3. • The misdiagnosis of epilepsy is estimated as high as 5 to 40 %
• Often all that is needed to differentiate non-epilectic paroxysmal
disorders from epilepsy is careful and detailed history in addition
to a thorough clinical examination, but sometimes an
electroencephalogram (EEG) or more advanced testing may be
necessary
4. Non-epileptic paroxysmal disorders can be classified according to
the age at presentation and the clinical manifestations
1. Syncope and other generalized paroxysms
2. Movement disorders and other paroxysmal movements and
postures
3. Oculomotor and visual abnormalities and visual hallucinations
4. Sleep related disorders
5. SYNCOPE AND OTHER GENERALIZED
PAROXYSMS
• Apnea, apneic episodes (cessation of breathing > 20 sec) in
neonates and apnea due to brainstem compression are usually
associated with bradycardia, while in seizures is usually
accompanied by tachycardia exceptions are there
• Sleep apnea can either be central (most commonly in premature
neonates) or obstructive.
• Apnea, can also be secondary to cerebral herniation and
brainstem compression due to increased ICP.
6. • Breath holding spell
• A breath-holding spell can be a frightening experience for parents because the infant
becomes lifeless and unresponsive .
• There are two major types of breath-holding spells: the more common cyanotic
form and t he pallid form.
• Cyanotic Spells: The episode is heralded by a brief, shrill cry followed by forced
expiration and apnea.
• There is a rapid onset of generalized cyanosis and a loss of consciousness that may
be associated with repeated generalized clonic jerks, opisthotonus, and bradycardia.
• Normal EEG.
• Rare before 6 months of age.
• They peak at about 2 yr of age, and they abate by 5 yr of age.
• The management is support and reassurance of the parents.
7. Pallid Spells:
• Less common than breath-holding spells.
• Initiated by a painful experience such as falling and striking the head, the child
stops breathing, rapidly loses consciousness, becomes pale and hypotonic.
• Bradycardia may occur.
• Management by conservative measures and atropine sulfate in some cases.
• Prolonged QT Syndrome:
• It is characterized by sudden loss of consciousness during exercise or an emotional
and stressful experience.
• During the period of syncope, various cardiac arrhythmias are evident.
• The child may recover within minutes or die during the event.
• Prolonged QT more than 0.46 msec support the diagnosis.
• B-Adrenergic- antagonist drugs are usually effective and may be lifesaving.
8. Psychological disorders (Pseudoseizures)
• Occur typically between 10 and 18 yr of age and are more frequent among
girls.
• Occur with patients with a past history of epilepsy.
• There are several distinguishing features of pseudoseizure, including lack of
cyanosis, normal reaction of the pupil to light, no loss of sphincter control,
normal plantar responses, and absence of tongue biting.
• The most reliable method of differentiation epilepsy from suspected
pseudoseizures is to record an attack.
• The EEG shows an excess of muscle artifact during the pseudoseizure.
• After a true epileptic seizure, there is a significant increase in serum
prolactin whereas pseudoseizure not.
9. Movement disorder and other paroxysmal
movements and postures
• Neonatal Jitteriness and clonus: Jitteriness consists of recurrent tremors.
Equal backward and forward movements of the limbs, spontaneous, or
triggered by touch or loud sounds.
• Movements suppression by stimulus removal or by relaxing the affected
limbs, the lack of autonomic symptoms and the clear difference from the two
faced (fast contraction, slow relaxation) clonic activity and the very quick
myoclonic jerks, point to a nonepileptic event
• Hypocalcemia, hypoglycemia, drug withdrawal, and hypoxic-ischecmic
encephalopathy or possible etiologies.
10. • Hyperekplexia (stiff baby syndrome) and pathologic startles
• Rare, sporadic or dominantly inherited disorder with neonatal
onset of life threatening episodes of tonic stiffening leads to apnea
and convulsive hypoxic seizure, characterized by a triad of
generalized stiffness, nocturnal myoclonus and startle reflex
• Stiffness in the neonatal form improves by one year of age and
may disappear during sleep
• Specific diagnostic sign can be elicited by tapping the nose, which
produces a nonfatigable startle reflex with head retraction.
