A case of cerebral aquaporinopathy

2010 ◽  
Vol 16 (10) ◽  
pp. 1252-1254 ◽  
Author(s):  
A. Tanaka ◽  
T. Yoshida ◽  
T. Yamada ◽  
R. Isayama ◽  
Y. Fujiwara ◽  
...  

A 35-year-old woman was hospitalized due to impaired consciousness. Magnetic resonance imaging (MRI) revealed multiple parenchymal lesions in supra and infratentorial brain regions, which were considered responsible for her declining consciousness level. She was treated with intravenous methylprednisolone. Neurological symptoms improved and she was discharged. She was readmitted 14 months later due to intractable hiccups. A follow-up brain MRI revealed an abnormal signal near the area postrema in the dorsal medulla. Serum aquaporin-4 antibody levels were positive, but there were no visual manifestations or myelitis. Spinal MRI was negative for longitudinally extended transverse myelitis throughout the clinical course.

2020 ◽  
Vol 35 (8) ◽  
pp. 501-508
Author(s):  
Mehmet Bastemur ◽  
Rahsan Gocmen ◽  
Safak Parlak ◽  
Deniz Yuksel ◽  
Elif Acar Arslan ◽  
...  

Aim: To investigate etiology and prognostic significance of pontine tegmentum lesions accompanying a cluster of acute flaccid myelitis. Method: We retrospectively examined patients from 6 centers in Turkey who manifested encephalitis or myelitis associated with dorsal pontine lesions on magnetic resonance imaging (MRI) between July 2018 and February 2019. Results: Twenty-two patients were evaluated. Ten of 22 (45%) presented with acute paralysis and 12 of 22 (55%) with brainstem symptoms only. Reverse transcription polymerase chain reaction for enterovirus was positive in 2 patients’ respiratory tract. Other etiologic factors were detected in 10 cases. On follow-up, patients presenting with symptoms of myelitis developed motor sequalae although spinal cord lesions on MRI resolved in 5 of 9 (55%). Encephalitic symptoms, present in 17 cases, recovered in 13 (76%), and brain MRI showed complete or near-complete resolution in 11 of 14 (78%). Conclusion: Various etiologic agents can be detected in patients with pontine involvement, even in a series collected during an outbreak of EV-D68. Encephalitis has a fair outcome but clinical recovery is slow and motor sequalae are frequent in spinal involvement, irrespective of follow-up spinal MRI findings.


2014 ◽  
Vol 20 (9) ◽  
pp. 1153-1164 ◽  
Author(s):  
George Tackley ◽  
Wilhelm Kuker ◽  
Jacqueline Palace

Neuromyelitis optica (NMO), or Devic’s disease, is a rare demyelinating disorder of the central nervous system that has a predilection for the optic nerve and spinal cord. Magnetic resonance imaging (MRI) is required to diagnose NMO. Longitudinally extensive transverse myelitis is NMO’s imaging hallmark and the presence of a brain MRI that is not diagnostic of multiple sclerosis (MS) also remains part of the diagnostic criteria. It is increasingly recognised that MS and NMO brain imaging can, however, have similar appearances but differences do exist: hypothalamic, periaqueductal grey and area postrema lesions implicate NMO whilst cortical, U-fibre or Dawson’s finger lesions are suggestive of MS. The timing of image acquisition, age, ethnicity and aquaporin-4 antibody status are all likely to alter the findings at MRI. This review therefore aims to overview and update the reader on NMO imaging, to provide clinically relevant guidance for diagnosing NMO and differentiating it from MS in order to guide management, and to highlight recent research insights.


ADMET & DMPK ◽  
2017 ◽  
Vol 5 (4) ◽  
pp. 234-241
Author(s):  
Xiuxin Wang ◽  
Yi Zhao ◽  
Huiling Zhang ◽  
Gang Yin ◽  
Yangkun Luo ◽  
...  

Radiotherapy (RT) is the standard treatment for nasopharyngeal carcinoma, which often causes inevitable brain injury in the process of treatment. The majority of patients has no abnormal signal or density change of the conventional magnetic resonance imaging (MRI) and computed tomography (CT) examination in the long-term follow-up after radiation therapy. However, when there is a visible CT and conventional MR imaging changes, the damage often has been severe and lack of effective treatments, seriously influencing the prognosis of patients. Therefore, the present study aimed to investigate the abnormal changes in nasopharyngeal carcinoma (NPC) patients after RT. In the present study, we exploited the machine learning framework which contained two parts: feature extraction and classification to automatically detect the brain injury. Our results showed that the method could effectively identify the abnormal regions reduced by radiotherapy. The highest classification accuracy was 82.5 % in the abnormal brain regions. The parahippocampal gyrus was the highest accuracy region, which suggested that the parahippocampal gyrus could be most sensitive to radiotherapy and involved in the pathogenesis of radiotherapy-induced brain injury in NPC patients.


