STATUS EPILIPITICUS & MANAGEMENT OF COMA

a ppt on status epilepticus and management of coma in children

1. STATUS EPILIPETICUS MANAGEMENT OF A CHILD WITH COMA

2. A seizure is defined as “a transient occurrence of signs and / or symptoms due to abnormal excessive or synchronous neuronal activity in the brain . The term transient is used as demarcated in time, with a clear start and finish .” Classically SE was defined as a “a condition characterized by an epileptic seizure that is sufficiently prolonged or repeated at sufficiently brief intervals so as to produce an unvarying and enduring epileptic condition

3. DEFINITION Continuous seizures activity > 5 min > 2 seizures without interictal recovery of consciousness to baseline Brought in active seizure to ER Early SE- 5-30 min Established SE- 30-120 min.

4. Classification of Status Epilepticus 1 Semiology 2 Etiology 3 EEG correlates 4 Age

5. Semiology This axis refers to the clinical presentation of SE and is therefore the backbone of this classification. The two main taxonomic criteria are: 1 . The presence or absence of prominent motor symptoms( Convulsive) 2. The degree (qualitative or quantitative) of impaired consciousness( Non Convulsive)

6. Etiology

7. Commonest cause of SE Febrile Seizure CNS Infections ¼ having SE have p/h of seizures Triggers for SE- poor drug compliance, sleep deprivation, inter current illness, puberty, drug interactions,

8. Indications for performing urgent EEG First , if psychogenic status epilepticus is suspected then diagnosis using EEG monitoring may avoid continued exposure to anticonvulsant medications Second , there is increasing data that after convulsive SE terminates, some patients have persisting EEG-only (non-convulsive) seizures. These may occur when convulsive seizures terminate with anticonvulsant administration but EEG seizures persist ( electroclinical uncoupling or dissociation), or when paralytics are required as part of the management strategy and clinically-evident convulsions are thereby masked.

9. Indications for performing urgent EEG Continuous EEG monitoring should be initiated within one hour of SE onset if ongoing seizures are suspected . 48 hours of EEG monitoring to identify possible non-convulsive seizures in children with recent SE with return to baseline mental status in 10 minutes, in patients with known acute symptomatic brain injury including traumatic brain injury, stroke, coma after cardiac arrest, or in patients with altered mental status

10. Age Neonatal (0 to 30 days). 2 Infancy (1 month to 2 years). 3 Childhood (> 2 to 12 years). 4 Adolescence and adulthood (> 12 to 59 years). 5 Elderly (≥ 60 years).

11. SE categorized by duration of seizures. Refractory status epilepticus : It is defined as continuous seizure activity despite administration of adequate doses of two antiepileptic drugs. Super-refractory SE: SE that continues for 24 hours or more after the use of anesthetic therapy, including cases that recur on weaning of the anesthetic agent.

12. In contrast to some earlier SE management algorithms which considered medications as first, second, and third line agents , The new guideline uses the terms emergent, urgent, and refractory to help convey a sense of time urgency.

13. Emergent Seizure Management: Initial Steps:  Ensure ABCs (airway, breathing, circulation), provide supplemental oxygen, and initiate continuous monitoring of vital signs.  Vascular Access:  Obtain IV access promptly.  Blood Glucose:  Check blood glucose levels to rule out hypoglycemia and treat accordingly.  Benzodiazepines:  Administer intravenous lorazepam (preferred for rapid onset) or diazepam as first-line therapy.  Antiepileptic Medications:  Administer concurrently with benzodiazepines, options include phenytoin, fosphenytoin , valproate, or levetiracetam .  Repeat Dosing:  If seizures persist, repeat benzodiazepine doses may be administered. 

14. Urgent Seizure Management: Continued Monitoring:  Maintain continuous monitoring of vital signs and airway.  Second-Line Therapy:  If initial therapy fails to control seizures, administer second-line medications as outlined above.  Consultation:  Consult with a neurologist early in the management process.  Intubation:  Consider intubation and mechanical ventilation if airway management or oxygenation/ventilation is compromised. 

