scholarly journals Kikuchi-Fujimoto Disease With Encephalopathy in Children: Case Reports and Literature Review

2021 ◽  
Vol 9 ◽  
Author(s):  
Yu-Ting Pan ◽  
Li-Ming Cao ◽  
Yan Xu ◽  
Zhi-Dan Fan ◽  
Hai-Guo Yu

Background: Kikuchi-Fujimoto disease (KFD) is a benign and self-limiting disease characterized by regional lymphadenitis and low-grade fever. Encephalopathy may present in children with KFD. We present three cases of KFD with encephalopathy in children and a literature review.Methods: Literature published between 2010 and 2020 was reviewed to understand the clinical features, laboratory findings, and treatments for encephalopathy occurring in children with KFD.Results: The interval between KFD and onset of neurological symptoms was 10 days to 3 months. Laboratory results were normal, except for high protein levels in cerebrospinal fluid findings. Brain magnetic resonance imaging (MRI) findings include hyperintense T2 and FLAIR signal in the supratentorial white matter, deep gray matter, brain stem, cerebellum, temporal lobes, pons, and basal ganglia. Glucocorticoids and immunoglobulin could be effective for treating KFD with encephalopathy.Conclusion: The early clinical manifestations of KFD with encephalopathy in children lack specificity, and the diagnosis is mainly based on CSF analysis and brain MRI findings. Early and timely immunomodulatory therapy is effective and can improve the prognosis of patients with KFD with encephalopathy.

2018 ◽  
Vol 19 (1) ◽  
Author(s):  
Yali Yue ◽  
Yongkang Liu ◽  
Lina Song ◽  
Xiao Chen ◽  
Yaohui Wang ◽  
...  

2020 ◽  
Vol 26 (3) ◽  
pp. 143-148
Author(s):  
Gordana Todorović ◽  
Aleksandar Joldžić ◽  
Slađana Anđelić ◽  
Darko Nedeljković

Introduction/Objective Severe acute respiratory distress syndrome caused by coronavirus 2 (SARS-COV-2) is a new respiratory disease -COVID-19. A virus from the Coronaviridae family, highly contagious and virulent took over the world in a very short time causing the 2019/2020 pandemic. We are presenting the case of COVID-19 transmission among family members, patients of various ages, sex, clinical presentation and findings, who have been infected in different ways. Case reports Three patients are described, all with different coronavirus-specific symptomatology. Symptoms ranged from fatigue and loss of appetite with no other, more prominent symptoms in the youngest patient, to fever, high temperature, diarrhoea, muscle ache and chest pain during inspiration in the oldest patient. The third patient's dominant symptoms were dry, non-productive cough, lack of oxygen, shortness of breath and perspiration on exertion, headache and normal temperature, with radiographically confirmed bilateral pneumonia. Laboratory findings (leukopenia, lymphocytopenia with elevated C-reactive protein levels, high erythrocyte sedimentation rate and lactate dehydrogenase levels) were consistent with a viral infection, highly suspicious of SARS-COV-2, which was confirmed with a real-time RT-PCR test in all three patients. After being hospitalized in the Clinical Hospital Center "Zemun" Department of Pulmonology and treated with symptomatic, antiviral and antibiotic therapy, the disease regressed and the RT-PCR tests became negative. Conclusion SARS-COV-2 is a very aggressive and potent cause of the coronavirus disease. The presented cases confirm the possibility of quick transmission within a family through direct and indirect contact, as well as the diversity of symptoms, laboratory and clinical findings. Our clinical examples are similar in symptomatology and available results to cases from other parts of the world hit with the pandemic.


