Imitators of Epilepsy Nabil J. Azar, M.D.

Transcription

1 Imitators of Epilepsy Nabil J. Azar, M.D. Vanderbilt University Medical Center Nashville, Tennessee

2 Disclosure I have no financial relationships to disclose that are relative to the content of my presentation.

3 Epilepsy differential diagnosis - The differential diagnosis of epileptic seizures is very broad, partly because the symptomatology of epileptic seizures is varied, depending on the eloquent cortical areas activated by the epileptic activity. - There is a great amount of diseases which can produce focal neurological symptoms and signs, occurring repeatedly in a paroxysmal way, not unlike epileptic seizures, and which can be mistaken for epilepsy. - To make a definite diagnosis of epilepsy, it is necessary to demonstrate the abnormal ictal activity associated with the recurrent attacks.

4 Outline Paroxysmal disorders in infants and children Physiologic nonepileptic imitators: - Cardiovascular - Syncope - Migraine - Sleep disorders - Movement disorders - Endocrine Non-physiologic nonepileptic imitators: - Psychiatric - Convulsive psychogenic attacks - Nonconvulsive psychogenic attacks

5 Imitators of epilepsy in infants Sleep: - Head banging: duration of min in transition to sleep, until the age of 1 - Benign neonatal myoclonus: usually not stimulus-induced, stops upon awakening - Infant apnea:? related to sudden infant death syndrome Wakefulness: - Jitteriness: mostly stimulus induced - Benign myoclonus of early infancy - Spasmodic torticollis : idiopathic or related to GE reflux (Sandifer syndrome) - Spasmus nutans: head nodding, head tilt and nystagmus, outgrown by age of Hyperexplexia or startle disease: rare familial disorder with variable prognosis - Shuddering attacks: excessive shivering,? familial, outgrown by age of 10 - Pallid syncope: due to transient asystole (tonic contractions)

6 Imitators of epilepsy in children Sleep: - Myoclonus - Hypnagogic paroxysmal dystonia - Parasomnias: nightmares or terrors or sleep walking Wakefulness: - Cyanotic breath holding spells: precipitated by fear or mild injury - Tics: disappear in sleep, may be complex (Tourette s syndrome) - Recurrent abdominal pain: vomiting, palor, often with headaches (15 % will have epilepsy) - Confusional migraines: lasting for hours - Munchausen syndrome by proxy

7 Cerebrovascular imitators Transient ischemic attacks (TIA) - vertebrobasilar > anterior circulation Lesser R. et al. Epilepsia 1985 Transient global amnesia (TGA): - confusion and amnesia - vascular>migrainous>epileptic Hodges J. WB Saunders 1991 Drop attacks: with/without loss of conscioussness or postictal state - compressive brainstem vascular disturbances - cervical cord diseases - colloid cysts Meissner I. Neurology 1985

8 Syncope: common features Sudden Unpredictable Recurrent loss of consciousness Common disorder (10%) Convulsive myoclonus Stereotyped Witness panic Potential serious injury High morbidity and mortality

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11 Factors causing syncope Decreased cardiac output: Asystole Tachy-arrhythmias Brady-arrhythmias Heart failure Decreased blood volume Decreased total peripheral resistance Hypotension Any combination of the above

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13 Classification of syncope types by precipitating cause: Reflex or vasovagal syncope (common faint): loss of vasomotor tone Respiratory syncope: rise in intrathoracic pressure impairing cardiac venous return (coughing, weight lifting) Cardiac syncope: rhythm abnormalities Obstruction of cardiac output Vascular: hypotension Areflexic or paralytic: neuropathic, drugs, trauma

14 Signs and Symptoms: seizure vs. syncope Prodrome: Onset: Vital functions: Position: Motor activity: Recovery: Incontinence: Syncope Common, light headedness, sweating, pallor, dimming vision Gradual onset and progression Depressed pulse, respiration and heart rate Usually occurs in upright position or upon exertion Motionless or limited clonic or myoclonic Usually prompt Less common Seizure Less common, very brief aura Sudden and quick Rapid heart rate, elevated blood pressure Occurs in any position Prominent tonic, clonic or myoclnic, automatism Slow More common

