scholarly journals Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration

2013 ◽  
Vol 7 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Chiadi U. Onyike ◽  
Olga Pletnikova ◽  
Kelly L. Sloane ◽  
Campbell Sullivan ◽  
Juan C. Troncoso ◽  
...  
Keyword(s):  
Psychiatric Disorder ◽  
Clinical Diagnosis ◽  
Hippocampal Sclerosis ◽  
Neuronal Loss ◽  
Executive Dysfunction ◽  
Convenience Sample ◽  
Illness Onset ◽  
Work Support ◽  
Resource Center ◽  
Johns Hopkins University

ABSTRACT Objective: To describe characteristics of hippocampal sclerosis dementia. Methods: Convenience sample of Hippocampal sclerosis dementia (HSD) recruited from the Johns Hopkins University Brain Resource Center. Twenty-four cases with post-mortem pathological diagnosis of hippocampal sclerosis dementia were reviewed for clinical characterization. Results: The cases showed atrophy and neuronal loss localized to the hippocampus, amygdala and entorrhinal cortex. The majority (79.2%) had amnesia at illness onset, and many (54.2%) showed abnormal conduct and psychiatric disorder. Nearly 42% presented with an amnesic state, and 37.5% presented with amnesia plus abnormal conduct and psychiatric disorder. All eventually developed a behavioral or psychiatric disorder. Disorientation, executive dysfunction, aphasia, agnosia and apraxia were uncommon at onset. Alzheimer disease (AD) was the initial clinical diagnosis in 89% and the final clinical diagnosis in 75%. Diagnosis of frontotemporal dementia (FTD) was uncommon (seen in 8%). Conclusion: HSD shows pathological characteristics of FTD and clinical features that mimic AD and overlap with FTD. The findings, placed in the context of earlier work, support the proposition that HSD belongs to the FTD family, where it may be identified as an amnesic variant.

scholarly journals An autopsy-proven case of Corticobasal degeneration heralded by Pontine infarction

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dallah Yoo ◽  
Sung-Hye Park ◽  
Sungwook Yu ◽  
Tae-Beom Ahn
Keyword(s):  
Neurodegenerative Disorders ◽  
Clinical Manifestations ◽  
Neuronal Loss ◽  
Corticobasal Degeneration ◽  
Executive Dysfunction ◽  
Lacunar Infarction ◽  
Cortical Atrophy ◽  
Dystonic Posturing ◽  
Proven Case ◽  
The Right

Abstract Background Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Case presentation A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration. Conclusions The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.

scholarly journals Cortical neuronal loss and hippocampal sclerosis are not detected by voxel-based morphometry in individual epilepsy surgery patients

2009 ◽  
Vol 30 (10) ◽  
pp. 3351-3360 ◽  
Author(s):  
Sofia H. Eriksson ◽  
Maria Thom ◽  
Mark R. Symms ◽  
Niels K. Focke ◽  
Lillian Martinian ◽  
...  
Keyword(s):  
Epilepsy Surgery ◽  
Hippocampal Sclerosis ◽  
Neuronal Loss ◽  
Voxel Based Morphometry

Accuracy of Clinical Diagnosis in Parkinsonism — A Prospective Study

1991 ◽  
Vol 18 (3) ◽  
pp. 275-278 ◽  
Author(s):  
A.H. Rajput ◽  
B. Rozdilsky ◽  
Alex Rajput
Keyword(s):  
Clinical Diagnosis ◽  
Correct Diagnosis ◽  
Neurofibrillary Tangle ◽  
Neuronal Loss ◽  
Final Diagnosis ◽  
Epidemiological Studies ◽  
Olivopontocerebellar Atrophy ◽  
Lewy Body Pathology ◽  
A Prospective Study ◽  
Critical Literature

