Paroxysmal disorders: Abrupt onset of a clinical episode that tends to be stereotyped and...
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Paroxysmal disorders:Abrupt onset of a clinical episode that Abrupt onset of a clinical episode that tends to be stereotypedtends to be stereotyped and repetitive , and repetitive , lasts seconds or minutes (rarely hours ), lasts seconds or minutes (rarely hours ),
and ends abruptlyand ends abruptly.. Definition of seizure:
transient and abruptly disturbance of transient and abruptly disturbance of cerebral function(cerebral function(impaired consciousness, impaired consciousness, abnormal abnormal motor activity, sensory motor activity, sensory disturbances or autonomic dysfunction) disturbances or autonomic dysfunction) caused by excessive or over synchronized caused by excessive or over synchronized cerebral neuronal dischargescerebral neuronal discharges..
Differential diagnosis of Paroxysmal disorders:
• Seizures
• Migraine
• TIA
• Syncope
• Breath holding spells
• Paroxysmal Vertigo
• hypoglycemia
• Tics• Pseudo seizures• Night terrors• Narcolepsy• Paroxysmal
torticollis• Paroxysmal
dystonia
Seizures ocurr 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
• Provokeing factors : fever ,infection, syncope, hypoxia, toxins, head trauma, stress,fatigue, cardiac arrhythmias
Epileptic syndromes classification
• Age of onset• Etiology• Seizure type• Cognitive
development• Neurologic
exam ,CT• prognosis
• Janz syndrom• West syndrom• Lennox-Gastaut syn.• BPEC• LKS• Benign neonatal
convulsion
Clinical Seizure classification
Partial (Only a portionof the brain)
- Simple(Normal consciousness)- Complex
(Impaired consciousness)
Generalized(Both hemispheres areinvolved)
Type of Seizure
Classification
• Partial (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
Classification
• 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 symptomology.
• Spread of partial seizure to the whole brain produced GTCS. (secondry generalization ).
• Only pshycic or autonomic symptoms can be difficult to diagnosis.
SPS : 10 -20 sec
• Uncinate seizure :) مياني ) ل تمپورا نامطبوع بوي احساس• Gelastic seizure : spells of uncontrolled laugher
hypothalamic tumors• Versive seizure : سمت يك به چشم و سر انحراف• 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, in one third (epigastric discomfort,
vague unpleasant feeling , fea vague unpleasant feeling , fearr))
• Automatisms: in 50-75 % in 50-75 % in infant :alimentary , in child:behavioral in infant :alimentary , in child:behavioral
• EEG: Spike or sharp in anterior temporal Spike or sharp in anterior temporal
• Neuroimaging: abnormal in 50-80% abnormal in 50-80% tumor, AVM, MTS, cortical tumor, AVM, MTS, cortical dysplasia,focal atrophy, gliosis, dysplasia,focal atrophy, gliosis, infarctioninfarction
• There may be a brief blank stare or a sudden cessation or pause in activity.
Goals of epilepsy treatment Indication for start of AEDs
Control of seizures
Minimal adverse events
Good patient compliance
AEDs suppress but do not cure seizures 80% of patients have control of seizures
60% seizure free, 20% drastically reducing
with treatment
Main aim is monotherapy
Significant improvement in only 10 % of
patients with second drug
Choosing AEDs : Seizure type & Epileptic syndrome
Pharmacokinetic profile
Medical history , interactions/potential for future problems
Efficacy
Expected adverse effects
Cost
Start at low dosage , gradually increase until seizure control or side effect threshold
Never abruptly stop medications
Principles of antiepileptic drugs treatment Pharmacokinetics : What the body does to drug
( absorption , distribution, metabolism, elimination )
Pharmacodynamics : What the drug does to body
( study of biochemical and physiologic effects of drug)
Toxicology
When a total daily dose is increased , sufficient
time (about 5 t 1/2) should be allowed for the
serum drug level to reach a new steady-state level
Withdrawal may be considered after
seizure- free period of 2-3 or more years.
