Epidemiology of subdural haemorrhage during infancy : A population-based register study

Objectives To analyse subdural haemorrhage (SDH) during infancy in Sweden by incidence, SDH category, diagnostic distribution, age, co-morbidity, mortality, and maternal and perinatal risk factors; and its association with accidents and diagnosis of abuse. Methods A Swedish population-based register study comprising infants born between 1997 and 2014, 0-1 years of age, diagnosed with SDH-diagnoses according to the (International Classification of Diseases, 10th version (ICD10), retrieved from the National Patient Register and linked to the Medical Birth Register and the Death Cause Register. Outcome measures were: 1) Incidence and distribution, 2) co-morbidity, 3) fall accidents by SDH category, 4) risk factors for all SDHs in the two age groups, 0-6 and 7-365 days, and for ICD10 SDH subgroups: S06.5 (traumatic SDH), I62.0 (acute nontraumatic), SDH and abuse diagnosis. Results Incidence of SDH was 16.5 per 100 000 infants (n = 306). Median age was 2.5 months. For infants older than one week, the median age was 3.5 months. Case fatality was 6.5%. Male sex was overrepresented for all SDH subgroups. Accidental falls were reported in 1/3 of the cases. One-fourth occurred within 0-6 days, having a perinatal risk profile. For infants aged 7-365 days, acute nontraumatic SDH was associated with multiple birth, preterm birth, and small-for-gestational age. Fourteen percent also had an abuse diagnosis, having increased odds of being born preterm, and being small-for-gestational age. Conclusions The incidence was in the range previously reported. SDH among newborns was associated with difficult birth and neonatal morbidity. Acute nontraumatic SDH and SDH with abuse diagnosis had similar perinatal risk profiles. The increased odds for acute nontraumatic SDH in twins, preterm births, neonatal convulsions or small-for-gestational age indicate a perinatal vulnerability for SDH beyond 1st week of life. The association between prematurity/small-for-gestational age and abuse diagnosis is intriguing and not easily understood.Peer reviewe

Normative spatiotemporal fetal brain maturation with satisfactory development at 2 years

Maturation of the human fetal brain should follow precisely scheduled structural growth and folding of the cerebral cortex for optimal postnatal function1 . We present a normative digital atlas of fetal brain maturation based on a prospective international cohort of healthy pregnant women2 , selected using World Health Organization recommendations for growth standards3 . Their fetuses were accurately dated in the first trimester, with satisfactory growth and neurodevelopment from early pregnancy to 2 years of age4,5 . The atlas was produced using 1,059 optimal quality, three dimensional ultrasound brain volumes from 899 of the fetuses and an automated analysis pipeline6–8 . The atlas corresponds structurally to published magnetic resonance images9 , but with finer anatomical details in deep grey matter. The between study site variability represented less than 8.0% of the total variance of all brain measures, supporting pooling data from the eight study sites to produce patterns of normative maturation. We have thereby generated an average representation of each cerebral hemisphere between 14 and 31 weeks’ gestation with quantification of intracranial volume variability and growth patterns. Emergent asymmetries were detectable from as early as 14 weeks, with peak asymmetries in regions associated with language development and functional lateralization between 20 and 26 weeks’ gestation. These patterns were validated in 1,487 three-dimensional brain volumes from 1,295 different fetuses in the same cohort. We provide a unique spatiotemporal benchmark of fetal brain maturation from a large cohort with normative postnatal growth and neurodevelopment

The application of cortical layer markers in the evaluation of cortical dysplasias in epilepsy

The diagnostic criteria for focal cortical dysplasia type I (FCD I) remain to be well and consistently defined. Cortical layer-specific markers (CLM) provide a potential tool for the objective assessment of any dyslamination. We studied expression patterns of recognised CLM using immunohistochemistry for N200, ER81, Otx1, Map1b (subsets of V/VI projection neurones), Pax6, Tbr1, Tbr2 (differentially expressed in cortical neurones from intermediate progenitor cells), Cux 1 (outer cortical layers) and MASH1 (ventricular zone progenitors). Dysplasia subtypes included FCD I and II, dysplasias adjacent to hippocampal sclerosis (HS) or dysembryoplastic neuroepithelial tumours (DNTs); all were compared to neonatal and adult controls. Laminar expression patterns in normal cortex were observed with Tbr1, Map1b, N200 and Otx1. FCDI cases in younger patients were characterised by abnormal expression in layer II for Tbr1 and Otx1. FCDII showed distinct labelling of balloon cells (Pax6, ER81 and Otx1) and dysmorphic neurones (Tbr 1, N200 and Map1b) supporting origins from radial glia and intermediate progenitor cells, respectively. In temporal lobe sclerosis cases with dysplasia adjacent to HS, Tbr1 and Map1b highlighted abnormal orientation of neurones in layer II. Dyslamination was not confirmed in the perilesional cortex of DNT with CLM. Finally, immature cell types (Otx1, Pax6 and Tbr2) were noted in varied pathologies. One possibility is activation of progenitor cell populations which could contribute to the pathophysiology of these lesions

26 cm fall caught on video causing subdural hemorrhages and extensive retinal hemorrhages in an 8‐month‐old infant

Key Clinical Message Severe, too many to count retinal hemorrhages (RH) in infants have been associated with abusive head trauma, but can occur in short falls. An 8‐month‐old male fell backward from a height of 26 cm, landing on his buttocks then hitting the back of his head on a vinyl floor. The fall was videotaped. Acute subdural hemorrhages were found along with extensive, too many to count intra‐RH in both eyes. Falls from small heights on to the occiput can lead to extensive RH of the type often associated with abusive head trauma

