The relationship between cartilage thickness and muscle thickness or leg length discrepancy in poliomyelitis sequelae.

OBJECTIVES: To evaluate the relationship between cartilage loss and differences in muscle thickness and/or leg length in poliomyelitis sequelae (PMS). Our study is the first to evaluate the relationship between cartilage loss and both muscle atrophy and leg length discrepancy in the same population. METHODS: 37 patients with PMS and 38 healthy controls were included. Talar and distal femoral cartilage thicknesses and gastrocnemius medialis and quadriceps femoris muscle thicknesses were measured via ultrasound. Leg length differences and manual muscle strength were also evaluated. RESULTS: The mean muscle thicknesses and cartilage thicknesses were thinner in the more affected legs than in the less affected legs in the patient group. All of the ultrasonographic measurements were thinner in the less affected legs of the patient group than in the right legs of the control group, except for the knee cartilage thicknesses. While there was a correlation between the cartilage thickness difference and the muscle thickness difference between the less and more affected legs in the patient group, there was no correlation between the cartilage thickness difference and leg length differences. CONCLUSIONS: Patients with PMS are predisposed to osteoarthritis. Talar and knee cartilage thicknesses may be more associated with the muscle thickness than the leg length discrepancy in PMS.

Propagation patterns in motor neuron diseases: Individual and phenotype-associated disease-burden trajectories across the UMN-LMN spectrum of MNDs.

Motor neuron diseases encompass a divergent group of conditions with considerable differences in clinical manifestations, survival, and genetic vulnerability. One of the key aspects of clinical heterogeneity is the preferential involvement of upper (UMN) and lower motor neurons (LMN). While longitudinal imaging patters are relatively well characterized in ALS, progressive cortical changes in UMN,- and LMN-predominant conditions are seldom evaluated. Accordingly, the objective of this study is the juxtaposition of longitudinal trajectories in 3 motor neuron phenotypes; a UMN-predominant syndrome (PLS), a mixed UMN-LMN condition (ALS), and a lower motor neuron condition (poliomyelitis survivors). A standardized imaging protocol was implemented in a prospective, multi-timepoint longitudinal study with a uniform follow-up interval of 4 months. Forty-five poliomyelitis survivors, 61 patients with amyotrophic lateral sclerosis (ALS), and 23 patients with primary lateral sclerosis (PLS) were included. Cortical thickness alterations were evaluated in a dual analysis pipeline, using standard cortical thickness analyses, and a z-score-based individualized approach. Our results indicate that PLS patients exhibit rapidly progressive cortical thinning primarily in motor regions; ALS patients show cortical atrophy in both motor and extra-motor regions, while poliomyelitis survivors exhibit cortical thickness gains in a number of cerebral regions. Our findings suggest that dynamic cortical changes in motor neuron diseases may depend on relative UMN and/or LMN involvement, and increased cortical thickness in LMN-predominant conditions may represent compensatory, adaptive processes.

Ultrasonographic evaluation of the distal femoral and talar cartilage thicknesses in patients with poliomyelitis: a cross-sectional observational study.