• Treatment of choice clonazepam
11. • Alternating hemiplegia of childhood
• This is a rare, often severe, disorder that consists of flaccid
hemiplegia affecting one or both sides lasting minutes to days,
starting in the first 18 months of life.
• Earlier manifestations include paroxysmal nystagmus, which is
monocular and ipsilateral to the hemiplegia or dystonia
• Most effected children also have ataxia and developmental delay,
choreoathetosis and behavioral problems
• Flunarizine 2.5-20 mg/day reduces the frequency and severity of
the attacks
12. • Motor tics
• Movements that are under partial control and are associated with
an urge to do them and with subsequent relief.
• Simple tics, occur at some time in about one in five children,
involve in one or two muscle groups
• Complex tics, involve multiple tics or muscle groups
• Tourette syndrome, consists of multiple motor tics and vocal tics
for more than a year, there is often a family history of tics and/or
obsessive-compulsive disorder or personality trait
13. • Episodic ataxias
• Form a clinically and genetically heterogeneous group of diseases that
manifest with recurrent truncal ataxia and incoordination of the eight
syndromes described so far, only two (types 1 and 2 ) have been reported in a
large number of families from different ethnic groups.
• Type 1, caused by mutations in the voltage-gated potassium channel. It
consists of brief episodes (sec to min) of cerebellar ataxia and partial seizures
• Type 2, characterized by longer attacks (min-hours) and interictal cerebellar
signs. Its caused by mutation in the voltage gated calcium channel gene. This
type is more responsive than type-1 to acetazolamide; the drug can reduce
frequency and severity of attacks
14. • Psychological disorders; many psychological disorders can be
mistaken for epileptic seizures
• Stereotypies repetitive movements that are more complex then
tics and do not changed and wax and wane as do tics (head-
banging, head-rolling, body rocking and hand flapping) usually
occur in neurological impaired children
• Hyperventilation spells; can be precipitated by anxiety and are
associated with dizziness, tingling, and, at times, carpopedal
spasm
• Transient global amnesia; consists of isolated short term memory
loss for minutes to hours have been reported in children
•
15. Oculomotor And Visual Abnormalities
• Spasmus Nutans ; triade of nystagmus head tilt and head nodding. Brain
MRI should be performed because the triade has been associated with masses
in the optic chiasm and third ventricle.
• In the absence of these associations remission occurs before 5 years of age.
• Opsoclonus-myoclonus syndrome; dancing eyes with continuous, random,
irregular, and conjugate eye movements that may fluctuate intensity
• Neuroblastoma (more commonly ), encephalitis and post infectious etiology
are possible causes and treated with clonazepam, corticosteroids, rituximab.
16. • Day dreaming and behavioral staring
• It is because of fatigue and inattention a differentiating point
from absence seizures, this is common in children with
attention deficit disorder
• Hyperventilation in the office precipitates absence seizure and
is useful clinical test
17. Sleep Related Disorders
• Paroxysmal nonepileptic sleep events are more common in
epileptic patients than in general population which makes there
diagnosis more difficult.
• Benign sleep myoclonus and neonatal sleep myoclonus
• Consists of repetitive, usually bilateral, rhythmic jerks involving
the upper and lower limbs during non rapid eye movement sleep,
sometimes mimicking clonic seizure.
• Remission is spontaneous usually at 2 to 3 months of age
18. • Sleep transition disorder
• Nocturnal head banging ( jactatio capitis nocturnal ), rolling, repetitive
limb movements, or body rocking occur in infants and toddlers as they
are trying to fall asleep and can be mistaken for seizure or spasms. They
usually remit spontaneously by 5 years of age, no specific therapy is
needed
• Narcolepsy-cataplexy
• Narcolepsy is characterized by excessive day time sleepiness, cataplexy,
sleep paralysis, hypnogogic hallucinations and disturb night time sleep
• Therapy relies on scheduled naps; medications such as amphetamines,
tricyclic antidepressants and counseling about precautions in work and
driving.