[S1-Guideline for Diagnosis and Treatment of Erectile Dysfunction]. / S1-Leitlinie Diagnostik und Therapie der erektilen Dysfunktion.

Die S1-Leitlinie "Diagnostik und Therapie der erektilen Dysfunktion" (AWMF-Registernummer 030 / 112) steht in einer vollständig überarbeiteten Neufassung zur Verfügung. Erektile Dysfunktion ist definiert als die fortwährende Unfähigkeit, eine penile Erektion, die für einen befriedigenden Geschlechtsverkehr ausreicht, zu erreichen oder aufrechtzuerhalten. Betroffen sind Millionen Bundesbürger: Bei Männern mit regelmäßiger sexueller Aktivität nimmt die erektile Dysfunktion von 2,3 Prozent in der dritten Lebensdekade auf 53,4 Prozent in der siebten Lebensdekade zu. Die Leitlinie gibt dezidierte Empfehlungen zur adäquaten Diagnose und zur Therapie der erektilen Dysfunktion, die seit der Einführung der Phosphodiesterase-5-(PDE-5-)Hemmer häufig unkritisch erfolgt.

Management of supine hypertension in patients with neurogenic orthostatic hypotension: scientific statement of the American Autonomic Society, European Federation of Autonomic Societies, and the European Society of Hypertension.

: Supine hypertension commonly occurs in patients with neurogenic orthostatic hypotension due to autonomic failure. Supine hypertension promotes nocturnal sodium excretion and orthostatic hypotension, thus, interfering with quality of life. Perusal of the literature on essential hypertension and smaller scale investigations in autonomic failure patients also suggest that supine hypertension may predispose to cardiovascular and renal disease. These reasons provide a rationale for treating supine hypertension. Yet, treatment of supine hypertension, be it through nonpharmacological or pharmacological approaches, may exacerbate orthostatic hypotension when patients get up during the night. Fall-related complications may occur. More research is needed to define the magnitude of the deleterious effects of supine hypertension on cardiovascular, cerebrovascular, and renal morbidity and mortality. Integration of more precise cardiovascular risk assessment, efficacy, and safety data, and the prognosis of the underlying condition causing autonomic failure is required for individualized management recommendations.

Clinical Rating Scales for Urinary Symptoms in Parkinson Disease: Critique and Recommendations.

BACKGROUND: The prevalence of lower urinary tract symptoms (LUTS) is high in Parkinson's disease (PD). These problems negatively affect quality of life and include both storage and voiding problems. The International Parkinson and Movement Disorder Society established a task force to review clinical rating scales/questionnaires for the assessment of urinary symptoms in PD. METHODS: According to prespecified criteria, these scales/questionnaires were classified as "Recommended" or "Recommended with caveats" when clinimetric properties were satisfactory for Recommended status but had not been assessed specifically in PD, "Suggested" or "Listed." These assessments were applied to rate scales as screening tools for the diagnosis of LUTS and for the rating of symptom severity. RESULTS: Among scales that included LUTS but focused on overall autonomic or non-motor symptoms in PD, no scale reached the clinimetric rigor to be designated as Recommended or Recommended with caveats, but some were Suggested for either diagnostic screening tools or severity measures. Among primary urological scales, most are well validated in urological setting, but none was validated specifically in PD. DAN-PSS (Danish PSS), ICIQ (International Consultation for Incontinence Questionnaire)-MLUTS (Male Lower Urinary Tract Symptoms), OABq, OABq-SF (ICIQ-OABqol), OAB-V8 (as screening tool), and OABSS (OAB Symptom Score) met criteria for Recommended with caveats. CONCLUSION: The Task Force does not recommend the development of a new scale. However, all above-mentioned questionnaires need to be studied further and specifically validated in PD.

Consensus statement on the definition of neurogenic supine hypertension in cardiovascular autonomic failure by the American Autonomic Society (AAS) and the European Federation of Autonomic Societies (EFAS) : Endorsed by the European Academy of Neurology (EAN) and the European Society of Hypertension (ESH).

