[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.

[Primary high-grade B-cell lymphoma of the CNS. Case report and review of the literature]. / Primäres hochmalignes B-Zell-Lymphom des ZNS. Fallpräsentation und Literaturübersicht.

We report the case of a 69-year-old patient referred to our clinic because of mania. When examined by neuroradiological imaging, there were lesions seen appearing and disappearing in different regions of the brain during a period of 2 months. Differential diagnosis of these changing lesions, progressive severe illness, and the role of glucocorticoid therapy concerning these lesions are discussed. The diagnosis of primary CNS lymphoma of the B-cell type could not made sure until autopsy.

[Adrenoleukodystrophy mimicking multiple sclerosis]. / Adrenoleukodystrophie kann Multiple Sklerose imitieren.

The article describes the development of symptoms in a 40-year-old female patient who is a symptomatic carrier of X-linked adrenoleucodystrophy (ALD). ALD is characterized by impaired peroxisomal beta-oxidation of very long chain fatty acids and is associated with mutations of the ALD gene, resulting in a defective peroxisomal membrane-transport protein. Our patient's symptoms are identical to those found in multiple sclerosis, showing spastic paraparesis of the lower limbs with marked sensory deficits, visual disturbances in the right eye, and bladder difficulties. Visual and auditory evoked potentials were pathological, and a cranial MRI revealed multiple periventrical white-matter lesions. We found increased intrathecal immunoglobulin production. Diagnosis was established by high concentrations of very long chain fatty acids in serum and in dermal fibroblasts after the same was found in our patient's son. In familial multiple sclerosis, ALD should be excluded in male and female patients.

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.

[Speech disturbances and dyskinesias as initial symptoms of cortico- basal degeneration]. / Sprachstörungen und Dyskinesien als Initialsymptome einer kortikobasalen Degeneration.

The article describes the development of symptoms in a 59-year-old patient. Dyskinesia and speech disorder were the only clinical features in the beginning. Increased immunological parameters and only slight hypokinetic-rigid signs for a long time made the diagnostical and therapeutical process more difficult, as well as atypical findings in neuroimaging techniques. Corticobasal degeneration was diagnosed about 6 years after onset of clinical symptoms.

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.

[Therapy approaches in cerebral cognitive deficits--neuropsychiatric aspects]. / Therapieansätze bei Hirnleistungsstörungen--neuropsychiatrische Aspekte.

According to the latest research the therapy of dementia includes following strategies: Above all there is a necessity for thoroughly diagnostic tests to exclude diseases which secondary induce reduced brain function. The early onset of non pharmacological treatments e.g. "brain-jogging" is essential. Pharmacological therapy with nootropics (e.g. Codergocrin, Nicergolin, Ginkgo biloba, Piracetam, Pyritinol, Naftidrofuryl) is recommended as early as possible, because they have no relevant side effects. Calcium antagonists may also be administered because of their neuroprotective properties. One pharmacological approach to enhance cholinergic functions involves inhibiting ACH-degradation by inhibiting acetylcholinesterase. Although this relatively new therapy has benefits, in some patients it has not been effective and has a potential to cause serious adverse (hepatic) events; only mild to medium severe dementias of Alzheimer's disease should be treated with this therapeutic principle. In the case of personality disorders there are psychotherapy and the administration of psychoactive drugs necessary.

[Ambulatory intensive therapy in the bulbar form of amyotrophic lateral sclerosis]. / Ambulante Intensivtherapie bei bulbärer Form der amyotrophen Lateralsklerose.

Due to the instability of their respiratory functions, patients suffering from ALS are potentially patients for permanent intensive care. The desire to provide care in the familiar environment at home on the one hand, and qualified professional support on the other hand, gives rise to the concept of ambulatory intensive care. This concept might be successfully implemented if one proceeds according to the motto: it is not the patient who is committed to technical facilities, but rather that the technical facilities ought to be committed to the patient. Home care, gastrostomy, tracheostomy, mobile suction drainage and the feasibility of home ventilation provide the groundwork for competent palliative medical care even in the bulbar form of ALS. Home care of such patients would be enhanced in terms of both security and quality if it were possible to have regular ambulatory check of the vital functions at intensive care units. In emergency cases, once their respiratory functions are stabilized patients could be discharged into home nursing after a short-term stay at the intensive care unit. Ambulatory intensive therapy would both serve to ease the burden of intensive care units in terms of costs and personnel, and to improve the life quality of patients.

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.

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.

[Hemorheologic effects of ginkgo biloba extract EGb 761. Dose-dependent effect of EGb 761 on microcirculation and viscoelasticity of blood]. / Hämorheologische Effekte des Ginkgo-biloba-Extrakts EGb 761. Dosisabhängige Wirkung von EGb 761 auf Mikrozirkulation und Viskoelastizität des Blutes.

METHOD: In a randomized open clinical trial involving 42 patients with pathological visco-elasticity values, the effect of a single intravenous injection of 50, 100, 150 or 200 mg of the Ginkgo biloba extract EGb 761, commercially available as Tebonin p.i. on the microcirculation of the skin (Doppler flowmetry) and the visco-elasticity of whole blood was investigated. RESULTS: A dose-dependent significant increase in the microcirculation was found. In the case of visco-elasticity, this dose-dependence was less marked. The present study thus confirms the positive effect of EGb 761 on the microcirculation and whole-blood visco-elasticity in patients with pathological visco-elasticity values, already found in earlier studies, and shows it to be dependent on the dose employed.

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.

[A comparative study of the sensory conduction velocity of the sural nerve using surface and needle electrodes]. / Vergleichende Untersuchung der sensiblen Leitgeschwindigkeit des N. suralis mit Oberflächen- und Nadelelektroden.

In 18 healthy persons aged 22-71 years and 17 patients suffering from clinically defined polyneuropathy the NCV of the sural nerve was measured both by antidromic technic recorded by surface electrodes and by orthodromic technique recorded by needle electrodes. Stimulation was done by bipolar surface electrodes in all cases. The skin temperature was warmed up to the minimum of 35 degrees C. The results showed in both groups a highly significant correlation of the NCV measured by antidromic and orthodromic technique. With increasing age the NCV decreased, but this was not significant. In all healthy persons sensory nerve potentials could be recorded with both techniques. In polyneuropathy missing of sensory potentials was higher in patients investigated by the antidromic technique than by orthodromic. For clinical practice measurement of NCV in sural nerve using antidromic technique should be preferred because of its simpler and faster achieving, its lower discomfort for the patient and avoiding risk of infection. However in case of missing nerve potentials, orthodromic investigation using needle electrodes for recording should be added.