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Hashimoto encephalopathy (HE) is a rare and often reversible neurological syndrome associated with autoimmune thyroiditis and steroid-responsiveness. This syndrome includes behavioral symptoms like delusions and delirium, mood disturbances, epilepsy, progressive cognitive impairment and alteration of vigilance and consciousness with confused state until coma. Two subtypes of clinical presentation are described: ictal onset with seizures and stroke like episode and insidious onset with progressive dementia. The pathogenesis is uncertain; several theories have been proposed: autoimmune, vasculitic and demyelinating. Here, we report the case of a patient with HE who was submitted to exhaustive neuropsychological exams in the premorbid and the acute phase, and following resolution of the acute phase. After the initial confusional state resolved, results of the neuropsychological exams revealed a diffuse pattern of cognitive impairment that eventually evolved toward a selective deficit in executive functions. This pattern of cognitive impairment suggests that, after an initial phase characterized by diffuse brain involvement, our patient was primarily affected by frontal lobe sufferance.  相似文献   

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Abstract. A 62-year-old woman, after a resection and ileostomy for multiple perforations of the terminal ileum and prolonged postoperative parenteral nutrition, developed thiamine deficiency with clinical and magnetic resonance imaging features of Wernickes disease. Later on the patient developed central pontine myelinolysis. For this condition, a pathogenetic role of a transient hypophosphatemia was suggested by both laboratory data and course of the disease.  相似文献   

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Wernicke’s encephalopathy (WE) is a severe brain disorder, first described in 1881, and is caused by a nutritional deficiency of thiamine (vitamin B1) found mostly in patients suffering from chronic alcoholism. In addition, WE can also complicate bariatric surgery if adequate vitamin supplementation is not insured. Without immediate treatment, the prognosis is poor and the mortality rate is high. Most patients present with atypical neurological symptoms, which hampers rapid diagnosis. We present a 40-year-old woman who underwent gastroplasty combined with gastric banding for severe obesity. She experienced repetitive vomiting and her diet was without vitamin supplementation. After three months she developed convergent strabismus, apathy and urinary incontinence, which was diagnosed as WE and treated as such. Six months later her recovery was incomplete, still showing gait difficulties and nystagmus. We aim to show that adequate vitamin supplementation in patients undergoing gastroplasty is necessary, especially considering the risk of permanent neurological deficits.  相似文献   

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ABSTRACT

We describe the case of a non-alcoholic patient with chronic myeloid leukemia who developed iatrogenic Wernicke’s encephalopathy (WE) following stem cell transplantation. Four years after the WE acute event, the patient’s cognitive profile was mainly characterized by moderate memory impairment, and functional and daily-living difficulties. Our report sustains the hypothesis that a iatrogenic form of WE may produce long-term cognitive sequelae even when thiamine therapy is administered in the acute phase until the resolution of the neurological signs.  相似文献   

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Wernicke's encephalopathy (WE) is a severe neurological disorder caused by thiamine deficiency. Clinically, it is most frequently observed in people with alcohol abuse. WE, however, can occur in any clinical condition associated with malnutrition or thiamine deficiency. We present the case of a 47-year-old woman with prolonged therapeutic fasting who presented with ophthalmoplegia, ataxia and deep coma. MRI showed unusual symmetric cortical abnormalities in the frontal and parietal lobes, as well as typical lesions surrounding the third ventricle and aqueduct. Although the patient entered a vegetative state, she finally regained consciousness after thiamine supplementation unexpectedly. To the best of our knowledge, it has never been reported to date that the patient with WE in a vegetative state with cortical damage shows a marvelous prognosis, which prompts us to report this case. In the present report, we highlight the role of MRI in the diagnosis of acute WE.  相似文献   

