[Reconstruction of Lower Extremity Palsy after Pelvic Fractures with the Muscle Transfers]. / Rekonstrukce paréz dolní koncetiny po zlomeninách pánve svalovými transfery.

PURPOSE OF THE STUDY The prevalence of nerve structure injuries accompanying pelvic and acetabular fractures is stated to be 5-25 %, with most frequent injuries to motor nerve structures associated with fractures of the posterior wall of the acetabulum. Prognostically worse outcomes of regeneration are documented mainly in iatrogenic, intraoperative injuries to nerve structures. This study aims to document the functional effect of muscle transfers restoring the movement of lower extremities with irreversible nerve lesion caused by the pelvic and acetabular fracture. MATERIAL AND METHODS A total of 18 patients with irreversible palsy of lower extremities in L4-S1 segments underwent a reconstruction surgery in the period 2006-2016, of whom 13 patients with the mean age of 42 (21-79) years arrived for a follow-up. The group included 10 patients with the loss of function of peroneal portion of the sciatic nerve, one patient sustained femoral nerve lesion and two patients suffered complete sciatic nerve lesion (both the peroneal and tibial portion). The patients were evaluated at the average follow-up of 77 (24-129) months after the reconstruction surgery. The average time interval from pelvic fracture to reconstruction by muscle transfer was 47 (18-151) months. Due to a wide spectrum of functional damage, the patients were evaluated in terms of the overall effect of the reconstruction surgery on the activities of daily living using the LEFS (The Lower Extremity Functional Scale). The surgical techniques used transposition of tensor fascie latae for femoral nerve lesion, transposition of tibialis posteriormuscle for palsy of the peroneal division of the sciatic nerve and tenodesis of tibialis anterior tendon and peroneus longustendon for the palsy of the peroneal and tibial portion of sciatic nerve. RESULTS The effect of movement restoration on daily living evaluated using the LEFS achieved 65 points (53-79) which is 85% of the average value of LEFS in healthy population. The transposition of active muscles tibialis posterior and tensor fasciae latae resulted in all the patients in active movement restoration. A loss of correction of foot position following the performed tenodesis of the paralysed tibialis anterior muscle was observed in one patient, with no significant impact on function. No infection complication was reported in the group. In 78% of patients the intervention was performed as day surgery. DISCUSSION There is a better prognosis for restoration in incomplete nerve lesion than in complete lesions and also in the loss of sensation than in the loss of motor function. The mini-invasive stabilisation of pelvic ring according to literature does not increase the risk of nerve lesions, while on the other hand a higher incidence of femoral nerve damage by INFIX fixator is documented. The type of muscle transfer is selected based on the availability of active muscles suitable for transposition and also with respect to functional requirements of the patient. CONCLUSIONS Irreversible palsy of lower extremity after the pelvic fracture is easily manageable as to the restoration of function. Surgical interventions using the preserved active muscles to restore the lost movement should be a component part of comprehensive surgical care for patients who sustained a pelvic fracture and should be performed centrally at a centre availing of comprehensive expertise. Key words: nerve lesion, tendon transfer, acetabulum, pelvis, fracture.

Age delays the recovery of distal motor latency after carpal tunnel syndrome surgery.

BACKGROUND: Studies have shown slow healing of peripheral nerve injury in elderly patients. Carpal tunnel syndrome (CTS) is the most frequent compressive mononeuropathy, affecting mostly older people and females. Few studies have assessed electrophysiological differences between younger and older patients. We aimed to evaluate age-dependent differences in electrophysiological parameters preoperatively and postoperatively over a 100-day postoperative period. METHOD: This retrospective study included 258 hands of patients who underwent conventional open-technique carpal tunnel syndrome surgery. Patients with paresthesia in the median nerve distribution or with impaired sensation or abnormal findings in sensory and motor median nerve conduction studies were enrolled. The age dependence of the preoperative values of distal motor latency, amplitude of the compound motor action potential and sensory conduction velocity was estimated using regression analysis. RESULTS: Statistically significant age dependence was found for the preoperative distal motor latency, compound motor action potential, amplitude and sensory conduction velocity. The repair of segmental demyelination was nearly twice as slow in the older group, at a 5 % significance level, even when comparing groups with the same preoperative distal motor latency. CONCLUSIONS: Analysis of preoperative nerve conduction parameters indicates that surgery for carpal tunnel syndrome is performed later in older patients.

