Transcript Paroxysmal disorders :

Paroxysmal disorders :
Abrupt onset of a clinical episode that
and repetitive , tends to be stereotyped
lasts seconds or minutes (rarely hours ),
and ends abruptly.
Definition of seizure :
transient and abruptly disturbance of
cerebral function(impaired consciousness,
abnormal motor activity, sensory
disturbances or autonomic dysfunction)
caused by excessive or over synchronized
cerebral neuronal discharges.
Differential diagnosis of Paroxysmal disorders:
• Seizure disorders
• Migraine and variants :
Paroxysmal torticollis , Paroxysmal vertigo (benign)
• Syncope and vasovagal events : Breach-holding spells
• Transient ischemic attack
• Metabolic disorders : Hypoglycemia
• Sleep disorders :Narcolepsy, Cataplexy , Night terrors
• Paroxysmal dystonia or choreoathetosis
• Shudder attacks
• Pseudoseizures
Seizures occur in 10% of children.
Less than one third of seizures in children
are caused by epilepsy.
• Epilepsy is the occurrence of two or
more unprovoked seizures at an
interval > 24 hours
• Provoking factors :
fever ,infection, syncope, hypoxia,
toxins, head trauma, stress, fatigue,
cardiac arrhythmias
Epileptic syndromes classification
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Age of onset
Etiology
Seizure type
Cognitive
development
• Neurologic exam
,CT
• prognosis
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Janz syndrome
West syndrome
Lennox-Gastaut syndrome
BPEC
LKS
Benign neonatal
convulsion
Clinical Seizure classification
Partial
(Only a portion
of the brain)
- Simple
(Normal consciousness)
- Complex
(Impaired consciousness)
Generalized
(Both hemispheres are
involved)
Type of Seizure
When partial seizures spread to involve the whole brain and
produce a generalized tonic-clonic seizure, they show secondary
generalization or Jacksonian seizures .
Partial or focal Seizures
• 40-60 % of epilepsy of childhood
• Simple partial
– Simple with motor signs
– Simple with sensory signs
– Autonomic: abdominal epilepsy
– Psychic : déjà vu, fear,…
• Complex partial : psychomotor seizures or temporal
lobe epilepsy or limbic
Generalized Seizures
– Absence Seizures (Petit mal)
– Tonic/Clonic (Grand mal)
– Atonic Seizures (Drop attacks)
– Myoclonic Seizures
– Clonic
– Tonic
Simple Partial seizures :
• The most common form : motor activity
• SPS arise from a anatomic specific focus.
Location and direction of spread of the
seizure focus determine clinical symptom
logy.
• Spread of partial seizure to the whole brain
produced GTCS(secondary generalization )
• Only psychic or autonomic symptoms can
be difficult to diagnosis.
SPS : 10 -20 sec
• Uncinate seizure :)‫احساس بوي نامطبوع (تمپورا ل مياني‬
• Gelastic seizure : spells of uncontrolled
laugher
hypothalamic tumors
• Versive seizure :‫انحراف سر و چشم به يك سمت‬
• lip smacking : anterior temporal lobe
• Macropsia , Micropsia, vertigo: posterior
temporal lobe
• Autonomic :fever ,tachycardia ,shivering and
increased GI motility
• Limbic seizure :dream like state and bizarre
psychic abnormalities
CPS
• Aura: in one third (epigastric discomfort,
vague unpleasant feeling , fear)
• Automatisms: in 50-75 %
in infant :alimentary , in child: behavioral
• EEG: Spike or sharp in anterior temporal
• Neuroimaging: abnormal in 50-80%
tumor, AVM, MTS, cortical dysplasia, focal
atrophy, gliosis, infarction
• There may be a brief blank stare or a
sudden cessation or pause in activity.
Treatment of generalized Seizures
GTCS:
First choice : valproate
Second : CBZ , PHT , TPM , LMC , Pb
CLZ , PRM , Zonisamide
Absence :
First choice :Valproate , ethoxysuximide , LMC
Second choice : TPM, Levetiracetam , Zoni
Myoclonic :
First : Valproate
Second : LMC , CLZ, Levetiracetam , Zoni
 Carbamazepine may exacerbate absence and
myoclonic seizures
Partial Onset Seizures
First-line drugs are carbamazepine and phenytoin.
PHT rarely used as first-line agent in children
because of toxicity.
 Carbamazepine : an acceptable first drug
 Gabapentine is another option
Adjunctive (add-on) therapy :
oxacarbamazepine , LMC, TPM, VPA , Pb, PRM ,
felbamate, levetiracetam , tiagabine, zonisamide
 WHO recommends Pb as choice treatment of partial &
TCG seizures in countries with restricted resources .
