Mouth-controlled mouse for quadriplegic disabled people: System design and validation.

Disabled people with a high cervical cord injury or quadriplegia face difficulties when controlling a computer. This study presents a digital mouth-controlled mouse-control aid called the bite-press mouth-controlled mouse (BPMCM) to replace the traditional computer mouse. The BPMCM is equipped with a joystick and micro switch, and the disabled person uses neck and head movements to push the joystick and control the cursor position while the three mouse functions (i.e., left-click, right-click, and drag) are activated by bite-pressing for different time intervals. The proposed design eliminates the sip-and-puff technique and the need to recite orders for reduced adaptation time and increased convenience. Furthermore, this design supports plug-and-play and hot plugging in modern mainstream operating systems that can often be directly operated via mouse functions. Experimental results demonstrated that disabled people using a BPMCM were as capable as healthy participants in operating a computer, with both experiments completed within 5 min, and voluntary disabled people immediately adapted to the BPMCM. The proposed design is expected to allow disabled people to operate computers at the same level as healthy participants. The BPMCM also required only half the physical exertion of other mouth-controlled mouse-control aids that require orders to be recited.

An Accurate and Rapidly Calibrating Speech Neuroprosthesis.

BACKGROUND: Brain-computer interfaces can enable communication for people with paralysis by transforming cortical activity associated with attempted speech into text on a computer screen. Communication with brain-computer interfaces has been restricted by extensive training requirements and limited accuracy. METHODS: A 45-year-old man with amyotrophic lateral sclerosis (ALS) with tetraparesis and severe dysarthria underwent surgical implantation of four microelectrode arrays into his left ventral precentral gyrus 5 years after the onset of the illness; these arrays recorded neural activity from 256 intracortical electrodes. We report the results of decoding his cortical neural activity as he attempted to speak in both prompted and unstructured conversational contexts. Decoded words were displayed on a screen and then vocalized with the use of text-to-speech software designed to sound like his pre-ALS voice. RESULTS: On the first day of use (25 days after surgery), the neuroprosthesis achieved 99.6% accuracy with a 50-word vocabulary. Calibration of the neuroprosthesis required 30 minutes of cortical recordings while the participant attempted to speak, followed by subsequent processing. On the second day, after 1.4 additional hours of system training, the neuroprosthesis achieved 90.2% accuracy using a 125,000-word vocabulary. With further training data, the neuroprosthesis sustained 97.5% accuracy over a period of 8.4 months after surgical implantation, and the participant used it to communicate in self-paced conversations at a rate of approximately 32 words per minute for more than 248 cumulative hours. CONCLUSIONS: In a person with ALS and severe dysarthria, an intracortical speech neuroprosthesis reached a level of performance suitable to restore conversational communication after brief training. (Funded by the Office of the Assistant Secretary of Defense for Health Affairs and others; BrainGate2 ClinicalTrials.gov number, NCT00912041.).

Locked-in syndrome: A qualitative study of a life story.

INTRODUCTION: Locked-in syndrome (LIS) is characterized by tetraplegia, anarthria, paralysis of cranial nerves, and facial musculature, with the preservation of consciousness and cognitive abilities, as well as vertical eye movements and eyelid movements, hearing, and breathing. Three types of LIS are distinguished: classic, incomplete, and total. The aim of the present study was to describe the life history of a person with LIS, as well as the wife's experience and perspective of this life history. METHODS: A qualitative life history study was conducted with two participants: a 54-year-old man diagnosed with LIS and his 50-year-old wife. Data were collected through interviews and autobiographical documents submitted by the participants and analyzed following Braun and Clarke's method of inductive thematic analysis. RESULTS: Five main themes were identified: (1) how to understand and overcome the new situation; (2) the process of care and rehabilitation; (3) communication; (4) writing as a way of helping oneself and others; and (5) personal autonomy and social participation. CONCLUSION: The participants valued the support of their friends and family in the acceptance stage of the new situation, giving special importance to the communication skills and medical attention received after diagnosis.

[Recovery from locked-in syndrome: rule rather than exception]. / Herstel na locked-insyndroom.

