Rare diseases · Sign or symptom
Status epilepticus
Repeated seizures without recovery between them
HP:0002133
What it means
Status epilepticus is a type of prolonged seizure resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms which lead to abnormally prolonged seizures (after time point t1). It is a condition that can have long-term consequences (after time point t2), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures.
In 2015 the ILAE Task Force on Classification of Status Epilepticus concluded that the evidence to define time points 1 and 2 in humans was incomplete. For tonic-clonic status epilepticus t1 is defined as 5 minutes and t2 as 30 minutes. For focal status epilepticus with impaired consciousness t1 is defined as 10 minutes and t2 over 60 minutes. For absence status epilepticus t1 is defined as 10-15 minutes and t2 is unknown.
Rare diseases that can present with this59
Very common80–99%
5Common30–79%
13- Combined oxidative phosphorylation defect type 27
- Desmosterolosis
- DNM1L-related encephalopathy due to mitochondrial and peroxisomal fission defect
- Kleefstra syndrome due to 9q34 microdeletion
- Lafora disease
- Nicolaides-Baraitser syndrome
- PCDH19 clustering epilepsy
- Primary hypomagnesemia-refractory seizures-intellectual disability syndrome
- Progressive myoclonic epilepsy with dystonia
- Pyridoxine-dependent-developmental and epileptic encephalopathy
- Succinic semialdehyde dehydrogenase deficiency
- Tuberous sclerosis complex
- Wrinkly skin syndrome
Sometimes5–29%
27- Alternating hemiplegia of childhood
- Angelman syndrome
- Autosomal dominant hyperinsulinism due to Kir6.2 deficiency
- Autosomal dominant hyperinsulinism due to SUR1 deficiency
- Bilateral generalized polymicrogyria
- Cysticercosis
- Folinic acid-responsive seizures
- Hemimegalencephaly
and 19 more in this range
Rare1–4%
13- Cocaine intoxication
- Familial or sporadic hemiplegic migraine
- FOXG1 syndrome
- Genetic epilepsy with febrile seizure plus
- Juvenile myoclonic epilepsy
- Leigh syndrome
- Neurodevelopmental delay-seizures-ophthalmic anomalies-osteopenia-cerebellar atrophy syndrome
- Niemann-Pick disease type C
and 5 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Prolonged seizure · Repeated seizure without recovery
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.