[Consensus statement "Dementia 2010" of the Austrian Alzheimer Society]. / Konsensusstatement "Demenz 2010" der Osterreichischen Alzheimer Gesellschaft.

The Austrian Alzheimer Society developed evidence-based guidelines based on a systematic literature search and criteria-guided assessment with subsequent transparent determination of grades of clinical recommendation. The authors evaluated currently available therapeutic approaches for the most common forms of dementia and focused on diagnosis and pharmacological intervention, taking into consideration the situation in Austria. The purpose of these guidelines is the rational and cost-effective use of diagnostic and therapeutic measures in dementing illnesses. Users are physicians and all other providers of care for patients with dementia in Austria.

Diagnostic value of different electrophysiologic tests in cervical disk prolapse.

We evaluated 24 patients with radiologically verified cervical disk prolapse. EMG abnormalities appeared in 67% of cases studied; dermatomal somatosensory evoked potentials revealed 85% abnormal findings on the affected side, but also revealed abnormalities in adjacent segments. In 38%, F responses were abnormal. We found electrodiagnostic abnormalities at levels without a disk prolapse, probably due to degenerative spondylopathic changes and beginning consecutive vascular compromise.

A sporadic form of hereditary neuropathy with liability to pressure palsies: clinical, electrodiagnostic, and molecular genetic findings.

We report a patient who had episodes of recurrent peripheral nerve pressure palsies. Electrodiagnostically, we found a clear decrease of nerve conduction velocity in affected and unaffected nerves. All the patient's relatives showed entirely normal clinical and electrodiagnostic findings. Histopathologically, there were extensive irregularities of the myelin sheaths with numerous tomaculous swellings. DNA analysis revealed a deletion for probes flanking the PMP-22 gene at the maternal chromosome 17 in our patient. His mother showed a normal gene dosage for all markers deleted in our patient, indicating a new mutation.

Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI.

Repeated bleeding into CSF spaces may cause superficial siderosis with progressive neurologic impairment. The unique physical properties of MRI provide a basis for the in vivo diagnosis of this entity.

Age and sex differences of dehydroepiandrosterone sulfate (DHEAS) and cortisol (CRT) plasma levels in normal controls and Alzheimer's disease (AD).

In 50 healthy subjects (23 female, 27 male, aged 18-81) and 24 patients with Alzheimer's disease (AD) (11 female, 13 male, aged 58-88) DHEAS and CRT plasma levels were studied. In normal subjects there was a clear negative correlation of DHEAS to age, while no significant age correlated decrease of CRT plasma levels was found. There was a significant decrease in the DHEAS/CRT ratio in elderly controls (aged > 60) as compared to young individuals (aged < 45). Overall there was a trend to lower DHEAS/CRT ratios in AD patients compared to age matched controls out of the total group of normals (P < 0.1), there was a significant decrease of this ratio in female AD patients (P < 0.05), compared to age matched female controls, but there was none in male Alzheimers; furthermore there was a significant difference in CRT plasma levels between female AD patients and age matched female controls (P < 0.01) and between female and male AD patients (P < 0.05). Considering the antiglucocorticoid effects of DHEAS, this ratio may account for its protective effect against hippocampal degeneration caused by glucocorticoids and possibly for the higher rate of AD in females.

Serum dehydroepiandrosterone and cortisol measurements in Huntington's chorea.

Serum levels of dehydroepiandrosterone sulfate (DHEAS), known to antagonize metabolic effects of glucocorticoids in animals, and cortisol (CRT), already shown to be related to cognitive dysfunction in man and animals, were measured in 11 drug-free male subjects with definite Huntington's chorea (HC) and in 25 age-matched male normal controls. Statistical difference was found between DHEAS serum levels (p < 0.05), CRT levels (p < 0.05) and the DHEAS/CRT ratio (p < 0.01) of HC subjects and normal individuals. These findings may indicate a dysfunction of the hypothalamic-pituitary-adrenal axis (HPAA) and possibly suggest a role of DHEAS as an antiglucocorticoid in HC.

Postmovement beta synchronization in patients with Parkinson's disease.

Event-related synchronization (ERS) after self-paced, voluntary brisk movement of the right and left thumb was studied in 17 patients with Parkinson's disease (PD) and 17 age-matched control subjects. All patients were receiving L-DOPA and/or DOPA-agonists. The movement-offset-triggered EEG data were analyzed in the 12- to 16-Hz, 16- to 20-Hz, and 20- to 24-Hz bands for eight time intervals after termination of movement. Significant differences in postmovement beta synchronization were observed in all three frequency bands. As compared with the control group, patients with PD showed a remarkably smaller beta ERS. This was the overall main effect for groups, as well as for interactions concerning side of movement and electrode positions. If beta ERS is a measure of recovery of the primary motor area after movement, our results indicate that this ability is impaired in PD patients.

Hypervolemic haemodilution and completed stroke: how important is the application time for its effect?

