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Therapeutic Advances in Neurological... 2023The locked-in syndrome (LiS) is characterized by quadriplegia with preserved vertical eye and eyelid movements and retained cognitive abilities. Subcategorization,... (Review)
Review
The locked-in syndrome (LiS) is characterized by quadriplegia with preserved vertical eye and eyelid movements and retained cognitive abilities. Subcategorization, aetiologies and the anatomical foundation of LiS are discussed. The damage of different structures in the pons, mesencephalon and thalamus are attributed to symptoms of classical, complete and incomplete LiS and the locked-in plus syndrome, which is characterized by additional impairments of consciousness, making the clinical distinction to other chronic disorders of consciousness at times difficult. Other differential diagnoses are cognitive motor dissociation (CMD) and akinetic mutism. Treatment options are reviewed and an early, interdisciplinary and aggressive approach, including the provision of psychological support and coping strategies is favoured. The establishment of communication is a main goal of rehabilitation. Finally, the quality of life of LiS patients and ethical implications are considered. While patients with LiS report a high quality of life and well-being, medical professionals and caregivers have largely pessimistic perceptions. The negative view on life with LiS must be overthought and the autonomy and dignity of LiS patients prioritized. Knowledge has to be disseminated, diagnostics accelerated and technical support system development promoted. More well-designed research but also more awareness of the needs of LiS patients and their perception as individual persons is needed to enable a life with LiS that is worth living.
PubMed: 37006459
DOI: 10.1177/17562864231160873 -
The Lancet. Psychiatry Jul 2019Catatonia is a psychomotor disorder featuring stupor, posturing, and echophenomena. This Series paper examines the evidence for immune dysregulation in catatonia.... (Review)
Review
Catatonia is a psychomotor disorder featuring stupor, posturing, and echophenomena. This Series paper examines the evidence for immune dysregulation in catatonia. Activation of the innate immune system is associated with mutism, withdrawal, and psychomotor retardation, which constitute the neurovegetative features of catatonia. Evidence is sparse and conflicting for acute-phase activation in catatonia, and whether this feature is secondary to immobility is unclear. Various viral, bacterial, and parasitic infections have been associated with catatonia, but it is primarily linked to CNS infections. The most common cause of autoimmune catatonia is N-methyl-D-aspartate receptor (NMDAR) encephalitis, which can account for the full spectrum of catatonic features. Autoimmunity appears to cause catatonia less by systemic inflammation than by the downstream effects of specific actions on extracellular antigens. The specific association with NMDAR encephalitis supports a hypothesis of glutamatergic hypofunction in catatonia.
Topics: Catatonia; Humans; Immune System; Immune System Diseases
PubMed: 31196793
DOI: 10.1016/S2215-0366(19)30190-7 -
Advances in Experimental Medicine and... 2020Under the partial influences of paradigm shift form category to dimension, the Diagnostic and Statistical Manual of Mental Disorder (DSM) was revised to the fifth... (Review)
Review
Under the partial influences of paradigm shift form category to dimension, the Diagnostic and Statistical Manual of Mental Disorder (DSM) was revised to the fifth edition (DSM-5); however, due to the lack of consistent biological makers and processes and the restricted availability of dimensional meta-structure, the revisions for the DSM-5 were based on a combination of categorical and dimensional approaches. Anxiety disorders were more clearly and consistently defined in the DSM-5 with the removal of obsessive compulsive, acute stress, and post-traumatic stress disorders. Differences between the childhood and adulthood categories of anxiety disorders were decreased, and overall, the symmetrical classification of anxiety subtypes was increased, since separation anxiety disorder and selective mutism were considered anxiety disorders, not neurodevelopmental disorders. Additionally, based on growing evidence, agoraphobia is distinct from panic disorder. Next, considering cultural syndromes including taijin kyofusho, khyal cap, trung gio attacks, and ataque de nervios, cultural influences are considered a significant factor for definitions and presentations of anxiety disorders. Controversies in the DSM-5 criteria for anxiety disorders are lowering the diagnostic thresholds of anxiety disorders and limiting the dichotomous view of anxiety and depression when defining generalized anxiety disorder. Further studies of alternative approaches to the restrictions of the DSM-5 criteria of anxiety disorders, including transdiagnostic specifiers and dimensional assessment tools, may be required.
