Acute flaccid paralysis: Do not forget beriberi neuropathy.

We aimed to elucidate characteristics of beriberi neuropathy (BB) in a general hospital (GH) setting. Nerve conduction studies (NCS), cross-referenced with clinical records of patients admitted to a GH (May 2011-July 2017), were reviewed for diagnosis of BB. Thirteen patients (age range 23-64 years; five women) were diagnosed with BB. Eleven were incarcerated (2-24 months) at time of index event. Eleven reported prior, severe anorexia (2-6 months); five reported significant weight loss, three had recurrent vomiting, and three reported alcohol misuse. Commonest presentation was weakness (12/13); nine had symptom evolution over ≥3 weeks. At nadir, 11/13 could not walk independently. Other features included numbness/paraesthesiae (10/13), dysautonomia (6/13), vocal cord dysfunction/dysphagia (4/13), nystagmus (3/13). Pain was not prominent. Cerebrospinal fluid, tested in five patients, was acellular; one showed mildly increased protein. NCS showed predominantly sensorimotor, axonal polyneuropathy, rarely asymmetric. Only one patient had sural-sparing pattern. All received high dose thiamine. Two of the thirteen received intravenous immunoglobulin for suspicion of Guillain-Barré syndrome (GBS). Eleven improved to independent ambulation. One patient died from pulmonary embolism; one was lost to follow-up. Two of the thirteen had residual neurocognitive effects; both misused alcohol. Besides GBS, BB is an important cause of acute to subacute flaccid paralysis, especially in incarcerated patients and those with significant dietary deprivation. Features favoring BB over GBS are ≥3 weeks of symptoms, nystagmus, confusion, vocal cord dysfunction, volume overload, normal spinal fluid, elevated lactate, and absence of sural-sparing pattern in NCS.

The Contribution of Surface Electromyographic Assessment for Defining the Stage of Peripheral Facial Paralysis: Flaccid or Sequelae Stage

Abstract Introduction Surface electromyographic activity may not be symmetric, even in subjects with no facial paralysis history. Objective To evaluate the contribution of the index of electromyographic (IEMG) activity in the identification of the two extremes of the facial paralysis course. Methods Thirty-four subjects with unilateral peripheral facial paralysis were selected. A control group was composed of volunteers without a history of facial paralysis. The electromyographic assessment of the facial muscle was performed by placing surface electrodes during movements of the forehead, eyes and lips using MIOTEC equipment, such as the MIOTOOL (Miotec, Porto Alegre, Brazil) software. The electromyographic activity was also recorded in other channels during the primary activity to identify the presence of synkinesis. The statistical analysis was performed using the Statistical Package for Social Sciences for Macintosh (SPSS Inc, Chicago, IL, USA). The IEMG activity was obtained from the division of the electromyographic activity root mean square (RMS) values on both sides. Results There was a statistically significant difference among the groups in all the analyzed indexes. The ocular-oral synkinesis in all patients must be correctly identified (with 100% sensitivity and specificity) using an IEMG activity of 1.62 as a cutoff point. The oral-ocular synkinesis must be correctly identified (93.3% sensitivity and 95.9% specificity) using the IEMG activity of 1.79 as a cutoff point. Conclusion The IEMG activity is below the normal scores in patients in the flaccid stage, whereas patients in the sequelae stage can either show normal values or values above or below the normal scores. The IEMG activity was shown to have high sensitivity and specificity in the identification of synkinesis.

Ventromedial medulla inhibitory neuron inactivation induces REM sleep without atonia and REM sleep behavior disorder.

Despite decades of research, there is a persistent debate regarding the localization of GABA/glycine neurons responsible for hyperpolarizing somatic motoneurons during paradoxical (or REM) sleep (PS), resulting in the loss of muscle tone during this sleep state. Combining complementary neuroanatomical approaches in rats, we first show that these inhibitory neurons are localized within the ventromedial medulla (vmM) rather than within the spinal cord. We then demonstrate their functional role in PS expression through local injections of adeno-associated virus carrying specific short-hairpin RNA in order to chronically impair inhibitory neurotransmission from vmM. After such selective genetic inactivation, rats display PS without atonia associated with abnormal and violent motor activity, concomitant with a small reduction of daily PS quantity. These symptoms closely mimic human REM sleep behavior disorder (RBD), a prodromal parasomnia of synucleinopathies. Our findings demonstrate the crucial role of GABA/glycine inhibitory vmM neurons in muscle atonia during PS and highlight a candidate brain region that can be susceptible to α-synuclein-dependent degeneration in RBD patients.

