Neurology Flashcards
A 3 year old is brought into accident and emergency by ambulance following a generalized tonic clonic seizure that lasted 2 minutes. She did not require any treatment to stop the seizure but on arrival the ambulance crew measured her temperature as 39.2°C and gave paracetamol. She is now apyrexial with a heart rate of 140, respiratory rate of 30 and capillary refill less than 2 seconds. On examination she has red enlarged tonsils with no pus, no neck stiffness or rash. What is the most appropriate management?
A. Oral penicillin
B. Lumbar puncture and IV ceftriaxone
C. Explain that this was a febrile convulsion and discharge home
D. Start phenytoin
E. Discharge home with rescue buccal midazolam for future seizures
C. Explain that this was a febrile convulsion and discharge home
1 C This is a typical febrile convulsion which are associated with rapid rises in temperature. The child is now stable and likely has a viral tonsillitis as the focus of infection. Parents need reassurance and explanation that febrile convulsions are not usually associated with later epilepsy but may recur in future febrile illnesses. Typically, children do not continue to have febrile convulsions beyond the age of 5 years. Management should be regular anti-pyretics and dress the child lightly during febrile illnesses. Oral penicillin (A) should be prescribed for bacterial tonsillitis, either with pus or if the child is unwell clinically. IV ceftriaxone and lumbar puncture (B) would be for suspected meningitis but this 3-year-old child has a clear focus for the fever, no neck stiffness or rash making this unlikely. In children having their first febrile convulsion under the age of 12 months, it is mandatory to rule out meningitis. Phenytoin (D) would be used for status epilepticus (seizure lasting longer than 30 minutes). Buccal midazolam (E) is used in the community for children who have seizures lasting longer than 5 minutes; febrile convulsions are usually short and unless a child has had a prolonged seizure they would not be sent home with rescue medication.
A 7 year old is referred to neurology due to frequent episodes of day-dreaming at school where she is unresponsive. She is falling behind in her work because of this. An electroencephalograph (EEG) shows three spike waves per second activity in all leads. What is the most likely diagnosis?
A. Temporal lobe epilepsy
B. Absence epilepsy
C. Day-dreaming
D. Benign Rolandic epilepsy
E. Narcolepsy
B. Absence epilepsy
2 B This is a typical presentation of absence epilepsy (B). This form of epilepsy often does not affect school performance, but frequent seizures interrupt the directions or information she receives at school and may contribute to her falling behind in her work. The EEG shows three spike waves per second in all leads, which is diagnostic for absence seizures. Temporal lobe epilepsy (A) would show an EEG with seizure activity in the temporal lobes and classically presents with a warning aura or sensation, impaired consciousness or unresponsiveness, and a focal seizure which may spread to become a generalized tonic clonic seizure. In day-dreaming (C), the person is responsive if called. Benign Rolandic epilepsy (D) causes partial seizures which usually occur in the early morning and affect young children, typically resolving in the teens. Narcolepsy (E) is a sleep disorder where affected persons spontaneously fall asleep at inappropriate times.
A mother brings her 2-year-old daughter to the GP on a Monday morning. Over the weekend she became very upset on being told ‘no’. She was screaming and then held her breath, went blue and fainted. She woke up quickly and seemed okay afterwards. However, it has just happened again this morning when she found some scissors and her mother took them away. On this occasion she had a brief generalized convulsion lasting about 10 seconds. What is the most likely explanation?
A. Breath holding attacks
B. Reflex anoxic seizures
C. Absence epilepsy
D. Wolff–Parkinson–White syndrome
E. Vasovagal syncope
A. Breath holding attacks
3 A Breath holding attacks (A) are not uncommon in toddlers at times of temper tantrums. They grow out of them and no treatment is required. Parents need to be reassured that the brief seizure is not harmful. A reflex anoxic seizure (B) typically occurs when a child is frightened or hurt, such as a bump to the head. The child goes pale and faints, hypoxia may cause a short seizure, but the child quickly recovers. Absence epilepsy (C) presents with short vacant periods where the child is unresponsive, followed sometimes by a brief period of confusion when they are aware they have missed something. Wolff–Parkinson–White syndrome is associated with a spontaneous onset re-entry tachycardia or supraventricular tachycardia which may lead to dizziness, shortness of breath and sometimes fainting. An ECG would show a delta wave. Vasovagal syncope, or a simple faint, is associated with standing for long periods of time in warm environments.
A mother with known placenta praevia with heavy vaginal bleeding was rushed into the labour ward and delivered by emergency caesarean section at 35 weeks’ gestation. Pre-delivery the fetus was bradycardic and after birth APGARs were three at 1 minute, five at 5 minutes and nine at 10 minutes. Thirty-six hours later on the special care baby unit the baby is irritable and requiring nasogastric tube feeds as he is not sucking well. The tone in his upper limbs is reduced and an EEG showed seizure activity which has been controlled by intravenous phenobarbitone. His cranial ultrasound is normal. His blood sugar monitoring is between 3.5 and 5 mmol/L, C-reactive protein (CRP) was less than 5 mg/L and is 7 mg/L today. He is apyrexial. What is the most likely diagnosis?
A. Intraventricular haemorrhage
B. Group B streptococcal meningitis
C. Hypoglycaemia
D. Mild hypoxic ischaemic encephalopathy (HIE)
E. Moderate HIE
E. Moderate HIE
4 E This child has experienced an hypoxic insult perinatally during his mother’s haemorrhage and is now showing signs of HIE: poor feeding, altered tone and seizure activity on EEG. Mild HIE (D) presents with irritability, startle responses, poor feeding and hyperventilation. Moderate HIE (E) also has altered tone or reduced movement and seizure activity. While ischaemic changes early on may be difficult to see on cranial ultrasound, intraventricular haemorrhage (A) is easily seen and is associated with both hypoxic insults and a cause of seizures in the neonate. This child’s cranial ultrasound was normal. Group B streptococcal meningitis (B) is a serious and life-threatening illness for the neonate and may cause seizures. It is therefore routine practice to treat unwell neonates with antibiotics, but the exposure for this infant is reduced as the delivery was a caesarean section and the CRP has been normal twice, making this an unlikely explanation for this illness. Hypoglycaemia (C) is also a cause of seizures in neonates but the definition is lower in this age group (below 2.5 mmol/L) so this child is not hypoglycaemic.
A 15-year-old girl comes to accident and emergency complaining of sudden right arm weakness and double vision. Last week she was incontinent of urine twice. She is normally fit and well. On examination she has a left-sided 6th nerve palsy and four out of five power in her right arm. The examination is otherwise unremarkable. An MRI head shows multiple hyperintense, inflammatory, white matter lesions. What is the most likely diagnosis?
A. Brain metastasis
B. Multiple sclerosis
C. Tuberous sclerosis
D. Tuberculous meningitis
E. Neurofibromatosis
B. Multiple sclerosis
5 B She has presented with neurology consistent with multiple lesions. All of the options may cause more than one lesion in the brain but multiple sclerosis (MS) (B) classically affects the white matter with demyelinating lesions which are inflamed, as seen in this case. Brain metastasis (A) and tuberculosis (D) would typically cause space occupying lesions with surrounding oedema. In tuberous sclerosis (C), the MRI typically shows subependymal calcifications and hypointense white matter lesions or tubers. Neurofibromatosis (E) may cause gliomas or acoustic neuromas which would be seen as a space occupying lesion. The MRI taken together with a history of intermittent and sudden changes in neurological function, especially vision and urinary incontinence, point towards the diagnosis of MS.
A 15-year-old girl is brought into accident and emergency from school having disclosed to a friend that she took 10 paracetamol tablets last night. Her blood level of paracetamol is below the treatment line, her liver function tests and clotting are normal. Her father died of a brain tumour 3 years ago and her mother is being treated for reactive depression. The girl tells you that she has been feeling low lately, particularly because she does not think she will do well in her up-coming exams. She regrets taking the tablets and does not think she will do it again. What is the most appropriate management?
A. Admit for monitoring of liver function and Child and Adolescent Mental Health Services (CAMHS) assessment
B. Refer to CAMHS as an outpatient and discharge as not currently suicidal
C. Refer to Social Services
D. Start antidepressant – fluoxetine
E. Start IV Parvolex
A. Admit for monitoring of liver function and Child and Adolescent Mental Health Services (CAMHS) assessment
6 A She should be admitted for observation until CAMHS have assessed her (A) and created a follow-up plan. As she presented after the window of intervention for her overdose, her liver function should be monitored.
While she denies feeling suicidal now, it is important that she be risk-assessed by mental health services and be discharged with a safety net that she can access if her feelings are driving her to repeat self-harm activities. She should therefore not be discharged (B). Social Services (C) may be required if it is felt that the mother’s depression is leading to issues of neglect, and sometimes Social Services and CAMHS will see patients together but this is more common with illicit drug or alcohol misuse. An antidepressant would not be the first line treatment for adolescent depression and she should first be assessed by CAMHS. Parvolex (E) is used to treat and reverse the toxicity of paracetamol; however it is ineffective beyond 15 hours post-overdose.
Which of the following is not a feature of a UMN lesion?
