How is status epilepticus diagnosed and treated in children? Take a look at the latest Chinese expert consensus

Status epilepticus (SE) is one of the critical diseases of the children's nervous system characterized by persistent seizures and may cause damage to the nervous system and even multiple organs, with an annual incidence of 17/100,000~23/100,000, and the national multicenter children's SE retrospective study conducted by the China Collaborative Group for Pediatric Status Epilepticus found that the case fatality rate was 3%, and the remaining neurological dysfunction
of different degrees.
There are many causes of SE, and the goal of treatment is to quickly stop the seizures, and timely and standardized treatment can reduce the mortality rate and improve the prognosis
.
Thus, by ending with "children/pediatric/children, status epilepticus, diagnosis/diagnosis, treatment/treatment" Based on the evidence of relevant guidelines and new clinical research of SE diagnosis and treatment at home and abroad, combined with the clinical diagnosis and treatment experience and opinions of experts of the Collaborative Group on Pediatric Status Epilepticus, this consensus was written to guide and standardize the diagnosis and treatment
of SE in children.

​

Recommendations for diagnostic evaluation in children with SE

Rapid evaluation of children with CSE is based on history and physical examination
.
CSE is a life-threatening emergency, so assessment and treatment are carried out simultaneously, and timely seizure control and monitoring of vital signs (including blood pressure, heart rate, and oxygen saturation) should be carried out in and after seizure control, etiology should be evaluated in a timely manner and treatment of the cause should be started as soon as possible
.

➤ Internal environmental homeostasis assessment

Detect blood glucose, electrolytes, liver and kidney function, etc.
to evaluate the internal environment, and correct the internal environment disorder
in time.

➤ EEG examination

EEG should be performed in all children with new episodes of SE, especially those with CSE episodes
.
If the state of consciousness quickly returns to normal after seizure control in SE, routine EEG can be performed, and if normalization is not possible, long-range EEG/video electroencephalogram (VEEG) monitoring
is recommended.
For cases with suspected NCSE, the duration of suspected clinical signs or symptoms of NCSE should be no less than 10 minutes, and diagnosis should be combined with EEG (recommended VEEG monitoring ≥ 24 hours) and clinical data
.

➤ Neuroimaging examination

Child-onset SE requires cranial imaging to aid clinical assessment and seizure localization, and Magnetic resonance imaging (MRI) is often recommended, but computed tomography (CT) is recommended for children with SE suspected intracranial hemorrhage
.

➤ CSF test

Acute CNS infection is most common in children with SE with a clear etiology, and a lumbar puncture should be performed when meningitis/encephalitis is clinically suspected or accompanied by fever, particularly in young children (< 2 years of age); Children with SE with suspected immune encephalitis should have cerebrospinal fluid/serum immunology
.
For children with suspected intracranial infection but unable to identify the causative agent by routine testing, tests such as cerebrospinal fluid metagenomic sequencing can be used to assist in etiological diagnosis
.

➤ Genetic testing

Genetic testing is recommended in children with SE who lack a clear cause, particularly those with
a family history or developmental disabilities.

➤ Other detections

According to the child's medical history and clinical manifestations, poison detection and hematuria tandem mass spectrometry
were selected as appropriate.
Children with severe disease require multimodal neuroassessment techniques
.

treat  

➤ Principles of treatment

(1) Early treatment, early and rapid termination of clinical convulsive seizures and persistent EEG epileptiform discharge;

(2) The drug selection is appropriate, the dose should be sufficient, the usage should be appropriate, and the treatment procedure should be continuous;

(3) Comprehensive treatment, including life support, anticonvulsant treatment, etiology treatment and treatment of complications;

(4) Neurological function assessment and rehabilitation treatment: early neurological function assessment and timely rehabilitation treatment
.

➤ CSE treatment

✔ Pre-hospital management

SE occurs mostly outside the hospital, and in the absence of venous access, prehospital treatment is usually given by mucosal or intramuscular administration: midazolam (intramuscularly/nasal/buccal mucosa) or diazepam (rectum).

