Efficacy of color lenses in abolishing photosensitivity: Beyond the one-type-fits-all approach?

OBJECTIVE: Red-light filtering lenses represent an additional option to medication in photosensitive epilepsy. Blue lenses (Clarlet Z1 F133) can dramatically reduce seizure frequency, with a substantial restriction in luminance that can limit their applicability in daily life. We investigated the efficacy of 4 blue lenses with higher transmittance and reduced chromatic distortion in abolishing the photoparoxysmal EEG response (PPR) compared to the gold-standard Z1 lenses. METHODS: We reviewed EEG data during photic-and pattern stimulation in 19 consecutive patients (6-39 years) with photosensitivity (PS). Stimulation was performed at baseline and while wearing Z1 and the four new lenses. Lenses were tested in the same session by asking the patient to wear them in a sequentially randomized fashion while stimulating again with the most provocative photic/pattern stimuli. The primary outcome was the change in the initial PPR observed for each lens, categorized as no change, reduction, and abolition. RESULTS: Photosensitivity was detected in 17 subjects (89.5%); pattern sensitivity (PtS) was identified in 14 patients (73.7%). The highest percentages of PPR abolition/reduction were observed with Z1, for both PS and PtS. Regarding the new lenses, B1 + G1 offered the best rates, followed by B1 + G2. B1 + G3 and B1 showed lower efficacy rates, particularly for PtS. In the comparative analysis, no significant differences in PPR suppression were detected between the five lenses for PS. For PtS, the capacity of Z1 for PPR abolition was significantly higher compared with B1 + G3 and B1. CONCLUSIONS: This preliminary study suggests efficacy of the new group of blue lenses with potentially greater tolerability, particularly in regions with fewer sunlight hours during winter. In line with the current trend for personalized approach to treatment, this study suggests that in some patients there might be scope in extending the testing to offer the lens with the higher transmittance effective in abolishing the PPR.

Analysis of the Attune tibial tray backside: A comparative retrieval study.

OBJECTIVES: The Attune total knee arthroplasty (TKA) has been used in over 600 000 patients worldwide. Registry data show good clinical outcome; however, concerns over the cement-tibial interface have been reported. We used retrieval analysis to give further insight into this controversial topic. METHODS: We examined 12 titanium (Ti) PFC Sigma implants, eight cobalt-chromium (CoCr) PFC Sigma implants, eight cobalt-chromium PFC Sigma rotating platform (RP) implants, and 11 Attune implants. We used a peer-reviewed digital imaging method to quantify the amount of cement attached to the backside of each tibial tray. We then measured: 1) the size of tibial tray thickness, tray projections, peripheral lips, and undercuts; and 2) surface roughness (Ra) on the backside and keel of the trays. Statistical analyses were performed to investigate differences between the two designs. RESULTS: There was no evidence of cement attachment on any of the 11 Attune trays examined. There were significant differences between Ti and CoCr PFC Sigma implants and Attune designs (p < 0.05); however, there was no significant difference between CoCr PFC Sigma RP and Attune designs (p > 0.05). There were significant differences in the design features between the investigated designs (p < 0.05). CONCLUSION: The majority of the earliest PFC Sigma designs showed evidence of cement, while all of the retrieved Attune trays and the majority of the RP PFC trays in this study had no cement attached. This may be attributable to the design differences of these implants, in particular in relation to the cement pockets. Our results may help explain a controversial aspect related to cement attachment in a recently introduced TKA design.Cite this article: A. Cerquiglini, J. Henckel, H. Hothi, P. Allen, J. Lewis, A. Eskelinen, J. Skinner, M. T. Hirschmann, A. J. Hart. Analysis of the Attune tibial tray backside: A comparative retrieval study. Bone Joint Res 2019;8:136-145. DOI: 10.1302/2046-3758.83.BJJ-2018-0102.R2.

A retrieval analysis of the Precice intramedullary limb lengthening system.

OBJECTIVES: The Precice nail is the latest intramedullary lengthening nail with excellent early outcomes. Implant complications have led to modification of the nail design. The aim of this study was to perform a retrieval study of Precice nails following lower-limb lengthening and to assess macroscopical and microscopical changes to the implants and evaluate differences following design modification, with the aim of identifying potential surgical, implant, and patient risk factors. METHODS: A total of 15 nails were retrieved from 13 patients following lower-limb lengthening. Macroscopical and microscopical surface damage to the nails were identified. Further analysis included radiology and micro-CT prior to sectioning. The internal mechanism was then analyzed with scanning electron microscopy and energy dispersive x-ray spectroscopy to identify corrosion. RESULTS: Seven male and three female patients underwent 12 femoral lengthenings. Three female patients underwent tibial lengthening. All patients obtained the desired length with no implant failure. Surface degradation was noted on the telescopic part of every nail design, less on the latest implants. Microscopical analysis confirmed fretting and pitting corrosion. Following sectioning, black debris was noted in all implants. The early designs were found to have fractured actuator pins and the pin and bearings showed evidence of corrosive debris. The latest designs showed evidence of biological deposits suggestive of fluid ingress within the nail but no corrosion. CONCLUSION: This study confirms less internal corrosion following modification, but evidence of titanium debris remains. We recommend no change to current clinical practice. However, potential reuse of the Precice nail, for secondary limb lengthening in the same patient, should be undertaken with caution.Cite this article: V. C. Panagiotopoulou, K. Davda, H. S. Hothi, J. Henckel, A. Cerquiglini, W. D. Goodier, J. Skinner, A. Hart, P. R. Calder. A retrieval analysis of the Precice intramedullary limb lengthening system. Bone Joint Res 2018;7:476-484. DOI: 10.1302/2046-3758.77.BJR-2017-0359.R1.

