Magnetoencephalography Atlas Viewer for Dipole Localization and Viewing.

Magnetoencephalography (MEG) is a noninvasive neuroimaging technique widely recognized for epilepsy and tumor mapping. MEG clinical reporting requires a multidisciplinary team, including expert input regarding each dipole's anatomic localization. Here, we introduce a novel tool, the "Magnetoencephalography Atlas Viewer" (MAV), which streamlines this anatomical analysis. The MAV normalizes the patient's Magnetic Resonance Imaging (MRI) to the Montreal Neurological Institute (MNI) space, reverse-normalizes MNI atlases to the native MRI, identifies MEG dipole files, and matches dipoles' coordinates to their spatial location in atlas files. It offers a user-friendly and interactive graphical user interface (GUI) for displaying individual dipoles, groups, coordinates, anatomical labels, and a tri-planar MRI view of the patient with dipole overlays. It evaluated over 273 dipoles obtained in clinical epilepsy subjects. Consensus-based ground truth was established by three neuroradiologists, with a minimum agreement threshold of two. The concordance between the ground truth and MAV labeling ranged from 79% to 84%, depending on the normalization method. Higher concordance rates were observed in subjects with minimal or no structural abnormalities on the MRI, ranging from 80% to 90%. The MAV provides a straightforward MEG dipole anatomic localization method, allowing a nonspecialist to prepopulate a report, thereby facilitating and reducing the time of clinical reporting.

5-hydroxytryptamine1A receptor density in the hippocampus of patients with temporal lobe epilepsy is associated with disease duration.

BACKGROUND AND PURPOSE: To date, no study has evaluated the association between serotonin receptor density and clinical variables in patients with temporal lobe epilepsy caused by hippocampal sclerosis (TLE-HS) using hippocampal tissue. We evaluated 5-hydroxytryptamine1A receptor (5-HT1AR) density in hippocampal tissue from patients with TLE-HS. METHODS: We analyzed the hippocampal tissue of 34 patients with pharmacoresistant unilateral TLE-HS. 5-HT1AR density was measured using semiquantitative western blotting. RESULTS: There was an association between higher density of 5-HT1AR and longer duration of epilepsy (Spearman correlation: P = 0.040; generalized linear model: P = 0.026). CONCLUSIONS: This study demonstrated that hippocampal 5-HT1AR density is associated with epilepsy duration in patients with TLE-HS. The authors postulate that this may represent a potential regulatory enhancement of endogenous serotonergic neurotransmission in response to prolonged and enduring epileptiform activity in the hippocampal tissue of patients with pharmacoresistant TLE-HS.

Spontaneous Resolution of a Ruptured Dissecting PICA Aneurysm. Report of Two Cases.

SUMMARY: Spontaneous dissecting aneurysms (SDAs) seldom involve the intradural vertebral artery VA, the posterior cerebral, basilar or postero inferior cerebellar arteries (PICAs), where they produce subarachnoid haemorrhage and/or ischaemia. These lesions may develop spontaneously or occasionally after minor trauma and occur in young people in whom there is no underlying abnormality to explain the appearance of the dissection in most cases. Spontaneous dissecting aneurysm of the PICA is rare and its natural history is not well understood. Surgery or endovascular treatment for PICA dissection remain controversial because they suggest vessel occlusion. Only in a few cases is the bypass between the occipital artery and the PICA possible with trapping of the dissected segment. Reinforcement of the arterial wall does not seem efficient and the surgical approach per se with sole exclusion of the aneurysm may be disastrous. We describe two cases of SDA of PICA that presented with subarachnoid haemorrhage (SAH), treated conservatively, with spontaneous cure of the lesions, angiographically confirmed at mid-time follow-up of five and four months. These favourable spontaneous thromboses, like 11 other similar case reported in the literature, did not show any rebleed. The possibility of a benign clinical course of this lesion exists; clinical and angiographic management of the patient before a decision for a aggressive treatment is proposed.

