Approach to Acute Confusion, Coma and Seizures

Acute Confusional State & Delirium

Clinical notes on the diagnosis and management of acute confusional states, delirium, and seizures.

Introduction

• Altered Mental Status: Can be divided into:
  • Acute (delirium or acute confusional state).
  • Chronic (dementia).
• Encephalopathy: A subacute organic brain syndrome, a gray zone between delirium and dementia. Its early course may fluctuate but is often persistent and progressive.

Definition of Delirium

• An acute change in mental status characterized by abnormal and fluctuating attention.
• Disturbance in the level of awareness and reduced ability to direct, focus, sustain, and shift attention (Bradleys Neurology 7th edition).

Synonyms for Delirium

• Altered mental status.
• Acute organic syndrome.
• Acute brain failure.
• Acute brain syndrome.
• Acute cerebral insufficiency.
• Exogenous psychosis.
• Metabolic encephalopathy.

Subtypes of Delirium

• Three Subtypes:
  • Hyperactive delirium.
  • Hypoactive delirium.
  • Mixed type.

Hyperactive Delirium

• Characterized by agitation, restlessness, and attempts to remove invasive tools (e.g., drug intoxication).

Hypoactive Delirium

• Characterized by withdrawal, flat affect, apathy, lethargy, and decreased responsiveness.

Mixed Delirium

• The patient's symptoms fluctuate between hyperactive and hypoactive states.

Subsyndromic Delirium (SSD)

• Patients show one or more symptoms of delirium (disattention, disorganized thinking, hallucinations, and delusions) but do not meet the criteria for diagnosing delirium.

Dementia-Superimposed Delirium (DSD)

• An acute change in mental state (characterized by fluctuating course, inability to maintain attention, disorganized thinking, or altered state of consciousness) occurring in a patient already suffering from dementia.

Sundowning Syndrome

• Sudden changes in behavior or mental state.
• May be considered circadian variations of symptoms.
• Associated with the mid-phase of Alzheimer's disease.

DSM-5 Diagnostic Criteria for Delirium

• A. A disturbance in attention (i.e., reduced ability to direct, focus, sustain, and shift attention) and awareness (reduced orientation to the environment).
• B. The disturbance develops over a short period of time (usually hours to a few days), represents a change from baseline attention and awareness, and tends to fluctuate in severity during the course of a day.
• C. An additional disturbance in cognition (e.g., memory deficit, disorientation, language, visuospatial ability, or perception).
• D. The disturbances in Criteria A and C are not better explained by another pre-existing, established, or evolving neurocognitive disorder and do not occur in the context of a severely reduced level of arousal, such as coma.
• E. There is evidence from the history, physical examination, or laboratory findings that the disturbance is a direct physiological consequence of another medical condition, substance intoxication or withdrawal (i.e., due to a drug of abuse or to a medication), or exposure to a toxin, or is due to multiple etiologies.

Pathophysiology of Delirium

• Delirium is the final common pathway of many pathophysiological disturbances that reduce or alter cerebral oxidative metabolism.
• Alteration in cortical brain function with abnormalities of deep brain structures.
• These conditions result from:
  • An exogenous insult or an intrinsic process that affects cerebral neurochemical functioning.
  • Physical or structural damage to the cortex, subcortex, or deeper structures involved with memory.
• End result of these disruptions of function or structure is impairment of cognition that affects alertness, orientation, emotion, behavior, memory, perception, language, praxis, judgment, problem-solving, and psychomotor activity.

How Common is Delirium?

• Delirium is a common behavioral disorder in medical-surgical settings.
• In general hospitals:
  • Prevalence ranges from 15\% to 24\% on admission.
  • Incidence ranges between 6\% and 56\% of hospitalized patients.
  • 11\% to 51\% postoperatively in elderly patients.
  • 80\% or more of intensive care unit (ICU) patients.
• Delirium is 'prevalent' when it is detected at admission and 'incidental' when it develops during the hospital stay.

Multi-factorial Etiology

• Delirium results from an interaction between predisposing factors (vulnerability) and precipitating factors (insults).
  • High Vulnerability + Less Noxious Insult → Delirium
  • Low Vulnerability + Noxious Insult → Delirium

Major Causes of Delirium

• Metabolic.
• Toxic.
• Infectious.
• Neurologic.
• Perioperative.

