14_McClure_14.indd 502 11/14/2012 3:36:06 PM PAROXYSMAL EPISODES: NOT EPILEPSY 503 Absences as part of a focal seizure disorder will only very rarely occur without some other suggestive feature such as an automatism, abnormal movement, or postictal state. Syncope \200 \200 Also known as fainting. Occurs from age 7mths onwards. There may well be a history of preci pitating events (e.g. fright, head bang, sudden standing, hair- brushing). Often the child has an aura of loss of vision, tingling, and auditory phenomen, this is followed by loss of consciousness and posture change: falls over if standing. Not all syncopal events result i n a loss of tone. In some, the fall is accompanied by increased tone. Myoclonic jerks may f ollow for a few seconds. Useful tools in diagnosis include: a history of a precipitant; jer king lasting less than 20secs; and the movements may not be rhythmic. If in doubt assume th at it is syncope until there is evidence otherwise (see Psychologically determined paroxysm al events (PDPE)). (cid:1) Caution If there is a history of sudden death in the family, or of syn- cope induce d by sudden physical stress such as exercise or sleep, long QT syndrome should be investiga ted. Psychologically determined paroxysmal events (PDPE) This is a less pejorative term to descr ibe episodes of psychological origin that used to be described as hysteria, and more recent ly as pseudosei- zures, malingering, factitious or conversion disorders. The episodes are a psychological phenomenon, although identifying or looking for the psy- chological causes a t time of diagnosis can be misleading or even counter- productive. There is no single event that will separate them from epilepsy. Some children rarely may even have both. Features suggestive of, but not diagnostic of PDPE \200 \200 Events triggered by speci \201 c situations Events with convulsive movements that are not explained anatomically, e.g. left arm jerking, then lull followed by right leg Thrashing mo vements that wax and wane, +/ \200\223 pelvic thrusting Eyes open during the episode Slumpi ng to the \202 oor in a dramatic manner. Falls without injury Violence, rather than viole nt movements Gain from the situation Generalized movements with rapid return to normal \200 \200 \200 \200 \200 These features are not diagnostic. There is no never or always. In partic- ular, young peop le can injure themselves and pass urine in PDPE, which are often misreported as a diagnosti c feature of genuine seizures (see b p.505). 14_McClure_14.indd 503 11/14/2012 3:36:06 PM CHAPTER 14 Neurology 504 Paroxysmal episodes: general management Assessment History The majority of paroxysmal episodes can be classi \201 ed with a careful his- tory . No episode can be safely classi \201 ed, even after EEG and MRI, if an adequate history has not been taken. Take details of the following: \200 when, where, what happened, and the child \200\231s First episode: responsiveness; how long , recovery, and talk to the witness. Subsequent episodes: Full medical history, family hist ory, developmental and psychosocial history. \200 situation, precipitants, duration, frequency. Video If you are unsure about the diagnosis, then request the carers to take a vid- eo reco rding of the event. Do not investigate or treat until the diagnosis is con \201 rmed. Chil dren are safer off treatment, when the clinician is unsure, if the caution below is followe d. Even when you are sure of the diagnosis, it is good clinical practice to request video r ecording of all different parox- ysmal events, since the episodes or events may evolve. (cid:1) Caution Even when the diagnosis could be a non-epileptic disorder, until this is co n \201 rmed, the carers should be advised how to manage a genuine seizure. The child shoul d avoid speci \201 c dangers: - - - - injury from fall proximity to swimming water without an identi \201 ed lifeguard unprotect ed heat moving objects and machinery Management Infantile non-epileptic disorders, syncopal episodes and jerks Allay the carers \200\231 concerns over the diagnosis. Psychologically determined paroxysmal events See b p.503. PDPE can be dif \201 cult to tre at, but these patients do respond to well organized management. The principal areas include the following: \200 Unambivalent diagnosis explained to both the parent and the child/ y oung person. Acknowledgement/acceptance by the young person, carers and all health professi onals that these are non-epileptic. Stabilization phase where the family is developing unde rstanding. Strengthen coping abilities and remove gain from the behavior. Psychological sup port is essential. Some families will feel very threatened when the possibility is raised o f looking at psychological issues that may have triggered these events in the child. \200 \200 14_McClure_14.indd 504 11/14/2012 3:36:06 PM SEIZURES AND CHILDHOOD