[Sleep in chronic neuropediatric diseases]. / Sueño en enfermedades neuropediátricas crónicas.

The prevalence of sleep disorders (SD) is notoriously increased in children with chronic neurological disease, with a negative bidirectional link that aggravates their symptomatology and has a negative impact on the quality of life of the child and their families. Identifying and recognizing this association is key for the child neurologist since the treatment of SD significantly improves daytime symptomatology in neurodevelopmental disorders, epilepsy, primary headaches, cerebral palsy and neuromuscular diseases.

La prevalencia de los trastornos del sueño (TS) se incrementa notoriamente en niños con enfermedad neurológica crónica, con un vínculo bidireccional negativo que agrava su sintomatología y repercute negativamente en la calidad de vida del niño y su familia. Identificar y reconocer dicha asociación es clave para el neuropediatra, ya que el tratamiento del TS mejora significativamente la sintomatología diurna de los trastornos del neurodesarrollo, epilepsia, cefaleas primarias, parálisis cerebral y enfermedades neuromusculares.

Assessing Delirium in Patients With Neurological Diseases.

ABSTRACT: BACKGROUND: Delirium is a common complication during hospitalization. Its consequences are severe, including reduced function, delayed rehabilitation, dementia, institutionalization, and death. Assessing delirium in neurological patients can be challenging due to the impact of neurological deficits. Therefore, the aim was to investigate the agreement between 2 delirium screening tools, factors associated with delirium, and assessing delirium in neurological patients. METHODS: This prospective observational study was conducted in 2 neurological units, using daily delirium screening. Intensive Care Delirium Screening Checklist and 2 versions of the Confusion Assessment Method were used to asses delirium in adult patients without baseline dementia, alcohol/drug detoxification, or palliative care. Descriptive analyses determined the number of delirium scores, and the analytical analyses were logistic regressions and a κ coefficient. RESULTS: The agreement between the screening tools was found to be substantial (κ = 0.71). Logistic regression analysis showed that the risk factors for a positive delirium screening were home care before admission (Confusion Assessment Method: odds ratio [OR], 4.21 [95% confidence interval (CI), 1.67-10.63]; Intensive Care Delirium Screening Checklist: OR, 6.14 [95% CI, 2.85-13.23]) and aphasia/dysarthria (Confusion Assessment Method: OR, 4.9 [95% CI, 1.32-6.81]; Intensive Care Delirium Screening Checklist: OR, 2.76 [95% CI, 1.3-5.87]). In total, 18.7% (n = 20/107) of the screening scores were positive. Specifically, the Confusion Assessment Method showed positive scores for 13.0% (n = 14/107) of participants, whereas the Intensive Care Delirium Screening Checklist showed positive scores for 16.8% (n = 18/107). CONCLUSION: The screening tools had a substantial degree of agreement. Therefore, nurses can use both screening tools to detect delirium in patients with neurological disorders. However, care should be taken in patients with aphasia to avoid misclassification with the Brief-Confusion Assessment Method. Moreover, special attention should be directed toward patients with language difficulties such as aphasia/dysarthria and those who received home care services before admission. These areas warrant further investigation in clinical practice and future studies.

How Do Nurses Assess Cognition in Adults With Neurological Conditions? A Scoping Review.

BACKGROUND: According to epidemiological studies, neurological cognitive problems are increasingly prevalent in the aging population, with estimates that the number of people living with cognitive impairment will triple by 2050. Therefore, early detection in rehabilitation settings is needed to manage cognitive changes to ensure that individuals living with these conditions receive care and support that addresses their needs. PURPOSE: This scoping review, based on the Arksey and O'Malley method, aims to investigate the cognitive assessments used for patients with neurological conditions in current nursing practice. METHOD: PubMed, Ovid Medline, and CINAHL databases were searched to identify relevant articles published from 2017 to 2023 in English. Twenty-four articles met the inclusion criteria. Cognitive assessments were evaluated across acute care/hospital, outpatient/clinic, community, and long-term care/nursing home settings. RESULTS: The Mini-Mental State Examination is the most frequently used tool across all settings except for long-term care. Cognition includes many different domains such as executive functioning and speed of processing information; however, most tools only capture memory. The nursing profession must expand its standardized nursing vocabulary to capture cognition better. CONCLUSIONS: As rehabilitation nurses navigate diverse clinical environments, recognition of contextual nuances is important in selecting cognitive function measurement tools most suitable for their setting.

