scholarly journals Cytotoxic lesion of the corpus callosum in a patient with aphasic status epilepticus

2020 ◽  
Vol 1 (1) ◽  
pp. 98-100
Author(s):  
Juan Ignacio Castiglione ◽  
Mario Emiliano Ricciardi ◽  
Catalina Bensi

A 47-year-old man with a history of aphasic seizures presented to the emergency room with a 12-hour global aphasia. Upon admission, brain MRI did not reveal acute lesions, and EEG showed sharp waves in the left frontal-temporal region. An Aphasic Status Epilepticus was diagnosed and antiepileptic treatment was initiated with adequate response. A week after the episode, a new brain MRI showed a high-signal ovoid lesion on T2-weighted and FLAIR sequences in the central part of the splenium of the corpus callosum. On diffusion-weighted images (DWI) the lesion was hyperintense with decreased apparent diffusion coefficient (ADC) values, indicating restricted diffusion consistent with a cytotoxic lesion of the corpus callosum (CLOCC). Follow-up MRI one month later showed complete image resolution. CLOCCs are secondary lesions associated with various entities in which high levels of cytokines and extracellular glutamate cause intracellular edema and reduced diffusion, a condition called cytotoxic edema, which affects vulnerable brain regions such as the splenium of the corpus callosum. In epileptic patients, CLOCCs may be due to the effect of seizures, especially prolonged ones, as well as antiepileptic treatment itself. CLOCCs are rare radiological findings and must be recognized to avoid misdiagnosis.

2020 ◽  
Vol 4 (1) ◽  
pp. 1-5
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Katia Orvin ◽  
Edna Inbar ◽  
Eldad Rechavia

Abstract Background Creutzfeldt–Jakob disease (CJD), the most common prion disease in humans, is primarily known for its adverse neurological impact and inevitable mortality. Data regarding myocardial involvement in CJD are scarce. Case summary A 54-year-old female patient, presented with progressive effort dyspnoea, was diagnosed with unexplained non-ischaemic cardiomyopathy. An extensive cardiac work-up including cardiac magnetic resonance imaging (MRI) did not reveal any underlying aetiology. Simultaneously, the patient developed involuntary limb movements and progressive cognitive decline. Thalamic high-signal abnormalities on diffusion-weighted images were apparent on brain MRI. Based on these findings, she was subsequently referred to a neurology department, where she suddenly died the day after her admission. Brain autopsy demonstrated spongiform encephalopathy. A genetic analysis performed to her son revealed a mutation in the PRNP gene; all of these were consistent with CJD. Discussion This case describes the clinical association of CJD and cardiomyopathy and the diagnosis prion-induced cardiomyopathy by exclusion. It is not inconceivable that the coexistence of these two clinical entities may be related to genetic expression and contemporaneously deposition of infectious prions in myocardial muscle and brain tissue. Awareness of this possible association could be of important public-safety concern, and merits further collaborative cardiac-neurological work-up to elucidate this phenotype among patients with unexplained cardiomyopathy with neurological symptoms that resemble CJD.


Neurology ◽  
2019 ◽  
Vol 94 (3) ◽  
pp. e232-e240 ◽  
Author(s):  
Charles Bernick ◽  
Guogen Shan ◽  
Henrik Zetterberg ◽  
Sarah Banks ◽  
Virendra R. Mishra ◽  
...  

ObjectiveThis study tests the hypothesis that certain MRI-based regional brain volumes will show reductions over time in a cohort exposed to repetitive head impacts (RHI).MethodsParticipants were drawn from the Professional Fighters Brain Health Study, a longitudinal observational study of professional fighters and controls. Participants underwent annual 3T brain MRI, computerized cognitive testing, and blood sampling for determination of neurofilament light (NfL) and tau levels. Yearly change in regional brain volume was calculated for several predetermined cortical and subcortical brain volumes and the relationship with NfL and tau levels determined.ResultsA total of 204 participants who had at least 2 assessments were included in the analyses. Compared to controls, the active boxers had an average yearly rate of decline in volumes of the left thalamus (102.3 mm3/y [p = 0.0004], mid anterior corpus callosum (10.2 mm3/y [p = 0.018]), and central corpus callosum (16.5 mm3/y [p = <0.0001]). Retired boxers showed the most significant volumetric declines compared to controls in left (32.1 mm3/y [p = 0.002]) and right (30.6 mm3/y [p = 0.008]) amygdala and right hippocampus (33.5 mm3/y [p = 0.01]). Higher baseline NfL levels were associated with greater volumetric decline in left hippocampus and mid anterior corpus callosum.ConclusionVolumetric loss in different brain regions may reflect different pathologic processes at different times among individuals exposed to RHI.


2016 ◽  
Vol 8 (3) ◽  
Author(s):  
Junko Tahara ◽  
Jun Shinozuka ◽  
Hitoshi Awaguni ◽  
Shin-ichiro Tanaka ◽  
Shigeru Makino ◽  
...  