15. Refractory Status Epilepticus Management: Further Anti-Seizure Medications: Continue or increase doses of existing anti-seizure medications.  Continuous Infusions: Begin continuous infusion of an anesthetic agent, such as midazolam or pentobarbital, to suppress seizures.  Ketamine: Consider the use of ketamine, especially in early phases of stage 3 status epilepticus , as a second-line treatment option.  ICU Admission: Transfer to an intensive care unit (ICU) for closer monitoring and advanced management.  Further Investigation: Investigate the underlying cause of the seizures to guide future management.  Surgical Interventions: In selected cases, consider surgical interventions for refractory status epilepticus , such as hemispheric disconnection ( hemispherotomy

16. COMPLICATIONS Respiratory arrest Arrythmias Hypotension Hemodynamic instability Prolonged seizures- rhabdomyolysis , hemolysis, myoglobinuria , AKI Uncontrolled seizures may cause – cerebral edema, raised ICT ,

17. MANAGEMENT OF COMA Consciousness : A state of being aware. A state of general wakefulness and responsiveness to environment . Coma : A state of deep, unarousable , sustained, pathologic unconsciousness in which the person shows no meaningful response to environmental stimuli. Consciousness – Coma continuum : Consciousness and coma are two extremes of an awareness scale as indicated here:

18. Descriptive terms such as: somnolence , stupor, obtundation , and lethargy used to denote different levels of wakefulness are best avoided Vegetative state describes a condition of complete unawareness of the self and the environment accompanied by sleep wake cycles with variable preservation of brainstem functions. The vegetative state is deemed to be permanent 12 months after traumatic brain injury and 3 months after non-traumatic injury . Minimally conscious state is defined as a condition of severely altered consciousness in which the patient demonstrates minimal but definite behavioural evidence of self- or environmental awareness. Brain death is defined as the permanent absence of all brain functions including those of the brainstem . Brain-dead patients are irreversibly comatose and apneic with absent brainstem reflexes.

19. Background Physiology Arousal or wakefulness, which is dependent on the function of the Reticular Activating System (RAS), a network of neurons located in the core of the brainstem extending from mid pons through the midbrain/hypothalamus to thalamus. Awareness , which is mediated through cerebral cortical neurons and their reciprocal projections to and from the major subcortical nuclei. Awareness requires arousal, but not vice versa.

20. Coma will result if there is either RAS or cerebral cortical dysfunction. RAS dysfunction, mostly caused by structural lesions in the brain stem tend to present with focal neurologic findings Cerebral cortical dysfunction is often caused by Toxic/Metabolic processes and present with non-focal neurologic findings.

21. Questions to ask in History Timing Acute onset – Suggests intracranial hemorrhage, non-convulsive status seizures, ischemic stroke, or acute trauma Onset of minutes to hours Suggests a metabolic cause, infection, or progressive injury from trauma Onset of hours to days – Suggests a shunt blockage, hydrocephalus, or tumor Associated symptoms Fever – Infections Preceding headache – Increased ICP Preceding somnolence – Toxic/Metabolic Ataxia, confusion or loss of milestones in recent weeks – Neoplasm Always be cautious of a history that is inconsistent with presentation, as this might suggest non-accidental injury (e.g. shaken baby syndrome)

22. “TIPS VOWELS” (AEIOU) T Trauma I Infection (systemic and neurologic) P Psychiatric S Space occupying lesion, stroke A Alcohol and drugs/toxins E Endocrine, environment I Insulin (hypo/hyperglycemia) O Oxygen (hypoxia), Opiates U Uremia

23. Physical Examination-Primary survey Airway (with C-spine precautions)   The tongue is the most common cause of obstruction in the unconscious child. Intubation is needed for: GCS less than 9 Poor airway or impending loss of airway Raised ICP (to optimally ventilate) Precaution during transport or CT scanning Breathing – must assure adequate oxygenation and ventilation Circulation – Is the patient in shock? Check pulses, BP, HR, perfusion, temperature . Is Cushing response (↑BP, ↓HR, abnormal breathing pattern due to ↑ICP) present ? Establish IV access. Disability – Glascow Coma Scale and Glascow Coma Scale-modification for children are useful for objective evaluation and quantification of the consciousness-coma continuum. But it does not account for the important brainstem reflexes ( pupillary, oculocephalic , oculovestibular , corneal).

