American Journal of Emergency Medicine 34 (2016) 342.e5–342.e7

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Case Report

A peculiar case of Lance-Adams syndrome☆ Lance-Adams syndrome (LAS) is a rare clinical entity characterized by myoclonus that starts days to weeks after cardiopulmonary resuscitation in patients who regained consciousness [1]. Herein we describe a peculiar case of Lance-Adams syndrome in a 29-year-old man after cardiac arrest in the context of acute myocarditis and successful cardiopulmonary resuscitation (CPR). A 29-year-old man was admitted to our intensive care unit (ICU) after an-out-of hospital cardiac arrest. He was found in gasping during the night while sleeping at home. Promptly rescued with cardiopulmonary resuscitation, defibrillation (because of ventricular fibrillation on electrocardiogram [ECG]-monitor) and drugs (adrenaline + amiodarone), he had return of spontaneous circulation after 20 minutes. His past medical history was unremarkable, but the evening before the event he had chills and muscle aches without fever self-treated with paracetamol (1-g tablet). At the moment of admission to ICU the patient was intubated and unresponsive (alert, voice, pain, unresponsive [AVPU] scale). Physical examination was unremarkable. A 12-lead ECG showed sinus rhythm with inverted T waves (Fig. 1). An echocardiogram was thus performed showing regional wall motion abnormalities in the anterior, anterolateral wall and apex with severe systolic dysfunction (ejection fraction, 25%). Coronary angiography was normal. Taking into account clinical presentation and instrumental findings, a myocarditis was suspected according to the current position statement of the European Society of Cardiology [2]. Unfortunately a cardiac MRI (CMR) was not readily available; the scan was performed about three weeks later but it did not show edema on T2-weighted images nor late gadolinium enhancement (LGE) on T1-weighted sequences (Fig. 2A and B). One day after the cardiac arrest the patient was still in coma. His electroencephalogram (EEG) showed a typical alpha coma (Fig. 3). Brain computed tomography was unremarkable. Three days later the patient gradually recovered consciousness, had no hemiparesis, and was able to follow simple commands; however, he started to have generalized myoclonus mainly involving lower limbs. Myoclonic jerks were triggered and aggravated by environmental stimuli, emotional stress, and by attempts to perform actions (intention myoclonus). A new EEG showed pseudocyclic epileptiform waves (Fig. 4). According to clinical presentation and instrumental findings a Lance-Adams Syndrome was thus diagnosed. A pharmacological treatment with levetiracetam (1500 mg twice daily) was started with low doses of benzodiazepines as an adjunct. The generalized myoclonus

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was clearly reduced with this therapy but did not completely disappeared; it was still present and aggravated during emotional changes or voluntary actions and decreased while the patient was sleeping. After 10 days the patient moved from the ICU to the cardiology department because of the myocarditis. A new echocardiogram confirmed the presence of wall motion abnormalities in the anterior and anterolateral wall with improved systolic function (ejection fraction 48%). The option of an implantable cardioverter-defibrillator in secondary prevention of sudden cardiac death was discussed. However, indication for implantable cardioverter-defibrillator in myocarditis is controversial because this condition may heal completely. Bridging with a life vest was considered a reasonable option but then abandoned. During his hospital stay the patient received progressive physical rehabilitation treatment because he had problems with activities of daily living. Three weeks after the cardiac arrest he still had some muscle jerks during attentive effort. A complete neuropsychological assessment revealed some cognitive impairment. He scored 20 in the mini-mental state examination, which confirmed cognitive disabilities. The patient also had a short-memory impairment and difficulties with attention and calculation. Therefore he was referred to a specialized rehabilitation center. We thought that this report is interesting for several reasons. First of all Lance-Adams syndrome (LAS) is per se a rare entity with fewer than 150 cases described so far [3]. Pharmacological treatment is still controversial, due mainly to a lack of clear understanding of the underlying pathophysiological mechanisms involved; however, a combined administration of sodium valproate, clonazepam and levetiracetam is usually reported [3,4]. In our patient levetiracetam alone was sufficient to control the myoclonus. Furthermore we would like to emphasize the peculiar electroencephalographic pattern in our patient; to the best of our knowledge this is the first reported case of alpha coma preceding the typical features of LAS. We conclude by recalling the impact of an early pharmacological treatment together with intensive rehabilitation on the prognosis of LAS. Giuseppe D. Sanna, MD Unità Operativa di Cardiologia, Ospedale “S.S Annunziata” Sassari Via Enrico De Nicola, 07100 Sassari, Sassari, Italy Corresponding author. Tel.: +39 0792061561; fax: +39 079210512 E-mail address: [email protected] Antonio Demurtas, MD1 Servizio di Neurologia e Neurofisiopatologia, Ospedale “S.S Annunziata” Sassari, Via Enrico De Nicola, 07100 Sassari, Sassari, Italy E-mail address: [email protected]


G.D. Sanna et al. / American Journal of Emergency Medicine 34 (2016) 342.e5–342.e7

Fig. 1. ECG. Twelve-lead ECG showing sinus rhythm with repolarization abnormalities (inverted T waves) in DI, aVL, V4-V6.

Stefano Cossa, MD2 Struttura Complessa di Radiologia, Azienda Ospedaliera “Brotzu” Piazzale Ricchi 1, 09134 Cagliari, Cagliari, Italy E-mail address: [email protected]

Annamaria Ara4 S.C. Psicologia Ospedaliera e delle Emergenze, Ospedale “S.S Annunziata” Sassari, Via Enrico De Nicola, 07100 Sassari, Sassari, Italy E-mail address: [email protected]

Francesca Murgia, MD Unità Operativa di Cardiologia, Ospedale “S.S Annunziata” Sassari Via Enrico De Nicola, 07100 Sassari, Sassari, Italy E-mail address: [email protected] Paola Murgia, MD3 Unità Operativa di Anestesia e Rianimazione, Ospedale “S.S Annunziata” Sassari, Via Enrico De Nicola, 07100 Sassari, Sassari, Italy E-mail address: [email protected]


Tel.: +39 0792061075 2

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Tel.: +39 0792061610


Tel.: +39 0792061234 References [1] Lance JW, Adams RD. The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy. Brain 1963;86:111–36.

Fig. 2. A, CMR. Turbo inversion recovery T2-weighted image (short axis) showing no oedema. B, CMR. T1-weighted LGE image showing the absence of subepicardially distributed LGE, typical for acute myocarditis.

G.D. Sanna et al. / American Journal of Emergency Medicine 34 (2016) 342.e5–342.e7


Fig. 3. EEG. EEG showing alpha waves, not responsive to acoustic stimuli (CLAP).

[2] Caforio AL, Pankuweit S, Arbustini E, Basso C, Gimeno-Blanes J, Felix SB, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2013;34(33):2636–48.

[3] Zhang YX, Liu JR, Jiang B, Liu HQ, Ding MP, Song SJ, et al. Lance-Adams syndrome: a report of two cases. J Zhejiang Univ Sci B 2007;8:715–20. [4] Lim Lee Ha, Kang Lee Ju. Lance-Adams syndrome. Ann Rehabil Med 2011;35: 939–43.

Fig. 4. EEG. EEG showing spikes and spikes/slow waves, synchronous with muscular jerkins with pseudorhythmic occurrence.

A peculiar case of Lance-Adams syndrome.

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