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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. Definition of seizure :

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Paroxysmal disorders:

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  1. Paroxysmal disorders: Abrupt onset of a clinical episode that tends to be stereotypedand repetitive , 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.

  2. Seizures Migraine TIA Syncope Breath holding spells Paroxysmal Vertigo hypoglycemia Tics Pseudo seizures Night terrors Narcolepsy Paroxysmal torticollis Paroxysmal dystonia Differential diagnosis of Paroxysmal disorders:

  3. 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

  4. Age of onset Etiology Seizure type Cognitive development Neurologic exam ,CT prognosis Janzsyndrom West syndrom Lennox-Gastaut syn. BPEC LKS Benign neonatal convulsion Epileptic syndromes classification

  5. Clinical Seizure classification - Simple (Normal consciousness) - Complex (Impaired consciousness) Partial (Only a portion of the brain) Generalized (Both hemispheres are involved)

  6. Type of Seizure

  7. 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

  8. Classification • Generalized Seizures • Absence Seizures (Petit mal) • Tonic/Clonic (Grand mal) • Atonic Seizures (Drop attacks) • Myoclonic Seizures • Clonic • tonic

  9. 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.

  10. 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

  11. 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.

  12. 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

  13. 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

  14. 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 %

  15. 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

  16. 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 .

  17. 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

  18. 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

  19. 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

  20. Rasmussen encephalitis:chronic , progressive focal inflammation of the brain 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

  21. 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

  22. 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

  23. Often referred as behavior problem,Poor school marks; disruptive behaviorSide 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

  24. 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

  25. 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

  26. 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 , cortone ( irritability , swelling ,hypertension , glycosuria, severe infection ) benzodiazepines , valproate

  27. 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 ) Etiologies of infantile spasms

  28. 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 : آمينوفيلين ،آنتي دپرسانت ،آنتي كولينرژيك،فنوتيازين ليتيوم ، ناركوتيك ، آمفتامين ، ساليسيلات ،ايزونيازيد

  29. Etiology of seizures Neurocutaneous syndromes : Tuberous sclerosis , NF,… • Systemic disorders : vasculitis ( CNS or systemic ) SLE hypertensive encephalopathy renal failure hepatic encephalopathy Infection:انسفاليت، مننژيت ، آبسه مغزي Other : trauma , tumor, idiopathic , familial Over-the-counter drugs, illicit substances herbal preparations, can precipitate seizure

  30. 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 Laboratory evaluation of seizures

  31. 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

  32. 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

  33. 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

  34. 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 factors of recurrence of FC

  35. 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

  36. ريسك فاكتورهاي احتمال مننژيت 1. ويزيت در 48 ساعت گذشته 2. تشنج در بدوورود به اورژانس 3. تشنج فوكال 4. معاينه عصبي غير طبيعي ا نديكاسيون ا نجام LP 1. تشنج با تب در زير يك سال 2. وجود لتارژي پرسيستانت 3.اولين تشنج تب كمپلكس 4. در بچه ايي كه قبلا آنتي بيوتيك گرفته و امكان پيگيري نباشد. Meningitis AND FC

  37. 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.

  38. Adverse effects of new AEDs

  39. 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

  40. 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.

  41. . What the common complications of status epilepticus ? • Reduction of CPP • Hyper/hypotension • Dysrhythmeia, CHF, Apnea, Aspiration • Non cardiogenic pulmonary edema, Rhabdomyolysis, • Hypo/hyperglycemia

  42. Status epilepticus classification Clinical • Simple partial • Complex partial • Generalized convulsive • Generalized non-convulsive Etiologic • Symptomatic ( acute or remote) • Idiopathic • Reactive : Prolong FC

  43. 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

  44. Pathophysiology : excessive excitatory neurotransmitters : glutamate , aspartate , acetylcholine Ineffectiveness of inhibition : GABA Neuronal loss with every episode, particularly if prolonged

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