EPILEPSIES 505 Seizures and childhood epilepsies One per cent of children will have had one seizure, not a ssociated with fever, by the age of 14yrs. The majority of these seizures will be general- ized tonic \200\223clonic episodes. Forms of epilepsy The two main forms of epilepsies can be categorized as having generalized or focal seizures. Generalized seizures These can be described as follows: \200 with shock-like movement of one/several parts or the whole Myoclonic: body. Tonic: with sustained contraction and stiffness. with rhythmic jerking of one limb, one side, or all of the Clonic: body. (See how this contrasts with the descriptio n of psychologically determined clinical events, b p.503). Tonic \200\223clonic: Absence: 5 \200\22315s in younger children, but can be longer in the older child. They can be associa ted with retropulsion of the head, upward deviation of eyes and eyelid, or perioral myoclon ia. (You should note that facial myoclonia can be asymmetrical and give the impression of a \200\230focal \200\231 seizure). \200 \200 a combination of the above forms. these are episodes of abrupt psychomotor arrest lasting Focal seizures These seizures start in one area of the brain and then may spread, and ultim ately generalize. If the latter part of the event is witnessed it may be described incorrec tly as being primarily generalized. The semiology depends on the locality of the initial el ectrical activity. \200\230Typical \200\231 seizure semiology includes the following: \200 Occipital: homonymous visual \201 elds. Centroparietal: marching up one side of the body. Temporal: disturbances (e.g. familiarity, \200\230d j vu \200\231), autonomia (e.g. au tomatisms such as nose rubbing), and contralateral clonic or dystonic movements. Frontal: multicolored bright lights spreading from one area of sensorimotor phenomena spreading from one limb and feelings of gastric discomfort, strangeness, anxiety, memory dystonic posturing and strange guttural noises. Status epilepticus (StE) StE can be convulsive with tonic/clonic movements. Alternatively, it can be non-convulsive with impairment of consciousness and often subtle twitch- ing. Tec hnically, StE is a seizure lasting for more than 30min, or repeated seizures lasting more t han 30min without recovery of consciousness in between. Practically, though, the treatment algorithm for StE can be used once a convulsive seizure has lasted longer than 5min (see b pp.80 \200\22381). 14_McClure_14.indd 505 11/14/2012 3:36:06 PM CHAPTER 14 Neurology 506 Seizures: management First unprovoked seizure \200 History: obtained. Examination: neurological diseases, particularly skin and dysmorphism. E lectroencephalography should be obtained. The current opinion is that, in most children, it is unlikely to in \202 uence management. Few specialists would start therapy at this poin t whatever the EEG showed. With expert neurophysiology a more accurate prognosis may be giv en, which in turn may in \202 uence therapy. Imaging: if abnormality is found on physical examination then MRI is very important to exclude a space-occupying lesion. a full account of personal, social, and family history should be perform a thorough examination looking for markers of (EEG): there is debate as to whether an EEG MRI is not indicated after a single seizure alone. However, Febrile seizures These can occur in infants or small children. Most last a minute or two, b ut it can be just a few seconds. Others last for more than 15min. \200 Typically, these children have no prior neurological disease or focal de \201 cits on exam ination. Here are some key facts about febrile seizures. They occur in up to 4% of all chil dren, generally between the ages of 6mths and 6yrs (although it is unusual to have one \200 \231s \201 rst episode when aged >4yrs). These children may have a temperature temperatur e may have become normal by the time it is measured. The seizure tends to occur during the \201 rst day of fever. Children prone to febrile seizures are not considered to have epil epsy. Recurrence risk of seizures is 35% over lifetime; 25% during the next 12mths. The vas t majority of febrile seizures are harmless. 95 \200\22398% of children who have experience d febrile seizures do not go on to develop epilepsy. Children who have febrile seizures tha t are lengthy, affect only part of the body, recur within 24hr, or who have neurological ab normalities have a higher incidence of subsequent epilepsy. \211 39*C, however the \200 \200 \200 Categorization \200 generalized tonic \200\223clonic activity lasting Simple febrile seizures (typical): <15min with associated fever. Complex febrile seizures ( atypical): these occur in up to 15% of cases and are characterized by focal seizure activit y, or prolonged seizure longer than 15min, or multiple seizures within a day. Convulsive se izures that occur in a child with no neurological problems, in the context of an intercurre nt infection, even without a recorded fever, are normally classi \201 ed as febrile. 