Functional hemispherectomy: postoperative motor state and correlation to preoperative DTI.

INTRODUCTION: Functional hemispherectomy (FH) is an infrequent method to reduce seizure frequency in patients with intractable epilepsy. The risk that hemispherotomy injures brain structures involved in residual motor function is challenging to predict. Our purpose was to evaluate MR diffusion tensor imaging (DTI) to preoperatively assess residual ipsilateral motor function prior to FH. METHODS: We applied DTI in 34 patients scheduled for FH to perform fiber tracking in healthy and damaged hemispheres of the corticospinal tracts (CSTs) and of the corpus callosum. We assessed the CSTs and the commissural fibers for streamline count, for fractional anisotropy (FA), and for respective ratios (affected/unaffected side). We correlated these DTI values to post-to-prior changes of muscle strength and evaluated their diagnostic accuracy. RESULTS: FA of the affected CSTs and of commissural fibers was significantly higher in patients with postoperative loss of muscle strength compared to patients without (p = 0.014 and p = 0.008). In contrast, CST FA from healthy hemispheres was not different between both groups. Ratios of streamline counts and FA from CSTs were higher in patients with postoperative reduced muscle strength compared to those without (1.14 ± 0.22 vs. 0.58 ± 0.14, p = 0.040; 0.93 ± 0.05 vs. 0.74 ± 0.03, p = 0.003). CSTs' normalized FA ratio greater than -0.085 predicted loss of muscle strength with 80 % sensitivity and 69.6 % specificity. CONCLUSION: Preoperative tracking of the CST and of commissural fibers contributes to the prediction of postoperative motor outcome after functional hemispherectomy.

Is the type and extent of hippocampal sclerosis measurable on high-resolution MRI?

INTRODUCTION: The purpose of this study is to relate hippocampal volume and FLAIR signal intensity to Wyler grading of hippocampal sclerosis (HS). METHODS: Of 100 consecutive patients with temporal lobe epilepsy and HS as histopathological diagnosis, 32 had high-resolution 3 Tesla MRI and anatomically well-preserved hippocampi following amygdalo-hippocampectomy. Hippocampal volume on 3D T1-weighted gradient echo and signal intensity on coronal FLAIR sequences were determined using FreeSurfer and SPM tools and related to Wyler grading. Seizure outcome was determined after 1 year. RESULTS: Histopathology showed four Wyler II, 19 Wyler III, and 9 Wyler IV HS. Hippocampal volumes were 3.08 ml for Wyler II (Wyler II/contralateral side: p > 0.05), 2.19 ml for Wyler III (p < 0.01), 2.62 ml for Wyler IV (p = 0.01), and 3.08 ml for the contralateral side. Normalized FLAIR signals were 1,354 (p = 0.0004), 1,408 (p < 0.0001), 1,371 (p < 0.04), and 1,296, respectively. Wyler II hippocampi were visually normal. Two of four (50%) Wyler II, 16/19 (84%) Wyler III, and 6/9 (66%) Wyler IV patients achieved Engel I outcome. CONCLUSIONS: Combined volumetry and quantitative FLAIR signal analysis clearly identifies Wyler III and IV HS. Quantitative FLAIR signal analysis may be helpful to identify Wyler II HS.

[Transient splenial lesion in influenza A H1N1 2009 infection]. / Transiente Spleniumläsion bei Influenza-A-H1N1-09-Infektion.

Severe neurologic complications have been rarely reported during novel pandemic influenza A(H1N1) virus infections. We describe the case of an 10-year-old boy with new onset seizures and proven influenza A(H1N1) 2009 infection showing a reversible hyperintense lesion in the splenium of the corpus callosum on T2-weighted and FLAIR magnetic resonance images without contrast enhancement. Transient splenial lesions have been described in the context of virus encephalopathy and do not require specific treatment.

Pattern of cortical changes in sporadic Creutzfeldt-Jakob disease.

