200 \200 s c i r t a d e a P osmolarity to 300 \200\223310, and CVP to 2 \200\2235cmH2O). \200 Pulse & BP continuously. \200 Send to neurosurgical center now. Herpes simplex encephalitis (HSE) is the most treat able encephalitis. \200\207Think of it in any febrile child with focal or general seizures and CNS (esp. temporal lobe) signs \200\201consciousness. Signs are often nonspeci \201 c. 675 Nasolabial herpes is often absent. CNS de \201 cits may be mild or gross (eg h emiparesis). Tests: CT, EEG and CSF often nonspeci \201 c (do PCR). MRI is better than CT. \201\227: \200 Start aci- clovir. If >12yrs old: 10mg/kgIBW/8h by IVI over 1h eg for 3wk s (20mg/kg/8h in neonates). 676 Monitor U&E & urine output; Mortality: 7%. 60% survive int act. CNS sequelae: Kluver \200\223Bucy syndrome (hypersexuality, rage, visual agnosia); aph asia; 677 amnesia; auditory agnosia; autism. 678 Brain tumors \200\207Arrange urgent refer ral if unexplained headache and/or fo- cal symptoms, eg progressive weakness or numbness, u nsteadiness, diffi culty speaking, or vision changes/VI nerve palsy. \205\224 are in the p osterior fossa. Con- sider brain tumors in children with lethargy, behavioral change, visua l dis- turbances, diabetes insipidus (polyuria/polydipsia), growth disturbances (e.g. growt h failure , delayed/arrested/precocious puberty), nausea vomiting. 679 Medulloblastoma: Midline cerebellar embryonal tumor (inferior vermis) causing ICP \200\200, speech diffi cul ty, truncal ataxia falls. \200 : \200 \211\210 4 : 1. Peak age: 4yrs. Seeding is alon g CSF pathways. Rx: surgical resection + radio/chemo-therapy. Brainstem astrocytoma: Most c ommon brain tumor in children. Associated with neuro \201 bromatosis 1) and prior radiatio n. Cranial nerve palsies; pyramidal tract signs (eg hemiparesis); cerebellar ataxia; signs of ICP \200\200 are rare. Midbrain and third ventricle tumors may be astrocytomas, pinealom as or colloid cysts (cause posture-dependent drowsiness). Signs: behavior change (early); p yramidal tract and cerebellar SIGNS; upward gaze defect. Suprasellar gliomas: Visual \201 eld defects; optic atrophy; pituitary disorders (growth arrest, hypothyroidism, delayed pu berty); diabetes insipidus (DI). Cra- nial DI is caused by ADH \200\201, so that there is p olyuria and low urine osmolality (always <800mosmol/L) despite dehydration. Cerebral hemisp heres: Usually gliomas. Meningiomas are rare. Fits are com- mon. Signs depend on the lobe i nvolved (OHCM p503). Tests: MRI/CT EEG. Op- tions: Excision if possible; CSF shunting; r adiotherapy; chemotherapy alone. 680 Other space-occupying lesions Aneurysms; haematomas; g ranulomas; tub- erculomas; cysts (cysticercosis); \200\207abscess: suspect if ICP \200\200 ; T \200\200; WCC \200\200. Get help. 1 Venous sinus thrombosis risk \200\200 if: infecti on; perinatal problems; blood dyscrasias. Signs: \200\201consciousness (50%), papilloedema (18%), cranial nerve palsy (33%), hemiparesis, hypotonia. Thrombolysis may be needed.681 i Children \200\231s coma scale (An objective record of coma level to help quantify prognosis and monitor progress.) Use if <4yrs; if >4, use Glasgow scale, p720. \200 Best motor res ponse (6 grades, quanti \201 ed in the blue numbers below) 6 Carrying out a request ( \200 \230obeying command \200\231): Moves to your request. 5 Localizing response to pain: Put pr essure on the patient \200\231s \201 nger nail bed with a pencil or sternal pressure: pur poseful movements towards chang- ing painful stimuli is a \200\230localizing \200\231 resp onse. 201 4 Withdraws to pain: Pulls limb away from painful stimulus. 3 Flexor response to pain: Pres sure on the nail bed causes abnormal \202 exion of limbs \200\224decorticate posture. 2 Extensor posturing to pain: The stimulus causes limb extension (adduction, internal rotation of shoulder, forearm pronation; decerebrate posture). s c i r t a i d e a P 1 No response to pain. Score best response of any limb. Best verbal response (5 grades) If intubated can the patient grimace? 5 Orientated: Smiles, is orientated to sounds, \201 xes and follows objects. 4 Crying but consolable (or inter action odd/inappropriate) 3 Inconsistently consolable (or moaning) 2 Inconsolable crying (o r irritable) 1 No response Eye opening (4 grades) 4 Spontaneous eye opening. 3 Eye opening in response to speech: Any, not just a request to open eyes. 2 Eye opening to response to pain: Pain to limbs as above. 