Mirrored-self misidentification in a patient with probable Alzheimer dementia

A 77-year-old-man who was a retired primary school teacher with probable Alzheimer dementia diagnosed after 2 years of progres sive short-term memory loss and predominant hippocampal atro phy onmagnetic resonance scan of the brain was brought to the out patient neurology clinic by his wife because of persistent ab normal behavior. The caretaker reported that he was frequently puzzled and astonished by his own mirror image. His medical his tory was remarkable for controlled hypertension and type 2 diabe tes. He did not have any personal or family history of psychiatric dis ease. There were no recent changes with the patient’s regular medications (rivastigmine, 9.5 mg daily transdermally; enalapril; and metformin). His wife described him as being a very quiet and relaxed person until the start of restrictions imposed by the COVID-19 pandemic, when he started with complaints of seeing a strangeman whenever he saw his face reflection in the mirror.info:eu-repo/semantics/publishedVersio

On the plausibility of late neuropsychiatric manifestations associated with the COVID-19 pandemic

Recognition of the association between acute and subacute different neuropsychiatric manifestations with the infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the cause of the ongoing coronavirus disease 2019 (COVID-19) pandemic, is progressively being recognized.info:eu-repo/semantics/publishedVersio

Pilocytic astrocytoma

Pilocytic astrocytoma (PA) is a benign tumour of childhood, often located in deep midline structures such as the brainstem and the cerebellum.1 Gross surgical resection is curative in the majority of patients.2 We report a case of recurrent PA diagnosed after an acute confusional state and rightsided spastic hemiparesis in a previously healthy 38-year-old woman. Her initial brain CT showed a left temporal mass causing obstructive hydrocephalus (figure 1A–C). Acute surgical extraction was performed (figure 1D) and the biopsy confirmed the diagnosis of PA grade I (WHO). The patient remained clinically asymptomatic for 1 year, when a progressive headache emerged. At this time, the neurological examination disclosed the presence of dysarthria and right-sided hemiparesis with facial involvement. Brain MRI showed the presence of recurrent PA (figure 1E, F). The patient again underwent surgery due to the mass effect and brainstem compression caused by the tumour, and the diagnosis of PA was reconfirmed histologically. The patient fully recovered after the intervention. PA occurs commonly in the first 2 decades of life.1 Information on the characteristics of PA in adulthood is scarce due to its rarity. Some studies indicate the adult prognosis to be similar to that in children, while others indicate that PA may show aggressive behaviour with tumour recurrence and death.2 Anaplastic features are associated with worse prognosis, but little is known about the value of genetic characterisation in adulthood PA.3 In conclusion, this case offers a unique description of adult onset PA in an atypical location outside the midline structures, and also provides an example of early recurrence

Dropped head and man-in-barrel syndrome in amyotrophic lateral sclerosis

We report a case of progressive symmetric brachial weakness followed by cervical muscle weakness. The electromyogram confirmed the diagnosis of amyotrophic lateral sclerosis. After 3 years the patient remained able to walk unassisted and without significant bulbar manifestations or upper neuron signs. The concomitant presence of dropped head syndrome and man-in-barrel syndrome in an amyotrophic lateral sclerosis patient makes our case unique.info:eu-repo/semantics/publishedVersio

IgG4: related disease presenting with headache and papilloedema

A previously healthy man aged 57years presented with a 4-week duration of predominant decubitus daily holocranial headache after minor head injury. Apart from unintentional weight loss of 8 pounds, there were no associated constitutional or systemic manifestations.The generalandneurologicalexamination was unremarkable. The funduscopy showed bilateral grade 2 papilloedema. Brain magnetic resonance (MRI) findings are shown in figure 1A–E. The brain MRI showed the presence of diffuse dural thickening (figure 1B–E) hypointense on unenhanced T1 imaging (figure 1B–F), with dural enhancement on T1-weighed imaging (figure 1B–F). The opening cerebrospinal fluid (CSF) pressure was 380mm Hg. With the exception of serum-elevated IgG4 immunoglobulin (IgG4=302 mg/dL) and elevated CSF protein level (68 mg/dL), the wide and extensive complementary study including body positron emission tomography scan, laboratory blood/CSF work-up was negative, excluding neoplastic, infectious, autoimmune and collagen vascular disorders. Meningeal biopsy showed fibrous thickening with moderate and predominant B and T cells inflammatory infiltrate. The patient improved with spinal taps evacuation and methylprednisolone treatment. Intracranial hypertrophic pachymeningitis is a severe and rare manifestation in the spectrum of IgG4-related diseases.12Biopsy of meninges is the gold standard for the diagnosis.3 Exclusion of systemic inflammatory disorders and demonstration elevated serum IgG4 can support the diagnosis in patients with typical diffuse dural thickening.13The disease respondsto immunosuppressive drugs such as corticosteroids, methotrexate, azathioprine, mycophenolate or cyclophosphamide.12Mass effect due to dural thickening can cause progressive neurological debilitation,blindness and cerebellarataxia.In the case of symptomatic hydrocephalus, surgical exploration for decompression maybe require

