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Indian Journal of Ophthalmology Jun 2022Marin-Amat syndrome is a rare acquired oculofacial synkinesis first reported in 1918. It manifests as involuntary eyelid closure on jaw opening or on lateral movement of...
BACKGROUND
Marin-Amat syndrome is a rare acquired oculofacial synkinesis first reported in 1918. It manifests as involuntary eyelid closure on jaw opening or on lateral movement of the jaw following a peripheral facial nerve palsy. The increased orbicularis tone due to aberrant connections between the cranial nerve (CN) V and CN VII results in an undesirable wink with major psychosocial impact.
PURPOSE
Most cases in literature were either observed or administered botulinum toxin injection to the orbicularis muscle. There are few sporadic reports of surgical interventions with successful outcomes.Hence there was a need to generate awareness regarding various modes of management of this rare entity.
SYNOPSIS
We present a video on the clinical presentation and management of six such patients, of whom one was bilateral. Five patients were females. Traumatic facial nerve paralysis and Bell's palsy was previously diagnosed in one and five patients respectively. The mean age was 52 ± 9.48 years. The mean MRD (margin reflex distance) 1 and MRD 2 was 3.17 ± 0.60 and 5.33± 0.65 mm respectively. On smiling or on movement of the jaw the MRD 1 and 2 was reduced by 2. 50±0.40 and 1.50+/-0.40 mm respectively. Of the six patients four patients opted for nil intervention.
HIGHLIGHTS
Botulinum toxin injection and preseptal orbicularis resection in the upper and lower eyelid along with blepharoplasty was performed in 1 patient each. Satisfactory reduction in the synkinetic movement was achieved in both. Marin-Amat syndrome is a rare often underdiagnosed synkinetic disorder following peripheral facial nerve palsy. Botulinum toxin injection and preseptal orbicularis resection are viable management options.
VIDEO LINK
https://youtu.be/YQbRecp449w.
Topics: Adult; Blinking; Botulinum Toxins; Facial Nerve; Facial Paralysis; Female; Humans; Male; Middle Aged; Syndrome; Synkinesis
PubMed: 35648031
DOI: 10.4103/ijo.IJO_1262_22 -
Journal of Ayub Medical College,... 2019Thyrotoxic periodic paralysis is an uncommon disorder characterized by hypokalaemia, thyrotoxicosis and paralysis, most commonly seen in South Asian males. Aim of our...
Thyrotoxic periodic paralysis is an uncommon disorder characterized by hypokalaemia, thyrotoxicosis and paralysis, most commonly seen in South Asian males. Aim of our case series is to highlight the significance of this reversible cause of patients presenting with neuromuscular paralysis. We present case series of 1 Asian and three Caucasian patients with thyrotoxic periodic paralysis who came with neuromuscular weakness secondary to thyrotoxicosis. All made a swift and uneventful recovery with no recurrence. Thyrotoxic periodic paralysis (TPP) is an infrequent condition having recurrent episodes of muscle weakness as main feature. Hypokalaemia is a common finding seen in these patients. Resolution of the attacks is achieved with correction of hypokalaemia and hyperthyroidism.
Topics: Adult; Aged, 80 and over; Female; Humans; Hyperthyroidism; Hypokalemia; Male; Muscle Weakness; Paralysis; Thyrotoxicosis; Young Adult
PubMed: 31933322
DOI: No ID Found -
Lancet (London, England) May 2017People with chronic tetraplegia, due to high-cervical spinal cord injury, can regain limb movements through coordinated electrical stimulation of peripheral muscles and... (Clinical Trial)
Clinical Trial Comparative Study
BACKGROUND
People with chronic tetraplegia, due to high-cervical spinal cord injury, can regain limb movements through coordinated electrical stimulation of peripheral muscles and nerves, known as functional electrical stimulation (FES). Users typically command FES systems through other preserved, but unrelated and limited in number, volitional movements (eg, facial muscle activity, head movements, shoulder shrugs). We report the findings of an individual with traumatic high-cervical spinal cord injury who coordinated reaching and grasping movements using his own paralysed arm and hand, reanimated through implanted FES, and commanded using his own cortical signals through an intracortical brain-computer interface (iBCI).
