Version July 2025
INTRODUCTION —
Epilepsy is a condition characterized by an underlying susceptibility for recurrent seizures. The term "seizure" refers to a transient occurrence of signs and/or symptoms due to abnormal excessive neuronal activity of the brain.
Paroxysmal events are common in infancy. In one population-based cohort, they occurred in 9 percent of those in the first year of life [1]. While seizures and epilepsy are commonly considered in the differential, these make up a small fraction (<10 percent) of these events.
Neonates and infants exhibit nonepileptic paroxysmal episodes that differ from those encountered in older children and adults (table 1). These may be difficult to differentiate from epileptic events because of the overlapping clinical features as well as the difficulty in interpreting neonatal and infant electroencephalograms (EEGs). It is important for clinicians to be aware of and recognize the transient nonepileptic events that resemble seizures to avoid unnecessary treatment and to institute the correct treatment when required.
This topic reviews nonepileptic paroxysmal events in the neonate and infant. The differential diagnosis of epilepsy in other age groups is reviewed separately. (See "Nonepileptic paroxysmal disorders in children" and "Nonepileptic paroxysmal disorders in adolescents and adults".)
DISTINGUISHING NORMAL BEHAVIORS FROM SEIZURES —
Healthy neonates and infants commonly exhibit a variety of paroxysmal movements including nonconjugate eye movements, sucking movements without associated eye abnormalities, and sleep-related myoclonus. (See "Clinical features, evaluation, and diagnosis of neonatal seizures", section on 'Normal newborn behaviors'.)
These normal behaviors as well as the pathologic conditions discussed below can be difficult to distinguish from epileptic seizures, which can also have somewhat subtle manifestations. Note that some of these conditions can persist into childhood.
Clinical features of the paroxysmal events, the setting in which they occur, and the presence or absence of provocation help distinguish these events from epileptic seizures. The history and physical examination should probe for associated features including change in vital signs, color change, autonomic disturbances, loss of consciousness, and head and eye deviation.
EEG, particularly video-EEG monitoring, can be invaluable in distinguishing among these entities. In one study of neonates, 90 percent of abnormal movements suspicious for seizures were found to be nonepileptic on EEG recording [2]. (See "Clinical features, evaluation, and diagnosis of neonatal seizures".)
NEONATES (BIRTH TO ONE MONTH OF AGE)
Apnea in neonates — Apnea is defined as a pause in breathing lasting >20 seconds.
●Apnea of prematurity – In preterm infants, apneic episodes are common during active sleep and are believed to be due to brainstem immaturity. Apnea of prematurity is discussed in detail elsewhere. (See "Pathogenesis, clinical manifestations, and diagnosis of apnea of prematurity" and "Management of apnea of prematurity".)
●Apnea in term neonates – Apnea in the first days of life can be a presenting feature of many conditions, including perinatal events, metabolic derangements, brain abnormalities (eg, stroke, congenital anomalies, trauma, and seizures), congenital or acquired upper airway obstruction, introduction of oral feeds, and congenital central hypoventilation [3].
Breath-holding spells typically present in older infants, but neonatal onset can occur. (See "Breath-holding spells", section on 'Epidemiology'.)
Recurrent unexplained apnea in neonates should prompt video-EEG monitoring (if available) to look for evidence of electrographic seizures. When video-EEG monitoring is not available, serial routine-length EEGs or continuous amplitude-integrated EEGs are alternatives. An epileptic seizure should be suspected if apnea is accompanied by eye closure or opening, eye deviation, mouth movement, hypertension, or tachycardia. However, bedside clinical observation alone is inadequate for accurate neonatal seizure diagnosis. (See "Clinical features, evaluation, and diagnosis of neonatal seizures".)
Jitteriness in neonates
●Clinical features – Jitteriness is a common movement seen in the neonatal period, often within the first days of life, and typically observed as an excessive response to stimulation such as touch or loud noise [4-6]. It has a tremulous quality, with a back-and-forth oscillation of equal amplitude and frequency.
In contrast to seizures, the child is typically awake during the events, and no associated autonomic disturbance is evident. The movement can be lessened by consoling the child, removing the stimulus, or relaxing the affected limb, interventions that do not affect seizure activity. The provocation of events by stimulus is also atypical for seizures. In addition, head or eye deviation during an episode suggests seizure.