Treatment of partial seizure
• Carbamazepine : drowsiness, dizziness,
diplopia ,SIADH (hyponatremia ) ,aplastic
anemia , agranulocytosis
• Phenytoin :
gingival hyperplasia , hirsutism ,rickets ,
SLE, ataxia, coarse face, nystagmus,
psudolymphoma, Stevens – Johnson
syndrome
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Benign focal epilepsy or rolandic epilepsy or
BPEC :
Age of onset : 5-10 years
16% of all afebrile seizure in <15 years
50-75 % occur during sleep(20 % only one
seizure)
Somatosensory aura around the face and mouth
and then focal motor (face and then ipsilateral
exterimity ) , and finial secondary generalized.
Good prognosis and normal CT,IQ,N.E
EEG :spike in centrotemporal
Treat: CBZ in frequent seizures for 2 yr
Absence seizures
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In 6-20% of epileptic children
No aura, no postictal, no loss of tone
Age: 4-6 yr NE & imaging :normal
A brief loss of environment awareness and staring or
eye fluttering or simple automatism such as head
bobbing &lip smacking
• EEG: 3cps spike and wave
• Seizures provoked by HV & flashing light
• 40-50% have generalized seizures(60%before
absence & 40% after the onset of absence.
• Treatment : Ethosuximide ,valproate
clonazepam as alternative
Differentiating of absence from CPS
1. The automatism of CPS are more complicated
(repetitive swallowing, picking of the hands
or walking in nonpurposeful circles
2. postictal confusion in CPS
3. Absence provoked by hyperventilation
4. CPS last few minutes ,absence :few sec
5. absence :dozens per day but patients with CPS
rarely have more than one or two seizures in
day
6. EEG
Often referred as behavior problem,
Poor school marks; disruptive behavior
Side effects of drugs:
• Valproate :pancreatitis, drowsiness, tremor ,
alopecia, weight gain, fatal liver failure
(Reye-like syndrome ) in <2yr
• Ethosuximide : nausea ,lethargy, hiccups
,SLE, Stevens – Johnson syndrome ; blood
dyscrasia
• Clonazepam :ataxia, lethargy, blood
dyscrasia ,depression, salivation
Myoclonus: a lightning-like jerk of part of the
body
1. Epileptic: EEG shows epileptiform discharges during
the jerk
2. Nonepileptic : may originate in the B.G ,BS, or spinal
cord
- Benign: sleep myoclonus
- Serious pathology
 The underlying illness producing myoclonic
epilepsy may be developmental and static or progressive
and associated with neurologic deterioration (neuronal
Ceroid lipofuscinosis).
Juvenile myoclonic epilepsy or Janz
syndrome :
• Onset age : 12-16 yr (adolescent )
• AD ( chromosome 6)
• Myoclonus in the morning (predominantly
within 90 minutes of awakening )with or
without generalized clonic and absence
seizure
• N.E : normal
• EEG : 4-6 / SWD
• Treat : valproic acid for lifelong
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Infantile spasms or west syndrome :
Age : 3-8 month , 86 % onset of seizures : < 1yr
Forms : mix , flexor , extensor
spasms occur in drowsiness state.
Repeated clusters occur each day.
EEG : hypsarrhythmia ( HVSW, spikes, polyspike and
disorganized background)
Poor prognosis
Classification : cryptogenic (40% )
symptomatic :TS is the most common
40 % of cryptogenic have a good intellectual.
Very Poor intellectual prognosis in symptomatic
Treat : ACTH , prednisolone ( irritability , swelling
,hypertension , glycosuria, severe infection )
benzodiazepines , valproate , vigabatrin (sabrile )
Etiologies of infantile spasms
• Methabolic : PKU,
MSUD, biotinidase
deficiency ,NKH,
hypoglycemia , B6
dependency,
lipidosis,…
• CNS dysgenesia :
polymicrogyria,
lissencephaly, Down
syndrome,…
• Neurocutaneous :
tuberous scerosis,….
