ZNA Middelheim General Hospital

Antwerpen, Belgium

ZNA Middelheim General Hospital

Antwerpen, Belgium

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Van Cauwelaert P.,ZNA Middelheim General Hospital | Dalton J.E.,Health Outcomes Sciences | Sessler D.I.,Cleveland Clinic | Sessler D.I.,McMaster University
Anaesthesia | Year: 2011

We conducted a randomised controlled trial to compare the efficacy of underbody forced-air warming (Arizant Healthcare Inc, Eden Prairie, MN, USA) with an underbody resistive heating mattress (Inditherm Patient Warming System, Rotherham, UK) and passive insulation in 129 patients having hypothermic cardiac surgery with cardiopulmonary bypass. Patients were separated from cardiopulmonary bypass at a core temperature of 35 °C and external warming continued until the end of surgery. Before cardiopulmonary bypass, the temperature-vs-time slopes were significantly greater in both active warming groups than in the passive insulation group (p < 0.001 for each). However, the slopes of forced-air and resistive warming did not differ (p = 0.55). After cardiopulmonary bypass, the rate of rewarming was significantly greater with forced-air than with resistive warming or passive insulation (p < 0.001 for each), while resistive warming did not differ from passive insulation (p = 0.14). However, absolute temperature differences among the groups were small. © 2011 The Authors.

Mehta A.,University College London | Beck M.,University of Mainz | Eyskens F.,ZNA Middelheim General Hospital | Feliciani C.,Catholic University of the Sacred Heart | And 6 more authors.
QJM | Year: 2010

Fabry disease is an X-linked inherited condition due to the absence or reduction of α-galactosidase activity in lysosomes, that results in accumulation of globotriaosylceramide (Gb3) and related neutral glycosphingolipids. Manifestations of Fabry disease include serious and progressive impairment of renal and cardiac function. In addition, patients experience pain, gastrointestinal disturbance, transient ischaemic attacks and strokes. Additional effects on the skin, eyes, ears, lungs and bones are often seen. The first symptoms of classic Fabry disease usually appear in childhood. Despite being X-linked, females can suffer the same severity of symptoms as males, and life expectancy is reduced in both females and males. Enzyme replacement therapy (ERT) can stabilize the progression of the disease. The rarity of the classic form of Fabry disease, however, means that there is a need to improve the knowledge and understanding that the majority of physicians have concerning Fabry disease, in order to avoid misdiagnosis and/or delayed diagnosis. This review aims to raise awareness of the signs and symptoms of Fabry disease; to provide a general diagnostic algorithm and to give an overview of the effects of ERT and concomitant treatments. We highlight a need to develop comprehensive international guidelines to optimize ERT and adjunctive therapy in patients with Fabry disease, including females and children. © The Author 2010. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved.

PubMed | icoMetrix, Unite dEtude du Mouvement, ZNA Middelheim General Hospital and Vrije Universiteit Brussel
Type: | Journal: Cerebellum & ataxias | Year: 2015

In the majority of right-handed subjects, language processing is subserved by a close interplay between the left cerebral hemisphere and right cerebellum. Within this network, the dominant fronto-insular region and the contralateral posterior cerebellum are crucially implicated in oral language production.We report atypical anatomoclinical findings in a right-handed patient with an extensive right cerebellar infarction and an older left fronto-insular stroke. Standardised neurolinguistic and neurocognitive test batteries were performed. In addition, fMRI, DTI, and SPECT results are reported. In this patient, disruption of the cerebellocerebral language network due to vascular damage in the left fronto-insular region and right posterior inferior cerebellar artery (PICA) territory did not induce any speech or language deficits. By contrast, executive and behavioural disturbances were found after the right cerebellar stroke. Evidence from fMRI and DTI suggests atypical bilateral language representation (Laterality Index=+0,11). At the cerebellar level, fMRI showed more activated voxels in the left than in the right hemisphere (Laterality Index=+0,66).We hypothesise congenital bilateral language representation in this patient which might be more advantageous than a typically lateralised distribution of linguistic functions to compensate acute damage to critical language regions. The more activated left cerebellum possibly compensated the functional loss in the right cerebellum after acute damage due to bilateral organisation of language function. However, more research is needed to confirm this hypothesis.