Potential Causes of Delirium (Differential Diagnosis)

• Infectious: Sepsis, encephalitis, meningitis, syphilis, central nervous system abscess.
• Withdrawal: Alcohol, barbiturates, sedative-hypnotics.
• Acute Metabolic: Acidosis, disturbances, hepatic/renal failure, other metabolic disturbances (glucose, magnesium, calcium).
• Trauma: Head, burns.
• CNS Disease: Hemorrhage, cerebrovascular accident, vasculitis, seizures, tumor.
• Hypoxia: Acute hypoxia, chronic lung disease, hypotension.
• Deficiencies: Vitamin B12, hypovitaminosis, niacin, thiamine.
• Environmental: Hypo/hyperthermia, endocrinopathies, diabetes, adrenal, thyroid.
• Acute Vascular: Hypertensive emergency, subarachnoid hemorrhage, sagittal vein thrombosis.
• Toxins/Drugs: Medications, street drugs, alcohols, pesticides, industrial poisons, carbon monoxide, cyanide, solvents, etc.
• Heavy Metals: Lead, mercury.

Differential Diagnosis of Acute Confusional State/Delirium

Neurological Causes

• Stroke.
• Traumatic brain injury, subdural hematoma.
• Infectious encephalitis/meningoencephalitis.
• CNS vasculitis, limbic encephalitis.
• Granulomatous meningoencephalitis (TB, sarcoidosis).
• Non-convulsive status epilepticus (NCSE).
• Migraine.

Non-Neurological Causes

• Hypoxia.
• Hypoglycemia, hyperglycemia.
• Hyponatremia ("dilution delirium").
• Hypernatremia.
• Hypocalcemia, hypercalcemia.
• Hepatic encephalopathy.
• Adrenal/pituitary insufficiency.
• Uremic encephalopathy.
• Vitamin B1 deficiency (Wernicke's encephalopathy).
• Hyperviscosity syndrome.
• Acute porphyria.

Drugs that can cause Delirium

• Levodopa.
• Dopamine agonists.
• Anticholinergics.
• Antidepressants.
• Chemotherapeutics.
• Intrathecal medications.
• Steroids.

Miscellaneous Causes

• Malignant neuroleptic syndrome.
• Serotonin syndrome.
• Alcohol withdrawal ("delirium tremens").
• Benzodiazepine/barbiturates withdrawal.

Multi-factorial Origin

• Intensive care ("ICU delirium").
• Postoperative.
• Advanced cancer.
• Older patients.

Clinical Characteristics of Delirium

• Acute onset of mental status change with fluctuating course.
• Attentional deficits.
• Confusion or disorganized thinking.
• Altered level of consciousness.
• Perceptual disturbances.
• Disturbed sleep/wake cycle.
• Altered psychomotor activity.
• Disorientation and memory impairment.
• Other cognitive deficits.
• Behavioral and emotional abnormalities.

Diagnostic Difficulty

• Differential diagnosis between Delirium, Dementia, and Psychosis.

Differential Diagnosis Table

Diagnostic Work-Up of Patients with Delirium

History

• Vulnerability profile.
• Triggering factors.
• Known medical, neurological, psychiatric disorders.

Clinical Examination

• Mental status.
• Focal neurological signs.
• Other neurological symptoms/signs.
• Other medical findings.

Investigations

• Laboratory Profile.
• Neuroimaging.
• Cerebrospinal fluid.
• EEG.
• Toxicological screening.
• ABG.

History Details

• Obtain a current and past history from other sources, including prehospital workers, family or friends, and past medical records.
• Look specifically for street drug, alcohol, and medication use.
• Preexisting endocrine disorders.
• Recent activities that may have resulted in exposure to toxins or environmental injury.
• Psychiatric illness and similar episodes of confusion in the past, to uncover a treatable or modifiable cause for the cognitive impairment.

Physical Examination Details

• Evaluate for pupillary, fundoscopic, and extraocular abnormalities, nuchal rigidity, thyroid enlargement, and heart murmurs or rhythm disturbances.
• Pulmonary examination that reveals wheezing, rales, or absent breath sounds.
• Abdominal examination that reveals hepatic or splenic enlargement.
• Cutaneous examination that shows rashes, petechiae, ecchymoses, track marks, or cellulitis.

Simplified Diagnostic Criteria: Confusion Assessment Method (CAM)

• Criteria:
  1. Acute onset and fluctuating course (Is there an acute change in mental state? Did this fluctuate during the past day?).
  2. Inattention (Is the patient easily distracted or does he have difficulty keeping track of what is being said?). can also be detected by asking for the days of the week to be recited backwards
  3. Disorganized thinking (Is the patient's speech disorganized, incoherent, rambling, irrelevant, unclear/illogical, or unpredictable switching between subjects?).
  4. Altered level of consciousness (Is the patient vigilant (hyper-alert) or lethargic/drowsy?).
• 1 + 2 + either 3 or 4 must be present to diagnose delirium.

