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¨A síndrome de Lennox-Gastaut (SLG) é uma das formas mais graves de epilepsia na infância devido à alta frequência de convulsões, resistência aos medicamentos e frequência associativa com comprometimento cognitivo. Se inicia comumente antes da idade de 5 anos, mais comum no sexo maisculino e se caracteriza por crises tônicas-axiais, atônicas e de ausência atípica. O EEG caracteriza-se por um padrão de ponta e onda frontocentral dominante lento (< 3 Hz) e os pacientes apresentam deficiência mental. As convulsões são de difícil controle e é comum um status epilepticus associado a torpor, espasmos e alterações do tônus. Em torno de 60% dos pacientes têm uma causa subjacente clara de encefalopatia (sintomática). Os demais casos são criptogênicos ou idiopáticos. Muitos medicamentos inicialmente reduzem a frequência de conulsões, mas, com o tempo, perdem sua eficácia. A Liga Internacional contra a Epilepsia (ILAE) enfatizou a importância da detecção precoce de crianças que são candidatas a procedimentos cirúrgicos no tratamento da SLG e recomendou o encaminhamento imediato para centros cirúrgicos especializados. Existem três opções na abordagem cirúrgica da epilepsia em crianças com SLG: ressecl~çao cirúrgica, calosotomia e estimulalçao do nervo vago.¨

 

■ Lennox-Gastaut syndrome in mitochondrial disease

Soonie Lee, Min-Seong Baek, Young-Mock Lee

Yonsei Med J. 2019 Jan 1; 60(1): 106–114

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6298891/

 

■ Burden of illness in patients with possible Lennox-Gastaut syndrome: a retrospective claims-based study

N L Reaven, S E Funk, G D Montouris et al

Epilepsy & Behavior 2018, vol 88: p66-67

https://www.epilepsybehavior.com/article/S1525-5050(18)30545-6/fulltext

 

■ The refractory epilepsy screening for Lennox-Gastaut syndrome (REST-LGS)

J E Piña-Garza, D Boyce, D M Tworek et al

Epilepsy & Behavior 2018, vol 90: p148-153

https://www.epilepsybehavior.com/article/S1525-5050(18)30566-3/fulltext

 

■ Optimizing clobazan treatment in patients with Lennox-Gastaut syndrome

J Isojarvi, B E Gidal, S Chung, R T Wechsler

Epilepsy & Behavior 2018, vol 78: p14—154

https://www.epilepsybehavior.com/article/S1525-5050(17)30721-7/fulltext

 

■ Síndrome de Lennox Gastaut: aproximación diagnóstica y avances terapéuticos: Fármacos antiepilépticos, Canabidiol y otras alternativas.

Herrera, Manuel L and Burneo, Jorge G

Rev Neuropsiquiatr, Abr 2018, vol.81, no.2, p.82-

http://www.scielo.org.pe/scielo.php?script=sci_arttext&pid=S0034-85972018000200005&lng=es&nrm=iso

 

■ Lennox-Gastaut syndrome and palliative surgical treatment – bibliographic review

B C Ribeiro, G Estevam, P H P de Aguiar, S Simis

Rev Chil Neurocirurgia 2018, vol 44: 83-88

http://www.neurocirugiachile.org/pdfrevista/v44_n1_2018/decastro_p83_v44n1_2018.pdf

 

■ Expert opinion on the management of Lennox-Gastaut syndrome: treatment algorithms and practical considerations

1. Helen Cross, Stéphane Auvin, Mercè Falip et al

Front Neurol. 2017; 8: 505

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649136/

 

■ Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends challenges and future directions

Adam P Ostendorf, Yu-Tze Ng

Neuropsychiatr Dis Treat. 2017; 13: 1131–1140

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5404809/

 

■ Ophthalmologic features of Lennox-Gastaut syndrome

Bo Hee Kim, Young Suk Yu, Seong-Joon Kim

Korean J Ophthalmol. 2017 Jun; 31(3): 263–26

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5469930/

 