Avaliação da força, desempenho e massa muscular de pacientes oncológicos e sua relação com parâmetros subjetivos e antropométricos / Evaluation of the strength, performance and muscle mass of cancer patients and its relationship with subjetive and anthropometric parameters

Introdução: o câncer e seu tratamento estão envolvidos na redução de massa muscular esquelética, força e desempenho físico, parâmetros que caracterizam a sarcopenia e que não estão inseridos na avaliação de rotina dos pacientes oncológicos. Objetivo: avaliar a massa, força e desempenho muscular de pacientes oncológicos e sua relação com métodos tradicionais de avaliação nutricional. Métodos: Incluídos pacientes com câncer, de ambos os sexos e idade entre 20 e 80 anos. A massa muscular foi estimada por equação preditiva e avaliada pelo índice de massa muscular, a força pelo pico de fluxo expiratório, e a capacidade funcional pelo teste Timed-Up-and-Go. Considerou-se sarcopenia quando massa muscular insuficiente associou-se à força e/ou desempenho físico insuficientes. Também foi avaliada a correlação da massa muscular, força e desempenho com os indicadores antropométricos e subjetivo. Resultados: Participaram 68 indivíduos, predominando o sexo feminino e o câncer de mama. Todas as mulheres apresentaram massa muscular adequada, porém 8 dos 12 homens apresentaram massa insuficiente e 91,6% deles apresentou pico de fluxo expiratório insuficiente. O desempenho foi insuficiente em 60,2% dos indivíduos. O índice de massa muscular se correlacionou com a circunferência do braço e da panturrilha, circunferência e área muscular do braço e com o índice de massa corporal. Conclusão: Os achados reforçam que os prejuízos à massa, força e desempenho físico devem ser considerados no paciente oncológico, sendo a antropometria uma aliada no diagnóstico da sarcopenia

Introduction: the cancer and its treatment are involved in loss of skeletal muscle, reduction in strength and muscular performance, parameters characterize sarcopenia, parameters that are not included in the routine evaluation of cancer patients. Objectives: to evaluate the muscle mass, strength and performance of cancer patients and its relationship with traditional methods of nutritional assessment. Methods: including cancer patients, of both sexes and age between 20 and 81 years. The muscle mass was estimated by a predictive equation and evaluated by muscle mass index, muscle strength assessed by peak expiratory flow, and functional capacity by the Timed-Up-and-Go test. Those with insufficient muscle mass associated with insufficient strength and/or physical performance were considered as sarcopenic. The possible correlation of the anthropometric and subjective indicators with the diagnostic parameters of sarcopenia was also evaluated. Results: 68 individuals participated, predominantly females and breast cancer. All of the women presented adequate muscle mass, but 8 of the 12 men had insufficient mass and 91.6% of them had insufficient peak expiratory flow. The performance was insufficient in 60,2% of the individuals. Muscle mass index correlated with arm and calf circumference, arm muscle circumference and area and body mass index. Conclusion: These findings reinforce that damage to the mass, strength and physical performance should be considered in the oncologic patient, and anthropometry may be an ally in the diagnosis of sarcopenia

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Can the Use of Neuromuscular Electrical Stimulation Be Improved to Optimize Quadriceps Strengthening?