A clinicopathological study of inclusion body myositis

Sporadic inclusion body myositis (IBM) is the commonest acquired myopathy in individuals aged over 50 years. Currently, diagnosis is based largely upon specific muscle biopsy findings, though these are thought to lack sensitivity. More recently, many abnormal protein aggregates have been described in IBM and some have been proposed as diagnostic markers. However, their diagnostic utility is untested and how they relate to the pathogenesis is uncertain. The clinical characteristics, pathological findings and disease course in 67 patients with histopathologically diagnosed and clinically diagnosed IBM were compared and revealed that patients with IBM have a characteristic pattern of weakness at presentation that is independent of the pathological findings. Current diagnostic pathological features are more common in older patients and therefore may be linked to disease duration. The pathology of IBM and the diagnostic utility of protein aggregates in IBM were studied in six clinicopathologically typical cases of IBM. Protein aggregates immunoreactive for p62, TDP-43, myotilin, α B-crystallin and ubiquitin were more abundant than the current diagnostic pathological features. The diagnostic potential of these pathological features was then assessed in pathologically typical and atypical IBM and controls. This revealed that staining for p62, MHC Class I, CD8 positive lymphocytes and mitochondrial abnormalities could support the diagnosis and should be included in diagnostic criteria for IBM. Laser microdissection and label-free mass spectrometry were used to validate the histological findings. The presence of nuclear and aggresomal proteins in rimmed vacuoles indicated that they are derived from myonuclei. Increased protein synthesis and ER stress suggest that abnormal protein aggregates may be the result of abnormal protein production. In conclusion, this thesis emphasises that a diagnosis of IBM can be confidently made on clinical grounds, with a supportive muscle biopsy. The histological and proteomic findings reveal that there is a spectrum of pathological features most likely associated with the disease duration and that adherence to the current pathological based diagnostic criteria may have led to some erroneous conclusions about the pathogenesis of this enigmatic disease.This thesis is not currently available in ORA

Do Inter-Country Differences in the Frequency of Abusive Head Trauma Reflect Different Proportions of Overdiagnosis of Abuse or True Differences in Abuse?

Non peer reviewe

Shaken Baby Syndrome, Abusive Head Trauma, and Actual Innocence: Getting it Right

In the past decade, the existence of shaken baby syndrome (SBS) has been called into serious question by biomechanical studies, the medical and legal literature, and the media. As a result of these questions, SBS has been renamed abusive head trauma (AHT). This is, however, primarily a terminological shift: like SBS, AHT refers to the two-part hypothesis that one can reliably diagnose shaking or abuse from three internal findings (subdural hemorrhage, retinal hemorrhage, and encephalopathy) and that one can identify the perpetrator based on the onset of symptoms. Over the past decade, we have learned that this hypothesis fits poorly with the anatomy and physiology of the infant brain, that there are many natural and accidental causes for these findings, and that the onset of symptoms does not reliably indicate timing. In the last volume of this journal, Dr. Sandeep Narang marshaled the arguments and evidence that he believes support the diagnostic specificity of the medical signs that are used to diagnose SBS/AHT. Dr. Narang does not dispute the alternative diagnoses but nonetheless argues that, in the absence of a proven alternative, the SBS/AHT hypothesis is sufficiently reliable to support criminal convictions. The cited studies do not, however, support this position since they assume the validity of the hypothesis without examining it and classify cases accordingly, often without considering alternative diagnoses. To address this problem, Dr. Narang argues that, in diagnosing SBS/AHT, we should rely on the judgment of child abuse pediatricians and other clinicians who endorse the hypothesis. Reliance on groups that endorse a particular hypothesis is, however, antithetical to evidence-based medicine and Daubert, which require an objective assessment of the scientific evidence. In the past decades, thousands of parents and caretakers have been accused-and many convicted-of abusing children based on a hypothesis that is not scientifically supported. While we must do everything in our power to protect children, we must refrain from invoking abuse as a default diagnosis for medical findings that are complex, poorly understood, and have a wide range of causes, some doubtlessly yet unknown. To this end, we are calling for collaboration between the medical and legal communities for the sole purpose of getting it right

Thrombosis is not a marker of bridging vein rupture in infants with alleged abusive head trauma

Aim Thrombosis of bridging veins has been suggested to be a marker of bridging vein rupture, and thus AHT, in infants with subdural haematoma. Methods This is a non-systematic review based on Pubmed search, secondary reference tracking and authors’ own article collections. Results Radiological studies asserting that imaging signs of cortical vein thrombosis were indicative of traumatic bridging vein rupture were unreliable as they lacked pathological verification of either thrombosis or rupture, and paid little regard to medical conditions other than trauma. Autopsy attempts at confirmation of ruptured bridging veins as the origin of SDH were fraught with difficulty. Moreover, microscopic anatomy demonstrated alternative non-traumatic sources of a clot in or around bridging veins. Objective pathological observations did not support the hypothesis that a radiological finding of bridging vein thrombosis was the result of traumatic rupture by AHT. No biomechanical models have produced reliable and reproducible data to demonstrate that shaking alone can be a cause of bridging vein rupture. Conclusion There is no conclusive evidence supporting the hypothesis that diagnostic imaging showing thrombosed bridging veins in infants correlates with bridging vein rupture. Hence, there is no literature support for the use of thrombosis as a marker for AHT