Richmond Agitation-Sedation Scale (RASS)

• Assessing Sedation:
  • If RASS is -2 to -3, proceed to CAM-ICU (patient CAM-ICU positive or negative?).
  • If RASS is -4 or -5, stop (patient unconscious) and recheck later.

EEG and Delirium

• Delirious patients showed significant reductions of alpha percentage, increased theta and delta activity, and slowing of the peak and mean frequencies.
• Engel and Romano (1959) described alpha slowing with delta and theta intrusions on electroencephalograms (EEGs) and correlated these changes with clinical severity.
• Treating the medical cause resulted in reversal of EEG changes of delirium.

Abnormal EEG Findings

• Delirium: In general, the EEG gets slow, theta and delta, alpha reduced.
• Hepatic Encephalopathy: Slowing and triphasic waves.
• Old variant CJD: Triphasic waves and slow.
• TLE: Temporal lobe rhythmic discharge and spikes.
• Dementia (Alzheimer's and others): In general slowing, loss of alpha and in Alzheimer's spikes.

Management of Delirium

Non-Pharmacological Prevention

• English National Clinical Guideline Centre has issued guidelines for nonpharmacological intervention:
  • Organizational interventions.
  • Orientation interventions.
  • Relaxation interventions.

Clinical Management Interventions

• Prompt correction of hypoxia, hypo/hypertension, anemia, and cardiac arrhythmias.
• Ensure adequate enteral hydration.
• Ensure intake of an adequate amount of calories, trace elements, and vitamins, preferably through the enteral route.
• Suspend unnecessary drug treatments, especially neuroactive drugs.

Pharmacotherapy

• Haloperidol - max dose up to 5 mg.
• Atypical antipsychotics - risperidone, olanzapine, quetiapine, and aripiprazole.
• Valproate, ondansetron, melatonin.
• Recent evidence indicates that low dose melatonin and Ramelteon, a melatonin receptor agonist, are effective at lowering the risk of delirium.
• Newer drug: Dexmedetomidine, (alpha-2 agonist).

Additional Management Strategies

• Institute supportive measures.
• Avoid restraints.
• Reduce stimuli (light, noise, frequent interaction).
• Discontinue focus of antibiotic
• Reassess patient, consider prolonged.
  * Does the patient behavior change ?:
  * NO: Patient discharged to rehab.
  * YES: Low dose neuroleptic (haloperidol, risperidone. Search for reversible causes.
  * No obvious cause after workup?

Conclusion About Delirium

• First, search for delirium!
• Prevention is better than curing: prevention is the most effective way to reduce delirium incidence.
• Search early for risk factors.
• Always try to use the lowest effective doses of neuroactive drugs, especially sedatives: less is better!
• Treat delirium as a medical emergency aggressively; search for underlying organic/metabolic causes and use of deliriogenic drugs.

Seizures and Epilepsy

Clinical notes covering the basics of seizure and epilepsy management.

Introduction to Seizures and Epilepsy

• Seizure: An abnormal, excessive, paroxysmal discharge of cerebral neurons.
  • 5-10% of the population will have at least one seizure, with the highest incidence occurring in early childhood and late adulthood.
• Epilepsy: A chronic condition characterized by recurrent, unprovoked seizures.
  • If a patient has 2 or more seizures, they are diagnosed as having epilepsy.
  • Incidence 0.3-0.5%.
  • Prevalence 5-30 persons per 1000.

Differential Diagnosis of Seizures

• Benign positional vertigo.
• Breath-holding spells in children.
• Cardiac arrhythmia.
• Hypoglycemia.
• Migraine.
• Narcolepsy/Cataplexy.
• Night terrors.
• Nightmares.
• Nocturnal myoclonus.
• Panic attacks.
• Periodic paralysis.
• Pseudoseizures/Hysterical seizures.
• Sleep apnea.
• Syncope.
• Transient ischemic attacks.

Syncope vs. Seizure

• Syncope: A transient loss of consciousness due to brief interruption of blood supply to the brain. Convulsive movements of the extremities may follow some prolonged episodes.

  • Vasovagal syncope: Secondary to fear, pain, or unpleasant sights such as blood or medical procedures.
  • Reflex syncope: Coughing, micturition, defecation, or Valsalva's maneuver.
  • Other causes, especially in the elderly: Orthostatic hypotension and cardiac arrhythmias.

