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Introduction

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Epilepsia partialis continua (EPC) is a rare presentation of epilepsy, but its recognition is important because it may be the harbinger of very serious disease. EPC was described by the International League Against Epilepsy as involving nonprogressive localized partial motor seizures followed by myoclonus at a later point in time.1 It is characterized by sustained and persistent repetitive focal motor activity, usually described as clonic or myoclonic in nature, typically involving a limited number of muscle groups. Both agonist and antagonist muscle groups can be involved, with any frequency, and it can persist to some degree in sleep. The face, shoulder, arm, and hand are most commonly affected (Videos 27-1, 27-2, 27-3, 27-4, and 27-5). The movement may have an arrhythmic character. Some patients report seizures worsening during activities, such as moving to pick up an object. In these cases, seizure exacerbations as the hand nears the target may be suggestive of an intention tremor, but the larger amplitude in EPC usually distinguish the two types of movements.

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Video 27-1: EPC in Patient with Rasmussen Syndrome, Part 1

Joseph I. Sirven

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Video 27-2: EPC in Patient with Rasmussen Syndrome, Part 2

Joseph I. Sirven

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Video 27-3: EPC in the Left Foot and Leg

Joseph I. Sirven

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Video 27-4: EPC in the Left Hand

Joseph I. Sirven

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Video 27-5: Left Hand Dystonic Posturing with Period Myoclonic-Like Jerking

Joseph I. Sirven

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Etiologies and Associations

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EPC may be due to a number of conditions (Table 27-1). Inflammatory causes comprise some of the best-known etiologies of EPC. Infectious agents and syndromes associated with EPC are primarily viral (Epstein-Barr virus, cytomegalovirus, JC polyomavirus of progressive multifocal leukoencephalopathy, human immunodeficiency virus, tick-borne encephalitis virus, and measles virus in subacute sclerosing panencephalitis), although bacterial causes (tuberculosis, cat scratch disease, and pertussis), fungal disease (cryptococcosis), and prion diseases (Creutzfeldt-Jakob disease) also have been seen.2,3 Rasmussen syndrome is an inflammatory condition that primarily affects children and is discussed in detail later in this chapter, along with the relationship of EPC to Kozhevnikov encephalitis. Other associations are multiple sclerosis, lupus erythematosus, and Sjögren syndrome. Paraneoplastic syndromes, which also are mediated by antibodies, may be a cause. One oncological association is gliomatosis cerebri.

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Table 27-1Conditions Associated with Epilepsia Partialis Continua

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