Gustave Moonen

• MD PhD
• Professor Emeritus at University of Liège

Functional neurological disorders consist of a group of neurological symptoms and syndromes for which a known “organic” cause cannot be identified. They still represent one of the most difficult diagnostic challenge for the neurologist, who can only rely on clinical criteria. Functional gait and movement disorders represent an important subgroup of...

Functional neurological disorders consist of a group of neurological symptoms and syndromes for which a known "organic" cause cannot be identified. They still represent one of the most difficult diagnostic challenge for the neurologist, who can only rely on clinical criteria. Functional gait and movement disorders represent an important subgroup of...

Les h o q u e t s : pa s to u j o u r s b a n a L s hiccups, not aLways unremarkabLe summary : Hiccups are most often benign and of short duration. However, they may also be persistent (> 48h) or even refractory (> 1 month). In such cases, they markedly alter the quality of life and can lead to severe impairment of health. We here review hiccups pa...

Hiccups are most often benign and of short duration. However, they may also be persistent (superior to 48h) or even refractory (superior to 1 month). In such cases, they markedly alter the quality of life and can lead to severe impairment of health. We here review hiccups pathophysiology, etiologies, work up and treatment. We suggest that hiccups s...

Neurological paraneoplastic syndromes are non-metastatic complications of systemic cancers, often resulting from an immune response triggered by the crossed expression of neuronal antigens by tumour cells. Several neurological syndromes such as cerebellar degeneration, sensory neuronopathy, limbic encephalitis, encephalomyelitis or the Lambert-Eato...

We discuss the diagnostic workup of a 62 year old woman without any significant past medical history. We take this opportunity to point out three aspects : 1. The necessary contextualization of the whole process allowing to avoid unrealistic differentials; 2. The requirement to prioritize the diagnostic tests as a function of their expected contrib...

We report the diagnostic workup of a 75-year old woman seen in the outpatient clinic and complaining of a very common symptom that is walking difficulty and falls. We stress the complexity of the clinical examination and of the pathophysiology of equilibrium and gait disorders. They are numerous aetiologies that are very often associated in elderly...

We report the case of a young patient who was seen at the outpatient clinic for recurring vertigo. The diagnosis was vestibular migraine. Considering the long delay between the onset of symptoms and the definite diagnosis, we found it appropriate to review the diagnostic workup in such cases which is multidisciplinary, implying otologists, ophtalmo...

The migration of cortical projection neurons is a multistep process characterized by dynamic cell shape remodeling. The molecular basis of these changes remains elusive, and the present work describes how microRNAs (miRNAs) control neuronal polarization during radial migration. We show that miR-22 and miR-124 are expressed in the cortical wall wher...

Neurotrophins are key players of neural development by controlling cell death programs. However, the signaling pathways that mediate their selective responses in different populations of neurons remain unclear. In the mammalian cochlea, sensory neurons differentiate perinatally into type I and II populations both expressing TrkB and TrkC, which bin...

SV2A, SV2B and SV2C are synaptic vesicle proteins that are structurally related to members of the major facilitator superfamily (MFS). The function and transported substrate of the SV2 proteins is not clearly defined although they are linked to neurotransmitters release in a presynaptic calcium concentration-dependent manner. SV2A and SV2B exhibit...

Aims: Synaptic vesicle proteins 2 (SV2) are neuronal vesicles membrane glycoproteins that appear as important targets in the treatment of partial and generalized epilepsies. Therefore, we analysed the expression of SV2 isoforms in the hippocampus of patients with temporal lobe epilepsy (TLE). Methods: SV2A, SV2B and SV2C immunostaining and Quant...

Cerebrotendinous xanthomatosis (CTX) is a rare and treatable autosomal recessive disease. The diagnosis should be suspected in the presence of a suggestive clinical triad characterized by early-onset cataract, tendinous xanthomata and neurological symptoms and signs, notably cerebellar ataxia, mental retardation and pyramidal syndrome.The diagnosis...