CONTEXT: Neuromuscular electrical stimulation (NMES) is a common modality used to retrain muscles and improve muscular strength after injury or surgery, particularly for the quadriceps muscle. There are parameter adjustments that can be made to maximize the effectiveness of NMES. While NMES is often used in clinical practice, there are some limitations that clinicians should be aware of, including patient discomfort, muscle fatigue, and muscle damage. EVIDENCE ACQUISITION: PubMed was searched through August 2014 and all articles cross-referenced. STUDY DESIGN: Clinical review. LEVEL OF EVIDENCE: Level 3. RESULTS: Clinicians can optimize torque production and decrease discomfort by altering parameter selection (pulse duration, pulse frequency, duty cycle, and amplitude). Pulse duration of 400 to 600 µs and a pulse frequency of 30 to 50 Hz appear to be the most effective parameters to optimize torque output while minimizing discomfort, muscle fatigue, or muscle damage. Optimal electrode placement, conditioning programs, and stimulus pattern modulation during long-term NMES use may improve results. CONCLUSION: Torque production can be enhanced while decreasing patient discomfort and minimizing fatigue.

Sensory abnormalities in autism. A brief report.

Sensory abnormalities were assessed in a population-based group of 208 20-54-month-old children, diagnosed with autism spectrum disorder (ASD) and referred to a specialized habilitation centre for early intervention. The children were subgrouped based upon degree of autistic symptoms and cognitive level by a research team at the centre. Parents were interviewed systematically about any abnormal sensory reactions in the child. In the whole group, pain and hearing were the most commonly affected modalities. Children in the most typical autism subgroup (nuclear autism with no learning disability) had the highest number of affected modalities. The children who were classified in an "autistic features" subgroup had the lowest number of affected modalities. There were no group differences in number of affected sensory modalities between groups of different cognitive levels or level of expressive speech. The findings provide support for the notion that sensory abnormality is very common in young children with autism. This symptom has been proposed for inclusion among the diagnostic criteria for ASD in the upcoming DSM-V.

An investigation of the neurophysiologic effect of tone-reducing AFOs on reflex excitability in subjects with spasticity following stroke while standing.

Tone-reducing ankle-foot orthoses (TRAFOs) are said to improve the control and functioning of spastic lower limbs by their biomechanic and neurophysiologic effects. Unfortunately, there is limited evidence in literature to support the theory that TRAFOs can effectively decrease spasticity in the foot and ankle neurophysiologically. The primary purpose of this investigation was to determine the neurophysiologic effect of TRAFOs on soleus muscle reflex excitability in subjects with spasticity following stroke while standing. A repeated-measures intervention study was conducted on 15 adult subjects with stroke who were recruited from the community. Custom-made articulated ankle-foot orthoses (AFOs) and TRAFOs with orthokinetic compression garments (OCGs) were fabricated for each subject. Five conditions were tested: (1) Shoes only, (2) AFO, (3) TRAFO, (4) TRAFO with OCG, (5) shoes only, to determine if the TRAFOs were most effective in decreasing spasticity as assessed by the ratio of maximum Hoffmann reflex amplitude to maximum muscle response amplitude (Hmax:Mmax ratio) of the soleus. The results found that there were no significant treatment effects for the interventions (F = 0.992, df = 2.167, p = 0.388), however, when analysed subject-by-subject, four subjects displayed significant increases in their Hmax:Mmax ratios to at least one treatment condition. Overall, the results demonstrated that the tone-reducing devices had no significant neurophysiologic effect on soleus reflex excitability in subjects with spasticity, however individual responses showed that the TRAFOs increased spasticity in some individuals.

A consensus definition of cataplexy in mouse models of narcolepsy.

People with narcolepsy often have episodes of cataplexy, brief periods of muscle weakness triggered by strong emotions. Many researchers are now studying mouse models of narcolepsy, but definitions of cataplexy-like behavior in mice differ across labs. To establish a common language, the International Working Group on Rodent Models of Narcolepsy reviewed the literature on cataplexy in people with narcolepsy and in dog and mouse models of narcolepsy and then developed a consensus definition of murine cataplexy. The group concluded that murine cataplexy is an abrupt episode of nuchal atonia lasting at least 10 seconds. In addition, theta activity dominates the EEG during the episode, and video recordings document immobility. To distinguish a cataplexy episode from REM sleep after a brief awakening, at least 40 seconds of wakefulness must precede the episode. Bouts of cataplexy fitting this definition are common in mice with disrupted orexin/hypocretin signaling, but these events almost never occur in wild type mice. It remains unclear whether murine cataplexy is triggered by strong emotions or whether mice remain conscious during the episodes as in people with narcolepsy. This working definition provides helpful insights into murine cataplexy and should allow objective and accurate comparisons of cataplexy in future studies using mouse models of narcolepsy.

