MedSecurance Project: Advanced Security-for-Safety Assurance for Medical Device IoT (IoMT).

The MedSecurance project focus on identifying new challenges in cyber security with focus on hardware and software medical devices in the context of emerging healthcare architectures. In addition, the project will review best practice and identify gaps in the guidance, particularly the guidance stipulated by the medical device regulation and directives. Finally, the project will develop comprehensive methodology and tooling for the engineering of trustworthy networks of inter-operating medical devices, that shall have security-for-safety by design, with a strategy for device certification and certifiable dynamic network composition, ensuring that patient safety is safeguarded from malicious cyber actors and technology "accidents".

Adults with Down syndrome: characterization of a Portuguese sample.

INTRODUCTION: Down syndrome or Trisomy 21 affects physical, behavioral and cognitive development. Ageing people with Down syndrome may suffer from several comorbidities that interfere with their daily life. OBJECTIVE: To analyze in a sample of adults with Down syndrome the presence of common medical diseases, regular use of medication, possible cognitive impairment and oral communication skills. MATERIAL AND METHODS: The socio-demographic and clinical data of 209 Portuguese adults (20-58 years) with Down syndrome were collected. Twenty-six subjects were assessed with the WAIS-III. Data was statistically analyzed--SPSS (v.19). RESULTS: Almost half (47.36%) of the sample suffers from comorbidities, with cardiac, psychiatric, epilepsy and hypothyroidism ranked as the most prevalent diseases. Seventy-two patients (34.45%) take medication that acts on the central nervous system. The WAIS-III results revealed IQ scores between 45-61 (mean = 49.65, SD = 4.93) and 141 individuals (67.5%) who can express themselves through basic oral language. DISCUSSION: In line with other studies, these individuals are vulnerable to various comorbidities. Most have basic oral language, but the IQ values are in the 'extremely low' range, with better performance on verbal tasks. CONCLUSION: The cognitive constraints and communication difficulties in the population with Down syndrome may difficult the early clinical recognition of comorbidities associated with ageing. The identification of new semiology, with caregiver support, is essential to ensure that appropriate care is provided to this population.

Introdução: A síndrome de Down ou Trissomia 21 afeta o desenvolvimento físico, comportamental e cognitivo. No decurso do envelhecimento, a ocorrência de comorbilidades aumenta nesta população, sendo a sua deteção e tratamento precoces determinantes na sua qualidade de vida. Objetivo: Analisar numa amostra de adultos com síndrome de Down a existência de doenças médicas comuns, uso regular de medicação, eventual disfunção cognitiva e capacidades de comunicação oral. Material e Métodos: Foram coletados os dados sociodemográficos e clínicos de 209 adultos portugueses (20 - 58 anos), com síndrome de Down. Vinte e seis indivíduos foram avaliados com a WAIS-III. Os dados foram analisados estatisticamente - SPSS (v.19). Resultados: Quase metade da amostra (47,36%) sofre de comorbilidades, sendo as doenças cardíacas, psiquiátricas, epilepsia e hipotiroidismo as mais prevalentes. Setenta e dois indivíduos (34,45%) tomam medicação com ação no sistema nervoso central. Na WAIS-III, os valores de QI situam-se entre 45-61 (m = 49,65, dp = 4,93) existindo 141 indivíduos (67,5%) capazes de se expressar através de linguagem oral básica. Discussão: Os indivíduos desta amostra são vulneráveis a várias comorbilidades. A maioria tem linguagem oral básica, mas os valores de QI situam-se na faixa 'extremamente baixo', sendo melhor o desempenho em tarefas verbais. Conclusão: As limitações cognitivas e dificuldades comunicacionais na população com síndrome de Down podem condicionar o reconhecimento clínico precoce de comorbilidades que lhe estão associadas no decurso do envelhecimento. A valorização semiológica, com apoio do cuidador, é essencial para garantir que os cuidados de saúde adequados são prestados a esta população.

