation from certain syndromes, which will be shortly described, is mandatory in order to achieve optimal therapeutic responses and for giving a prognosis.

The treatment of patients suffering from idiopathic Parkinson's disease has become more and more complex. From its beginning the treatment has to be tailored to the needs of each patient. Not only the individual symptomatology and its course have to be taken into consideration, but also the short- and longtime reaction to treatment.

In the group of medication acting on the dopaminergic system the transdermal application of a dopamine agonist (Rotigotine) and a new MAO B inhibitor (Rasigiline) are receiving most attention. With some concern we had to learn that pergolide, a potent dopamine agonist, may be the cause of clinically relevant valvulopathies. Our possibilities to act on non-dopaminergic deficits is still very limited. The studies demonstrating a positive effect of cholinesterase inhibitors on cognitive decline are at least a positive signal.

Recent clinical studies in multiple sclerosis (MS) provide new data on the treatment of clinically isolated syndromes, on secondary progression, on direct comparison of immunomodulatory treatments and on dosing issues. All these studies have important implications for the optimized care of MS patients. The multiple sclerosis therapy consensus group (MSTCG) critically evaluated the available data and provides recommendations for the application of immunoprophylactic therapies. Initiation of treatment after the first relapse may be indicated if there is clear evidence on MRI for subclinical dissemination of disease. Recent trials show that the efficacy of interferon beta treatment is more likely if patients in the secondary progressive phase of the disease still have superimposed bouts or other indicators of inflammatory disease activity than without having them. There are now data available, which suggest a possible dose-effect relation for recombinant beta-interferons. These studies have to be interpreted with caution, as some potentially important issues in the design of these studies (e. g. maintenance of blinding in the clinical part of the study) were not adequately addressed. A meta-analysis of selected interferon trials has been published challenging the value of recombinant IFN beta in MS. The pitfalls of that report are discussed in the present review as are other issues relevant to treatment including the new definition of MS, the problem of treatment failure and the impact of cost-effectiveness analyses. The MSTCG panel recommends that the new diagnostic criteria proposed by McDonald et al. should be applied if immunoprophylactic treatment is being considered. The use of standardized clinical documentation is now generally proposed to facilitate the systematic evaluation of individual patients over time and to allow retrospective evaluations in different patient cohorts. This in turn may help in formulating recommendations for the application of innovative products to patients and to health care providers. Moreover, in long-term treated patients, secondary treatment failure should be identified by pre-planned follow-up examinations, and other treatment options should then be considered.

Treatment of idiopathic Parkinson's has become increasingly complex during the past years. A revision of the guidelines for treatment which had been published in 1998 by a study group of the Swiss Neurological Society became necessary. Emphasis is again put on the correct choice of medication for the start of treatment. The new guidelines also contain hints to the management of the problems of long term treatment.

This was a phase-IV double-blind equivalence trial designed to assess the efficacy and tolerability of two doses of flunarizine (10 mg o.d. = FLU 10 mg and 5 mg o.d. = FLU 5 mg) in the prophylaxis of migraine, in comparison with slow-release propranolol (160 mg o.d.). A total of 808 subjects were treated in a treatment period of 16 weeks. 142 subjects discontinued the trial prematurely, mainly because of adverse events ( n = 58). The mean attack frequency in the double-blind period was 2.0 for the FLU 5 mg group, 1.9 for the FLU 10 mg group, and 1.9 for the propranolol group. The mean attack frequency in the last 28 days of the double-blind period was 1.8 for FLU 5 mg, 1.6 for FLU 10 mg, and 1.7 for propranolol. Both flunarizine groups were at least as effective as propranolol ( P< 0.001 in one-sided test). The percentage of responders (defined as subjects for whom attack frequency decreased by at least 50% compared to run-in) in the last 28 days of the double-blind period was 46% (118/259) for FLU 5 mg, 53% (141/264) for FLU 10 mg, and 48% (125/258) for propranolol. Statistical analysis showed that FLU 10 mg is at least as effective as propranolol ( P< 0.001) and showed a trend for noninferiority of FLU5 and propranolol ( P = 0.053). No statistically significant differences between the treatment groups were found for any of the secondary parameters. Overall, 190 subjects reported one or more adverse events during the run-in phase: 54 (20.5%) in the FLU 5 mg group, 76 (27.7%) in the FLU 10 mg group and 60 (22.3%) in the propranolol group. The results of this equivalence trial show that 10 mg flunarizine daily with a drug-free weekend is at least as effective as 160 mg propranolol in the prophylaxis of migraine for all evaluated parameters (one-sided equivalence tests) after 16 weeks of treatment. In addition, 5 mg flunarizine proves to be at least as effective as 160 mg propranolol when looking at the mean attack frequency for both the whole double-blind period and the last 28 days of treatment. However, in the analysis of responders, 160 mg propranolol seems to be slightly better than 5 mg flunarizine. In addition, no significant differences between the three treatments were found with regard to safety: all three treatments were generally well-tolerated and safe.

