General Data

• Name: JS• Age: 14 months old• Gender: Female• Date of Birth: August 12, 2009• Religion: Roman Catholic• Informant: Mother• Reliability: Good

Chief Complaint

• generalized seizures

History of Present IllnessSince birth

•Recurrent grand mal seizures•lasting up to 1 hour•maintained on carbamazepine and topiramate.

Few hours PTC •6 times vomiting and high grade fever.

30 minutes PTC •Generalized tonic clonic seizures with upward rolling of eyeballs and circumoral cyanosis.

Review of Systems General: (-) weight loss, (-) diminished activity,

(-) delayed growth Cutaneous: (-) rash, (-) pigmentation, (-) hair

loss HEENT: (-) headache, (-) dizziness, (-) visual

difficulties, (-) excessive lacrimation, (-) hearing difficulties, (-) aural discharge, (-) nasal discharge, (-) toothache, (-) sore throat

Cardiovascular: (-) easy fatigability, (-) cyanosis, (-) fainting spells

Review of Systems Gastrointestinal: (-) constipation, (-) diarrhea,

(-) abdominal pain, (-) jaundice, (-) pica Genitourinary: (-) dysuria, (-) edema, (-)

discharge Endocrine: (-) heat/cold intolerance, (-)

polyuria, (-) polydipsia, (-) polyphagia Musculoskeletal: (-) joint pain, (-) limitation of

motion Hematopoietic: (-) pallor, (-) easy bruisability

Personal HistoryGestational History

Patient was born to a 23-year old G1P0 housewife married to a 24-year old driver.

Patient’s mother had regular prenatal check-up at USTH with an OB-GYN. She was given unrecalled medication.

She denied illicit drug use, alcoholic intake, exposure to viral exanthems, teratogenic drugs, cigarette smoke and radiation.

Personal HistoryBirth, Neonatal History

Mother denied any illnesses during the pregnancy. No maternal fever, no pruritis and no discharge before labor.

Patient was born live, term (39-40 weeks), singleton, female, via NSD at USTH.

The patient weighed 3 kg upon delivery.

Personal History

Feeding history Patient was exclusively breastfed during the first

three months. she was then shifted to Bonamil on the 4th

month and then Nido fortified milk on his 1st year.

Complementary food was introduced at 6 months.

The patient has good appetite and is not a picky eater. He likes to eat biscuits, cookies, and milk.

Physical Examination• Awake, irritable, not in cardiorespiratory

distress, poorly hydrated• HR 180, RR 28, T 40.3, O2 sat 82%• Warm to touch skin, poor skin turgor, no

active dermatoses, pale nail beds• Normocephalic head, symmetric face, no

lumps/bumps, black hair evenly distributed • Pale palpebral conjunctivae, anicteric sclerae,

pupils 2-3 mm ERTL, (+) ROR, sunken eyes

Physical Examination• No aural discharge, no tragal tenderness, non

hyperemic EAC, tympanic membrane intact• No nasal discharge, midline septum, turbinates

congested, no alar flaring• dry buccal mucosa, no oral ulcers, non-

hyperemic posterior pharyngeal wall, circumoral cyanosis

• Supple neck, no palpable cervical lymph nodes, thyroid not enlarged

• Symmetrical chest expansion, no retractions, equal tactile and vocal fremiti, clear breath sounds

Physical Examination

• Adynamic precordium, AB at 4th LICS MCL, no thrills, no heaves, S1 and S2 normal, no murmurs

• globularabdomen, normoactive bowel sounds, soft, no direct or rebound tenderness, no appreciable masses

• No limitations in range of motion, no joint swelling, no erythema

• Pulses full and equal, no edema, no cyanosis, no clubbing

Neurologic Examination

Alert, awake, irritableNo asymmetry, no gross deformities, no bulging of fontanels, no hydrocephalusSpontaneous muscle movements, no involuntary movements, no tremors

Neurologic Examination• Cranial Nerves: • CN2- visual tracking, blinks with bright light• CN3, 4, 6- no ptosis, pupils 2-3 mm ERTL• CN5- blinks upon gentle air blowing • CN7- no facial asymmetry• CN8- turns head to stimulus• CN9, 10- normal suck and swallowing • CN 11- symmetry of SCM muscle bulk • (-) Involuntary movements• MMT 5/5 in all extremities• DTR: ++ on all• No meningeal signs: (-) nuchal rigidity, Kernig’s and Brudzinski’s• (-) Babinski

Diagnosis

Status epilepticus• Continuous clinical or EEG seizures lasting

atleast 30 minutes or recurrent seizures without return of consciousness during interictal period; the series lasting for 30 minutes or more

Pertinent History

• History of infections, including meningitis• Any history of previous seizures• Compliance to anticonvulsant drugs/ abrupt

withdrawal• Drug use (INH) or exposure to toxins• Anoxia• Vascular disturbances• Metabolic disturbances ( electrolyte

abnormalities)

Pertinent History

• History of static encephalopathies• Cerebral palsy• Head trauma• Underlying chronic illness