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The primary objective of this study was to test hypotheses about the relationship between HMPAO-SPECT findings and probable Alzheimer’s disease (DAT) in a relatively large sample of patients diagnosed according to DSM-III-R. SPECT patterns of 20 controls and 116 DAT patients were investigated. Left and right frontal, temporal, parietal and occipital regions of the brain were rated as showing a hypoperfusion or not. A wide variety of patterns were found and these are described in detail below. In DAT patients, temporal and/or parietal regions were affected significantly more often (88%, p > 0.001) than frontal and/or occipital regions (70%). A bilateral temporoparietal pattern, which has been repeatedly reported as typical for DAT, was observed in 48% of DAT patients, but also in 25% of controls, and did not differentiate significantly between these two groups (p > 0.05). Conversely, more than three regions with hypoperfusion were observed significantly more often in DAT patients (48%, p < 0.01) than in controls (10%). In DAT patients, the number of regions with hypoperfusion correlated significantly with the score of the Mini Mental State Examination (MMSE, r = 0.33, p < 0.001). The frequency of at least one hypoperfusion was approximately equal in left and right hemispheres (77% vs. 73%, p = 0.2). The hypothesis that cognitive decline in DAT starts in the temporal regions was tested in 14 SPECT patterns showing only one region with hypoperfusion. In 12 of these patterns, a temporal region was in fact affected (p < 0.001). Whereas hypoperfusion in frontal areas was not accompanied by a significantly lower MMSE than when only temporoparietal regions were affected, MMSE scores were significantly lower when occipital regions were affected in addition to temporoparietal regions (p < 0.05). The clinical use of SPECT findings was tested in discriminating analyses with the MMSE and a delayed recall test as additional predictors of DAT. Whereas the MMSE and the delayed recall test differentiated significantly between DAT patients and controls, SPECT findings yielded no further differentiation. In conclusion, the theoretical and clinical implications of SPECT findings and their relationships to other physiological and psychological variables deserve further investigation. Received: 3 December 1998 / Accepted: 1 April 1999  相似文献   

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Patients with Wernicke’s encephalopathy (WE) often have unusual patterns of vertical nystagmus. Initially there is often a spontaneous upbeating nystagmus that may change to downbeat nystagmus with a change in the direction of gaze, convergence or with vestibular stimuli. Patients also often show a profound loss of the horizontal but not the vertical vestibulo-ocular reflex (VOR). Furthermore, the acute upbeat nystagmus may change to a chronic downbeat nystagmus. We present hypotheses for these features based on (1) the location of vertical gaze-holding networks near the area postrema of the dorsomedial medulla where the blood–brain barrier is located, which we suggest becomes compromised in WE, (2) the location of the vestibular nuclei in the brainstem, medially for the horizontal VOR, and laterally for the vertical VOR, (3) neuronal circuits differ in susceptibility to and in the ability to recover from thiamine deficiency, and (4) impaired processing of otolith information in WE, normally used to modulate translational vestibulo-ocular reflexes, leads to some of the characteristics of the spontaneous vertical nystagmus including the peculiar reversal in its direction with a change in gaze or convergence.

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Hashimoto’s encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto’s thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966 U/mL. After one month of 60 mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved. Physicians should strongly consider this uncommon diagnosis in patients with rapid cognitive decline and no other clear etiology.  相似文献   

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There are no confirmatory or diagnostic tests or tools to differentiate between essential tremor (ET) and tremor in idiopathic Parkinson’s disease (PD). Although a number of imaging studies have indicated that there are differences between ET and PD, the functional imaging study findings are controversial. Therefore, we analyzed regional cerebral blood flow (CBF) by perfusion brain single-photon emission computed tomography (SPECT) to identify differences between ET and tremor-dominant Parkinson’s disease (TPD). We recruited 33 patients with TPD, 16 patients with ET, and 33 healthy controls. We compared the severity of tremor symptoms by comparing the Fahn-Tolosa-Marin rating scale (FTM) score and the tremor score from Unified Parkinson’s Disease Rating Scale (UPDRS) between TPD and ET patients. Subjects were evaluated by neuropsychological assessments, MRI and perfusion SPECT of the brain. Total FTM score was significantly higher in ET patients than TPD patients. However, there was no significant difference in FTM Part A scores between the two patient groups, while the scores for FTM Part B and C were significantly higher in ET patients than TPD patients. Brain SPECT analysis of the TPD group demonstrated significant hypoperfusion of both the lentiform nucleus and thalamus compared to the ET group. Brain perfusion SPECT may be a useful clinical method to differentiate between TPD and ET even during early-phase PD, because the lentiform nucleus and thalamus show differences in regional perfusion between these two groups during this time period. Additionally, we found evidence of cerebellar dysfunction in both TPT and ET.  相似文献   

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