Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy

2008 ◽  
Vol 25 (3) ◽  
pp. E8 ◽  
Author(s):  
Bassam Addas ◽  
Elisabeth M. S. Sherman ◽  
Walter J. Hader

Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.

Neurology ◽  
2020 ◽  
Vol 95 (9) ◽  
pp. e1244-e1256 ◽  
Author(s):  
Dileep R. Nair ◽  
Kenneth D. Laxer ◽  
Peter B. Weber ◽  
Anthony M. Murro ◽  
Yong D. Park ◽  
...  

ObjectiveTo prospectively evaluate safety and efficacy of brain-responsive neurostimulation in adults with medically intractable focal onset seizures (FOS) over 9 years.MethodsAdults treated with brain-responsive neurostimulation in 2-year feasibility or randomized controlled trials were enrolled in a long-term prospective open label trial (LTT) to assess safety, efficacy, and quality of life (QOL) over an additional 7 years. Safety was assessed as adverse events (AEs), efficacy as median percent change in seizure frequency and responder rate, and QOL with the Quality of Life in Epilepsy (QOLIE-89) inventory.ResultsOf 256 patients treated in the initial trials, 230 participated in the LTT. At 9 years, the median percent reduction in seizure frequency was 75% (p < 0.0001, Wilcoxon signed rank), responder rate was 73%, and 35% had a ≥90% reduction in seizure frequency. We found that 18.4% (47 of 256) experienced ≥1 year of seizure freedom, with 62% (29 of 47) seizure-free at the last follow-up and an average seizure-free period of 3.2 years (range 1.04–9.6 years). Overall QOL and epilepsy-targeted and cognitive domains of QOLIE-89 remained significantly improved (p < 0.05). There were no serious AEs related to stimulation, and the sudden unexplained death in epilepsy (SUDEP) rate was significantly lower than predefined comparators (p < 0.05, 1-tailed χ2).ConclusionsAdjunctive brain-responsive neurostimulation provides significant and sustained reductions in the frequency of FOS with improved QOL. Stimulation was well tolerated; implantation-related AEs were typical of other neurostimulation devices; and SUDEP rates were low.ClinicalTrials.gov identifierNCT00572195.Classification of evidenceThis study provides Class IV evidence that brain-responsive neurostimulation significantly reduces focal seizures with acceptable safety over 9 years.


2019 ◽  
Vol 160 (7) ◽  
pp. 270-278 ◽  
Author(s):  
Katalin Nóra Lőrincz ◽  
Beáta Bóné ◽  
Márton Tóth ◽  
Réka Horváth ◽  
Norbert Kovács ◽  
...  

Abstract: Introduction: Epilepsy as a chronic, severe neurologic disease significantly influences the quality of life of the epileptic patients. In candidates well selected for surgery, the seizure freedom is realistically achievable, and the quality of life can be further improved with complex individual rehabilitation. Aim: We aimed to evaluate the postoperative outcome of patients who underwent epilepsy surgery between 2005 and 2016 at the Epilepsy Center at Pécs. Method: We evaluated seizure status at regular follow-up visits after surgery and the quality of life using questionnaires focusing on employment and social status. Results: 76% of the 72 patients who underwent surgical resection for epilepsy were free from disabling seizures , and 10% had rare disabling seizures (almost seizure-free), 7% experienced worthwhile improvement and 7% had no worthwhile improvement. Comparing the employment status of patients free from disabling seizures to patients not free from disabling seizures, we found that the employment status is significantly influenced by seizure freedom (p<0.01, Fisher’s exact test). While 67% of seizure-free patients were employed, only 19% of patients not free from disabling seizures were hired. Conclusion: Our results resemble the international tendencies and success rate, proving epilepsy surgery as an available, valid and effective treatment in well selected patients. Orv Hetil. 2019; 160(7): 270–278.


2015 ◽  
Vol 28 (4) ◽  
pp. 851-858 ◽  
Author(s):  
Amanda Braga de Louredo ◽  
Ana Luiza Coelho Leite ◽  
Gisela Rosa Franco Salerno ◽  
Marcelo Fernandes ◽  
Silvana Blascovi-Assis

Abstract Introduction : Heart failure (HF) is a complex clinical syndrome representing the common final pathway of various heart diseases. It is characterized by low exercise tolerance, low survival rates and deteriorated quality of life. Several studies mention Quality of Life (QoL) as an important source of information on how disease truly affects patient's lives. In this context, the assessment of QoL is extremely important to provide data that support the choice of a therapeutic strategy and the assessment of the effectiveness of a treatment. Objectives : This study aimed to investigate and identify the most appropriate and widely used instrument for the assessment of quality of life in patients with HF. Methods : We searched the databases of Lilacs, Medline, Pubmed, Scielo and CAPES to identify relevant articles published in English and Portuguese between 2000 and 2010. Results : We found 25 papers that described, quoted or used instruments for the assessment of QoL in patients with HF. Conclusion : The MLHFQ is the most widely used instrument to assess QoL in patients with HF. Its good metric properties have been confirmed in a large number of studies. In addition, it has a simple structure and is easy to administer, which makes it the most recommended instrument for this purpose.


