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OBJECTIVES Aortic stenosis or regurgitation that requires operations in children often results from unicuspid valve morphology. In all paediatric patients with this anomaly, we have performed unicuspid valve repair by bicuspidization, creating a new commissure via adding patch material. This study reviewed our experience with this procedure. METHODS All patients with a unicuspid aortic valve who underwent bicuspidization at ≤18 years of age between 2003 and 2018 were evaluated. Autologous pericardium had initially been used for cusp augmentation. Since 2014, decellularized xenogeneic tissue or expanded polytetrafluoroethylene membrane has been applied. RESULTS There were 60 consecutive patients. The median operative age was 13 (1–18) years. Thirty patients had prior surgical or catheter valvuloplasties. Aortic regurgitation, aortic stenosis and their combination were present in 22, 11 and 27 patients, respectively. Autologous pericardium decellularized tissue and expanded polytetrafluoroethylene were used in 45, 11 and 4 patients, respectively, without intraoperative conversion to valve replacement. The overall survival was 96% at 10 years with 2 late deaths. Twenty patients underwent aortic valve reoperation due to patch degeneration (n = 11), suture dehiscence of the patch (n = 3), subaortic stenosis (n = 3) or other reasons (n = 3). Freedom from aortic valve reoperation was 73% and 50% at 5 and 10 years, respectively. Fifteen patients (25%) required valve replacement with pulmonary autograft (n = 14) or prosthesis (n = 1) 5.2 (0.2–13) years after bicuspidization at the age of 19 (10–32) years. CONCLUSIONS Bicuspidization is a safe and reproducible alternative to valve replacement with acceptable freedom from reoperation. It can serve as a bridge to valve replacement providing superior outcomes in adults.

Aortic valve-sparing operations are meant to stabilize the root in order to provide stable aortic valve function. The possibility of graft dilatation has been known in other forms of aortic replacement but is largely ignored in valve-preserving surgery. We describe graft dilatation as a cause of failure late after aortic valve reimplantation. It was corrected by applying the concepts of aortic valve repair.

OBJECTIVE: To assess the feasibility and accuracy of seizure detection based on heart rate variability (HRV) using a wearable electrocardiography (ECG) device. Noninvasive devices for detection of convulsive seizures (generalized tonic-clonic and focal to bilateral tonic-clonic seizures) have been validated in phase 2 and 3 studies. However, detection of nonconvulsive seizures still needs further research, since currently available methods have either low sensitivity or an extremely high false alarm rate (FAR). METHODS: In this phase 2 study, we prospectively recruited patients admitted to long-term video-EEG monitoring (LTM). ECG was recorded using a dedicated wearable device. Seizures were automatically detected using HRV parameters computed off-line, blinded to all other data. We compared the performance of 26 automated algorithms with the seizure time-points marked by experts who reviewed the LTM recording. Patients were classified as responders if >66% of their seizures were detected. RESULTS: We recruited 100 consecutive patients and analyzed 126 seizures (108 nonconvulsive and 18 convulsive) from 43 patients who had seizures during monitoring. The best-performing HRV algorithm combined a measure of sympathetic activity with a measure of how quickly HR changes occurred. The algorithm identified 53.5% of the patients with seizures as responders. Among responders, detection sensitivity was 93.1% (95% CI: 86.6%-99.6%) for all seizures and 90.5% (95% CI: 77.4%-97.3%) for nonconvulsive seizures. FAR was 1.0/24 h (0.11/night). Median seizure detection latency was 30 s. Typically, patients with prominent autonomic nervous system changes were responders: An ictal change of >50 heartbeats per minute predicted who would be responder with a positive predictive value of 87% and a negative predictive value of 90%. SIGNIFICANCE: The automated HRV algorithm, using ECG recorded with a wearable device, has high sensitivity for detecting seizures, including the nonconvulsive ones. FAR was low during the night. This approach is feasible in patients with prominent ictal autonomic changes.