Machado-Joseph disease of Azorean ancestry in Brazil: the Catarina kindred; neurological, neuroimaging, psychiatric and neuropsychological findings in the largest known family, the Catarina kindred

Há, até o momento, notícia de 9 famílias näo aparentadas com o diagnóstico clínico de doença de Machado-Joseph (MJD) no Brasil. Esta é a maior família do mundo com doença. É de origem açoriana e contem 622 indivíduos na árvore fenealógica. Destes, 236 foram examinados. Dois examinadores julgaron 39 como afetados. Respectivamente 12, 23 e 4 pacientes tinham os fenôtipos I, II e III da doença, com idades no início variando entre 10-48, 14-54 e 30-55. Doença tipo I de início juvenil näo mostrou atrofia täo severa nas imagens por ressonância magnética (RM) quanto doença tipo II de duraçäo igual, demonstrando que severidade clínica e grau de atrofia näo caminham paralelamente. Nenhum dos 8 pacientes examinados por RM tinha atrofia olivar ou anormalidades no globo pálido. Doze pacientes e 23 sob risco foram submetidos a avaliaçäo neuropsicológica. A atençäo foi normal em todos. Memória verbal estava pior nos doentes com maior decaimento com o tempo que nos sob risco. Ambos os grupos tiveram pontuaçäo abaixo do normal na identificaçäo de silhuetas e praxia construtiva. Memória visual estava bem abaixo do normal para ambos, com muitas rotaçöes, porém sem omissöes ou confabulaçäo. Padräo peculiar de multiplicaçäo dos detalhes internos, que denominamos o "efeito olho de mosca" foi visto em 6 doentes e 8 sob risco. Discriminaçäo defeituosa de cores, quando múltiplas cores eram apresentadas lado a lado, na ausência de anomia ou cegueira a cores, caracterizável como "simultagnosia a cores", surgiu como achado e foi pesquisada em 29 sujeitos 4/10 doentes e 6/19 sob risco mostram esta dificuldade. Conclui-se que disfunçöes cognitivas na esfera visual säo proeminentes nesta família. Se seriam próprias da doença e manifestaçäo precoce daqueles sob risco, está ainda para ser estabelecido. Depressäo foi avaliada com critérios do DSM III-R e com o Montgomery-Asberg Depression Rating Scale. Näo houve diferença entre doentes e sob risco. Entretanto, os pacientes tiveram menos depressäo do que tinham tido antes ou nas fases precoces da doença. A MJD plenamente instalada parecia exercer efeito protetor da depressäo

Machado-Joseph disease of Azorean ancestry in Brazil: the Catarina kindred. Neurological, neuroimaging, psychiatric and neuropsychological findings in the largest known family, the "Catarina" kindred.

At the moment 9 seemingly independent families with the clinical diagnosis of MJD are known in Brazil. The largest family tree of Azorean ancestry contains 622 individuals in 9 generations. 236 were examined, 39 found to be affected by two examiners. Phenotypes I, II and III were expressed by 12, 23 and 4 patients with age of onset by phenotypes being 10-48, 14-54 and 30-55 respectively. Although clinically more severe, juvenile onset type I disease did not show as severe a ponto-mesencephalic atrophy on MRI as the father with type II disease of similar symptomatic duration. None of the 8 patients examined with MRI showed olivary atrophy or pallidal abnormalities. 12 affected and 23 at risk were evaluated with neuropsychological tests. Attention was normal in both groups. Verbal memory scores were below normal in the affected and there was greater decay with time than in the risk group. Both scored below normal in identifying silluettes and constructional praxis. Visual memory scores were well below normal for both, with many rotations but no omissions or confabulations. A peculiar pattern of multiplying internal details called "the fly-eye effect" was observed in 6 affected and 8 at risk. Defective color distinction when multiple colors presented close to each other, in face of proper naming of individual colors ("color simulatanagnosia"), was looked for in 29 people. 4/10 affected and 4/19 at risk showed this phenomenon. Cognitive dysfunctions in this MJD family are prominent in the sphere of vision. Whether they constitute an early manifestation in those at risk and thus serve as a clinical identifier of the illness is yet to be established. Depression was looked for in the history of the family with DSM III-R criteria and an attempt at quantification with the Montgomery-Asberg Rating Scale. There was no significant quantitative difference between affected and at risk. Once undeniably symptomatic however, the patients had no, or less depression than themselves before or at the early stages of the illness. Covert depression was appropriately excluded. Fully established MJD in this family seemed to exert a protective effect from depression.