Infantile spasmsInfantile spasms

Dr/Nabil El-mansouryDr/Nabil El-mansoury

DefinitionDefinition West syndrome is composed of the triad of West syndrome is composed of the triad of

infantile spasms, an interictal EEG pattern infantile spasms, an interictal EEG pattern termed hypsarrhythmia, and mental termed hypsarrhythmia, and mental retardation, although the diagnosis can be retardation, although the diagnosis can be made even if one of the 3 elements is missing made even if one of the 3 elements is missing (according to the international classification).(according to the international classification).

This severe epilepsy syndrome is an age-This severe epilepsy syndrome is an age-dependent expression of a damaged brain. dependent expression of a damaged brain. The term infantile spasms has been used to The term infantile spasms has been used to describe the seizure type, the epilepsy describe the seizure type, the epilepsy syndrome, or both syndrome, or both

the the syndrome’s namesake, Dr W J West, gave the first

detailed description of infantile spasms, as they

occurred in his child.[ in a letter to the editor of The

Lancet in 1841,dr West described the events as

“bobbings” that “cause a complete heaving of the

head forward towards his knees, and then

immediately relaxing into the upright position …

these bowings and relaxings would be repeated at

intervals of a few seconds, and repeated from 10 to

20 or more times at each attack, which would not

continue more than 2 or 3 minutes; he sometimes has

2, 3 r more attacks in the day.

This detailed clinical description was This detailed clinical description was followed 100 years later by the report of followed 100 years later by the report of the typical interictal EEG pattern termed the typical interictal EEG pattern termed hypsarrhythmia. Most patients with hypsarrhythmia. Most patients with infantile spasms have some degree of infantile spasms have some degree of developmentaldevelopmental

retardationretardation.The West syndrome was created in the

early 1960by Drs. Gastaut, Poirier, and Pampiglione.

IntroductionIntroduction

WestWest’’s syndrome is a unique, age-s syndrome is a unique, age-specific epilepsy of early infancy. specific epilepsy of early infancy.

Spasms are:Spasms are: Different from myoclonic and tonic Different from myoclonic and tonic

seizuresseizures Characterized by an initial contraction Characterized by an initial contraction

phase followed by a more sustained phase followed by a more sustained tonic phasetonic phase

Divided into flexor, extensor, and mixed Divided into flexor, extensor, and mixed flexor-extensor spasms and they can also flexor-extensor spasms and they can also be asymmetricalbe asymmetrical

Infantile spasms are believed to reflect abnormal Infantile spasms are believed to reflect abnormal interactions between the cortex and brainstem interactions between the cortex and brainstem structures. Focal lesions early in life may structures. Focal lesions early in life may secondarily affect other sites in the brain, and secondarily affect other sites in the brain, and hypsarrhythmia may represent this abnormal hypsarrhythmia may represent this abnormal activity arising from multiple brain sites. activity arising from multiple brain sites.

The frequent onset of infantile spasms in infancy The frequent onset of infantile spasms in infancy suggests that an immature central nervous system suggests that an immature central nervous system may be important in the pathogenesis. The brain-may be important in the pathogenesis. The brain-adrenal axis also may be involved.adrenal axis also may be involved.

One theory states that the effect of different One theory states that the effect of different

stressors in the immature brain produces an stressors in the immature brain produces an abnormal excessive secretion of corticotropin-abnormal excessive secretion of corticotropin-releasing hormone, causing spasms. releasing hormone, causing spasms.

IncidinceIncidince

Incidence of infantile spasms is estimated Incidence of infantile spasms is estimated between 0.25 to 0.60 per 1000 live births.between 0.25 to 0.60 per 1000 live births.

Prevalence rate is 0.15-0.2 per 1000 children Prevalence rate is 0.15-0.2 per 1000 children ten years of age or younger.ten years of age or younger.

United StatesUnited States Infantile spasm constitutes 2% of childhood Infantile spasm constitutes 2% of childhood

epilepsies but 25% of epilepsy with onset in epilepsies but 25% of epilepsy with onset in the first year of life. the first year of life.

SexSexAlthough males are affected slightly Although males are affected slightly more often than females, no significant more often than females, no significant gender difference is notedgender difference is noted..

AgeAge90% of infantile spasms begin in those 90% of infantile spasms begin in those younger than 12 months. Peak onset is younger than 12 months. Peak onset is at age 4-6 monthsat age 4-6 months..

