How do you treat Norse?

How do you treat Norse?

NORSE does not respond to standard treatment of status epilepticus (SE). Standard treatment would normally involve benzodiazepines followed by a standard anti- seizure medicine such as valproic acid, phenytoin, levetiracetam, phenobarbital, or lacosamide (preferably given intravenously).

How common is Norse?

NORSE is a rare disorder with one estimate being around 3,200 cases per year in the U.S. Underreporting and misdiagnoses are likely since the term itself is new and not well established.

What is the most common treatment for Norse?

There is currently no known specific therapy for NORSE and studies are urgently needed to determine what the best treatment options are. The treatment of SE initially consists of benzodiazepines (lorazepam, diazepam, or clonazepam), followed by a standard anticonvulsant medication, as in most cases of SE.

What is fire syndrome?

Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic syndrome that strikes previously healthy children aged 3-15 years and has an unknown pathogenesis and few treatments. These children experience a nonspecific febrile illness that is followed by prolonged refractory status epilepticus.

What causes Norse disease?

The most common causes of NORSE and FIRES are autoimmune/paraneoplastic disorders, such as encephalitis associated with anti-neuronal antibodies (anti-NMDA receptor, anti-voltage-gated potassium channel complex, etc.), followed by viral encephalitis.

What is refractory status epilepticus?

Background Refractory status epilepticus (RSE) is a life-threatening condition in which seizures do not respond to first- and second-line anticonvulsant drug therapy.

What is focal epilepsy?

Focal epilepsy is a neurological condition in which the predominant symptom is recurring seizures that affect one hemisphere (half) of the brain.

Is there a syndrome that qualifies as Norse?

The infantile hemiconvulsion‐hemiplegia and epilepsy syndrome (IHHE) is a specific syndrome that also qualifies as NORSE.

What kind of seizures do people with Norse have?

[1] The seizures that begin in people with NORSE are typically either focal impaired awareness seizures or tonic-clonic seizures (more commonly known as grand mal seizures). [1] The seizures become very prolonged or frequent over time and transition to status epilepticus (SE) over a few days.

When to know if you have Norse of unknown cause?

If no cause is found after extensive evaluation, this is considered “cryptogenic NORSE” or “NORSE of unknown cause”’. ‘FIRES is a subcategory of NORSE that requires a prior febrile infection, with fever starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus’.

Who are new-onset refractory status epilepticus ( Norse )?

New-onset refractory status epilepticus and its subcategory febrile infection-related epilepsy syndrome are rare devastating clinical presentations in those without pre-existing relevant history, often in schoolchildren or young adults, without a clear cause on initial investigations.