OBJECTIVES Some studies have suggested that root remodelling is unsuitable as valve-sparing aortic root replacement in children because of the increased risk of valve failure. This study reviewed our experience with root remodelling in children. METHODS All patients who underwent root remodelling at the age of ≤18 years between 1999 and 2016 were evaluated. In 2004, cusp effective height was measured intraoperatively and prolapse was corrected with central plication on the cusp. Suture annuloplasty was introduced after 2009 for annular dilatation. RESULTS There were 17 consecutive patients. The median age at operation was 14 (2.8–18) years. Aortic valve morphology was tricuspid in 10 patients, bicuspid in 5 patients, unicuspid in 1 patient and a pulmonary autograft in 1 patient. Marfan syndrome, Loeys-Dietz syndrome and other connective tissue diseases were present in 11, 1 and 2 patients, respectively. Five patients had more than moderate aortic regurgitation. The median graft size used for root remodelling was 24 (18–26) mm. Cusp repair and annuloplasty were performed in 15 and 14 patients, respectively. The mean follow-up time was 6.5 ± 4.3 years. One patient with preoperatively severely depressed ventricular function died in the hospital from persistent heart failure. One patient (operated on before 2004) required aortic valve reoperation due to cusp prolapse. One patient with a unicuspid valve had developed moderate aortic regurgitation, and the other 14 patients had mild or less regurgitation. The median diameter of the sinus of Valsalva at the last follow-up was 36 (30–43) mm, Z-score of 1.5 (−3.5 to 3.9). CONCLUSIONS Root remodelling can be performed in children with favourable results by appropriate cusp repair and annuloplasty.

We describe a root remodeling technique for the unicuspid and unicommissural aortic valve with a moderately hypoplastic anterior commissure. This technique achieves symmetric bicuspidization without patch insertion to cusps. Partial cusp fusion of the anterior commissure is divided, and two symmetric tongues of a Dacron graft are sutured to each center of cusp insertion lines. The cusp nadirs are relocated posteriorly, and the bicuspidized valve has two sufficiently high commissures from a new angulated virtual basal ring. To optimize valve configuration, annuloplasty and prolapse correction according to effective height concept are added. This technique is a promising option in select patients.

Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder, characterized by cafe-au-lait macules, benign neurofibromas as well as malignant peripheral nerve sheath tumours, freckling in the axillary or inguinal regions, optic glioma and Lisch nodules (iris hamartomas) and further manifestations like bone deformities etc. Additionally, NF1 patients are at increased risk of early-onset cerebrovascular diseases, the pathogenesis of which has not been clarified yet. Here we report the first case of two siblings with NF1 who suffered an acute ischemic stroke. Professionals treating NF1 patients should be aware of the elevated risk of stroke in this population. Large prospective studies are needed to establish optimal guidelines for diagnosis, monitoring and treatment of cerebrovascular disease in patients suffering from NF1, as well as to achieve a consensus on routine vascular screening in NF1.

Introduction We examined the role of ultrasound in diagnosing and localizing ulnar neuropathy (UN) at the elbow. Methods Forty-one patients referred for UN and 43 healthy subjects were included. Cross-sectional area (CSA) of the ulnar nerve was measured by ultrasound at 5 locations across the elbow and at the wrist. Patients underwent near-nerve conduction studies (N-NCS). Results Ultrasound (US) had a sensitivity of 80.5%, N-NCS had a sensitivity of 70.7%, and combined sensitivity was 85.4% in diagnosing UN. Using N-NCS as a reference standard, US reached a sensitivity of 93.1% and a specificity of 50.0% in diagnosing UN, and a sensitivity of 77.3% and a specificity of 42.9% in localizing UN at the elbow. There was a reduction in mean CSA at 3-month follow-up. Conclusions N-NCS and US are complementary methods and have the potential to increase the sensitivity for diagnosing and localizing UN at the elbow. Muscle Nerve 52: 759–766, 2015

