scholarly journals Leucine-rich glioma-inactivated protein 1 antibody-mediated autoimmune encephalitis in a 4-year-old girl: a case report

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Junxia Luo ◽  
Jianguo Shi ◽  
Yehong Chen ◽  
Wandong Hu ◽  
Yujie Guo ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Case Report ◽  
Antiepileptic Drugs ◽  
Brain Magnetic Resonance Imaging ◽  
Autoimmune Encephalitis ◽  
Epileptic Seizures ◽  
Case Presentation ◽  
Csf Levels ◽  
Clonic Seizures ◽  
Magnetic Resonance Imaging Mri

Abstract Background Leucine-rich glioma-inactivated protein 1 (LGI1) antibody-mediated encephalitis is a rare subtype of autoimmune encephalopathy, which is associated with autoimmunity against the neuronal plasma membrane proteins. The characteristic symptoms of this disease are memory dysfunction, seizures, faciobrachial dystonic seizures, cognitive deficits, neuropsychiatric disturbances, and intractable hyponatremia. The diagnosis of this disease mainly depends on the presence of anti-LGI1 antibody in serum or cerebrospinal fluid of patients. LGI1 antibody encephalitis has been reported mostly in adults, with rare occurrences in children. Case presentation In this report, we described a 4-year-old girl with typical seizures. Seizure types included focal seizures and generalized tonic-clonic seizures. The electroencephalogram findings showed focal discharges. Brain magnetic resonance imaging (MRI) showed normal. The cerebrospinal fluid (CSF) levels of cells, glucose, and chloride were within the normal range, and the culture did not reveal growth of any pathogen. Test of serum LGI1-Ab was positive, while the tests for autoimmune encephalitis antibody series in CSF were negative. The seizures of the patient were completely controlled after the therapy of immunoglobulin, methylprednisolone and antiepileptic drugs (AEDs), and the mental state almost returned to normal. Conclusion To our knowledge, the patient described here may be the youngest case of LGI1 antibody encephalitis reported to date. Children with the LGI1 antibody-associated encephalitis may present only with single symptoms such as epileptic seizures and have good response to the therapy of immunoglobulin, methylprednisolone and antiepileptic drugs. Our case report will provide hints for pediatricians in the diagnosis and treatment of LGI1-antibody encephalitis.

scholarly journals Lymphangiography and focal pleurodesis treatment of chylothorax with an aberrant thoracic duct following oesophagectomy: a case report

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Tomoyuki Ishida ◽  
Jun Kanamori ◽  
Hiroyuki Daiko
Keyword(s):  
Magnetic Resonance Imaging ◽  
Interventional Radiology ◽  
Case Report ◽  
Magnetic Resonance ◽  
Thoracic Duct ◽  
Rare Case ◽  
Resonance Imaging ◽  
Case Presentation ◽  
Thoracostomy Tube ◽  
Magnetic Resonance Imaging Mri

Abstract Background Management of postoperative chylothorax usually consists of nutritional regimens, pharmacological therapies such as octreotide, and surgical therapies such as ligation of thoracic duct, but a clear consensus is yet to be reached. Further, the variation of the thoracic duct makes chylothorax difficult to treat. This report describes a rare case of chylothorax with an aberrant thoracic duct that was successfully treated using focal pleurodesis through interventional radiology (IVR). Case presentation The patient was a 52-year-old man with chylothorax after a thoracoscopic oesophagectomy for oesophageal cancer. With conventional therapy, such as thoracostomy tube, octreotide or fibrogammin, a decrease in the amount of chyle was not achieved. Therefore, we performed lymphangiography and pleurodesis through IVR. The patient appeared to have an aberrant thoracic duct, as revealed by magnetic resonance imaging (MRI); however, after focal pleurodesis, the leak of chyle was diminished, and the patient was discharged 66 days after admission. Conclusions Chylothorax remains a difficult complication. Focal pleurodesis through IVR can be one of the options to treat chylothorax.

scholarly journals Case Presentation: Unusual Association Between Possible Bilateral Intraventricular Xanthogranulomas, Postero-inferior Cerebellar Artery Aneurysm and Thrombophilia

2018 ◽  
Vol 64 (4) ◽  
pp. 173-176
Author(s):  
Sebastian Razvan Andone ◽  
Andreea Romaniuc ◽  
Zoltan Bajko ◽  
Smaranda Maier ◽  
Laura Barcutean ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Metabolic Diseases ◽  
Artery Aneurysm ◽  
Epileptic Seizures ◽  
Mthfr C677t ◽  
Lower Limbs ◽  
Case Presentation ◽  
High Cholesterol Level ◽  
Cerebellar Artery ◽  
Magnetic Resonance Imaging Mri

