scholarly journals Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease

2021 ◽  
Vol 15 (4) ◽  
pp. 548-549
Author(s):  
Janaina Mariana de Araujo Miranda Brito-Marques ◽  
Eduardo Sousa de Melo ◽  
Fabíola Lys de Medeiros ◽  
Cristiano Sobral de Carvalho ◽  
Paulo Roberto de Brito-Marques

ABSTRACT. We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted diffusion in the diffusion-weighted imaging and hypointense areas corresponding to the apparent diffusion coefficient in the cerebral cortex. The autopsy revealed positive immunohistochemistry for the PrPSc protein. Creutzfeldt–Jakob disease presenting with hearing loss is unusual.

2020 ◽  
pp. 028418512091403
Author(s):  
Somkiat Huaijantug ◽  
Paranee Yatmark ◽  
Phanupong Chinnabrut ◽  
Nutruja Rueangsawat ◽  
Avirut Wongkumlue ◽  
...  

Background Quantitative magnetic resonance imaging (MRI) is used to study the anatomy of the brain in dogs with idiopathic epilepsy. Purpose To quantitate MRI images in terms of volumetric ratios and histogram analyses of the following regions of interest (ROI) in dogs with idiopathic epilepsy: frontal; parietal; temporal; piriform; thalamic; and hippocampal regions. Material and Methods Nine dogs with epilepsy and four healthy controls were evaluated. We examined the volumetric ratios and histogram analyses of six ROIs in all dogs. Results MR images, in T1-weighted, T2-weighted, FLAIR, diffusion-weighted imaging, and apparent diffusion coefficient sequences detected changes in 4/9 (44%) epileptic dogs found in 5/6 regions: frontal; parietal; temporal; piriform; and hippocampal regions. However, no such changes were observed in the thalamic region. Interestingly, the frontal and piriform volumetric ratios of epileptic dogs were significantly lower than those of control dogs. The histogram analyses in 4/6 regions were significantly increased in epileptic dogs. Conclusion Our results demonstrated MRI finding abnormalities in several regions of the brain in several sequences including T1-weighted, T2-weighted, FLAIR, diffusion-weighted imaging, and apparent diffusion coefficient in epileptic dogs. In several regions of the brain, atrophy may exist, and hyperintensity may be present on MR images in epileptic dogs. These findings suggest that the diagnostic yield of MRI, which is an advanced neuroimaging technique, is high in epileptic dogs and has good reliability and sensitivity in detecting abnormal areas in patients.


2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Soha Khan ◽  
Asma AlNajjar ◽  
Abdullah Alquaydheb ◽  
Shahpar Nahrir

Celiac disease epilepsy and occipital calcification (CEC) syndrome is a rare, emerging disease first described in 1992. To date, fewer than 200 cases have been reported worldwide. CEC syndrome is generally thought to be a genetic, noninherited, and ethnically and geographically restricted disease in Mediterranean countries. However, we report the first ever case of probable CEC in a Saudi patient. Furthermore, the patient manifested a magnitude of brain magnetic resonance imaging (MRI) signal abnormalities during the periictal period which, to the best of our knowledge, has never been described in CEC. The brain MRI revealed diffusion-weighted imaging (DWI) restriction with a concordant area of apparent diffusion coefficient (ADC) hypointensity around bilateral occipital area of calcification. An imbalance between the heightened energy demand during ictal phase of the seizure and unadjusted blood supply may have caused an electric pump failure and cytotoxic edema, which then led to DWI/ADC signal alteration.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Elijah Lackey ◽  
Deepal P. Shah-Zamora ◽  
Jodi Hawes ◽  
Andy J. Liu

A 76-year-old Caucasian woman initially presented to the Duke Memory Disorders clinic with a 9-month history of a rapid decline in cognitive, motor, and neuropsychiatric function. On initial presentation, the patient required assistance with activities of daily living. On neurological examination, she was found to have Gerstmann’s syndrome along with appendicular apraxia. A positional tremor was noted without myoclonus or fasciculations. She had a paucity of speech and was unable to write her own name. Snout and grasp reflexes were present. Episodes of inappropriate laughter were noted during the exam. She was admitted to the inpatient neurology service for further evaluation. The Diffusion Weighted Imaging sequence on Magnetic Resonance Imaging of the brain was negative for restricted diffusion. An electroencephalogram was unremarkable. Cerebrospinal fluid analysis for Real-Time Quaking-Induced Conversion assay was positive along with an elevated 14-3-3 and increased total Tau protein levels. There was no family history of Creutzfeldt–Jakob disease. The cerebral spinal fluid results were consistent with a diagnosis of Creutzfeldt–Jakob disease, despite the negative MRI brain findings.


2020 ◽  
Vol 4 (4) ◽  
pp. 626-627
Author(s):  
Boris Ryabtsev ◽  
Matthew Slane

Case Presentation: A 53-year-old male presented to the emergency department with acute onset of bilateral hearing loss as well as vertigo and severe vomiting. The Head Impulse– Nystagmus–Test of Skew exam was indicative of a central neurologic process. Computed tomography angiogram of the head and neck revealed near-total bilateral vertebral artery occlusions in the second and third segments. The patient was admitted for further evaluation; subsequent magnetic resonance imaging revealed multiple areas of infarction in the cerebellar hemispheres, medulla, and occipital lobes. Discussion: This case describes a unique presentation of a posterior stroke. Common symptoms include vertigo, loss of balance, and vomiting. However, bilateral hearing loss as a prominent symptom is uncommon. Imaging revealed a rare finding of bilateral vertebral artery occlusion.


2007 ◽  
Vol 48 (7) ◽  
pp. 755-762 ◽  
Author(s):  
A. Aalto ◽  
J. Sjöwall ◽  
L. Davidsson ◽  
P. Forsberg ◽  
Ö. Smedby

Background: Borrelia infections, especially chronic neuroborreliosis (NB), may cause considerable diagnostic problems. This diagnosis is based on symptoms and findings in the cerebrospinal fluid but is not always conclusive. Purpose: To evaluate brain magnetic resonance imaging (MRI) in chronic NB, to compare the findings with healthy controls, and to correlate MRI findings with disease duration. Material and Methods: Sixteen well-characterized patients with chronic NB and 16 matched controls were examined in a 1.5T scanner with a standard head coil. T1- (with and without gadolinium), T2-, and diffusion-weighted imaging plus fluid-attenuated inversion recovery (FLAIR) imaging were used. Results: White matter lesions and lesions in the basal ganglia were seen in 12 patients and 10 controls (no significant difference). Subependymal lesions were detected in patients down to the age of 25 and in the controls down to the age of 43. The number of lesions was correlated to age both in patients (ρ = 0.83, P<0.01) and in controls (ρ = 0.61, P<0.05), but not to the duration of disease. Most lesions were detected with FLAIR, but many also with T2-weighted imaging. Conclusion: A number of MRI findings were detected in patients with chronic NB, although the findings were unspecific when compared with matched controls and did not correlate with disease duration. However, subependymal lesions may constitute a potential finding in chronic NB.


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