Definition
- Paroxysmal events that appear similar to an epileptic seizure but do not involve abnormal, rhythmic discharges of neurons in the brain.
Types
- Pseudo-seizure
- Psychogenic seizures
- Psychogenic seizures are real events and may not be under voluntary control
Differentiating NES from epileptic seizures
Feature | Epileptic seizure | NES |
% males | 72% | 20% |
Clonic UE movement | - In-phase: 96% - Out-of-phase: 0 | - In-phase: 20% - Out-of-phase: 56% |
Clonic LE movement | - In-phase: 88% - Out-of-phase: 0 | - In-phase: 16% - Out-of-phase: 56% |
Vocalizations | - None: 16% - Start of seizure: 24% - Middle: 60% “epileptic cry” - Types: only sounds of tonic/clonic respiratory muscle contraction | - None: 56% - Start of seizure: 44% - Middle: 0 - Types: moans, screams, grunts, snorts, gagging, retching, understandable statements, gasps |
Head turning | - Unilateral: 64% - Side-to-side: 8% (slow, low amplitude) | - Unilateral: 16% - Side-to-side: 36% (violent, high amplitude) |
Features often associated with NES
- Frequent seizures despite therapeutic AEDs
- Before
- Suggestible or inducible seizures
- Provoked with stimuli that would not cause a seizure (e.g. tuning fork to the head, alcohol pad to the neck, IV saline…)
- Multiple or variable seizure types (ES is usually stereotypical), fluctuating level of consciousness, denial of correlation of seizure with stress
- During
- Arching of the back: 90% specific for NES
- Fluctuating intensity and severity during seizure
- Side-to-side rolling, pelvic thrusting, wild movements
- Bilateral motor activity with preserved consciousness
- Non-physiologic spread of neurologic signs
- Disproportionate postictal mental status changes
- Expression of relief or indifference
- Crying or whimpering
- Asynchronous movement
- Intermittent arrhythmic and out-of-phase convulsive activity
- Stop & go: seizures usually build and then gradually subside
- Forced eye closing during entire seizure
- Weeping (whining): highly specific
- Bilateral shaking with preserved awareness.
- Exception: supplementary motor area seizures (mesial frontal area)—these seizures are usually tonic (not clonic)
- After
- Absence of laboured breathing or drooling after generalized convulsion
- No postictal confusion or lethargy
- Multiple different-physician visits
- Lingering prodrome or gradual ictal onset (over minutes)
- Prolonged seizure duration (> 5 mins)
- Manifestations altered by distraction
Features common to both
- Verbal unresponsiveness,
- Rarity of automatisms
- Whole-body flaccidity
- Rarity of urinary incontinence
More definitive findings
- If any two of the following are demonstrated, 96% of time this will be NES:
- Out-of-phase clonic UE movement
- Out-of-phase clonic LE movement
- No vocalization or vocalization at start of event
- Lateral tongue laceration: very specific for seizures.
Tests
- Minnesota Multiphasic Personality Inventory (MMPI) scales in hypochondriasis, depression hysteria, and schizophrenia
- Chen 2005
- Elevated serum prolactin assay, when measured in the appropriate clinical setting at 10 to 20 minutes after a suspected event, is a useful adjunct for the differentiation of generalized tonic–clonic or complex partial seizure from psychogenic nonepileptic seizure among adults and older children
- Serum prolactin assay does not distinguish epileptic seizures from syncope
- The use of serum PRL assay has not been established in the evaluation of status epilepticus, repetitive seizures, and neonatal seizures
- EEG
- Muscle enzyme studies
Events in children that can stimulate epileptic seizure
Event | Description |
Reflex anoxic seizure (pallid Breath holding spell) | Common in young children, especially under 2 years. Unexpected stimulus (pain, shock, fright) causes excessive vagal activity—heart and respiration stops transiently and child becomes pale |
Cyanotic breath holding spell | Reflex expiration in response to anger/frustration causes child to become cyanotic |
Cardiogenic syncope | Syncope resulting from structural or functional cardiac abnormality—no convulsive movements associated |
Cough syncope | Prolonged cough spasms (e.g. asthmatic, infection) can reduce venous return and lead to syncope with incontinence |
Gastroesophageal reflux | Can lead to paroxysmal dystonic posturing associated with meals due to discomfort |