■ Assessment of treatment patterns and healthcare cost associated with probable Lennox-Gastaut syndrome

J E Piña-Garza, G D Montouris, F Vekeman et al

Epilepsy & Behaviour 2017, vol 73: 46-50

http://www.epilepsybehavior.com/article/S1525-5050(17)30286-X/fulltext

 

■ Electroencephalography network effects of corpus callosotomy in patients with Lennox-Gastaut syndrome

Jun-Ge Liang, Dongpyo Lee, Song Ee Youn, Heung Dong Kim, Nam-Young Kim

Front Neurol. 2017; 8: 456

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5591410/

 

 Recent developments in the genetics of childhood epileptic encephalopathies: impact in clinical practice

■ Treatment of adults with Lennox-Gastaut syndrome: further analysis of efficacy and safety/tolerability of rufinamide

Rob McMurray, Pasquale Striano

Neurol Ther. 2016 Jun; 5(1): 35–43

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4919131/

 

■ Comprehensive overview: efficacy, tolerability, and cost-effectiveness of clobazan in Lennox-Gastaut syndrome

Michele A Faulkner

Ther Clin Risk Manag. 2015; 11: 905–914

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4467745/

 

■ Retention rates of rufinamide in pediatric epilepsy patients with and without Lennox-Gastaut syndrome

Sudha Kilaru Kessler, Ann McCarthy, Avital Cnaan, Dennis J. Dlugos

Epilepsy Res. 2015 May; 112: 18–2

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4805421/

 

■ Síndrome de Lennox-Gastaut, una revisión actualizada

P David, V Garcia, S Meneses

Revista Chilena de Epilepsia 2014, Año 14 (3)

http://www.revistachilenadeepilepsia.cl/wp-content/uploads/2015/04/201403_sindrome_lennox_gastaut_revision.pdf

 

■ Conceptualizing Lennox-Gastaut syndrome as a secondary network epilepsy

John S. Archer, Aaron E. L. Warren, Graeme D. Jackson, David F. Abbott

Front Neurol. 2014; 5: 225.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4214194/

 

■ Experience in the use of clobazan in the treatment of Lennox-Gastaut syndrome

Gabriela Purcarin, Yu-Tze Ng

Ther Adv Neurol Disord. 2014 May; 7(3): 169–176

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3994921/

 

■ Stable dosages of clobazan for Lennox-Gastaut syndrome are associated with sustained dro-seizure and total-seizure improvements over 3 years

Joan A Conry, Yu-Tze Ng, Lydia Kernitsky et al

on-behalf-of the OV-1004 Study Investigators

Epilepsia. 2014 Apr; 55(4): 558–56

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4303987/

 

■ Cost-effectiveness analysis of antiepileptic drugs in the treatment of Lennox-Gastaut syndrome

K M Clements, M Skornicki, A K O´Sullivan

Epilepsy & Behavior 2013, vol 29: p184-189

https://www.epilepsybehavior.com/article/S1525-5050(13)00334-X/fulltext

 

■ Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox-Gastaut syndrome: a meta-analysis

Guido Lancman, Michael Virk, Huibo Shao et al

Seizure. 2013 Jan; 22(1): 3–8.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3655762/

 

■ Síndrome de Lennox-Gastaut

G ZAldívar-Pascua, G Dávila-Gutiérrez

Acta Pediátrica de México 2011, vol 32: 75-76

https://www.redalyc.org/pdf/4236/423640327012.pdf

 

■ Treating Lennox-Gastaut syndrome in epileptic pediatric patients with third-generation rufinamid

Jessica Gresham, Lea S Eiland, Allison M Chung

Neuropsychiatr Dis Treat. 2010; 6: 639–645.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2951747/

 

■ Síndrome de Lennox-Gastaut

L B Maldonado, A J M Avellan

El Residente 2009, vol IV (2)

http://www.medigraphic.com/pdfs/residente/rr-2009/rr092e.pdf

 