Background Routine quantitative assessment of left ventricular (LV) volumes with echocardiography is hindered by time-consuming methods requiring a manual trace of the LV cavity from two apical two-dimensional planes. Thus, the aim of this study was to evaluate faster new semiautomatic echocardiographic methods that could represent a feasible alternative for the assessment of LV volumes and ejection fraction (EF) in clinical practice. Methods Two semiautomatic methods, the automated EF (Auto-EF) for two-dimensional echocardiography and the 4D Auto LVQ tool for three-dimensional echocardiography (3DE), were compared with the biplane modified Simpson's method and cardiac magnetic resonance (CMR) imaging in 47 patients. To evaluate the accuracy of volumetry, additional in vitro measurements using water-filled latex balloons were performed with both modalities. Results Results of balloon volumetry by echocardiography and CMR measurements were in good agreement with real balloon volumes. The mean LV EF was 45 ± 11% by Auto-EF, 45 ± 11% by 3DE, 48 ± 11% by Simpson's method, and 54 ± 12% by CMR. Linear regression and Bland-Altman analyses showed good associations for semiautomatic methods with Simpson's method (Auto-EF, r = 0.85, bias = 3%, limits of agreement [LOA] = 12%; 3DE, r = 0.79, bias = 3%, LOA = 14%), as well as with CMR (Auto-EF, r = 0.74, bias = 9%, LOA = 17%; 3DE, r = 0.73, bias = 9%, LOA = 17%). Intra- and interobserver variability were 6% and 12% with Auto-EF and 8% and 11% with 3DE, respectively. Conclusions Good correlations between semiautomatic echocardiographic parameters for assessment of LV volumes and EF could be observed when compared with Simpson's method or CMR. However, intertechnique agreement analysis of absolute LV volumes revealed considerable differences, with significant underestimation of volumes and EF with respect to CMR.

Purpose To compare the value of Dobutamine stress echocardiography (DSE) with that provided by Dobutamine Cardiac Magnetic Resonance (DCMR) for the non-invasive risk stratification of patients with suspected or known coronary artery disease (CAD). Methods Patients with suspected or known CAD underwent either DSE or DCMR using the same standardized protocol. Patient matching was then performed retrospectively for age, gender and risk factors. Outcome data including cardiac death and non-fatal myocardial infarction (defined as hard cardiac events) and ‘late' revascularization performed >90days after the diagnostic procedures were collected during at least 6months. Results Follow-up data were available in 1852 patients who completed either DSE (n=884) or DCMR (n=884) during a mean follow-up duration of 4.1±2.4 and 3.9±1.9years, respectively (p=NS). Matched patients exhibited an overall high risk profile (69±9years; 69% male, 70% history of CAD and 26% diabetes mellitus in both groups). Using multivariable analysis, both modalities successfully identified patients with inducible ischemia at higher risk for subsequent hard cardiac events, surpassing the value of conventional risk factors like age, male gender and diabetes (HR=9.2; 95%CI=5.6–14.9 for DCMR versus 2.5; 95%CI=1.7–3.7 for DSE). By testing for interaction the predictive capacity of DCMR was higher than that provided by DSE (p=0.02). Patients with negative DCMR exhibited lower event rates compared to those with negative DSE (annual hard cardiac event rate of 0.8% versus 3.2%, p=0.002). Conclusions DSE & DCMR aid the risk stratification of CAD patients. However, inducible WMA during DCMR are associated with a higher risk for subsequent cardiac events. Patients with negative DCMR on the other hand, exhibited a lower event rate compared to those with negative DSE.

Background So far, only generalized tonic-clonic seizures can be reliably detected with non-invasive wearable devices. We aimed to develop an automated seizure detection algorithm using a wearable ECG-device for detecting both GTC and focal seizures. Material and methods We recorded ECG using a dedicated wearable device (ePatch®) during long-term video-EEG monitoring. In this phase-2 clinical study, 100 patients were prospectively recruited; 43 of the patients had 126 seizures (108 focal, 18 GTC) of >20 s duration during recording (941 h training data, 2238 h test data). We analyzed 20 heart rate variability (HRV)-parameters and 6 combinations of these using either 50 or 100 R-R intervals sliding window with maximum overlapping. Each HRV-parameters cut-off value for seizure-alarm was set to 105% of the highest non-seizure period during training data of the same patient. Positive responders of seizure detection were defined, for each HRV-parameter, as patients with >66% of seizures detected. Results In total, 53.5% of the patients were responders for the best performing algorithm. In these patients, the method achieved a sensitivity of 93.1% and false detection rate of 1.1/day. An average of >50 beats/minute HR increase or decrease during seizure(s) is a positive predictor of being a responder of seizure detection (PPV: 87.0%, NPV: 90.0%), making it easy to define for which patients a reliable seizure alarm is feasible. Conclusions High sensitivity and low false positive alarm rates can be achieved with our algorithm analyzing ECG-signals using the wearable device in persons with average HR changes >50 beats/min during seizures.

