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Volume 01

November 2007

Issue 11

Editorial Gaining a Perspective on Childhood Seizures. Experiencing or witnessing a seizure can be frightening, particularly if the seizure occurs in a child. It is likely that recurrent seizures are caused by the brain's underlying predisposition to the development of epilepsy. There is concern that this pattern of seizures may progress to status epilepticus. Several attempts have been made to classify epileptic syndromes and these definitions continue to be refined. Overlapping clinicoelectrical features make classification of patients within a specific syndrome often difficult. The electoencephalogram of these cases may show evolving changes with time and often serial tracings are needed to reach a definite diagnosis. The CT/MRI scan may also be normal initially and show abnormality only later on. Thus, these children, who suffer from profound physical, social and mental handicap, often shift from one physician to another. Proper classification of these patients by understanding their etiology and clinical features is a keenly felt issue. While it is critical to diagnose and apply the best approach to treat seizures, the impact of epilepsy on a child's life often goes beyond the seizures themselves. It is very sad to say Teaching Hospital, Jaffna is not having a Neurologist or a Neuro-Surgeon to provide services for the patients with neurological problems.

Hope these vacancies will be filled soon !

CONTENTS 1. Childhood Epilepsy 2. Group B Streptococcus Disease 3. Hepatitis B 4. Paediatrics-MCQ


Childhood Epilepsy ¬If in doubt about diagnosis, do not label as epilepsy but watch and wait or refer to specialist

DEFINITONS ¬Seizures/convulsions: Paroxysmal disturbance of consciousness, behaviour, motor function, sensation singly or in combination.

¬Diagnosis of epilepsy is clinical

SEIZURE TYPES

¬Epilepsy: Recurrent seizures without any provoking factor and not happening in one situation only.

Generalized

RECOGNITION AND ASSESSMENT

¬Tonic

¬Tonic-clonic ¬Clonic

¬Detailed and accurate history from an eyewitness (beginning, middle and end of episode)

¬Absence ¬Myoclonic

¬When the history is unclear, recording the episode can be useful

Focal ¬W i t h o u t i m p a i r m e n t o f c o n s c i o u s n e s s ( f o c a l m o t o r, sensory, or other types)

¬Episodes occurring only in certain provoking factors (such as fall, emotions, certain posture etc., except photosensitive stimuli) are likely to be non-epileptic

¬With impairment of consciousness ¬Focal with secondary generalizationhistory of aura, Todd's paralysis, focal feature on EEG

learning ¬any underlying problem difficulties, cerebral palsy, HIE, head injury or other CNS insult ¬Look for any co-morbidity

EPILEPSY SYNDROME

¬Family history - may be positive in certain idiopathic generalized epilepsies, some symptomatic epilepsies (tuberous sclerosis), autosomal dominant frontal epilepsies

¬In most cases epilepsy is idiopathic but a few cases have an underlying cause ¬Actively look for cause to guide prognosis, other treatment and option for epilepsy surgery

¬Genetic conditions, e.g. Angelman's syndrome ¬Neurocutaneous syndrome stigmata

¬Identification based on

look for

- Seizure type - Age of onset

¬Neurological examination

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- Neurodevelopmental status 2

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- Appearance of EEG (ictal and interictal)

and arm with gurgling and salivation ¬May become secondary generalized

Childhood absence epilepsy

¬Spikes in one or the other centrotemporal area

¬Usually presents at 3-8 yrs old

¬Awake EEG could be normal and usually asleep EEG would show the spikes

¬More common in girls ¬Several (up to 100) brief episodes in a day

Panyiotopoulos syndrome

¬No post-ictal features

¬Younger children (peak 5 yrs old)

¬Typical EEG three per sec spike and wave

¬Autonomic seizures and behavioral disturbances usually nocturnal

¬10-30% of children have generalized seizures at some stage

¬Usually starts with vomiting and child initially conscious

Juvenile absence epilepsy

¬Subsequent deviation of eyes to one side, impaired consciousness or may end in hemiclonic seizure or (rarely) generalized seizure

¬Usually presents after 9-10 yrs old ¬Absence frequency is less than in childhood absence epilepsy

