scholarly journals Rasmussen's Encephalitis. A Case Report

2018 ◽  
Vol 3 (2) ◽  
pp. 849-854
Author(s):  
Tania Licona ◽  
Alejandra Mazariegos-Rivera ◽  
Morgan Medina
Keyword(s):  
Secondary Trauma ◽  
Intractable Epilepsy ◽  
Personal History ◽  
Neurological Dysfunction ◽  
Brain Disorder ◽  
Rasmussen’S Encephalitis ◽  
Intractable Seizures ◽  
Immune Mediated ◽  
History Of ◽  
Rasmussen’S Syndrome

Rasmussen's encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction with intellectual decline, and intractable seizures. It is a well-established cause of pharmacologically intractable epilepsy. The report is on a 17-month-old infant, treated at the Mario Catarino Rivas Hospital Honduras. Family history: grandfather epileptic secondary trauma from 20 years. Personal history: two previous emergency visits (at 16 months and 16 months 8 days) for convulsions for which she was admitted three days and was treated with valproic acid 30 mg/kg per day. The infant is admitted in the emergency, with a history of about three hours after onset of tonic convulsions, focused on left-side with drooling, oculogiros and relaxation of sphincters and fever of 38.5 ° C. Entered as convulsive syndrome in the study, however, as the days passed the number of seizures increased to 60 per day and was gradually presenting alterations in neurodevelopment. MRI reported leukoencephalopathy of undetermined origin and biopsy reported findings consistent with Rasmussen's syndrome. She was treated with immunoglobulin every two weeks for six doses after two months of hospitalization with achieved improvement. Currently, episodes of seizures have decreased significantly and almost not convulsing, she presented alterations in neurodevelopment.

Schizophrenia with Good and Poor Outcome

1985 ◽  
Vol 146 (4) ◽  
pp. 348-357 ◽  
Author(s):  
T. Kolakowska ◽  
A. O. Williams ◽  
K. Jambor ◽  
M. Ardern
Keyword(s):  
Cognitive Impairment ◽  
Psychiatric Symptoms ◽  
Stable Condition ◽  
Neurological Dysfunction ◽  
Brain Disorder ◽  
Developmental Abnormalities ◽  
Poor Outcome ◽  
History Of ◽  
Organic Brain ◽  
Treatment Deficits

SummaryFifty-six patients with RDC schizophrenia (42) or schizoaffective disorder (14), of two to 20 years' duration, were assessed for neurological ‘soft’ signs and cognitive impairment when in a stable condition—the ‘outcome’. Neurological dysfunction (46% of 50 examined patients) was associated with a history of developmental abnormalities, but was unrelated to outcome, psychiatric symptoms, or treatment. Deficits in particular cognitive fields were related to two independent factors: overall severity of residual psychiatric disorder (outcome) and neurological dysfunction. There was no relationship between the size of the lateral brain ventricles on CT scan and either ‘soft’ signs or cognitive impairment.The findings do not provide evidence for an association between the presence of organic brain disorder (as indicated by the joint occurrence of neurological dysfunction and cognitive impairment) and either poor outcome or particular symptoms of schizophrenia.

scholarly journals Dual Pathology in Rasmussen’s Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Richard A. Prayson
Keyword(s):  
Chronic Inflammation ◽  
Focal Cortical Dysplasia ◽  
Intractable Epilepsy ◽  
Cortical Dysplasia ◽  
Cortical Atrophy ◽  
Review Of The Literature ◽  
Dual Pathology ◽  
Rasmussen’S Encephalitis ◽  
Intractable Seizures

Rasmussen’s encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology). This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diagnosis of coexistent Rasmussen’s encephalitis, marked by chronic inflammation, microglial nodules, and focal cortical atrophy, combined with focal cortical dysplasia (Palmini et al. type IIA, ILAE type IIA). The literature on dual pathology in the setting of Rasmussen’s encephalitis is reviewed.

BIiochemical Markers in Pregnancy Associated with Sj�gren�s Syndrome and Thrombophilia

2018 ◽  
Vol 69 (8) ◽  
pp. 2300-2303
Author(s):  
Anca Daniela Braila ◽  
Adrian Neacsu ◽  
Anca Emanuela Musetescu ◽  
Elena Luminita Vircan ◽  
Alesandra Florescu ◽  
...  
Keyword(s):  
Multidisciplinary Approach ◽  
Personal History ◽  
Obstetrics And Gynecology ◽  
Maternal History ◽  
Immune Mediated ◽  
Multisystemic Disease ◽  
History Of ◽  
One Year ◽  
Neonatal Monitoring ◽  
In Pregnancy

Sj�gren�s syndrome (SS) is a multisystemic disease mainly characterized by the hypofunction of the lachrymal and salivary glands and can be either primary or secondary, when related to other autoimmune pathologies. We present the case of a 35-year-old female admitted in the Department of Obstetrics and Gynecology for pregnancy monitoring. The patient had a personal history of a spontaneous abortion one year prior to admission at 5 months of gestation and a maternal history of SS. A multidisciplinary approach with solid obstetrical, rheumatological and neonatal monitoring is essential for best outcomes of the mother and fetus. An early detection of maternal and fetal immune-mediated threats and judicious use of medication is essential in women with autoimmune diseases who plan conceiving.