BACKGROUND AND PURPOSE: High cortical signal intensity on diffusion-weighted (DW) or fluid-attenuated inversion recovery (FLAIR) images is increasingly described in sporadic Creutzfeldt-Jakob disease (sCJD). The aim of this study was to assess the extent and location of high cortical signal intensity, to investigate whether DW or FLAIR is superior in showing changes in cortical signal intensity, and to find out whether the distribution of the signal intensity changes is random or follows a common pattern. MATERIALS AND METHODS: We analyzed FLAIR and DW MR imaging scans of 39 patients with sCJD for hyperintense cortical signal intensity. We compared the sensitivity of the DW and FLAIR scans. We correlated the extent and location of the cortical signal intensity changes with concomitant changes in deep gray matter and the genotype of codon 129 of the prion protein gene. RESULTS: There was high signal intensity in the insula, the cingulate gyrus, and the superior frontal gyrus in 95%. The cortical areas near the midline also frequently showed the abnormal signal intensity (precuneus 87%, paracentral lobe 77%). The precentral and postcentral gyri were affected less frequently (41% and 28%, respectively). The DW MR imaging showed the cortical changes more effectively than FLAIR. There was no correlation between the distribution of changes and additional signal alterations in deep gray matter or the genotype of codon 129. CONCLUSION: The distribution of cortical signal intensity abnormalities in patients with sCJD follows a common pattern, affecting mainly the cortical areas near the midline, the insula, cingulum, and the superior frontal cortex. DW imaging is superior to FLAIR in the detection of cortical high signal intensity.

Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences.

BACKGROUND AND PURPOSE: MR imaging has played an increasingly important role in the diagnosis of Creutzfeldt-Jakob disease (CJD) since basal ganglia abnormalities on T2-weighted images have been described; thus, the aim of our study was to compare the value of different MR images in the diagnosis of CJD. METHODS: One hundred fifty-seven patients with CJD underwent MR imaging examinations. Ninety-two patients were neuropathologically confirmed, and 65 were clinically classified as having CJD through the CJD Surveillance Unit (probability of 95%). There was no standardized MR imaging protocol; thus, the examinations included 143 T2-weighted, 43 proton attenuation (PD)-weighted, 84 fluid-attenuated inversion recovery (FLAIR), and 44 diffusion-weighted images (DWI). The MR images were reviewed for pathologic changes of the basal ganglia, thalamus, and cerebral cortex. RESULTS: Cortical abnormalities were present in 70 patients (45%) and were visible in 80% (35/44) of all available DWI examinations. The basal ganglia were affected in 94 patients (60%), in particular in the caudate nucleus; the most sensitive sequences were DWI (64%) and PD-weighted (63%). A thalamic involvement was more frequently diagnosed on PD-weighted images (19%) and DWI (14%) than on FLAIR or T2-weighted images. CONCLUSION: PD-weighted images and DWI showed better results in the diagnosis of signal intensity changes in the basal ganglia compared with T2-weighted or FLAIR images; however, in the diagnosis of cortical changes, DWI was clearly superior. Our data suggest that DWI is the most sensitive MR imaging technique in the diagnosis of CJD.

Very early detection of response to imatinib mesylate therapy of gastrointestinal stromal tumours using 18fluoro-deoxyglucose-positron emission tomography.

AIM: To investigate whether 18F-FDG-PET allows early assessment of response to imatinib mesylate in GIST patients. PATIENTS AND METHODS: Five gastrointestinal stromal tumour (GIST) patients and one patient with a KIT-positive small cell cancer were studied. Treatment consisted of 400 mg imatinib mesylate daily. 18F-deoxyglucose-positron emission tomography (18F-FDG-PET) scans were done before and one week after starting imatinib mesylate therapy. RESULTS: Metabolic responses were detected after only one week of therapy in all GIST patients (four partial and one complete response). The mean decrease of the standardised uptake value was 60% (range 43 to 77%). In contrast, the tumour of the non-GIST patient was metabolically stable. Four of the 5 GIST patients achieved a partial response on CT after a mean duration of 23 weeks (range 6 to 48 weeks). CONCLUSION: 18F-FDG-PET is a valuable method for the detection of response to imatinib mesylate in patients with KIT-positive tumours as early as one week after starting therapy.

Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease.

OBJECTIVE: To evaluate the diagnostic usefulness of magnetic resonance imaging (MRI) in the clinical diagnosis of Creutzfeldt-Jakob disease (CJD). BACKGROUND: Creutzfeldt-Jakob disease is a rare neurodegenerative disease that belongs to the group of human spongiform encephalopathies and usually affects elderly people. It is clinically characterized by rapidly progressive dementia and development of neurological symptoms, such as myoclonus or ataxia. Until now, neuroradiologic investigations have only played a minor role in establishing the clinical diagnosis of CJD, and they are often performed to exclude differential diagnoses. SETTING: A university hospital, base of the German National Creutzfeldt-Jakob Disease Surveillance Study. METHODS AND PATIENTS: In this study, MRIs from suspected cases of CJD were examined by one investigator blinded to the diagnosis. Patients were classified according to the established clinical and neuropathological criteria. RESULTS: Bilateral symmetric, high signal intensities on T2-weighted MRIs were present in the basal ganglia of 109 (67%) of 162 patients with CJD. In the control group, which consisted of non-CJD dementia patients, these abnormalities on T2-weighted MRIs were found in 4 (7%) of 58 patients. This corresponds to a high specificity in the differential diagnosis of CJD. CONCLUSION: These results indicate that MRI is a useful and valuable tool with reasonable sensitivity (67%) and high specificity (93%) and should be considered as an additional cornerstone in the clinical diagnosis of CJD.

[Creutzfeldt-Jakob disease: value of MRI]. / Creutzfeldt-Jakob-Krankheit: Stellenwert der MRT.

PURPOSE: To define the role of MRI in the diagnosis of Creutzfeldt-Jakob disease (CJD). METHODS: 14 patients with suspected CJD were studied within 3 years. MRI findings were correlated with WHO established diagnostic criteria (clinical findings, EEG, CSF with 14-3-3 protein assay). RESULTS: 12 patients had CJD. One patient each suffered from Hashimoto's encephalitis and ALS dementia complex, respectively. Nine of 12 CJD patients had increased signal intensity of the striatum (n = 8), pulvinar thalami (n = 5) and/or cerebellar and cerebral cortex (n = 3), respectively. Signal intensity was most pronounced on FLAIR sequences; six patients were studied with diffusion-weighted MRI and showed impaired diffusion in these areas. Both patients without CJD did not show the abovementioned signal changes (sensitivity 75%, specificity and positive predictive value 100%, respectively). CONCLUSION: If patients with suspected CJD are studied with FLAIR and diffusion-weighted sequences, this disorder can reliably be proven or ruled out. Typical MRI findings narrow down the differential diagnosis and should be included in the WHO diagnostic criteria.

Noninvasive evaluation of cerebral arteriovenous malformations by 4D-MRA for preoperative planning and postoperative follow-up in 56 patients: comparison with DSA and intraoperative findings.

