Subtrochanteric atypical femoral fracture induced solely by glucocorticoid without bisphosphonate treatment: a case report.

An AFF is a form of stress fracture induced by excessive physiologic repetitive stress over the bone remodeling capacity. Although glucocorticoid administration is a known risk factor for AFF, no case of AFF with glucocorticoid administration as the only risk factor has been previously reported. In this report, we aimed to highlight the risk of AFF associated with long-term administration of glucocorticoids, and the importance of surveillance and correction of risk factors in patients undergoing long-term glucocorticoid therapy. A 58-year-old male patient was diagnosed with subtrochanteric AFF. He had no medical history of any condition that might disrupt bone metabolism and no known risk factors for AFF, except for long-term administration of glucocorticoid. After fixation of the fracture, the glucocorticoid was replaced with an alternative medication. Although AFF is notorious for delayed union or nonunion, complete union of the fracture was obtained at 14 months postoperatively. This case brought to our attention the possibility that glucocorticoids alone may be responsible for inducing AFF and highlighted the importance of regular assessments in case of necessity of glucocorticoid administration. Additionally, correction of risk factors might expedite the union of AFF.

A focused update on preventing ceramic fractures in hip arthroplasty: is the 'cup' half full?

Ceramic bearings have several desirable properties, such as resistance to wear, hardness, and biocompatibility, that favour it as an articulating surface in hip arthroplasty. However, ceramic fracture remains a concern. We have reviewed the contemporary literature, addressing the factors that can influence the incidence of ceramic bearing surface fracture. Cite this article: Bone Joint J 2019;101-B:897-901.

Ultrasound-guided versus blind temporomandibular joint injections: a pilot cadaveric evaluation.

Temporomandibular disorders are painful conditions that require precise injection therapy in selected patients. This pilot cadaveric study was undertaken to compare the accuracy of temporomandibular joint (TMJ) injection between the anatomical landmark-based (blind) technique and an ultrasound-guided technique. TMJ injections using the blind technique or the ultrasound-guided technique were performed in 10 non-embalmed cadavers. After dissection, the accuracy of the TMJ injections was found to be significantly greater for the ultrasound-guided injections than for the blind technique (blind 55% vs. ultrasound 95%, P=0.008). For injections into the upper joint space of the TMJ, the success rate of the injection was comparable for the two techniques (blind 80% vs. ultrasound 100%, P=0.474). However, ultrasound-guided injections into the lower joint space had a much higher success rate than the blind technique (blind 30% vs. ultrasound 90%, P=0.020). The blind technique was associated with a considerable proportion of failed or inappropriate injections, especially for lower joint space injections. Ultrasound-guided TMJ injections were accomplished with a higher accuracy than the conventional blind technique, especially in the case of injections targeting the lower joint space of the TMJ.

Teriparatide therapy for severe, refractory osteoradionecrosis of the jaw.

Although osteoradionecrosis (ORN) is a serious complication of craniofacial radiotherapy, the current management methods remain suboptimal. Teriparatide (TPTD), a recombinant human parathyroid hormone (1-34), has shown beneficial effects on osseous regeneration in medication-related osteonecrosis of the jaw or periodontitis. However, TPTD therapy in irradiated bones has not been indicated yet because of the theoretical risk of osteosarcoma seen in rat models. Hence, we first report here two patients with tongue cancer with late-emerging ORN who were successfully treated with TPTD for 4-6 months with serum calcium and vitamin D supplementation. In contrast to the usual progress of ORN, the bone defect regenerated well and bone turnover markers including serum C-terminal telopeptide of type 1 collagen and osteocalcin were restored with TPTD therapy. Our experience might suggest that TPTD therapy with careful monitoring can provide an effective treatment option for patients with ORN in select refractory cases, with the benefits outweighing the potential risks.

Association of benign recurrent vertigo and migraine in 208 patients.

The aim of this study was to determine the association of benign recurrent vertigo (BRV) and migraine, using standardized questionnaire-based interview of 208 patients with BRV recruited through a University Neurotology clinic. Of 208 patients with BRV, 180 (87%) met the International Classification of Headache Disorders 2004 criteria for migraine: 112 migraine with aura (62%) and 68 without aura (38%). Twenty-eight (13%) did not meet criteria for migraine. Among patients with migraine, 70% experienced headache, one or more auras, photophobia, or auditory symptoms with some or all of their vertigo attacks, meeting the criteria for definite migrainous vertigo. Thirty per cent never experienced migraine symptoms concurrent with vertigo attacks. These met criteria for probable migrainous vertigo. Among patients without migraine, 21% experienced either photophobia or auditory symptoms with some or all of their vertigo attacks; 79% experienced only isolated vertigo. The age of onset and duration of vertigo attacks did not differ significantly between patients with (34 +/- 1.2 years) and patients without migraine (31 +/- 3.0 years). In patients with migraine, the age of onset of migraine headache preceded the onset of vertigo attacks by an average of 14 years and aura preceded vertigo by 8 years. The most frequent duration of vertigo attacks was between 1 h and 1 day. Benign recurrent vertigo is highly associated with migraine, but a high proportion of patients with BRV and migraine never have migraine symptoms during their vertigo attacks. Other features such as age of onset and duration of vertigo are similar between patients with or without migraine.

