Neuro

Question 1

Multi-system atrophy?

Answer 1

o Rare condition where the neurons of multiple systems in the brain degenerate, affects the basal ganglia as well as multiple other areas.
o The degeneration of the basal ganglia which can occur in this disease process present with Parkinonism
o The degeneration in other areas lead to conditions such as Autonomic dysfunction (post hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia)

Question 2

• Dementia with Lewy bodies:

Answer 2

o This is a type of dementia that is associated with features of parkinsonism.
o Leads to progressive cognitive decline.
o Associated visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness.

Question 3

What do you need to give with levodopa?

Answer 3

peripheral decarboxylase inhibitors- prevent it being broken down before it reaches the brain

Question 4

COMT inhibitors?

Answer 4

o Used in PD
The main example of these is entacapone.
o These are inhibitors of catechol-o-methyltransferase, an enzyme that metabolises levodopa in the body and the brain.
o Entacapone is taken with levodopa (and a decarboxylase inhibitor) to slow breakdown of the levodopa in the brain. Increasing the lengths of affect it has

Question 5

Progressive Supranuclear palsy

Answer 5

o	Aka- steele-richardson-olszewski syndrome 
o	Features;
	Impairement of the vertical gaze (difficulty reading or climbing stairs)
	Parkinsonism 
	Falls 
	Slurring of speech
	Cognitivie impairement 
o	Management:
	Poor response to levodopa

Question 6

What chromosome and gene is affected in huntington’s?

Answer 6

ch4

o MUTATION on ch4 leading to a repeated CAG sequence Huntingtin gene (HTT) (Trinucleotide repeat)

Question 7

First line generalised seizures?

Answer 7

Sodium valproate, usually second line is lamotrigine

Question 8

EEG finding for west syndrome?

Answer 8

hypsarrithmia
Hypsarrhythmia is very chaotic and disorganized brain electrical activity with no recognizable pattern, whereas a normal brain electrical activity shows clear separation between each signal and visible pattern. It is an abnormal interictal pattern, consisting of high amplitude and irregular waves and spikes in a background of chaotic and disorganized activity seen on electroencephalogram (EEG), and frequently encountered in infants diagnosed with infantile spasms, although it can be found in other conditions

Question 9

Lennoux-Gastaut syndrome:

Answer 9

o Need the 3 findings to diagnose:
 Atypical absences, falls and jerks (multiple seizure types)
 90% have a moderate to severe handicap
 EEG- abnormally slow basic rhythm interrupted with Slow spike and wave pattern.

Question 10

How is status epilepticus defined?

Answer 10

• Defined as seizures lasting more than 5 mins or more than 3 seziures in an hour.

Question 11

EEG: 3Hz generalized, symmetrical

Answer 11

Absence seizures
3 Hz spike-and-wave discharge
(petit mal)

Question 12

Ix after seizure?

Answer 12

Blood tests including serum calcium, and an ECG (rhythm, conduction abnormalities, QT interval) are necessary in most patients following an episode of loss of consciousness

MRI in most after first seizure- particularly focal onset and elderly

Question 13

What are the 4 meds used for neuropathic pain?

Answer 13

Amitriptyline is a tricyclic antidepressant
Duloxetine is an SNRI antidepressant
Gabapentin is an anticonvulsant
Pregabalin is an anticonvulsant

Question 14

Typical features of neuropathic pain?

Answer 14

Burning
Tingling
Pins and needles
Electric shocks
Loss of sensation to touch of the affected area

Question 15

Features of complex regional pain syndrome?

Answer 15

Occurs after local injury/ surgery to an area, within this area:

Features
progressive, disproportionate symptoms to the original injury/surgery
allodynia
temperature and skin colour changes
oedema and sweating
motor dysfunction

Manage like other neuropathic pain

Question 16

Causes of CNVII LMN (excluding bells and RHS)?

Answer 16

•	Infection:
o	Otitis media
o	Malignant Otitis externa
o	HIV
o	Lyme disease
•	Systemic disease:
o	DM 
o	Sarcoidosis 
o	Leukaemia
o	MS 
o	GBS
•	Tumours:
o	Acoustic neuroma
o	Parotid tumours
o	Cholesteatomas
•	Trauma:
o	Direct nerve damage
o	Damage in parotid surgery 
o	Base of skull fracture.

Question 17

Mx of ramsay hunt syndrome?

Answer 17

o Treat with Prednisolone and Aciclovir

Question 18

Ab in myasthenia Gravis?

Answer 18

o Acetylcholine receptor Ab (Ach-R) Ab (85%)
o Muscle-specific kinase (MuSK) Ab (10%)
o LRP4 (less than 5%)

Question 19

Typical features of myasthenia gravis?

Answer 19

o Diploplia- Extraocular muscle weakness causing double vision
o Ptosis- Eyelid weakness leading to drooping of the eyelid
o Weakness in the facial movements
o Issues swallowing
o Fatigue in the jaw when chewing
o Slurred speech
o Progressive weakness in repetitive movements

Question 20

The edrophonium test?

