1/43. Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature.Hughes (antiphospholipid) syndrome (APS) can mimic multiple sclerosis (MS). We analyzed the clinical, laboratory, and imaging findings of MS-like expression in a cohort of patients with APS in an attempt to identify parameters that might differentiate the 2 entities. We studied 27 patients who were referred to our unit with the diagnosis of probable or definite MS made by a neurologist. All patients were referred to our lupus clinic because of symptoms suggesting an underlying connective tissue disease, uncommon findings for MS on magnetic resonance imaging (MRI), atypical evolution of MS, or antiphospholipid antibody (aPL) positivity. aPL, antinuclear antibody (ANA), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies were measured by standard methods. MRI was performed in every patient and compared with MRI of 25 definite MS patients who did not have aPL. An index severity score was calculated based on the size and number of increased signal intensity areas in MRI. In the past medical history, 8 patients with primary APS and 6 with APS secondary to systemic lupus erythematosus (SLE) had had symptoms related to these conditions. Neurologic symptoms and physical examination of the patients were not different from those common in MS patients. Laboratory findings were not a useful tool to distinguish APS from MS. When MRI from APS patients was compared globally with MRI from MS patients, MS patients had significantly increased severity score in white matter (p < 0.001), cerebellum (p = 0.035), pons (p < 0.015), and when all areas were taken together (p < 0.001). patients with APS had significantly increased scores in the putamen (p < 0.01). No differences were noticed in the degree of atrophy. When taken individually, MRI from APS patients could not be distinguished from MRI from MS patients. Most of the patients with primary APS showed a good response to oral anticoagulant treatment. In patients with secondary APS, the outcome was poorer. Hughes syndrome (APS) and MS can be difficult to distinguish. A careful medical history, a previous history of thrombosis and/or fetal loss, an abnormal localization of the lesions in MRI, and the response to anticoagulant therapy might be helpful in the differential diagnosis. We believe that testing for aPL should become routine in all patients with MS.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/43. 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 = physical (Clic here for more details about this article) |
3/43. Current therapy of multiple sclerosis.The list of medications for both immune modulation and symptomatic relief continues to grow. Ideally, however, drug therapy should be part of a multidisciplinary approach that also includes such elements as patient education and physical therapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/43. Perinephric abscesses in the neurologically impaired.STUDY DESIGN: Retrospective chart review. OBJECTIVES: To document the occurrence and management of large perinephric abscesses in neurologically impaired patients at high risk for this infectious complication. SETTING: US veterans Affairs hospital. methods: The records, radiographs, operative findings and outcomes of all patients who presented with perinephric abscesses evident on physical exam within the last 5 years were reviewed. RESULTS: Four patients presented with large perinephric abscesses evident on physical examination. All had severe neurologic impairment with high sensory levels; three had spinal cord injuries, one had advanced multiple sclerosis. All had neurogenic bladders and recurrent urinary tract infections. The diagnosis was made through a combination of history, physical examination and computed tomography (CT) examination. All were found to have upper tract obstruction. All were managed with immediate abscess drainage and three had elective nephrectomy once the infection had resolved. No patients died of their perinephric abscess. CONCLUSIONS: These four cases illustrate that although advances in antibiotics, imaging and percutaneous management have improved the speed of diagnosis and reduced the mortality in patients with perinephric abscesses, the neurologically impaired population continues to remain at significant risk for the development and the delayed diagnosis of these morbid renal infections.- - - - - - - - - - ranking = 3keywords = physical (Clic here for more details about this article) |
5/43. Monofocal acute inflammatory Demyelination (MAID): a unique disorder simulating brain neoplasm.BACKGROUND: Monofocal acute inflammatory demyelinating (MAID) lesions present as large masses that mimic brain tumors. The natural history and nosologic classification are not well defined. methods: Five patients with MAID from our files and 4 from the literature are presented. We defined MAID as a mass-like lesion, >4 cm in diameter. Demyelination was pathologically proven in all patients. RESULTS: Headaches and motor deficits were the most common presenting symptoms. language problems and motor deficits were the most common findings on physical examination. After a median follow-up of 44 months, none of our 5 patients had multiple sclerosis. In similar cases, additional lesions indicative of multiple sclerosis developed within an average of 8 months. CONCLUSIONS: It may be difficult to distinguish MAID from brain tumor. It may be that MAID represents a unique form of isolated demyelinating disease, or it may represent transition to multiple sclerosis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/43. Cognitive presentation of multiple sclerosis: evidence for a cortical