Thyrotoxic periodic paralysis presenting with quadriparesis and hyperreflexia.

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism that manifests as painless flaccid paralysis. An East Asian man in his late 20s presented to the emergency department with an acute onset of quadriparesis associated with hypertonia and hyperreflexia. His initial symptoms and signs suggested involvement of the brain and spinal cord; however, MRI of the neuroaxis was normal. His serum potassium concentration was low, and thyroid test results were consistent with hyperthyroidism. The patient was diagnosed with TPP associated with Graves' disease and was treated with potassium supplementation, propranolol and methimazole. Motor strength improved to his baseline level of power; bulk was normal, and tone was increased. Although flaccid paralysis is a typical presentation of TPP, brisk reflexes and muscle spasticity cannot rule out this condition. This case highlights the importance of considering TPP as a possible diagnosis in patients presenting with acute quadriparesis.

Prospective analysis of a surgical algorithm to achieve ventilator weaning in cervical tetraplegia.

Objectives: Chronic ventilator dependency in cervical tetraplegia is associated with substantial morbidity. When non-invasive weaning methods have failed the primary surgical treatment is diaphragm pacing. Phrenic nerve integrity and diaphragm motor units are requirements for effective pacing but may need to be restored for successful weaning. A surgical algorithm that includes: 1. Diaphragm pacing, 2. Phrenic nerve reconstruction, and 3. Diaphragm muscle replacement, may provide the capability of reducing or reversing ventilator dependency in virtually all cervical tetraplegics.Design: Prospective case series.Setting: A university-based hospital from 2015 to 2019.Participants: Ten patients with ventilator-dependent cervical tetraplegia.Interventions: I. Pacemaker alone, II. Pacemaker + phrenic nerve reconstruction, or III. Pacemaker + diaphragm muscle replacement.Outcome measures: Time from surgery to observed reduction in ventilator requirements (↓VR), ventilatory needs as of most recent follow-up [no change (NC), partial weaning (PW, 1-12 h/day), or complete weaning (CW, >12 h/day)], and complications.Results: Both patients in Group I achieved CW at 6-month follow-up. Two patients in Group II achieved CW, and in another two patients PW was achieved, at 1.5-2-year follow-up. The remaining two patients are NC at 6 and 8-month follow-up, respectively. In group III, both patients achieved PW at 2-year follow-up. Complications included mucous plugging (n = 1) and pacemaker malfunction requiring revision (n = 3).Conclusion: Although more investigation is necessary, phrenic nerve reconstruction or diaphragm muscle replacement performed (when indicated) with pacemaker implantation may allow virtually all ventilator-dependent cervical tetraplegics to partially or completely wean.

A case for inspiratory muscle training in SCI: potential role as a preventative tool in infectious respiratory diseases like COVID-19.

INTRODUCTION: Respiratory complications (RC) are a leading cause of death after spinal cord injury (SCI) due to compromised immune function and respiratory muscle weakness. Thus, individuals with SCI are at high risk of developing COVID-19 related RC. Results of a SCI clinical trial showed a supervised respiratory muscle training (RMT) program decreased risk of developing RC. The feasibility of conducting unsupervised RMT is not well documented. Four publications (n = 117) were identified in which unsupervised RMT was performed. Significant improvements in respiratory outcomes were reported in two studies: Maximal Inspiratory and Expiratory Pressure (MIP40% and MEP25%, respectively), Peak Expiratory Flow (PEF9%), seated and supine Forced Vital Capacity (FVC23% and 26%, respectively), and Peak Cough Flow (28%). This review and case report will attempt to show that an inspiratory muscle training (IMT) home exercise program (HEP) is feasible and may prepare the respiratory system for RC associated with COVID-19 in patients with SCI. CASE PRESENTATION: A 23-year-old with tetraplegia (P1), history of mechanical ventilation, and hospitalization for RC, completed 27 IMT HEP sessions in one month. MIP and sustained MIP (SMIP) increased from baseline by 28% and 26.5%, respectively. Expiratory volumes and rates also improved (FVC, FEV1, and PEF: 11.7%, 8.3%, and 14.2%, respectively). DISCUSSION: The effects of COVID-19 on patients with SCI remains inconclusive, but recent literature and the results of this case suggest that unsupervised IMT is feasible and may limit the severity of RC in patients with SCI who contract COVID-19.