Relapse rate after withdrawn AEDS : 20 -40 %
•
Treatment of generalized Seizures GTCS: First choice : valproate Second : CBZ , PHT , TPM , LMC , Pb CLZ , PRM , Zonisamide Absence :First choice :Valproate , ethoxysuximide , LMCSecond choice : TPM, Levetiracetam , ZoniMyoclonic : 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
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 finialy secondry generalized.
• Good prognosis and normal CT,IQ,N.E• EEG :spike in centrotemporal• Treat: CBZ in frequent seizures for 2 yr
Landau-Kleffner syndrome or Acquired Epileptic Aphasia:
• Onset age :early childhood (4-7 yr)• Cortical auditory deficit and language
disability • Seizure in 80% (partial and generalized)• EEG :highly epileptiform during sleep• CT or MRI :normal • Prognosis :not good• Treat :Nav, clobazam, steroid ,IVIG
Rasmussen encephalitis:chronic , progressive focal inflammation of the chronic , progressive focal inflammation of the
brain of unknown originbrain of unknown origin • Usual age of onset :6-10 yr• Nonspevific viral disease focal persistent
motor activity (epilepsia partialis continiua ) hemiplegia and cognitive deterioriation.
• EEG:focal spike and slow wave activity• Imaging :initially normal and then show atrophy in
the involved area• Prognosis :progressive and lethal• Hemispherectomy for seizure eradication and
prevention of cognitive deterioration but with permanent hemiparesis
Absence seizures• 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 enviroment awareness and starig 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
• EEG
Often referred as behavior problem,Often referred as behavior problem,Poor school marks; disruptive behaviorPoor 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
• Epileptic: EEG shows epileptiform discharges during the jerk.
• Nonepileptic :may originate in the B.G ,BS, or spinal cord.
• Benign: sleep myoclonus• Serious pathology• Underlying illness produce myoclonic epilepsy:
- developmental or static - progressive
Juvenile myoclonic epilepsy or Janz syndrom :
• Onset age : 12-16 yr (adolesenct )• AD ( chromosome 6)• Myoclonus in the morning with or without
generalized clonic and absence seizure .• N.E is normal. • EEG : 4-6 / SWD• Treat : valproic acid for lifelong
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% ) Classification : cryptogenic (40% ) • symptomatic :TS is the most commonsymptomatic :TS is the most common• 40 % of cryptogenic have a good intellectual.• Very Poor intellectual prognosis in symptomatic • Treat : ACTH , cortone ( irritability , swelling ,hypertension , Treat : ACTH , cortone ( irritability , swelling ,hypertension ,
glycosuria, severe infection ) benzodiazepines , valproate glycosuria, severe infection ) benzodiazepines , valproate
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 seizuresPerinatal 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 :
،آنتي دپرسانت ،آنتي آمينوفيلين ، ناركوتيك ، ليتيوم كولينرژيك،فنوتيازين
،ايزونيازيد ساليسيالت ، آمفتامين
Etiology of seizuresNeurocutaneous syndromes :
Tuberous sclerosis , NF,… Systemic disorders : vasculitis ( CNS or systemic ) SLE
hypertensive encephalopathy
renal failure hepatic encephalopathy
Infection: مغزي آبسه ، 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 :Neuroimaging :Neonatal seizureFocal seizure focal EEGFocal neurologic finding Neurodevelopmental delayDysmorphic 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 (choromosome 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
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 of recurrence of FC
• Positive family history of FC
• FC in < 1 yr• Seizure with
T< 40 degree • Seizure with
fever <1 hour• Complex FC
prophylaxis of recurrent FC:
Oral diazepam at the onset of each febrile illness
Prolong anticonvulsant : pb or Nav
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
Meningitis AND FC
فاكتورهاي ريسكمننژيت احتمال
در. 1 ساعت 48ويزيتگذشته
به. 2 بدوورود در تشنجاورژانس
فوكال. 3 تشنجغير. 4 عصبي معاينه
طبيعي
نجام ا نديكاسيون LPا
زير. 1 در تب با تشنجسال يك
لتارژي. 2 وجودپرسيستانت
تب. 3 تشنج اولينكمپلكس
قبال. 4 كه ايي بچه درو گرفته بيوتيك آنتي
نباشد پيگيري .امكان
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 (Stevens- Johnson 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.