Many patients suffering from Parkinson's disease complain about chronic fatigue and daytime somnolence. During the last few years attention has been drawn to "sleep attacks", which are supposed to be due mainly to dopamine agonists. Sleep disturbances during the night are quite frequent. It is important to search for the probable causes in each individual case in order to be able to install an efficacious treatment.

It lasted some 150 years after the first description by James Parkinson in 1817 until levodopa, the first really efficacious treatment, was available for Parkinson's disease. Previously only anticholinergic substances and stereotaxic operations, both acting merely exclusively on tremor, were used for many and a few years respectively. Even today the treatment is purely symptomatic. There is no treatment in sight which would cure the disease or at least stop or slow down its progression.

Epilepsy is an important factor of neurologic morbidity in the elderly population. The incidence is higher in the age group over 65 years than in children and adolescents. Cerebrovascular disturbances are the most frequent cause of seizures in the elderly. The so-called idiopathic epilepsies on the other hand are rather rare. The frequent co-morbidity and co-medications have to be taken in consideration when evaluating and treating such patients. There is a number of efficacious antiepileptic drugs at disposition. The selection of the drugs and their dosage have to be adapted to the needs of the particular age group.

Two female patients with recurrent optic neuritis and severe myelitis were described. In the first patient the illness began with recurrent myelitis, in the second one with optic neuritis. In both patients spinal MRI showed extensive enhancing lesions of the cervical respectively thoracic spinal cord. An initial cranial MRI was normal. In the first patient an MRJ demonstrated after several years lesions not typical for multiple sclerosis. In both patients cerebrospinal fluid showed especially mononuclear pleocytosis with cell counts between 11 and 126/microliter and severely elevated total protein, while intrathecal oligoclonal bands were not found. During exacerbations in both patients ENA-autoantibody-screening was positive. Intravenous treatment with methylprednisolone improved the clinical situation for some time. In both patients cyclophosphamide at a dose of 100 mg daily had to be given due to relapsing neurological deficits.

Objective: To reevaluate concordance rates in 9 monozygotic (MZ) and 12 dizygotic (DZ) twin pairs with PD 8 years after the initial study. Background: Longitudinal investigations increase accuracy in clinical diagnosis of PD. Methods: Follow-up by personal interview and clinical examination. Results: Concordance rates were not different between MZ (3/9) and DZ (5/12) twin pairs at follow-up, even when PD-associated dementia and isolated postural tremor were considered diagnostic of familial Lewy body parkinsonism. Evaluation of medical history, personality traits, and blink rate did not reveal putative early or premorbid parkinsonism in 9 co-twins who were motor-asymptomatic during the follow-up interval. However, these co-twins had reduced semantic fluency in comparison with a healthy control group of similar age. None of 7 co-twins without motor signs who underwent PET investigation 6 years previously showed signs of extrapyramidal disease at follow-up, but verbal memory continued to be reduced in 5 of these co-twins. Conclusion: Based on concordance rates only, the findings in our twin sample do not support a major genetic impact for the motor expression of PD.