Narcolepsy | Sudden loss of muscular tone secondary to cataplexy, usually an emotional trigger, no postictal state or loss of consciousness, EEG shows recurrent attacks of REM sleep |
Night terrors | Brief nocturnal episodes of terror without typical convulsive movements, common in ages 4–6 |
Paroxysmal dyskinesias | Precipitated by sudden movement or startle, no associated change in consciousness |
Paroxysmal vertigo | Common in toddlers, triggered by fright/crying—seen to stagger, fall and possibly vomit |
Non-epileptiform attack disorder (pseudoseizure) | No EEG changes except movement artefact during episode |
Rage attacks/Tantrum | Common in children aged 6–12 years, outburst is explosive and out of proportion to trigger (tantrums are goal directed) |
Shuddering attack | Shiver-like movement of the trunk with associated stiffening, neck flexion and arm adduction, each episode lasts seconds and there is no change in consciousness |
Vasovagal syncope (neurally mediated syncope) | Loss of consciousness triggered by postural change, heat or emotion; there is presyncopal dizziness, clouded/tunnel vision before a slow collapse |
Tic | Involuntary, non-rhythmic, repetitive movements not associated with impaired consciousness |
Non-epileptic 'status'
- Many different types
- Lie still
- Prolonged events, waxing and waning
- Thrashing
- Shaking/tremor all over (rarely jerking)
- Back arching
- Drug effect
- Benzodiazepine use may disinhibit!
- Other features
- Resisting eye opening
- Injuries can occur during an attack
- Recovery rapid
- Risk factors
- LD, young/middle aged, prev head injury, female gender, sexual/physical abuse, FHx epilepsy, depression
Non-convulsive status epilepticus (NCSE)
- Nonconvulsive seizures (NCSz)
- Purely electrographic seizures
- Subtle clinical signs:
- Face and limb twitching
- Nystagmus
- Eye deviation
- Pupillary abnormalities
- Autonomic instability
- Negative clinical features
- Hippus can occur in NCSE
Differential diagnosis
Category | Subtypes / Examples |
Psychologic disorders (psychogenic seizures) | - Somatoform disorders: especially conversion disorder - Anxiety disorders: especially panic attacks and PTSD - Dissociative disorders - Psychotic disorders - Impulse control disorders - Attention-deficit disorders¹ - Factitious disorders (including Munchausen's syndrome) |
Cardiovascular disorders | - Syncope - Cardiac arrhythmias - Transient ischemic attacks - Breath-holding spells¹ |
Migraine syndromes | - Complicated migraines¹ - Basilar migraines |
Movement disorders | - Tremors - Dyskinesias - Tics¹, spasms - Other (including shivering) |
Parasomnias & sleep-related | - Night terrors¹, nightmares¹, somnambulism¹ - Narcolepsy, cataplexy - Rapid eye movement behavior disorder - Nocturnal paroxysmal dystonia |
Gastrointestinal disorders | - Episodic nausea or colic¹ - Cyclic vomiting syndrome¹ |
Other | - Malingering - Cognitive disorders with episodic behavioral or speech symptoms - Medication effects or toxicity - Daydreams¹ |
- ¹usually encountered in children
- Psychogenic:
- 20–90% of patients with intractable seizures referred to epilepsy centers.
- These patients carry the diagnosis of seizures from 5–7 years.
- Up to 50% of these may have legitimate seizures at some time as well.
- Tic:
- Can be suppressed, is not repetitive
- If repetitive, may be hemifacial spasm
- Movement disorder: myoclonus (can be epileptic or non-epileptic)
- Cataplexy: sudden muscular weakness triggered by strong emotions such as laughter, anger and surprise.
- e.g. with narcolepsy often provoked by laughter or other emotional stimulus (can rarely be caught on EEG, and when it is, it shows REM intrusion into wakefulness)
- Parasomnia: a group of sleep disorders that involve unwanted events or experiences (abnormal movements, behaviours, emotions, perceptions or dreams) that occur while you are falling asleep, sleeping or waking up.
- Experiences
- Night terrors (occurs in slow wave sleep, vs. nightmare which occurs in REM),
- Sleep walking,
- REM behaviour disorders (usually occurs in older men)
- There is a high probability they will go on to have degenerative brain disease (used to be called paroxysmal nocturnal PNT).
- Syncope:
- 90% of the time people who faint have myoclonic jerks or shaking
- TIA