■ Treatment of Lennox-Gastaut syndrome: overview and recent findings

Kenou van Rijckevorsel

Neuropsychiatr Dis Treat. 2008 Dec; 4(6): 1001–1019.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2646636/

 

■ Lennox-Gastaut syndrome, review of the literature and a case report

T A Saleh, L Stephen

Head Face Med 2008, vol 4: 9

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2483705/

 

■ Role of rufinamide in the management of Lennox-Gastaut syndrome (childhood epileptic encephalopathy)

Gerhard Kluger, Bettina Bauer

Neuropsychiatr Dis Treat. 2007 Feb; 3(1): 3–11

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2654531/

 

by Dr Paulo Fernando Leite

Médico Cooperado Unimed BH – CRMMG: 7026

Cardiologia – Centro Médico Unimed BH

Belo Horizonte/Minas Gerais/Brasil

Email: pfleite1873@gmail.com

¨A síndrome hepatorrenal (SHR) é o desenvolvimento do quadro de insuficiência renal em pacientes com doença hepática crônica prévia sem evidências clínica ou laboratorial de nefropatia prévia. Atinge até 18% dos pacientes cirróticos com ascite em um ano, chegando a 39% em cinco anos, com uma sobrevida média em torno de duas semanas após estabelecido o quadro. O diagnóstico da SHR baseia-se em critérios clínicos e laboratoriais. Critérios diagnósticos do Síndrome Hepatorrenal (SHR): cirrose com ascite; creatinina sérica >1.5 mg/dL (persistente 2 dias apos suspensão de diuréticos e albumina); exclusão de choque, nefrotoxicos e doença  renal parenquimatosa aguda. Classificação do SHR: tipo 1 (duplicação da creatinina inicial para >2.5 mg/dL em <2 semanas) e tipo 2 (agravamento mais lento e ligeiro da função renal). Recentemente, descreve-se ainda o SHR tipo 3, em doentes com SHR e patologia renal simultâneos. Sem tratamento, a sobrevida no SHR tipo 1 e aproximadamente 2 semanas e no SHR tipo 2 seis meses. O tratamento do SHR baseia-se na reversão da doença hepática de base / no transplante hepático (transplantes de fígado-rim reservam-se para doentes em dialise ha mais de 8 semanas). No entanto, com a terapêutica atualmente disponível, pode haver reversão do SHR sem transplante hepático. O SHR tipo 1 trata-se com vasoconstritores (principalmente terlipressina; se doente em Unidade de Cuidados Intensivos, a noradrenalina pode ser uma alternativa) e albumina. Espera-se reversão do SHR em aproximadamente metade dos doentes. Se a creatinina não diminuir e o doente tiver indicações clássicas para dialise, esta pode ser utilizada ate a recuperação hepatica / ao transplante. O tratamento do SHR tipo 2 consiste em paracenteses evacuadoras periódicas com albumina. Se ineficazes, podem-se usar vasoconstritores. Como a disfunção renal e ligeira, a dialise nao esta indicada. Seu aparecimento está relacionado ao mecanismo de formação de ascite, que envolve vasoconstrição e hipofluxo renal, retenção de água e sódio, aumento do volume plasmático, e conseqüentemente hiperfluxo no território esplâncnico. Mediadores vasoativos renais e humorais, como a endotelina 1, tromboxano A2 e leucotrienos, estão ainda envolvidos na gênese desta síndrome que culmina com insuficiência renal funcional. O tratamento preconizado da SHR pode ser farmacológico ou cirúrgico, sendo o transplante de fígado o único efetivo e permanente, com sobrevida de até 60% em quatro anos. Após melhora da função hepática, geralmente há a reversão da insuficiência renal. O diagnóstico precoce e a rápida terapêutica podem ampliar a expectativa de vida destes hepatopatas enquanto se aguarda o transplante hepático para seu tratamento definitivo.¨

