Optische Kohärenztomographie in der Mittelohrchirurgie

The application of optical coherence tomography (OCT) in middle ear surgery has a high diagnostic potential, especially for intraoperative evaluation of the cause of stapes fixation, intraoperative assessment of the morphology of the stapes footplate in revision stapes surgery, and as an orientation guide in cochlear implantation in congenital anomalies. OCT displays the middle and inner ear structures precisely. This technology enables the surgeon to use this information for further specification of the intraoperative modus operandi. Considering our own in vivo and ex vivo investigations and animal experiments, potential areas of application can be defined: visualization of the oval window niche in revision stapesplasty and reconstructive middle ear surgery, as well as during explorative tympanotomy for intraoperative assessment of perilymph fistula, and demonstration of structures of the exposed but not opened inner ear.     

Paradoxical response of VEGF expression to hypoxia in CSF of patients with ALS

Vascular endothelial growth factor (VEGF) is implicated in motor neurone degeneration. In normal individuals, hypoxia is known to induce an overexpression of VEGF, as measured in CSF. We show that patients with ALS do not manifest this VEGF overexpression in the presence of hypoxia. Although VEGF gene expression is mainly stimulated by hypoxia, we have measured lower VEGF levels in cerebrospinal fluid (CSF) from hypoxaemic patients with amyotrophic lateral sclerosis (ALS) than in CSF from normoxaemic patients with ALS. In contrast, hypoxaemic neurological controls displayed higher levels than normoxaemic neurological controls. There was a negative correlation between VEGF levels and the severity of hypoxaemia in patients with ALS, suggesting deregulation of VEGF in ALS.     

Good outcome in adult‐onset Rasmussen's encephalitis syndrome: is recovery possible?

A healthy 29‐year‐old man suffered from adult‐onset epilepsy, characterized by polymorphic progressive seizures resistant to AEDs, leading to unilateral cortical deficits and atrophy of the left hemisphere. The disorder satisfied the clinical, EEG, and imaging criteria for a diagnosis of Rasmussen's encephalitis. During the acute phase of the disease, intrathecal synthesis of specific anti‐CMV IgG was identified. This case was characterized by a very mild course and remission of seizures following a treatment with high‐dose intravenous polyvalent immunoglobulins containing a high anti‐CMV titre. The patient remained symptomless for more than 15 years from clinical onset and more than eight years after the discontinuation of immunological therapy. In agreement with a recent report, this case confirms that adult‐onset Rasmussen's encephalitis syndrome may occur with a very mild clinical picture and persistent remission. In this case, the specific index for intrathecal production of anti‐CMV antibodies suggested possible CMV involvement, indicating specific immuno‐therapy as a treatment choice.     

Desmopressin therapy to assist the functional identification and characterisation of von Willebrand disease: Differential utility from combining two (VWF:CB and VWF:RCo) von Willebrand factor activity assays?

We performed a retrospective audit of desmopressin (DDAVP) usage to assist in the functional characterisation of von Willebrand disease (VWD). Data was evaluated for 208 patients, comprising those with VWD (Type 1 [n = 160], Type 2A [n = 19], Type 2M [n = 10]), plus 19 individuals with haemophilia or carriers of haemophilia. Laboratory testing comprised pre- and post-DDAVP evaluation of factor VIII (FVIII:C), von Willebrand factor (VWF) antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo) activity, VWF collagen binding (VWF:CB) activity, and in one laboratory an alternate VWF activity assay. In brief, combined usage of VWF:RCo and VWF:CB appears to provide improved functional characterisation and/or ‘classification’ of VWD types, in particular better differentiation of Type 2A and 2M VWD, and clearer validation of a Type 1 VWD diagnosis. Thus, (i) Type 1 VWD displayed generally good absolute and relative rises in all test parameters, although relative rises were greatest for FVIII:C and VWF:CB, and CB/Ag ratio increases overshadowed those for RCo/Ag; (ii) Type 2A VWD patients showed good absolute and relative rises in both FVIII:C and VWF:Ag, but poor absolute rises in both VWF:CB and VWF:RCo; although small rises in both CB/Ag and RCo/Ag were also observed, both ratios tended to remain below 0.7; (iii) finally, Type 2 M VWD patients generally showed good absolute and relative rises in FVIII:C, VWF:Ag and VWF:CB, but a poor absolute and relative rise in VWF:RCo; thus, there were good rises in CB/Ag ratios but little change in RCo/Ag, which tended to remain below 0.7. Future multi-centre prospective investigations are warranted to validate these findings and to investigate their therapeutic implications.     

Complete remission of cutaneous and subcutaneous melanoma metastases of the scalp with imiquimod therapy

Multiple cutaneous and subcutaneous melanoma metastases represent a therapeutic challenge. A 63‐year‐old man presented with multiple cutaneous and subcutaneous melanoma metastases on his right parieto‐occipital region that appeared ten weeks after surgical excision of the primary tumor. Staging showed no further metastases. Because of the large area of cutaneous metastatic spread, the location and the limited possibility of a complete excision, we decided to begin immunomodulatory therapy with imiquimod applied for eight hours daily five days a week. After six weeks of imiquimod monotherapy, a partial remission of the cutaneous metastases had occurred. After 17 months, the remission of these metastases was complete. Four months later the patient is still free of cutaneous, visceral, cerebral and lymph node metastases.     

Modality‐independent representations of small quantities based on brain activation patterns

Machine learning or MVPA (Multi Voxel Pattern Analysis) studies have shown that the neural representation of quantities of objects can be decoded from fMRI patterns, in cases where the quantities were visually displayed. Here we apply these techniques to investigate whether neural representations of quantities depicted in one modality (say, visual) can be decoded from brain activation patterns evoked by quantities depicted in the other modality (say, auditory). The main finding demonstrated, for the first time, that quantities of dots were decodable by a classifier that was trained on the neural patterns evoked by quantities of auditory tones, and vice‐versa. The representations that were common across modalities were mainly right‐lateralized in frontal and parietal regions. A second finding was that the neural patterns in parietal cortex that represent quantities were common across participants. These findings demonstrate a common neuronal foundation for the representation of quantities across sensory modalities and participants and provide insight into the role of parietal cortex in the representation of quantity information. Hum Brain Mapp 37:1296‐1307, 2016. © 2016 Wiley Periodicals, Inc.