'Positive' inter-ictal clinical signs of functional neurological disorders are found in patients with functional dissociative seizures.

BACKGROUND AND PURPOSE: Prior studies highlighted the high diagnostic specificity (ranging from 92% to 100%) of clinical signs observed in functional neurological disorders (FNDs). However, these signs are rarely looked for by epileptologists when trying to distinguish between functional dissociative seizure (FDS) and epileptic seizure. The aim of this study was to determine the prevalence of inter-ictal clinical signs of FND in a cohort of patients with probable FDS. The secondary objective was to compare the prevalence of inter-ictal FND clinical signs in FDS patients with age- and gender-matched epileptic patients without FDS. METHODS: Patients diagnosed with FDS seen at two tertiary care centres and epileptic outpatients were included in the study. Each patient underwent a physical examination, searching for inter-ictal clinical signs of FND. RESULTS: In the FDS group, 79% of patients presented at least one sign of FND, compared to 16.6% of patients with epilepsy (p < 0.001). Moreover, 66.6% of FDS patients presented three or more FND signs, whereas only 4.1% of epileptic patients did (p < 0.001). The median number of FND clinical signs in the FDS group was four (SD 1.7; 5.5). Using the threshold of three signs or more, the specificity of detecting three or more FND signs was 83.3%, with a sensitivity of 79.2%. CONCLUSION: Inter-ictal clinical signs of FND are present in patients with FDS and should be looked for during neurological examination.

Neurologic comorbidity in psychiatric inpatients: evidence from neurologic consultations in a Spanish center. / Comorbilidad neurológica en pacientes psiquiátricos ingresados: evidencia sobre interconsultas de neurología en un centro español.

INTRODUCTION: The presence of psychiatric comorbidity in some neurological disorders is common. A bi-directional influence between some psychiatric and neurological disorders has been discussed, but not widely studied. There is an absence of literature on the typology and rates of neurology consultations in different types of psychiatric inpatients. MATERIALS AND METHODS: Cross-sectional study based on real world data on patients who had a neurological consultation during hospitalization on a psychiatric ward. RESULTS: The most frequent reasons for visits to neurologists in our study were cluster 'Epilepsy/other types of non-epileptic seizures' (n = 177, 36.44%), followed by cluster 'Movement disorders' (n = 77, 20.48%), 'Cognitive disorder' (n = 69, 18.35%), and finally cluster 'Neuropathy' (n = 21, 5.59%). The most frequent type of psychiatric patient who required neurologic consultation presented a psychotic disorder (n = 100, 26.60%), follow by problem behavior (n = 82, 21.81%), bipolar disorder (n = 78, 20.78%), depressive disorder (n = 42, 11.17%) and autism spectrum disorder (n = 20, 5.32%). We found a statistically significant relationship between (problem behavior and intellectual disability) and neurologic consultation for epilepsy/other types of non-epileptic seizures, and between (depressive disorder, bipolar disorder, autism spectrum disorder and intellectual disability) and neurologic consultation for movement disorders. CONCLUSIONS: This is the first study in the literature which analyzes the rates and typology of neurologic consultations in people hospitalized with psychiatric disorders. A deep knowledge of epilepsy, movement disorders and cognitive disorders should be required for health professionals to treat psychiatric inpatients appropriately. Patients with particular psychiatric disorders seem to require a higher number of neurologic consultations than others during their hospitalization.