24. Secondary survey-Head and Neck Bruises, cephalohematoma , swelling suggest -cranial trauma. Bleeding or clear fluid from nose or ears suggest -basilar skull fracture. Check fundi for papilledema (increased ICP) and hemorrhage (occult trauma). Many metabolic/toxic causes for coma result in symmetrically small pupils with preserved reactivity to light. Hypoxic-ischemic injury produces symmetric dilated pupils that may not respond to light . Unilateral pupillary dilatation in the comatose patient should be as considered evidence of oculomotor nerve compression from ipsilateral uncal herniation , unless proved otherwise Passive resistance to neck flexion suggests meningeal irritation, tonsillar herniation. Horizontal divergence of eys at rest-normal, conjugate roving eyes- good, to one side- frontal of same or pons of other side ,(eyes look towards hemisphere and away from brainstem lesion) Ocular bobbing- PONS Ocular dipping-DIFFUSE CORTICAL ANOXIC DAMAGE

25. Secondary survey-Head and Neck Positive Kernig’s and Brudzinski’s sign can also indicate meningeal irritation. Cranial Nerve Exam: II, IV, VI – Extra ocular movement (brainstem integrity) Note that the Oculocephalic reflex should not be done if neck injury is suspected V, VII -Corneal reflexes. A normal response is bilateral closure of eyelids after unilateral stimulation of the cornea. Metabolic causes tend to produce bilateral loss of response. IX, X -Gag reflex

26. Fever- infective process e.g. sepsis, pneumonia , meningitis, encephalitis, or a brain abscess; Hypothermia- alcohol, barbiturate,sedative , phenothiazine intoxication, hypoglycemia, extreme hypothyroidism May also indicate heat stroke or abnormality of hypothalamic temperature regulatory mechanisms. Tachycardia- fever, hypovolemic or septic shock, heart failure or arrhythmias. Bradycardia -raised intracranial pressure or a result of myocardial injury (due to myocarditis, hypoxia , sepsis, or toxins ). Tachypnea- features of respiratory distress signifying increased work of breathing is an indicator of lung pathology e.g. pneumonia, pneumothorax, empyema or asthma.

27. Quiet tachypnea- acidosis which maybe present in diabetic ketoacidosis, uremia , or some poisonings (e.g. ethylene glycol). Hypertension- maybe the cause of alteration in sensorium in hypertensive encephalopathy or maybe a compensatory mechanism to maintain cerebral perfusion in children with increased intracranial pressure or stroke; in the former, there maybe features of hypertensive retinopathy and findings suggestive of left ventricular hypertrophy (ECG, Echocardiography ). Hypotension due to sepsis, cardiac dysfunction toxic ingestion, or adrenal insufficiency, may lead to poor cerebral perfusion, resulting in diffuse or watershed hypoxic-ischemic injury, profiund hypothyroidism.

28. Sensory and Motor reflexes Sensation is grossly tested through the GCS evaluation Deep tendon reflex (DTR) and/or motor tone asymmetry are important focal findings. Posturing indicates level of disease in midbrain with reference to the red nucleus: Flexor (previously called decorticate): Arms flexed and hands towards midline; legs extended. Indicates damage above the level of the red nucleus in midbrain. Extensor (previously called decerebrate ): Arms and legs extended; teeth clenched. Indicates severe disease involving central midbrain below the level of the red nucleus.

29. Cardiovascular- Congenital heart disease or endocarditis may be sources of intracranial thrombi.   Skin Cyanosis – poor oxygenation Jaundice – liver failure Extreme pallor – anemia or shock Cherry red skin – CO poisoning Rashes/ petechiae – may be seen with certain infections such as meningococcemia, TTP, Bleeding diathesis Increased pigmentation – Addison’s Disease Neuro -cutaneous lesions – e.g. tuberous sclerosis may suggest intracranial tumor Odor of exhaled breath Alcohol intoxication DKA (sweet fruity) Uremia (urine like) Hepatic coma (musty)

30. Clinical Category of Causes 1 ) Coma with focal signs 2) Coma without focal signs and without meningeal irritation 3) Coma without focal signs and with meningeal irritation