Malignant schwannoma of the obturator nerve.

Lesions of obturator nerve are rare. Tumours and mainly malignant schwannoma of this nerve are extremely rare. The authors describe an unusual case of a gigantic schwannoma of the obturator nerve in 69 year old woman. Due to tumour expansion in the proximal part of the thigh MRI was performed and demonstrated extensive tumour originating most probably from the obturator nerve. The patient had no neurological symptoms. Biopsy from the lesion was taken at the Department of Orthopaedics with the following conclusion: malignant schwannoma. The patient received neoadjuvant chemotherapy due to diffuse metastatic spread on the chest X ray, after which metastatic spread subsided. The main lesion reduced its size by 1 cm. In 4 months after biopsy the patient was referred for operation to neurosurgery. The tumour was removed along its borders and except of minimal weakness of adduction of the right thigh there was no neurological deterioration. She was subsequently referred for further care to oncology and radiotherapy.The goal of this work is to emphasize the extremely rare occurrence of tumours of this nerve and suggest therapeutic options (Fig. 4, Ref. 11).

Mild traumatic brain injury.

Traumatic Brain Injury (TBI) is among the most frequent neurological disorders. Of all TBIs 90% are considered mild with an annual incidence of 100­300/100.000. Intracranial complications of Mild Traumatic Brain Injury (MTBI) are infrequent (10%), requiring neurosurgical intervention in a minority of cases (1%), but potentially life-threatening (case fatality rate 0,1%). Hence, a true health management problem exists because of the need to exclude the small chance of a life threatening complication in large numbers of individual patients. The 2002 EFNS guidelines used a best evidence approach based on the literature until 2001 to guide initial management with respect to indications for CT, hospital admission, observation and follow up of MTBI patients. This updated EFNS guideline version for initial management inMTBI proposes a more selectively strategy for CT when major (dangerous mechanism, GCS<15, 2 points deterioration on the GCS, clinical signs of (basal) skull fracture, vomiting, anticoagulation therapy, post traumatic seizure) or minor (age, loss of consciousness, persistent anterograde amnesia, focal deficit, skull contusion, deterioration on the GCS) risk factors are present based on published decision rules with a high level of evidence. In addition clinical decision rules for CT now exist for children as well. Since 2001 recommendations, although with a lower level of evidence, have been published for clinical in hospital observation to prevent and treat other potential threads to the patient including behavioral disturbances (amnesia, confusion and agitation) and infection.

[Peripheral nerve injury during laparoscopic procedures]. / Poranení periferních nervu pri laparoskopických operacích.

Laparoscopic approach predominates in almost all surgical procedures. In comparison with classical surgery the laparoscopic procedures are more considerable for most tissues. Nevertheless, after learning of laparoscopic surgical techniques, iatrogenic peripheral nerve lesions still occure. The authors present a review of the most frequent laparoscopic operations and occurrence of peripheral nerve injuries in particular procedures. They try to point to the situations leading to iatrogenic lesions, to identify different causes of nerve damage and to deduce necessary preventive measures.

[Lesions of peripheral nerves in obstetrics and gynecology. A review]. / Léze periferních nervu v porodnictví a gynekologii. Prehled.

Peripheral nerves in obstetrics and gynecology are damaged with moderate frequency. Mostly there are iatrogenic lesions, that most often develop during delivery, operations and also in course of smaller invasive procedures. In genitofemoral nerve lesions neuralgic pain in groin and vulva is the most striking symptom, in cutaneous femoris lateralis nerve damage there are unpleasant paresthesias of lateral thigh. In case of femoral or obturator nerve lesion a weakness of thigh muscles with difficulties of standing and walking is the most typical. In pudendal nerve damage the autonomic disturbances (incontinence of feaces and urine) and pain in perineum are in the foreground of the patient's complains. After a knowledgeable history taking and detailed clinical examination the next step of diagnostics are neurophysiological investigations (neurography and needle electomyography). In special cases there is racional to use imaging methods (ultrasonography, MRI, CT). In majority of these iatrogennic nerve lesions the conservative approach is successful (rehabilitation, medicaments against neuropathic pain) and only in a small proportion of patients the neurosurgeon consultation is necessary.