1 No eye opening. Add the score in the 3 areas. Eg: no response to pain + no verbalization + no eye opening = 3. As a rule of thumb, <8 \211\210intubation needed; 4 \200\2238 \211\210 intermediate p rognosis; 3 \211\210 bad prognosis. Migraine In children we modify migraine criteria to include bilateral or frontal headache l asting 1 \200\22372h with nausea/vomiting + any 2 of: photophobia, phonophobia, dif- \201 culty thinking, lightheadedness, or fatigue 682 (vertigo and abdo pain also occur) sometim es heralded by visual or sensory aura. Most children have to lie down during attacks; sleep resolves the attack. Prevalence: 5% (10% in adolescence). Triggers: School pressure, tantr ums, excitement: upset, hypoglycaemia, sleep lack or excess (weekend migraine), sensory eve nts (noise, light, heat/cold), sympathetic activity (sports). Drugs: (as early as possible in an attack); par- acetamol (p143); ibuprofen 10mg/kg PO (see p143). 683 If this fails and over 6yrs, try sumatriptan, 684 eg if weight >30kg. 683 If vomiting, use rectal (paracetam ol suppositories) or nasal routes (sumatriptan dose: 12 \200\22318 years 10 \200\22320mg st at, repeated once after \211 2h if needed) or IV chlorpromazine (0.15mg/kg if >8y; max 10m g). 685 Non-drug treatments: Relaxation training, biofeedback, self- hypnosis, and guided i magery have a role. 686 Prophylaxis: Encourage regular sleep. Consider propranolol and pizo tifen. Amitriptyline and sodium valproate are used, but note lack of robust evidence of eff i cacy. 687 Nimodipine, timolol, pa- paverine, trazodone, clonidine, metoclopramide, and do mperidone are \200\230ineff ec- tive \200\231. 688 Signs for prompt referral: \200 Heada ches of increasing frequency or severity, or if aged <6yrs. \200 Headache unrelieved by p aracetamol or ibuprofen. \200 Irritable; loss of interest/skills; slowing of physical or cognitive development. \200 Head circumference above the 97th centile, or greatly out of line. Other causes of headache Viruses; meningitis; sinusitis (frontal sinus not de- velope d until >10yrs); hypertension (always do BP), stress, behavioral. (OHCM p462) Encephalitis Signs Flu-like prodome, consciousness \200\201; odd behavior; vomiting; \201 ts; T \200\200; meningism. Infective causes: include HSV ( \200 aciclovir, p200); mumps (ask about parotiditis/testicular pain); varicella zoster (recent chicken pox?); rabies (do g-bite abroad); parvovirus (slapped cheek syndrome), immunocompromise (CMV, EBV, HHV-6); in \202 uenza; toxoplasmosis; TB; mycoplasma; malaria (if a possi- bility do a thick blood \201 lm and enlist specialist help, OHCM p394); dengue; Rick- ettsia. 689 Do CSF PCR; test stool, urine, and blood. Non-infective differential: hypoglycaemia; DKA; kernicterus (p115 ), hepatic failure (eg Reye \200\231s syndrome), lead or other poisoning, subarachnoid hemo rrhage, malignancy, lupus. 690 Investigations: CSF MC&S and PCR; bloods, stool (enterovirus es), urine \200\207Prolonged fevers \200\224consider: endocarditis; Still \200\231s (p654) ; malignancy. \200 Meningitis APLS, NICE Suspect this in any ill baby or child. Symptoms m ay be subtle, especially in infants \200\224irritability, abnormal cry, lethargy and diffi culty feeding. Signs in- clude fever, seizures, apnoea, bulging fontanelle. \200\207Get ex pert help from your senior. If there is any hint of meningococcal disease, \200 GIVE IV CE FTRIAXONE (50 \200\22380mg/kg; max 2 \200\2234g). See BNFC 5.1.2. Sending blood/CSF cultu res must not delay this. Septic signs: commonly present before meningeal signs \200\224incl ude T \200\200; cold hands/feet, limb/joint pain, abnormal skin color, odd behavior; rash (don \200\231t expect petechiae with meningococcus); DIC; pulse \200\200; BP \200\201; tac hypnoea; WCC \200\200. Meningeal signs: comparatively late, and less common in young childr en, they are neither sensitive nor speci \201 c 691 \200\224include stiff neck ("unable to kiss knee"; often absent if <18 months); Kernig \200\231s sign (resistance to extending kn ee with hip \202 exed); Brudzinski \200\231s sign (hips \202 ex on bending head forward ); photopho- bia; opisthotonus Other causes of stiff neck: Tonsillitis, pneumonia, subarach noid bleed. Lumbar puncture: Often inappropriate (see p200); contraindicated if: focal sign s; DIC; purpura or brain herniation is near (odd posture or breathing; coma scale <13, p201 ; dilated pupils, doll \200\231s eye re \202 exes, BP \200\200, pulse \200\201, papilloede ma). Pre- liminary CT cannot show LP will be safe. Technique: (OHCM p782) Learn from an exp ert. \200 Explain everything to mother. \200 Get IV access \201 rst: acute deteriorat ion is possible. Ask an experienced nurse to position child fully \202 exed (knees to chi n) on the side of a bed, with his back exactly at right angles with it. \200 Mark a point just above (cranial to) a line joining the spinous processes between the iliac crests. \200 Drape & sterilize the area; put on gloves. \200 In \201 ltrate 1mL of 1% lidocaine super \201 cially in the older child. \200 Insert LP needle aiming towards umbilicus. K eep the needle perpendicular to the back at all times. \200 Catch 4 CSF drops in each of 3 bottles for: urgent Gram stain, culture, virology, glucose, protein (do blood glucose too ). \200 Do CNS observations often. \200 Report to mother. After LP: FBC, U&E, culture b lood, urine, nose swabs, stool virology. CRP: is it >20mg/L? CXR. Fluid bal- ance, TPR & BP hourly. Is CSF lactate >3mg/L? (a more sensitive way to distinguish bacterial from aseptic meningitis than the blood/CSF glucose ratio.) Treating pyogenic meningitis before the orga nism is known \200 Protect airway; give high- \202 ow O2; set up IVI: if in shock give 0. 