Spinal intramedullary schwannomas-report of a case and extensive review of the literature.

Intramedullary schwannomas (IMS) represent exceptional rare pathologies. They commonly present as solitary lesions; only five cases of multiple IMS have been described so far. Here, we report the sixth case of a woman with multiple IMS. Additionally, we performed the first complete systematic review of the literature for all cases reporting IMS. We performed a systematic review of the literature in PubMed, EMBASE and Cochrane Central Register of Controlled (CENTRAL) to retrieve all relevant studies and case reports on IMS. In a second step, we analysed all reported studies with respect to additional cases, which were not identified through the database search. Studies published in other languages than English were included. One hundred nineteen studies including 165 reported cases were included. In only five cases, the patients harboured more than one IMS. Gender ratio showed a ratio of nearly 3:2 (male:female); mean age of disease presentation was 40.2 years; 11 patients suffered from neurofibromatosis (NF) type 1 or 2 (6.6%). IMS are rare. Our first systematic review on this pathology revealed 166 cases, including the here reported case of multiple IMS. Our review offers a basis for further investigation on this disease.

Suitability of intravascular imaging for assessment of cerebrovascular diseases.

PURPOSE: Arteriosclerosis of the vascular system is associated with many accompanying diseases. Especially cerebral arteriosclerosis is a main risk factor for ischemic strokes. We want to verify the practicability of intravascular imaging like intravascular ultrasound and optical coherence tomography for the assessment of cerebral vessel walls and plaques. METHODS: We examined 18 Circuli arteriosi willisii postmortem. The data contained 48 plaques from 48 different vessel parts. The samples underwent intravascular and histological imaging to conduct a quantitative assessment of vessel wall parameters (healthy vessel wall, thinnest vessel wall, plaque thickness and vessel diameter) as well as to qualitatively evaluate the healthy vessel wall, fibrotic plaques, calcifications and cholesterol deposits in diseased vessels. RESULTS: The comparison showed statistically significant smaller measurements for thinnest vessel walls, normal vessel walls and vessel diameters in histology than in imaging. No statistically significant difference was reached for plaque diameters. Fibrotic plaques were characterized as hyper-intense with dorsal attenuation and calcifications as hypo-intense with dorsal attenuation in optical coherence tomography. In intravascular ultrasound, fibrotic plaques showed a homogeneous echogenicity without distal attenuation and calcifications were depicted as hyperechoic with dorsal sound shadows. Cholesterol deposits were hyper-intense in optical coherence tomography with strongly attenuated signals and in intravascular ultrasound; the deposits were hyper-intense with almost no attenuation. CONCLUSION: Both intravascular methods allow for plaque characterization and quantification of plaque diameter in cerebral vessel walls. When compared with histology, a statistically significant bias was obtained for the ex vivo measurements of the normal vessel wall diameters.

Decerebrate posturing following traumatic brain injury: MRI findings and their diagnostic value.

AIM: To determine the pathomorphological and clinical background to decerebrate posturing in humans following serious traumatic brain injury. MATERIALS AND METHODS: One hundred and twenty patients who had been unconscious for more than 24 h underwent diagnostic MRI within 8 days after trauma. The presence of decerebrate rigidity as the clinical parameter was correlated to MRI findings, such as traumatic lesions in defined brain areas. Significance was presumed as p < 0.05. RESULTS: On the day of MRI 43 (36%) patients exhibited decerebrate posturing: 19 (23%) cases were unilateral and 24 (77%) bilateral. There was a significant correlation between midbrain lesions and the presence of rigidity. If a midbrain lesion was found in the absence of pontine lesions, decerebrate rigidity could be concluded (p < 0.05). There was no significant correlation to the rigidity in the case of midbrain lesions accompanied by pontine lesions, and no correlation to the rigidity could be detected for other regions of the brain. Both the occurrence of decerebrate posturing and the detection of brainstem lesions at MRI correlated with the Glasgow Outcome Scale. The combination of both parameters improved the probability of predicting the outcome. CONCLUSION: The rate of decerebrate posturing increases significantly in the presence of midbrain lesions. The presence of pontine lesions appears to be of secondary importance. The chances of predicting the Glasgow Outcome Scale are improved by the combination of clinical information (decerebrate posturing) and radiological parameters (type of brainstem lesion).

Detecting artery occlusion and critical flow diminution in the case of an acute ischemic stroke--methodological pitfalls of common vascular diagnostic methods.

