Electroencephalographic and imaging profile in a subacute sclerosing panencephalitis (SSPE) cohort: a correlative study.

OBJECTIVE: There are only a few studies correlating diverse radiological and EEG features of subacute sclerosing panencephalitis (SSPE). The objective of the study was to (a) describe EEG profile and (b) correlate it with the clinical and imaging data of patients with confirmed SSPE. METHODS: This study was conducted at a University teaching hospital in south India and involved 58 patients (M:F=37:21, age: 12.3, SD 4.8 years) of SSPE. Diagnosis of SSPE was based on the characteristic clinical manifestations, and raised IgG (1:625) anti-measles antibody in cerebrospinal fluid (CSF) by ELISA in all the patients. Scalp EEGs were recorded on 16 channel machines using standard parameters and procedures. The EEG, clinical and imaging data were reviewed. RESULTS: EEGs were frequently abnormal: typical (37) and atypical (21). Diffuse slowing of background activity (BGA) was noted in 46 records being asymmetrical in six. Periodic complexes were periodic (32), quasi-periodic (21) or a-periodic (4). Periodic complexes (PC) (amplitude: 370.7, SD 171.2 microV; duration - 1.7, SD 2.0 s; inter-complex interval: 8.4, SD 9.2s) were symmetrical in 39 and asymmetrical in 19. CT (32) and MRI (23) scans were normal in 16 patients while others had white matter (15), cerebral edema (8), cerebral atrophy (8), basal ganglia (2), and thalamic (2) changes. There was an independent association of frontally dominant slowing of BGA (p=0.04) and typical PCs (p=0.03) with the diffuse cerebral edema on imaging. White matter changes correlated with slowing of BGA (p=0.04), but not with typical PC (p=0.16). CONCLUSIONS: This study provides valuable insight into the structural and clinical correlates of EEG changes in SSPE. SIGNIFICANCE: Irrespective of the incidence of occurrence of SSPE in a community, a clinician should be aware of the wide spectra of EEG findings. This study also discusses the possible underlying structural and clinical correlates.     

Erythrodermic bullous pemphigoid

We report a case of erythrodermic type of bullous pemphigoid which is a rare variant of bullous pemphigoid. Our patient had a peculiar clinical presentation with bullae, erosions and extensive erythrodermic areas, and distinct direct immunofluorescent findings which included linear IgG and C3 deposits in the basement membrane and also IgG in the intercellular spaces. Very high levels of serum IgE were also detected in our patient.     

Heparin in haemorrhagic infarction in cerebral venous sinus thrombosis

Morbidity and mortality in puerperal cerebral venous thrombosis (CVT) can be reduced by arresting the progression of thrombosis using heparin. However, conventional dose of heparin requires monitoring of coagulation parameters and carries a risk of haemorrhage. The present study involved 56 patients of puerperal CVT with CT evidence of haemorrhagic infarction. Twenty nine of these patients received low dose heparin till 30th post-partum day or symptomatic relief. Their clinical features and severity were similar to 27 patients who did not receive heparin. The mortality and morbidity at discharge was significantly less (P < 0.001) in heparin treated group. There were no haemorrhagic complications. Low dose heparin is safe and effective in cerebral venous thrombosis, even with haemorrhagic infarction.     

Dapsone - the treatment of infectious and inflammatory diseases in dermatology

Dapsone is known as useful in the treatment of infectious diseases. The use of the drug in infectious and inflammatory diseases in dermatology is reviewed.     

From the Cover: Echolocating bats rely on an innate speed-of-sound reference

Animals must encode fundamental physical relationships in their brains. A heron plunging its head underwater to skewer a fish must correct for light refraction, an archerfish shooting down an insect must “consider” gravity, and an echolocating bat that is attacking prey must account for the speed of sound in order to assess its distance. Do animals learn these relations or are they encoded innately and can they adjust them as adults are all open questions. We addressed this question by shifting the speed of sound and assessing the sensory behavior of a bat species that naturally experiences different speeds of sound. We found that both newborn pups and adults are unable to adjust to this shift, suggesting that the speed of sound is innately encoded in the bat brain. Moreover, our results suggest that bats encode the world in terms of time and do not translate time into distance. Our results shed light on the evolution of innate and flexible sensory perception.

