Hypoxaemia and interstitial lung disease in an infant with hypothyroidism and hypotonia.

A 7-month-old-term male infant presented with cough, tachypnoea, hypoxaemia and post-tussive emesis. Clinical history was significant for respiratory failure and pulmonary hypertension in the neonatal period requiring assisted ventilation, congenital hypothyroidism, mild hypotonia, recurrent respiratory infections, hypoxaemia requiring supplemental oxygen and nasogastric tube feeds. Physical examination showed tachypnoea, coarse bilateral breath sounds and mild hypotonia. Chest radiograph revealed multifocal pulmonary opacities with coarse interstitial markings and right upper lobe atelectasis. Following antibiotic therapy for suspected aspiration pneumonia, chest CT scan was performed and showed multiple areas of pulmonary consolidation and scattered areas of bilateral ground-glass opacities. Genetic studies showed a large deletion of chromosome 14q13.1-14q21.1, encompassing the NK2 homeobox 1 (NKX2-1) gene consistent with a diagnosis of brain-thyroid-lung (BTL) syndrome. Our case highlights the importance of genetic studies to diagnose BTL syndrome in infants with hypothyroidism, hypotonia and lung disease.

[Professor NI Guang-xia's clinical experience in treatment of wei syndrome with xingshen tongyang needling technique of acupuncture].

According to the effect of the spirit and yang qi in the treatment of wei syndrome and the theory as "acupoints of yangming meridians especially for treatment of wei syndrome", professor NI Guang-xia proposes the treatment principle of xingshen tongyang (regaining consciousness/spirit and promoting yang). Guided by this treatment principle, the theory of traditional meridians and acupoints is coordinated with the achievement of modern medicine research. Consequently, xingshen tongyang needling technique of acupuncture is suggested. In the treatment of wei syndrome, the predominated prescriptions are formed for regaining consciousness/spirit, i.e. Baihui (GV 20), Sishencong (EX-HN 1), Fengfu (GV 16) and Fengchi (GB 20) and for promoting yang circulation, i.e. Jianyu (LI 15), Quchi (LI 11), Hegu (LI 4) and Zusanli (ST 36). The acupoint prescriptions are modified align with the individual cases. This treatment regimen effectively relieves the symptoms and delayed the progress of disease.

Efeito da fisioterapia aquática na força muscular respiratória de crianças e adolescentes com Síndrome de Down / Effect of aquatic physioterapy on the respiratory muscle strength of children and teenagers with Down Syndrome

A força muscular respiratória em crianças e adolescentes com Síndrome de Down é comprometida pela hipotonia generalizada que os acometem. Analisar os efeitos da fisioterapia aquática na força muscular respiratória em crianças e adolescentes com síndrome de Down. Estudo de intervenção, quasi-experimental, com amostra constituída de oito crianças e adolescentes diagnosticados com SD e média de idade de 12 anos (± 3,8). Foram realizadas 10 sessões de fisioterapia aquática, com 50 minutos de duração cada, em piscina com água aquecida. A força muscular respiratória foi avaliada a partir da pressão inspiratória máxima (PImáx) e pressão expiratória máxima (PEmáx) com auxílio do manuvacuômetro, sendo obtido seus valores antes do primeiro atendimento e após o último. Analisou-se ainda a saturação periférica de oxigênio e frequência cardíaca. Para comparação das médias antes e depois da intervenção foi utilizado o Teste T pareado. Amostra de indivíduos predominantemente do sexo feminino (75,0%), pardos (75,0%) e residentes em zona urbana (87,5%). A comparação da PImáx e PEmáx antes e após as 10 sessões de fisioterapia aquática evidenciou melhora da força muscular inspiratória e expiratória, sendo tais diferenças estatisticamente significantes (valor de p<0,01). Também foram notadas melhorias na frequência cardíaca e saturação de oxigênio (valor de p<0,05) com a intervenção. Destaca-se neste estudo que a fisioterapia aquática parece ser um recurso terapêutico eficiente para o fortalecimento da musculatura respiratória e melhora dos sinais vitais de crianças e adolescentes de com diagnóstico de Síndrome de Down.

