Abstract
We report a case of a rapidly progressing, relapsing-remitting, steroid-responsive granulocytic encephalitis without any signs of peripheral nervous system or other organ involvement. It ...apparently had an immune-mediated etiology that could not be attributed to any known disease entity. A 22-year-old man presented with rapidly progressive severe neurological symptoms caused by encephalitis. Examination of the cerebrospinal fluid as well as brain biopsy showed extensive accumulation of neutrophilic granulocytes with no hints of an infectious agent. Magnetic resonance imaging revealed multiple T2/FLAIR demarcated lesions. Subsequent to a steroid pulse therapy, the clinical symptoms and imaging abnormalities improved rapidly. Ten months later, the patient experienced a disease relapse, which again responded well to steroids. Forty months after the relapse, he is currently doing well on azathioprine. This case highlights that an immunosuppressive treatment should be considered in patients with extensive neutrophilic encephalitis when no infectious agent is detected. A new immune-mediated relapsing-remitting CNS disease entity might need to be considered.
Diagnostic imaging criteria of multiple sclerosis (MS) include the spatial and temporal dissemination of cerebral and/or spinal cord lesions. Magnetic resonance imaging (MRI) is the method of choice ...for initial diagnosis and follow-up disease monitoring. Current guidelines for spinal MRI recommend sagittal imaging of the spinal cord and lesion confirmation on axial planes if lesions are detected. Sagittal imaging is, however, hampered by technical (e.g. partial volume effects, motion artifacts) and anatomical (e.g. scoliosis) limitations. We hypothesized that long coverage of the spinal cord by axial image acquisition has superior diagnostic performance compared to sagittal imaging and can identify otherwise undetected lesions. Our prospective clinical study included 119 MS patients. Axial MRI revealed ~2.5-fold more lesions than the sagittal angulation (axial lesion load: 4.0 ± 2.4 vs. 1.6 ± 1.2 lesions on sagittal planes,
p
< 0.001). Importantly, 20 patients (17%) with normal sagittal MRI scans had unequivocal lesions only visible on axial planes (mean lesion number on axial planes in these patients: 2.0 ± 1.3). Moreover, 45 patients (38%) showed a discrepancy of ≥3 lesions that were found additionally on axial scans (mean difference 4.4 ± 1.7). Additionally identified lesions were on average smaller in size and located more laterally within the spinal cord. No lesion on sagittal images was missed on the axial angulation. Our study demonstrates that imaging of small axial segments for lesion confirmation is insufficient in spinal imaging. We recommend implementing a long coverage axial MRI sequence for spinal imaging of MS patients.
The endovascular treatment of wide-necked aneurysms remains challenging. The “Y”-stenting technique has been used for stent-assisted coil embolization of wide-necked bifurcation aneurysms. So far, ...this technique has been described for aneurysms of the basilar apex or the middle cerebral artery bifurcation and only for open stent systems using the Neuroform stent. We report a 52-year-old woman with recurrence of a wide-necked aneurysm of the anterior cerebral artery that was successfully retreated by stent-assisted coiling using the “Y”-stenting technique with the Enterprise stent system.
This book provides a comprehensive overview of inflammatory brain diseases from a neuroradiological point of view, encompassing both infectious (e.g., viral encephalitis and pyogenic brain abscess) ...and non-infectious (e.g., multiple sclerosis) diseases. Neuroimaging contributes greatly to the differentiation of infectious and noninfectious brain diseases and to the distinction between brain inflammation and other diseases. In order to ensure a standardized approach throughout the book, each chapter is subdivided into three principal sections: epidemiology, clinical presentation and therapy, imaging, and differential diagnosis. A separate chapter addresses technical and methodological issues and imaging protocols. All of the authors are recognized experts in their fields, and numerous high-quality and informative illustrations are included. This book will be of great value not only to neuroradiologists but also to neurologists, neuropediatricians, and general radiologists.
Summary Background and purpose CT angiography (CTA) is an increasingly used method for evaluation of stented vessel segments. Our aim was to compare the appearance of different carotid artery stents ...in vitro on CTA using different CT scanners. Of particular interest was the measurement of artificial lumen narrowing (ALN) caused by the stent material within the stented vessel segment to determine whether CTA can be used to detect in-stent restenosis. Material and methods CTA appearances of 16 carotid artery stents of different designs and sizes (4.0 to 11.0 mm) were investigated in vitro. CTA was performed using 16-, 64- and 320-row CT scanners. For each stent, artificial lumen narrowing (ALN) was calculated. Results ALN ranged from 18.77% to 59.86%. ALN in different stents differed significantly. In most stents, ALN decreased with increasing stent diameter. In all but one stents, ALN using sharp image kernels was significantly lower than ALN using medium image kernels. Considering all stents, ALN did not significantly differ using different CT scanners or imaging protocols. Conclusion CTA evaluation of vessel patency after stent placement is possible, but is considerably impaired by ALN. Investigators should be informed about the method of choice for every stent and stent manufacturers should be aware of potential artifacts caused by their stents during noninvasive diagnostic methods such as CTA.