●Diagnosis – The diagnosis of jitteriness is based upon the presence of typical clinical features described above in an otherwise healthy infant with a normal neurologic examination. Concern should arise if the jitteriness is associated with altered awareness or is clearly focal or lateralized. If these or any other atypical features are present, an EEG should be considered.
Secondary jitteriness should be suspected in children with a complicated birth or delivery and in those with an abnormal physical examination. In these cases, the jitteriness can be associated with drug withdrawal, hypocalcemia, hypoglycemia, and hypoxic-ischemic encephalopathy, conditions that can also produce seizures; other associated conditions include congenital heart defects and maternal cannabis use [7].
●Prognosis – In most cases, jitteriness in healthy neonates diminishes with the first two weeks of life and resolves before the age of one year [7]. No specific treatment is needed aside from reassurance of the family and caregivers.
In cases of secondary jitteriness, the prognosis is less certain and is likely related to the nature of the underlying disorder [7].
Benign neonatal sleep myoclonus
●Definition and clinical features – Benign neonatal sleep myoclonus (BNSM), also called benign sleep myoclonus of infancy, refers to a phenomenon of repetitive myoclonic jerks that occur primarily during non-rapid eye movement (NREM) sleep but can occur in all stages of sleep, or in the transition from sleep to wakefulness [8-14]. Onset is in the first few days to weeks of life [8,10,14]. The jerks are typically bilateral, symmetric movements of the arms and/or legs.
●Diagnosis and differential diagnosis – The diagnosis of BNSM can often be made based upon a good, informative history and examination, particularly if there are home cell phone or baby monitor video recordings of the events. Features that help distinguish myoclonic jerks of BNSM from epileptic events include [8-11,15]:
•Absence of autonomic disturbances
•Myoclonic jerks occurring only while asleep and disappearing abruptly with arousal
•Normal EEG
•Normal neurologic and developmental examination
Unlike epileptic seizures, the movements of BNSM will cease when the baby is aroused.
Neonates with severe cerebral dysfunction can also have myoclonic jerks, but these typically occur with a stimulus or upon wakening or falling asleep.
Unlike BNSM, the onset of benign myoclonus of infancy occurs beyond the immediate newborn period, and the myoclonus generally occurs during wakefulness [7,16,17]. (See 'Benign myoclonus of infancy' below.)
●Management and prognosis – The condition usually resolves spontaneously by two to three months of age but may persist in a minority until six months to one year of age. No treatment is necessary. Affected children have normal development [14].
Hyperekplexia — Hyperekplexia (stiff baby syndrome or startle disease) is a rare genetic disease that has been associated with pathogenic variants in various genes, often affecting glycine receptors [18-20].
●Pathogenesis and genetics – Both hereditary and sporadic hyperekplexia are genetically heterogeneous. The most common hereditary form (MIM 149400) is caused by variants in the glycine receptor alpha 1 (GLRA1) gene on chromosome 5, which lead to decreased inhibitory glycine signaling and hyperexcitability in motor nerves [21,22]. Both autosomal dominant and recessive forms have been described, and the phenotypic spectrum includes milder as well as later-onset cases [22].
Other hyperekplexia genes include glycine receptor beta (GLRB) [23], ATPase family AAA domain containing 1 (ATAD1) [24], and solute carrier family 6 member 5 (SLC6A5), which encodes a presynaptic glycine transporter [25]. The SLC6A5 variants are predominately associated with recessive hyperekplexia; symptoms include life-threatening neonatal apnea and breath-holding spells [25].
●Clinical features – The disease is characterized by a triad of generalized stiffness (hypertonia ) while awake, nocturnal myoclonus, and an exaggerated startle reflex with eye blinking and flexor spasm of the trunk [20]. These features are often apparent at birth [26]. Episodes of hypertonia or tonic spasms occur upon awakening or with auditory, tactile, or visual stimuli; consciousness is not affected. When severe, these episodes may interfere with breathing and can even cause hypoxic brain injury [27]. Attacks may be resolved by the Vigevano maneuver, which is performed by manual flexion of the neck or hips [28,29]. Tapping the nasal tip, nose bridge, glabella, upper lip, or chin may trigger generalized rigidity without habituation [16,28,30]. All of these features are atypical for epileptic seizures.