¬Child looks unwell, unresponsive

¬Cluster after awakening

¬Other autonomic features very common e.g. dilated pupils, pale skin or flushing, incontinence

¬90% of children have generalized seizures at some stage

¬EEG faster spike and wave

¬Usually lasts for a few to 30 min, occasionally for several hours

Juvenile myoclonic epilepsy

¬Typical EEG changes are spike in occipital area

¬Usually presents between 12-18 yrs old

Temporal lobe epilepsy

¬Jerks after awakening common and often unrecognized

¬Focal seizures with impaired consciousness and complex automatism

¬90% of children have generalized seizures at some stage

¬Children with history of prolonged febrile seizure in the early years of life may have mesial temporal sclerosis as a cause of their seizures

¬15-30% of children will have absences

Benign epilepsy of childhood with rolandic spike

¬Other known causes: cortical dysplasia, gliomas, disembryonic neuroectodermal tumor

¬Usually nocturnal seizures ¬Unilateral focal motor seizures of face November 2007

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syndrome and non-convulsive status

Frontal lobe epilepsy ¬Focal motor seizures

EEG not indicated

¬Tonic or clonic seizures may have speech arrest and head rotation

¬Funny turns, apnoeic attacks, dizzy spell, strange behaviour

¬Complex partial seizures ¬Nocturnal seizures are characteristic feature of frontal lobe epilepsy

¬Non-convulsive episodes {e.g. syncope, reflex anoxic seizures, breath-holding episodes (ECG more appropriate)

¬Ictal EEG can be normal

¬Febrile seizures

¬Can mimic pseudo-seizures

¬Single uncomplicated generalized tonic-clonic seizure

Other epilepsy syndromes

¬To monitor progress in wellcontrolled epilepsy

¬Multiple brief seizures in the night

¬Epileptic encephalopathy in newborns (myoclonic or Ohtohara syndrome)

¬Prior to stopping treatment

¬West's syndrome (infantile spasms)

Indications for MRI of brain

¬Severe myoclonic epilepsy of infancy (Dravet syndrome) ¬Lennox-Gastaut syndrome

¬Focal epilepsy (including TLE) except Rolandic seizure

¬Laundau-Kleffner syndrome

¬Epilepsy in children < 2-yrs-old

¬For other epilepsy syndromes see International League against Epilepsy website (http://www.ilae-epilepsy.org)

¬Myoclonic epilepsy

INVESTIGATIONS

¬Seizures continuing in spite of firstline medication

¬Intractable seizures ¬Loss of previous good control

Indications for EEG

¬Associated neurological deficits or appearance of new neurological signs

«Clinically diagnosed epilepsy «After an episode of status epilepticus «Unexplained coma or encephalopathy

¬Developmental regression in children with epilepsy

«Suspicion of non-convulsive status in children with learning difficulties and epilepsy

¬Infantile spasm (West's syndrome)

Other investigations

«Acquired regression of speech or language function

¬Sleep or sleep-deprived EEG useful

«Developmental regression suspected to have neuro-degenerative condition

in all children in whom there is a high clinical suspicion but awake EEG normal (Sleep EEG is useful to pick

«To monitor progress in West's November 2007

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¬Start antiepileptic only if diagnosis certain (two or more unprovoked seizures)

up focal epilepsies and sleep-deprived EEG is useful in generalized epilepsies including JME. Sleep EEG should be done with Melatonin

¬Preferably after initial EEG results obtained

¬Video telemetry useful if diagnostic dilemma, pseudoseizures or prior to surgery

¬Start with small dose and build up to half maintenance increase to full maintenance if seizures continue

¬Drug levels only if concerns about

¬Increase dose stepwise every two to three weeks

compliance and overdose

¬Biochemistry glucose, calcium, LFT, lactate, ammonia: metabolic and genetic investigations where suspicion of metabolic disorder e.g. progressive developmental delay

First line drugs ¬Carbamazepine start with 2.5 - 5 mg/kg per day in two divided doses increasing to 20 - 30 mg/kg per day OR

¬Epileptic encephalopathies, such as West's syndrome, need a series of investigations (discuss with paediatric neurologist)

¬Valproate start with 5 - 10 mg/kg/day in two divided doses increasing to 40 mg/kg per day

