PEDIATRIC MENINGITIS

INTRODUCTION Mortality 20-40% in neonates Mortaility 5-10% in infants and children Pediatric incidence is highest in neonatal period Next highest incidence is between 3-8months 90% of cases occur before 5 yo

INFECTIOUS MENINGITIS BACTERIAL

Pneumococcus Meningococcus Listeria Hflu Staph aureus E. coli Borrelia (LYME) Treponema (SYPHILUS) Mycoplasma

VIRAL Enteroviruses: coxsachi, echo, polio Herpes virus: HSV, EBV, CMV, VZV Arboviruses: WEE, EEE, Japaneses, St. Louis, WNV Other: rabies, HIV, HTLV, measles, mumps

FUNGAL Cryptococcus Coccidiomyocosis Candida Histoplasma Blastomyces

PARASITES Toxoplasma Cysticercosis Amoeba

RICKESIA RMSF

NON-INFECTIOUS MENINGITITS CDs C - Cancer (carcinomatous meningitis) D - Druges (Septra, isoniazid, NSAIDS) SIN S - Serum sickness S - Sarcoidosis S - SLE, Bechets

NON-INFECTIOUS CAUSES UNCOMMON IN KIDS

PEDIATRIC BACTERIAL MENINGITISETIOLOGIES Neonatal Meningitis

Group B streptococcus: 50% E. Coli: 25% Others: other coliforms, Staph eip, Staph aureus, pneumococcus,

meningococcus, group D strep, ureaplasma, Heamophillus, , Listeria Note that Listeria is actually fairly

Infants/Children Pneumococcus: 45% Meningococcus: 45% H.flu: 5% Other: salmonaella, camplylobacter, listeria, group B strep, anaerobes

PATHOPHYSIOLOGY Neonates are immunologic immaturity = essentially immunosuppressed Immunosuppression: AIDS, DM, sickle cell, corticosteroids Other risks: poor living conditions, head trauma, neurosurgery, mastoiditis, recent AOM,

daycare, antibiotic use

CLINICAL FEATURES General

Presentation depends on age: more nonspecific with younger kids 3/4 have subacute presentation over 2-5 days Fever, malaise, lethargy, irritability, anorexia, N/V, diarrhhae Symptoms are OFTEN nonspecific 1/4 have acute presentation within 24hrs Look for locus of infection: AOM, mastoiditis, etc

Neonatal Period Temperature is easiest clue Temp > 38: 10% with SBI and 1-2% with bacterial meningitis 50% will be afebrile or hypothermic: absence of temp does not r/o

meningitis in a neonate Other vital sign changes are a clue May present with ALTE: apnea secondary to seizure or respiratory center

depression Behavioural changes: irritable, lethargic Irritability that is WORSE with consoling is a clue Seizures: vacant stare, hypertonicity, trembling chin, bicycling motion of

extremities Nuchal rigidity < 25% Bulging fontanelle 15% Rashes infrequent Livedo reticularis: generalized pallor accompanied by indistinctly outlined

truncal pathces of blue discoloration

Infants (1mo-1yr) Similar to newborns Febrile illness, generally look toxic

Nuchal rigidity again uncommon but specific if present Headache + fever (90% but not 100%!)

Child 1-5yo Fever, headache, photophobia, neck stiffness Neck stiffness becomes more reliable after the first year Passive testing of neck stiffness: supine position, seated with legs

outstretched (better way of passive testing) Active testing: distraction with an object and observe neck ROM with

tracking of object, also look for spontaneous ROM of neck Sensitivity and specificity of Brudinskys and Kernigs are poor Brudinskys sign: flexion of the Back of the neck produces involuntary hip

flexion Kernigs sign: extension of the Knee causes involuntary contraction of the

hamstring :. inability to straighten knee; also described as extension of the Knee causes pain in the neck

BUG NOTES Meningococcus

Gram -ve intracellular diplococci 5 major serotypes: A, B, C, Y, W-135 (B,C,Y are now most prevalent) Vaccine covers A,C,Y, W-135 (NOT B): Vaccine is not 100% effective; even less effective < 2yo Vaccine duration is not permanent Adrenal hemorrhage more common than pneumococcus

Pneumococcus Many serotypes Vaccine covers most 50% have pneumonia on CXR

DIFFERENTIAL DIAGNOSIS Infectious

Septicemia, encephalitis, subdural empyema, epidural abscess, brain abscess, viral/fungal/TB meningitis, myocarditis, rickettsemia

Traumatic Shaken baby, NAT, closed head injury

Metabolic Hypoglycemia, DKA, hypo/hypernatremia, uremia, urea cycle defects

Must suspect meningitis in any infant with fever.

Infants do not present with neck stiffness.