BACKGROUND: Several EEG criteria have been proposed for diagnosis of non-convulsive status epilepticus (NCSE), but none have been clinically validated. We aimed to assess the diagnostic accuracy of the EEG criteria proposed by a panel of experts at the fourth London-Innsbruck Colloquium on Status Epilepticus in Salzburg, 2013 (henceforth called the Salzburg criteria).METHODS: We did a retrospective, diagnostic accuracy study using EEG recordings from patients admitted for neurological symptoms or signs to three centres in two countries (Danish Epilepsy Centre, Dianalund, Denmark; Aarhus University Hospital, Aarhus, Denmark; and Paracelsus Medical University, Salzburg, Austria). Participants were included from the Danish centres if they were aged 4 months or older, and from the Austrian centre if aged 18 years or older. Participants were sorted into two groups: consecutive patients under clinical suspicion of having NCSE (the clinical validation group) or consecutive patients with abnormal EEG findings but no clinical suspicion of NCSE (the control group). Two raters blinded to all other patient data retrospectively analysed the EEG recordings and, using the Salzburg criteria, categorised patients as in NCSE or not in NCSE. By comparing with a reference standard inferred from all clinical and para-clinical data, therapeutic response, and the final outcome, we calculated sensitivity, specificity, overall diagnostic accuracy, positive and negative predictive values, and inter-rater agreement for the Salzburg criteria. The reference standard was inferred by two raters who were blinded to the scorings of the Salzburg criteria.FINDINGS: We retrospectively reviewed EEG data from 220 patients. EEGs in the clinical validation group were recorded in 120 patients between Jan 1, and Feb 28, 2014 (Austria), and Aug 1, 2014, and Jan 31, 2015 (Denmark). EEGs in the control group were recorded in 100 patients between Jan 13 and Jan 22, 2014 (Austria) and Jan 12 and Jan 26, 2015 (Denmark). According to the reference standard, 43 (36%) of the 120 patients in the validation group had NCSE. In the validation cohort sensitivity was 97·7% (95% CI 87·9-99·6) and specificity was 89·6% (80·8-94·6); overall accuracy was 92·5% (88·3-97·5). Positive predictive value was 84·0% (95% CI 74·1-91·5) and negative predictive value was 98·6% (94·4-100). Three people in the control group (n=100) fulfilled the Salzburg criteria and were therefore false positives (specificity 97·0%, 95% CI 91·5-99·0; sensitivity not calculable). Inter-rater agreement was high for both the Salzburg criteria (k=0·87) and for the reference standard (k=0·95). Therapeutic changes occurred significantly more often in the group of patients fulfilling Salzburg criteria (42 [84%] of 50 patients) than in those who did not (11 [16%] of 70; pINTERPRETATION: The Salzburg criteria for diagnosis of NCSE have high diagnostic accuracy and excellent inter-rater agreement, making them suitable for implementation in clinical practice.FUNDING: None.

Peripheral artery disease (PAD) is a complex, often underdiagnosed illness with rising prevalence in western world countries. During the past decade there has been a rapid advance especially in the field of endovascular treatment of PAD. Here we present for the first time a case reporting on the placement of coronary stent graft in a peripheral vessel for the management of a peripheral side branch perforation. Interventional angiologists or radiologists may consider such an option for complication management after injury of smaller vessels during peripheral percutaneous interventions. Further specialization and novel options of complication management as described in our case may shift the treatment from surgical to even more endovascular treatment procedures in the future.

