Characteristic brain magnetic resonance imaging abnormalities in central nervous system aquaporin-4 autoimmunity

2010 ◽  
Vol 16 (10) ◽  
pp. 1229-1236 ◽  
Author(s):  
Woojun Kim ◽  
Min Su Park ◽  
Sang Hyun Lee ◽  
Su-Hyun Kim ◽  
In Ja Jung ◽  
...  

Background: Although neuromyelitis optica has been traditionally regarded as a disease without brain involvement, brain abnormalities are not uncommon in patients with neuromyelitis optica-related disorders. Methods: We aimed to characterize the brain magnetic resonance imaging (MRI) abnormalities in neuromyelitis optica spectrum disorder patients who are seropositive for anti-aquaporin-4 autoantibody (AQP4 Ab). Of 236 consecutive patients with inflammatory demyelinating central nervous system diseases, we retrospectively analyzed MRI characteristics of 78 patients who were seropositive for AQP4 Ab. Results: For an average observational period of 6.3 years, 62 patients (79%) had brain lesions on MRI. Twenty-four patients (31%) had brain MRI abnormalities at the onset of disease, and 35 (45%) had symptomatic brain involvement. Characteristic brain MRI abnormalities were classified into five categories: (1) lesions involving corticospinal tracts (e.g. posterior limb of internal capsule and cerebral peduncle (44%); (2) extensive hemispheric lesions likely due to vasogenic edema (29%); (3) periependymal lesions surrounding aqueduct and the third and fourth ventricles (22%); (4) periependymal lesions surrounding lateral ventricles (40%); and (5) medullary lesions, often contiguous with cervical lesions (31%). Fifty-four patients (69%) showed at least one kind of brain abnormality among the five characteristic MRI lesions. Ten patients showed gadolinium-enhancing lesions, which were characterized by multiple patchy enhancing patterns with blurred margins. Conclusions: In central nervous system AQP4 autoimmunity, brain MRI abnormalities were more common than is generally appreciated and were characterized by their unique localization and configuration.

2018 ◽  
Vol 128 (2) ◽  
pp. 157-161 ◽  
Author(s):  
Tara J. Wu ◽  
Sean M. Lewis ◽  
Peak Woo

Introduction: The presentation, course, and management of a rare laryngeal manifestation of neurosarcoidosis due to central nervous system (as opposed to peripheral nervous system) injury are described. Methods: The authors present 3 cases of vocal cord paralysis as the initial symptom of isolated neurosarcoidosis at a tertiary care laryngology clinic. Results: Laryngoscopy diagnosed unilateral vocal cord paralysis. Laryngeal electromyography revealed high vagal injury, prompting workup on brain magnetic resonance imaging. On magnetic resonance imaging, 2 cases showed basilar leptomeningeal inflammation and 1 case showed a brainstem mass. Patients were found at follow-up to have severe, progressive vagal injury, with patients developing severe quality of life impairments and medical complications. Conclusions: Neurosarcoidosis is not usually considered in the differential diagnosis of vocal cord paralysis. At initial presentation, all patients lacked other cranial neuropathies and systemic sarcoidosis manifestations, making diagnosis difficult. Otolaryngologists should be aware of this rare presentation, as prompt diagnosis by brain magnetic resonance imaging with or without central nervous system biopsy, as opposed to traditional chest radiography or computed tomography for the workup of peripheral nerve injury, is necessary. Serial laryngeal examinations are recommended for close monitoring of progressive disease and recommending treatment. Injection or medialization laryngoplasty can provide improvements in voicing but not swallow.


2021 ◽  
pp. 189-191
Author(s):  
Catalina Sanchez Alvarez ◽  
Kenneth J. Warrington