scholarly journals SREAT presenting as decades of intractable seizures and isolated delusional episodes with clinical, laboratory, and EEG confirmation of treatment response

2019 ◽  
Vol 7 ◽  
pp. 2050313X1985005
Author(s):  
Elysia Tjong ◽  
Rachael Gardner ◽  
Yen-Yi Peng
Keyword(s):  
Epilepsy Surgery ◽  
Autoimmune Thyroiditis ◽  
Clinical Laboratory ◽  
Intractable Epilepsy ◽  
Temporal Lobectomy ◽  
Intractable Seizures ◽  
Autoimmune Epilepsy ◽  
History Of ◽  
Quantitative Electroencephalogram

We report a case of a 60-year-old woman with a history of intractable seizures and isolated delusional psychosis who was later diagnosed with steroid-responsive encephalopathy associated with autoimmune thyroiditis. The patient underwent right temporal lobectomy (epilepsy surgery) 15 years before coming to this clinic, but continued to have focal seizures, resulting in frequent emergency room visits thereafter. After admission for intensive inpatient video electroencephalogram monitoring and subsequent 7 months of close follow-up, both the electroencephalogram abnormalities and isolated delusional psychosis were found to be responsive to immunotherapy. This suggests that her epilepsy may be autoimmune in nature. Steroid-responsive encephalopathy associated with autoimmune thyroiditis was diagnosed after 26 years since the onset of seizures. Performing invasive epilepsy surgery in patients with autoimmune epilepsy cannot reverse the inflammatory process; therefore, it is reasonable to test for autoimmune etiologies before excision surgery on patients with medically intractable epilepsy. This case demonstrates the clinical use of quantitative electroencephalogram in assisting with the diagnosis of steroid-responsive encephalopathy associated with autoimmune thyroiditis and supports that it is a spectrum disorder with protean manifestations.

scholarly journals Epilepsy in children with subacute sclerosing panencephalitis

2013 ◽  
Vol 141 (7-8) ◽  
pp. 434-440 ◽  
Author(s):  
Nebojsa Jovic
Keyword(s):  
Measles Virus ◽  
Clinical Course ◽  
Subacute Sclerosing Panencephalitis ◽  
Intractable Epilepsy ◽  
Complex Partial Seizures ◽  
Disease Evolution ◽  
Intractable Seizures ◽  
Initial Symptoms ◽  
History Of ◽  
History Of Epilepsy

Introduction. Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. Objective. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Methods. Retrospective study was carried out on 19 children (14 boys, 5 girls) with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years). Results. SSPE onset ranged from 4.5 to 16.5 years (mean 10.05). Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6%) cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Conclusion. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

Pharmacologically intractable epilepsy in children: diagnosis and preoperative evaluation

2008 ◽  
Vol 25 (3) ◽  
pp. E2 ◽  
Author(s):  
Cristina Go ◽  
O. Carter Snead
Keyword(s):  
Risk Factors ◽  
Surgical Treatment ◽  
Preoperative Evaluation ◽  
Intractable Epilepsy ◽  
Evaluation Process ◽  
Intractable Seizures ◽  
History Of ◽  
History Of Epilepsy ◽  
Definition Of ◽  
Causes And Risk Factors

It is important to correctly diagnose medically intractable epilepsy in children and to identify those children whose medically refractory, localization-related seizures may be surgically remediable as soon as possible to optimize the surgical outcome. In this paper the authors review the definition of medically intractable seizures and discuss the various causes and risk factors for this disorder in children. They also outline the presurgical diagnostic evaluation process for pharmacologically intractable epilepsy in children who may be candidates for surgical treatment of localization-related seizures. The treatment of children with medically intractable epilepsy is both challenging and rewarding. Surgery has the potential of altering the natural history of epilepsy by improving or eliminating seizures in carefully selected patients.

Out of the Whirlwind Reconsidered

2020 ◽  
Vol 53 (2) ◽  
pp. 1-12
Author(s):  
Michael Berkowitz
Keyword(s):  
United States ◽  
The United States ◽  
Personal History ◽  
Historical Writing ◽  
Holocaust Literature ◽  
The Holocaust ◽  
History Of ◽  
Mutual Accountability ◽  
Union College

This article argues that Albert Friedlander’s edited book, Out of the Whirlwind (1968), should be recognised as pathbreaking. Among the first to articulate the idea of ‘Holocaust literature’, it established a body of texts and contextualised these as a way to integrate literature – as well as historical writing, music, art and poetry – as critical to an understanding of the Holocaust. This article also situates Out of the Whirlwind through the personal history of Friedlander and his wife Evelyn, who was a co-creator of the book, his colleagues from Hebrew Union College, and the illustrator, Jacob Landau. It explores the work’s connection to the expansive, humanistic development of progressive Judaism in the United States, Britain and continental Europe. It also underscores Friedlander’s study of Leo Baeck as a means to understand the importance of mutual accountability, not only between Jews, but in Jews’ engagement with the wider world.

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