ManifestationsManifestations Spasms begin with a sudden, rapid, tonic Spasms begin with a sudden, rapid, tonic

contraction of trunk and limb musculature contraction of trunk and limb musculature that gradually relaxes over 0.5-2 seconds.that gradually relaxes over 0.5-2 seconds. Contractions can last 5-10 seconds.Contractions can last 5-10 seconds. The intensity may vary from a subtle head The intensity may vary from a subtle head

nodding to a powerful contraction of the body.nodding to a powerful contraction of the body. Infantile spasms usually occur in clusters, Infantile spasms usually occur in clusters,

often several dozens, separated by 5-30 often several dozens, separated by 5-30 seconds.seconds.

Spasms frequently occur just before sleep or Spasms frequently occur just before sleep or upon awakening. They can be observed during upon awakening. They can be observed during sleep, although this is rare.sleep, although this is rare.

Spasms can be flexor, extensor, or a mixture Spasms can be flexor, extensor, or a mixture of flexion and extension.of flexion and extension.

Flexor spasms consist of brief contractions of Flexor spasms consist of brief contractions of the flexor muscles of the neck, trunks, and the flexor muscles of the neck, trunks, and limbs, resulting in a brief jerk. They may limbs, resulting in a brief jerk. They may resemble a self-hugging motion and often are resemble a self-hugging motion and often are associated with a cry. The patient then associated with a cry. The patient then relaxes, and the jerk repeats. These attacks relaxes, and the jerk repeats. These attacks occur in clusters throughout the day and last occur in clusters throughout the day and last anywhere from less than 1 minute to 10-15 anywhere from less than 1 minute to 10-15 minutes or longer in some patients.minutes or longer in some patients.

-Extensor spasms consist of contractions of -Extensor spasms consist of contractions of the extensor muscles with sudden extension the extensor muscles with sudden extension of the neck and trunk with extension and of the neck and trunk with extension and abduction of the limbs. Extensor spasms abduction of the limbs. Extensor spasms and asymmetric or unilateral spasms often and asymmetric or unilateral spasms often are associated with symptomatic cases.are associated with symptomatic cases.

Mixed spasms are the most common type, Mixed spasms are the most common type, consisting of flexion of the neck and arms consisting of flexion of the neck and arms with extension of the legs, or flexion of the with extension of the legs, or flexion of the legs with extension of the arms.legs with extension of the arms.

In different series the frequency of the 3 In different series the frequency of the 3 spasm types were 42-50% mixed, 34-42% spasm types were 42-50% mixed, 34-42% flexor, and 19-23% ex flexor, and 19-23% ex

General physical General physical examinationexamination Physical examination can be important in helping to Physical examination can be important in helping to

identify specific etiologies that may have both identify specific etiologies that may have both systemic and neurological symptoms (eg, tuberous systemic and neurological symptoms (eg, tuberous sclerosis complex).sclerosis complex).

Often a patient with infantile spasms has normal Often a patient with infantile spasms has normal findings on general physical examination. No findings on general physical examination. No pathognomonic physical findings are present in pathognomonic physical findings are present in patients with infantile spasms.patients with infantile spasms.

If abnormalities in the general physical examination If abnormalities in the general physical examination are noted (eg, adenoma sebaceum, ash leaf macules), are noted (eg, adenoma sebaceum, ash leaf macules), specific etiologies may be suggested.specific etiologies may be suggested.

Patients may exhibit moderate-to-severe growth delay; Patients may exhibit moderate-to-severe growth delay; this is a nonspecific finding and more a reflection of this is a nonspecific finding and more a reflection of the underlying brain injury than of a specific epilepsy the underlying brain injury than of a specific epilepsy syndrome.syndrome.

NeurologicNeurologic examinationexamination

The neurologic examination in patients with The neurologic examination in patients with infantile spasms demonstrates abnormalities in infantile spasms demonstrates abnormalities in mental status function, specifically deficits in mental status function, specifically deficits in cognitive function consistent with developmental cognitive function consistent with developmental delay or regression.delay or regression.

Abnormalities in level of consciousness, cranial Abnormalities in level of consciousness, cranial nerve function, and motor/sensory/reflex nerve function, and motor/sensory/reflex examination are nonspecific findings and more a examination are nonspecific findings and more a reflection of the underlying brain injury or effect of reflection of the underlying brain injury or effect of anticonvulsant medications than of the syndrome.anticonvulsant medications than of the syndrome.