A. Slow-relaxing Achilles tendon reflex
B. Brisk reflexes
C. Increased tone
D. Decreased power
E. Up-going plantar reflex
A. Slow-relaxing Achilles tendon reflex
7 A Slow-relaxing reflexes may be a sign of a systemic illness such as hypothyroidism or lower motor neuron diseases such as Guillain–Barré syndrome. Increased reflexes (B) and tone (C), decreased power (D) and up-going plantar responses (E) are all features of UMN lesions.
Which for the following is not a feature of raised ICP?
A. Headache
B. Morning vomiting
C. Sun setting eyes
D. Bulging anterior fontanelle
E. Papilloedema
D. Bulging anterior fontanelle
8 D All of these options are associated with intracranial pathology but a bulging anterior fontanelle occurs when there is increased fluid in the brain in a young child before the fontanelle closes; thus there will be no raised ICP as the open fontanelle provides a space for the extra fluid. In an older child with the same underlying pathology and a closed fontanelle, the result is raised ICP. Symptoms and signs may include: headache (A), morning vomiting (B), sun setting eyes (C) (a late sign of raised ICP caused by pressure on cranial nerves III, IV and VI) and papilloedema (again a late sign of raised ICP).
A 10-year-old girl with sickle cell disease presents to her GP on Monday morning complaining of weakness in her right leg. She says she collapsed on Saturday afternoon and has not felt right since. What is the most likely diagnosis?
A. Sickle cell painful crisis
B. Parvovirus B19 infection
C. Aplastic crisis
D. Cerebral infarction
E. Osteomyelitis of the right femur
D. Cerebral infarction
9 D Here the worrying and important diagnosis to rule out is a stroke (D). Sickle cell patients are at risk of stroke, especially if their sickle cell percentage is above 30 per cent of the total red blood cell population. She is not complaining of pain in her leg, just weakness; therefore this is not a painful crisis (A) or osteomyelitis (E). Sickle cell patients with parvovirus B19 infection (B) are at risk of an aplastic crisis (C) with associated secondary infections, bleeding and severe anaemia, but these would not explain her symptoms.
A 6-year-old boy is registering with a new GP, having just moved to the area. He is in a wheelchair but is able to mobilize with a fast scissoring gait over short distances. He has increased tone in his legs and has scars from previous tendon release surgeries. His upper limbs are normal. His mother says that his school performance is good and he is writing well. She thinks he was going to have a Statement of Special Educational Needs assessment before they moved. As the GP, what is the most appropriate next step in management?
A. Reassure his mother that as he is doing well at school he does not need a statement
B. Refer to a community paediatrician
C. Refer to the physiotherapists and occupational therapists
D. Liaise with his new school teacher to make sure the school is able to support his physical needs
E. Refer to an educational psychologist
B. Refer to a community paediatrician
10 B This child has diplegic cerebral palsy and needs multidisciplinary support to manage his care. He should have a formal statement to create a plan of what physical support he will need for school, so (A) is wrong. The community paediatrician (B) is best placed to coordinate this process, although assessments by physiotherapists and occupational therapists (C), liaison with the school (D), and educational psychology (E) reports will be needed. In addition he will need to be referred to orthopaedic specialists and possibly Social Services to assess eligibility for grants to adapt the home.
A 6-year-old boy is taken to see the GP by his mother because he has been getting severe abdominal pains, sometimes with vomiting and yesterday with a headache as well. He has no diarrhoea or constipation. His growth and examination are normal. He has no significant past medical history. In his family history, his maternal grandfather recently died of gastric cancer and mum’s migraines have been worse since his death. She is worried her son is getting gastric cancer too. What is the most likely diagnosis?
A. Crohn’s disease
B. Brain tumour
C. Somatization disorder
D. Gastric cancer
E. Coeliac disease
C. Somatization disorder
11 C This child presents with abdominal pain, sometimes with vomiting or associated headache, with a family history of migraine and distinct psychological triggers, making the most likely diagnosis a somatization disorder (C). This term encompasses various different descriptive terms such as abdominal migraine, recurrent abdominal pain, non-organic pain, functional pain and irritable bowel syndrome. Children may experience migraines as abdominal pain and as they get older they develop more classical migraine headaches. Crohn’s disease (A) would usually present in an older child (but can occur in this age group) with changes in bowel habit and failure to thrive. There is often a family history, which is not present in this case. A brain tumour (B) would be more likely to present with headaches rather than abdominal pain as well as having neurological signs and symptoms. Gastric cancer (D) is very rare in children but would have features of poor appetite and weight loss as well. Coeliac disease (E) usually presents with abdominal distension, diarrhoea and failure to thrive.
Which of the following is not the correct side effect of anti-epileptic medicine?