METHODS
We recruited a participant into the BrainGate2 clinical trial, an ongoing study that obtains safety information regarding an intracortical neural interface device, and investigates the feasibility of people with tetraplegia controlling assistive devices using their cortical signals. Surgical procedures were performed at University Hospitals Cleveland Medical Center (Cleveland, OH, USA). Study procedures and data analyses were performed at Case Western Reserve University (Cleveland, OH, USA) and the US Department of Veterans Affairs, Louis Stokes Cleveland Veterans Affairs Medical Center (Cleveland, OH, USA). The study participant was a 53-year-old man with a spinal cord injury (cervical level 4, American Spinal Injury Association Impairment Scale category A). He received two intracortical microelectrode arrays in the hand area of his motor cortex, and 4 months and 9 months later received a total of 36 implanted percutaneous electrodes in his right upper and lower arm to electrically stimulate his hand, elbow, and shoulder muscles. The participant used a motorised mobile arm support for gravitational assistance and to provide humeral abduction and adduction under cortical control. We assessed the participant's ability to cortically command his paralysed arm to perform simple single-joint arm and hand movements and functionally meaningful multi-joint movements. We compared iBCI control of his paralysed arm with that of a virtual three-dimensional arm. This study is registered with ClinicalTrials.gov, number NCT00912041.
FINDINGS
The intracortical implant occurred on Dec 1, 2014, and we are continuing to study the participant. The last session included in this report was Nov 7, 2016. The point-to-point target acquisition sessions began on Oct 8, 2015 (311 days after implant). The participant successfully cortically commanded single-joint and coordinated multi-joint arm movements for point-to-point target acquisitions (80-100% accuracy), using first a virtual arm and second his own arm animated by FES. Using his paralysed arm, the participant volitionally performed self-paced reaches to drink a mug of coffee (successfully completing 11 of 12 attempts within a single session 463 days after implant) and feed himself (717 days after implant).
INTERPRETATION
To our knowledge, this is the first report of a combined implanted FES+iBCI neuroprosthesis for restoring both reaching and grasping movements to people with chronic tetraplegia due to spinal cord injury, and represents a major advance, with a clear translational path, for clinically viable neuroprostheses for restoration of reaching and grasping after paralysis.
FUNDING
National Institutes of Health, Department of Veterans Affairs.
Topics: Brain; Brain-Computer Interfaces; Electric Stimulation Therapy; Electrodes, Implanted; Feasibility Studies; Hand; Hand Strength; Humans; Male; Microelectrodes; Middle Aged; Motor Cortex; Movement; Muscle, Skeletal; Quadriplegia; Self-Help Devices; Spinal Cord Injuries; United States; United States Department of Veterans Affairs; User-Computer Interface
PubMed: 28363483
DOI: 10.1016/S0140-6736(17)30601-3 -
Hand (New York, N.Y.) Jan 2018We evaluated the effectiveness of robot-assisted motion and activity in additional to physiotherapy (PT) and occupational therapy (OT) on stroke patients with hand... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
We evaluated the effectiveness of robot-assisted motion and activity in additional to physiotherapy (PT) and occupational therapy (OT) on stroke patients with hand paralysis.
METHODS
A randomized controlled trial was conducted. Thirty-two patients, 34.4% female (mean ± SD age: 68.9 ± 11.6 years), with hand paralysis after stroke participated. The experimental group received 30 minutes of passive mobilization of the hand through the robotic device Gloreha (Brescia, Italy), and the control group received an additional 30 minutes of PT and OT for 3 consecutive weeks (3 d/wk) in addition to traditional rehabilitation. Outcomes included the National Institutes of Health Stroke Scale (NIHSS), Modified Ashworth Scale, Barthel Index (BI), Motricity Index (MI), short version of the Disabilities of the Arm, Shoulder and Hand (QuickDASH), and the visual analog scale (VAS) measurements. All measures were collected at baseline and end of the intervention (3 weeks).