No pathognomonic findings are present on No pathognomonic findings are present on neurologic examination in patients with infantile neurologic examination in patients with infantile spasmspasm

CausesCausesInfantile spasms (West syndrome) can Infantile spasms (West syndrome) can

be classified according to its be classified according to its suspected etiology assuspected etiology as

Symptomatic:, 70-75% Symptomatic:, 70-75% cryptogenic, 8-42%cryptogenic, 8-42% . idiopathic. 9-14%.. idiopathic. 9-14%.

SymptomaticSymptomatic-: -: Patients are diagnosed with symptomatic infantile Patients are diagnosed with symptomatic infantile

spasms if an identifiable factor is responsible for spasms if an identifiable factor is responsible for the syndrome. any disorder that can produce the syndrome. any disorder that can produce brain damage can be associated with infantile brain damage can be associated with infantile spasms.spasms.

The list of etiologies can be subdivided into The list of etiologies can be subdivided into prenatal disorders, perinatal disorders, and prenatal disorders, perinatal disorders, and postnatal disorders.postnatal disorders. Prenatal disorders includePrenatal disorders include hydrocephalus, hydrocephalus,

microcephaly, hydranencephaly, schizencephaly, microcephaly, hydranencephaly, schizencephaly, polymicrogyria, Sturge-Weber syndrome, incontinentia polymicrogyria, Sturge-Weber syndrome, incontinentia pigmenti,pigmenti,  tuberous sclerosis, trisomy 21, hypoxic-tuberous sclerosis, trisomy 21, hypoxic-ischemic encephalopathies, congenital infections, and ischemic encephalopathies, congenital infections, and trauma.trauma.

Perinatal disorders includePerinatal disorders include hypoxic- hypoxic-ischemic encephalopathies, meningitis, ischemic encephalopathies, meningitis, encephalitis, trauma, and intracranial encephalitis, trauma, and intracranial hemorrhages.hemorrhages.

Postnatal disorders includePostnatal disorders include pyridoxine pyridoxine dependency, nonketotic hyperglycinemia, dependency, nonketotic hyperglycinemia, maple syrup urine disease, maple syrup urine disease, phenylketonuria, mitochondrial phenylketonuria, mitochondrial encephalopathies, meningitis, encephalopathies, meningitis, encephalitis, degenerative diseases, encephalitis, degenerative diseases, biotinidase deficiency, and trauma.biotinidase deficiency, and trauma.

CryptogenicCryptogenic -: -:

Patients have cryptogenic infantile spasms if no Patients have cryptogenic infantile spasms if no cause is identified but a cause is suspected and cause is identified but a cause is suspected and the epilepsy is presumed to be symptomatic.the epilepsy is presumed to be symptomatic.

The proportion of cryptogenic cases varies from The proportion of cryptogenic cases varies from 8-42%. This wide range may be related to 8-42%. This wide range may be related to variations in the definition of the term variations in the definition of the term cryptogenic and the age of diagnosis, since cryptogenic and the age of diagnosis, since assessment of developmental level in early assessment of developmental level in early infancy is difficult.infancy is difficult.

IdiopathicIdiopathic-:-:

Patients may be considered to have idiopathic Patients may be considered to have idiopathic infantile spasms if normal psychomotor infantile spasms if normal psychomotor development occurs prior to the onset of development occurs prior to the onset of symptoms, no underlying disorders or definite symptoms, no underlying disorders or definite presumptive causes are present, and no presumptive causes are present, and no neurological or neuroradiological abnormalities neurological or neuroradiological abnormalities exist. Some investigators use the terms exist. Some investigators use the terms idiopathic and cryptogenic interchangeably.idiopathic and cryptogenic interchangeably.

The percentage of idiopathic cases reportedly The percentage of idiopathic cases reportedly is 9-14%.is 9-14%.