9% saline in 20mL/kg boluses. If shock persists beyond 3 boluses \200\224consider intubatio n and inotropic support. Call a pediatric intensivist early. 202 \200 \200 s c i r t a d e a P i \200 Ceftriaxone 50 \200\22380mg/kg/day (max2 \200\2234g) IV infusion if >3months \200\223 18yrs. \200 Cefotaxime 50mg/kg \200\224/12h if <7d; /8h if 7 \200\22321d; /6h if <21 days \200\2233months) PLUS amoxicillin/ampicillin p204. \200 Dexamethasone (0.15mg/kg/6h IV eg for 4 days) with 1st antibiotic dose (in de- velope d countries) 692 unless the organism is very likely to be N. meningitidis. 693 \200 If pre-hernia signs, treat for ICP \200\200 (p200), eg mannitol 20%, 2.5 \200\2235mL/ kg IVI. Treat for cryptococcus if HIV+ve. After culture, check the minimum inhibitory conce ntration (MIC) of the antibiotics used to the organism in vitro. Complications Disseminated sepsis, subdural eff usion, hydrocephalus, ataxia, paralysis, deafness (steroids prevent t his), IQ \200\201, epilepsy, brain abscess. 203 CSF in meningitis Pyogenic: Tubercular: Aseptic: Normal range if >1 month old clear \211 5 lymphocytes; 0 neutrophils > \205\224 of blood Appearance 694,695 often turbid may be \201 brin web usually clear mononuclear Predominan t cell polymorphs 50 \200\2231500/mm3 eg 1000/mm3 > \205\224 of blood < \205\224 of blood mononuclear 10 \200\223350/mm3 < \205\224 of blood Glucose level Protein (mg/dL) \200\200 (mean \211\210300) \200\200 >40 (mean \211\21020 0) \200\20640 and <1500 <40 Bacterial antigen detection for N. meningitidis, H. in \202 u enzae & streps helps in partially treated meningi- tis. CSF lactate typically rises before the CSF glucose falls in pyogenic meningitis. Preventing deaths from meningococcal disease Because death is so swift, this can seem a hopeless task. Nevertheless, the following (and using your 6th sense) can save lives, and the meningococcus is not always that fast. \200 Rapid skilled assessment of all febrile children. Nurse triage is unreliable. \200 Don \200\231t expect meningeal signs; septicaemic signs are more fatal. \200 Any rash (or non e) will do for the meningococcus. If you wait for purpura, s c i r t a i d e a P you may be waiting until the disease is untreatable. For any acutely unwell child leave your consulting room door ajar. Explain that a doctor can be contacted at any time if: \200\223He seems to be worsening \200\223A rash develops \200\223Poor response to antipyretics \200\223Cold hands or feet; odd skin color \200\223Unrousable or crying in an odd way \200\223Odd features eg limb/joint pain Beware fever + lethargy + vomiting, even if no headache or photophobia. \200 Give parente ral penicillin early (p205), before admission to hospital. \200 Monitor pulse, BP, respir atory rate, pupil size/reactivity, level of conscious- ness (AVPU, p103); WCC and platelets (both may be \200\201). \200 Urgent activation of and transfer to pediatric intensive c are unit (PICU). Do blood gasses to assess degree of acidosis. Get fresh frozen plasma to t he bed- side now to deal with coagulopathy. Intubation, ventilation and vigorous IV \202 uid resuscitation must be prompt. Monitor catheter urine output. Cross- match blood. Contin uously monitor ECG. \200 Inotropes may be needed: dopamine or dobutamine (same dose) eg a t 10 g/ kg/min (put 15mg/kg in 50mL of 5% glucose and infuse at 2mL/h). 696 This is OK by peripheral vein, but if adrenaline is needed, use a central line (0.1 g/kg/ min, ie 300 g /kg in 50mL of 0.9% saline at 1mL/h). 696 \200 If plasma glucose <3, give 10% glucose 5mL /kg as a bolus, then as needed. Heroic, non-standard ideas: Extracorporeal membrane oxygena tion; termi- nal fragment of human bactericidal/permeability-increasing protein (rBPI21) to \200\201cytokines; heparin with protein C concentrate to reverse coagulopathy; plasma- ph eresis to remove cytokines, and thrombolysis (rTPA) for limb reperfusion. 697 Other issues Ensure immunization (protein \200\223polysaccharide conjugate vaccine prevents type C but n ot the common type which is B; p151). Inform your local CDC (consultant in communicable dis eases). \200\207Stop parents smoking! 37% of cases are put down to aerosolized spread via smokers coughs. 698 Meningococcal prophylaxis for contacts with aerosolized meningococci (o r deep kissing). 