PURPOSE: None of the vascular emergency diagnostic methods commonly used in the case of acute ischemic stroke, i. e. CTA, color-coded duplex sonography (CCDS), MRA, and DSA, is free of restrictions due to physical and physiological characteristics. As a result, misleading results initiating an inappropriate acute therapeutic intervention or hampering a promising one cannot be excluded. We aimed to assess the type and frequency of methodological pitfalls occurring in this situation. MATERIALS AND METHODS: We retrospectively analyzed data of 269 consecutive patients admitted to our stroke unit with a clinical syndrome of an acute stroke. All patients underwent one or more vascular emergency diagnostic methods on a routine basis. RESULTS: 37 patients were excluded because of a final diagnosis other than ischemic stroke. 76 of 232 ischemic stroke patients underwent emergency diagnostic methods with two or more vascular examination techniques. Controversial results occurred in 20 patients and related to the detection and localization of large artery occlusion and its differentiation from a low/slow flow situation and the identification of critical cerebral flow diminution distal to large artery occlusion/severe stenosis. Methodological pitfalls were able to be most reliably resolved by CCDS. Within the whole cohort of ischemic stroke patients, vascular constellations susceptible to misinterpretation were diagnosed in 40 (17.2 %) patients. CONCLUSION: We recommend providing several techniques including CCDS in an emergency stroke setting and applying techniques with respect to diagnostic findings.

[Cranial magnetic resonance imaging and serum marker S-100 for expert opinions in severe brain injuries]. / Kranielle Kernspintomographie und S-100 Serumdiagnostik in der Begut- achtung schwerer Schädelhirntraumen.

Detailed imaging of the brain is necessary to come to an expert opinion on the consequences of severe brain injuries (BI). As lesions are not visualised on computerised tomography, magnetic resonance imaging (MRI) data and serum marker S-100 are currently favoured. Our study analyses the predictive qualities of MRI and S-100 for the outcome in severe BI in 100 patients. The location of brain stem lesions identified with the aid of MRI significantly correlated with the outcome. Special types of brain stem lesions could be distinguished. S-100 was a rough measure for the amount of destruction of CNS tissue, but the exact location of the destroyed tissue appeared to be more significantly related with the outcome than its volume. For an expert opinion on severe BI, follow up MRI after brain injury can definitely not exclude relevant lesions. Early MRI is recommended.

The predictive value of ICP as compared to magnetic resonance imaging in comatose patients after head injury.

INTRODUCTION: While highly increased intracranial pressure (ICP) is of high predictive value indicating a fatal outcome, the predictive value of moderately increased ICP early after head injury remains uncertain. We compared the predictive value of ICP to the predictive value of magnetic resonance imaging (MRI) early after head injury. METHODS: 55 patients with a Glasgow Coma Scale (GCS) of less than 8, for more than 24 hours after head injury were investigated. Outcome was classified according to the Glasgow Outcome Scale (GOS). All patients received registration of ICP upon arrival at the hospital and an initial cranial computerized tomography scan. An MRI study was subsequently performed within 10 days of admission. The highest mean ICP registered within one hour in the first day of admission and the location of lesions as identified by MRI were related with outcome. RESULTS: ICP was neither related with mortality nor with GOS of survivors. The location of lesions as depicted by MRI proved to be statistically significantly related with the GOS (p < 0.001). Age proved to be clearly and significantly related with outcome (p = 0.019). CONCLUSIONS: Our current MRI findings suggest that the location of the initial brain injury lesion correlates with outcome at 6 months. No such correlation could be identified for intracranial pressure on the first day after head injury (p = 0.766).

A gesture-controlled projection display for CT-guided interventions.

PURPOSE: The interaction with interventional imaging systems within a sterile environment is a challenging task for physicians. Direct physician-machine interaction during an intervention is rather limited because of sterility and workspace restrictions. METHODS: We present a gesture-controlled projection display that enables a direct and natural physician-machine interaction during computed tomography (CT)-based interventions. Therefore, a graphical user interface is projected on a radiation shield located in front of the physician. Hand gestures in front of this display are captured and classified using a leap motion controller. We propose a gesture set to control basic functions of intervention software such as gestures for 2D image exploration, 3D object manipulation and selection. Our methods were evaluated in a clinically oriented user study with 12 participants. RESULTS: The results of the performed user study confirm that the display and the underlying interaction concept are accepted by clinical users. The recognition of the gestures is robust, although there is potential for improvements. The gesture training times are less than 10 min, but vary heavily between the participants of the study. The developed gestures are connected logically to the intervention software and intuitive to use. CONCLUSIONS: The proposed gesture-controlled projection display counters current thinking, namely it gives the radiologist complete control of the intervention software. It opens new possibilities for direct physician-machine interaction during CT-based interventions and is well suited to become an integral part of future interventional suites.