A 53-year-old man with hypertension, hyperlipidemia, and a remote, cryptogenic, multifocal, posterior circulation ischemic stroke, came to the emergency department with 1 day of vertigo, ataxic gait, nausea, occipital headache, and painless binocular diplopia. Symptoms were present upon awakening on the day of presentation and progressed throughout the day. The erythrocyte sedimentation rate was 10 mm/h and C-reactive protein level was less than 3 mg/L. Cerebrospinal fluid examination indicated mild lymphocytic pleocytosis with 9 cells/µL, protein value of 45 mg/dL, and glucose level within normal limits. Brain magnetic resonance imaging and magnetic resonance angiography with contrast demonstrated a left caudate head infarction and leptomeningeal and perivascular enhancement involving bilateral temporal lobes, basal ganglia, and frontal lobes. Right middle cerebral artery wall enhancement was also noted. Conventional cerebral angiography showed diffuse dilatation and mural irregularity of the right middle cerebral artery M1 segment, as well as dilatation of the first 2 mm of the left anterior cerebral artery A1 segment. These findings were associated with vessel wall gadolinium enhancement on magnetic resonance imaging, which raised concern for vasculitis. The patient was diagnosed with primary angiitis of the central nervous system. The diagnosis was based on the presence of multiple ischemic infarcts, without cardioembolic source, abnormal brain magnetic resonance imaging and magnetic resonance angiography findings consistent with vasculitis, and the absence of systemic vasculitis, infection, and cancer. After diagnosis, the patient was started on intravenous methylprednisolone, followed by oral prednisone and intravenous cyclophosphamide. Because of some new areas of enhancement on magnetic resonance imaging, the patient was subsequently treated with rituximab. Clinical and radiologic remission was achieved, although the patient had permanent residual gait difficulties. Central nervous system vasculitis is an inflammatory process of the blood vessels in the brain, meninges, and spinal cord. It is called primary angiitis of the central nervous system when the process is limited to the brain and, rarely, the spinal cord. In other circumstances, central nervous system vasculitis can be secondary to a systemic inflammatory syndrome or infectious process.


2007 ◽  
Vol 14 (2) ◽  
pp. 248-251 ◽  
Author(s):  
JA Cabrera-Gómez ◽  
A. Saiz-Hinarejos ◽  
F. Graus ◽  
A. González-Quevedo ◽  
R. Rodríguez-Rojas ◽  
...  

We studied cranial magnetic resonance imaging (MRI) lesions in three women with acute attacks of recurrent longitudinally extensive transverse myelitis (r-LETM), recurrent-optic neuritis (r-ON) and r-LETM-CNS. Neuromyelitis Optica -immunoglobulin (IgG) antibody was positive in all cases. Brain MRI (1.5 Tesla) was performed according to protocol from consortium MS centre. We described the cranial lesions in brain MRI of acute relapses. These lesions were different from MS, most had an asymptomatic course which disappeared with time, protocol from consortium of MS centre criteria for brain MRI and seropositivity of NMO-IgG are useful tools for differentiate acute lesions of NMO/MS. Multiple Sclerosis 2008; 14: 248—251. http://msj.sagepub.com


2007 ◽  
Vol 13 (2) ◽  
pp. 186-192 ◽  
Author(s):  
José A Cabrera-Gómez ◽  
L Quevedo-Sotolongo ◽  
A González-Quevedo ◽  
S Lima ◽  
Y Real-González ◽  
...  

Background Some studies showed abnormalities in brain magnetic resonance imaging (MRI) of relapsing neuromyelitis optica (R-NMO) from 12 to 46%. These abnormalities are described as compatible/non-compatible with multiple sclerosis (MS). Objective To describe the abnormal brain MRI lesions in R-NMO with imaging studies conducted with more sensitive white matter change techniques. Methods Thirty patients with R-NMO were selected. All MRI brain studies were performed with a 1.5-T Siemens MRI system according to the Standardized MR Imaging Protocol for Multiple Sclerosis from the Consortium of MS Centers Consensus Guidelines. Results Brain MRI images were evaluated in 29 R-NMO cases because in one case the MRI images were not appropriate for the study. Of these 29 brain MRI studies, 19 cases (65.5%) had at least one or more lesions (1–57) and 10 were negative (34.4%). Brain MRI findings in 19 cases were characterized in T2/fluid-attenuated inversion-recovery (FLAIR) by the presence of subcortical/deep white matter lesions in 16 (84.2%) cases (1–50), most of them < 3 mm and without juxtacortical localization. Periventricular lesions were observed in 13 (68.4%) cases, but morphologically they were not oval, ovoid or perpendicularly orientated. Infratentorial lesions, all >3 mm, were observed in 4 (21.05%) cases without cerebellar involvement. T1 studies demonstrated absence of hypointense regions. Optic nerve enhancement was observed in 6/19 patients (31.5%). None of the brain MRI abnormalities observed were compatible with Barkhof et al. criteria of MS. Conclusions This study, based on a Cuban patient population, with long duration of disease, good sample size and detailed characterization by MRI, demonstrated the brain MRI pattern of R-NMO patients, which is different from MS. Multiple Sclerosis 2007; 13: 186–192. http://msj.sagepub.com