The prognosis for locked-in syndrome after acquired brainstem injury is unfavourable. However, partial recovery of motor function occurs in many patients and benefits from intensive rehabilitation. Here we evaluate two patient cases and results of a questionnaire among medical doctors specialised in rehabilitation. We define bottlenecks in the treatment of acute locked-in syndrome in the ICU. Locked-in patients have a years-long life expectancy once they have survived the acute phase. There is no validated prognostic instrument to predict recovery, but even small neurological recovery can have large functional benefits. Recovery may take place over an extended period of time, up to years after onset. To unlock the potential to recover we recommend to start with early rehabilitation while the patient is still in the ICU on life sustaining treatment This may set the patient off along the road from locked-in to unlocked.

Atypical presentation of biotinidase deficiency: masquerading neuromyelitis optica spectrum disorder.

Biotinidase deficiency (BTD) is a treatable, inherited metabolic disorder commonly characterised by alopecia, dermatitis, seizures and developmental delay. It can also manifest as optic neuritis and myelitis; however, these are infrequently described in the literature. We report three cases who presented with quadriplegia and vision loss, initially managed as neuromyelitis optica spectrum disorder (NMOSD), based on neuroimaging findings. Two of them initially responded to immune therapy but relapsed after a few months, while one case showed no clinical improvement with immune therapy. The clinical presentation and neuroimaging findings in all three cases were consistent with NMOSD, leading to a delayed diagnosis of BTD. Antiaquaporin4 and antimyelin oligodendrocyte glycoprotein antibodies were negative in all patients. Urine organic acids reported raised markers of biotinidase or holocarboxylase synthase deficiency. Two of them had a dramatic response to biotin supplementation, showing significant improvement in motor function and vision.

[Clinical characteristics analysis of 4 cases with acute flaccid myelitis in children].

Objective: To summarize the clinical manifestations, diagnosis, treatment and prognosis of acute flaccid myelitis (AFM) in children. Methods: Clinical characteristics of 4 AFM cases from Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from September 2018 to November 2022, were analyzed retrospectively. Results: The age of 4 children with AFM was 7 years, 4 years and 3 months, 7 years and 1 month, 6 years and 5 months, respectively. There were 2 boys and 2 girls. Prodromal infection status showed 3 children of respiratory tract infection and 1 child of digestive tract infection. The main manifestation was asymmetrical limb weakness after infection, and the affected limb range was from monoplegia to quadriplegia. Cranial nerve injury was involved in 1 child, no encephalopathy. Magnetic resonance imaging in the spinal cord of all 4 children showed long T1 and T2 signals, mainly involving gray matter. Cerebrospinal fluid cell-protein separation was observed in 2 children. Pathogen detected in 1 child pharyngeal swab was enterovirus D68. Antibody IgM to adenovirus was positive in the blood of 1 child. Antibody IgG against Echo and Coxsackie B virus were positive in the blood of another child. After glucocorticoid, human immunoglobulin or simple symptomatic treatment and at the same time under later rehabilitation training, muscle strength recovered to different degrees, but there were disabilities left in 3 children. Conclusions: AFM should be considered in children with acute and asymmetrical flaccid paralysis accompanied by abnormal magnetic resonance imaging signal in the central region of spinal cord, especially post-infection. The effective treatment is limited and the prognosis is poor.

Extensor Carpi Radialis Brevis Tendon Transfer for Thumb and Finger Flexion Reconstruction after Failed Extensor Carpi Radialis Brevis Motor Branch Transfer in a Tetraplegic Patient.

Distal nerve transfers can restore precise motor control in tetraplegic patients. When nerve transfers are not successful, tendon transfers may be used for subsequent reconstruction. In this case, an extensor carpi radialis brevis (ECRB) tendon transfer was used to restore thumb and finger flexion following an unsuccessful ECRB to anterior interosseous nerve transfer in a young tetraplegic patient. Twelve months following tendon transfer, the patient demonstrated functional grip and pinch strength and was using both hands for daily activities. Level of Evidence: Level V (Therapeutic).

Effects of two external cooling strategies on physiological and perceptual responses of athletes with tetraplegia during and after exercise in the heat.