Impact of respiratory muscle training on respiratory muscle strength, respiratory function and quality of life in individuals with tetraplegia: a randomised clinical trial.

BACKGROUND: Respiratory complications remain a leading cause of morbidity and mortality in people with acute and chronic tetraplegia. Respiratory muscle weakness following spinal cord injury-induced tetraplegia impairs lung function and the ability to cough. In particular, inspiratory muscle strength has been identified as the best predictor of the likelihood of developing pneumonia in individuals with tetraplegia. We hypothesised that 6 weeks of progressive respiratory muscle training (RMT) increases respiratory muscle strength with improvements in lung function, quality of life and respiratory health. METHODS: Sixty-two adults with tetraplegia participated in a double-blind randomised controlled trial. Active or sham RMT was performed twice daily for 6 weeks. Inspiratory muscle strength, measured as maximal inspiratory pressure (PImax) was the primary outcome. Secondary outcomes included lung function, quality of life and respiratory health. Between-group comparisons were obtained with linear models adjusting for baseline values of the outcomes. RESULTS: After 6 weeks, there was a greater improvement in PImax in the active group than in the sham group (mean difference 11.5 cmH2O (95% CI 5.6 to 17.4), p<0.001) and respiratory symptoms were reduced (St George Respiratory Questionnaire mean difference 10.3 points (0.01-20.65), p=0.046). Significant improvements were observed in quality of life (EuroQol-Five Dimensional Visual Analogue Scale 14.9 points (1.9-27.9), p=0.023) and perceived breathlessness (Borg score 0.64 (0.11-1.17), p=0.021). There were no significant improvements in other measures of respiratory function (p=0.126-0.979). CONCLUSIONS: Progressive RMT increases inspiratory muscle strength in people with tetraplegia, by a magnitude which is likely to be clinically significant. Measurement of baseline PImax and provision of RMT to at-risk individuals may reduce respiratory complications after tetraplegia. TRIAL REGISTRATION NUMBER: Australian New Zealand Clinical Trials Registry (ACTRN 12612000929808).

Breathing-synchronised electrical stimulation of the abdominal muscles in patients with acute tetraplegia: A prospective proof-of-concept study.

OBJECTIVE: To examine whether, by enhancing breathing depth and expectoration, early use of breathing-synchronised electrical stimulation of the abdominal muscles (abdominal functional electrical stimulation, AFES) is able to reduce pulmonary complications during the acute phase of tetraplegia. DESIGN: Prospective proof-of-concept study. SETTING: Spinal cord unit at a level 1 trauma center. METHOD: Following cardiovascular stabilisation, in addition to standard treatments, patients with acute traumatic tetraplegia (ASIA Impairment Scale A or B) underwent breathing-synchronised electrical stimulation of the abdominal muscles to aid expiration and expectoration. The treatment was delivered in 30-minute sessions, twice a day for 90 days. The target was for nine of 15 patients to remain free of pneumonia meeting Centers for Disease Control and Prevention (CDC) diagnostic criteria. RESULTS: Eleven patients were recruited to the study between October 2011 and November 2012. Two patients left the study before completion. None of the patients contracted pneumonia during the study period. No complications from electrical stimulation were observed. AFES led to a statistically significant increase in peak inspiratory and expiratory flows and a non-statistically significant increase in tidal volume and inspiratory and expiratory flow. When surveyed, 6 out of 9 patients (67%) reported that the stimulation procedure led to a significant improvement in breathing and coughing. CONCLUSION: AFES appears to be able to improve breathing and expectoration and prevent pneumonia in the acute phase of tetraplegia (up to 90 days post-trauma). This result is being validated in a prospective multicentre comparative study.

