scholarly journals Antibody Negative Autoimmune Encephalitis: A Case Report

2020 ◽  
Vol 33 (13) ◽  
Author(s):  
Carolina Sequeira ◽  
Pedro Lopes

Encephalitis is characterized by inflammation of the brain. Literature describes autoimmune as one of the most common aetiology of non-infectious encephalitis. Given the similarities in clinical, imagological and laboratory findings with viral encephalitis and due to the wide variety of clinical features, the diagnosis is rather challenging and therefore physicians need an increased clinical suspicion to make the correct diagnosis. We report a case of a 35-year-old male with no past medical history that presented with two episodes of autoimmune encephalitis in a 6-month period. Despite having the typical clinical presentation and imagological findings consistent with autoimmune encephalitis, this case had negative results for antibodies, which delayed the diagnosis. It is essential to highlight the importance of considering the hypothesis of autoimmune aetiology on the differential diagnosis of all patients presenting with clinical and magnetic resonance imaging results suggestive of probable encephalitis, regardless of the negative antibodies results. This case clearly depicts the difficulties of diagnosing and treating an autoimmune encephalitis. The main goal of this case report is to increase awareness towards early diagnosis to promptly implement a specific treatment that has proven to improve the outcome and prognosis.


2021 ◽  
Vol 74 (1-2) ◽  
pp. 33-37
Author(s):  
Sanja Jakovljevic ◽  
Ljuba Vujanovic ◽  
Dejan Ogorelica ◽  
Aleksandra Fejsa-Levakov ◽  
Jasmina Sekulic

Introduction. Grover?s disease is characterized by pruriginous polymorphic rash with a variable course and duration. Although the etiology is still unknown, the disease is often associated with other dermatoses, malignant diseases, use of certain medications, as well as immunosuppression. Case Report. We report a case of a 70-year-old male patient who was referred for examination to the Clinic of Dermatovenereology Diseases, Clinical Center of Vojvodina, due to a rash that lasted for nine months. The first lesions on the skin appeared around the nipples as exudative eczematous plaques. A few months later, identical lesions appeared on the lower legs. During treatment with systemic antihistamines and topical corticosteroids, there were episodes of transient improvements and re-exacerbations. In the meantime, erythematous brownish, round and oval papules appeared on the abdomen and the back, accompanied by intense itch. Laboratory findings revealed eosinophilia and elevated serum immunoglobulin E levels. A skin biopsy of the back lesion was performed and the histopathological examination confirmed the diagnosis of Grover?s disease. After the systemic treatment using corticosteroids and antihistamines, with gradual dose reduction and application of topical corticosteroids and emollients, complete regression of the skin lesions was achieved. Conclusion. Since the clinical manifestations of the disease may be nonspecific and discrete, dermatopathological analysis is of crucial importance in making the correct diagnosis. In patients with atopy, the treatment with systemic corticosteroids, antihistamines and topical agents may lead to regression of skin lesions with a significant improvement in the quality of life.



2020 ◽  
Vol 1 (2) ◽  
pp. 143-148
Author(s):  
Nikola Grubor ◽  
Dragan Erić ◽  
Ivana Pavlović ◽  
Goran Tasić ◽  
Slavko Matić

Introduction: Benign tumors of the spleen are a rare condition. Their pathogenesis and clinical presentation represent a diagnostic and surgical challenge in the sense of establishing the correct diagnosis and selecting the appropriate surgical approach. Case report: In our paper, we are presenting a 45-year-old female patient who was initially tested and examined because of nonspecific complaints manifesting as occasional pain in the left upper quadrant of the abdomen. The values of the laboratory findings were within the reference range and preoperative radiological diagnostics indicated the existence of a mass in the spleen which had the features of a tumor. Based on the preoperative diagnostic finding we decided to perform laparoscopic splenectomy. Postoperative recovery was without adverse events, and the patient has prescribed the necessary vaccination and antibiotic prophylaxis. Conclusion: Myofibroblastic tumor of the spleen represents, as far as its origin is concerned, a mystery, with its very limited and nonspecific presentation of symptoms, which is why each individual case must be approached seriously. Laparoscopy has a very important role in the treatment of this disease, bearing in mind all the advantages of a minimally invasive approach over open surgery.