AbstractIntroduction: Xanthogranulomas are rare, benign, usually asymptomatic, cutaneous tumors most frequently seen in children (juvenile xanthogranulomas). Some lesions can be found accidentally at randomly performed cerebral computer tomography (CT) or magnetic resonance imaging (MRI) or even on autopsy.Case report: We present the case of a 44 year-old woman, known with a thrombophilic disorder (PAI-1 gene mutation, MTHFR C677T and A1298C) on chronic anticoagulant treatment. The onset of symptoms was in 2010, when she presented paresthesia and lower limbs weakness. Two years later the patient presents with severe intermittent headache and left hemicrania and a cerebral angio-MRI is performed showing a left postero-inferior cerebellar artery aneurysm and two choroid plexus intraventricular masses in the lateral ventricles. The patient developed a new symptom, dysarthria in 2014 and in 2015 has multiple episodes of loss of consciousness, interpreted as epileptic seizures. Routine blood tests were within normal range, except for a high cholesterol level. The patient was tested for autoimmune, infectious, endocrine and metabolic diseases that were negative. Surgical treatment and biopsy from the lesion was proposed, however the patient refused both procedures.Conclusions: There is an association between xanthogranulomas localization and the choroid plexus, the most frequent CNS origin being in the trigon of the lateral ventricle. Our case does not resemble with any other case published, mostly because the unusual presentation, symptomatology and the association between xanthogranulomas, thrombophilia and postero-inferior cerebellar artery aneurysm which were never reported before in other cases of xanthogranulomas from the literature.

Unusual presentation of acute encephalopathy with biphasic seizures and late reduced diffusion in Miller–Dieker syndrome

2019 ◽  
Author(s):  
Satoru Kobayashi ◽  
Mai Kamishima ◽  
Akari Tago ◽  
Kyoko Yokoi ◽  
Satoshi Suzuki
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Subcortical White Matter ◽  
Acute Encephalopathy ◽  
Case Presentation ◽  
Delta Waves ◽  
Diffusion Weighted Images ◽  
Disturbance Of Consciousness ◽  
Intravenous Diazepam ◽  
Severe Motor ◽  
Magnetic Resonance Imaging Mri

Abstract Background: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a unique subtype of acute encephalopathy that occurs in children. To date, there is no description of a patient with concurrent Miller–Dieker syndrome (MDS) and AESD. Case presentation: We present a case of 2-year-old girl with MDS, who was admitted for status epilepticus with a high fever, was diagnosed with AESD. She exhibited seizure for more than 1 h, which disappeared after administration of intravenous diazepam and phenobarbital. Brain magnetic resonance imaging (MRI), performed on the third day post-admission because she had not regained consciousness after the seizure ceased, showed abnormally high intensities in subcortical white matter, predominantly in the frontal areas on diffusion-weighted images (DWI). Acute encephalitis/encephalopathy was diagnosed based on electroencephalography (EEG) findings of diffuse high-voltage delta waves without seizure activity. Six days post-admission, frequent apneic episodes were observed, with oxygen desaturation due to clustered seizures. Although seizures disappeared with continuous intravenous midazolam, subclinical seizures were present in amplitude-integrated EEG (aEEG); these were suppressed by increasing the midazolam dose. Conclusion: This is the first report of AESD in a patient with MDS. Ther disturbance of consciousness was difficult to recognize because of severe motor and intellectual disabilities due to MDS. EEG aids the evaluation of consciousness in the acute phase, and aEEG can be helpful for monitoring and controlling subclinical seizures in the biphasic phase of AESD, especially patients with underlying neurological disorders.

scholarly journals Reversible splenial lesion syndrome due to oxcarbazepine withdrawal: case report and literature review

2018 ◽  
Vol 46 (3) ◽  
pp. 1277-1281 ◽  
Author(s):  
Chaoyang Jing ◽  
Lichao Sun ◽  
Zhuo Wang ◽  
Chaojia Chu ◽  
Weihong Lin
Keyword(s):  
Case Report ◽  
Corpus Callosum ◽  
Epileptic Seizures ◽  
Resonance Imaging ◽  
Splenial Lesion ◽  
History Of ◽  
Reversible Lesion ◽  
Magnetic Resonance Imaging Mri ◽  
Reversible Splenial Lesion Syndrome

