Neurology

Question 1

What are the meninges layers?

Answer 1

1. Extra -dural space = minimal/narrow, associated meningeal vessels
2. Dura mater = outermost, tough, fibrous, forms folds, encloses dural venous sinuses
3. Sub-dural space = narrow, contains bridging veins
4. Arachnoid mater = soft, thin, loose
5. Subarachnoid space = contains CSF + cerebral vessels
6. Pia mater = innermost, adhered to the surface of the brain and contributes to BBB

Question 2

Recap - Outline the main roles of the
a) Frontal lobe
b) Temporal Lobe
c) Parietal Lobe
d) occipital lobe

Answer 2

Frontal - decision making, movement, executive function, personality.

Temporal - hearing (primary auditory cortex), memory and language, smell, facial recognition

Parietal - Sensory info

Occipital lobe - Vision

Question 3

Recap - What are the main responsibilities for the
a) Brainstem
b) Cerebellum

Answer 3

brainstem - controls Heart and breathing rate, Blood pressure and GI function, as well as consciousness

Cerebellum - Muscle coordination, and balance

Question 4

Recap - what are the two arteries that supply the brain?

Answer 4

Internal carotid
Vertebral arteries

Question 5

What does the internal carotid artery branch off to supply?

Answer 5

branches off to create the Anterior cerebral artery, as well as posterior communicating artery to join the circle of Willis

After this the ICA continues on as the Middle cerebral artery, which supplies the lateral portions of the cerebrum.

Question 6

After entering the cranium through the foramen magnum, what branches does the vertebral artery give off? What do the 2 vertebral arteries then go on to do?

Answer 6

Give off Spinal arteries, supply the entire length of spine
Gives off The Posterior Inferior cerebellar artery - supplies cerebellum
also gives off a menigeal branch

But after this two vertebral arteries converge to form the basilar artery

Question 7

What arteries branch off the basilar artery?

Answer 7

Superior cerebellar artery (SCA)
Anterior inferior cerebellar artery (AICA) - Both to supply the cerebellum
The Pontine arteries

Question 8

What are the layers of the cerebellum?

Answer 8

1. moleucular layer
2. purkinje cell layer (most important as only ouput)
3. granule cell layer
4. white matter

Question 9

What does the posterior cerebral artery go on to supply? What is it a branch of?

Answer 9

Supplies occipital lobe, posteromedial temporal lobes, midbrain, thalamus,

It is the terminal branch of the basilar arteries,

Question 10

What does the anterior cerebral artery supply?

Answer 10

ANTERIOR CEREBRAL ARTERY (supplies and runs over Corpus Callosum and supplies Medial aspects of Hemispheres (anteromedial aspects of the cerebrum)

Question 11

What is the cause of an extra-dural haemorrhage?

Answer 11

Traumatic – typically caused by bleeding from the meningeal arteries as a result of skull fracture that tears the dura

Middle meningeal runs close to the pterion

Question 12

What is the weakest part of the skull?

Answer 12

Pterion point

Question 13

What is the clinical presentation of extra-dural haemorrhage?

Answer 13

Patient may have extensive traumatic injuries

In other cases, patient sustains a head injury but appears to be OK for a while (‘lucid period’)

Bleeding into the extradural space > rapid rise in intracranial pressure (ICP) > brain is compressed

Headache, drowsiness, rapid neurological deterioration

Death if not treated rapidly (neurosurgical emergency

Question 14

What is the imaging look like for an Extra-dural haemorrhage?

Answer 14

Acute (fresh) bleed appears hyperdense on CT (bright white)

Convex; does not conform to surface of the brain as bleeding is limited by dural attachments to the skull (lemon shape)

Compression of the brain – midline shift (falx cerebri, lateral ventricles)

Skull fracture may be seen

Question 15

What is the cause of a sub-dural haemorrhage?

Answer 15

Usually caused by trauma – typically a fall leading to bleeding from dural bridging veins

Low pressure bleeding

Gradual rise in ICP (over several weeks or months)

Most likely in patients with brain atrophy (elderly, dementia, history of excess alcohol intake) – bridging veins

Question 16

WHat is the clincal presentation for sub dural haemorrhage?