Physical & Neurologic Examination

• Vital signs• Observation of seizure activity• Skin bruises, petechiae or needle marks• Papilledema and retinal hemorrhage• Organomegaly and abdominal tenderness

Diagnostic Tests• Determine the cause of the seizure1. Blood glucose level2. Toxic scree3. CBC4. Electrolytes including Ca5. BUN6. Anticonvulsant drug level7. ABG8. Bacterial and viral cultures9. EEG10. CT/MRI11. CSF determination

Classification of status epilepticus

1. Overt generalized convulsive status epilepticus- continuous convulsive activity and intermittent convulsive activity without regaining full consciousness

• Convulsive (tonic-clonic)• Tonic• Clonic• Myoclonic

Classification of status epilepticus

2. Subtle generalized convulsive status epilepticus• Coma following generalized convulsive status

epilepticus with or w/o motor activity3. Simple status epilepticus• Consciousness preserved• Simple motor status epilepticus• Sensory status epilepticus• Aphasic status epilepticus

Classification of status epilepticus

4. Non convulsive status epilepticus• Consciousness impaired; twilight or fugue

state• Petit mal seizures (absence seizure)• Complex partial status epilepticus

Management

Goals of emergency management for status epilepticus

• Ensure adequate brain oxygenation and cardiorespiratory function

• Terminate clinical and electrical activity as rapidly as possible

• Prevent seizure recurrence• Identify precipitating factors such as hypoglycemia,

electrolyte imbalance, lowered drug levels, infection and fever

• Correct metabolic imbalance• Prevent systemic complications• Further evaluate and treat etiology of status epilepticus

• In practice, treatment should start within, and certainly 10 minutes of continuous generalized tonic-clonic seizure activity

Protocol

• ABC principle of resuscitation– Oral airway– High flow O2 (100% O2 by nasal cannula or

nonrebreathing mask), intubate if necessary– Continuous ECG monitoring and pulse oximetry– IV 5% dextrose in 0.3% saline or PNSS– In the absence of dehydration or shock, fluids may

be given at 75% of maintenance level or at 100 mk/m2

• Blood glucose by stick testing– If hypoglycemic, give 2-4 mg/kg of 25% glucose

solution by bolus injection

• Drug therapy– IV diazepam 0.3 mg/kg or lorazepam 0.1 mg/kg– If IV access failed, recatl diazepam 0.5 mg/kg– 2nd dose hould be given in children who do not

respond after 10 mins and with recurrence of convulsions

– If following the 1st dose of rectal diazepam an IV access is still not established, rectal paraldehyde 0.4 mg/kg

• If seizures persist– Verify that the convulsion is a genuine epileptic

seizure– Confirm that no treatable cause like

hyperglycemia is overlooked– If IV access is still not possible, interosseous

needle should be inserted

Phenobarbital

• Loading dose of 20 mg/kg by slow IV bolus• If seizures are controlled before full loading

dose is given, remaining dose may be given 1-2h after by either slow IV or IM

• May produce respiratory depression and arrest – elective intubation

Phenytoin

• 20 mg/kg (made up of 0.9% saline at a maximum concentration of 10 mg in 1 mL, infused at no more than 1 mg/kg/min)

• Preferred drug• Less respiratory and CNS depression

• Children on maintenance oral phenytoin and compliant, IV phenobarbital over 10 min should be given

• Other management– Diazepam or midazolam infusion– Barbiturate coma– General anesthesia

• If after 20 mins after phenobarbital and phenytoin infusion, patient remains in convulsive status epilepticus, consider rapid sequence induction of anesthesia by experienced anesthesia personnel using thiopental or propofol

• Ideally, EEG should be done• Children <3 years old with prior history of CSE

should be treated with IV pyridoxine

• Diazepam, loeazepam, or valproate – best drug for treatment of nonconvulsive status

• Valproate – administered by NGT or rectally; effective in absence status epilepticus

• Common medical complications: – CHF– Hypertension– Hypotension– Pulmonary edema– Pneumonia– Oliguria

Treatment guideline for an acute tonic-clonic convulsion including established convulsive status epilepticus.

et al. Arch Dis Child 2000;83:415-419

©2000 by BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health

CURRENT GUIDELINES FOR TREATMENT OF CONVULSIVE STATUS EPILEPTICUS BY COUNTRY

• Japan: “Research Committee on Clinical Evidence of Medical Treatment for Status Epilepticus in Childhood has a proposed guideline for the treatment of CSE in childhood. Initial management of seizures should be attempted mainly with i.v. diazepam, the second-line treatment involves i.v. midazolam followed by i.v. phenytoin if seizures persist, and the third-line treatment requires barbiturate coma”

• France: “as intravenous lorazepam not available, clonazepam, rectal diazepam or buccal midazolam as the best choice for initial therapy of CSE in infants and young children. Intravenous phenytoin / fosphenytoin and phenobarbital are the second-line drugs. Of the third line AEDs, high-dose midazolam infusion rather than thiopental to minimize serious side effects from barbiturate anesthesia”

The Role of Intravenous Valproate in Convulsive Status Epilepticus in the Future. Shang-Yeong Kwan, Acta Neurol Taiwan 2010;19:78-81