scholarly journals Case Report: Anti-LGI1 Limbic Encephalitis Associated With Anti-thyroid Autoantibodies

2021 ◽  
Vol 11 ◽  
Author(s):  
Ricardo Otiniano-Sifuentes ◽  
Anali Cuba Antezana ◽  
Walter F. De La Cruz Ramirez ◽  
Kevin Pacheco-Barrios ◽  
Darwin A. Segura Chavez

Anti-LGI1 encephalitis is an autoimmune encephalitis with antibodies against leucine-rich glioma-inactivated 1 (LGI1), first described in 2010. It is a non-frequent and poorly understood entity that represents the second most frequent cause of autoimmune encephalitis. This entity is characterized by the presence of limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Herein, we present the case of a male patient with an onset of epileptic seizures (generalized tonic-clonic seizure), and involuntary dystonic movements that affect the right side of his face and right upper limb associated with mental disorder, and affectation of higher functions. The electroencephalogram showed continuous generalized slowing of the background activity. The brain magnetic resonance imaging showed signal hyperintensity at the level of both mesial temporal lobes and hippocampi and in the head of the right caudate nucleus. Anti-thyroglobulin antibodies were positive, and he was initially diagnosed as Hashimoto's encephalopathy (HE). However, the response to corticosteroids was not completed as it is usually observed in HE. For that, antibodies for autoimmune encephalitis were tested, and the anti-LGI1 antibodies were positive in serum and cerebrospinal fluid. HE is an important differential diagnosis to consider. Furthermore, the presence of Anti-thyroglobulin antibodies should not be taken as the definitive diagnostic criteria, since these antibodies could be associated with other autoimmune encephalopathies, which include in addition to anti-LGI1, anti-NMDA and anti-Caspr2.

2008 ◽  
Vol 109 (4) ◽  
pp. 742-745 ◽  
Author(s):  
Tracy Weimer ◽  
Warren Boling ◽  
David Pryputniewicz ◽  
Adriana Palade

The authors report a case of status epilepticus secondary to limbic encephalitis that was successfully treated with temporal lobectomy. A 45-year-old woman presented in status epilepticus refractory to high-dose suppressive medical therapy. Magnetic resonance imaging of the brain showed T2- and FLAIR-weighted hyperintensities in the right temporal lobe, left and right frontal lobes, and pons. A lumbar puncture revealed normal findings. Continuous electroencephalography monitoring showed continued right temporal seizure activity. A paraneoplastic panel was positive for N-type voltage-gated calcium channels. Subsequent bronchial biopsy revealed small cell carcinoma of the lung. A right temporal lobectomy was performed due to refractory status, resulting in resolution of seizure activity and recovery of good neurological function. The authors describe their case and review the literature on surgical therapy for refractory status epilepticus and limbic encephalitis.


2020 ◽  
Vol 33 (4) ◽  
pp. e100196
Author(s):  
Homayun Shahpesandy ◽  
Rosemary Mohammed-Ali ◽  
Ahmed Oladosu ◽  
Tarik Al-Kubaisy ◽  
Moses Anene ◽  
...  

Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psychosis, short-term memory impairment as well as faciobrachial and grand mal seizures. We aim to present a case of a 53-year-old man with positive anti–voltage-gated potassium channel (VGKC) complex antibodies who initially presented with symptoms of psychotic mania. Six weeks post-psychiatric symptomatology, he presented with neurological symptoms such as faciobrachial jerking and tonic-clonic seizure. The patient had no previous psychiatric history and was initially treated with psychotropic medications. Our experience emphasises the fact that limbic encephalitis is not easy to identify as most patients initially present with psychiatric symptomatology than neurological symptoms. Furthermore, immunological and laboratory testing takes a rather long time to determine the diagnosis. What is more, few psychiatrists consider autoimmune nature of the neuropsychiatric presentation. Therefore, it is important to consider autoimmune encephalitis in patients with new-onset psychosis or mania who also present with neurological symptoms at some stage of their illness. Characteristic indicators of autoimmune encephalitis include neurological symptoms such as facial twitching, seizures, confusion and cognitive decline.


2017 ◽  
Vol 16 (04) ◽  
pp. 239-242
Author(s):  
Gunnar Buyse ◽  
Lieven Lagae ◽  
Philippe Demaerel ◽  
Frank Kesteloot ◽  
Ingele Casteels ◽  
...  