Sacral nerve stimulation as an option for the treatment of faecal incontinence in patients suffering from cauda equina syndrome.

STUDY DESIGN: Retrospective clinical study with 1-year follow-up. OBJECTIVES: Treatment of faecal incontinence by permanent sacral nerve stimulation (SNS) in patients suffering from cauda equina syndrome (CES). SETTING: Klosterneuburg, Austria. BACKGROUND AND PURPOSE: A flaccid paresis of the sphincter ani muscle and the pelvic floor combined with faecal incontinence can occur in patients suffering from CES as a result of a trauma in the region of the lumbar spine. If the incontinence cannot be managed by the use of laxatives or anal tampons, the patient is restricted in his/her quality of life. Thus, it was our aim to improve sphincter function and anal sensitivity to achieve voluntary rectal defaecation. MATERIALS AND METHODS: The functional integrity of at least one sacral root (S2-S4) was determined through percutaneous nerve evaluation (PNE). Following this analysis, a period of external temporary SNS was performed to evaluate the functional effect. If there was a decrease in the number of episodes of faecal incontinence during this evaluation period, a neurostimulation device (InterStim; Medtronic) was implanted. PATIENTS: A total of 11 patients suffering from flaccid paresis of the anal sphincter muscle and faecal incontinence caused by CES underwent PNE, which was successful in 8 patients. Two of these patients were eliminated from the procedure at the end of the temporary SNS period, one patient refused the permanent implantation. Therefore, five patients proceeded to permanent implantation, which led to an improved continence in all the cases. DISCUSSION: In the synopsis of the preoperative proctologic and neurological findings, successful electric stimulation of the sacral roots can be expected in incomplete CES. In the case of flaccid paresis of the anal sphincter muscles caused by an incomplete CES, permanent SNS offers a promising option for the treatment of faecal incontinence. .

[The value of intravesical electrostimulation in the treatment of acute prolonged bladder overdistension]. / Der Stellenwert der intravesikalen Elektrostimulation in der Therapie der akuten prolongierten Blasenüberdehnung.

BACKGROUND: The effectiveness of intravesical electrostimulation (IVES) in the treatment of acute prolonged bladder overdistension (PBO) was investigated. METHODS: Sixteen patients (female 11, male 5, ø 54 years) after PBO (bladder filling volume: 1317+/-320 ml) were evaluated: 11 after surgery and 5 after polytrauma, psychosomatic disorder or LV4 fracture. After exclusion of a neurogenic aetiology and a urodynamic examination, IVES was performed besides IC or suprapubic catheter. RESULTS: Group 1: six patients with a weak detrusor (p(detr. max.)<30 cmH(2)O); group 2: ten patients had detrusor acontractility. After 25 IVES sessions, group 1 showed a significant increase of p(detr. max.) (p=0.01) as well as a decrease in PVR (31% to 3% of bladder capacity, p=0.02). Group 2 had no significant increase of p(detr. max). CONCLUSIONS: Two-thirds of patients with a weak detrusor after PBO will regain balanced voiding after IVES due to detrusor reinforcement. With an acontractile detrusor only bladder sensation improves.

Lack of effect of acupuncture on electromyographic (EMG) activity--a randomised controlled trial in healthy volunteers.

