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seizures, and typical absences, which occur in around one-third of patients. Seizures have an
age-related onset. Myoclonic jerks are the defining seizures starting in the mid-teens and
occurring mainly on awakening, particularly after sleep deprivation. The tendency to seizures
is probably life-long. The management of JME differs from standard medical practice for the
treatment of ‘epilepsy’ in several important respects20,21.

An editorial in the Lancet by Grunewald and Panayiotopoulos in 1992 states the
following12,21: ‘There is no better example of the importance of syndrome classification than
juvenile myoclonic epilepsy. JME accounts for between 5.4% and 10.2% of cases of epilepsy,
but, despite clinical and electroencephalographic features that should enable its easy
identification, the rate of misdiagnosis remains high. Accepted practice for management of
‘epilepsy’ will often be inappropriate in this condition  e.g. the withholding of treatment in
patients who have had a single generalised tonic-clonic seizure, drug withdrawal after two or
three years’ freedom from seizures, and stopping sodium valproate or substituting
carbamazepine in women who plan to become pregnant. Accurate diagnosis does more than
improve patient management and well-being; it also allows proper advice on prognosis,
genetic risk, and employment. Failure to diagnose JME represents a serious medical error;
how can diagnostic accuracy and management be improved? Physicians should be ever alert
to the possibility of JME21.’

The syndromes of temporal lobe epilepsy
The syndromes of temporal lobe epilepsy comprise more than 30% of epilepsies. They are
an heterogeneous group of disorders sharing the same topographic seizure onset but often of
diverse aetiology, age at onset, prognosis, response to medical or surgical management.
Aetiology may be symptomatic, idiopathic or metabolic. The commonest of all, hippocampal
epilepsy, is found in around 20% of patients with epilepsies. Hippocampal epilepsy is a
distinct epileptic disease with defined pathology (hypocellular and gliotic ‘sclerotic’
hippocampus with a unique pattern of cellular loss, not found in other brain diseases). High-
resolution magnetic resonance imaging (MRI) identifies existing pathology in around 90%
of patients. Drug treatment is similar to other partial seizure types. Carbamazepine and
phenytoin are the most effective of the older drugs. Of the newer drugs, all claim efficacy:
lamotrigine, vigabatrin, topiramate, tiagabine, gabapentin, zonisamide. These treatments may
be relatively effective in 80% of patients. If one or two of the main drugs fail, the chances of
achieving medical control are negligible. These patients, even in childhood, need urgent
evaluation for neurosurgical treatment for which they are the best candidates and the most
likely to have excellent and sustained benefit22.

Epilepsy or epilepsies? Is this controversial?

Even the most sceptical physicians who doubt the clinical or practical significance of the
syndromic diagnosis of epilepsies have to accept that BCSSS, JME and syndromes of
temporal lobe epilepsy have more differences than similarities. They all require different
management and their short and long-term treatment strategies are entirely different. What
may be the best drug for one may be deleterious for the other.

The time is right for eradicating the traditional diagnostic label of ‘epilepsy’. This change
may favourably influence the diagnosis, management, and welfare of people with epileptic
seizures. The treatment of epilepsies will change but their correct diagnosis will always be
the medical target. This concept is not difficult to understand and need not be controversial.