Miscellaneous Intussuception, intoxication, toxic exposure, seizure disorder, brain tumor,

AVM, ruptured dermoid cysts

INVESTIGATIONS General

CBC, etc Blood culture a must

Indications for Lumbar Puncture Suspected neonatal sepsis: fever, toxic appearing, seizure, ALTEs Febrile illness between 1-2months: difficult to exclude meningitis by exam Febrile illness after close contact to patients with a SBI Nuchal signs Toxic appearance Febrile seizures: LP if < 1year, toxic (generally lower threshold) Fever and petechiae: not all petechiae are meningitis related, LP if unwell Immunocompromised Dural penetration: craniofacial trauma; LP after CT Acute hearing loss

Indications for CT scanning b/f LP Focal neurological deficit Focal seizure (vs ? all seizures) Papilledema Marked decreased LOC Hx or evidence of head trauma (recent or remote) Known intracranial mass ALL immunocompromised patients

NORMAL CSF WBC < 5 and PMNs < 1 NO esoinophils May have occasional basophil

CSF CELL COUNT Bacterial meningitis usually > 1000 cells/mm3 Normal CSF wbc counts vary widely Normal wbc counts also vary with ageAge Mean Range Treatment ThresholdPreterm 7 0-44 >9Term 8 0-32 >220-4weeks 11 0-50 >354-8weeks 7 0-50 >10>8weeks 2 0-8 >6

CSF DIFFERENTIAL Bacterial usually higher wbc count; viral usually lower wbc count Classical is neutrophils with bacterial and lymphs with viral, TB, fungal Bacterial early can have diff with primarily lymphs Viral early can have diff with primarily PMNs Neonates: 60% of cells can be PMNs > 1 month: maximum of 3 PMNs per mm3 Recent pediatric study: found NO predictive value of differential on initial

LP and 24hr LP: early neutrophilia or abscence there of can predict or rule out viral vs bacterial etiology

Powers et al 1984- 10% of bacterial meningitis with early lymphocytic %- 2/3 of viral had early neutrophil predominance- 10% of bacterial had wbc count < 1000

Traumatic LP- No more than 1 wbc per 500 rbc are allowed- WBCcsf = Measured wbcin CSF [(rbccsf X wbcblood)]

RBC blood Gram Stain

60 - 90% sensitive depending on source Gram stain sensitivity decreases 20% w/ abx on board Clues on gram stain ....

Gram -ve intracellular diplococci MeningococcusGram + diplococci PneumococcusGram + bacilli ListeriaGram -ve coccobacilli H.fluGram -ve bacilli Ecoli

Cultures 85% sensitive with bacterial meningitis Sensitivity < 50% with previous abx Viral cultures detect 25% Fungal cultures detect 75% TB cultures detect 55% Opening pressure: normal 5-20 cm Can do viral cultures: consider if you need to know the bug

Glucose Normal CSF/serum ratio is 0.6:1.0 unless serum hyperglycemia Ration < 0.4 suggests bacterial, TB or fungal meningitis (only mild

decrease in glucose with viral meningitis) Protein

Normal range = 0.2 - 0.4 g/L Can increase with blood from traumatic tap Viral: normal to mild increase (0.4 - 1.0) Bacterial, fungal, TB: larger increased protein (0.4 - 5.0) NOTE: can increase with SAH, vasculitis, cancer, MS

India Ink Staining Budding microorganism = cryptococcus NOTE: only 30% sensitive thus must do cryptococcal antigen testing

Antigen Detection Counter Immuno Electrophoresis (CIE): not used as much Latex Agglutination

- More sensitive, faster, replacing CIE- Variable reports of sensitivity- Meningococcus: 50 - 90%- Pneumococcus: 50 - 100- Hflu 80%- Cryptococcal agglutination: 90%- Xanthochromia: takes 2hrs to devp after blood, can be due

to bloody tapETIOLOGY and EMPIRIC ANTIBIOTICS

Note similarity to sepsis in peds Ceftriaxone/Cefotaxime: excellent CSF penetration, good

pneumococcus/meningococcus coverage, make sure to give at CNS penetration doses Add ampicillin for coverage of Listeria if > 50yo or < 3mo Add vancomycin for coverage of penicillin resistant pneumococcus (20% here) Resistant pneumococcus overall is 20% Note: cefotaxime and ceftriaxone can cause biliary sludging < 1mo Allergic to pencillins: cephalosporins OK unless anaphylactic Allergic to cephalosporins: Meropenum When to start acyclovir empirically?

Cold sores Genital ulcers Neonate with mom with ulcers ? any focal seizure (increased risk of HSV)

Special Circumstances Closed head trauma: pneumococcus, meningo, GBS; ceftriaxone 2g iv

q12h Open head trauma, neurosx, CSF shunt: staph aureus/epi; vancomycin +

ceftazidime

< 1 month 1 - 3 months 3 mo - 50 yrs > 50yrs

ETIO Group B StrepE. coliListeriaConsider HSV encephalitis-mom w/ HSV-baby with vesicles-focal sz-focal neuro s/s

E.coliListeriaPneumococcusMeningococcusH.fluGroup B Strep (uncommon 1-3mo)

PneumococcusMeningococcusH.flu

Pneumococcus 40%Meningococcus 25%Listeria 5%Other gram -ve 10% (klebsiella, ecoli,hflu)

EMPABx

Amp 50 mg/kg iv q12hGent 2.5 mg/kg iv q12

Amp 50 mg/kg iv q6hCefotax 50 mg/kgq6h

Ceftriaxone50 mg/kg iv q 12h (max 4g/d)Vancomycin (pen resistant pneumococcus 20% here)