• Clinical presentation of Syncope:

  • Dizzy/light-headed.
  • Fullness in ears.
  • Nauseous.
  • Often gradual graying or blurring of the vision.

• Patients who faint tend to go down more "gracefully" than those with seizures.

• Stereotypical provoking factors such as prolonged periods of standing in the heat, sight of blood, micturition, or abruptly assuming an erect posture after prolonged recumbence.

• The absence of an aura, tongue bite, urinary incontinence, and prolonged tonic-clonic activity in the presence of a provoking factor would be more suggestive of syncope.

Transient Ischemic Attacks (TIA) vs. Seizure

• TIA results from temporary interruption of blood supply in the distribution of a cerebral vessel.
• "Negative symptoms" such as numbness and weakness are more likely to manifest as compared to the "positive symptoms" (stiffness and twitching) seen with seizures.
• Patients will likely have risk factors for cerebrovascular disease such as hypertension, diabetes, and/or coronary artery disease.

Migraines vs. Seizure

• A classic migraine with visual aura, nausea/vomiting, and pounding hemicranial headache can be differentiated easily from a seizure based on history alone.
• However, migraines presenting with isolated symptoms such as vertigo, episodic vomiting (cyclic vomiting), visual changes, and aphasia with/without headaches can be a challenge.
  • A detailed history of previous attacks.
  • Certain triggers (caffeine, sleep withdrawal, chocolate).
  • Family history of migraines.
• Empirical treatment with antiepileptic/anti-migraine medications may clarify the diagnosis in some instances.

Pseudoseizures and Hysterical Seizures vs. Seizure

• Pseudoseizures are paroxysmal attacks of non-epileptic etiology.
• Often characterized by:
  • Waxing and waning movements during a single attack.
  • Prolonged tonic-clonic activity without postictal disorientation.
  • Non-rhythmic pelvic thrusting.
  • Non-physiological evolution of symptoms such as motor activity spreading from one hand to the other without first affecting the ipsilateral face or leg.

Classification of Seizures

• Seizures may be either focal or generalized.

Focal Seizures

• Originate within networks limited to one cerebral hemisphere.
• Can occur with or without impairment of cognition.
• Focal seizures are usually associated with structural abnormalities of the brain.

Generalized Seizures

• Arise within and rapidly engage networks distributed across both cerebral hemispheres.
• Generalized seizures may result from cellular, biochemical, or structural abnormalities that have a more widespread distribution.

Seizure Classification Table

1. Focal seizures (Can be further described as having motor, sensory, autonomic, cognitive, or other features).
2. Generalized seizures
   • Absence
     • Typical
     • Atypical
   • Tonic clonic
   • Clonic
   • Tonic
   • Atonic
   • Myoclonic
3. May be focal, generalized, or unclear
   • Epileptic spasms

Focal Seizures: Detailed

• Arise from a neuronal network either discretely localized within one cerebral hemisphere or more broadly distributed but still within the hemisphere.
• Focal seizures with or without dyscognitive features ("simple focal seizures" and "complex focal seizures").
• Focal seizures can also evolve into generalized seizures (focal seizures with secondary generalization).
• Interictal EEG: Often normal or may show brief discharges termed epileptiform spikes or sharp waves.

Focal Seizures Without Dyscognitive Features

• Can cause motor, sensory, autonomic, or psychic symptoms without impairment of cognition.

• Ictal EEG: Abnormal discharges in a very limited region over the appropriate area of cerebral cortex if the seizure focus involves the cerebral convexity.

• Focal motor seizures:

  • Abnormal motor movements may begin in a very restricted region such as the fingers and gradually progress (over seconds to minutes) to include a larger portion of the extremity ("Jacksonian march").
  • Localized paresis (Todd's paralysis) may occur for minutes to many hours in the involved region following the seizure.
  • Seizure may continue for hours or days (epilepsia partialis continua).

• Focal seizures may also manifest as:

  • Changes in somatic sensation (e.g., paresthesias).
  • Vision (flashing lights or formed hallucinations).
  • Equilibrium (sensation of falling or vertigo).
  • Autonomic function (flushing, sweating, piloerection).

• Focal seizures arising from the temporal or frontal cortex may also cause alterations in hearing, olfaction, or higher cortical function (psychic symptoms):

  • Sensation of unusual, intense odors (e.g., burning rubber or kerosene) or sounds (crude or highly complex sounds).
  • Epigastric sensation that rises from the stomach or chest to the head.
  • Fear.
  • Sense of impending change, detachment, depersonalization, déjà vu.
  • Illusions that objects are growing smaller (micropsia) or larger (macropsia).

