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Journal of Neural Transmission (Vienna,... Aug 2023Depression is one of the most frequent neuropsychiatric symptoms in progressive supranuclear palsy (PSP), a four-repeat tauopathy and most common atypical parkinsonian... (Review)
Review
Depression is one of the most frequent neuropsychiatric symptoms in progressive supranuclear palsy (PSP), a four-repeat tauopathy and most common atypical parkinsonian disorder, but its pathophysiology and pathogenesis are poorly understood. Pubmed/Medline was systematically analyzed until January 2023, with focus on the prevalence, major clinical features, neuroimaging findings and treatment options of depression in PSP. The average prevalence of depression in PSP is around 50%; it does usually not correlate with most other clinical parameters. Depression is associated with multi-regional patterns of morphometric gray matter variations, e.g., reduced thickness of temporo-parieto-occipital cortices, and altered functional orbitofrontal and medial frontal circuits with disturbances of mood-related brain networks. Unfortunately, no specific neuropathological data about depression in PSP are available. Antidepressive and electroconvulsive therapies are effective in improving symptoms; the efficacy of transcranial stimulation needs further confirmation. Depression in PSP is a common symptom, related to multi-regional patterns of cerebral disturbances and complex pathogenic mechanisms that deserve further elucidation as a basis for adequate treatment to improve the quality of life in this fatal disease.
Topics: Humans; Supranuclear Palsy, Progressive; Depression; Quality of Life; Parkinsonian Disorders; Brain
PubMed: 36933007
DOI: 10.1007/s00702-023-02621-w -
Survey of Ophthalmology 2023The consequences of facial nerve palsy and the secondary inability of eyelid closure and blink may lead to devastating complications for the patient, including... (Review)
Review
The consequences of facial nerve palsy and the secondary inability of eyelid closure and blink may lead to devastating complications for the patient, including blindness. Reconstruction techniques to improve eyelid position and function can be broadly classified into 'static' and 'dynamic' techniques. Generally, ophthalmologists have been familiar with static procedures such as upper eyelid loading, tarsorrhaphy, canthoplasty, and lower eyelid suspension. Recently, dynamic techniques are being increasingly described for patients who require definitive strategies to improve eyelid function, once the initial critical goals of corneal protection and vision preservation have been achieved. The choice of technique(s) is dependent upon the status of the main protractor of the eyelid region, as well as the age of the patient, the patient's morbidities and expectations, and surgeon preference. This review shall first describe the clinical and surgical anatomy relevant to the ophthalmic consequences of facial paralysis and discuss methods of defining function and outcomes. A comprehensive review of dynamic eyelid reconstruction is then presented with a discussion of the literature. These various techniques may not be familiar to all clinicians. It is important that ophthalmic surgeons are aware of all options available for their patients. Furthermore, eye care providers must have an understanding of when referral may be appropriate to provide timely intervention and optimal chances of recovery.
Topics: Humans; Facial Paralysis; Facial Nerve; Eyelids; Cornea
PubMed: 37201598
DOI: 10.1016/j.survophthal.2023.05.003 -
CNS & Neurological Disorders Drug... 2024Bell palsy is a non-progressive neurological condition characterized by the acute onset of ipsilateral seventh cranial nerve paralysis. People who suffer from this type... (Review)
Review
Bell palsy is a non-progressive neurological condition characterized by the acute onset of ipsilateral seventh cranial nerve paralysis. People who suffer from this type of facial paralysis develop a droop on one side of their face, or sometimes both. This condition is distinguished by a sudden onset of facial paralysis accompanied by clinical features such as mild fever, postauricular pain, dysgeusia, hyperacusis, facial changes, and drooling or dry eyes. Epidemiological evidence suggests that 15 to 23 people per 100,000 are affected each year, with a recurrence rate of 12%. It could be caused by ischaemic compression of the seventh cranial nerve, which could be caused by viral inflammation. Pregnant women, people with diabetes, and people with respiratory infections are more likely to have facial paralysis than the general population. Immune, viral, and ischemic pathways are all thought to play a role in the development of Bell paralysis, but the exact cause is unknown. However, there is evidence that Bell's hereditary proclivity to cause paralysis is a public health issue that has a greater impact on patients and their families. Delay or untreated Bell paralysis may contribute to an increased risk of facial impairment, as well as a negative impact on the patient's quality of life. For management, antiviral agents such as acyclovir and valacyclovir, and steroid treatment are recommended. Thus, early diagnosis accompanied by treatment of the uncertain etiology of the disorder is crucial. This paper reviews mechanistic approaches, and emerging medical perspectives on recent developments that encounter Bell palsy disorder.
Topics: Pregnancy; Humans; Female; Bell Palsy; Facial Paralysis; Quality of Life; Antiviral Agents; Acyclovir
PubMed: 36959147
DOI: 10.2174/1871527322666230321120618 -
Practical Neurology Nov 2023Classic Raymond syndrome is a rare neurological presentation comprising ipsilateral abducens palsy, contralateral facial paresis and contralateral hemiparesis. We...