TITLE: Comorbilidad neurológica en pacientes psiquiátricos ingresados: evidencia sobre interconsultas de neurología en un centro español.Introducción. La presencia de comorbilidad psiquiátrica en algunos trastornos neurológicos es frecuente. Se ha discutido sobre una influencia bidireccional entre algunos trastornos psiquiátricos y neurológicos, pero este hecho no se ha estudiado ampliamente. Existe escasa información en la bibliografía sobre la tipología y la prevalencia de las interconsultas de neurología en los diferentes tipos de pacientes psiquiátricos hospitalizados. Materiales y métodos. Estudio transversal basado en datos reales sobre pacientes que necesitaron una interconsulta de neurología durante su hospitalización en salud mental. Resultados. Los motivos más frecuentes que requirieron una interconsulta de neurología en nuestro estudio fueron el clúster 'Epilepsia/otros tipos de crisis no epilépticas' (n = 177; 36,44%), seguido del clúster 'Trastornos del movimiento' (n = 77; 20,48%), el clúster 'Trastorno cognitivo' (n = 69; 18,35%) y, por último, el clúster 'Neuropatía' (n = 21; 5,9%). El tipo más frecuente de paciente psiquiátrico que requirió consulta neurológica presentó un trastorno psicótico (n = 100; 26,6%), seguido de problemas de conducta (n = 82; 21,81%), trastorno bipolar (n = 78; 20,78%), trastorno depresivo (n = 42; 11,17%) y trastorno del espectro autista (n = 20; 5,32%). Se encontró una relación estadísticamente significativa entre problemas de conducta y discapacidad intelectual e interconsulta de neurología por epilepsia/otros tipos de crisis no epilépticas, y entre trastorno depresivo, trastorno bipolar, trastorno del espectro autista y discapacidad intelectual e interconsulta de neurología por trastornos del movimiento. Conclusiones. Éste es el primer estudio en la bibliografía que analiza la frecuencia y la tipología de las interconsultas de neurología en personas hospitalizadas con trastornos psiquiátricos. Debe requerirse a los profesionales que traten a pacientes de salud mental hospitalizados un conocimiento profundo en epilepsia, trastornos del movimiento y trastornos cognitivos. Algunos pacientes con determinados trastornos psiquiátricos parecen necesitar un mayor número de interconsultas de neurología que otros durante su hospitalización.

Long-term neurological and neurocognitive impairments after tick-borne encephalitis in Lithuania - a prospective study.

BACKGROUND: The aim of this study was to characterise long-term neurological and neurocognitive sequelae after tick-borne encephalitis (TBE) in adults. METHODS: 98 prospective consecutive TBE patients, classified by disease severity, were included. Immediate outcomes were evaluated with Glasgow Outcome Scale (GOS) and Rankin Scale (RS). After 6 and 18 months, long-term disability was evaluated using Modified Rankin Scale (MRS) and neurocognitive assessment was performed with Matrics Consensus Cognitive Battery (MCCB), measuring processing speed, attention/vigilance, working memory, verbal learning, visual learning, reasoning/problem solving and social cognition. The MCCB results were compared to healthy age, gender and education-matched controls. RESULTS: Mild, moderate, and severe TBE was diagnosed in 53.1%, 38.8%, and 8.2% of cases, respectively. At discharge, 25.5% of the patients had major or moderate impairments (GOS) and various levels of disability in 34.7% (RS). Up to 18 months from the onset of TBE, over 20% remained with slight to moderate disability (MRS). GOS, RS and MRS scores correlated with disease severity. At 6 months after the onset, TBE patients scored significantly lower than controls in processing speed, verbal, and visual learning. Two latter domains were significantly more impaired in patients with mild TBE. Patients aged 18-39 performed significantly worse in attention/vigilance and working memory, whereas aged 60+ in verbal learning. A year later, significant improvement was observed in six of seven cognitive domains. CONCLUSIONS: Long-term neurological sequelae persist in a substantial proportion of TBE patients with significant impairment in several cognitive domains, especially in younger patients and even after mild TBE.

A systematic review of the perspectives of botulinum toxin use on the quality of life of neurological patients with drooling.

