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Nonepileptic motor
phenomena in the
neonate
RAAFAT SALAMA EGYPTIEN/ARAB BOARDS IN PEDIATRICS
The newborn infant is prone to clinical motor phenomena
that are not epileptic in nature. These include
tremors, jitteriness, various forms of
myoclonus and brainstem release
phenomena, HYPEREKPLEXIA. They are
frequently misdiagnosed as seizures, resulting in
unnecessary investigations and treatment with
anticonvulsants, which have potentially harmful side effects
TREMOR AND JITTERINESS
Tremor can be defined as an involuntary, rhythmical oscillatory
movement of equal amplitude around a fixed axis.
It can be either fine with a high frequency (greater than 6 Hz) and
low amplitude (lower than 3 Hz) or coarse with a low frequency and
higher amplitude
Jitterinessrefers to recurrent tremor
Tremor is the most common abnormal movement encountered in
the neonate. Up to two-thirds of healthy newborns will have some
fine tremor in the first three days of life
reported that up to 44% of newborns were jittery.
Why tremor in neonate considered benign or
pathological in contrast to child or adult which
always pathological?
One theory is that neonatal tremor is due to immaturity of spinal
inhibitory interneurons causing an excessive muscle stretch reflex. As
the neonate gets older and the interneurons mature, the tremor
resolves
Another theory is that elevated levels of circulating catecholamines
account for the tremor
Pathological conditions that may be associated with tremor include
hypoglycemia, hypocalcemia, sepsis, hypoxic ischemic
encephalopathy, intracranial hemorrhage, hypothermia,
hyperthyroid state and drug withdrawal
In general, fine tremor is usually benign or secondary to
metabolic disturbance such as hypoglycemia
Coarse tremor should raise suspicion of intracranial
pathology, such as hypoxic ischemic encephalopathy and
intracranial hemorrhage
Coarse tremor is frequently associated with the ‘neonatal
hyperexcitability syndrome’ in mildly asphyxiated
neonates with increased tendon reflexes and excessive
Moro response
The neurological outcome of neonates with tremor is good
as long as there are no perinatal complications, such as
asphyxia, identified.
Jittery neonates with a history of perinatal complications
were at a 30% risk of adverse neurodevelopmental outcome,
in particular, those with coarse tremor as part of the
‘neonatal hyperexcitability syndrome’
Differentiated tremor from seizure
1-The tremor can be brought on with stimuli
2-Can be stopped with gentle passive flexion and restraint of the
affected limb
4-Is not associated with ocular phenomena, such as forced eye
deviation
5-Not associated with significant autonomic changes such as
hypertension or apnea
3-One can determine whether the tremor is benign by placing the
neonate supine with hands free at his or her side. A benign tremor will
resolve when the neonate is allowed to suck on the examiner’s finger
Investigation of the jittery neonate should depend on the
perinatal history and physical examination
If appears the neonate well and has no history of perinatal
complications, blood glucose measurement alone will suffice
Further investigations should be performed in those neonates
that appear unwell, have coarse tremor, have a history of
perinatal complications and whose tremor does not settle with
soothing or suckling.
Investigations performed ultimately depend on the clinical
situation, but consideration should be given toward doing a sepsis
workup, urine drug screening, neuroimaging, thyroid screening and
metabolic workup
Special consideration should be given to tremor as part of a neonatal
withdrawal syndrome. Excessive tremor and jitteriness have been
reported in newborns of mothers who had been prescribed opiates,
selective serotonin reuptake inhibitors, marijuana, inhaled volatile
substances and cocaine can also cause a withdrawal syndrome in which
coarse tremor is prominent
Interestingly, tremor does not appear to be more common in neonates
of alcohol-or nicotine-dependent mothers
A form of tremor that only involves the perioral muscles is
familial trembling of the chin, which is an autosomal dominant
condition in which the cutaneous muscles of the chin tremble. In
the neonate, it is frequently brought on by crying
Treatment of neonates with tremor and jitteriness should be aimed at
correcting the underlying cause if identified
-who appear unwell or have a history of perinatal complication should be
observed in a neonatal intensive care unit
-Special care must also be paid to mother-newborn bonding because
jittery neonates tend to have decreased visual attention and are more
difficult to console
-Treatment of familial trembling of the chin with botulinum toxin
injections into the perioral muscles is reserved only for cases in which the
trembling causes difficulty eating or drinking, or causes social
embarrassment
MYOCLONUS
Myoclonus is a brief shock-like movement of a limb caused by
muscle contraction. It can be either localized to one body part or
generalized
Unlike tremor, it is irregular and arrhythmic. Myoclonus also tends to
have a higher amplitude than tremor.
