scholarly journals Creutzfeldt-Jakob disease Heidenhain variant: case report with progressive cognitive decline recorded by videos

2021 ◽  
Author(s):  
Pedro Neves Fortunato ◽  
Hilton Mariano da Silva Júnior ◽  
Rosana Carandina Maffeis ◽  
Caroline Moraes Tapajós Bolzani
Keyword(s):  
Cognitive Decline ◽  
Brain Mri ◽  
Video Recording ◽  
Infectious Complications ◽  
Psychomotor Slowing ◽  
Restricted Area ◽  
Periodic Discharges ◽  
Jakob Disease ◽  
Visual Complaints ◽  
The Brain

Context: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative disease. Heidenhain’s variant has isolated visual symptoms that persist even without any cognitive decline for a few weeks. Objective: To report a case of the Heidenhain variant of the CJD with evolution documented. Methods: Case report:A 67-year-old woman was admitted to the emergency room with a report of “seizures and dementia for 1 month”. It all started in August 2020 with a decrease in visual acuity. The son started to record the evolution of the disease in vídeos on his mobile phone. It got worse, with an inability to reach objects and optical apraxia,psychomotor slowing, abnormal repetition of acts and impairment of judgment. MRI of the brain (normal) and EEG was performed, with diffuse slowing of the base activity. It evolved with intermittent myoclonus and abulia. Extensive investigation for encephalitis: normal. Treatment with acyclovir and pulsetherapy with methylprednisolone were performed. Results: new brain MRI: restricted area following the diffusion and T2 / FLAIR hypersignal exclusively cortical involving both posterior hemispheres. Also new EEG: generalized periodic discharges. The 14-3-3 protein was detected in her CSF.The patient died 4 months after the onset of the condition due to infectious complications. Conclusions: The Heidenhain variant of CJD should be considered a differential diagnosis in all patients who have isolated visual complaints, especially if associated with cognitive complaints. The video recording of the patient allows a detailed analysis of the clinical picture and becomes an important diagnostic complementation tool.

Familial multiple cavernomatosis and neuropsychiatric symptoms: Is there any relation?

2016 ◽  
Vol 33 (S1) ◽  
pp. S146-S147
Author(s):  
V. Espirito Santo ◽  
R. Almendra ◽  
A.R. Figueiredo ◽  
A. Almeida ◽  
I. Rego ◽  
...  
Keyword(s):  
Hemorrhagic Stroke ◽  
Past History ◽  
Neuropsychiatric Symptoms ◽  
Brain Mri ◽  
Brain Network ◽  
Sudden Onset ◽  
Psychomotor Slowing ◽  
Autosomal Dominant Disorder ◽  
The Impact ◽  
The Brain

IntroductionCavernomas are clusters of abnormal blood vessels found in the brain and spinal cord. The familiar form is an autosomal dominant disorder associated with the presence of multiple cavernomas in both locations.Clinical CaseA 84-year-old man was admitted in our neurologic department for a sudden onset of difficulty in walking associated with loss of urinary sphincter control. Past history included a major depressive disorder with psychotic features since youth, epilepsy since 33 years old and, at 77 years old, he had a hemorrhagic stroke resulting from cavernous malformation haemorrhage. Medication consisted of clopidogrel 75 mg id, risperidone 3 mg id, venlafaxine 37.5 mg bid and clobazam 10 mg id. On neurological examination, he showed psychomotor slowing, dysexecutive syndrome, paraparesis and hypoesthesia with sensitive level by D10. Blood test was normal. Dorsolumbar spine-TC showed intradural hyperdensity by D12-L1, probably because of a hemorrhage lesion, that MRI revelled to be a cavernoma. Brain-MRI demonstrated 3 massive cavernomas in cortical-subcortical right occipital lobe, left lenticular nucleus and left pre-central gyrus and countless small infratentorial and supratentorial cavernomas. We inquired his family and we found out that one of his daughters also had multiple brain cavernomas, diagnosed after a hemorrhagic stroke when she was 55 years old.ConclusionFamiliar multiple cavernomatosis is associated with neuropsychiatric disorders. We enhance the impact that such a diffuse form of the disease has on the brain network causing atypical psychocognitive symptoms. In all cases a detailed neuropsychiatric family history should be sought and all should be followed regularly clinically and by MRI.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals Content-Based Estimation of Brain MRI Tilt in Three Orthogonal Directions

