Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity.     

Correlates of figure-ground segregation in fMRI

We investigated which correlates of figure-ground-segregation can be detected by means of functional magnetic resonance imaging (fMRI). Five subjects were scanned with a Siemens Vision 1.5 T system. Motion, colour, and luminance-defined checkerboards were presented with alternating control conditions containing one of the two features of the checkerboard. We find a segregation-specific activation in V1 for all subjects and all stimuli and conclude that neural mechanisms exist as early as in the primary visual cortex that are sensitive to figure-ground segregation.     

DOCK6 Mutations Are Responsible for a Distinct Autosomal‐Recessive Variant of Adams–Oliver Syndrome Associated with Brain and Eye Anomalies

Adams–Oliver syndrome (AOS) is characterized by the association of aplasia cutis congenita with terminal transverse limb defects, often accompanied by additional cardiovascular or neurological features. Both autosomal‐dominant and autosomal‐recessive disease transmission have been observed, with recent gene discoveries indicating extensive genetic heterogeneity. Mutations of the DOCK6 gene were first described in autosomal‐recessive cases of AOS and only five DOCK6‐related families have been reported to date. Recently, a second type of autosomal‐recessive AOS has been attributed to EOGT mutations in three consanguineous families. Here, we describe the identification of 13 DOCK6 mutations, the majority of which are novel, across 10 unrelated individuals from a large cohort comprising 47 sporadic cases and 31 AOS pedigrees suggestive of autosomal‐recessive inheritance. DOCK6 mutations were strongly associated with structural brain abnormalities, ocular anomalies, and intellectual disability, thus suggesting that DOCK6‐linked disease represents a variant of AOS with a particularly poor prognosis.     

Bipolar radiofrequency ablation of spinal tumors: predictability,safety and outcome

BackgroundBone metastases are often the cause of tumor-associated pain and reduction of quality of life. For patients that cannot be treated by surgery, a local minimally invasive therapy such as radiofrequency ablation can be a useful option. In cases in which tumorous masses are adjacent to vulnerable structures, the monopolar radiofrequency can cause severe neuronal damage because of the unpredictability of current flow.PurposeThe aim of this study is to show that the bipolar radiofrequency ablation provides an opportunity to safely treat such spinal lesions because of precise predictability of the emerging ablation zone.Study designProspective cohort study of 36 patients undergoing treatment at a single institution.Patient sampleThirty-six patients in advanced tumor stage with primary or secondary tumor involvement of spine undergoing radiofrequency ablation.Outcome measuresPrediction of emerging ablation zone. Clinical outcome of treated patients.MethodsX-ray–controlled treatment of 39 lesions by bipolar radiofrequency ablation. Magnetic resonance imaging was performed pre- and postinterventionally. Patients were observed clinically during their postinterventional stay.ResultsThe extent of the ablation zones was predictable to the millimeter because it did not cross the peri-interventional planned dorsal and ventral boundaries in any case. No complications were observed.ConclusionsAblation of tumorous masses adjacent to vulnerable structures is feasible and predictable by using the bipolar radiofrequency ablation. Damage of neuronal structures can be avoided through precise prediction of the ablation area.     

Neuron-specific enolase and tau protein as neurobiochemical markers of neuronal damage are related to early clinical course and long-term outcome in acute ischemic stroke

OBJECTIVES: Analyses of neuron-specific enolase (NSE) and tau protein in patients with hyperacute ischemic stroke, their association with infarct volume, severity of the neurological deficit, the neurovascular status and functional outcome. PATIENTS AND METHODS: In 66 consecutive patients, serial venous blood samples were taken at 3, 6, 12, 18, 24, 48, 72, 96, and 120 h after stroke onset. The neurovascular status was assessed by repetitive extra- and transcranial duplex sonography. Neurological deficits were quantified by the NIH stroke scale, and functional outcome was assessed with the modified Rankin scale (mRS). RESULTS: After a first rise within 3 h, NSE decreased followed by a secondary increase until Day 5. Tau protein concentrations showed a continuous increase from admission onward. NSE and tau release were highly correlated with severity of neurological deficits and infarct volume (P = 0.001). NSE, but not tau protein, release was associated to the neurovascular status on admission. NSE and tau protein values were significantly correlated with the functional outcome at 3 months (P < 0.001). CONCLUSION: Release kinetics of NSE and tau protein are associated with patients' clinical deficits and infarct volume, and may be used as an additional predictor of the early course and functional outcome.     

