Brain : a journal of neurology
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Are the oculomotor disturbances in myotonic dystrophy (MD), i.e. reduced smooth pursuit (SP) gain and reduced saccadic peak velocity (PV), of muscular or central origin? To answer this question the following two approaches were used. (i) The performance of SP was compared with the patient's ability to suppress the vestibulo-ocular reflex (VOR) visually (VOR suppression; VOR-S). In the latter task the SP system is involved, but the eyes hardly move within the orbits. A parallel impairment of SP and VOR-S would indicate a central dysfunction. (ii) Peak saccadic velocity was compared between two saccades performed to and fro in rapid succession. ⋯ The increase in saccadic latency for centripetal saccades at the short ISI also reflects a central deficit. However, the observed slowing of saccades might have a myopathic or neural origin; a distinction was not possible at present. A myotonic origin of the saccade slowing seems unlikely, because the effect was independent of the presaccadic activation of the relaxing (antagonistic) eye muscle.
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Face perception and emotion recognition were investigated in a group of people with Huntington's disease and matched controls. In conventional tasks intended to explore the perception of age, sex, unfamiliar face identity (Benton test) and gaze direction from the face, the Huntington's disease group showed a borderline impairment of gaze direction perception and were significantly impaired on unfamiliar face matching. With a separate set of tasks using computerinterpolated ('morphed') facial images, people with Huntington's disease were markedly impaired at discriminating anger from fear, but experienced less difficulty with continua varying from male to female, between familiar identities, and from happiness to sadness. ⋯ Questionnaires were also used to examine self-assessed emotion, but did not show such striking problems. Taken together, these data reveal severe impairments of emotion recognition in Huntington's disease, and show that the recognition of some emotions is more impaired than others. The possibility that certain basic emotions may have dedicated neural substrates needs to be seriously considered: among these, disgust is a prime candidate.
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Mechanical distortion of the human cranial venous sinuses is painful as is cranial venous sinus distension during migraine. Sumatriptan, the serotonin (5HT)IB/D-like receptor agonist, is highly effective in relieving migraine headache and part of its action may be due to constriction of cranial dural blood vessels. Using immunohistochemical detection of the immediate early gene Fos, we have mapped the spatial pattern of neural activation in the caudal medulla and the upper cervical spinal cord (C1, C2 and C3) in cats following either electrical or mechanical stimulation of the superior sagittal sinus. ⋯ Treatment with sumatriptan reduced the numbers of Fos-positive cells found in laminae I and IIo of the TNC and C2 (6, 13 cells and 9 cells, respectively) after mechanical stimulation. These data suggest that the neural effect of sumatriptan alone is sufficient for significant attenuation of transmission in the trigeminal system. The fact that sumatriptan can inhibit trigeminal activation without its vascular effects suggests that drugs without a significant activity on blood vessels may be effective in the treatment of migraine.
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Randomized Controlled Trial Clinical Trial
Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study.
Thirty patients with definite or probable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of chronic progressive (16 patients) or relapsing (14 patients) course were randomly assigned to receive intravenous immunoglobulin (IvIg) 0.4 g per kg body weight or a placebo treatment on 5 consecutive days in a double-blind, cross-over trial. Neurological function was monitored by serial quantitative assessments [neurological disability score (NDS); clinical grade (CG) and grip strength (GS) measurements] and by electrophysiological studies before and after each treatment period. Twenty-five patients completed both treatment periods. ⋯ All 10 patients have been maintained and stabilized with IvIg pulse therapy of 1 g per kg body weight or less, given as a single infusion prior to the expected relapse. A beneficial response to IvIg was found to be most likely in patients with acute relapse or with disease of one year or less. Patients with predominantly sensory signs did not improve.
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We studied 31 consecutive patients with temporal and extratemporal epilepsy who underwent presurgical evaluation with stereotaxic depth EEG (SEEG) to assess the relationships between amygdalo-hippocampal (AM-HF) atrophy and the location of SEEG seizure onset and SEEG interictal abnormalities. Scalp EEG recordings with sphenoidal electrodes had shown bitemporal ictal or interictal epileptic abnormalities in all. Patients underwent high quality MRI scans, including MRI volumetric measurements of mesial temporal structures. ⋯ We conclude that unilateral mesial atrophy predicts ipsilateral mesial SEEG seizure onset despite bitemporal extracranial EEG foci. However, in patients with significant bilateral mesial atrophy, SEEG seizures may originate from either side, even in the presence of significant asymmetry. Finally, the identification of unilateral mesial atrophy has prognostic importance.