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Background: Isolated actinomycosis of the humerus is a very rare entity. Method: A 30-year-old male had an erythematous plaque with a pus-(minimal) discharging sinus over left lower arm. The patient had no discharge of sulphur granules from the sinus. The patient had raised ESR with a single lytic lesion with minimal sclerosis and inconspicuous periosteal reaction on radiographs. Such atypical clinical and radiological features lead to initial wrong diagnosis of tuberculosis. A diagnosis of Actinomycosis of humerus became possible after demonstration of filamentous bacilli in culture and on histopathology from the sulphur grains obtained by open biopsy. Result: The patient recovered completely after administering PenicillinG 24 million units intravenous for 7 days followed by 1.2 g of oral amoxicillin in three divided doses for 3 months and did not show any recurrence during last 2 years of follow up. Conclusion: Surgeons should be aware of this rare entity and difficulty in its diagnosis due to its variable manifestations, including confusion with highly endemic tuberculous infection. Awareness of full spectrum of the diseases and careful evaluation in individual cases will expedite diagnosis and avoid unnecessary surgical interventions. [ABSTRACT FROM AUTHOR]

Low grade central osteosarcoma is a rare primary bone tumor. This fibro-osseous lesion shares some radiological and histopathological resemblance with fibrous dysplasia, which is a more common entity. Thus it may be mistaken as fibrous dysplasia and may receive inadequate treatment resulting in a more malignant recurrent bone tumor. We present a case of low grade central osteosarcoma of the right tibia, which was initially treated as fibrous dysplasia with curettage and bone grafting. Three years later the tumor recurred with greater soft tissue and bony involvement but without metastasis. The patient was treated with above knee amputation and has been asymptomatic for the last two years. [ABSTRACT FROM AUTHOR]

The brain-specific enzyme CYP46A1 controls cholesterol turnover by converting cholesterol into 24 S -hydroxycholesterol (24OH). Dysregulation of brain cholesterol turnover and reduced CYP46A1 levels are observed in Alzheimer's disease (AD). In this study, we report that CYP46A1 overexpression in aged female mice leads to enhanced estrogen signaling in the hippocampus and improved cognitive functions. In contrast, age-matched CYP46A1 overexpressing males show anxiety-like behavior, worsened memory, and elevated levels of 5α-dihydrotestosterone in the hippocampus. We report that, in neurons, 24OH contributes to these divergent effects by activating sex hormone signaling, including estrogen receptors. CYP46A1 overexpression in female mice protects from memory impairments induced by ovariectomy while having no effects in gonadectomized males. Last, we measured cerebrospinal fluid levels of 24OH in a clinical cohort of patients with AD and found that 24OH negatively correlates with neurodegeneration markers only in women. We suggest that CYP46A1 activation is a valuable pharmacological target for enhancing estrogen signaling in women at risk of developing neurodegenerative diseases.

Background: Male estrogen receptor beta (ERβ) knockout (BERKO) mice display anxiety and aggression linked to, among others, altered serotonergic signaling in the basolateral amygdala and dorsal raphe, impaired cortical radial glia migration, and reduced GABAergic signaling. The effects on primary motor cortex (M1 cortex) and locomotor activity as a consequence of ERβ loss have not been investigated., Objective: The aim of this study was to determine whether locomotor activity is altered as a consequence of the changes in the M1 cortex., Methods: The locomotor activity of male wild-type (WT) and BERKO mice was evaluated using the open-field and rotarod tests. Molecular changes in the M1 cortex were analyzed by RNA sequencing, electron microscopy, electrophysiology, and immunohistological techniques. In addition, we established oligodendrocyte (OL) cultures from WT and BERKO mouse embryonic stem cells to evaluate OL function., Results: Locomotor profiling revealed that BERKO mice were more active than WT mice but had impaired motor coordination. Analysis of the M1 cortex pointed out differences in synapse function and myelination. There was a reduction in GABAergic signaling resulting in imbalanced excitatory and inhibitory neurotransmission as well as a defective OL differentiation accompanied by myelin defects. The effects of ERβ loss on OL differentiation were confirmed in vitro., Conclusion: ERβ is an important regulator of GABAergic interneurons and OL differentiation, which impacts on adult M1 cortex function and may be linked to increased locomotor activity and decreased motor coordination in BERKO mice., (© 2020 The Author(s) Published by S. Karger AG, Basel.)