Surpass flow diverter in the treatment of intracranial aneurysms: a prospective multicenter study.

BACKGROUND AND PURPOSE: Incomplete occlusion and recanalization of large and wide-neck brain aneurysms treated by endovascular therapy remains a challenge. We present preliminary clinical and angiographic results of an experimentally optimized Surpass flow diverter for treatment of intracranial aneurysms in a prospective, multicenter, nonrandomized, single-arm study. MATERIALS AND METHODS: At 24 centers, 165 patients with 190 intracranial aneurysms of the anterior and posterior circulations were enrolled. The primary efficacy end point was the percentage of intracranial aneurysms with 100% occlusion on 6-month DSA. The primary safety end point was neurologic death and any stroke through a minimum follow-up of 6 months. RESULTS: Successful flow-diverter delivery was achieved in 161 patients with 186 aneurysms (98%); the mean number of devices used per aneurysm was 1.05. Clinical follow-up (median, 6 months) of 150 patients (93.2%), showed that the primary safety end point occurred in 18 subjects. Permanent neurologic morbidity and mortality were 6% and 2.7%, respectively. Morbidity occurred in 4% and 7.4% of patients treated for aneurysms of the anterior and posterior circulation, respectively. Neurologic death during follow-up was observed in 1.6% and 7.4% of patients with treated intracranial aneurysms of the anterior and posterior circulation, respectively. Ischemic stroke at ≤30 days, SAH at ≤7 days, and intraparenchymal hemorrhage at ≤7 days were encountered in 3.7%, 2.5%, and 2.5% of subjects, respectively. No disabling ischemic strokes at >30 days or SAH at >7 days occurred. New or worsening cranial nerve deficit was observed in 2.7%. Follow-up angiography available in 158 (86.8%) intracranial aneurysms showed 100% occlusion in 75%. CONCLUSIONS: Clinical outcomes of the Surpass flow diverter in the treatment of intracranial aneurysms show a safety profile that is comparable with that of stent-assisted coil embolization. Angiographic results showed a high rate of intracranial aneurysm occlusion.

Friedreich's ataxia with retained tendon reflexes: molecular genetics, clinical neurophysiology, and magnetic resonance imaging.

Lower limb areflexia is generally regarded as an essential criterion for the diagnosis of Friedreich's ataxia (FRDA). We describe a family with a recessive form of early-onset ataxia in which one member had a phenotype typical of FRDA whereas another, with retained tendon reflexes in the lower limbs, did not have electrophysiologic evidence of the usual severe afferent axonal neuropathy of FRDA. In contrast, somatosensory evoked potentials, eye-movement recordings, and MRI of the head and cervical cord provided results highly suggestive of FRDA in both patients. We performed genetic linkage analysis in this family, using markers tightly linked to the FRDA locus on chromosome 9. Inheritance of identical paternal and maternal genotypes by the affected members, but not by their unaffected siblings, provided supporting evidence that this disorder may result from mutation within the FRDA gene or is tightly linked to the investigated loci on chromosome 9.

Detection and follow-up of homonymous visual field defects - perimetric essentials for evaluation of spontaneous recovery.

Clinical detection and follow-up of homonymous visual field defects require appropriate perimetric procedures: since postgeniculate lesions are usually characterised by absolute scotomata, time consuming threshold methods can be replaced by supraliminal strategies with comparatively high stimulus densities. Compared with equidistant rectangular grids, a centripetal stimulus condensation represents the physiological conditions more adequately and thus is more effective. It allows one to differentiate central changes of the visual field, like macular sparing or splitting, which also interfere with reading performance. This procedure requires test points to be located to either side of the vertical meridian, rather than directly on it. Multimodal assessment of visual subfunctions (using static, kinetic or colour test points, random dot patterns or optokinetic stimulation) specifies the effect of the lesion in different channels or regions of the visual pathways. Automation of perimetric procedures and continuous monitoring of fixation are important tools, enhancing the quality of examination and follow-up. The above mentioned psychophysical techniques for detection of functional defects and documentation of eventual recovery, as well as matching neuroimaging findings, are demonstrated by illustrative cases.

Ultrasonography and contrast-enhanced MRA in ICA-stenosis: is conventional angiography obsolete?