ABSTRACT:Clinical diagnosis of Parkinson's syndrome (PS) is reasonably easy in most cases but the distinction between different variants of PS may be difficult in early cases. The correct diagnosis is not only important for counselling and management of patients but also in conducting pharmacological and epidemiological studies. There is very little critical literature on the pathological verification of the clinical diagnosis in PS. We report our 22 year experience to address that issue. Between 1968 and 1990, 65 PS patients came to autopsy. Complete data are available in 59 (M- 50, F-19) cases. The initial diagnosis made by a qualified neurologist was idiopathic Parkinson's disease (IPD) in 43 cases. Of those 28 (65%) had Lewy body pathology. After a mean duration of 12 years the final diagnosis was IPD in 41 cases which was confirmed in 31 (76%). The IPD could not be clinically distinguished from cases with severe substantia nigra neuronal loss without inclusions or from those with neurofibrillary tangle inclusions and neuronal loss at the anatomical sites typically involved in IPD. All progressive supra-nuclear palsy, olivopontocerebellar atrophy, Jakob-Creutzfeldt's disease and the majority of the multiple system atrophy cases were diagnosed correctly during life. The correct clinical diagnosis in most non-IPD variants of PS was possible within 5 years of onset (range: 2 months to 18 years). We recommend that studies aimed at including only the IPD cases restrict the enrollment to those cases that have had PS motor manifestations for five years or longer duration.

scholarly journals Survey of Physicians Concerning the Use of Chest Radiography in the Diagnosis of Pneumonia in Out-Patients

1997 ◽  
Vol 8 (2) ◽  
pp. 95-98
Author(s):  
Thomas J Marrie
Keyword(s):  
Clinical Diagnosis ◽  
Chest Radiograph ◽  
Chest Radiography ◽  
Chest Radiographs ◽  
Smoking History ◽  
Convenience Sample ◽  
Clinical Appearance ◽  
Study Population ◽  
Physical Findings

OBJECTIVE: To determine how physicians use chest radiography in the diagnosis of pneumonia in ambulatory patients.STUDY POPULATION: A convenience sample of 176 Nova Scotia family physicians and internists selected to represent all geographic areas of the province proportional to population.STUDY INSTRUMENT: A 35-item questionnaire covering demographics, experience with out-patients with pneumonia, use of chest radiographs to make this diagnosis and factors that were considered important in the decision to perform initial and follow-up chest radiographs. Two skill-testing questions were also included.RESULTS: One hundred and fourteen of 176 (64.7%) responded; 88% had treated out-patients with pneumonia in the previous three months. Fifty-seven per cent of physicians requested chest radiographs on 90% to 100% of out-patients in whom they had made a clinical diagnosis of pneumonia. These physicians were more likely to be internists and to have graduated before 1970. Factors that ranked most important in the decision to request the initial chest radiograph were clinical appearance, respiratory distress and physical findings, while age and smoking history contributed most to the decision to perform a follow-up chest radiograph.CONCLUSIONS: There is considerable variability among physicians in requesting chest radiographs on out-patients with a clinical diagnosis of pneumonia. Physician and patient factors contribute to this variability.

scholarly journals Reconcile the debate over protective effects of BCG vaccine against COVID-19

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Wei Fu ◽  
Pei-Chuan Ho ◽  
Chia-Lun Liu ◽  
Kai-Teh Tzeng ◽  
Nawar Nayeem ◽  
...  
Keyword(s):  
Potential Role ◽  
Early Stage ◽  
Bcg Vaccine ◽  
Protective Effects ◽  
Young Population ◽  
Resource Center ◽  
Country Level ◽  
Johns Hopkins University ◽  
Clinical Scenarios ◽  
Program Data

AbstractWhile awaiting the COVID-19 vaccines, researchers have been actively exploring the effectiveness of existing vaccines against the new virus, among which the BCG vaccine (Bacillus Calmette-Guérin) receives the most attention. While many reports suggest a potential role for BCG immunization in ameliorating SARS-CoV-2 infection, these findings remain controversial. With country-level COVID-19 outbreak data from Johns Hopkins University Coronavirus Resource Center, and BCG program data from World Atlas of BCG Policies and Practices and WHO/UNICE, we estimated a dynamic model to investigate the effect of BCG vaccination across time during the pandemic. Our results reconcile these varying reports regarding protection by BCG against COVID-19 in a variety of clinical scenarios and model specifications. We observe a notable protective effect of the BCG vaccine during the early stage of the pandemic. However, we do not see any strong evidence for protection during the later stages. We also see that a higher proportion of vaccinated young population may confer some level of communal protection against the virus in the early pandemic period, even when the proportion of vaccination in the older population is low. Our results highlight that while BCG may offer some protection against COVID-19, we should be cautious in interpreting the estimated effectiveness as it may vary over time and depend on the age structure of the vaccinated population.

