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1
Q

Examples of disorders affecting brainstem

A

Tumours
Inflammatory disorders e.g. MS
Metabolic e.g. central pontine myelonecrosis (Na+)
Trauma
Spontaneous aneurysm e.g. AVM, aneurysm
Infarction e.g. vertebral artery dissection
Infection e.g. cerebellar abscess from ear

2
Q

Criteria from brainstem death

A
Pupils
Corneal reflex
Caloric vestibular reflex
Cough reflex
Gag reflex
Respirations
Response to pain
3
Q

Different types of sensory fibres (large and small)

A

Aα and Aβ (large myelinated fibres)

Aδ and C (thin, small fibres)

4
Q

Difference between Aα and Aβ (large myelinated) sensory fibres

A

Αα are transmitting signals regarding proprioception and Aβ about light touch, pressure and vibration.

5
Q

Difference and similarity between Aδ and C fibers small, thin sensory fibres

A

Aδ are myelinated and C unmyelinated
Both transmit signals regarding pain
Additionally, Aδ signals are about cold sensation and C signals are about warm sensation.

6
Q

What is result of demyelinating neuropathies

A

Myelin is destroyed

Leads to slower conduction velocities

7
Q

What is difference between mononeuropathy and polyneuropathy

A
Mononeuropathy = problem with one nerve
Polyneuropathy = problem with multiple nerves
8
Q

Examples of common mononeuropathies

A

Carpal tunnel syndrome
Ulnar neuropathy
Peroneal neuropathy
Cranial mononeuropathies e.g. III or VII cranial nerve palsy

9
Q

What nerve is compressed in carpal tunnel syndrome

A

Median nerve

10
Q

Cause of ulnar neuropathy

A

Entrapment at the cubital tunnel

11
Q

Cause of peroneal neuropathy

A

Entrapment at the fibular head

12
Q

Cause of cranial neuropathies such as III or VII cranial nerve palsy

A

Idiopathic
Immune mediated
Ischaemic

13
Q

Subtypes of large fibre neuropathies

A

Axonal

Demyelinating

14
Q

Define peripheral neuropathy

A

Any disorder of the peripheral nervous system

Can be acute or chronic

15
Q

Types of causes of chronic demyelinating neuropathies

A

Genetic

Inflammatory

16
Q

What is CIDP

A

Chronic inflammatory demyelinating polyneuropathy (CIDP)
A rare neurological disorder in which there is inflammation of nerve roots and peripheral nerves and destruction of the fatty protective covering (myelin sheath) over the nerves

17
Q

What is ataxia

A

Poor balance

18
Q

Causes of ataxia

A

Sensory - loss of proprioception
OR
Cerebellar

19
Q

In sensory ataxia, what makes it worse

A

Eyes closed or when dark

20
Q

Motor symptoms of peripheral neuropathies

A
Muscle cramps
Weakness
Fasciculations - muscle twitches
Atrophy
High arched feet (pes cavus)
21
Q

3 categories of axonal peripheral neuropathies

A
Symmetrical sensorimotor (or sensory neuropathies)
Sensory gangliopathies (or asymmetrical sensory)
Asymmetrical sensorimotor neuropathies (or mononeuritis multiplex)
22
Q

What is the most common type of peripheral neuropathy

A

Symmetrical sensori-motor PNs

23
Q

Presentation of symmetrical sensori-motor peripheral neuropathies

A

Initially sensory symptoms affecting the toes and fingers (Length-dependent pattern) and eventually motor symptoms again in a length dependent pattern.
Long fibres are affected first

24
Q

Example of asymmetrical sensory neuropathy

A

Sensory gangliopathy

25
Q

In sensory gangliopathy, what structure is affected

A

Dorsal root ganglia

26
Q

Characteristics of Sensory gangliopathy (asymmetrical sensory PN)

A

Purely sensory and characteristically presents with patch distribution of symptoms.
Dorsal root ganglia are affected.
Relatively uncommon overall, but common as a paraneoplastic manifestation or in the context of Sjogren’s disease and gluten sensitivity

27
Q

What is Sjogren’s disease

A

A condition that mainly affects the glands that produce saliva and tears, causing a dry mouth and/or eyes

28
Q

Example of asymmetrical sensorimotor PN

A

Mononeuritis multiplex

Occurs in the context of vasculitis

29
Q

Characteristics of asymmetrical sensorimotor PN

A

Multiple nerve involvement
Can randomly affect any nerve without a specific pattern
Very common but painful and occurs in the context of Vasculitis

30
Q

Diagnostic approach to PN (first steps)

A

History
Clinical examination
Neurophysiological examination i.e. NCS/QST

31
Q

Diagnostic approach to PN: Clinical examinations done

A

Reduced or absent tendon reflexes
Sensory deficit
Weakness - muscle atrophies

32
Q

Diagnostic approach to PN: Neurophysiological examinations

A

Nerve conduction studies - determine type of neuropathy

33
Q

How would you diagnose a demyelinating PN in nerve conduction study

A

Reduced conduction velocities and/or conduction block in sensory and motor nerves with minimal or no abnormalities in the SNAP and CMAPs

34
Q

How would you diagnose mononeuritis multiplex PN in nerve conduction study

A

SNAPs and CMAPs of affected nerves are reduced

Asymmetrical

35
Q

How would you diagnose SMA (Spinal Muscular Atrophy) PN in nerve conduction study