TREATMENT

¬Avoid polypharmacy; do not add a second medication unless the full or maximum tolerated dose of the medication has been reached (discuss with a paediatrician with

General guidelines ¬Discuss treatment with a consultant before starting

Drugs of 1st, 2nd and 3rd choice in the treatment of seizure types Seizure type

First

Generalized Epilepsy

Sodium valproate or Carbamazepine

Childhood Absence Focal

Sodium valproate Ethosuximide Carbamazepine

Infantile spasms

Vigabatrin or ACTH

Second Carbamazepine + or Sodium valproate Lamotrigine* Sodium valproate Lamotrigine Topiramate Levitiricetam Prednisolone Sodium valproate Nitrazepam

Third Lamotrigine*, Topiramate Levitiricetam Levitiricetam Topiramate Clobazam Phenytoin

Trail of pyridoxine

+ Carbamazepine should be avoided in childhood absences, juvenile absences and juvenile myoclonic epilepsy and can increase seizure in some epileptic encephalopathies and generalized epilepsies * Lamotrigine can increase myoclonic seizure in some myoclonic epilepsy syndromes

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special interest or paediatric neurologist before adding second drug)

FURTHER OPINION/REFERRAL TO SPECIALIST SERVICE ¬Behavioural or developmental regression

¬Aim to switch to monotherapy after a period of overlap

¬Epilepsy syndrome cannot be identified

¬Sugar-free preparations of liquids should be used

¬Child < 2 yrs-old

¬Potential adverse effects must be discussed with parents and documented in the notes

(AED) do not ¬Antiepileptic drug control seizures ( within 2 yrs) ¬2 AEDs have been tried and are unsuccessful

¬If child develops adverse effects, discuss and reduce dose

¬Unilateral structural lesion

¬Additional advice regarding safety (e.g. supervision when swimming) must be discussed and documented

¬Diagnostic doubt about seizure type and/or syndrome ¬Specific syndromes such as SturgeWeber syndrome, Rasmusen's encephalitis

¬Discuss and prescribe rescue treatment, especially in generalized epilepsy, with training for the parents

¬Risk of unacceptable side effect of medication

¬Provide written information, including about epilepsy associations

WITHDRAWAL OF ANTIEPILEPTIC DRUGS

¬Parents and children should be allowed to ask questions, especially about sensitive issues such as sudden death

¬Consider when child has been seizure free for two years

SUBSEQUENT MANAGEMENT

¬Discuss the risks of recurrence (25-30 %) if this occurs, recommence treatment

Increase dose of antiepileptic until good control towards full dose or maximum to lerated dose

¬Recurrence is very high in some syndromes (e.g. juvenile myoclonic epilepsy 70-80%

If suboptimal control with one drug or unacceptable side effects, start 2nd-line drug

- usually require lifelong treatment) ¬Postpone withdrawing AED if important events such as grade 5 scholarship are looming

OUT-PATIENT MANAGEMENT ¬Initial follow-up at six to eight weeks

¬Gradual withdrawal over 2 to 3 months is usual

¬Subsequent follow-up/structured review every 3-12 months based on clinical need

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¬Some drugs (Phenobarbital or benzodiazepines) need very slow withdrawal over 6-12 months 6

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ACUTE SEIZURE MANAGEMENT

Airway, High flow O2, Glucose Stix

Vascular access

Yes

No

Lorazepam 0.1 mg/kg IV/IO (dilute 1:1 with 0.9% Sodium Chloride)

Diazepam 0.5 mg/kg PR OR Midazolam 0.5 mg/kg buccal

10 min

10 min Yes

Lorazepam 0.1 mg/kg IV/IO

Vascular access?