Classic Raymond syndrome is a rare neurological presentation comprising ipsilateral abducens palsy, contralateral facial paresis and contralateral hemiparesis. We present a man in his late 60s who presented with diplopia, dysarthria and right-sided limb weakness. This syndrome is one of a group of 'crossed paralyses' of the caudal pons.
Topics: Male; Humans; Brain Ischemia; Stroke; Pons; Facial Paralysis; Paresis; Ischemic Stroke
PubMed: 37524438
DOI: 10.1136/pn-2023-003782 -
Journal of Bodywork and Movement... Jul 2023Cerebral palsy (CP) can be described as a group of permanent non-progressive disorders that occur in the developing fetal or infant brain. Studies have shown that... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Cerebral palsy (CP) can be described as a group of permanent non-progressive disorders that occur in the developing fetal or infant brain. Studies have shown that children and adolescents with CP have low cardiorespiratory fitness and higher energy expenditure during daily activities when compared to typical children. Therefore, interventions focused on the physical conditioning of this population could be critical.
OBJECTIVE
To evaluate the effect of physical conditioning training on distance walked and maximum oxygen consumption (VO₂ max) in individuals with CP, through a systematic review.
METHOD
Two independent researchers performed systematic searches in the PUBMED, SciELO, PEDro, ERIC, and Cochrane databases using the search terms "physical fitness", "aerobic training" or "endurance" combined with "cerebral palsy".
INCLUSION CRITERIA
(1) population (children and adolescents with CP between 5 and 18 years old); (2) type of study (experimental studies); (3) type of intervention (physical conditioning protocols); (4) outcome (distance walked in the 6 min walking test - 6MWT, and VO₂ max).
RESULTS
386 studies were identified and 5 articles were considered eligible. After physical conditioning training, there was an increase of 46.34 m (p = 0.07) and 5.93. ml. kg-1. min -1 (p < 0.001) in the 6MWT and VO₂ max, respectively.
CONCLUSION
Physical conditioning training appears to be clinically beneficial to the cardiorespiratory fitness of children and adolescents with CP.
Topics: Humans; Adolescent; Child; Child, Preschool; Exercise; Physical Fitness; Walking; Exercise Test; Cerebral Palsy; Paralysis
PubMed: 37330763
DOI: 10.1016/j.jbmt.2023.04.036 -
Stroke Feb 2024Stroke is a leading cause of paralysis, most frequently affecting the upper limbs and vocal folds. Despite recent advances in care, stroke recovery invariably reaches a... (Review)
Review
Stroke is a leading cause of paralysis, most frequently affecting the upper limbs and vocal folds. Despite recent advances in care, stroke recovery invariably reaches a plateau, after which there are permanent neurological impairments. Implantable brain-computer interface devices offer the potential to bypass permanent neurological lesions. They function by (1) recording neural activity, (2) decoding the neural signal occurring in response to volitional motor intentions, and (3) generating digital control signals that may be used to control external devices. While brain-computer interface technology has the potential to revolutionize neurological care, clinical translation has been limited. Endovascular arrays present a novel form of minimally invasive brain-computer interface devices that have been deployed in human subjects during early feasibility studies. This article provides an overview of endovascular brain-computer interface devices and critically evaluates the patient with stroke as an implant candidate. Future opportunities are mapped, along with the challenges arising when decoding neural activity following infarction. Limitations arise when considering intracerebral hemorrhage and motor cortex lesions; however, future directions are outlined that aim to address these challenges.
Topics: Humans; Brain-Computer Interfaces; Paralysis; Stroke; Stroke Rehabilitation; Prostheses and Implants
PubMed: 38018832
DOI: 10.1161/STROKEAHA.123.037719 -
MBio Aug 2023Enterovirus D68 (EV-D68) is a nonpolio enterovirus associated with severe respiratory illness and acute flaccid myelitis (AFM), a polio-like illness causing paralysis in...
Enterovirus D68 (EV-D68) is a nonpolio enterovirus associated with severe respiratory illness and acute flaccid myelitis (AFM), a polio-like illness causing paralysis in children. AFM outbreaks have been associated with increased circulation and genetic diversity of EV-D68 since 2014, although the virus was discovered in the 1960s. The mechanisms by which EV-D68 targets the central nervous system are unknown. Since enteroviruses are human pathogens that do not routinely infect other animal species, establishment of a human model of the central nervous system is essential for understanding pathogenesis. Here, we describe two human spinal cord organoid (hSCO)-based models for EV-D68 infection derived from induced, pluripotent stem cell (iPSC) lines. One hSCO model consists primarily of spinal motor neurons, while the another model comprises multiple neuronal cell lineages, including motor neurons, interneurons, and glial cells. These hSCOs can be productively infected with contemporary strains, but not a historic strain, of EV-D68 and produce extracellular virus for at least 2 weeks without appreciable cytopathic effect. By comparison, infection with hSCO with another enterovirus, echovirus 11, causes significant structural destruction and apoptosis. Together, these findings suggest that EV-D68 infection is not the sole mediator of neuronal cell death in the spinal cord in those with AFM and that secondary injury from the immune response likely contributes to pathogenesis. IMPORTANCE AFM is a rare condition that causes significant morbidity in affected children, often contributing to life-long sequelae. It is unknown how EV-D68 causes paralysis in children, and effective therapeutic and preventative strategies are not available. Mice are not native hosts for EV-D68, and thus, existing mouse models use immunosuppressed or neonatal mice, mouse-adapted viruses, or intracranial inoculations. To complement existing models, we report two hSCO models for EV-D68 infection. These three-dimensional, multicellular models comprised human cells and include multiple neural lineages, including motor neurons, interneurons, and glial cells. These new hSCO models for EV-D68 infection will contribute to understanding how EV-D68 damages the human spinal cord, which could lead to new therapeutic and prophylactic strategies for this virus.