These tonic spasms can also cause older infants to fall suddenly in response to surprise, sensory stimuli, strong emotion, or stress [26]. As a result, walking is often delayed.
●Diagnosis – The diagnosis of hyperekplexia is clinical; EEG should be done to exclude seizures but is typically normal [31]. Other laboratory studies are uninformative.
The evaluation for a genetic diagnosis involves a three-generation family history (to identify relatives with hyperekplexia) and molecular genetic testing [30]. Family history is usually positive with hereditary hyperekplexia, but a negative family history does not exclude the hereditary form. The sporadic form is diagnosed if there is no family history, genetic basis, or other neurologic cause of excessive startle [26,32].
Hyperekplexia with an excessive startle response may also be an acquired feature of brainstem dysfunction (eg, pontocerebellar hypoplasia type 2), and several severe neurometabolic and neurodevelopmental disorders such as Tay-Sachs disease and some early-infantile epileptic encephalopathies [30,31]. These conditions should be considered when the evaluation reveals significant developmental delay or unexpected neurologic findings (eg, spasticity, ataxia, hypotonia, dystonia, chorea, visual impairment, or seizures).
●Management and prognosis – For patients with hyperekplexia, we suggest initial treatment with clonazepam, starting at 0.01 to 0.03 mg/kg per day, and titrated to effect up to 0.1 mg/kg per day [20,33,34]. Clonazepam, a gamma-aminobutyric acid (GABA) receptor agonist, appears to be effective for alleviating hypertonia and apneic episodes in small studies of patients with hyperekplexia [19,26,28,35]. Phenobarbital, phenytoin, diazepam, and sodium valproate also have been cited in the literature as effective therapies for hypertonia and/or abnormal startle response [26].
For emergency treatment of attacks, the Vigevano maneuver can be taught to family, caregivers, and health care professionals [29,32].
Startle episodes can result in falls and injuries. Protective gear, including helmets, and supervision of swimming and outdoor activities may be required for affected children and adults [32].
The spasms lessen in severity and usually disappear as the child grows older, but an excessive startle response may persist throughout life, elicited even by minor visual, auditory, or tactile stimuli [32]. Delay in motor milestones (particularly walking) may be present, but cognition and psychomotor development are typically normal.
Severe neonatal episodic laryngospasm (SNEL)
●Pathophysiology and genetics – SNEL is a sodium channelopathy causing episodic laryngospasm, along with face and limb myotonia. It was initially described in three neonates carrying a de novo pathogenic variant in the muscle sodium channel gene SCN4A (sodium voltage-gated channel alpha subunit 4) [36].
●Clinical features – During the neonatal phase, the condition is characterized by episodic, potentially life-threatening attacks of laryngospasm with initial stridor, apnea, cyanosis, generalized stiffness, and bradycardia. Onset is typically in the first days or weeks of life. Most infants have variable feeding difficulty [36-38].
With time, affected infants and children exhibit recurrent attacks of muscle stiffness and develop muscle hypertrophy.
●Diagnosis – Electromyography reveals spontaneous muscle activity with myotonic discharges [36].
The diagnosis is confirmed by detection of an SCNA4 pathogenic variant on genetic analysis.
●Treatment and prognosis – Treatment with sodium channel blockers (eg, carbamazepine 12 to 40 mg/kg per day) has been associated with reducing or eliminating attacks [36-38].
In a review of 16 reported cases, one infant died of respiratory failure at 2.5 months and another of pneumonia at 20 months [38]. Among survivors, the prognosis was generally good, although some patients experienced a delay in developmental milestones.
INFANTS (ONE MONTH TO ONE YEAR OF AGE) —
The differential diagnosis of epileptic seizures in infancy includes events with a broad range of clinical manifestations and underlying pathologies. Although clinical symptomatology helps to differentiate these from epileptic seizures, EEG, particularly video-EEG monitoring, can be invaluable in the diagnosis [39-41]. (See "Seizures and epilepsy in children: Clinical and laboratory diagnosis".)