10 min

No

Paraldehyde 0.4 mL/kg PR, dilute with equal volume of olive oil Phenytoin â&#x20AC;&#x201C; with cardiac monitoring - 18 mg/kg IV/IO over 20 min (dilute in 0.9% sodium chloride) If already taking phenytoin, give Phenobarbitone 20 mg/kg IV/IO over 10 min

Call Anaesthetist

Thiopentone 4 mg/kg IV/IO - Intra Venous { IV IO - Intra Osseous} November 2007

Compiled by: Dr. S.K.Arulmoli

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Group B Streptococcus Disease «If caesarean section with intact membranes and no suspicion of chorio-amnionitis or maternal fever:

RECOGNITION AND ASSESSMENT DEFINITION Group B streptococcus (GBS)

- no investigation necessary

disease is classified as follows:

«If one baby from multiple birth has GBS disease, treat other babies empirically

«Early Onset within first 7 days of life: - often with rapid onset in first hours after birth

«Chorio-amnionitis is treated with broad-spectrum antibiotics

- risk can be reduced by giving maternal intrapartum antibiotics

SUBSEQUENT MANAGEMENT

«Late onset cannot be prevented

«If

antibiotics were indicated but not given more than 4 hr before delivery:

RISK FACTORS

- observe for 12 hr

«Give intrapartum antibiotics to the mother with:

«If signs of neonatal sepsis take blood culture and surface swabs, perform lumbar puncture and give empirical treatment with benzylpenicillin and gentamicin

- previous infant with invasive GBS disease - GBS bacteriuria this pregnancy «2 or more of the following present:

«O b s e r v a t i o n d o e s n o t n e e d admission to neonatal unit

- GBS on vaginal swab in this pregnancy

«The following are NOT indications for starting antibiotics:

- fever >38°C during labour - prematurity <37 weeks

- vaginal GBS before labour if mother asymptomatic

- rupture of membranes >18 hr in labour

- vaginal GBS in previous pregnancy

IMMEDIATE TREATMENT

- term rupture of membranes when not in labour

«Vaginal delivery: give mother benzylpenicillin 3 g IV, then 1.5 g 4 hrly during labour

- a well baby more than 12 hr after delivery even if risk factors present

- if allergic to penicillin, give clindamycin 900 mg 8 hrly until delivery November 2007

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Hepatitis B 0.5mL(lomcg)for: - all babies born to mothers at high risk

RECOGNITION AND ASSESSMENT Investigation

- (Caution: other brands have different doses)

«For

all newborns, check antenatal screening results for mother’s tests

«HBIG 2 mL (whole ampoule) 200 units for babies of mothers who:

«If

antenatal testing not done (e.g. concealed pregnancy) request urgent maternal HBsAg test

- are HBeAg +ve - if it is not known whether eAg +ve or not

«I f

a w a r e a n t e n a t a l l y, i n f o r m obstetrician, neonatologist, and public health team P of the plan to immunise

OR - if acute hepatitis in pregnancy

«Give mother information about hepatitis B, modes of transmission and prevention of spread

«Both should be given within 48 hr of birth, ideally as soon as possible after delivery

Immediate Postnatal Treatment of the baby

«Use two separate injection sites, in the anterolateral thighs (not buttocks)

«Labour ward must inform on-call neonatal team when a mother who is HBsAg +ve arrives in labour or for caesarean section

Subsequent Management «Local hospital to give the subsequent doses of hepatitis B vaccine

«Hepatitis B vaccine e.g. Engerix B - Second dose at 1month

Vaccine required by baby

Immunoglobulin required by baby

HBs Ag positive, HbeAg positive

Yes

Yes

HbsAg positive without e markers (or where e marker has not been determined)

Yes

Yes

Acute hepatitis B during pregnancy

Yes

Yes

HbsAg positive, anti-Hbe positive

Yes

No

Other high risk group

Yes

No

Maternal status

- Third dose at 6 months «Check the child's HBsAg status 6 weeks after the 6 month dose - Optional

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News in Brief *************** Dialysis unit, at Teaching Hospital, Jaffna, started to function again after a lapse of nearly one year, with the fixation of water purification plant and other accessories. The main source of fund to rectify this unit was donated by IMHO(USA). At present three patients can be dialyzed simultaneously. ************ Dr.(Mrs).A.Narani and Dr.T.Pratheepan were successful in the MD Part I Examination and Dr.S.Sribavanantharajah was successful in the MSc Medical Administration Examination that were conducted by the PGIM, University of Colombo. JMA News Letter would like to congratulate these medical officers. ************* This year Ministry of Health has posted 66 medical officers to Jaffna District in the post intern list. Among them 42 were allocated to Teaching hospital, Jaffna and 24 were allocated to the RDHS Sector to serve in the peripheral hospitals.