PURPOSE: Patients suffering from cardiovascular autonomic failure often develop neurogenic supine hypertension (nSH), i.e., high blood pressure (BP) in the supine position, which falls in the upright position owing to impaired autonomic regulation. A committee was formed to reach consensus among experts on the definition and diagnosis of nSH in the context of cardiovascular autonomic failure. METHODS: As a first and preparatory step, a systematic search of PubMed-indexed literature on nSH up to January 2017 was performed. Available evidence derived from this search was discussed in a consensus expert round table meeting in Innsbruck on February 16, 2017. Statements originating from this meeting were further discussed by representatives of the American Autonomic Society and the European Federation of Autonomic Societies and are summarized in the document presented here. The final version received the endorsement of the European Academy of Neurology and the European Society of Hypertension. RESULTS: In patients with neurogenic orthostatic hypotension, nSH is defined as systolic BP ≥ 140 mmHg and/or diastolic BP ≥ 90 mmHg, measured after at least 5 min of rest in the supine position. Three severity degrees are recommended: mild, moderate and severe. nSH may also be present during nocturnal sleep, with reduced-dipping, non-dipping or rising nocturnal BP profiles with respect to mean daytime BP values. Home BP monitoring and 24-h-ambulatory BP monitoring provide relevant information for a customized clinical management. CONCLUSIONS: The establishment of expert-based criteria to define nSH should standardize diagnosis and allow a better understanding of its epidemiology, prognosis and, ultimately, treatment.

Heart Rate Spectra Confirm the Presence of Autonomic Dysfunction in Dementia Patients.

Recent data suggest autonomic dysfunction in patients suffering dementia. This study evaluated autonomic modulation in dementia patients with and without autonomic involvement, employing ECG spectral analysis in the time-frequency domain (wavelet transform) in supine resting and head-up tilt (HUT) position. Thirty-six patients were prospectively evaluated at the Department of Neurology and Psychiatry, General Hospital of the City of Linz, between 2009 and 2014. A standard cardiovascular autonomic test series (Ewing battery) was performed to screen for autonomic dysfunction. The Ewing battery diagnoses were used as reference standard and compared to the diagnostic results obtained by spectral analysis (time-frequency domain) of ECG recordings. Based on the Ewing battery results, 14 patients suffered autonomic dysfunction, while 22 did not. Time frequency domain was accessed by using the continuous wavelet transformation (CWT) with an analytical Morlet mother wavelet in supine resting and HUT position. Within each cohort the modification of spectral components from supine resting to HUT was analyzed reflecting the autonomic modulation. For patients without autonomic dysfunction, a significant increase of autonomic modulation was detected by wavelet transformed ECG recordings (8%, p < 0.05; low frequency content) during HUT compared to supine resting. There was no significant modulation between HUT and supine resting in patients suffering autonomic dysfunction. In dementia patients suffering autonomic dysfunction, CWT identified blunted autonomic regulation only by analysis of ECG recordings without the need to assess other biosignals or tests depending on the patient's cooperation. Further studies are needed to evaluate whether CWT is a suitable method to support the standard Ewing battery in demented patients.

[The seriously affected stroke patient who is not able to communicate - treatment to the best of one's knowledge and ethical principles]. / Der schwer betroffene kommunikationsunfähige Schlaganfallpatient - Handeln nach bestem Wissen und ethischen Grundlagen der medizinischen Behandlung?

Choosing the right therapy for a patient affected by a severe stroke that resulted in communicative inability is a real challenge for an interdisciplinary team. In the following case prognostic predictors will be investigated and ethical issues for a better decision making will be discussed.

Incidence of cerebrovascular lesions in pure autonomic failure.

In pure autonomic failure (PAF) - a rare form of primary dysautonomia - some patients show cerebrovascular lesions usually found in hypertensive subjects. In an autonomic laboratory records of patients with a definitive diagnosis of PAF having had cerebral imaging (cMRI, cCT) were analysed retrospectively. Tilt table data (supine/tilted), 24 hour blood pressure recordings (day/night) and serum catecholamine levels were correlated with cerebrovascular lesions and also compared to published normal values. 50 PAF patients (23 female, 27 male) were identified, mean age 67 years (sd 9.5). Out of these 35 (70%) had pathologic cerebral scans showing white matter lesions (WML) in 30, lacunar strokes in 5 and hemispheric stroke and microbleeds each in 1. Age and supine systolic blood pressure were significantly elevated in patients with pathologic scans (70 compared to 61 years [p=0.007], and 170 compared to 154 mmHg [p=0.045]). Out of 28 patients with WML and ambulatory blood pressure recordings available 24 were non-dippers. The data show that the frequency of WML is lower in PAF patients aged 60 to 80 years compared to age matched community based samples. Although PAF usually results in hypotension, a frequent complication is supine hypertension. Although the overall frequency of WML seems to be reduced in PAF, a number of patients with elevated supine systolic blood pressure (>160 mmHg) develop WML and some of these suffer stroke.

The Movement Disorders task force review of dysautonomia rating scales in Parkinson's disease with regard to symptoms of orthostatic hypotension.