Recent meta-analyses found an association between prenatal exposure to the antidepressant fluoxetine (FLX) and an increased risk of autism in children. This developmental disorder has been related to dysfunctions in the brains' rewards circuitry, which, in turn, has been linked to dysfunctions in dopaminergic (DA) signaling. The present study investigated if FLX affects processes involved in dopaminergic neuronal differentiation. Mouse neuronal precursors were differentiated into midbrain dopaminergic precursor cells (mDPCs) and concomitantly exposed to clinically relevant doses of FLX. Subsequently, dopaminergic precursors were evaluated for expression of differentiation and stemness markers using quantitative polymerase chain reaction. FLX treatment led to increases in early regional specification markers orthodenticle homeobox 2 ( Otx2 ) and homeobox engrailed-1 and -2 ( En1 and En2 ). On the other hand, two transcription factors essential for midbrain dopaminergic (mDA) neurogenesis, LIM homeobox transcription factor 1 α ( Lmx1a ) and paired-like homeodomain transcription factor 3 ( Pitx3) were downregulated by FLX treatment. The stemness marker nestin ( Nes ) was increased, whereas the neuronal differentiation marker β 3-tubulin ( Tubb3 ) decreased. Additionally, we observed that FLX modulates the expression of several genes associated with autism spectrum disorder and downregulates the estrogen receptors (ERs) α and β Further investigations using ER β knockout (BERKO) mDPCs showed that FLX had no or even opposite effects on several of the genes analyzed. These findings suggest that FLX affects differentiation of the dopaminergic system by increasing production of dopaminergic precursors, yet decreasing their maturation, partly via interference with the estrogen system., (Copyright © 2018 by The Author(s).)

The ability to propagate mature cells and tissue from pluripotent stem cells offers enormous promise for treating many diseases, including neurodegenerative diseases. Before such cells can be used successfully in neurodegenerative diseases without causing unwanted cell growth and migration, genes regulating growth and migration of neural stem cells need to be well characterized. Estrogen receptor beta (ERβ) is essential for migration of neurons and glial cells in the developing mouse brain. To examine whether ERβ influences differentiation of mouse embryonic stem cells (mESC) into neural lineages, we compared control and ERβ knockout (BERKO) mESCs at defined stages of neural development and examined the effects of an ERβ-selective ligand (LY3201) with a combination of global and targeted gene-expression profiling and the expression of key pluripotency markers. We found that ERβ was induced in embryoid bodies (EBs) and neural precursor cells (NPCs) during development. Proliferation was higher in BERKO NPCs and was inhibited by LY3201. Neurogenesis was reduced in BERKO ES cells, and oligodendrogliogenesis was enhanced. BERKO EBs expressed higher levels of key ectodermal and neural progenitor markers and lower levels of markers for mesoderm and endoderm lineages. ERβ-regulated factors are involved in cell adhesion, axon guidance, and signaling of Notch and GABA receptor pathways, as well as factors important for the differentiation of neuronal precursors into dopaminergic neurons (Engrailed 1) and for the oligodendrocyte fate acquisition (Olig2). Our data suggest that ERβ is an important component for differentiation into midbrain neurons as well as for preventing precocious oligodendrogliogenesis., Competing Interests: The authors declare no conflict of interest.

We compared the radiological and functional outcomes in patients with radial longitudinal deficiency treated surgically with those managed nonoperatively and correlated the ease of performance of activities with radiological and functional parameters in the two groups. A retrospective analysis was done in 446 type 3 and 4 hands (modified Bayne classification) over 20 years comparing the subjective assessment, objective and functional scores in nonoperatively managed hands (Group 1; n = 137) to hands managed with centralization or radialization (Group 2; n = 309). The subjective, objective and functional measures were significantly better in Group 2. Improved alignment, finger and wrist range of motion and grip strength were related to improvement in functional score. Surgical treatment of radial longitudinal deficiency provides improvement in cosmesis, function and ease of performance of activities when compared with nonoperatively managed hands.