2013 ◽  
Vol 34 (6) ◽  
pp. E7 ◽  
Author(s):  
Sandeep Mittal ◽  
Monika Mittal ◽  
José Luis Montes ◽  
Jean-Pierre Farmer ◽  
Frederick Andermann

Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.


2015 ◽  
Vol 207 (3) ◽  
pp. 198-206 ◽  
Author(s):  
Paolo Fusar-Poli ◽  
Matteo Rocchetti ◽  
Alberto Sardella ◽  
Alessia Avila ◽  
Martina Brandizzi ◽  
...  

BackgroundThe nosology of the psychosis high-risk state is controversial. Traditionally conceived as an ‘at risk’ state for the development of psychotic disorders, it is also conceptualised as a clinical syndrome associated with functional impairment.AimsTo investigate meta-analytically the functional status of patients at high clinical risk for psychosis and its association with longitudinal outcomes.MethodThree meta-analyses compared level of functioning (n = 3012) and quality of life (QoL) (n = 945) between a high-risk group, a healthy control group and group with psychosis, and baseline functioning in people in the high-risk group who did or did not have a transition to psychosis at follow-up (n = 654).ResultsPeople at high risk had a large impairment in functioning (P<0.001) and worse QoL (P = 0.001) than the healthy control group, but only small to moderately better functioning (P = 0.012) and similar QoL (P = 0.958) compared with the psychosis group. Among the high-risk group, those who did not develop psychosis reported better functioning (P = 0.001) than those who did.ConclusionsOur results indicate that the high-risk state is characterised by consistent and large impairments of functioning and reduction in QoL similar to those in other coded psychiatric disorders.


2021 ◽  
Vol 12 ◽  
Author(s):  
Yuriz Bakhtiar ◽  
Surya Pratama Brilliantika ◽  
Jacob Bunyamin ◽  
Muhammad Thohar Arifin ◽  
Hardian Hardian ◽  
...  

Background: Besides seizure control, quality of life (QoL) should be considered as an equally important outcome for epilepsy surgery service providers. The paucity of QoL reports from developing countries has enlarged the representation gap between wealthy countries and countries with fewer resources. In this study, we evaluated postoperative QoL in the Indonesian drug-resistant epilepsy cohort where the epilepsy surgery service faces limited resource availability.Methods: We evaluated the QoL in patients with temporal lobe epilepsy who underwent surgery in our epilepsy surgery center in Semarang, Indonesia, from 2001 until 2015. The follow–up period started in 2018 through 2019. Postoperative QoL, depression, and anxiety were evaluated with self-reporting questionnaires including the Quality of Life in Epilepsy Inventory-31, Beck Depression Inventory-II, and Zung Self-Rating Anxiety Scales.Results: Forty returned questionnaires were included in the analysis (male 25, 62.5%; mean age 27.6 ± 9.05 years). The seizure-free cohort (n = 22, 55.0%) reported higher scores in most QoL dimensions particularly adjustment, overall QoL, and seizure worry compared to those with persistent seizures. The overall QoL level was correlated with seizure freedom and surgery type. QoL dimensions were negatively correlated with anxiety and depression levels.Conclusions: Postoperative seizure freedom was a major factor of postoperative QoL level. Besides seizure freedom, anxiety and depression levels were also negatively correlated with QoL levels in the Indonesian population.


Author(s):  
Jules C. Beal ◽  
Emilio Perucca

Epilepsy affects approximately 65 million people worldwide, leading to significant morbidity and mortality including injuries, psychiatric comorbidities, social stress, and the risk of sudden death. The best indicator of quality of life in patients with epilepsy is seizure freedom. The chapter discusses the medical management of epilepsy, focusing on decision making, when to initiate treatment, how to choose an appropriate medication, and how to proceed when a medication fails. The treatment of epilepsy is a highly individualized process that must take into account an individual’s seizure type, risk of seizure recurrence, age, sex, medical comorbidities, and personal goals and preferences.


2019 ◽  
Vol 62 (5) ◽  
pp. 600-608 ◽  
Author(s):  
Puneet Jain ◽  
Mary Lou Smith ◽  
Kathy Speechley ◽  
Mark Ferro ◽  
Mary Connolly ◽  
...  

2018 ◽  
Author(s):  
Lennox Hoyte ◽  
Renee Bassaly ◽  
Stuart Hart ◽  
Mona McCullough ◽  
Elisha Jackson

Pelvic organ prolapse (POP) is the descent of one of more aspects of the vagina and/or uterus. Evaluation of POP always begins with a thorough history and physical examination. Management choices include observation, conservative options, and surgical options. Surgical management is divided into two categories, which are restorative and obliterative. It is important to counsel patients that although POP may affect quality of life, it is not life-threatening. This chapter is designed to guide the healthcare provider with a review of epidemiology, anatomy, evaluation, and management of POP. The chapter contains 14 figures that illustrate examples of common examination findings, devices, and treatment options. Also, there are 5 tables that provide concise reference materials to help guide the healthcare provider. This review contains 91 references, 14 figures, and 6 tables. Key Words: constipation, mesh, pain, pelvic floor, pessary, POP-Q, risk factors, surgery, vaginal wall


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