14_McClure_14.indd 506 11/14/2012 3:36:06 PM SEIZURES: MANAGEMENT 507 Management of febrile seizure Safety \200 \200 \200 Move any danger away from the child and consider their privacy Place the child on a protect ed surface on their side It is good practice to note the time Assistance \200 \200 The family should call for help if unfamiliar with febrile seizures Then call ambulance Treatment \200 If the seizure lasts >10min, the child should be treated for status epilepticus Once the se izure has ended, the child should be assessed for the source of the fever, investigated, an d treated appropriately Consider admission and observation, especially if this is the \201 rst episode Meningitis? \200 Consider meningitis if the child shows symptoms of stiff neck, extreme lethargy more than 4 hr post-seizure, abundant vomiting, or is <12mths old If there is concern perform a lumbar puncture as long as the child is not encephalopathic Seizure prevention and home care \200 There is poor evidence to support interventions to prevent febrile seizures Parents should give standard antipyretics early in any febrile illness Parents should get expert advice if a previous seizure lasted >10min \200 14_McClure_14.indd 507 11/14/2012 3:36:06 PM CHAPTER 14 Neurology 508 Epilepsies: neonatal See also b p.134. Neonatal seizures are rarely part of a benign epilep sy syndrome, expert advice should be sought. \200 They are more commonly a symptom of underlying, severe cerebral dysfunction. Seizures are n ever generalized tonic \200\223clonic seizures because the brain has not matured enough to produce synchronous epileptic activity. Management \200 History: prognosis? Take a history for cerebral insults such as hypoxia- ischaemia. Is there a relevant family history, including consanguinity? Examination: featur es. Blood investigations: (calcium and phosphate). Lumbar cell count (WCC); CSF microscopy and growth culture; CSF lactate and glycine; CSF latex agglutin (group B streptococci). is there a family history of similar convulsions with benign look for neurocutaneous stigmata and dysmorphic FBC; CRP; blood glucose; serum electrolytes puncture: CSF glucose, red blood cell count (RCC), and white Epileptic encephalopathy If no cause is evident consider the investiga- tions for epileptic encephalopathy (see Box 14.1). Follow advice of the biochemist for further investigation o r management of relevant results Drug treatment \200 Phenobarbital: once daily for at least 2wks. Pyridoxal Phenobarbital, treat with pyridoxal phosphate. If possible wait for 48hr to assess effect. Clonazepam: th e seizures continue to give the child signi \201 cant cardiorespiratory compromise, commen ce infusion of IV clonazepam at 5micrograms/ kg/min increasing as seizures continue up to a maximum of 20micrograms/kg/min. treat by loading with 20mg/kg IV. Continue on 5mg/kg phosphate 10mg/kg/qds PO: if the infant is unresponsive to if the pyridoxal phosphate has proved ineffective or 14_McClure_14.indd 508 11/14/2012 3:36:07 PM EPILEPSIES: NEONATAL 509 Box 14.1 Investigations for epileptic encephalopathy Examinations \200 Wood \200\231s light: \200 Ophthalmology: ophthalmological markers of neurological disorders MRI: tuberous sclerosis retinitis pigmentosa, phakomata, and other neuronal migration defects, structural abnormalities Blood: routine \200 U&E, urate: \200 Liver function tests: \200 Ammonia: \200 Lacta te \200 Thyroid function tests \200 Chromosomes: renal and purine disorders liver dysfunction urea cycle defects and liver failure : mitochondrial disease : thyroid disease major structural chromosomal abnormalities Blood: special biochemistry \200 Plasma amino acid (including glycine and serine) and total homocysteine: aminoacidaemias an d defects in homocysteine remethylation Transferrin isoelectric focusing: Biotinidase: Carn itine pro \201 le: \200 \200 congenital defects of glycosylation biotinidase de \201 ciency mitochondrial fatty acid B-oxidation defects Blood and CSF \200 Plasma glucose matched with CSF glucose: de \201 ciency CSF lactate: CSF glycine and serin e: 3-phosphoglycerate dehydrogenase de \201 ciency glucose carrier transportation \200 mitochondrial cytopathies non-ketotic hyperglycinaemia and Urine \200 Amino and organic acids: oxidase de \201 ciency, and molybdenum cofactor de \201 ciency d isorders of purine and pyrimidine metabolism Purine and pyrimidine: Creatine to guanidinoac etate ratio- disorders of creatine metabolism amino and organic acidurias, sulphite \200 14_McClure_14.indd 509 11/14/2012 3:36:07 PM CHAPTER 14 Neurology 510 Epilepsies: infantile Infantile epilepsies are challenging and expert advice should be soug ht. Benign myoclonic epilepsy of infancy This form of epilepsy