Hearing requires an optimal afferent innervation of sensory hair cells by spiral ganglion neurons in the cochlea. Here we report that complementary expression of ephrin-A5 in hair cells and EphA4 receptor among spiral ganglion neuron populations controls the targeting of type I and type II afferent fibres to inner and outer hair cells, respectively...

The goal of our study was to investigate different aspects of sleep, namely the sleep-wake cycle and sleep stages, in the vegetative state/unresponsive wakefulness syndrome (VS/UWS) and minimally conscious state (MCS). 24h polysomnography was performed in 20 patients in a UWS (n=10) or in a MCS (n=10) due to brain injury. The data were first tested...

Histoire c l i n i qu e Nous rapportons le cas d'un homme de 33 ans reçu à la consultation de neurologie pour des lombalgies mécaniques. L'examen clinique démontre essentiellement une surcharge pondérale importante et un pli fessier asymétrique, en baïonnette (fig. 1), qui nous fait évoquer la possibilité d'une moëlle attachée. L'anamnèse complémen...

Today's market in micro-wind turbines is in constant development introducing more efficient solutions for the future. Besides the private use of tower supported turbines, opportunities to integrate wind turbines in the built environment arise. The Integrated Roof Wind Energy System (IRWES) presented in this work is a modular roof structure integrat...

Wind is an attractive renewable source of energy. Recent innovations in research and design have reduced to a few alternatives with limited impact on residential construction. Cost effective solutions have been found at larger scale, but storage and delivery of energy to the actual location it is used, remain a critical issue. The Integrated Roof W...

The migration of cortical interneurons is characterized by extensive morphological changes that result from successive cycles of nucleokinesis and neurite branching. Their molecular bases remain elusive, and the present work describes how p27(Kip1) controls cell-cycle-unrelated signaling pathways to regulate these morphological remodelings. Live im...

Natalizumab-associated progressive multifocal leukoencephalopathy (N-PML) in multiple sclerosis (MS) is due to CNS infection by the opportunistic JC virus (JCV). As of December 2011, 193 confirmed cases of N-PML have been observed, giving rise to an overall risk of approximately 0.202%.(1) N-PML pathogenesis remains partially elusive although risk...

Sudden sensorineural hearing loss is a perplexing entity in otology. Susac's syndrome (also called retinocochleocerebral vasculopathy) is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear, and usually affects women in young adulthood. We describe the clinical aspects, radiographic findings, and management of one s...

The relationship between sunlight exposure and the incidence of multiple sclerosis and the understanding of immunomodulatory effects of vitamin D triggered, in recent years, a broad range of investigations. Immunological studies performed in vitro and in vivo have demonstrated how tolerogenic vitamin D can be. Epidemiological studies confirmed an i...

MicroRNAs (miRNAs) are non-coding RNAs that promote post-transcriptional silencing of genes involved in a wide range of developmental and pathological processes. It is estimated that most protein-coding genes harbor miRNA recognition sequences in their 3' untranslated region and are thus putative targets. While functions of miRNAs have been extensi...

Les surdites de perception se caracterisent, pour la plupart, par une atteinte de l'oreille interne, plus particulierement des cellules ciliees et des neurones auditifs. A l'heure actuelle, aucun traitement permettant de restaurer la fonction auditive ne peut etre propose. La regeneration des cellules ciliees de la portion auditive de l'oreille int...

No clinical test is currently available and validated to measure the maximum walking speed (WS) of multiple sclerosis (MS) patients. Since the Timed 25-Foot Walk Test (T25FW) is performed with a static start, it takes a significant proportion of the distance for MS patients to reach their maximum pace. In order to capture the maximum WS and to quan...

Motor fatigue and ambulation impairment are prominent clinical features of people with multiple sclerosis (pMS). We hypothesized that a multimodal and comparative assessment of walking speed on short and long distance would allow a better delineation and quantification of gait fatigability in pMS. Our objectives were to compare 4 walking paradigms:...