15 Syncope-induced myoclonus Incidence: up to 90 % of all syncopal attacks More common in younger adults Multifocal > unifocal Unilateral or bilateral No prognostication for recovery except in anoxic injury

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18 Diagnosis of syncope Diagnostic testing: Long-term video-eeg monitoring Routine EEG ECG / holter monitoring Tilt table test / orthostatic blood pressure Carotid ultrasound

19 Migraines and epilepsy Common neurological disorders Could trigger each other Coexists in about 25 % of patients Relationship between migraine and epilepsy? Overlap syndromes: - Benign occipital epilepsy - Migrainous and convulsive hemiplegia - Mitochondrial encephalopathy Barolin G. Epilepsia 1966 Panayiotoupoulos C. Neurology 1980

20 Migraine and epilepsy: common features GI upset Flushing and autonomic symptoms Vertiginous disequilibrium Complex visual hallucinations Uncinate (smell/taste) auras Paroxysmal and transient focal neurological deficits Provoked by hormonal changes Occasional CSF abnormalities Abnormal EEG during attacks (slow activity, sharply contoured waves, posterior sharps for hours or days) Response to anticonvulsants Comorbidities: anxiety and depression Positive family history Anderman E. et al. Butterworths 1987

21 Migraine and epilepsy: distinguishing features Favor migraines: Recurrent headaches Photophobia Scotoma Simple visual hallucination Provoked by diet Response to anti-migraine medications Gradual onset Prolonged duration Absence of postictal state Favor epilepsy: Partial motor manifestations Generalized tonic-clonic events Nocturnal occurrence Photic/HV provocation Response to anticonvulsants Sudden onset Short duration Presence of postictal state Rapoport A. Neurology 1992

22 Atypical migraines Acephalgic migraine: controversial Basilar artery migraine: vertigo, ataxia, change in level of consciousness Hemiplegic migraine Postictal migraine epileptic cephalgia Migraine-triggered seizures Cluster headaches (autonomic manifestations ) Niedermeyer E. Clin Electroencephalogr 1993 Bickerstaff E. Lancet 1961

23 Sleep conditions imitating epilepsy REM behavior disorder Nightmares Night terror Sleep walking (somnanbulance) Period leg movements of sleep Bruxism Cataplexy Sleep paralysis Daytime sleep attacks

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26 Parasominas vs frontal lobe seizures Carreno M. Seminars in Neurology 2008

27 Movement disorders and epilepsy Chorea and athetosis Ballismus Paroxysmal ataxia Dystonia Tics and Tourette s syndrome Paroxysmal dystonic choreoathetosis (min-hours, not precipitated by mvt) Paroxysmal kinesigenic choreoathetosis (sec-min, precipitated by mvt) Blepharospam Hemifacial spasm Tardive dyskinesia Akathisia Cramps and spasms Stiff person Myoclonus Asterxis Tremor Startle disease (hyperekplexia) Restless leg syndrome

28 Paroxysmal choreoathethosis

29 Myoclonus Physiologic: Hypnic jerks, sleep-related, coughinduced, exercise or anxiety related Essential: hereditary or sporadic Infectious: SSPE, CJD, postviral Metabolic: uremic, hepatic, alcohol-related, toxic (AEDs, l-dopa etc) Neoplastic: basal ganglia Cerebrovascular: CVA, anoxic.. Degenerative d/s: Wilson s, HVZ, Alzheimer, Huntington, PD Storage diseases: Gaucher, Tay-Sachs Epileptic: - Non-progressive: idiopathic or symptomatic - Progressive: progressive myoclonic epilepsies Fahn S. et al. Adv Neurol 1986