In 75 patients with completed stroke hypervolemic haemodilution was performed for 1 day with 500 ml 6% HES 200.000/0.6-0.66 (Elohäst) to test the acute influence of application time for haemorheological changes. The patients were divided into 3 groups randomly selected: group A received the infusion for 2 hours, group B for 4 hours and group C for 6 hours. Haematocrit (HCT), whole blood viscosity (VI-VB), blood elasticity (EL-VB) and plasma viscosity (VI-PL) were determined before onset of infusion, after 6 and 18 hours subsequently. The values obtained were compared with the onset results. In all groups and parameters after 6 hours there was a significant decrease. After 18 hours group A showed a significant higher value of HCT and increasing trends for VI-VB and EL-VB. In group B after 18 hours a significant decrease of VI-VB could be observed. In group C all values remained significantly below the onset. The results show, that one important haemorheological argument for an effective form of haemodilution should also be a long application time. This might be one of the reasons, why in some studies with hypervolemic haemodilution no positive results could be obtained.

Compactotomy in Huntington's chorea.

Advances in neuroradiological and neurosurgical techniques have lead to a growing interest in functional neurosurgical interventions for medically intractable movement disorders. The majority of these procedures are performed in patients with hypokinetic movement disorders, especially Parkinson's disease. However, relatively few interventions were done in hyperkinetic disorders such as Huntington's disease (HD), mainly owing to the lack of an adequate target nucleus. We have recently described the case of a reversible chorea in a genetically confirmed HD patient. We subsequently identified a marked bilateral degeneration of the substantia nigra as the probable reason for choreatic cessation. We therefore suggest that primary striatal atrophy causing hyperkinesia and secondary substantia nigra atrophy favouring hypokinesia were balanced in this patient, thus resulting in a close-to-physiologic GABAergic basal ganglia output. We postulate that deep brain stimulation of the substantia nigra pars compacta may ameliorate hyperkinesia in choreatic movement disorders, thus representing the first effective therapy in Huntington's chorea. Several lines of evidence in recent neurophysiological research support our hypothesis and are discussed below.

[Comments on epileptic psychosis (two case reports) (author's transl)]. / Zum Problem der epileptischen Psychosen.

Nosological interpretation and therapy of epileptic psychosis is difficult, since psychotic and epileptic activities are of quite different time relationships. Case reports of two patients suffering from psychosis and showing generalized spike-wave paroxysms, without evidence of clinical seizures, are presented. The problems of diagnosing epileptic psychosis under these circumstances and the therapeutic approach are discussed.

[Somatosensory evoked potential in general anesthesia]. / Das somatosensorisch evozierte Potential unter Bedingungen der Allgemeinanästhesie.

Somatosensory evoked potentials (SSEP) were investigated under general anaesthesia in 12 patients with no history of previous cerebral disease. With the commonly-used stimulation of the median nerve on the wrist, conduction from the homolateral Erb's point, and contralateral conduction from the mastoid and sensory cortical area, SSEP were registered during the first 100 msec. after the stimulation. Latency period, shape and amplitude of the spinal and subcortical components and early cortical potentials (N 20, P 28) show no change, whereas the consecutive negative and positive cortical SSEP show a definite change in shape and latency period. No change was registered in somatosensory impulse conduction and early cortical response under general anaesthesia and these can, therefore, be used during neurosurgery as a parameter for the control of cortical and subcortical functioning.

[Value of multimodal evoked potentials in the diagnosis of multiple sclerosis]. / Zum Stellenwert multimodal evozierter Potentiale in der Diagnostik der multiplen Sklerose.

94 Patients diagnosed as having multiple sclerosis (MS) were examined by multimodal evoked potentials. According to the criteria of Bauer and Poser the patients were classified as possible, probable and certain forms of MS. Abnormalities were found in the VEP in 82.6% of cases (possible - 44.4%, probable - 87.5%, certain - 95.2%), in the BAEP in 51.3% of cases (possible - 27.3%, probable - 46.2%, certain - 68.8%) and in the SSEP in 71.1% of cases (possible - 35.3%, probable - 67.7%, certain - 88.1%). Subclinical lesions were detected by VEP in 37.0% of cases, by BAEP in 43.6% and by SSEP in 21.1%. Reclassification according to the results of the evoked potentials studies was required in 21.2% of cases. The value of evoked potentials in the diagnosis of MS is discussed with regard to the different forms of evoked potentials, as well as in comparison with other examination techniques.

[The treatment of blepharospasm with botulinum toxin A]. / Die Behandlung des Blepharospasmus mit Botulinum-Toxin A.

12 patients suffering from essential blepharospasm were treated by injection of 4 ng botulinum toxin A into the Musculus orbicularis oculi of each eye. The effect was followed up over a period of 6 weeks by means of a subjective rating scale, a visual function score and a score for the overall clinical impression. After 4 days an improvement was observed in all patients and it was still present after 6 weeks. After a time interval of 12 weeks reinjection was performed in 8 cases with half the initial dose, since the beneficial effect had decreased to under 50%. Follow-up showed an impressive response to reinjection of botulinum toxin A, which remained undiminished still after 6 weeks. No serious side effects were observed. Hence, this form of therapy appears to be an effective alternative to all other therapeutic measures in this disease.