OBJECTIVE: To investigate the effectiveness of botulinum toxin in the salivary glands of patients with neurological impairment and drooling and its impact on the quality of life. MATERIALS AND METHODS: This systematic review was registered with the International Prospective Register of Systematic Reviews (CRD 42,023,435,242) and conducted using the Preferred Reporting Items for Systematic Reviews and Meta-analyses. An electronic search was performed in the PubMed/MEDLINE, Embase, Scopus, Cochrane Library, and clinical trial databases until August 2023, no language restriction. Cohort studies and randomized clinical trials of patients diagnosed with drooling and neurological impairment who used botulinum toxin on the salivary gland were included, which evaluated subjective quality of life parameters. The risk of bias was assessed using the Joanna Briggs Institute Critical Appraisal Checklist and Risk of Bias 2 tools. The certainty of the evidence was analyzed using the Grading of Recommendations Assessment, Development, and Evaluation approach. RESULTS: Eight studies involving 317 patients were included. All studies, through subjective parameters, suggested the effectiveness of botulinum toxin in reducing drooling, resulting in an improvement in the quality of life. Three studies demonstrated improvements in swallowing and four in cases of respiratory diseases. Two clinical trials had a high risk of bias, whereas one had low risk. The five cohort studies that were evaluated had a high risk of bias. The certainty of the evidence was considered low. CONCLUSIONS: Based on the patient/caregivers' perception of improvement in drooling, dysphagia, and respiratory symptoms, it can be inferred that botulinum toxin application reduces subjective drooling in neurologically compromised patients. Its impact contributes to the general well-being and quality of life. CLINICAL RELEVANCE: Injection of botulinum toxin into the salivary glands can be considered an alternative technique to surgical or medicinal approaches in reducing drooling. It is effective, less invasive and without significant side effects. It promotes a positive impact on the well-being and quality of life of neurological patients.

High Prevalence of Psychological Comorbidities and Functional Neurological Symptoms in Women With Urinary Retention.

PURPOSE: Chronic idiopathic urinary retention (CIUR) in young women is poorly understood and a probable etiology is established only in around 40%, most commonly a primary disorder of external urethral sphincter relaxation, sometimes referred to as Fowler's syndrome. A high prevalence of psychological and functional comorbidities is reported, however these have been poorly characterized. MATERIALS AND METHODS: Women consecutively referred for the assessment and management of CIUR were evaluated cross-sectionally for 13 psychological/behavioral domains using a structured clinical interview: depression, anxiety, post-traumatic stress disorder (PTSD), other psychiatric history, functional neurological disorder, other functional syndromes, childhood and adult trauma, personality disorder, and self-harm (ever/current). RESULTS: A total of 91 women (mean age [SD]: 34 [11] years) were evaluated. Women with Fowler's syndrome (n = 69) were younger (mean age [SD]: 32 [9] vs 40 [13] years) than women without Fowler's syndrome and reported shorter mean duration of urinary symptoms (mean [SD]: 5 [6] vs 10 [9]). A high prevalence of psychiatric and psychological comorbidities was reported (97%) including current depression (77%), current anxiety (78%), and PTSD (32%). A high prevalence of functional neurological disorder (56%) and other functional symptoms (65%) was also reported. Self-harm was reported in (14%) and personality disorder in 16%. Childhood trauma was reported in 35% of women. CONCLUSIONS: Young women with CIUR report a high burden of psychiatric disorders, affective symptoms, trauma, PTSD, self-harm, and functional neurological disorder, particularly in those with Fowler's syndrome. These factors can undermine the engagement with health care professionals and affect management and should therefore be addressed during the urological assessment.

Neurologic Dysphagia.

Dysphagia is commonly associated with neurologic/neuromuscular disorders including prematurity, cerebral palsy, traumatic brain injury, brain tumors, genetic disorders, and neuromuscular diseases. This article aims to review the major categories of neurologic dysphagia, to outline specific findings and special considerations for each population, and to acknowledge the importance of integrating each patient's medical prognosis, goals of care, and developmental stage into a multidisciplinary treatment plan.