BACKGROUND AND PURPOSE: 4D-MRA is a promising technique in the diagnosis and follow-up of cAVMs. The purpose of this study was to compare 4D-MRA in the pre- and postoperative evaluation of cAVMs with DSA or intraoperative findings as the standards of reference regarding qualitative and quantitative parameters. MATERIALS AND METHODS: Fifty-six consecutive patients with cAVMs (30 women) underwent both 4D-MRA and DSA. Preoperative 4D-MRA was excluded from analysis in 1 patient (movement artifacts). Twenty-five patients underwent surgery on cAVMs and underwent both imaging modalities pre- and postoperatively. 4D-MRA was performed with either 0.5-mol/L gadolinium-diethylene-triamine pentaacetic acid (group 1: voxel size, 1.1 × 1.1 × 1.4 mm(3); 608 ms/dynamic frame; 19 patients) or 1.0-mol/L gadobutrol (group 2: voxel size, 1.1 × 1.1 × 1.1 mm(3); 572 ms/dynamic frame; additional alternating view sharing; 37 patients). Two readers independently reviewed 4D-MRA and DSA regarding the Spetzler-Martin classification, arterial feeders, and postoperative residual filling. Vessel sharpness, vessel diameter, and VBC of 4D-MRA were quantified. RESULTS: Preoperative Spetzler-Martin classification 4D-MRA and DSA ratings matched in 55/55 patients (Spetzler-Martin grades: I, 12; II, 22; III, 15; IV, 5; V, 1), and 93/100 arterial feeders were correctly identified by preoperative 4D-MRA (7 additional arterial feeders identified by DSA only: group 1, 3/19; group 2, 4/36). Postoperative 4D-MRA and DSA matched in 25/25 patients (residual filling, 1/25). Vessel sharpness and diameters did not differ substantially between the 2 groups. VBC was significantly higher in group 2 (P < .005). CONCLUSIONS: 4D-MRA is a reliable tool that allows predicting Spetzler-Martin classification and postoperative residual filling; it hence allows substituting DSA in the pre- and postoperative evaluation of patients with cerebral AVMs.

Postsurgical intracranial hypotension: diagnostic and prognostic imaging findings.

BACKGROUND AND PURPOSE: CSF loss with consecutive intracranial hypotension has been discussed as a possible pathogenetic mechanism in poor clinical outcome after uneventful neurosurgery and appears to be correlated to specific imaging findings. The purpose of this study was to describe the clinical and imaging findings of symptomatic intracranial hypotension likely induced by wound suction drainage. MATERIALS AND METHODS: This is a review of previously published cases of patients in whom this condition developed after uneventful intracranial surgery. We performed an analysis of 3 more cases, of which 2 occurred after spinal surgery with accidental dural opening. RESULTS: Sixteen patients who remained unconscious or did not become fully responsive after surgery showed symmetric bilateral thalamic/basal ganglia signal intensity changes on CT and MR imaging studies. Of these 16 patients, 4 died and 2 also had brain stem signal intensity changes. All patients had rapid and distinct intraoperative and postoperative CSF loss documented on CT and/or MR imaging studies by a transient increase of the sag ratio, defined as maximal anteroposterior midbrain diameter by maximal bipeduncular diameter. CONCLUSIONS: The clinical course and typical MR imaging findings characterize the disease entity postsurgical intracranial hypotension. These findings also underline the potential danger of wound suction drainage in the case of possible CSF loss.

Gated multidetector computed tomography. A technique to reduce intracranial aneurysm clip and coil artifacts.

BACKGROUND AND PURPOSE: Streak artifacts caused by aneurysm clips and coils impede image quality in multidetector computed tomography (MDCT). The authors propose a technique to minimize these artifacts by gated data reconstruction and shifting the reconstruction window. PATIENTS AND METHODS: Intracranial CT angiograms were acquired in the follow-up of six patients with clipped and coiled intracranial aneurysms, respectively. Images were reconstructed from four consecutive 45 degrees rotated segments with an acquisition time of 52.5 ms/segment. Data acquisition was gated via an external pacemaker cable-connected to the scanner. RESULTS: Artifact orientation could be rotated by shifting the reconstruction window and interesting vessel segments visualized without disturbing streak artifacts. This allowed to assess the posterior communicating artery origin in two cases and a middle cerebral artery aneurysm remnant in another case, respectively. However, due to a higher noise interesting vessel segments were not adjustable in another three patients. CONCLUSION: Gated MDCT is a promising technique to reduce the amount and to change the position of artifacts induced by clips or coils.