De Smet H.J.,Ghent University | Marien P.,ZNA Middelheim General Hospital | Marien P.,Vrije Universiteit Brussel
Cerebellum | Year: 2012

The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms and neurobehavioural abnormalities that typically develop in children following posterior fossa tumour resection. Although PFS has been documented in more than 350 paediatric cases, reports of adult patients with a vascular aetiology are extremely rare. In addition, the pathophysiological substrate of the syndrome remains unclear. We report an adult patient with PFS after surgical evacuation of a cerebellar bleeding. After 45 days of (akinetic) mutism, the patient's cognitive and behavioural profile closely resembled the "cerebellar cognitive-affective syndrome". A quantified SPECT study showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, executive functioning, spatial cognition and affective regulation. We hypothesize that cerebello-cerebral diaschisis might be an important pathophysiological mechanism underlying akinetic mutism, cognitive deficits and behavioural- affective changes in adult patients with PFS. ©Springer Science+Business Media, LLC 2011.

Marien P.,Royal Flemish Academy of Belgium for science and the Arts | Marien P.,ZNA Middelheim General Hospital | Marien P.,Vrije Universiteit Brussel | De Smet E.,MS en Revalidatiecentrum | And 5 more authors.
Cerebellum | Year: 2013

Apraxic agraphia is a peripheral writing disorder caused by neurological damage. It induces a lack or loss of access to the motor engrams that plan and programme the graphomotor movements necessary to produce written output. The neural network subserving handwriting includes the superior parietal region, the dorsolateral and medial premotor cortex and the thalamus of the dominant hemisphere. Recent studies indicate that the cerebellum may be involved as well. To the best of our knowledge, apraxic agraphia has not been described on a developmental basis. This paper reports the clinical, neurocognitive and (functional) neuroimaging findings of a 15-year-old left-handed patient with an isolated, non-progressive developmental handwriting disorder consistent with a diagnosis of «apraxic dysgraphia». Gross motor coordination problems were objectified as well but no signs of cerebellar, sensorimotor or extrapyramidal dysfunction of the writing limb were found to explain the apraxic phenomena. Brain MRI revealed no supra- and infratentorial damage but quantified Tc-99m-ECD SPECT disclosed decreased perfusion in the anatomoclinically suspected prefrontal and cerebellar brain regions crucially involved in the planning and execution of skilled motor actions. This pattern of functional depression seems to support the hypothesis that «apraxic dysgraphia» might reflect incomplete maturation of the cerebello-cerebral network involved in handwriting. In addition, it is hypothesized that "apraxic dysgraphia" may have to be considered to represent a distinct nosological category within the group of the developmental dyspraxias following dysfunction of the cerebello-cerebral network involved in planned actions. © 2012 Springer Science+Business Media, LLC.

PubMed | Ludwig Maximilians University of Munich, Human Motor Control Section, Vrije Universiteit Brussel, Yale University and 8 more.
Type: Journal Article | Journal: Cerebellum (London, England) | Year: 2016