Nicht immer prasentiert sich die Lyme-Borreliose bei Manifestationen im Zentralnervensystem mit ihren typischen Fruh- oder Spatinfektionssyndromen. Es wird uber eine seltene Erstmanifestation—Schizophrenie—berichtet. Das Magnetresonanztomogramm (MRT) wurde wegen Therapieresistenz initiiert und zeigte vaskulitische Lasionsmuster. Die laborchemische Analyse ergab einen erhohten Borrelientiter. Bei einer zweiten Patientin war im kranialen Computertomogramm (CT) eine Enzephalitis mit raumforderndem Charakter zu erkennen und lenkte deshalb von der Verdachtsdiagnose Lyme-Neuroborreliose ab. Ein Zeckenbis war bei beiden Patienten nicht eruierbar. Zur Klarung uncharakteristischer klinisch-neurologischer Symptome fuhren die Bildgebung, der lymphozytare Liquorbefund sowie der Nachweis borrelienspezifischer Antikorper im Serum zur Zieldiagnose Neuroborreliose.

In order to demonstrate the involvement of nitric oxide synthases (NOS)--in particular the inducible isoform (iNOS)--in inflammatory processes within the nasal airways, we used organ-bath incubation to study isolated inferior turbinates and mucosa of the maxillary sinus of guinea pigs. The pattern of the expression in various substructures of the nasal mucosa was of special interest. Mucosa was incubated for 6 h with lipopolysaccharides (LPS) produced by E. coli, interleukin II (IL-2) or tumor necrosis factor-alpha (TNF-alpha). Saline was used as the control solution. Following incubation the specimens were fixed in buffered 4% formaldehyde solution over a period of 4 h. Tissues were next exposed to nicotinamide adenine dinucleotide phosphate (NADPH)-diaphorase-reaction and immunostained with specific antibodies to iNOS. Results then showed a clearly increased or initiated expression of iNOS in epithelium, glands, leucocytes and blood vessels of treated tissues in comparison to the control specimens. The inflammatory mediator LPS and the cytokines Il-2 or TNF-alpha alone were found to be capable of increasing the expression of iNOS, although the effects of LPS clearly exceeded those of the cytokines. This finding implicates iNOS-generated nitric oxide as a key factor for causing nasal swelling, secretion and obstruction during nasal infections and allergic episodes.

Background The discrimination between atrial flutter (AFlu) and atrial fibrillation (AFib) can be made difficult by an irregular ventricular response owing to complex conduction phenomena within the atrioventricular (AV) node, known as multilevel AV block. We tested the hypothesis that a mathematical algorithm might be suitable to discriminate both arrhythmias. Objectives To discriminate AFlu with irregular ventricular response from AFib based on the sequence of R-R intervals. Methods Intracardiac recordings of 100 patients (50 patients with AFib and 50 patients with AFlu) were analyzed. On the basis of a numerical simulation of variable flutter frequencies followed by 2 levels of AV block in series, a given sequence of R-R intervals was analyzed. Results Although the ventricular response displays absolute irregularity in AFib, the sequences of R-R intervals follow certain rules in AFlu. We find that using a mathematical simulation of multilevel AV block, based on the R-R sequence of 16 ventricular beats, a stability of atrial activation could be predicted with a sensitivity of 84% and a specificity of 74%. When limiting the ventricular rate to 125 beats/min, discrimination could be performed with a sensitivity of even 89% and a specificity of 80%. In cases of AFlu, the atrial cycle length could be predicted with high accuracy. Conclusion On the basis of the electrophysiological mechanism of multilevel AV block, we developed a computer algorithm to discriminate between AFlu and Afib. This algorithm is able to predict the stability and cycle length of atrial activation for short R-R sequences with high accuracy.