Athletes with tetraplegia may experience marked hyperthermia while exercising under environmental heat stress due to their limited ability to dissipate heat through evaporative means. This study investigated the effectiveness of two external cooling strategies (i.e., spraying water onto the body surface or using a cooling vest) on physiological and perceptual variables in tetraplegic athletes during and after an aerobic exercise session in a hot environment. Nine male wheelchair rugby players performed an incremental test to determine their maximum aerobic power output. After that, they were subjected to three experimental trials in a counter-balanced order: control (CON, no body cooling), cooling vest (CV), and water spraying (WS). During these trials, they performed 30 min of a submaximal exercise (at 65% of their maximum aerobic power) inside an environmental chamber set to maintain the dry-bulb temperature at 32 °C. The following variables were recorded at regular intervals during the exercise and for an additional 30 min following the exertion (i.e., post-exercise recovery) with the participants also exposed to 32 °C: body core temperature (TCORE), skin temperature (TSKIN), heart rate (HR), rating of perceived exertion (RPE), thermal comfort (TC), and thermal sensation (TS). While exercising in CON conditions, the tetraplegic athletes had the expected increases in TCORE, TSKIN, HR, RPE, and TC and TS scores. HR, TC, and TS decreased gradually toward pre-exercise values after the exercise, whereas TCORE and TSKIN remained stable at higher values. Using a cooling vest decreased the temperature measured only on the chest and reduced the scores of RPE, TC, and TS during and after exercise but did not influence the other physiological responses of the tetraplegic athletes. In contrast, spraying water onto the athletes' body surface attenuated the exercise-induced increase in TSKIN, led to lower HR values during recovery, and was also associated with better perception during and after exercise. We conclude that water spraying is more effective than the cooling vest in attenuating physiological strain induced by exercise-heat stress. However, although both external cooling strategies do not influence exercise hyperthermia, they improve the athletes' thermal perception and reduce perceived exertion.

A Multimodal Assistive-Robotic-Arm Control System to Increase Independence After Tetraplegia.

Following tetraplegia, independence for completing essential daily tasks, such as opening doors and eating, significantly declines. Assistive robotic manipulators (ARMs) could restore independence, but typically input devices for these manipulators require functional use of the hands. We created and validated a hands-free multimodal input system for controlling an ARM in virtual reality using combinations of a gyroscope, eye-tracking, and heterologous surface electromyography (sEMG). These input modalities are mapped to ARM functions based on the user's preferences and to maximize the utility of their residual volitional capabilities following tetraplegia. The two participants in this study with tetraplegia preferred to use the control mapping with sEMG button functions and disliked winking commands. Non-disabled participants were more varied in their preferences and performance, further suggesting that customizability is an advantageous component of the control system. Replacing buttons from a traditional handheld controller with sEMG did not substantively reduce performance. The system provided adequate control to all participants to complete functional tasks in virtual reality such as opening door handles, turning stove dials, eating, and drinking, all of which enable independence and improved quality of life for these individuals.

An analysis of clinical characteristics of rare bilateral medial medullary infarction: An observational study.

This study aimed to characterize the risk factors, etiology, clinical manifestations, anatomical characteristics, stroke mechanisms, imaging features, and prognosis of bilateral medial medullary infarction (BMMI). A retrospective analysis was conducted on 11 patients with BMMI who met the inclusion criteria at the Affiliated Hospital of Xuzhou Medical University from January 2013 to January 2023. The patients' imaging and clinical features were analyzed and summarized. Eleven patients (7 male, 4 female), aged 46 to 62 years, met the inclusion criteria. Common clinical presentations included dysarthria (90.9%), dysphagia (90.9%), quadriplegia (81.8%), and so on. Within 72 hours of onset, 8 cases presented with quadriplegia, 2 cases with hemiplegia, and 1 case without limb paralysis. The main risk factor for BMMI was hypertension, followed by diabetes. "Heart appearance" infarcts occurred in 4 cases (36.4%), while "Y appearance" infarcts occurred in 7 cases (63.6%). Among the patients, 3 had unilateral vertebral artery stenosis or occlusion, 5 had bilateral vertebral artery stenosis or occlusion, 2 had normal vertebral basilar artery, and 1 did not undergo cerebrovascular examination. All patients received standardized treatment for cerebral infarction. The prognosis was poor, with 81.8% of patients having an unfavorable outcome, including 1 death, 9 cases of disability, and only 1 patient achieving self-care ability after recovery. BMMI is more prevalent in males aged 45 to 60 years. The main risk factors are hypertension and diabetes. Atherosclerosis is the primary etiological subtype. The main clinical manifestations are dyskinesia, dizziness, quadriplegia, and dysarthria. The prognosis of BMMI is poor. The specific imaging features of "heart appearance" or "Y appearance" infarcts aid in the diagnosis of BMMI.

Chasing Gold: Heat Acclimation in Elite Handcyclists with Spinal Cord Injury.