Ocorrência circadiária de crises em doentes com epilepsia

Tese de mestrado, Ciências do Sono, Faculdade de Medicina, Universidade de Lisboa, 2016A ocorrência de crises epiléticas em determinados tipos de epilepsia pode obedecer a um padrão circadiário não uniforme ou não aleatório. Pretendeu-se com este trabalho caracterizar o padrão circadiário de crises epiléticas em doentes com epilepsia focal submetidos a monitorização vídeo-eletroencefalográfica. Realizou-se uma revisão retrospetiva dos registos consecutivos de 123 doentes com epilepsia focal incluídos no programa de cirurgia da epilepsia, que realizaram a monitorização vídeo-eletroencefalográfica. As crises foram distribuídas em blocos de 3 e 4 horas ao longo de 24 horas. Somente doentes cuja localização da epilepsia foi definida o suficiente para serem candidatos a cirurgia da epilepsia foram incluídos. A idade média dos doentes foi de 37.7 anos, com desvio padrão de 11.5 anos, e mediana de 37. A maioria (70/56%) era do sexo feminino. Em termos etiológicos, a esclerose mesial temporal (86/69.9%) foi a forma mais comum de epilepsia focal. Tumores (18/14.6%), malformações do desenvolvimento cortical (7/5.7%), gliose reativa (5/4%), lesões vasculares (4/3.2), e perturbações da migração neuronal (3/2.6%) contribuíram como lesões causativas da epilepsia focal nesta série. A maior parte dos doentes teve a sua epilepsia focal topograficamente localizada ao lobo temporal mesial (102/83%) e no lobo temporal neocortical (13/11%). Os restantes doentes, agrupados na categoria epilepsia focal extra-temporal (8/6%) tinham lesões localizadas no lobo frontal (4) e parietal (4). Oitenta e quatro (68.3%) doentes foram operados, com evolução favorável em todos: classe 1 (74/88%), classe 2 (6/7.1%), e classe 3 (4/4.9%), validando a correta localização topográfica do foco epilético nesta série. Foi identificado um padrão não uniforme de distribuição de crises com origem no lobo temporal, em localização mesial e neocortical, com dois picos de ocorrência, tanto na distribuição por bins de 3 ou 4 horas, entre as 10:00–13:00/16:00-19:00 e 08:00-12:00/16:00-20:00 horas respetivamente (p=0.004). Não foi identificado nenhum padrão circadiário em doentes com crises com origem extra-temporal. A ocorrência circadiária de crises é reconhecida em determinados tipos de epilepsia, mas estudos sobre a ritmicidade da ocorrência de crises são escassos. Neste sentido, a sua replicação e validação é fundamental. O presente estudo confirma o padrão bimodal de crises na epilepsia com origem no lobo temporal. No entanto, o pico de crises varia entre os diferentes estudos, sugerindo que o ambiente, fatores exógenos rítmicos ou ainda zeitgebers sociais possam modular o ritmo endógeno circadiário de ocorrência de crises epilépticas. A caraterização da ritmicidade circadiária de crises pode influenciar o diagnóstico e o tratamento de determinados tipos de epilepsia, em concreto da epilepsia do lobo temporal, que é a forma mais comum de epilepsia refractária ao tratamento médico.The occurrence of seizures in specific types of epilepsies can follow a circadian non uniform or non-random pattern. This study aimed to characterize the circadian patterns of clinical seizures associated with focal epilepsy who underwent video-encephalography. We retrospectively reviewed the scalp EEG recordings from 125 consecutive adult patients with FE, included in our epilepsy surgery program and undergoing video-EEG. Distribution along 3 or 4 hour time blocks or bins throughout 24 hours. Only those patients in whom the epilepsy focus was defined and then candidates to epilepsy surgery were included and etiology were analyzed. The mean age was 37.7 years, with standard deviation of 11.5 years, median of 37. The majority were females (70/56%). The most common etiology was mesial temporal sclerosis (86/69.9%). Neoplasms (18/14.6%), malformations of cortical development (7/5.7%), reactive gliosis (5/4%), vascular lesions (4/3.2) and neuronal migration disorders (3/2.6%) also contributed as a causative lesions in this series. The majority of patient had their focal epilepsy located in the mesial temporal lobe (102/83%) and in the neocortical temporal lobe (13/11%). The remaining patients with extra-temporal lobe location (8/6%) had their lesions located in the frontal lobe (4) and parietal lobe (4). Favorable Engel outcome occurred in all patients submitted to surgery (n=84), class 1 (74/89%), class 2 (6/7.1%) and 3 (4/4.9%), supporting correct localization of epileptogenic zone in this series. Non-uniform seizure distribution was observed in seizures arising from the temporal lobe (mesial temporal lobe and neocortical temporal lobe), with two peaks in both 3 and 4 hours bins between 10:00–13:00/16:00-19:00 and 08:00-12:00/16:00-20:00 respectively (p=0.004) identified. No specific circadian pattern was identified in seizures from extra-temporal location. Circadian pattern is recognized in certain types of epilepsy, but studies on 24-h rhythmicity in seizure occurrence are scarce. Their replication and validation is therefore fundamental. Our study confirms the bimodal pattern of temporal lobe epilepsy independently of the nature of the lesion. However, peak times differ between different studies, suggesting that the ambient, or rhythmic exogenous factors or environmental/social zeitgebers, may modulate the endogenous circadian rhythms of seizures. Characterization of chronotypes can influence diagnosis and treatment in selected types of epilepsy, such as the case of temporal lobe epilepsy, which is the most common medical refractory epilepsy