Answer 16

Typical picture is of gradual cognitive deterioration

May be a history of a fall, maybe not

Patient may have old bruising on their head (or elsewhere) suggesting frequent / recent falls

Question 17

What is the appearance of imaging for sub dural?

Answer 17

(banana shape)
Chronic (old) bleed appears hypodense on CT (dark)

Concave; conforms to surface of the brain as bleeding is not limited by dural attachments

Compression of the brain – signs include midline shift (falx cerebri, lateral ventricles)

Question 18

What are the investigations for a SDH?

Answer 18

Immediate CT head

Question 19

WHat is the cause of a sub-arachnoid haemorrhage?

Answer 19

Usually spontaneous from rupture of an aneurysm on a cerebral artery

Can be traumatic, but this is less common

Question 20

What is the management for a SDH?

Answer 20

Drainage:
- small SDH are drained via a burr hole washout a small tube called
- large SDH requires a craniotomy which is when part of the skull bone is removed

Question 21

What are the complications a the raised intercranial pressure in a SDH?

Answer 21

Supratentorial herniation: cerebrum is pushed against the skull or the tentorium, can compress the arteries that nourish the brain leading to an ischaemic stroke

Infratentorial herniation: cerebellum is pushed against the brainstem, can compress the vital area in the brainstem that control consciousness, respiration, and heart rate

Question 22

WHat is the clinical presentation for subarachnoid?

Answer 22

Typically present with sudden onset severe ‘worst-ever headache’ (‘thunderclap’)

Patient may:
be conscious
have reduced GCS
be unconscious (poorer prognosis)

Can be rapidly fatal

Question 23

What is the appearacne of a sub-arachnoid bleed?

Answer 23

Acute (fresh) bleed is hyperdense on CT (bright white)

Blood seen in fissures and cisterns +/- ventricles
(not a large mass like SD and ED)

Blood in the SA space:
irritates the meninges
irritates cerebral vessels and causes vasospasm > hypoxic injury
may track back into the ventricular system > hydrocephalus

(looks like spider)

Question 24

What is the cause of an intra-cerebral haemorrhage?

Answer 24

• Spontaneous due to aneurysm or vessel rupture
• Small perforating vessels prone to rupture, especially if hypertensive
• Clinical presentation determined by the size of the bleed and brain region affected

E.g.
Coma
Weakness (facial, limbs)
Seizure

Question 25

What is the appearance of a intra-cerebral bleed on imaging?

Answer 25

Acute (fresh) bleed is hyperdense on CT (bright white) Blood seen in the substance of the brain Mass effect seen if large (e.g. midline shift)

Question 26

What is an epidural haemorrhage?

Answer 26

Bleeding above the dura matter (same as extra dural haemorrhage)

Question 27

Who is an EDH most common in?

Answer 27

They usually occur in young adults

Question 28

What are the symptoms of a EDH?

Answer 28

Reduced GCS: loss of consciousness after the trauma due to concussion - There might be a lucid interval after initial trauma if there is a slower bleed. This is followed by rapid decline. Headaches Vomiting Confusion Seizures Pupil dilation if bleeding continues

Question 29

What are the differentials for a EDH?

Answer 29

Epilepsy Carotid dissection Carbon monoxide poisoning Subdural haematoma Subarachnoid haemorrhage

Question 30

What is the management for a EDH

Answer 30

Clot evacuation Craniotomy: part of the skull bone is removed in order to remove accumulated blood below. Followed by ligation of the vessel.

Question 31

Which part of the brain do the middle, anterior and posterior arteries supply?

Answer 31

Anterior = front and midline Middle = lateral lobes Posterior = posterior part of brain

Question 32

What is Broca's and Wernicke's area?

Answer 32

Broca - motor control of speech Wernicke - understanding of speech

Question 33

What artery and location of the primary motor cortex control upper and lower limb?

Answer 33

Lower limb - represented the medial surface of the cerebral hemisphere = anterior cerebral artery territory Upper limb and face represented on the lateral surface = middle cerebral artery territory

Question 34

What is the difference between CNS and PNS?

Answer 34

CNS = brain and spinal cord PNS = cranial nerves, spinal nerves, ganglia

Question 35

Where are motor neurones located in the spinal cord?