Abdominal functional electrical stimulation to enhance mechanical insufflation-exsufflation.

CONTEXT: Respiratory complications, attributed to the build-up of secretions in the airway, are a leading cause of rehospitalisation for the tetraplegic population. Previously, we observed that the application of Abdominal Functional Electrical Stimulation (AFES) improved cough function and increased demand for secretion removal, suggesting AFES may aid secretion clearance. Clinically, secretion clearance is commonly achieved by using Mechanical insufflation-exsufflation (MI-E) to simulate a cough. In this study the feasibility of combining AFES with MI-E is evaluated. FINDINGS: AFES was successfully combined with MI-E at eight fortnightly assessment sessions conducted with one sub-acute participant with tetraplegia. By using the signal from a pressure sensor, integrated with the MI-E device, AFES was correctly applied in synchrony with MI-E with an accuracy of 96.7%. Acute increases in exhaled volume and peak flow were observed during AFES assisted MI-E, compared to MI-E alone, at six of eight assessment sessions. CONCLUSION: The successful integration of AFES with MI-E at eight assessment sessions demonstrates the feasibility of this technique. The acute increases in respiratory function observed at the majority of assessment sessions generate the hypothesis that AFES assisted MI-E may be more effective for secretion clearance than MI-E alone.

Respiratory neuromodulation in patients with neurological pathologies: for whom and how?

Implanted phrenic nerve stimulation is a technique restoring spontaneous breathing in patients with respiratory control failure, leading to being dependent on mechanical ventilation. This is the case for quadriplegic patients with a high spinal cord injury level and for patients with congenital central hypoventilation syndrome. The electrophysiological diaphragm explorations permits better patient selection, confirming on the one hand a definite issue with central respiratory command and on the other hand the integrity of diaphragmatic phrenic nerves. Today there are two different phrenic stimulation techniques: the quadripolar intrathoracic stimulation and the bipolar intradiaphragmatic stimulation. Both techniques allow patients to be weaned off their mechanical ventilator, improving dramatically their quality of life. In fact, one of the systems (phrenic intradiaphragmatic stimulation) was granted social security reimbursement in 2009, and now both are reimbursed. In the future, phrenic intradiaphragmatic stimulation may find its place in the intensive care unit, for patients needing it temporarily, for example, after certain surgeries with respiratory complications as well as diaphragmatic atrophies induced by prolonged mechanical ventilation.

Video-assisted thoracoscopic implantation of a diaphragmatic pacemaker in a child with tetraplegia: indications, technique, and results.

We report the case of a child with tetraplegia after cervical trauma, who subsequently underwent diaphragmatic pacemaker implantation. We reviewed the major indications for diaphragmatic pacing and the types of devices employed. We highlight the unequivocal benefit of diaphragmatic pacing in the social and educational reintegration of individuals with tetraplegia.

Relatamos o caso de uma criança tetraplégica após trauma cervical que foi posteriormente submetida a implante de marca-passo diafragmático. Revisamos as principais indicações da estimulação diafragmática e os tipos de dispositivos empregados, assim como apontamos o inequívoco benefício da reinserção socioeducacional desses indivíduos na sociedade.

Video-assisted thoracoscopic implantation of a diaphragmatic pacemaker in a child with tetraplegia: indications, technique, and results / Implante de marca-passo diafragmático por videotoracoscopia em criança com tetraplegia: indicações, técnica e resultados

We report the case of a child with tetraplegia after cervical trauma, who subsequently underwent diaphragmatic pacemaker implantation. We reviewed the major indications for diaphragmatic pacing and the types of devices employed. We highlight the unequivocal benefit of diaphragmatic pacing in the social and educational reintegration of individuals with tetraplegia.

Relatamos o caso de uma criança tetraplégica após trauma cervical que foi posteriormente submetida a implante de marca-passo diafragmático. Revisamos as principais indicações da estimulação diafragmática e os tipos de dispositivos empregados, assim como apontamos o inequívoco benefício da reinserção socioeducacional desses indivíduos na sociedade.