Several new methods for the treatment of patients suffering from Parkinson's disease have been introduced during the last few years. COMT inhibitors are available in addition to levodopa with a decarboxylase inhibitor. COMT inhibitors enhance and prolong the effect of single levodopa doses. Severe side effects have been mentioned in connection with the COMT inhibitor tolcapone in recent months. Several new dopaminergic agonists now provide efficacious monotherapy in the early stages of the disease. This brings about a reduction in late problems of treatment such as dyskinesias and fluctuations. Stereotaxic operations in the pallidum and the subthalamic afford relief for these late complications of treatment in selected patients.

The criteria for the diagnosis of dementia and the inherent problems are discussed. From a neurological point of view the most important differential diagnoses are described. Special attention is paid to potentially treatable conditions.

According to the classification of the International Headache Society, an episodic and a chronic form of tension-type headache are distinguished. In most cases the headache is less severe than in migraine patients. For this reason many patients never consult their doctor. In the treatment of such patients, one must be very cautious with analgesics. There is a considerable risk of chronic use of these medications, which may be the cause of a chronic daily headache. Relaxation techniques and cognitive training have been shown to be helpful. Most experts consider amitriptyline to be the best long-term medication.

In developing countries tetanus is still a real problem because of its high incidence and mortality. In all countries with high medical standards it has become rare. For this reason many clinicians are no longer familiar with the disease and its diagnosis. Until now no laboratory test has been readily available to confirm or rule out tetanus. However, the diagnosis can be performed by a simple and readily available electromyogram (EMG). We performed EMGs in 13 patients in whom tetanus was suspected but whose case history or clinical findings left some doubt. In 7 cases the EMG was typical for tetanus, showing spontaneous activity of motor units which could not be suppressed voluntarily and with shortening or absence of the silent period after a stretch reflex or after electrical stimulation of the nerve. In 2 cases we found one of the two diagnostic features. In all these 9 cases the diagnosis of tetanus was confirmed by the further development of the disease. In the remaining 4 patients the EMG was normal and in the course it was confirmed that they were not suffering from tetanus. Therefore, we consider electromyography a very useful and reliable tool either to confirm or rule out the diagnosis of tetanus.

Headache takes a large place in daily medical practice, and it is important to try and establish the most precise possible diagnosis. An outline of the diagnosis and differential diagnosis of migraine is followed by a discussion of interval drug treatment forms and counselling. Today's recommended treatment for attacks and the commonest sources of error in migraine management are summarized.

The history of clinical application of electromyographic and electroneurographic techniques is not even 60 years old. For a long time the clinicians' need could not be fulfilled, due to the imperfection of the electronic equipment. Nowadays, electromyography and electroneurography are established methods not only for the diagnosis of neuromuscular disorders, but since the recording of cortical and spinal evoked potentials has been introduced, diseases of the central nervous system lie also within its scope.

Elektrophysiologische Untersuchungen konnen in der Neuroorthopadie von grosem Nutzen sein. Wie bei allen Hilfsuntersuchungen ist es aber unerlaslich, das sie methodisch korrekt durchgefuhrt und kritisch beurteilt werden. Sie liefern nie eine fertige Diagnose, sondern sie geben nur Auskunft uber den funktionellen Zustand der untersuchten Strukturen. Die Untersuchungen mussen daher immer im klinischen Kontext gesehen werden. Die Moglichkeiten und die Grenzen der einzelnen Methoden mussen dem Untersucher und dem Kliniker bekannt sein. Leider werden immer wieder Fragen an die klinische Neurophysiologie gestellt, die sie gar nicht beantworten kann, und es werden Untersuchungsresultate in unzulassiger Weise uberinterpretiert. Die eigentlich selbstverstandliche Regel, das Untersuchungen in der Klinik nur eingesetzt werden sollten, wenn auch verlasliche Normwerte vorliegen, wird leider sehr haufig misachtet.