699 Rifampicin \200\22412hrly for 4 doses PO: if <1yr, 5mg/kg; 1 \200\2231 2yrs: 10mg/kg (max 600mg); >12yrs: 600mg. It interacts with the contraceptive Pill, can sta in contact lenses, and turns urine red. Ceftriaxone single IM dose: 125mg if <12yr; 250mg i f >12yrs. Cipro \202 oxacin 500mg PO is an option if \211 12yrs old (250mg if 5 \200\2231 2yrs). Meningitis: the organisms The meningococcus, the pneumococcus, and, in the unvaccinated, Ha emophi- lus in \202 uenzae are the great killers. In the former, the interval between seem - ing well and coma may be counted in hours. If you suspect meningococcal disease, give ben zyl-penicillin (BOX) before hospital admission \200 700 (do not worry about ruining the ch ance of a positive blood or CSF culture later). Neisseria meningitiis Abrupt onset rash (purpuric or not, eg starting as pink macules on legs in 20%); septicaemia may occur with n o meningitis (Wa- terhouse \200\223Friderichsen, OHCM p707), so early LPs may be normal, gi ving false reassurance. Arthritis, conjunctivitis, myocarditis & DIC may coexist. Typical a ge: Any. Movie: Gram \200\223ve cocci in pairs (long axes parallel), often within poly- mo rphs. Drug of choice: cefotaxime/ceftriaxone (p202) or benzylpenicillin (G) 50mg/kg/4h IV. If penicillin and cephalosporin-allergic, give chloramphenicol (below). Treat shock with co lloid ( inotropes, p203). Prevention: p202. Haemophilus in \202 uenzae (Rare if immunize d). Typical age: <4yrs. CSF: Gram \200\223 ve rods. The lower the CSF glucose, the worse t he infection. Drugs: Ceftriax- one (p202) or, where there is no resistance, chloramphenicol 12 \200\22325mg/kg/6h IVI (neonates: see BNF) + IV ampicillin \200\224if \211 1wk old, 30 \200\22360mg/kg/12h (/8h if 1wk \200\2233wks old; /6h if 3 \200\2234wks old). If >4wks old 25mg/kg/6h (max 1g), doubled in severe infections. Rifampicin (below) may also be needed. With chloram- phenicol, monitor peak levels; aim for 20 \200\22325 g/mL; usual doses may b e far exceeded to achieve this. (Trough level: <15 g/mL.) As soon as you can, switch to PO (more eff ective). Steroids (p202) prevent hearing loss. Strep pneumoniae Typical age: Any . Risk factors: Respiratory infections, skull fracture ( \200\207is this \200\230minor run ny nose \200\231 CSF rhinorrhoea?), meningocele, HIV. 701 Movie: Gram +ve cocci. \201\227: Ceftriaxone, p202 or benzylpenicillin 50mg/kg/4h slow IV \200\224or, if resistance likely (eg parts of Europe and USA) rifampicin. As an add-in or an alternative, consider vancom ycin, if >1 month old: 15mg/kg/8h (max 2g/24h) IVI over 1h, but CSF penetration is unreliab le. 702 Monitor U&E. E. coli This is a major cause of meningitis in neonates (in whom signs may consist of feeding diffi culties, apnoea, seizures, and shock). Drug of choice: ce- fo taxime (p202) or gentamicin (p175). Group B haemolytic streptococci eg via mother \200\231s vagina (so swab mothers whose infants suddenly fall ill at 24h-old). Infection may be del ayed a month. Drug of choice: benzylpenicillin 25 \200\22350mg/kg/8 \200\22312h slow IV. Li steria monocytogenes presents soon after birth with meningitis or septi- caemia ( pneumon ia). It is rare unless immunocompromised. Microabscesses form in many organs (granulomatosi s infantiseptica). \202\202 : Culture blood, CSF, placenta, amniotic \202 uid. \201\227 : IV ampicillin (above) + gentamicin (p175). 703 TB can cause CNS infarcts, demyelination w ith cranial nerve lesions, and tuber- culomas meningitis (long prodrome with lethargy, m alaise, and anorexia). Photophobia and neck stiff ness are likely to be absent. The 1st few CSFs may be normal, or show visible \201 brin webs and widely varying cell counts. Dose examples: Isoniazid 10mg/kg/24h PO max 500mg (? with vit B6) + rifampicin 10mg/kg/24h ( \211 600mg/day) for 1yr + (for 2 months) pyrazinamide 35mg/kg/ day with eg streptomycin if >4wks old, eg 20 \200\22330mg/kg once daily (max 1g) IM adjusted to give a peak plasma leve l <40 g/mL and a trough of <3 g/mL; alternative: ethambutol 15mg/kg/day PO if old enough to report visual prob- lems. 704,705 Adding dexamethasone improves survival (at least in th ose >14yrs old) but probably does not prevent disability. 706 Children <14 should be given prednisolone 4mg/kg/24h for 4 weeks then follow a reducing course. 705 Other bacteria Lepto spiral species (canicola); Brucella; Salmonella. Causes of \200\230aseptic \200\231 mening itis Viruses (eg mumps, echo, herpes, polio), par- tially treated bacterial infections, cry ptococcus (use ink stains). 204 \200 s c i r t a d e a P i 205 Giving IM benzylpenicillin before hospital admission \200 300mg IM up to 1 year old. \200 600mg if 1 \200\2239yrs. \200 1.2g if >10yrs. \200 When in doubt, give it: it may be negligent not to do so. \200 If penicillin-allergic, cefotaxime may be used (50mg/kg IM stat; if >12yrs 1g). 707 s c i r t a i d e a P Fig 1. Image of glass test in purpuric rashes (eg meningitis). The rash has stained, and do es not blanch. Dr Petter Brandtzaeg, and the Meningitis Trust. Fig 3. 