Neurology Questions
Dr Helen Nightingale
Correct stems highlighted in bold
Question 1
Which of the following are true about confirming brainstem death?
1.
2.
3.
4.
5.
There should be absence of pupil responses
There should be no plantar responses
The oculo-vestibular reflexes should be absent
There should be no cough reflex
It should be undertaken by one doctor who has been qualified for more
than five year
Extended answer
According to the academy of royal colleges to confirm brainstem death there
should be:
 No brain-stem reflexes
 Fixed pupils which do not respond to “ sharp changes” in light intensity
 No corneal reflex
 No oculo-vestibular reflex
This test involves the slow injection into each external auditory meatus of (at
least) 50mls of ice cold water. It is essential that there is clear access to the
tympanic membranes and the head should be at 30o . An intact brainstem
response would elicit nystagmuss with fast movements away from the injected
ear. In brainstem death no response is seen.
 No cranial nerve motor responses
Central (or peripheral) painful stimuli would normally produce a response in
the cranial nerve territory - this is not the case in brainstem death.
 No cough or gag response
 No chest movement in response to hypercapnia – the apnoea test
This should only be performed if the other tests are consistent with brainstem
death. The patient is preoxygenated with 100% oxygen for 10 minutes to
achieve a PCO2 of > 5 kPa. The patient is then disconnected from the
ventilator but 6 L/min of oxygen is still deliveryed by the endotracheal tube.
The pCO2 should then rise to > 6.65 kPa. If there are no chest movements
this confirms brainstem death. The patient can then be again attached to the
ventilator.
This must be carried out by 2 or more medical practitioners who have been
registered for more than 5 years. At least one must be a consultant and they
must be competent in undertaking and interoperating brain-stem testing.
Please see the link below for more information
http://www.bts.org.uk/Documents/A%20CODE%20OF%20PRACTICE%20FO
R%20THE%20DIAGNOSIS%20AND%20CONFIRMATION%20OF%20DEAT
H.pdf
Question 2
A 40-year-old woman is admitted to A+E with a 4-day history of progressive
lower limb and numbness. She is normally fit and well apart from vomiting and
diarrhoea for a few days 9-10 days ago. Which of the following investigations
would help you make the diagnosis?
1. Lumbar puncture
2. Nerve conduction studies
3. CT brain
4. MRI Brain
5. Autoantibodies
Extended answer
This history is consistent with Gullian Barre syndrome (GBS). GBS is an acute
onset usually demyelinating (but sometimes axonal) motor polyneuropathy
with prominent proximal weakness. This tends to be preceded by sensory
symptoms. Typically this starts distally and ascends unlike its variant Miller
Fisher syndrome. Miller Fisher syndrome is associated with GQ1b antibodies
and comprises of ataxia, opthalmoplegia, reduced reflexes with spared power
in the limbs.
Prior to the onset of GBS patients have often experienced in the preceding 12 weeks a (generally) mild illness such as an URTI or D+V. Known triggers
include:
 Campylobacter jejuni
 HIV
 CMV
 EBV
 Mycoplasma species
Investigations include:
 Autoantibodies:
o GM1 is associated with a worse prognosis
o GQ1B associated with Miller-Fisher variant
 CSF
o Usually acellular with raised protein
 Nerve conduction studies
o Usually show demyelination although some patients have
axonal loss
 Serology for campylobacter jejuni
 Pulmonary function tests may show a reduced forced vital capacity
Question 3
You are placed on a neurology ward and are watching the registrar examine
one of the patients. You observe that the patient has bilateral extensor plantar
responses but absent ankle reflexes bilaterally. What is the differential
diagnosis for this finding?
1.
2.
3.
4.
5.
Friedreich’s ataxia
Parkinsons disease
Middle cerebral artery infarct
Motor neuron disease
Wilson’s disease
Extended answer
This is a classical examination question any of the following would be
potential options:
• Subacute combined degeneration of the spinal cord
• motor neurone disease
• Syphilitic tabo-paresis
• Friedreich's ataxia
• A lesion at the conus medullaris or cauda equina lesion
• Multiple sclerosis
• Niacin deficiency
• Stroke + diabetic neuropathy
Question 4
A 23-year-old male economics study is admitted to your hospital whilst he was
on his Easter break from University. He doesn’t really seem his normal self
and has not been sleeping. His father has seen him crying a few times. He
has also been unsteady on his feet and has started to talk like “he is drunk”
and this morning wet himself. What is this most likely diagnosis?





Schizophrenia
Wilson’s disease
Variant CJD
Alcoholism
Multiple sclerosis
Extended Answer
Variant Creutzfeldt-Jakob Disease tends to affect younger patients that the
sporadic form. It is caused by a prion protein, which undergoes
posttranslational modification into beta sheets. These accumulate in neurones
ultimately leading to cell death.
Typical features include those describe in the history a more comprehensive
list is given below:
 Ataxia and dysarthria
 Psychiatric disturbance and insomnia
 Sensory disturbance
 Myoclonus and involuntary movements
 Dysphagia
 Urinary incontinence
 Immobility, mutism and cortical blindness occur in the late stages.
Question 5
You are in the neurology outpatient clinic and meet a 40-year-old man known
to have myotonic dystrophy. Which of the below are typical of the genetic of
this condition?
1.
2.
3.
4.
5.
Autosomal recession inheritance
Autosomal dominant inheritance
Exhibits anticipation
X-linked inheritance
Located on the X chromosome
Extended answer
Myotonic dystrophy is an autosomal dominant disorder, which is caused by a
trinucleotide repeat (AGC) located on chromosome 19. With each successive
generation the number of repeats and thus the severity of the disease may
increase this is termed anticipation.