Thermoregulation is impaired in individuals with a spinal cord lesion (SCI), affecting sweat capacity, heat loss, and core temperature. This can be particularly problematic for athletes with SCI who exercise in hot and humid conditions, like those during the Tokyo 2020 Paralympic Games. Heat acclimation can support optimal preparation for exercise in such challenging environments, but evidence is limited in endurance athletes with SCI. We evaluated whether seven consecutive days of exercise in the heat would result in heat acclimation. Five elite para-cycling athletes with SCI participated (two females, three males, median (Q1-Q3) 35 (31-51) years, four with paraplegia and one with tetraplegia). All tests and training sessions were performed in a heat chamber (30°C and 75% relative humidity). A time-to-exhaustion test was performed on day 1 (pretest) and day 7 (posttest). On days 2-6, athletes trained daily for one hour at 50-60% of individual peak power (PPeak). Comparing pretest and posttest, all athletes increased their body mass loss (p=0.04), sweat rate (p=0.04), and time to exhaustion (p=0.04). Effects varied between athletes for core temperature and heart rate. All athletes appeared to benefit from our heat acclimation protocol, helping to optimize their preparation for the Tokyo 2020 Paralympic Games.

Non-invasive spinal cord electrical stimulation for arm and hand function in chronic tetraplegia: a safety and efficacy trial.

Cervical spinal cord injury (SCI) leads to permanent impairment of arm and hand functions. Here we conducted a prospective, single-arm, multicenter, open-label, non-significant risk trial that evaluated the safety and efficacy of ARCEX Therapy to improve arm and hand functions in people with chronic SCI. ARCEX Therapy involves the delivery of externally applied electrical stimulation over the cervical spinal cord during structured rehabilitation. The primary endpoints were safety and efficacy as measured by whether the majority of participants exhibited significant improvement in both strength and functional performance in response to ARCEX Therapy compared to the end of an equivalent period of rehabilitation alone. Sixty participants completed the protocol. No serious adverse events related to ARCEX Therapy were reported, and the primary effectiveness endpoint was met. Seventy-two percent of participants demonstrated improvements greater than the minimally important difference criteria for both strength and functional domains. Secondary endpoint analysis revealed significant improvements in fingertip pinch force, hand prehension and strength, upper extremity motor and sensory abilities and self-reported increases in quality of life. These results demonstrate the safety and efficacy of ARCEX Therapy to improve hand and arm functions in people living with cervical SCI. ClinicalTrials.gov identifier: NCT04697472 .

Representation of internal speech by single neurons in human supramarginal gyrus.

Speech brain-machine interfaces (BMIs) translate brain signals into words or audio outputs, enabling communication for people having lost their speech abilities due to diseases or injury. While important advances in vocalized, attempted and mimed speech decoding have been achieved, results for internal speech decoding are sparse and have yet to achieve high functionality. Notably, it is still unclear from which brain areas internal speech can be decoded. Here two participants with tetraplegia with implanted microelectrode arrays located in the supramarginal gyrus (SMG) and primary somatosensory cortex (S1) performed internal and vocalized speech of six words and two pseudowords. In both participants, we found significant neural representation of internal and vocalized speech, at the single neuron and population level in the SMG. From recorded population activity in the SMG, the internally spoken and vocalized words were significantly decodable. In an offline analysis, we achieved average decoding accuracies of 55% and 24% for each participant, respectively (chance level 12.5%), and during an online internal speech BMI task, we averaged 79% and 23% accuracy, respectively. Evidence of shared neural representations between internal speech, word reading and vocalized speech processes was found in participant 1. SMG represented words as well as pseudowords, providing evidence for phonetic encoding. Furthermore, our decoder achieved high classification with multiple internal speech strategies (auditory imagination/visual imagination). Activity in S1 was modulated by vocalized but not internal speech in both participants, suggesting no articulator movements of the vocal tract occurred during internal speech production. This work represents a proof-of-concept for a high-performance internal speech BMI.

Unusual presentation of Sjogren's syndrome during pregnancy: a case report.

