Face shape and face identity processing in behavioral variant fronto-temporal dementia: A specific deficit for familiarity and name recognition of famous faces.

Deficits in face processing have been described in the behavioral variant of fronto-temporal dementia (bvFTD), primarily regarding the recognition of facial expressions. Less is known about face shape and face identity processing. Here we used a hierarchical strategy targeting face shape and face identity recognition in bvFTD and matched healthy controls.

Safety and efficacy of natalizumab in Belgian multiple sclerosis patients: subgroup analysis of the natalizumab observational program.

Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multiple sclerosis (MS) patients. As it is one of the more recent therapies for MS, there remains a need for long-term safety and efficacy data of natalizumab in a clinical practice setting. The Tysabri observational program (TOP) is an open-label, multicenter, multinational, prospective observational study, aiming to recruit up to 6,000 patients with relapsing-remitting MS from Europe, Canada and Australia.

A Belgian ancestral haplotype harbours a highly prevalent mutation for 17q21-linked tau-negative FTLD.

Among patients with frontotemporal lobar degeneration (FTLD), the respective frequencies of dominant 17q21-linked tau-negative FTLD (with unidentified molecular defect) and 17q21-linked tau-positive FTLD (due to MAPT mutations) remain unknown. Here, in a series of 98 genealogically unrelated Belgian FTLD patients, we identified an ancestral 8 cM MAPT containing haplotype in two patients belonging to multiplex families DR2 and DR8, without demonstrable MAPT mutations, in which FTLD was conclusively linked to 17q21 [maximum summed log of the odds (LOD) score of 5.28 at D17S931].

Long-term follow up of glatiramer acetate compassionate use in Belgium.

Between June 1995 and November 1998, 228 patients with relapsing-remitting Multiple Sclerosis started treatment with glatiramer acetate (Copaxone) 20 mg once daily in the frame of a "compassionate use" protocol in 15 Belgian centers. Following an average treatment period of 5.8 years, treating neurologists were requested to fill in follow-up forms indicating neurological disability status and side effects during the previous 6 months.

Objective pulsatile tinnitus caused by intrapetrous dissecting aneurysm.

Intrapetrosal dissections is a special kind of intracranial dissection since it is located intracranially but extradurally. Only three reports of spontaneous intrapetrosal dissection have been published so far, the posttraumatic intrapetrosal dissections caused by penetrating or blunt trauma not included. Based on the clinical and radiological presentation a treatment with anticoagulantia (a coumarine derivative) was successfully installed.

Primary brain stem tethering: a rare cause of geniculate neuralgia.

This rare case of brain stem tethering presented with chronic and progressive geniculate neuralgia. In view of the fact that an occipital subcutaneous lipoma had been resected in childhood, it probably concerned a primary tethering, fitting in with an occult occipital dysraphism. Magnetic resonance imaging (MRI) clearly demonstrated an underlying tethering, causing a distortion of the brain stem.

Self-treatment of acute migraine with subcutaneous sumatriptan using an auto-injector device: comparison with customary treatment in an open, longitudinal study.

In a multicenter open longitudinal clinical trial where 479 patients suffering from migraine with or without aura were recruited, patients treated at home one to three migraine attacks with their customary treatment, and subsequently, over a 3-month period, one to three migraine attacks with 6 mg sumatriptan sc using an autoinjector. The headache response to customary treatment was 19% at 1 h and 30.5% at 2 h, and was not significantly different when only attacks treated "adequately" according to accepted treatment recommendations were considered: 16% at 1 h and 35% at 2 h.

Auditory information processing in sleep: late cortical potentials in an oddball paradigm.

Information processing of auditory stimuli in sleeping healthy volunteers is studied by means of an oddball paradigm. Latencies of the early (N1P2) complex are affected by NREM sleep depth and show a progressive slowing. The amplitude of the early N1 component depends on stimulus type and probability and is increased following deviant stimuli.

Auditory information processing in sleep: habituation to repetitive stimuli.

Habituation to evoked responses is obvious in waking but still controversial in sleep. Single-response analysis proves short-term habituation of auditory evoked potentials in stage 2 NREM sleep. The data are discussed referring to the two-system hypothesis of sensory processing in sleep and to DC instability and sleep maintenance mechanisms in stage 2 NREM.

Lack of asymmetry of middle cerebral artery blood velocity in unilateral migraine.

Background And Purpose: A recent transcranial Doppler study found reduced blood velocity in seven patients during migraine attacks in the middle cerebral artery at the headache side. This would implicate vasodilation of the middle cerebral artery in the pathogenesis of headache in migraine. We attempted to confirm this finding.

Antimigraine drug sumatriptan increases blood flow velocity in large cerebral arteries during migraine attacks.

Sumatriptan, a novel selective 5-hydroxytryptamine1d (5-HT1d) receptor agonist, which is highly effective in the acute treatment of migraine attacks, blocks dural neurogenic plasma extravasation and constricts cranial blood vessels in animal experiments. We measured intra- and extracranial blood flow velocities (BFV) with a transcranial Doppler device in 67 patients during a spontaneous migraine attack, before and after treatment with 3 mg or 6 mg subcutaneous sumatriptan or placebo. Sumatriptan, but not placebo, significantly increased BFV (cm/sec) in the internal carotid and middle cerebral arteries on both sides, without detectably changing the BFV in the common and external carotid arteries.

