Imitators of epilepsy Flashcards
Imitators of epilepsy in neonates and infants
- Sandifer syndrome,
- Self-gratification syndrome,
- Benign myoclonus of early infancy,
- Shuddering attacks,
- Startle disease or hyperekplexia,
- Benign neonatal myoclonus,
- Jitteriness, head banging and body
rocking, - Spasmodic torticollis, and
- Apnea.
Imitators of epilepsy in older children
- Breath-holding spells,
- Movement disorders (tics, paroxysmal
kinesigenic choreoathetosis, etc.), - Parasomnias and sleep disorders,
- Migraine headaches,
- Psychogenic nonepileptic seizures,
- Behavioral disorders (rage attacks, inat- tentiveness), and
- Syncope.
Imitators of epilepsy in adolescences and adults
- Nonepileptic psychogenic seizures (also known as pseudoseizures),
- Syncope,
- Panic attacks and hyperventilation,
- Migraines, and
- Parasomnias and sleep disorders (such
as narcolepsy and cataplexy).
Sandifer syndrome
Neonates and infants
This syndrome consists of intermittent abnormal posturing such as stiffen- ing and opisthotonic posturing usually primarily due to gastroesophageal reflux. It is usually associated with feedings. It can be mistaken as paroxysmal dystonia, epileptic spasms, or tonic seizures. This usually improves with antireflux medications.
Self-gratification syndrome
Neonates and infants
This is also referred to as infantile masturbation. This occurs in infants and young children. It involves rubbing of the thighs against each other, thrusting of the pelvis associated with sweating, grunting, or flushing of the face with variable degree of responsiveness. After the event, the child is back to baseline. Treatment involves reassuring the family of its self-limiting and benign nature.
Benign myoclonus of infancy
Neonates and infants
This usually occurs in the first year of life, mostly seen between the age of 3–8 months. It consists of brief tonic or myoclonic contractions involving the axial muscles. These spasms usually occur in cluster and are different from benign neonatal sleep myoclonus. Neurological examination, EEG, and development are all normal. Treatment involves reassurance since myoclonus usually resolves by 2 years of age.
Shuddering attacks
Neonates and infants
These usually occur between the ages of 4–6 months. The events consist of tremor in the head, arms, and trunk with adduction and flexion of the elbows. Com- munication and responsiveness are usually impaired. They can be triggered by emotions such as fear, anger, or frustration. They tend to gradually subside with age and completely resolve by 10 years of age. Reassurance of the family is usually enough.
Hyperekplexia
Neonates and infants
This is also referred as stiff baby syndrome or startle disease. It appears more like an exaggerated startle response and may result in falling. It usually occurs at the age of 6 months to 6 years and peaks at the age of 2– 3 years old. It consists of a triad of generalized stiffness, nocturnal myoclonus, and tonic spasms with auditory or tactile stimuli. In affected neo- nates, tapping the tip of the nose or glabella may elicit a typical response. It is secondary to gene mutations affecting inhibitory glycine receptor (GLRA1 and GLRB). Commonly used treat- ments are clonazepam, followed by valproic acid or levetiracetam.
Breath-holding spells
Older children
Breath-holding spells commonly occur between the ages of 6 and 18 months. Spells are divided as cyanotic or pallid.
Cyanotic breath-holding spells are usually precipitated by an emotional cause such as frus- tration. The child cries and then holds the breath in expiration resulting in cyanosis and may be followed by loss of consciousness and loss of tone. The precipitating event can be from the child being upset. If the apnea is prolonged, clonic jerks may be observed. Treatment is usu- ally reassurance and behavioral modification of parental response. If recurrent, then screening for iron deficiency anemia can be done.
Pallid breath-holding spells usually occur in response to a minor trauma or fright. The child stops breathing, becomes pale, may have a brief cry followed by loss of consciousness. Clonic jerks and incontinence may also occur. Bradycardia or asystole may occur. Main treat- ment is also parental reassurance, with some studies suggesting a role for atropine.
Migraines
Older children, adoescents, adults
Migraines are common causes of recurrent headaches. The dilemma occurs if the neurological event occurs without significant headaches and the event can be mistaken for seizures, such as in the case of confusional migraine or migraine with aura.
For example, confusional migraines are epi- sodes of confusion, agitation/hyperactivity, par- tial or total amnesia, disorientation, lethargy, or vomiting that may last for several minutes or hours. Headache and visual complaints may occur but are not prominent symptoms. These may be mistaken for temporal lobe seizures but other etiologies such as encephalitis, substance abuse, metabolic causes, and vasculitis should be considered.
On the other hand, migraines with aura can appear as focal seizures if the visual disturbances are more prominent than the headache, such as “Alice in wonderland” syndrome where visual micropsia, macropsia, and distortions can all occur. This can be easily mistaken for temporal or occipital lobe seizures.
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Parasomnias and sleep disorders
Older children, adoescents, adults
Some sleep disorders can be mistaken for complex partial seizures, commonly of frontal lobe origin due to their hypermotor features and nocturnal occur- rence. These mostly include night terrors (oc- curring in non-REM sleep) and REM behavior disorder (occurring in REM sleep).