Answer 35

Ventral horn = motor neurones

Question 36

Where are sensory neurones located in the spinal cord?

Answer 36

Dorsal root ganglion

Question 37

What nerve fibres are carried in the spinal cord?

Answer 37

Sympathetic - smooth muscle, sweat glands Somatic motor Somatic sensory

Question 38

What are the main ascending sensory tracts?

Answer 38

Dorsal columns Spinothalamic tract

Question 39

What is the main descending motor tract?

Answer 39

Lateral corticospinal tract

Question 40

What is the route of the lateral corticospinal tracts? And consequence of lesion?

Answer 40

Descending motor control decussate in the medulla enter the contralateral lateral CST UMN synapses with LMN in the ventral grey hron axon leaves in the spinal nerve lesions in cord = ipsilateral weakness

Question 41

What sensations does the dorsal column pathway control?

Answer 41

Touch, proprioception, vibration Cuneate fasciculus – info from the UL Gracile fasciculus – info from the LL

Question 42

What sensations does the spinoithalamic tract control?

Answer 42

pain and temperature - ventrolateral

Question 43

What is the route of the dorsal column pathway? And consequence of lesion?

Answer 43

1.Sensory primary axons ascend in the ipsilateral dorsal columns (cuneate or gracile fasciculus 2. Synapse with 2nd neuron in the cuneate (UL) or gracile (LL) nucleus 3. Axons decussate in the medulla then ascend 4. Synapse with 3rd neuron in thalamus 5. Axons project to the somatosensory cortex Lesions in the cord: ipsilateral loss of / impaired fine touch and proprioception

Question 44

What is the route of the spinothalamic tract? And consequence of lesion?

Answer 44

(travel up on opposite side) Sensory primary axon synapses with 2nd neuron in the grey horn Axons decussate then ascend Synapse with 3rd neuron in thalamus Axons project to the somatosensory cortex Lesions in the cord: contralateral loss of / impaired pain and temp sensation

Question 45

What is the difference between a dermatome and myotome?

Answer 45

Dermatome = area of skin supplied by a single spinal nerve Myotome = muscles supplied by a single spinal nerve

Question 46

What is the function of cranial nerve 1?

Answer 46

Olfactory - sense of smell - receptor in nasal cavity - travel from cribriform plate > olfactory bulb > tracts > temporal lobe

Question 47

What is the function of cranial nerve 2?

Answer 47

optic nerve - Fibres travel from the retina to the primary visual cortex (calcarine sulcus, medial aspect of occipital lobe)

Question 48

What is the function of cranial nerve 3?

Answer 48

Oculomotor nerve - innervates eye muscles except from superior oblique + lateral rectus - parasympathetic fibres = constrict the pupil

Question 49

What is the function of the 4th nerve?

Answer 49

Trochlear - Superior oblique muscle (test by looking medially and down)

Question 50

What is the function of 6th cranial nerve?

Answer 50

Abducens Lateral rectus = abducts the eye

Question 51

What is the function of 5th cranial nerve?

Answer 51

Trigeminal Sensory and motor; 3 divisions: Ophthalmic V1 > sensory Maxillary V2 > sensory Mandibular V3 > sensory plus motor to the muscles of mastication

Question 52

What is the function of 7th cranial nerve?

Answer 52

Facial Taste anterior 2/3 tongue Muscles of facial expression: Muscles of the upper face bilaterally innervated by the motor cortex Muscles of lower face contralaterally innervated by the motor cortex Parasympathetics to the lacrimal, submandibular and sublingual salivary glands (stimulates secretion)

Question 53

What is the function of the 8th cranial nerve?

Answer 53

Vestibulocochlear - hearing + balance

Question 54

WHat is the function of the 9th cranial nerve?

Answer 54

Glossopharyngeal Taste posterior 1/3 of the tongue General sensation: (touch, temp, pain) Pharynx, posterior 1/3 of the tongue Parasympathetics to parotid gland

Question 55

What is the function of the 10th cranial nerve?

Answer 55

Vagus General sensation - pharynx, larynx, oesophagus. Motor - muscles of the soft palate, pharynx and larynx – vital for swallowing and speech Parasympathetics: thoracic and abdominal viscera

Question 56

What is the function of the 11th cranial nerve?