• Congenital infections :
toxo, CMV, syphilis
• Encephalopathies:
postasphyxia
posttraumatic
posthemorragic
postinfections
postimmunization
(pertusis )
Etiology of seizures
Perinatal conditions :
CNS malformation, hemorrhage, HIE, congenital
infection, trauma
 Metabolic conditions:
hypoglycemia,hypocalcemia,B6 deficiency
hypomagnesemia, hypo or hypernatremia , storage
disease, degenerative , Reye syndrome
 Poisoning :
lead , cocaine, cyanide , co, pesticide, strychnine
 drugs :
، ‫فنوتيازين ليتيوم‬،‫آنتي كولينرژيك‬، ‫آنتي دپرسانت‬، ‫آمينوفيلين‬
‫ايزونيازيد‬، ‫ ساليسيالت‬، ‫ آمفتامين‬، ‫ناركوتيك‬
Etiology of seizures
Neurocutaneous
syndromes :
Tuberous sclerosis , NF,…
 Systemic disorders :
vasculitis ( CNS or systemic ) SLE
hypertensive encephalopathy
renal failure
hepatic encephalopathy
 Infection: ‫ آبسه مغزي‬، ‫ مننژيت‬،‫انسفاليت‬
 Other : trauma , tumor, idiopathic , familial
 Over-the-counter drugs, illicit substances herbal
preparations, can precipitate seizure
Laboratory evaluation of seizures
• CBC
• Glucose,
ca,mg,p
• Na,K,Bun,Cr
• Toxicology
• CSF
• EEG
• Imaging
• metabolic
Neuroimaging :
 Neonatal seizure
 Focal seizure
 focal EEG
 Focal neurologic finding
 Neurodevelopmental delay
 Dysmorphic face
Breath holding spells
• In 5 % of children, rare in <6mo and >5-8 yr,
80% <18mo all in <3yr,
• Cyanotic BHS :crying, prolonged expiratory
apnea, cyanosis,UWG, tonic-clonic movement
treat: reassurance, piracetam, ferrous
• Pallid BHS: painful stimuli, asystole, pallor,
bradycardia, opisthotonos ,seizure
treat : atropine , less benign than cyanotic
Fever and seizure:
• 1. CNS infection
• 2. Epilepsy triggered by fever
• 3. Febrile seizure
 F.C occur in 2- 4 % of children.
 AD (chromosome 19 & 8)
 50 % in 1-2 yr , 93% in < 3 yr
 URI, roseola, AOM are the most common causes of
F.C.
Recurrent F.C :
first FC in <1yr
50 %
first FC in >1yr
28 %
10 % of children have 3 or more recurrence
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Febrile Seizures
Fever of over 38.5C (even 37.8)
Age range of 6 mo to 7 yr
No infection of the CNS or electrolyte abnormality
No previous non-febrile seizure or neonatal seizure
Simple:
– Generalized
– Less than 15 minutes
– One in 24h
• Complex:
– Focal
– Over 15 minutes
– More than one in 24h
– Focal neurologic sign in postictal state
Risk factors for recurrence of FC
Major :
1. Age <1 yr
2. Duration of fever <24 hr
3. Fever 38-39
Minor:
1. Family history of FC
2.Family history of epilepsy
3.Complex febrile seizure
4. Day care
5. Male gender
6. Lower serum sodium
Having no risk factors
carries a recurrence risk
of about 12%;
1 risk factor, 25-50%
2 risk factors, 50-59%
3 or more, 73-100%
prophylaxis of recurrent
FC:
Oral diazepam at the
onset of each febrile
illness
Prolong
anticonvulsant pb or
Risk of epilepsy in FC
 Abnormal neurologic examination or
development
 Positive family history of epilepsy
 Complex FC
 FC in < 1 yr
 Recurrent FC
 Seizure with T< 40 degree
prophylaxis :
Prolong anticonvulsant : pb or Nav
Risk factors for occurrence of subsequent
epilepsy
Risk factor
Risk for subsequent epilepsy
Simple febrile seizure
1%
Neurodevelopmental abnormalities
33%
Focal complex FC
29%
Family history of epilepsy
18%
Fever <1 hr before febrile seizure
11%
Complex febrile seizure, any type
6%
Recurrent febrile seizures
4%
‫‪Meningitis AND FC‬‬
‫ريسك فاكتورهاي احتمال مننژيت‬
‫‪ .1‬ويزيت در ‪ 48‬ساعت گذشته‬
‫‪ .2‬تشنج در بدوورود به اورژانس‬
‫‪ .3‬تشنج فوكال‬
‫‪ .4‬معاينه عصبي غير طبيعي‬
‫انديكاسيون انجام ‪LP‬‬
‫‪ .1‬تشنج با تب در زير يك سال‬
‫‪ .2‬وجود لتارژي پرسيستانت‬
‫‪.3‬اولين تشنج تب كمپلكس‬
‫‪ .4‬در بچه ايي كه قبال آنتي بيوتيك گرفته و امكان پيگيري نباشد‪.‬‬
Routine laboratory testing in epileptic patients
 Routine laboratory testing is not cost effective or
necessary with the exception of felbamate, which is
associated with a relatively high risk of aplastic anemia
and requires close laboratory monitoring.