Nasogastric feeding with chloral hydrate or retention enema
can also be used.

✔ In-hospital treatment

First-line agents are preferred: diazepam or midazolam intravenously
.
After failure of the preferred medical therapy for intravenous infusion, second-line agents
can be followed.
Stage RSE treatment is preferred with midazolam or propofol anesthesia
.
Regardless of whether it is refractory or not, SE must always have second- and third-line drugs
readily available.

Insufficient initial treatment dose and untimely treatment are common and important reasons for the development of SE into RSE, and the delay in the treatment time of anesthesia drug treatment in patients with RSE is an important factor
affecting the treatment effect.
Therefore, it is very important to establish the SE treatment process, and the CSE diagnosis and treatment process recommended by this consensus is shown in Figure 1
.

Figure 1 CSE diagnosis and treatment flow chart (GS: Glucose solution; EEG: EEG; ECG: Electrocardiogram, electrocardiogram; RSE: refractory status epilepticus; SRSE: super refractory status epilepticus; ASMs: anti-seizure drugs
.
At present, there is no midazolam nasal dosage form in China)

✔ Inter-hospital transfer

Grassroots hospitals that lack the conditions for further treatment of children with SE should transport children with SE according to the graded referral model, fully assess the vital signs, state of consciousness and seizures of SE before transport, equip emergency personnel, first-aid drugs and rescue equipment according to the condition, pay attention to maintaining stable respiratory and circulatory functions during transportation, give mechanical ventilation and other supportive treatment when necessary, pay special attention to whether there are signs and symptoms of intracranial hypertension, and give symptomatic treatment
.
According to the principle of proximity, shorten the transit time
.

✔ Choice of therapeutic drug

Table 1 lists the usage and precautions
for CSE treatment drugs.

Table 1 Usage and precautions of commonly used therapeutic drugs for CSE

✔ SRSE treatment

SSE can lead to severe neurological sequelae and even death, but its treatment is still in the exploratory stage
.
The main treatment is the combination of anesthetic drugs with ASMs, and appropriate non-anesthesia treatment can be selected according to the child's condition (Table 2), mainly including the treatment of NCSE:

Table 2 SRSE non-anesthesia treatment

At present, there is no unified process for the treatment of NCSE, and it is necessary to choose an individualized treatment plan
according to the clinical manifestations and EEG of the child.

The main treatment principles: (1) actively look for the cause and carry out the cause treatment; (2) For children with NCSE with epilepsy, benzodiazepines can be temporarily used, and the dose or type of oral anti-seizure drugs can be adjusted; (3) For critically ill children with NCSE after CSE, the treatment principle is the same as CSE, and it is recommended to use anesthetic drugs (third-line drugs for CSE) for treatment under EEG monitoring; (4) For children with brain injury NCSE after hypoxia, especially those with hypotension, treatment can be relatively conservative
.
Continuous EEG monitoring is necessary
for the treatment of NCSE patients.

Neurological function assessment, rehabilitation and prognostic follow-up

After SE seizure control, quantifiable neurological, psychological, and rehabilitation assessments should be carried out as soon as possible, using the modified Rankin scale (mRS), Children's intelligence test scales are measured to provide early intervention and improve prognosis
.

SE is a common neurological critical illness in children, and clinical evaluation should be carried out clinically in combination with the clinical manifestations of children with SE, to clarify the cause and guide clinical diagnosis and treatment
.
The treatment of SE should be a race against time, rather early than late, the dose should be sufficient, and the control should be as
soon as possible.
At the same time, neurological function assessment and rehabilitation should be carried out early to obtain a good prognosis
.
This consensus is based on the existing SE diagnosis and treatment evidence, aiming to standardize the diagnosis and treatment decisions of children SE, this consensus is not a mandatory standard, and there are many points that need to be improved and revised, such as the treatment of SE onset 40~60min stage still lacks sufficient clinical evidence guidance and standardization, therefore, clinicians should choose a reasonable diagnosis and treatment plan
according to their own professional knowledge, clinical experience and available medical resources when facing specific SE children.