F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 60 ◽  
Author(s):  
Rashmi Kumar

Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae  globally. Causes are diverse and include viral and non-viral infections of the brain as well as autoimmune processes. In the West, the autoimmune encephalitides are now more common than any single infectious cause, but, in Asia, infectious causes are still more common. In 2006, the World Health Organization coined the term “acute encephalitis syndrome”, which simply means acute onset of fever with convulsions or altered consciousness or both. In 2013, the International Encephalitis Consortium set criteria for diagnosis of encephalitis on basis of clinical and laboratory features. The most important infectious cause in the West is herpes simplex virus, but globally Japanese encephalitis (JE) remains the single largest cause. Etiologic diagnosis is difficult because of the large number of agents that can cause encephalitis. Also, the responsible virus may be detectable only in the brain and is either absent or transiently found in blood or cerebrospinal fluid (CSF). Virological diagnosis is complex, expensive, and time-consuming. Different centres could make their own algorithms for investigation in accordance with the local etiologic scenarios. Magnetic resonance imaging (MRI) and electroencephalography are specific for few agents. Clinically, severity may vary widely. A severe case may manifest with fever, convulsions, coma, neurologic deficits, and death. Autoimmune encephalitis (AIE)  includes two major categories: (i) classic paraneoplastic limbic encephalitis (LE) with autoantibodies against intracellular neuronal antigens (Eg: Hu and Ma2) and (ii) new-type AIE with autoantibodies to neuronal surface or synaptic antigens (Eg: anti-N-methyl-D-aspartate receptor). AIE has prominent psychiatric manifestations: psychosis, aggression, mutism, memory loss, euphoria, or fear. Seizures, cognitive decline, coma, and abnormal movements are common. Symptoms may fluctuate rapidly. Treatment is largely supportive. Specific treatment is available for herpesvirus group and non-viral infections. Various forms of immunotherapy are used for AIE.



2020 ◽  
Vol 31 (01) ◽  
pp. 076-082
Author(s):  
Doug Garrison ◽  
Laura Barth ◽  
David Kaylie ◽  
Kristal Riska

AbstractDizziness is a common complaint that can arise from multiple systems in the body. Objective vestibular tests are used to understand the underlying function of the vestibular system and whether or not it may be contributing to the dizziness symptoms experienced by the patient. Even when comprehensive case history is consistent with an otologic etiology, audiometric and vestibular tests are ordered to objectively characterize inner ear function to help further refine the differential diagnoses and aid in guiding treatment options. Few reports in the literature describe audiometric and vestibular results in patients with multiple concurrent otologic etiologies.This case provides a description of audiometric, vestibular, and imaging results in a case of concurrent bilateral superior canal dehiscence, right-sided vestibular schwannoma, and right-sided posterior canal benign paroxysmal positional vertigo. The patient’s symptoms and laboratory findings are described in detail and, where appropriate, highlight challenges that may arise in interpretation.A case report.The patient presented for evaluation of dizziness, asymmetric hearing loss, and autophony. Comprehensive audiometric evaluation shows asymmetric sensorineural hearing loss and an air-bone gap at 250 Hz in the right ear. Vestibular evaluation shows right caloric asymmetry along with abnormal cervical vestibular– and ocular vestibular–evoked myogenic potentials, with the left ear showing results consistent with the third-window pathology.Comprehensive assessment of symptoms and critical thinking while performing testing are necessary when examining multiple concurrent otologic etiologies in a patient. Knowledge of anticipated test results and physiology may help the audiologist to synthesize results and make appropriate clinical recommendations as part of the multidisciplinary team.



2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Deepak Vallabhaneni ◽  
Muhammad Atif Naveed ◽  
Rajiv Mangla ◽  
Awss Zidan ◽  
Rashi I. Mehta

Encephalitis is characterized by inflammation of brain tissue and has various infectious and noninfectious causes. CSF analysis and MRI usually reveal inflammatory changes although sometimes brain imaging may be normal. Autoimmune encephalitis is caused by antibodies against neuronal synaptic receptors, surface proteins, or intracellular proteins. In this case report, we present a 65-year-old female who presented with a fall and altered mental status. Workup for infectious etiologies was negative and MRI of the brain displayed focal restricted diffusion with corresponding T2-FLAIR hyperintensity involving gray matter structures, making the diagnosis unclear. CT perfusion of the brain demonstrated increased cerebral blood volume and cerebral blood flow in the left parietooccipital gray matter, with corresponding normal mean transit time. Following treatment failure with acyclovir, antibiotics, and steroids, the patient was found to be positive for GAD65 antibodies and diagnosed with autoimmune encephalitis. Symptoms markedly improved with plasmapheresis. Autoimmune encephalitis rarely causes restricted diffusion and this is the first case report to describe corresponding hyperperfusion on CT perfusion study.



2008 ◽  
Vol 63 (suppl_4) ◽  
pp. ONSE315-ONSE315
Author(s):  
Ricardo J. Komotar ◽  
Brad E. Zacharia ◽  
J Mocco ◽  
Michael G. Kaiser ◽  
Stephen J. Frucht ◽  
...  

