Neuro

Question 1

Seizures Ix

allowed one simple seizure without investigation

Answer 1

Bloods: FBC, UandE, cultures, blood glucose

Orifices: LP, urine

X:

ECG

S: MRI??
EEG
PET/SPECT pre surgery??

Question 2

Seizures MRI indications

Answer 2

under 2
focal
no response to tx

Question 3

Tonic Clonic Management

Answer 3

First line: sodium valproate

Second line: lamotrigine or carbamazepine

Valproate:
Teratogenic, so patients need careful advice about contraception
Liver damage and hepatitis
Hair loss
Tremor

Question 4

Focal Seizures TX

Answer 4

One way to remember the treatment is that the choice of medication is the reverse of tonic-clonic seizures:

First line: carbamazepine or lamotrigine
Second line: sodium valproate or levetiracetam

Carbemazepine:
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions

Question 5

Generalised seizure definition

Answer 5

TLOC >3s, symmetrical, no aura

Question 6

Focal seizure definition

Answer 6

aura, local group, hallucinations, deja vu, flashbacks, autopilot

Question 7

Absence Seizures tx

Answer 7

First line: sodium valproate or ethosuximide

Question 8

Atonic Seizures tx (Lennox Gastaut??)

Answer 8

First line: sodium valproate

Second line: lamotrigine

Question 9

Myoclonic Seizures

Answer 9

First line: sodium valproate

Other options: lamotrigine, levetiracetam or topiramate

Question 10

Infantile Spasms (West Syndrome)

Answer 10

Prednisolone

Vigabatrin

Question 11

Seizures advice

Answer 11

Take showers rather than baths
Be very cautious with swimming unless seizures are well controlled and they are closely supervised
Be cautious with heights
Be cautious with traffic
Be cautious with any heavy, hot or electrical equipment

avoid driving if teen

Question 12

Tension Headaches tx

Answer 12

reassurance, analgesia, regular meals, avoiding dehydration and reducing stress.

VISION CHECK

Question 13

Migraines tx

Answer 13

Rest, fluids and low stimulus environment
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics, such as domperidone (unless contraindicated)

VISION CHECK

Question 14

Migraine prophylaxis

Answer 14

Propranolol (avoid in asthma)
Pizotifen (often causes drowsiness)
Topiramate (girls with child bearing potential need highly effective contraception as it is very teratogenic).

Question 15

Simple Febrile Convulsions def

Answer 15

tonic clonic, less than 15 mins, once in illness

Question 16

Complex Febrile Convulsions def

Answer 16

partial/focal, more than 15 mins, multiple in one illness

need investigations

Question 17

Febrile convulsion management

Answer 17

identify and manage the underlying source of infection and control the fever with simple analgesia such as paracetamol and ibuprofen.

Simple febrile convulsions do not require further investigations and parents can be reassured and educated about the condition. Complex febrile convulsions may need further investigation.

Question 18

Febrile convulsion advice

Answer 18

Stay with the child
Put the child in a safe place, for example on a carpeted floor with a pillow under their head
Place them in the recovery position and away from potential sources of injury
Don’t put anything in their mouth
Call an ambulance if the seizure lasts more than 5 minutes

visit their GP at the next available opportunity

Question 19

Anoxic Seizures (reflex/cyanotic) management

Answer 19

After excluding other pathology and making a diagnosis, educating and reassuring parents about breath holding spells

iron deficiency anaemia

Question 20

Seizures management - general principles

Answer 20

Drugs not for all
Monotherapy first
vagal stimulation?
keto diet (low carb, high fat)

Question 21

Floppy infant central causes

Answer 21

Encephalopathy (HIE, trauma, BM) –> CP
Chronic encephalopathy (metabolic inborn errors, hypothyroid)
CTD - ehlers danlos, oi
Hydrocephalus (arachnoid cysts, aqueductal stenosis, chiari malformation, chromosomal abnormalities)

Question 22

Floppy infant peripheral causes

Answer 22

SMA
NMJ e.g. myaesthenia
Poliomyelitis
congential myopathies e.g. dmd

Question 23

Floppy infant ix

Answer 23

Central:
Bloods - metabolic, tft, u and e, plasma aminos, fatty acids

Urine GAGs

Medical genetics/ chromosomes

CT/MRI

Peripheral:
muscle biopsy
emg
ck and lactate
SMN gene (SMAT1)

Question 24

Signs and symptoms of cerebral palsy

Answer 24

Failure to meet milestones
Increased or decreased tone, generally or in specific limbs
Hand preference below 18 months is a key sign to remember for exams
Problems with coordination, speech or walking
Feeding or swallowing problems
Learning difficulties

Question 25

Medications for CP (alongside MDT)

Answer 25

Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling

Question 26

Gower’s Sign

Answer 26

proximal muscle weakness - DMD

using their hands on their legs to help them stand up

X-linked recessive

Question 27

DMD tx

Answer 27

MDT

surgical and medical management of complications such as spinal scoliosis and heart failure

Oral steroids have been shown to slow the progression of muscle weakness by as much as two years.

Creatine supplementation can give a slight improvement in muscle strength.

Question 28

Duchennes Muscular Dystrophy

Answer 28

dystrophin on the X-chromosome

Boys with Duchennes present around 3 – 5 years with weakness in the muscles around their pelvis.

Question 29

Beckers Muscular Dystrophy

Answer 29

dystrophin gene is less severely affected and maintains some of its function

Symptoms only start to appear around 8 – 12 years

Question 30

Myotonic Dystrophy

Answer 30

presents in adulthood

Progressive muscle weakness
Prolonged muscle contractions - E.G. can’t release handshake or doorknob
Cataracts
Cardiac arrhythmias

Question 31

Facioscapulohumeral Muscular Dystrophy

Answer 31

Facioscapulohumeral muscular dystrophy usually presents in childhood with weakness around the face, progressing to the shoulders and arms

Question 32

Oculopharyngeal Muscular Dystrophy

Answer 32

late adulthood

bilateral ptosis, restricted eye movement and swallowing problems

Question 33

Limb-girdle Muscular Dystrophy

Answer 33

Limb-girdle muscular dystrophy usually presents in teenage years with progressive weakness around the limb girdles (hips and shoulders).

Question 34

Emery-Dreifuss Muscular Dystrophy

Answer 34

Emery-Dreifuss muscular dystrophy usually presents in childhood with contractures, most commonly in the elbows and ankles

Question 35

SMA signs

Answer 35

lower motor neurone:

fascinations, reduced muscle bulk, reduced tone, reduced power and reduced or absent reflexes

Question 36

SMA type 1

Answer 36

first few months of life, usually progressing to death within 2 years

Question 37

SMA type 2

Answer 37

onset within the first 18 months. Most never walk, but survive into adulthood

Question 38

SMA type 3

Answer 38

onset after the first year of life. Most walk without support, but subsequently loose that ability. Respiratory muscles are less affected and life expectancy is close to normal

Question 39

SMA type 4

Answer 39

onset in the 20s. Most will retain the ability to walk short distances but require a wheelchair for mobility. Everyday tasks can lead to significant fatigue. Respiratory muscles and life expectancy are not affected.

Question 40

SMA tx

Answer 40

MDT
Physio
NIV (SMA1 - trachy)
PEG feeding if unsafe swallow