Identical twin brothers developed mild encephalopathy at the age of 7.0 and 9.7 years (Patient 1) and 10.7 years (Patient 2). Patient 1 had influenza A at the time of his second episode, but triggering agents were not evident at the first episode. The triggering agents in Patient 2 were unclear. The neurological features of both patients included transient facial numbness, left arm paresis, dysarthria, and gait disturbance. Diffusion-weighted images from magnetic resonance imaging showed high signal levels at the splenium of corpus callosum and in the bilateral cerebral deep white matter. These results are characteristic of mild encephalitis/encephalopathy with a reversible isolated splenium of corpus callosum lesion. All three episodes were treated with a methylprednisolone pulse. Acyclovir was also administered to Patient 2 and to Patient 1 during his first episode. Patient 1 received an anti-influenza agent and intravenous immunoglobulin during his second episode. Both patients recovered completely without sequelae. Genetic factors, which may predispose identical twins to develop encephalopathy, are discussed.


2014 ◽  
Vol 6 (1) ◽  
Author(s):  
Chih-Ming Lin

Methomyl is a potent pesticide that is widely used in the field of agriculture. The systemic toxic effects of methomyl have been well described. However, the neurological effects of methomyl intoxication are not well understood. In this study, we report a 61-year-old Taiwanese man sent to our emergency department because of altered mental status. His family stated that he had consumed liquid methomyl in a suicide attempt. He was provided cardiopulmonary resuscitation because of unstable vital signs. He was then sent to an intensive care unit for close observation. On the second day of admission, he regained consciousness but exhibited irregular limb and torso posture. On the sixth day, he started to complain of blurred vision. An ophthalmologist was consulted but no obvious abnormalities could be identified. On suspicion of cerebral disease, a neurologist was consulted. Further examination revealed cortical blindness and decorticate posture. Cerebral magnetic resonance imaging (MRI) was arranged, which identified bilateral occipital regions lesions. The patient was administered normal saline and treated with aspirin and piracetam for 3 weeks in hospital. During the treatment period, his symptom of cortical blindness resolved, whereas his decorticate posture was refractory. Follow-up brain MRI results supported our clinical observations by indicating the disappearance of the bilateral occipital lesions and symmetrical putaminal high signal abnormalities. In this article, we briefly discuss the possible mechanisms underlying the cerebral effects of methomyl poisoning. Our study can provide clinicians with information on the manifestations of methomyl intoxication and an appropriate treatment direction.


2016 ◽  
Vol 48 (3) ◽  
pp. 209-216 ◽  
Author(s):  
Ebru Nur Vanli-Yavuz ◽  
Betul Baykan ◽  
Serra Sencer ◽  
Altay Sencer ◽  
Isin Baral-Kulaksizoglu ◽  
...  

Purpose. There is a lack of knowledge on consecutive patients with epilepsy associated with bilateral hippocampal sclerosis (BHS). We aimed to investigate the differentiating features of BHS in comparison with unilateral HS (UHS). Method. We investigated our database for patients with epilepsy fulfilling the major magnetic resonance imaging criteria for BHS; namely, presence of bilateral atrophy and high signal changes on T2 and FLAIR series in the hippocampi. UHS patients seen in past 2 years were included as the control group. Clinical, EEG, and other laboratory findings, data on treatment response and epilepsy surgery were investigated from their files. Results. A total of 124 patients (31 with BHS and 93 with UHS; 49 right-sided and 44 left-sided) were included. We found that 16.1% of the BHS and 18.3% of the UHS groups were not drug-refractory. A binary logistic regression analysis performed with significant clinical features disclosed that history of febrile status epilepticus, mental retardation, and status epilepticus were statistically more common in BHS group. Moreover, diagnosis of psychosis established by an experienced psychiatrist and slowing of the EEG background activity were both found significantly more frequent in BHS. 66.67% of the operated BHS patients showed benefit from epilepsy surgery. Conclusions. BHS is a heterogeneous group, showing significant differences such as increased frequencies of mental retardation, status epilepticus, febrile status epilepticus and psychosis, in comparison to UHS. In all, 16.1% of the BHS cases showed a benign course similar to the UHS group and some patients with drug-resistant epilepsy may show benefit from epilepsy surgery.


2020 ◽  
Author(s):  
Christoph Fraenz ◽  
Dorothea Metzen ◽  
Christian J. Merz ◽  
Helene Selpien ◽  
Nikolai Axmacher ◽  
...  

AbstractResearch has shown that fear acquisition, in reaction to potentially harmful stimuli or situations, is characterized by pronounced interindividual differences. It is likely that such differences are evoked by variability in the macro- and microstructural properties of brain regions involved in the processing of threat or safety signals from the environment. Indeed, previous studies have shown that the strength of conditioned fear reactions is associated with the cortical thickness or volume of various brain regions. However, respective studies were exclusively targeted at single brain regions instead of whole brain networks. Here, we tested 60 young and healthy individuals in a differential fear conditioning paradigm while they underwent fMRI scanning. In addition, we acquired T1-weighted and multi-shell diffusion-weighted images prior to testing. We used task-based fMRI data to define global brain networks which exhibited increased BOLD responses towards CS+ or CS- presentations, respectively. From these networks, we obtained mean values of gray matter density, neurite density, and neurite orientation dispersion. We found that mean gray matter density averaged across the CS+ network was significantly correlated with the strength of conditioned fear reactions quantified via skin conductance response. Measures of neurite architecture were not associated with conditioned fear reaction in any of the two networks. Our results extend previous findings on the relationship between brain morphometry and fear learning. Most importantly, our study is the first to introduce neurite imaging to fear learning research and discusses how its implementation can be improved in future research.