Statistical comparisons of the MWS (Student T-tests) in the T25FW, the T25FW+, the T100MW and the T500MW across different subsets of the pMS population stratified according to their pyramidal (P), cerebellar (C) and sensitive (S) Functional System Scores (FS). (DOC)

Pain, suffering and positive emotions in patients in vegetative state/unresponsive wakefulness syndrome (VS/UWS) and minimally conscious states (MCS) pose clinical and ethical challenges. Clinically, we evaluate behavioural responses after painful stimulation and also emotionally-contingent behaviours (e.g., smiling). Using stimuli with emotional v...

Cranial neuropathies are frequent and their semiological analysis is the basis of the diagnostic workup. This is even more true in the case of multiple cranial neuropathies. We here propose a diagnostic exercise in the case of a simultaneous cranial nerves IX (glossopharyngeal), X (vagus) and XI (spinal) deficit. This case exemplifies that knowledg...

Transient impairment of consciousness frequently prompts the patient to consult a neurologist or a cardiologist. Detailed medical history and physical examination allow to distinguish fainting from epileptic seizure, metabolic or psychogenic events. We report the history of an 83-year-old woman who presented a transient loss of consciousness.The va...

Early detection of progressive multifocal leucoencephalopathy (PML) in the setting of natalizumab therapy currently is performed by rapid evaluation of new symptoms occurring in treated patients. The role of MR scanning has not been investigated but holds promise since MR detection is highly sensitive for PML lesions. The authors report a case of p...

Development of a renewable energy policy has become a priority task among researchers and governors all over the world due to an increase in energy consumption, rise in prices for fossil fuels and electricity, global climate change, environmental disasters, etc. Regarding ecological value and economical prospects, wind energy generation is consider...

Patients in a minimally conscious state (MCS) show restricted signs of awareness but are unable to communicate. We assessed cerebral glucose metabolism in MCS patients and tested the hypothesis that this entity can be subcategorized into MCS− (i.e., patients only showing nonreflex behavior such as visual pursuit, localization of noxious stimulation...

The adult brain most probably reaches its highest degree of plasticity with the lifelong generation and integration of new neurons in the hippocampus and olfactory system. Neural precursor cells (NPCs) residing both in the subgranular zone of the dentate gyrus and in the subventricular zone of the lateral ventricles continuously generate neurons th...

Consciousness can be defined by two components: arousal and awareness. Disorders of consciousness (DOC) are characterized by a disrupted relationship between these two components. Coma is described by the absence of arousal and, hence, of awareness whereas the vegetative state is defined by recovery of arousal in the absence of any sign of awarenes...

Monitoring the level of consciousness in brain-injured patients with disorders of consciousness is crucial as it provides diagnostic and prognostic information. Behavioral assessment remains the gold standard for assessing consciousness but previous studies have shown a high rate of misdiagnosis. This study aimed to investigate the usefulness of el...

Functional neuroimaging and electrophysiology studies are changing our understanding of patients with coma and related states. Some severely brain damaged patients may show residual cortical processing in the absence of behavioural signs of consciousness. Given these new findings, the diagnostic errors and their potential effects on treatment as we...

Previous European surveys showed the support of healthcare professionals for treatment withdrawal [i.e., artificial nutrition and hydration (ANH) in chronic vegetative state (VS) patients]. The recent definition of minimally conscious state (MCS), and possibly research advances (e.g., functional neuroimaging), may have lead to uncertainty regarding...

The vegetative state is a devastating condition where patients awaken from their coma (i.e., open their eyes) but fail to show any behavioural sign of conscious awareness. Locked-in syndrome patients also awaken from their coma and are unable to show any motor response to command (except for small eye movements or blinks) but recover full conscious...

We discuss the case of two patients who developped delayed malignant peripheral nerve sheath tumor (MPNST) following radiotherapy.

The presence of neurogenic precursors in the adult mammalian brain is now widely accepted, but the mechanisms coupling their proliferation with the onset of neuronal differentiation remain unknown. Here, we unravel the major contribution of the G(1) regulator cyclin-dependent kinase 6 (Cdk6) to adult neurogenesis. We found that Cdk6 was essential f...