BACKGROUND: Sequelae of poliomyelitis, coupled with asymmetric impairment and weight-bearing, typically alter walking biomechanics which can be associated with the knee and ankle osteoarthritis. AIM: We aimed to investigate whether the distal femoral and talar cartilage thicknesses were different in patients with poliomyelitis. DESIGN: Cross-sectional observational study. SETTING: Outpatients, tertiary care center. POPULATION: Thirty-six patients (12 males, 24 females) with a history of poliomyelitis and 36 age, gender and body mass index similar healthy subjects (11 males, 25 females) were enrolled. Mean values for age, body mass index and age of the poliomyelitis onset were 70.2±4.6 years, 27.2±5.7 kg/m2, and 3.6±2.4 years. METHODS: Visual Analogue Scale (VAS) was used to assess pain. Lower limb muscle strengths were measured by manual muscle testing. The more affected side was identified according to the lower limb manual muscle testing. Bilateral distal femoral cartilage from the lateral femoral condyle, intercondylar area, medial femoral condyle and talar cartilage thicknesses were measured using ultrasound imaging. RESULTS: Among patients, the onset of poliomyelitis was at 3.6±2.4 years of age, and Functional Ambulation Category scores were 5 (3-5). VAS scores were similar between the sides affected more and less by poliomyelitis. All cartilage thicknesses (except the talar cartilage) of the patients were found to be thinner on the more affected side than the less affected side (all P<0.001). The thickness of talar cartilage was thinner compared to control subjects (P<0.001). Among the patients, quadriceps muscle strength was positively correlated with medial condyle (r=0.377, P=0.024), intercondylar area (r=0.399, P=0.016) and lateral condyle (r=0.363, P=0.030) thicknesses. Knee VAS scores were negatively correlated with talar cartilage thicknesses (r=-0.393, P=0.018). CONCLUSIONS: We found a thinning of the distal femoral condyle in the more affected paretic sides of poliomyelitis patients as compared to both those of less affected sides and those of healthy controls. Talar cartilages on both sides of the patients were thinner compared to control subjects. CLINICAL REHABILITATION IMPACT: Our preliminary findings may contribute to the long-term management of patients with long-term poliomyelitis sequelae.

Radiographic and Clinical Outcomes of Surgical Correction of Poliomyelitis-Related Spinal Deformities: A Comparison Among Three Types of Pelvic Instrumentations.

BACKGROUND: We compared the clinical and radiographic outcomes of corrective surgery in patients with poliomyelitis-related spinal deformity (PSD) using 3 types of pelvic fixation and investigated the incidence and risk factors for complications. METHODS: We reviewed the data from 42 patients with PSD who had undergone spinopelvic reconstruction at a single institution from 2000 to 2016. Of the 42 patients, 15 had been treated with the Galveston technique, 13 with iliac screw fixation, and 14 with S2-alar-iliac (S2AI) screw fixation. Demographic data, radiographic parameters, and complications were analyzed. Health-related quality of life was determined using Scoliosis Research Society (SRS) 22-item questionnaires and the Oswestry Disability Index scores. RESULTS: After surgery, the correction rate of the main curve was 51.7%, 57.8%, and 52.1% in the 3 groups, with significant improvement in regional kyphosis, coronal balance, and pelvic obliquity (PO) (P < 0.05). The correction of PO was similar among the 3 types of pelvic fixation; however, the patients treated with S2AI fixation required significantly less operative time (P < 0.05) and blood loss (P < 0.006). The overall complication rate was 40.5%, with a major complication rate of 23.8%. Age at surgery (P = 0.006) and grade >2 SRS-Schwab osteotomy (P = 0.036) were significant risk factors for complications. Significant improvement was found in the SRS-22 and Oswestry Disability Index scores at the final follow-up examination in the 3 groups. CONCLUSIONS: The present study showed satisfactory correction of spinopelvic deformity for 42 patients with PSD. Compared with the Galveston technique and iliac screw fixation, the use of S2AI significantly decrease the operative time and estimated blood loss and obtained similar correction of PO. Patient age at surgery and grade >2 SRS-Schwab osteotomy were significant risk factors for complications.

Long-term Outcome of Unconstrained Primary Total Hip Arthroplasty in Ipsilateral Residual Poliomyelitis.

