DURAL AVF CAUSING A TREATABLE CJD LIKE COGNITIVE DYSFUNCTION

2015 ◽  
Vol 86 (11) ◽  
pp. e4.135-e4
Author(s):  
Anna Randall ◽  
Richard James Booth Ellis ◽  
Brython Hywel ◽  
Christopher Andrew McCoy ◽  
Rhys Davies ◽  
...  

A 67-year-old woman presented with a seven-month history of progressive psychiatric disturbance and sub-acute cognitive decline mimicking Creuztfeldt-Jakob Disease.She presented initially to Psychiatry with worsening anxiety, depression, lassitude, impaired concentration and insomnia. She was given a diagnosis of agitated depression.She subsequently experienced episodes of disorientation, indecision and inability to complete routine, learned tasks as well as visual hallucinations. Initial investigations for causes of subacute dementia were unremarkable including a non-diagnostic MRI. However, five months into her presentation, there was further rapid deterioration with fluctuating consciousness and deteriorating mobility. She became bedbound, incontinent and cortically blind. She had right hemiparesis, hemineglect, extrapyramidal features, and extensor plantars.A 4D computerised tomography angiogram demonstrated a dural arteriovenous (AV) fistula with retrograde filling of superior sagittal sinus and cortical venous congestion. She underwent emergency Onyx embolisation. Over a few weeks, there was gradual improvement in her anxiety, cognition and mobility. She had a mild residual right hemiparesis.Intracranial dural AV fistulae can present with a spectrum of neurological symptoms, including cognitive decline. A lowhigh index of suspicion combined with close liaison between Neuroradiology, Neurology and Neurosurgery ensures prompt diagnosis and maximises the potential for cognitive recovery.

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
R. Williams ◽  
F. Cresswell ◽  
M. McClure ◽  
R. Lane

Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1) Electroencephalogram demonstrating periodic sharp wave complexes, (2) MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3) presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain histology confirmed a typical spongiform encephalopathy. Establishing an underlying aetiology is dementia is important not only for prognostic reasons but in order to detect potentially reversible causes. In cases of an atypical dementing illness our proposed investigations may assist in confirming or excluding underlying Creutzfeltd-Jakob disease.


2020 ◽  
Vol 13 (9) ◽  
pp. 550-556
Author(s):  
Minal Karavadra ◽  
Ricky Bell

The intensive care department may seem a long way from the GP's consulting room, but every year tens of thousands of critically ill patients are admitted to intensive care units (ICUs) across the UK. Patients are often left with long term sequelae that may require GP input. Physical weakness, psychiatric disturbance and cognitive decline are not uncommon after an illness that requires a stay in an ICU. These hinder a patient’s return to their previous level of function and impact caregivers after discharge. This article aims to highlight the chronic symptoms patients can acquire during ICU admission that may come to the attention of GPs for their advice and treatment.


2013 ◽  
Vol 70 (6) ◽  
pp. 729-735 ◽  
Author(s):  
Kaarin J. Anstey ◽  
Kerry Sargent-Cox ◽  
Nicolas Cherbuin ◽  
Perminder S. Sachdev

2021 ◽  
pp. practneurol-2021-002942
Author(s):  
Yue Hui Lau ◽  
Ahmad Shahir Mawardi ◽  
Norzaini Rose Zain ◽  
Shanthi Viswanathan

A 33-year-old man with a history of chronic toluene abuse through glue sniffing, developed tremors, cerebellar signs and cognitive decline. MR scan of the brain showed global cerebral and cerebellar atrophy with symmetrical T2-weighted hypointensities in the basal ganglia, thalami and midbrain. After stopping glue sniffing, his tremors, ataxia of gait, speech and cognition partially improved. Early recognition and intervention of toluene-induced leukodystrophy could prevent ongoing morbidity and premature mortality.


Author(s):  
Dharnaben A. Patel ◽  
Dhruv J. Patel ◽  
N. D. Kantharia

Background: Diabetes Mellitus is a chronic metabolic disorder characterized by hyperglycemia, polyuria, hyperlipidemia etc, resulting from defects in insulin secretion, insulin action or both. It affects various organs of the body including the brain. Cognitive function is the thinking process of the brain. In any chronic disease evaluation of cognitive function is justified as it may affect various common day to day activities.Methods: It is a prospective, observational and non-interventional study. Thirty diabetic patients who were recently started on insulin i.e. within 7 days were enrolled in the study based on inclusion and exclusion criteria. Thirty non diabetic healthy individuals served as a control. Cognitive function was accessed by Adenbrooke’s Cognitive Examination (ACE III) at the time of enrollment.Results: The results were analysed using paired t-test. Attention, Memory and Visiospatial ability was significantly reduced in diabetic patients compared to control. Verbal fluency and language was also reduced but the change was not significant. Total ACE III score was significantly reduced in diabetic patients compared to control.Conclusions: Cognitive function is significantly reduced in Diabetic patients recently started on insulin. Hyperglycemia could be the possible reason of cognitive decline. Proper understanding of the natural history of Diabetes and the pathogenesis of cognitive decline as well as control of Diabetes can help to prevent development of cognitive dysfunction.