2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Rotem Semo Oz ◽  
Melissa S. Tesher

Abstract Background Lipopolysaccharide (LPS)-responsive and beige like anchor (LRBA) deficiency is categorized as a subtype of common variable immune deficiency (CVID). A growing number of case reports and cohorts reveal a broad spectrum of clinical manifestations and variable phenotype expression, including immune dysregulation, enteropathy and recurrent infections. The association between rheumatic disease and CVID generally has been well established, arthritis has been less frequently reported and minimal data regarding its clinical features and characteristic in LRBA deficiency has been published. This case report and literature review evaluates the characteristics and features of arthritis in LRBA deficiency patients. Case presentation and review results Herein, we describe a unique case of LRBA deficiency first presented with poly articular arthritis. Alongside the report, a literature review focusing on LRBA deficiency, rheumatic disease and arthritis has been conducted. We reviewed 43 publications. Among these, 7 patients were identified with arthritis. Age of first presentation was six weeks to 3 years. Male to female ratio was 4/3. Two patients were diagnosed with polyarticular Juvenile idiopathic arthritis (JIA) and three with oligoarticular JIA. Each patient was found to have different genomic mutation. The treatment was diverse and included corticosteroids, cyclosporine, methotrexate, adalidumab and abatacept. Conclusion Joint involvement is variable in LRBA deficiency, hence it should always be kept in mind as a differential diagnosis for a patient with combination of juvenile arthritis and clinically atypical immune dysregulation and / or immunodeficiency.


2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Ekaterina Baron ◽  
Vadim Gushchin ◽  
Mary Caitlin King ◽  
Andrei Nikiforchin ◽  
Armando Sardi

Background. Clinical decisions in patients with peritoneal dissemination of low-grade appendiceal mucinous neoplasms (LAMN) diagnosed during pregnancy are challenging. However, their slow progression and favorable prognosis allow deferring definitive treatment until after spontaneous delivery, a reasonable period of breastfeeding, and fertility preservation. Case Presentation. Two pregnant patients were incidentally diagnosed with LAMN and extensive peritoneal spread at 20 weeks gestation and at cesarean section. Treatment with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in both cases was delayed until spontaneous delivery at term and breastfeeding in the first patient and breastfeeding and fertility preservation in the second patient. Both patients remain disease-free for over 5 years, and their children are healthy. The literature review highlights the challenges that physicians face in treating pregnant patients with stage IV appendiceal tumors. Conclusion. Pregnancy management decisions in patients with peritoneal spread from mucinous appendiceal tumor should be based on understanding the tumor biology and prognosis. Definitive treatment in pregnant patients with favorable tumors, such as LAMN, may be delayed until spontaneous delivery without compromising maternal survival.


2019 ◽  
Vol 8 (2) ◽  
pp. 136
Author(s):  
Jae Han ◽  
Yun Bae ◽  
Seul Song ◽  
Jae-Jin Song ◽  
Ja-Won Koo ◽  
...  

The goal of this study was to elucidate radiologic biomarker that can predict the outcome of cochlear implantation (CI) in congenital cytomegalovirus (cCMV) related deafness. A retrospective survey of speech perception after CI and an evaluation of brain magnetic resonance imaging (MRI) findings were performed in 10 cochlear implantees with cCMV-related prelingual deafness. Specifically, a special attention was paid to the degree of white matter (WM) abnormality shown in brain MRI, which was used to divide our cohort into two groups: The mild and severe pathology groups. Age-matched prelingual deaf patients with idiopathic sensorineural hearing loss were selected as controls. Subjects in mild pathology groups showed higher a Category of Auditory Performance (CAP) score (5.2 ± 0.8) than those with severe pathologies (3.4 ± 1.5) (P = 0.041). Importantly, speech performance from subjects with mild pathology was comparable to that of the control group (mean CAP score of 5.2 ± 0.8 vs. 5.1 ± 1.2) (P = 0.898). Mild pathologies related to the limited WM lesion in MRI not accompanied by severe MRI pathologies, such as diffuse WM abnormality, myelination delay, ventriculomegaly, migration abnormality, and cerebellar hypoplasia, can be tolerated and do not adversely affect the CI outcome in cCMV deafness.