[Restoration of upper-limb function in patients with cervical spinal cord lesion]. / Obnova pohybu horní koncetiny u pacientu s vysokou mísní lézí.

PURPOSE OF THE STUDY: Injury to the spinal chord at the C5 level results in a severe handicap to the patient because of a loss of active motion of the wrist and hand. Selected surgical procedures can effectively restore active extension of the wrist and provide conditions for some vital pinching and gripping abilities. The deltoid muscle also has a potential for restoration of upper limb function, because its posterior portion can be used to restore elbow extension. The aim of this clinical retrospective study was to describe and evaluate the techniques used to improve the quality of life in patients with spinal chord lesions at the C5 level. MATERIAL AND METHODS: The group studied consisted of 15 patients (3 women and 12 men; average age, 34 years) with a spinal chord lesion at the C5 segment who had zero wrist extension or in whom wrist extensor muscle force was equal to the third degree of muscle strength or less. The procedures to restore active extension of the wrist involved the radiobrachialis muscle, the reconstruction of elbow extension was activated by the posterior portion of the deltoid muscle, and a fixed supination posture of the forearm was corrected by elongating the distal tendon of the biceps muscle which was derotated and attached to the proximal radius. The interval between injury and reconstructive surgery was 36 months on the average. RESULTS: The reconstructive surgery described resulted in restoration of hand and wrist function with sufficient muscle strength in all patients, which had a favourable effect on many aspects of their daily lives. The average values of muscle strength were 4.1° (3° to 5°). for wrist extension and 3.3° (2° to 4°) for elbow extension. Key-pinch force was on average 1.2 kg (0.2 to 2.1). A failure of thumb carpometacarpal arthrodesis was the most frequent complication and occurred in two patients. On clinical evaluation, all patients reported satisfaction with the restored function of their upper extremities and expressed their willingness to undergo the same procedure again. DISCUSSION: Reconstructive surgery of the upper extremity in tetraplegic patients has been performed with success in several countries, namely USA and Sweden, for many years. Since 2003 the results in the Czech Republic have also shown positive effects of reconstructive procedures on better quality of life in patients with cervical spinal chord injury at the lower levels, in whom the goal is to restore vital pinching and gripping abilities. A good outcome of surgical treatment requires good quality post-operative care provided by a team of physical therapists fully informed of the reconstructive procedures involved. CONCLUSIONS: A restored function of the upper extremity in the patients with spinal chord injury at the C level plays a decisive role in the whole spectrum of the patient's daily activities, such as dressing, communication, etc., and thus improves their self- care. The restoration of active elbow extension has a positive effect on the patient's mobility.

Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly.

Vertebrate-striated muscle is assumed to owe its remarkable order to the molecular ruler functions of the giant modular signaling proteins, titin and nebulin. It was believed that these two proteins represented unique results of protein evolution in vertebrate muscle. In this paper we report the identification of a third giant protein from vertebrate muscle, obscurin, encoded on chromosome 1q42. Obscurin is approximately 800 kD and is expressed specifically in skeletal and cardiac muscle. The complete cDNA sequence of obscurin reveals a modular architecture, consisting of >67 intracellular immunoglobulin (Ig)- or fibronectin-3-like domains with multiple splice variants. A large region of obscurin shows a modular architecture of tandem Ig domains reminiscent of the elastic region of titin. The COOH-terminal region of obscurin interacts via two specific Ig-like domains with the NH(2)-terminal Z-disk region of titin. Both proteins coassemble during myofibrillogenesis. During the progression of myofibrillogenesis, all obscurin epitopes become detectable at the M band. The presence of a calmodulin-binding IQ motif, and a Rho guanine nucleotide exchange factor domain in the COOH-terminal region suggest that obscurin is involved in Ca(2+)/calmodulin, as well as G protein-coupled signal transduction in the sarcomere.