The cerebellum is involved in sensorimotor operations, cognitive tasks and affective processes. Here, we revisit the concept of the cerebellar syndrome in the light of recent advances in our understanding of cerebellar operations. The key symptoms and signs of cerebellar dysfunction, often grouped under the generic term of ataxia, are discussed. Vertigo, dizziness, and imbalance are associated with lesions of the vestibulo-cerebellar, vestibulo-spinal, or cerebellar ocular motor systems. The cerebellum plays a major role in the online to long-term control of eye movements (control of calibration, reduction of eye instability, maintenance of ocular alignment). Ocular instability, nystagmus, saccadic intrusions, impaired smooth pursuit, impaired vestibulo-ocular reflex (VOR), and ocular misalignment are at the core of oculomotor cerebellar deficits. As a motor speech disorder, ataxic dysarthria is highly suggestive of cerebellar pathology. Regarding motor control of limbs, hypotonia, a- or dysdiadochokinesia, dysmetria, grasping deficits and various tremor phenomenologies are observed in cerebellar disorders to varying degrees. There is clear evidence that the cerebellum participates in force perception and proprioceptive sense during active movements. Gait is staggering with a wide base, and tandem gait is very often impaired in cerebellar disorders. In terms of cognitive and affective operations, impairments are found in executive functions, visual-spatial processing, linguistic function, and affective regulation (Schmahmanns syndrome). Nonmotor linguistic deficits including disruption of articulatory and graphomotor planning, language dynamics, verbal fluency, phonological, and semantic word retrieval, expressive and receptive syntax, and various aspects of reading and writing may be impaired after cerebellar damage. The cerebellum is organized into (a) a primary sensorimotor region in the anterior lobe and adjacent part of lobule VI, (b) a second sensorimotor region in lobule VIII, and (c) cognitive and limbic regions located in the posterior lobe (lobule VI, lobule VIIA which includes crus I and crus II, and lobule VIIB). The limbic cerebellum is mainly represented in the posterior vermis. The cortico-ponto-cerebellar and cerebello-thalamo-cortical loops establish close functional connections between the cerebellum and the supratentorial motor, paralimbic and association cortices, and cerebellar symptoms are associated with a disruption of these loops.

Marien P.,ZNA Middelheim General Hospital | Wackenier P.,ZNA Middelheim General Hospital | De Surgeloose D.,ZNA Middelheim General Hospital | De Deyn P.P.,ZNA Middelheim General Hospital | Verhoeven J.,City University London
Cerebellum | Year: 2010

Little is known about the neurobiological substrate of developmental coordination disorder (DCD), a neuro-developmental syndrome with significant, negative impact on the motor, cognitive and affective level throughout lifespan. This paper reports the clinical, neurocognitive and neuroradiological findings of a 19-year-old patient with typical DCD. As demonstrated by mild ataxia and a close semiological correspondence with the recently acknowledged 'cerebellar cognitive affective syndrome', clinical and neurocognitive investigations unambiguously indicated functional disruption of the cerebellum. Structural MRI of the brain confirmed cerebellar involvement revealing a slight anterior/superior asymmetry of vermal fissures consistent with rostral vermisdysplasia. Although this abnormality of vermal fissuration is generally considered an incidental neuroradiological finding without any clinical relevance, a potentially subtle impact on the developmental level has never been formally excluded. In addition to a generally decreased perfusion of the cerebellum, a quantified Tc-99m-ECD SPECT disclosed functional suppression of the anatomoclinically suspected supratentorial regions involved in the execution of planned actions, visuo-spatial processing and affective regulation. Based on these findings, it is hypothesised that the cerebellum is crucially implicated in the pathophysiologcial mechanisms of DCD, reflecting disruption of the cerebello-cerebral network involved in the execution of planned actions, visuo-spatial cognition and affective regulation. © 2010 Springer Science+Business Media, LLC.

Moens M.,Universitair Ziekenhuis Brussel | Sunaert S.,Catholic University of Leuven | Marien P.,ZNA Middelheim General Hospital | Marien P.,Vrije Universiteit Brussel | And 7 more authors.
Neuroradiology | Year: 2012

Introduction: Although spinal cord stimulation (SCS) is widely used for chronic neuropathic pain after failed spinal surgery, little is known about the underlying physiological mechanisms. This study aims to investigate the neural substrate underlying short-term (30 s) SCS by means of functional magnetic resonance imaging in 20 patients with failed back surgery syndrome (FBSS). Methods: Twenty patients with FBSS, treated with externalized SCS, participated in a blocked functional magnetic resonance imaging design with stimulation and rest phases of 30 s each, repeated eight times in a row. During scanning, patients rated pain intensity over time using an 11-point numerical rating scale with verbal anchors (0 = no pain at all to 10 = worst pain imaginable) by pushing buttons (left hand, lesser pain; right hand, more pain). This scale was back projected to the patients on a flat screen allowing them to manually direct the pain indicator. To increase the signal-to-noise ratio, the 8-min block measurements were repeated three times. Results: Marked deactivation of the bilateral medial thalamus and its connections to the rostral and caudal cingulate cortex and the insula was found; the study also showed immediate pain relief obtained by short-term SCS correlated negatively with activity in the inferior olivary nucleus, the cerebellum, and the rostral anterior cingulate cortex. Conclusions: Results indicate the key role of the medial thalamus as a mediator and the involvement of a corticocerebellar network implicating the modulation and regulation of averse and negative affect related to pain. The observation of a deactivation of the ipsilateral antero-medial thalamus might be used as a region of interest for further response SCS studies. © 2012 Springer-Verlag.

