ABSTRACT
Despite the high relevance of emotion processing for social functioning, the study of the impairment of facial affect in multiple sclerosis (MS) has received little attention. Previous research reported evidence for emotion processing deficits but the nature and extent are not fully explained. Thirty-five MS patients underwent dedicated neuropsychological assessment of emotion processing using two facial affect recognition tasks and self-report measures of alexithymia. For comparison, healthy participants served as controls. Relative to healthy controls, MS patients were impaired in facial affect recognition on four of the six Ekman basic emotions, except happiness and disgust. The MS patients were more alexithymic than the healthy controls. These data provide evidence for deficits in the recognition of emotional face expressions and emotional introspection.
Subject(s)
Affective Symptoms/diagnosis , Affective Symptoms/etiology , Facial Expression , Multiple Sclerosis/complications , Perceptual Disorders/diagnosis , Perceptual Disorders/etiology , Adult , Affective Symptoms/physiopathology , Aged , Case-Control Studies , Female , Humans , Male , Middle Aged , Multiple Sclerosis/physiopathology , Multiple Sclerosis/psychology , Pattern Recognition, Visual/physiology , Perceptual Disorders/physiopathology , Prospective Studies , Young AdultABSTRACT
Cerebral lesions are held to induce plastic changes of the brain. Less well established, however, is how much space-occupying brain lesions may only displace functional representations. In a 66-year-old man we show, by means of functional magnetic resonance imaging and transcranial magnetic stimulation, a profound displacement of the motor cortex due to a large asymptomatic arachnoid cyst. Thus, the chronically compressed brain is capable of sustaining normal brain function without utilizing the potential of cortical plasticity.
Subject(s)
Adaptation, Physiological , Arachnoid Cysts/physiopathology , Motor Cortex/pathology , Neuronal Plasticity , Psychomotor Performance , Aged , Arachnoid Cysts/pathology , Evoked Potentials, Motor , Humans , Magnetic Resonance Imaging , Male , Motor Cortex/physiopathology , Neuropsychological Tests , Transcranial Magnetic StimulationABSTRACT
In this study, we sought to investigate if brain tissue affected by ischemia can accommodate areas of activation related to restoration of brain function following ischemic stroke. In two patients perfusion imaging (PI) and diffusion weighted imaging (DWI) obtained in the acute phase after stroke was coregistered with BOLD imaging of brain functions acquired when profound recovery had occurred. Both patients suffered from thrombembolic brain infarction due to dissection of the internal carotid artery (ICA) characterized by a severe PI-DWI mismatch in the acute stage of stroke. Following ICA recanalization and clinical recovery BOLD imaging showed task-specific activation adjacent to the infarct lesion within the former PI-DWI mismatch area. The data in these two stroke patients provide evidence that brain tissue at risk of infarction as shown by the PI-DWI mismatch can survive and, thereby, constitute the major site underlying post-ischemic recovery.
Subject(s)
Brain Ischemia/pathology , Brain/anatomy & histology , Cerebral Infarction/diagnosis , Adult , Aphasia/etiology , Brain/pathology , Brain/physiology , Brain Mapping/methods , Facial Paralysis/etiology , Female , Functional Laterality , Humans , Magnetic Resonance Imaging , Middle AgedABSTRACT
The classical McCune-Albright syndrome (MAS) consists of peripheral precocious puberty (PPP), fibrous bone dysplasia and café-au-lait spots. We conducted a survey among pediatric endocrinologists in Germany, Austria and Switzerland, most of them participating in the German Working Group for Pediatric Endocrinology (APE). Thirty-three physicians reported 58 patients. A detailed questionnaire yielded extensive data from 41 patients. Patients (36 females, 5 males) were diagnosed between the 4th week of life and 8 years. Symptoms included precocious puberty (37 patients), café-au-lait spots (35), fibrous bone dysplasia (32), hyperthyroidism (5), liver disease (5), phosphate wasting (4), GH hypersecretion (3), anemia in infancy (2), hyperprolactinemia (1), and Cushing's disease (1). Therapy of PPP included testolactone (15), tamoxifen (7), cyproterone acetate (CPA) (5), anastrozole (1) and exemestane (1). Testolactone, tamoxifen and CPA showed varying degrees of clinical remission; none was unanimously effective, but side effects were very rare. New aromatase inhibitors were not well validated in MAS. Eleven children received bisphosphonate therapy (pamidronate i.v. q 3 months) for fibrous bone dysplasia. Pamidronate was well tolerated, and pain improved when present, but data on preventive effects are not yet available. In conclusion, this survey describes a large cohort of patients with MAS, many with severe clinical problems, including major physical handicaps. Our results show that there is no 'gold standard' for the therapy of PPP; tested treatment regimens are not ideal, and new aromatase inhibitors need to be evaluated in controlled studies. Pamidronate was well tolerated, but preventive effects on bone dysplasia have not yet been proven.