Apnea in term infants
●Etiologies – In a retrospective review of 101 full-term infants (average age two months) with apnea defined by sleep study, the most common etiologies were gastroesophageal reflux disease (48 percent), upper airway abnormalities or obstruction (37 percent), and neurologic diseases (19 percent), including seizures (5 percent) [42]. Other less common etiologies included genetic disorders, passive smoke exposure, infections, high altitude, cardiac diseases, pulmonary hypertension, lower airway obstruction, hyperbilirubinemia, and intrauterine drug exposure. Breath-holding spells occur only during wakefulness and thus were not evaluated by this study.
●Breath-holding spells – Breath-holding spells are frightening but innocuous events that can be mistaken for epileptic seizures. The first episode usually occurs between 6 and 18 months of age with a range that extends from the neonatal period to six years of age. The two clinical types are cyanotic and pallid. Both types are typically preceded or provoked by an injury or emotional upset that, along with the presence of pallor or cyanosis, helps distinguish them from epileptic seizures. Breath-holding spells are reviewed in detail elsewhere. (See "Breath-holding spells".)
●Brief resolved unexplained event – When apnea occurs in older infants, it may represent a brief resolved unexplained event (BRUE). BRUE is not a specific diagnosis but a description of a sudden, brief, resolved episode characterized by an unexpected change in breathing, appearance, or behavior (ie, cyanosis or pallor; absent, decreased, or irregular breathing; marked change in tone [hypertonia or hypotonia], or an altered level of responsiveness) (table 2).
●Evaluation – For infants with unexplained paroxysmal apnea, evaluation should be guided by specific concerns. Neurologic abnormality on examination or recurrent events suggestive of seizure (eg, apnea accompanied by hypertension, tachycardia, color change, autonomic disturbances, eye closure or opening, eye deviation, mouth movement, and/or loss of consciousness) should prompt video-EEG monitoring (if available) to look for evidence of electrographic seizures. Serial routine-length EEGs or continuous amplitude-integrated EEGs are alternatives.
The history and physical examination of infants who have experienced a BRUE (table 2) should identify features that suggest a specific cause of the event (eg, choking/laryngospasm or an upper respiratory infection) or characteristics that suggest a higher risk for having a serious underlying disease. The evaluation and management of BRUE are reviewed in detail separately. (See "Acute events in infancy including brief resolved unexplained event (BRUE)".)
Jitteriness in infancy — Mild degrees of jitteriness beyond the neonatal period are common in healthy infants [43]. More severe jitteriness can interfere with feeding and other aspects of normal infant care.
Benign myoclonus of infancy — Benign myoclonus of infancy is also known as benign spasms of infancy or benign nonepileptic infantile spasms [44].
●Clinical features – The spasms can manifest as myoclonus, brief tonic contractions, shuddering, and/or atonia (negative myoclonus) [45]. Spasms occur in clusters, frequently at mealtimes.
First occurring at three to eight months of age, these clusters increase in intensity and severity over weeks or months and then remit spontaneously at two to three years of age [46,47].
●Diagnosis and differential diagnosis – Benign myoclonus of infancy can be identified by a normal neurologic examination and normal ictal and interictal EEG recordings. Video-EEG of ictal episodes is the gold standard for distinguishing epileptic from nonepileptic infantile spasms [44].
The clinical features of benign spasms of infancy may be indistinguishable from other types of progressive infantile spasms (eg, infantile epileptic spasms syndrome) associated with developmental delay and epileptic seizures, although these are distinctive in that they typically occur only while awake and are associated with abnormal EEG findings [45,46,48]. (See "Infantile epileptic spasms syndrome: Clinical features and diagnosis", section on 'Electroencephalography'.)
●Treatment and prognosis – No treatment is required. Infants with benign myoclonus of infancy have normal neurologic development without subsequent epilepsy.
Shuddering attacks — Shuddering attacks usually begin in infancy, less commonly in childhood. The pathophysiology is unknown.
●Clinical features – These brief episodes of altered muscle tone often manifest as a rapid tremor of the head, shoulder, and trunk reminiscent of a "shudder" or "shiver" from a chill. The episodes last a few seconds and can occur multiple times a day [49,50]. There may be stiffening, flexion, and elevation of the arms with a low-amplitude tremor [49]. If seated, the child may lean in one direction or even fall. The child may stare, appear to be unaware of the surroundings, and then promptly return to the task at hand [51]. However, affected children are fully conscious during and after an attack, which helps to differentiate shuddering attacks from seizures [49].