Adverse effects of new AEDs AEDSerious Adverse EventsNonserious Adverse
Events
GabapentinNoneWeight gain, peripheral edema, behavioral changes*
LamotrigineRash, including Stevens Johnson and toxic epidermal necrolysis (increased risk for children, also more common with concomitant valproate use and reduced with slow titration); hypersensitivity reactions, including risk of hepatic and renal failure, DIC, and arthritis
Tics* and insomnia
AEDSerious Adverse EventsNonserious Adverse Events
LevetiracetamNoneIrritability
behavior change
OxcarbazepineHyponatremia (more common in elderly), rash
None
TiagabineStupor or spike wave stuporWeakness
TopiramateNephrolithiasis, open angle glaucoma, hypohidrosis (predominantly children)
Metabolic acidosis, weight loss, language
dysfunction
ZonisamideRash, renal calculi, hypohidrosis (predominantly
children)
Irritability, photosensitivity,
weight loss
Status epilepticus Definition :as a seizure that lasts for 30 minutes
or longer or is repeated frequently so as consciousness not regained between seizures
Seizure lasting more than five minutes has a high risk of lasting 30 minutes and treatment delay is associated with delayed treatment response.
In 1.3 to 16% of epileptic patients. Mortality: 2.7 - 20 percent
SE occurs in 1.3 to 16% of epileptic patients
Mortality: 2.7 - 20 percent
Incidence rates, causes, and prognosis vary substantially by age
Most in the first year of life
Febrile SE is the most common etiology
60 % of children are neurologically healthy prior to the first episode of SE.
.What the common complications of status
epilepticus ?• Reduction of CPP• Hyper/hypotension• Dysrhythmeia, CHF, Apnea, Aspiration• Non cardiogenic pulmonary edema,
Rhabdomyolysis,• Hypo/hyperglycemia
Status epilepticus classification Clinical Simple partial
Complex partial
Generalized convulsive
Generalized non-convulsive
Etiologic
• Symptomatic ( acute or remote)
• Idiopathic
• Reactive : Prolong FC
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
Pathophysiology : excessive excitatory neurotransmitters : glutamate , aspartate , acetylcholineIneffectiveness of inhibition : GABANeuronal loss with every episode, particularly if prolonged
Complication: Hypoxemia and acidosis Hyperglycemia & hypoglycemia Hyper tension & hypotension IICP and cerebral herniation Hyperpyrexia Hyperkalemia Myoglobinuria Non cardiogenic pulmonary edema Leukocytosis(60%) CSF pleocytosis(13%)
Risk Factors • Partial seizures with clustering
• Focal background EEG abnormalities
• Secondary generalized Partial seizures
• Generalized abnormalities on neuroimaging
• Younger age of epilepsy (1 year or less) at onset
• Symptomatic etiology of epilepsy
• Occurrence of SE as the first seizure
Management Assess respiratory & circulatory status &
maintenance of an adequate airway and o2 therapy , cardiac & BP monitoring, pulse oximetry
A rapid history & neurologic exam: to determine classification & etiology
Establishment of IV line and obtain blood samples : CBC, glucose , Ca ,P , Mg , Cr, ABG, LFT, Na, K .
Toxic and metabolic screens ,AED level, blood culture,…in some patients
Correction of metabolic abnormalities: hypoglycemia
Management Neuroimaging: after stabilization of patient , especially in a patient with focal neurologic signs EEG for background activity evaluation : as soon as possible after the seizure stops, ideally in 1-2 hour If the patient has not regained a relatively
normal mental state within a few hours after SE has stopped, an EEG should be performed to evaluate the possibility of subclinical seizures.
Drugs:• Oral or IM medication (Pb, Pht, VPA ,
OXCBZ , Levetiracetam) : in partial seizures or brief generalized motor seizures have stopped before the child arrives in the emergency department, which are short in duration, or the child is conscious despite multiple seizures.