2021 ◽  
Author(s):  
Pooja Prabhu ◽  
A. K. Karunakar ◽  
Sanjib Sinha ◽  
N. Mariyappa ◽  
G. K. Bhargava ◽  
...  
Keyword(s):  
Large Scale ◽  
Brain Mri ◽  
Similarity Measures ◽  
Principal Component ◽  
Brain Anatomy ◽  
Morphological Operations ◽  
Mr Images ◽  
Tilt Correction ◽  
Brain Mr Images ◽  
The Brain

AbstractIn a general scenario, the brain images acquired from magnetic resonance imaging (MRI) may experience tilt, distorting brain MR images. The tilt experienced by the brain MR images may result in misalignment during image registration for medical applications. Manually correcting (or estimating) the tilt on a large scale is time-consuming, expensive, and needs brain anatomy expertise. Thus, there is a need for an automatic way of performing tilt correction in three orthogonal directions (X, Y, Z). The proposed work aims to correct the tilt automatically by measuring the pitch angle, yaw angle, and roll angle in X-axis, Z-axis, and Y-axis, respectively. For correction of the tilt around the Z-axis (pointing to the superior direction), image processing techniques, principal component analysis, and similarity measures are used. Also, for correction of the tilt around the X-axis (pointing to the right direction), morphological operations, and tilt correction around the Y-axis (pointing to the anterior direction), orthogonal regression is used. The proposed approach was applied to adjust the tilt observed in the T1- and T2-weighted MR images. The simulation study with the proposed algorithm yielded an error of 0.40 ± 0.09°, and it outperformed the other existing studies. The tilt angle (in degrees) obtained is ranged from 6.2 ± 3.94, 2.35 ± 2.61, and 5 ± 4.36 in X-, Z-, and Y-directions, respectively, by using the proposed algorithm. The proposed work corrects the tilt more accurately and robustly when compared with existing studies.

scholarly journals Innumerable cerebral microbleeds in hepatitis B virus-related decompensated liver cirrhosis: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Chi Hyuk Oh ◽  
Jin San Lee
Keyword(s):  
Liver Cirrhosis ◽  
Hepatic Encephalopathy ◽  
Hepatitis B Virus ◽  
Hepatitis B ◽  
Brain Mri ◽  
Cerebral Microbleeds ◽  
Amyloid Angiopathy ◽  
Decompensated Liver Cirrhosis ◽  
B Virus ◽  
The Brain

Abstract Background Cerebral microbleeds (CMBs) are small, rounded, dark-signal lesions on brain MRI that represent cerebral hemosiderin deposits resulting from prior microhemorrhages and are neuroimaging biomarkers of cerebral amyloid angiopathy (CAA). Here, we report a case of innumerable CMBs in a patient with hepatic encephalopathy underlying decompensated liver cirrhosis. Case presentation An 83-year-old woman diagnosed with hepatitis B virus-related liver cirrhosis 40 years before was referred to our neurology clinic for progressive disorientation of time and place, personality changes, and confusion with somnolence over 2 weeks. Based on the laboratory, neuroimaging, and electrophysiological findings, we diagnosed the patient with hepatic encephalopathy, and her symptoms recovered within 12 h after proper medical management. Brain MRI showed innumerable CMBs in the bilateral frontal, parietal, temporal, and occipital lobes. Since the distribution of CMBs in the patient was mainly corticosubcortical and predominantly in the posterior cortical regions, and the apolipoprotein E genotype was ε4/ε4, we speculated that CAA and hepatic encephalopathy coexisted in this patient. Conclusions We suggest that severe liver dysfunction associated with long-term decompensated liver cirrhosis may be related to an increased number of CMBs in the brain. Our findings indicate that decompensated liver cirrhosis may be a risk factor for the development of CMBs and corroborate a link between the liver and the brain.

scholarly journals Cognitive trajectories of patients with focal ß-amyloid deposition

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Si Eun Kim ◽  
Byungju Lee ◽  
Hyemin Jang ◽  
Juhee Chin ◽  
Ching Soong Khoo ◽  
...  
Keyword(s):  
Cognitive Decline ◽  
Neuropsychological Tests ◽  
Mixed Effects ◽  
Amnestic Mild Cognitive Impairment ◽  
Emission Tomography ◽  
Amyloid Pet ◽  
Cognitive Level ◽  
Visual Rating ◽  
Positron Emission ◽  
The Brain