Incapacitating articular sequelae in the hip joint have been described for patients with late effects of poliomyelitis. In these patients, total hip arthroplasty (THA) has been associated with a substantial rate of dislocation. This study was conducted to evaluate the long-term clinical and radiologic outcomes of unconstrained THA in this specific group of patients. The study included 6 patients with ipsilateral polio who underwent primary THA between 1985 and 2006. Patients with polio who underwent THA on the nonparalytic limb were excluded. Mean follow-up was 119.5 months (minimum, 84 months). Clinical outcomes were evaluated with the modified Harris Hip Score (mHHS) and the visual analog scale (VAS) pain score. Radiographs were examined to identify the cause of complications and determine the need for revision surgery. All patients showed significantly better functional results when preoperative and postoperative mHHS (67.58 vs 87.33, respectively; P=.002) and VAS pain score (7.66 vs 2, respectively; P=.0003) were compared. Although 2 cases of instability were diagnosed, only 1 patient needed acetabular revision as a result of component malpositioning. None of the patients had component loosening, osteolysis, or infection. Unconstrained THA in the affected limb of patients with poliomyelitis showed favorable long-term clinical results, with improved function and pain relief. Nevertheless, instability may be a more frequent complication in this group of patients compared with the general population. [Orthopedics. 2017; 40(2):e255-e261.].

Is there a role for total ankle replacement in polio patients?: A case report and review of the literature.

Although poliomyelitis is almost eradicated, as orthopaedic surgeons we occasionally encounter residual deformities in patients who suffered the disease in the past. An equino-varus deformity of the foot is the commonest deformity seen. In this report we present a 53 years old lady who had poliomyelitis as a child and presented with a painful, degenerate ankle and 12 degrees of varus deformity. CT confirmed advanced degenerative changes. The management options included realignment and fusion, supramalleolar ostoetomy or total ankle replacement. In view of the satisfactory active range of motion and advanced OA confirmed by CT scan we considered the option of total ankle replacement. To our knowledge this has never been reported before. At two and half years follow up the patient showed satisfactory functional results (AOFAS score 88/100) and high satisfaction. Plain radiographs revealed a well aligned joint with no evidence of loosening or osteolysis.

Asymmetrical hemochromatosis arthropathy in a patient with a history of poliomyelitis.

Herein, we report a case of a 67-year-old man with hereditary hemochromatosis and a history of poliomyelitis that had caused a paresis of the right arm. Hemochromatosis-associated arthropathy developed in all but the paretic limb. X-ray and MRI revealed degenerative, massive inflammatory and destructive changes in the joints of the non-paretic hand only. These findings argue for the contribution of physical exposure and non-mechanical factors to the development of the hemochromatosis arthropathy.

Imported case of poliomyelitis, Melbourne, Australia, 2007.

Wild poliovirus-associated paralytic poliomyelitis has not been reported in Australia since 1977. We report type 1 wild poliovirus infection in a man who had traveled from Pakistan to Australia in 2007. Poliomyelitis should be considered for patients with acute flaccid paralysis or unexplained fever who have been to poliomyelitis-endemic countries.

Cesarean delivery under ultrasound-guided spinal anesthesia [corrected] in a parturient with poliomyelitis and Harrington instrumentation.

PURPOSE: To describe the anesthetic implications, and management of a medically complex parturient, who presented for Cesarean delivery (CD). The patient had poliomyelitis complicated with severe kyphoscoliosis, which had been treated with extensive spinal surgery. We used ultrasound guidance to facilitate successful spinal analgesia and anesthesia. CLINICAL FEATURES: A 27-yr-old woman, with a history of poliomyelitis and moderate restrictive lung disease secondary to kyphoscoliosis, presented at 38 weeks gestation for elective CD because of cephalopelvic disproportion. The woman had Harrington rods in situ from the level of the second thoracic vertebra, to the level of the fourth lumbar vertebra. Ultrasound guidance enabled one intervertebral space to be visualized (L5-S1), 3 cm from the expected spinal midline, and spinal anesthesia was performed at this interspace without any complications. A healthy infant was delivered, and the mother recovered uneventfully. CONCLUSIONS: Spinal anesthesia can be effectively performed in patients with poliomyelitis and severe kyphoscoliosis, that has been treated with extensive Harrington instrumentation. To facilitate regional techniques in such patients, bedside ultrasound may be greatly beneficial in identifying the correct spinal interspace.

[The prevalance of orthopedic disabilities in the district of Cay, Afyon, Turkey]. / Afyon'un Cay ilçesinde ortopedik özürlerin sikligi.