BACKGROUND: Pregnancy imposes significant physiological changes, including alterations in electrolyte balance and renal function. This is especially important because certain disorders might worsen and make people more susceptible to electrolyte abnormalities. One such condition is Sjogren's syndrome (SS), an autoimmune disease that can cause distal renal tubular acidosis (dRTA). This case report offers a unique perspective on the intricate physiological interplay during pregnancy, emphasizing the critical importance of recognizing and managing electrolyte abnormalities, particularly in the context of autoimmune disorders such as Sjogren's syndrome. CASE PRESENTATION: We report a case of a 31-year-old pregnant Indian woman at 24 weeks gestation presenting with fever, gastrointestinal symptoms, and progressive quadriparesis followed by altered sensorium. Severe hypokalaemia and respiratory acidosis necessitated immediate intubation and ventilatory support. Investigations revealed hypokalaemia, normal anion gap metabolic acidosis, and positive autoimmune markers for SS. Concurrently, she tested positive for IgM Leptospira. Management involved aggressive correction of electrolyte imbalances and addressing the underlying SS and leptospirosis. CONCLUSION: This case underscores that prompt recognition and management are paramount to prevent life-threatening complications in pregnant patients with autoimmune disease. This report sheds light on the unique challenge of managing hypokalaemic quadriparesis in the context of Sjogren's syndrome during pregnancy.

Vertebral artery dissection as the underlying cause of ventral spinal epidural haematoma.

Spontaneous spine epidural haematoma is a rare occurrence, with an incidence of 0.1/100 000 inhabitants/year. The anterior location of the haematoma is very uncommon since the dural sac is firmly attached to the posterior longitudinal ligament. Vertebral artery dissection as its underlying cause is an exceptionally rare event, with only two documented cases.This article presents the case of young woman who arrived at the emergency room with a spinal ventral epidural haematoma extending from C2 to T10, caused by a non-traumatic dissecting aneurysm of the right vertebral artery at V2-V3 segment. Since the patient was tetraparetic, she underwent emergent laminectomy, and the vertebral artery dissection was subsequently treated endovascularly with stenting.Vertebral artery dissection with subsequent perivascular haemorrhage is a possible cause of spontaneous spine epidural haematoma, particularly when located ventrally in the cervical and/or high thoracic column. Hence the importance of a thorough investigation of the vertebral artery integrity.

Rarely Seen Compartment Syndrome of the Shoulder and Back: Diagnosis and Management.

Compartment syndrome (CS) is a well-known surgical emergency with high morbidity including potential long-term disability and limb loss. The most important factor determining the degree of morbidity with CS is time to treatment; therefore, early diagnosis and surgery are vital. We present a patient who fell off his bicycle and sustained cervical spine fractures causing near complete quadriplegia. He was found by the road over 12 hours later, so his creatine phosphokinase (CPK) was trended and serial examinations were performed. We identified tight deltoid, trapezius, and latissimus compartments and brought him to the operating room for fasciotomies. Although lab values and compartment pressures can be helpful, they should not guide treatment. It is important to consider atypical sites for CS and complete a head to toe physical examination. Patients should proceed to the operating room if clinical suspicion exists for CS because of the morbidity associated with a missed diagnosis.

A case of iatrogenic acute spinal cord injury with tetraplegia following thyroid surgery.

AIM: Iatrogenic acute spinal cord injury with tetraplegia is a serious consequence of non-spinal surgery.We report a case of acute spinal cord injury with tetraplegia after thyroid surgery. METHOD: The patient was pathologically diagnosed with papillary carcinoma, underwent left thyroidectomy, and developed tetraplegia after surgery. RESULT: The patient was diagnosed with acute spinal cord injury with tetraplegia and cured after anti-inflammatory and dehydrating treatment. CONCLUSION: Iatrogenic spinal cord injuries after elective non-spinal surgery can have catastrophic consequences, and clinicians must be alert to this possibility in clinical practice.

Dengue-associated longitudinally extensive transverse myelitis.

We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases.

Patient Resiliency and Caregiver Burden After Traumatic Spinal Cord Injury Resulting in Quadriplegia.

Traumatic spinal cord injury (tSCI) resulting in quadriplegia is a life-altering injury for patients and caregivers. We conducted a retrospective review of patients treated for tSCI and quadriplegia at a level 1 trauma center to assess quality of life (QOL), socioeconomic factors, and mortality. Patients and caregivers were surveyed. Of the 65 patients included, 33 contacts were made. Seventeen surveys were completed (12 caregivers and 5 patients). Six unreachable patients were confirmed alive via medical record. Mortality rate among these 39 accessible patients was 23% (n = 9). Medicaid and uninsured patients experienced longer hospital length of stay (P < .0001) and discharged to home or nursing facilities (P < .0001) more often than those with private insurance or Medicare. Patients reported overall "good" QOL (80%) while caregivers reported overall decreased QOL markers. Our results reflect the resilience among this patient population, but also highlight the impact of this life-altering injury on the caregiver.