Monthly Case

A 31-year-old male patient is seen regularly in out epilepsy outpatient clinic for more than 10 years. At age 18 years, he suffered a first tonic clonic generalized epileptic seizure early after awakening following a short night with 3 hours of sleep. In the following 13 years, he had another two generalized tonic clonic seizures, both of which occurred after sleep deprivation and directly after awakening. Family history is unremarkable, multiple routine EEGs were normal.

Beyond the epileptic seizures, the patient reported episodes on awakening with complete paralysis with the exemption of tongue and eye movements. This sleep paralysis lasts 1-2 min and is associated with anxiety. So far, these episodes have occurred five times. In addition, the patient reports excessive daytime sleepiness. Polysomnography with multiple sleep latency test revealed SOREM, i.e. sudden onset rapid eye movement sleep.

In this patient, two diagnoses can be made: Firstly, he suffers from epilepsy which likely is idiopathic generalized epilepsy, subsyndrome: epilepsy with grand mal on awakening. Secondly, he suffers from narcolepsy which in this case is characterized by sleep paralysis and striking daytime sleepiness. The diagnosis of narcolepsy was confirmed by the finding of SOREM in the multiple sleep latency test.

Treatment of choice in idiopathic generalized epilepsies is valproic acid. Unfortunately, the current patient developed some unspecific adverse events from this substance. With carbamazepine 900 mg, the patient is seizure-free for the last 3 years. However, this antiepileptic drug is not without problems in idiopathic generalized epilepsies as this sodium channel blocker provides the risk to facilitate occurrence of myoclonic and absence seizures. Gladly, this didn’t occur in this patient. Narcolepsy was treated with modafinil. Since regular administration of this drug, the patient did not experience any more sleep paralyses, excessive daytime sleepiness has improved significantly.

Figures on coincidence of idipathic generalized epilepsies and narcolepsy has not been reported in the literature and genetic similarities have not been demonstrated so far. However, it has to be considered that narcolepsies with a prevalence of 1 in 5,000 is a rather rare condition.

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