Neurology

Question 1

Cerebral palsy; definition, epi, aetio.

Answer 1

CP is a disorder of posture and movement due to non-progressive damage to the developing brain.

Presents by the age of 3yrs, 2per 1000 live births.

Aetio
-80% are antenatal in origin (due to genetic defects)
-10% due to HIE during delivery (preterms are esp vulnerable to brain damage from periventricular leucomalacia)
-10% are postnatal (meningitis, TBI, hydrocephalus, hyperbilirubinemia…)

Question 2

Differentiating CP with other neurological conditions.

Answer 2

In CP, the child will not lose any milestones they’ve already gained even if motor disorder will evolve w/o Rx. (The milestones may be delayed)

In other neuro disorders, there is progressive loss of already gained milestones.

Question 3

Classification of cerebral palsy.

Answer 3

Spastic 90%
Dyskinetic 6%
Ataxic 4%

Hemiplegic, diplegic, quadriplegic, monoplegic & triplegic

Question 4

Categorisation of severity in CP.

Answer 4

Gross Motor Function Classification System (GMFCS)

I= walks w/o limitations
II= walks w/ limitations
III= walks w/ handheld mobility devices (frames)
IV= self mobility w/ limitations (may use powered ones)
V= transported in a manual wheelchair

Question 5

Spastic CP features

Answer 5

Damage to the UMN in the pyramidal/corticospinal tract with spasticity and exaggerated reflexes. May have initial hypotonia.

Has types; hemiplegia, diplegia, quadriplegia

Question 6

Dyskinetic CP features

Answer 6

Basal ganglia are affected with affection of the upper limb being more severe.

Most commonly due to HIE and kernicterus

CF are floppiness, poor trunk control and delayed motor development in infancy.

Question 7

Ataxic CP features

Answer 7

Parkinsonism traits in gait etc and usually affects the same side and are mostly genetically determined.

Question 8

Mx of CP

Answer 8

MDT consisting of parents, paediatrician, physiotherapists, occupational therapists, ENT surgeons, eye surgeons, orthopaedic surgeons, social workers, community care nurse and teachers.

Question 9

Drugs used in CP

Answer 9

Baclofen to reduce muscle tone
Bensexole (Artaine) to reduce dystonia
Botulinum toxin
Surgery for contractures

Question 10

Bacterial causes of meningitis in different age groups

Answer 10

Neonate - 3 months = GBS, E.coli, Listeria
1m- 6yrs = Neisseria meningitidis, S. pneumoniae, H. influenza
>6 yrs = Neisseria, Strept

Question 11

When to consider neuroimaging in meningitis?

Answer 11

When fever doesn’t settle with ABx
Signs of brain abscess
Signs of increased ICP
In focal (one-sided) seizures

Question 12

Contraindications for LP(8)

Answer 12

Cardiorespiratory instability
Focal neurological signs
Signs of increased ICP
Coagulopathy
Thrombocytopenia
Location infection at site of LP
Shock
No consent

Question 13

CSF findings in partially treated meningitis

Answer 13

Lymphocytes will be high instead of neutrophils, proteins remain high, sugar remains low and culture is -ve.

Question 14

Complications of meningitis

Answer 14

Hearing loss
Local vasculitis leading to cranial nerve palsies
Local cerebral infarctions
Subdural effusions
Hydrocephalus
Cerebral Abscess (vanco and drainage)

Question 15

Causes of un-resolving fever in meningities

Answer 15

Wrong ABx and/or dose, viral/fungal aetio, TB, formation of a brain abscess

Question 16

Common causes of encephalitis

Answer 16

HSV (ass. w/ temporal lobe epilepsy) Rx acyclovir IV
JE
Dengue

Question 17

Clinical features of Febrile Seizures

Answer 17

Generalised
<15 minutes
One episode/day
No brain damage

Question 18

Mx of febrile seizures

Answer 18

Advise parents to not out anything in the mouth during the seizure, and if it lasts more than 5 min; to hospitalise the child

Antiepileptics (eg: clobazam) is given only during fever days

Question 19

Complication of Guillain-Barre Syndrome

Answer 19

Miller- Fischer Syndrome; ataxia, areflexia and external ophthalmoplegia

Question 20

Duchenne’s muscular dystrophy; aetio, epi

Answer 20

X-linked recessive disease that affects the gene coding for dystrophin leading to necrosis of muscle cells and their replacement with fat

Usually present by 3-5yrs

Question 21

Duchenne’s; CF

Answer 21

Problems in gross motor development milestones
Gower’s sign (stand using arms)
Pseudohypertrophy of calf muscles
Waddling gait
Language delay