Myoclonus has also been reported in premature neonates after
receiving intravenous benzodiazepines
Nonepileptic pathological myoclonus most likely represents
brainstem release phenomena, in which cortical inhibition of
normally suppressed brainstem activity is lost due to diffuse cerebral
injury
Benign neonatal sleep myoclonusis characterized by
rhythmical myoclonic jerks seen only during sleep. It is common and
frequently misdiagnosed as seizures.
-It tends to occur in healthy, full-term newborns. While it can be seen
in any stage of sleep, it tends to occur predominantly in quiet sleep
-Unlike sleep myoclonus in adults, which is usually an asymmetric
single jerk, benign neonatal sleep myoclonus is bilateral and
repetitive
-Onset is usually in the first few days of life and usually remits
spontaneously by four months of age
Unlike tremor and jitteriness, gentle restraint
may worsen the myoclonus. Because
myoclonus can last up to 1 h, it can be
mistaken for status epilepticus, leading to
treatment with anticonvulsants, which provide
no benefit and often worsen the myoclonus
In Benign neonatal sleep myoclonus
Benign myoclonus of early infancy, which has
usual onset between three to nine months, has also been reported
in the neonatal period
In this condition, the infant will have recurrent clusters of
myoclonic jerks when awake.
The EEG is normal even during events. Resolution is by nine
months of age
NEONATAL HYPEREKPLEXIA
Generalized muscle rigidity in the neonate, nocturnal myoclonus and
an exaggerated startle reaction to auditory, tactile and visual stimuli.
The startle reaction is a normal response to stimuli that consists of
facial grimace and blinking followed by flexion of the trunk.
The startle response is exaggerated when it interferes with normal
activities, and causes apnea and frequent falls
There are two forms of hyperekplexia
The minor form has an exaggerated startle response
only(generalized tonic spasm with tonic flexion of the limbs and
trunk and clenching of the fists. The eyes often remain open in
an anxious stare) Apnea is common during the spasms
The major form, there is also a generalized muscle rigidity seen
only when the infant is awake in addition to nocturnal myoclonus
The exaggerated startle response can be
elicited by tapping the nasal bridge,
thus differentiating it from seizures for
which it is often mistaken
CONCLUSION
1.Differentiating epileptic seizures from nonepileptic motor phenomena
is extremely important
2.While neonatal seizures are usually a sign of serious intracranial
pathology, nonepileptic motor events may be benign. If they are
pathological, the underlying cause may be different from those that
cause seizures, requiring a different treatment approach.
3.The standard anticonvulsants used in neonates have potentially
harmful side effects
4.Anticonvulsants should only be used in the neonate
when the likelihood that the events are truly epileptic is
high
Thank you

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Benign abnormal movements in neonate

  • 1. Nonepileptic motor phenomena in the neonate RAAFAT SALAMA EGYPTIEN/ARAB BOARDS IN PEDIATRICS
  • 2. The newborn infant is prone to clinical motor phenomena that are not epileptic in nature. These include tremors, jitteriness, various forms of myoclonus and brainstem release phenomena, HYPEREKPLEXIA. They are frequently misdiagnosed as seizures, resulting in unnecessary investigations and treatment with anticonvulsants, which have potentially harmful side effects
  • 4. Tremor can be defined as an involuntary, rhythmical oscillatory movement of equal amplitude around a fixed axis. It can be either fine with a high frequency (greater than 6 Hz) and low amplitude (lower than 3 Hz) or coarse with a low frequency and higher amplitude Jitterinessrefers to recurrent tremor Tremor is the most common abnormal movement encountered in the neonate. Up to two-thirds of healthy newborns will have some fine tremor in the first three days of life reported that up to 44% of newborns were jittery.