• If IV access is delayed or impossible, many AEDs can be given by alternative routes : interosseous , IM, rectal , intranasal
Missed medication
Paradoxical effects
Nonprescription medications : over-the-counter drugs , illicit substances, herbal preparations
Some AEDs commonly used to treat SE may worsen seizures caused by illicit drugs .
In SE is induced by cocaine, other local anesthetics, theophyliine , lindane treatment with benzodiazepines is recommended ( not phenytoin )
Drugs:Benzodiazepines:Diazepam : 0.3mg/kg, 2mg/min, Max :10mg duration of anticonvulsant effect <20 minutes first choice in outside of emergency department is not considered first-line therapy for SE(less efficacy and
more respiratory depression) Lorazepam : Effective duration of action : 4-6 hours IV-rectal , 0.5 - 2 mg/min , Max: 4mg <12 yr : 0.1 mg/kg >12yr : 0.05 mg/kg Midazolam : IV, IM, rectal , nasal ,oral 0.15-0.2mg/kg
Antiepileptic DrugsPhenytoin: IV,15-30 mg/kg each time 10mg/kg , 1mg/kg/min
or
Fosphenytoin (IV-IM,15-20 mg/kg , 3mg/kg/min) with monitoring of HR and BP and avoiding of venous extravasations
Side effects :
hypotension ,arrhythmias, local pain and injury, venous thrombosis and purple glove syndrome (edema, discoloration ,and pain in distal extremity to the site of infusion)
Phenobarbital : IV-IM-SC, 15-20mg/kg , 2mg/kg/min
Side effects : sedation, respiratory depression
IV Lidocaine: (1 - 2 mg/kg ,once or twice) is one of the second-line drugs in SE treatment with favorable properties of prompt responses, less alteration of consciousness and respiratory depression
Valproic acid : 20-40 mg/kg IV(diluted 1:1 with normal saline or DW 5% ) over 5 to 10 minutes can be used as a second or third-line treatment.
Refractory status epilepticus
is defined as ongoing seizures despite the use of two first-line drugs, usually a benzodiazepine plus either phenytoin or phenobarbital, or which continues for > 60-90 minutes in spite of adequate treatment
Admit in P-ICU treatment of cerebral edema
Drugs in refractory SE• Pentobarbital : 5-15 mg/kg stat , and 0.5-5 mg/kg/h in
stable hemodynamic Side effects: hypotension , pulmonary edema, ileus,
delayed neurologic recovery• Midazolam:0/15-0/2mg/kg stat, 1-5 µg/ kg /min• Propofol:1-3mg/kg stat, 2-10mg/kg/h , should not be used
in children on the ketogenic diet Adverse effects:
bradycardia , apnea, hypotension , hypertriglyceridemia , acidosis , rhabdomyolysis
• IV valproic acid : 15-20mg/kg in 1-5 min, 5mg/kg/h• lidocaine drip• Paraldehyde• General anesthesia • Topiramate
Neonatal seizures• 1.subtle :the most common forms
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• Hypomagnesemia• Hypo or hypernatremia• Congenital brain
malformation• IVH :between 1 and 3
days of age in preterm
• Vit 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
• In 5 % of fullterm neonate
• Seizure in 4- 6 days
• Multifocal clonic seizure
• Duration of seizures : 24 hours
• Prognosis : good
• Mental development : normal
Benign familial neonatal seizure
• AD , choromosom 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 ocurr in crying or examining jit : IDM, after asphyxia,infants with narcotic withdrawal
Treatment of neonatal seizure• Treat of specific cause:
Hypoglycemia Hypocalcemia, Hypomagnesemia, hypo or hypernatremia and B6 deficiency
• In the absence of an indentifiable cause ,pb 20 – 40 mg/Kg and then dilantin 10 -20 mg/Kg od diazepam 0.1- 0.2 mg/Kg followed by one of the two longer- acting drugs
• Prognosis : dependent to underlying cause
Imitators of epilepsy : Nonepileptic paroxysmal disorders
Paroxysmal disorders Abrupt onset of a clinical episode that tends to be stereotyped and repetitive , lasts seconds or minutes (rarely hours) , and ends abruptly 25 % of patients referred to epilepsy clinic don't have epilepsy & recognition is important: to avoid unnecessary treatmentto avoid unnecessary treatment to institute the correct treatment when requiredto institute the correct treatment when required