Answer 56

Accessory Motor to sternocleidomastoid and trapezius

Question 57

What is the function of cranial nerve 12?

Answer 57

Hypoglossal Motor to the tongue muscles. - Nerve lesion > ipsilateral tongue muscles are paralysed (left deviation = left hypoglossal lesion)

Question 58

What is Amaurosis Fugax?

Answer 58

A classical syndrome of painless short-lived monocular blindness. It is a term usually reserved for transient visual loss of ischaemic origin.

Question 59

What is the aetiology of Amaurosis Fugax?

Answer 59

- temporary reduction in the retinal, ophthalmic or ciliary blood flow leading to temporary retinal hypoxia - transient obstruction from emboli of ophthalmic artery - can occur in TIA (other causes are giant cell arteritis and central retinal artery occlusion)

Question 60

What is an ischaemic stroke?

Answer 60

An episode of neurological dysfunction caused by focal cerebral, spinal, or retinal infarction (cell death due to lack of blood supply)

Question 61

What is a stroke due to intracerebral haemorrhage?

Answer 61

Rapidly developing clinical signs of neurological dysfunction attributable to a focal collection of blood within the brain parenchyma or ventricular system not caused by trauma.

Question 62

What is a stroke due to subarachnoid haemorrhage?

Answer 62

Rapidly developing signs of neurological dysfunction and/or headache because of bleeding into the subarachnoid space (the space between the arachnoid membrane and the pia mater of the brain or spinal cord), which is not caused by trauma.

Question 63

What symptoms does a stroke in the dominant hemisphere cause?

Answer 63

(usually left hemisphere - right handed) Language dysfunction Expressive dysphasia Receptive dysphasia Dyslexia Dysgraphia

Question 64

What symptoms does a stroke in a non-dominant hemisphere cause?

Answer 64

Non-dominant hemisphere (usually RIGHT hemisphere) Anosognosia - Neglect of paralysed limb (unaware of side of body) - Denial of weakness Visuospatial dysfunction - Geographical agnosia - Dressing apraxia - Constructional apraxia

Question 65

What are the symptoms of a anterior cerebral artery stroke?

Answer 65

Contralateral hemiparesis and sensory loss with upper limbs > lower limbs Homonymous hemianopia Aphasia: if the affecting dominant hemisphere 95% of right handed people this is the left side Hemineglect syndrome if affecting the non-dominant hemisphere patients won’t be aware of one side

Question 66

What are the symptoms of posterior cerebral artery strokes?

Answer 66

Contralateral homonymous hemianopia with macular sparing contralateral loss of pain and temp due to spinothalamic damage Unsteadiness Visual disturbance Slurred speech Headache (MC in posterior) Vomiting Others e.g. memory loss, confusion

Question 67

What are the symptoms of a vertebrobasilar artery stroke?

Answer 67

Cerebellar signs Reduced consciousness Quadriplegia (affects all 4 limbs + torso) or hemiplegia (one side of body)

Question 68

What is Weber’s syndrome and what are the symptoms of it?

Answer 68

It is a midbrain infarct that leads to oculomotor palsy and contralateral hemiplegia (lack of control in one side of body)

Question 69

What are the symptoms of lateral medullary syndrome (posterior inferior cerebellar artery occlusion)

Answer 69

ischaemia in lateral part of medulla oblongata **Ipsilateral** facial loss of pain and temperature Horner’s syndrome: miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face) Ipsilateral cerebellar signs Contralateral loss of pain and temperature

Question 70

What is used to classify strokes and how does it do it?

Answer 70

The Bamford classification and it categorises strokes based on the area of circulation affected

Question 71

What is used to identify strokes in the community?

Answer 71

F- Face A- Arm S- Speech T- Time (this is a stupid one because it is not a symptom just there to make the word fast)

Question 72

What is used to identify strokes in hospital?

Answer 72

Recognition of Stroke in the Emergency Room (ROSIER) scale.

Question 73

What are the criteria for the ROSIER scale?

Answer 73

Loss of consciousness Seizure activity New, acute onset of: - Asymmetric facial/arm/leg weakness - Speech disturbance - Visual filed defect

Question 74

When would a stroke be possible using the ROSIER scale and what would happen as result?