OBJECTIVES: Data were collected on disabilities from a sample population in order to estimate the prevalence of orthopedic disabilities in the general population. METHODS: A two-stage field study was conducted in Cay, a district of Afyon, Turkey. In the first stage, a list of all individuals with orthopedic disabilities was derived. Then, medical histories were elicited and examinations were made by a team of specialists of orthopedics and traumatology. Radiographic studies were made when necessary. Orthopedic disabilities were defined as any muscle or skeletal abnormality that was associated with a permanent functional loss and were classified as congenital, traumatic, cerebral palsy, poliomyelitis, and other causes. RESULTS: The overall population was 35,571, of whom 189 had orthopedic disabilities (53/10,000). The male-to-female ratio was 1.8. The most common cause was congenital diseases (25.9%; 13.7/10,000), followed by trauma (23.8%; 12.6/10,000), cerebral palsy (21.7%; 11.5/10,000), poliomyelitis (10.1%; 5.3/10,000), and others (18.5%; 9.8/10,000). The lowest mean age (19+/-5 years) was found in those with cerebral palsy, which was associated with the highest degree of functional loss. The mean age in patients with poliomyelitis was 29+/-7 years. It was found that 37% of the disabled could benefit from either a limited or extensive surgical intervention, and 9% could have significant improvement both in functional ability and life quality through physical therapy or the use of a prosthesis/orthosis. Only 18% were under the coverage of a health insurance, though. CONCLUSION: The data of this study may throw some new light on the prevalence and implications of orthopedic disabilities in Turkey.

Computed tomographic study of the skeletal musculature of the lower body in 45 postpolio patients.

Muscle computed tomography (CT) and muscle strength assessment of the pelvic girdle and leg muscles were performed in 32 postpolio patients experiencing new muscle weakness, and in 13 postpolio patients with stable neuromuscular condition. Muscles of the postpolio patients experiencing new muscle weakness showed significantly more CT scan abnormalities as compared with the stable postpolio patients. No other features discriminative of symptomatic postpolio patients were found. In individual patients, muscle CT scan evaluation is a useful adjunct to muscle strength assessment.

Fixed pelvic obliquity after poliomyelitis: classification and management.

We classified fixed pelvic obliquity in patients after poliomyelitis into two major types according to the level of the pelvis relative to the short leg. Each type was then divided into four subtypes according to the direction and severity of the scoliosis. In 46 patients with type-I deformity the pelvis was lower and in nine with type II it was higher on the short-leg side. Subtype-A deformity was a straight spine with a compensatory angulation at the lower lumbar level, mainly at L4-L5, subtype B was a mild scoliosis with the convexity to the short-leg side, subtype C was a mild scoliosis with the convexity opposite the short-leg side, and subtype D was a moderate to severe paralytic scoliosis with the convexity to the short-leg side in type I and to the opposite side in type II. A combination of surgical procedures improved the obliquity in most patients. These included lumbodorsal fasciotomy, abductor fasciotomy and stabilisation of the hip by triple innominate osteotomy with or without transiliac lengthening. In patients with type ID or type IID appropriate spinal fusion was usually necessary.

[Computed tomography of the skeletal muscles in neuromuscular diseases]. / Computertomographie der Skelettmuskulatur bei neuromuskulären Erkrankungen.

CT-documentation of skeletal muscular lesions caused by neuromuscular diseases implies an essential contribution to conventional techniques in the macroscopic field. Size, distribution and degree of lesions as well as compensatory mechanisms are proved thereby. We report about the different effects on muscle appearance referring to 106 patients of our own experience in amyotrophic lateral sclerosis, spinal muscular atrophy, poliomyelitis, polyradiculitis, polyneuropathy as well as peripheral traumatic nerve lesions.

Skeletal changes in neuromuscular disorders mimicking juvenile rheumatoid arthritis and hemophilia.