Sleep disruption in tetraplegia: a randomised, double-blind, placebo-controlled crossover trial of 3 mg melatonin.

STUDY DESIGN: Randomised, double-blind, placebo-controlled crossover trial of melatonin supplementation to people with complete tetraplegia. OBJECTIVES: To investigate the effect that 3 mg melatonin supplementation has on objective and subjective sleep, quality of life and mood of people living with complete tetraplegia. SETTING: Austin Hospital Sleep Laboratory and participants' homes, Melbourne, Victoria, Australia. METHODS: Two week run-in followed by 3 week nightly administration of 3 mg melatonin or placebo, 2-week washout and further 3 week administration of the opposite treatment. Four testing sessions were conducted; the last nights of the run-in, treatment and washout periods. Testing sessions involved recording full polysomnography, completing a questionnaire battery and collecting urine and blood samples. The questionnaires assessed mood, sleep symptoms and health-related quality of life, and the urine and plasma samples assayed 6-sulphatoxymelatonin (aMT6s) and melatonin levels, respectively. A sleep diary was completed throughout the study. RESULTS: Eight participants (mean (s.d.): age 49.5 years (16), postinjury 16.9 years (7.1)) were recruited in which seven concluded the protocol. Endogenous-circulating melatonin was significantly higher (P < or = 0.01) following melatonin (urine: 152.94 µg h(-1) (74.51), plasma: 43,554.57 pM (33,527.11)) than placebo (urine: 0.86 µg h(-1) (0.40), plasma: 152.06 pM (190.55)). Subjective sleep improved significantly following melatonin specifically for duration of sleep per night and psychological wellbeing. Objective sleep showed a significant increase in light sleep with melatonin, with all other sleep parameters being unchanged. CONCLUSION: These results suggest that increasing melatonin in people with complete tetraplegia is beneficial, especially for subjective sleep. Investigation of the pharmacokinetics of melatonin metabolism in this population is warranted. SPONSORSHIP: This project is proudly supported by the Transport Accident Commission.

Threshold currents of platinum electrodes used for functional electrical stimulation of the phrenic nerves for treatment of central apnea.

BACKGROUND: Stability of threshold currents during long-term use of phrenic nerve stimulation has been questioned. METHODS AND RESULTS: Between January 5, 1988, and March 5, 2008, 49 patients with functional C2-tetraplegia received an Atrostim PNS (Atrotech Ltd., Tampere, Finland) as treatment of their respiratory insufficiency; a follow-up of 35 of such patients was carried out exclusively in our institution for 6.3 (4.44) 0.04-15.75 years (mean [standard deviation (SD)] range). The device employed four-pole sequential nerve stimulation, which provided four threshold currents subsequently evaluated for each phrenic nerve. Stimulation data were prospectively recorded. The differences between threshold currents recorded 1 year after implantation and the last recorded values were 0.33-0.43 (0.44-0.63) 0-2.9 mA. After having excluded the data of eight patients with values >1 mA (= mean + SD), we registered the differences for the remaining patients of 0.15-0.24 (0.14-0.24) 0-0.95 mA, which is just twice the adjustment accuracy of the device. Out of the eight problem cases one had, and two were suspected to have, surgical trauma; all three nerves recovered. In two cases the values steadily increasing over years might have been caused by unspecific foreign body reaction. Two cases with values >1 mA for different durations at different electrodes might be caused by biofilm, and one patient displaying steadily increasing values lived, unwilling to live, only 2 years after the implantation. CONCLUSION: Thus, there was no permanent nerve injury and in 77% of the presented cases threshold currents remained stable.

Recognition and mental manipulation of body parts dissociate in locked-in syndrome.