requires no further investigatio n or therapy provid- ing that what is observed meets the following criteria. \200 \200 \200 \200 Myoclonic seizures only. No other seizure type. Normal interictal EEG. Normal development. West \200\231s syndrome The diagnosis of this condition is based on a classic triad. \200 Infantile spasms: elevation of arms; may be confused with gastro-oesophageal re \202 ux or colic. Developmental: Hypsarrhythmia: short tonic contraction of trunk with upward \200 delay or regression. on the EEG. Often children have only some of these or the EEG is reported as being chaotic, with high v oltage sharp and slow waves, but not \200\230classical hypsarrhythmia \200\231. Investigations Take a thorough history and examination and make sure that you have excluded tuberous sclerosis (see b pp.530, 947). Then, use the series of investigations on the prev ious page for epileptic encephalopathy. Treatment Children with West \200\231s Syndrome are best cared for at home. Prednisolone therapy \200 \200 \200 Step 1: Step 2: Step 3: 15mg* oral/tds for the \201 rst week Continuing seizures. Increase oral dose to 20mg*, qd s at 14 days. Withdraw \200\224in four steps over 15 days This is the complete dose, NOT per kg Note: The child will be immunosuppressed whilst and for 2wks after therapy. Many will devel op hypertension, it is useful to let the GP know the dose of nifedipine should the child be come hypertensive. Some authors prefer ACTH IM injections, but evidence of its superiority is poor, and administration is more dif \201 cult Second line therapy is with oral vigabatrin First 24hr: 25mg/kg bd, Next 2 days: 50mg/kg bd , Day 4 \200\224if there are continuing seizures \200\224 75mg/kg, twice daily for no more than 20wks, due to the risk of visual \201 eld damage. 14_McClure_14.indd 510 11/14/2012 3:36:07 PM EPILEPSIES: INFANTILE 511 Severe myoclonic epilepsy of infancy Seizures occur from the \201 rst year onwards, and i nclude: \200 prolonged (>1hr) febrile and shorter afebrile seizures; \200 focal seizure s; \200 atypical absences; \200 segmental myoclonia. Investigations \200 EEG: may be normal initially, but may develop photosensitivity (i.e. within 12mths in 50%) and generalized discharges once the seizures are frequent. Genetics: if negative and the cl inical picture is atypical, then use the screening investigations for epileptic encephalopa thy (b p.509). over 70% have a mutation in the SCN1a gene. However, Treatment The treatment should follow a sequence, adding anticonvulsants if there is no res ponse. Lamotrigine should be avoided. The sequence is as follows: \200 \200 \200 Start with sodium valproate. Add clobazam. Consider stiripentol if resistant to therapy (ne eds expert supervision). Myoclonic astatic epilepsy A condition with: \200 \200 \200 myoclonic astatic seizures; myoclonic jerks; generalized tonic \200\223clonic seizures. The EEG demonstrates predominantly generalized discharges once sei- zures are established. Seek advice about further investigation and treat as for idiopathic generalized epilepsy (b p.513). Seizures in this condition are likely to be unresponsive, so consider using the ke togenic diet early in refractory cases. Lennox \200\223Gastaut syndrome A condition with: \200 Tonic seizures with trunk \202 exion (often evolving out of infantile spasms). Atonic sei zures, myoclonic jerks, atypical absences. Invariably there is developmental delay once the seizures are established. This condition r arely responds to drugs. Seek advice about further inves- tigation and treat as for an idio pathic generalized epilepsy (b p.513), initially, then getting expert help. 14_McClure_14.indd 511 11/14/2012 3:36:07 PM CHAPTER 14 Neurology 512 Epilepsies: mid to late childhood (1) Idiopathic generalized epilepsies These epilepsies ar e better described as genetic epilepsies. The diagno- sis of epilepsy rests on the history. The EEG helps with classi \201 cation. It should be remembered that generalized discharge s on EEG (particular with photic stimulation) may occur in children without seizures. There is no need for MRI or blood tests after the \201 rst episode. Seizures include combinati ons of: \200 \200 \200 \200 typical absences; myoclonia; tonic seizures and generalized tonic \200\223clonic seizures; myoclonic jerks. In these patients more than 80% of standard, and 95% of standard + sleep- deprived EEG reco rdings will show generalized discharges. Myoclonic absence epilepsy \200 Typical absences with short symmetrical jerks of mainly the upper limbs with abduction and elevation. Early onset <5yrs of age. EEG demonstrates generalized discharges of 3cycles/s s pike and wave, that are not well formed and, in addition, may have short bursts of polyspik es. Poor prognosis and can deteriorate into an epileptic encephalopathy, may require treatm ent with the ketogenic diet. \200 