2017 ◽  
Vol 41 (2) ◽  
pp. 74-75
Author(s):  
Laligam Sekhar ◽  
Kyra Becker ◽  
Anne Moore ◽  
Vanessa Tran

A 40-year-old woman with prior history of headaches, left-sided weakness, and diplopia was diagnosed with right sigmoid and transverse sinus thrombosis; increased intracranial pressure and associated cerebral venous infarction that underwent 12 months anticoagulation and then stopped. She has been off anticoagulation for about a year then started not “feeling right,” and was diagnosed with pneumonia and pulmonary embolism. During her course of hospitalization, she presented with progressive headache, possible left-sided weakness, associated blurry vision, nausea, and vomiting. Magnetic resonance venogram (MRV) shows lack of flow-related signals within the left half of the distal superior sagittal sinus, left transverse sinus, and nonocclusive thrombus in the left sigmoid sinus. Mechanical sinus thrombectomy attempted without significant difference in clot burden. A transcranial Doppler (TCD) emboli monitoring exam was ordered and was performed on the bilateral internal jugular for 15 min each. Microembolic signals detected: 52 emboli per hour right internal jugular vein (IJV), and 32 emboli per hour left IJV. She was discharged on methazolamide, furosemide, topiramate, and lifelong warfarin. No major events have been reported since discharge from 2008 to 2016.


Viruses ◽  
2019 ◽  
Vol 11 (3) ◽  
pp. 232 ◽  
Author(s):  
Paweł Liberski ◽  
Agata Gajos ◽  
Beata Sikorska ◽  
Shirley Lindenbaum

Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology, and molecular genetics. We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt–Jakob disease, as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.


2010 ◽  
Vol 1 (1) ◽  
pp. 3 ◽  
Author(s):  
Vicky Chau ◽  
Sadhana Prasad ◽  
Dwight Stewart ◽  
George Heckman

Patients with Creutzfeldt–Jakob disease (CJD) may exhibit characteristic abnormalities on the electroencephalogram (EEG). However, these abnormalities have been associated with a number of cases of drug toxicity. We report a case of CJD-like syndrome associated with gabapentin. A 78-year-old man was hospitalized for recurrent falls. Three months prior to admission, gabapentin was prescribed to treat symptoms of trigeminal neuralgia. The patient subsequently presented with a two-month history of worsening gait abnormalities, negative myoclonus, and cognitive impairment. The EEG showed diffuse background slowing with larger amplitude delta discharges, which at times appeared triphasic, raising the possibility of CJD. The gait abnormalities and myoclonus resolved and the EEG normalized after the gabapentin was discontinued. Several cases of drug-induced CJD-like syndrome have been reported, mainly presenting with cognitive impairment, myoclonus, Parkinsonism, and EEG abnormalities. This patient may have been predisposed to adverse neurological effects from gabapentin owing to age, concurrent renal insufficiency, and cardiac disease. We concluded that it is imperative to include drug toxicity in the differential diagnosis of patients presenting with clinical manifestations and EEG findings suggestive of CJD, particularly in the setting of advanced age and comorbidities.


Author(s):  
Matthew J Lennon ◽  
Ben Chun Pan Lam ◽  
John Crawford ◽  
Henry Brodaty ◽  
Nicole A Kochan ◽  
...  

Abstract Background While midlife hypertension is deleterious, late-life hypertension has been associated with better cognitive outcomes in several studies. Many questions remain, including the relative benefit or harm of a blood pressure (BP) target and antihypertensive therapy of <120 in very old individuals. Methods The Sydney Memory and Aging Study (n = 1015) comprises a cohort of 70- to 90-year-olds, who were followed biennially for 8 years. Global cognition was assessed with a battery of 10 neuropsychological tests. Blood pressure was measured at Waves 1 and 2 and classified into 3 systolic groupings: group 1 (≤120 mmHg), group 2 (121–140 mmHg), and group 3 (>140 mmHg). Multiple regression, linear mixed modeling, and Cox regression examined the effect of BP and antihypertensives. Results There were no overall significant differences in global cognition or dementia between the disparate BP groups. However, in those not taking antihypertensives, the systolic BP (SBP) > 140 mmHg group had a significantly worse global cognitive trajectory compared to SBP ≤ 120 mmHg (b = −0.067, 95% CI [−0.129, −0.006], p = .030). Within the SBP ≤ 120 mmHg group those taking antihypertensives had significantly worse global cognition trajectories compared to those not taking antihypertensives even when controlling for past history of hypertension (b = −0.077, 95% CI [−0.147, −0.007], p = .030). Conclusions Untreated hypertension in old age is related to worse global cognitive decline. However, ongoing treatment at new recommendations of lower SBP targets may be related to poorer cognitive decline and should be considered carefully in older populations.


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