scholarly journals GERIATRIC SYNDROMES AND SARS-COV-2: MORE THAN JUST BEING OLD

2020 ◽  
pp. 1-3
Author(s):  
I. Aprahamian ◽  
M. Cesari
Keyword(s):  
Economic Consequences ◽  
World Health ◽  
Disease Spread ◽  
Major Health ◽  
Economic Problems ◽  
Resource Center ◽  
Large Size ◽  
Johns Hopkins University ◽  
The World ◽  
Health Organization

At the end of 2019, China released a warning about an outbreak of pneumonia due to unknown causes (1). It was subsequently identified as a new coronavirus, named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a zoonotic large size RNA-based genome virus, responsible for this infectious disease (called Covid-19) (2). The disease spread worldwide. On March 11th 2020, the World Health Organization (WHO) declared the state of pandemic, foretelling a catastrophe with major health and economic consequences. On April 3th, 1,039,166 cases of SARS-CoV-2 and 55,092 deaths have already been reported around the world (Johns Hopkins University & Medicine Coronavirus Resource Center; https://coronavirus.jhu.edu/map.html; last access April 4th). Unfortunately, the complete clinical and epidemiological picture of Covid-19 is still unclear. This coronavirus is highly contagious (primarily airborne, but also through contact with infected surfaces), and its severity ranges from asymptomatic/mild forms (the majority) to very severe cases (3). Currently, we are facing several challenges of difficult solution, such as lack of hospital and intensive care beds, shortage of personal protective equipment for health professionals, the loneliness and economic problems of isolated people, and the race for a vaccine and effective treatments.

scholarly journals Pre- and post-operative Wisconsin card sorting test performance in patients with temporal lobe epilepsy due to hippocampal sclerosis

2007 ◽  
Vol 1 (2) ◽  
pp. 173-180 ◽  
Author(s):  
Luciana Tisser ◽  
Andre Palmini ◽  
Eliseu Paglioli ◽  
Mirna Portuguez ◽  
Ney Azambuja ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Test Performance ◽  
Hippocampal Sclerosis ◽  
Executive Dysfunction ◽  
Wisconsin Card Sorting Test ◽  
Card Sorting ◽  
Neuropsychological Profile ◽  
Temporal Structures ◽  
Sorting Test

Abstract Patients with temporal lobe epilepsy due to hippocampal sclerosis (TLE/HS) have a distinct neuropsychological profile, but there is still debate on whether executive dysfunction is part of this profile and also whether temporal lobe surgery can modify this dysfunction. Objective: To study the presence and reversibility of executive dysfunction in patients with unilateral TLE/HS. Methods: Twenty-five patients with refractory seizures due to TLE/HS underwent presurgical evaluation which included the application of the Wiconsin Card Sorting Test (WCST). Nineteen were re-evaluated in follow up, at least 6 months after selective amygdalo-hippocampectomy (SAH). Twenty-two control subjects matched for age and education also performed the WCST. Results: Sixteen of the 25 patients (64%) completed fewer than four categories in the WCST whereas only 4 of the 22 controls (18%) did not complete at least four categories (p<0.005). In addition, the performance of the patients involved significantly more perseverative responses and errors compared to controls. The patient group demonstrated significant post-operative improvement in many measures of the WCST following SAH. Conclusions: These findings support the presence of executive dysfunction in patients with TLE/HS and suggest that such dysfunction can be partially reversed by selective resection of epileptogenic mesial temporal structures.

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