Childhood absence epilepsy \200 Previously known as \200\230 \200 Typical absences onl y, but very frequent. \200 Present during the \201 rst decade. \200 Rarely develop ge neralized tonic \200\223clonic seizures. \200 Absences can be associated with mild myoclo nia, asymmetry, or automatisms. EEG demonstrates regular bursts of 3cycles/s spike and wave . petit mal \200\231. Juvenile absence epilepsy \200 \200 \200 \200 Onset towards the end of the \201 rst and during the second decade. All have absences. Up to 30% have myoclonic jerks. Majority develop generalized tonic \200\223clonic seizures du ring second decade if untreated. EEG discharges more fragmented and irregular than in child hood absence epilepsy, with more bursts of polyspike. Prognosis is guarded even after many years of being seizure-free as an adult, relapse is common. 14_McClure_14.indd 512 11/14/2012 3:36:07 PM EPILEPSIES: MID TO LATE CHILDHOOD (1) 513 Juvenile myoclonic epilepsy \200 \200 \200 Onset in the second decade. Invariable myoclonic jerks classically within the \201 rst ho ur of awakening. High risk of generalized tonic \200\223clonic seizures, up to 80% of adole scent girls will have further generalized tonic \200\223clonic seizures if they withdraw me dication completely. EEG may have absences and photosensitivity; discharges are more fragme nted and irregular than in juvenile absence epilepsy with bursts of polyspike. Treatment of idiopathic generalized epilepsies \200 First-line: sodium valproate is normally used as \201 rst-line therapy, except in childho od absence epilepsy, where ethosuximide can be considered. In girls aged >9yrs, families sh ould be counseled that it is up to twice as likely to produce seizure freedom as other drug s. Some experts (but not others) consider it may stimulate appetite and increase the incide nce of polycystic ovary syndrome. Both of which reverse on drug withdrawal. All experts agr ee that it is signi \201 cantly more teratogenic than other anti-epileptic drugs if taken during pregnancy at a dose of more than 1000mg per day. Second-line: to be introduced more slowly when sodium valproate is being used concurrently. Third-line: there is no consensus. Some clinicians advocate using a benzodiazepine and suggest clonazepam as the most effecti ve. However, it is extremely dif \201 cult to withdraw if it is used in moderate to high d osage. Therefore, others advise using clobazam, topiramate, or levetiracetam. lamotrigine is the next choice, but take note that it needs 14_McClure_14.indd 513 11/14/2012 3:36:07 PM CHAPTER 14 Neurology 514 Epilepsies: mid to late childhood (2) Idiopathic focal epilepsies The term idiopathic focal epilepsy, or benign focal epilepsy, is used less frequently due to the severity of the sei zures in some children. Benign childhood epilepsy with centrotemporal spikes \200\224Rolandic Epilepsy \200\231 The classic presentation of this condition is: \200 \200 Predominantly nocturnal sensorimotor seizures. Onset in one side of face or a hand, then sp reading down one side and may generalize. EEG may be relatively normal whilst awake. EEG in slow wave sleep or drowsiness will develop frequent centrotemporal spike and wave discharg es with an easily recognizable shape and distribution. \200 The majority of children with this condition have infrequent, short seizures and the decisi on whether to treat or not is taken after discussion with the parents and child. Some clini cians feel strongly that therapy should be the same as in idiopathic generalized epilepsy, but others will consider using carbamazepine. Benign childhood occipital seizure syndrome (Panayiotopoulos syndrome) \200 \200 Young children (aged 1 \200\2237yrs). Bizarre seizures: encephalopathy often associated wit h ictal vomiting, headache, and eye deviation. Often misdiagnosed. Heterogeneous EEG abnorm alities. Good prognosis. Treatment is rarely indicated. prolonged (<30min), stereotyped episodes of \200 \200 \200 Landau \200\223Kleffner syndrome (LKS) and electrical status in slow wave sleep These condi tions are considered to be an extreme variant, marked by the following: \200 \200 \200 Intellectual regression with relatively few seizures. Striking language impairment \200\224 an epileptic aphasia in LKS. EEG may show non-speci \201 c abnormalities in the waking sta te, but once drowsy or in slow wave sleep the EEG develops electrical status. They are dif \201 cult to treat and normally refractory to \201 rst-line drugs. Steroids are advocated and have been shown to be of temporary bene \201 t. They may even improve l ong-term outcome. 