کننده کمک سواالت گريه نظير خاصي شرايط بدنبال حمالت اين هميشه آيا
، کردنگيرد؟ ... می صورت و خواب ، غذاخوردن ، ورزش ، سرفه
می مختل حمالت اين بروز زمان در کودک هوشياری آياو شود
؟ آورد می ياد به کامال را آن طفل اينکه يا می ،تغيير حمالت اين حين در کودک رنگ آيا
می کند؟ حرکت خاصی جهت در چشمها آيا؟ ساير کنند از بيش بدن از قسمت کدام
شود؟ می درگير ها از قسمت تواند می کودک، آيا؟ کند جلوگيری حاالت اين می بروز والدين آيا
؟ کنند سازی شبيه پزشک، نزد را حمالت اين آيا تواننددارد؟ وجود خانواده در حمالت اين از مثبت فاميلی سابقه
حاالت بروز زمان شده گرفته فيلم مشاهده
Nonepileptic paroxysmal eventsNeonate :
Apnea
Jitteriness
Sleep myoclonus
Hyperkplexia
Infants :
Syncope (BHS ,…)
Benign myoclonus of infancy
Shuddering attacks
Sandifer syndrome
Paroxysmal torticollis of infancy
Extrapyamidal drug reaction
Benign paroxysmal vertigo
Spasmus nutans
Opsoclonus-myoclonus
Masturbation
Nonepileptic paroxysmal eventsChildren
BHS
Vasovagal syncope
Migraine
Tic disorders
Staring spells
Sleep disorders
BPV
Masturbation
Movement disorder
Adolescents and young adults
Vasovagal syncope
Migraine
Daydreaming
Sleep disorders
Paroxysmal dyskinesia
Tic disorders
Hemifacial spasm
Stiff person syndrome
Pseudoseizures
Episodic rage
In neonate Apneic seizure : if is accompanied by abnormal eye
movement, mouth movement , in BP or HR Jitteriness :
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 in seizure
Jit occur in crying or examining
Severe jitteriness :
IDM, HIE, narcotic withdrawal, hypocalcemia,
hypoglycemia
Benign neonatal sleep myoclonus Bilateral, symmetric myoclonic jerks of the arms
and/or legs, during non-REM sleep in the first few weeks of life and resolve by 2-3months
Absence of autonomic disturbances
EEG , neurologic exam & development : normal
Sleep myoclonus will cease when the baby is aroused
Neonates with severe cerebral dysfunction can also have myoclonic jerks, but these typically occur with a stimulus, or upon wakening or falling asleep
SyncopeAn abrupt loss of consciousness due to sudden in
cerebral perfusion ( brief tonic contraction in muscles of face , trunk, extremities in 50 % or UWG )
Causes : vasovagal (painful procedures, blood drawing warm setting),cardiac arrhythmia, HV & panic attack , Reflex syncope (micturition or cough, carotid sinus hypersensitivity)
Predisposing situation , accompanying pallor, nausa , perspiration , prodromal lightheadedness and visual changes, lack of postictal state , short duration
EEG : transient slowing in attack , CPK in seizure
Tilt table testing can provoke vasovagal syncope
Breath-holding spells In 5 % of children, rare in <6mo and >5-8 yr
80% <18mo , all in <3yr
Iron deficiency is more prevalent in BHS
A positive FH is present in 20-35%
An autosomal dominant trait in some families
The two clinical types of BHS:
Cyanotic
Pallid
Family members and individual children can demonstrate both types, usually one predominates
Cyanotic BHS Crying, prolonged expiratory apnea, cyanosis, UWG,
followed by limpness and loss of consciousness
Decorticate or decerebrate posture in prolong BHS
A few children have generalized tonic or clonic seizures , prolonged postictal unconsciousness
15 -25 % have multiple episodes daily ,most children have one to six spells per week
Treat : reassurance
Ferrous sulphate :5-6 mg/kg/ day
Piracetam, a GABA-derivative
Pallid BHS It is less common than the cyanotic variety
The event is caused by cardiac bradycardia
Occur after a minor fall or blow to the head or upper body, then child stops breathing and becomes pale, diaphoretic, and limp
May followed by opisthotonos, incontinence & low amplitude clonus , confusion or sleepy for several minutes afterward.
