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Orphanet Journal of Rare Diseases May 2007Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or... (Review)
Review
Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. PNS are rare, affecting less than 1/10,000 patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small cell lung cancer. PNS can affect any part of the central and peripheral nervous system, the neuromuscular junction, and muscle. They can be isolated or occur in association. In most patients, the neurological disorder develops before the cancer becomes clinically overt and the patient is referred to the neurologist who has the charge of identifying a neurological disorder as paraneoplastic. PNS are usually severely disabling. The most common PNS are Lambert-Eaton myasthenic syndrome (LEMS), subacute cerebellar ataxia, limbic encephalitis (LE), opsoclonus-myoclonus (OM), retinopathies (cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), Stiff-Person syndrome (SPS), chronic gastrointestinal pseudoobstruction (CGP), sensory neuronopathy (SSN), encephalomyelitis (EM) and dermatomyositis. PNS are caused by autoimmune processes triggered by the cancer and directed against antigens common to both the cancer and the nervous system, designated as onconeural antigens. Due to their high specificity (> 90%), the best way to diagnose a neurological disorder as paraneoplastic is to identify one of the well-characterized anti-onconeural protein antibodies in the patient's serum. In addition, as these antibodies are associated with a restricted range of cancers, they can guide the search for the underlying tumor at a stage when it is frequently not clinically overt. This is a critical point as, to date, the best way to stabilize PNS is to treat the cancer as soon as possible. Unfortunately, about one-third of patients do not have detectable antibodies and 5% to 10% have an atypical antibody that is not well-characterized. As PNS are believed to be immune-mediated, suppression of the immune response represents another treatment approach.
Topics: Cerebellar Ataxia; Dermatomyositis; Encephalomyelitis; Humans; Intestinal Pseudo-Obstruction; Lambert-Eaton Myasthenic Syndrome; Limbic Encephalitis; Opsoclonus-Myoclonus Syndrome; Paraneoplastic Polyneuropathy; Retinal Diseases; Sensation Disorders; Stiff-Person Syndrome; Syndrome
PubMed: 17480225
DOI: 10.1186/1750-1172-2-22 -
Current Opinion in Neurobiology Aug 2015The cerebellum is connected to cerebral areas that subserve a range of sensory and motor functions. In this review, we summarize new literature demonstrating deficits in... (Review)
Review
The cerebellum is connected to cerebral areas that subserve a range of sensory and motor functions. In this review, we summarize new literature demonstrating deficits in visual perception, proprioception, motor control, and motor learning performance following cerebellar damage. In particular, we highlight novel results that together suggest a general role of the cerebellum in estimating and predicting movement dynamics of the body and environmental stimuli. These findings agree with the hypothesized role of the cerebellum in the generation and calibration of predictive models for a variety of functions.
Topics: Animals; Cerebellar Diseases; Cerebellum; Humans; Movement Disorders; Perception; Sensation Disorders
PubMed: 25863011
DOI: 10.1016/j.conb.2015.03.013 -
Neurobiology of Disease May 2011The pathophysiology of dystonia has been best studied in patients with focal hand dystonia. A loss of inhibitory function has been demonstrated at spinal, brainstem and... (Review)
Review
The pathophysiology of dystonia has been best studied in patients with focal hand dystonia. A loss of inhibitory function has been demonstrated at spinal, brainstem and cortical levels. Many cortical circuits seem to be involved. One consequence of the loss of inhibition is a failure of surround inhibition, and this appears to directly lead to overflow and unwanted muscle spasms. There are mild sensory abnormalities and deficits in sensorimotor integration; these also might be explained by a loss of inhibition. Increasing inhibition may be therapeutic. A possible hypothesis is that there is a genetic loss of inhibitory interneurons in dystonia and that this deficit is a substrate on which other factors can act to produce dystonia. This article is part of a Special Issue entitled "Advances in dystonia".