Chronic idiopathic urinary retention: Comorbidity and outcome in 102 individuals.

OBJECTIVES: Chronic Idiopathic urinary retention is poorly understood. One small study suggests higher than expected rates of functional neurological disorder and pain comorbidity which may have implications for understanding the disorder. We investigated the frequency of functional neurological disorder, chronic pain other medical and psychiatric comorbidity, triggers of urinary retention, results of urodynamic assessment, medication history, management, and outcome in patients with chronic idiopathic urinary retention. METHODS: A consecutive retrospective electronic notes analysis was undertaken of patients with chronic idiopathic urinary retention presenting to a secondary care urology clinic between Jan 2018-Jan 2021, with follow-up to their most recent urological appointment. RESULTS: 102 patients were identified (mean age of 41.9 years, 98% female). 25% had functional neurological disorder (n = 26), most commonly limb weakness (n = 19, 19%) and functional seizures (n = 16, 16%). Chronic pain (n = 58, 57%) was a common comorbidity. Surgical and medical riggers to urinary retention were found in almost half of patients (n = 49, 48%). 81% of patients underwent urodynamic assessment (n = 83). Most frequently no specific abnormality was reported (n = 30, 29%). Hypertonic urethral sphincter was the most identified urodynamic abnormality (n = 17, 17%). We noted high levels of opioid (n = 50, 49%) and benzodiazepine (n = 27, 26%) use. Urinary retention resolved in only a small number of patients (n = 6, 6%, median follow up 54 months), in three cases spontaneously. CONCLUSION: This preliminary data suggests idiopathic urinary retention is commonly comorbid with functional neurological disorder, and chronic pain, suggesting shared mechanisms.

Painful Eyes in Neurology Clinic: A Guide for Neurologists.

Eye pain is a common complaint among patients presenting to the neurology clinic. It can be related to neurologic diseases, but it can also be a localized eye condition. Such disorders can be misleading, as their benign appearance might mask more grave underlying conditions, potentially leading to misdiagnoses or delayed treatment. Clinicians should be aware of the specific neurologic or systemic disorders (eg, demyelinating diseases or vascular abnormalities) that might first manifest as eye pain. Formal ophthalmic consultation is recommended for patients presenting with eye pain as the predominant complaint especially when red flags for more serious pathology are present.

Other Secondary Headaches: Odontogenic Pain and Other Painful Orofacial Conditions.

This article discusses extremely common odontogenic pain conditions, which may occasionally present to the neurology clinic mimicking headache, and other uncommon orofacial pain conditions, which may do the same. Typical presentations, investigative strategies, and management are discussed, as well as highlighting key diagnostic criteria and the importance of involving oral or dental specialists where diagnostic uncertainty exists.

Cognitive Impairment in Neurological Diseases.

The complex link between cognitive impairment and neurological disorders underscores the intricacies of neurological sciences [...].

Dual-Task Assessments for Predicting Future Falls in Neurologic Conditions: A Systematic Review.

ABSTRACT: This review investigated the ability of dual-task tests to predict falls in people with neurological disorders. Databases were searched to identify prospective cohort studies that analyzed dual-task testing and falls in people with neurological disorders. Reviewers screened studies for eligibility and extracted key information like participant characteristics, intervention details, outcome measures, and significant outcomes. Reviewers assessed methodological quality of eligible studies using the Standard Quality Assessment Criteria. Eighteen studies of strong methodological qualified with 1750 participants were included in the review. Dual-task performances were predictive of future falls in people with Huntington's disease, spinal cord injury, and moderate cognitive impairment, although only one independent study was included for each disability type. In people with stroke, 37% of eligible studies showed dual-task assessments to be predictive of future falls. No dual-task tests predicted prospective falling in people with Alzheimer's or Parkinson's disease. Complex dual tasks seemed to be more predictive of fall risk than simpler dual tasks. Results suggest that disability type, severity of disability, and task complexity play a role in the predictive ability of dual-task assessments and future falling in neurological disorders. Future studies may benefit from using this review to guide the design of effective dual-task assessments and fall interventions.