• These subjective, "internal" events that are not directly observable by someone else are referred to as auras.

Focal Seizures with Dyscognitive Features

• Transient impairment of the patient's ability to maintain normal contact with the environment.
  • Unable to respond appropriately to visual or verbal commands during the seizure.
  • Impaired recollection or awareness of the ictal phase.
    1. Aura (i.e., a focal seizure without cognitive disturbance).
       PN-
    2. Sudden behavioral arrest or motionless stare.
    3. Automatisms: Involuntary, automatic behaviors.
    • Basic behaviors such as chewing, lip smacking, swallowing, or "picking" movements of the hands.
      • More elaborate behaviors such as a display of emotion or running.
    1. The patient is typically confused following the seizure. Transition to full recovery of consciousness may range from seconds up to an hour.
• Anterograde amnesia.
• Postictal aphasia.

Evolution of Focal Seizures to Generalized Seizures

• Tonic-clonic variety.
• Focal seizures arising from a focus in the frontal lobe.
• History of preceding aura.

Generalized Seizures: Detailed

• Arise at some point in the brain but immediately and rapidly engage neuronal networks in both cerebral hemispheres.

Typical Absence Seizures

• Sudden, brief lapses of consciousness without loss of postural control.
• Typically lasts for only seconds.
• No postictal confusion.
• Subtle, bilateral motor signs such as rapid blinking of the eyelids, chewing movements, or small-amplitude, clonic movements of the hands.
• Associated with a group of genetically determined epilepsies; onset usually in childhood or early adolescence, representing the main seizure type in 15-20% of children with epilepsy.
• EEG: Generalized, symmetric, 3-Hz spike-and-wave discharge that begins and ends suddenly, superimposed on a normal EEG background.

Atypical Absence Seizures

• Lapse of consciousness of longer duration and less abrupt in onset and cessation.
• More obvious motor signs.
• EEG: Generalized, slow spike-and-wave pattern with a frequency of ≤2.5 Hz.
• Diffuse or multifocal structural abnormalities of the brain, signs of neurologic dysfunction such as mental retardation.
• Less responsive to anticonvulsants.

Generalized, Tonic-Clonic Seizures

• Main seizure type in ~10% of all persons with epilepsy.

• Most common seizure type resulting from metabolic derangements.

• The seizure usually begins abruptly without warning.

  • May have vague premonitory symptoms in the hours leading up to the seizure, distinct from the stereotypic auras associated with focal seizures that generalize.

• Tonic contraction of muscles throughout the body, including muscles of expiration and the larynx, resulting in a loud moan or "ictal cry."

• Respirations are impaired.

• Secretions pool in the oropharynx, leading to cyanosis.

• Contraction of jaw muscles, potentially causing biting of the tongue.

• Marked enhancement of sympathetic tone, increasing heart rate, blood pressure, and pupillary size.

• After 10-20 seconds, the clonic phase begins, characterized by the superimposition of periods of muscle relaxation on the tonic muscle contraction.

  • Periods of relaxation progressively increase until the end of the ictal phase, which usually lasts no more than 1 min.

• Postictal phase includes unresponsiveness, muscular flaccidity, and excessive salivation that can cause stridorous breathing and partial airway obstruction.

  • Bladder or bowel incontinence may occur.
  • Followed by postictal confusion involving headache, fatigue, and muscle ache.

• EEG:

  • Tonic phase: Progressive increase in generalized low-voltage fast activity, followed by generalized high-amplitude, polyspike discharges.
  • Clonic phase: High-amplitude activity is typically interrupted by slow waves to create a spike-and-wave pattern.
  • Postictal EEG: Diffuse slowing that gradually recovers as the patient awakens.

• Brief tonic seizures lasting only a few seconds are seen in Lennox-Gastaut syndrome.

Atonic Seizures

• Sudden loss of postural muscle tone lasting 1-2 seconds.
• Consciousness is briefly impaired with no postictal confusion.
• EEG: Brief, generalized spike-and-wave discharges followed immediately by diffuse slow waves that correlate with the loss of muscle tone.
• Atonic seizures are usually seen in association with known epilepsy syndromes.