DRAL is a p53-responsive gene whose four and a half LIM domain protein product induces apoptosis.

DRAL is a four and a half LIM domain protein identified because of its differential expression between normal human myoblasts and the malignant counterparts, rhabdomyosarcoma cells. In the current study, we demonstrate that transcription of the DRAL gene can be stimulated by p53, since transient expression of functional p53 in rhabdomyosarcoma cells as well as stimulation of endogenous p53 by ionizing radiation in wild-type cells enhances DRAL mRNA levels. In support of these observations, five potential p53 target sites could be identified in the promoter region of the human DRAL gene. To obtain insight into the possible functions of DRAL, ectopic expression experiments were performed. Interestingly, DRAL expression efficiently triggered apoptosis in three cell lines of different origin to the extent that no cells could be generated that stably overexpressed this protein. However, transient transfection experiments as well as immunofluorescence staining of the endogenous protein allowed for the localization of DRAL in different cellular compartments, namely cytoplasm, nucleus, focal contacts, as well as Z-discs and to a lesser extent the M-bands in cardiac myofibrils. These data suggest that downregulation of DRAL might be involved in tumor development. Furthermore, DRAL expression might be important for heart function.

Alterations at the intercalated disk associated with the absence of muscle LIM protein.

In this study, we investigated cardiomyocyte cytoarchitecture in a mouse model for dilated cardiomyopathy (DCM), the muscle LIM protein (MLP) knockout mouse and substantiated several observations in a second DCM model, the tropomodulin-overexpressing transgenic (TOT) mouse. Freshly isolated cardiomyocytes from both strains are characterized by a more irregular shape compared with wild-type cells. Alterations are observed at the intercalated disks, the specialized areas of mechanical coupling between cardiomyocytes, whereas the subcellular organization of contractile proteins in the sarcomeres of MLP knockout mice appears unchanged. Distinct parts of the intercalated disks are affected differently. Components from the adherens junctions are upregulated, desmosomal proteins are unchanged, and gap junction proteins are downregulated. In addition, the expression of N-RAP, a LIM domain- containing protein located at the intercalated disks, is upregulated in MLP knockout as well as in TOT mice. Detailed analysis of intercalated disk composition during postnatal development reveals that an upregulation of N-RAP expression might serve as an early marker for the development of DCM. Altered expression levels of cytoskeletal proteins (either the lack of MLP or an increased expression of tropomodulin) apparently lead to impaired function of the myofibrillar apparatus and to physiological stress that ultimately results in DCM and is accompanied by an altered appearance and composition of the intercalated disks.

[Compressive syndromes of the suprascapular nerve--experience with surgery]. / Kompresivní syndromy n. suprascapularis--zkusenosti s chirurgickou lécbou.

Compression of suprascapular nerve at the suprascapular notch or spinoglenoid notch can be a cause of a rare mononeuropathy. Patients complain of pain in scapula and shoulder, and selective wasting and weakness of the supraspinatus and infraspinatus muscles. Tenderness at the suprascapular notch and pain by forced abduction of arm are typical and useful signs. Electromyographic signs of various grade denervation are regularly found in both muscles. Development of entrapment syndromes is associated with chronic overload, shoulder injuries, with surgical procedures, or with shoulder-arm fixation. The authors present their own group of 10 patients, who were operated on for entrapment syndrome of suprascapular nerve during 7 year period. Direct or indirect traumatic mechanisms of development of nerve compression were disclosed in most patients. During postoperative period there was not only substantial decrease of pain, but also regression of weakness and wasting of both muscles. In discussion, necessity of differential diagnostic procedures in case of pain and muscle wasting, use of appropriate imaging techniques, demand of purposeful EMG examination, and postoperative follow-ups are emphasized.

Correction to: The Sydney Heart Bank: improving translational research while eliminating or reducing the use of animal models of human heart disease.

In the original version of this article, the name of one of the authors is not correct. The correct name should be W. A. Linke, which is shown correctly in the authorgroup section above.

Different domains of the M-band protein myomesin are involved in myosin binding and M-band targeting.

