1/8. Confabulation and multiple sclerosis: a rare association.BACKGROUND: Fantastic confabulation in the context of multiple sclerosis (MS) has not previously been reported in the literature. The association is of interest because clearly demonstrable brain pathology in MS together with other cognitive and behavioural correlates may further our understanding of the neural basis underlying confabulation. methods: A single case report with magnetic resonance imaging of the brain and detailed neuropsychological evaluation. RESULTS: Confabulation occurred together with disinhibited and stimulus bound behaviour. While the patient's physical and emotional state limited the range of psychometric tests administered, the results revealed an inability to maintain focused, regulated information processing. Although memory difficulties were present, they were not in the nature of a severe amnesic disorder. The patient appeared to have a broad fund of knowledge, but the associations binding the information together and putting it into context were loose. All three features of a triad of responses previously described in confabulating patients were present, namely an inability to withhold answers, to monitor one's own responses and provide verbal self corrections. MRI of the brain showed bilateral periventricular lesions and discrete frontal lesions with 53% of the total lesion volume distributed in frontal areas. Cortical atrophy, most marked in frontal regions also was conspicuous. CONCLUSIONS: Confabulation linked to frontal lobe involvement may occur as part of the changes in mentation found in MS. It is, however, rare and although associated with impaired memory, may be found in the absence of a severe amnesic disorder. This conclusion is discussed in the light of observations from the literature suggesting that frontal involvement is a prerequisite before fantastic confabulation occurs.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/8. Central hyperacusis with phonophobia in multiple sclerosis.hearing disorders are a well-described symptom in patients with multiple sclerosis (MS). Unilateral or bilateral hyperacusis or deafness in patients with normal sound audiometry is often attributed to demyelinating lesions in the central auditory pathway. Less known in MS is a central phonophobia, whereby acoustic stimuli provoke unpleasant and painful paresthesia and lead to the corresponding avoidance behaviour. In our comparison collective, patient 1 described acute shooting pain attacks in his right cheek each time set off by the ringing of the telephone. Patient 2 complained of intensified, unbearable noise sensations when hearing nonlanguage acoustic stimuli. Patient 3 noticed hearing unpleasant echoes and disorders of the directional hearing. All patients had a clinical brainstem syndrome. ENT inspection, sound audiometry and stapedius reflex were normal. All three patients had pathologically changed auditory evoked potentials (AEPs) with indications of a brainstem lesion, and in magnetic resonance imaging (MRI) demyelinating lesions in the ipsilateral pons and in the central auditory pathway. The origin we presume in case 1 is an abnormal impulse conduction from the leminiscus lateralis to the central trigeminus pathway and, in the other cases, a disturbance in the central sensory modulation. All patients developed in the further course a clinically definite MS. Having excluded peripheral causes for a hyperacusis, such as, e.g., an idiopathic facial nerve palsy or myasthenia gravis, one should always consider the possibility of MS in a case of central phonophobia. Therapeutic possibilities include the giving of serotonin reuptake inhibitors or acoustic lenses for clearly definable disturbing frequencies.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
3/8. Korsakoff's syndrome as the initial presentation of multiple sclerosis.A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. memory performance was investigated on several occasions during an 11-year follow-up. diagnosis of laboratory-supported definite multiple sclerosis was established and magnetic resonance imaging showed disseminated white matter lesions, especially in both medial temporal lobes. No other cause than multiple sclerosis was found for the amnestic syndrome. Among the rare cases of Korsakoff's syndrome in the course of multiple sclerosis, this is to our knowledge the first case in which a memory deficit was the initial manifestation of the disease.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
4/8. prognosis of patients who present with an episode of myelopathy of unknown origin in malaysia: a retrospective study of 52 patients.Fifty-four per cent of 52 patients presenting to the University of Malaya Medical Centre with a myelopathy for which appropriate investigations uncovered no definite etiology, subsequently developed clinically definite or probable multiple sclerosis. In the subgroup of patients with a presentation indicative of acute/subacute transverse myelopathy, 14 or 52% also went on to develop clinically definite or probable multiple sclerosis, a far higher proportion than previously recorded in the literature. This finding is probably a further manifestation of racial difference in the behaviour of multiple sclerosis. For the group as a whole, the only factor which appeared to be associated with an increased risk of developing multiple sclerosis was female sex; 67% of 33 female patients went on to develop multiple sclerosis after a mean follow-up period of 5.5 years. Other factors such as age of onset, racial composition, level of spinal cord involvement, presence of fever and CSF finding were found not to be important.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