Several lines of evidence demonstrate that the motor system is involved in motor simulation of actions, but some uncertainty exists about the consequences of lesions of descending motor pathways on mental imagery tasks. Moreover, recent findings suggest that the motor system could also have a role in recognition of body parts. To address these issues in the present study we assessed patients with a complete damage of descending motor pathways (locked-in syndrome, LIS) on the hand laterality task, requiring subjects to decide whether a hand stimulus in a given spatial orientation represents a left or a right hand. LIS patients were less accurate than healthy controls in judging hand laterality; more importantly, LIS patients' performance was modulated by spatial orientation of hand stimuli whereas it was not affected by biomechanical constraints. These findings demonstrate a dissociation between spared hand recognition and impaired access to action simulation processes in LIS patients.

A rare instance of 'cardio-respiratory pacing': permanent pacemaker insertion for symptomatic bradycardia in a quadriplegic man dependent on diaphragmatic pacing by phrenic nerve stimulators.

Patients with high spinal cord injury may present with significant cardiac dysautonomia. There is a dearth of data regarding electromechanical interference to cardiac pacemakers from phrenic nerve stimulators which are used in such patients for respiratory support. We report an instance of bipolar lead permanent pacemaker insertion for ventricular standstill in a man with quadriplegia following C2 fracture and the measures we adopted to minimise electromagnetic interference with phrenic nerve stimulators. To the best of our knowledge, this is the first reported instance of successful pacemaker insertion in a quadriplegic patient on long-term diaphragmatic pacing.

Phrenic nerve stimulation: the Australian experience.

Phrenic nerve stimulation is a technique whereby a nerve stimulator provides electrical stimulation of the phrenic nerve to cause diaphragmatic contraction. The most common indications for this procedure are central alveolar hypoventilation and high quadriplegia. This paper reviews the available data on the 19 patients treated with phrenic nerve stimulation in Australia to date. Of the 19 patients, 14 required pacing due to quadriplegia, one had congenital central hypoventilation syndrome and one had brainstem encephalitis. Information was unavailable for the remaining three patients. Currently, 11 of the pacers are known to be actively implanted, with the total pacing duration ranging from 1 to 21 years (mean 13 years). Eight of the 19 patients had revision surgeries. Four of these were to replace the original I-107 system (which had a 3-5-year life expectancy) with the current I-110 system, which is expected to perform electrically for the patient's lifetime. Three patients had revisions due to mechanical failure. The remaining patients' notes were incomplete. These data suggest that phrenic nerve stimulation can be used instead of mechanical ventilators for long-term ongoing respiratory support.

Laparoscopic diaphragm pacing for tetraplegia.

(1) The NeuRx DPS is a laparoscopically implanted device that provides ventilatory support. (2) This device stimulates the diaphragm muscle, rather than the phrenic nerve, and is intended to lead to less risk of nerve damage than other therapies.(3) This technology provides an alternative to mechanical ventilation, and allows patients to increase day-to-day freedom and minimize the risk of respiratory infection. (4) The NeuRx DPS safety profile is based on clinical testing, which began with clinical trials starting in 2000. It has the potential to reduce costs, but this has not been well established.

Polineuropatía tardía secundaria a intoxicación por insecticidas organofosforados / Delayed polyneuropathy induced by organophosphorus insecticides poisoning

Los insecticidas organofosforados presentan como mecanismo tóxico más importante la inhibición directa de la acetilcolinesterasa.También pueden producir efectos tóxicos directos, un síndrome intermedio y con me-nor frecuencia una polineuropatía tardía, que afecta fundamentalmente a nervios periféricos y que puede evolucionar de forma retrógrada y ascendente, afectando al sistema nervioso central. Presentamos el caso de un paciente de 72 años, que dos semanas después de fumigar con insecticidas organofosforados inició un cuadro progresivo de disestesias, déficit de fuerza de predominio en extremidades inferiores y progresivo trastorno de la marcha, que desembocó en una tetraparesia flácida. El electromiograma confirmó una polineuropatía mixta motora y sensitiva, compatible con neuropatía desmielinizante con componente axonopático sensitivo grave. No existe tratamiento farmacológico específico para la polineuropatía tardía.Tras el tratamiento sintomático de las complicaciones en fase aguda, únicamente el tratamiento rehabilitador puede tener utilidad a la hora de minimizar las secuelas funcionales (AU)