14_McClure_14.indd 514 11/14/2012 3:36:07 PM EPILEPSIES: MID TO LATE CHILDHOOD (2) 515 Focal epilepsies These epilepsies are symptomatic of a focal area of dysfunction, but the e lectrical discharges may generalize (i.e. secondary generalization). \200 While the elect rical discharges are focal, consciousness may be maintained (previously known as simple par tial seizures). When the discharges become more widespread consciousness will be impaired o r lost (previously known as complex partial seizures). They all may develop into a secondar ily generalized seizure. At that point it is not possible to classify them if the onset has not been witnessed. Their expression will depend on the principally affected area of the b rain. Frontal lobe epilepsies These children tend to have short, but frequent seizures \200\224pa rticularly arising out of sleep. They are often associated with asymmetric dystonic posturi ng and brought on by loud noises. Recovery can be quick and they may be dif \201 cult to a ssess on the EEG. Temporal lobe epilepsies The seizures affect memory and emotion with disturbances such as \200\230d j vu \200\231, fear, abdominal discomfort, and automatisms. Occipital lobe epilepsies These episodes are associated with simple multicolored blobs of l ight in one side of a visual \201 eld. They often produce headache and vomiting. Management of focal epilepsies Investigation MRI is always indicated. Children rarely have malignant brain tumors. However , they can have dysplasias, gliosis, and benign tumors. The tem- poral lobe may show hippoc ampal sclerosis. Treatment \200 First-line: \200 Second-line: comparing anti-epileptic drugs against eac h other. However, sodium valproate is a logical choice amongst the older anticonvulsants (b ut not in girls >9yrs of age). Of the newer anticonvulsants, lamotrigine, topiramate, and l evetiracetam could be used, but licensing conditions should be noted. carbamazepine is generally recommended. therapy is widely debated. There are few good studies 14_McClure_14.indd 515 11/14/2012 3:36:07 PM CHAPTER 14 Neurology 516 Headache Children with headache are commonly referred to general pediatricians. \200 Over 90% will have chronic childhood headache, with no identi \201 able physical cause. Some h ave migraine. Malignant brain tumors obstructing CSF \202 ow, causing hydrocephalus and c onsequent headaches, are less common. These are almost always associated with focal signs o n examination or a suggestive history (see b pp.653, 662), if present for more than 6wks. \200 Chronic, tension type headache This form of headache is: \200 \200 \200 regular; often frontal; not associated with vomiting, paraesthesia, visual disturbance, or abnormality on examination (including BP). History The headache may be reported to be severe enough to take time off school, but with few objective signs of pain. A full history is important, not only to exclude migraine and symptoms of raised ICP, but also to elucidate stresses that may be causing the headache or gains the child may have from the behavior. It should be assumed to be chronic if present f or more than 6wks. Treatment \200 Reassure the family that, with the thorough history and examination, migraine and tumors ca n be excluded. It is inappropriate to perform either a CT or an MRI scan. Sympathize with the family over the problem and suggest analgesia, but at best it is likely to make no difference. Therefore dosage and number of drugs should be reduced to the minim um acceptable. Encourage the child or young person to continue doing all the normal activit ies for somebody of their age. \200\230I can \200\231t take away the headache, but the mor e normal things you do and the fewer drugs you take, the less you will notice the pain \200 \231. Raised ICP This is a potent cause of headache and will be associated with either or both of the following: \200 Abnormal co-ordination, eye movements, and fundi (i.e. papilloedema). Severe examination: in particular, heel \200\223toe walking, \201 nger \200\223nose short history: vomiting, morning headache, visual disturbance. Clinically, the main concern is a mass obstructing CSF \202 ow, particularly a malignant posterior fossa tumor. Therefore the children need expert opinion on neuroimaging as soon a s possible. MRI superior, but CT head is performed if MRI not immediately available. However, the ICP can be raised without abnormality evident on CT scan. In some of these chi ldren there may be thrombosis of a cerebral sinus. Therefore, MRI and MRV are recommended. 