4-month-old girl with gangrene of hands due to meningococcaemia. CDC. Fig 2. Tuberculoma. Caseous (cheese-like) nec- rotic material is surrounded by epithelioid cell granulomas. Rupture releases mycobacteria into the subarachnoid space, hence causing T B meningitis. Prof. Dimitri Agamanolis. Epilepsy and febrile convulsions EPILEPSY is a tendency to intermittent abnormal electrical brain activity. Clas- si \201 cation depends on whether signs are referable to one part o f a hemisphere (partial epilepsy) or not (generalized), and on whether consciousness is af- fected (complex) or not (simple). Seizures in generalized epilepsy may be: \200 Tonic/cl onic (grand mal): Limbs stiff en (the tonic phase) and then jerk 206 \200 forcefully (clonic phase), with loss of consciousness. Absences (petit mal): Brief (eg 10sec) pauses ( \200\230he stops in mid-sentence, and carri es on where he left off \200\231); eyes may roll up; he/she is unaware of the attack. Infantile spasms/West syndrome: Peak age: 5 months. Clusters of head nodding ( \200\230Sala am attack \200\231) and arm jerks, every 3 \200\22330sec. IQ \200\201 in 70%. 708 EEG is c haracteristic (hypsarrythmia). \200\224: vigabatrin (SE: visual \201 eld defects); 709 A CTH. 710 \200 Myoclonic seizures: 1 \200\2234yrs; eg \200\230thrown \200\231 suddenly to the ground. \200\224: valproate. Partial epilepsy Signs are referable to part of one hemi sphere. Complex phe- nomena: (temporal lobe \201 ts) consciousness \200\201; automatisms (lip smacking, rub- bing face, running); \201 ts of pure pleasure. 711,712 Causes Often n one is found. Infection (eg meningitis); U&E \200\200, glucose \200\201, Ca2+ \200\201, Mg2 + \200\201; Na+ \200\200 \200\201; toxins; trauma; metabolic defects; tuberous sclerosis; C NS tu- mour (<2%) or malformation; \202 ickering lights, eg TV; exercise. 713 \201 \201 : Arrhythmias, migraine, narcolepsy, night terrors, faints (re \202 ex anoxic sei- zures, p207), tics, M nchausen \200\231s (eg by proxy, OHCM p720). Tests Expert EEG; MRI is the preferred choice- it is more sensitive and has no exposure to radiation. CT may be more ava ilable and not require an anaes- thetic \200\224use in emergent situations to look for acut e hemorrhage/lesions. \200 714 A SIMPLE FEBRILE CONVULSION is a single tonic \200\223cloni c, symmetrical generalized seizure lasting <20min, occurring as T \200\200 rises rapidly in a febrile illness \200\224typi- cally in a normally developing child ( \200\2235yrs ol d). Think of meningo-encephali- tis, CNS lesion, epilepsy, trauma, glucose \200\201, Ca2+ \200\201, or Mg2+ \200\201 if: \200 Focal CNS signs or CNS abnormality \200 Previous hi story of epilepsy \200 The seizure lasts >15min \200 There is >1 attack in 24h. Lifetim e prevalence: 3% of children have at least one febrile convulsion. Examination: Find any i nfection; if any neck stiff ness consider meningitis. \201\227: Put in recovery position; if \201 t is lasting >5min: lorazepam IV, buccal midazolam (p208), or diazepam PR. Tepid sponging if hot; 715 paraceta- mol syrup (p143). Consider FBC, U&E, Ca2+, glucose, MSU, CXR , ENT swabs. 716 To LP or not LP? Risk of pyogenic meningitis is as low (<1.3%) as the risk in a febrile child with no seizures 717 if all the above criteria are ful \201 lled. Avoi d LP in the post ictal period as a CNS assessment will be impossible. If you suspect mening itis, then treat now. Parental education: Allay fear (a child is not dying during a \201 t). Fe- brile convulsions don \200\231t usually ( \200\2053%) mean risk of epilepsy. For th e 30% having recurrences, teach carers to use buccal midazolam or rectal diazepam 0.5mg/ kg , eg with a 5mg tube (Stesolid ) if 1 \200\2233yrs,1 or a 10mg tube if older only dur- ing seizures. Further prevention: Diazepam PR during fevers has a role; 718 other anticonvulsa nts are \200\230never \200\231 needed. Explain that all fevers (eg vaccination-asso- ciate d) should prompt oral antipyretics (p142), but that this does not necessar- ily avoid anoth er seizure, with diazepam PR to hand if needed. Prognosis: In typical febrile convulsions ( de \201 ned above) there is no progress to epilepsy in 97%. 