31. Coma with focal signs • Intracranial hemorrhage • Stroke: arterial ischemic or sinovenous thrombosis • Tumors • Focal infections-brain abscess • Post seizure state: Todd’s paralysis • Acute disseminated encephalomyelitis

32. Coma without focal signs and without meningeal irritation Hypoxia-Ischemia: Cardiac or pulmonary failure, Cardiac arrest , Shock, Near drowning Metabolic disorders: Hypoglycemia Acidosis , Hyperammonemia (e.g. Hepatic encephalopathy , urea cycle disorders, Reye syndrome) Uremia, Fluid and Electrolyte disturbances (dehydration, hyponatremia , hypernatremia ) Systemic Infections: Bacterial: gram-negative sepsis, meningitis, toxic shock syndrome , Enteric encephalopathy Post infectious disorders: Acute necrotizing encephalopathy, Hemorrhagic shock and encephalopathy syndrome Post immunization encephalopathy: Whole cell pertussis vaccine, Drugs and toxins Cerebral malaria Rickettsial : Lyme disease, Rocky mountain spotted fever Hypertensive encephalopathy Post seizure states Non-convulsive

33. Coma without focal signs and with meningeal irritation Meningitis Encephalitis Subarachnoid hemorrhage

34. Uncul - large ipsilateral pupil, babinski sign, ipsilateral hemiparesis ( kernohan woltman sign) Central Transtentoria - symmetric downward movement, compression of midbrain,miotic pupil,and drowsiness Resp affected late

35. Cingulate and trans tentorial - early compressionof medulla and resp arrest and death.

36. Management The next most important step is establishment of vascular access. If there is evidence of circulatory failure, fluid bolus (20 mL/kg- Normal saline) should be administered. Samples should be drawn for various investigations. If there are features of raised intracranial pressure (asymmetric pupils, tonic posturing, papilledema, evidence of herniation ), measures to decrease intracranial pressure should be rapidly instituted (hyperventilation, mannitol etc.). Acid base and electrolyte abnormalities should be corrected. Normothermia should be maintained . Mannitol is administered as an initial bolus of 0.25–1 g/kg (the higher dose for more urgent reduction of ICP) followed by 0.25–0.5 g/kg boluses repeated every 2– 6 h as per requirement. Hypertonic saline is administered as a continuous infusion at 0.1 to 1.0 mL/kg/h, to target a serum sodium level of 145–155 meq /L.

37. MANAGEMENT Identify and treat hypoglycemia with intravenous dextrose (2 ml/kg 10% D, Then glucose infusion rate of 6–8 mg/kg/min). Identification and treatment of seizures. If the child is having tonic- clonic movements, tonic deviation of eyes or nystagmus , or there is history of a seizure preceding the encephalopathy , anticonvulsant should be administered. Lorazepam should be given (0.1 mg/kg), followed by phenytoin loading (20 mg/kg). Non convulsive status epilepticus (NCSE) maybe seen in comatose children, and should be looked for in all children with unexplained encephalopathy . Treatment of infections. In case of suspected sepsis/ meningitis, broad spectrum antibiotics (ceftriaxone, vancomycin ) should be instituted immediately. If viral encephalitis is likely, then samples for PCR for herpes simplex virus should be sent and acyclovir should be started (dose).

38. Antimalarials (quinine/ artesunate ) should be started if there is a clinical suspicion of cerebral malaria. Antidotes: Naloxone (0.1 mg/kg) should be used in case of suspected opiate poisoning. Flumazenil is useful for benzodiazepine overdosage . Steroids are of benefit in acute disseminated encephalomyelitis, meningococcemia with shock, enteric encephalopathy, tubercular meningitis, and pyogenic meningitis. If metabolic causes have been identified, e.g. diabetic ketoacidosis , hepatic encephalopathy, uremia or hyperammonemia , these should treated appropriately. In sick children with acute febrile encephalopathy, empirical therapy with antibiotics, acyclovir and antimalarial agents should be considered while the results of investigations are awaited . The clinical course of the child should be monitored closely and documented on a daily basis. Particular attention should be paid to changing level of consciousness, fever, seizures , autonomic nervous system dysfunction , increased intracranial pressure, and speech and motor disturbances . Nosocomial infections are important complications during hospitalization, and must be prevented and treated promptly.

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