The spells often occur with feeding or when the child is excited or distressed, suggesting they are a pattern of stimulus overflow in a young child. They never occur during sleep and virtually never when being held and cuddled.
The episodes may continue at lower frequency into the second half of the first decade and can, on rare occasions, cause the child to fall in the middle of activities such as playing ball.
●Evaluation and diagnosis – The diagnosis of shuddering attacks is based on the clinical history, video review of attacks, and EEG. The family and caregivers should record attacks with video if possible. Evaluation with EEG or video-EEG is generally advocated for patients with suspected shuddering attacks, since they can be confused with seizures and infantile epileptic spasms syndrome [52]. The EEG during the spells is normal or shows only muscle artifact [49,51,53]. Neurologic examination and developmental assessments also are normal.
●Treatment and prognosis – No intervention is required. Education and reassurance of the family and caregivers about the innocuous nature of shuddering attacks is all that is needed. The attacks spontaneously resolve by the second decade without treatment. There is no association with subsequent epilepsy. Occasionally, a family history of benign essential tremor exists, but the children themselves do not have a chronic tremor [53,54].
Sleep-related rhythmic movement disorder — Rhythmic movements such as nocturnal head banging, body rocking, and head rolling typically occur in children younger than one year of age as they try to fall asleep [9,55,56]. They also can be present in deep sleep and in wakefulness. Episodes usually begin in infancy and resolve by five years of age but can persist into adult life. The characteristic nature of the movements is not typical for epilepsy, and patients often can be directed to stop the movements, which is generally not true for an epileptic seizure. (See "Sleep-related movement disorders in childhood", section on 'Rhythmic movement disorder'.)
Sandifer syndrome — Sandifer syndrome refers to the intermittent paroxysmal spells of generalized stiffening and opisthotonic posturing (ie, dystonic posterior arching of the neck and back due to dystonic contraction of midline truncal extensor muscles) that are caused by gastroesophageal reflux in infants [57,58]. These spells may be associated with other abnormal movements [59,60]. Additional manifestations may include seizure-like episodes, ocular symptoms, irritability, growth delay, developmental delay, and iron deficiency anemia. The spells are associated with feedings, usually occurring within 30 minutes following a meal. (See "Acquired torticollis in children", section on 'Sandifer syndrome'.)
If the history suggests Sandifer syndrome, a gastroesophageal work-up is warranted [50,58]. Treatment of gastroesophageal reflux reduces the frequency and severity of attacks. (See "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis" and "Gastroesophageal reflux disease in children and adolescents: Management".)
Dystonic drug reactions — Dystonia is an abnormal posture due to sustained contraction of both the agonist and antagonist muscle groups. A dystonic posture may be generalized or focal; examples include opisthotonic posturing, torticollis, and oculogyric crisis (ie, irregular prolonged deviations of the eyes, usually up and lateral).
A common etiology of dystonia in infants is an acute reaction to a drug such as metoclopramide, phenothiazines, or haloperidol. (See "Hyperkinetic movement disorders in children", section on 'Acute dystonic reaction'.)
Benign paroxysmal torticollis in infancy — Benign paroxysmal torticollis is an idiopathic disorder, characterized by periods of an abnormal, sustained posture of the head and neck in which the head tilts to one side, with the face rotated toward the opposite side. The events begin and end suddenly, with a duration between a few hours and a few days. The child is alert and responsive during an attack. (See "Acquired torticollis in children", section on 'Benign paroxysmal torticollis'.)
Episodes usually first occur in the first three months of life and may recur at varying intervals, resolving by the age of three years in most cases [61-63]. EEG and neurologic examinations are normal, as is subsequent neurologic development; any delays in motor development improve with resolution of episodes [63]. No treatment is required.
A relationship between this disorder and migraine is suggested. A family history of migraines is common in children with this disorder, and they may develop migraine later in life [62,63]. In one family, benign paroxysmal torticollis appeared to be associated with inheritance of a pathogenic variant in the calcium voltage-gated channel subunit alpha1 A (CACNA1A) gene, which is also linked to familial hemiplegic migraine [64].