Author(s):  
Mariam Raafat ◽  
Mahmoud Alalfy ◽  
Omar Nagy ◽  
Samira Saraya

Abstract Background Central nervous system (CNS) anomalies are the most commonly diagnosed abnormalities of all fetal malformations and are usually primarily discovered on routine prenatal ultrasonography (US). Fetal magnetic resonance imaging (MRI) is a non-invasive technology with high soft tissue contrast that is documented to increase the diagnostic accuracy for detection of fetal brain anomalies. The aim of our study is to analyze the value of adding magnetic resonance imaging (MRI) of the fetal brain to antenatal ultrasound in the diagnosis of fetal central nervous system (CNS) anomalies. Results We diagnosed various CNS anomalies including twelve cases with infra- and supra-tentorial arachnoid cysts, six cases had Dandy-Walker malformation (DWM) and its variants, 1 case with mega cisterna magna, 2 cases of holoprosencephaly, 1 case of hydranencephaly, 2 cases with supratentorial hydrocephalus, 1 case of craniopharyngioma, 6 cases with corpus callosum (CC) agenesis, 1 case of extradural hematoma, and 8 cases with Meckel-Gruber syndrome (MGS). MRI diagnosis confirmed the ultrasound finding, without additional information in 23 cases (57.5%%), added an extra finding in 11 cases (27.5%), differentiated between 2 pathologies in 3 cases (7.5%), and changed the diagnosis in 3 cases (7.5 %). The 40 pregnancies resulted in 27 births (67.5%), 2 died directly after birth (5%), 7 terminations (17.5%), and 4 intrauterine fetal deaths (IUFD) (10 %). Conclusion Ultrasound is the gold standard imaging modality for anomaly scan in the second and third trimesters; however, MRI of the fetal brain might be a clinically valuable complement especially when ultrasound examination is inconclusive due to maternal obesity, severe oligohydramnios, or in complicated cases with unclear diagnosis.


2021 ◽  
Author(s):  
Tomer Stern ◽  
Liora Kornreich ◽  
Hadassa Goldberg

Abstract Background We aimed to find the clinical significance of brain abnormalities on magnetic resonance imaging (MRI) in epilepsy and the lateralization of these findings with electroencephalogram (EEG). Methods We retrospectively analyzed the results of all EEGs and brain MRIs of 600 consecutive epilepsy patients from 1998 to 2020. Results Data were available for 563 cases (267 females). Ninety percent of the patients were 18 years old or younger. A total of 345 patients (61.3%) had focal epilepsy, 180 (32%), generalized, and 38 (6.7%), inconclusive. In 187 (33.2%), the first MRI was abnormal and in 81 (out of 108 repeated MRI), the second was pathological. The most frequent brain abnormalities were cortical dysplasia in 41 (18.1%), other structural abnormalities in 25 (11%), various phacomatoses in 23 (10.1%), and mesial temporal sclerosis in 17 (7.5%). Among 226 patients with abnormal MRI, 171 (75.6%) had focal epilepsy when compared with 36 (15.9%) with generalized epilepsy (p <0.001). In 121 patients (53.5%), the result of the abnormal MRI contributed significantly to the understanding of the epilepsy etiology. The side of abnormality was lateralized to the EEG focus in 120 cases (53%); in 10/15 cases with infantile spasms (66%), MRI was significantly abnormal. In 33, in whom the first MRI was normal, a second MRI revealed a significant abnormality. Conclusion Brain MRI is an important tool in epilepsy diagnosis, mainly in focal seizures and infantile spasms. A repeat MRI is mandatory in intractable focal cases to improve the yield of this test.


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