Publications by authors named "Erik Hoff"

Background: Obstructive sleep apnoea (OSA) is a common disease with breathing disturbances during sleep. Sulthiame (STM), a carbonic anhydrase (CA) inhibitor, was recently shown to reduce OSA in a significant proportion of patients. CA activity and hypoxia-inducible factor (HIF)-1α are two potential biomarkers reported in severe OSA and hypoxia.

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Background And Objectives: Autoimmune encephalitis (AIE) and paraneoplastic neurologic syndromes (PNSs) encompass a heterogeneous group of antibody-associated disorders. Both the number of syndromes and commercially available antibody tests have increased considerably over the past decade. High-quality population-based data on epidemiology of these disorders and real-world performance of antibody tests are needed.

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Objective: This systematic review aimed at synthesizing current evidence on biomarkers associated with cognitive impairment (CI) in Post-Traumatic Stress Disorder (PTSD).

Methods: A systematic literature search was conducted for studies assessing biomarkers associated with CI in PTSD.

Results: Of the 10,149 titles screened, 8 studies met our inclusion criteria.

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Background: The carbonic anhydrase inhibitor sulthiame reduces OSA severity, increases overnight oxygenation, and improves sleep quality. Insights into how sulthiame modulates OSA pathophysiologic features (endotypic traits) adds to our understanding of the breathing disorder itself, as well as the effects of carbonic anhydrases in respiratory regulation.

Research Question: How does sulthiame treatment modify endotypic traits in OSA?

Study Design And Methods: Per-protocol tertiary analysis of a randomized controlled trial with the inclusion criteria as follow: BMI, ≥ 20 to ≤ 35 kg/m; age, 18-75 years; apnea-hypopnea index (AHI) ≥ 15 events/h; Epworth sleepiness scale score, ≥ 6; as well as nonacceptance or nontolerance of positive airway pressure treatment.

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Objective: New drug treatments are under development in obstructive sleep apnea (OSA). The placebo effect is well recognized in various conditions, but its relevance in OSA is debated. In the current study we determined the influence of a placebo effect in studies of drug therapy in OSA.

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Article Synopsis
  • The study investigates how changes in brain connectivity, specifically in the rich-club of the structural covariance network, are related to Alzheimer's disease and cognitive impairments.
  • Participants included those with normal cognition, mild cognitive impairment, and Alzheimer-type dementia, with cortical thickness measured using structural MRI.
  • Results showed that lower connectivity in key brain regions (rich-club) correlates with poorer memory performance and reduced hippocampal volume, highlighting the impact of Alzheimer's on brain organization.
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Current therapies for obstructive sleep apnea (OSA) are limited by insufficient efficacy, compliance, or tolerability. An effective pharmacological treatment for OSA is warranted. Carbonic anhydrase inhibition has been shown to ameliorate OSA.

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The vascular and neurodegenerative processes related to clinical dementia cause cell loss which induces, amongst others, an increase in interstitial fluid (ISF). We assessed microvascular, parenchymal integrity, and a proxy of ISF volume alterations with intravoxel incoherent motion imaging in 21 healthy controls and 53 memory clinic patients - mainly affected by neurodegeneration (mild cognitive impairment, Alzheimer's disease dementia), vascular pathology (vascular cognitive impairment), and presumed to be without significant pathology (subjective cognitive decline). The microstructural components were quantified with spectral analysis using a non-negative least squares method.

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Blood-brain barrier (BBB) dysfunction is one of the pathophysiological mechanisms in cerebral small vessel disease (SVD). Previously, it was shown that BBB leakage volume is larger in patients with SVD compared with controls. In this study, we investigated the link between BBB leakage and cognitive decline over 2 years in patients with cSVD.

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Objectives: Obstructive sleep apnea (OSA) is a common sleep disorder that has several health hazards, including cognitive dysfunction. Studies have thus far primarily focussed on the prevalence of cognitive impairment in patients diagnosed with OSA at sleep clinics. The present study aims to investigate the prevalence of OSA at an outpatient memory clinic.

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Introduction: Anosognosia is a common but underrated symptom in dementia and has significant impact on both patients and caregivers. A proper evaluation of anosognosia is therefore desirable. There are three common methods to determine anosognosia: (1) clinical rating, (2) patient-caregiver discrepancies, and (3) prediction of performance discrepancies.

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Whole blood carbonic anhydrase activity (CAa) is increased in patients with obstructive sleep apnea (OSA). Our study investigated the influence of positive airway pressure (PAP) or CA inhibitor acetazolamide (ACT) therapy on CAa, OSA and blood pressure. Thirty-three OSA patients (21 hypertensive, body mass index (BMI) 37 ± 7 kg/m and apnea-hypopnea index (AHI) of 47 ± 31 events/hr) were followed-up after PAP treatment (compliance, 4.