Background Reversible splenial lesion syndrome is a distinct entity radiologically characterized by a reversible lesion in the splenium of the corpus callosum. According to previous reports, this condition may be associated with antiepileptic drug use or withdrawal. We herein report a case of reversible splenial lesion syndrome associated with oxcarbazepine withdrawal. Case Report A 39-year-old man presented with an 8-year history of epileptic seizures. During the previous 3 years, he had taken oxcarbazepine irregularly. One week prior to admission, he withdrew the oxcarbazepine on his own, and the epilepsy became aggravated. Magnetic resonance imaging (MRI) revealed an isolated lesion in the splenium of the corpus callosum with slight hypointensity on T1-weighted imaging and slight hyperintensity on T2-weighted imaging. Regular oxcarbazepine was prescribed. Over a 5-month follow-up period, repeat MRI showed that the abnormal signals in the splenium of the corpus callosum had completely disappeared. Conclusion Reversible splenial lesion syndrome is a rare clinicoradiological disorder that can resolve spontaneously with a favorable outcome. Clinicians should be aware of this condition and that oxcarbazepine withdrawal is a possible etiological factor.

scholarly journals Hypertrophic olivary degeneration concomitant with bilateral middle cerebellar peduncles Wallerian degeneration following unilateral pontine infarction

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Bing Bao ◽  
Xiangbin Wu ◽  
Zhongbin Xia ◽  
Yaoyao Shen
Keyword(s):  
Wallerian Degeneration ◽  
Corticospinal Tract ◽  
Brain Magnetic Resonance Imaging ◽  
Acute Onset ◽  
Case Presentation ◽  
Hypertrophic Olivary Degeneration ◽  
Pontine Infarction ◽  
Cerebellar Peduncles ◽  
Magnetic Resonance Imaging Mri

Abstract Background Wallerian degeneration (WD) can occur in different projecting systems, such as corticospinal tract, dentate-rubro-olivary pathway, and corticopontocerebellar tract. However, the co-occurrence of hypertrophic olivary degeneration (HOD) and middle cerebellar peduncles (MCPs) degeneration secondary to unilateral pontine infarction in a single patient is extremely rare. Case presentation A 71-year-old man presented with acute onset of dizzness, slurred speech, and right-sided weakness. On the next day, his previous neurologic deficits deteriorated. Brain magnetic resonance imaging (MRI) revealed acute ischemic stroke of the left pons. After treatment with thrombolysis, antiplatelets, and rehabilitation training, his speaking and motor function improved moderately. At the 3-month follow-up, the MRI showed hyperintensity in the left medulla oblongata and bilateral MCPs on T2-weighted and FLAIR images, suggesting HOD as well as MCPs degeneration. Conclusions It is of great importance for us to know the anatomic knowledge of dentate-rubro-olivary and corticopontocerebellar pathways.

scholarly journals Pelizaeus-Merzbacher Disease: A Case Report

2020 ◽  
Author(s):  
Ghazaleh Jamalipour Soufi ◽  
Siavash Iravan
Keyword(s):  
Case Report ◽  
White Matter ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Proteolipid Protein ◽  
Radiology Department ◽  
Magnetic Resonance Imaging Mri ◽  
Normal White Matter ◽  
Pelizaeus Merzbacher Disease ◽  
The Brain

Pelizaeus-Merzbacher Disease (PMD), as a rare genetically x-linked leukodystrophy, is a disorder of proteolipid protein expression in myelin formation. This disorder is clinically presented by neurodevelopmental delay and abnormal pendular eye movements. The responsible gene for this disorder is the proteolipid protein gene (PLP1). Our case was a oneyear-old boy referred to the radiology department for evaluating the Central Nervous System (CNS) development by brain Magnetic Resonance Imaging (MRI). Clinically, he demonstrated neuro-developmental delay symptoms. The brain MRI results indicated a diffuse lack of normal white matter myelination. This case report should be considered about the possibilityof PMD in the brain MRI of patients who present a diffuse arrest of normal white matter myelination.

scholarly journals Dyke-Davidoff-Masson Syndrome: A case Report in a patient presenting with drug-resistant epilepsy and pseudoseizures

2021 ◽  
Author(s):  
Pedro Schmidt dos Reis Matos Figueiredo ◽  
Thiago Oliveira Chaves
Keyword(s):  
Case Report ◽  
Cognitive Dysfunction ◽  
Brain Magnetic Resonance Imaging ◽  
Rare Condition ◽  
Signs And Symptoms ◽  
Drug Resistant ◽  
Cerebral Hemiatrophy ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Drug Resistant Epilepsy