Assessment of the equivalence of a generic to a branded femoral stem.

AIMS: The aim of this study was to compare the design of the generic OptiStem XTR femoral stem with the established Exeter femoral stem. MATERIALS AND METHODS: We obtained five boxed, as manufactured, implants of both designs at random (ten in total). Two examiners were blinded to the implant design and independently measured the mass, volume, trunnion surface topography, trunnion roughness, trunnion cone angle, Caput-Collum-Diaphyseal (CCD) angle, femoral offset, stem length, neck length, and the width and roughness of the polished stem shaft using peer-reviewed methods. We then compared the stems using these parameters. RESULTS: We found that the OptiStems were lighter (p < 0.001), had a rougher trunnion surface (p < 0.001) with a greater spacing and depth of the machined threads (p < 0.001), had greater trunnion cone angles (p = 0.007), and a smaller radius at the top of the trunnion (p = 0.007). There was no difference in stem volume (p = 0.643), CCD angle (p = 0.788), offset (p = 0.993), neck length (p = 0.344), stem length (p = 0.808), shaft width (p = 0.058 to 0.720) or roughness of the polished surface (p = 0.536). CONCLUSION: This preliminary investigation found that whilst there were similarities between the two designs, the generic OptiStem is different to the branded Exeter design. Cite this article: Bone Joint J 2017;99-B:310-16.

Regression versus no regression in the autistic disorder: developmental trajectories.

Developmental regression is a complex phenomenon which occurs in 20-49% of the autistic population. Aim of the study was to assess possible differences in the development of regressed and non-regressed autistic preschoolers. We longitudinally studied 40 autistic children (18 regressed, 22 non-regressed) aged 2-6 years. The following developmental areas were considered fundamental in the first years of life, and were assessed at ages 2, 3, 4, 5, and 6: receptive and expressive language, communicative and request modalities, play activities, and mental age. Children who regressed showed lower mean performances than those who did not regress and, in the time intervals considered, non-regressed children improved their ratings in the above mentioned variables significantly more than regressed children.

An open-label study of controlled-release melatonin in treatment of sleep disorders in children with autism.

Long-term effectiveness of controlled-release melatonin in 25 children, aged 2.6-9.6 years with autism without other coexistent pathologies was evaluated openly. Sleep patterns were studied using Children's Sleep Habits Questionnaire (CSHQ) and sleep diaries at baseline, after 1-3-6 months melatonin treatment and 1 month after discontinuation. Sleep diary and CSHQ showed a more problematic sleep in autistic children compared with controls. During treatment sleep patterns of all children improved. After discontinuation 16 children returned to pre-treatment score, readministration of melatonin was again effective. Treatment gains were maintained at 12 and 24-month follow-ups. No adverse side effects were reported. In conclusion, controlled-release melatonin may provide an effective and well-tolerated treatment for autistic children with chronic sleep disorders.

Autism in tuberous sclerosis: evoked potential evidence for a deficit in auditory sensory processing.

OBJECTIVE: Autism is a frequent manifestation of tuberous sclerosis complex (TSC) being reported in up to 60% of the patients. Its presence is in association with cortical and subcortical lesions involving the temporal lobes. This study was designed to shed light on the functional mechanisms linking anatomical lesions of TSC and behavioural phenotype by investigating scalp recorded event related potentials to auditory stimuli. METHODS: Fourteen children with TSC, seven of which fulfilled the DSM IV criteria for autistic disorder were selected for this study. All of the subjects underwent high resolution MRI, EEG, brainstem auditory evoked potentials, cognitive and behavioural evaluation. Electrical evoked responses to two different pitches, presented with different probability (80% 1000 Hz, 20% 1500 Hz) were recorded from 21 scalp electrodes in the autistic and non-autistic subgroups, to assess central auditory processing and automatic memory. RESULTS: The first component of the long latency auditory response (N1) had a significantly prolonged latency with lower amplitude in all of the patients with autistic behaviour who, contrary to non-autistics had MRI lesions involving one or both temporal lobes. A mismatch negativity was detected in all subjects and had a longer latency in subjects with autistic behaviour. CONCLUSIONS: To our knowledge this is the first electrophysiological evidence of a deficit in auditory information processing and automatic memory in TSC patients with autistic behaviour.