14_McClure_14.indd 516 11/14/2012 3:36:07 PM HEADACHE 517 A subgroup has raised pressure of unknown cause \200\224idiopathic intrac- ranial hypertens ion, where the only sign on examination will be papil- loedema +/ \200\223 reduced visual a cuity, with normal cranial imaging, except for the lateral venous sinuses, which can look c ompressed. Idiopathic intracranial hypertension (IIH) IIH or benign IH or pseudotumour cerebri typical ly is associated with obe- sity, female sex, and adolescence. It is important to exclude se condary cases caused by: \200 Drugs: contraceptive pills; phenothiazines. Systemic lupus erythematosus. changes: adrenal failure; hyperthyroidism; Endocrine hypoparathyroidism; menarche; pregnancy; obesity. Head steroid withdrawal; vitamin A; thyroid replacement; oral disease: iron de \201 ciency; Guillain \200\223Barr syndrome; systemic injury. History Early morning headache blurred or double vision, vomiting. Examination and investigation \200 check BP. \200 \200 \200 \200 General: Neurology: Eyes: Imaging: Lumbar protein, and glucose. there may be ataxia. papilloedema; scotoma on visual \201 eld testing. normal. puncture: raised ICP (>20cm CSF); normal CSF cell count, Management \200 \200 \200 \200 Weight loss in the obese. Try and remove the causal medication. Diuretics: Steroids: withdr awn. Serial lumbar punctures or surgical intervention. Monitoring of eyes and visual \201 elds: do well, but some patients with eye problems may deteriorate. to reduce CSF formation (e.g. acetazolamide, furosemide). may be effective, but can cause r ebound problems when \200 most patients without visual de \201 cit 14_McClure_14.indd 517 11/14/2012 3:36:07 PM CHAPTER 14 Neurology 518 Headache: migraine Up to 10% of children may have migraine. These are debilitating episodes and the criteria are listed below. If they occur frequently (more than 4 times per month f or more than 3mths), the diagnosis is unlikely. If the headache occurs daily then the term chronic headache should be used and managed as described on b p.516. Treatment \200 Exclude \200 Paracetamol of symptoms, as they will treat the headache an d nausea. Prophylaxis: if the migraine is frequent enough to disrupt schooling or social ac tivity, then consider prophylaxis. The evidence-base for different therapies is poor. Initi ally try a 3mths trial of pizotifen. If this is not effective, then try propranolol. Antide pressants such as amitriptyline have also been used. Sumatriptan may be used in children ol der than 12yrs at the onset of symptoms, if other treatments are ineffective. triggers: such as diet, dehydration, overtiredness, and stress. and domperidone: these can be tried initially, at the onset Diagnostic criteria for pediatric migraine Migraine without aura A At least 5 attacks ful \201 lling B \200\223D B Headache attack la sting 1 \200\22348hr C Headache has at least two of the following: \200 \200 \200 \200 D During headache, at least one of the following: \200 \200 Bilateral (temporal or frontal) or unilateral location Pulsating quality Moderate to severe intensity Aggravation by routine physical activity Nausea and/or vomiting Photophobia and/or phonophobia Migraine with aura A Idiopathic recurring disorder: headache that usually lasts 1 \200\2234 8hr B At least two attacks ful \201 lling C C At least three of the following: \200 One or more fully reversible aura symptoms indicating focal cortical and/or brainstem dysfu nction At least one aura developing gradually over >4min, or two or more symptoms occurring in succession No aura lasting >60min Headache follows in <60min \200 14_McClure_14.indd 518 11/14/2012 3:36:07 PM BELL \200\231S PALSY 519 Bell \200\231s palsy \200 Acute paralysis of the muscles of facial expression may be unable to close the eye on the a ffected side. Normally unilateral, but may be bilateral lower motor neuron lesion. to oedem a of the facial nerve as it passes through the temporal s \200 bone. Etiology \200 Idiopathic. \200 Varicella and other viruses. \200 Borrelia burgdorferi (Lyme disease), particularly if bilateral. Examination \200 Check: hyperacusis. Full systemic examination: vasculitides. Full neurological examination: which would exclude an idiopathic Bell \200\231s palsy. whether other branches of the facial nerve are affected, e.g. in particular, look for signs of leukemia and look for other signs, the presence of Investigation \200 \200 \200 FBC and \201 lm (leukemia). Varicella titres. Borrelia investigation, in suspicious cases , only after discussion with microbiology as this is a dif \201 cult infection to either r efute or con \201 rm. Treatment \200 Steroids: opinion is to use 2mg/kg (maximum 60mg) prednisolone, once daily for 5 days if the symptoms are less than 7 days old. Aciclovir 10 days, irrespective of va ricella status, may be useful. evidence for the use of steroids is limited, but the general : recent evidence indicates that oral aciclovir (40mg/kg/day) for Prognosis Most children will either recover fully or recover to a good degree. When this do es not occur after 6mths, referral for facial nerve grafting is appropriate. 