A

Symmetrical type of sensorimotor pn

Reduced SNAPs in beginning and eventually CMAPs

36
Q

Nerve conduction study: difference between demyelinating and axonal

A
Demyelinating = slow conduction velocities
Axonal = reduced amplitudes of the potentials
37
Q

Causes of axonal PN

A

Associated with systemic diseases
Inflammatory – immune mediated (mainly acute)
Infectious (i.e. hepatitis, HIV, Lyme)
Ischemic (i.e. vasculitis)
Metabolic (i.e. Fabry’s, porphyria)
Hereditary (CMT, HLPP)
Toxins (pharmaceuticals, environmental toxins, B6)

38
Q

Systemic diseases associated with axonal PN

A
Diabetes
Vitamin deficiency  (commonly B12)
Gluten sensitivity / coeliac disease
Chronic renal disease
Excessive alcohol drinking
Hypothyroidism
Amyloidosis
Connective tissue disease
Paraproteinemia
Paraneoplastic
Critical illness polyneuropathy
39
Q

What is meant by chronic idioapthic axonal polyneuropathy

A

Develops over at least 6 months (chronic)
No aetiology can be identified despite extensive and appropriate investigations (Idiopathic)
Axons are affected, most commonly in proportion to their length (Axonal)

40
Q

Examples of immune mediated chronic demyelinating neuropathy

A

CIDP (chronic inflammatory demyelinating polyneuropathy)

Multifocal motor neuropathy (MMN)

41
Q

Examples of genetic chronic demyelinating neuropathy

A

Charcot Marie Tooth disease

Hereditary sensory and autonomic neuropathies

42
Q

Example of an acute polyneuropathy

A

Guillain Barre syndrome

43
Q

What is Guillain Barre syndrome

A

Autoimmune disorder in which the immune system attacks healthy nerve cells in your peripheral nervous system (PNS). This leads to weakness, numbness, and tingling, and can eventually cause paralysis.

44
Q

Clinical presentation of Guillain Barre syndrome

A

Progressive ascending limb weakness (usually symmetrical) that peaks at 4 weeks, usually lost reflexes, some paraesthesia (‘walking on rubber’). Little to no muscle wasting.
Infection (e.g. GI) may precede the disease (campylobacter)

45
Q

What type of neuropathy is Guillain Barre syndrome

A

Demyelination and axonal degradation -> progressive ascending neuropathy

46
Q

What is Miller Fisher syndrome

A

Variant of Guillain Barre syndrome that affects cranial nerves of the eye muscles (opthalmoplegia and ataxia)

47
Q

Aetiology of Guillain Barre syndrome

A

Most have preceding infection, usually of respiratory and GI. Potentially antibodies to infection also attack peripheral nerves. Usually caused by campylobacter jejuni.
Can be CMV

48
Q

Pathophysiology of Guillain Barre syndrome

A

Demyelination and axonal degeneration -> acute, ascending and progressive neuropathy
Probably antibodies from the infection succumb to molecular mimicry and attack myelination

49
Q

Diagnosis of Guillain Barre syndrome

A
Clinical. 
Antibody screen. 
Nerve conduction studies. 
CSF: raised protein level with a normal white cell count. 
Monitor FVC for respiratory involvement
50
Q

Treatment of Guillain Barre syndrome

A

IV immunoglobulin.
Plasma exchange possible.
Ventilate if necessary.

51
Q

Complications of Guillain Barre syndrome

A

Life threatening respiratory failure

Lower Motor Neurone lesion (sequelae)

52
Q

Treatment of neuropathy

A
Aim to identify any reversible cause and stop progression if possible
Symptomatic treatment:
Pain 
Cramps (Quinine)
Balance (physiotherapy/walking aids)
53
Q

Treatment of pain symptoms in neuropathy (medical)

A

Amitryptilline
Gabapentin
Pregabalin

54
Q

A 72 year old patient with a 15 year history of diabetes complains of burning sensation and tingling in his both feet for 3 years. Over the last 12 months the symptoms have progressed up to the level of the knees.

On examination he has reduced tendon reflexes in the upper limbs and the knees and absent tendon reflexes in the ankles. He has reduced vibration sensation up to the knees.

What is the most likely diagnosis?

A

Peripheral neuropathy of axonal type (length dependent)

Diabetes
Burning sensation and tingling
Reduced tendon reflexes
Absent tendon reflexes in the ankles
Reduced vibration sensation up to the knees
55
Q

A 32 year old woman with a 5 day history of progressive weakness and tingling which started from the lower limbs but now has affected the upper limbs too.

On examination she has absent tendon reflexes.

Her medical history is unremarkable, however she reports D&V about 3 weeks ago.

What is the most likely diagnosis?

A

Acute polyneuropathy
Guillain Barre syndrome

5 day
started from the lower limbs
absent tendon reflexes
D and V about 3 weeks ago

56
Q

A 52 year old man reports patchy sensory loss and complains of poor balance, symptoms which are getting gradually worse.

Ten year earlier he was diagnosed with coeliac disease.

On examination he has mild sensory ataxia, reduced reflexes and patchy loss of pinprick and vibration sensation.

What is the most likely diagnosis?

A

Sensory ganglionopathy

Patchy sensory loss
Poor balance
Coeliac disease