Answer 74

A stroke is possible if they have any of the criteria and hypoglycaemia has been excluded WOULD REQUIRE URGENT NON-CONTRAST CT - Aspirin 300mg stat (after the CT)

Question 75

What are key features of stroke symptoms?

Answer 75

Onset = abrupt (secs) Nature of symptoms = Focal (localised to an area of the brain) Quality = negative Maximal deficit = at the start (symptoms are worse)

Question 76

What are differential diagnosis for stroke?

Answer 76

Epileptic seizure = history + symptoms (unconscious) Space occupying lesion (e.g. subdural, tumour, arteriovenous malformation) = gradual onset, headache, confusion, drowsiness, deterioration Infection (MC in elderly - can unmask deficit from previous stroke) = gradual onset, fever Metabolic (e.g. hyponatraemia, hypoglycaemia, hypercalcaemia, uraemia) = confusion, reduced consciousness, ataxia Multiple sclerosis = young, sub acute progression Functional neurological disorder (FND) = inconsistencies, 'give way' weakness Migraine = history, previous attacks Hepatic encephalopathy

Question 77

What investigations are done for a stroke?

Answer 77

1st line: CT head (rule out anything serious like a tumour or bleed - often normal after a stroke) GOLD: MRI brain with diffusion weighted imaging (can see ischaemia when a CT can't) - Blood tests (metabolites, electrolytes) - ECG +- 72 hour tape - carotid doppler - ECHO - CT or MR angiogram

Question 78

What are the 4 categories for ischaemic stroke?

Answer 78

- large vessel disease (50%) - small vessel disease - cardioembolic - cryptogenic/ rarities

Question 79

What are the 2 categories of haemorrhagic stroke?

Answer 79

- primary intracerebral haemorrhage - subarachnoid haemorrhage

Question 80

What causes ischaemic stroke in large vessel iscahemia?

Answer 80

- atherosclerosis - blood vessel occlusion, thrombus - embolism

Question 81

What causes small vessel ischaemic stroke?

Answer 81

- small deep perforator arteries blocked - caused by high blood pressure, diabetes, smoking, age - in situ microatheroma or lipohyalinosis

Question 82

What are the causes of cardioembolic stroke?

Answer 82

Cardiogenic embolus: Mural thrombus Atrial fibrillation Endocarditis (vegetation can embolise) Atrial septal defect (

Question 83

What is the most common cause of stroke in young people?

Answer 83

Dissection (carotid or vertebral) Risk factors: - trauma or cervical manipulation - vigorous physical activity (eg weightlifting) - vasculopathy (fibromuscular dysplasia, Marfan’s) - sympathomimetic drug abuse (can also be spontaneous)

Question 84

What is a primary intracerebral haemorrhage stroke and its causes?

Answer 84

leakage of blood directly into brain tissues due to - hypetension - amyloidosis - arteriovenous malformation - aneurysm rupture

Question 85

What is a secondary intracerebral haemorrhage?

Answer 85

NOT classed as a stroke - due to trauma, warfarin or bleeding into a tumour - but can cause similar symptoms as primary

Question 86

What are the risk factors for developing an intracerebral stroke?

Answer 86

Head injury Hypertension Aneurysm Brain tumour Anticoagulant

Question 87

What are the presentations of an intracerebral haemorrhage?

Answer 87

Sudden headache is a key feature Weakness Seizure Vomiting Reduced consciousness

Question 88

What does a 'thunder clap headache signify?

Answer 88

Subarachnoid haemorrhage

Question 89

What are the investigations for a intracerebral haemorrhage?

Answer 89

CT/MRI to confirm size and location of the haemorrhage Check FBC and clotting Angiography to visualise the exact location of the haemorrhage

Question 90

What is the management for a intracerebral haemorrhage?

Answer 90

Consider ICU and intubation and ventilation if there is reduced consciousness Correct any clotting abnormalities Correct severe hypertension but avoid hypotension Drugs to relieve intercranial pressure mannitol

Question 91

What are the surgeries that can be performed for an intracerebral haemorrhage?