AbstractWyburn–Mason syndrome is a rare condition that is characterized by ipsilateral arteriovenous malformations affecting the eye, brain, and facial skin. A conjunctival vascular dilation can be a rare ocular presenting sign. We report a 6-year-old boy who attended the hospital because of the sudden appearance of a conjunctival vascular lesion in his right eye. Inspection of his facial skin showed a subtle discoloration along the right trigeminal nerve and a vascular structure of the conjunctiva. Fundoscopy showed dilated and tortuous retinal vessels. Brain magnetic resonance imaging (MRI) revealed a large arteriovenous malformation involving the thalamus and perimesencephalic area. Ophthalmologic and neuroradiologic findings were consistent with the diagnosis of Wyburn–Mason syndrome. The sudden emergence of a vascular malformation in the conjunctiva should alert the clinician to perform an ophthalmoscopy, and in our patient, this finding was the clue to diagnosis of Wyburn–Mason syndrome. Because of the association between retinal and intracranial arteriovenous malformations, an MRI of the brain is strongly recommended in all patients with ocular arteriovenous malformations.


Author(s):  
N. V. Yurovskikh ◽  
G. V. Jarygina

Introduction. In recent years, the use of oral anticoagulants for the prevention of ischemic strokes (IS) in patients with atrial fibrillation (AF), as well as the prevention of deep vein thrombosis and thromboembolism has been increasing in clinical practice. According to epidemiological studies, ischemic strokes account for about 85% of all AMI. Cardioembolic strokes (CES), which develop due to embolism to the cerebral vessels from the heart chambers, deserve special attention. The presence of AF is associated with a twofold increase in the risk of death from cardiovascular disease and death from any cause. Timely anticoagulant therapy can reduce the risk of thromboembolic complications and improve the prognosis. Materials and Methods. The authors present a clinical observation in which a 47-year-old female patient diagnosed with venous insufficiency, who took dabigatran 150 mg twice a day for a year, developed IS with left-sided pronounced hemiparesis. After 170 minutes from the development of the stroke, idarucizumab was administered to neutralize the anticoagulant effect of dabigatran and thrombolytic therapy (TLT) was performed. Results. Twenty-four hours after TLT, the National Institutes of Health Stroke Scale (NIHSS) score decreased from 16 to 6. A repeat CT scan of the brain revealed a focus of ischemia in the right frontal, parietal, and temporal lobes. On the 19th day of the AI, dabigatran 150 mg 2 times a day was resumed. Conclusion. The presented observation confirms that in patients with AI developed against the background of dabigatran, the administration of idarucizumab is safe and allows for TLT.


2020 ◽  
Vol 19 (8) ◽  
pp. 584-598
Author(s):  
Mette Scheller Nissen ◽  
Matias Ryding ◽  
Morten Meyer ◽  
Morten Blaabjerg

Autoimmune Encephalitides (AE) comprises a group of diseases with antibodies against neuronal synaptic and cell surface antigens. Since the discovery of the most common subtype, NMethyl- D-Aspartate (NMDA) receptor encephalitis, an astonishing number of novel disease-causing antibodies have been described. This includes other glutamatergic and GABAergic receptor antibodies and antibodies against various other surface proteins. Many of these novel conditions present as limbic encephalitis with memory impairment, psychiatric features and epileptic seizures, often alongside subtype specific clinical features. Others present with a clinical disease course specific to the antibody. In contrast to the well-known paraneoplastic syndromes with antibodies directed against intracellular antigens (e.g. limbic encephalitis with Hu antibodies), autoimmune encephalitides are often highly responsive to immunotherapy, with a good outcome if diagnosed and treated early. Prognosis depends on aggressive immunotherapy, often with a combination of corticosteroids, intravenous immunoglobulin, plasma exchange or in some cases anti-CD20 therapy and cyclophosphamide. Other treatment regimens exist, and prognosis varies between disease subtypes and occurrence of underlying cancer. We review current knowledge on subtype-specific clinical presentation, disease mechanisms, diagnosis including pitfalls, treatment paradigms and outcome in autoimmune encephalitides, and provide suggestions for future research.


Encyclopedia ◽  
2021 ◽  
Vol 2 (1) ◽  
pp. 26-35
Author(s):  
Natalia A. Shnayder ◽  
Timur K. Sirbiladze ◽  
Irina V. Demko ◽  
Marina M. Petrova ◽  
Regina F. Nasyrova

Limbic encephalitis (LE) is an inflammatory disease of the brain, in which lesion is anatomically limited in structures of the limbic system. In some cases, LE can start with symptoms of limbic dysfunction with further involvement of other regions of the brain. Classic LE syndrome includes such symptoms as the development of personality disorders, depression, sleep disorders, epileptic seizures, hallucinations and cognitive disorders (short-term and long-term memory impairment). The information of clinical examination, electroencephalogram (EEG), magnetic resonance imaging (MRI) and cerebrospinal fluid studies (CSF) suggest the diagnosis of LE in most patients with Coronavirus Disease 2019 (COVID-19).