Organizational factors determining access to reperfusion therapies in Ischemic Stroke-Systematic literature review

Background: After onset of acute ischemic stroke (AIS), there is a limited time window for delivering acute reperfusion therapies (ART) aiming to restore normal brain circulation. Despite its unequivocal benefits, the proportion of AIS patients receiving both types of ART, thrombolysis and thrombectomy, remains very low. The organization of a stroke care pathway is one of the main factors that determine timely access to ART. The knowledge on organizational factors influencing access to ART is sparce. Hence, we sought to systematize the existing data on the type and frequency of pre-hospital and in-hospital organizational factors that determine timely access to ART in patients with AIS. Methodology: Literature review on the frequency and type of organizational factors that determine access to ART after AIS. Pubmed and Scopus databases were the primary source of data. OpenGrey and Google Scholar were used for searching grey literature. Study quality analysis was based on the Newcastle-Ottawa Scale. Results: A total of 128 studies were included. The main pre-hospital factors associated with delay or access to ART were medical emergency activation practices, pre-notification routines, ambulance use and existence of local/regional-specific strategies to mitigate the impact of geographic distance between patient locations and Stroke Unit (SU). The most common intra-hospital factors studied were specific location of SU and brain imaging room within the hospital, and the existence and promotion of specific stroke treatment protocols. Most frequent factors associated with increased access ART were periodic public education, promotion of hospital pre-notification and specific pre- and intra-hospital stroke pathways. In specific urban areas, mobile stroke units were found to be valid options to increase timely access to ART. Conclusions: Implementation of different organizational factors and strategies can reduce time delays and increase the number of AIS patients receiving ART, with most of them being replicable in any context, and some in only very specific contexts.info:eu-repo/semantics/publishedVersio

Cognitive function, cerebral microbleeds, radiotherapy, and bevacizumab in survivors of pediatric brain tumors

We read with great interest a recent paper published by Roddy et al showing a high (48.8%) 5-year cumulative incidence of cerebral microbleeds (CMB) and its association with cognitive dysfunction in a group with pediatric brain tumors who received cranial radiation therapy (CRT).1 We believe that exposing the association of CMB (or the associated underlying microvascular pathology) with cognitive impairment in this population will help draw attention to the importance of the topic in this specific growing population.info:eu-repo/semantics/publishedVersio

Internuclear ophthalmoplegia, skew deviation and nystagmus from facial colliculus infarction: small lesion big trouble

Isolated internuclear ophthalmoplegia (INO) is an extremely rare presentation of stroke [1–3]. INO is defned by the presence of ipsilateral adduction paresis with contralateral dissociated nystagmus on horizontal gaze and is caused by strategic brainstem lesions involving the medial longitudinal fasciculus (MLF) [2, 3].info:eu-repo/semantics/publishedVersio

Determinants of quality of life after stroke in Southern Portugal: a cross sectional community-based study

Introduction: the perception of Quality of Life (QoL) has been used to evaluate the treatment and evolution of several pathologies. QoL evaluation allows a better understanding of the patient and his adaptation to the disease. An observational, community-based and descriptive correlational study was carried out to analyze stroke survivors’ perception of QoL. Methods: consecutive case-series of stroke survivors (≥3 months) followed in a single public primary health center (“Tavira Primary Health Centre”) from Algarve, southern Portugal. The Portuguese version of the World Health Organization Quality of life instrument was administered in 102 stroke survivors. Results: Perception of QoL was associated (p 2 = 0.406; F = 8.757; p 2 = 0.286; F = 5.536; p 2 = 0.357; F = 7.287; p 2 = 0.160; F = 3.156; p < 0.005) on environmental domain, emerged as predictors of QoL. Conclusion: Different socio-demographic, economic and health factors are associated with post-stroke QoL. Description of QoL contributes to the overall evaluation of the impact of stroke on health and should be a priority for health professionals.info:eu-repo/semantics/publishedVersio