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Progressive multifocal leukoencephalopathy (PML) may develop in follicular lymphoma patients treated with bendamustine-rituximab. In this report, treatment with pembrolizumab successfully inhibited the clinical progression of PML by promoting radiologically demonstrated immune restoration inflammatory syndrome (IRIS), allowing complete clearance of the virus. These findings may further support the use of pembrolizumab in PML with special consideration for the potential occurrence of IRIS.

Magnetic resonance imaging is of paramount importance in the presurgical evaluation of drug resistant epilepsy. Detection of a potentially epileptogenic lesion significantly improves seizure outcome after surgery. To optimize the detection of subtle lesions, MRI post-processing techniques may be of essential help. In this study, we aimed to evaluate the detection rate of the voxel-based morphometric analysis program (MAP) in a prospective trial. We aimed to study the MAP+ findings in terms of their clinical value in the decision-making process of the presurgical evaluation. We included, prospectively, 21 patients who had negative MRI by visual analysis. In a first step, results of the conventional non-invasive presurgical evaluation were discussed, blinded to the MAP results, in multidisciplinary patient management conferences to determine the possible seizure onset zone and to set surgical or invasive evaluation plans. Thereafter, MAP results were presented, and the change of initial clinical plan was recorded. All MAP detections were reaffirmed by a neuroradiologist with epilepsy expertise. For the 21 patients included, mean age at the time of patient management conference was 26 years (SD 15 +/- years, range: 5-54 years). In total, 4/21 had temporal lobe epilepsy and 17/21 had extra-temporal lobe epilepsy. MAP was positive in 10/21 (47%) patients and in 6/10 (60%) a diagnosis of focal cortical dysplasia was confirmed after neuroradiologist review, corresponding to a 28% detection rate. MAP+ findings had a clear impact on the initial management in 7/10 patients (7/21, 33% of all patients), which included an adaptation of the intracranial EEG plan (6/7 patients), or the decision to proceed directly to surgery (1/7 patients). MRI post-processing using the MAP method yielded an increased detection rate of 28% for subtle dysplastic lesions in a prospective cohort of MRI-negative patients, indicating its potential value in epilepsy presurgical evaluation.

Contains fulltext : 282718.pdf (Publisher’s version ) (Closed access) BACKGROUND AND PURPOSE: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by clinical manifestations involving the brain, eye and skin. SWS is commonly caused by somatic mutations in G protein subunit Alpha Q (GNAQ). Five cases of subunit Alpha 11 (GNA11) mutations have been reported. We studied phenotypic features of GNA11-SWS and compared them with those of classic SWS. METHODS: Within two European multidisciplinary centers we looked for patients with clinical characteristics of SWS and a GNA11 mutation. Clinical and radiological data were collected retrospectively and prospectively. RESULTS: We identified three patients with SWS associated with a somatic GNA11 mutation. All had disseminated capillary malformation (CM) and hyper- or hypotrophy of an extremity. At birth, the CMs of the face, trunk and limbs were pink and patchy, and slowly darkened with age, evolving to a purple color. Two of the patients had glaucoma. All had neurological symptoms and moderate brain atrophy with a lower degree of severity than that classically associated with SWS. Susceptibility-weighted imaging (SWI) and contrast-enhanced fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging demonstrated the best sensitivity to reveal the pial angiomas. CONCLUSIONS: We have differentiated two distinct clinical/radiological phenotypes of SWS; GNAQ- and GNA11-SWS. The classic GNAQ-SWS is characterized by a homogeneous dark-red CM, commonly associated with underlying soft tissue hypertrophy. The CM in GNA11-SWS is more reticulate and darkens with time, and the neurological picture is milder. SWI and post-contrast FLAIR sequences appear to be necessary to demonstrate leptomeningeal angiomatosis. Anti-epileptic medication or future targeted therapies may be useful, as in classic SWS.

Primary cutaneous apocrine adenocarcinoma (PCAC) is an extremely rare neoplasm involving the sweat glands. Due to a lack of cases, there is no consensus for the systemic treatment of locally advanced or metastatic PCAC. Anti‐androgen therapy may have activity in inoperable or metastatic PCAC with high androgen receptor (AR) expression.