Respiratory muscle strength in children and adolescents with Down syndrome is compromised by the generalized hypotonia that affects them. This study aims to analyze the effects of aquatic physical therapy on respiratory muscle strength in children and adolescents with Down syndrome. Material and method: A quasi-experimental study with a sample consisting of eight children and adolescents diagnosed with DS and mean age of 12 years (± 3.8). Ten sessions of aquatic physiotherapy were performed, each with a duration of 50 minutes, in a pool with heated water. Respiratory muscle strength was assessed from maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) using a manuvacuometer, and its values were obtained before the first session and after the last one. Peripheral oxygen saturation and heart rate were also analyzed. The paired T-test was used to compare the means before and after the intervention. Sample of predominantly female (75.0%), brown (75.0%) and urban residents (87.5%). The comparison of MIP and MEP before and after the 10 sessions of aquatic physiotherapy showed an improvement in inspiratory and expiratory muscle strength, and these differences were statistically significant (p <0.01). Improvements in heart rate and oxygen saturation (p value <0.05) were also noted with the intervention. In this study, aquatic physiotherapy seems to be an efficient therapeutic resource for the strengthening of respiratory muscles and improvement of the vital signs of children and adolescents diagnosed with Down's Syndrome.

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.

[Post-stroke flaccid limb dysfunction treated with the comprehensive therapy of acupuncture, Chinese herbal medicine and rehabilitation: muti-center randomized controlled trial].

OBJECTIVE: To evaluate the clinical value of the comprehensive therapy of acupuncture, Chinese herbal medicine and rehabilitation in the treatment of post-stroke flaccid limb dysfunction. METHODS: The four-center, single-blind, randomized and controlled research method was adopted, 240 qualified subjects were randomized into a comprehensive therapy group, an acupuncture group, a rehabilitation group and a Chinese herbal medicine group, 60 cases in each one, at the ratio of 1 1. In the comprehensive therapy group, the comprehensive therapy of acupuncture, Chinese herbal medicine and rehabilitation was applied. The acupuncture therapy included the scale acupuncture at middle line of vertex, lateral line 1 of vertex, lateral line 2 of vertex, etc. with the single reinforcing and reducing technique by the speed of needle insertion and withdrawal, and the body acupuncture therapy at the acupoints on the antagonistic muscles with the reinforcing and reducing technique by the needle rotation. The Chinese herbal medicine therapy included No. 1 stroke formula for the cases of liver and kidney yin deficiency and the upward disturbance of wind yang, No. 2 stroke formula for qi deficiency and blood stagnation, and the stagnation in meridians and No. 3 stroke formula for the interaction of phlegm and stasis and blockage of meridians according to the pattern/syndrome differentiation. The rehabilitation therapy focused on the promotion technique by putting the healthy limb. The simple acupuncture, rehabilitation and Chinese herbal medicine therapies as the comprehensive therapy group were applied in the acupuncture group, rehabilitation group and Chinese herbal medicine group separately. The Chinese medicine symptom, the limb motor function, the daily life activity, fainting needle reaction, allergic reaction and the others were taken as indices to evaluate the efficacy and safety of the treatment. RESULTS: (1) The results of the four indices named the Chinese medicine symptom, the limb motor function, the limb balance function, the daily life activity were all improved significantly after treatment as compared with those before treatment in four groups (all P < 0.01). (2) Concerning to the improvement degrees, the improvements of the above four indices in the comprehensive therapy group were more significant than those in the other three groups (P < 0.01, P < 0.05). The improvement in Chinese medicine symptom in the acupuncture group and the Chinese herbal medicine group were more significant than that in the rehabilitation group (both P < 0.05). The improvement of the upper limb motor function in the acupuncture group was more significant than that in the rehabilitation group and the Chinese herbal medicine group separately (both P < 0.05). CONCLUSION: The comprehensive therapeutic program of acupuncture, Chinese herbal medicine and rehabilitation is safe and effective in the treatment of post-stroke flaccid limb dysfunction. It is more advantageous in efficacy as compared with any simple therapy.

Does physical therapy improve outcome in infants with joint hypermobility and benign hypotonia?