719 Risk is much higher if pre -existing CNS abnormality (50%), epilepsy in a \201 rst degree relative, or complex febri le seizures. s c i r t a d e a P i 1 If <3yrs insert PR half-way to nozzle mark. 2.5mL is expellable; don \200\231t worry abo ut the bit left behind; it \200\231s allowed for by the manufacturer. Alternative: diazepam Rectubes : its license is for a 0.5mg/kg single dose (adults & children >10kg, a typical weight for a 1yr-old), which shouldn \200\231t be repeated until after 12h; very few report s of respiratory problems at this dose: all survived. Only insert half-way if <15kg, and al ways press the buttocks together after giving.720 Dose example: 1 month \200\2232yrs: 5mg: 2 \200\22312yrs: 5 \200\22310mg. 207 Are these paroxysmal/episodic \200\230spells \200\231 epilepsy? This is often a true dilem ma, and it matters as epilepsy treatment can be toxic. Also, if it is harmful to label any child, it is doubly so to mislabel a child. So always get help with the diagnosis. Watching and waiting, repeat EEGs, and videos of attacks (eg on mobile phone) may be need- ed. 721 In one study of 22 babies <1yr old referred for \200\230?epilepsy \200\231, 9 did have epi lepsy. The other 13 showed one of \201 ve patterns of non-epileptic par- oxysmal events ( NEPE) \200\2241 blinking 2 Head-shaking movements 3 Body posturing with head and arm jerk 4 Rhythmic masturbation movements ( \200\230grati \201 cation disorder \200\231, eg with gr unts, leg-crossing, \202 ushing etc \200\224in the presence of a normal EEG) 5 Myoclonic head \202 exion. NEPE may be clini- cally indistinguishable from epilepsy. In the infants described above, \211 4 interictal EEGs were normal, the spells completely resolved after a fairly short period without anticonvulsants, and the infants continued to de- velop norm ally with no evidence of epilepsy at follow-up. 722 Some speci \201 c types of seizure Re \202 ex anoxic attacks Paroxysmal, self-limited brief (eg 15sec) asystole triggered by pai n, fear (eg at venepuncture) or anxiety, or an overwhelming confrontation with reality, per haps in the form of a bath which is unexpect- edly hot or cold. During this time the child is deathly pale \200\224 hypotonia, rigidity, upward eye deviation, clonic movements, and urinary incontinence. Typical age: 6 months to 2 years (but may be much older). Prevalence : 0.8% of preschool children. \201\231 \201\231: Epilepsy is often misdiagnosed, as the tr igger as- pect to the history is ignored or unwitnessed. When in diffi culty, refer to a sp ecialist for vagal excitation tests under continuous EEG & ECG monitoring (ocular compressi on induces the oculo-cardiac re \202 ex; do not do this test if there is glaucoma, or know n arrhythmia, or if you lack experience: it is uncomfortable). NB: tongue-biting is not des cribed in re \202 ex anoxic seizures. Management: Drugs are rarely, if ever, needed. Atrop ine has been tried, to reduce sensitivity to vagal in \202 uences. Anticonvulsants are not needed. Pacemakers might be an option. What to tell parents: Avoid the term \200\230sei- zure \200\231, as this is all that is likely to be remembered, however careful your explana tion. Pallid syncopal attack, or white breath-holding attacks are useful synonyms. Emphasiz e its benign nature, and that the child usually grows out of it (but it may occur later in life, and in older siblings). 723 Panayiotopoulos syndrome (6% of all epilepsies) A benign focal seizure disorder occurring in early and mid-childhood (peak age: 5yrs). Autonomic sym ptoms may predominate. EEG: shifting and/or multiple foci, often with occipital predominanc e. It occurs mainly at night, with vomiting and eye deviation with impaired consciousness b efore the convulsion starts. Many seizures last for 30 minutes (some may last hours) \200 \224but there is no perma- nent brain damage. Treatment: as remission often occurs within 2 years, antiepileptic medication is often not needed. Reassure. 724 Age-dependent epileptic encephalopathy Ohtahara syndrome: tonic spasms clustering. EEG: suppression-burst. 725 Chloral hydrate may help. 725 This transforms over time into West syndrome, and thence to L ennox \200\223Gas- taut syndrome. Think of these as age-speci \201 c epileptic reactions t o non- speci \201 c exogenous CNS insults, acting at speci \201 c developmental stages. 7 26 Rolandic epilepsy (benign epilepsy with centrotemporal spikes, BECTS) 15% of all childho od epilepsy. Infrequent, brief partial \201 ts with unilateral facial or oropharyngeal se nsory-motor symptoms, speech arrest hypersaliva- tion. Sulthiame may be used in some uni ts. 727,728 s c i r t a i d e a P ? y p a r e h t Mozart \200\231s sonata for 2 pianos, K448, has been found to reduce interictal epi- leptif orm EEG discharges (compared with Beethoven \200\231s F r Elise) in a rand- omized, single -blind, crossover, placebo-controlled trial. 729 t r a z o M Epilepsy: management Stepwise \201 of status epilepticus Supportive therapy: \200 Secur e airway; give O2. Set a clock in motion \200 Check T ; if \200\200, give rectal parace tamol (it may be a febrile convulsion) \200 Do BP, pulse, glucose, Ca2+ ( Mg2+). \200 \207If hypoglycaemic, give glucose 5ml/kg IV of 10% solution, then 5 \200\22310mg/kg/min as 10% glucose IVI. \200 Seizure control: proceed to the next step only if \201 ts continu e \200 714 0min \200 ABC. High \202 ow O2. Estimate weight. Check blood glucose. IV acc ess 5min \200 Lorazepam 0.1mg/kg IV; slow bolus via a large vein OR buccal midazolam 0.3 \200\2230.5mg/kg; squirt half between lower gum and the cheek on each side. 208 (Neonates: p112; see also APLS) \200 15min Repeat lorazepam. Call for senior help. Prepare phenytoin. 