Jorien van der Burg

• MD, PhD
• PostDoc Position at Leiden University

Objective Weight loss is associated with clinical progression in Huntington disease (HD), but whether body weight causally affects disease onset or progression is unknown. Therefore, we aimed to assess whether genetically determined variations in body weight are causally related to age at onset in HD. Methods Using data from different recent genom...

Background: Heart failure and chronic obstructive pulmonary disease (COPD) are leading causes of disability and lead to substantial healthcare costs. The aim of this study was to evaluate the effectiveness of home telemonitoring in reducing healthcare usage and costs in patients with heart failure or COPD. Methods: The study was a retrospective obs...

UNSTRUCTURED Objective The aim of this study was to evaluate the effectiveness of home telemonitoring in reducing healthcare usage and costs in patients with heart failure or chronic obstructive pulmonary disease (COPD). Design The study was a retrospective observational study with a pre-post research design and a follow-up duration of up to 3 year...

Objective: A fundamental but still unresolved issue regarding Huntington disease (HD) pathogenesis is whether the factors that determine age at onset are the same as those that govern disease progression. Because elucidation of this issue is crucial for the development as well as optimal timing of administration of novel disease-modifying therapie...

Unintended weight loss is a hallmark of Huntington disease (HD), but it is unknown to what extent weight loss impacts the rate of disease progression. Therefore, using longitudinal data from the Enroll-HD study, we assessed the association between baseline body mass index (BMI) and the rate of clinical progression in 5821 HD mutation carriers. We f...

Liplikeczeem (lip-licking dermatitis) is een contacteczeem dat ontstaat door het frequent likken van de lippen en de omliggende huid. Door het herhaaldelijk contact met speeksel wordt de huid beschadigd en raakt vervolgens ontstoken.

An 86-year-old man presented at the Emergency Department with pain in his right leg and the inability to extend his knee after a fall. An ultrasound of the leg revealed a quadriceps tendon rupture. Surgical repair was performed and we expect complete recovery.

A 10-year-old boy consulted a dermatologist with a painful, subungual tumour on one of his toes. X-ray showed an outgrowth of the distal phalanx and histopathological investigation showed osteochondromatous proliferation. The patient was diagnosed with exostosis. Subungual exostoses are benign osteocartilaginous tumours that occur beneath the nail...

This fourth edition of Huntington's disease presents a comprehensive summary of the current knowledge of this disease, including the major scientific and clinical advances that have occurred since publication of the third edition in 2002. Completely updated and expanded, chapters in this volume are organized in five sections, which include clinical...

Weight loss is the most important non-neurological complication of Huntington's disease (HD). It correlates with disease progression and affects the quality of life of HD patients, suggesting that it could be a valuable target for therapeutic intervention. The mechanism underlying weight loss in HD is unknown. Mutant huntingtin, the protein that ca...

Huntington's disease (HD) is an inherited neurodegenerative disorder caused by a polyglutamine stretch in the huntingtin protein. Today, more than 15 years after the genetic defect underlying HD was discovered, the pathogenesis is still not well understood and there is no adequate treatment. Research into this disorder has conventionally focused on...

Huntington disease (HD) is a hereditary neurodegenerative disorder caused by an expanded number of CAG repeats in the huntingtin gene. A hallmark of HD is unintended weight loss, the cause of which is unknown. In order to elucidate the underlying mechanisms of weight loss in HD, we studied its relation to other disease characteristics including mot...

While Huntington's disease (HD) is a condition that primarily involves the basal ganglia, there is evidence to suggest that the hypothalamus is also affected. Because the osmoreceptors regulating thirst are situated in the circumventricular region of the hypothalamus, we were interested in whether altered thirst is a part of the HD phenotype. We us...

Huntington's disease (HD) is a hereditary disorder characterized by personality changes, chorea, dementia and weight loss. The cause of this weight loss is unknown. The aim of this study was to examine body weight changes and weight-regulating factors in HD using the R6/2 mouse model as a tool. We found that R6/2 mice started losing weight at 9 wee...

Song acquisition in songbird males is a prominent model system for the study of the brain mechanisms of memory. Male zebra finches (Taeniopygia guttata) learn their songs from an adult conspecific tutor early in life. Previous work has shown that exposure of males to their tutor song leads to increased expression of immediate early genes (IEGs) in...

The aromatase knockout (ArKO) mouse is estrogen deficient. Using reverse-transcription and real-time PCR, we showed that transcript levels of the N-methyl-d-aspartate (NMDA) receptor subunit NR2B are significantly higher in the hippocampus of female ArKO mice compared to wild-type (WT) littermates. Expression levels of NR1, NR2A, but not NR2C, also...