The treatment of idiopathic Parkinson's disease has been divided in 3 different phases. In the first phase the patient's disability is still minimal and a treatment with selegiline (possibly combined with an anticholinergic drug) is sufficient. In the second phase the treatment with levodopa (plus an decarboxylase inhibitor) becomes indispensable. In order to minimize the problems of long-term therapy a dopaminergic agonist (mostly bromocriptine) is added. The third phase is characterized by these problems. Some measures are discussed which may help to increase the patients' quality of life.

A classification of the disturbances of consciousness is given, and the possible causes are listed. The criteria used in Switzerland for the diagnosis of brain death are presented in detail.

28 patients suffering from idiopathic Parkinson's disease received subcutaneous apomorphine for the treatment of painful dystonia and of off-phases of long duration. After a mean period of 12.4 months 20 patients still used apomorphine. In this time the mean dose per injection had fallen from 2.5 mg to 2.1 mg and the mean number of daily applications from 2.6 to 2.2. Most patients considered dystonias and off-phases to respond satisfactorily to apomorphine. However, in most cases the treatment effect was impaired by side effects, especially nausea.

At present we dispose of a number of medical, surgical and other measures for the treatment of patients suffering from idiopathic Parkinson's disease. Some guidelines for the adaptation of the treatment for the individual patient will be given. Special attention will be given to the question of a possible neuroprotective treatment.

In eight patients psychogenic para- or quadriparesis was clinically diagnosed. The following features were identified as positive criteria for psychogenic etiology: (1.) contradictory clinical findings (normal muscle tone, normal reflexes, no pyramidal signs, alternating muscle power at varying situations of clinical examination etc.), (2.) paradoxical behavior ("belle indifférence"), (3.) normal electrophysiologic, neuroradiologic and cerebrospinal fluid findings. Overemphasizing technical versus clinical diagnosis involves the risk of expensive diagnostic and therapeutic procedures and favors fixation on the symptoms.

Headache is very frequent and it has many different causes. It is caused by a severe organic disease only in a small number of patients. In most cases a clinical examination, with special emphasis on the history, is sufficient for a correct evaluation. Only in selected cases additional investigations are needed. Headaches which necessitate an urgent examination are rather infrequent. In most cases the natural course may be observed for a certain period of time without the risk of missing important therapeutic measures.

Peripheral and central mechanisms involved in generation, modulation and perception of pain are discussed. Lesions of peripheral nerves are a particular cause of pain, which has to be recognized as such, as it requires specific therapeutic measures. Emphasis is put on the role of ephaptic transmission.

ADRs presenting as neurologic or psychic disturbances are rather frequent; therefore, it will not be possible to give a complete overview. Drug-induced extrapyramidal syndromes which are due to an interference of the drug or a drug metabolite with cerebral dopaminergic mechanisms in most instances are discussed in more detail. Neuroleptics are prominent in this group of preparations. Drug-induced Parkinsonism, acute dystonia, tardive dyskinesia and the neuroleptic malignant syndrome are the ADRs presented herein.

In the course of long-term treatment of Parkinson's disease many problems, which cannot yet be satisfactorily solved, arise with time in a large number of patients. This overview describes some strategies which are used nowadays to tackle these problems. Special attention is drawn to the treatment of painful dystonias and off-periods with apomorphine.

Surgical interventions can give rise to peripheral nerve lesions by various mechanisms. The neurologist is asked to define which nerve has been lesioned at which site. We also have to define the type of lesion and its severity. The electrophysiological investigation is a very important diagnostic tool in this particular situation. Together with the clinical examination it will allow to plan an optimal treatment for the patient.