RESULTS
A significant effect of time interaction existed for NIHSS, BI, MI, and QuickDASH, after stroke immediately after the interventions (all, P < .001). The experimental group had a greater reduction in pain compared with the control group at the end of the intervention, a reduction of 11.3 mm compared with 3.7 mm, using the 100-mm VAS scale.
CONCLUSIONS
In the treatment of pain and spasticity in hand paralysis after stroke, robot-assisted mobilization performed in conjunction with traditional PT and OT is as effective as traditional rehabilitation.
Topics: Aged; Aged, 80 and over; Disability Evaluation; Double-Blind Method; Female; Hand; Humans; Male; Middle Aged; Orthotic Devices; Paralysis; Robotics; Stroke Rehabilitation; Visual Analog Scale
PubMed: 28719996
DOI: 10.1177/1558944717692096 -
Anesthesiology Jul 2023The dose of sugammadex recommended by the manufacturer for reversal of rocuronium is 2 mg/kg when the train-of-four count is 2 or more and 4 mg/kg when it is less... (Clinical Trial)
Clinical Trial
BACKGROUND
The dose of sugammadex recommended by the manufacturer for reversal of rocuronium is 2 mg/kg when the train-of-four count is 2 or more and 4 mg/kg when it is less than 2 but there is a posttetanic count of at least 1. The purpose of this dose-finding study was to titrate sugammadex to produce a train-of-four ratio 0.9 or greater at the conclusion of cardiac surgery, and to continue monitoring neuromuscular blockade in the intensive care unit to identify recurrent paralysis. The hypothesis was that many patients would require less than the recommended dose of sugammadex, but that some would require more, and that recurrent paralysis would not occur.
METHODS
Neuromuscular blockade was monitored using electromyography during cardiac surgery. Administration of rocuronium was at the discretion of the anesthesia care team. During sternal closure, sugammadex was titrated in 50-mg increments every 5 min until a train-of-four ratio 0.9 or greater was obtained. Neuromuscular blockade was monitored with electromyography in the intensive care unit until sedation was discontinued before extubation or for a maximum of 7 h.
RESULTS
Ninety-seven patients were evaluated. The dose of sugammadex required to achieve a train-of-four ratio of 0.9 or greater varied from 0.43 to 5.6 mg/kg. There was a statistically significant relationship between the depth of neuromuscular blockade and the sugammadex dose required for reversal, but there was a large variation in dose required at any depth of neuromuscular blockade. Eighty-four of 97 patients (87%) required less than the recommended dose, and 13 (13%) required more. Two patients required additional sugammadex administration for recurrent paralysis.
CONCLUSIONS
When sugammadex was titrated to effect, the dose was usually less than the recommended dose, but it was more in some patients. Therefore, quantitative twitch monitoring is essential for ascertaining that adequate reversal has taken place after sugammadex administration. Recurrent paralysis was observed in two patients.
Topics: Humans; Androstanols; Cardiac Surgical Procedures; gamma-Cyclodextrins; Neuromuscular Blockade; Neuromuscular Nondepolarizing Agents; Paralysis; Rocuronium; Sugammadex
PubMed: 37027807
DOI: 10.1097/ALN.0000000000004578 -
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 -
Journal of Ayub Medical College,... 2022Acute hypokalemic paralysis (AHP) is a life-threatening emergency. It is exceptionally unusual for hypothyroidism to present with AHP. This association can be either...
Acute hypokalemic paralysis (AHP) is a life-threatening emergency. It is exceptionally unusual for hypothyroidism to present with AHP. This association can be either primary or secondary through distal renal tubular acidosis. We report two cases who presented with acute quadriplegia. The succeeding investigations revealed severe hypokalemia and autoimmune hypothyroidism. The second case was found to have Sjogren's syndrome additionally. The underlying aetiology of hypokalemia in both cases was found to be dRTA. The combination of such conditions is reported sporadically. Here we also discuss the potential association of AHP with autoimmune conditions by proxy through dRTA.
