Neurology

Question 1

Describe the difference between primary and secondary headache

Answer 1

Primary headaches are those where the headache and its associated features are the disorder (i.e. there is no underlying cause), for example; migraine, tension headache, cluster headache

Secondary headaches are secondary to an underlying cause, for example, subarachnoid haemorrhage, space-occupying lesion, meningitis, temporal arteritis etc.

Question 2

State important features of taking a headache history

Answer 2

Onset (time to maximal symptoms and circumstances at onset)

Severity and Quality of Pain

Location/Radiation of Pain

Presence of Aura/Prodrome

Periodicity (Duration and Frequency)

Associated Features (Photophobia, Nausea, Phonophobia etc)

Age at Onset

Triggers/Exacerbating/Relieving Factors

FHx

Social/Employment Hx

Mediation Hx

Co-Morbid Depression and Sleep Disturbance

Question 3

State red flag signs and symptoms for headache

Answer 3

Age >50yrs

Thunderclap Headache

Focal/Non-Focal Neurological Deficit

Worsening of Symptoms with Posture, Valsalva or Physical Exertion

Early Morning Headaches

Fever

Weight Loss

Seizures

Meningism

Temporal Artery Tenderness/Jaw Claudication

Specific Situations - Cancer, Pregnancy, Post-Partum, HIV, Immunosuppression

Question 4

State features of high and low pressure headaches

Answer 4

• Raised Pressure
  
  • Worse on lying flat, in the morning, on Valsalva and physical exertion
  • Improved on sitting/standing
  • Persistent N/V
  • Optic disc swelling, impaired visual acuity, restricted visual fields
  • 3rd and 6th CN Palsy
  • Caused my mass effect, increased venous pressure, obstruction to CSF flow
• Reduced Pressure
  
  • Worse on sitting/standing
  • Relieved by lying down
  • Results from CSF leak

Question 5

Describe the presentation and management of migraine

Answer 5

Triggers include hormones, weather stress, hunger, sleep disturbance, exertion, alcohol excess, foods

Prodrome in up to 60% of patients up to 48 hours before headache (could include mood disturbance, restlessness, hyperosmia, photophobia, diarrhoea)

Aura reported in up to 30% of cases and is a recurrent, reversible focal neurological symptoms (visual, sensory or motor)

Character often throbbing/pulsatile, moderate to severe, unilateral in 60%, gradual onset, lasting 4-72 hours

Associated symptoms include N/V, photophobia, phonophobia, osmophobia, mood disturbance, diarrhoea

Lifestyle management includes avoidance of triggers, reduction of caffeine/alcohol intake, encourage regular meals/sleep patterns

Acute management with simple analgesia, triptans and anti-emetics

Prophylaxis with beta-blockers, TCAs and anti-epileptics

Question 6

Describe the assessment and investigation of thunderclap headache

Answer 6

‘abrupt onset of severe headache which reaches maximal intensity in <5 mins and lasts >1 hour’

worst headache of my life/like being hit over the head

Causes include SAH, ICH, Arterial Dissection, Cerebral Venous Sinus Thrombosis, Bacterial Meningitis, Primary Headache

Investigate with Bloods, ECG, Urgent CT Head and Lumbar Puncture (after 12 hours)

Question 7

Describe the pathophysiology of stroke

Answer 7

After stoppage of blood flow to the brain, neurons depolarise within minutes (causing instant onset of early symptoms) and irreversible brain tissue death occurs within 12 hours

Therefore, the sooner the blood flow is restored the more brain death is prevented

Question 8

Describe the clinical presentation of stroke/TIA

Answer 8

Visual Loss

Weakness

Slurred Speach

Ataxia

Aphasia

Question 9

Describe the management of stroke

Answer 9

• Ischaemic Stroke
  
  • IV Thrombolysis (within 4.5h) +/- Thrombectomy (within 6-8h)
  • Aspirin
  • Stroke Unit
  • Hemicraniectomy
• Haemorrhagic Stroke
  
  • Aggressive BP Control
  • Stroke Unit
  • Neurosurgical Evacuation

Question 10

Define ‘seizure’

Answer 10

‘a transient occurrence of signs and/or symptoms due to abnormal, excessive or synchronous neuronal activity in the brain’

Question 11

Define ‘epilepsy’

Answer 11

‘a disorder of neuronal activity in the brain defined as two or more unprovoked seizures, or one unprovoked seizure with a lesion in the CNS that increases the probability of a second unprovoked seizure’