2018 ◽  
Vol 1 ◽  
pp. 251581631879054
Author(s):  
J Matthijs Biesbroek ◽  
Dirk R Rutgers ◽  
Sander van Gulik ◽  
Catherina JM Frijns

Short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) is a rare form of trigeminal autonomic cephalalgia. SUNA is frequently associated with a trigeminal neurovascular conflict and rarely occurs secondary to other intracranial pathology. We report a patient with SUNA that was associated with ipsilateral meningeal inflammation caused by idiopathic hypertrophic pachymeningitis (HP). During the 9-year follow-up, she experienced multiple episodes of SUNA, most of which occurred during exacerbations of the pachymeningitis. This is the third case of SUNA associated with HP reported in the literature. Based on this observation, we suggest that in patients presenting with SUNA, besides dedicated magnetic resonance imaging (MRI) of the trigeminal nerve, gadolinium-enhanced brain MRI should be performed to rule out HP.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Amir M. Torabi

Bilateral medial medullary stroke is a very rare type of stroke, with catastrophic consequences. Early diagnosis is crucial. Here, I present a young patient with acute vertigo, progressive generalized weakness, dysarthria, and respiratory failure, who initially was misdiagnosed with acute vestibular syndrome. Initial brain magnetic resonance imaging (MRI) that was done in the acute phase was read as normal. Other possibilities were excluded by lumbar puncture and MRI of cervical spine. MR of C-spine showed lesion at medial medulla; therefore a second MRI of brain was requested, showed characteristic “heart appearance” shape at diffusion weighted (DWI), and confirmed bilateral medial medullary stroke. Retrospectively, a vague-defined hyperintense linear DWI signal at midline was noted in the first brain MRI. Because of the symmetric and midline pattern of this abnormal signal and similarity to an artifact, some radiologists or neurologists may miss this type of stroke. Radiologists and neurologists must recognize clinical and MRI findings of this rare type of stroke, which early treatment could make a difference in patient outcome. The abnormal DWI signal in early stages of this type of stroke may not be a typical “heart appearance” shape, and other variants such as small dot or linear DWI signal at midline must be recognized as early signs of stroke. Also, MRI of cervical spine may be helpful if there is attention to brainstem as well.


2016 ◽  
Vol 22 (9) ◽  
pp. 1174-1183 ◽  
Author(s):  
Mike P Wattjes ◽  
Martijn T Wijburg ◽  
Anke Vennegoor ◽  
Birgit I Witte ◽  
Stefan D Roosendaal ◽  
...  

Background: In natalizumab-treated multiple sclerosis (MS) patients, magnetic resonance imaging (MRI) is considered as a sensitive tool in detecting both MS disease activity and progressive multifocal leukoencephalopathy (PML). Objective: To investigate the performance of neuroradiologists using brain MRI in detecting new MS lesions and asymptomatic PML lesions and in differentiating between MS and PML lesions in natalizumab-treated MS patients. The secondary aim was to investigate interrater variability. Methods: In this retrospective diagnostic study, four blinded neuroradiologists assessed reference and follow-up brain MRI scans of 48 natalizumab-treated MS patients with new asymptomatic PML lesions ( n = 21) or new MS lesions ( n = 20) or no new lesions ( n = 7). Sensitivity and specificity for detection of new lesions in general (MS and PML lesions), MS and PML lesion differentiation, and PML detection were determined. Interrater agreement was calculated. Results: Overall sensitivity and specificity for the detection of new lesions, regardless of the nature of the lesions, were 77.4% and 89.3%, respectively; for PML-MS lesion differentiation, 74.2% and 84.7%, respectively; and for asymptomatic PML lesion detection, 59.5% and 91.7%, respectively. Interrater agreement for the tested categories was fair to moderate. Conclusion: The diagnostic performance of trained neuroradiologists using brain MRI in pharmacovigilance of natalizumab-treated MS patients is moderately good. Interrater agreement among trained readers is fair to moderate.