Ceftriaxone 2 g iv q12 +/-Vancomycin 1gm iv q12 +/-Ampicillin 2g iv q4hr

AlternAbx

Amp + cefotaxime Cefotaxime

TREATMENT General

Empiric antibiotics as above Do not delay antibiotics: ie, order them up while doing LP; give before CT Fluids: 20 ml/kg for shock X 2 Pressors: start norepinephrine if hypotensive after two boluses Treat hypoglycemia if present (D10 for neonates, D25 for infants, D50 for

kids) Seizures: benzo > dilantin > phenobarb Increased ICP: intubate, mild hyperventilation

Steroids Role of decadron in bacterial meningitis controversial Animal studies: decreased CSF pressure, lactate, brain edema Given before or at time of first antibiotic dose to decrease mortality Adults: recent evidence from NEJM Nov 2002: dexamethasone 10 mg iv

q6hr Children: data is in group with Hflu, shown to decrease hearing

complications; not generally used now as Hflu rates decreases due to vaccine

Consider in kids who are > 2yo and unimmunized (higher risk of Hflu) AAP recommendations: limit steroid use to those with presumptive Hflu Dose: 0.15 mg/kg

Contact treatment Inform public health Pneumococcus

- none H.flu

- prophylaxis required only if there is non-immunized siblings or contacts > 25 hr/wk who are less than 4 years old

- rifampin 20 mg/kg (max 600mg) od X 4 days- ALL daycare contacts if > 1 case- F is for HFlu for four days

Meningococcus- prophylaxis required for all close contacts such as

household members, daycare, including adults; medical personel only necessary if in contact with mucosal secretions

- only eradicates nasopharyngeal colonization- rifampin 10 mg/kg (max 600mg) bid X 2 days- ceftriaxone im, or cipro are alternatives

COMPLICATIONS Acute/Subacute

Sepsis: DIC, ARDS, ARF, hepatobiliary Dehydrataion Pericardial effusion Adrenal hemorrhage Abcess SIADH and hyponatremic seizures Seizures: ativan > phenytoin > phenobarb Cerebral edema: thus only give 70% maintenance fluids to prevent this Subdural effusions: liquidification of pus Hydrocephalus: results from pus in arachnoid villi preventing drainage Increased ICP: treat w/ ETT and hyperventilation, mannitol 1 g/kg iv

Chronic Deaf: sensorineural deafness in 5%

Blind: uncommon Dumb: MR or devtal delay, learning disabilities Ataxia Seizure disorder Focal paralysis Hydrocephalus

PEDIATRIC SEIZURES

CLASSIFICATION OF SEIZURES

GENERALIZED (Loss of consciousness) Tonic Clonic Tonic - Clonic (GrandMal) Absence (PetitMal) Myoclonic Atonic

FOCAL/PARTIAL (NO loss of consciousness) Simple (consciousness/mentation NOT affected)

Sensory: auditory, visual, olfactory, gustatory, vertiginous Motor: tonic, clonic, tonic-clonic, jacksonian Automomic Psychic

Complex (consciouness/mentation affected but NO loss of consciousness) Visceral symptoms Hallucinations Memory disturbance Dream - like state Affective disorder Automatisms

Secondarily Generalized

MODIFIED CLASSIFICATION OF EPILEPTIC SYNDROMES Idiopathic

Benign neonatal covulsions Benign childhood epilepsy (includes Benign Rolandic Epilepsy) Childhood/juvenile absense epilepsy Jeuvenile myoclonic epilepsy Idiopathic epilepsy - otherwise unspecified

Symptomatic Epilepsy Syndromes West synrome(Infantile Spasms) Lennox - Gaustaux Early Myoclonic encephalopathy Temporal Lobe epilepsy Epilepsia Partialis continua Frontal Lobe epilepsy Post traumatic epilepsy

Mixed or Uncertain Classification Neonatal seizures Febrile seizures Reflex epilepsy

PATHOPHYSIOLOGY Any disturbance of excitatory or inhibatory mechanisms Spread of seizure activity limited by active synapse inhibition Young, immature nervous system MORE prone to seizures because excitatory systems

are relatively over - developed compared to the inhibatory systems (period of vulnerability)

Seizures do affect long term behaviour and cognition: the longer and the more frequent the seizures the more likely that development will be abnormal

INFANTS have different seizures Poor white matter connections b/w hemispheres thus more likely to be

focal and thus generalized tonic-clonic seizures uncommon Blinking, lip-smacking, hand twitching, pill rolling, ALTEs (parents miss the

short seizure activity but notice the apnea)

NOTES ON GENERALIZED SEIZURES Begin with abrupt loss of consciousness with NO aura Motor activity when present involves all four extremities and is usu symmetrical May have prodrome of irritability, tension, isolated myoclonic jerks but NO true aura Hx of aura implies focal onset with secondary generalization Post-ictal phase of variable duration is universal Convulsive Generalized Seizures

Tonic/Clonic (GrandMal)- abrupt loss of consciousness w/o warning and no true aura- b/cms tonic: rigid, trunk extended - falls to ground, may b/cm apneic, vomit, incontinent,

cyanotic- b/cms clonic: rhythmic jerking

Clonic- rhythmic flexor mvmts of muscles which gradually slow

Non-Convulsive Generalized Seizures Absence (PetitMal)

- sudden interuption of consciousness; stairs, stops talking, not responsive, doesnt fall, lasts seconds +/- automatisms, NO post ictal phase , no incontinence