Topics: Age Factors; Anxiety; Anxiety Disorders; Depression; Diagnostic and Statistical Manual of Mental Disorders; Humans; Stress Disorders, Post-Traumatic
PubMed: 32002930
DOI: 10.1007/978-981-32-9705-0_12 -
Neuroscience and Biobehavioral Reviews May 2020Akinetic mutism (AM) is a rare neurological disorder characterized by the presence of an intact level of consciousness and sensorimotor capacity, but with a simultaneous... (Review)
Review
Akinetic mutism (AM) is a rare neurological disorder characterized by the presence of an intact level of consciousness and sensorimotor capacity, but with a simultaneous decrease in goal-directed behavior and emotions. Patients are in a wakeful state of profound apathy, seemingly indifferent to pain, thirst, or hunger. It represents the far end within the spectrum of disorders of diminished motivation. In recent years, more has become known about the functional roles of neurocircuits and neurotransmitters associated with human motivational behavior. More specific, there is an increasing body of behavioral evidence that links specific damage of functional frontal-subcortical organization to the occurrence of distinct neurological deficits. In this review, we combine evidence from lesion studies and neurophysiological evidence in animals, imaging studies in humans, and clinical investigations in patients with AM to form an integrative theory of its pathophysiology. Moreover, the specific pharmacological interventions that have been used to treat AM and their rationales are reviewed, providing a comprehensive overview for use in clinical practice.
Topics: Adrenergic Uptake Inhibitors; Akinetic Mutism; Animals; Dopamine Agonists; Dopamine Uptake Inhibitors; Dopaminergic Neurons; GABA-A Receptor Agonists; Gray Matter; Humans; Motivation; Zolpidem
PubMed: 32044373
DOI: 10.1016/j.neubiorev.2020.02.006 -
Indian Pediatrics Jul 2020Autoimmune encephalitis has acquired immense significance as a treatable cause of encephalopathy, epilepsy and movement disorders in children. In this review, we discuss... (Review)
Review
CONTEXT
Autoimmune encephalitis has acquired immense significance as a treatable cause of encephalopathy, epilepsy and movement disorders in children. In this review, we discuss the various clinical syndromes, diagnosis, treatment and prognosis in children.
EVIDENCE ACQUISITION
A MEDLINE search strategy using the following terms (1998-2019) was adopted for this review. Limits of 'Human' and 'English' were applied. Search terms included: "autoimmune encephalitis", "autoimmune encephalitis AND epidemiology", "pathophysiology", "diagnosis" and "treatment" for studies in children. Review articles, practice parameters, guidelines, systematic reviews, meta-analyses, randomized controlled trials, cohort studies, case series and case reports were included.
CONCLUSIONS
Autoimmune encephalitis is being increasingly recognized in children. Anti-NMDAR encephalitis is the most common form. Children present with a polysymptomatic presentation including behavioral changes, psychosis, sleep disturbances, mutism, seizures, movement disorders, memory impairment as well as other neurocognitive deficits. Diagnosis is based on suggestive history and ancillary investigations including magnetic resonance imaging, cerebrospinal fluid analysis, and serology for autoantibodies. Treatment is based on immunomodulation of the acute episode followed by maintenance therapy, with earlier initiation being associated with better outcomes. Prognosis depends on the type of clinical syndrome.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Child; Encephalitis; Epilepsy; Hashimoto Disease; Humans; Movement Disorders; Seizures
PubMed: 32727942
DOI: No ID Found -
F1000Research 2020Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae globally. Causes are diverse and include viral and non-viral infections... (Review)
Review
Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae globally. Causes are diverse and include viral and non-viral infections of the brain as well as autoimmune processes. In the West, the autoimmune encephalitides are now more common than any single infectious cause, but, in Asia, infectious causes are still more common. In 2006, the World Health Organization coined the term "acute encephalitis syndrome", which simply means acute onset of fever with convulsions or altered consciousness or both. In 2013, the International Encephalitis Consortium set criteria for diagnosis of encephalitis on basis of clinical and laboratory features. The most important infectious cause in the West