Treat: in severe attacks with prolonged, severe bradycardia or asystole, atropine and cardiac pacing
Benign myoclonus of infancy
Known as benign nonepileptic infantile spasms
Clusters of spasms at mealtime at 3-8 months
Clusters increase in intensity and severity over weeks or months and then remit spontaneously at two to three years of age
EEG, neurologic exam , development : normal
An anticonvulsant is not indicated
Without subsequent epilepsy
Masturbation In girls ( 2 mo – 3 yr ) , in boredom or stress
Episodes of tonic posturing with copulatory movements without manual stimulation of genitalia
Suddenly flushed & perspires , may grunt & breathe irregularly without loss of consciousness
Persists for a few minutes ( rarely hours )
Search for evidence of sexual abuse or abnormality of perineum
Subside by 3 yr of age
No specific therapy is required
Sandifer syndrome Intermittent paroxysmal spells of generalized
stiffening & opisthotonic posturing with feedings (within 30 minutes following a meal) in infants due to gastroesophageal reflux
These spells may be associated with apnea, staring, and minimal jerking of the extremities
Can be seen in normal children or children with hypotonia and tracheomalacia
Treatment of GER reduces the frequency and severity of attacks.
Benign paroxysmal torticollis in infancy Periodically, head tilts to one side, with the face
rotated toward the opposite side with pallor , agitation & vomiting , begin and end suddenly, with a duration between a few hours and a few days. The child is alert and responsive during an attack.
Episodes usually first occur in the first 3 months of life and resolving by the age of 3 years
EEG, neurologic exam , development : normal,
A FH of migraines is common , may develop migraine later in life
Benign paroxysmal vertigo (BPV) In toddlers ,sudden attacks of ataxia, ± nystagmus,
diaphoresis, nausea, and vomiting , without altered consciousness, the child appears frightened and pale
Episodes : <1minute,clusters: daily for several days
then remitting for several weeks & recurring again
Usually resolves by 3-5yr, may develop migraine several years later
BPV remains a diagnosis of exclusion.
Treat in clusters of attacks : Diphenhydramine 5mg/kg/day PO, IM ,IV, rectal
Shuddering attacks Brief episodes a rapid tremor of the head, shoulder, and
trunk as "shiver" from a chill in 4-6 mo
Episodes last a few seconds, can occur multiple times a day (100 times ) and occur with feeding ,or when child is excited or distressed
They never occur during sleep and virtually never when being held and cuddled
EEG ,Neurologic exam , development: normal
FH of benign essential tremor exists
The spells spontaneously resolve by the second decade without treatment
Nonepileptic staring spells or pseudoabsences In MR, ADHD, autism & in normal children
Spells in boredom or inactive (watching TV or sitting in a class)
Staring interrupted by tactile or vocal stimulation (but usually not hand-waving )
HV in young children :blow out imaginary candles
In HV , Video-EEG show generalized HVSW in the healthy child, but no epileptiform activity. An inexperienced electroencephalographer may confuse this striking slow activity, "build-up" with the paroxysmal activity of an absence seizure.
Dystonia An abnormal posture due to sustained
contraction of both the agonist and antagonist muscle groups
A dystonic posture may be generalized or focal: examples include opisthotonic posturing, torticollis, oculogyric crisis , facial spasm
A common etiology of dystonia in infants is an acute reaction to drugs such as : metoclopramide ,phenothiazines , haloperidol
In infants : generalized
In children: face and trunk