A clinical case of pseudotumorous chronic parainfectious limbic encephalitis

Parainfectous limbic encephalitis (PILE) associated with viruses of the Herpesviridae family is one of the forms of chronic herpes encephalitis characterized by limbic system dysfunction and a prolonged course with frequent exacerbations. There are two types of the course of the disease: latent auto...

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Main Authors: N. A. Shnaider, Yu. S. Panina, T. E. Popova
Format: Article
Language:Russian
Published: IMA-PRESS LLC 2014-10-01
Series:Неврология, нейропсихиатрия, психосоматика
Subjects:
Online Access:https://nnp.ima-press.net/nnp/article/view/437
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author N. A. Shnaider
Yu. S. Panina
T. E. Popova
author_facet N. A. Shnaider
Yu. S. Panina
T. E. Popova
author_sort N. A. Shnaider
collection DOAJ
description Parainfectous limbic encephalitis (PILE) associated with viruses of the Herpesviridae family is one of the forms of chronic herpes encephalitis characterized by limbic system dysfunction and a prolonged course with frequent exacerbations. There are two types of the course of the disease: latent autoimmune limbic encephalitis (LE) progressing to mesial temporal sclerosis and pseudotumorous granulomatous LE. The latter (inflammatory pseudotumor or granuloma) is characterized by the formation of a polymorphic inflammatory infiltrate with the elements of fibrosis, necrosis, and a granulomatous reaction and by myofibroblast cells. This is a slowly growing benign pseudotumor that contains much more plasma cells than inflammatory ones. The diagnosis of pseudotumorous LE is difficult and requires the participation of a neurologist, an immunologist, an oncologist, and a neurosurgeon. Perfusion computed tomography, magnetic resonance imaging, and magnetic resonance spectroscopy give proof to the adequacy of the term inflammatory pseudotumor because it is histologically difficult to characterize the lesion as a tumor or inflammation. When a chronic lesion in the central nervous system is lately diagnosed, the prognosis of the disease may be poor and complicated by the development of resistant symptomatic focal epilepsy and emotional, volitional, and cognitive impairments. It was differentially diagnosed from brain tumors (astrocytic, oligodendroglial, and mixed gliomas, ependymal, neuronal, neuroglial, and embryonal tumors, meningiomas, cholesteatomas, dermoid cysts, teratomas, and cysts), other reactive and inflammatory processes (leukemic infiltrations, systemic lupus erythematosus, multiple sclerosis, encephalomyelitis), hypoparathyroidism, Addison's disease, vitamin A intoxication, and the long-term use of glucocorticoids and contraceptives. The authors describe a clinical case of the pseudotumorous course of chronic PILE in a 28-year-old woman. They discuss difficulties in differential diagnosis and the specific features of the clinical course and treatment of the disease. Inflammatory pseudotumor is an indication for surgery, but realizing the fact that this false tumor may avoid an unnecessary radical operation in some cases, which demonstrates the given clinical observation.
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spelling doaj.art-da09dc445508457d86e7054c0ec844072023-03-13T08:42:14ZrusIMA-PRESS LLCНеврология, нейропсихиатрия, психосоматика2074-27112310-13422014-10-0163495410.14412/2074-2711-2014-3-49-54424A clinical case of pseudotumorous chronic parainfectious limbic encephalitisN. A. Shnaider0Yu. S. Panina1T. E. Popova2Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia, Krasnoyarsk, Russia Neurological Center of Epileptology, Neurogenetics, and Brain Research, University Clinic, Krasnoyarsk, RussiaProf. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia, Krasnoyarsk, RussiaProf. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia, Krasnoyarsk, Russia Neurological Center of Epileptology, Neurogenetics, and Brain Research, University Clinic, Krasnoyarsk, RussiaParainfectous limbic encephalitis (PILE) associated with viruses of the Herpesviridae family is one of the forms of chronic herpes encephalitis characterized by limbic system dysfunction and a prolonged course with frequent exacerbations. There are two types of the course of the disease: latent autoimmune limbic encephalitis (LE) progressing to mesial temporal sclerosis and pseudotumorous granulomatous LE. The latter (inflammatory pseudotumor or granuloma) is characterized by the formation of a polymorphic inflammatory infiltrate with the elements of fibrosis, necrosis, and a granulomatous reaction and by myofibroblast cells. This is a slowly growing benign pseudotumor that contains much more plasma cells than inflammatory ones. The diagnosis of pseudotumorous LE is difficult and requires the participation of a neurologist, an immunologist, an oncologist, and a neurosurgeon. Perfusion computed tomography, magnetic resonance imaging, and magnetic resonance spectroscopy give proof to the adequacy of the term inflammatory pseudotumor because it is histologically difficult to characterize the lesion as a tumor or inflammation. When a chronic lesion in the central nervous system is lately diagnosed, the prognosis of the disease may be poor and complicated by the development of resistant symptomatic focal epilepsy and emotional, volitional, and cognitive impairments. It was differentially diagnosed from brain tumors (astrocytic, oligodendroglial, and mixed gliomas, ependymal, neuronal, neuroglial, and embryonal tumors, meningiomas, cholesteatomas, dermoid cysts, teratomas, and cysts), other reactive and inflammatory processes (leukemic infiltrations, systemic lupus erythematosus, multiple sclerosis, encephalomyelitis), hypoparathyroidism, Addison's disease, vitamin A intoxication, and the long-term use of glucocorticoids and contraceptives. The authors describe a clinical case of the pseudotumorous course of chronic PILE in a 28-year-old woman. They discuss difficulties in differential diagnosis and the specific features of the clinical course and treatment of the disease. Inflammatory pseudotumor is an indication for surgery, but realizing the fact that this false tumor may avoid an unnecessary radical operation in some cases, which demonstrates the given clinical observation.https://nnp.ima-press.net/nnp/article/view/437limbic systemencephalitisherpesinflammatory pseudotumorepilepsy
spellingShingle N. A. Shnaider
Yu. S. Panina
T. E. Popova
A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
Неврология, нейропсихиатрия, психосоматика
limbic system
encephalitis
herpes
inflammatory pseudotumor
epilepsy
title A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
title_full A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
title_fullStr A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
title_full_unstemmed A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
title_short A clinical case of pseudotumorous chronic parainfectious limbic encephalitis
title_sort clinical case of pseudotumorous chronic parainfectious limbic encephalitis
topic limbic system
encephalitis
herpes
inflammatory pseudotumor
epilepsy
url https://nnp.ima-press.net/nnp/article/view/437
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