Myoclonic Seizures

• Myoclonus is a sudden and brief muscle contraction that may involve one part of the body or the entire body.
  • Physiologic form of myoclonus is sudden jerking movement observed while falling asleep.
  • Pathologic myoclonus may arise from metabolic disorders, degenerative CNS diseases, or anoxic brain injury.
• Myoclonic seizures are considered to be true epileptic events because they are caused by cortical dysfunction.
• EEG: Bilaterally synchronous spike-and-wave discharges synchronized with the myoclonus.
• Myoclonic seizures are most commonly associated with juvenile myoclonic epilepsy.

Causes of Focal Seizures

• Idiopathic.
  * Benign Rolandic epilepsy of childhood
  * Benign occipital epilepsy of childhood
• Genetic- Tuberous sclerosis
• Focal structural lesions.
  * Auto somal dominant frontal lobe epilepsy
  * Auto somal dominant partial epilepsy with auditory features
• dysembryonic- Cortical dysgenesis
• Infantile hemiplegia.
• Cerebral migration Abnormalities- Sturge- weber syndrome
• von Hippel-Lindau disease.
• Neurofibromatosis.
• Vasculitis
• Cerebrovascular disease
• Mesial temporal sclerosis
  • Trauma
  • Intra cerebral hemorrhage.
  • Cerebreal infarction
• Tuberculoma
• Intracranial tumors:
  • Cerebral Accesses
  • CNS infection- Toxoplasmosis, syphilis , Cisticercosis

Causes of Generalized Seizures

• Generalization from focal seizures
• Drug use- Antibiotics/ Antimalarials/ Ciclosporin/ Cardiac antiarrhythmics/ Psychotropic agents
• Infective or Inflammation- post infectious encephalopathy / Meningitis / SLE/ Multiple Sclerosis
• Congenital disease
• Metabolic disorders- Hypoglycemia/ Hypomagnesemia/ Hypocalcemia
• Cerebral birth injury
• Cerebral Anoxia
• Renal failure

Epilepsy Syndromes

• Epilepsy syndromes are disorders in which epilepsy is a predominant feature.
• There is sufficient evidence (e.g., through clinical, EEG, radiologic, or genetic observations) to suggest a common underlying mechanism.

Electroclinical Epilepsy Syndromes

• Adolescence to adulthood
  • Juvenile absence epilepsy (JAE).
  • Juvenile myoclonic epilepsy (JME).
  • Epilepsy with generalized tonic-clonic seizures alone.
  • Progressive myoclonus epilepsies (PME).
  • Autosomal dominant epilepsy with auditory features (ADEAF).
  • Other familial temporal lobe epilepsies.
• Less specific age relationship
  • Familial focal epilepsy with variable foci (childhood to adult).
  • Reflex epilepsies.
• Distinctive constellations
  • Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS).
  • Rasmussen's syndrome.
  • Gelastic (from the Greek word for laughter) seizures with hypothalamic hamartoma.
  • Hemiconvulsion-hemiplegia-epilepsy.

Epilepsies with Structural-Metabolic Causes

• Malformations of cortical development (hemimegalencephaly, heterotopias etc.).
• Neurocutaneous syndromes (tuberous sclerosis complex, Sturge-Weber etc.).
• Tumor.
• Infection.
• Trauma.
• Angioma.
• Perinatal insults.
• Stroke etc.

Epilepsies of Unknown Cause

• Conditions with epileptic seizures traditionally not diagnosed.
  • Benign neonatal seizures (BNS).
  • Febrile seizures (FS).

Common Epilepsy Syndrome Table

Juvenile Myoclonic Epilepsy (JME)

1. Generalized seizure disorder of unknown cause that appears in early adolescence.
2. Bilateral myoclonic jerks that may be single or repetitive, most frequent in the morning after awakening, and can be provoked by sleep deprivation. Consciousness is preserved unless the myoclonus is especially severe.
3. Many patients also experience generalized tonic-clonic seizures; up to one-third have absence seizures.
   Complete remission is relatively uncommon, seizures usually respond well to appropriate anticonvulsant medication.
4. Often a family history of epilepsy and genetic linkage studies suggest a polygenic cause.

Lennox-Gastaut Syndrome

• Occurs in children.
• Triad:
  1. Multiple seizure types: Generalized tonic-clonic, atonic, and atypical absence seizures.
  2. EEG: Slow (<3 Hz) spike-and-wave discharges and a variety of other abnormalities; and
  3. Impaired cognitive function in most but not all cases.
• Associated with CNS disease or dysfunction from a variety of causes:
  De novo mutations/ Developmental abnormalities/ Perinatal hypoxia/ischemia/ Trauma/Infection.