History of Status Epilepticus

Fig. 1.1

Map of Paris in the 1880s showing the peregrinations taken by a mailman with bromide-responsive epileptic “wanderings”—an early case of nonconvulsive status epilepticus? (From Shorvon [4], with permission)

After epileptic fits of moderate severity, the patient may pass into a condition of mental automatism, in which various acts are performed in an apparently conscious manner, but of which no recollection is afterwards retained.

Gowers further [13], refers to the studies of Bourneville and provides one clinical description, noting:

the intervals between the fits becomes shorter, the coma deepens, the pulse and respiration become very frequent, and the temperature rises, it may be of 104°, 105°, or even 107°. Sometimes hemiplegia comes on after the condition has existed for several days. The patient may die in a state of collapse, death being apparently due to the violent and almost continuous convulsions, or, the fits ceasing, he may become delirious and present symptoms of meningitis, with rapid formation of bedsores, and may die in this stage. At any period, the symptoms may lessen, and the patient recover. A large proportion of the cases, however, end fatally. Fortunately, this severe degree of the status epilepticus is very rare, at any rate out of asylums for the insane. No instance in which death occurred has come under my own observation, although I have seen many examples of a slighter degree of the condition, from which the patients have recovered [13, p. 193–4].

While addressing treatment, Gowers [23] offers little reassurance:

In the ‘status epilepticus,’ in which attacks recur with great frequency for several days, and in which bromide often fails entirely, I have known hypodermic injections of morphia, in doses of 1/16th of a grain to be of great service, and Sieveking has found it useful, given by the mouth, in the same state. But morphia is a remedy which can only be employed hypodermically in epileptics with extreme caution. If an attack occurs, and the post-epileptic coma coincides with the sleep induced by morphia, the patient’s life is in great danger [13, p. 273].

He goes on to note:

In the status epilepticus, bromide often fails. Inhalations of nitrite of amyl have been found useful by Crichton Browne. Chloroform inhalations rarely have a permanent effect. The remedies from which I have seen most good are repeated dosages of chloral, the subcutaneous injection of morphia, and the application of ice to the spine [13, p. 290–1].

Thus long neglected in the written records of medicine, it would seem that status epilepticus had been addressed within the greater context of epilepsy only over the past few hundred years. Careful observations of individual cases in association with gross anatomical and classic histological correlation led to its identification as the “maximum expression of epilepsy” [4].

The Advent of Electroencephalography

After 1924 when Hans Berger discovered the recordable electrical impulses from the brain using electroencephalography (EEG), there was a concerted effort to link the now-measurable brain dysfunction with its clinical correlates, and the study of epilepsy was rapidly transformed into a technically driven clinical science for the 50 years that followed. (More recently, neuroimaging, especially MRI, appears to have taken over a part of this role.) Berger was the first to systematically study patients with epilepsy using EEG. In his seventh report in 1933, he recorded the first EEG of focal motor seizures in a patient with epilepsia partialis continua, and recorded another patient with 3/sec spike and wave discharges [23]. Following this, a series of single case reports and series used a different terminology (e.g. epilepsia minor continua, status pyknolepticus, absence continua, absence status spike-wave stupor, and others). It soon became clear that not every patient with a milder form of status had generalized spike-wave discharges [24]. The influence of EEG on scientific thought was so dominant that etiologic and pathological research dawning in the nineteenth century almost stopped completely. In Germany, the Nazi regime and the Second World War took its toll, and many of the researchers in this field had to leave their countries.

As with convulsive status epilepticus, it was the advent of EEG that proved beyond doubt that nonconvulsive status epilepticus derived from an epileptic brain, and not, as some had suspected, from hysterical or nonepileptic fugue states. Gibbs, Gibbs, and Lennox regarded a seizure as a “paroxysmal cerebral dysrhythmia” [25], and in 1945 Lennox described the clinical and EEG features of absence status in his cousin Ann Lennox and coined the term petit mal status [26]. This was followed in 1956 by Gastaut and Roger, who described a nurse with complex partial status epilepticus (CPSE) that may have lasted several months. They called the condition état de mal temporal [24].

From about the 1950s onwards, a somewhat more detailed investigation of the consequences of SE came with the publication of case series of SE. Whitty and Taylor noted that a longer duration of SE appeared to correlate with a worse outcome [27], with 1/3 of their patients dying. It was Janz in 1953 who systematically studied the relationship of the number of grand mal seizures and the average interval between the seizures preceding status epilepticus [28]. Based on the analysis of 103 grand mal seizures he found that 3 or 4 more seizures would follow when the inter-seizure interval dropped to 2–6 h. If the inter-seizure interval were <1 h at least 6 seizures, and most likely “true” status epilepticus, would ensue if seizures were not terminated by treatment. In the 42 cases of SE reported by Janz in 1964, those that were symptomatic were found to have a frontal origin [29]. In general, symptomatic SE was commoner than idiopathic types. Hunter’s review of the Register General in the United Kingdom reported that SE accounted for a third of the cases of death in patients with epilepsy [5]. About 25% of status seen at Queen Square was thought to be precipitated by changes in medication, with some 30% associated with an inter-current infection.

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