Topics: Child; Humans; Animals; Mice; Enterovirus D, Human; Spinal Cord; Paralysis; Motor Neurons; Enterovirus Infections
PubMed: 37535397
DOI: 10.1128/mbio.01058-23 -
Nature Genetics Jul 2023Hereditary congenital facial paresis type 1 (HCFP1) is an autosomal dominant disorder of absent or limited facial movement that maps to chromosome 3q21-q22 and is...
Hereditary congenital facial paresis type 1 (HCFP1) is an autosomal dominant disorder of absent or limited facial movement that maps to chromosome 3q21-q22 and is hypothesized to result from facial branchial motor neuron (FBMN) maldevelopment. In the present study, we report that HCFP1 results from heterozygous duplications within a neuron-specific GATA2 regulatory region that includes two enhancers and one silencer, and from noncoding single-nucleotide variants (SNVs) within the silencer. Some SNVs impair binding of NR2F1 to the silencer in vitro and in vivo and attenuate in vivo enhancer reporter expression in FBMNs. Gata2 and its effector Gata3 are essential for inner-ear efferent neuron (IEE) but not FBMN development. A humanized HCFP1 mouse model extends Gata2 expression, favors the formation of IEEs over FBMNs and is rescued by conditional loss of Gata3. These findings highlight the importance of temporal gene regulation in development and of noncoding variation in rare mendelian disease.
Topics: Animals; Mice; Facial Paralysis; GATA2 Transcription Factor; Motor Neurons; Neurogenesis; Neurons, Efferent
PubMed: 37386251
DOI: 10.1038/s41588-023-01424-9 -
Critical Care Nursing Clinics of North... Dec 2023Progressive supranuclear palsy (PSP) is a fatal neurodegenerative disorder. Care requires a patient-centered approach encompassing compassion, communication, and... (Review)
Review
Progressive supranuclear palsy (PSP) is a fatal neurodegenerative disorder. Care requires a patient-centered approach encompassing compassion, communication, and empathy. Despite concerted actions to streamline PSP patient transitions, the care is multifaceted and cause of concern. Patients with PSP undergoing transitional care are at an increased risk of undesirable outcomes, frequently endure poor communication, and encounter inconsistent care. Therefore, patients with PSP and families worry about the uncertainty in care, including who is accountable for the care and available resources. Through the three spheres of impact, Clinical Nurse Specialists can educate and assist intensive care unit nurses caring for patients with PSP /families, aiding in the successful care transition.
Topics: Humans; Supranuclear Palsy, Progressive; Patient Transfer
PubMed: 37838414
DOI: 10.1016/j.cnc.2023.05.008 -
Journal of Neurology Nov 2023To systematically review the published cases of bilateral facial palsy (BFP) to gather evidence on the clinical assessment and management of this pathology. (Review)
Review
OBJECTIVE
To systematically review the published cases of bilateral facial palsy (BFP) to gather evidence on the clinical assessment and management of this pathology.
METHODS
Following PRISMA statement recommendations, 338 abstracts were screened independently by two authors. Inclusion criteria were research articles of human patients affected by BFP, either central or peripheral; English, Italian, French or Spanish language; availability of the abstract, while exclusion criteria were topics unrelated to FP, and mention of unilateral or congenital FP. Only full-text articles reporting the diagnostic work-up, the management, and the prognosis of the BFP considered for further specific data analysis.
RESULTS
A total of 143 articles were included, resulting a total of 326 patients with a mean age of 36 years. The most common type of the paralysis was peripheral (91.7%), and the autoimmune disease was the most frequent aetiology (31.3%). The mean time of onset after first symptoms was 12 days and most patients presented with a grade higher than III. Associated symptoms in idiopathic BFP were mostly non-specific. The most frequently positive laboratory exams were cerebrospinal fluid analysis, autoimmune screening and peripheral blood smear, and the most performed imaging was MRI. Most patients (74%) underwent exclusive medical treatment, while a minority were selected for a surgical or combined approach. Finally, in more than half of cases a complete bilateral recovery (60.3%) was achieved.
CONCLUSIONS
BFP is a disabling condition. If a correct diagnosis is formulated, possibilities to recover are elevated and directly correlated to the administration of an adequate treatment.
Topics: Humans; Adult; Facial Paralysis; Facial Nerve Diseases; Causality; Magnetic Resonance Imaging
PubMed: 37523065
DOI: 10.1007/s00415-023-11897-7