- school age children; rare in adult (temp lobe sz mc)- ppt by hyperventilation - good office test- 3 Hz spike and wave on EEG

Atonic (Akinetic): - sudden loss of postural tone; drops to ground abruptly w/

no postural reflexes. Often confused w/ syncope Myoclonic

- sudden, brief muscle group contraction without LOC- drop-attack occurs when the entire body is involved- shock-like contraction of groups of muscles, often irregular

in rhythm and amplitude, and may not be repetitive Tonic

- trunk flexion, open eyes and mouth, upward eye deviation, neck extension

NOTES ON PARTIAL/FOCAL Local electrical discharges which may spread More often secondary seizures Can figure out location from symptoms Aura is characteristic of focal sz Sensory hallucination: sensory cortex Focal motor mvmt: motor cortex Tonic deviation of head and eyes away from side of discharge: frontal cortex Bizzare olfactory/gustatory hallucination: medial-temporal cortex Types .....

Simple- localized, consciousness not affected- generally NO post-ictal phase- motor: focal, jacksonian spread, todds paralysis (a/f up to

hrs),march may occur- motor autmatisms: lipsmaking, fiddling, repeating words- sensory: hallucinations, smells, tastes- autonomic: pupil dilation, salivation, lacrimation, visceral

symptoms (butterflies in stomach)- psychic: fear, deja vu, dream-like states, paranoia, elation

Complex- locaized with altered level of consciousness but not

unconscious- usually amnestic to event but may be responsive during

event- post-ictal state is common- motor, sensory, autonomic, psychic- often caused by focal d/c in temporal lobe :. has been

called temporal lobe szs (poorly); also often affect thinking and behaviour :. has been called psyhomotor szs (poorly)

- commonly misdiagnosed as psychiatric disorder- aura for seconds - min then pt appears distant and

unresponsive, sutomatisms occur in 90% (chewing, swallowing, lipsmaking, scratching, fumbling, disrobing)

Secondarily Generalized- begins focally then spreads- hx will differentiate this- starts w/ aura, lip-smaking, etc- important b/c focal sz implies underlying structural lesion

NOTES ON EPILEPTIC SYNDROMES Infantile Spasms (West Syndrome)

Presents in first year Rapid, jackknife flexor or extensor spasms in clusters Misdiagnosis of colic common as they tend to cry and draw legs up West syndrome = infantile spasms + abnormal psychomotor development

+ hypsarrhythmia on EEG 2/3 have underlying CNS anomaly: brain malformation, tuberous sclerosis LOOK for ash leaf spots, adenoma sebaceum, periungual fibromas (T.S.) 95% mentally retarded Seizures well controlled in < 50% Tx: ACTH, prednisone, bigabatrin

Lennox - Gastaut syndrome Mental retardation, multiple seizure types, classic EEG pattern (spike and

wave) Onset b/w 1-6yrs and may evolve from infantile spasms Frequent seizures of various types Valproic acid usually first line tx but usually end up on several meds

Jeuvenile Absence Epilepsy Begins at 4-12 yo in most Spike and wave on EEG co-incides with absence spell Generalized convulsions occur in 50% Hyperventilation can trigger Prognosis excellent Valproate or ethosuximide for those with generalized seizures

Benign Rolandic Epilepsy Partial epilepsy, 3-13yo Classically seizures occur while sleeping (twisting of mouth) Rolandic region spikes on EEG Treatment controversial

FEBRILE SEIZURE Introduction

Definition = seizure in presense of a fever w/o evidence of CNS infection or other caused cause

Common: 5% of children Occurs b/w 5mo - 5yo; peak at 9-20 months Note age range: most will say < 5 monthers do not get febrile seizures

thus they all require abx pending septic workup Various types: clonic, tonic, tonic - clonic Very low mortality

Classification Simple febrile seizure (97%): non of criterion for complex/complicated Complex febrile seizure (3%)

- duration > 15 min- multiple seizures within the same illness- partial/focal features- neurologically abnormal child (devt, sz, structural)

Features Why did it happen? Thought to occur while temperature rising; 50% have

temp > 39 degrees on first examination; EEG does NOT reveal paroxysmal epileptic activity; ? related to bug and not temperature

What is the rate of recurrence of febrile sz?- 30% have 2nd seizure- 50% of those have 3rd seizure

Is it genetic? Fhx positive in 30% Will the child get epilepsy?

- General population incidence is 0.5-1%- Incidence after SIMPLE febrile seizure is 3%- Incidence after COMPLEX febrile seizure is 10%- Risk increased with fhx of epilepsy

Do febrile seizures have a post-ictal phase: some texts say NO post-ictal phase; it is commonly seen; length of post-ictal phase not predictive of pathology

Will giving the child tylenol prevent the next febrile seizure: NO (Multiple studies including a cochrane review)

General approach Stop the seizure Examine for a source of infection Examine for a neurologic abnormality Decide simple vs complex seizure Decide source vs no source for infection; normal vs abnormal neuro exam Decide well vs unwell child

Approach to Investigations and Management Stop the seizure

- Ativan- Dilantin- Phenobarb

Complex febrile seizure- Labs: CBC, lytes, Ca, Mg, PO4, ? cap gas, ammonia- Draw blood cultures, do LP and full septic work up- Give antibiotics ASAP (before LP if there will be a delay)- CT head (may need to do before LP if there is decreased