30 Movement disorder side effects of AEDs Phenytoin: dystonia, asterexis, chorea, dyskinesia, ballismus Phenobarbital: dystonia, tics Carbamazepine: orofacial dyskinesia, myoclonus, dystonia Valproate: asterexis, tremor (parkinsonism) Ethosuximide: dyskinesia Lamotrigine: Oculogyric crisis Karas B. et al. Neurology 1983 Chadwick D. et al. J Neurol Neurosurg Psych 1976 Joyce R. et al. Neurology 1980

31 Endocrine imitators of epilepsy Hypoglycemia (double vision, confusion, odd behavior, slurred speech, tremor, weakness, vertigo, anxiety, ataxia, palpitation.true seizures) Hyperglycemia (altered consciousness but very gradual true focal seizures) Hyponatremia / hepernatremia Hypocalcemia: mental status changes, carpopodeal spasm Hyperthyroidism (Hashimoto s) / hypothyroidism Pheochromocytoma/paragangliomas (syncope, flushing, epigastric sensation) Carcinoid (serotonin releasing tumors) Mastocytosis (mainly histamine release) Acute porphyria (heme synthesis disturbances) The majority of the above conditions may also provoke epileptic seizures

32 Vertigo and epilepsy Dizziness is nonspecific and seldom associated with epilepsy Dizziness is very common in patients with epilepsy (AED side effects) Vertigo: - Peripheral: BPPV, Menier s, acoustic neuroma - Central: CVA, migraine, MS Hughes J. et al. Dis Nerv System 1983

33 Psychiatric imitators of epilepsy Coversion disorder Delirium Panic disorders Dissociative disorders (alteration in memory, behavior or identity) Schizophrenia Delusional disorders Mutism Mania Major depressive episode

34 Delirium and epilepsy: common features Critically-ill patients in whom non-convulsive status epilepticus is often under diagnosed Fluctuating consciousness, inappropriate behavior and subtle motor manifestations (absence, complex partial seizures) Similar causes: intoxication (including AEDs), organ failure, electrolytes imbalance, CVA, intracranial infections Preictal, ictal and postictal delirium The electrographic distinction maybe challenging because of nonspecific EEG patterns that could be interpreted as encephalopathic, postictal or ictal. Lipowski Z. Oxford Press 1990

35 Ictal fear vs. panic attack Carreno M. Seminars in Neurology 2008

36 Psychosis and epilepsy Ictal psychosis Postictal psychosis: may be delayed 1-7 days Interictal psychosis: - Prolonged or brief - Forced normalization - Not correlated with seizure frequency or epilepsy duration - May develop at anytime Chronic psychosis in epilepsy epileptic schizophrenia : unknown relationship especially that these two disorders are thought to be antagonistic (ECT) Geschwind syndrome: in patients with temporal lobe epilepsy, characterized by sexual behavioural disorders, hyper-religiosity, hypergraphia and viscosity (interictal>ictal) ECT and seizures McKenna P. et al. Am J. Psychiat 1985

37 Psychogenic Seizures - Patterns 192 patients with confirmed diagnosis of psychogenic nonepileptic seizures: witnesses survey Generalized motor activity 66 Migratory motor activity 93 Unilateral motor activity 13 Altered responsiveness only 12 Collapse 8 89 % 11 % Moore D. et al. J. Epilepsy 1998

38 Frontal lobe complex partial seizures Stereotyped pattern Frequent seizures, often in clusters Brief seizures, under 1 minute Bizarre attacks that appear hysterical Prominent motor automatisms, usually complex Aggressive sexual automatisms Vocalizations, with variable complexity Short postictal period, rapid clearing Complex partial status epilepticus common Williamson J. et al. Annals of Neurology 1995

39 Hypermotor seizure

40 Coexistence of psychogenic and epileptic seizures %: including staring or unresponsive spells %: based on clinical impression. Holmes M. et al. Epilepsia 1993 Cohen B. et al. Seizure %: confirmed by long-term video-eeg. Sutter M. et al. Epilepsia %: confirmed by long-term video-eeg. Lesser T. Epilepsy & Behavior 2004