Abnormal eye movements
●Paroxysmal tonic downgaze of infancy – This rare disorder affects otherwise healthy neonates and infants. It is characterized by sudden sustained downward eye deviation ("setting sun sign") lasting seconds to minutes, associated in some cases with abnormal movements or stiffening of the head and/or limbs, and followed by quick correction to primary gaze [7,65-67]. Onset is typically shortly after birth (preterm infants may be affected) with resolution by six months of age, but onset has been reported up to five months of age [65,68,69].
Although the reported spells have been self-limited, investigations including brain imaging are warranted for neonates and infants with paroxysmal tonic gaze deviations to exclude seizures and brain pathology [65].
●Paroxysmal tonic upgaze of childhood – The onset of this disorder is typically in infancy or early childhood with conjugate tonic upward eye deviation, incomplete downward saccades or down-beating nystagmus with attempted downgaze, and normal horizontal eye movements [66,70]. These episodes are usually associated with neck flexion and a downward chin position. They can last minutes to hours or longer and may occur several times a day. There is no impairment of consciousness. The episodes may be exacerbated by fatigue or illness and alleviated with sleep [70].
Evaluation should include EEG and brain imaging. The EEG is normal during episodes, but affected individuals may have concomitant epileptic seizures [71,72]. Neuroimaging is typically normal, but some cases are associated with various structural brain abnormalities such as hydrocephalus, pinealoma, hypomyelination, and brainstem lesions [66].
Resolution usually occurs between one month and six years of age. While most patients have normal neurodevelopment, permanent sequelae have been reported including ataxia, ocular movement abnormalities, and developmental delay [67].
●Oculomotor apraxia – Oculomotor apraxia is a condition with impaired saccadic eye movements in which the child will turn their head suddenly to move the direction of gaze. These peculiar rapid head movements are rarely mistaken for epileptic seizure. Oculomotor apraxia may be seen in ataxia-telangiectasia and lysosomal diseases. (See "Ataxia-telangiectasia", section on 'Differential diagnosis'.)
●Spasmus nutans – Spasmus nutans consists of a triad of nystagmus, head nodding (titubation), and head tilt. When the symptoms fluctuate during the day, they may be confused with epileptic seizures. Consciousness is not impaired. The onset is usually the first few months of life and resolves by five years of age. (See "Pendular nystagmus", section on 'Spasmus nutans'.)
●Opsoclonus – Opsoclonus consists of rapid, erratic, involuntary conjugate eye movements ("dancing eyes"). These eye movements are usually associated with myoclonus and ataxia, also described as dancing eyes-dancing feet syndrome. Most cases occur between ages 6 and 18 months. Fifty percent of cases are related to an underlying neoplasm, especially neuroblastoma [73,74]. (See "Clinical presentation, diagnosis, and staging evaluation of neuroblastoma" and "Hyperkinetic movement disorders in children", section on 'Myoclonus'.)
SUMMARY AND RECOMMENDATIONS
●Distinction from seizures – The differential diagnosis of epileptic seizures in neonates and infants includes a variety of benign, physiologic phenomena as well as pathologic conditions (table 1). Clinical features of the events, the setting in which they occur, and the presence or absence of provocation help distinguish these events from epileptic seizures. The history and physical examination should probe for associated features including change in vital signs, color change, autonomic disturbances, loss of consciousness, and head and eye deviation. In challenging cases, EEG, particularly video-EEG monitoring, is useful in the diagnostic evaluation. (See "Clinical features, evaluation, and diagnosis of neonatal seizures" and "Seizures and epilepsy in children: Clinical and laboratory diagnosis".)
●Neonates – Healthy neonates commonly exhibit a variety of physiologic paroxysmal movements during both wakefulness and sleep. Pathologic conditions in neonates that can have overlapping features or be difficult to distinguish from epileptic seizures include apnea, jitteriness, benign neonatal sleep myoclonus, and hyperekplexia. Some of these conditions can persist into childhood. (See 'Neonates (birth to one month of age)' above.)
•Apnea in neonates – In preterm infants, apneic episodes are common during active sleep and are believed to be due to brainstem immaturity. (See "Pathogenesis, clinical manifestations, and diagnosis of apnea of prematurity" and "Management of apnea of prematurity".)