Orthostatic hypotension is defined as a blood pressure fall of > 20 mm Hg systolic and/or 10 mm Hg diastolic within 3 minutes of an upright position. The Movement Disorders Society commissioned a task force to assess existing clinical rating scales addressing symptoms of orthostatic hypotension in Parkinson's disease. Seven neurologists and a clinimetrician assessed each scale's previous use and critiqued its clinimetric properties. A scale was "recommended" if it had been applied to populations of patients with Parkinson's disease, with data on its use in studies beyond the group that developed the scale, and was found to be clinimetrically valid. A scale was considered "suggested" if it had been applied to Parkinson's disease, but only 1 of the other criteria was applied. A scale was "listed" if it met only 1 criterion. Symptoms of orthostatic hypotension are generally assessed in scales on wider autonomic or nonmotor symptoms. Some scales designed to detect orthostatic hypotension-related symptoms provide information on their severity: the AUTonomic SCale for Outcomes in PArkinson's Disease and the COMPosite Autonomic Symptom Scale met criteria for recommended with some limitations; the Novel Non-Motor Symptoms Scale and the Orthostatic Grading Scale were classified as suggested. The Self-completed Non-Motor Symptoms Questionnaire for Parkinson's Disease was classified as suggested as a tool for screening orthostatic symptoms. However, these and the listed scales need further validation and application before they can be recommended for clinical use in patients with Parkinson's disease.

Clinical core symptoms of posterior spinal artery ischemia.

INTRODUCTION: Ischemia in the distribution of the posterior spinal artery is rare. METHODS: Between 2004 and 2008 we observed 4 patients with posterior spinal artery ischemia (PSAI). RESULTS: All patients had the expected loss of posterior column function but also suffered from motor deficits which were severe, and the leading symptom in 2 patients. Severe pain, bowel and bladder dysfunction were other concomitant symptoms. In 3 of 4 patients, a follow-up spinal MRI was needed to show the cord infarct together with vertebral body ischemia in 2 patients. CONCLUSION: An acute onset of posterior column dysfunction with a variable degree of motor deficit and dorsalgia suggests PSAI.

[BIPAP-mask-ventilation in terminal amyotrophic lateral sclerosis (ALS)]. / Die BIPAP-Maskenbeatmung in der Terminalphase der amyotrophen Lateralsklerose (ALS) - Wenn das Sterben zu Hause von einem medizinischen Gerät begleitet wird.

Amyotrophic lateral sclerosis (ALS) is a progressive, neuromuscular disease without any curative therapy at the moment. Non-invasive BIPAP-ventilation has proven to be helpful to cope with the increasing hypoventilation due to weakness in ventilatory muscles in ALS. If BIPAP-ventilation is well tolerated by the patient, it can be very helpful for symptom control in palliative homecare. In this case presentation we discuss how non-invasive ventilation can influence perception of dying at home and thereby quality of life of patients, caregivers and members of palliative teams. We will see that especially during this sensible terminal phase various questions and fears concerning the mechanically assisted ventilation may arise. These issues should be addressed in advance with careful attention and information. Members of palliative teams should therefore be provided with good training and regular supervision.

Myeloid sarcoma in the central nervous system: case report and review of the literature.

Myeloid sarcomas are rare manifestations of mainly myeloblastic leukemia. Their occurrence in the central nervous system is exceptional and current literature is limited to case studies. A case is added herewith and a review was performed to investigate clinical characteristics and treatment options of central nervous system myeloid sarcoma. A 61-year-old female with acute myeloblastic leukemia (FAB M5) and progressive left sided hemiparesis showed a right parieto-occipital epidural lesion mimicking meningioma. Partial resection was performed to reveal a myeloid sarcoma. Reviewing the literature we identified 44 cases with sufficient description of the diagnosis, treatment and follow up to one year. In these patients different treatment regimens were applied. However, when systemic chemotherapy or irradiation was included in the treatment regimen, patients showed the best 1-year survival proportion.

The end-of-life hospital setting in patients with glioblastoma.

Despite aggressive treatment, outcome of patients with glioblastoma is poor. Several distinct clinical problems arise in the terminal stage of this disease. The purpose of this study was to evaluate the end-of-life phase in a hospital setting in patients with glioblastoma. Twenty-nine consecutive patients with glioblastoma, who died in our department, were included in this analysis regarding symptoms, medication, diagnostics, and interventional procedures. The patients were comparable with respect to age, gender, and overall survival with data from the literature. Relevant clinical symptoms, medications, diagnostics, well as interventional procedures increased continuously toward end of life. Pain, epileptic seizures, and symptoms of brain edema were the most frequent clinical symptoms. According to this, most patients were on antiepileptic drugs (AED), steroids, and analgesics. In the last phase, symptoms from brain edema, fever, decrease of vigilance, dysphagia, and pneumonia were the prominent clinical features. Our study demonstrates that the end of life in patients with glioblastoma has several periods with different clinical aspects with respect to symptoms and treatment.