Paraneoplastic movement disorders.

Paraneoplastic movement disorders are diverse autoimmune neurological illnesses occurring in the context of systemic cancer, either in isolation or as part of a multifocal neurological disease. Movement phenomena may be ataxic, hypokinetic (parkinsonian), or hyperkinetic (myoclonus, chorea, or other dyskinetic disorders). Some disorders mimic neurodegenerative or hereditary illnesses. The subacute onset and coexisting nonclassic features of paraneoplastic disorders aid distinction. Paraneoplastic autoantibodies provide further information regarding differentiating cancer association, disease course, and treatment responses. A woman with cerebellar ataxia could have metabotropic glutamate receptor 1 autoimmunity, in the setting of Hodgkin lymphoma, a mild neurological phenotype and response to immunotherapy. A different woman, also with cerebellar ataxia, could have Purkinje cytoplasmic antibody type 1 (anti-Yo), accompanying ovarian adenocarcinoma, a rapidly progressive phenotype and persistent disabling deficits despite immune therapy. The list of antibody biomarkers is growing year-on-year, each with its own ideal specimen type for detection (serum or CSF), accompanying neurological manifestations, cancer association, treatment response, and prognosis. Therefore, a profile-based approach to screening both serum and CSF is recommended. Immune therapy trials are generally undertaken, and include one or more of corticosteroids, IVIg, plasma exchange, rituximab, or cyclophosphamide. Symptomatic therapies can also be employed for hyperkinetic disorders.

Pathogenesis and immunopathology of paraneoplastic disorders.

Paraneoplastic neurologic syndromes (PNS) represent a rare group of immune-mediated complications associated with an underlying tumor. Ectopic protein expression in neoplastic cells or an aberrant immune regulation in the course of hematooncologic diseases or thymomas trigger an autoimmune response that may affect any part of the central and/or peripheral nervous system. Recent advances in drug therapies as well as novel animal models and neuropathologic studies have led to further insights on the immune pathomechanisms of PNS. Although the syndromes share common paths in pathogenesis, they may differ in the disease course, prognosis, and therapy targets, depending on the localization and type of antibody epitope. Neuropathologic hallmarks of PNS associated with antibodies directed against intracellular epitopes are characterized by T cell-dominated inflammation, reactive gliosis including microglial nodules, and neuronal degeneration. By contrast, the neuropathology of cell surface antibody-mediated PNS strongly depends on the targeted antigen and varies from B cell/plasma cell-dominated inflammation and well-preserved neurons together with a reduced expression of the target antigen in anti-NMDAR encephalitis to irreversible Purkinje cell loss in anti-P/Q-type VGCC antibody-associated paraneoplastic cerebellar degeneration. The understanding of different pathomechanisms in PNS is important because they strongly correspond with therapy response and prognosis, and should guide treatment decisions.

Paraneoplastic autoimmune neurologic disorders associated with thymoma.

Thymoma is often associated with paraneoplastic neurologic diseases. Neural autoantibody testing is an important tool aiding diagnosis of thymoma and its autoimmune neurologic complications. Autoantibodies specific for muscle striational antigens and ion channels of the ligand-gated nicotinic acetylcholine receptor superfamily are the most prevalent biomarkers. The autoimmune neurologic disorders associating most commonly with thymoma are myasthenia gravis (MG), peripheral nerve hyperexcitability (neuromyotonia and Morvan syndrome), dysautonomia, and encephalitis. Patients presenting with these neurologic disorders should be screened for thymoma at diagnosis. Although they can cause profound disability, they usually respond to immunotherapy and treatment of the thymoma. Worsening of the neurologic disorder following surgical removal of a thymoma may herald tumor recurrence. Prompt recognition of paraneoplastic neurologic disorders is critical for patient management. A multidisciplinary approach is required for optimal management of neurologic autoimmunity associated with thymoma.