Neurology and Neurosurgery

Which condition is caused by central inflammation?

Multiple sclerosis

What causes inflammatory conditions in neurology?

Autoimmune demyelination

What type of matter is affected in multiple sclerosis?

The white matter as the tracts are affected

What locations are affected in multiple sclerosis?

The brain, spinal cord, and optic nerves

What is the typical age of onset of multiple sclerosis?

20 to 40

What are the clinical features of multiple sclerosis? (18)

Relapsing and progressing
Sensory symptoms such as numbness and tingling
Upper motor neurone symptoms
Ataxia
Diplopia
Visual symptoms, commonly pain on eye movement and rapid deterioration in central vision
Urinary symptoms
Fatigue
Low mood
Spasticity
Worse symptoms with heat or exercise
Swallowing disorders
Constipation
Pupil defects
Intention tremor
Scanning speech
Reduced cognition and memory
Mania

What is the diagnostic test for multiple sclerosis?

Lumbar puncture - will show CSF oligoclonal bodies (antibodies) which confirm inflammation

How do you manage multiple sclerosis? (4)

Steroids in acute disease
Immunomodulation
Treat the symptoms
PTOT

What is the pathophysiology of Guillain-Barre syndrome?

Peripheral inflammation that affects the longest nerves first

What causes Guillain-Barre syndrome?

A pathogen causes the formation of antibodies which attack the nerve cells

How long is the progressive phase of Guillain-Barre syndrome?

Up to 4 weeks

What are the clinical features of Guillain-Barre syndrome? (8)

Sensory symptoms
Lower motor neurone weakness
Absent reflexes
Face and bulbar symptoms
Pain
Respiratory distress
Autonomic dysfunction
Upper motor neurone dysfunction - late sign

How do you diagnose Guillain-Barre syndrome?

Lumbar puncture will show high protein due to demyelination of the nerve roots that sit in the CSF, especially at the cauda equina

How do you manage Guillain-Barre syndrome?

IV immunoglobulins

What are the clinical features of giant cell arteritis? (5)

Headache
Scalp tenderness
Jaw claudication
Amaurosis fugax
Sudden blindness in one eye

What will the bloods show in giant cell arteritis? (5)

Raised ESR
Raised CRP
Raised platelets
Raised alkaline phosphatase
Reduced Haemoglobin

What are the risk factors for giant cell arteritis? (2)

Polymyalgia
Over 55 years old

What is the management for giant cell arteritis? (2)

Prednisolone
Osteoporosis prophylaxis

What causes myasthenia gravis?

Acetylcholine receptor antibodies

What are the clinical features of myasthenia gravis? (3)

Similar to Guillain-Barre
No sensory symptoms as acetylcholine is only used in neuromuscular junction
Typically starts with the eyes

What is the management for myasthenia gravis? (3)

Steroids
IV immunoglobulins
Acetylcholinesterase inhibitors

Why can encephalitis not be bacterial?

Bacteria will be engulfed and produce an abscess

What are the clinical features of encephalitis? (11)

Altered mental state
Fever
Seizures
Focal neurological signs
Odd behaviour
Reduced consciousness
Cranial nerve lesions
Rash
Lymphadenopathy
Cold sores
Conjunctivitis

How do you manage encephalitis? (2)

Aciclovir
Steroids for raise intracranial pressure

What are the early clinical features of meningitis? (4)

Headache
Leg pain
Cold hands and feet
Abnormal skin colour

What are the later clinical features of meningitis? (10)

Meningism
Photophobia
Kernig's sign
Coma
Sepsis
Focal neurological signs
Oedema
Raised intracranial pressure
Seizures
Petechial rash

How do you manage meningitis? (2)

Antibiotics
Fluid and oxygen if shocked

When does Bells palsy typically present?

During sleep , abruptly

What are the clinical features of Bells palsy? (9)

Complete unilateral facial weakness
Ipsilateral numbness and pain around the ear
Reduced taste
Hypersensitivity to sounds
Unilateral mouth sagging
Food trapped between gum and cheek
Saliva leaking out
Speech difficulty
Failure of eye closure

How do you manage Bells palsy? (3)

Steroids within 6 days of presentation
Antivirals may help due to link with herpes zoster
Protect the eye

What are the complications of Bells palsy? (3)

Incomplete recovery
Synkinesis - blinking causes upturning of the mouth
Misconnection of the parasympathetic fibres causing unilateral lacrimation when eating instead of salivation

Which herpes typically causes encephalitis?

HSV 1

How do you diagnose herpes zoster encephalitis?

CSF PCR

How does herpes simplex cause encephalitis?

There is spread from the cranial nerve ganglia to the frontal and temporal lobes

How do degenerative diseases cause disease?

Proteins become misfolded and this causes other proteins near them to misfold. Normally these would be removed but this does not occur. The proteins aggregate and destroy the cells that they are in

What are the three main neurodegenerative disease?

Alzheimer's disease
Parkinson's disease
Motor neurone disease

What is the pathophysiology of motor neurone disease?

Degeneration of neurones in the motor cortex, cranial nerve nuclei, and anterior horn cells. Affects upper and lower motor neurones

What three things are spared in motor neurone disease?

Sensation
Sphincter function
Eye movements

What are the clinical features of motor neurone disease (32)

Upper and lower motor neurone symptoms at the same time
Brisk reflexes
Wasting
Fasciculations
Memory, language, executive and visuospatial dysfunction
REM sleep behaviour disorder
Periodic limb movements
Detrusor sphincter dysfunction
Social changes
Depression
Hallucinations
Paranoia
Compulsions
Apathy
Disinhibition
Insomnia
Parkinsonism
Ataxia
Apraxia
Erectile dysfunction
Constipation
Sialorrhea
Postural hypotension
Dystonia
Tremor
Myoclonus
Chorea
Seizures
Agraphaesthesia
Astereognosis
Insensitivity to pain

What are the clinical features of bulbar palsy? (3)

Palsy of the tongue, muscles of mastication, and facial muscles

What is the cause of bulbar palsy?

Loss of function of the motor nuclei in the medulla

What is amyotrophic lateral sclerosis?

Combined lower motor neurone wasting and upper motor neurone signs causing weakness

What is progressive muscular atrophy?

Anterior horn cell lesion affecting the distal muscles before proximal

What is the difference between Lewy body dementia and Parkinson's disease?

Parkinson's disease starts with the motor and autonomic symptoms and Lewy body dementia starts with the cognitive and psychiatric symptoms

What are the clinical features of Parkinson's disease and Lewy body dementia? (21)

Memory loss
Paranoia
Compulsions
Insomnia
Dystonia
Tremor
Constipation
Sialorrhea
Visuospatial and executive dysfunction
Non-threatening hallucinations
Reduced REM sleep
REM sleep behaviour disorder
Periodic limb movements
Erectile dysfunction
Detrusor-sphincter dysfunction
Depression
Apathy
Stereotypy
Parkinsonism
Apraxia
Postural hypotension

What causes Alzheimer's disease?

Accumulation of beta-amyloid peptide which is a degradation product of amyloid precursor protein

What is the pathophysiology of Alzheimer's disease? (4)

Progressive neuronal damage
Neurofibrillary tangles
Increased number of senile plaques
Loss of acetylcholine

Which areas of the brain are the most vulnerable to the neuronal loss of Alzheimer's disease? (4)

Hippocampus
Amygdala
Temporal neocortex
Some subcortical nuclei

What are the clinical features of Alzheimer's disease? (11)

Forgetting recent events
Way-finding problems
Word finding issues
Loss of practical skills
Behavioural changes
Reduced cognition
Hallucinations
Delusions
Apathy
Depression
Irritability

How do you manage Alzheimer's disease? (2)

Cholinesterase inhibitors
Memantine for late stage

What causes Wernicke-Korsakoff syndrome?

Thiamine deficiency that is normally linked to alcohol abuse

What are the clinical features of Wernicke-Korsakoff syndrome? (13)

Vision changes
Hallucinations
Polyneuropathy
High heart rate
Confabulation
Fixed pupils
Decreased reflexes
Abnormal eye movements
Loss of muscle coordination
Memory loss - can be profound
Inability to form new memories
Muscle atrophy and weakness
Not orientated to time and place

How do you manage Wernicke-Korsakoff syndrome? (3)

Thiamine
B vitamins
Beware of refeeding syndrome

What are the features of alcohol dependence? (5)

A strong desire to drink
Difficulty controlling alcohol intake
Physiological withdrawal when intake is reduced
Tolerance
Harm due to continued alcohol use

What is the typical distribution of polyneuropathies?

Bilateral, symmetrical, and widespread

What are polyneuropathies?

Generalised disorders of the peripheral nerves or the cranial nerves

What are the motor features of neuropathies? (6)

Often progressive symptoms
Weakness or clumsiness
Difficulty walking
Respiratory difficulties
Wasting - most noticeable in distally
Reduced or absent reflexes

What are the sensory features of neuropathies? (4)

Numbness
Tingling
Burning
Difficulty handling small objects

What are the cranial nerve features of neuropathies? (3)

Swallowing difficulty
Speech difficulty
Diplopia

What does does demyelinating neuropathy affect?