Evidence from functional neuroimaging studies on resting state suggests that there are two distinct anticorrelated cortical systems that mediate conscious awareness: an “extrinsic” system that encompasses lateral fronto-parietal areas and has been linked with processes of external input (external awareness), and an “intrinsic” system which encompas...

Ambulation impairment is a major component of physical disability in multiple sclerosis (MS) and a major target of rehabilitation programs. Outcome measures commonly used to evaluate walking capacities suffer from several limitations. To define and validate a new test that would overcome the limitations of current gait evaluations in MS and ultimat...

Natalizumab (Tysabri) is a monoclonal antibody that was recently approved for the treatment of relapsing-remitting multiple sclerosis (RRMS). Our primary objective was to analyse the efficacy of natalizumab on disability status and ambulation after switching patients with RRMS from other disease-modifying treatments (DMTs). A retrospective, observa...

We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) in a 76-year-old male presenting with confusion, dysarthria, diplopia, lumbal pain and headaches of recent onset. Neurological examination revealed nuchal rigidity and bilateral sixth cranial nerve palsy. The cerebrospinal fluid showed a marked hyperproteinorachia (4711 mg/L) and...

Human pluripotent stem cells, including embryonic (hES) and induced pluripotent stem cells (hiPS), retain the ability to self-renew indefinitely, while maintaining the capacity to differentiate into all cell types of the nervous system. While human pluripotent cell-based therapies are unlikely to arise soon, these cells can currently be used as an...

Assessment of visual fixation is commonly used in the clinical examination of patients with disorders of consciousness. However, different international guidelines seem to disagree whether fixation is compatible with the diagnosis of the vegetative state (i.e., represents "automatic" subcortical processing) or is a sufficient sign of consciousness...

The 'default network' is defined as a set of areas, encompassing posterior-cingulate/precuneus, anterior cingulate/mesiofrontal cortex and temporo-parietal junctions, that show more activity at rest than during attention-demanding tasks. Recent studies have shown that it is possible to reliably identify this network in the absence of any task, by r...

Eye movements abnormalities are common symptoms in neurology. We report a clinical observation of ischemic unilateral internuclear ophtalmoplegia to illustrate how much anatomical diagnosis is based on 1) a detailed neurological examination and 2) a deep knowledge and understanding of the anatomy and physiology of ocular movements. We also take thi...

Pain management in severely brain-damaged patients constitutes a clinical and ethical stake. At the bedside, assessing the presence of pain and suffering is challenging due to both patients' physical condition and inherent limitations of clinical assessment. Neuroimaging studies support the existence of distinct cerebral responses to noxious stimul...

The locked-in syndrome is a rare neurologic disorder defined by (1) the presence of sustained eye opening; (2) preserved awareness; (3) aphonia or hypophonia; (4) quadriplegia or quadriparesis; and (5) a primary mode of communication that uses vertical or lateral eye movement or blinking. Five cases are reported here, and previous literature is rev...

Assessing behavioral responses to nociception is difficult in severely brain-injured patients recovering from coma. We here propose a new scale developed for assessing nociception in vegetative (VS) and minimally conscious (MCS) coma survivors, the Nociception Coma Scale (NCS), and explore its concurrent validity, inter-rater agreement and sensitiv...

Following coma, some patients will recover wakefulness without signs of consciousness (only showing reflex movements, i.e., the vegetative state) or may show non-reflex movements but remain without functional communication (i.e., the minimally conscious state). Currently, there remains a high rate of misdiagnosis of the vegetative state (Schnakers...

Recent studies on spontaneous fluctuations in the functional MRI blood oxygen level-dependent (BOLD) signal in awake healthy subjects showed the presence of coherent fluctuations among functionally defined neuroanatomical networks. However, the functional significance of these spontaneous BOLD fluctuations remains poorly understood. By means of 3 T...

Case reports are the most accessible publications to the medical student or the practitioner. The appropriate selection of the clinical case that deserves publication is an important first step in the process. Afterwards, the report of the case requests much attention, as far as both content and presentation are concerned. The key-message emerging...