Myomesin is a 185-kDa protein located in the M-band of striated muscle where it interacts with myosin and titin, possibly connecting thick filaments with the third filament system. By using expression of epitope-tagged myomesin fragments in cultured cardiomyocytes and biochemical binding assays, we could demonstrate that the M-band targeting activity and the myosin-binding site are located in different domains of the molecule. An N-terminal immunoglobulin-like domain is sufficient for targeting to the M-band, but solid-phase overlay assays between individual N-terminal domains and the thick filament protein myosin revealed that the unique head domain contains the myosin-binding site. When expressed in cardiomyocytes, the head domains of rat and chicken myomesin showed species-specific differences in their incorporation pattern. The head domain of rat myomesin localized to a central area within the A-band, whereas the head domain of chicken myomesin was diffusely distributed in the cytoplasm. We therefore conclude that the head domain of myomesin binds to myosin but that this affinity is not sufficient for the restriction of the domain to the M-band in vivo. Instead, the neighboring immunoglobulin-like domain is essential for the precise incorporation of myomesin into the M-band, possibly because of interaction with a yet unknown protein of the sarcomere.

[The usefulness of carpal tunnel pressure measurement during endoscopic surgery of carpal tunnel syndrome]. / Vyuzití merení tlaku v karpálním tunelu behem operace syndromu karpálního tunelu.

The usefulness of carpal tunnel pressures measurement during surgery of carpal tunnel syndrome is documented by the authors. 31 patients underwent endoscopic uniportal approach, the pressure in different positions (neutral position, flexion and extension in wrist, grip of the fingers) and in different levels (distal forearm, proximal, central and distal part of carpal tunnel, palm) was measured. The significant drop of pressure was observed after dissection of ligament in all levels. The results are presented in the form of several graphs.

The Sydney Heart Bank: improving translational research while eliminating or reducing the use of animal models of human heart disease.

The Sydney Heart Bank (SHB) is one of the largest human heart tissue banks in existence. Its mission is to provide high-quality human heart tissue for research into the molecular basis of human heart failure by working collaboratively with experts in this field. We argue that, by comparing tissues from failing human hearts with age-matched non-failing healthy donor hearts, the results will be more relevant than research using animal models, particularly if their physiology is very different from humans. Tissue from heart surgery must generally be used soon after collection or it significantly deteriorates. Freezing is an option but it raises concerns that freezing causes substantial damage at the cellular and molecular level. The SHB contains failing samples from heart transplant patients and others who provided informed consent for the use of their tissue for research. All samples are cryopreserved in liquid nitrogen within 40 min of their removal from the patient, and in less than 5-10 min in the case of coronary arteries and left ventricle samples. To date, the SHB has collected tissue from about 450 failing hearts (>15,000 samples) from patients with a wide range of etiologies as well as increasing numbers of cardiomyectomy samples from patients with hypertrophic cardiomyopathy. The Bank also has hearts from over 120 healthy organ donors whose hearts, for a variety of reasons (mainly tissue-type incompatibility with waiting heart transplant recipients), could not be used for transplantation. Donor hearts were collected by the St Vincent's Hospital Heart and Lung transplantation team from local hospitals or within a 4-h jet flight from Sydney. They were flushed with chilled cardioplegic solution and transported to Sydney where they were quickly cryopreserved in small samples. Failing and/or donor samples have been used by more than 60 research teams around the world, and have resulted in more than 100 research papers. The tissues most commonly requested are from donor left ventricles, but right ventricles, atria, interventricular system, and coronary arteries vessels have also been reported. All tissues are stored for long-term use in liquid N or vapor (170-180 °C), and are shipped under nitrogen vapor to avoid degradation of sensitive molecules such as RNAs and giant proteins. We present evidence that the availability of these human heart samples has contributed to a reduction in the use of animal models of human heart failure.

Oral metoclopramide with or without diphenhydramine: potential for prevention of late nausea and vomiting induced by cisplatin.