The most important poisoning mechanism of organophosphorus insecticides is the direct inhibition of acetylcholinesterase.This may also cause direct toxicity, an intermediate syndrome and less frequently delayed polyneuropathy, which mainly affects peripheral nerves and may progress in an ascending retrograde way, compromising the central nervous system.We present the case of a 72-year old man who at two weeks of fumigating with organophosphorus insecticides developed a progressive picture of paresthesias, dysesthesias, lower limb weakness and gait disorders that resulted in flaccid tetraparesia.The electromyography confirmed the presence of mixed sensorimotor polyneuropathy in the lower limbs consistent with demyelinating neuropathy and severe axonopathy component.There is no specific pharmacological treatment for delayed polyneuropathy and once the symptomatic treatment has been provided in the acute phase, only rehabilitation has proven to be effective in minimizing functional sequelae of these patients (AU)

Virtual reality hypnosis in the treatment of chronic neuropathic pain: a case report.

This case report evaluates virtual reality hypnosis (VRH) in treating chronic neuropathic pain in a patient with a 5-year history of failed treatments. The patient participated in a 6-month trial of VRH, and her pain ratings of intensity and unpleasantness dropped on average 36% and 33%, respectively, over the course of 33 sessions. In addition, she reported both no pain and a reduction of pain for an average of 3.86 and 12.21 hours, respectively, after treatment sessions throughout the course of the VRH treatment. These reductions and the duration of treatment effects following VRH treatment were superior to those following a trial of standard hypnosis (non-VR) treatment. However, the pain reductions with VRH did not persist over long periods of time. The findings support the potential of VRH treatment for helping individuals with refractory chronic pain conditions.

Selective motor imagery defect in patients with locked-in syndrome.

Recent studies indicate that motor imagery is subserved by activation of motor information. However, at present it is not clear whether the sparing of motor efferent pathways is necessary to perform a motor imagery task. To clarify this issue, we required patients with a selective, severe de-efferentation (locked-in syndrome, LIS) to mentally manipulate hands and three-dimensional objects. Compared with normal controls, LIS patients showed a profound impairment on a modified version of the hand-laterality task and a normal performance on mental rotation of abstract items. Moreover, LIS patients did not present visuomotor compatibility effects between anatomical side of hands and spatial location of stimuli on the computer screen. Such findings confirmed that the motor system is involved in mental simulation of action but not in mental manipulation of visual images. To explain LIS patients' inability in manipulating hand representations, we suggested that the pontine lesion, both determined a complete de-efferentation, and affected a component of the motor system, which is crucial for mental representation of body parts, probably the neural connections between parietal lobes and cerebellum.

Management of pathologic laughter and crying in patients with locked-in syndrome: a report of 4 cases.

Emotional lability and pathologic laughter and crying (PLC) have been frequently mentioned in patients with locked-in syndrome (LIS) without giving any detail about the clinical characteristics and possible consequences in terms of symptoms burden, functional impact, and recovery. In the present report, we describe our approach and management of 4 patients with LIS and PLC. PLC caused discomfort to the patients and hindered the different components of their rehabilitation program, limiting communication, the execution of swallowing testing and training, and the improvement of any residual motor function. PLC was unrelated to depression, did not ameliorate after pharmacologic treatment, and improved with cognitive-behavior treatment. Our findings suggest that, in LIS patients, laughter and crying alterations do not represent symptoms of a mood disorder but are the result of the same pontine lesion that causes LIS. In relation to the complex pathway regulating laughter and crying, we hypothesized that, in patients with LIS, PLC may be the result of a direct damage to the pontine center or of an alteration in the ponto-cerebellar pathway linking emotional behavior to contextual information. Presence of PLC in patients with LIS severely affects their intelligent adaptation to the environment. Direct explanation to the patients of the origin of PLC may be helpful as a cognitive-behavior treatment, with resulting benefits to the entire rehabilitation program.