Right ventricular (RV) function determines long-term outcome in many cardiopulmonary diseases. However, the assessment of RV function is time-consuming and surrogate parameters derived from two-dimensional (2D) or Doppler echocardiography show poor consistency. Forty consecutive patients were examined within 30 min after magnetic resonance imaging (MRI) with comprehensive echocardiography, including strain imaging and real-time three-dimensional echocardiography. A new parameter, the RV automated systolic index (RV-ASI), was obtained from the apical four-chamber view using semi-automated border detection. RV-ASI could be assessed by 2D echocardiography in 38 of 40 patients. RV ejection fraction assessed by MRI was 48 ± 9 %, while RV-ASI was 52 ± 11 % (r = 0.74, SEE = 6 %, p

The aim of this study was to investigate the involvement of oxidative stress and apoptosis in an animal model of Meniere's disease. Endolymphatic hydrops (ELH) is generally accepted as the decisive histological characteristic of Meniere's disease. Closure of the endolymphatic duct (Kimura's method) was used to induce endolymphatic hydrops in guinea pigs. Sham-operated animals served as controls. After 4 weeks the animals operated showed a significant elevation of the hearing thresholds as measured by audiometric brainstem responses (ABR) pre- and postoperatively. Immediately after the second ABR measurement, the animals were sacrificed for further immunohistological examinations of the inner ear with specific antibodies to active caspase-3 (cas-3) as a marker for apoptosis and antibodies to 8-isoprostane (8-iso) and nitrotyrosine (NT) as indicators of oxidative stress. Compared with the sham-operated controls, hydropic cochleae showed strong immunostaining for both oxidative stress markers in spiral ganglion cells, in the blood-vessels and fibrocytes of the lateral wall, as well as in supporting cells of the organ of Corti. Activation of cas-3 in spiral ganglion cells and the lateral wall was found exclusively in hydropic cochleae. Our findings suggest that oxidative stress is involved in the development of endolymphatic hydrops and may lead to cellular damage which induces apoptosis by activation of cas-3. Apoptotic cell death might contribute to the sensorineural hearing loss found in later stages of Meniere's disease.

A 37-year-old male patient presented to the stroke unit of our neurology department with weakness and paresthesia of the right arm and slight anomia. Physical examination revealed distal palsy of both arms, predominantly on the right side. No other pathologic findings were observed. Cranial MRI revealed bilateral territorial infarction in the supply territories of the middle and posterior cerebral arteries. A 12-lead electrocardiography showed incomplete right bundle branch block, negative T waves and ST depression in II, III, aVF and V4–V6 (Fig. 1a). Transesophageal and transthoracic echocardiography revealed normal dimensions of both ventricles with regular systolic and diastolic function. No intracardiac thrombi and no patent foramen ovale could be detected at that time. HolterECG did not show paroxysmal atrial fibrillation. Carotid artery stenosis was ruled out by duplex ultrasound. Blood examination showed increased creatinine (1.59 mg/dL) and urea (60 mg/dL). The white cell count was 13.2 9 10/L, with 6.9 9 10/L eosinophils (52%). A screening for vasculitis was unremarkable aside from an ANA titer of 1:320. Stool analysis and serologic tests were unsuspicious for parasitic helminth infection and there was no apparent primary allergic disorder. Because of the drastically increased eosinophils, we suspected a myeloproliferative disorder. A bone marrow biopsy was done, showing increased myelopoiesis and megakaryopoiesis as well as massive eosinophilia. These findings led us to the diagnosis of hypereosinophilic syndrome (HES). The firstline therapy for HES is steroids [3]; so, we started treatment with prednisone. Antiplatelet therapy with aspirin was administered because of the stroke. As it may have influence on the treatment, it is necessary to further classify the disease by cytogenetic and molecular genetic analysis. During these diagnostic procedures, a chromosomal deletion on 4q12 leading to creation of the FIP1L1–PDGFRA fusion gene was detected. This gene encodes a protein displaying constitutive tyrosine kinase activity. Thus, the final diagnosis was F/P ? chronic eosinophilic leukemia (CEL) [7] and the tyrosine kinase inhibitor imatinib, which is regarded as first line therapy for this special disease subgroup [3, 7], was administered at a dose of 100 mg/day. A control echocardiography was performed 2 months later, revealing an elastic echogenic mass filling the left ventricular apex (Fig. 1b; Movie 1) and right ventricular involvement. These findings could be confirmed in further follow-up studies. Cardiac magnetic resonance (CMR) also confirmed the presence of thrombotic formations in the left and right ventricular apex (Fig. 1d). Strikingly, leukocyte count was normalized to 9.1 9 10/L with 0.5% eosinophils at this point of time. No FIP1L1–PDGFRA transcripts were detectable, so a complete molecular genetic remission was achieved. The patient remained clinically stable without symptoms. Echocardiography follow-up was continued and after 1 year of therapy with imatinib, a clear regression of cardiac involvement could be assessed, since the left and right ventricular echogenic masses could not be detected any longer (Fig. 1c; Movie 2). Chronic eosinophilic leukemia is part of the heterogenous group of HESs. As these are very rare, there are no published Electronic supplementary material The online version of this article (doi:10.1007/s00392-010-0143-9) contains supplementary material, which is available to authorized users.