  • 5. Why tremor in neonate considered benign or pathological in contrast to child or adult which always pathological? One theory is that neonatal tremor is due to immaturity of spinal inhibitory interneurons causing an excessive muscle stretch reflex. As the neonate gets older and the interneurons mature, the tremor resolves Another theory is that elevated levels of circulating catecholamines account for the tremor Pathological conditions that may be associated with tremor include hypoglycemia, hypocalcemia, sepsis, hypoxic ischemic encephalopathy, intracranial hemorrhage, hypothermia, hyperthyroid state and drug withdrawal
  • 6. In general, fine tremor is usually benign or secondary to metabolic disturbance such as hypoglycemia Coarse tremor should raise suspicion of intracranial pathology, such as hypoxic ischemic encephalopathy and intracranial hemorrhage Coarse tremor is frequently associated with the ‘neonatal hyperexcitability syndrome’ in mildly asphyxiated neonates with increased tendon reflexes and excessive Moro response
  • 7. The neurological outcome of neonates with tremor is good as long as there are no perinatal complications, such as asphyxia, identified. Jittery neonates with a history of perinatal complications were at a 30% risk of adverse neurodevelopmental outcome, in particular, those with coarse tremor as part of the ‘neonatal hyperexcitability syndrome’
  • 8. Differentiated tremor from seizure 1-The tremor can be brought on with stimuli 2-Can be stopped with gentle passive flexion and restraint of the affected limb 4-Is not associated with ocular phenomena, such as forced eye deviation 5-Not associated with significant autonomic changes such as hypertension or apnea 3-One can determine whether the tremor is benign by placing the neonate supine with hands free at his or her side. A benign tremor will resolve when the neonate is allowed to suck on the examiner’s finger
  • 9. Investigation of the jittery neonate should depend on the perinatal history and physical examination If appears the neonate well and has no history of perinatal complications, blood glucose measurement alone will suffice Further investigations should be performed in those neonates that appear unwell, have coarse tremor, have a history of perinatal complications and whose tremor does not settle with soothing or suckling. Investigations performed ultimately depend on the clinical situation, but consideration should be given toward doing a sepsis workup, urine drug screening, neuroimaging, thyroid screening and metabolic workup
  • 10. Special consideration should be given to tremor as part of a neonatal withdrawal syndrome. Excessive tremor and jitteriness have been reported in newborns of mothers who had been prescribed opiates, selective serotonin reuptake inhibitors, marijuana, inhaled volatile substances and cocaine can also cause a withdrawal syndrome in which coarse tremor is prominent Interestingly, tremor does not appear to be more common in neonates of alcohol-or nicotine-dependent mothers A form of tremor that only involves the perioral muscles is familial trembling of the chin, which is an autosomal dominant condition in which the cutaneous muscles of the chin tremble. In the neonate, it is frequently brought on by crying
  • 11. Treatment of neonates with tremor and jitteriness should be aimed at correcting the underlying cause if identified -who appear unwell or have a history of perinatal complication should be observed in a neonatal intensive care unit -Special care must also be paid to mother-newborn bonding because jittery neonates tend to have decreased visual attention and are more difficult to console -Treatment of familial trembling of the chin with botulinum toxin injections into the perioral muscles is reserved only for cases in which the trembling causes difficulty eating or drinking, or causes social embarrassment
  • 13. Myoclonus is a brief shock-like movement of a limb caused by muscle contraction. It can be either localized to one body part or generalized Unlike tremor, it is irregular and arrhythmic. Myoclonus also tends to have a higher amplitude than tremor.
  • 14. Myoclonus has also been reported in premature neonates after receiving intravenous benzodiazepines Nonepileptic pathological myoclonus most likely represents brainstem release phenomena, in which cortical inhibition of normally suppressed brainstem activity is lost due to diffuse cerebral injury
  • 15. Benign neonatal sleep myoclonusis characterized by rhythmical myoclonic jerks seen only during sleep. It is common and frequently misdiagnosed as seizures. -It tends to occur in healthy, full-term newborns. While it can be seen in any stage of sleep, it tends to occur predominantly in quiet sleep -Unlike sleep myoclonus in adults, which is usually an asymmetric single jerk, benign neonatal sleep myoclonus is bilateral and repetitive -Onset is usually in the first few days of life and usually remits spontaneously by four months of age
  • 16. Unlike tremor and jitteriness, gentle restraint may worsen the myoclonus. Because myoclonus can last up to 1 h, it can be mistaken for status epilepticus, leading to treatment with anticonvulsants, which provide no benefit and often worsen the myoclonus In Benign neonatal sleep myoclonus
  • 17. Benign myoclonus of early infancy, which has usual onset between three to nine months, has also been reported in the neonatal period In this condition, the infant will have recurrent clusters of myoclonic jerks when awake. The EEG is normal even during events. Resolution is by nine months of age
  • 19. Generalized muscle rigidity in the neonate, nocturnal myoclonus and an exaggerated startle reaction to auditory, tactile and visual stimuli. The startle reaction is a normal response to stimuli that consists of facial grimace and blinking followed by flexion of the trunk. The startle response is exaggerated when it interferes with normal activities, and causes apnea and frequent falls There are two forms of hyperekplexia The minor form has an exaggerated startle response only(generalized tonic spasm with tonic flexion of the limbs and trunk and clenching of the fists. The eyes often remain open in an anxious stare) Apnea is common during the spasms
  • 20. The major form, there is also a generalized muscle rigidity seen only when the infant is awake in addition to nocturnal myoclonus The exaggerated startle response can be elicited by tapping the nasal bridge, thus differentiating it from seizures for which it is often mistaken
  • 21. CONCLUSION 1.Differentiating epileptic seizures from nonepileptic motor phenomena is extremely important 2.While neonatal seizures are usually a sign of serious intracranial pathology, nonepileptic motor events may be benign. If they are pathological, the underlying cause may be different from those that cause seizures, requiring a different treatment approach. 3.The standard anticonvulsants used in neonates have potentially harmful side effects 4.Anticonvulsants should only be used in the neonate when the likelihood that the events are truly epileptic is high