LOC but dont delay abx for CT or LP- EEG if CT and LP normal

Simple febrile seizure- Looks unwell: full septic workup, antibiotics after cultures,- Looks well + source of infection: treat source- Looks well and no source of infection

Approach as per any febrile kid Febrile seizures < 5 months uncommon thus

lower threshold for cultures, abx, admit Febrile seizure > 5 months approached as

any other febrile kid (r/o UTI)- AAP guideline 1996 for Febrile seizures: strongly

consider LP in infants < 1yo; NO routine CT/MRI/EEG for febrile sz

- Complete hx, PE looking for causes of fever and focal neurological signs

Treatment Tx cause of fever Admission criteria: complex febrile seizure, social reasons, cause of fever Prophylaxis: AAP recommendation -> NO routine febrile seizure

prophylaxis Febrile seizure prophylaxis is occasionally used for very frequent

recurrences- Do NOT start in ED without neuro consultation- NNT 4-8 to prevent one seizure- High side effects

Discharge instructions- Seizure instructions: turn on side, nothing in mouth, safe

env- When to bring back: as per febrile illness- Follow up: GP or peds- Tylenol prn for fever symptoms

NEONATAL SEIZURES Present DIFFERENTLY than older children and adults --------------------------> Subtle physical

findings are common: lip smacking, staring spells, tongue thrusting, bicycling, apnea, prolonged eye deviation, rhythmic movement, bicycling, peddling, posturing of limbs (sustained)

Generalized tonic - clonic less common (connections between sides are not well developed -corpus callosum - thus harder for seizure activity to become generalized)

Structural Etiologies: CNS bleed: IVH, SAH CNS infarct: hypoxemic ischemic encephalopathy CNS tumor Cerebral dysgenesis

Metabolic Etiologies: Meningitis Sepsis Hypoglycemia

Hypocalcemia Hyponatremia Hypernatremia Pyridoxine deficiency Narcotic (etc) withdrawl

Investigations Septic work up Gluc, Ca, lytes Metabolic d/o screen: lytes, cap gas, ammonia, lactate, serum amino acids, urine organic acids Urine drug tox screen prn CT head EEG

Managment Phenobarbital is first line in neonates 20 mg/kg iv Dilantin, lorazepam second lines Hypoglycemia: 6 ml/kg of D10 Hypocalcemia: 0.2 ml/kg of Calcium Chloride Pyridoxine: 50 mg iv Admit to PICU

DIFFERENTIAL DIAGNOSIS OF PEDIATRIC SEIZURES(similar to altered LOC)

STRUCTURAL Trauma Bleed Infarct Tumor Abscess AVM Hydrocephalus Increased ICP

METABOLIC Major organ failure

Heart: arrrythmia Lungs: hypoxia, hypercarbia Kidneys: uremia Liver: hepatic encephalopathy Brain: hypertensive encephalopathy

Endocrine Hypoglycemia Thyroid storm Myxedemic coma Addisonian crisis

Electrolytes Na: up or down Ca: up or down

Mg P04

Toxicologic EtOH withdrawl Glucose related:Insulin, Oral hypoglycemics Cardiotoxic: BB, CCB TCA, Lithium, Seritonin syndrome, ASA, INH, anticholinergics Anticonvulsants Toxic alcohols

Acidosis: Congenital inborn errors of metabolism causing acidosis Base excess Other

PREGNANCY (ECLAMPSIA) Neurocutanous disorders: sturg weber, neurofibromatosis, tuberous

sclerosis Lytes Infectious

Meningitis, Encephalitis Sepsis TORCH infections

Conversion disorder/psychogeni

NOTES OF VARIOUS ETIOLOGIES Hypoglycemia: common cause, must check chemstrip, can be focal or generalized Ketotic hypoglycemia

MCC of childhood hypoglycemia, presents with new-onset seizure Episodes of symptomatic hypoglycemia associated with periods of calorie

deprivation Onset usually 6 - 18 months Symptoms commonly in morning Seizure often ppt by vomiting/diarrhea Chemstrip normal b/w attacks but low during episode ED dx = hypoglycemia + ketonuria Can be provocated by ketogenic diet Avoid ketogenic diets effective Cataracts are complication: refer to optho (recurrent lens swelling)

Electrolytes Hypernatremia > 160 Hyponatremia < 120 although rate of devt important Hypercalcemia: rarely can cause seizure Hypocalcemia: rarely Hypomagnesemia: rarely

Drugs and Toxins Extensive list of drugs/toxins: see box 168-7 Plants, insecticides, hydrocoarbons, rodenticides also Common: amphetemines, cocaine, PCPs, TCAs, EtOH w/drawl, BZD

withdrawl Less common: ASA, theophylline, isoniazid, lithium, phenytoin,

carbemezepine, Post Traumatic Seizures

Up to 10% after head injuries Impact seizures within 1-2 min not associated with severe injury or

epilepsy Severe injuries more likely to go on to epilepsy

Neurofibromatosis Caf-au-lait lesions Axillary freckling Subcutaneous nodules Seizures and mental retardation are common Optic gliomas, CNS tumors also common

