Pediatric Status Epilepticus Clinical Presentation

Updated: Oct 06, 2014
  • Author: Rajesh Ramachandrannair, MBBS, MD, FRCPC; Chief Editor: Timothy E Corden, MD  more...
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Presentation

History

In the initial presentation of status epilepticus (SE), a directed history suffices. Obtain a more detailed history after stabilization, including the following details about the current seizure activity:

  • Time and nature of onset of seizure activity

  • Involvement of extremities or other body parts

  • Nature of movements (eg, eye movements, flexion, extension, stiffening of extremities)

  • Incontinence

  • Cyanosis (perioral or facial)

  • Duration of seizure activity prior to medical attention

  • Mental status after cessation of seizure activity

  • Postictal neurologic deficit

Other important information to elicit in the history includes the following:

  • Fever or intercurrent illnesses

  • Prior history of seizures - If present, specify medications, anticonvulsant use, and compliance.

  • Head injury (recent and remote)

  • Central nervous system (CNS) infection or disease (eg, meningitis, neurocutaneous syndrome)

  • Intoxication or toxic exposure (see Etiology for examples)

  • Other CNS abnormality (eg, ventricular-peritoneal shunt, prior CNS trauma)

  • Birth history and developmental delay (eg, anoxic encephalopathy, cerebral palsy)

  • Other medical history (eg, acquired immunodeficiency syndrome, systemic lupus erythematosus, type 1 diabetes mellitus)

Phases of convulsive status epilepticus

Generalized tonic-clonic SE (GTCSE) has 3 phases. In phase 1, discrete partial seizures or, less frequently, generalized seizures can be observed both clinically and on electroencephalography (EEG). Blood pressure usually remains within the reference range, but metabolic acidosis may be observed in association with elevated serum lactate and glucose levels.

In phase 2, discrete SE events fuse and partial seizures become secondarily generalized. The main outward manifestation of continuous clinical and EEG seizure activity consists of a tonic phase (sustained muscle contraction) followed by clonic jerks (alternating contraction and relaxation of the 4 limbs). Phase 2 may include altered blood pressure.

In phase 3, clinical seizures may become quite subtle, with brief rhythmic clonic or myoclonic movements often restricted to a single part of the body. During this period, the patient's EEG findings start to show slow-frequency discharges similar to periodic lateralizing epileptiform discharges (PLEDs). Rhythmic activity may be observed as myoclonus that affects only the feet, hands, facial muscles, or eyes (as nystagmus).

As the episode progresses, a motionless patient's EEG may reveal generalized or PLED-like discharges. This type of activity is thought to represent a burned-out form of SE. This conclusion is supported by cases in which positron emission tomography (PET) scanning revealed hypermetabolism of the mesiotemporal region in patients with abnormal mental status and PLED-like discharges after an episode of SE.

Hyperthermia, respiratory compromise, hypotension, and hypoglycemia may be observed. If not promptly treated, these metabolic, cardiovascular, and respiratory complications can exacerbate the patient's clinical condition and neurologic deficit.

Nonconvulsive status epilepticus

Patients with nonconvulsive SE are described as appearing forgetful and sleepy, behaving as if deaf and blind (“like a zombie”), or having the appearance of being drugged. In more severe cases, patients are described as unresponsive. Sometimes parents describe the motor component of frequent falls, poor motor control, or abnormal balance.

Next:

Physical Examination

Perform a rapid, directed physical and neurologic examination during SE, followed by a detailed examination when the child is stabilized. During the initial physical examination, seek signs of sepsis or meningitis and of head trauma or CNS injury.

Signs of sepsis or meningitis include the following:

  • Temperature above 38.5°C; in patients younger than 2-3 months, above 38.0°C

  • Respiratory distress

  • Cyanosis

  • Poor peripheral perfusion

  • Bulging fontanelles in infant

  • Meningismus (in children >12-18 mo)

  • Presence of petechiae or purpura, herpetic vesicles

When the patient's situation stabilizes, look for lymphadenopathy, which suggests catscratch fever.

Evidence of head or other CNS injury includes the following:

  • Bradycardia, tachypnea, and hypertension (Cushing triad for signs of increased intracranial pressure)

  • Poor pupillary response

  • Asymmetry on neurologic examination

  • Abnormal posturing

  • Gross deformity or soft tissue injury to head

Hallmarks of neurocutaneous syndromes (eg, port wine stain) may also be found.

Patients with GTCSE usually have bilateral and synchronous movements of the extremities. Although asynchronous alternating movements of the extremities are often thought to be caused by pseudoseizures, a similar pattern can be observed in cases of frontal lobe epilepsy. Epilepsia partialis continua manifests by unilateral and, at times, focal (eg, one hand or even one finger) clonic activity (ie, twitching).

Patients with absence SE present with altered consciousness, with or without clonic movements of the eyelids or upper extremities, and automatisms involving the hands and face. A child may sometimes continue to perform a motor act that he or she was engaged in before onset of the absence seizure (eg, bouncing a basketball). In some cases, the patient may answer simple questions, but detailed examination reveals slowed mentation and poor processing of complex information. Episodes of absence SE may last 12 hours or longer.

In patients who present to the emergency department (ED) after an episode of prolonged seizure, carefully observe for signs of subtle seizures or SE, such as clonic or myoclonic rhythmic movements involving the limbs or face and eyes. These movements often are easy to recognize in overt generalized tonic-clonic seizures and in SE. Clonic activity may start focally then spread to the hemibody and finally become generalized. Focal clonic activity may assume the form of rhythmic facial muscle contractions, or it may involve the limbs.

Complications

The most feared complication of GTCSE is brain damage associated with neuronal loss mediated by sustained electrical seizure activity in the brain. Other complications of prolonged seizures may include the following:

  • Aspiration

  • Fluid, electrolyte, and metabolic disturbances

  • Trauma

  • Cardiopulmonary problems

  • Complications as a result of the underlying cause

Fluid, electrolyte, and metabolic complications include lactic acidosis, dehydration, and hypotension. Myoglobinuria caused by muscle breakdown during a seizure may lead to renal dysfunction.

Traumatic complications of SE include oral trauma, both internal (eg, biting the tongue or oral mucosa) and external (eg, hitting the lips). Many patients incur closed head or facial injuries during the clonic phase of seizures. Posterior shoulder dislocation is a classic complication and is difficult to diagnose in the unconscious patient

Pulmonary edema and cardiac arrhythmias may be complications of SE or its treatment.

Disseminated intravascular coagulation in association with significant leukocytosis and mild cerebrospinal fluid pleocytosis may produce a clinical picture similar to sepsis or CNS infection. In these cases, patients are often treated for a severe infection until sepsis or meningitis/encephalitis can be safely ruled out.

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