14_McClure_14.indd 519 11/14/2012 3:36:08 PM CHAPTER 14 Neurology 520 Acute disseminated encephalomyelitis ADEM is an immune mediated disease. It usually occurs following a viral infection, but may follow other infections or vaccination. It involves au to- immune demyelination, it is similar to multiple sclerosis- although mono- phasic. ADEM produces multiple in \202 ammatory lesions in the brain and spinal cord, particularly in t he white matter. Usually these are found in the subcortical/central white matter and cortic al gray-white junction of both cerebral hemispheres, cerebellum, brainstem, and spinal cord , but other areas including the basal ganglia may also be involved. Presentation \200 \200 \200 The average age around 5 \200\2238yrs old. Abrupt onset and a monophasic course. Symptoms u sually begin 1 \200\2233wks after infection or vaccination and include fever, headache, dro wsiness, coma, and seizures. Average time to maximum severity about four and a half days. A dditional symptoms include hemiparesis, paraparesis, and cranial nerve palsies. \200 Diagnosis This is based on \201 nding typical changes on MRI- as above in the subcor- tic al/central white matter, cortical gray-white junction, cerebellum, brain- stem, and spinal cord. The basal ganglia may also be involved (Fig. 14.3). CSF may show a mild lymphocytosis , with normal glucose, but there may be a mild rise in protein. Fig. 14.3 MRI head scan of child with ADEM on FLAIR sequence, which shows the subcortical/c entral white matter, cortical gray-white junction lesions well. 14_McClure_14.indd 520 11/14/2012 3:36:08 PM ACUTE DISSEMINATED ENCEPHALOMYELITIS 521 Treatment It is important to exclude other causes of encephalopathy (b pp.72 \200\22379, 55 2). Then supportive measures such as hydration/feeding, bulbar func- tion and respiration s hould be instituted. Pulsed intravenous methylpred- nisolone is widely recommended as de \201 nitive treatment, and is normally associated with improvement within days. ADEM may relapse once or twice, it is then called M(ultiple)DEM. Multiple Sclerosis rarely occurs in childhood, but becomes more com- mon as children approach adulthood. It presents with demyelinating plaques, which differ from ADEM in their distribution- more periventricu - lar white matter, and with much less encephalopathy, seizures, and coma, but more focal n eurological signs. 14_McClure_14.indd 521 11/14/2012 3:36:08 PM CHAPTER 14 Neurology 522 Stroke in childhood Cerebrovascular stroke \200\224although commonly presenting with congen ital hemiplegia \200\224is rare in childhood, but it does cause signi \201 cant morbidity. The cause can be arterial-ischaemic, haemorrhagic, or venous in origin. The majority of ca ses will have a likely cause identi \201 ed on history and/or examination. The main causes are: \200 \200 \200 \200 sickle cell disease; congenital cardiac defects; cerebral infection; trauma (arterial disse ction). Management Children with stroke will need initial attention to ABC (see b p.46) and treatme nt of acute conditions such as mastioditis/meningitis before early transfer to a specialist unit. Investigation Once stable all children will require brain imaging \200\224preferably magnet - ic resonance imaging and angiography of both cerebral and neck vessels, rather than CT sc an (although CT will show the distribution of injury and exclude hemorrhage). Even with a k nown cause such as trauma, all child- ren require screening for underlying thrombophilia as these conditions may co-exist. If there is no obvious cause then the investigations in the box should be considered. Treatment After stabilization, acute treatment should be undertaken in a specialist center. Subsequent management, although acute, would be undertaken with the same team and aims as that outlined for cerebral palsy. 14_McClure_14.indd 522 11/14/2012 3:36:08 PM STROKE IN CHILDHOOD 523 Investigation for stroke Blood: hematology \200 \200 FBC, ESR: polycythaemia Thrombophilia screen, \201 brinogen: thrombophilia Blood: biochemistry \200 \200 \200 \200 \200 \200 \200 \200 \200 Electrolytes, magnesium Liver function tests CRP: in \202 ammation Plasma lactate and CSF lactate: Fasting glucose: Fasting lipid screen: Thyroid function tests: Ammonia: Homocystei ne (free and total): methyltetrahydrofolate reductase (MTHFR) de \201 ciency can also be p icked up by common mutation analysis on the thrombophilia screen, and if symptomatic has a raised plasma homocysteine Serum iron, total iron binding capacity, ferritin, red cell fola te, and vitamin B12: iron de \201 ciency and other nutritional disorders Plasma amino acid s: Carnitine (acyl, free, and total): mitochondrial disorders diabetes hyperlipidaemias Hashimoto thyroiditis/encephalopathy urea cycle disorders \200 aminoacidurias B-oxidation defects Urine: biochemistry Urine organic and amino acids: homocystinuria, MTHFR de \201 ciency Blood immunology and infection screen \200 \200 immunode \201 ciency IgG, IgM, IgA: Titres for infection screen of: Mycoplasma, Chlamydia, Helicobacter, Borreli a, Brucella; viruses (echo, Coxsackie, Epstein \200\223Barr, Varicella, hepatitis B) ASOT, Anti DNAase B: ANA, ANCA, autoimmune disease \200 streptococcal disease