BACKGROUND: Acupuncture is used clinically to treat muscle spasticity and flaccidity. Claims have been made that acupuncture can affect muscle EMG activity, though there is some doubt about the reliability of these studies. This study's aim was to examine the immediate effects of acupuncture on the EMG activity of the common wrist extensor muscles. METHODS: Thirty five right-handed healthy volunteers, 17 male and 18 female, aged 18-70 years were recruited from a convenience sample. Five subjects provided reliability data, and the remaining 30 took part in the main study. The study was a crossover, within-subject design, with the interventions counterbalanced. The three conditions were genuine acupuncture to LI4 (Hegu) and LI10 (Shousanli) for 20 minutes, with de qi; inappropriate acupuncture to PC3 (Quze) and PC6 (Neiguan) for 20 minutes; and 20 minutes of a no intervention control. All participants received each condition in random order. The outcome measure was surface EMG activity recorded over the common wrist extensor muscles during a 10 second, sub-maximal, isometric contraction. The average of three readings was used. The reliability of the outcome measurement was assessed in five volunteers selected at random who received repeated EMG recordings without acupuncture. RESULTS: The reliability tests showed the EMG procedure was highly reliable, with an intraclass correlation coefficient (ICC 2,1) of 0.9996 and a standard error of measurement of 0.014 mV. In the main study, paired t tests for the effect of the order of the first two interventions showed no detectable carry-over effect. Freidman analysis of variance found no difference between the three conditions (P=0.573). CONCLUSION: No meaningful change was detected in EMG activity following acupuncture interventions, but this study provides a sound protocol and normative values on which to plan future research.

[Potentialities of reflexotherapy in complex treatment of hypotonic states]. / Vozmozhnosti refleksoterapii v kompleksnom lechenii gipotonicheskikh sostoianii.

Methods of reflexotherapy in complex treatment of hypotonic state have significant advantage before traditional medicamentous therapy. Patients with hypotonic conditions which received traditional treatment are exposed to the greater risk in development of various forms cerebrovascular pathologies.

[Mixed hypotonia, neurological regression and atrophy of the cerebellum: manifestations that suggest infantile neuroaxonal dystrophy]. / Hipotonía mixta, regresión neurológica y atrofia cerebelosa: manifestaciones sugestivas de distrofia neuroaxonal infantil.

INTRODUCTION: Infantile neuroaxonal dystrophy (INAD), or Seitelberger disease, is a neurodegenerative disease of unknown origin which is transmitted by autosomal recessive inheritance. Clinically, it courses with psychomotor stagnation and regression that begins at the age of one or two years, associated to hypotonia with mixed clinical features (segmentary and suprasegmentary) that progresses towards spastic tetraplegia and progressive optic atrophy and dementia; this leads to death before the age of ten years. AIMS. To present the case of a 30 month old child with INAD, in whom a N acetylgalactosaminidase deficiency and mitochondrial cytopathy were ruled out. CASE REPORT: Male aged 30 months with an initial overall retardation, and later regression, of psychomotor acquisitions. In the physical exploration the patient displayed serious neurological involvement with mixed hypotonia, muscular hypotrophy with generalised weakness and mild bilateral horizontal nystagmus. Complementary explorations with neuroimaging revealed a slight increase in the subarachnoid space, with atrophy of the vermis and cerebellar hemispheres. Neurophysiological tests (EMG and ENG), which were initially normal, later showed signs of denervation in the EMG, and the ENG revealed a decreased amplitude of motor responses, with preservation of conduction speed. Histological tests showed the presence of axons with axoplasm expanded by the inclusion of typical tubulovascular structures. CONCLUSION: The clinical features of our patient met all the criteria to satisfy a diagnosis of INAD, and he displayed a classic form of the disease. INAD must be considered when the clinician is faced with: 1. A clinical picture of stagnation and later regression of psychomotor development before the age of two years; 2. Hypotonia, muscular atrophy and initial overall areflexia, with later progression towards pyramidalism; 3. Initially normal EMG findings, with later signs of denervation; 4. Cerebellar atrophy (hemispheres and vermis); 5. Visual deficit, and 6. Histopathological proof of characteristic findings.

Response of prolonged flaccid paralysis to FNS rehabilitation techniques.