Topics: Humans; Hypokalemia; Paralysis; Autoimmune Diseases; Hypothyroidism
PubMed: 36566415
DOI: 10.55519/JAMC-04-10918 -
Medecine Sciences : M/S Oct 2017Since centuries, scientists, clinicians and philosophers have been debating about the interactions between the body and the mind. Researches and advances in... (Review)
Review
Since centuries, scientists, clinicians and philosophers have been debating about the interactions between the body and the mind. Researches and advances in neurophysiology over the last decades have challenged many principles and theories, mainly based on empirical observations, generally well accepted in clinical practice. These new findings, achieved through functional "magnetic resonance imaging", awake brain surgery and allow new techniques and opportunities in physical rehabilitation. More than ever the body and the brain must be considered and treated as a functional entity, the distinction between body and soul developed by Platon should no longer be applied. The development of new technologies, in particular the virtual reality, lends itself perfectly to this global approach. The aim of this article is to present how a purely cognitive training can have beneficial effects on the body and the motor performances in clinics.
Topics: Amputation, Surgical; Amputees; Brain; Cognition; Exercise; Functional Laterality; Humans; Paralysis; Physical Education and Training; Physical Stimulation; Psychophysiology
PubMed: 28994385
DOI: 10.1051/medsci/20173310021 -
BMJ Case Reports Jan 2015Periodic thyrotoxic paralysis is a genetic condition, rare in the West and in Caucasians. Thyrotoxicosis, especially in western hospitals, is an easily overlooked cause...
Periodic thyrotoxic paralysis is a genetic condition, rare in the West and in Caucasians. Thyrotoxicosis, especially in western hospitals, is an easily overlooked cause of sudden-onset paralysis. We present a case of a 40-year-old man who awoke one morning unable to stand. He had bilateral lower limb flaccid weakness of 0/5 with reduced reflexes and equivocal plantars; upper limbs were 3/5 with reduced tone and reflexes. ECG sinus rhythm was at a rate of 88/min. PR interval was decreased and QT interval increased. Bloods showed potassium of 1.8 mEq/L (normal range 3.5-5), free T4 of 29.2 pmol/L (normal range 6.5-17) and thyroid-stimulating hormone (TSH) of <0.01 mIU/L (normal range 0.35-4.94). Random urinary potassium was 8.8 mEq/L (normal range 12.5-62.5). The patient was admitted initially to intensive therapy unit and given intravenous potassium. His symptoms resolved within 24 h. He was diagnosed with thyrotoxic periodic paralysis. He was discharged on carbimazole and propanolol, and follow-up was arranged in the endocrinology clinic.
Topics: Adult; Antithyroid Agents; Arrhythmias, Cardiac; Brugada Syndrome; Carbimazole; Cardiac Conduction System Disease; Heart Conduction System; Humans; Hypokalemia; Hypokalemic Periodic Paralysis; Male; Muscle Weakness; Paralysis; Potassium; Propranolol; Thyrotoxicosis; Thyrotropin
PubMed: 25566931
DOI: 10.1136/bcr-2014-206872 -
Endokrynologia Polska 2022Thyrotoxic myopathy is hyperthyroidism accompanied by muscle lesions. It is recognized as the general term for a group of symptoms with several main manifestations of... (Review)
Review
Thyrotoxic myopathy is hyperthyroidism accompanied by muscle lesions. It is recognized as the general term for a group of symptoms with several main manifestations of several hyperthyroidism patients in the course (e.g. muscle weakness, muscle paralysis, or pain). From the clinical perspective, it may only be manifested as muscle-related symptoms. The symptoms of high metabolic syndrome (e.g. thyrotoxicosis) are absent, obscured, or relatively delayed, so it can be easily misdiagnosed. Accordingly, patients experiencing the first symptom of myopathy should concentrate on the possibility of thyrotoxic myopathy. Given the clinical characteristics, thyrotoxic myopathy can be devided into chronic thyrotoxic myopathy, thyrotoxicosis with periodic paralysis, acute thyrotoxic myopathy, hyperthyroidism with myasthenia gravis, as well as infiltrating exophthalmos with ophthalmoplegia. In this paper, we review thyrotoxic myopathy research status, diagnoses, and treatments.
Topics: Humans; Hyperthyroidism; Muscle Weakness; Muscular Diseases; Paralysis; Thyrotoxicosis
PubMed: 35119093
DOI: 10.5603/EP.a2022.0004