Jacksonian seizures

Dawn Eliashiv MD (Dr. Eliashiv of the David Geffen School of Medicine at the University of California, Los Angeles, received honorariums from Eisai, Cyberonics (LivaNova Inc), Neuropace Inc, Sunovion, and UCB for consulting work.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released September 13, 2011; last updated January 16, 2017; expires January 16, 2020

This article includes discussion of Jacksonian seizures, Bravais-Jacksonian seizures, and Jacksonian march seizures (with local spread). The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Historical note and terminology

Historical note. Since the time of Hippocrates, focal motor seizures have been a clinically recognized phenomenon. Hippocrates astutely understood that seizures arose contralateral to the diseased hemisphere. However, predating the Greeks, the Sakkiku, a medical textbook written in Assyrian Babylonia around 1000 BCE, provides accounts concerning focal motor seizures (Wilson and Reynolds 1990).

Louis François Bravais described a form of focal motor epilepsy in his doctoral thesis published in 1827, roughly 40 years before Jackson's descriptions. He gave this disease, in which there were only hemibody convulsions, the name l'epilepsie hémiplégique, derived from the Greek hemiplegia, literally meaning “I strike by half” (Maisonneuve 1805; Bravais 1827). He described 5 different entities of l'epilepsie hémiplégique, which are as follows: (1) those beginning in the face or tongue, (2) those beginning in the upper limbs, (3) those beginning in the lower limbs, (4) those beginning in the abdomen, and (5) those beginning in the course of a peripheral nerve (Eadie 2010). His thesis provided a detailed description of l'epilepsie hémiplégique beginning in the face or tongue. He recognized that the convulsions retreated in the reverse order in which they appeared. Bravais was also aware of a unilateral transient paralysis following an attack and described prodromata of the somatosensory variety.

John Hughlings Jackson furthered our understanding by ascribing the clonic activity to aberrant electrical discharges in the contralateral motor cortex (Taylor 1931). He observed that the face was more commonly involved than the leg and impairment of consciousness did not occur in seizures affecting only 1 side of the body prior to spread to contralateral extremities.

Jackson reported a series of 8 patients in a lecture in 1870 titled “A Study of Convulsions.” Therewithin, he relayed the following:

 

A married woman, 43 years of age…consulted me at London hospital on December 13, 1864….Exactly one week before at 9 or 10 a.m., her right forefinger and thumb began to work, and the working continued up to the elbow, and then all the fingers worked. (She imitated the movements by alternately shutting and opening the hand.) There was no attendant sensation…She had had three attacks, and after each the hand felt “heavy and dead,” and for some time (she) could not use it well. (Taylor 1931).

His understanding of this disease was augmented by emerging knowledge of pathology, which was not readily available in Bravais' time. With information derived from necropsy, Jackson was able to deduce that the motor activity was a cortical function, debunking the prevailing belief that epilepsy was a disease originating in the brainstem.

Nomenclature. It was Charcot who coined the term Jacksonian epilepsy, using it to denote seizures with a march of symptoms (Charcot and Pitres 1877; Charcot 1881). He was aware of the neglect Bravais' work had received; hence, “Bravais-Jacksonian seizures” is another eponymous term used to describe the same phenomenon.

When the condition becomes persistent, it is deemed epilepsia partialis continua, a term coined by Kozhevnikov, who remarked:

 

In recent years I happened to observe several cases of cortical epilepsy…that it may be called epilepsia corticalis sive partialis continua, in that here the convulsive manifestations were continuous….The question of the nature of the disease process is much more difficult…in all cases the illness developed little by little and once it had developed persisted for a very long time, so that we can postulate only chronic processes here….Thus, of the chronic processes, encephalitis with transition to secondary hardening of the brain, or sclerosis cerebri, is almost the only possibility….Thus, not knowing precisely what we are dealing with, [I propose] the presence of a chronic encephalitis (Kozhevnikov 1895; Gastaut 1968).

The classification for the different types of epilepsies has undergone several revisions in the 20th century. Jacksonian seizures were categorized under the term “simple partial seizures with motor signs” by the ILAE in 1981 (Anonymous 1981). In the 1989 classification, the term Jacksonian seizures was still utilized. In later classifications, these seizures are considered “focal clonic seizures (without spread)” and “Jacksonian march seizures (with local spread)” (Anonymous 1989; Engel 2006a; Engel 2006b). They are also referred to as “focal motor seizures with elementary clonic motor signs” (Engel 2006a; Engel 2006b), “clonic seizures,” “simple motor seizures,” or “somatomotor seizures” (Luders et al 1998). If the entire hemibody is involved, then they are termed “hemiclonic seizures” (Engel 2006a). If the focal clonic activity persists for a prolonged time, the term epilepsia partialis continua is applied (Engel 2006b). The ILAE classification suggested the term “focal seizures without impairment of consciousness with observable motor components” (Berg et al 2010; Panayiotopoulos 2010). The ILAE defines Jacksonian march as a “traditional term indicating spread of clonic movements through contiguous body parts unilaterally.” Therefore, one must recognize that the term Jacksonian seizure, which is synonymous with focal motor seizures in clinical parlance, is indeed not synonymous with Jackonsian march. In the proposed ILAE 2016 operational classification of seizures, the terminology used is “focal aware motor (Jacksonian)” (Fisher et al 2016).

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