| We describe a patient who developed korsakoff syndrome following a spontaneous chronic subdural hematoma. The present case demonstrates persistence of both amnesia and confabulation long after recovery from the acute phase of spontaneous chronic subdural hematoma. There are few reports describing persistent amnesia with confabulation following brain damage. We considered that chronic subdural hematoma in the bilateral frontal and temporal lobes caused amnesia and confabulations, and these conditions persisted as a result of organic atrophic changes of both the frontal and temporal lobes due to long-term compression by chronic subdural hematoma. ( info) |
| We report the case of a 40-year-old alcoholic male patient, hospitalized with an acute ataxia of stance and gait, ocular muscle weakness with nystagmus and a global apathetic-confusional state. After admission, an amnestic syndrome with confabulation was also observed and diagnosis of Wernicke-korsakoff syndrome was made. Under treatment with intravenous thiamine, the patient recovered completely from gaze weakness and ataxia, whereas a severe amnestic syndrome persisted. Fluorodeoxyglucose (FDG) positron emission tomography (PET) showed bilateral thalamic and severe bilateral temporal-parietal hypometabolism resembling a pattern typical for Alzheimer's disease. Longitudinal assessment of the alcohol-abstinent and thiamine-substituted patient revealed improvements of clinical state and neuropsychological performance that were paralleled by recovered cerebral glucose metabolism. In contrast to metabolic rates that increased between 7.1% (anterior cingulate, left) and 23.5% (parietal, left) in cortical areas during a 9-month remission period, thalamic glucose metabolism remained severely disturbed over time (change: left 0.2%, right 0.3%). ( info) |
| AIMS: To investigate a possible relationship between cerebellar glucose metabolism and recovery from ataxia in the first months of acute Wernicke-korsakoff syndrome. methods: Two cases of alcoholic Wernicke-korsakoff syndrome were followed up with the clinical status and cerebral glucose metabolism over a 4- and 9-month period. RESULTS: Initially both patients showed severe ataxia and elevated cerebellar glucose metabolism that decreased corresponding to the restitution of stance and gait. CONCLUSION: Increased cerebellar glucose metabolism at the onset of the illness may reflect the reorganization process of disturbed motor skills and may indicate cerebellar plasticity. ( info) |
| There are limited case reports of structural lesions causing korsakoff syndrome. This report describes acute korsakoff syndrome following localized, bilateral infarction of the mammillothalamic tracts (MTTs). Axial T2-weighted imaging revealed the lesions at the lateral wall level of the third ventricle and diffusion-weighted imaging confirmed that the left lesion was new and the right old. korsakoff syndrome persisted 6 months after the onset. This case suggests that bilateral MTT dysfunction can lead to korsakoff syndrome. ( info) |
15/20. The cost of living in purgatory's shadow. A 40-year-old alcoholic man developed Wernicke-korsakoff syndrome. His examination showed ophthalmoplegia, ataxia and memory difficulties. His cranial magnetic resonance imaging scan showed increased signal in the paraventricular regions of the thalamus on T-2 weighted, diffusion and axial fluid-attenuated inversion recovery sequences consistent with the syndrome. The ophthalmoplegia resolved with thiamine administration; however, his memory did not improve. ( info) |
16/20. acetylcholinesterase inhibitors for the treatment of Wernicke-korsakoff syndrome--three further cases show response to donepezil. Three patients diagnosed with Wernicke-korsakoff syndrome were treated with the acetylcholinesterase inhibitor, donepezil, for periods of 6 to 8 months. Cognitive testing [Alzheimer's disease assessment scale-cognitive subscale (ADAS-Cog), Mini-mental state examination (MMSE), Clock drawing test and six item 2 min recall] and carer questionnaires [Informant Questionnaire (IQ Code), Neuropsychiatric inventory scale (NPI)] were performed at baseline, mid- and endpoint of the treatment period and post-discontinuation. Progressive partial improvement occurred in cognitive measurements through the treatment period, some of which was sustained after discontinuing donepezil. Carer questionnaires also indicated improvement. Confounding factors necessitate caution when attributing improvements to the medication, but these cases suggest that this option merits further investigation. ( info) |