In full-term infants, apnea in the first days of life can be a presenting feature of many conditions, including perinatal events, metabolic derangements, brain abnormalities (eg, stroke, congenital anomalies, trauma, and seizures), congenital or acquired upper airway obstruction, introduction of oral feeds, and congenital central hypoventilation. (See 'Apnea in neonates' above.)
•Jitteriness – Jitteriness is a common movement seen in the neonatal period, often in response to stimulation. In contrast to seizures, the child is typically awake during the events, and no associated autonomic disturbance is evident. (See 'Jitteriness in neonates' above.)
•Benign neonatal sleep myoclonus – This condition is characterized by repetitive myoclonic jerks that occur primarily during non-rapid eye movement (NREM) sleep but can occur in all stages of sleep, or in the transition from sleep to wakefulness. The movements cease abruptly with arousal. (See 'Benign neonatal sleep myoclonus' above.)
•Hyperekplexia – Hyperekplexia is a rare genetic disease characterized by a triad of generalized stiffness while awake, nocturnal myoclonus, and an exaggerated startle reflex. Severe episodes can interfere with breathing.
For patients with hyperekplexia who require treatment for hypertonia or tonic spasms, we suggest initial treatment with clonazepam (Grade 2C). Treatment with clonazepam may alleviate hypertonia and apneic episodes. Alternatives are phenobarbital, phenytoin, diazepam, and sodium valproate. (See 'Hyperekplexia' above.)
•Severe neonatal episodic laryngospasm (SNEL) – This disorder is a sodium channelopathy caused by pathogenic variants in the SCN4A gene causing episodic, potentially life-threatening attacks of laryngospasm with stridor, apnea, cyanosis, generalized stiffness, and bradycardia.
For patients with SNEL, we suggest treatment with carbamazepine (Grade 2C), which may reduce or eliminate attacks. (See 'Severe neonatal episodic laryngospasm (SNEL)' above.)
●Infants – The differential diagnosis of epileptic seizures in infancy includes events with a broad range of clinical manifestations. Some of these conditions can persist into childhood. (See 'Infants (one month to one year of age)' above.)
•Apnea in term infants – The most common causes of apnea in full-term infants include gastroesophageal reflux disease, upper airway abnormalities or obstruction, and neurologic diseases including seizures. Breath-holding spells, which occur during wakefulness, are frightening but innocuous events that can be mistaken for epileptic seizures. The first episode may occur as early as the neonatal period but usually occurs between 6 and 18 months of age. The two clinical types are cyanotic and pallid. Both types are typically preceded or provoked by an injury or emotional upset. (See 'Apnea in term infants' above and "Breath-holding spells".)
•Benign myoclonus of infancy – This condition, also known as benign spasms of infancy, involves spasms that can manifest as myoclonus, brief tonic contractions, shuddering, and/or atonia (negative myoclonus). The diagnosis is supported by a normal neurologic examination and normal ictal and interictal EEG recordings. No intervention is required. The spasms remit spontaneously at two to three years of age. Neurologic development is normal without subsequent epilepsy. (See 'Benign myoclonus of infancy' above.)
•Shuddering attacks – Shuddering attacks last a few seconds and often occur with feeding or when the child is excited or distressed, suggesting they are a pattern of stimulus overflow in a young child. (See 'Shuddering attacks' above.)
•Sleep-related disorders – Sleep-related rhythmic movement disorder should be considered when events are confined to sleep or the transitions between wakefulness and sleep. (See 'Sleep-related rhythmic movement disorder' above.)
•Increased muscle tone – Nonepileptic events that involve stiffening or sustained changes in head or limb tone and posture include Sandifer syndrome, which occurs in response to gastroesophageal reflux, and dystonias, which include a variety of conditions that produce paroxysmal eye and/or head movements. (See 'Sandifer syndrome' above and 'Dystonic drug reactions' above and 'Benign paroxysmal torticollis in infancy' above.)
•Disorders causing abnormal eye movements – Abnormal eye movements that may be confused with seizures include paroxysmal tonic downgaze of infancy, paroxysmal tonic upgaze of childhood, oculomotor apraxia, spasms nutans, and opsoclonus. (See 'Abnormal eye movements' above.)
●Older children and adolescents – Separate topics discuss paroxysmal movement disorders in older age groups. (See "Nonepileptic paroxysmal disorders in children" and "Nonepileptic paroxysmal disorders in adolescents and adults".)