Every organism must reliably sense its environment in order to survive and reproduce (1). Some sensory systems are innate and unalterable (2), allowing for efficient use even by naïve newborn animals (3–5). Others require learning or experience-dependent development—usually during a critical period during ontogeny (6, 7), though sometimes retained through adulthood (8), allowing for adapting sensing to changing environments (9, 10). The ability to accurately estimate distances with sub-centimeter accuracy is a hallmark of bat echolocation (11–13). Bats achieve this accuracy by means of delay-tuned neurons—neurons that are activated by specific call–echo time delays, supposedly encoding target distance (14–19), although it should be noted that some work suggests that the tuning width of delay-tuned neurons might not allow the accuracy that bats exhibit in delay perception (20). Though delay tuning has been shown to be (at least partially) innate at the neural level (21), this has never been tested behaviorally. Namely, when a newborn bat takes off for the first time, does its brain correctly translate time delays into distance?Translating time into distance relies on a reference of the speed of sound (SOS). This physical characteristic of the environment is not as stable as it may seem. The SOS may change considerably due to various environmental factors such as humidity, altitude, and temperature (22). Bats (Chiroptera) are a specious and widely distributed order of highly mobile and long-lived animals. They therefore experience a range of SOSs (with more than 5% variation, see below) between species, among species, and even within the life of a single individual. We therefore speculated that the reference of the SOS may not be innate to allow for the environmentally dependent SOS experienced by each animal.To test this, we examined the acquisition of the SOS reference by exposing neonatal bats to an increased SOS environment from birth (Materials and Methods). We reared two groups of bats from birth to independent flight in two flight chambers: six bats in normal air (henceforth: “air pups”) and five bats in a helium-enriched air environment (Heliox), where the speed of sound was 15% higher (henceforth: “Heliox pups”). Notably, Heliox pups were never active and did not echolocate in non-Heliox environment (Materials and Methods). This 15% shift is higher than the ecological range and was chosen because it is high enough to enable us to document behavioral changes but low enough so as to allow the bats to function (that is, to fly despite the change in air density). In order to feed, the bats had to fly to a target positioned 1.3 m away from their wooden slit roost. Once the bats learned to fly to the target independently (after ca. 9 wk), we first documented their echolocation in the environment where they were brought up, and we then moved them to the other treatment for testing (Materials and Methods). Because bats adjust their echolocation parameters to the distance of the target, before and during flight (23), we used their echolocation to assess the bats’ target range estimates. If the SOS reference is learned based on experience, the bats raised in Heliox should have learned a faster reference, so that when they flew in normal air, they would have perceived the target as farther than it really was. We also ran the same experiments on adult bats to test adult plasticity.     

Health‐related quality of life among Darier’s disease patients

Background Darier’s disease (DD) is an autosomal dominant skin disorder characterized by persistent eruption of hyperkeratotic papules. The effect of DD on quality of life (QOL) has been assessed in only one study, which found no correlation between the Dermatology Life Quality Index (DLQI) score and clinical severity of the disease. The correlation between health‐related quality of life (HRQL) and other diseases and patient characteristics has not been studied. Objectives To examine the HRQL of patients with DD and to evaluate the association between HRQL scores and disease and patient characteristics. Methods A total of 74 DD patients completed three QOL questionnaires: DLQI, EQ‐5D, and one specially designed for the study. The data reported in this study were collected as part of a larger study on the clinical characteristics of DD; the socio‐demographic and clinical data were used in the statistical analysis of the current study. Results Mean DLQI was 5.41 ± 5.57 and the mean EQ‐Visual Analogue Scale (VAS), was 70.84 ± 19.25. DLQI and EQ‐VAS were significantly associated with skin area affected, disease severity, age at onset of DD and a seborrhoeic distribution pattern of DD. Stepwise linear regression showed skin area affected to be the most significant variable in the predication of DLQI (beta = 0.183; SE = 0.04; P < 0.001), and disease severity the most significant variable in the predication of EQ‐VAS (beta = ?9.15; SE = 3.21; P < 0.006). Conclusions Darier’s disease has a negative impact on HRQL of patients and the HRQL is associated with various disease characteristics, mainly skin area affected and clinical severity.     

Just Culture: Practical Implementation for Radiologist Peer Review

Peer review is a cornerstone of quality improvement programs and serves to support the peer learning process. Peer review in radiology incorporates the review of diagnostic imaging interpretation, interventional procedures, communication, and the evaluation of untoward patient events. A just culture is an environment in which errors and near-miss events are evaluated in a deliberately nonpunitive framework, avoiding a culture of blame and responsibility and focusing instead on error prevention and fostering a culture of continuous quality improvement. Adoption of a just culture requires careful attention to detail and relies on continuous coaching of individuals and teams to ensure future systems improvements and a culture of safety. The authors describe the practical implementation of a just culture framework for peer review in an academic radiology department and highlight its application to interpretive, noninterpretive, and procedural domains through case examples.