5/8. How often is multiple sclerosis mistaken for a psychiatric disorder?In a defined population of 112,000, an attempt was made to discover every patient with multiple sclerosis. Using strict diagnostic criteria, 91 patients were identified. A search of psychiatric records for the same population revealed that 15 (16%) of these patients were referred to psychiatrists between the onset of their symptoms and the diagnosis of multiple sclerosis. Ten (11%) were seen with symptoms attributable to multiple sclerosis. These symptoms were recognized as neurological in only two cases, while the other eight patients received a variety of psychiatric diagnoses. Possible reasons for diagnostic error included the subjective nature of many early symptoms, histrionic behaviour, and psychiatric disturbance which drew attention away from physical symptoms. The results underline the caution needed when patients with physical symptoms are referred for psychiatric assessment.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
6/8. Diffential diagnosis of the caloric nystagmus.Diagnostic considerations based upon the nystagmogram are limited. Quantitative assessment of horizontal canal sensitivity is available through the use of culmination frequency or culmination slow phase velocity. Qualitative characteristics of nystagmometry have been sought but with no satisfactory results. Three distinctive features of the caloric nystagmus were evaluated and were found to be suggestive or outrightly pathognomonic for retrolabyrinthine or central nervous system abnormalities. These are: (1) Vestibular decruitment. The disproportionate caloric responsiveness when a weak stimulus elicits a more intense nystagmic reaction than a strong stimulus is capable of creating. (2) Hyperactive vestibular responsiveness (3) Ocular fixation reversal phenomenon. Contrary to the normal behaviour, the elimination of fixation decreases the nystagmus intensity instead of facilitating the evoked nystagmus. The assessment of these qualitative features of the caloric nystagmus in addition to the quantitative measurements widens the scope of our diagnostic capabilities.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
7/8. An investigation of the effects of intracerebral injection in the marmoset of cytopathic cerebrospinal fluid from patients with schizophrenia or neurological disease.In experiments designed to investigate transmission, cerebrospinal fluid (CSF) from patients with schizophrenia and neurological disease (huntington's chorea and multiple sclerosis) which had been found to induce cytopathic effects in human embryonic fibroblast cell culture was injected intracerebrally into mice, hamsters and marmosets (small New World primates). No evidence was obtained of transmission to mice or hamsters. A total of 15 marmosets (callithrix jacchus) was injected intracerebrally with CSF [8 with samples from 4 patients with schizophrenia. 3 with samples from patients with neurological disease (2 with Huntington's chorea and 1 with multiple sclerosis) and 4 with samples from 3 patients without neurological or psychiatric disease] and was observed over a period of 2 1/2 years. analysis of variance on data obtained from behavioral observations averaged over 6-month periods revealed that animals injected with CSF from patients with schizophrenia and neurological disease became progressively more inactive when compared with animals injected with CSF from control patients. The change detected by behavioural observation was confirmed as a difference 2 and 2 1/2 years after injection by automated activity monitoring. There was an incidence of reproductive anomalies (including two occipital encephalocoeles) in the females in the experimental group, but the numbers are too small to draw firm conclusions from this observation. Many reported differences in biological samples from schizophrenic patients and normal controls have subsequently been found to be due to factors unrelated to the disease state. This may prove to be the case with the changes observed in this experiment. Nevertheless, the fact that marmosets injected with CSF from patients suffering from neuropsychiatric disease, including schizophrenia, subsequently differed in their behaviour from those injected with control CSF warrants further investigation.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/8. dementia in two histologically confirmed cases of multiple sclerosis: one case with isolated dementia and one case associated with psychiatric symptoms.During the past 10 years, considerable attention has been devoted to cognitive impairment in multiple sclerosis. Occasionally this impairment may be so severe that multiple sclerosis presents as a dementia associated with only minor neurological signs and symptoms. The cases of two women affected by multiple sclerosis who presented with a pure dementia are reported. In the first patient, a progressive apragmatic behavioural disturbance with reduced short term memory and learning abilities were the main clinical features. Neuropathological examination of the brain disclosed numerous plaques in the periventricular white matter, with severe atrophy of the corpus callosum. Plaques were also seen in the white matter of both hippocampus and in the columns of the fornix. The impairment of short term memory could be linked to these lesions. Behavioural changes were probably related to the bilateral lesions of the long associative bundles that disconnected the frontal lobes from other parts of the cerebral hemispheres. In the second patient, visual hallucinations were associated with cognitive dysfunction. MRI showed large plaques in the white matter of both left frontal and temporal lobes. Smaller plaques were also present in the periventricular white matter of the occipital lobes, the nature of which were confirmed by a stereotactic biopsy.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |