A five-year-old boy is seen regularly in the community paediatric clinic for developmental delay.

He first presented with a history of infantile spasms. What is the most likely diagnosis? (Choose ONE) Tuberous sclerosis Sturge-Weber syndrome Neurofibromatosis type-1 Neurofibomatosis type-2 Landau-Kleffner syndrome YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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Which of the following features would you expect to see in this child? (Choose TWO) EEG showing centrotemporal spikes Periungual fibromas Ashleaf macules Intracranial calcification Optic gliomas Adenoma sebaceum Lisch nodules

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Ashleaf macules Intracranial calcification Tuberous sclerosis is a neurocutaneous syndrome loved by examiners! Inheritance is autosomal dominant with a 70 % new mutation rate. Features include Skin: Ash leaf patches (hypopigmented patches seen best with a Woods light), Shagreen patches and occasionally, caf au lait patches. Look out for adenoma sebaceum and periungual fibromas after puberty. Central Nervous System: developmental delay, seizures (infantile spasms), periventricular and subependymal intracranial calcification. Eyes: retinal phacomata Kidneys: angiomas, cysts Heart: rhabdomyomas. 13030
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A 6-year-old boy is referred to the community paediatrician because of educational difficulties. The paediatrician notes that he has multiple caf au lait patches. Which of the following features would help to make a diagnosis of neurofibromatosis type 1? (Choose TWO) 1x neurofibroma Four caf au lait patches Inguinal freckling

Ash leaf macule Macrocephaly 1x plexiform neurofibroma Medulloblastoma Neuroblastoma

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Inguinal freckling 1x plexiform neurofibroma What other specialist input would be most useful in making a diagnosis? (Choose ONE) Physiotherapist Ophthalmologist Speech and language therapist Endocrinologist Neurologist

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Neurofibromatosis type-1 (NF-1) is an autosomal dominant condition with a 50% new mutation rate. For diagnosis, 2 or more of the following features are required: 6 or more caf au lait patches (>5mm pre puberty, >15mm post puberty) axillary/ inguinal freckling 2 or more neurofibromas, or one plexiform neurofibroma (these are more diffuse in appearance) optic glioma two or more Lisch nodules (melanocytic iris hamartomas) fibrous dysplasia of bone (e.g. sphenoid, tibia) first degree relative with neurofibromatosis according to above criteria. Macrocephaly is a feature of NF-1 but is not a diagnostic criterion. Similarly, children with NF-1 are predisposed to having many types of tumour, including neuroblastomas and medulloblastomas. An ophthalmology review to look for Lisch nodules would be most useful in making a diagnosis.

A three-week-old baby presents with lethargy, floppiness and poor feeding. As part of a septic screen a lumbar puncture is done. The following results are obtained on microscopy of the cerebro-spinal fluid.
Protein glucose red blood cell count white blood cell count 3g/l 2.5 mmol/l (blood glucose 5 mmol/l) 1000 cells/ l 50 cells/l

What is the most likely diagnosis? (Choose ONE) Traumatic lumbar puncture Viral meningitis Tuberculous meningitis Normal CSF Bacterial meningitis

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What are the most likely organisms to cause meningitis in this age group? (Choose TWO) Salmonella Group B streptococcus Staphylococcus aureus Escherichia coli Staphylococcus epidermidis Neisseria meningitides Klebsiella

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Group B streptococcus Escherichia coli This picture is consistent with bacterial meningitis (high protein, glucose <2/3 of blood glucose value, high white cell count). If the white cell: red cell ratio is

greater than 1:500 this indicates a raised white cell count. (because the ratio of white cell to red cell in the plasma is approximately 1:500) In viral meningitis lymphocytes predominate and the CSF protein is < 1.5g/l while the CSF glucose is not reduced compared to plasma. In TB meningitis the CSF appears cloudy (sometimes with a fibrin web developing). There is a high white cell count, high protein and very low CSF glucose. Ziehl-Neelsen staining of the CSF will reveal acid fast bacilli on microscopy. Also beware of normal neonatal CSF ranges: White cell count 0- 15, polymorphs 0, lymphocytes 0-15, red cells 0- 500, protein 0.3 -2. Meningitis in the neonatal period is caused by organisms from the maternal genitourinary tract.

Theme: Diagnosis A Benign myoclonus B Infantile spasms C LennoxGastaut syndrome D Absence seizures E Febrile seizures F Benign rolandic epilepsy G Rasmussens syndrome H LandauKleffner syndrome I Juvenile myoclonic epilepsy J Neonatal encephalopathy For each of the scenarios described below, select the single most likely option above. Each option can be used once, more than once or not at all. Scenario 1 A term infant is born by normal vaginal delivery. He is discharged at 6 h of age. His parents bring him back after 12 h having noticed that he is having symmetrical jerks of his arms and legs while he is sleeping in his cot.

Benign myoclonus CORRECT ANSWER

Benign myoclonus Benign myoclonus can be seen in term infants. It only occurs in sleep and the myoclonic jerks are abolished if the child is wakened. It is a benign condition and the parents may be reassured that these are not seizures.

YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 2 A 5-year-old boy comes to clinic. His parents describe that he has had two seizures. The first, 6 months ago, affected his face and left arm, He was distressed, as he could not communicate with his parents during the seizure although he was conscious. One month ago he was found by his parents having a generalised tonic clonic seizure at night.

Benign rolandic epilepsy CORRECT ANSWER Benign rolandic epilepsy Benign rolandic epilepsy (sometimes called benign epilepsy with centro-temporal spikes) presents in school age children. It is seen more commonly in boys. Typical seizures are nocturnal partial seizures, most commonly unilateral motor seizures involving the face and upper limbs. Speech arrest occurs during the seizure. The seizures may secondarily generalised. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3 A term infant is admitted to NICU with meconium aspiration syndrome. He is sedated and ventilated. While on the ventilator he was noted to have clinical seizures. After a prolonged stay on NICU he is discharged. At his 3-month clinic follow-up his parents say that they have noted runs of seizures where he stretches forward in flexion and then relaxes. This is most prominent when he awakes from sleep.

Infantile spasms CORRECT ANSWER Infantile spasms Infantile spasms are a typical seizure activity associated with developmental delay. In 50% the infant may already have developmental delay and in the other 50% developmental delay occurs after the onset of seizures. Treatment is with vigabatrin or steroids but the seizures may be difficult to control. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 4 A 3.5-year-old boy is referred to community paediatricians by the speech and language team. He was speaking in full sentences until six months ago when he began to loose his speech skills. He has begun to have epileptic seizures. His parents complain that his behaviour is becoming difficult to manage.

LandauKleffner syndrome CORRECT ANSWER LandauKleffner syndrome LandauKleffner syndrome consists of infrequent seizures and an acquired receptive aphasia. Severe behavioural disturbance with hyperactivity is frequently seen. Early recognition and treatment may slow or arrest the language deterioration. (Candidates should be aware of the new five-axis classification of epilepsy. http://www.ilae.org/Visitors/Centre/ctf/ctfoverview.cfm ) - To view this page you will need to retype the address into your browser. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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An 11-year-old boy is brought to clinic by his parents. His parents have noticed that he constantly pulls faces and appears to have facial tics. He was originally referred for twitching two years ago when he had an electroencephalogram (EEG) arranged. This was unremarkable. He was then lost to follow up and his parents explained that this is because his problem seemed to have resolved. However, over the last few months he has been having more twitching episodes. These vary in intensity and in duration. His father expresses the view that he thinks that the boy is in control of his twitching as he can stop if told to. In clinic the boy cannot sit still. He is constantly moving and pushing his hair out of his eyes and blinking. He also shrugs his shoulders especially on his right hand side. He repeatedly clears his throat. On speaking with the boy you notice that he has pressure of speech and constantly changes subject. He acknowledges that the twitches and tics have worsened but that he cannot completely suppress them. He describes them as being a pressure building up inside him until he starts to twitch. The boy has been doing well at school but his teacher has raised concerns about his inability to sit still and wondered if his twitches were a manifestation of epilepsy. What is the most likely diagnosis? Choose ONE answer only. Attention deficit hyperactivity disorder Partial seizures Tourettes syndrome Behavioural difficulties Early-onset schizophrenia

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Tourettes syndrome is an inherited neurological disorder with repeated involuntary movements shown as tics or twitches. There are also uncontrollable sounds; in only a minority of cases is this shown as inappropriate words. Patients with Tourettes syndrome can temporarily suppress their tics or vocalisation but

describe them building up inside until they are expressed. The movements may be disguised as semi purposeful movements such as pushing hair, blinking or shrugging shoulders. The vocalisations may also be expressed as socially acceptable noises such as clearing of the throat and sniffing. However, on further questioning most patients will have a history of shrieking, barking or other noises. Exploring the family history will often reveal relatives with tics and/or vocalisations. Again this may not be revealed unless directly asked for. The symptoms of Tourettes may be worse at some times than others leading the parents to think at first that the child is going through a behaviourally challenging phase which passes. As the child enters adolescence the symptoms may be more pronounced. There is an overlap with ADHD and Tourettes syndrome. This can lead to difficulties in treatment of ADHD, as methylphenidate should be used with caution in those with Tourettes as it can worsen tics. Any treatment should be under the supervision of a child psychiatrist. Tourettes syndrome may be treated with haloperidol. Again treatment should be initiated and monitored by a child psychiatrist. Tourettes syndrome will frequently become less severe in late adolescence and adulthood.

An 11-year-old boy attends his annual medical at the school for those with physical disability that he attends. He was born at 26 weeks gestation and had a stormy neonatal course. He developed signs of spastic diplegia as he grew. He sat at the age of 13 months and started walking at the age of 24 months. He has maintained mobility with the use of crutches and anklefoot orthoses. He is seen regularly by physiotherapists in school and has an annual review by the local paediatric orthopaedic surgeon. His mother tells you that her son appears to be having more difficulty in walking. She describes how he appears to be sinking at the knees. On examination you notice that he stands and walks with both knees flexed. He has no scoliosis, there is full range of movement in both hips and he can fully extend both knees when lying supine. His mother tells you that he still seems to enjoy swimming but walking and standing appears to be more difficult. What is the most likely cause of his deterioration? Choose ONE answer only. Natural progression of his cerebral palsy

Dislocation of one or both of his hips Duchennes muscular dystrophy Tightening of his hip adductor muscles Shortening of his gastrocnemius muscle

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As children with cerebral palsy get older they experience increasing difficulty supporting their frame. The increasing body mass around the time of puberty is often too much for their relatively hypotonic muscles to support. Therefore children who gained independent mobility, albeit with the use of mobility aids, may go off their feet around the time of adolescence. Non-weight-bearing exercise, such as swimming, provides less of a problem. Physiotherapy has a role in maximising the muscle strength that remains, but the natural course is for these children to have increasing difficulty with mobilisation. Children and families cope with this loss of independence in a variety of ways. Some find that the use of a wheelchair for longer journeys helps the family get out more as they can travel further than when the child was slowly mobile. However, some children and parents find this loss of independence difficult. It is wise to involve the multi-disciplinary team in management of such cases. Regular review by physiotherapist and paediatric orthopaedic surgeons is advised. Botulinum toxin and/or surgery can sometimes be of benefit. Occupational therapists and social work departments may help in providing aids for the child and family. Schoolteachers and nurses in schools for the physically disabled are useful sources of advice and encouragement for the children and their parents.

A male infant is born to healthy consanguineous parents. The antenatal scans had all shown polyhydramnios and only one scan had demonstrated a small stomach bubble.

The fetus had always been shown to have a bradycardic heart rate but four-chamber view of the fetal heart had been normal. Amniocentesis had been performed and chromosome study of the infant showed 46,X,Y. The infant was born at 35 weeks gestation with a birth weight of 1.5 kg. He was noted to have fixed contractures of the elbows, wrists and hips. He had severe fixed talipes equinovarus. He needed resuscitation at birth and made little spontaneous movement. A nasogastric tube was passed easily to the stomach. Chest X-ray showed small lung fields. What is the most likely diagnosis? Choose ONE answer only. Infantile cerebral palsy Arthrogryposis Osteogenesis imperfecta Achondroplasia Myotonic dystrophy

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Arthrogryposis describes a collection of conditions characterised by fixed contractures at birth. The majority of cases are caused by fetal akinesia, i.e. reduced fetal movements. The joints are normal physiologically early in pregnancy but develop contractures in pregnancy. Normal development of joints requires motion. Lack of movement causes extra connective tissue to develop around the joints. Muscle atrophy may also be seen through lack of movement. Many cases have a neurological origin. There may be evidence of poor swallowing and poor intrauterine gasps and breathing. There is no evidence of intrauterine fracture. The condition is more common in consanguineous families. There are probably a number of (mostly neurological) causes.

An 8-month-old boy was brought to the A&E by his mother. She was concerned that he had symptoms of a cold for 3 weeks that did not seem to be improving. During this time he had been off his food and irritable. She had also noticed that he could no longer pull to sit independently. More recently she had noticed that he tended to fall over to one side if placed in the sitting position. She has taken him to the general practitioner earlier in the week who had reassured the mother and explained that when the boys cold improved his motor skills would return to normal. On examination the boy had a temperature of 37.8 o C. He had a runny nose and inflamed posterior pharyngeal wall. He had some shotty cervical lymphadenopathy. He could not sit unsupported and did not have a parachute reflex. When sitting on his mothers lap the boys legs crossed at the ankles. No deficit of the cranial nerves could be elicited. Which investigation would be the most important? Choose ONE answer only. Computed tomography (CT)/magnetic resonance imaging (MRI) brain Electroencephalogram (EEG) Lumbar puncture Muscle biopsy

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Computed tomography (CT)/magnetic resonance imaging (MRI) brain Loss of motor skills and scissoring of the legs are warning signs of serious neurological pathology. The boy shows signs of an upper motor neurone deficit affecting both sides of the body. A CT/MRI scan of the head and cervical cord should be arranged urgently.

Which diagnoses would be MOST likely? Choose TWO answers only. Cerebral palsy Duchennes muscular dystrophy Intracranial space-occupying lesion Meningitis Leucodystrophy Spinal muscular atrophy Freidrichs ataxia

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Intracranial space-occupying lesion Leucodystrophy A space-occupying lesion would be high on the list of differentials. Meningitis would not cause a slow deterioration over 3 weeks. Duchennes muscular dystrophy causes hypotonia not hypertonia and presents at a later age. Leucodystrophy may present in the first year of life with developmental regression and loss of motor skills. Cerebral palsy does not cause regression of skills alrady obtained. The diagnostic feature is abnormalities of white matter seen on CT or MRI of the brain. An MRI is more likely to pick up a leucodystrophy or a brainstem tumour. Psychomotor deterioration and irritability are cardinal signs. Those with a rapid regression have a poor prognosis.

A 6-month-old girl is referred to the Out-patients Department with pronounced head lag. She is the only child of non-consanguineous parents and was born at term. All antenatal bloods and scans were normal. She had been born by emergency Caesarean section for failure to progress and had been discharged from hospital on day 2 of life after a normal baby check. At the 6-week check she had been noted to have a degree of head lag but passed her check. At 6 months of age her parents became concerned that she still needed to have her head supported when she was held. She was smiling in clinic but her parents said that visitors had commented that she was irritable. Her mother also mentioned that she would sleep with her neck extended so that her head almost touches her bottom. When changing her nappy her mother would have to wait for her to relax so that she could part her legs. On examination the girl was irritable. She lay supine with her legs extended and raised off the bed. Reflexes were brisk throughout more noticeably in the legs. She would fix and follow and was interested in what happened around her. She responded appropriately to noises. She demonstrated a palmar grasp but tended to hold her fists clenched. She had marked head lag and could not hold her head up when held sitting. What is the most likely cause of the girls symptoms? Choose ONE answer only. Space-occupying lesion Neuronal migration defect Cerebral palsy Subdural haemorrhage Spinal muscular atrophy

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Cerebral palsy is a fixed, non-progressive lesion of the central nervous system occurring within the first two years of life. Most cases of cerebral palsy are antenatal in origin, although it is always useful reviewing the delivery notes in case there was an intra-partum event that might explain the symptoms. Central hypotonia with limb hypertonia is classical of the picture seen. The hypertonia may be more severe in the lower limbs in the case of diplegia. Which investigation would be most appropriate? Choose ONE answer only. Full blood count MRI scan Serum lactate SMN genotype Cranial ultrasound X-ray of back Muscle biopsy

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MRI scan

Although in many cases causation for the cerebral palsy is not found, cranial scanning should be performed. Computed tomography (CT) scan is most readily accessible and will demonstrate structural lesions, but MRI will show more subtle lesions.

Which two initial referrals would be most useful? Choose TWO answers only. Social services Physiotherapy referral Neurodevelopmental paediatrician referral Community dentistry Occupational therapy Speech and language therapy Geneticist

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Physiotherapy referral Neurodevelopmental paediatrician referral Referral to physiotherapy is of importance as attention to posture and movement will help to prevent scoliosis developing and prevent the development of

contractures by strengthening opposing muscle groups. Neurodevelopmental paediatricians take a key role in the assessment and ongoing management of these infants co-ordinating a multi-disciplinary team to address all areas of development.

A 6-month-old girl is referred to the Out-patients Department with pronounced head lag. She is the only child of non-consanguineous parents and was born at term. All antenatal bloods and scans were normal. She had been born by emergency Caesarean section for failure to progress and had been discharged from hospital on day 2 of life after a normal baby check. At the 6-week check she had been noted to have a degree of head lag but passed her check. At 6 months of age her parents became concerned that she still needed to have her head supported when she was held. She was smiling in clinic but her parents said that visitors had commented that she was irritable. Her mother also mentioned that she would sleep with her neck extended so that her head almost touches her bottom. When changing her nappy her mother would have to wait for her to relax so that she could part her legs. On examination the girl was irritable. She lay supine with her legs extended and raised off the bed. Reflexes were brisk throughout more noticeably in the legs. She would fix and follow and was interested in what happened around her. She responded appropriately to noises. She demonstrated a palmar grasp but tended to hold her fists clenched. She had marked head lag and could not hold her head up when held sitting. What is the most likely cause of the girls symptoms? Choose ONE answer only. Space-occupying lesion Neuronal migration defect Cerebral palsy Subdural haemorrhage Spinal muscular atrophy

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Cerebral palsy is a fixed, non-progressive lesion of the central nervous system occurring within the first two years of life. Most cases of cerebral palsy are antenatal in origin, although it is always useful reviewing the delivery notes in case there was an intra-partum event that might explain the symptoms. Central hypotonia with limb hypertonia is classical of the picture seen. The hypertonia may be more severe in the lower limbs in the case of diplegia. Which investigation would be most appropriate? Choose ONE answer only. Full blood count MRI scan Serum lactate SMN genotype Cranial ultrasound X-ray of back Muscle biopsy

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MRI scan Although in many cases causation for the cerebral palsy is not found, cranial scanning should be performed. Computed tomography (CT) scan is most readily accessible and will demonstrate structural lesions, but MRI will show more subtle lesions.

A C3C4 B T4T5 C T12L1 D C7T1 E T10T11 F L1L2 G T7T8 H C5C6 Match the options above to the questions below. Motor roots: C56 arm flexion C78 extend wrist C8 T1 thumb flexion/lumbricals L13 hip flexion L24 knee extension L5S2 knee flexion S12 ankle flexion S1 hallux extension

Scenario 1 At what spinal level is the lesion in a patient with a spinal injury if sensation is absent from the level of the umbilicus?

T10T11 CORRECT ANSWER T10T11 YOUR ANSWER WAS CORRECT

YOUR ANSWER WAS INCORRECT Scenario 2 What nerve roots are injured in a baby who suffers from an Erbs palsy at birth?

C5C6 CORRECT ANSWER C5C6 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3 What is the most caudal upper level of the lesion in a newborn with spina bifida that has absent hip flexion?

L1L2 CORRECT ANSWER L1L2 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 4 At what spinal level is the lesion in a patient with a transverse myelitis if sensation is absent from the level of the nipples?

T4T5 CORRECT ANSWER

T4T5 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

A 15 B 13 C 12 D 11 E8 F7 G5 H4 I3 What are the childrens coma scores for the following patients? Eye Opening 4 Spontaneous 3 To verbal command 2 To painful stimuli 1 No eye opening Verbal (adult/older child) 5 Oriented 4 Confused conversation 3 Uses inappropriate words 2 Makes incomprehensible sounds 1 None Best grimace (young child) 5 Spontaneous, normal facial/oromotor activity 4 Less than usual spontaneous ability, only responsive to touch 3 Vigorous grimace to pain 2 Mild grimace to pain 1 No response to pain Motor 6 Obeys verbal commands 5 Localizes pain 4 Normal flexion to pain 3 Abnormal flexion to pain (Decorticate posturing) 2 Extension to pain (Decerebrate posturing) 1 - No response

NICE Guideline: Head injury: Triage, assessment, investigation and early management of head injury in infants, children and adults. http://www.nice.org.uk/page.aspx?o=77174 Scenario 1 Eye opening: spontaneousVerbal response: orientatedBest motor response: obeys verbal commands

13 YOUR ANSWER 15 CORRECT ANSWER YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 2 Eye opening: to verbal command

Verbal response: uses inappropriate words Best motor response: obeys verbal commands 11 YOUR ANSWER 12 CORRECT ANSWER 12 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3 Eye opening: no eye opening Verbal response: none Best motor response: extension to pain (decerebrate posturing)

5 YOUR ANSWER 4 CORRECT ANSWER 4 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 4 In a young child. Eye opening: to painful stimuli Verbal response: vigorous grimace to pain Best motor response: abnormal flexion to pain (decorticate posturing)

7 YOUR ANSWER 8 CORRECT ANSWER 8 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 5 In a young child. Eye opening: to voice Verbal response: less than usual spontaneous ability, only responsive to touch Best motor response: localizes pain

8 YOUR ANSWER 12 CORRECT ANSWER 12 YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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Theme: Antiepileptic management A Vigabatrin B Acetazolamide C Sodium valproate D Lamotrigine E Levetiracetam F Phenytoin G Ethosuximide H Carbamazepine I Clobazam For each of the following scenarios, select the most appropriate antiepileptic from the options above. Scenario 1 Second line drug treatment of generalised absences with 3 cycles/second spike and wave on electroencephalogram (EEG).

Ethosuximide YOUR ANSWER Levetiracetam CORRECT ANSWER Current NICE guidelines recommend Levetiracetam and Topiramate as second line drugs in the treatment of childhood absence epilepsy.

YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 2 First line treatment in myoclonic epilepsy.

Sodium valproate CORRECT ANSWER Treatment of myoclonic seizures can be difficult. Topiramate is also considered as first line treatment. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3 Useful adjunct to treatment when risk of seizures increases in cluster epilepsy or seizures that worsen at other times (eg with the menstrual cycle).

Acetazolamide YOUR ANSWER Clobazam CORRECT ANSWER Treatment can be initiated when a cluster of seizures begins. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 4 First choice for seizures consisting of stereotypical mannerisms and bizarre, repetitive behaviour with clouding of consciousness and temporal lobe focus on EEG.

Carbamazepine CORRECT ANSWER

Valproate can be used in these complex partial seizures but carbamazepine is usually the first-line treatment. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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A Vigabatrin B Topiramate C Sodium valproate D Lamotrigine E Levetiracetam F Phenytoin G Ethosuximide H Carbamazepine I Clobazam Patients/carers should be warned about the important side-effects of antiepileptic medication. Suggest the drug most likely to cause the following side-effects. Scenario 1 Permanent visual field loss in up to 30% patients.

Vigabatrin CORRECT ANSWER Vigabatrin Visual fields should be monitored regularly. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 2 Liver failure.

Sodium valproate CORRECT ANSWER Sodium valproate Hepatotoxicity is more common in children under 3 years and those with metabolic/degenerative disorders. Raised liver enzymes are not uncommon in patients on sodium valproate and are usually transient. LFTs (including INR) should be monitored until they return to normal. Treatment should be stopped if INR is significantly raised. Patients/carers should be warned to recognised signs of liver disorders. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3 Dizziness, headache, tremor, gum hypertrophy, coarse face, hirsutism.

Phenytoin CORRECT ANSWER Phenytoin Phenytoin is less commonly used these days for long-term seizure control. Gum hypertrophy, coarse face and hirsutism are usually seen in those who have been on the medication for a number of years. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 4 Nausea, agitation, tremor, increased appetite, weight gain, hair loss.

Sodium valproate CORRECT ANSWER Sodium valproate Like most antiepileptics, valproate has a number of side-effects. These include behavioural and neurological symptoms. Many antiepileptics have a sedating

effect. Valproate often causes agitation. Increased appetite, weight gain, hair loss are specific to valproate. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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A 9-month-old baby is brought to the ward after a fit. The child was crying, went blue and floppy then started jerking in all four limbs for about 3 min. During this time he was unresponsive. He recovered over the next 10 min and now seems back to normal. Parents report that on three previous occasions he has gone blue and floppy while crying but only for a few moments. They had seen the GP who told them the baby would grow out of it. There are no developmental problems. Examination is normal. What is the most likely diagnosis? Infantile spasms Severe myoclonic epilepsy of infancy Reflex anoxic seizure Rasmussens syndrome Ohtaharas syndrome

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This is a form of reflex anoxic seizure (previously called a blue-breath-holding episode). The crying causes hypoxia and bradycardia. Sometimes a hypoxic seizure occurs. Most children eventually outgrow the problem although the attacks can frighten parents. It is important the parents do not keep giving in to

the childs wishes to stop them crying. There is no evidence that the hypoxia or, indeed the seizure, causes any harm to the child. Another form of reflex anoxic seizure (previously called a reflex pallid syncope) occurs in response to a sudden shock or pain. This is probably a vagally mediated response directly affecting the cardiovascular system.

What is the next step in management? Cranial MRI EEG and ECG Start phenobarbital Explain diagnosis to parents and reassure Start atropine

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Reassurance and explanation is all that is required. Atropine can be used in severe cases.

A 4-year-old is brought to clinic. Parents give a history of a shaking episode. She was playing in the sitting room when she suddenly went stiff and gave a short cry. Dad went to pick her up and noticed her eyes rolled up. She was unresponsive and her limbs were stiff. He lowered her to the floor. On the floor all 4 limbs start to jerk rhythmically for 34 min. She then relaxed and went quite limp. After a few minutes she started to wake up. By about 10 min she was awake and responsive but then fell asleep for 23 h after which she seemed fully recovered. The GP who examined her once she had recovered referred her to the clinic for a routine appointment. Since the referral some 2 months ago she has had a further

episode with no obvious trigger. There is no family history of epilepsy. The girl is otherwise well. Development has been normal. Examination is unremarkable. The most likely diagnosis is? Focal epilepsy with secondary generalisation Cerebral tumour Generalised epilepsy presenting with tonicclonic seizures Temper tantrums Reflex anoxic seizures

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Epilepsy is, by definition, recurrent seizures. As she has had two episodes a diagnosis can be made. The seizures sound like classic tonicclonic convulsions (axis 1). In an otherwise normal child primary/idiopathic generalised epilepsy (axis 2/3) is more likely than focal epilepsy with secondary generalisation.

What initial investigation might be appropriate? Electroencephalogram (EEG) Computed tomography (CT) Magnetic resonance imaging (MRI)

Trial of anticonvulsants Liver function tests

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An EEG would help confirm the type of seizure (ie primary generalised). Cranial imaging may be requested but is unlikely to demonstrate any abnormality if primary generalised epilepsy presenting with generalised tonicclonic seizures is confirmed.

What initial treatment might be appropriate? Choose TWO options only. Sodium valproate Lamotrigine Phenytoin Carbamazepine Levetiracetam Ethosuximide

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Sodium valproate Carbamazepine Sodium valproate and carbamazepine are both appropriate treatments for primary generalised epilepsy presenting as tonic clinic seizures. Sodium valproate is probably more likely to be chosen by most clinicians. Lamotrigine is frequently used as first line but is not licensed at this age. Phenytoin is generally not used as first line because of side-effects and a narrow therapeutic window. Ethosuximide is a treatment for absence seizures. Levetiracetam is not a first line treatment. Topiramate might also be considered in generalised tonicclonic seizures. http://www.nice.org.uk/pdf/CG020NICEguideline.pdf

Parents are reluctant to start therapy in view of possible side-effects. What is the most appropriate action? Choose ONE option only. Suggest strongly that they she start treatment immediately Explain the risk that seizures will worsen if left untreated Instigate child protection procedures (Children Act 1989) Respect their wishes and offer to review the child should they reconsider or if further seizures occur Explain that side-effects are minimal and that treatment is the best course of action

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There is no evidence that delay in treatment will cause the condition to worsen in most cases (though there are some exceptions eg LandauKleffner Syndrome). Parents should be told what to do if a further seizure occurs and how to minimise risks to the child (eg climbing frames, bathing although these are probably less significant in a 4-year-old). School will need to know when she starts. Side-effects of anticonvulsants can be unpleasant and even dangerous (eg StevensJohnson syndrome, liver failure, agranulocytosis). The frequency of seizures and hence the impairment they cause (axis 5) is not yet established.

A 7-year-old presents with blank episodes during which he is unresponsive for 10 15 s. They are sometimes associated with eye flicking. In the clinic an episode is provoked by hyperventilation. Examination is otherwise normal. Which of the following medications is likely to be the most effective in controlling these episodes? Carbamazepine Sodium valproate Phenytoin Topiramate Gabapentin

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Sodium valproate is the treatment for this condition. Ethosuximide is an alternative. Carbamazepine often worsens the condition.

What is his electroencephalogram (EEG) likely to show? Three cycles per second spike and wave Temporal lobe focus Centro-temporal spikes Frontal focus Hypsarhythmia

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This child has primary/idiopathic generalised epilepsy presenting with absences (petit mal). This is associated with three cycles per second spike and wave activity.

Which of the following are criteria used for the diagnosis of tuberose sclerosis? Choose THREE options only. Sex-linked dominant inheritance pattern Malignant degeneration of periventricular tubers Adenoma sebaceum Developmental delay Enamel pits in teeth

Ash-leaf spots Macrocephaly

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Adenoma sebaceum Enamel pits in teeth Ash-leaf spots

The diagnosis is based on two or more of the following: skin: hypo-pigmented maculae, ash-leaf patches, adenoma sebaceum, shagreen patches teeth: enamel pits eye: choroidal hamartomas, hypo-pigmented patches in iris CNS: periventricular tubers, cerebral astrocytomas, sacrococcygeal chordomas CVS: cardiac rhabdomyomas renal: angiomyolipomas lungs: diffuse interstitial fibrosis malignant degeneration of benign tumours is not a typical feature of TS. Developmental delay is common but not diagnostic.

Which of the following are criteria used for the diagnosis of neurofibromatosis type 1? Choose TWO options only. Inguinal freckling Six caf-au-lait patches measuring 710 mm in a post-pubertal child

Eighth nerve mass Cousin with NF-1 The presence of a neurofibroma Optic pathway glioma Macrocephaly

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Inguinal freckling Optic pathway glioma According to the 1987 National Institute of Health Consensus Developmental Conference on NF the diagnosis of NF-1 is based on evidence of two of the following: six or more caf-au-lait patches (>5 mm prepuberty, >15 mm post-puberty) two or more neurofibromas or 1 plexiform neurofibroma axillary or inguinal freckling optic pathway glioma two or more Lisch nodules osseous lesion (sphenoid dysplasia, thinning of cortex on long bone) 1st degree relative with NF-1.

Which of the following would help establish a diagnosis of bacterial meningitis in a 4-month-old boy? Choose THREE options only. Bulging fontanelle

Neck stiffness Lumbar puncture Neutrophil count of 23 x 109/l CRP 65 mg/l Photophobia

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Bulging fontanelle Lumbar puncture Neutrophil count of 23 x 109/l Neck stiffness is an unreliable sign in children under 18 months and would be particularly difficult to accurately elicit in a 4-month-old. A neutrophil count > 15 x 109/l is suggestive of a bacterial infection (though not diagnostic). Level of Creactive protein (CRP) has not been shown to help differentiate bacterial from viral infections.

A previously well 3-year-old girl presents to the Emergency Department following a head injury. The following would suggest an increased risk of intra-cranial injury and hence the possible need for computed tomography (CT) scan. Choose THREE options only. Basal skull fracture Loss of consciousness for 5 min CORRECT ANSWER Dizziness immediately after injury

Fall from height > 3m Headache on arrival that responds to paracetamol 2 vomits in the 90 min since the injury

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Basal skull fracture Loss of consciousness for 5 min Fall from height >3m NICE guideline 56 gives the detail of when to image children with head injuries. CT scanning is indicated in children under 16 if any of the following are present;

Witnessed loss of consciousness lasting > 5 minutes Amnesia (antegrade or retrograde) lasting > 5 minutes Abnormal drowsiness 3 or more discrete episodes of vomiting Clinical suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy Age > 1 year: GCS < 14 on assessment in the emergency department Age < 1 year: GCS (paediatric) <15 on assessment in the emergency department Suspicion of open or depressed skull injury or tense fontanelle Any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from ears or nose, Battle's sign) Focal neurological deficit Age <1 year: presence of bruise, swelling or laceration > 5cm on the head Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from > 3 m, high-speed injury from a projectile or an object)

www.nice.nhs.uk/nicemedia/pdf/CG56QuickRedGuide.pdf

A 14-year-old girl presents to the clinic with facial twitching, lip smacking and grimacing, which has progressed over the last 3 months. Initially just her face was involved but is now starting to affect her arms, causing unexpected jerks and her legs causing some difficulty walking. She tells you she is unable to prevent the movements. Parents report mood swings. GP has done routine blood tests (FBC, U&E, LFT) all normal. Past medical history: nil significant other than an episode of glandular fever about 4 months ago rash, high fever and sore throat resolved spontaneously. Parents are 46 and 49 years old. Both are well. No siblings. Medications: nil. Denies trying any illicit drugs. On examination: looks well repeated facial twitching, lip smacking and grimacing noted throughout consultation CVS/RS/AS normal tone, power, reflexes normal cranial nerves normal pronator drift bilaterally it was unclear if this was due to poor compliance co-ordination affected by random sudden movements of limbs. Which of the following investigations is most likely to suggest the cause of her problems? Choose ONE answer only. Chromosomal studies Cranial MRI Serum copper/ceruloplasmin/urinary copper ASOT Psychiatric/psychology referral

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This girl has chorea. The age of her parents makes Huntingtons unlikely. The absence signs/history or liver disease and normal LFTs make Wilsons disease unlikely although formal testing is probably indicated. In view of the previous pyrexial illness and mood swings, Sydenhams chorea is most likely.

A 4-month-old boy is brought to the clinic with a 3-week history of funny turns. Mum reports that his arms both lift up beside his head and he flexes at the hips. Afterwards he cries and appears upset. Initially he had one every few days but now has 34 per day. He used to be a content, happy baby and had begun to develop good head control. Now he is frequently unsettled and seems to be more floppy. On examination there is little to find apart from more than expected head lag when he is pulled to sitting. The most likely diagnosis is? Choose ONE answer only. Generalised tonicclonic seizures Juvenile myoclonus Complex partial seizures Infantile spasms Reflex anoxic seizures

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This is a classic description of infantile spasms. They are usually associated with developmental regression. Infantile myoclonic seizures may present at this age but the jack-knife/salam spasm is typical of infantile spasms. These were first described by West and his name is sometimes given to the syndrome.

What is the electroencephalogram (EEG) likely to show? Choose ONE answer only. Hypsarhythmia Three cycles per second spike and wave Temporal lobe focus Normal Burst-suppression pattern

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Hypsarhythmia is the classic finding associated with infantile spasms. If it is not found on a standard EEG it may be manifest in sleep. Three cycles per second spike and wave is associated with generalised absences (petit mal). Burst-suppression is classically seen in newborns following asphyxial insult. Which of the following therapies is indicated? Choose ONE answer only. Carbamazepine Phenytoin Steroids Phenobarbital

Levetiracetam

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ACTH or prednisolone can be given. Vigabatrin has been used in the past and is still in some centres; however, the high incidence of visual field deficits has raised concerns regarding its use. Steroids have also been shown to be more effective in controlling seizures. Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW, OCallaghan FJ, Verity CM, Osborne JP. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multi-centre, randomised controlled trial. Lancet. 2004 Nov 1319; 364(9447): 17738.

A 14-year-old with epilepsy is not fully controlled on sodium valproate. The decision has been made to add in lamotrigine. Which of the following options is correct? A higher then normal dose of lamotrigine should be started because of enzyme induction Lamotrigine should not be added, as it is contraindicated in a patient on sodium valproate A lower then normal dose of lamotrigine should be started because valproate increases plasma concentration of lamotrigine The normal dose of lamotrigine should be started The dose of lamotrigine used depends on the electroencephalogram (EEG) findings

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Most antiepileptic drugs interact in some way and care should be taken when using them in combination. Ideally monotherapy should be used where possible.

A previously well 4-year-old boy presents to the Emergency Department following a head injury. The following would suggest an increased risk of intra-cranial injury and hence the need for an urgent computed tomography (CT) scan. Choose THREE of the following. CSF rhinorrhoea Loss of consciousness for a few seconds Penetrating head injury Post-traumatic seizure Headache on arrival that responds to paracetamol 1-2 vomits in the 90 min since the injury

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CSF rhinorrhoea Penetrating head injury Post-traumatic seizure CT scanning is indicated in children under 16 if any of the following are present;

Witnessed loss of consciousness lasting > 5 minutes Amnesia (antegrade or retrograde) lasting > 5 minutes Abnormal drowsiness 3 or more discrete episodes of vomiting Clinical suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy Age > 1 year: GCS Age > 1 year: GCS < 14 on assessment in the emergency department Age < 1 year: GCS (paediatric) <15 on assessment in the emergency department Suspicion of open or depressed skull injury or tense fontanelle Any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from ears or nose, Battle's sign) Focal neurological deficit Age < 1year: presence of bruise, swelling or laceration >5cm on the head Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from > 3 m, high-speed injury from a projectile or an object)

www.nice.nhs.uk/nicemedia/pdf/CG56QuickRedGuide.pdf

A 5-month-old baby is sent to the clinic by the health visitor because of concerns regarding developmental delay. Mum reports that at 3 months he seemed to be developing head control but more recently has had difficulty holding his head up. He otherwise seems well. He follows mum round the room with his eyes, is smiley and responsive and starting to vocalise. On examination: looks well not dysmorphic smiles responsively CVS/RS/AS normal tone generally reduced marked head lag spontaneous movement of limbs against gravity you are unable to elicit tendon reflexes cranial nerves appear normal as far as testing allows.

What is likely to be the most useful investigation? Choose ONE answer only. Chromosomes Creatine kinase Cranial MRI Muscle biopsy SMN genotype

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This baby has spinal muscular atrophy type I (WerdnigHoffman disease). There is progressive degeneration of the anterior horn cells leading to progressive lower motor neurone signs. Death from respiratory failure is typical in the first year of life. The diagnosis can be confirmed by genotype (chromosome 5q13). Previously nerve conduction studies were required. The history of regression of muscle tone and power and the absence of reflexes point to spinal muscular atrophy.

A 13-year-old girl present to the clinic with dizziness, ataxia and diplopia, which began about 36 h ago and has gradually worsened. She also has a slight headache and feels nauseated. She was diagnosed as having complex partial seizures at the age of 8 years and successfully treated with carbamazepine. Previous seizures are described as some twitching of the right arm and right side of the face, drooling and confused behaviour. An electroencephalogram (EEG) at the time of diagnosis confirmed a temporal lobe

focus. No intra-cranial imaging was performed. Six months ago she had been fit-free for 2 years and so stopped her medication (carbamazepine 300 mg bid) over a period of 2 months. Unfortunately a few days ago she had a further seizure and her GP restarted her carbamazepine. Current medications: carbamazepine 300 mg bid. On examination: apyrexial orientated tone, power and reflexes normal difficulty standing and walking wide-based gait poor co-ordination extra-ocular movements: complaining somewhat inconsistently of diplopia fundi normal Kernigs sign negative CVS/RS/AS normal What is the most likely cause of her condition? Temporal lobe glioma Intracranial bleed from arterio-venous malformation Carbamazepine toxicity Partial status epilepticus Viral encephalitis

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Carbamazepine must be started at a low dose and gradually increased to a treatment dose. After 4 months any liver enzyme induction would have resolved. A space-occupying lesion is a possibility in view of her history and EEG but it is likely the onset of symptoms would have been more insidious. A lesion is more likely to take the form of mesial temporal sclerosis. An intracranial bleed is likely

to have a more sudden onset. Partial status should be considered but the findings are not typical of her seizure type and she is fully orientated.

A 12-year-old child with learning difficulties presents with generalised tonicclonic seizures. He is having 12 per week. His electroencephalogram (EEG) shows some abnormalities but nothing diagnostic. He is commenced on sodium valproate but despite reaching maximum doses he continues to have at least one seizure per week. Which of the following anticonvulsant would be most appropriate second line treatment? Topiramate Levetiracetam Ethosuximide Phenytoin Lamotrigine

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According to the NICE guidelines relating to The diagnosis and management of the epilepsies in adults and children in primary and secondary care (October 2004), generalized tonic-clonic seizures should be treated with Carbamazepine, Lamotrigine, Sodium valproate and Topiramate as first line drugs. Topiramate has been shown to be effective in children with learning difficulties. Kelly K, Stephen LJ, Sills GJ, Brodie MJ. Topiramate in patients with learning disability and refractory epilepsy. Epilepsia, 2002; 43: 399402.

Recommended second line drugs are Clobazem, Levetiracetam and Oxcarbazepine. Ethosuximide is a treatment for absence seizures. Phenytoin is used less commonly at present due to side-effects and narrow therapeutic window. Lamotrigine is sometimes used in combination with valproate in children.

A 15-year-old girl presents with a history of three generalised tonicclonic seizures over a period of 4 months. Her electroencephalogram (EEG) confirms primary generalised epilepsy. She requests treatment. What is the most appropriate treatment? Choose ONE answer only. Carbamazepine Sodium valproate Lamotrigine Topiramate Levetiracetam

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Lamotrigine is generally considered the best choice in girls because it does not interact with the oral contraceptive pill and does not have the cosmetic sideeffects of sodium valproate. Carbamazepine, sodium valproate and topiramate might also be considered as first line treatments of primary generalised epilepsy presenting with generalised tonicclonic seizures.

Which of the following would you also recommend? Choose THREE options only. It is safer to take a shower rather than a bath She will not be able to learn to drive She should not have children, as there is a high risk they will have seizures She should take care at heights Buccal midazolam to control seizures She should avoid flashing lights, as this will provoke a seizure

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It is safer to take a shower rather than a bath She should take care at heights Buccal midazolam to control seizures She can bathe but someone should be aware she is doing so and be able to reach her if she has a seizure. People with epilepsy can learn to drive of they are on treatment and have not had a fit for at least 1 year. Some forms of epilepsy are hereditary but the risk of her children having a similar problem is small. Patients with epilepsy should take care in situations where loss of control (caused by having a seizure) would injure them. Buccal mid-azolam is probably more effective in terminating seizures than PR diazepam. Unless there is a history of photosensitivity or evidence on the EEG there is no need to avoid flashing lights. Only a small proportion of epilepsies are photosensitive.

A 14-year-old girl presents with a 2-month history of headaches. They are more severe in the early morning. On examination she has papilloedema but no focal neurological signs. An urgent MRI scan arranged later that day is normal. Which of the following are the most appropriate management options? Choose TWO of the following. Formal visual field assessment Reassure, suggest analgesia and review in 2 months Psychology referral Cerebral angiogram Lumbar puncture

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Formal visual field assessment Lumbar puncture This girl has the features of Idiopathic Intracranial Hypertension (IIH). Ocular palsy can sometimes be present. Lumbar puncture will confirm the diagnosis by demonstrating the raised pressure and will probably ease the symptoms. Visual fields must be assessed regularly as the increased pressure can lead to permanent visual field loss. This condition used to be called benign intracranial hypertension but the name was changed given the permanent effects, which can result. The condition may resolve spontaneously. Treatment can be given to ease symptoms and/or reduce visual field loss in the form of acetazolamide, steroids, serial lumbar punctures or surgery.

A Carbamazepine B Gabapentin C No medication is indicated D Phenobarbital E Phenytoin F Sodium valproate G Vigabatrin Which of the above would be the firstchoice medication in the following situations? Each choice may be used once, more than once, or not at all. Scenario 1 A 3-year-old boy referred to your clinic with a history of 10 generalised seizures, each associated with fever. The boy achieved all developmental milestones at appropriate ages. His father had convulsions as a child.

No medication is indicated CORRECT ANSWER Randomised controlled trials have demonstrated that maintenance treatment with anticonvulsants in children with febrile seizures does not reduce the risk of further febrile seizures. YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 2 An 8-year-old girl with a history of looking into space on a daily basis and recent onset of poor school performance. An electroencephalogram (EEG) demonstrates a regular spike-wave discharge at 3 Hz.

Sodium valproate CORRECT ANSWER This is a case of absence seizures (also known as petit mal or absence seizures). YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT Scenario 3

An 8-month-old boy with developmental delay and recurrent episodes where he jerks his upper body forward, appearing to bend in half at the waist. An electroencephalogram (EEG) demonstrates hypsarhythmia.

Vigabatrin CORRECT ANSWER This is a case of infantile spasms (also known as Salaams seizures or Wests syndrome). YOUR ANSWER WAS CORRECT YOUR ANSWER WAS INCORRECT

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A five-year-old boy, who has not had any immunisations presents to casualty with a 3-hour history of onset of a non-blanching rash. He was rushed to hospital by ambulance and on examination; he has widespread purpurea, a capillary refill time of 5 seconds and a heart rate of 160. His airway is secure and intravascular access has been obtained. Following 3 sets of 20 ml/kg fluid boluses, his hear rate drops to 90 and his central capillary refill time is 1 second. He has been given 80 mg/kg of ceftriaxone. The anaesthetist has been called to help intubate him. You remember reading about the importance of recognizing raised intracranial pressure in a child with meningitis. Which of the following are characteristic clinical features of a patient with raised intracranial pressure? Choose FOUR answers. Hypertension Bradycardia Sunsetting Absence of papilloedema Vomiting

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Hypertension Bradycardia 'Sunsetting' Vomiting It is vital not to carry out a lumbar puncture in an acutely unwell child in whom the possibility of raised intracranial pressure exists, as this procedure could lead to the death of the child secondary to coning. In a child with raised intracranial pressure, the priorities are to nurse in the midline, restrict maintenance fluids to two-thirds of normal, optimise metabolic state, ventilate to achieve low normal pa(CO2) and the use of osmotherapy such as mannitol or hypertonic saline. Arrangements should be made to transfer the child to a paediatric intensive care unit.