41 Discriminating ictal features of nonepileptic seizures 1- Out of phase upper extremity movements 2- Out of phase lower extremity movements 3- No vocalization, or vocalization at onset 4- Forward pelvic thrusting 5- Absence of whole body rigidity 6- Side to side head movements Gates J. et al. Arch Neurology 1985

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43 Psychogenic monepileptic attacks: red flags Multiple types of attacks Changing types of attacks Prolonged and repetitive attacks Atypical auras (headache, numbness, chest tightness, dizziness ) Variable degree of responsiveness Variable degree of awareness and memory Poor response to antiepileptic drugs Beware of : - pseudosleep - pseudo-pseudoseizures - pelvic thrusting Bendabis S. et al. Epilepsy Research 2005

44 Distinguishing ictal features GTC PNES FLHS Eyes open 91 % (p<0.05) 27 % 67 % Vocalization 54 % 62 % 41 % Asynchronous movement Side to side head/body 9 % 96 % (p<0.01) 90 % (p<0.05) 5 % 63 % (p<0.05) 76 % (p<0.01) Mean ictal duration (sec) (p<0.05) 34 GTC: generalized tonic-clonic seizures, PNES: psychogenic nonepileptic seizures, FLHS: frontal lobe hypermotor seizures Azar N. et al. Epilepsia 2008

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46 Postictal breathing pattern Epileptic: - Deep - Loud with snoring - Regular - Prolonged inspiratory and expiratory phases - Duration 1-5 minutes Nonepileptic: - Shallow - Quiet - Irregular with brief pauses - Short inspiratory and expiratory phases - Duration 1< minute p < for all features Azar N. et al. Epilepsia 2008

47 Postictal breathing- epileptic

48 Postictal breathing- nonepileptic

49 Injury and incontinence Telephone survey to witnesses and families Epileptic seizures: - 38 patients - 48 % tongue biting - 54 % urinary incontinence - 48 % injuries - Burns, bone fracture more common (p<0.05) Nonepileptic seizures: - 73 patients - 44 % tongue biting - 45 % urinary incontinence - 40 % injuries - Suicide attempts more common (p<0.05) Peguero E. et al. Epilepsia 1995

50 Facial muscle involvement Video-EEG review and witnesses survey of: epileptic events observed in 257 patients nonepileptic events observed in 159 patients Open eyes recorded in 90 % of epileptic events in the tonic phase Forceful eye closure recorded in 60 % of nonepileptic events Tongue biting on the side reported in 54 % of patients with epileptic seizures Tongue biting at the tip reported in 44 % of patients with nonepileptic seizures DeToledo J. et al. Neurology 1996

51 Nonepileptic seizures: suggestive features Atypical auras Pre-ictal behavior changes Gradual onset Pseudo-sleep at onset Eye closure during unresponsiveness Eye fluttering Discontinuous seizure activity Gradual cessation Non-physiologic progression Absence of postictal state High seizure frequency Excessive variability in seizure manifestations Funny vocalization Resistance to eye opening Prolonged duration Occurrence during clinic visit Suggestibility Precipitation of typical attacks by suggestion Attacks only in the presence of others Vocalizations consisting of gagging, retching, gasping, screaming, crying or moaning Emotional display during events Emotional triggers Retained consciousness and recollection of events with bilateral jerking activity

52 Questions 1: In neonates, which of the following seizure imitators includes abnormal eye movements? A- Hyperekplexia B- Spasmus nutans C- Sandifer s syndrome D- Pallid syncope

53 Questions 2: Which of the following is most likely to occur with syncope? A- Multifocal myoclonus B- Urine incontinence C- Postictal confusion D- Prolonged postictal state

54 Questions 3: All is true about frontal lobe seizures except: A- Often nocturnal B- Tend to cluster C- Rarely include motor manifestations D- Brief in duration

55 Questions 4: Which is least likely to occur in psychogenic nonepileptic seizures? A- Ictal eye closure B- Side-to-side movements C- Stertorous post-ictal breathing D- Asynchronous movements