Giant Cell Arteritis Presenting with Bilateral Subdural Haematomas of Arterial Origin

Giant cell arteritis or temporal arteritis is an inflammatory condition affecting medium to large sized vessels, particularly the cranial arteries. A 76-year-old man with no significant past medical history presented to the emergency department with a 3-week history of diffuse headaches associated with fever, loss of appetite, weight loss and general malaise. A CT scan of the brain showed bilateral shallow chronic low density subdural haematomas. A complete laboratory panel was unremarkable except for a raised erythrocyte sedimentation rate and elevated C-reactive protein. A transthoracic echocardiogram and CT scan of the body were unremarkable. MRI of the brain confirmed bilateral old subdural collections and showed marked vessel wall enhancement in the frontal branches of the left superficial temporal artery. A left temporal artery biopsy confirmed giant cell temporal arteritis. We speculate that a vasculitic process in the small subdural arteries may have contributed to our patient’s spontaneous subdural haematomas

Application of recent international epidemiological guidelines to a prospective study of the incidence of first seizures, newly-diagnosed epilepsy and seizure mimics in a defined geographic region in Ireland

Studies adherent to international guidelines and epilepsy classification are needed to accurately record the incidence of isolated seizures, epilepsy and seizure-mimics within a population. Because the diagnosis of epilepsy is largely made through clinical assessment by experienced physicians, seizures and epilepsy are susceptible to misdiagnosis. Previous epidemiological studies in epilepsy have not captured â seizure mimicsâ . We therefore sought to quantify the incidence of isolated seizures, epilepsy and seizure-mimics using the International League Against Epilepsy (ILAE) classification system. In this study multiple overlapping methods of case ascertainment were applied to a defined geographic region from January 1 to March 31, 2017 to identify all patients presenting with first seizures (provoked and unprovoked), new diagnoses of epilepsy and seizure mimics. Over a 3 month period, from a population of 542,869 adults and children, 442 potential presentations were identified, and 283 met the inclusion criteria. Radiology databases were the source of the largest number of individual cases (n = 153, 54%), while electroencephalogram (EEG) databases were the source of the highest number of unique-to-source cases (those not identified elsewhere, n = 60, 21%). No single case was picked up in every method of ascertainment. Among the 283 included presentations, 38 (13%) were classed as first provoked seizures, 27 (10%) as first unprovoked seizures, 95 (34%) as new diagnosis of epilepsy and 113 (40%) as seizure mimics. Ten (3%) presentations were indeterminate. We present and apply a rigorous study protocol for investigation of the incidence of first seizures, new diagnosis of epilepsy and seizure mimics in a geographically defined region which is adherent to recently published international guidelines for epidemiologic studies and epilepsy classification. We highlight the challenges in making a diagnosis of new-onset epilepsy in patients presenting with a first seizure using the current ILAE definition of epilepsy, when epilepsy can be diagnosed in situations where the treating physician anticipates the risk of further seizures exceeds 60%

The incidence of first seizures, epilepsy and seizure mimics in a geographically defined area.

To determine the incidence of first seizures, epilepsy and seizure mimics in a geographically defined area using the updated 2014 International League Against Epilepsy (ILAE) definition which allows an epilepsy diagnosis following a single seizure where risk of further seizures over the next 10 years is approximately 60% or more. This replaced the 1993 definition where epilepsy was diagnosed when a person had two or more seizures separated by 24 hours. Using multiple overlapping methods of case ascertainment followed by individual case classification by an epileptologist we identified all first seizures, new diagnosis of epilepsy, and seizure mimics occurring in a defined geographical area (population 542,868) 01/01/2017-12/31/2017. Incidence was age-standardised to the Standard European Population. We compared incidence rates when using the 2014 and 1993 ILAE definitions. When applying the 2014 ILAE definition of epilepsy the incidence of new diagnosis of epilepsy was 62 per 100,000 (age-standardised 74), compared to 41 per 100,000 (age-standardised 48) when applying the 1993 definition, and the difference was more pronounced at older ages. The incidence of all first seizures and of seizure mimics was 102 per 100,000 (age-standardised 123) and 94 per 100,000 (age-standardised 111), respectively. The most frequently encountered seizure mimic was syncope. Application of the 2014 ILAE definition of epilepsy resulted in higher incidence of new diagnosis of epilepsy compared to the 1993 definition. The incidence of seizure mimics almost equals that of all first seizures. Seizures, epilepsy and seizure mimics represent a significant burden to healthcare systems

Heritability of subcortical volumetric traits in mesial temporal lobe epilepsy.