2020 ◽  
Author(s):  
Ryuzo Orihashi ◽  
Yoshito Mizoguchi ◽  
Yoshiomi Imamura ◽  
Shigeto Yamada ◽  
Akira Monji

Abstract BackgroundThis study aimed to investigate the association between serum levels of soluble triggering receptor expressed on myeloid cells 2 (sTREM2), a soluble form of an innate immune receptor expressed on the microglia, and brain volume in elderly people.MethodsThe survey was conducted twice in Kurokawa-cho, Imari, Saga Prefecture, Japan, among people aged 65 years and older. We collected data from 596 residents. Serum sTREM2 level measurements, brain MRI, Mini-Mental State Examination (MMSE), and Clinical Dementia Rating (CDR) were performed at Time 1 (2009–2011). Follow-up brain MRI, MMSE, and CDR were performed at Time 2 (2016–2017). The interval between Time 1 and Time 2 was approximately 7 years. Sixty-nine participants (16 men, mean age 72.69 ± 3.18 years; 53 women, mean age 72.68 ± 4.64 years) completed this study. We analyzed the correlation between serum sTREM2 levels (Time 1) and brain volume (Time 1, Time 2, and Time 1–Time 2 difference) using voxel-based morphometry implemented with Statistical Parametric Mapping.ResultsParticipants in this study had lower MMSE and CDR scores 7 years after the baseline evaluation. However, analyses at the cluster level by applying multiple comparison corrections (family wise error; P < 0.05) showed no correlation between serum sTREM2 levels and volume of different brain regions, either cross-sectional or longitudinal. ConclusionsSerum sTREM2 level could not serve as an immune biomarker of aging-related volume changes in brain regions closely related to cognitive function in elderly people aged 65 years and above.


2010 ◽  
Vol 17 (2) ◽  
pp. 164-172 ◽  
Author(s):  
Olivier Gout ◽  
Annie Bouchareine ◽  
Antoine Moulignier ◽  
Romain Deschamps ◽  
Caroline Papeix ◽  
...  

Background and Objective: This study aimed to assess the value of cerebrospinal fluid (CSF) findings for predicting conversion to clinically definite multiple sclerosis (CDMS). Methods: From a database of 447 patients with a first demyelinating event, the records of 208 patients less than 51 years old who had baseline magnetic resonance imaging (MRI) and CSF examinations and a follow-up of at least 1 year were included. A multivariable Cox model was used to assess the short-term risk of CDMS according to baseline CSF findings after adjustment for prognostic factors (including brain MRI) and to provide a simple classification for predicting CDMS. Results: During a median follow-up of 3.5 years, 141 (67.8%) patients converted to CDMS. In multivariate analysis, younger age (hazard ratio [HR]: 1.44 [95% CI 1.02–2.01]), spatial dissemination on brain MRI (HR: 2.07 [95% CI 1.47–2.91]) and more than 4 WBC/mm3 in CSF (HR: 1.44 [95% CI 1.03–2.02]) were independently associated with CDMS. The Cox score obtained from these three predictors enabled patients to be divided into three groups with significant increased risks of CDMS at 1, 2 and 3 years; groups were classified as high-risk (64.7%, 77.4%, 96.1%), intermediate-risk (33.3%, 51.5%, 61.5%), and low-risk (11.1%, 18.3%, 40.3%). Conclusions: Age at onset, spatial dissemination on brain MRI and CSF white blood cell count are independently associated with short-term conversion to CDMS. The three proposed risk group classifications could be a useful tool to select patients for early therapeutic intervention.


2014 ◽  
Vol 20 (11) ◽  
pp. 1485-1493 ◽  
Author(s):  
P Meyer ◽  
N Leboucq ◽  
N Molinari ◽  
A Roubertie ◽  
M Carneiro ◽  
...  

Background: Acute transverse myelitis (ATM) in children is a rare and often severe disease for which there are few known prognostic factors, particularly the subsequent risk of multiple sclerosis (MS) diagnosis. Objectives: To determine the clinical course and prognostic factors after a first episode of ATM in children. Methods: Thirty children below 16 years of age diagnosed with a first neurological episode of ATM were included retrospectively. Clinical evaluation, treatment, laboratory, and MRI data were collected. Results: Median age at onset was 11 years (range 3–15 years). Follow-up data were available for a median of 4 years (range 0.5–16.7 years). Five patients subsequently had a diagnosis of MS (17%), which was associated with acute partial transverse myelitis (odds ratio 5; 95% confidence interval 2.3–11), with a 60% probability of having a relapse at five years ( p < 0.01). The 2011 Verhey criteria correctly identified MS in children with the highest specificity (96%) and sensitivity (80%). Conclusion: Acute partial transverse myelitis and brain MRI abnormalities at initial presentation are significantly predictive of a subsequent diagnosis of MS in children with ATM. These findings suggest that closer brain MRI monitoring after acute partial transverse myelitis might make the earlier introduction of disease-modifying therapies possible.


Sign in / Sign up

Export Citation Format

Share Document