Tuberous Sclerosis Ash leaf spots Adenoma sebaceum Mixed seizure disorder of infantile spasms, severe MR Brain stones: calcified intracranial tumors

Sturge - Weber Port-wine stain, hemiplegia, seizures

CLINICAL APPROACH TO Szs

HISTORY Was it really a seizure? Consider paroxysmal events. Automatisms = coordinated activity which occurs during the state of clouding of

consciousness and for which the patient is amnesic Occur most often complex partial sz but may be seen in absence sz Ex: eating, mimicry (anger, fear), perseverative

Key points in hx Onset: when, where, what doing, rapid, slow, breathholding, grey over

eyes, sweating, lipsmaking, blinking, staring, aura During: mvmts, symmetrical, purposeful, rhythmic Duration: how long After: post-ictal confusion, lethargy, memory of attack

No previous SZ hx vmts) Shx: EtOH, drugs Fhx: szs, neuro disorders ROS: systemic features, fever, any other neurological s/s

Previous SZ hx As above but less detailed Look for change in dose, changed medication, missed dose, change from

brand name to generic medication, noncompliance, drug interactions, new Rx

Look for precipitants: substances, sleep deprivation, stress, infections, strobe light, dehydration, blood sugar, endocrine

Progression of underlying dz Superimposed head trauma Complications of Mx: toxic level of Rx; phenytoin may inc myoclonic sz;

valproate in complex partial sz may inc focal sz; anticonvulsant induced osteomalacia :. hypocalcemic :. inc sz (7 yrs of Rx)

PHYSICAL EXAMINATION Gen: level of consciousness, confusion Vit: fever Derm: lesions of neurocut syndromes (neurofibromatosis, tuberous sclerosis,

sturge/weber H/N: tongue lacerations, broken teeth, auscultate for bruits (AVMs) orbital/cranial/carotid Resp: aspiration MSK: #s, posterior shoulder dislocation Neuro: full exam important - look for focal finding, papilledema

INVESTIGATIONS First SZ

See AAN guidelines Routine blood work has extremely low yield Short seizure, neurologically normal child: no investigations neccessary Persistent altered LOC: CBC, urea, Cr, lytes, Mg, Ca, PO4, toxicology Emergent CT head for suspected serious structural lesion: focal deficit,

persistent altered LOC, persistent h/a, cancer hx, anticoagulant use, partial seizure at onset

AAN recomendation: imaging not routinely indicated for first nonfebrile sz MRI actually better than CT LP in child > 18 months: normal neuro and general examination, no

suspicion for meningitis; does not need LP and can be diagnosed with febrile seizure

LP in child < 12 months: strongly consider LP in all presumed febrile seizures as meningeal signs are unreliable (AAP recommendation)

EEG is recommended for all (AAP recommendation) Previous SZ

CT for change in seizure pattern, prolonged postictal state, persistent abnormal mental status, fever, or other suspicion for new structural lesion

Anticonvulsant level: interpret w/ caution (MCC is Rx noncompliance) May do full w/u depending on presentation

PROBLEMS W/ ANTICONVULSANTS Side-effects: lethargy, irritability, rash Remember serious side-effects: Stevens - Johnson syndrome, hepatic failure

(valproate) Phenytoin toxicity: Nausea, dysarthria, dipolpia, ataxia, impaired LOC ---->

Chronic use: neurtopenia, osteopmalacia, anemia, lupu-like syn, myasthenia, etc Thrombocytopenia Drug interactions can be very important Mvmt disorders

NON-EPILEPTIC PAROXYSMAL EVENTS

PSEUDOSEIZURES Difficult dx that may occur in pts that do have epilepsy (commonly) Ddx w/ EEG monitor: may induce by hyperventilation, tuning forks or IV saline Trick: insert NG tube, the pseudosz pt will b/cm immediately responsive Consider dx when .....

Long hx with no modification by medications Exacerbated by stress or emotional upset Highly suggestible Sz only occur when witnesses present Lack of incontinence, injury, post-ictal phase

SYNCOPE History is the key to distinguish; what happened right b/f you went out Presyncope: lightheadedness, faint, vertigo, greying of vision, pallor, nausea,

diaphoresis Tone decreased vs increased Lack of prolonged post-event confusion Incontinence, injury, fhx, previous sz hx less common

BREATH-HOLDING SPELLS Multiple names: infantile syncope, anoxic convulsion, anoxic seizures, white reflex

syncope MCC of non-ictal paroxysmal events in children Onset in infancy and toddler age and usu resolves by 5 yo 5% of children; fhx in 25%; Simple is only color change; complex if there is loss of tone Breath-holding is a misnomer: involuntary and reflexive and occurs during active or full

expiraiton Commonly initiated by emotional or provocative stimuli Starts by becoming quiet, mouth is wide open in full expiration as the face and trunk

changes color; returns to normal breathing before LOC if simple; complex spells have deepening of the color change then loss of consciousness; tone then changes from limp to opisthotonos and there is occasionally body jerking and urinary incontinence (anoxic seizure); inspiratory gasp then normal breathing; event lasts about 40 seconds; may be hypotonic for few minutes after

Two types: cyanotic (Blue): 60%; palid (white) in 20% or mixed in 20% Ocular compression test: bilateral ocular compression for 10 seconds leads to

bradycardia, asystole > 2 sec, precipitates breath-holding spell Frequently misdiagnosed as seizures:

Seizure: loss of muscle tone and posture BEFORE color change, post ictal, no crying before episode, precipitating event, no opisthotonos

Breath-hold: loss of tone/posture AFTER color change, no post ictal, crying before episode, precipitating event, opisthotonus

EEG useful to help distinguish Other ddx: anemia, brain stem event, syncope, apneas Mangement: generally conservative, atropine orally and scopolamine patches have been

used

OTHER Migraine variant: motor, sensory, autonomic deficits Sleep disorders: nightmares, night terrors Tics: Intermittent, non-rythmic mvmts or utterances Shuddering attacks: shuddering infants, fhx of same or tremors Daydreaming spells: mimics absence sz Panic attacks/hyperventilation Sandifers syndrome: abnormal arching of back and torticollis seen in infants w/ GE

STATUS EPILEPTICUS IN CHILDREN General

See Canadian Pediatric Society Guidelines (cps.ca) Protect from injury, recovery position Airway: push mandible forward, nasopharyngeal airway, intubate if any

concern re airway protection or for need to ventilate, suction airway When to intubate? 45 min and failure to respond to bzd/dilantin (CPS

recmdtn) Breathing: ventilate, pulsox, give oxygen by face mask or np Circulation: establish large bore iv and give NS Emergency tx: chemstrip and glucose 2 ml/kg of DW50 iv + thiamine

100mg iv/im Adjuncts: NG tube to dec aspiration risk, cardiac monitor, pulsox, BP

monitor History: ask parents hpi, pmhx, meds, what controlled previous seizures,

etc Physical: trauma, fever, rash, locus of infection Investigations: CBC, Urea, Cr, lytes, Ca, Mg, P04, glucose, toxicology

screen, myoglobinuria, CK, blood culture Anticonvulsant hierarchy

Lorazepam 0.1 mg/kg iv/sl/pr over 2 min repeat in 15 & 30 min if necc. [or Diazepam 0.2 mg/kg and repeat up to max 2.6 mg/kg or resp depression] +Phenytoin 20 mg/kg iv at maximum of 50 mg/min or Fosphenytoin

Phenobarbital iv 20 mg/kg iv at rate of 1mg/kg/min Barbituate coma (pentobarbitol 2 mg/kg bolus then 1 mg/kg/hr), general

anesthesia, diazepam drip, propofol drip Specifics

Benzodiazepines- respiratory depression and hypotension may occur esp if

coningested alcohol, barbituates, narcotics, or other sedatives

- lorazepam has slower onset of action but longer duration than diazepam :. theoretically preferred over diazepam

- rectal lorazepam: same does; syringe in 2 inch, squeeze buts

- can be injected sublingual

Phenytoin- most imp drug- do NOT give w/ glucos (precipitates), do NOT give im- mixed in propylene glycol: hypotension, tissue toxicity- hypotension, decreased contractility, AV block- contraindications: 2nd or 3rd degree AV block- watch closesly, stop if s/e devp

Fosphenytoin- Produrg, water-soluble, rapidly converted to phenytoin- Can be given im or iv; can be given with dextrose solutions- Can be given faster (150 mg PE/min)- Doesnt contain propylene glycol thus less hyptotension

and tissue/vascular toxicity- Dose: 20 mg PE/kg iv given at rate of 150 mg PE/min- Phenobarbital- respiratory depression common; watch for- ventilator support commonly reqd- may result in prolonged obtundation or coma

Phenobarbitol- Generally third line- Usually first-line in infants < 1yo (dilantin is erratically

absorbed and difficult to maintain appropriate levels) Paraldehyde

- Must be rectal- Must be glass syringe (eats plastic)-

Other Mx Hyponatremia: 4 ml/kg of 3% NS over 30 minutes Hypocalcemia: 0.1 ml/kg of 10% calcium chloride Isoniazid: pyridoxime 1 mg per mg of INH (or 5 mg on spec)

DISPOSITION Indications for admission

Prolonged seizure Abnormal neurological examination Prolonged post-ictal phase Social factors Significant underlying medical conditions

Anticonvulsant Therapy with first unprovoked seizure 1/3 will have recurrence and 75% of those will have third seizure Risk of recurrence increases with +ve fhx, abnormal EEG, seizure during

sleep General recommendation is NOT to treat after first seizure No evidence that early treatment with anticonvulsant effects rates of

further seizures DONT start in ED

Problems with Anticonvulsants Side-effects common problem

Sedation, dizziness, blurry vision, ataxia, GI upset Hepatoxocity, aplastic anemias, agranulocytosis, serum sickness STEVENS - JOHNSON SYNDROME: rash + anticonvulsant, must think

of this, stop anticonvulsant, consult Discharge education

Educate pt and family: can drive a/f sz free for one year whether on or off Rx, take drug at same time, dont miss doses, avoid alcohol, refill prescriptions regularly, wear medi-alert bracelet

Advise about swimming, ladders, dangerous equipment, driving, boating, climbing, gum

Pregnancy: tertaogens, monitorin Inform govt about driving Support groups F/U

NEUROCUTANEOUS DISORDERS

NEUROFIBROMATOSIS Neural crest cells proliferate in multiple foci Diagnostic criteria exist NF1 and NF2 are very different (NF1 is much more common) NF1 Features