In the case of night terrors, the child wakes up from sleep, appears terrified and agitated, is inconsolable, and has no recollection of the event. They occur around the age of 4 years and typically resolve by 8 years.
While in REM behavior, excessive and vio- lent motor activity with no recollection of events is frequent. Polysomnography (with EEG normal recording) is the gold standard diagnostic tool.
On the other hand, narcolepsy is characterized by a tetrad of cataplexy, sleep paralysis, hypna- gogic hallucinations, and excessive daytime sleepiness. Cataplexy is a sudden loss of muscle tone precipitated by touch, emotional excitement, or laughter. It may occur several times during the day and may often be mistaken for atonic sei- zures. Two tests that are commonly used in diagnosing narcolepsy are the polysomnogram and multiple sleep latency test (MSLT).
Syncope
Older children, adoescents, adults
Syncope is very common and up to 10% of the population will experience it at least one time during their life. It is caused by a transient interruption of cerebral blood flow to the brain resulting in loss of consciousness.
Neurocardiogenic syncope (or vasovagal syncope) is the most common type of syncope. The event is often preceded by prodromal symptoms of feeling of warmth, nausea, blurring or tunnel vision, diaphoresis, and lightheaded- ness. Events are usually provoked by emotions (pain, anxiety, blood drawing) particular situa- tions (crowded environment, hot weather, pro- longed standing, fatigue). It may be caused by decreased blood volume or venous return, or parasympathetic cardioinhibitory response caus- ing vasodepression. Common reflex causes of syncope are coughing, micturition, and swal- lowing. Tilt table tests are occasionally done in recurrent syncopal cases. Diagnosis is made by history, orthostatic measurements, or tilt table testing. Treatment includes reassurance, avoiding precipitating factors, increasing fluid and salt intake. If needed, beta-blockers, alpha-adrenergic agonists, anticholinergics, and mineralocorti- coids (such as fludrocortisone) can be used.
In contrast, cardiogenic syncope is less com- mon but may be more life threatening. In such case, syncopal episodes occur without warning or prodromal symptoms and may occur during physical exertion. Common causes include structural cardiac diseases (such as hypertrophic cardiomyopathy, aortic stenosis), or dysrhyth- mias. An initial cardiology workup including cardiac echocardiography and Holter monitoring is warranted.
Convulsive syncope refers to syncope that is followed by brief tonic or rarely clonic activity, which may occur in prolonged cerebral hypop- erfusional states.
During syncopal episodes, the EEG typically show transient high-voltage delta activity or complete voltage attenuation.
PNES
Older children, adolescent, adults
PNES are by far the most common imitators of epilepsy. It has been reported that 25–40% of all admissions to inpatient video-EEG studies end up with the diagnosis of PNES. However, despite the ability to diagnose PNES with high confi- dence using video-EEG monitoring, the delay in diagnosis is long, averaging about 7–10 years.
The patient’s history may suggest the diag- nosis. Several clues are useful in clinical practice and should raise the suspicion that seizures may be psychogenic rather than epileptic. One of the first clues is resistance or worsening of seizures to antiepileptic drug trials. The presence of prominent comorbid psychiatric conditions should also raise the suspicion of PNES, partic- ularly the presence of depression, anxiety, or PTSD (mainly secondary to physical or sexual abuse). Psychiatric comorbidities are found in the majority of patients with PNES, with depression being the most common.
In addition, several specific triggers that are atypical for epilepsy may suggest the diagnosis of PNES, such as emotional triggers (whether positive or negative stress), headache or pain, specific sounds or lights. Also, certain situations may trigger PNES, for example, the presence of specific audience and occurrence in the physi- cian’s office or waiting room.
A detailed description of events by witnesses is always helpful since specific clinical features have been shown to favor the diagnosis of PNES. Some of these clinical features include eye clo- sure, side-to-side head or body shaking, bilateral asynchronous and often discontinuous move- ments, crying, speech stuttering, and arching of the back. With convulsive attacks, rapid or short postictal recovery, shallow irregular, and soft postictal breathing pattern also favor PNES. Despite the fact that urinary incontinence, tongue biting, bodily injuries can be commonly seen in both epileptic and nonepileptic seizures, serious injuries and tongue biting at the side are more typical for epileptic seizures.
PNES diagnosis requires video-EEG recording of typical events. Withdrawal of antiepileptic drugs is also essential for excluding a concomitant diagnosis of epilepsy, which can be seen in up to 10% of patients suffering from PNES. A formal psychiatric evaluation with additional neuropsy- chological testing is also an integral part of the initial assessment.
Treatment of PNES is targeted toward specific psychiatric conditions and is often very individ- ualized. Therapy typically includes psychother- apy and the use of adjunctive medications to treat coexisting depression or anxiety.
Successful treatment and seizure freedom may be a challenging task, and in several instances hard to reach. Some of the common treatment obstacles include effective delivery of the diag- nosis, acceptance of the diagnosis and patient commitment to therapy and follow-up. In gen- eral, patients who accept their diagnosis and follow through with therapy are more likely to experience a successful outcome.