 

■ Clinical outcomes after liver transplantation for hepatorenal syndrome: a systematic review and meta-analysis

Piyapon Utako, Thapanakul Emyoo, Thunyarat Anothaisintawee et al

Biomed Res Int. 2018; 2018: 5362810

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5994306/

 

■ Noradrenaline for reverting hepatorenal syndrome: a prospective observational, single-center study

Kamesh Gupta, Pooja Rani, Anurag Rohatgi et al

Clin Exp Gastroenterol. 2018; 11: 317–324

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6151092/

 

■ Vasoactive agents for hepatorenal syndrome: a mixed treatment comparison network meta-analysis and Trial sequential analysis of randomized clinical trials

Kannan Sridharan, Gowri Sivaramakrishnan

J Gen Intern Med. 2018 Jan; 33(1): 97–102

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5756164/

 

■ EASL clinical practice guideline for the management of patients with decompensated cirrhosis

European Association for the Study of the Liver

Journal of Hepatology 2018, vol 69 (2): 406-460

https://www.journal-of-hepatology.eu/article/S0168-8278(18)31966-4/fulltext

 

■ Renal dysfunction in cirrhosis: acute kidney injury and the hepatorenal syndrome

T Bucsics, E Krones

Gastroenterology Report 2017, vol 5 (2); 127-137

https://academic.oup.com/gastro/article/5/2/127/3752478

 

 

■ Hepatorenal syndrome: update on diagnosis and therapy

J G Acevedo, M E Cramp

World J Hepatol 2017, vol 9 (6): 293-299

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5332418/

 

■ Asociación de insuficiencia renal aguda con mortalidad y complicaciones en pacientes hospitalizados con cirrosis hepática

Castro-Serna, D et al.

Med. interna Méx., Feb 2017, vol.33, no.1, p.41-47

http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0186-48662017000100041&lng=es&nrm=iso

 

■ Renal dysfunction in cirrhosis: acute kidney injury and the hepatorenal syndrome

Theresa Bucsics, Elisabeth Krones

Gastroenterol Rep (Oxf) 2017 May; 5(2): 127–137

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5421450/

 

■ Biomarkers of renal injury in cirrhosis: association with acute kidney injury and recovery after liver transplantation

Ashwani K Singal, Bradford Jackson, Glauber B Pereira et al

Nephron. 2018; 138(1): 1–12

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5828970/

 

■ Terlipressin versus noradrenaline for hepatorenal syndrome – Economic evaluation under the perspective of the Brazilian Public Health System

Mattos, Ângelo Zambam de, Mattos, Angelo Alves de and Ribeiro, Rodrigo Antonini

Arq. Gastroenterol., June 2016, vol.53, no.2, p.123-126

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032016000200123&lng=en&nrm=iso&tlng=en

 

■ Síndrome hepatorrenal: fisiopatologia, diagnóstico y manejo.

Ospina T, Juan Ricardo and Restrepo G, Juan Carlos

Rev Col Gastroenterol, Jun 2016, vol.31, no.2, p.146-15

http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0120-99572016000200007&lng=en&nrm=iso

 

■ Outcomes of liver transplantation in patients with hepatorenal syndrome

Rohan M Modi, Nishi Patel, Sherif N Metwally, Khalid Mumtaz

World J Hepatol. 2016 Aug 28; 8(24): 999–1011.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5002501/

 

■ Cirrhotic cardiomyopathy: another case of a successful approach to treatment of hepatorenal syndrome

Luis Otávio Mocarzel, Jessica Bicca, Luiza Jarske et al

Case Rep Gastroenterol. 2016 Sep-Dec; 10(3): 531–537

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5091268/

 

■ Hepatorenal syndrome: aetiology, diagnosis, and treatment

1. Low, G. J. M. Alexander, D. J. Lomas

Gastroenterol Res Pract. 2015; 2015: 20701

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4306364/

 