This study examined the effect of the frequency of physical therapy on the outcome of infants referred for delayed motor development due to joint hypermobility and benign hypotonia. The study groups comprised 29 infants (8-12 months) who were randomly placed into a monthly and weekly treatment groups. No difference was found between the 2 study group scores on the different tests at all assessment points. However, assessment of walking at the age of 15 months revealed a clear advantage of the infants who were treated weekly. Our study demonstrated a minor benefit of weekly treatment protocol only in the achievement of independent walking in children with joint hypermobility and benign hypotonia. It did not prove an advantage of weekly physical therapy. It seems that monthly physical therapy combined with a home treatment protocol implemented by the primary caregivers is sufficient to achieve motor catch-up.

[Study on treatment of flaccidity from the Governor Vessel].

The relationship of affected regions, cause of disease, pathogenesis of flaccidity with physiological functions of the Governor Vessel is expounded, indicating that "skin, muscle, tendon, vessel and joint" are basic structures of performing motor function of the organism, which depend on nourishing of qi, blood, body fluid, essence and marrow; "insufficiency of essence and marrow" and "deficiency of qi and blood" are keys of pathogenensis of flaccidity; "deficiency or obstruction of the Governor Vessel" and "yang of the Governor Vessel unable to function" are the basis of channels and collaterals for flaccidity attack. Clinically, regulation of the Governor Vessel can promote repair of morphosis and functions of the Governor Vessel (brain and spinal cord), and rebuilding of the regulative system of the brain-the Governor Vessel (spinal cord)-viscera-channels, qi and blood functional activities, so as to restore the normal distribution and transduction of qi, blood, body liquid, essence and marrow to nourish skin, muscle, tendon, vessel and joint.

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. .

[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.

Long term effects of the palatal plate therapy for the orofacial regulation in children with Down syndrome.

The objective of this study was to evaluate long term effects of orofacial regulation therapy with modified Castillo-Morales palatal plate on 68 Down children that attended our Unit from 1992 to 2001. Corrections obtained with palatal plate therapy were evaluated according to the following parameters: spontaneous lingual protrusion based on three level scale, position "open mouth", labial hypotonia and sialorrhea. The results showed distinct improvement in nearly all of the parameters compared to initial conditions.

Use of functional electrical stimulation in the lower extremities of incomplete spinal cord injured patients.

After a program of therapeutic electrical stimulation, 3 groups of incomplete spinal cord injured (SCI) patients were identified, those in whom an improvement of both voluntary and stimulated muscle force was observed, those with an increase in stimulation response only, and patients in whom no effect of electrical stimulation training could be recorded. As it is difficult to predict the outcome of the electrical stimulation rehabilitation process, a diagnostic procedure was developed to predict soon after accidents which incomplete SCI patients are candidates for permanent use of a functional electrical stimulation (FES) orthotic aid. The candidates for chronic use of FES are patients with weak ankle dorsiflexors and sufficiently strong knee extensors. These patients are equipped with a single channel peroneal stimulator augmenting dorsiflexion and knee and hip flexion in a total lower limb flexion response. By applying FES to the ankle plantar flexors, the swing phase of walking can be significantly shortened and faster walking obtained.

The effects of functional electrical stimulation on shoulder subluxation, arm function recovery, and shoulder pain in hemiplegic stroke patients.

The purpose of this study was to evaluate the effectiveness of a functional electrical stimulation (FES) treatment program designed to prevent glenohumeral joint stretching and subsequent subluxation and shoulder pain in stroke patients. Twenty-six recent hemiplegic stroke patients with shoulder muscle flaccidity were randomly assigned to either a control group (n = 13; 5 female, and 8 male) or experimental group (n = 13; 6 female, and 7 male). Both groups received conventional physical therapy. The experimental group received additional FES therapy where two flaccid/paralyzed shoulder muscles (supraspinatus and posterior deltoid) were induced to contract repetitively up to 6 hours a day for 6 weeks. Duration of both the FES session and muscle contraction/relaxation ratio were progressively increased as performance improved. The experimental group showed significant improvements in arm function, electromyographic activity of the posterior deltoid, range of motion, and reduction in subluxation (as indicated by x-ray) compared with the control group. We concluded that the FES program was effective in reducing the severity of shoulder subluxation and pain, and possibly facilitating recovery of arm function.

Effects of long-impulse electrical stimulation on atrophy and fibre type composition of chronically denervated fast rabbit muscle.