Abstract Objective: In this case report, we present a patient with normal pressure hydrocephalus in whom a lumbar drainage trial yielded a false-negative result secondary to cervical spondylosis. Clinical Presentation: An 80-year-old woman presented with classic symptoms of normal pressure hydrocephalus as well as evidence of cervical myelopathy. Magnetic resonance imaging of the brain and spine showed enlarged ventricles and single-level cervical canal narrowing. Intervention: An initial lumbar drainage trial was performed, which revealed negative results. The patient then underwent cervical decompression and fusion. Despite this procedure, the patient’s symptoms continued to worsen. A repeat lumbar drainage trial was performed with positive results. Subsequently, a ventriculoperitoneal shunt was placed, resulting in significant improvement of her symptoms. Conclusion: This case report illustrates how altered cerebrospinal fluid flow dynamics may impact the accuracy of the lumbar spinal drainage trial in patients with normal pressure hydrocephalus.



2021 ◽  
Author(s):  
Felipe Fanine de Souza ◽  
Ana Luiza da Silva Wendhausen ◽  
Felipe Reinert Avilla Machado ◽  
Gustavo Figueiredo da Silva ◽  
Maria Eduarda Angelo de Mendonça Fileti ◽  
...  

Context: Non-ketotic hyperglycemia is a rare cause of chorea. Clinical findings are usually unilateral and potentially reversible after treatment for hyperglycemia. Hyperglycemia leads to asymmetric multifocal petechial hemorrhages of the basal ganglia, leading to a dysfunction of neuronal networks that connect the basal ganglia and the motor cortical areas, mainly affecting the subthalamic nucleus and contralateral striatum, which is highlighted by typical hyperdense lesions of the basal ganglia in computed tomography (CT) of the brain. This study aimed to report a case of a patient with choreiform movements due to a rare etiology of hyperglycemia nonketotic in a Hospital Public of Joinville, SC. The study was carried out through the collection and analysis of a patient’s medical record. Case report: Female patient, 54 years old, who presented for 6 days choreiform movements in the face, left upper limb and, discreetly, in the left lower limb. Snake tongue sign and milkmaid’s grip positive, without dysarthria. In the laboratory exam, glucose of 600 mg / dL; without further changes. Cranial tomography showed hyperdensity in the putamen region on the right. The treatment was started to obtain better glycemic control and Risperidone 3 mg / day. Conclusions: It is concluded, then, that non-ketotic hyperglycemia is an uncommon, but reversible cause of chorea, and may manifest itself due to an uncontrolled non-ketotic diabetes mellitus. Its pathogenic mechanism remains to be clarified. In addition, clinical, epidemiological, imaging and laboratory findings, together, corroborate for early diagnosis and proper management.



Author(s):  
Mitchell W. Couldwell ◽  
Samuel Cheshier ◽  
Philipp Taussky ◽  
Vance Mortimer ◽  
William T. Couldwell

Moyamoya is an uncommon disease that presents with stenoocclusion of the major vasculature at the base of the brain and associated collateral vessel formation. Many pediatric patients with moyamoya present with transient ischemic attacks or complete occlusions. The authors report the case of a 9-year-old girl who presented with posterior fossa hemorrhage and was treated with an emergency suboccipital craniotomy for evacuation. After emergency surgery, an angiogram was performed, and the patient was diagnosed with moyamoya disease. Six months later, the patient was treated for moyamoya using direct and indirect revascularization; after surgery there was excellent vascularization in both regions of the bypass and no further progression of moyamoya changes. This case illustrates a rare example of intracerebral hemorrhage associated with moyamoya changes in the posterior vascularization in a pediatric patient and subsequent use of direct and indirect revascularization to reduce the risk of future hemorrhage and moyamoya progression.



2004 ◽  
pp. 406-412
Author(s):  
Paul Okunieff ◽  
Michael C. Schell ◽  
Russell Ruo ◽  
E. Ronald Hale ◽  
Walter G. O'Dell ◽  
...  

✓ The role of radiosurgery in the treatment of patients with advanced-stage metastatic disease is currently under debate. Previous randomized studies have not consistently supported the use of radiosurgery to treat patients with numbers of brain metastases. In negative-results studies, however, intracranial tumor control was high but extracranial disease progressed; thus, patient survival was not greatly affected, although neurocognitive function was generally maintained until death. Because the future promises improved systemic (extracranial) therapy, the successful control of brain disease is that much more crucial. Thus, for selected patients with multiple metastases to the brain who remain in good neurological condition, aggressive lesion-targeting radiosurgery should be very useful. Although a major limitation to success of this therapy is the lack of control of extracranial disease in most patients, it is clear that well-designed, aggressive treatment substantially decreases the progression of brain metastases and also improves neurocognitive survival. The authors present the management and a methodology for rational treatment of a patient with breast cancer who has harbored 24 brain metastases during a 3-year period.



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