Topics: Animals; Dystonic Disorders; Humans; Motor Cortex; Neural Inhibition; Sensation Disorders; Transcranial Magnetic Stimulation
PubMed: 20817092
DOI: 10.1016/j.nbd.2010.08.025 -
Neuropsychology Review Mar 2016The aim of this systematic review was to integrate and assess evidence for the effectiveness of multisensory stimulation (i.e., stimulating at least two of the following... (Review)
Review
The aim of this systematic review was to integrate and assess evidence for the effectiveness of multisensory stimulation (i.e., stimulating at least two of the following sensory systems: visual, auditory, and somatosensory) as a possible rehabilitation method after stroke. Evidence was considered with a focus on low-level, perceptual (visual, auditory and somatosensory deficits), as well as higher-level, cognitive, sensory deficits. We referred to the electronic databases Scopus and PubMed to search for articles that were published before May 2015. Studies were included which evaluated the effects of multisensory stimulation on patients with low- or higher-level sensory deficits caused by stroke. Twenty-one studies were included in this review and the quality of these studies was assessed (based on eight elements: randomization, inclusion of control patient group, blinding of participants, blinding of researchers, follow-up, group size, reporting effect sizes, and reporting time post-stroke). Twenty of the twenty-one included studies demonstrate beneficial effects on low- and/or higher-level sensory deficits after stroke. Notwithstanding these beneficial effects, the quality of the studies is insufficient for valid conclusion that multisensory stimulation can be successfully applied as an effective intervention. A valuable and necessary next step would be to set up well-designed randomized controlled trials to examine the effectiveness of multisensory stimulation as an intervention for low- and/or higher-level sensory deficits after stroke. Finally, we consider the potential mechanisms of multisensory stimulation for rehabilitation to guide this future research.
Topics: Acoustic Stimulation; Brain; Cognition Disorders; Humans; Perceptual Disorders; Photic Stimulation; Physical Stimulation; Sensation Disorders; Stroke Rehabilitation; Treatment Outcome
PubMed: 26490254
DOI: 10.1007/s11065-015-9301-1 -
Seminars in Neurology Nov 2009Mal de debarquement (MdD), the "sickness of disembarkment," occurs when habituation to background rhythmic movement becomes resistant to readaption to stable conditions... (Review)
Review
Mal de debarquement (MdD), the "sickness of disembarkment," occurs when habituation to background rhythmic movement becomes resistant to readaption to stable conditions and results in a phantom perception of self motion typically described as rocking, bobbing, or swaying. Although several studies have shown that brief periods of MdD are common in healthy individuals, this otherwise natural phenomenon can become persistent in some individuals and lead to severe balance problems. Increased recognition of MdD in a persistent pathological form occurred after the publication of a case series of six patients by Brown and Baloh in 1987. Over 20 years later, although more is known about the clinical syndrome of persistent MdD, little is known about what leads to this persistence. This review addresses the clinical features of MdD, the associated symptoms in the persistent form, theories on pathogenesis, experience with treatment, and future directions for research.
Topics: Humans; Motion Sickness; Sensation Disorders; Syndrome
PubMed: 19834863
DOI: 10.1055/s-0029-1241038 -
Journal of Autism and Developmental... Jan 2006Research studies on sensory issues in autism, including those based on questionnaires, autobiographical accounts, retrospective video observations and early experimental... (Review)
Review
Research studies on sensory issues in autism, including those based on questionnaires, autobiographical accounts, retrospective video observations and early experimental approaches are reviewed in terms of their strengths and limitations. We present a cognitive neuroscience theoretical perspective on multisensory integration and propose that this may be a useful way of conceptualizing and studying sensory integration and the perceptual experience of persons with autism. Our goal is to operationalize the concept of sensory integration, a notion that is frequently alluded to in the field of autism yet rarely defined in empirical terms. We conclude with a discussion of how this re-conceptualization and study of sensory integration may generate testable hypotheses and lead to refinements in current perceptual theories of autism.
Topics: Autistic Disorder; Humans; Perceptual Disorders; Sensation Disorders
PubMed: 16395537
DOI: 10.1007/s10803-005-0044-3 -
Physical & Occupational Therapy in... 2019: Sensory processing impairments are well characterized in children with neurodevelopmental disorders, particularly autism, and have been associated with maladaptive...
: Sensory processing impairments are well characterized in children with neurodevelopmental disorders, particularly autism, and have been associated with maladaptive behaviors. However, little is known regarding sensory processing difficulties within Down syndrome, or how these difficulties may influence maladaptive behavior. This study aims to characterize sensory processing difficulties within the Down syndrome phenotype and determine the influence of processing difficulties on maladaptive behavior. : To explore this issue, we administered the Short Sensory Profile and the Developmental Behavior Checklist to parents or primary caregivers of young children with DS ( = 49; M nonverbal mental age (NVMA) = 30.92 months (SD = 12.30); M chronological age (CA) = 67.04 (SD = 25.13). : Results indicated that Low Energy/Weak, Under-responsive/Seeks Sensation, and Auditory Filtering were the areas of greatest sensory regulation difficulty, and that Self-Absorbed behavior and Disruptive/Antisocial behavior were elevated areas of maladaptive behavior. Multivariate regression analyses indicated that Under-responsive/Seeks Sensation was the only sensory regulation domain significantly associated with Self-Absorbed and Disruptive/Antisocial behavior. : Findings indicate a consistent pattern of sensory processing impairments and associations with maladaptive behavior in children with DS. Implications for interventions are discussed.
