AN UNCOMMON CASE OF FILUM TERMINALE HEMANGIOBLASTOMA NON-ASSOCIATED WITH VON HIPPEL – LINDAU DISEASE

A 55-year-old man presented with low backache, paraesthesia, and spasticity of both lower limbs. He had urinary retention and constipation. Investigations revealed a vascular intradural cauda equina tumor. MRI scan demonstrated an enhancing mass at the third and fourth lumbar vertebral levels. There were multiple dilated and tortuous veins draining from both poles of the tumor. At surgery, we found a well-defined tumor with an orange hue and fleshy consistency was encountered arising from the filum terminale, comprising a few adjacent nerve roots. The tumor was excised en bloc. The filum terminale was also been resected. A sporadic hemangioblastoma arising from the filum terminale should be considered as a rare cause of back pain and sciatica. Total excision of the tumor offers a cure

NEUROINFLAMAÇÃO NA ESCLEROSE LATERAL AMIOTRÓFICA

A esclerose lateral amiotrófica (ELA) esporádica é uma doença neurodegenerativaque acomete o neurônio motor. Sua etiologia ainda não foi totalmente esclarecida e é considerada multifatorial. O objetivo desse trabalho é fazer uma revisão narrativa sobre os mecanismos fisiopatológicos da ELA esporádica, com foco no papel da neuroinflamação, além de descrever estudos envolvendo a pesquisa de biomarcadores de diagnóstico e prognóstico. O processo de neuroinflamação na ELA é considerado secundário às alterações que levam à morte neuronal, podendo influenciar na taxa de progressão dadoença. Existem diversos estudos sobre o perfil dos fatores inflamatóriosna ELA, por vezes com resultados contraditórios, reforçando a dificuldade de análise desses fatores num organismo dinâmico. Ainda assim, no contexto da ELA, o estudo de possíveis biomarcadores diagnósticos e/ou prognósticos é válido e de grande interesse, pois permitiria um avanço nos ensaios clínicos que buscam novos tratamentos, bem como na condução e planejamento de cada caso

Papez circuit gray matter and episodic memory in amyotrophic lateral sclerosis and behavioural variant frontotemporal dementia

Amyotrophic lateral sclerosis and behavioural variant frontotemporal dementia are two different diseases recognized to overlap at clinical, pathological and genetic characteristics. Both conditions are traditionally known for relative sparing of episodic memory. However, recent studies have disputed that with the report of patients presenting with marked episodic memory impairment. Besides that, structural and functional changes in temporal lobe regions responsible for episodic memory processing are often detected in neuroimaging studies of both conditions. In this study, we investigated the gray matter features associated with the Papez circuit in amyotrophic lateral sclerosis, behavioural variant frontotemporal dementia and healthy controls to further explore similarities and differences between the two conditions. Our non-demented amyotrophic lateral sclerosis patients showed no episodic memory deficits measured by a short-term delayed recall test while no changes in gray matter of the Papez circuit were found. Compared with the amyotrophic lateral sclerosis group, the behavioural variant frontotemporal dementia group had lower performance on the short-term delayed recall test and marked atrophy in gray matter of the Papez circuit. Bilateral atrophy of entorhinal cortex and mammillary bodies distinguished behavioural variant frontotemporal dementia from amyotrophic lateral sclerosis patients as well as atrophy in left cingulate, left hippocampus and right parahippocampal gyrus. Taken together, our results suggest that sub-regions of the Papez circuit could be differently affected in amyotrophic lateral sclerosis and behavioural variant frontotemporal dementia

Deficits in emotion recognition as markers of frontal behavioral dysfunction in Amyotrophic Lateral Sclerosis

Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with prominent motor symptoms. Patients with ALS may also manifest frontal behavior symptoms and cognitive decline, including impairment in facial emotion recognition. The authors aimed to investigate whether deficits in emotion recognition were associated with frontal behavior symptoms in ALS. Methods: Participants were patients with probable or definite sporadic ALS (N521; male:female ratio, 11:10; median age, 62 years; median disease duration, 3 years) and agematched and education-matched healthy control subjects (N525; male:female ratio, 14:11; median age, 61 years). The Facial Emotion Recognition Test (FERT) was administered to all participants. Patients with ALS were assessed using the Cambridge Behavior Inventory-Revised and were classified into two groups according to the presence of frontal behavioral symptoms: ALS with no behavioral symptom (ALSns; N59) and ALS with at least one behavioral symptom (ALSbs; N512). Results: Apathy and mood symptoms were the most frequent neuropsychiatric symptoms in the patient group. Patients with ALS performed worse than control subjects in the recognition of sadness (p,0.004). There were no differences between control subjects and patients in the ALSns group in all FERT scores, but the ALSbs group had lower performance than control subjects in sadness (p,0.003). Conclusions: Emotion recognition deficit may be a marker of frontal behavior in ALS

Normative resistance to responsibility to protect in times of emerging multipolarity: the cases of Brazil and Russia

This article assesses the normative resistance to Responsibility to Protect adopted by Brazil and Russia against the backdrop of their international identities and self-assigned roles in a changing global order. Drawing upon the framework of Bloomsfield's norm dynamics role spectrum, it argues that while the ambiguous Russian role regarding this principle represents an example of 'norm antipreneurship', particularities of Brazil's resistance are better grasped by a new category left unaccounted for by this model, which this study portrays as 'contesting entrepreneur'.- (undefined