To report unusual occurrence of angiomyolipoma at intraarticular location with another lesion in the same side foot. A 12-year-old girl was referred to us after initial inconclusive work-up done elsewhere for swelling of left knee joint. There was a 15 × 12 cm swelling in the knee joint partially encasing patella while also a similar hourglass shaped swelling measuring 9 × 4 cm was noted in the same side foot. After clinical and radiological evaluation an excision biopsy was planned for both sites. The specimen sent for histopathological evaluation revealed angiomyolipoma with identical characteristics in the two locations and was HMB45 immunostain negative. Patient was evaluated for possibility of tuberous sclerosis but there was no contributory evidence. Angiomyolipoma is typically a solitary renal tumor with rare occurrence at musculoskeletal sites. Multicentric variety is still infrequent. Intraarticular occurrence of angiomyolipoma presents a diagnostic challenge not only in terms of unfamiliarity but also unusual presentation with absence of characteristic immunostaining and multicentricity requiring careful exclusion of other lesions that may require a more radical approach for treatment., (Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Background: Minimally invasive approaches such as sclerotherapy have been introduced to treat aneurysmal bone cysts. Sclerotherapy has been associated with reasonable healing rates during the past two decades. However, it is unclear whether sclerotherapy compares with the more traditional extended curettage and bone grafting., Questions/purposes: We therefore compared the healing rates and functional scores in patients having percutaneous repetitive sclerotherapy using polidocanol (Group 1) with those with intralesional excision (extended curettage with a high-speed burr) and bone grafting (Group 2) for treatment of aneurysmal bone cyst., Patients and Methods: We randomly divided 94 patients into two treatment groups. We assessed healing rates (primary outcome measure), pain relief, time to healing and recurrence, hospital stay, and the Enneking functional score. Forty-five patients from Group 1 and 46 from Group 2 were available for study. The minimum followup was 3.2 years (mean, 4.4 years; range, 3.2-6.1 years)., Results: At last followup, 93.3% in Group 1 and 84.8% in Group 2 had achieved healing. Complications in Group 1 were minor and resolved. In Group 2, three patients had deep infections and five had superficial infections, and two had growth disturbances. Although the healing rates were similar, we found higher rates of clinically important complications, worse functional outcomes, and higher hospital burden associated with intralesional excision., Conclusions: Repetitive sclerotherapy using polidocanol is a minimally invasive, safer method of treatment for aneurysmal bone cysts compared with intralesional excision and bone grafting. In this preliminary study, we found similar recurrence rates for the two treatment methods, however, this will require confirmation in larger studies., Level of Evidence: Level II, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Background: Complications that develop after femoral neck fracture in children-especially osteonecrosis-have been retrospectively attributed to inadvertent delayed fixation and fracture type. Prospective evaluation of results after delayed fixation of femoral neck fractures in children beyond the first 24 h is not reported in the literature and requires evaluation to increase our understanding of the procedure and improve fixation methods. Also, the role of capsular decompression in initial management needs to be elucidated., Materials and Methods: Radiological and functional evaluation was done for delayed fixation (>24 h) of displaced fractures in the femoral neck in 21 children (21 hips) treated over 11 years. Mean patient age was 11.8 (median 12, range 5-15) years. Extraphyseal fixation was done using partially threaded cannulated cancellous screws after closed or open reduction. Patients were allowed full weight bearing after 12-18 weeks. Results were assessed on the basis of modified Ratliff criteria. Patients were followed for a mean of 81 (range 66-129) months., Results: All fractures united at a mean duration of 12 (range 10.6-14) weeks. Three (14.3%) patients had osteonecrosis of the hip, which was significantly related to poor outcome (r = 0.495; P = 0.022). There was a significant correlation (r = 0.52) between development of osteonecrosis and delayed fracture fixation of >10 days (P = 0.016) and open reduction (P = 0.016)., Conclusions: Outcome following temporal delay in fracture fixation of the femoral neck is primarily affected by osteonecrosis of the femoral head, whereas restriction of movements, shortening, and premature physeal closure has no significant influence. Osteonecrosis is primarily linked to delay and open reduction, whereas fracture type, age, and sex seem insignificant factors. Capsular decompression does not seem to affect the outcome in delayed presentations and may hinder definitive treatment.