The efficacy of electrical stimulation on a chronically denervated muscle depends on stimulus parameters, which have an important influence on the development of atrophy. Stimulus frequency and/or total activity are particularly responsible for the development of some histological, biochemical and contractile features. The present study in 18 rabbits deals with a recently developed electrical stimulus, which had proved effective in maintaining muscle force following denervation. This current has (1) unusual long bidirectional rectangular impulses (20 ms) and (2) a frequency of 25 Hz, which is between the frequencies of fast- and slow-firing motor units. Electrical stimulation began 28 (in one animal 53) days after total motor and sensory denervation of the right hindleg, and was continued until the end of the experiment, up to 205 days. To mimic a therapeutic regimen, which should be agreeable to patients, daily treatment times were kept to a minimum (2 x 6 min), and surface electrodes were used. Morphometric evaluation of the fast flexor digitorum sublimis muscle showed that such electrical stimulation was able to preserve fibre diameter at a level of 72-86% of the initial values for several months, while unstimulated fibres showed the usual atrophy with a decrease of diameters below 40% of normal. The stimulation induced a "hybrid" fibre type with properties of a slow muscle (rich in mitochondria in NADH-dependent tetrazolium reductase staining and electron microscopy) as well as of a fast-twitch muscle (fibre type IIb in myofibrillar ATPase stainings).

Carnitine and weakness in haemodialysis patients.

Weakness in haemodialysis patients has been attributed to several factors including carnitine deficiency. Malnutrition, neuropathy, uraemic myopathy and parathyroid hormone excess may all be important. Six haemodialysis patients were shown to have reduced muscle power compared with a normal population, and to be malnourished by dietary assessment, and features of their weakness were investigated. Total carnitine was normal in plasma but elevated in muscle, with an excess of esterified carnitine in both plasma and muscle and diminished free plasma carnitine. Muscle biopsy showed no features of carnitine deficiency and electromyography showed a non-specific neuropathy with additional myopathic changes in some. Dietary supplementation with L-carnitine (2 g/day) for 6 weeks in a placebo-controlled trial showed a redistribution of carnitine fractions but no subjective or objective improvement in muscle function. There was no improvement in the plasma lipid profile. The weakness of haemodialysis patients is multifactorial. We have not demonstrated total carnitine depletion in either muscle or plasma, and oral supplementation of L-carnitine has no demonstrable effect in this group.

Effects of electrical muscle stimulation combined with voluntary contractions after knee ligament surgery.

The aim of the present study is to compare the effect of electrical muscle stimulation combined with voluntary muscle contractions with a program only with voluntary muscle contractions during immobilization in casts after anterior cruciate ligament surgery. Twenty-three patients, 7 women and 16 men with a mean age of 28 yr, were randomized into two groups: an experimental group (13 patients) and a control group (10 patients). Post-operatively, the patients were immobilized for 3 wk in a full leg cast with the knee flexed at an angle of 20 degrees to 30 degrees and then in a knee cast for another 3 wk. All patients had a standard program with quadriceps muscle contractions. In addition, the experimental group received electrical stimulation of the quadriceps muscle 4 X 10 min, 3 times.wk-1, at a frequency of 30 Hz. During each stimulation, the patients were requested to contract the quadriceps muscle voluntarily as well. When pre-operative measurements were compared with those at the end of the immobilization period (6 wk after the operation), a significantly larger reduction in the knee extension isometric muscle strength was found for the control group than for the experimental group. In comparisons of the data of the male subjects only, this difference was still seen to be significant. The cross-sectional area of the quadriceps muscle measured with computed tomography was significantly less reduced during the immobilization period in the experimental group than in the control group.(ABSTRACT TRUNCATED AT 250 WORDS)

[Principles of electric stimulation in flaccid paralysis]. / Zasdy elektrostymulacji w porazeniach wiotkich.

Electrostimulation must be selective and sparing. Selectivity is obtained establishing which denervated muscles are responsible for loss of a given function, choosing a proper size of the stimulating electrode and its point of application, and testing the muscle bioelectrically. Sparing electrostimulation includes the application of current of appropriate parameters, that is: impulse duration, current intensity, frequency of impulses and duration of procedure. The results obtained by this method assessed according the Lovett's scoring system were very good and good in moderately advanced paresis, but unsatisfactory in severe paralysis.