Seven patients with neuromuscular disorders were examined, including one with cerebral palsy, one with Duchenne muscular dystrophy, two with paraplegia, and three with poliomyelitis; all exhibited skeletal changes mimicking those found in juvenile rheumatoid arthritis and hemophilia. These findings included apparent overgrowth of the epiphyses, periarticular osteoporosis, and joint-space narrowing in seven subjects; accentuation of the trabecular pattern in six; gracile bones and soft-tissue wasting in five; tibiotalar slant in two; and premature epiphyseal closure in one. Changes in osseous vascular dynamics and the debilitation or immobilization found both in patients with neuromuscular disorders and those with arthritis may help explain these overlapping findings. While the clinical distinction between the neuromuscular and arthritic disorders is straightforward, the similarity in radiographic appearance has received little attention. If the clinical history is inadequate, this may result in confusion or misinterpretation by the radiologist. In the absence of more specific findings, such as articular erosions or erosions of the femoral intercondylar notch, the differential diagnosis may be mistakenly limited to juvenile rheumatoid arthritis and hemophilia. In such cases, the neuromuscular disorders should also be considered in the differential diagnosis.

Fatty replacement of lower paraspinal muscles: normal and neuromuscular disorders.

The physiologic replacement of the lower paraspinal muscles by fat was evaluated in 157 patients undergoing computed tomography for reasons unrelated to abnormalities of the locomotor system. Five patients with neuromuscular disorders were similarly evaluated. The changes were graded according to severity at three spinal levels: lower thoracic-upper lumbar, midlumbar, and lumbosacral. The results were analyzed in relation to age and gender. It was found that fatty replacement of paraspinal muscles is a normal age-progressive phenomenon most prominent in females. It progresses down the spine, being most advanced in the lumbosacral region. The severest changes in the five patients with neuromuscular disorders (three with poliomyelitis and two with progressive muscular dystrophy) consisted of complete muscle group replacement by fat. In postpoliomyelitis atrophy, the distribution was typically asymmetric and sometimes lacked clinical correlation. In muscular dystrophy, fatty replacement was symmetric, showing relative sparing of the psoas and multifidus muscles. In patients with neuromuscular diseases, computed tomography of muscles may be helpful in planning a better rehabilitation regimen.

Roentgenmorphological muscle changes in anterior horn cell lesions.

The author examined muscles in the extremities of 42 poliomyelitis patients using a special roentgenological technique. This method made it possible to objectively determine the size of the muscles in the soft tissues of the extremities. The volume and distribution of the increased amounts of fatty tissue in the muscle and the volume of normal muscle tissue led to draw conclusions regarding the histological state of the muscle. Roentgenmorphological changes are closely linked to muscle dysfunction. Roentgenological examination of the soft tissue provides a useful supplement to a muscle biopsy and to electromyographic examinations and is of value for the rehabilitation of the patients.

[Roentgenological findings in muscular alterations of extremities (author's transl)]. / Uber die Röntgenmorphologie der Veränderungen der Extremitätenmuskulatur.

A survey of roentgenological findings in muscular alterations of extremities based on the author's experiences and on the literature is presented. Following a description of the normal roentgen anatomy, the alterations in different diseases of interstitial lipomatosis are demonstrated. By roentgenological examinations different muscular lesions of the extremities can be differentiated and the clinical follow-up verified.

Combined immunodeficiency and vaccine-related poliomyelitis in a child with cartilage-hair hypoplasia.

Patients previously described with cartilage-hair hypoplasia, a distinctive form of short-limbed dwarfism, have been found to have deficient cell-mediated immunity with intact antibody-mediated immunity. The patient with cartilage-hair hypoplasia described in the present report is unusual in that she had both deficient antibody-mediated immunity and deficient cell-mediated immunity. In addition, she developed severe, vaccine-related paralytic poliomyelitis. This complication suggests that live viral vaccines should not be administered to children with short-limbed dwarfism until the form of short-limbed dwarfism is established and immunologic evaluation is performed when indicated.