Purpose: Because of its paramagnetic properties, oxygen may act as an endogenous magnetic resonance imaging contrast agent by changing proton relaxation rates. Changes in tissue oxygen concentrations have been shown to produce changes in relaxation rate R1 of water. The aim of the study was to improve the sensitivity of oxygen enhanced R1 imaging by exploiting the higher solubility of oxygen in lipids (as compared with water) to sensitively monitor changes in tissue oxygen levels by selectively measuring the R1 of lipids. Methods: The method, with the acronym ‘‘MOBILE’’ (mapping of oxygen by imaging lipids relaxation enhancement), was applied in different mouse models of hypoxic processes on a 11.7 T magnetic resonance imaging system. MOBILE was compared with R*2, R1 of water, and with pO2 measurements (using electron paramagnetic resonance oximetry). MOBILE was also applied in the brain of healthy human volunteers exposed to an oxygen breathing challenge on a 3 T magnetic resonance imaging system. Results: MOBILE was shown to be able to monitor changes in oxygenation in tumor, peripheral, liver, and brain tissues. The clinical translation was demonstrated in human volunteers. Conclusion: MOBILE arises as a promising noninvasive and sensitive tool for diagnosis and therapeutic guidance in disorders involving hypoxia. Magn Reson Med 70:732–744, 2013. V C 2012 Wiley Periodicals, Inc.

Objectives Several prognostic factors influencing the recovery from olfactory dysfunction have been described. The aim of this study was to investigate whether olfactory bulb volume could be used as a new predictor of olfactory recovery in postinfectious and posttraumatic olfactory loss. Study Design Cohort study; Level of evidence, 4. Setting Tertiary university clinic, department of otolaryngology. Subjects and Methods A cohort of 60 patients with postinfectious (n = 28) and posttraumatic olfactory loss (n = 32) was investigated. Assessment of olfactory function was performed using orthonasal (Sniffin' Sticks test) and retronasal psychophysical olfactory tests, at the time of the diagnosis (t1) and 15 months later (t2). All patients were examined on 3 tesla magnetic resonance imaging, and the olfactory bulbs volume was assessed using planimetric contouring at the time of the diagnosis (t1). Results Recovery rate was 25% in patients with posttraumatic olfactory loss and 36% in patients with postinfectious olfactory loss. There was a correlation between both orthonasal and retronasal olfactory testing and the initial measurement of the total olfactory bulb volume. In addition, we observed a significant correlation between changes in olfactory functions and initial measurement of the total olfactory bulb volume, with larger volumes relating to higher improvement of olfactory function. Finally, we found that none of the patients with a total olfactory bulb volume of 40 mm(3) or less exhibited recovery of olfactory function. Conclusion Olfactory bulb volume seems to be a predictor of olfactory recovery in patients with postinfectious and posttraumatic olfactory loss.

This report describes cartilaginous regeneration in a cricoarytenoid joint affected by spondyloarthropathy using tumor necrosis factor-alpha (TNF-α) blockade, monitored by magnetic resonance (MR) and computed tomography (CT) imaging. This case is interesting for several reasons. It is only the eighth case of destructive ankylosing spondylitis-related cricoarytenoid arthritis published in the English language literature. It describes, for the first time, full recovery of vocal cord mobility following TNF-α blockade. It is also the first case to be published with MR imaging demonstrating regeneration of the cricoarytenoid cartilage following treatment. This case represents a landmark in the treatment of patients presenting with destructive arthritis involving the cricoarytenoid joint.

OBJECTIVE: The aims of this study were to evaluate olfactory function with orthonasal and retronasal testing in patients with posttraumatic olfactory loss and to investigate the relation between residual olfactory function and olfactory bulb (OB) volume. METHOD: A retrospective study of 25 patients with posttraumatic olfactory loss was performed. Orthonasal olfactory function was assessed with the Sniffin' Sticks test kit; retronasal olfactory function was assessed with intraorally applied odors. Magnetic resonance imaging was used to determine OB volume and cortical damage in the frontal and temporal areas. RESULTS: The main outcomes of the present study were the demonstration of a correlation between olfactory function and OB volume, which was more pronounced for retronasal than for orthonasal olfactory function; retronasal olfactory function was most affected in the patients with the most extensive cerebral damage and was least compromised in patients without such damage; OB volumes were smaller in patients with parosmia compared with those without; and the presence of parosmia was clearly associated with the presence of cerebral damage. CONCLUSION: The data confirm that OB volume is an indicator of olfactory function but, interestingly, in this study, it is largely determined by retronasal olfactory sensitivity. In addition, these results emphasize the role of higher cortical centers in olfactory function, and especially in parosmia, which may, at least in some cases, be related to lesions in the fronto-orbital and anterior temporal cortices. It would be of interest to investigate OB volume further in relation to the prognosis of the disorder.

OBJECTIVE: The study aimed to investigate whether the degree of postinfectious olfactory loss is reflected in volume of the olfactory bulb (OB). STUDY DESIGN: Retrospective study of 26 patients with postinfectious olfactory loss. MATERIALS AND METHODS: Olfactory function was assessed with the "Sniffin' Sticks" test kit, and the magnetic resonance imaging study focused on OB volume and the olfactory sulcus. RESULTS: The study revealed that 1) OB volume varies with regard to olfactory function, 2) OB volume decreases with duration of olfactory loss, and 3) patients with parosmia had smaller OB volumes than patients who did not report such smell distortions, although their overall olfactory function was not significantly different from each other. CONCLUSION: The study emphasizes that OB volume is a gauge of olfactory function.