[Supportive care and palliation in patients with malignant gliomas]. / Supportive Therapie und Palliativmedizin bei Patienten mit malignen Gliomen.

The primary aim of supportive care in patients with malignant gliomas is accurate symptom control in order to optimize quality of life. The palliative approach should be considered from the beginning of treatment, due to the intractable and incurable character of the disease. Neurological signs and symptoms have to be investigated clinically, as well as neuroradiologically in order to evaluate treatment response and to provide adequate supportive care.

[Amyotrophic lateral sclerosis--when planning is almost too late]. / Amyotrophe Lateralsklerose--wenn die Planung fast zu spät kommt.

Amyotrophic lateral sclerosis (ALS) is a disease with progressive muscle weakness, also affecting respiratory muscles. In the terminal phase most patients experience a progression. Nutrition, speech and breathing capacity decrease. It is important to inform the patient and relatives in time and to give them a chance to decide. "Care Planning" and "Advance Directives" especially concerning ventilation reduces fear and helps the doctors and carers to decide, following the will of the patient. Nobody knows the speed of the progression. The patient in this case had few subjective symptoms at the time of the family conference. Progression till death lasted one month only. Treatment of his dyspnoe was not optimised, but during care all decisions were based on the actual will of the patient. Generally nocturnal hypoventilation, for instance non-invasive ventilation by BiPAP-mode, can relieve symptoms of dyspnoe in ALS patients. Low-dose morphine and/or benzodiazepine relieve respiratory discomfort and remove the negative spiral of dysnoe-fear-dyspnoe. Oxygen therapy is usually not needed (only in the very last stages of the disease) and is not recommended especially during the night. Hypercapnia can occur because of hypoventilation. This can cause growing unconsciousness and maybe death during sleep. Prolonging life is only possible by invasive long-term ventilation with all the problems of intensive care measures. The patient could have been given low dose morphine from the time of the family conference. Ventilation by CPAP-mode was insufficient for him.

Trigeminal neuralgia in two patients with glioblastoma.

Headache in glioblastoma patients often indicates raised intracranial pressure by either tumor edema or tumor progression. We report local glioblastoma growth causing cranial nerve lesions as well as trigeminal neuralgia, and highlight pain management in these patients.

P450 enzyme inducing and non-enzyme inducing antiepileptics in glioblastoma patients treated with standard chemotherapy.

The co-administration of antiepileptic drugs (AED) and chemotherapeutic agents in patients with glioblastoma multiforme (GBM) is common. Interactions of chemotherapeutic agents and AED have not been investigated sufficiently. The purpose of this study is to evaluate the effects of enzyme inducing (EI-AED) and non-EI-AED in patients with GBM treated with standard chemotherapeutic agents on survival and haematotoxicity. One hundred and sixty eight glioblastoma patients with standard treatment including surgery, radiotherapy and chemotherapy were retrospectively analysed. Patients were separated into three groups: Group A patients without AED (n=88), Group B patients with EI-AED (n=43), and Group C patients with non-EI-AED (n=37). CCNU was the most frequently used first-line drug in all three groups (Group A: 77%; Group B: 81%; Group C: 78%). Second line treatment, mainly temozolomide, was applicated in 58 of patients and third-line treatment in 9. Carbamazepine was the most frequently administered AED in Group B (81%) and valproic acid in Group C (85%). For statistical analysis, only patients with CCNU first line treatment were calculated. A significant difference regarding survival was detected between Group B (10.8 month) and Group C (13.9 month), as well as increased haematotoxicity for Group C. These results indicate that AED influence the pharmacokinetics of chemotherapeutic drugs in patients with GBM. Valproic acid might be responsible for increasing haematotoxicity. Whether the difference regarding survival between Group B and Group C is due to a decrease of efficacy of chemotherapeutic agents by EI-AED, or due to increased efficacy of chemotherapeutic agents caused by the enzyme inhibiting properties of valproic acid, has to be evaluated in future studies.

Expiratory muscle weakness and assisted cough in ALS.

Elimination of airway secretion is a major issue in the care of patients with ALS. Sufficient cough flows have to be generated by expiratory muscles to allow airway clearance. Bulbar and expiratory muscle weakness are often reasons for failure of non-invasive ventilation (NIV) and may lead to tracheostomy. Expiratory aids may help to overcome these problems, at least for some time. We report a patient with advanced ALS, receiving nocturnal NIV, who gained much benefit from regular use of a mechanical in-exsufflation device.