2020 ◽  
Author(s):  
Zhongping Zhang ◽  
Dhanashree Vernekar ◽  
Wenshu Qian ◽  
Mina Kim

Abstract Background: To investigate the effect of using an Rician nonlocal means (NLM) filter on quantification of diffusion tensor (DT)- and diffusion kurtosis (DK)-derived metrics in various anatomical regions of the human brain and the spinal cord, when combined with a constrained linear least squares (CLLS) approach.Methods: Prospective brain data from 9 healthy subjects and retrospective spinal cord data from 5 healthy subjects from a 3T MRI scanner were included in the study. Prior to tensor estimation, registered diffusion weighted images were denoised by an optimized blockwise NLM filter with CLLS. Mean kurtosis (MK), radial kurtosis (RK), axial kurtosis (AK), mean diffusivity (MD), radial diffusivity (RD), axial diffusivity (AD) and fractional anisotropy (FA), were determined in anatomical structures of the brain and the spinal cord. DTI and DKI metrics, signal-to-noise ratio (SNR) and Chi-square values were quantified in distinct anatomical regions for all subjects, with and without Rician denoising. Results: The averaged SNR significantly increased with Rician denoising by a factor of 2 while the averaged Chi-square values significantly decreased up to 61 % in the brain and up to 43% in the spinal cord after Rician NLM filtering. In the brain, the mean MK varied from 0.70 (putamen) to 1.27 (internal capsule) while AK and RK varied from 0.58 (corpus callosum) to 0.92 (cingulum) and from 0.70 (putamen) to 1.98 (corpus callosum), respectively. In the spinal cord, FA varied from 0.78 in lateral column to 0.81 in dorsal column while MD varied from 0.91 × 10−3 mm2/s (lateral) to 0.93 × 10−3 mm2/s (dorsal). RD varied from 0.34 × 10−3 mm2/s (dorsal) to 0.38 × 10−3 mm2/s (lateral) and AD varied from 1.96 × 10−3 mm2/s (lateral) to 2.11 × 10−3 mm2/s (dorsal).Conclusions: Our results show Rician denoising NLM filter incorporated with CLLS significantly increases SNR and reduces estimation errors of DT- and KT-derived metrics, providing the reliable metrics estimation with adequate SNR levels.


2016 ◽  
Vol 4 (2) ◽  
pp. 72-75
Author(s):  
Arthur Joseph ◽  
Jacob Core ◽  
Daniel Solano ◽  
Marquand Patton ◽  
Shaun Smart

Background: Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by misfolded proteins that lead to neurodegeneration and inevitable death. Classic sporadic CJD presents primarily with cognitive symptoms and ataxia without visual impairment at the onset of the illness. Seizure activity is a rare presentation of patients with sporadic CJD. Case: We present a rare case of rapidly progressive encephalopathy in a 57-year-old female who presented to the emergency department with bizarre behavior and vision deterioration. Imaging was unrevealing, and infectious and organic causes were ruled out. Electroencephalogram showed evidence of encephalopathy and non-convulsive status epilepticus. Magnetic resonance imaging conducted later displayed high signal intensity in centrum ovale. The patient’s history, results from diagnostic analyses, and clinical presentation suggested the diagnosis of CJD (sporadic type). Conclusion: Due to the low incidence and varying clinical presentations, it is difficult to include CJD in a differential diagnosis without specific analytic measures. However, for the benefit of the patient and healthcare resources, CJD needs to be quickly considered when rapid neurological decline or non-convulsive status epilepticus is not suggestive of another entity


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Abdullah Abdulruhman Aljasser

Abstract Webb-Dattani syndrome (WEDAS) is an autosomal recessive disorder caused by mutation in the ARNT2 gene characterized by frontotemporal hypoplasia, globally delayed development, and pituitary and hypothalamic insufficiency. The condition is reported to be associated with consanguinity and with Saudi Arabian ancestry. We presented twin baby girls with developmental delayment seizures, and microcephaly. They have also hypopituitarism in the form of diabetes insipidus and hypocortlisim. also they have cortical blindness. Their brain MRI shows brain atrophic changes and delayed myelination thin corpus callosum,and small pituitary gland ad absence posterior high signal spot and pituitary stalk. Genetic testing by Exome sequencing was done and it shows A homozygous variant of ARNT2 (ARNT2:NM_014862:exon3:c.147-1G&gt;A). One of this twin her condition deteriorated with uncontrolled seizures and spasticity and died at age 22 months. Conclusion: we report another cases of the ARNT2 mutation in a Saudi family illustrating the disease of webb-dattani Syndrome with seizures and hypopituitarism and severe visual impairment and global developmental delayment.


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