N170 asymmetry as an index of inferior occipital dysfunction in patients with symptomatic occipital lobe epilepsy.

OBJECTIVE: Localizing epileptic foci in posterior brain epilepsy remains a difficult exercise in surgery for epilepsy evaluation. Neither clinical manifestations, neurological, EEG nor neuropsychological evaluations provide strong information about the area of onset, and fast spread of paroxysms often produces mixed features of occipital, temporal and parietal symptoms. We investigated the usefulness of the N170 event-related potential to map epileptic activity in these patients. METHODS: A group of seven patients with symptomatic posterior cortex epilepsy were submitted to a high-resolution EEG (78 electrodes), with recordings of interictal spikes and face-evoked N170. Generators of spikes and N170 were localized by source analysis. Range of normal N170 asymmetry was determined in 30 healthy volunteers. RESULTS: In 3 out of 7 patients the N170 inter-hemispheric asymmetry was outside control values. Those were the patients whose spike sources were nearest (within 3cm) to the fusiform gyrus, while foci further away did not affect the N170 ratio. CONCLUSIONS: N170 event-related potential provides useful information about focal cortical dysfunction produced by epileptic foci located in the close neighborhood of the fusiform gyrus, but are unaffected by foci further away. SIGNIFICANCE: The N170 evoked by faces can improve the epileptic foci localization in posterior brain epilepsy.

The electroclinical-imagiological spectrum and long-term outcome of transient periictal MRI abnormalities.

The electroclinical-imagiological spectrum and long-term outcome of transient periictal MRI abnormalities (TPMA) remains largely unclear. This prompted us to perform a prospective observational cohort study, including electroencephalography (EEG) and multi-sequence MRI, in 19 consecutive patients (8 female, mean age 51.7 years) with TPMA induced by convulsive and non-convulsive status epilepticus (n=14) or isolated seizures. TPMA were associated with focal, lateralized or diffuse EEG abnormalities, and were mostly focal unilateral and cortico-subcortical (n=11), less frequently cortically restricted, bilateral, hemispheric and with remote lesions (pulvinar, cerebellum); 66.7% had cortico-pial contrast enhancement and 93.7% restriction on diffusion-weighted imaging, with cortical cytotoxic edema on apparent-diffusion coefficient, only tumor-like TPMA (n=5) presenting noticeable subcortical vasogenic edema. The heterogeneity of clinical, EEG and MRI findings contributed to a 38.6% strict focal topographic concordance between them, with the more widespread findings also attributable to the time lag between studies, seizure dynamics/etiologies and cerebral reserve. At follow-up (mean duration 29.6 months, 3-120), the brain damage induced by TPMA was responsible for a high incidence of clinical and MRI sequelae (63.2%), only tumor-like/small TPMA induced by acute symptomatic seizures presenting good clinical outcomes. Our findings may contribute to a better definition and comprehension of the TPMA electroclinical-imagiological spectrum, pathophysiology and long-term outcome.

Cerebral tuberculoma presenting as flexion spasms.

A 10-month-old girl was admitted with refractory infantile spasms. Video EEG demonstrated focal epileptic activity, and MRI revealed a conglomeration of annular lesions. Surgical excision was performed and pathology was consistent with tuberculoma. After antituberculous therapy, the outcome was favorable. Despite all investigations, Mycobacterium tuberculosis's mode of transmission was unclear, and both congenital and postnatal acquired forms were considered.

[Significance of neuroimaging in the diagnosis of Boucher-Neuhauser syndrome]. / A importância da neuroimagem no diagnóstico da Síndrome de Boucher-Neuhauser.

The authors report a case of a young patient with the clinic triad of cerebellar ataxia, retinal dystrophy and hypogonadotropic hypogonadism (Boucher Neuhauser Syndrome), of probable autosomal recessive inheritance, in which the brain MRI has a major role to the diagnosis.