Laboratory workupLaboratory workup

Prior to initiating therapy, consider obtaining Prior to initiating therapy, consider obtaining some or all of the following laboratory studies:some or all of the following laboratory studies:

Complete blood count with differential, liver Complete blood count with differential, liver panel, renal panel with electrolytes and glucose, panel, renal panel with electrolytes and glucose, calcium, magnesium, phosphorus, and urinalysis calcium, magnesium, phosphorus, and urinalysis with microscopic examinationwith microscopic examination

Metabolic workup including glucose, liver panel, Metabolic workup including glucose, liver panel, serum lactate and pyruvate, plasma ammonia, serum lactate and pyruvate, plasma ammonia, serum and urine amino acids, urine organic serum and urine amino acids, urine organic acids, and serum biotinidaseacids, and serum biotinidase

Blood, urine, and cerebrospinal fluid cultures if Blood, urine, and cerebrospinal fluid cultures if an infection is suspectedan infection is suspected

Cerebrospinal fluid analysis for cell count, Cerebrospinal fluid analysis for cell count, glucose, protein, bacterial and viral culture, glucose, protein, bacterial and viral culture, lactate, pyruvate, and amino acidslactate, pyruvate, and amino acids

ImagingImaging

About 70-80% of patients have abnormal About 70-80% of patients have abnormal findings on neuroimaging studies.findings on neuroimaging studies.

Magnetic resonance imaging (MRI) of the brain Magnetic resonance imaging (MRI) of the brain provides a more detailed evaluation than does a provides a more detailed evaluation than does a computed tomography (CT) scan of the brain.computed tomography (CT) scan of the brain.

Imaging studies should be obtained prior to Imaging studies should be obtained prior to starting ACTH or steroid therapy, as these starting ACTH or steroid therapy, as these therapies are associated with the appearance of therapies are associated with the appearance of apparent brain atrophy as treatment continues.apparent brain atrophy as treatment continues.

CT scanCT scan Structural brain anomalies such as Structural brain anomalies such as

hydrocephalus, hydranencephaly, hydrocephalus, hydranencephaly, schizencephaly, and agenesis of corpus schizencephaly, and agenesis of corpus callosum can be recognized easily by CT callosum can be recognized easily by CT scans.scans.

In addition, cerebral calcifications can be In addition, cerebral calcifications can be observed in patients with tuberous observed in patients with tuberous sclerosis or congenital infections.sclerosis or congenital infections.

- MRI scansMRI scans are superior to CT scans in detecting areas are superior to CT scans in detecting areas

of cortical dysgenesis, disorders of neuronal of cortical dysgenesis, disorders of neuronal migration, or disorders of myelination. migration, or disorders of myelination.

Electroencephalogram(EEGElectroencephalogram(EEG))

Interictal electroencephalogramInterictal electroencephalogramHypsarrhythmia is the characteristic interictal Hypsarrhythmia is the characteristic interictal

EEG pattern and consists of chaotic, high- to EEG pattern and consists of chaotic, high- to extremely high-voltage polymorphic delta extremely high-voltage polymorphic delta and theta rhythms with superimposed and theta rhythms with superimposed multifocal spikes and wave discharges. multifocal spikes and wave discharges. Multiple variations of this pattern are Multiple variations of this pattern are possible, including focal or asymmetric possible, including focal or asymmetric hypsarrhythmia. hypsarrhythmia.

Ictal electroencephalogramIctal electroencephalogram

Eleven different types of ictal patterns have Eleven different types of ictal patterns have been identified in patients with West syndrome.been identified in patients with West syndrome.

In one study, the most common pattern found In one study, the most common pattern found in 38% of patients with seizures was a high-in 38% of patients with seizures was a high-voltage, frontal dominant, generalized slow-voltage, frontal dominant, generalized slow-wave transient followed by voltage attenuation, wave transient followed by voltage attenuation, also termed an electrodecremental episode. also termed an electrodecremental episode. These electrodecremental episodes were a These electrodecremental episodes were a feature in 71% of the seizures.feature in 71% of the seizures.

No close correlation exists between the type of No close correlation exists between the type of seizure and the EEG pattern seizure and the EEG pattern

Ophthalmic Ophthalmic examinationexamination::

Ophthalmic examination may reveal Ophthalmic examination may reveal chorioretinitis from congenital chorioretinitis from congenital infections,infections,

chorioretinal lacunar defects in chorioretinal lacunar defects in patients with Aicardi syndrome, patients with Aicardi syndrome,

retinal tubers in patients with retinal tubers in patients with tuberous sclerosis.tuberous sclerosis.

Wood lampWood lamp

: Tuberous sclerosis is the single most : Tuberous sclerosis is the single most common recognizable cause of West common recognizable cause of West syndrome. Therefore, a careful syndrome. Therefore, a careful examination of the skin for the examination of the skin for the characteristic hypopigmented lesions of characteristic hypopigmented lesions of tuberous sclerosis is mandatory. The tuberous sclerosis is mandatory. The unaided bedside identification of these unaided bedside identification of these lesions may be more difficult in patients lesions may be more difficult in patients with light complexions.with light complexions.