variant.BACKGROUND: Although neuropsychiatric complications are well recognised, the presentation of multiple sclerosis with cognitive or neuropsychiatric symptoms has generally been considered a rare occurrence and to reflect subcortical pathology. OBJECTIVES: To document the clinical, neuropsychological, and radiological features of six cases of cognitive presentation of multiple sclerosis, to review the relevant literature, and to propose a possible cortical basis for this clinical presentation. SUBJECTS: Six patients (five women; age range 38 to 60 years) presented to the memory and cognitive disorders clinic in Cambridge with an initially undiagnosed cognitive/neuropsychiatric syndrome. All underwent neuropsychological evaluation, brain imaging, and ancillary investigations to establish a diagnosis of multiple sclerosis. RESULTS: The six cases all had a progressive dementia syndrome with prominent amnesia, often accompanied by classic cortical features including dysphasia, dysgraphia, or dyslexia. Mood disturbance was ubiquitous and in three patients there was a long history of preceding severe depression. All six developed characteristic physical signs on follow up, with marked disabilities. A review of 17 previously reported cases highlighted the prominence of memory impairment and depression in the early stages. CONCLUSIONS: On clinical, pathological, and radiological grounds, the neuropsychiatric presentation of multiple sclerosis may represent a clinicopathological entity of "cortical multiple sclerosis." Failure to recognise this will delay diagnosis and may expose patients to potentially dangerous and invasive investigation. Because the neuropsychiatric features of cortical multiple sclerosis are a major cause of handicap, their early recognition may be particularly important in view of emerging treatments.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/43. Normal conduction in pathways traversing an asymptomatic multiple sclerosis plaque.A 31-year-old woman developed right facial myokymia as the initial manifestation of multiple sclerosis (MS). An MRI scan revealed a focal signal abnormality confined to the left dorsolateral pontomedullary region. brain-stem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SEPs), and blink reflex (BR) failed to show a conduction abnormality through the left brain-stem lesion. Instead, BAEP and BR indicated a conduction defect in the right pons and EMG showed myokymic discharges in right facial muscles. Our findings provide rare documentation of normal conduction through a presumably asymptomatic MS plaque. The abnormal MRI signal likely represents tissue edema, rather than demyelination. This case demonstrates that physical findings in MS patients may correlate better with electrophysiological abnormalities than with MRI abnormalities.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/43. The Cotard syndrome. Report of two patients: with a review of the extended spectrum of 'delire des negations'.The Cotard syndrome is characterized by the delusion where an individual insists that he has died or part of his body has decayed. Although described classically in schizophrenia and bipolar disorder, physical disorders including migraine, tumour and trauma have also been associated with the syndrome. Two new cases are described here, the one associated with arteriovenous malformations and the other with probable multiple sclerosis. The delusion has been embarrassing to each patient. Study of such cases may have wider implications for the understanding of the psychotic interpretation of body image, for example that occurring in anorexia nervosa.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/43. Childhood-onset multiple sclerosis and mood disorders: a case study.multiple sclerosis (MS) is rare in children. Little research exists regarding emotional and behavioral disorders in childhood-onset MS, despite the occurrence of such problems in adults with MS. This paper describes the cognitive and behavioral characteristics of a boy diagnosed with MS at age 9 and mood disorder at age 10. He displayed no cognitive or behavioral problems prior to the onset of physical symptoms of MS. Three years after diagnosis, this child showed persistent problems with speed of processing, visual-motor skills, and parent and teacher-reported executive functioning. In addition, he had difficulties with emotional lability, behavioral disinhibition, depression, and social interaction. As with adults, children with MS may be at increased risk for mood disorder compared to their peers. mood disorders in children with MS are likely to be multiply determined, although the specific causal mechanisms are unknown.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/43. multiple sclerosis: long-term remission after a high dose of cyclophosphamide.The objective of this case report is to document the possibility that immunoablative doses of cyclophosphamide may provide a long-term remission of multiple sclerosis (MS). We report the case of a 48-year-old woman with definite MS diagnosed in 1994 who has been in complete remission since a dose of 3800 mg of cyclophosphamide was accidentally given intravenously in early 1997. For 7 years there have been no signs of disease activity on history, physical examination, or on high-quality magnetic resonance imaging (MRI) with appropriate contrast-enhancement methodology. This case includes information on the possibility that less aggressive chemotherapy than that used with stem cell transplantation may be effective in the long-term control of MS.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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