Answer 20

• The edrophonium test can be helpful if there is a doubt re diagnosis
o Patients are given an IV dose of edrophonium (or neostigmine).
o Normally these cholinesterase enzymes in the neuromuscular joints break down Ach.
o Edrophonium stops this process to stop the breakdown of Ach.
o This leads to raise in Ach at the NM joint.
o It briefly relieves the weakness- diagnosis of MG

Question 21

Treatment of myasthenia gravis?

Answer 21

Treatment options:
• Reversible acetylcholinesterase inhibitors (usually pyridostigmine or neostigmine) increases the amount of Ach in the neuromuscular joints and improve the symptoms
• Immunosuppression (e.g. prednisolone or azathioprine) supresses the Ab
• Thymectomy can improve symptoms even in patients without a thymoma

Monoclonal Ab:
• Rituximab: Is a monoclonal Ab that targers B cells and reduces the production of Ab. (on NHS if other meds don’t work)
• Eculizumab: A monoclonal Ab that targets complement protein C5. Could prevent the complement activation and destruction of the Ach receptors. Ongoing research and debate if it is good enough to use on NHS.

Question 22

Myasthenic crisis management?

Answer 22

o Immunomodulatory therapies such as IV Ig and plasma exchange
• Patients may need non-invasive ventilation with BiPAP or full intubation and ventilation.

Question 23

What is a myasthenic crisis?

Answer 23

worsening of symptoms, often triggered by another illness such as a RTI

Question 24

EMG findings for MG?

Answer 24

Decrement and increment. In myasthenia gravis, a
characteristic decrement in evoked muscle action
potential follows repetitive motor nerve stimulation.
The reverse occurs, i.e. increment, following repetitive
stimulation in LEMS,

Question 25

Causes of GBS?

Answer 25

o Campylobacter Jejuni
o CMV
o Epstein Barr virus

Question 26

Criteria for diagnosis neurofibromatosis type 1?

Answer 26

o There are clear diagnostic criteria for NF1 based on the classical features of the condition. There must be at least 2 of the 7 features to indicate a diagnosis. You can remember the mnemonic CRABBING.
 C- Café au lait spots (6 or more) measuring >5 mm in children or >15 mm in adults
 R- Relative with the NF1 gene
 A- Axillary or inguinal freckles
 BB- Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
 I- Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris
 N- Neurofibromas (2 or more) or 1 plexiform neurofibroma
 G- Glioma of the optic nerve

Question 27

NFT1 gene?

Answer 27

o The neurofibromatosis type 1 gene is found on chromosome 17.
o It codes for a protein called neurofibromin, which is a tumour suppressor protein.
o Inheritance of this mutation follow an AD pattern.

Question 28

Complications of NFT1?

Answer 28

• Complications:
o Migraines
o Epilepsy
o Renal artery stenosis causing hypertension
o Learning and behavioural problems (e.g. ADHD)
o Scoliosis of the spine
o Vision loss (secondary to optic nerve glioma)
o Malignant peripheral sheath tumours
o GI stromal tumours
o Brain tumours
o Spinal cord tumours with associated neurology (paraplegia)
o Increased risk of cancer (e.g. breast cancer)
o Leukaemia

Question 29

Neurofibromatosis Type 2:

Answer 29

• The neurofibromatosis type 2 gene is found on chromosome 22 .
• It codes for a protein called merlin, which is a tumour suppressor protein particularly important in schwann cells.
• Mutations in this gene lead to the development of schwannomas (benign nerve sheath tumours of the Schwann cells).
• Inheritance is autosomal dominant.
• NFT2 is most associated with acoustic neuromas.
• These are tumours of the auditory nerve innervating the ear.
• Symptoms are:
o Hearing loss
o Tinnitus
o Balance issues
• Schwannomas can also develop in the brain and spinal cord with symptoms based on the location of the lesion.
• Surgery can be used to resect them but there is a risk of permanent nerve damage.

Question 30

What is the classical presentation of idiopathic normal pressure hydrocephalus?

Answer 30

Reversible cause of dementia in elderly patients:

• urinary incontinence
• dementia and bradyphrenia
• gait abnormality (may be similar to Parkinson’s disease)

Question 31

Imaging findings in normal pressure hydrocephalus?

Answer 31

hydrocephalus with an enlarged fourth ventricle

in addition to the ventriculomegaly there is typically an absence of substantial sulcal atrophy

Question 32

Management of NPH?

Answer 32

• ventriculoperitoneal shunting
  
  • around 10% of patients who have shunts experience -significant complications such as seizures, infection and intracerebral haemorrhages

Question 33

2 reversible causes of dementia picked up on imaging (MRI/CT)?

Answer 33

NPH, SDH

Question 34

What EEG changes would you find in CJD?

Answer 34

• EEG- biphasic, high amplitude sharp waves - unique

Question 35

Myopathic EMG?

Answer 35

Myopathy. Short-duration spiky polyphasic muscle
action potentials are seen. Spontaneous fibrillation is
occasionally recorded.
 Myotonic discharges. A characteristic high-frequency
whine is heard.
 Decrement and increment. In myasthenia gravis, a
characteristic decrement in evoked muscle action
potential follows repetitive motor nerve stimulation.
The reverse occurs, i.e. increment, following repetitive
stimulation in LEMS, page 1152.
 In denervation, profuse fibrillation potentials are seen.