To the Editor: Some years ago I was asked by an asymptomatic cotwin of a patient with PD whether an [18F]dopa PET scan could predict whether he would develop the illness. Vieregge et al.’s article1 is the second recent study examining the progress of asymptomatic twins identified as having abnormal, and putatively presymptomatic, scans. The editor and authors should be congratulated for publishing what is essentially a negative study; not one of seven asymptomatic cotwins with an abnormal scan has developed PD in 6 years. An important explanation is omitted from the discussion. [18F]dopa PET is a technique that has limited reproducibility, numerous potential technical errors, and in the identification of preclinical disease, is likely to produce false positives and false negatives.2 Without reproducibility data, the authors and readership cannot be confident of the reliability of identification of abnormal subjects. This suggests that the technique may have erroneously identified preclinical subjects. The recent similar follow-up study from this country3 does not give any greater confidence in the technique. In that study, 10 of 18 asymptomatic monozygotic (MZ) cotwins and 3 …

The efficacy of 5-hydroxytryptophan and propranolol has been studied in the interval treatment of migraine. 39 migraine patients have participated in a double-blind trial. After a placebo run-in of one month, the patients received either 5-hydroxytryptophan or propranolol for 4 months. The treatment with both substances resulted in a statistically significant reduction in frequency of migraine attacks. Furthermore, in the propranolol group the duration of the attacks and the number of analgesics used for treatment of the attacks were significantly reduced. Although propranolol, which is considered a reference for the interval treatment of migraine, is more effective, 5-hydroxytryptophan is a possible alternative for many patients.

Two patients with isolated neurogenic hypertrophy of the trapezius muscle due to accessory nerve injury and a patient with neurogenic hypertrophy of the anterior tibial muscle due to chronic radicular lesion L4 are described. Electromyography of the affected muscles showed dense continuing spontaneous discharges of complex potentials. Muscle biopsy performed in two patients showed abundant hypertrophic muscle fibres, identified in one case by ATP-ase reaction as being of predominantly type I. In the majority of previously reported patients with neurogenic muscle hypertrophy confined to the calf muscle, a passive stretch mechanism was suggested as a cause of the hypertrophy. It is assumed that the excessive spontaneous muscle activity gave rise to the hypertrophy in these patients. This may also be true in previously reported patients with neurogenic hypertrophy and similar spontaneous activity in electromyography.

Migraine patients suffering from frequent and severe attacks may need prophylactic treatment. Propranolol, a beta-receptor blocker, and flunarizine, a calcium antagonist, are considered to be the most effective compounds for the prophylaxis of migraine. In a number of controlled studies, flunarizine has been shown to reduce the number of migraine attacks significantly. In migraine studies, sedation and weight gain are the most frequent side effects of flunarizine.

Patients with cervical radicular lesions present themselves with neck pain and stiffness on the one side and with neurological deficits of radicular distribution on the other side. The most frequent cause is a root compression due to a herniated disk or spondylotic changes of the spine. The management of the patients and the differential diagnosis are discussed.

Patients suffering from Parkinson's disease frequently complain of dizziness, postural instability and falls. Vestibular tests have been performed in 30 parkinsonian patients and in 28 controls. The results suggest a central vestibular disturbance in Parkinson's disease which correlates with the clinical disability. This vestibular disturbance is assumed to be due to dysfunction of the nigro-striato-collicular tracts.

Voiding disorders are common in patients with myelomeningocele (MMC) and are of great prognostic importance. We performed urodynamic-electromyographic measurements in 11 adolescent MMC patients who had undergone immediate postnatal surgery. None of our patients was able to urinate normally, and 9 suffered from some form of incontinence. The detrusor vesicae was hyper-reflexive in 6 patients and hypo-reflexive in 4. The bladder neck was never obstructive, but the striated sphincter was obstructive in 4. In 2 of these detrusor sphincter dyssynergy was found, and in 2 patients a non-relaxing sphincter was present. There was no correlation between the localization of the MMC and the type of dysfunction. The aim of any treatment of these disorders should be to prevent the development of vesico-ureteral reflux. Appropriate treatment requires the type of bladder dysfunction to be precisely known. Hence a urodynamic-electromyographic assessment should be performed in all MMC patients with voiding problems.