Comparing motion- and imagery-related activation in the human cerebellum: A functional MRI study

Cerebellar activation during execution and imagination of a finger movement was compared. Functional magnetic resonance imaging was used to detect cerebellar activation during execution and imagination of an untrained self-paced finger-to-thumb movement (left and right hand separately). The four fingers were opposed to the thumb in changing sequences freely chosen by the subjects. The activation maps of 10 right-handed healthy subjects were averaged after transformation into a common coordinate space. Averaged activation maps revealed strong motion-related bilateral activation in the anterior lobe of the cerebellum and in the paravermal regions of the posterior lobe. Ipsilateral activity predominated significantly. Compared to motion, imagination of the same task produced lower signal changes, and foci were more variable in position and strength. The averaged activation maps showed activity in the same regions as in motion. Activation in the posterior cerebellar lobe was more prominent extending into the lateral hemispheres. Ipsilateral dominance was significant for right-hand imagery. The left-hand task only showed marginally stronger ipsilateral activation. The activation pattern observed during execution of the finger-to-thumb movement is in agreement with theories of functional cerebellar topography. For imagery, activation at a comparable location may reflect common functionality, e.g., motor preparation and/or timing. Additional activation in the lateral hemispheres may be related to an imagery-specific function. Hum. Brain Mapping 6:105–113, 1998. © 1998 Wiley-Liss, Inc.     

Impact of acetylsalicylic acid in patients undergoing cerebral aneurysm surgery – should the neurosurgeon really worry about it?

There has been an increase in the use of acetylsalicylic acid (ASA, Aspirin®) among patients with stroke and heart disease as well as in aging populations as a means of primary prevention. The potentially life-threatening consequences of a postoperative hemorrhagic complication after neurosurgical operative procedures are well known. In the present study, we evaluate the risk of continued ASA use as it relates to postoperative hemorrhage and cardiopulmonary complications in patients undergoing cerebral aneurysm surgery. We retrospectively analyzed 200 consecutive clipping procedures performed between 2008 and 2018. Two different statistical models were applied. The first model consisted of two groups: (1) group with No ASA impact - patients who either did not use ASA at all as well as those who had stopped their use of the ASA medication in time (>?=?7 days prior to operation); (2) group with ASA impact - all patients whose ASA use was not stopped in time. The second model consisted of three groups: (1) No ASA use; (2) Stopped ASA use (>?=?7 days prior to operation); (3) Continued ASA use (did not stop or did not stop in time, <7 days prior to operation). Data collection included demographic information, surgical parameters, aneurysm characteristics, and all hemorrhagic/thromboembolic complications. A postoperative hemorrhage was defined as relevant if a consecutive operation for hematoma removal was necessary. An ASA effect has been assumed in 32 out of 200 performed operations. A postoperative hemorrhage occurred in one out these 32 patients (3.1%). A postoperative hemorrhage in patients without ASA impact was detected and treated in 5 out of 168 patients (3.0%). The difference was statistically not significant in either model (ASA impact group vs. No ASA impact group: OR?=?1.0516 [0.1187; 9.3132], p?=?1.000; RR?=?1.0015 [0.9360; 1.0716]). Cardiopulmonary complications were significantly more frequent in the group with ASA impact than in the group without ASA impact (p?=?0.030). In this study continued ASA use was not associated with an increased risk of a postoperative hemorrhage. However, cardiopulmonary complications were significantly more frequent in the ASA impact group than in the No ASA impact group. Thus, ASA might relatively safely be continued in patients with increased cardiovascular risk and cases of emergency cerebrovascular surgery.

     

Localised in vivo 1H spectroscopy of human bone and soft tissue tumours

Localised ¹H in vivo magnetic resonance spectroscopy was applied to fibrous and bone tumours before and during cytostatic treatment and radiotheraphy. The results of 24 studies in 18 patients with malignant tumours of the leg or pelvis are presented including cases of sarcoma, malignant fibrous histiocytoma, multiple myeloma, malignant lymphoma and metastasis. A double spin echo localisation method with water suppression was implemented on a 1.5 Tesla whole body unit. Voxel size was (13 mm)³ or (20 mm)³. The most common resonances besides lipids (16/18) were those of choline (10/18) and creatine (5/18). Creatine was always decreased in comparison to choline and often absent from tumour spectra. Additional resonances with phase distorsions from J-coupling (chemical shift region 1.8–2.5 and 2.2–4.0 ppm) were recorded. In the presence of lipids, lactate remained undetectable because special editing techniques were not available. Significant spectral differences between different tumour types were not evident. In about 30% of the investigations the spectra contained only water and lipid signals. Follow-up studies in three patients during radio- and chemotheraphy showed a decrease in metabolites (choline, creatine, unassigned signals between 1.0 and 2.5 ppm) after weeks and months. The decrease in choline was most pronounced paralleled by an increase in lipid/choline ratios. Correspondence to: H. BongersPresented at the European Congress of Radiology 1991 in Vienna     

Intraventricular meningioma with fatal haemorrhage: clinical and autopsy findings

Only a few cases of intraventricular meningioma have been reported and the association with intracranial haemorrhage is even rarer. More than ever, autopsy findings are scarce. Here, we report a case of primary intraventricular meningioma with intraventricular haemorrhage in a 57-year-old woman. A CT scan of the head initially suggested a malignant brain tumour as the lesion was quite inhomogeneous with hyper- and hypodense sections accompanied by fresh haemorrhage. At autopsy, the tumour was histologically diagnosed as a fibroblastic meningioma WHO-Grade I. The source of haemorrhage was most likely the tumour itself as it contained focally rather large angiomatous and additionally small cavernous vessels and acute haemorrhage in various sections. The assumptive adherence of the tumour to the choroid plexus was probably disrupted by the haematoma.