Neurosurgery ◽  
2010 ◽  
Vol 66 (6) ◽  
pp. E1206-E1207 ◽  
Author(s):  
Taylor J. Abel ◽  
Adam O. Hebb ◽  
C. Dirk Keene ◽  
Donald E. Born ◽  
Daniel L. Silbergeld

Abstract OBJECTIVE Corpora amylacea (CA) normally accumulate within perivascular, subpial, and subependymal astrocytic processes. CA are associated with a number of conditions including normal aging, hippocampal sclerosis associated with temporal lobe epilepsy, multiple sclerosis, Lafora-type progressive myoclonic epilepsy, and adult polyglucosan body disease. Reports of massive localized accumulation of CA in the brain outside of these conditions are rare. CLINICAL PRESENTATION A 49-year-old woman, with a long-standing history of migraine headaches, presented to her primary care provider for increased headache duration. Brain magnetic resonance imaging (MRI) revealed a left parahippocampal lesion, suggestive of low-grade glioma. INTERVENTION Given the MRI suggestive of left parahippocampal glioma, left-sided frontotemporal craniotomy was performed for resection of the lesion. Specimens obtained during the operation revealed focal high-density accumulation of CA with no evidence of neoplasm, ischemia, or hypoxic injury. CONCLUSION This case illustrates the possibility that localized high-density CA accumulation can present as an intrinsic lesion on brain MRI. CA should be included in the differential diagnosis for patients presenting with brain MRI suggestive of nonenhancing space-occupying lesions.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Amir M. Torabi

Bilateral medial medullary stroke is a very rare type of stroke, with catastrophic consequences. Early diagnosis is crucial. Here, I present a young patient with acute vertigo, progressive generalized weakness, dysarthria, and respiratory failure, who initially was misdiagnosed with acute vestibular syndrome. Initial brain magnetic resonance imaging (MRI) that was done in the acute phase was read as normal. Other possibilities were excluded by lumbar puncture and MRI of cervical spine. MR of C-spine showed lesion at medial medulla; therefore a second MRI of brain was requested, showed characteristic “heart appearance” shape at diffusion weighted (DWI), and confirmed bilateral medial medullary stroke. Retrospectively, a vague-defined hyperintense linear DWI signal at midline was noted in the first brain MRI. Because of the symmetric and midline pattern of this abnormal signal and similarity to an artifact, some radiologists or neurologists may miss this type of stroke. Radiologists and neurologists must recognize clinical and MRI findings of this rare type of stroke, which early treatment could make a difference in patient outcome. The abnormal DWI signal in early stages of this type of stroke may not be a typical “heart appearance” shape, and other variants such as small dot or linear DWI signal at midline must be recognized as early signs of stroke. Also, MRI of cervical spine may be helpful if there is attention to brainstem as well.


1994 ◽  
Vol 52 (1) ◽  
pp. 58-63 ◽  
Author(s):  
Marcelo P. Ferreira ◽  
Nelson P. Ferreira ◽  
Rene Lenhardt

Two patients with cerebellopontine angle (CPA) lipoma were studied. They were submitted to surgical treatment. Available literature was reviewed and 29 cases with same lesion were identified which had been treated by surgery. Clinical manifestations, possibility of diagnostic methods, surgical indications and treatment strategies are discussed. Attention is called to the peculiarities of CPA lipomas and the doubtful vality of attempting complete excision in all cases.


e-GIGI ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 256
Author(s):  
Yobel R. Woran ◽  
Lydia E. N. Tendean ◽  
Christy N. Mintjelungan

