We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI. The lesion was resected, and the histological and immunohistochemical studies revealed an IgG4-related tumefactive fibroinflammatory lesion of the retroperitoneum.

BACKGROUND: Immunoglobulin G4 (Ig G4)-related disease is rare; however, it is a fibroinflammatory disease that has been studied a lot so far. Although the expression pattern varies depending on the organ affected, it usually manifests as organ hypertrophy and organ dysfunction.
METHODS: A 46-year-old man was referred to our otorhinolaryngology department for left submandibular swelling and tenderness that occurred 2 weeks ago. He was treated with antibiotics (augmentin 625mg, per oral) for 2 weeks, but his symptoms did not improve, and his white blood cell (WBC) count was 10,500 /μL (normal 3,800-10,000 /μL).
CONCLUSIONS: A mass-like lesion of the submandibular space has been concluded and the laboratory findings have been satisfactory (IgG4 level); IgG4-related disease, which is rare, but recently often reported, can be included in the differential diagnosis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease that occurs predominantly in children. According to the guidelines, ADEM belongs to the myelin oligodendrocyte glycoprotein (MOG)-associated diseases and usually manifests after febrile infections (also after SARS-CoV-2) or postvaccinally.
OBJECTIVE: Incidence, course and clinical, and as well, as radiological features and new developments and treatment of ADEM.
METHODS: Analysis and review of the literature on ADEM and of notable cases and guidelines.
RESULTS: The first signs of ADEM include fever, nausea and vomiting, headache and meningism as well as, by definition, encephalopathy, which usually manifests as drowsiness and confusion. The radiological diagnosis is made by magnetic resonance imaging (MRI). Here, the asymmetrically distributed, diffuse and tumefactive lesions can be located supra- and infratentorially. In the acute phase, the lesions usually show contrast enhancement and restricted diffusion. Spinal involvement of the gray matter with the typical H‑pattern with myelitis transversa is not uncommon. ADEM has mostly a monophasic course, with a recurrent form (\"relapsing ADEM\") in 1-20% of cases. For treatment, steroids and in severe cases immunosuppressive drugs are used.
CONCLUSIONS: ADEM is generally a monophasic disease whose symptoms usually last for a few weeks or months. It is crucial to differentiate ADEM from other demyelinating diseases, like for example multiple sclerosis, in order not to delay the proper treatment.
UNASSIGNED: HINTERGRUND: Die akute disseminierte Enzephalomyelitis (ADEM) gehört zu den seltenen demyelinisierenden Erkrankungen, die meistens bei Kindern auftreten. ADEM gehört laut Leitlinien zu den Myelin-Oligodendrozyten-Glykoprotein(MOG)-assoziierten Krankheiten und manifestiert sich in der Regel nach febrilen Infektionen (auch nach SARS-CoV-2) oder, deutlich seltener, nach Impfungen.
UNASSIGNED: Inzidenz, Verlauf und klinische sowie radiologische Diagnostik sowie Entwicklung und Therapieoptionen von ADEM.
UNASSIGNED: Analyse und Auswertung der Literatur über ADEM sowie Analyse der bemerkenswerten Fälle und Leitlinien.
UNASSIGNED: Zu den ersten Anzeichen von ADEM gehören Fieber, Nausea bis zum Erbrechen sowie Kopfschmerzen und Meningismus sowie per definitionem eine Enzephalopathie, die am Anfang wenig ausgeprägt sein kann und sich meistens als Schläfrigkeit und Verwirrung manifestiert. Die radiologische Diagnose wird in der Magnetresonanztomographie (MRT) gestellt. Hier sind asymmetrisch verteilte, unscharf abgrenzbare, tumorsimulierende Läsionen supra- und infratentoriell abgrenzbar. In der akuten Phase nehmen die Läsionen meistens ringförmig Kontrastmittel auf und zeigen eine Diffusionsrestriktion. Spinaler Befall der grauen Substanz mit dem typischen H‑Muster mit Myelitis transversa ist nicht selten. Die ADEM hat meistens einen monophasischen Verlauf, wobei eine rekurrierende Form („relapsing ADEM“) in 1–20 % der Fälle zu erwarten ist. Bei der Behandlung kommen Steroide und in schweren Fällen Immunsuppressiva zum Einsatz.
UNASSIGNED: ADEM ist eine meist monophasische Erkrankung, deren Symptome nach einigen Wochen/Monaten abklingen sollten. Es ist wichtig, sie von anderen demyelinisierenden Krankheiten wie der multiplen Sklerose zu unterscheiden, um die nötige Therapie nicht zu verzögern.

Multiple sclerosis (MS) is the autoimmune, neurodegenerative disease of the central nervous system (CNS). Typically, it affects the young adult population, however, up to 10% of the cases, it can develop in childhood. Atypical manifestations, such as the tumefactive variant (tMS) or acute disseminated encephalomyelitis (ADEM), especially coupled with fulminant disease course, are even more rare and pose a considerable differential diagnostic and therapeutic challenge. Recently, the therapeutic strategy on the use of disease modifying therapies (DMTs) in MS has shifted to the direction of a more individualized approach, that takes the personal differences heavily into account, in particular regard to the activity and prognosis of the disease. Despite this change has only been applied to adults yet, it is plausible to predict, that it will soon be applied to pediatric patients as well, particularly, as several randomized studies are under way concerning DMTs in pediatric populations. To our best knowledge, we are the first to report a successful natalizumab treatment of pediatric fulminant tMS, in case of a 13.5 years old girl. We feel that this report demonstrates the need of early and adequate treatment in such an aggressive case, because it can reverse the course of a possibly fatal disease.

Acute disseminated encephalomyelitis is a monophasic demyelinating disorder of the central nervous system associated with various viral infections including HIV infection. We present the findings of seven HIV-infected patients with mild to moderate immunosuppression presenting with atypical features. Four patients had a multiphasic course; three patients had tumefactive lesions, and two patients had corpus callosum lesions. Two patients with the multiphasic course also had tumefactive lesions. Their clinical and radiological findings are presented. Despite the few cases, we propose that the dysimmune process lying between marked immunosuppression (CD4 < 200 cells/μL) and normal CD4 counts (CD4 > 500 cells/μL) might be responsible for these atypical presentations.

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