PURPOSE: The purpose of this study was to investigate the response of muscles with prolonged flaccid paralysis (a year after stroke) to two types of treatment: (1) functional neuromuscular stimulation (FNS) with surface electrodes; and (2) FNS with intramuscular (IM) electrodes (FNS-IM). A second purpose was to compare FNS-gait versus volitional gait (no FNS activation). METHOD: We used a single case study design; our patient was age 72, with flaccid paralysis of knee flexors and ankle dorsiflexors. RESULTS: Following four months of treatment with surface-stimulation, there was no change in muscle function or gait. Following treatment with FNS-IM, the patient regained partial volitional control of knee flexors and dorsiflexors; untreated muscles did not change. CONCLUSION: FNS-gait provided more normal knee and ankle dorsiflexion during swing phase versus volitional gait swing phase (no FNS activation).

Functional electrical stimulation treatment of postradiculopathy associated muscle weakness.

Eighteen chronic pain patients (CPPs) with postradiculopathy-associated muscle weakness were entered into a nonrandomized functional electrical stimulation (FES) clinical treatment study to determine if FES can improve postradiculopathy associated muscle weakness. Because of the clinical nature of the unit, the CPPs could not be denied treatments other than FES. To control for these other treatments, the CPPs were used as their own controls with two control conditions, ie, where possible, the contralateral probable normal muscle was used as a control (control 1) and; in a subgroup of CPPs (n = 6), FES treatment was initially withheld to the probable weak muscle (control 2). Strength was measured as isometric maximum voluntary contraction (IMVC) and was used as the outcome treatment variable. IMVC was measured in both the probable weak and contralateral probable normal muscles at entrance into the Pain Center, at beginning of FES treatment, at completion of FES treatment, and for the control 2 condition at the end of the non-FES treatment period. Statistical analyses of the IMVC strength results using the two control conditions indicated (1) both the FES-treated and untreated muscles increased significantly in IMVC strength, (2) improvement in IMVC strength for FES-treated muscles was significantly greater than for probable normal FES-untreated muscles and, (3) improvement in IMVC strength in FES-treated muscles was significantly greater during the FES-treatment period than during the non-FES treatment period. FES treatment of postradiculopathy-associated muscle weakness in CPPs seems to increase the strength of the probable weak muscle above the increase in strength provided by other concurrent treatments.(ABSTRACT TRUNCATED AT 250 WORDS)

[Role of the respiratory muscles in weaning from mechanical ventilation]. / Rôle des muscles respiratoires dans le sevrage de la ventilation mécanique.

Weaning from mechanical ventilation is difficult when the respiratory muscles are unable, because of weakness or fatigue, to assume the entire work of breathing. Partial ventilatory support may then be used to rest the respiratory muscles. However, the degree of muscle rest achieved varies according to the ventilatory mode. Inspiratory muscle electromyographic activity is only moderately reduced with assisted ventilation (AMV) and synchronized intermittent ventilation (SIMV). On the other hand, inspiratory pressure support (IPS) is able to markedly relieve the inspiratory muscles. With noninvasive techniques, muscle rest is better achieved by positive pressure than by negative pressure ventilation. The opposite option, training of the respiratory muscles, has also been found to facilitate difficult weaning. The role of resting and overloading the respiratory muscles in the management of difficult weaning remains to be established.

Two theories of muscle strength augmentation using percutaneous electrical stimulation.

Electrical stimulation of muscle is a commonly used, well-substantiated strategy that physical therapists use to augment strength in patients with muscle weakness. Two distinctly different theories of strength augmentation using percutaneous muscle stimulation are presented. The first theory proposes that augmentation of muscle strength with electrically elicited muscle contractions occurs in a similar manner to augmentation of muscle strength with voluntary exercise. Electrically elicited muscle contractions of relatively high intensity with low numbers of repetitions strengthen muscle proportionally to the external load on the muscle in a manner that is equivalent to voluntary contraction. The second theory proposes that augmentation of muscle strength using percutaneous stimulation is fundamentally different from augmentation of strength with voluntary exercise. This theory uses the physiological differences between electrically elicited and voluntary contractions, such as the reversal of motor unit recruitment order, as a basis for argument. Both theories are partially substantiated using published literature. Strategies for testing both theories are also presented.