Question 12

Outline the different types of seizure

Answer 12

Focal seizures begin in one part of the brain, and may spread to become secondary generalised

Generalised involves multiple areas of the brain or spread from one area to both sides of the brain

Absence seizures are common in young children

Tonic-Clonic seizures involve LOC and a short tonic phase (muscle suddenly tense and patient falls) and a longer clonic phase (rapid convulsions)

Question 13

Describe investigations for epilepsy

Answer 13

Diagnosis is largely based on a detailed history from the patient and eyewitness

ECG to check for underlying cardiac problems

EEG is not generally diagnostic but may assist in classification

MRI

Question 14

Describe the management of epilepsy

Answer 14

1. Reduce Pre-Synaptic Excitability
   
   1. Voltage Gated Na⁺ Channel Antagonists
      
      1. Carbamazepine
      2. Lamotrigine
   2. Voltage Gated K⁺ Channel Agonist
      
      1. Retigabine
2. Stops Neurotransmitter Release
   
   1. SV2A Vesicle Antagonist
      
      1. Levetiracetam
   2. Voltage Gated Ca²⁺ Channel Antagonist
      
      1. Pregabalin
      2. Gabapentin
3. GABA-ergic System Agonists
   
   1. GABA Metabolism Inhibitor
      
      1. Valproate
      2. Vigabatrin
   2. GABA Transporter Antagonists
      
      1. Tiagabine
4. Reduces Post-Synaptic Excitability
   
   1. GABA Receptor Agonist
      
      1. Benzodiazepines
   2. AMPA and NMDA Receptor Antagonist

Usual treatment for Focal is Lamotrigine, Carbamazepine or Levetiracetam and for Generalised it is Valproate, Levetiracetam and Lamotrigine

Question 15

Describe the management of Status Epilepticus

Answer 15

‘a single clinical seizure lasting more than 30 minutes or repeated seizures over a period of time greater than 30 minutes without intervening recovery of consciousness’

MEDICAL EMERGENCY

May cause profound systemic/neurological damage if left untreated

Immediate management includes assessing and securing the airway, administering oxygen and assessing pulse, BP and RR

If seizures continue for more than 5 minutes, the seizure should be treated with IV benzodiazepines

If seizures continue, the patient may need sedated and intubated

Question 16

State causes of loss of consciousness

Answer 16

Epilepsy (Seizures)

Syncope

Stroke/TIA

Drugs

Hypoglycaemia

Alcohol

Hypoxia

Question 17

Describe the pathogenesis of subarachnoid haemorrhage

Answer 17

Most SAH are caused by aneurysms which form under haemodynamic stress

Extensive inflammatory and immunological reactions are common in unruptured intracranial aneurysm and may be related to aneurysm formation and rupture

A small number (15-20%) are due to other causes. e.g. Arterio-Venous Malformation or Neoplasia

Question 18

Describe the presentation of subarachnoid haemorrhage

Answer 18

Sudden Onset Thunderclap Headache

LOC

Seizures

Visual, Speech and Limb Disturbance

Sentinel Headache

Photophobia

Meningism

Subhyaloid Haemorrhages

Vitreous Haemorrhages

Pulmonary Oedema

Question 19

Describe the investigation of subarachnoid haemorrhage

Answer 19

CT - Confirms diagnosis and may give clues to aetiology - Very accurate

LP - For presence of xanthochromia (bilirubin or OxyHb) in the CSF - Conducted at least 12 hours after onset of symptoms

CT/MRI Angiography

Digital Subtraction Angiography

Question 20

Describe the management of subarachnoid haemorrhage

Answer 20

Bed Rest

Fluids (2.5-3 Litres of Normal Saline)

Anti-Embolic Stockings

Nimodipine

Analgesia

Surgical Clipping (10-15%)

Endovascular Repair (Coils, Stents and Glue) (80-85%)

Question 21

Describe the complications of subarachnoid haemorrhage

Answer 21

• Rehaemorrhage
  
  • 5-10% incidence in the first 72 hours
  • Immediate repair reduces risk
• Delayed Ischaemia
  
  • At Days 3-10
  • Progressive deterioration in LOC associated with new deficit
  • Managed with fluid resus, nimodipine, inotropes, angioplasty
• Hydrocephalus
• Hyponatraemia
• ECG Changes
• LRTI
• PE
• UTI
• Seizures
  