Message of Condolence We inform with great pain, the untimely demise of Dr.(Mrs).G.Kugathas who shouldered the dermatology services single handed in Jaffna peninsula for more than three decades Her praiseworthy contribution to the betterment of Health Service and Medical Education in Jaffna will be remembered and respected for ever It is prudent that the vacuum created by her loss should be filled by her students with great enthusiasm We Sincerely feel that this is the least respect we can show to Late Dr.(Mrs).G.Kugathas November 2007

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Paediatrics-MCQ 1. In Tuberculous Meningitis (TBM): A. The signs and symptoms normally develop two to three weeks after initial infection with the pathogen B. Most patients are young children C. A negative tuberculin PPD skin test reliably discounts the possibility of infection in young infants D. Patients invariably have a primary site of infection outside the CNS E. Normally, antimicrobial treatment should be withheld until a positive identification of the pathogen becomes available 2. The following is an absolute or relative contraindication for lumbar puncture: A. Raised intracranial pressure D. Platelets less than 20 x 109/L B. Headache E. Raised INR C. High grade fever 3. BCG vaccination is contraindicated if the patient is: A. HIV positive B. Living in an area with a high incidence of tuberculosis C. Suffering from leukaemia D. On steroid therapy E. Receiving antibacterial agents for a concurrent non-mycobacterial infection 4. In acute exacerbations of Cystic Fibrosis (CF) associated with Pseudomonas aeruginosa: A. A combination of two intravenous aminoglycosides at low doses has been shown to be superior to one aminoglycoside given at conventional dosage B. The prime aim of treatment is to eradicate the pathogen C. IV antibiotics have been shown to be more effective than nebulised antibiotics D. IV antibiotic therapy achieves lower sputum levels of drug than nebulised therapy E. Oral ciprofloxacin is often given as long term prophylaxis 5. Use of nebulised tobramycin in patients with CF: A. Causes fewer long term side effects than intravenous treatment B. Achieves higher sputum concentrations than intravenous treatment C. Is more effective in the treatment of acute Pseudomonas aeruginosa infections than intravenous tobramycin alone D. Requires plasma level monitoring and dosage adjustment to minimise the risk of toxicity E. Is commonly associated with bronchospasm November 2007

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6. With the gastrointestinal complications of CF: A. Up to 90 per cent of patients have some pancreatic insufficiency B. Doses of lipase enzymes above 5,000 units/kg/day are likely to cause colonic strictures in children C. If patients have diabetes, total calorie intake should be low D. Pancreatic enzyme supplementation may result in hyperuricaemia E. Most pancreatic enzymes currently used are produced by recombinant DNA techniques 7. Adverse effects sometimes associated with anti-epileptic include: A. Vigabatrin causing epidermal necrolysis B. Topimarate causing renal stone formation C. Lamotrigine causing drowsiness D. Sodium valproate causing liver failure E. Lamotrigine causing epidermal necrolysis 8. The ILEA classification of syndrome: A. Makes use of an understanding of the associated anatomy B. Makes use of information from EEG recording C. Divides partial seizures from partial seizures with secondary generalization D. Divides location-related epilepsies into three main categories E. Classifies each syndrome as representing a specific condition 9. In the interaction between sodium valproate and phenytoin A. Sodium valproate displaces phenytoin from its binding site B. More phenytoin is initially available for metabolism C. Sodium valproate acts as an enzyme inducer D. Sodium valproate acts as an enzyme inhibitor E. Hypoalbuminaemia affects the amount of free phenytoin 10. A vaccine is available for the prevention of meningitis caused by the following organisms: A. Group B meningococci D. Group C meningococci B. Streptococcus pneumoniae E. Herpes viruses By: Dr. S.K.Arulmoli C. Polioviruses Answers for Ophthalmology MCQ (Volume 1, Issue 10) 1. TTFFT 6.TFFTF

2. FFFTT 7. TFFTF

3. TTFFT 8. TTFTT

4. TFFFT 9.FTFTF

5. TFTFF 10. FTFFT

Answers for the Paediatrics MCQ will be given in the next month issue.

JMA Newsletter 11/07  

Newsletter for November 2007 for the Jaffna Medical Association, Issue 11