Background EEG reactivity in auditory event-related potentials.

Mental activity has influences on auditory event-related potentials (AERP) as well as on some EEG rhythms, notably the alpha rhythm. In this study, background reactivity (BR) of the EEG was investigated in the context of AERPs. Single responses of 14 healthy subjects were analysed in 3 reaction time AERP experiments of increasing difficulty: the first involved one type of tone delivered at random intervals, the second offered two types of tone (an "oddball" design), and the third offered 3 types of tone.

Are event-related potentials in multiple sclerosis indicative of cognitive impairment? Evoked and event-related potentials, psychometric testing and response speed: a controlled study.

Bimodal event-related potentials (ERPs), together with evoked potentials (EPs), measures of motor speed (tapping test, EMG latencies and reaction times (RT)), and psychometric test results were studied in a group of 30 multiple sclerosis (MS) patients and 19 controls. ERPs have been advocated as objective tests of cognitive function. In the present study ERPs were compared with the results of psychometric tests, which have a proven validity in measuring aspects of cognitive function that are important in daily life.

What is the validity of an "abnormal" evoked or event-related potential in MS? Auditory and visual evoked and event-related potentials in multiple sclerosis patients and normal subjects.

The predictive validity of evoked potentials (EPs) and event-related potentials (ERPs) in multiple sclerosis (MS) has not yet been fully investigated, as only the sensitivity of these tests has sofar been reported. EPs (short, middle and long latency auditory evoked potentials and visual evoked potentials) and ERPs (visual and auditory) were studied in 19 controls and 30 multiple sclerosis (MS) patients. Abnormality thresholds of peak latencies were defined on the basis of the mean plus 2 or 3 standard deviations, based on data from the control group.

Blood flow velocities in the vertebrobasilar system during migraine attacks--a transcranial Doppler study.

In this study, blood flow velocity in the basilar artery and both vertebral and middle cerebral arteries was measured with a transcranial Doppler device in 23 migraineurs during and outside a migraine attack. The aim of the study was to compare blood flow velocities during and outside an attack and to examine vascular reactivity to voluntary hyperventilation during both conditions. No differences in blood flow velocity were found.

Vascular reactivity during migraine attacks: a transcranial Doppler study.

We measured vascular reactivity--expressed i) as decrease in blood flow velocity (cm/sec) per vol% CO2 decrease due to voluntary hyperventilation and ii) as increase of blood flow velocity during the first minute after resuming normal ventilation, per vol% CO2 increase--in the middle cerebral and basilar artery of 48 migraineurs with attacks without aura, and 17 normal controls. We found no differences for both determinants of vascular reactivity between migraineurs during and outside an attack, and between migraineurs and healthy volunteers. We conclude that the vasomotor reactivity is normal during migraine attacks without aura.

Blood flow velocity changes in migraine attacks--a transcranial Doppler study.

A pulsed Doppler device was used to measure blood flow velocities in the common carotid artery, the extracranial part of the internal carotid artery, the external carotid artery, the middle cerebral artery, and the anterior cerebral artery in 31 migraineurs without aura (n = 27) and with aura (n = 4), both during and outside an attack. The aims were to compare blood flow velocity during and between migraine attacks and to study asymmetries of the blood flow velocity. Compared with blood flow velocity values obtained in the attack-free interval, blood flow velocity was lower during attacks without aura in both common carotid arteries, but not in the other extra- and intracranial vessels which were examined.

The interpretation of dysprosody in patients with Parkinson's disease.

Prosodic features in the speech production of 21 patients with idiopathic Parkinson's disease were tested. The appreciation of vocal and facial expression was also examined in the same patients. Significant intergroup differences were found in the prosody production tasks but, in contrast to previous results, not in the receptive tasks on the recognition and appreciation of prosody and of facial expression.

Cerebral vasculopathy associated with primary varicella infection.

A previously healthy 5-year-old boy developed cerebral vasculopathy, presenting as two episodes of acute hemiparesis 3 and 9 months, respectively, after a primary varicella infection (chickenpox). This association has not been reported before, to our knowledge, although cerebral vasculopathy is a well-known complication of herpes zoster ophthalmicus. The diagnosis was based on the presence of oligoclonal varicella-specific IgG in the cerebrospinal fluid and angiographic findings.

Habituation of K-complexes or event-related potentials during sleep.

We examined habituation and dishabituation of evoked K-complexes (equivalent to auditory event-related potentials) during sleep. Habituation is investigated by presenting trains of stimuli with a 5 sec inter-stimulus interval and a 4 min inter-train interval. Habituation is complete by the third identical stimulus in a train and dishabituation occurs after deviant stimuli and after the inter-train interval.

Cerebral venous thrombosis as presenting sign of myeloproliferative disorders.

Two patients with cerebral venous thrombosis are described. In both patients laboratory findings suggested an underlying haematological disorder and bone marrow biopsy showed a myeloproliferative disorder. Both chronic cerebral venous thrombosis and early myeloproliferative disorders are difficult to diagnose.