Background: The aim of the study was to evaluate statistically the occurrence and causes of accidental fall of instruments and implants during orthopaedic procedures., Methods: A prospective study was carried out over a period of 18 months. Total of 120 randomly chosen major orthopaedic surgeries were observed. The observer counted the number of times an instrument fell during surgery. The offending operating room personnel were identified. A note of the nature of instrument falling was made., Results: A total of 120 surgeries were observed. There were 15 falls (38.5%) during elective surgeries and 24 (61.5%) during emergency (trauma) surgeries. Falls were classified as major and minor. There were a total of 39 falls with 11 major and 28 minor falls. Seven surgeries had two or more falls. In 26 instances (66.7%) the operating surgeon was responsible for the fall. In seven (17.9%) assisting surgeon and in three (7.7%) of the instances the scrub nurse was responsible for the fall of the instruments. Three falls (7.7%) were unaccountable. An average delay of 7.6 min was noted after the fall of the instrument or implant., Conclusions: Fall of instruments in the theatre is a common problem all over the world. It leads to increased operating time and extra resources and can hamper the end result of surgery. Our study is the first of its kind to evaluate the problem. Most falls occurred because of the operating surgeon. A course in instrument handling similar to one conducted by the theatre nurses may be helpful in preventing such falls.

Kimura's disease is a rare, chronic inflammatory disorder, presenting usually as head and neck swellings and regional cervical lymphadenopathy. The disease is more often described from oriental literature but the osseous manifestations and involvement of extremity are rare and unidentified in literature. We present an unusual case of long bone periosteal reaction in a patient of Kimura's disease of extremity. This potentially innocuous disorder must be kept in mind as a radiological differential of periosteal reaction.

Spinal tuberculosis constitutes 50% of all musculoskeletal tuberculosis. However, literature regarding congenital spinal tuberculosis is very scanty. Congenital spinal tuberculosis was diagnosed in a two-month-old child on the basis of age at presentation (gibbus since three weeks of age), hepatomegaly, raised ESR, radiological destruction of D10-D11 vertebrae, asymptomatic maternal endometrial tuberculosis and tuberculous histopathology from CT guided biopsy specimen from D10 vertebra. Both child and mother were treated by antitubercular treatment. Child improved symptomatically, gibbus became less prominent and ESR became normal at the end of one year of treatment. Patient had no recurrence during last two years of follow-up. This is the first case of congenital tuberculosis of spine with a documented source of infection from asymptomatic maternal endometrial tuberculosis. CT guided core biopsy from vertebra/aspiration from the paravertebral abscess help in early diagnosis and treatment to reduce neurological morbidity and mortality. Endometrial biopsy help in establishing the diagnosis of congenital tuberculosis and adequate antitubercular treatment in such cases may provide protection to fetus in subsequent pregnancy.

Desmoplastic fibroma of bone is an extremely rare locally aggressive primary bone tumour. Diagnosis is tricky and treatment experience limited with tumor surgeons. We studied three rare cases of desmoplastic fibroma arising primarily in bone at unusual sites. Diagnosis was established by radio-histopathologic correlation. Patients were operated with various modalities. Results were evaluated over long term follow-up (6-9 years). The disease was eradicated in all the three cases and no recurrence was seen over long term follow-up. Functional outcome was good and no complications were encountered. Wide resection of the tumor is favoured as the recurrence rates after surgical treatment are high.