is herpes simplex virus, but globally Japanese encephalitis (JE) remains the single largest cause. Etiologic diagnosis is difficult because of the large number of agents that can cause encephalitis. Also, the responsible virus may be detectable only in the brain and is either absent or transiently found in blood or cerebrospinal fluid (CSF). Virological diagnosis is complex, expensive, and time-consuming. Different centres could make their own algorithms for investigation in accordance with the local etiologic scenarios. Magnetic resonance imaging (MRI) and electroencephalography are specific for few agents. Clinically, severity may vary widely. A severe case may manifest with fever, convulsions, coma, neurologic deficits, and death. Autoimmune encephalitis (AIE) includes two major categories: (i) classic paraneoplastic limbic encephalitis (LE) with autoantibodies against intracellular neuronal antigens (Eg: Hu and Ma2) and (ii) new-type AIE with autoantibodies to neuronal surface or synaptic antigens (Eg: anti-N-methyl-D-aspartate receptor). AIE has prominent psychiatric manifestations: psychosis, aggression, mutism, memory loss, euphoria, or fear. Seizures, cognitive decline, coma, and abnormal movements are common. Symptoms may fluctuate rapidly. Treatment is largely supportive. Specific treatment is available for herpesvirus group and non-viral infections. Various forms of immunotherapy are used for AIE.
Topics: Acute Disease; Animals; Autoantibodies; Encephalitis; Hashimoto Disease; Humans; Receptors, N-Methyl-D-Aspartate
PubMed: 32047620
DOI: 10.12688/f1000research.20634.1 -
Clinical Child and Family Psychology... Jun 2021In current classification systems, selective mutism (SM) is included in the broad anxiety disorders category. Indeed, there is abundant evidence showing that anxiety,... (Review)
Review
In current classification systems, selective mutism (SM) is included in the broad anxiety disorders category. Indeed, there is abundant evidence showing that anxiety, and social anxiety in particular, is a prominent feature of SM. In this article, we point out that autism spectrum problems in addition to anxiety problems are sometimes also implicated in SM. To build our case, we summarize evidence showing that SM, social anxiety disorder (SAD), and autism spectrum disorder (ASD) are allied clinical conditions and share communalities in the realm of social difficulties. Following this, we address the role of a prototypical class of ASD symptoms, restricted and repetitive behaviors and interests (RRBIs), which are hypothesized to play a special role in the preservation and exacerbation of social difficulties. We then substantiate our point that SM is sometimes more than an anxiety disorder by addressing its special link with ASD in more detail. Finally, we close by noting that the possible involvement of ASD in SM has a number of consequences for clinical practice with regard to its classification, assessment, and treatment of children with SM and highlight a number of directions for future research.
Topics: Anxiety; Anxiety Disorders; Autism Spectrum Disorder; Child; Child Behavior Disorders; Humans; Mutism; Phobia, Social
PubMed: 33462750
DOI: 10.1007/s10567-020-00342-0 -
Advances in Experimental Medicine and... 2020Anxiety disorders, including panic disorder/agoraphobia (PDA), generalized anxiety disorder (GAD), social anxiety disorder (SAD), and others, are the most prevalent... (Review)
Review
Anxiety disorders, including panic disorder/agoraphobia (PDA), generalized anxiety disorder (GAD), social anxiety disorder (SAD), and others, are the most prevalent mental disorders. In this paper, recommendations are given for the psychopharmacological treatment of these disorders which are based on comprehensive treatment guidelines, meta-analyses, and systematic reviews of available randomized controlled studies. Anxiety disorders can effectively be treated with psychotherapy, pharmacotherapy, or a combination of both. First-line drugs are the selective serotonin reuptake inhibitors (SSRIs) and serotonin-norepinephrine reuptake inhibitors (SNRIs). Benzodiazepines are not recommended for routine use due to their possible addiction potential. Other treatment options include the calcium modulator pregabalin, tricyclic antidepressants, buspirone, moclobemide, and others. Drug treatment can be combined with psychological treatments. Novel treatment strategies include medications that act on GABA, glutamate, and other neurotransmitter systems. After remission, medications should be continued for 6 to 12 months.