Late nausea and vomiting lasting 2-7 days occurs in 20%-68% of patients receiving cisplatin. We therefore studied the effects of oral metoclopramide given at doses of 20, 50, or 100 mg four times a day for 25 doses (7 days) beginning after cisplatin to determine the maximum tolerated dose (MTD) for prophylactic oral antiemetic regimens. Patients were stratified into younger (less than or equal to 30 yr old) and older (greater than 30 yr old) groups. Dose escalation was performed without or with concomitant oral diphenhydramine. For the younger group, the MTD for metoclopramide without diphenhydramine was less than 20 mg, and the MTD with diphenhydramine was 20 mg. For the older group, the MTD without diphenhydramine was 20 mg, and the MTD with diphenhydramine was 50 mg. Extrapyramidal reactions in the younger group and agitated depression in the older group were the major dose-terminating toxic effects. Patient acceptance of these regimens was excellent.

[Granulomatous myopathy in patients with sarcoidosis and myasthenia gravis]. / Granulomatózní myopatie u pacientu se sarkoidózou a myasthenia gravis.

Granulomatous myopathies are extremely rare. The finding of epithelioid granulomas in muscle biopsy indicates mostly an involvement of the skeletal muscle in systemic sarcoidosis. In this report we provide description of five patients with previously diagnosed sarcoidosis (pulmonary or cutaneous), in which a clinically significant muscle weakness developed. We aim at demonstrating the value of muscle biopsy for further treatment of the patients, since clinically indistinguishable myopathies can arise not due to the involvement of muscles in the granulomatous process, but due to the corticosteroid-induced changes (chronic steroid myopathy). The demonstration of a selective atrophy of type II muscle fibers can provide the clue for distinguishing the patients, in which the corticosteroid treatment should continue, from those, in which the treatment should be modified. Further, we discuss a rare finding of granulomas in muscle biopsies of two patients with myasthenia gravis (MG) associated with thymoma. Although it is difficult to explain the pathogenesis of this event, MG should be considered in the differential diagnosis of granulomatous myopathies. Moreover, the finding of granulomas along with lymphocytic infiltration in MG muscle should lead to a search for an underlying thymic neoplasm.

[PCR-multiplex system for analysis of Y-chromosomal microsatellite polymorphisms DYS449, DYS456, DYS458, and DYS464]. / PCR-multiplex systém pro analýzu Y chromosomálních mikrosatelitních polymorfismu DYS449, DYS456, DYS458 a DYS464.

DNA profiling--inclusive sex determination-- with microsatellite markers is currently a commonly used genetic method of studying humans. An efficient technique of producing the genetic data is amplification of multiple microsatellites in a single PCR reaction. Here we introduce a novel PCR-multiplex system for analysis of four polymorphic Y-STRs. Specifically, these are DYS449, DYS456, DYS458, and DYS464. These loci were chosen because of their reported high diversity in Euroamerican population (10), as well as their absence in the commercial analytical kits at the time of beginning of this study. Our objective was to design this PCR-multiplex for use of fragmentation analysis by electrophoresing samples on a capillary semi-automated genetic analyzer applying only one fluorescent dye. The PCR system we propose, may be notably used in fields such as forensic and human population genetics.

Distinct role of protein phosphatase 2A subunit Calpha in the regulation of E-cadherin and beta-catenin during development.

Protein phosphatase 2A (PP2A) plays central roles in development, cell growth and transformation. Inactivation of the gene encoding the PP2A catalytic subunit Calpha by gene targeting generates a lethal embryonic phenotype. No mesoderm is formed in Calpha(-/-) embryos. Here, we found that during normal early embryonic development Calpha was predominantly present at the plasma membrane whereas the highly homologous isoform Cbeta was localized to the cytoplasm and nuclei, suggesting the inability of Cbeta to compensate for vital functions of Calpha in Calpha(-/-) embryos. In addition, PP2A was found in a complex containing the PP2A substrates E-cadherin and beta-catenin. In Calpha(-/-) embryos, E-cadherin and beta-catenin were redistributed from the plasma membrane to the cytosol. Cytosolic concentrations of beta-catenin were low. Our results suggest that Calpha is required for stabilization of E-cadherin/beta-catenin complexes at the plasma membrane.