BACKGROUND: The standard techniques for the screening and staging of internal carotid artery (ICA) stenosis are Doppler (continuous wave) and Duplex sonography. For the imaging of ICA occlusive disease, magnetic resonance angiography (MRA) is replacing digital subtraction angiography (DSA). The purpose of this observational study was to assess whether contrast enhanced MRA (CE-MRA) combined with ultrasound provided sufficient information for the planning of surgical treatment. METHODS: CE-MRA was performed in 195 patients (mean age 67.5 years) with sonographic evidence of severe ICA stenosis. The MRA examination protocol contained a heavily T1-weighted contrast bolus enhanced 3D-gradient echo sequence. The degree of stenosis was estimated retrospectively by two experienced neuroradiologists who were blinded to the sonographic findings. RESULTS: The consistency of MRA and ultrasound was sufficient to plan thrombendarterectomy in 182/195 patients. The estimations of the degree of stenosis were congruent between MRA and ultrasound in 91% of 197 vessels with high-degree carotid artery stenosis. CE-MRA evaluation had a high interobserver agreement. In 3 cases ultrasound examination diagnosed a filiform ICA stenosis which was not visible with MRA. In all these cases, DSA and the intraoperative findings revealed very short (1-2 mm), high-grade, excentric stenosis. CE-MRA correctly detected patency in 5 patients with high-grade and low-flow carotid artery stenosis, which had been regarded as occluded by ultrasound. Conversely with, in CE-MRA two occluded vessels were falsely considered as open. CONCLUSION: The combination of sonography and CE-MRA is a powerful tool for the non-invasive presurgical evaluation of the carotid arteries. DSA should be reserved for selected cases.

Clinical and genetic analysis of three German kindreds with autosomal dominant cerebellar ataxia type I linked to the SCA2 locus.

The detailed clinical, electrophysiological and imaging data of three German autosomal dominant cerebellar ataxia (ADCA) families are reported. Linkage to SCA2 was established using microsatellite markers D12S105, D12S1339(1328), D12S1304(1329) yielding a lod score exceeding +3.0 for the combined data. Analysis of the pedigree data provided evidence of anticipation as observed in other neurodegenerative disorders due to polyglutamine expansion encoded by a CAG repeat. This hypothesis was confirmed by the detection of the SCA2-specific pathological protein using the 1C2 monoclonal antibody which selectively recognizes large polyglutamine expansions and the characterization of a CAG expansion in the patients. Clinically, the families were characterized by progressive ataxia of stance, gait and limbs. Saccade velocity was markedly reduced in SCA2. Further oculomotor findings were gaze palsy, impaired smooth pursuit and reduced optokinetic reflex. Dementia and pyramidal tract signs were rather rare, while peripheral involvement (reduced or absent ankle reflexes, fasciculation-like movements, amyotrophy) was a prominent feature. Electrophysiological investigations provided evidence of sensory neuropathy of the axonal type and degeneration of the posterior columns. Imaging studies demonstrated severe shrinkage of brain-stem structures even in early stages of the disease.

Hyperexcitatory activity in visual cortex in homonymous hemianopia after stroke.

OBJECTIVES: Damage to and destruction of neural afferents result in a disruption of sensory input, which causes reduced activity in the corresponding cortical areas. Conversely, there is also evidence that lesions in the sensory pathway induce changes in the intracortical connectivity resulting in augmented cortical activity due to disinhibition. As disinhibition is assumed to be involved in the reconfiguration of neural networks, its appearance after brain lesions might be relevant for the restitution of impaired brain functions. METHODS: The effects of lesions in the visual pathway on the activity in visual cortex were studied using magnetoencephalography. In order to compare the neural activity affected by the lesion with the activity associated with intact visual processing, only patients with unilateral, post-chiasmatic lesions resulting in homonymous hemianopia were examined. RESULTS: Stimulation within the scotoma resulted in reduced magnetic activity compared to the stimulation of the intact hemifield. Increased activity was observed when the border region of the scotoma was stimulated. CONCLUSIONS: It is concluded that the magnetic hyperactivity reflects cortical disinhibition induced by lesions in the visual system. Furthermore, the possible role of cortical disinhibition as a basis for cortical reorganization and as a precondition for the recovery of impaired visual functions is discussed.

Small rosarylike infarctions in the centrum ovale suggest hemodynamic failure.

