Imitators of Epilepsy - mc.vanderbilt.edu of... · Epilepsy differential diagnosis - The...
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Imitators of Epilepsy
Nabil J. Azar, M.D. Vanderbilt University Medical Center
Nashville, Tennessee
Disclosure
I have no financial relationships to disclose that are relative to the content of my
presentation.
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.
Outline
• Physiologic non-epileptic imitators:
- Cardiovascular - Syncope - Migraine - Sleep disorders - Movement disorders - Endocrine
• Non-physiologic nonepileptic imitators:
- Psychiatric - Convulsive
psychogenic attacks - Nonconvulsive
psychogenic attacks
• Paroxysmal disorders in infants and children
Imitators of epilepsy in infants Sleep:
- Head banging: duration of 15-30 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 5-8 - 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)
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
Cerebrovascular imitators • Transient ischemic attacks (TIA) - vertebrobasilar > anterior circulation • Transient global amnesia (TGA):
- confusion and amnesia - vascular>migrainous>epileptic
• Drop attacks: with/without loss of conscioussness or postictal state - compressive brainstem vascular disturbances - cervical cord diseases - colloid cysts
Hodges J. WB Saunders 1991
Lesser R. et al. Epilepsia 1985
Meissner I. Neurology 1985
Syncope: common features • Sudden • Unpredictable • Recurrent loss of consciousness • Common disorder (10%) • “Convulsive ” myoclonus • Stereotyped • Witness panic • Potential serious injury • High morbidity and mortality
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
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
Signs and Symptoms: seizure vs. 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
• 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
Syncope Seizure
Prodrome:
Onset:
Vital functions:
Position:
Motor activity:
Recovery:
Incontinence:
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
Diagnosis of syncope
Diagnostic testing: • Long-term video-EEG monitoring • Routine EEG • ECG / holter monitoring • Tilt table test / orthostatic blood pressure • Carotid ultrasound
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
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
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
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
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
Parasominas vs frontal lobe seizures
Carreno M. Seminars in Neurology 2008
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
Paroxysmal choreoathethosis
Myoclonus • Physiologic: Hypnic jerks,
sleep-related, cough-induced, 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
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
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
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
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
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
Ictal fear vs. panic attack
Carreno M. Seminars in Neurology 2008
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
Psychogenic Seizures - Patterns
• Generalized motor activity 66 • Migratory motor activity 93 • Unilateral motor activity 13 • Altered responsiveness only 12 • Collapse 8
Moore D. et al. J. Epilepsy 1998
192 patients with confirmed diagnosis of psychogenic nonepileptic seizures: witnesses survey
89 %
11 %
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
Hypermotor seizure
Coexistence of psychogenic and epileptic seizures
• 50-58 %: including staring or unresponsive spells.
• 30-45 %: based on clinical impression.
• 18-20 %: confirmed by long-term video-EEG.
• 16-20 %: confirmed by long-term video-EEG.
Holmes M. et al. Epilepsia 1993
Cohen B. et al. Seizure 1997
Lesser T. Epilepsy & Behavior 2004
Sutter M. et al. Epilepsia 2002
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
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
Distinguishing ictal features
Azar N. et al. Epilepsia 2008
GTC PNES FLHS Eyes open 91 % (p<0.05) 27 % 67 %
Vocalization 54 % 62 % 41 % Asynchronous
movement 9 % 96 % (p<0.01) 90 % (p<0.05)
Side to side head/body
5 % 63 % (p<0.05)
76 % (p<0.01)
Mean ictal duration (sec)
42 185 (p<0.05)
34
GTC: generalized tonic-clonic seizures, PNES: psychogenic nonepileptic seizures, FLHS: frontal lobe hypermotor seizures
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 < 0.001 for all features
Azar N. et al. Epilepsia 2008
Postictal breathing- epileptic
Postictal breathing- nonepileptic
Injury and incontinence
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
Telephone survey to witnesses and families
Facial muscle involvement
• 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
Video-EEG review and witnesses’ survey of: - 654 epileptic events observed in 257 patients
- 457 nonepileptic events observed in 159 patients
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
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
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
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
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