[Involvement of the optic nerve in neural muscular atrophy]. / Zur Frage der Optikusmitbeteiligung bei neuraler Muskelatrophie.

Visual evoked potentials (VEP) were investigated in 12 members of a family with Charcot-Marie-Tooth disease, 7 of whom showed manifest clinical signs of the disease. The mean value of the latency P100 of VEP in the 12 patients did not differ significantly from the value in normal controls, nor did the values in members with considerably reduced nerve conduction velocities differ significantly from those in members with normal nerve conduction velocity. Among the investigated members of the family, however, there was a significant difference in mean P100 latency between family H.G. with no clinical signs of the disease and the family of the twin brother, F.G. with clear clinical and neurographic signs of HMSN I. The divergent opinions given in the literature may be explained by heterogeneity, as well as an extreme expression of the inherited defect and a combination of two independently inherited diseases.

[Transient global amnesia]. / Die transiente globale Amnesie.

The clinical picture of transient global amnesia (TGA) is discussed on the basis of the clinical findings in 19 patients. The central symptom is an acute memory loss, leading to disorientation, helplessness and pseudo-confusion. The symptoms last for several hours and then fade away. Pathogenetically a functional impairment of the limbic system is proposed. Most cases of TGA are due to ischaemic disease; rare causes are epileptic seizures, tumours, encephalitis and toxic or metabolic conditions. In order to clarify the aetiology and initiate appropriate therapy meticulous neurological investigation is necessary. The spontaneous prognosis of ischaemic forms is favourable, whilst in other cases it depends on the primary condition.

[Effect of naftidrofuryl on viscoelasticity, thrombocyte aggregation and erythrocyte fluidity of blood]. / Die Wirkung von Naftidrofuryl auf die Viskoelastizität, die Thrombozytenaggregation und die Erythrozytenfluidität des Blutes.

In 30 patients suffering from cerebrovascular disease stage I with atherosclerotic plaques in the carotid's bifurcation the effect of a 7-days application with 400 mg Naftidrofuryl versus NaCl was tested. As parameters viscosity and elasticity of whole blood, plasma viscosity, erythrocytes' and membrane fluidity point, as well as platelet aggregation (spontaneous, ADP-, Epinephrine- and Collagen-induced) were determined before onset, after 4 days and after 8 days. After 8 days treatment with naftidrofuryl there was a significant improvement in viscosity and elasticity of whole blood as well as in erythrocytes' elongation, membrane-fluidity and in the induced test of platelet aggregation.

[Peroperative evoked potentials in neurosurgery]. / Les potentiels évoqués per-opératoires en neuro-chirurgie.

The application of somatosensory evoked potentials in different fields of neurosurgical procedures are presented. SSEP are used for the intra-operative control in aneurysm surgery, during a.v. malformation surgery specially in the posterior fossa and in the operative treatment of large acoustic neurinomas. Finally the authors describe their technic of anterior spinal cord stimulation in transthoracic approach in spinal cord traumatic lesions. In this patients proven intra-operative neuronal continuity allows good prognosis of functional recovery.

[Temporary bypass in carotid surgery; peroperative study of various metabolic parameters and the EEG]. / Le by-pass temporaire en chirurgie carotidienne; étude per-opératoire de quelques paramètres métaboliques et de l'EEG.

Out of a whole of patients of nearly 1000 reconstructive operations of the extracranial carotid artery, which were carried out by a systematic application of the intraluminal shunt, 32 selected patients with extracranial stenosis of the internal carotid artery were examined intraoperatively as to the following parameters of the internal carotid artery and the internal jugular vein: pH, pCO2, pO2, B.E. and the lactate were determined as well. Statistic analysis shows a significant increase of the arteriovenous difference of the lactate from the first to the second and from the first to the third sample.

[Intraoperative somatosensory evoked potentials (SSEP) in surgery of cerebral aneurysms: topographical information]. / Intraoperative somatosensorisch evozierte Potentiale (SSEP) in der Chirurgie cerebraler Aneurysmen: topische Aussagen.

Out of 114 patients who were operated because of cerebral aneurysms, 16 patients were monitored by means of Somatosensory Evoked Potential (SSEP) during the operation. In 9 of these, successful monitoring was possible during the whole duration of the surgical procedure. In the remaining seven, monitoring was impossible over a long period so that these patients were excluded from the present study. Monitoring of the cerebral functions by means of SSEP provides help in aneurysm surgery as follows: Objective evaluation of the function of the somatosensory system. The induced arterial hypotension--usually done in our department--implies danger for the brain, particularly, if rupture of the aneurysm occurs at the same time. In this case, Somatosensory Evoked Potentials provide the possibility to detect lesions of somatosensory neurons at different levels. This makes prognostic conclusions possible. Pathological changes of some SSEP components during temporary occlusion of arteries of the Circle of Willis indicate developing lesions of brain structures. Intraoperative SSEP data were correlated with the postoperative course in all 9 patients. Prognostic conclusions derived from these data are discussed.