The myelin but not the nerves. Can affect sensory or motor neurones, or both

What are the common causes of demyelinating neuropathies? (3)

Autoimmune
Drugs
Genetic

What is an example of a demyelinating neuropathy?

Guillain-Barre syndrome

What is peripheral neuropathy?

Mononeuropathy where only one nerve is affected

What is the common cause of peripheral neuropathy?

Compression

What are the metabolic causes of neuropathy? (5)

Diabetes mellitus
Renal failure
Hypothyroidism
Hypoglycaemia
Mitochondrial disorders

What are the vascular causes of neuropathy? (3)

Polyarteritis nodosa
Rheumatoid arthritis
Wegener's granulomatosis

Which infections can cause neuropathy? (4)

Leprosy
HIV
Syphilis
Lyme disease

Which vitamin abnormalities can cause neuropathy? (5)

B1 deficiency
B6 deficiency
B12 deficiency
Folate deficiency
B6 excess

Which inherited syndromes can cause neuropathy? (4)

Refsum's
Charcot-Marie-Tooth
Porphyria
Leukodystrophy

Which drugs can cause neuropathies? (7)

Vincristine
Nitrofurantoin
Metronidazole
Phenytoin
Alcohol
Cisplatin
Isoniazid

What are the causes of autonomic neuropathy? (9)

Diabetes mellitus
Amyloid
Guillain-Barre
HIV
Leprosy
Systemic lupus erythematosus
Paraneoplastic syndrome
Toxins
Genetics

What are the signs of autonomic neuropathy? (9)

Postural hypotension
Erectile dysfunction
Ejaculatory failure
Reduced sweating
Constipation
Nocturnal diarrhoea
Urine retention
Horner's syndrome
Holmes-Adie pupil

How do you diagnose autonomic neuropathy?

Autonomic function tests

How does primary autonomic failure happen?

Alone, as part of multisystem atrophy, or with Parkinsonism

What does axonal polyneuropathy affect?

The axons, not the nerves. It affects the amplitude of the signals

What are the length dependent causes of axonal polyneuropathy? (3)

LINKED TO METABOLISM:
Diabetes mellitus
B12 deficiency
Alcohol excess

How do you manage neuropathy? (5)

Treat the cause
PTOT
Steroids
Immunosuppressants
Amitriptyline or gabapentin for pain

What are mononeuropathies?

Lesions of individual nerves

What are the main causes of mononeuropathies? (4)

Trauma
Entrapment
Leprosy
Diabetes mellitus

What is mononeuritis multiplex?

More than two nerves affected by mononeuropathies

Which nerve roots does the ulnar nerve come from?

C7-T1

Where is there weakness and wasting in ulnar nerve mononeuropathy? (4)

Wrist flexors
Interossei
Medial two lumbricals
Hypothenar muscles

Where is there sensory loss in ulnar nerve mononeuropathy?

The medial 1.5 fingers and the ulnar side of the hand

Which function will be weak in ulnar nerve mononeuropathy?

Flexion of the 4th and 5th DIP joints

How do you manage ulnar nerve mononeuropathy? (2)

Rest
Avoiding pressure on the elbow

Which nerve roots does the median nerve come from?

C6-T1

Which muscle will be weak in median nerve mononeuropathy?

Abductor pollicis brevis

Where will the sensory loss be in the median nerve mononeuropathy?

The radial fingers and palm

Where will there be weakness if there is a lesion of the anterior interosseous nerve?

Flexion of the distal phalanx of the thumb and index finger

What are the clinical features of carpal tunnel syndrome? (5)

Achy pain in the hand and arm, often worse at night
Sensory loss in the thumb, index, and middle fingers
Impaired light touch
Impaired two point discrimination
Impaired sweating

How can you manage carpal tunnel syndrome? (3)

Splinting
Local steroid injection
Surgical decompression

Which nerve roots supply the radial nerve?

C5-T1

What is the function of the radial nerve?

Opens the fist

How is the radial nerve damaged?

Compression against the humerus

How do you assess the radial nerve?

Test for wrist and finger drop with the elbow flexed and the arm pronated

Where is there sensory loss in radial nerve damage?

The dorsal aspect of the hand and the root of the thumb

Which nerve roots supply the common perineal nerve?

L4-S2

Where is the common perineal nerve often damaged?

Around the fibular head

What are the clinical features of common perineal nerve damage? (4)

Foot drop
Weak ankle dorsiflexion
Weak ankle eversion
Sensory loss over the dorsum of the foot

Which nerve roots supply the tibial nerve?

S1-S3