Lymphoid pneumonitis as an immune reconstitution inflammatory syndrome in a patient with CD4 cell recovery after HAART initiation.

In the era of highly active antiretroviral therapy (HAART), immune reconstitution inflammatory syndrome (IRIS) has come to present a significant clinical challenge. Following the recovery of memory T cells, latent infections may lead to clinical and laboratory deterioration despite immunological and virological reconvalescence. However, many other forms of complications after induction of HAART, which are not entirely understood, must be included in the entity of IRIS. Here we report a case of a patient complaining of respiratory distress and fever 10 days after initiating HAART. Radiologically and clinically, his findings mimicked Pneumocystis jiroveci pneumonia. However, no infectious agent could be detected, and bronchoalveolary lavage showed a high cell count (90% lymphocytes and 4% eosinophils) consistent with interstitial pneumonitis. He improved dramatically after treatment with oral steroids.

Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease.

OBJECTIVES: To describe the clinical presentation of patients with Alzheimer's disease (AD) or dementia with Lewy bodies (DLB) who were suspected of having Creutzfeldt-Jakob disease (CJD) and to investigate whether current clinical diagnostic criteria cover these atypical forms of AD and DLB. METHODS: Brains from necropsy were examined for the diagnosis of CJD at the German reference centre for spongiform encephalopathies. Symptoms and signs in patients with suspected CJD in whom necropsy showed AD (n=19) or DLB (n=12) were analysed. Their data were compared with a group of patients with CJD (n=25) to determine overlapping and discriminating clinical features. All patients were classified according to clinical diagnostic criteria for CJD, AD, and DLB. RESULTS: Demented patients were suspected of having CJD if disease was rapidly progressing and/or focal neurological signs appeared and/or an EEG showed sharp wave complexes. Myoclonus and limb rigidity were the most common neurological signs in all three dementias. DLB was not suspected in any patient, although patients with DLB showed parkinsonism (58%) and fluctuations (58%). Periodic sharp wave complexes (PSWCs) in EEG typical of CJD were found in five patients with AD and one patient with DLB. 14-3-3 Protein in CSF was detected in 20 patients with CJD, in two patients with AD, but not in any patient with DLB. Although most patients with DLB or AD met the clinical criteria for their respective diagnosis (74% and 90%), they also fulfilled criteria for CJD (42% and 58%). CONCLUSIONS: In patients with rapidly progressive dementia and focal neurological signs, CJD should be the first line diagnosis. Facing the triad dementia, myoclonus, and rigidity, AD should be considered if the disease course is longer and DLB is the differential diagnosis if parkinsonism or fluctuations are present. Findings on EEG or CSF typical of CJD do not exclude AD or DLB.

Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants.

According to the recently established molecular basis for phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD), six different phenotypes are characterized by the size of the protease-resistant fragment of the pathological prion protein (types 1 and 2) and homozygosity or heterozygosity for methionine or valine at codon 129 of the prion protein gene (designated by MM1, MM2, MV1, MV2, W1, and W2). In the present investigation, we analyzed the value of commonly used clinical tests (electroencephalogram [EEG], detection of 14-3-3 protein in cerebrospinal fluid [CSF], and hyperintensity of the basal ganglia in magnetic resonance imaging) for the clinical diagnosis in each CJD phenotype. The detection of periodic sharp and slow wave complexes in the EEG is reliable in the clinical diagnosis of MM1 and MV1 patients only. The CSF analysis for 14-3-3 protein showed high sensitivity in all analyzed subgroups with the exception of MV2 patients. Valine-homozygous patients had a negative EEG, but most had detectable levels of neuronal proteins in the CSF. The sensitivity of the magnetic resonance imaging was 70%, irrespective of the subgroup, but was particularly reliable in the clinical diagnosis of MV2 patients. The widening spectrum of diagnostic techniques in CJD is not only useful in the increased accuracy of the clinical diagnosis but should also lead to the identification of more atypical cases of sporadic CJD.