■ Current position of vasoconstrictor and albumin infusin for type 1 hepatorenal syndrome

Abhasnee Sobhonslidsuk

World J Gastrointest Pharmacol Ther. 2015 Aug 6; 6(3): 28–31

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4526839/

 

■ Treatment and management of ascites and hepatorenal syndrome: an update

Kurt Lenz, Robert Buder, Lisbeth Kapun, Martin Voglmayr

Therap Adv Gastroenterol. 2015 Mar; 8(2): 83–100

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4314304/

 

■ Hepatorenal syndrome: a review of pathophysiology and current treatment options

Brian Erly, William D. Carey, Baljendra Kapoor et al

Semin Intervent Radiol. 2015 Dec; 32(4): 445–454

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4640915/

 

■ Síndrome hepatorenal

V R Emilio, J A Rodrigo, C D Luis et al

Revista Médica 2015, vol 5 (3): 134-140

http://www.medigraphic.com/pdfs/revmed/md-2014/md142h.pdf

 

■ Hyponatremia and hepatorenal syndrome

Arpan Mohanty, Guadalupe Garcia-Tsao

Gastroenterol Hepatol (N Y) 2015 Apr; 11(4): 220–229.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4836593/

 

■ Hepatorenal syndrome: update on diagnosis and treatment

Olga Baraldi, Chiara Valentini, Gabriele Donati et al

World J Nephrol. 2015 Nov 6; 4(5): 511–52

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4635371/

 

■ What’s new in hepatorenal syndrome? An updated review for the nephrologist

Magrico, Rita, Mateus, Ana and Ramos, Aura

Port J Nephrol Hypert, Dec 2013, vol.27, no.4, p.249-260

http://www.scielo.mec.pt/scielo.php?script=sci_arttext&pid=S0872-01692013000400005&lng=pt&nrm=iso&tlng=en

 

■ Medical management of hepatorenal syndrome

A Davenport, J Ahmad, A Al-Khafaji et al

Nephrology Dialysis Transplantation 2012, vol 27 (1)

https://academic.oup.com/ndt/article/27/1/34/1933244

 

■ Hepatorenal syndrome

Jan Lata

World J Gastroenterol. 2012 Sep 28; 18(36): 4978–4984

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3460323/

 

■ Hepatorenal syndrome: the 8^th international consensus conference of the Acute Dialysis Quality Initiative (ADQI) Group

M K Nadim, J A Kellum, A Davenport et al

Critical Care 2012, vol 16: R23

https://ccforum.biomedcentral.com/articles/10.1186/cc11188

 

■ Conceptos actuales en síndrome hepatorrenal

M F Higuera-de la Tijera, J M Abdo-Francis, A I Servin-Caamano et al

Rev Med Hosp Gen Méx 2011, vol 74 (1): 42-49

http://www.elsevier.es/es-revista-revista-medica-del-hospital-general-325-pdf-X0185106311025501

 

■ Poor outcomes with treatment of hepatorenal syndrome type 1 with splancnic vasoconstrictors and albumin: report of seven cases and review of the literature

Kalil, Jamile Rosário et al.

Arq. Gastroenterol., Sept 2009, vol.46, no.3, p.214-218

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032009000300014&lng=en&nrm=iso&tlng=en

 

■ Progress in treatment of massive ascites and hepatorenal syndrome

Alexander L Gerbes, Veit Gulberg

World J Gastroenterol. 2006 Jan 28; 12(4): 516–519

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4066081/

 

■ Hepatorenal syndrome: an update.

Genzini, Tércio and Torricelli, Fábio César Miranda

Sao Paulo Med. J., Jan 2007, vol.125, no.1, p.50-56

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802007000100010&lng=en&nrm=iso&tlng=en

 

by Dr Paulo Fernando Leite

Médico Cooperado Unimed BH – CRMMG: 7026

Cardiologia

Belo Horizonte/Minas Gerais/Brasil

Email: pfleite1873@gmail.com