2021 ◽  
Vol 12 ◽  
Author(s):  
Yuanyuan Fang ◽  
Dengji Pan ◽  
Hao Huang

Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor encephalitis is a relatively rare anti-neuronal surface antigen autoimmune encephalitis (LE). We described a case of a 47-year-old Chinese man having anti-AMPA receptor limbic encephalitis initially presented with cognitive decline, undetectable antibodies, and normal imaging findings in magnetic resonance image (MRI) and then developed into typical autoimmune limbic encephalitis a few months later with a course of multiple relapses. In addition, we found progressive brain atrophy in our case, which was a rare presentation of LE. This report also summarized the characteristics of nine reported cases of anti-AMPA receptor limbic encephalitis with relapse up to date. This case highlighted that autoimmune limbic encephalitis is an important differential diagnosis for patients with typical symptoms even when the MRI and antibodies are normal, and more attention should be paid to the relapse of anti-AMPA receptor encephalitis.


QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
R M H Zaitoun ◽  
O A Abdelsalam ◽  
H H Aly

Abstract A 4-year-old boy presented to the emergency room with severe abdominal pain, tachypnea and vomiting followed by progressive obtundation. He was found to have hyperglycemia, ketonuria and metabolic acidosis. A diagnosis of Diabetic ketoacidosis was hence made and the boy was managed as such, with improvement in his laboratory parameters as well as in his level of consciousness. On day 6 after admission, the boy developed a recurrence of altered consciousness and repeated seizures. On examination the boy had spastic quadriparesis lateralizing to the right side. MRI of the brain showed multiple inflammatory lesions involving both temporal lobes. CSF analysis was unremarkable and CSF Herpes Simplex virus PCR was negative. Based on the inflammatory nature of lesions on MRI, with negative PCR for HSV and otherwise unremarkable CSF, together with progressive worsening of level of consciousness and spasticity, the possibility of autoimmune encephalitis was raised and the boy received 2 grams per kilogram of intravenous immunoglobulin with mild improvement in level of consciousness. The boy then received intravenous pulsed methylprednisolone, with a clear improvement in both his motor and mental capabilities. Three months post hospital discharge, the child was back to normal. This case highlights the importance of considering autoimmune encephalitis in the differential diagnosis of altered level of consciousness in a child with type 1 diabetes, especially in the presence of suggestive MRI findings and a lack of an alternative explanation.


2021 ◽  
pp. 1-11
Author(s):  
Akash Sharma ◽  
Neeraj Kumar ◽  
Ayush Kumar ◽  
Karan Dikshit ◽  
Kusum Tharani ◽  
...  

In modern day Psychiatric analysis, Epileptic Seizures are considered as one of the most dreadful disorders of the human brain that drastically affects the neurological activity of the brain for a short duration of time. Thus, seizure detection before its actual occurrence is quintessential to ensure that the right kind of preventive treatment is given to the patient. The predictive analysis is carried out in the preictal state of the Epileptic Seizure that corresponds to the state that commences a couple of minutes before the onset of the seizure. In this paper, the average value of prediction time is restricted to 23.4 minutes for a total of 23 subjects. This paper intends to compare the accuracy of three different predictive models, namely – Logistic Regression, Decision Trees and XGBoost Classifier based on the study of Electroencephalogram (EEG) signals and determine which model has the highest rate of detection of Epileptic Seizure.


2019 ◽  
Vol 10 (01) ◽  
pp. 154-157
Author(s):  
M. S. Umashankar ◽  
A. Bharath Kumar

ABSTRACTAbsence seizures are often associated with impaired or loss of consciousness clinically proved to have an impact on motor and cognitive abnormalities of the nerve cells of the brain. Seizure admits several etiopathophysiological events leading to several neurofunctional changes in the reticulothalamocortical circuitry zones of the central nervous system. This paves the episodes of absence seizure events. A clinical case report of absence seizure in a 25 years age adult patient came to the hospital with impaired consciousness. The brain magnetic resonance imaging scanning of the patient detected a small focal flair hypertensive area in the right parasellar region close to cavernous sinus with mild flair hypersensitivity in the left cavernous sinuses, right maxillary, and ethmoid sinusitis. The electroencephalogram of the brain showed normal waves with electrode artifacts was observed. The patient was confirmed with absence seizures, and he was treated with oxcarbazepine 150 mg twice daily. The patient was recovered from seizure and discharged with medications. He was called for follow-up examination once in 3-month period.


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