25min \200 Phenytoin 20mg /kg IVI; over 20mins. Monitor ECG OR (if on regular phenytoin) \200 Phenobarbital 20mg/kg over 5mins \200 Paraldehyde mixed with equal part of olive oil \200\2240.8mL/kg of mixture (max 10mL) as a single dose PR; a void contact with plastics/rubber may be given on direction of senior staff . Call PICU & y our anesthetist \200\224prepare for intubation locate ET tube,etc p627 45min \200 Rapid sequence induction use \200 thiopental 4mg/kg. Transfer to PICU \200 \207These times refer to elapsed time on the clock from the 1st drug, not gaps between each drug. Some authorities recommend starting ventilation earlier, and always be ready to do t his to protect the airway. Tests SAO2, ECG monitor, glucose, U&E, Ca2+, Mg2+, arterial gass es, FBC, platelets, ECG. Consider anticonvulsant levels, toxicology screen, blood ammonia, lumbar puncture (after resolution), culture blood and urine, virology, EEG, MRI, CT, car- b on monoxide level, lead level, amino acid levels, metabolic screen. Once the crisis is over Refer to a specialist: is MRI or prophylaxis, eg with sodium valproate or carbamazepine (p 209) needed? Aim to use one drug only. Increase dose until \201 ts stop, or toxic levels reached. Out of the context of sta- tus, prophylaxis is typically started after the 2nd sei zure. Choice of anti-epilep- tic drug (AED) should be based on epilepsy syndrome/presenting seizure type. Carbamazepine SE: Rash ( exfoliation); platelets \200\201, agranulocytosis , aplasia (all rare). It induces its own enzymes, so increasing doses may be needed. Sodium valproate (200mg/5mL). SE: vomiting, appetite \200\200, drowsiness, platelets \200\201 (do FBC pre- \200\224). Rare hepatotoxicity can be fatal (eg if coexisting unsuspected metabol ic disorder). Monitor LFT, eg in 1st 6 months. When prescribing to girls of present and fut ure childbearing potential, warn of teratogenic risk. Ethosuximide The syrup is 250mg/5mL. SE: D&V, rashes, erythema multiforme, lupus syndromes, agitation, headache. Indication: abs ence epilepsy. Lamotrigine Uses: absences and intractable epilepsy as an add-on. Dose when given with valproate if 2 \200\22312yrs: 0.15mg/kg/day PO for 2wks, then 0.3mg/kg daily for 2wks, then \200\200 by up to 0.3mg/kg every 1 \200\2232 weeks. Usual maintenance: 1 \200 \2235mg/ kg/day (higher if with non-valproate anticonvulsants: 2.5 \200\2237mg/kg/12h). Vi gabatrin (May be 1st choice in infantile spasms and tuberous sclerosis \201 ts.) This blo cks GABA transaminase. Consider adding it to regimens if partial sei- zures are uncontrolle d. Starting dose: 15 \200\22320mg/kg/12h increased after 2wks to a typical dose of 30 \200 \22340mg/kg/12h (max 150mg/kg/day). Blood levels do not help (but monitor concurrent phenyt oin: it may fall by 20%). SE: Drowsiness, depression, psychosis, amnesia, diplopia, and \201 eld defects (test every year). Levetiracetam licensed for used as monotherapy for foca l seizures \200\224but thought not cost eff ective (NICE \200 714). SE: depression; lethar gy; weakness Diet Consider a high-fat ketogenic diet if 2 drugs fail to work (it can \200 \201 \201 ts by \205\224). 730 It needs supervision. SE: constipation, vomiting, \200 \201energy, hunger. Education If \201 ts are few, educate teachers on lifting bans on sup ervised swimming, cycling etc. Showers are safer than baths. Emphasize compliance/ concorda nce (one seizure may \200\201the threshold for the next, ie kindling). 731 s c i r t a d e a P i Newer anticonvulsants NICE reserves gabapentin, lamotrigine, oxcarbaz- epine, tiagabine, to piramate, and vigabatrin (as an adjunct for partial seizures) for children not bene \201 t ing from (or able to tolerate) older drugs (above) or older drugs have contraindications/in teractions (the Pill) \200\224or the child is of childbearing potential or is likely to nee d drugs into her childbearing years. 706 209 , e n p e z a m a b r a C e n p e z a m a b r a c 0 0 7 \200\223 0 8 2 L / l o m a g n i r e p a t 0 8 1 \200\223 0 6 M J E N e d o r B d n a a m s a l p n 0 8 \200\223 0 4 i r o i h g u o r t t e g r a T y r e g r u s l l u f p e h u f p e h i e h t l l i l e v e l . , t o N t o N e t a m a r i p o t f i y s p e s c i r t a i d e a P . r e t a e r g o n s n o i t a n b m o c L / g m 9 9 \200\223 0 4 0 2 \200\223 0 1 0 4 \200\223 5 1 2 1 \200\223 4 g u r d r o ; l i n o m o o S p e ) s e r u z i e s t n a t s i s e r - g u r d n I i l r o m a t e c a r i t e v e s i . e n , k o o b t x e t s s e c o r p n w o d g n i r e p a t y r t i l y a d r e p c i n o l c o y m e t a b r e c a x e s e s o D . d n o c e s s e r u z i e s 3 \200\223 2 3 \200\223 2 2 \200\223 1 2 \200\223 1 2 \200\223 1 n e h t 2 e v i s n e h e r p m o c . e n p e z a b r a c x o , e n g i r t o m a 2 3 7 . I R M d n a e r a e c n e s b a i l i , e s o d e v i t c e ff e m a z a b o l c h 4 2 / g k / g m e t a m a r i p o t 1 . 