Clubfoot is the commonest congenital deformity in babies. More than 100,000 babies are born worldwide each year with congenital clubfoot. Around 80% of the cases occur in developing nations. We treated 154 feet [mean Pirani score (total) 5.57] in 96 children (78 males, 18 females) by the Ponseti method from January 2003 to December 2005. A prospective follow-up for a mean duration of 19.5 months (range 6-32 months) was undertaken. After six months of treatment the Pirani score was reduced to zero for all patients. The results show that corrective surgery, sometimes multiple, can be avoided in most cases which are usually associated with the development of a stiff, painful foot. Low socio-economic status and illiteracy prevailing in developing nations increases the prevalence of neglected clubfoot that is still harder to correct. Integration into various programs and proper use of available resources can decrease neglected clubfoot and improve chances of successful and timely correction of deformity. Bracing constitutes an important part of treatment and proper motivation and education of the parents mitigates the chances of losing correction. The Ponseti method of correcting clubfoot is especially important in developing countries, where operative facilities are not available in the remote areas and well-trained physicians and personnel can manage the cases effectively with cast treatment only.

Background: Aim of our study was to evaluate the use of Locking Compression Plate (LCP) in patients with long standing nonunions of the clavicle with osteoporotic bones., Material and Methods: There were a total of 11 patients. Surgery was performed for painful nonunion with shoulder dysfunction. Average time since injury was 6.9 months (3-39 months). Nonunions were stabilized by a 3.5-mm LCP, applied on the antero-inferior surface of the clavicular shaft. A six-hole LCP was used in eight cases. In three patients seven-hole LCP and in one patient eight-hole LCP was used. The fixation was supplemented by autogenous cancellous bone graft (except in hypertrophic nonunions). Patients were followed for a mean of 2.8 (1-3) years., Results: The average time of radiologic union was 5.3 months (3-9 months). Union was achieved in all patients. Subjective clinical assessment was performed using the Disabilities of the Arm, Shoulder, and Hand functional score (DASH). The mean DASH score preoperatively was 42 (25-52) and postoperatively was 24 (11-34). Superficial infection and features of reflex sympathetic dystrophy were seen in one patient each., Conclusion: Long standing clavicular nonunion with osteopenic bone is a difficult problem to treat. Our study shows that LCPs can be an improved implant option with good fracture healing.

Although uncommon in developed world, tuberculosis is one of the most dreaded diseases in the under-developed and developing countries. Resurgence of tuberculosis and its increasing incidence, with widespread HIV infection has lead to the reporting of infections at unusual sites. Tubercular tenosynovitis not involving bone is an extremely rare and perplexing presentation. A rare case of isolated tuberculosis of Achilles tendon without any evident primary contiguous or distant focus in a 55-year-old immunocompetent male is presented.

Giant cell tumour of bone is an aggressive benign bone tumour. It rarely affects the ribs. It usually involves the posterior end of the ribs and literature regarding anterior end involvement is scanty. We report a case of giant cell tumour of the anterior end of the rib masquerading as a sub-mammary abscess in lactating women.

Tuberculosis is as old as mankind. Vertebral tuberculosis is fairly common form of bone and joint tuberculosis. However, isolated sacral tuberculosis is rare. It may present as an atypical sacral lesion having epidural granuloma without destruction of sacrum on MRI and plain X-rays. This atypical presentation may lead to delay in diagnosis and treatment. This case report intends to emphasize that spinal tuberculosis should be the first and foremost differential diagnosis in the presence of atypical clinical and radiological features of a sacral lesion particularly in developing countries. Early diagnosis and treatment can prevent or minimize the neurological morbidity in such cases.

Vertebral disease constitutes approximately 50% of all skeletal tuberculosis. We describe a patient who developed a discharging sinus at the tip of the coccyx. Extensive examination revealed isolated tuberculosis of the coccyx. Although rare, the condition should be suspected in patients presenting with a chronic sinus in the sacrococcygeal area and a lytic lesion in the coccyx on CT or MRI, particularly in the developing world.