Context: Dyke-Davidoff-Masson (DDM) syndrome is a rare neurological condition, first described in 1933. Characteristics include cerebral hemiatrophy, contralateral hemiparesis, seizures, and cognitive dysfunction, combined into different degrees and patterns. Brain magnetic resonance imaging (MRI) is used to perform diagnosis throughout its specific findings. Case Report: A eighteen-year-old female presented to our service with a history of cognitive dysfunction and seizures since early childhood, which persistence even with adequate use of antiepileptic drugs. During Investigation were found signs and symptoms compatible with DDM syndrome, and evidence of pseudoseizures captured in a video electroencephalography monitoring. Conclusion: DDM syndrome is a rare condition that must be part of differential diagnosis in patients with seizures and cerebral hemiatrophy. Management is based on adequate control of seizures and other comorbidities.

scholarly journals Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination

2021 ◽  
Vol 12 ◽  
Author(s):  
Bing-Yan Ren ◽  
Yi Guo ◽  
Jing Han ◽  
Qian Wang ◽  
Zai-Wang Li
Keyword(s):  
Case Report ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Autoimmune Disorder ◽  
Pulse Therapy ◽  
Nmdar Encephalitis ◽  
Cns Demyelination ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

Clinical Pharmacology of Modern Antiepileptic Drugs

2017 ◽  
Vol 10 (6) ◽  
pp. 3875-3890
Author(s):  
D Samba Reddy
Keyword(s):  
Antiepileptic Drugs ◽  
Enzyme Induction ◽  
Epileptic Seizures ◽  
Drug Of Choice ◽  
Voltage Gated Sodium Channels ◽  
Intractable Seizures ◽  
Spread Of Excitation ◽  
Clonic Seizures ◽  
Excitability Of Neurons ◽  
Orally Active

This article describes current antiepileptic drugs (AEDs) that are available for treatment of epilepsy. Epilepsy is characterized by repeated occurrence of seizures.  Epileptic seizures are classified into focal and generalized types. Around two-dozen AEDs are available for treating epilepsy. AEDs act on diverse molecular targets to selectively modify the abnormal excitability of neurons by reducing the focal seizure discharges or preventing spread of excitation. AEDs suppress seizures by blocking the voltage-gated sodium channels (phenytoin, carbama-zepine, valproate, lamotrigine, oxcarbazepine, topiramate), voltage-activated calcium channels (ethosuximide, gabapentin), potentiation of GABA inhibition (barbiturates, benzodiazepines, tiagabine), and reduction of glutamate excitation (felbamate, parampanel). Carbamazepine, phenytoin, and valproate are the first-line agents for partial seizures and generalized tonic-clonic seizures. Ethosuximide is the drug of choice for absence seizures. AEDs are orally-active and show unique pharmacokinetic features. Some AEDs cause enzyme induction and hence produce drug-drug interactions. Newer AEDs such as gabapentin, levetiracetam, tiagabine, and pregabalin do not cause enzyme induction. Despite many advances in epilepsy research, nearly 30% of people with epilepsy have drug-resistant or intractable seizures. Presently, there is no cure for epilepsy. Thus, newer and better AEDs that can better prevent refractory seizures and modify the disease are needed for curing epilepsy.    

scholarly journals Transient Edematous Lesions of the Splenium in Epileptic Patients

2005 ◽  
Vol 32 (3) ◽  
pp. 352-355 ◽  
Author(s):  
Giovanna Carrara ◽  
Edoardo Ferlazzo ◽  
Donatella Tampieri ◽  
Frederick Andermann ◽  
Denis Melanson
Keyword(s):  
Antiepileptic Drugs ◽  
Case Reports ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
High Signal ◽  
Focal Lesions ◽  
Epileptic Patients ◽  
Magnetic Resonance Imaging Mri ◽  
Patients With Epilepsy

ABSTRACT:Background:Transient focal lesions in the splenium of the corpus callosum (SCC) have been previously described in patients with epilepsy or without epilepsy but receiving antiepileptic drugs (AED).Case reports:Two epileptic patients were admitted to our long-term monitoring unit. Antiepileptic drugs were completely discontinued a few days later. One patient had no seizures. The other had three attacks, the last of which occurred two days before a brain magnetic resonance imaging (MRI) was performed. In both cases brain MRI showed a lesion in the SCC characterized by high signal on T2-weighted images and no enhancement after Gadolinium infusion. The patients were discharged with their pre-admission medications. A follow-up MRI five weeks later showed resolution of the SCC lesions.Conclusions:The pathogenesis of transient SCC lesions in epileptic patients is still unclear. In our patients, either the sudden AED withdrawal or the seizures activity may be presumed to be the cause, though an individual susceptibility must also be considered.

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