TreatmentTreatment

The goals of treatment for infants with West The goals of treatment for infants with West syndrome are the best quality of life with no syndrome are the best quality of life with no seizures, the fewest adverse effects from seizures, the fewest adverse effects from treatment, and the least number of medications.treatment, and the least number of medications.

Medications such as ACTH and conventional Medications such as ACTH and conventional antiepileptic medications (AEDs) are the antiepileptic medications (AEDs) are the mainstay of therapy for infants with West mainstay of therapy for infants with West syndrome. Unfortunately, no one medical syndrome. Unfortunately, no one medical treatment gives satisfactory relief for all infants treatment gives satisfactory relief for all infants with West syndrome.with West syndrome.

The various medical treatment options for The various medical treatment options for infants with West syndrome can be divided infants with West syndrome can be divided into 2 major groups:into 2 major groups:

Commonly used first-line treatments (ie, Commonly used first-line treatments (ie, ACTHACTH  , prednisone, vigabatrin, prednisone, vigabatrin  , pyridoxine , pyridoxine [vitamin B-6][vitamin B-6]

Second-line treatments (ie, benzodiazepines, Second-line treatments (ie, benzodiazepines, valproic acid, lamotriginevalproic acid, lamotrigine  , topiramate, topiramate  , , zonisamidezonisamide  , levetiracetam , levetiracetam

In 2007, an expert survey concluded that 1-3 trials of monotherapy should be implemented before considering epilepsy surgery. In patients with tuberous sclerosis or symptomatic infantile spasms, vigabatrin was the drug of choice. Alternative options for symptomatic spasms included ACTH and prednisone.

Focal cortical resection: In some patients, resection of a localized region can lead to freedom from seizures.

Vigabatrin (Sabril)Vigabatrin (Sabril)

Indicated as monotherapy for children aged Indicated as monotherapy for children aged 1 mo to 2 y with infantile spasms. Precise 1 mo to 2 y with infantile spasms. Precise mechanism unknown. mechanism unknown. Irreversible inhibitor of gamma-aminobutyric acid transaminase (GABA-T). GABA-T metabolizes GABA, an inhibitory neurotransmitter, thereby increasing CNS GABA levels. Use must be must be weighed against risk of permanent vision weighed against risk of permanent vision loss. Approved by the FDA August, 2009. loss. Approved by the FDA August, 2009. Available only from restricted access Available only from restricted access program.program.

ACTHACTH

A 2004 American Academy of Neurology and A 2004 American Academy of Neurology and Child Neurology Society practice parameter Child Neurology Society practice parameter concluded that "ACTH is probably effective concluded that "ACTH is probably effective for the short-term treatment of infantile for the short-term treatment of infantile spasms and in resolution of hypsarrhythmia spasms and in resolution of hypsarrhythmia and "There is insufficient evidence to and "There is insufficient evidence to recommend the optimum dosage and recommend the optimum dosage and duration of treatment with ACTH for the duration of treatment with ACTH for the treatment of infantile spasms .treatment of infantile spasms .

PrednisonePrednisone

   A 2004 American Academy of A 2004 American Academy of

Neurology and Child Neurology Neurology and Child Neurology Society practice parameter Society practice parameter concluded that "there is insufficient concluded that "there is insufficient evidence that oral corticosteroids evidence that oral corticosteroids are effective in the treatment of are effective in the treatment of infantile spasms .infantile spasms .

Levetiracetam (Keppra, Levetiracetam (Keppra, Keppra XR)Keppra XR)

Mechanism of action: inhibition of N-type calcium Mechanism of action: inhibition of N-type calcium channels, modulation of GABA and glycine channels, modulation of GABA and glycine receptors and binding to SVA2 proteinreceptors and binding to SVA2 protein

One small open label trial of 5 infants with new One small open label trial of 5 infants with new onset cryptogenic infantile spasms showed clinical onset cryptogenic infantile spasms showed clinical effectiveness. Two children became seizure free, effectiveness. Two children became seizure free, while 2 others showed a minimum of 50% while 2 others showed a minimum of 50% reduction in seizures. The dose ranged from 30-60 reduction in seizures. The dose ranged from 30-60 mg/kg/d.mg/kg/d.