BACKGROUND AND PURPOSE: Lesions in the centrum ovale may be classified as microangiopathic (lacunar) lesions and hemodynamic infarctions. To distinguish between them, a size of more than 2 cm has been postulated for hemodynamic infarctions. The reliability of this criterion was assessed with MR imaging. METHODS: In 16 patients with unilateral or bilateral occlusion or high-grade stenosis of the internal carotid artery (ICA), CO2 testing revealed an ipsilateral hemodynamic failure. Each hemisphere in these patients was assessed separately for the presence and size of centrum ovale lesions. RESULTS: Five of the 16 patients suffered from large cortical infarctions with a probable embolic pathogenesis. In the remaining 11 patients (22 hemispheres), a hemodynamic failure was found in 15 hemispheres, due to occlusion (13 hemispheres) or high-grade ICA stenosis (two hemispheres). MR imaging revealed centrum ovale infarctions with a size of more than 2 cm in three of the 15 hemispheres. In eight hemispheres, multiple small lesions (< 1.5 cm; three to 30 per hemisphere) could be found with a rosarylike or sickle-shaped distribution. In none of these eight cases did MR images show lacunar infarctions in the typical regions of the brain. CONCLUSION: Our results favor the assumption that the MR finding of multiple small (< 1.5 cm) rosarylike lesions in the centrum ovale seems to be typical in patients with hemodynamic failure due to severe ICA disease.

Spontaneous occlusion of a cerebral arteriovenous malformation: angiography and MR imaging follow-up and review of the literature.

We present the angiographic and MR imaging course of a 62-year-old man with a right parietal high-flow arteriovenous malformation (AVM), which was diagnosed because of seizures. A spontaneous, complete, and asymptomatic occlusion of the AVM was confirmed by a second angiography 3 months later. The possible mechanisms leading to the occlusion are discussed, and a brief review of the literature is given.

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.

Lesion location influences perception of homonymous scotomata during flickering random dot pattern stimulation.

An attempt was made to clarify whether the site of postchiasmal lesions affects subjective perception of homonymous visual field defects during stimulation with flickering random dot patterns (white noise-field). Out of 56 patients with homonymous hemianopia, 38 (68%) perceived scotomata in this situation, but 18 (32%) discerned none at all. Neuroradiologic superposition of cerebral lesions detected by computed tomography (CT) or magnetic resonance imaging (MRI) showed that nearly all patients who perceived their scotomata had lesions involving the primary visual cortex or the perigeniculate region, whereas those who received no scotoma had lesions centered within the optic radiation. Functional MRI of six normal subjects during stimulation with flickering random dot patterns indicated predominant activation of the primary visual cortex. Since noise-field defects were most frequently perceived by patients whose lesion involved the primary visual cortex, it appears that the sensitivity of noise-field campimetry depends on the site of damage in the visual pathway. The explanation for this may be that damage to long-range horizontal connections impairs filling-in processes.

Localized in vivo 1H spectroscopy of human skeletal muscle: normal and pathologic findings.

To obtain high signal to noise ratio in small volume elements (8 cm3), in vivo 1H NMR spectroscopy of normal and diseased human skeletal muscle was performed using a double spin-echo localization method on a 1.5-T whole body system. High resolved spectra of normal calf muscle show the well known resonances of lipids (methyl, methylene, olefinic, and other fatty acid resonances), creatine/phosphocreatine, choline/carnitine, taurine, and histidine with good intraindividual reproducibility. Pronounced intraindividual differences in the lipid range were found between different upper thigh muscle groups. On pathologic conditions like myopathy, myositis or irradiation damage the spectral lipid content was increased. Three months after local irradiation of the medial vastus muscle (50 Gy), the localized 1H NMR spectrum showed a complete loss of the choline and creatine signals. In a case of M. Behçet with muscular involvement the relative reduction of the choline signal may provide an insight in the pathobiochemistry. The results of our investigations in nine healthy volunteers and three patients are presented in detail including relaxation times of the metabolites.

Localized Larmor frequency-guided fat and water proton MRI of the spine: a method to emphasize pathological findings.

A measurement protocol providing a correct adjustment of the irradiation frequencies for well separated fat and water images of the lumbar spine is presented. To determine accurately the Larmor frequencies of water and fat protons within the vertebral bodies, a volume selective spectrum of a volume element (13 mm)3 located in a lumbar vertebral body was acquired with the 90 degrees-180 degrees-180 degrees double spin-echo method. These Larmor frequencies are used to adjust the frequency-selective pulse of the SENEX chemical-shift imaging sequence. This procedure provides well separated fat and water images for a large field of view even in the inhomogeneous region of the vertebral column. Their clinical importance is demonstrated by localized Larmor frequency-guided (LLFG) SENEX 1H images of the lumbar spine in healthy persons of different age and in a patient with acute myeloid leukemia.