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Blood-brain barrier (BBB) leakage is considered an important underlying process in both cerebral small vessel disease (cSVD) and Alzheimer's disease (AD). The objective of this study was to examine associations between BBB leakage, cSVD, neurodegeneration, and cognitive performance across the spectrum from normal cognition to dementia. Leakage was measured with dynamic contrast-enhanced magnetic resonance imaging in 80 older participants (normal cognition, n = 32; mild cognitive impairment, n = 34; clinical AD-type dementia, n = 14).

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Objective: To investigate the link between blood-brain-barrier (BBB) permeability and cerebral blood flow (CBF) and the relation with white matter hyperintensities (WMH) in cerebral small vessel disease (cSVD).

Methods: Twenty-seven patients with cSVD received dynamic susceptibility contrast and dynamic contrast-enhanced MRI to determine CBF and BBB permeability (expressed as leakage rate and volume), respectively. Structural MRI were segmented into normal-appearing white matter (NAWM) and WMH, for which a perilesional zone was defined.

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Study Objectives: The carbonic anhydrase inhibitor acetazolamide (AZT) modulates blood pressure at high altitude and reduces sleep-disordered breathing in patients with obstructive sleep apnea (OSA). We aimed to investigate the treatment effect of AZT and in combination with continuous positive airway pressure (CPAP) on blood pressure in patients with hypertension and OSA.

Methods: In a prospective, randomized, three-way crossover study, 13 male patients with hypertension and moderate to severe OSA (age 64 ± 7 years, body mass index 29 ± 4 kg/m, and mean apnea-hypopnea index 37 ± 23 events/h) received AZT, CPAP, or AZT plus CPAP for 2-week periods.

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Introduction: Cerebrospinal fluid collection by lumbar puncture (LP) is performed in the diagnostic workup of several neurological brain diseases. Reluctance to perform the procedure is among others due to a lack of standards and guidelines to minimize the risk of complications, such as post-LP headache or back pain.

Methods: We provide consensus guidelines for the LP procedure to minimize the risk of complications.

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Background And Purpose: Cerebral small vessel disease (SVD) is associated with cognitive impairment. This may be because of decreased microstructural integrity and microvascular perfusion, but data on these relationships are scarce. We determined the relationship between cognition and microvascular perfusion and microstructural integrity in SVD patients, using intravoxel incoherent motion imaging-a diffusion-weighted magnetic resonance imaging technique designed to determine microvascular perfusion and microstructural integrity simultaneously.

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Article Synopsis
  • Neuropsychological assessments (NPA) were evaluated to determine their added value alongside standard clinical evaluations in memory clinic patients.
  • In a study with 221 patients, NPA led to significant changes in diagnoses: 22% of syndromal diagnoses and 15% of etiological diagnoses were updated based on NPA findings.
  • The inclusion of NPA improved diagnostic accuracy by 18% for syndromal diagnoses and increased clinician confidence by 6-7% overall, especially benefiting patients with subjective cognitive impairment (SCI) and mild cognitive impairment (MCI).
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Article Synopsis
  • Limited information exists on how Alzheimer's disease (AD) biomarkers in cerebrospinal fluid (CSF) affect short-term prognoses alongside standard diagnostic assessments.
  • In a study involving 114 memory clinic patients, experts evaluated cognitive and functional symptoms based on routine tests, both with and without CSF biomarkers, to see how well they could predict clinical decline over 2 years.
  • The findings showed no significant difference in prediction accuracy between assessments with (74%) and without (79%) CSF biomarkers, indicating that standard diagnostic methods alone can make fairly accurate predictions about the progression of symptoms in suspected cognitive disorders.
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Photosensitivity in a patient with C9orf72 repeat expansion.

Amyotroph Lateral Scler Frontotemporal Degener

January 2017

The phenotype of C9ORF72 repeat expansions is rapidly expanding. Originally found to be the major genetic cause of familial frontotemporal dementia with amyotrophic lateral sclerosis, several other clinical characteristics have been described more recently. Here, we report on a family diagnosed with 'degenerative schizophrenia' and harbouring a C9ORF72 repeat expansion, in which at least the index patient and an uncle showed a photoparoxysmal response during electroencephalography.

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After an episode of diarrhoea, a previously healthy young man developed a splenic abscess due to invasive non-typhoidal Salmonella. The patient was presented with >1 month of fever, diffuse abdominal pain, raised C reactive protein and increased white cell count. Ultrasonography revealed a 5 × 5 cm abscess in the spleen.

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Introduction: Lumbar puncture (LP) is increasingly performed in memory clinics. We investigated patient-acceptance of LP, incidence of and risk factors for post-LP complications in memory clinic populations.

Methods: We prospectively enrolled 3868 patients (50% women, age 66 ± 11 years, mini mental state examination 25 ± 5) at 23 memory clinics.

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