  • 1-7% of patients
• DVT
  
  • SAH induces a prothrombotic state

Question 22

Describe the Glasgow Coma Scale

Answer 22

Question 23

Describe the clinical presentation of radial nerve mononeuropathy

Answer 23

Wrist and Finger Drop

Usually Painless

Motor Weakness; Wrist/Finger Extension and Elbow Flexion in Mid-Pronation

Sensory Change: Radial Part of Dorsum of Hand

Question 24

Describe the clinical presentation of ulner nerve mononeuropathy

Answer 24

Weak Grip

Usually Painless

Motor Weakness: Index Finger/Pinkie Abduction, Wrist Flexion and Thumb Adduction

Sensory Change: Medial 1.5 Fingers

Question 25

Describe the clinical presentation of median nerve mononeuropathy

Answer 25

Hx of Intermittent Nocturnal Pain, Numbness and Tingling Weak Grip +ve Tinel's Sign/Phalen's Test Motor Weakness: MCP/Thumb Flexion, Thumb Opposition and Thumb Abduction Sensory Change: Lateral 3.5 Fingers on Palm and Tips of Those Fingers on Dorsum

Question 26

Describe the clinical presentation of femoral nerve mononeuropathy

Answer 26

Weakness of Quadriceps Weak Hip Flexion Numbness in Medial Shin Motor Weakness: Knee Extension, Hip Flexion, Hip Adduction Sensory Change: Medial Lower Limb

Question 27

Describe the clinical presentation of common peroneal nerve mononeuropathy

Answer 27

Acute Onset Foot Drop and Sensory Disturbance Usually Painless Motor Weakness: Ankle Dorsiflexion, Great Toe Extension Sensory Change: Shin and Dorsum of Foot

Question 28

Describe the epidemiology of multiple sclerosis

Answer 28

UK prevalence of 1.2/1000 More prevalent in Scotland and in northern latitudes Twice as common in women than men

Question 29

Describe the pathophysiology of multiple sclerosis

Answer 29

Plaques of demyelinating lesions are the cardinal features of MS Plaques can occur anywhere in the CNS white matter, however, they have a predilection for distinct sites: optic nerves, the periventricular region, the corpus callosum, the brainstem and its cerebellar connections and the cervical cord Grey matter of the cortex and the sub-pial meninges are also affected in the early stages however peripheral nerves are not Acute relapses are caused by focal inflammation causing myelin damage and conduction block and recovery follows as inflammation subsides and re-myelination occurs In severe damage, secondary permanent axonal destruction occurs which is the pathological basis of the progressive disability in progressive forms of MS

Question 30

Describe the clinical presentation of multiple sclerosis

Answer 30

Optic Neuritis Spinal Cord Lesions (Paraparesis, Difficulty Walking, Limb Numbness, Tingling) Brainstem Demyelination (Diplopia, Vertigo, Facial Numbness/Weakness, Dysarthria, Dysphagia) Visual Change, Sensory Symptoms, Clumsy Hand or Limb, Unsteadiness, Ataxia, Urinary Symptoms, Pain, Fatigue, Spasticity, Depression, Sexual Dysfunction, Temperature Sensitivity

Question 31

Describe the investigation of multiple sclerosis

Answer 31

MRI Brain and Spinal Cord - To investigate for demyelinating lesions Bloods to exclude other inflammatory conditions such as Sarcoidosis and SLE Visual Evoked Response LP - IgG Oligoclonal Bands in 85-90%

Question 32

Describe the main subtypes of multiple sclerosis

Answer 32

* Relapsing Remitting * Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission * Primary Progressive * Steady increase in disability without attacks * Secondary Progressive * Initial relapsing-remitting pattern that suddenly begins to decline without periods of remission * Progressive Relapsing * Steady decline from onset with super-imposed attacks

Question 33

Describe the pathology of Parkinson's Disease

Answer 33

Loss of dopaminergic neurones within substantia nigra PD manifests clinically after loss of approximately 50% of dopaminergic neurons Surviving neurones contain Lewy Bodies (aggregates of misfolded proteins in the cytoplasm) Progresses from Stage 1/2 (Medulla/Pons), to Stage 3/4 (Midbrain, Substantia Nigra, Pars Compacta - Parkinsonism) to Stage 5/6 (Neocortex - PD Dementia)

