8/25/2017 Misdiagnois NOT Uncommon Differential Diagnosis of SEIZURES Epilepsy Epilepsy Reasons for referral confirmed NOT confirmed Doubt about diagnosis 7 (18%) 32 (82%) Lunliya Thampratankul, MD No doubt about Division of Neurology, Department of Pediatrics 129 (70%) 55 (30%) diagnosis Ramathibodi Hospital, Mahidol University Misdiagnois NOT Misdiagnois NOT Uncommon Uncommon Diagnosis No. Staring episodes Mental retard 22 Autistic 4 Learning disorder 3 Self stimulation 2 Abnormal EEG 7 Normal child 8 Psychogenic non epileptic 9 Syncope 4 Dystonia 4 Parasomnias 4 Hyperventilation 3 Migraine 3 Breath holding spells 2 Munchhausen by proxy 2 Other 4 Not defined 6 Misdiagnois NOT Evaluation of Seizure Uncommon Hardly anyone with epilepsy will come to any harm • อาการทีเกิดขึนเป็นอาการชักหรือไม่ from a delay in diagnosis whereas a false positive diagnosis is gravely damaging. ประวัตจิ ากผู้เหน็ เหตุการณ์โดยละเอียด • Diagnosis of epilepsy lead to significant การตรวจร่างกาย psychological consequences of patients and families. Preictal Postictal • The underlying disease may not receive properly treatment. • Some cardiac cause carry a considerable mortality Ictal risk ปัจจัยกระตุ้น เวลาและสถานที กิจกรรมทีทาํ ลําดับเหตุการณ์ 1 8/25/2017 Differential Diagnosis of Seizure • Syncope PNEA • Jitteriness Paroxysmal Non-Epileptic Attack • Arrhythmia (Psychogenic/pseudoseizure) • Physiologic myoclonus • GERD • Hyperventilation • Breath holding spell • Sleep disorders • Benign paroxysmal • Migraine vertigo • Metabolic disorders • Cyclic vomiting • Hypoglycemia • Movement disorders • TIA • Hyperekplexia • Psychogenic seizure Jerky movement Neonatal sleep myoclonus อาการกระตุก Neurological • Myoclonic jerks เกิดขึนเฉพาะ ช่วงหลับ • Seizure: • Bilateral, synchronous, repetitive • Clonic seizure • Distal part of upper extremities • Myclonic seizure • Epileptic spasms • Unilateral, isolated, transition from one limb to • Abnormal movement: the others • Tics • พบได้ในเด็กปกติ อาการดีขึนเมื อโตขึน • Hypnic jerk, sleep myoclonus • Opsoclonus-myoclonus syndrome • ไม่สมั พนั ธ์กบั ชกั อาจสมั พนั ธ์กบั ไมเกรนตอนโต Other: • ข้อสงั เกต: ถ้าพบในเด็กทีมีปัญหาซมึ หรือ เกิดในขณะตืน ระวงั ภาวะ • Jitteriness ชกั Jitteriness vs Jitteriness Neonatal Seizure • อาการสัน (rhythmic tremor)ของแขนขา Seizure Jitteriness • Triggered by stimulation; loud noise External stimulus can No Yes • Stopped by passive movement (flex) of initiate limbs, initiate baby’s sucking Symmetrical fine Movements Irregular, jerky • No autonomic change tremor • Hyperexciatbility Associated HR Yes No • พบใน ภาวะปกติ hypocalcemia, hypoglycemia, Associated apnea ± No maternal drug withdrawal Movements can be No Yes easily stopped Self limited Bending/holding 2 8/25/2017 Bobble-head doll Opsoclonus-myoclonus- syndrome ataxia syndrome • Repetitive antero-posterior head movements, 2-3 • Opsoclonus: multidirectional conjugate eye Hz frequency movement with horizontal, vertical and torsional • Disappear or attenuate on volitional activities directions • Often associated with trunkal tremor • Difficulty walking, behavioral change • Associated with lesions in the 3rd ventricular • Autoimmune, inflammatory reaction, region: suprasellar cyst, aqueductal stenosis, etc paraneoplastic, infectious • 50% of children associated with occult neuroblastoma • Small cell lung CA, breast adenoCA • Rx immunotherapy Subacute sclerosing Hypnic Jerk panencephalitis (SSPE) • Hypnagogic jerk, Sleep start • Dawson disease, measles encephalitis • @ beginning to fall asleep • 1: 10,000 measle cases • “jump with a falling sensation” might lead to • 6 years after measle infection arousal • Behavior change, irritability dementia-like myoclonus intellectual decline loss of motor • Post-sleep deprived, irregular sleep control of walking, speaking, swallowing coma schedule, caffeine, stress • Dx: CSF and blood specific IgG, CSF PCR • DDx: Periodic limb movement disorders of sleep (might related with RLS, insomnia) SSPE-EEG Epileptic spasms • West syndrome Triad: (1) Epileptic spasms (2) Developmental delay (3) Hypsarrhythmia • Onset: early infancy, peak 4 - 6 mo. • Etiology: HIE, structural brain defect, TSC, Down syndrome, inborn error of metabolism • Need urgent evaluation 3 8/25/2017 Tonic posturing Hyperekplexia อาการเกร็ง Startle syndrome Neurological: Non-neurological: • Pronounced startle responses to tactile or acoustic • Seizure . Gastroesophageal stimuli and hypertonia • ICP: decorticate, reflux • Generalized stiffness (limb extension), forced eye decerebrate posturing . Breath holding spells closure falling • Dystonia . Hyperventilation • Episodic neonatal apnea sudden infant death syndrome (SIDS) • Hyperekplexia syndrome . Gratification disorder • “NOSE TAPPING” (glabellar tapping) (infantile • Glycine, AD AR X-linked: GLRA1 (glycine receptor masturbation) alpha 1 subunit), GLRB, SLC6A5, GPHN, ARHGEF9 . Shuddering attacks • Rx clonazepam Breath holding spells Gratification disorder Infantile masturbation • Prolonged expiratory apnea • Age onset: 3 mo-3 yr (upto 5 yr), mean 10.5 mo • Provocation (pain, fear, upset) crying • Stereotype episodes of variable duration noiseless pause with an open mouth and • Vocalization with quiet grunting change in color • Facial flushing with diaphoresis loss of consciousness and postural tone • Pressure on the perineum with the characteristic post anoxic seizure posturing of lower extremities (e.g. rocking with crossed legs) • Less than 1 min • No LOC, cessation with distraction • Age 6-18 mo • Rx reassure, Rx iron deficiency Shuddering Paroxysmal dyskinesia • Shivering episode mainly involve head, neck, • Paroxysmal kinesiogenic dyskinesia shoulder and occasionally trunk (PKD) “ice cube was dropped down their back” • Paroxysmal non-kinesiogenic dyskinesia • No impairment of consciousness; staring but (PNKD) alert • Intermittent + hyperkinetic movement • Abrupt onset, 5-15 seconds (chorea/dystonia) • Infancy to early childhood • Abnormal movement, good conscious • Precipitated by fear, anger • PKD: Rx low dose carbamazepine 4 8/25/2017 Dystonia Catatonia • Abnormal movement • Psychogenic motor immobility • Awkward, unusual, painful posture • Unresponsiveness to external stimuli in a • Focal/segmental/hemi/generalized person who is apparently awake • Etiology: • Associated with psychological condition, • Drugs (extrapyramidal side effect) autoimmune disorder, alcohol & • Autoimmune benzodiazepine withdrawal, neuroleptic • Wilson disease malignant syndrome • Structural brain • S&S; mutism, negativism, echopraxia, • Genetic,etc echolalia, waxy flexibility, withdrawal • Rx benzodiazepine, underlying disease Loss of tone Narcolepsy with ตัวอ่อน ล้มบ่อย cataplexy Neurological • Excessive daytime sleepiness • Seizure (drop attacks): atonic seizure, • Cataplexy: sudden loss of tone myoclonic seizure precipitated by emotion • Cataplexy • Sleep paralysis Non-neurological • Sleep onset/offset hallucination • Syncope: vasovagal, cardiogenic • Inv: Overnight polysomnography + • Anaphylactic shock multiple sleep latency test, MRI brain Loss of consciousness Syncope vs Seizure ภาวะหมดสติ Neurological Syncope Seizure Decreased cerebral Abnormal electrical • Generalized seizure Mechanism • Stroke blood flow activity Position, state Upright, awake Any, awake-sleep Non-neurological Dizziness, blurry Aura: motor/sensory/ • Syncope Preictal vision, sweating, visual/ déjà vu . Vasovagal palpitation . Cardiogenic-arrhythmia, aortic stenosis Onset of LOC Gradual Sudden • Hypoglycemia Duration 10-20 sec Minutes Eyes closed, Eyes opened, • Intoxication Eyes (up rolling) eye deviation • Anaphylaxis 5 8/25/2017 Nocturnal Syncope vs Seizure paroxysmal events Syncope Seizure • At sleep onset: Facial color Pale Cyanosis • Rhythmic movement disorder of sleep Twitching Rare, < 15 sec Frequent • During early sleep Enuresis Rare Yes • NonREM parasomnias Tongue biting No Yes • Sleep terrors, confusional arousal, sleep walking Amnesia Yes Yes • During late sleep Postictal Brief somnolence Confusion, headache, • REM parasomnias: Nightmare symptoms Rapid recovery focal neuro deficit • Precipitating: fear, Any time in sleep: sleep myoclonus, seizure Note stress, anxiety, pain, All events stereotype sleep deprivation Sleep related Rhythmic Parasomnia Movement Disorder • Occur while drowsy or falling asleep • นอนละเมอ • Body rocking, head banging (Jactatio captitis • Sleep talking, sleep terrors, sleep walking, nocturna), head rolling confusional arousal • Body rolling, leg rolling, leg banging • เป็นภาวะปกติและพบบ่อยในเด็ก ส่วนในผู้ใหญ่พบไม่บ่อย ควรต้องหา • Normal infant, children with intellectual สาเหตุ disability • มกั เกิดในช่วง 2-3 ชัว โมงแรกหลงั จากเริมหลบั • Family concerns • เกิดได้ 1-3 ครังต่อคืน • ปัจจยั กระต้นุ ได้แก่ อดนอน ยานอนหลบั เจ็บป่วย ความเครียด Management of Parasomnia and Nocturnal Parasomnia Frontal Lobe Epilepsy NREM parasomnia NFLE • REASSURE Age at onset Usually < 10 yrs Variables • Sleep hygiene, adequate amount of sleep (+) Family Hx 60-90% Up to 40% • Manage triggering factors • Noise and light stimuli contribute to partial arousal, Attacks per night 1 or 2 3 or more extreme exercise, stress Episode per month < 1 -4 20 -40 • antihistamine, alcohol, antidepressant, sedative Clinical course Tends to disappear by Often stable with • Environmental safety adolescence increasing ages • Search for and treat, if present, other sleep disorder, Episode duration Secondsto 30 min Seconds to 3 min (often such as OSA < 2 min) Semiology of Variable complexity, Highly stereotyped • Clonazepam for