Lynch Syndrome (LS) is a cancer-predisposing condition resulting from hereditary mutation of DNA mismatch repair genes. Gastrointestinal, urogenital, and endometrial carcinomas are well-known to ...predominantly occur in LS patients. In contrast, there are only few reports on brain tumours in the context of LS and to date intracranial tumour manifestation appear to be rather coincidental.
We present the case of a 56-year-old female developing aggressive lactotroph pituitary adenoma following a history of multiple Lynch-associated malignomas and having a confirmed
mutation. Furthermore, we performed a literature review via PubMed using the search terms 'Lynch Syndrome', 'HNPCC', 'MMR mutation' combined with 'intracranial tumour', 'sellar tumour', 'pituitary adenoma', or 'pituitary carcinoma', focusing on other reported cases and treatment regimens.
A handful of studies have indicated an increased frequency of brain tumours in the context of LS, predominantly glioblastoma and less frequently low-grade glioma or other brain tumours. Based on our literature review, we summarized the known instances of pituitary adenoma in LS patients, including the present case. Furthermore, we reviewed the common recommendation of using temozolomide (TMZ) for treatment of aggressive pituitary adenoma or carcinoma and found strong indication that it might be insufficient in LS patients, while PD-1 blockade could be a promising treatment option.
Combined with our case, there is a growing body of evidence that intracranial tumours and in particular those of the sellar region might be more prevalent in LS patients than previously assumed, due to their genetic profile substantially affecting viability and efficacy of treatment options. Clinical signs of aggressive tumour growth in combination with irresponsiveness to standard treatment in case of recurrence should lead to further diagnostic measures, because revelation of germline MMR mutations would call for an extended screening for other neoplastic manifestations and would markedly influence further treatment.
Steady-state auditory evoked fields were recorded from 15 subjects using a whole head MEG system. Stimuli were 800 ms trains of binaural clicks with constant stimulus onset asynchrony (SOA). Seven ...different SOA settings (19, 21, 23, 25, 27, 29 and 31 ms) were used to give click rates near 40 Hz.
Transient responses to each click were reconstructed using a new algorithm that deconvoluted the averaged responses to the different trains. Spatio-temporal multiple dipole modelling in relation to 3D MRI scans revealed two overlapping source components in both the left and right auditory cortex. The primary sources in the medial part of Heschl's gyrus exhibited a N19-P30-N40 m pattern. The secondary, weaker sources at more lateral sites on Heschl's gyrus showed a N24-P36-N46 m pattern. When applied to transient middle latency auditory evoked fields (MAEFs) recorded at SOAs of 95-135 ms, the primary sources imaged activities similar to the deconvoluted steady-state responses, but the secondary source activities were inconsistent.
Linear summation of the deconvoluted source waveforms accounted for more than 96% of the steady-state variance. This indicates that the primary activity of the auditory cortex remains constant up to high stimulation rates and is not specifically enhanced around 40 Hz.
There is a lack of systematic data regarding local intra-arterial fibrinolysis (LIF) of thromboemboli occurring during neuroendovascular procedures with the use of recombinant tissue plasminogen ...activator (rtPA). We report our technique for treating LIF of intracerebral thromboemboli occurring during neuroendovascular procedures.
Nine of 723 patients (1.2%) who underwent neuroendovascular procedures during the period from January 1997 to September 2002 suffered thromboembolic complications. These patients were treated by LIF with a maximum dose of 0.9 mg rtPA per kilogram body weight. Recanalization was categorized as successful (Thrombolysis in Myocardial Infarction TIMI grade 2 or 3) versus unsuccessful (TIMI grade 0 or 1), and clinical outcome was categorized as independent (Rankin Scale score 0 to 2) versus dependent or dead (Rankin Scale score 3 to 6).
The minimum time between thrombus detection and beginning of LIF was 10 minutes, and the maximum time was 90 minutes. Successful recanalization was achieved in 4 of 9 patients (44%). All 9 patients suffered cerebral ischemic infarctions, and none of the patients sustained intracerebral hemorrhage. Two patients (22%) died from malignant brain infarctions. Four patients (44%) remained moderately disabled, and 3 patients (33%) were severely disabled 3 months after LIF.
Although we used relatively high doses of rtPA, the recanalization rates and clinical outcome of LIF in our patients were not satisfactory. Strategies for the prevention of thromboemboli during neuroendovascular procedures must be improved, and novel fibrinolytic or thrombolytic techniques should be developed.
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