Aneurysmal bone cyst is a rare tumour-like lesion which develops during growth. Our aim was to determine the efficacy of the administration of percutaneous intralesional 3% polidocanol (hydroxypolyaethoxydodecan) as sclerotherapy. Between July 1997 and December 2004 we treated 72 patients (46 males, 26 females) with a histologically-proven diagnosis of aneurysmal bone cyst, at various skeletal sites using this method. The sclerotherapy was performed under fluoroscopic guidance and general anaesthesia or sedation and local anaesthesia. The mean follow-up period was 34 months (26.5 to 80). The patients were evaluated using the Enneking system for functional assessment and all the lesions were radiologically quantified into four grades. The mean age of patients was 15.6 years (3 to 38) and the mean number of injections was three (1 to 5). Ten patients were cured by a single injection. The mean reduction in size of the lesion (radiological healing) was found to be 76.6% (61.9% to 93.2%) with a mean clinical response of 84.5% (73.4% to 100%). Recurrence was seen in two patients (2.8%) within two years of treatment and both were treated successfully by further sclerotherapy. Percutaneous sclerotherapy with polidocanol is a safe alternative to conventional surgery for the treatment of an aneurysmal bone cyst. It can be used at surgically-inaccessible sites and treatment can be performed on an out-patient basis.

The authors performed a prospective study to evaluate limb length discrepancy in children following titanium elastic nailing for femoral shaft fractures. Thirty-seven children (28 boys and 9 girls) were included in the study. The average time to radiological union in our study was 7.8 weeks (range: 5 to 14). Results after 3 years were evaluated for 29 children. Limb lengthening was noted in the first year in 15 children: at the time of nail removal, an average of 10.6 mm and at the end of one year 8.7 mm. After three years only nine were lengthened an average of 2.7 mm. An average of 12.6 mm shortening was seen in four patients at the time of nail removal, reduced to 12.1 mm at the end of one year. After three years three remained short, an average of 11.7 mm. No limb length discrepancy was seen in 10 patients. We conclude that limb length discrepancy is common following elastic nailing in paediatric femoral fractures, with lengthening being more frequent than shortening. Lengthening tends to decline with time at an average rate of around 1.5 mm per year.

Actinomycosis of spine is a very rare disease. Very few cases have been studied and reported in the past. The dilemma of distinguishing the condition from other disorders relies on the competency of the treating physician and a proper knowledge of the subtle radiological differences between these disorders especially in underdeveloped and developing countries where tuberculosis still has a very strong foothold. A rare atypical case of actinomycosis of spine resembling tuberculosis is presented.

Tuberculosis is 1 of the most deadly diseases in the world. With an increase in the incidence of HIV worldwide, tubercular infections at unusual sites are being reported. Skeletal muscle tuberculosis without bony involvement is an extremely rare presentation of tuberculosis. A case of isolated tuberculosis of the vastus lateralis muscle without any evident primary focus in a 30-y-old immunocompetent female is presented.

Tuberculosis of the ilium is a rare identity, accounting for less than 1% of all skeletal tuberculosis. We report two such lesions in immunocompetent individuals. Tuberculosis remains an important differential diagnosis when faced with unusual or chronic bony lesions, especially in endemic areas, even in non-immunocompromised individuals. It can involve any site and affect people of any age.

Pyrazinamide (PZN) was administered to 10 patients of pulmonary tuberculosis (for 7 consecutive days) each day after an overnight fast. On 8th day serum levels and urinary elimination were measured at 2,4,6 and 8 hours. Simultaneous administration of isoniazid to same patients significantly decreased the peak serum concentration (Cmax). Although, time to peak serum concentration (Tmax) remained unaffected, serum half life (t1/2) prolonged, the elimination rate constant (Kel) and area under serum concentration time curve (AUC) decreased and apparent volume of distribution (Vd) and plasma clearance (Clp) of PZN increased significantly. However, the cumulative per cent dose of PZN excreted in urine was not changed significantly. Although, serum levels of PZN were decreased at 2, 4, 6, and 8 hours, PZN levels remained above minimum effective concentration thereby not affecting the therapeutic status of PZN administered in combination with isoniazid, if PZN is administered in moderate doses.