0 \200\223 5 2 0 0 y 0 4 \200\223 5 1 0 2 \200\223 0 1 l l a u s U 5 2 \200\223 0 1 5 1 \200\223 5 8 \200\223 4 r o f d e d n e m m o c e r l y r t e c i v d a e h t g n i r u d e c n e r r u c e r s e d u l c n . a , r o y a m k r o w , y s p e r o m a t e c a r i t e v e L e t a o r p a v m u d o s l a c i g r u s o r u e n ( l i i r e h t i e n e n g i r t o m a 3 3 7 . 3 4 \200\223 8 3 : ) 1 ( 8 3 8 0 0 2 p E t n e m t a e r t l a v r e t n I s y a d n i , l 7 0 0 2 0 1 0 1 t n e m e r c n 5 7 7 1 t o n f I i l e g n E J . e t a o r p a v m u d o s e r a i e v i t c n u d A r o f l y r t ; e r u z i e s h 4 2 / g k / g m n i r t a b a g v d n a i e s o D r e f e r \200\207 . n e h t e n e b a t i u s n u : e b a t l 5 2 0 0 l e z i S 0 0 1 j l i l 5 5 0 0 5 0 . 1 ; l . . . . o r u e N r t a d e P d n a 2 t s r \201 s i . ; . f o : s n o i t a n b m o c e n g i r t o m a . ) s e r u z i e s i r o f . ) s d o r e t s o c i t r o c k s i r i i e c r u o S i f I e h T e t a o r p a v m u d o S t n e m s s e s s a e s o d t e g r a T e n b a g a i t , i h 4 2 / g k / g m . i d n o c e s e t a o r p a v m u d o s l a i t i n t c e ff e f o i . 7 9 4 p M C H O e c n e s b a d n a 5 0 0 5 . l s h t n o m 9 0 2 5 1 . 6 7 r o l 2 1 . c i x o t i r o f l ; e d m x u s o h t e e n p e z a m a b r a c , E T I S O P P O e e S n o t y n e h p i i i e r o m r o ( n i r t a b a g V i i e e S s t n a s l u v n o c i t n a g n p p o t S h t i i h 4 2 / g k / g m w d e r a p m o c g n i t r a t S c i n o l c o y m e t a b r e c a x e r e v e : s t \201 c i t e n 5 2 0 0 e s o d y r t , 0 1 0 1 e n p e z a b r a c x o 5 5 3 e s u . i . , t s r i F p e h e c i o h c i l y r t t o n : s m s a p s y a m i : s t \201 c i t s r i F 4 3 7 . 8 1 k a s k e e w 6 o D 4 1 7 \200 ? g u r d h c i h W t s r i F e n p e z a m a b r a C r o t e d c i n e g o t e k l l i a f e d m x u s o h t E a t i b r a b o n e h P i , : s t \201 8 6 1 m a p e z a n o C i , n o c i : s e c n e s b A n i t n e p a b a g g u r D i n o t y n e h P e l i t n a f n I e t a o r p a V n o s g u r d f I l c \200\223 c i i i 4 3 3 s i l i a l c o y M d o i r e p i t r a P y a m ; 6 9 9 1 n o T l l ( Behavioral problems \200\207Only enter battles you can win. If the child can win, be more subtle, eg con- sistent rewards, not inconsistent punishments. Get a health visitor \200 \231s advice; ensure everyone is encouraging the same response from the child. Entrances Fo od refusal and food fads are common. Reducing pressure on the child, discouraging parental over-reaction, and gradual enlarging of tiny portions of attractive food are usually all th at is needed. Check Hb. Keep a watchful eye on growth and weight gain. Overeating: Eating c omforts, and if the child is short on comfort, or if mother feels inadequate, the scene is set for overeating and lifelong patterns are begun. Diets may fail until the child is hospi talized (p348). If obese (p226, p530), remember hypothalamic syndromes (eg Prader \200\223W illi, p652), but the treatment remains the same. Pica is eating things which are not food, eg plastic, or feces (coprophagia); if persistent, look for other disturbed behaviors, auti sm, or \200\201IQ. Causes: iron or other mineral de \201 ciency; obsessive-compulsive dis order. Complications: lead poisoning; worm infestations. 735 Behavior modi \201 cation may help. 736 Exits Constipation is diffi culty in defecation; it may comprise of <3 stools pe r week; large hard stool; \200\230rabbit dropping \200\231 stool; distress/straining/bleed ing with passage of stool. It may lead to abdominal pain, abdominal masses, over \202 ow s oiling \200\230lavatory-blocking \200\231 enormous stools (megarectum), and anorexia. C aus- es: diet, poor \202 uid, or \201 bre intake \200\224or fear, eg as a result of a \201 ssure. Rarely Hirschprung \200\231s disease (p130). Failure to pass meconium in \201 rst 48h? Ask about onset of constipation and precipitants ( \201 ssure/change in diet /timing of potty training/fears and phobias/moving house/acute infections/family upheavals) . Red \202 ags include. \200 737 210 \200 s c i r t a d e a P i Constipation from birth or \201 rst few weeks. Failure to pass meconium within 48h. Falte ring growth (an amber \202 ag really; consider coeliac disease/hypothyroid). New weakness /abnormal re \202 exes in legs, delayed locomotion. Abnormal appearance of anus / skin in sacral/gluteal region (look for sacral dimples/hairy patches/ \202 attening of gluteal mus cles/multiple \201 ssures). Gross abdominal distension with vomiting.