A. Sodium valproate – aplastic anaemia
B. Carbamazepine – visual disturbance
C. Lamotrigine – rash
D. Vigabatrin – behavioural disturbance
E. Levetiracetam – anorexia
A. Sodium valproate – aplastic anaemia
12 A Sodium valproate (A) side effects include increased appetite, weight gain, hair loss and liver failure, but not aplastic anaemia. Carbamazepine (B) is associated with lupus erythematosus syndrome, dizziness and visual disturbances. Lamotrigine (C) is associated with rash, behavioural changes and irritability. Vigabatrin (D) side effects include behavioural changes, retinopathy, sleep disturbance and weight gain. Levetiracetam (Keppra) is associated with anorexia, abdominal pain, vomiting, diarrhoea, behavioural changes and thrombocytopenia.
A 13-year-old Somali girl presents to accident and emergency with a 1-month history of headaches, weight loss and night sweats. Her father is concerned that she seems confused and is more unwell with her headache despite paracetamol. She was born in the UK and has had all her immunizations. She travelled to Somalia 6 months ago. The rest of the family is well although dad has a cough. On examination she is thin and looks unwell but is neurologically intact with no abnormal findings on clinical examination. Which diagnosis needs to be ruled out first?
A. Brain tumour
B. Tuberculous meningitis
C. Pulmonary tuberculosis
D. Migraines
E. HIV infection
B. Tuberculous meningitis
13 B A brain tumour (A) is unlikely with a normal neurological examination. She has weight loss and night sweats which should raise a suspicion of tuberculosis (TB), which is particularly common in the Somali community. There is no specific history of cough and more concerning is her headache and confusion, making the most likely diagnosis tuberculous meningitis (B). She should have a lumbar puncture, provided there are no signs of raised ICP. A chest x-ray would be performed routinely in this case to look for pulmonary TB (C). While migraines (D) may be very troublesome and frequent, they do not cause weight loss and night sweats and investigations for lymphoma or tuberculosis should always be carried out when these symptoms are present. Patients diagnosed with TB should be screened for HIV (E) co-infection, but this does not need to be done immediately as it will not change the initial management.
A 4-month-old baby being investigated for infantile spasms is noted to have an ash leaf macule on his back under Wood’s light. His EEG shows hypsarrythmia. The report of his MRI brain states there are subependymal nodules. What is the diagnosis?
A. Neurofibromatosis type I
B. Neurofibromatosis type II
C. West’s syndrome
D. Tuberous sclerosis
E. Tay–Sachs disease
D. Tuberous sclerosis
14 D Ash leaf macules are a skin manifestation of tuberous sclerosis (D) and the typical MRI findings of this are subependymal nodules. Tuberous sclerosis is a cause of infantile spasms, associated with hypsarrythmia on EEG, which are treated with vigabatrin. Seizures do occur in neurofibromatosis ((A) and (B)) but are much less common and not usually associated with any imaging changes. West’s syndrome (C) is a syndrome of infantile spasms and developmental regression, one cause of which is tuberous sclerosis. As you are given no information on this child’s development, West’s syndrome is not the best answer when there is clear evidence to support the cause as tuberous sclerosis. Tay–Sachs disease (E) is an autosomal recessive deficiency of hexosaminidase A and presents with seizures, developmental regression, deafness, progressive loss of motor function and increased tone, which is most common in Ashkenazi Jewish populations.
A 3 month old is brought into accident and emergency with a generalized tonic clonic seizure. She is apyrexial and the seizure stopped after 15 minutes with rectal diazepam given by the ambulance crew. Her heart rate is 130, respiratory rate of 36 and capillary refill is less than 2 seconds. On examination she is drowsy, has a port wine stain on her forehead but is otherwise normal on examination. What is the most likely cause of her seizure?
A. Sturge–Weber syndrome
B. Tuberous sclerosis
C. Neurofibromatosis type I
D. Meningitis
E. Neurofibromatosis type II
A. Sturge–Weber syndrome
15 A A port wine stain is a flat purple haemangioma which is present from birth. Any baby born with a port wine stain in the trigeminal region or in the hair should have an MRI brain to look for intracranial haemangiomas as they are at risk of epilepsy. This is called Sturge–Weber syndrome (A). Tuberous sclerosis (B) causes infantile spasms which are brief tonic movements sometimes called ‘salaam attacks’ as a description of the movement and often mistaken for infant colic. Neurofibromatosis ((C) and (E)) rarely causes seizures so this would not be the most likely cause. A child with meningitis (D) which has caused a seizure would be unwell with fever and possibly evidence of shock, whereas this child has a normal capillary refill, a normal heart rate and is apyrexial.