Abstract Background The presence of ß-amyloid (Aß) in the brain can be identified using amyloid PET. In clinical practice, the amyloid PET is interpreted based on dichotomous visual rating, which renders focal Aß accumulation be read as positive for Aß. However, the prognosis of patients with focal Aß deposition is not well established. Thus, we investigated cognitive trajectories of patients with focal Aß deposition. Methods We followed up 240 participants (112 cognitively unimpaired [CU], 78 amnestic mild cognitive impairment [aMCI], and 50 Alzheimer’s disease (AD) dementia [ADD]) for 2 years from 9 referral centers in South Korea. Participants were assessed with neuropsychological tests and 18F-flutemetamol (FMM) positron emission tomography (PET). Ten regions (frontal, precuneus/posterior cingulate (PPC), lateral temporal, parietal, and striatum of each hemisphere) were visually examined in the FMM scan, and participants were divided into three groups: No-FMM, Focal-FMM (FMM uptake in 1–9 regions), and Diffuse-FMM. We used mixed-effects model to investigate the speed of cognitive decline in the Focal-FMM group according to the cognitive level, extent, and location of Aß involvement, in comparison with the No- or Diffuse-FMM group. Results Forty-five of 240 (18.8%) individuals were categorized as Focal-FMM. The rate of cognitive decline in the Focal-FMM group was faster than the No-FMM group (especially in the CU and aMCI stage) and slower than the Diffuse-FMM group (in particular in the CU stage). Within the Focal-FMM group, participants with FMM uptake to a larger extent (7–9 regions) showed faster cognitive decline compared to those with uptake to a smaller extent (1–3 or 4–6 regions). The Focal-FMM group was found to have faster cognitive decline in comparison with the No-FMM when there was uptake in the PPC, striatum, and frontal cortex. Conclusions When predicting cognitive decline of patients with focal Aß deposition, the patients’ cognitive level, extent, and location of the focal involvement are important.

scholarly journals The Role of Vitamin D as a Biomarker in Alzheimer’s Disease

2021 ◽  
Vol 11 (3) ◽  
pp. 334
Author(s):  
Giulia Bivona ◽  
Bruna Lo Sasso ◽  
Caterina Maria Gambino ◽  
Rosaria Vincenza Giglio ◽  
Concetta Scazzone ◽  
...  
Keyword(s):  
Vitamin D ◽  
Risk Factor ◽  
Cognitive Decline ◽  
Immune Cell ◽  
Response To Treatment ◽  
Cellular Processes ◽  
Vitamin D Levels ◽  
The Brain ◽  
Key Questions

Vitamin D and cognition is a popular association, which led to a remarkable body of literature data in the past 50 years. The brain can synthesize, catabolize, and receive Vitamin D, which has been proved to regulate many cellular processes in neurons and microglia. Vitamin D helps synaptic plasticity and neurotransmission in dopaminergic neural circuits and exerts anti-inflammatory and neuroprotective activities within the brain by reducing the synthesis of pro-inflammatory cytokines and the oxidative stress load. Further, Vitamin D action in the brain has been related to the clearance of amyloid plaques, which represent a feature of Alzheimer Disease (AD), by the immune cell. Based on these considerations, many studies have investigated the role of circulating Vitamin D levels in patients affected by a cognitive decline to assess Vitamin D’s eventual role as a biomarker or a risk factor in AD. An association between low Vitamin D levels and the onset and progression of AD has been reported, and some interventional studies to evaluate the role of Vitamin D in preventing AD onset have been performed. However, many pitfalls affected the studies available, including substantial discrepancies in the methods used and the lack of standardized data. Despite many studies, it remains unclear whether Vitamin D can have a role in cognitive decline and AD. This narrative review aims to answer two key questions: whether Vitamin D can be used as a reliable tool for diagnosing, predicting prognosis and response to treatment in AD patients, and whether it is a modifiable risk factor for preventing AD onset.

scholarly journals Pure Motor Stroke Secondary to Cerebral Infarction of Recurrent Artery of Heubner after Mild Head Trauma: A Case Report

2016 ◽  
Vol 4 (1) ◽  
pp. 139-141
Author(s):  
Ali Yilmaz ◽  
Zahir Kizilay ◽  
Ayca Ozkul ◽  
Bayram Çirak
Keyword(s):  
Ischemic Stroke ◽  
Head Trauma ◽  
Distal Part ◽  
Brain Mri ◽  
Artery Occlusion ◽  
Acute Ischemia ◽  
Post Traumatic ◽  
The Brain ◽  
Perforating Arteries ◽  
Pure Motor

