Journal of Parkinson's Disease and Movement Disorders

Yavuz ALTUNKAYNAK, Devrimsel Harika ERTEM, Ayhan KÖKSAL, Alkin YILMAZ, Sevim BAYBAŞ

Bakırköy Ruh Sağlığı Sinir Hastalıkları Eğitim ve Araştırma Hastanesi, 2. Nöroloji Kliniği, İstanbul, Türkiye

Keywords: Action myoclonus; clonazepam; Lance- Adams syndrome; levetiracetam; posthypoxic myoclonus; valproic acid.


First described in 1963, Lance-Adams Syndrome (LAS) is a posthypoxic myoclonic encephalopathy due to several factors following cerebral hypoxia. It is accompanied by action myoclonus, cerebellar ataxia and cognitive decline. Resistant, action-induced and stimulus-sensitive asyncronised myoclonic jerks are seen in the distal extremities. No specific diagnostic or therapeutic procedure is available for LAS, a rare condition. The prognosis of the disease is also unclear. A 50-year-old male patient with a history of cardiac arrest during an inguinal hernia surgery and who underwent cardiopulmonary resuscitation was admitted to the emergency department with the complaints of behavioral changes, imbalance and involuntary movements in the limbs. The patient was diagnosed with LAS based on the history, neurological examination and test results and clonazepam and valproic acid primarily were initiated. Levetiracetam was also added for the treatment of resistant myoclonus. Following treatment, a dramatic improvement in the clinical presentation was observed. Although several drugs are available for the treatment of LAS, currently there is no definitive treatment proposed. In this article, we present a case with LAS and discuss his clinical findings and our treatment approach with regard to the literature review.