VIP Service Call Girls Sindhi Colony š³ 7877925207 For 18+ VIP Call Girl At Th...
Ā
Infantile Spasms.ppt
1. WEST SYNDROME &
INFANTILE SPASMS
MITCHELL P. CREED, MD, MA
PEDIATRIC NEUROLOGY
STONY BROOK UNIVERSITY CHILDRENāS HOSPITAL
2. INFANTILE SPASMS
ā¢ Represents 2% of all epilepsies.
ā¢ 25 % of all that present in the first year of life
ā¢ 90% start before the age of 12 months.
ā¢ Peak at 4-6 months.
3. WEST SYNDROME
Triad of
ā¢ infantile spasms,
ā¢ an interictal EEG pattern termed
hypsarrhythmia,
ā¢ Development delay or regression
4. DO YOU KNOW WHO DESCRIBED FOR
THE FIRST TIME?
ā¢ West described the events in 1841as ābobbingsā that
ācause a complete heaving of the head forward towards
his knees, and then immediately relaxing into the
upright position ā¦
5. DO YOU KNOW WHO DESCRIBED FOR
THE FIRST TIME?
ā¢ ā¦ these bowings and relaxings would be repeated
alternately at intervals of a few seconds, and repeated
from 10 to 20 or more times at each attack, which attack
would not continue more than 2 or 3 minutes; he
sometimes has 2, 3 or more attacks in the day.ā
6. INFANTILE SPASMS
ā¢ Spasms begin with a sudden, rapid, tonic
contraction of trunk and limb musculature that
gradually relaxes over 0.5-2 seconds.
ā¢ Contractions can last 5-10 seconds.
ā¢ The intensity may vary from a subtle head
nodding to a powerful contraction of the body.
8. INFANTILE SPASMS
ā¢ Infantile spasms usually occur in clusters,
often several dozens, separated by 5-30
seconds.
ā¢ Spasms frequently occur just before sleep or
upon awakening. They can be observed during
sleep, although this is rare.
9. INFANTILE SPASMS
ā¢ Spasms, the seizure type, may have
variable features
ā¢ Three subtypes (flexor, extensor, and
mixed flexor-extensor) based on postural
manifestations and patterns of muscle
involvement during the seizure.
ā¢ Flexor spasms involve flexion of the neck,
trunk, and extremities, resulting in jack-
knifing at the waist and a self-hugging
motion of the arms..
10. INFANTILE SPASMS
ā¢ Extensor spasms consist of extension of the
neck, trunk, and extremities. Mixed flexor-
extensor spasms involve combinations of the
above.
13. FAMILY HISTORY
ā¢ Only in 17 % of patients
ā¢ 10% have Hx of febrile Seizure
ā¢ Incidence 1.6-4.5/10,000 live births (2000-
2500 new cases/year in US)
19. ETIOLOGY
ā¢ Infantile spasms are a SYMPTOM, not an underlying
condition
ā¢ Any disorder that can produce brain damage can
be associated with infantile spasms.
ā¢ Prenatal
ā¢ Perinatal.
ā¢ Postnatal.
23. INFANTILE SPASMS
ā¢ Pt must be evaluated for
Tuberous sclerosis
ā¢ Manifestation of TS are:
ā¢ Cardiac tumors
ā¢ kidney tumors
ā¢ cutaneous malformations
such as ash-leaf
hypopigmented lesions
ā¢ seizures
24. INFANTILE SPASMS
Cryptogenic
ā¢ Patients have cryptogenic infantile spasms if
developmental delay and no cause is identified
but a cause is suspected and the epilepsy is
presumed to be symptomatic.
ā¢ Account for 8-42% of the cases (wide range)
25. CLASSIFICATION
Idiopathic
ā¢ Normal psychomotor development prior to the onset of
symptoms
ā¢ Does NOT meet West Syndrome criteria
ā¢ No underlying disorders or definite causes are present
ā¢ No neurological or neuroradiological abnormalities .
26. DIFFERENTIAL DIAGNOSIS
ā¢ Benign myoclonus in infancy,
which consists of clusters of nonepileptic spasms and a
normal EEG, occurs in infants with normal psychomotor
development.
ā¢ Hyperplexia,
a startle jerk, is triggered by touching the nose and eventually
the upper limbs.
ā¢ Tonic seizures
ā¢ Shuddering
ā¢ Sandifer syndrome,
due to GE reflux, may be difficult to detect and mimic
infantile spasms.
27. DIFFERENTIAL DIAGNOSIS
ā¢ Early breath-holding spells and aversive reactions
to stimuli can result in dystonic postures or jerks.
ā¢ Jactatio capitis,
or head banging, occurs in older infants on falling
asleep.
ā¢ Spasmus nutans associated with neck tilt and
nystagmus.
ā¢ Moro reflex must be distinguished from IS
28. LAB STUDIES
ā¢ CBC diff , LFTs , renal panel with electrolytes
and glucose, calcium, magnesium,
phosphorus, and urinalysis with microscopic
examination
ā¢ Metabolic workup including glucose, liver
panel, serum lactate and pyruvate, plasma
ammonia, serum and urine amino acids, urine
organic acids, and serum biotinidase
29. LAB STUDIES
ā¢ Blood, urine, and cerebrospinal fluid cultures if
an infection is suspected
ā¢ Cerebrospinal fluid analysis for cell count,
glucose, protein, bacterial and viral culture,
lactate, pyruvate, and amino acids
30. NEUROIMAGING
ā¢ 70-80% of patients have abnormal findings on
neuroimaging studies.
ā¢ MRI more sensitive than CT scan of the brain.
ā¢ Imaging studies should be obtained prior to
starting ACTH or steroid therapy, as these
therapies are associated with the appearance
of apparent brain atrophy as treatment
continues.
37. ICTAL EEG
ā¢ High-voltage, frontal dominant, generalized
slow-wave transient followed by voltage
attenuation, also termed an
electrodecremental episode
38. WHAT IS HYPSARRHYTMIA?
ā¢ Chaotic, high- to extremely high-voltage
polymorphic delta and theta rhythms with
ā¢ Superimposed multifocal spikes and wave
discharges
39.
40.
41. HYPSARRHYTHMIA
ā¢ At onset usually only during drowsiness and
light sleep.
ā¢ EEG may be normal (modified hypsarrhythmia).
43. GOAL OF TREATMENT
ā¢ Cessation of spasms & resolution of
hypsarrhythmia in 2-4 weeks
ā¢ Quality of life with no seizures
ā¢ Fewest adverse effects from treatment
ā¢ The least number of medications.
46. TREATMENT
ā¢ Focal cortical resection
ā¢ In some patients, resection
of a localized region can
lead to freedom from
seizures.
ā¢ Ketogenic Diet
47. COMPLICATIONS OF TX
ā¢ Hypertension, metabolic abnormalities, severe
irritability, osteoporosis, sepsis, and
congestive heart failure
48. PROGNOSIS
ā¢ Only 14% have normal or borderline normal
cognitive development
ā¢ Drastically improves if*:
ā¢ Treatment started within 1 month
ā¢ Developmentally normal at onset (idiopathic)
ā¢ No cause found (cryptogenic/idiopathic)
ā¢ Some associations may have better outcome,
such as Down Syndrome, NF.
*Kivity S et al. Epilepsia 2004;