Spike-induced interference in auditory sensory processing in Landau-Kleffner syndrome.

OBJECTIVES: Landau-Kleffner Syndrome (LKS) is an epileptic syndrome characterised by a deficit in language comprehension and production, paroxysmal epileptiform activity in the posterior temporal leads, and by the inconsistent presence of epileptic fits. Its interest lies in the fact that it stands as a model for the study of interference of epileptiform activity on cognitive function, although the pathophysiology of the decline in language skills that follows its onset has not yet been clarified. METHODS: We have recorded spike-triggered auditory evoked responses in a group of 6 children with LKS, to investigate whether the occurrence of individual EEG paroxysms is able per se to induce a decline in the response of the auditory cortex. RESULTS: Results have indicated that left hemisphere spikes are associated with a greater reduction in amplitude and an increase in latency of the NI, than spikes occurring in the right hemisphere. No stable change in the evoked response has been detected outside of the EEG paroxysm. CONCLUSIONS: We postulate EEG interictal activity is able to induce impairment in processing auditory information and that this may play a role in the pathogenesis of language deficit in LKS.

Frontal lobe epilepsy associated with tuberous sclerosis: electroencephalographic-magnetic resonance image fusioning.

We studied the topographic relationships between cortical and subcortical lesions shown on magnetic resonance images (MRI) and sources of epileptiform activity in a series of nine children with intractable epilepsy and tuberous sclerosis complex. Although video-electroencephalographic (EEG) monitoring was suggestive of a frontal seizure onset, interictal EEG was, in seven of nine cases, in the form of apparently bisynchronous discharges. In all cases, the use of a short time lag estimation procedure based on a nonlinear correlation function between surface recorded EEG signals allowed the detection of a lateralized onset of EEG paroxysmal activity. Furthermore, a computerized method based on a source localization EEG-MRI image fusioning procedure, has revealed a topographic concordance between well-defined frontal cortical lesions shown on MRI and site of onset of paroxysmal discharges. Lennox-like EEG patterns frequently reported in children with tuberous sclerosis complex could be the result of the tendency of frontal tubers to induce secondary bilateral synchrony, with implications in the medical and eventually surgical management of the often drug-resistant associated seizures.

Computerized electroencephalographic assessment of congenital brain infarction.

We studied 12 children (8 female and 4 male) aged 2.2-14.3 years, whose computed tomographic (CT) examination had shown evidence of malacic and/or porencephalic outcomes of early vascular brain infarction. Topographic spectral electroencephalographic (EEG) analysis was performed in all patients in the awake state. The following spectral EEG variables were studied: topography, absolute and relative power of delta, theta, alpha, beta bands, overall power, and peak alpha frequency asymmetries. The results of topographic spectral EEG analysis were compared with the localization and nature of lesions as detected by CT scans. Depending on the nature of the lesions, we were able to identify two different spectral patterns. Porencephalic cysts were characterized by an increase in delta and theta bands in the areas surrounding the lesion sites, as identified by CT. Spectral EEG patterns of malacic outcomes resulted in a focal increase in theta and delta band power, corresponding to the topography of lesions. Moreover, in 9/12 subjects an asymmetry of alpha rhythm in occipital leads was found homolaterally to the lesion sites, associated with a decrease in power, without any CT evidence of an occipital lesion.

Computerized EEG topography in childhood migraine between and during attacks.

Topographic EEG mapping was performed in 58 migrainous children (mean age: 12.9 years; 39 without, 19 with aura) between attacks. Ten children were also recorded during an attack with visual aura. Between attacks there were no significant differences between migraineurs and age-matched controls. During visual aura a decrease in occipital alpha power contralateral to the affected hemifield was found in all patients. This was followed by a bilateral frontal increase in delta power, and, during the headache, by an increased delta activity in posterior-temporal and occipital electrode sites. The possible brain mechanisms underlying these EEG changes are discussed.

Tuberous sclerosis: relationships between topographic mapping of EEG, VEPs and MRI findings.

A group of 10 patients suffering from tuberous sclerosis (TS) and epilepsy was studied by means of topographic mapping of EEG and visual evoked potentials. The localizing ability of the technique was compared to the topography of hyperintense T2-dependent areas seen on magnetic resonance images (MRI). Data were collected from 19 electrodes, free from interictal EEG transients and artifacts, spectral analysis was performed using the FFT algorithm and color maps were produced by specialized equipment. Data were analyzed with respect to the presence of interhemispheric asymmetries and significant differences with age-matched normal controls. The topography of slow frequency components was the best clue for localization, with a concordance between imaging and spectral EEG data in 76% of the lesions detected by MRI. In 7 patients topographic mapping revealed spectral abnormalities in areas where MRI was not able to detect morphological lesions. These abnormalities were characterized by an increased delta (5 patients), theta (4 patients), or lowered ipsilateral alpha (1 patient) power. Although full agreement between imaging and electrophysiological data was not observed, we believe that in patients with TS the study of EEG rhythm activity can add valuable information to the visual inspection of the tracings.