Of brain and bone: the unusual case of Dr. A.

Frontotemporal dementia (FTD) is a clinical syndrome characterized by progressive decline in social conduct and a focal pattern of frontal and temporal lobe damage. Its biological basis is still poorly understood but the focality of the brain degeneration provides a powerful model to study the cognitive and anatomical basis of social cognition. Here, we present Dr. A, a patient with a rare hereditary bone disease (hereditary multiple exostoses) and FTD (pathologically characterized as Pick's disease), who presented with a profound behavioral disturbance characterized by acquired sociopathy. We conducted a detailed genetic, pathological, neuroimaging and cognitive study, including a battery of tests designed to investigate Dr. A's abilities to understand emotional cues and to infer mental states and intentions to others (theory of mind). Dr. A's genetic profile suggests the possibility that a mutation causing hereditary multiple exostoses, Ext2, may play a role in the pattern of neurodegeneration in frontotemporal dementia since knockout mice deficient in the Ext gene family member, Ext1, show severe CNS defects including loss of olfactory bulbs and abnormally small cerebral cortex. Dr. A showed significant impairment in emotion comprehension, second order theory of mind, attribution of intentions, and empathy despite preserved general cognitive abilities. Voxel-based morphometry on structural MRI images showed significant atrophy in the medial and right orbital frontal and anterior temporal regions with sparing of dorsolateral frontal cortex. This case demonstrates that social and emotional dysfunction in FTD can be dissociated from preserved performance on classic executive functioning tasks. The specific pattern of anatomical damage shown by VBM emphasizes the importance of the network including the superior medial frontal gyrus as well as temporal polar areas, in regulation of social cognition and theory of mind. This case provides new evidence regarding the neural basis of social cognition and suggests a possible genetic link between bone disease and FTD.

Generation of intense proton beams from plastic targets irradiated by an ultraintense laser pulse.

Proton beams generated from thin aluminum and Mylar foil targets that are irradiated by a 30fs Ti:sapphire laser pulse with an intensity of 2.2x10;{18}Wcm;{2} were investigated. Protons from the Mylar targets were observed to have an energy higher by a factor of 2 and were higher in number by an order of magnitude as compared with those generated from the aluminum targets. The maximum proton energy of 1.3+/-0.12MeV obtained from the Mylar target was found to be similar with previous observations that used laser pulses with different intensities. To address the anomalous behavior of the maximum proton energy for plastic targets, an acceleration model is proposed. In this model, the protons are accelerated by a resistively induced electric field in the front of the target, which can account for the experimental observations.

Silver-mirror-based multipass preamplifier for a broadband terawatt Ti:sapphire laser.

We have developed a silver-mirror-based multipass preamplifier for a broadband amplification in a terawatt Ti:sapphire laser. With the extremely broad bandwidth of the silver mirrors, a very broad amplified spectrum can be generated at an amplified energy of 4 mJ; the amplified spectral width is 65 nm at half maximum and 160 nm at -25 dB without any spectral shaping technique. Such a broad amplification can be explained well by the simulation that includes gain narrowing and gain saturation. Even after a further amplification to an energy of 600 mJ, the amplified spectrum is broad enough to support an approximately 20 fs transform-limited pulse duration.

The relevance of migraine in patients with Menière's disease.

CONCLUSION: Coexistent migraine affects relevant clinical features of patients with Ménière's disease (MD). OBJECTIVE: Epidemiological studies have shown an association between migraine and MD. We sought to determine whether the coexistence of migraine affects any clinical features in patients with MD. PATIENTS AND METHODS: In this retrospective case-control study of University Neurotology Clinic patients, 50 patients meeting 1995 AAO-HNS criteria for definite MD were compared to 18 patients meeting the same criteria in addition to the 2004 IHS criteria for migraine (MMD). All had typical low frequency sensorineural hearing loss and episodes of rotational vertigo. Outcome measures included: sex, age of onset of episodic vertigo or fluctuating hearing loss, laterality of hearing loss, aural symptoms, caloric responses, severity of hearing loss, and family history of migraine, episodic vertigo or hearing loss. RESULTS: Age of onset of episodic vertigo or fluctuating hearing loss was significantly lower in patients with MMD (mean +/- 1.96*SE = 37.2 +/- 6.3 years) than in those with MD (mean +/- 1.96*SE = 49.3 +/- 4.4 years). Concurrent bilateral aural symptoms and hearing loss were seen in 56% of MMD and 4% of MD patients. A family history of episodic vertigo was seen in 39% of MMD and 2% of MD patients.