17/20. Functional MRI study of diencephalic amnesia in Wernicke-korsakoff syndrome. Anterograde amnesia in Wernicke-korsakoff syndrome is associated with diencephalic lesions, mainly in the anterior thalamic nuclei. Whether diencephalic and temporal lobe amnesias are distinct entities is still not clear. We investigated episodic memory for faces using functional MRI (fMRI) in eight controls and in a 34-year-old man with Wernicke-korsakoff syndrome and diencephalic lesions but without medial temporal lobe (MTL) involvement at MRI. fMRI was performed with a 1.5 tesla unit. Three dual-choice tasks were employed: (i) face encoding (18 faces were randomly presented three times and subjects were asked to memorize the faces); (ii) face perception (subjects indicated which of two faces matched a third face); and (iii) face recognition (subjects indicated which of two faces belonged to the group they had been asked to memorize during encoding). All activation was greater in the right hemisphere. In controls both the encoding and recognition tasks activated two hippocampal regions (anterior and posterior). The anterior hippocampal region was more activated during recognition. Activation in the prefrontal cortex was greater during recognition. In the subject with Wernicke-korsakoff syndrome, fMRI did not show hippocampal activation during either encoding or recognition. During recognition, although behavioural data showed defective retrieval, the prefrontal regions were activated as in controls, except for the ventrolateral prefrontal cortex. fMRI activation of the visual cortices and the behavioural score on the perception task indicated that the subject with Wernicke-korsakoff syndrome perceived the faces, paid attention to the task and demonstrated accurate judgement. In the subject with Wernicke-korsakoff syndrome, although the anatomical damage does not involve the MTL, the hippocampal memory encoding has been lost, possibly as a consequence of the hippocampal-anterior thalamic axis involvement. Anterograde amnesia could therefore be the expression of damage to an extended hippocampal system, and the distinction between temporal lobe and diencephalic amnesia has limited value. In the subject with Wernicke-korsakoff syndrome, the preserved dorsolateral prefrontal cortex activation during incorrect recognition suggests that this region is more involved in either the orientation or attention at retrieval than in retrieval. The lack of activation of the prefrontal ventrolateral cortex confirms the role of this area in episodic memory formation. ( info) |
| Wernicke-korsakoff syndrome (WKS) and disordered eating behavior have been reported separately after bariatric surgery. We report a patient who following a bariatric operation developed WKS associated with a disturbed eating behavior without vomiting. This morbidly obese man developed an intense fear of gaining weight in the postoperative period and engaged in an extreme form of "food avoidance behavior". 2 months postoperatively after severe weight loss, he was hospitalized with disorientation and an amnesic syndrome. He was discharged 2 months later with stable weight and regular eating habits. Despite this, at the last follow-up visit 2 years postoperatively, he still had a residual partial amnesic syndrome. The surgical team must be aware of peculiar forms of pathological eating that may appear after bariatric surgery; the emergence of an eating avoidance disorder may be associated with the development of WKS. ( info) |
| PURPOSE: The authors hereby describe a case of Wernicke-korsakoff syndrome with accompanying ocular findings that is caused by hyperernesis gravidarum. methods: We experienced a 27-year-old female at 12 weeks of pregnancy, who visited our clinic because of weight loss, gait disturbance, decreased mentality and dizziness after prolonged vomiting for 2 months. Neurological examination demonstrated ataxia of gait and loss of orientation. Ophthalmologic examination showed decreased visual acuity, upbeat nystagmus, diplopia and retinal hemorrhage. RESULTS: We report a relatively rare case of Wernicke-korsakoff syndrome with ophthalmic symptoms induced by hyperemesis gravidarum. CONCLUSIONS: If a pregnant women has symptoms of severe vomiting along with other ocular findings such as retinal hemorrhage or restricted extraocular movement, one must suspect the diagnosis of Wernicke-korsakoff syndrome, and should start appropriate treatment immediately. ( info) |
20/20. The anatomy of amnesia: neurohistological analysis of three new cases. The most useful information about the anatomy of human memory comes from cases where there has been extensive neuropsychological testing followed by detailed post-mortem neurohistological analysis. To our knowledge, only eight such cases have been reported (four with medial temporal lobe damage and four with diencephalic damage). Here we present neuropsychological and post-mortem neurohistological findings for one patient (NC) with bilateral damage to the medial temporal lobe and two patients (MG, PN) with diencephalic damage due to bilateral thalamic infarction and Korsakoff's syndrome, respectively. All three patients exhibited a similar phenotype of amnesia with markedly impaired declarative memory (anterograde and retrograde) but normal performance on tests of nondeclarative memory (e.g., priming and adaptation-level effects) as well as on tests of other cognitive functions. Patient NC had damage to the hippocampus (dentate gyrus and the CA1 and CA3 fields) and layer III of the entorhinal cortex, but with relative sparing of the CA2 field and the subiculum. Patient MG had damage to the internal medullary lamina and mediodorsal thalamic nuclei. Patient PN had damage to the mammillary nuclei, mammillothalamic tracts, and the anterior thalamic nuclei. These findings illuminate several issues regarding the relation between diencephalic and medial temporal lobe amnesia, the status of recognition memory in amnesia, and the neuroanatomy of memory. ( info) |