Abstract: The COVID-19 outbreak is caused by SARS-CoV-2 that spread rapidly throughout the world. The most common clinical manifestations of COVID-19 are fever, fatigue, and dry cough. Some patients experience nasal congestion, runny nose, headache, conjunctivitis, sore throat, diarrhea, skin rash, loss of smell and taste. Oral manifestations of COVID-19 infection are also reported. Dentists are prone to cross-infections of several infectious diseases because they are often exposed to saliva and blood. These viruses are transmitted through inhalation of aerosols and droplets containing the viruses or direct contact with mucous membranes, oral fluids, dental instruments, and surfaces contaminated with the virus. This study was aimed to determine the oral manifestations of COVID-19 infection. This was a literature review study searching three databases, namely Pubmed, ClinicalKey and Google Scholar. The keywords used were oral AND manifestations AND COVID-19. Selection based on inclusion and exclusion criteria was carried out by critical appraisal. There were eight literatures in the form of case reports. The results showed that oral manifestations commonly found in patients with clinical COVID-19 were ulcers, petechiae, macules, and plaques with variations in quantity, color appearance, and localization. Lesions were found on the palate, tongue, labial mucosa, gingiva, lips, and oropharynx. In conclusion, oral manifestations could be found in clinical COVID-19 patients, however, it is not certain whether these manifestations are directly caused by SARS-CoV-2 or are as secondary manifestations.Keywords: oral manifestations; COVID-19 Abstrak: Wabah COVID-19 disebabkan oleh SARS-CoV-2 yang menyebar dengan cepat hingga ke seluruh dunia. Infeksi COVID-19 mempunyai manifestasi klinis paling umum seperti demam, rasa lelah, dan batuk kering. Beberapa pasien mengalami hidung tersumbat, pilek, nyeri kepala, konjungtivitis, sakit tenggorokan, diare, ruam kulit, hilang penciuman dan pengecapan. Bahkan terdapat laporan penemuan manifestasi oral pada infeksi COVID-19. Dokter gigi sebagai profesi yang rentan terjadi infeksi silang beberapa penyakit menular karena sering terpapar dengan saliva dan darah. Virus ini memungkinkan terjadinya penularan lewat terhirupnya aerosol dan droplet yang mengandung virus atau kontak langsung dengan membran mukosa, cairan mulut, instrumen kedokteran gigi dan permukaan yang terkontaminasi virus. Penelitian ini bertujuan untuk mengetahui manifestasi oral infeksi COVID-19. Jenis penelitian ialah suatu literature review dengan pencarian menggunakan tiga database yaitu Pubmed, ClinicalKey dan Google Scholar. Kata kunci yang digunakan yaitu oral AND manifestations AND COVID-19. Setelah seleksi berdasarkan kriteria inklusi dan ekslusi dilakukan critical appraisal dan didapatkan delapan literatur yang berupa laporan kasus.Hasil penelitian mendapatkan bahwa manifestasi oral pada pasien klinis COVID-19 yang sering ditemukan, seperti ulkus, petekie, makula, dan plak dengan variasi kuantitas, penampakan warna, dan lokalisasi. Lokasi lesi ditemukan pada palatum, lidah, mukosa labial, gingiva, bibir dan orofaring. Simpulan penelitian ini ialah manifestasi oral dapat ditemukan pada pasien klinis COVID-19 tetapi belum diketahui pasti apakah secara langsung disebabkan oleh SARS-CoV-2 atau merupakan manifestasi sekunder.Kata kunci: manifestasi oral; COVID-19


Author(s):  
Razieh Fallah ◽  
Mohammad Javad Asadi ◽  
Reza Nafisi Moghadam ◽  
Mohammad Hossein Ahrar Yazdi

Introduction: In neonatal period, brain magnetic resonance imaging (MRI) is the best neuroimaging to find etiology of seizure. The aim of this study was to evaluate brain MRI findings of neonates with clinical seizure and its effect on diagnostic and therapeutic interventions of newborn. Methods: In a retrospective study, medical records and brain MRI findings of neonates with clinical seizure admitted to Neonatal Intensive Care Unit or Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran from September 2018 and before were evaluated. The data were analyzed using SPSS version 16 software , the required indicators and tables were prepared and Fisher exact test and Chi-square test were used to determine the relationship between qualitative variables and independent t-test was used to compare the means in the two groups Results: Twenty-five girls and 38 boys were studied. The cause of seizures was found in 94% and the most common cause of congenital hypoxia was in 22 infants (35%). The most common type of seizure was tonic in 23 infants (36.5%) and the most common type of generalized seizure was in 47 infants (75%). Brain MRI was abnormal in 19 neonates (30%). Based on the MRI results, there was a change in therapeutic interventions (brain surgery) in four infants (6.3%) and in diagnostic interventions (metabolic tests) in 19 infants (30%). Abnormal brain MRI was more frequent in neonates by cesarean section (46%)  than  normal vaginal delivery (19%), (p= 0.01) and also neonates with partial seizure (37.5%) had an abnormal MRI than  generalized seizure (21%), (p= 0.03). Mean of hospitalization days was longer in neonates with abnormal brain MRI (12.32±2.76 days) than neonates with normal MRI (8.57±2.82 days) (P = 0.02). Conclusion: Based on the results of this study, brain MRI might be useful in finding intracranial pathology that causes seizure in neonates by cesarean section to detect birth asphyxia and in newborns with partial seizure.  


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