Toxocara canis causes the visceral larva migrans syndrome in which central nervous involvement is rare. We report the case of a 40-year-old woman presenting with a subacute weakness of the right leg and dysaesthesiae in the right Th8-Th10 dermatomas. Spinal magnetic resonance imaging examination showed abnormal hyperintensity within the spinal cord. Cerebrospinal fluid analysis revealed eosinophilic pleocytosis. Antibody titres to Toxocara canis were higher in the cerebrospinal fluid than in the serum. Treatment using mebendazole led to a complete clinical recovery, normalization of cerebrospinal fluid parameters and improvement in spinal magnetic resonance imaging abnormalities.

Three different deconvolution techniques for quantifying cerebral blood flow (CBF) from whole brain T*(2)-weighted bolus tracking images were implemented (parametric Fourier transform P-FT, parametric single value decomposition P-SVD and nonparametric single value decomposition NP-SVD). The techniques were tested on 206 regions from 38 hyperacute stroke patients. In the P-FT and P-SVD techniques, the tissue and arterial concentration time curves were fit to a gamma variate function and the resulting CBF values correlated very well (CBF(P-FT) = 1.02 x CBF(P-SVD), r(2) = 0.96). The NP-SVD CBF values (i.e., original unfitted curves were used) correlated well with the P-FT CBF values only when a sufficient number of time series volumes were acquired to minimize tracer time curve truncation (CBF(P-FT) x 0.92 x CBF(NP-SVD), r(2) = 0.88). The correlation between the fitted CBV and the unfitted CBV values was also maximized in regions with minimal tracer time curve truncation (CBV(fit) = 1.00 x CBV(unfit), r(2) = 0.89). When a sufficient number of time series volumes could not be acquired (due to scanner limitations) to avoid tracer time curve truncation, the P-FT and P-SVD techniques gave more reliable estimates of CBF than the NP-SVD technique.

A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. Magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.

A robust whole brain magnetic resonance (MR) bolus tracking technique based on indicator dilution theory, which could quantitatively calculate cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MTT) on a regional basis, was developed and tested. T2*-weighted gradient-echo echoplanar imaging (EPI) volumes were acquired on 40 hyperacute stroke patients after gadolinium diethylene triamine pentaacetic acid (Gd-DTPA) bolus injection. The thalamus, white matter (WM), infarcted area, penumbra, and mirror infarcted and penumbra regions were analyzed. The calculation of the arterial input function (AIF) needed for absolute quantification of CBF, CBV, and MTT was shown to be user independent. The CBF values (ml/min/100 g units) and CBV values (% units, in parentheses) for the thalamus, WM, infarct, mirror infarct, penumbra, and mirror penumbra (averaged over all patients) were 69.8 +/- 22.2 (9.0 +/- 3.0 SD); 28.1 +/- 6.9 (3.9 +/- 1.2); 34.4 +/- 22.4 (7.1 +/- 2.7); 60.3 +/- 20.7 (8.2 +/- 2.3); 50.2 +/- 17.5 (10.4 +/- 2.4); and 64.2 +/- 17.0 (9.5 +/- 2.3), respectively, and the corresponding MTT values (in seconds) were 8.0 +/- 2.1; 8.6 +/- 3.0; 16.1 +/- 8.9; 8.6 +/- 2.9; 13.3 +/- 3.5; and 9.4 +/- 3.2. The infarct and penumbra CBV values were not significantly different from their corresponding mirror values, whereas the CBF and MTT values were (P < 0.01). Quantitative measurements of CBF, CBV, and MTT were calculated on a regional basis on data acquired from hyperacute stroke patients, and the CBF and MTT values showed greater sensitivity to areas with perfusion defects than the CBV values. J. Magn. Reson. Imaging 2000;12:400-410.

Objective: The olfactory bulb (OB) is essential for olfactory perception, and its volume is correlated to the olfactory function in normal and pathologic cases. We hypothesized that olfactory bulb volume could also be used as a new predictor of olfactory recovery.Method: A cohort of 60 patients suffering from PI (n = 28) and PT olfactory loss (n = 32) was studied. Assessment of olfactory function was performed using orthonasal and retronasal tests, at the time of the diagnosis and 15 months later. All patients were examined on a 3T-MRI. The olfactory bulb volume was calculated.Results: Recovery rate was 25% in patients with PT olfactory loss and 35.7% in patients with PI olfactory loss. There was a correlation between both the orthonasal and the retronasal score, and between olfactory testing and the total olfactory bulb volume, both at T1 and at T2. In addition, we observed a significant correlation between changes in olfactory functions and initial measures of the total OB volume, with larger volumes re...