 Vomiting (symptom of hepatotoxicity or pancreatitis),
prolonged unexplained fever, easy bruising, extreme
fatigue or lethargy, flu-like symptoms, worsening of
seizures, change in mental status, and abdominal pain
should lead to further investigations
 Many idiosyncratic reactions of AEDs (StevensJohnson syndrome, TEN, serum sickness , pancreatitis)
are not predicted by presymptomatic blood test
abnormalities
 Patients taking AEDs should be monitored for
emergence or worsening of suicidal ideation or
depression.
Status epilepticus:
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Generalized tonic-clonic activity lasting longer
than 20 minutes, or repeated seizures without
restoration of consciousness for more than 30
minutes, is defined as status epilepticus and may
lead to irreversible brain injury.
Complications of SE:
Reduction of CPP
Hyper/hypotension
Dysrhythmia, CHF, Apnea, Aspiration
Non cardiogenic pulmonary edema,
Rhabdomyolysis,
Hypo/hyperglycemia
Subtypes of Status epilepticus
• Symptomatic :
- acute brain injury : ( 25 % of children )
CNS infections , electrolyte disorder, acute
anoxia
- congenital malformation or previous brain
injury ( in 10 % of children )
- other : hypoglycemia , hypocalcemia, poisoning,
Reye ,lead, frontal tumor,…
• Prolonged FC: most common cause in <3yr
• Idiopathic : sudden cessation of AED
Management of status epilepticus
• Open the airway / administer oxygen, if
necessary intubate
• Check the circulation
• Obtain vital signs
• Insert NG tube & IV line
• Laboratory evaluation :
CBC , glucose, Ca, Mg, P, BUN, Cr, lactate
other
per case
• IV Anticonvulsant medications
Drugs in SE
1.Benzodiazepine: max rate of administration :1 mg/min
Diazepam :
IV: 0.2-0.4 mg/Kg (up to 10mg) ,Rectal : 0.3 -0.5 mg/Kg
Midazolam 0.1-0.2 mg/kg
Lorazepam 0.05 – 0.1 mg/Kg IV
2. Phenytoin :10- 20 mg/Kg (up to 1- 1.5 g) IV at a rate
1mg/Kg/m in with ECG & heart rate monitoring
3. Phenobarbital :10 -20 mg/Kg IV at a rate of
1mg/Kg/min
4. Valproic acid : 20 mg/kg
Then: Continuous drip of diazepam, midazolam , pb
and general anesthesia
Neonatal seizures
1.Subtle :the most common form
apnea, eye deviation ,tongue thrusting, eye
blinking, staring, bicycling, fluctuation of vital
sign, sialorrhea, pedaling movements
2. Focal or generalized tonic : sustained posturing
of limbs or trunk
3.focal or multifocal clonic: repetitive ,rhythmic
contraction of muscles of limbs, face, or trunk
4. Focal or generalized myoclonic :arrhythmic
contraction of muscles of limbs, face, or trunk
Etiology of neonatal seizures
• HIE :the most common
cause in full term, occur
12- 24 hours after birth
asphyxia
• Hypoglycemia
• Hypocalcemia
• Hypomagnesaemia
• Hypo or hypernatremia
• Congenital brain
malformation
• IVH :between 1 and 3 days
of age in preterm
• B6 dependency
• Injection of local
anesthetic agents into
fetal scalp (transient
bradycardia, fixed
mydriasis) in lab. room
• Sepsis: after 5 days
• Drug withdrawal
• IEM :lethargy, acidosis,
FH of infant death
• SAH: sudden onset on
days1-3,short duration
,do not recur
Benign idiopathic neonatal seizure
or fifth day fits
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In 5 % of full term neonate
Seizure in 4- 6 days
Multifocal clonic seizure
Duration of seizures : 24 hours
Prognosis : good
Mental development : normal
Benign familial neonatal seizure
• AD , chromosome 20
• Generalized clonic seizures occurring
toward the end of first week of life (2-3 )
• 10- 20 times in a day
• Outlook : favorable
• Seizures stop in 6 months of age.
• Response to treat is variable.
Differentiate of seizure from
jitteriness or tremulousness:
• Jit are sensory dependent, elicited by stimuli,
interrupted by holding the limb
• Jit is fine &rapid, seizure is coarse, fast and
slow clonic activity
• Abnormal eye movement in seizure
• Autonomic abnormality ( increased in BP or
PR ) in seizure
• Jit occur in crying or examining
Jit : IDM, after asphyxia, infants with narcotic
withdrawal
Treatment of neonatal seizure
• Treat of specific cause:
Hypoglycemia , Hypocalcemia ,
Hypomagnesaemia,hypo or hypernatremia ,
B6 deficiency
• In the absence of an identifiable cause , pb
20 – 40 mg/Kg and then dilantin 10 -20
mg/Kg or diazepam 0.1- 0.2 mg/Kg followed
by one of the two longer- acting drugs
• Prognosis : dependent to underlying cause