From a behavioral as well as neurobiological point of view, sleep and consciousness are intimately connected. A better understanding of sleep cycles and sleep architecture of patients suffering from disorders of consciousness (DOC) might therefore improve the clinical care for these patients as well as our understanding of the neural correlations o...

This study describes the neuropsychological assessment of 34 patients with questionable Alzheimer's disease (QAD) followed up for 3 years. Several measures were selected from the California Verbal Learning Test (CVLT) and compared to other cognitive tasks to assess the best neuropsychological indices for (a) detecting early memory impairment in QAD...

The neural mechanisms underlying the antinociceptive effects of hypnosis still remain unclear. Using a parametric single-trial thulium-YAG laser fMRI paradigm, we assessed changes in brain activation and connectivity related to the hypnotic state as compared to normal wakefulness in 13 healthy volunteers. Behaviorally, a difference in subjective ra...

Guillain-Barré syndrome (GBS) is often triggered by a preceding bacterial or viral infection. Occasionally, it has been observed in association with acute hepatitis A, B and C, and three cases have been previously described in India in which GBS was associated with acute hepatitis E. A molecular mimicry mechanism is supposed to be involved in the p...

We present a discussion on the treatment options in the case of a patient seen at the outpatient abnormal movement clinic for a resting tremor of both hands. Signs and symptoms of the parkinsonian syndrome are summarized as well as the current treatment options of early Parkinson's disease.

Since their inception in the 1970's, monoclonal antibody therapies became increasingly efficient and common in numerous medical conditions and their use in neurology has been boosted during the last couple of years with the rise of natalizumab (Tysabri). Furthermore, if most monoclonal antibodies currently assessed in neurologic conditions remain c...

Newborn granule neurons are generated from proliferating neural stem/progenitor cells and integrated into mature synaptic networks in the adult dentate gyrus of the hippocampus. Since light/dark variations of the mitotic index and DNA synthesis occur in many tissues, we wanted to unravel the role of the clock-controlled Period2 gene (mPer2) in timi...

The relationship between the Bispectral Index (BIS), an EEG-based monitor of anesthesia, and brain activity is still unclear. This study aimed at investigating the relationship between changes in BIS values during natural sleep and regional cerebral blood flow (rCBF) variations, as measured by Positron Emission Tomography (PET). Data were obtained...

mPER2 is expressed in GABAergic neurons but not in astroglial and oligodendroglial cells in the adult dentate gyrus. White arrow point towards a neural stem/progenitor cell located in the SGL of the DG expressing mPER2 (A) and Sox2 (B). (C) This cell is negative for the astroglial marker S100β (C). Astroglial cell co-expressing S100β and Sox2 (whit...

No significant difference between the volume of the dentate gyrus in WT and Per2Brdm1 mice. (A-B) Confocal images of coronal sections of the DG counterstained with NeuN in order to study the absence of morpho-volumetric differences between WT and mPer2Brdm1 mutant mice at the same level of the rostro-caudal axis. (C) We have compared similar hippoc...

Expression of mPER2 protein in adult dentate gyrus sections using different antibodies. (A-B) Panels representing confocal images of DG sections simultaneously processed for immunostaining with ADi Per2 antibody (A) or Santacruz Per2 antibody (B) at ZT12. The two antibodies recognize identically mPER2. (C) Western blot analysis were carried out on...

Total locked-in syndrome is characterized by tetraplegia, anarthria and paralysis of eye motility. In this study, consciousness was detected in a 21-year-old woman who presented a total locked-in syndrome after a basilar artery thrombosis (49 days post-injury) using an active event-related paradigm. The patient was presented sequences of names cont...

For a long time it was believed that the adult mammalian brain was completely unable to regenerate after insults. However, recent advances in the field of stem cell biology, including the identification of adult neural stem cells (NSCs) and evidence regarding a continuous production of neurons throughout life in the dentate gyrus (DG) and the subve...

The generation of cortical projection neurons relies on the coordination of radial migration with branching. Here, we report that the multisubunit histone acetyltransferase Elongator complex, which contributes to transcript elongation, also regulates the maturation of projection neurons. Indeed, silencing of its scaffold (Elp1) or catalytic subunit...