Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011551 (depersonalization)
1,117 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In his classic overview, Mayer-Gross indicated two clinical features of depersonalization to be taken as starting points for future investigation: excessive difficulty in describing it and its relatively rare appearance in organic disorders. Neither characteristic has so far been discussed sufficiently in psychopathology and neurobiology. In this article, we examine the language aspect of depersonalization by comparisons with aphasia, in which the two objects of study described by Mayer-Gross, speech and organic disorders, intertwine. Concerning amnestic aphasia, Gelb and Goldstein insist that an object cannot be grasped as a generally understood fact using a categorical attitude but only experienced subjectively in its this-ness with a concrete attitude. The particular experience of depersonalization is the reverse of that in amnestic aphasia, as the relation of the depersonalized patient to this-ness is disturbed but an ideal view of the generality remains.
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PMID:[The psychopathology of depersonalization]. 1652 52

Background: Alice in Wonderland syndrome (AIWS) is a rare neurological disorder characterized by distortions of visual perception (metamorphopsias), the body image, and the experience of time, along with derealization and depersonalization. Some 85% of patients present with perceptual distortions in a single sensory modality, e.g., only visual or only somesthetic in nature. Moreover, the majority experience only a single type of distortion, e.g., only micropsia or only macropsia. AIWS has many different etiologies, and hence an extensive differential diagnosis. Its amenability to treatment depends on the underlying pathological process, which in children is mostly encephalitis, and in adults, migraine. In the literature, no more than 180 "clinical" cases of AIWS have been described (i.e., cases in need of medical attention). Of them, some 50% showed a favorable prognosis. However, non-clinical cases (i.e., fleeting, transient cases of AIWS for which no professional help is needed) have been described in up to 30% of the general population. This indicates that AIWS is perhaps not as rare as traditionally assumed, and has led some authors to conclude that, prognostically, AIWS is usually harmless. Methods: From our own clinical practice, we describe the first known case of Creutzfeldt-Jakob Disease (CJD, Heidenhain variant) that presented with symptoms of AIWS. Results: In our patient, disease onset was sudden and rapidly progressive, starting with isolated visual symptoms. Symptoms of AIWS comprised akinetopsia, chloropsia, micropsia, macropsia, zoom vision, and time distortions (quick-motion phenomenon and protracted duration). Soon, these were complicated by paraesthesias, gait instability, aphasia, expressive amusia, cognitive decline, and behavioral changes in the form of agitation and emotional lability. The diagnosis of probable sporadic CJD was confirmed with the aid of a head MRI and cerebrospinal fluid (14-3-3 protein). In the absence of any treatment options, our patient was discharged home and died within 2 months after his visual symptoms had begun. Autopsy consent was not obtained. Conclusion: We conclude that AIWS is not always as harmless as sometimes suggested, and that CJD, although extremely rare, must be part of its extensive differential diagnosis, notably in the presence of rapid cognitive decline.
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PMID:Alice in Wonderland Syndrome as a Presenting Manifestation of Creutzfeldt-Jakob Disease. 3114 56