Question 22

Duchenne’s; Prognosis and complications

Answer 22

Poor prognosis w/o Rx; death by late 20s due to respiratory failure or ass. cardiomyopathies (dilated)
If good RX; 50-60s w/ loss of independent ambulation.
Scoliosis is another common complication

Question 23

Duchenne’s; Investigations

Answer 23

Serum creatinine phosphokinase is markedly elevated (x1000)
Muscle biopsy
DNA testing

Question 24

Duchenne’s; Mx

Answer 24

Medical = corticosteroids 10d each month
MDT consisting of physiotherapy, paediatricians, speech therapist, occupational therapist, orthopaedics, social worker etc

Question 25

Causes of Bell’s palsy in children

Answer 25

CP angle tumour
Parotid enlargement due to mumps
Forceps delivery
Herpes
Idiopathic

Question 26

Juvenile myasthenia; patho, epi, CF

Answer 26

Progressive muscle weakness throughout the day due to declining levels of acetylcholine due to antibodies against ACh receptors.

Presentation is usually by 10y/o w/ ophthalmoplegia and ptosis, difficulty chewing and loss of facial expression (+generalised muscle weakness).

Question 27

Juvenile myasthenia; Ix, Mx

Answer 27

Ix = IV edraphonium over minutes followed by pyridostigmine/neostigmine over days in the HDU/ICU

Mx = ACh inhibitors; pyridostigmine, neostigmine, immunosuppressive therapy, immuno-modulating drugs (MMF, prednisolone, azathioprine)
Thymectomy is thymoma present

Plasma exchange is used during a crisis.

Question 28

Neurofibromatosis 1; genetics, criteria

Answer 28

De novo (50%) mutation of the neurofibromin-1 (NF-1) gene w/ autosomal dominant inheritance

Criteria; >2 more of the following;
->6 cafe-au-lait spots prepuberty, >15 after puberty
->1 neurofibroma
-axillary freckling
-optic glioma
-1 Lisch nodule (hamartoma on the iris seen on slit-lamp exam)
-bony lesions from sphenoid dysplasia
-1st degree relative w/ NF1

Question 29

Neurofibromatosis 2; gene, CF

Answer 29

Less common, autosomal dominant inheritance of the NF-2 gene presenting w/ multiple inherited Schwannomas, meningiomas and ependymomas

Question 30

Associated conditions w/ neurofibromatosis

Answer 30

MENS (endocrine disorders)
Phaeochromocytoma
Pulmonary HTN
Renal artery stenosis

Question 31

Tuberous sclerosis; cutaneous features

Answer 31

Depigmented ash leaf patches or a melanotic naevi (light up under UV)
Shagreen patches; roughened patches of skin over the lumbar spine
Angiofibromata (adenoma sebaceum) over the bridge of the nose and cheek; may be present from birth/ present at 3yrs

Question 32

Tuberous sclerosis; neurological features

Answer 32

Infantile spasms
Epilepsy (often focal)
Intellectual disability (often w/ autism)

Question 33

Tuberous sclerosis; other features

Answer 33

Subungual fibromata
Phakomata (dense white areas on the retina from degeneration)
Rhabdomyomata of the heart in infancy that resolves
Angiomyolipomas
Polycystic kidneys
Cysts in lungs

Question 34

Tuberous sclerosis; Mx

Answer 34

Seizures; vigabatrine
cardiology follow-up
USS KUB
Special education

Question 35

Sturge-Weber syndrome

Answer 35

Sporadic disorder w/ a haemangiomatous facial lesion (port-wine stain) in the Cr5 distribution and ass. intracranial lesion (leptomeningeal angioma)

There is a high risk of ipsilateral glaucoma

Question 36

DDx of meningocele vs. meningomyelocele

Answer 36

Meningocele; covered by skin and nerve tissue is normal

Meningomyelocele; not covered by skin therefore is at a higher risk of rupture, infection and meningitis. Nerve tissue lies outside

Question 37

Myelomeningocele associations

Answer 37

Variable paralysis of the legs
Muscle imbalance
Sensory loss
Bladder denervation
Bowel denervation
Scoliosis
Hydrocephalus from a Chiari malformation

Question 38

Types of hydrocephalus with their relative causes.

Answer 38

Communicating = obstruction at the arachnoid villi (the site of absorption of CSF).
- SAH, meningitis

Non-communicating = obstruction w/i the ventricular system or aqueduct.
- Congenital malformation (Chiari, aqueduct stenosis), posterior fossa neoplasm or vascular malformation, intraventricular haemorrhage in a preterm infant

Question 39

10 features to look for in hydrocephalus

Answer 39

Sunsetting sign
Papilloedema
Cushing’s reflex; HTN w/ bradycardia
Examine shunt
Features of shunt infection
Association; meningomyelocele
Hepatosplenomegaly
Severity
Vision, hearing, bowel and bladder function