Topics: Child; Child, Preschool; Developmental Disabilities; Down Syndrome; Female; Humans; Male; Phenotype; Problem Behavior; Sensation Disorders; Surveys and Questionnaires
PubMed: 31070074
DOI: 10.1080/01942638.2019.1575320 -
Comprehensive Psychiatry Jan 2018Preliminary studies in children and adults with Attention Deficit Hyperactivity Disorder (ADHD) report both hypo-responsiveness and hyper-responsiveness to sensory...
Preliminary studies in children and adults with Attention Deficit Hyperactivity Disorder (ADHD) report both hypo-responsiveness and hyper-responsiveness to sensory stimuli, as well as problems modulating sensory input. As it has been suggested that those with ADHD exist at the extreme end of a continuum of ADHD traits, which are also evident in the general population, we investigated the link between ADHD and sensory sensitivity in the general population. Two online questionnaires measuring ADHD traits and sensory responsivity across various sensory domains were administered to 234 participants. Results showed a highly significant positive correlation between the number of ADHD traits and the frequency of reported sensory processing problems. An increased number of sensory difficulties across all modalities were associated with the level of ADHD. Furthermore, ADHD traits predicted sensory difficulties and exploratory factor analysis revealed a factor that combined ADHD trait and sensory processing items. This is the first study to identify a positive relationship between sensory processing and ADHD traits in the general population. Our results suggest that sensory difficulties could be part of the ADHD phenotype.
Topics: Adolescent; Adult; Aged; Attention Deficit Disorder with Hyperactivity; Female; Humans; Male; Middle Aged; Population Surveillance; Self Report; Sensation; Sensation Disorders; Surveys and Questionnaires; United Kingdom; Young Adult
PubMed: 29121555
DOI: 10.1016/j.comppsych.2017.10.008 -
European Neurology 2007Synaesthesia is the intriguing, involuntary experience of feeling one sensation in response to a different sensory stimulus. Recognised since described in 1890 by John... (Review)
Review
Synaesthesia is the intriguing, involuntary experience of feeling one sensation in response to a different sensory stimulus. Recognised since described in 1890 by John Locke and clarified by Galton in the 1880s, it has been analysed in the last 50 years. Grapheme-colour synaesthesia is the commonest form, but many other sensory linkages are reported. Experiments show that it is a genuine immediate perception, not merely a memory or learned association. Many of the mechanisms posited are based on indirect methods, and we know little of the neurophysiological mechanisms.
Topics: Brain; History, 15th Century; History, 19th Century; Humans; Perception; Sensation Disorders
PubMed: 17179720
DOI: 10.1159/000098101 -
Journal of Neurology Aug 2009Parkinson's disease is a hypokinetic movement disorder with cardinal motor features of bradykinesia, resting tremor and rigidity. However, non-motor symptoms, such as... (Review)
Review
Parkinson's disease is a hypokinetic movement disorder with cardinal motor features of bradykinesia, resting tremor and rigidity. However, non-motor symptoms, such as cognitive, neuropsychiatric, sleep, autonomic and sensory disturbances are gaining increasing attention. These non-motor symptoms may be intrinsic to the disease pathology or may be results of treatment with dopaminergic agents. Given that most, if not all, patients with Parkinson's disease will experience non-motor symptoms, it is important to be sensitive to these phenomena, especially since some non-motor signs may precede motor impairment. Treatment may include interventions independent of traditional, dopaminergic anti-Parkinson therapy or may be tailored to increase or reduce dopamine responsiveness of the symptom.
Topics: Antiparkinson Agents; Autonomic Nervous System Diseases; Brain; Cognition Disorders; Dopamine; Humans; Neurocognitive Disorders; Parkinson Disease; Sensation Disorders; Sleep Wake Disorders
PubMed: 19711119
DOI: 10.1007/s00415-009-5240-1