Adult-onset hemiplegic migraine with cortical enhancement and oedema.

We present genetically identical twin patients who experienced late-onset migraine with visual and somatosensory auras and later developed hemiplegic migraines associated with severe cortical oedema and enhancement. Both positron emission tomography and electroencephalography showed an increase in activity contralateral to the hemiplegic side. Brain biopsy during the attack showed reactive astrogliosis and microgliosis. Mutations in CACNA1A, ATP1A2, SLC1A3 and NOTCH3 were ruled out by sequencing. This report shows the clinical and genetic evaluation of a severe form of familial hemiplegic migraine as well as the evolution of the imaging changes.

Episodic vertical oscillopsia with progressive gait ataxia: clinical description of a new episodic syndrome and evidence of linkage to chromosome 13q.

We describe four families with late onset episodic vertical oscillopsia and progressive gait ataxia. Probands presented between the ages of 40 and 64 years with initial symptoms of episodic vertical oscillopsia and interictal downbeat nystagmus. A mild gait ataxia developed over several years. Triggers included physical exertion, alcohol and caffeine. Patients did not respond to acetazolamide. Genetic screening for episodic ataxia types 1 and 2, and spinocerebellar ataxias 1, 2, 3 and 6 were negative. Using ancestral identity by descent analysis and dense single nucleotide polymorphism (SNP) genotyping throughout the genome, an interval of 28.6 cM (approximately 14.2 Mb) on chromosome 13q12.11-q13.3, composed of 1259 SNPs, was shared between affected individuals in two of the four families and highlighted a region of suggestive linkage (LOD >2.7).

Genetic heterogeneity of autosomal dominant nonprogressive congenital ataxia.

We studied a family with nonprogressive congenital ataxia (NPCA) previously reported in 1985. Follow-up evaluation documented a nonprogressive course. Older family members developed ataxic spells and vertical oscillopsia triggered by stress and exercise. Linkage analysis using a 10K single-nucleotide polymorphism array found suggestive linkage to four loci on chromosomes 1q44, 5q35.1-35.3, 7q36.2-36.3, and 9q31.2-32 and ruled out linkage to the NPCA locus on 3p, proving genetic heterogeneity for autosomal dominant NPCA.

Relativistic nonlinear Thomson scattering as attosecond x-ray source.

Relativistic, nonlinear Thomson scattering by an electron of an intense laser field has been investigated by computer simulation. Under a laser field with a pulse duration of 20-fs full width at half maximum and an intensity of 10(20) W/cm(2), the motion of an electron is highly relativistic and generates an ultrashort radiation of 2 as with photon energies from 100 to 600 eV. An interesting modulated structure of the spectrum is observed and analyzed. A radiation produced by the zigzag motion of an electron under a linearly polarized laser has better characteristics than by a helical motion under a circularly polarized laser pulse in terms of an angular divergence and an energy spectrum. The effect of ion field in a plasma was also investigated, which shows that for a laser intensity of 10(20) W/cm(2), the ion field due to an ion density of up to 7 x 10(18) cm(-3) can be ignored during the laser pulse.

Local structure and tunneling kinetics of ammonium aluminum alum by infrared hole-burning.

The title compound, crystallized with a few percent of deuterium, contains some NH(3)D(+) and HOD. At low temperatures, five N-D stretch bands are observed. These belong to two ammonium sites with apparent C(s) symmetry in the low-temperature phase. The N-D bands can be hole-burned with an infrared laser. Burning bands belonging to the A-sites transforms some of them to the higher-energy B-sites. The A- and B-sites probably differ from each other by the arrangement of water and sulfate about the ammonium.

Wnt signaling and an APC-related gene specify endoderm in early C. elegans embryos.

In a 4-cell stage C. elegans embryo, signaling by the P2 blastomere induces anterior-posterior polarity in the adjacent EMS blastomere, leading to endoderm formation. We have taken genetic and reverse genetic approaches toward understanding the molecular basis for this induction. These studies have identified a set of genes with sequence similarity to genes that have been shown to be, or are implicated in, Wnt/Wingless signaling pathways in other systems. The C. elegans genes described here are related to wnt/wingless, porcupine, frizzled, beta-catenin/armadillo, and the human adenomatous polyposis coli gene, APC. We present evidence that there may be partially redundant inputs into endoderm specification and that a subset of these genes appear also to function in determining cytoskeletal polarity in certain early blastomeres.