Caf-au-lait spots: seen in essentially all; appear by age 1 Neurofibromas: usually appear by teens Lisch nodules of the eye Optic nerve gliomas Astrocytomas Intraspinal tumors: back pain, scoliosis, neuro deficits Hypertension, pheochromocytoma Vascular anomalies Wilms tumor, sarcomas

TUBEROUS SCLEROSIS Disorder of cellular differentiation Ash leaf spots: seen better with woods light Adenoma sebaceum (looks like acne) Peri/subungual fibromas Seizures, infantile spasms Developmental delay Renal angiomas

OTHER Sturge Weber: portwine stain of V1: seizures, intracranial angiomas Ataxia Telangiectasia: progressive ataxia Von Hippel-Lindau: cerebellar or spinal hemangiomas :. present with CB or SC findings

PEDIATRIC HEADACHES

INTRODUCTION Common problem, usually benign but not always Migraines occur in 1% by age 7, 5% by age 15 Vascular theory: vasodilation of cranial arteries causes headache Neuronal theory: wave of neuronal depression and decreased cerebral blood flow Trigeminal theory: headache is an expression of the nerve-blood vessel interaction

CLINICAL FEATURES Acute, chronic progressive or non-progressive History is the key (as per adults): onset, associations, description, pattern, prior headaches,

therapies used, trauma, red flags for tumors Physical: look for HTN, neurocutaneous disorders, neuro findings, fundoscopy

DIFFERENTIAL DIAGNOSIS Benign

Tension Migraine Cluster:

Malignant Infectious: any viral illness with fever, meningitis, encephalitis, sinusitis, otitis

media, mastoiditis, dental absess Traumatic: recall leptomeningeal cysts with recent skull # Hypertension Toxic: sympathomimetics, analgesia rebound, CO CNS tumor AVMs SAH Hydrocephalus Pseudotumor cerebri Congenital Malformations

ACUTE HEADACHES Viral illness with fever is MC diagnosis SAHs due to cerebral aneurysms does occur Same approach as for adults Pediatric Migraines common

CHRONIC NON-PROGRESSIVE HEADACHES Tension Migraine Depression Conversion

CHRONIC - PROGRESSIVE HEADACHES Main concern is increased ICP: tumors, abscess, hydrocephalus, bleeding Signs of increased ICP

Headaches that awaken child or are present first thing in the am Postural changes Noctural or morning emesis Papilledema

Pseudotumor Cerebri Benign intracranial hypertension Normal CT head; normal CSF, high opening pressure on LP Females, younger Obesity, tetracycline, OCP, vitamin A, steroids Tx: diuretics and repeat LPs

DIAGNOSIS History and physical are key CT head prn LP prn MRI

PEDIATRIC MIGRAINES Classification

Classic migraine = migraine with aura Common migraine = migraine without aura Complicated migraine = migraine with hemiplegia, opthalmoplegia, basilar artery,

acute confusion, alice-in-wonderland syndrome Migraine variants: abdominal migraine, BPV, torticollis, ocular migraine

Pediatric Criteria for Migraine without aura (box 168-12) Hemiplegic migraine: sudden onset of hemiparesis or hemisensory loss followed by headache,

more frequent in kids than adults Opthalmopleic migraine: severe unilateral eye pain and headach followed by 3rd nerve palsy Basilar artery: common in kids, visual symptoms, vertigo, ataxia, LOC, drop attacks Acute confusional state: change of personality or behavior with migraine Alice-in-wonderland syndrome: distortions in body images and shapes; objects appear larger or

smaller before, during, or after a headache

PEDIATRIC ATAXIA

DIFFERENTIAL DX Toxic ingestions: EtOH, bzd, lithium, dilantin, carbon monoxide, antiHT, anticonvulsants Posterior fossa structural lesion: tumor, AVM, abscess, infarct, MS, dandy walker cysts Elevated ICP any reason: tumor, hydrocephalus, etc Trauma, non-accidental truma Post infectious cerebellitis Acute Disseminated EncephaloMyelitis

Non-convulsive status Vertebrobasillar migraine Inborn errors of metabolism Peripheral vertigo Peripheral neuropathy: GBS, botulism, Millar fishcer

ACUTE POST-INFECTIOUS CEREBELLITIS Demyelination post viral infection Fairly common Can be any virus but common with VARICELLA, EBV, coxsachie (and mycoplasma) Afebrile, nystagumus, ataxic Investigations

CT head normal LP normal or mild increased wbc MRI with diffuse uptake if MR is done EEG if there is concern for non-convulsive status

Nhx: most resolve over 10 days but occasionally persist for months (worst at onset) Has been treated with steroids Disposition

Do CT and LP Discuss with ped neurology: ? MR, ? EEG, ? d/c home if well

ACUTE DISSEMINATED ENCEPHALOMYELITIS = ADEM Similar to post infectious cerebellitits except has BRAIN STEM findings Brain stem findings = altered LOC, facial palsy, nystagmus, EOM abnormality, other CN palsy May have meningismus CT may be normal LP may be normal or mild incr wbc MRI = multifocal lesions Tx = steroids + IVIG ADEM vs HSV encephalitis may be difficult to distinguish

Send PCR for HSV and treat with acyclovir until pending