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AIM: The results of the Asymptomatic Carotid Atherosclerosis Study (ACAS) study have provided the first scientific evidence that in patients with asymptomatic carotid stenosis greater than 60% carotid endarterectomy reduces the risk of stroke from 2% to 1% per year. The implications are that approximately 20 operations need to be performed in order to prevent 1 stroke in 5 years. The aims of the Asymptomatic Carotid Stenosis and Risk of Stroke (ACSRS) study are to identify a subgroup or subgroups at a risk for stroke higher than 4% and a group at a risk for stroke less than 1% per year using systemic and local risk factors (plaque characterization) in addition to the degree of stenosis. The aim of this paper is to present the protocol and the results of the quality control. METHODS: The ACSRS is a multicentre natural history study of patients with asymptomatic internal carotid diameter stenosis greater than 50% in relation to the bulb. The degree of stenosis is graded using multiple established ultrasonic duplex criteria. In addition, ultrasonic plaque characterization is performed and clinical risk factors and medications are recorded. Training is provided centrally. All carotid ultrasound examinations are recorded on video-tape which together with CT-brain scans and ECG are analysed at the coordinating centre with feedback information to partner centres. RESULTS: The video recordings and analysis of data centrally with feed back information have provided quality control with a significant improvement not only in the completion of data forms but also in the grading of internal carotid stenosis and plaque recordings using ultrasound. CONCLUSION: The high level of quality of data collected will add credibility to the results of the ACSRS study and may eventually promote the development of international standards of plaque imaging and characterization

Sudden sensorineural hearing loss is a perplexing entity in otology. Susac's syndrome (also called retinocochleocerebral vasculopathy) is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear, and usually affects women in young adulthood. We describe the clinical aspects, radiographic findings, and management of one such case.A 30-year-old woman was admitted to the hospital because of sudden onset of bilateral deafness and headache. During her hospitalization, she developed discrete right hemiparesis and hypoesthesia.Magnetic resonance imaging revealed multiple signal hyperintensities and atrophy of the corpus callosum. The differential diagnosis was a myelinating condition, such as multiple sclerosis or acute demyelinating encephalomyelitis.Retinal fluorescein angiography helped the diagnosis of Susac's syndrome.

We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) in a 76-year-old male presenting with confusion, dysarthria, diplopia, lumbal pain and headaches of recent onset. Neurological examination revealed nuchal rigidity and bilateral sixth cranial nerve palsy. The cerebrospinal fluid showed a marked hyperproteinorachia (4711 mg/L) and mild cytorachia (5-10 leucocytes/mm3) with a few atypical lymphoid cells. On admission, brain CT scan and MRI demonstrated diffuse and nodular leptomeningeal contrast enhancement predominant at the skull base and several osteolytic lesions in the right parietal bone. Extensive serological studies for infectious, autoimmune or neoplastic diseases were negative. The work-up diagnosis was neurosarcoidosis or multiple meningeal and osseous metastases of an unknown primary cancer. Surgical biopsy of the right parietal bone lesion showed only fibrous tissue with no evidence of tumour or inflammation. The patient was treated with high dose corticosteroids but its neurological status progressively worsened and he died of aspiration pneumonia 35 days after admission. Post-mortem examination revealed a PDLG, a rare fatal tumour with about 60 cases reported. PDGL is characterized by the diffusion of neoplastic glial cells throughout the leptomeninges without evidence of a primary intra-parenchymal lesion. Recognition of this rare brain tumour is important as recent reports suggest that radiotherapy and chemotherapy can improve patient survival.

Traumatic and non-traumatic brain injured disorders of consciousness patients are still challenging for diagnosis, prognosis, ethical and socio-economic reasons. Currently, there remains a high rate of misdiagnosis of the vegetative state (Schnakers, et al. 2009). Recent advances in MRI techniques (diffusion tensor, magnetic resonance spectroscopy and functional imaging) provide data that could improve the diagnostic and prognostic evaluation and management of these patients.

Pain is a subjective experience. Its assessment is based on the subject's direct verbal report. This method of assessment is, however, impossible in patients who cannot communicate their feelings. In this context, indirect measurements such as behavioral observations or physiological measurements are needed. To facilitate the assessment of pain in non-communicative patients, numerous standardized behavioral scales have been developed. The aim of this review is to discuss the main validated pain scales employed in end-stage dementia, newborn and preverbal children, and severely brain damaged patients with a disorder of consciousness such as coma, the vegetative state or the minimally conscious state.

Spontaneous brain activity has recently received increasing interest in the neuroimaging community. However, the value of resting-state studies to a better understanding of brain-behavior relationships has been challenged. That altered states of consciousness are a privileged way to study the relationships between spontaneous brain activity and behavior is proposed, and common resting-state brain activity features observed in various states of altered consciousness are reviewed. Early positron emission tomography studies showed that states of extremely low or high brain activity are often associated with unconsciousness. However, this relationship is not absolute, and the precise link between global brain metabolism and awareness remains yet difficult to assert. In contrast, voxel-based analyses identified a systematic impairment of associative frontoparieto-cingulate areas in altered states of consciousness, such as sleep, anesthesia, coma, vegetative state, epileptic loss of consciousness, and somnambulism. In parallel, recent functional magnetic resonance imaging studies have identified structured patterns of slow neuronal oscillations in the resting human brain. Similar coherent blood oxygen level-dependent (BOLD) systemwide patterns can also be found, in particular in the default-mode network, in several states of unconsciousness, such as coma, anesthesia, and slow-wave sleep. The latter results suggest that slow coherent spontaneous BOLD fluctuations cannot be exclusively a reflection of conscious mental activity, but may reflect default brain connectivity shaping brain areas of most likely interactions in a way that transcends levels of consciousness, and whose functional significance remains largely in the dark.

The field of neurology was long infamous for a lack of therapeutic options. How many of you have once thought: "Neurologists don't cure the disease, they admire it". But those days have passed into history, and the field is now vibrant with new treatments and hope even for patients with the worst neurodegenerative diseases. We summarized in the present review the latest major advances in therapeutic principles and practice for some of the most frequent chronic neurological disorders such as headaches, epilepsy, multiple sclerosis, dementias, Parkinson's disease, sleep/wake disturbances and peripheral neuropathies. We cannot cure or prevent, but we can now halt or control symptoms and disease progression to provide physical and psychological relief, and a better quality of life for patients who suffer from these otherwise devastating neurological conditions.