In another small open label trial of 7 children, 5 In another small open label trial of 7 children, 5 with symptomatic infantile spasms, treated with with symptomatic infantile spasms, treated with 20-80 mg/kg/d of levetiracetam, all responded to 20-80 mg/kg/d of levetiracetam, all responded to therapy. Two patients had >75% reduction in therapy. Two patients had >75% reduction in spasms and one had complete cessation of spasms.spasms and one had complete cessation of spasms.

Valproic acid Valproic acid (Depakene)(Depakene)

A 2004 American Academy of Neurology and A 2004 American Academy of Neurology and Child Neurology Society practice parameter Child Neurology Society practice parameter concluded that "there is insufficient evidence concluded that "there is insufficient evidence to recommend valproic acid for treatment of to recommend valproic acid for treatment of infantile spasms infantile spasms

Pyridoxine (vitamin Pyridoxine (vitamin B-6)B-6)

A 2004 American Academy of Neurology and Child A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded Neurology Society practice parameter concluded that "there is insufficient evidence to recommend that "there is insufficient evidence to recommend pyridoxine for the treatment of infantile spasms pyridoxine for the treatment of infantile spasms

Two distinct treatment situations exist in which Two distinct treatment situations exist in which pyridoxine is used in patients with West pyridoxine is used in patients with West syndrome:syndrome:

(1) IV administration during diagnostic EEG to (1) IV administration during diagnostic EEG to assess whether patient's seizures and EEG assess whether patient's seizures and EEG abnormalities are related to pyridoxine abnormalities are related to pyridoxine deficiency. In this approach, administer 50-100 deficiency. In this approach, administer 50-100 mg IV during diagnostic EEG; if dramatic mg IV during diagnostic EEG; if dramatic improvement noted in EEG, patient believed to improvement noted in EEG, patient believed to have pyridoxine-dependent seizureshave pyridoxine-dependent seizures

(2) Long-term oral administration: (2) Long-term oral administration: Effectiveness of long-term oral high-dose Effectiveness of long-term oral high-dose pyridoxine in West syndrome has been pyridoxine in West syndrome has been investigated in multiple open-label studies investigated in multiple open-label studies with promising results; most patients who with promising results; most patients who respond to long-term oral high-dose respond to long-term oral high-dose pyridoxine do so within 1-2 wk of initiation.pyridoxine do so within 1-2 wk of initiation.

PrognosisPrognosis The long-term overall prognosis is poor and is The long-term overall prognosis is poor and is

related directly to the etiology.related directly to the etiology. Infants with idiopathic West syndrome have Infants with idiopathic West syndrome have

better prognosis than do infants with better prognosis than do infants with symptomatic West syndrome. Only 14% of infants symptomatic West syndrome. Only 14% of infants with symptomatic West syndrome have normal or with symptomatic West syndrome have normal or borderline normal cognitive development borderline normal cognitive development compared with 28-50% of infants with idiopathic compared with 28-50% of infants with idiopathic West syndrome. Mental retardation is severe in West syndrome. Mental retardation is severe in 70% of patients, often with psychiatric problems 70% of patients, often with psychiatric problems such as autistic features or hyperactivity. such as autistic features or hyperactivity. Infrequently spasms may persist in adulthood. 50-Infrequently spasms may persist in adulthood. 50-70% of patients develop other seizure types and 70% of patients develop other seizure types and 18-50% of patients will develop18-50% of patients will develop  Lennox Gastaut Lennox Gastaut syndrome.syndrome.

Favorable prognostic factors include Favorable prognostic factors include cryptogenic etiology, age of onset >4 cryptogenic etiology, age of onset >4 months, absence of atypical spasms and months, absence of atypical spasms and partial seizures, absence of asymmetric EEG partial seizures, absence of asymmetric EEG abnormalities, short time from onset to abnormalities, short time from onset to treatment, and early sustained response to treatment, and early sustained response to treatment.treatment.

Infants with symptomatic infantile spasms Infants with symptomatic infantile spasms have been shown to be at higher risk for the have been shown to be at higher risk for the development of autism spectrum disorders, development of autism spectrum disorders, compared with those infants with compared with those infants with cryptogenic or idiopathic spasmscryptogenic or idiopathic spasms

This done aty 26-10-2011 (11/2 year child)reveals marked atrophy and volume loss of left temporal and parietal lobe,encephalmalacia .Widenining of the ipsilateral left lateral ventricle is notead”(porencephaly)Conclusion :-chronic sequalae of an old brain insult for clinical correlation

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