Question 34

Describe the clinical features of Parkinson's Disease

Answer 34

Bradykinesia, Muscular Rigidity, Postural Instability, 5-6Hz Rest Tremor Dementia, Depression, Anxiety Constipation, Urgency, Nocturia, Erectile Dysfunction, Excessive Salivation, Postural Hypotension, Sweating REM Sleep Behaviour Disorder, Restless Leg Syndrome, Daytime Somnolence Reduced Olfactory Function, Fatigue, Pain and Sensory Symptoms

Question 35

Describe the differential diagnosis of Parkinson's Disease

Answer 35

Benign Tremor Disorders (e.g. Essential Tremor) Dementia with Lewy Bodies Vascular Parkinsonism Parkinson Plus Disorders Drug Induced Parkinsonism/Tremor

Question 36

Describe the pharmacological management of Parkinson's Disease

Answer 36

* L-Dopa * Taken up by dopaminergic neurones and decarboxylated to dopamine within presynaptic terminals * Adverse effects include nausea, vomiting and postural hypotension (peripheral) and confusion and hallucinations (central) * Prescribed with a dopa-carboxylase inhibitor * Dopamine Agonists * e.g. Ropinirole, Pramipexole * Acts directly on post-synaptic striatal dopamine receptors (D2) * Longer half-life, lower efficacy but fewer motor complications than L-Dopa * MAO-B Inhibitors * e.g. Selegiline, Rasagiline * Prevents dopamine breakdown by binding irreversible to monoamine oxidase * COMT Inhibitors * e.g. Entacapone, Tolcapone * Inhibiting COMT results in longer L-Dopa half-life/duration of action

Question 37

Describe Myasthenia Gravis

Answer 37

Autoimmune disorder with auto-antibodies to acetylcholine receptor at post-synaptic neuromuscular junction Association with other autoimmune disorders May be associated with thymic hyperplasia or thymoma Affects young women in 20’s and older men in 70’s Causes fatigable weakness of ocular, bulbar, neck, respiratory and/or limb muscles Investigated with antibodies to AChR (present in 85% of cases) and abnormal single fibre EMG Managed with Pyridostigmine (anti-acetylcholine esterase) and immunosuppressive therapies (e.g. steroids and intravenous Immunoglobulin)

Question 38

Describe the pathogenesis and clinical presentation of length-dependent axonal neuropathy

Answer 38

Most common cause in the western world in Diabetes Mellitus Severely damaged axons degenerate distally Within a week the nerve becomes electrically inert Muscle fibres supplied by damaged motor nerves atrophy EMG records show fibrillation potentials Undamaged motor fibres sprout to supply more muscle fibres Demyelination: Demyelination of peripheral nerve fibres initially leaves the axon intact The result is blockage or slowing of conduction Pressure or entrapment neuropathies are primarily caused by demyelination Inflammatory processes e.g. Guillain-Barre are caused by demyelination Presents with tingling, numbness and in the peripheries, typically in a gloves and stocking pattern

Question 39

Describe the pathology of common dementias

Answer 39

Alzheimer - Generalised atrophy, beta-amyloid plaques and neurofibrillary tangles Vascular - Strokes, lacunar infarcts and white matter lesions Lewy Body - Generalised atrophy with lew bodies in the cortex and midbrain Frontotemporal - Frontotemporal atrophy and pick cells/bodies in the cortex

Question 40

Describe the clinical presentation of common dementias

Answer 40

Alzheimers - Memory loss, aggression, language deficit, impaired visuospatial Vascular - Focal neurological deficits, evidence of vascular disease Lewy Body - Fluctuating cognition, visual hallucinations, shuffling gait, increased tone, tremors Frontotemporal - Disinhibition, socially inappropriate behaviours, poor judgement and cognitive function

Question 41

Describe the investigation of common dementia syndromes

Answer 41

All patients should have a routine dementia screen, including FBC, U&Es, LFTs, TFTs, Glucose, VitB12 and Folate Cognitive function should be assessed using the Mini Mental State Exam (

Question 42

Describe the management of Multiple Sclerosis

Answer 42

Education from an MS Nurse Specialist Immunisations (avoid live vaccines if on disease-modifying drugs) Early management of infections Symptomatic treatment (e.g. pain, spasticity and urinary features) Physiotherapy/Occupational Therapy Short courses of steroids such as IV Methylprednisolone for 3 days (or high dose oral steroids) are used for severe relapses