BACKGROUND: The recurrent Heubner's artery is the distal part of the medial striate artery. Occlusion of the recurrent artery of Heubner, classically contralateral hemiparesis with fasciobrachiocrural predominance, is attributed to the occlusion of the recurrent artery of Heubner and is widely known as a stroke syndrome in adults. However, isolated occlusion of the deep perforating arteries following mild head trauma also occurs extremely rarely in childhood.CASE REPORT: Here we report the case of an 11-year-old boy with pure motor stroke. The brain MRI showed an acute ischemia in the recurrent artery of Heubner supply area following mild head trauma. His fasciobrachial hemiparesis and dysarthria were thought to be secondary to the stretching of deep perforating arteries leading to occlusion of the recurrent artery of Heubner.CONCLUSION: Post-traumatic pure motor ischemic stroke can be secondary to stretching of the deep perforating arteries especially in childhood.

Sporadic Creutzfeldt-Jakob Disease: Diagnosing Typical and Atypical Presentations under Limited Circumstances

2021 ◽  
pp. 1-7
Author(s):  
Shazma Khan ◽  
Sara Khan
Keyword(s):  
Developing Countries ◽  
Brain Mri ◽  
Tertiary Care ◽  
Rapid Onset ◽  
Spongiform Encephalopathy ◽  
Care Hospital ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Missed Diagnosis ◽  
Atypical Presentations

<b><i>Introduction:</i></b> Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible disorder of the central nervous system caused by the transformation of normal prion protein into an abnormal misfolded form. The process begins spontaneously and runs a vicious cycle to cause spongiform encephalopathy, rapidly resulting in death. Amply described in the western literature, CJD is scarcely reported in Asia due to certain limitations including missed diagnosis, under-reporting, and rarity of the disease. Brain MRI, electroencephalogram, cerebrospinal fluid testing, and biopsy of the infected brain tissue support the diagnosis in cases of clinical suspicion. However, the diagnosis can still be made with limited available resources in developing countries. <b><i>Method:</i></b> A review of CJD cases evaluated in the neurology department of a tertiary care hospital in Pakistan was done from 2002 to 2018. <b><i>Results:</i></b> Eleven cases labeled as sCJD are identified based on the European MRI-CJD consortium criteria. This is the first study on CJD from Pakistan, which includes both the typical and atypical presentations. <b><i>Conclusion:</i></b> Even with limited testing available, the diagnosis of CJD can be made with confidence in the developing countries, provided the suspicion is kept high in cases of rapid onset dementia and acute behavioral changes.

Synergistic Effect of Serum Homocysteine and Diabetes Mellitus on Brain Alterations

2021 ◽  
pp. 1-9
Author(s):  
Gihwan Byeon ◽  
Min Soo Byun ◽  
Dahyun Yi ◽  
Jun Ho Lee ◽  
So Yeon Jeon ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Older Adults ◽  
Cognitive Decline ◽  
Brain Atrophy ◽  
Homocysteine Level ◽  
Cognitive Impairments ◽  
Interactive Effect ◽  
Brain Mri ◽  
Amyloid Β ◽  
Serum Homocysteine

Background: Both elevated blood homocysteine and diabetes mellitus (DM) are related to cognitive impairments or dementia. A previous study also demonstrated that the association between homocysteine and cognitive decline was much stronger in individuals with DM than in those without DM. Objective: This study aimed to examine the interactive effect of blood homocysteine and DM on brain pathological changes including brain atrophy, amyloid-β and tau deposition, and small vessel disease (SVD) related to cognitive impairments. Methods: A total of 430 non-demented older adults underwent comprehensive clinical assessment, measurement of serum homocysteine level, [11C] Pittsburgh Compound B (PiB) PET, [18F] AV-1451 PET, and brain MRI. Results: The interactive effect of homocysteine with the presence of DM on brain atrophy, especially in aging-related brain regions, was significant. Higher homocysteine concentration was associated with more prominent brain atrophy in individuals with DM, but not in those without DM. In contrast, interaction effect of homocysteine and DM was found neither on Alzheimer’s disease (AD) pathologies, including amyloid-β and tau deposition, nor white matter hyperintensity volume as a measure of SVD. Conclusion: The present findings suggest that high blood homocysteine level and DM synergistically aggravate brain damage independently of AD and cerebrovascular disease. With regard to preventing dementia or cognitive decline in older adults, these results support the importance of strictly controlling blood glucose in individuals with hyperhomocysteinemia and lowering blood homocysteine level in those with DM.

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