To date few reports have discussed the presence and function of nitric oxide (NO) in structures of the facial nerve. We performed nicotinamide adenine dinucleotide phosphate (NADPH-d)-diaphorase-histochemistry and immunohistochemistry on the intratemporal portion of the facial nerve, including the geniculate ganglion, of guinea pigs using specific antibodies to the three known isoforms of NO synthase and soluble guanylyl-cyclase (sGC). Normal facial nerves were compared to those treated intratympanically with bacterial lipopolysaccharides (LPS) and tumor necrosis factor-alpha (TNF-alpha). Both constitutive NOS isoforms and sGC could be detected in the bipolar ganglion cells of normal animals, while the inducible isoform (iNOS or NOS II) was not found. Endothelial NOS (NOS III) and sGC were present in blood vessels and were predominantly found in the perineurial sheath and less in the endoneurium. sGC could be detected in all fibers in a cross section of the facial nerve. LPS and TNF treatment led to the detection of iNOS in the perikaryia of the geniculate ganglion and the perineural sheath. These findings imply that NO may be involved in neurotransmission at least in the visceroafferent system. NO regulates vascular tone of nutrient blood vessels in the perineural sheath and endoneurium. The presence of sGC indicates that NO acts via its second messenger cGMP. NOS II expression may be a contributing factor to facial nerve palsy via two different mechanisms: NOS II-generated NO may lead to an overstimulation of the visceroefferent nerve fibers and motor fibers of the facial nerve. Dysregulation in facial nerve blood vessels could lead to edema and elevated pressure on the nerve within its osseous canal.

The factors involved in the development of chronic inflammation and edema in nasal polyps remain to be clarified. The expression of vascular endothelial growth factor (VEGF) has been described in plasma cells, suggesting that plasma cells may play a major role in the development of edema in nasal polyps through the production of VEGF. We performed immunohistochemical analysis using specific antibodies to VEGF and to the known VEGF receptors, VEGFR-1 and VEGFR-2, on paraffin sections of human nasal polyps ( n=11) and controls of human mucosa of the normal middle turbinates ( n=6). In normal turbinate mucosa, sporadic immunostaining for VEGF was observed throughout the endothelial cells of the small veins and arteries. VEGFR-1 and VEGFR-2 expression was faint in the healthy turbinates. In nasal polyp tissues, strong immunostaining for VEGF was found in the endothelium of blood vessels and in the infiltrating perivascular inflammatory cells. Fibroblasts also stained for VEGF. Strong immunolabeling to VEGFR-1 was evident in the vascular endothelium, whereas weak to moderate VEGFR-1-staining was generally confined to scattered mononuclear round cells. Mononuclear round cells and the endothelium of capillaries revealed immunoreactivity to VEGFR-2. These findings support a role for VEGF and its receptors, VEGFR-1 and VEGFR-2, in the development and perpetuation of edema and angiogenesis in nasal polyps.

Background: We report on a 42-year-old female patient who presented with a schizophreniform disorder and complete relief of symptoms after specific therapy. Methods: Cerebrospinal fluid and magnetic resonance imaging findings led to the diagnosis of Lyme disease. Results: To our knowledge this is the first reported case with an exclusive psychiatric manifestation of Lyme disease. Conclusions: In case of first manifestation of psychotic disorder, although neurological symptoms are lacking, Lyme disease should be considered and be excluded by cerebrospinal fluid analysis.