OBJECTIVES: We aimed to 1) determine if subcortical volume deficits are common to mesial temporal lobe epilepsy (MTLE) patients and their unaffected siblings 2) assess the suitability of subcortical volumetric traits as endophenotypes for MTLE. METHODS: MRI-based volume measurements of the hippocampus, amygdala, thalamus, caudate, putamen and pallidium were generated using an automated brain reconstruction method (FreeSurfer) for 101 unrelated 'sporadic' MTLE patients [70 with hippocampal sclerosis (MTLE+HS), 31 with MRI-negative TLE], 83 unaffected full siblings of patients and 86 healthy control subjects. Changes in the volume of subcortical structures in patients and their unaffected siblings were determined by comparison with healthy controls. Narrow sense heritability was estimated ipsilateral and contralateral to the side of seizure activity. RESULTS: MTLE+HS patients displayed significant volume deficits across the hippocampus, amygdala and thalamus ipsilaterally. In addition, volume loss was detected in the putamen bilaterally. These volume deficits were not present in the unaffected siblings of MTLE+HS patients. Ipsilaterally, the heritability estimates were dramatically reduced for the volume of the hippocampus, thalamus and putamen but remained in the expected range for the amygdala. MRI-negative TLE patients and their unaffected siblings showed no significant volume changes across the same structures and heritability estimates were comparable with calculations from a healthy population. CONCLUSIONS: The findings indicate that volume deficits for many subcortical structures in 'sporadic' MTLE+HS are not heritable and likely related to acquired factors. Therefore, they do not represent suitable endophenotypes for MTLE+HS. The findings also support the view that, at a neuroanatomical level, MTLE+HS and MRI-negative TLE represent two distinct forms of MTLE

Genome-wide mapping for clinically relevant predictors of lamotrigine- and phenytoin-induced hypersensitivity reactions.

An association between carbamazepine-induced hypersensitivity and HLA-A*3101 has been reported in populations of both European and Asian descent. We aimed to investigate HLA-A*3101 and other common variants across the genome as markers for cutaneous adverse drug reactions (cADRs) attributed to lamotrigine and phenytoin

Heritability of subcortical volumetric traits in mesial temporal lobe epilepsy.

OBJECTIVES: We aimed to 1) determine if subcortical volume deficits are common to mesial temporal lobe epilepsy (MTLE) patients and their unaffected siblings 2) assess the suitability of subcortical volumetric traits as endophenotypes for MTLE. METHODS: MRI-based volume measurements of the hippocampus, amygdala, thalamus, caudate, putamen and pallidium were generated using an automated brain reconstruction method (FreeSurfer) for 101 unrelated 'sporadic' MTLE patients [70 with hippocampal sclerosis (MTLE+HS), 31 with MRI-negative TLE], 83 unaffected full siblings of patients and 86 healthy control subjects. Changes in the volume of subcortical structures in patients and their unaffected siblings were determined by comparison with healthy controls. Narrow sense heritability was estimated ipsilateral and contralateral to the side of seizure activity. RESULTS: MTLE+HS patients displayed significant volume deficits across the hippocampus, amygdala and thalamus ipsilaterally. In addition, volume loss was detected in the putamen bilaterally. These volume deficits were not present in the unaffected siblings of MTLE+HS patients. Ipsilaterally, the heritability estimates were dramatically reduced for the volume of the hippocampus, thalamus and putamen but remained in the expected range for the amygdala. MRI-negative TLE patients and their unaffected siblings showed no significant volume changes across the same structures and heritability estimates were comparable with calculations from a healthy population. CONCLUSIONS: The findings indicate that volume deficits for many subcortical structures in 'sporadic' MTLE+HS are not heritable and likely related to acquired factors. Therefore, they do not represent suitable endophenotypes for MTLE+HS. The findings also support the view that, at a neuroanatomical level, MTLE+HS and MRI-negative TLE represent two distinct forms of MTLE

Genomic microdeletions associated with epilepsy: not a contraindication to resective surgery.

Several recent reports of genomic microdeletions in epilepsy will generate further research; discovery of more microdeletions and other important classes of variants may follow. Detection of such genetic abnormalities in patients being evaluated for surgical treatment might raise concern that a genetic defect, possibly widely expressed in the brain, will affect surgical outcome.Journal ArticleResearch Support, Non-U.S. Gov'tFLWINSCOPUS: ar.jinfo:eu-repo/semantics/publishe