Topics: Anti-Anxiety Agents; Anxiety Disorders; Humans; Psychotherapy; Selective Serotonin Reuptake Inhibitors; Serotonin and Noradrenaline Reuptake Inhibitors
PubMed: 32002937
DOI: 10.1007/978-981-32-9705-0_19 -
Pediatrics Jun 2020Down syndrome disintegrative disorder (DSDD), a developmental regression in children with Down syndrome (DS), is a clinical entity that is characterized by a loss of... (Review)
Review
Down syndrome disintegrative disorder (DSDD), a developmental regression in children with Down syndrome (DS), is a clinical entity that is characterized by a loss of previously acquired adaptive, cognitive, and social functioning in persons with DS usually in adolescence to early adulthood. Initially reported in 1946 as "catatonic psychosis," there has been an increasing interest among the DS community, primary care, and subspecialty providers in this clinical area over the past decade. This condition has a subacute onset and can include symptoms of mood lability, decreased participation in activities of daily living, new-onset insomnia, social withdrawal, autistic-like regression, mutism, and catatonia. The acute phase is followed by a chronic phase in which baseline functioning may not return. No strict criteria or definitive testing is currently available to diagnose DSDD, although a comprehensive psychosocial and medical evaluation is warranted for individuals presenting with such symptoms. The etiology of DSDD is unknown, but in several hypotheses for regression in this population, psychological stress, primary psychiatric disease, and autoimmunity are proposed as potential causes of DSDD. Both psychiatric therapy and immunotherapies have been described as DSDD treatments, with both revealing potential benefit in limited cohorts. In this article, we review the current data regarding clinical phenotypes, differential diagnosis, neurodiagnostic workup, and potential therapeutic options for this unique, most disturbing, and infrequently reported disorder.
Topics: Activities of Daily Living; Adolescent; Autistic Disorder; Catatonia; Child; Developmental Disabilities; Down Syndrome; Female; Humans; Male; Mood Disorders; Psychotic Disorders; Review Literature as Topic
PubMed: 32471843
DOI: 10.1542/peds.2019-2939 -
The World Journal of Biological... Feb 2023This is the third version of the guideline of the World Federation of Societies of Biological Psychiatry (WFSBP) Task Force for the Pharmacological Treatment of Anxiety,...
World Federation of Societies of Biological Psychiatry (WFSBP) guidelines for treatment of anxiety, obsessive-compulsive and posttraumatic stress disorders - Version 3. Part I: Anxiety disorders.
AIM
This is the third version of the guideline of the World Federation of Societies of Biological Psychiatry (WFSBP) Task Force for the Pharmacological Treatment of Anxiety, Obsessive-Compulsive and Posttraumatic Stress Disorders (published in 2002, revised in 2008).
METHOD
A consensus panel of 33 international experts representing 22 countries developed recommendations based on efficacy and acceptability of available treatments. In total, 1007 RCTs for the treatment of these disorders in adults, adolescents, and children with medications, psychotherapy and other non-pharmacological interventions were evaluated, applying the same rigorous methods that are standard for the assessment of medications.
RESULT
This paper, Part I, contains recommendations for the treatment of panic disorder/agoraphobia (PDA), generalised anxiety disorder (GAD), social anxiety disorder (SAD), specific phobias, mixed anxiety disorders in children and adolescents, separation anxiety and selective mutism. Selective serotonin reuptake inhibitors (SSRI) and serotonin-norepinephrine reuptake inhibitors (SNRIs) are first-line medications. Cognitive behavioural therapy (CBT) is the first-line psychotherapy for anxiety disorders. The expert panel also made recommendations for patients not responding to standard treatments and recommendations against interventions with insufficient evidence.
CONCLUSION
It is the goal of this initiative to provide treatment guidance for these disorders that has validity throughout the world.
Topics: Adult; Adolescent; Child; Humans; Stress Disorders, Post-Traumatic; Biological Psychiatry; Anxiety Disorders; Selective Serotonin Reuptake Inhibitors; Anxiety; Obsessive-Compulsive Disorder
PubMed: 35900161
DOI: 10.1080/15622975.2022.2086295