A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system
Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesio...
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| Format: | Article |
| Language: | Russian |
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IMA-PRESS LLC
2020-12-01
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| Series: | Неврология, нейропсихиатрия, психосоматика |
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| Online Access: | https://nnp.ima-press.net/nnp/article/view/1479 |
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| author | L. V. Lukina V. A. Mikhailov N. I. Ananyeva G. E. Mazo L. I. Sitnik E. D. Kasyanov |
| author_facet | L. V. Lukina V. A. Mikhailov N. I. Ananyeva G. E. Mazo L. I. Sitnik E. D. Kasyanov |
| author_sort | L. V. Lukina |
| collection | DOAJ |
| description | Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material. |
| first_indexed | 2024-04-10T02:01:11Z |
| format | Article |
| id | doaj.art-a7cd4f68e65c4bc2946e98b429c61bcb |
| institution | Directory Open Access Journal |
| issn | 2074-2711 2310-1342 |
| language | Russian |
| last_indexed | 2024-04-10T02:01:11Z |
| publishDate | 2020-12-01 |
| publisher | IMA-PRESS LLC |
| record_format | Article |
| series | Неврология, нейропсихиатрия, психосоматика |
| spelling | doaj.art-a7cd4f68e65c4bc2946e98b429c61bcb2023-03-13T08:42:20ZrusIMA-PRESS LLCНеврология, нейропсихиатрия, психосоматика2074-27112310-13422020-12-0112611712310.14412/2074-2711-2020-6-117-1231075A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous systemL. V. Lukina0V. A. Mikhailov1N. I. Ananyeva2G. E. Mazo3L. I. Sitnik4E. D. Kasyanov5V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthV.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthV.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthV.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthV.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthV.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Ministry of HealthLangerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material.https://nnp.ima-press.net/nnp/article/view/1479langerhans cell histiocytosiscentral nervous systemfixation amnesianeuroimaging |
| spellingShingle | L. V. Lukina V. A. Mikhailov N. I. Ananyeva G. E. Mazo L. I. Sitnik E. D. Kasyanov A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system Неврология, нейропсихиатрия, психосоматика langerhans cell histiocytosis central nervous system fixation amnesia neuroimaging |
| title | A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system |
| title_full | A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system |
| title_fullStr | A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system |
| title_full_unstemmed | A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system |
| title_short | A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system |
| title_sort | case of fixation amnesia in langerhans cell histiocytosis involving the central nervous system |
| topic | langerhans cell histiocytosis central nervous system fixation amnesia neuroimaging |
| url | https://nnp.ima-press.net/nnp/article/view/1479 |
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