Vandenborre D.,Vrije Universiteit Brussel | Vandenborre D.,Rehabilitation Center | van Dun K.,Vrije Universiteit Brussel | Marien P.,Vrije Universiteit Brussel | Marien P.,ZNA Middelheim General Hospital
Brain and Cognition | Year: 2015

Objectives: Apraxic agraphia (AA) is a peripheral writing disorder generally considered to result from a causative lesion in the parietal and/or prefrontal lobe of the language dominant hemisphere (De Smet, Engelborghs, Paquier, De Deyn, & Mariën, 2011). De Smet et al. (2011), however, confirmed that AA might be associated with lesions outside the typical language areas such as the cerebellum or the thalamus. We report a 32-year-old ambidextrous patient with a left frontal lobectomy who following bilateral thalamic damage developed AA. Method: Detailed neurolinguistic and neurocognitive test results were obtained after resection of an extensive left frontal lobe tumour by means of a set of standardised tests. Repeated investigations were performed after a bithalamic stroke. Functional imaging was performed by means of quantified SPECT. Results: Normal neurolinguistic test results were obtained after tumour resection. Neurocognitive test results, however, showed a dysexecutive syndrome and frontal behavioural deficits, including response inhibition. AA occurred after a bithalamic stroke while non-handwriting written language skills, such as typing, were normal. Quantified SPECT showed a significant bifrontal hypoperfusion. Conclusion: Neurolinguistic follow-up findings and SPECT evidence in this unique patient with bithalamic damage for the first time indicate that AA in the alphabetic script may result from diaschisis affecting the frontal writing centre. The findings suggest that the thalamus is critically implicated in the neural network subserving graphomotor processing. © 2015 Elsevier Inc.

D'aes T.,Vrije Universiteit Brussel | Marien P.,Vrije Universiteit Brussel | Marien P.,ZNA Middelheim General Hospital
Cerebellum | Year: 2015

Although insights in cerebellar neurocognition and affect are continuously growing, little is known about the role of the brainstem in cognitive and behavioural processing. In this paper, it is hypothesized that the brainstem is an inherent functional part of the cerebellocerebral network subserving cognition and affect, and that isolated brainstem damage may cause a constellation of symptoms closely resembling the cerebellar cognitive affective syndrome (CCAS) following cerebellar pathology. In order to investigate these premises, the available literature on cognitive and affective disturbances following brainstem lesions was critically reviewed starting from the pioneer descriptions in the 1950s till June 2012. Three personal cases were added to a study group of 75 cases with isolated vascular brainstem damage. In a cohort of 30 patients that allowed construction of anatomoclinical correlations in a reliable way, a range of cognitive and behavioural symptoms, typically associated with impairment of cortical or limbic areas, were identified. Executive dysfunction, attentional deficits and a decline in general intellectual capacity represent the most common cognitive findings, but memory, visuospatial skills, language and praxis may be impaired as well. Almost half of the cases presented with behavioural or affective changes. Analysis of SPECT findings indicates that functional suppression of frontal, parietal and to a lesser extent also the temporal areas are common phenomena after isolated brainstem stroke. As reflected by diaschisis affecting the cerebellocerebral network, a loss of excitatory input from the brainstem to the cerebellum and cerebrum may induce disruption of several cortical regions as well as emotional control centres resulting in and a constellation of symptoms closely resembling the CCAS. The pathophysiological mechanism underlying brainstem-induced cognitive and affective disturbances is discussed. © 2014, Springer Science+Business Media New York.

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