Answer 91

Craniotomy = part of the skull bone is removed to drain any blood and relieve pressure Stereotactic aspiration = aspirate off blood and relieve intracranial pressure guided by a CT scanner. Good for bleeding that is located deeper in the brain

Question 92

What is the treatment for a ischaemic stroke?

Answer 92

Antiplatelets: aspirin given as soon as possible once haemorrhagic stroke is excluded Thrombolysis: alteplase (tissue plasminogen activator)- given if to re-establish blood flow is <4.5 hours of symptom onset Thrombectomy must score > 5 on NIH Stroke Scale/Score (NIHSS) and pre-stroke functional status < 3 on the modified Rankin scale

Question 93

What should be performed before thrombectomy?

Answer 93

CT angiogram (CTA): identifies arterial occlusion

Question 94

What are secondary stroke preventions?

Answer 94

- Antiplatelets agents = Clopidogrel (Warfarin/DOAC superior to aspirin in AF/mural thrombus) - statins = stabilis atherosclerotic plaque - long term BP lowering - carotid intervention (remove blockage to prevent it happening again)

Question 95

What can cause Subarachnoid Haemorrhage?

Answer 95

Trauma is a key factor Atraumatic cases are referred to as spontaneous SAH

Question 96

What is the most common cause of a spontaneous SAH?

Answer 96

Berry aneurysm- they account for 80% of cases. Arise at points of bifurcation within the circle of Willis; the junction between the anterior communicating and anterior cerebral artery They are associated with PKD, coarctation of the aorta, and connective tissue disorders (Marfan)

Question 97

What are the risk factors for having a SAH?

Answer 97

Cocaine use Sickle cell anaemia Connective tissue disorders Neurofibromatosis: tumours form on your nerve tissues PKD Alcohol excess

Question 98

What can occur as a result of a subarachnoid haemorrhage?

Answer 98

Blood vessels that are bathing in a pool of blood can start to intermittently vasoconstrict (vasospasm). If this occurs in the circle of Willis it will reduce the supply of blood flow to the brain causing further injury Over time blood in the subarachnoid space can irritate the meninges and cause inflammation which leads to scarring of the surrounding tissue. The scar tissue can obstruct the normal outflow of CSF causing fluid to build up leading to hydrocephalous

Question 99

What are the signs of a SAH?

Answer 99

3rd nerve palsy- if the aneurysm occurs in posterior communicating artery 6th nerve palsy a non-specific sign which indicates raised intercranial pressure Reduced GCS

Question 100

What are the symptoms of a SAH?

Answer 100

Thunderclap headache during strenuous activity or sex. It’s like being hit really hard on the back of the head Neck stiffness photophobia Vison changes

Question 101

What are the initial investigations for SAH?

Answer 101

FBC Serum glucose Clotting screening Urgent non-contrast CT of the head. - Blood will cause ** hyperattenuation (this means becoming more dense on CT will show as white)** in the subarachnoid space

Question 102

What tests would you perform if CT is negative but a SAH is still suspeccted?

Answer 102

Lumbar puncture: will show RBCs or xanthochromia (yellow pigmentation due to degradation of haemoglobin to bilirubin - so need to wait till 12 hours after SAH)

Question 103

What is the management to prevent vasospasm?

Answer 103

Nimodipine is a CCB and prevents vasospasms

Question 104

What is the management to stop the bleeding?

Answer 104

First line: Endovascular coiling of the aneurysm Second line: surgical clipping via craniotomy

Question 105

What are the complications of a SAH?

Answer 105

Rebleeding 22% risk at one month Vasospasm: accounts for 23% of deaths; at highest risk for the first 2-3 weeks after SAH; treated with (induced) hypertension,hypervolemia andhaemodilution (triple-H therapy). Hydrocephalus: acutely managed with external ventricular drain (CSF drainage into an external bag) or a long-term ventriculoperitoneal shunt, if required Seizures: seizure-prophylaxis is often administered (e.g. Keppra) Hyponatraemia: commonly due to syndrome of inappropriate antidiuretic hormone secretion (SIADH)

Question 106

What is the treatment for a haemorrhagic stroke?

Answer 106

- Admit to neurocritical care: patients will need intensive monitoring - If features of raised intracranial pressure: consider intubation with hyperventilation, head elevation (30°) and IV mannitol (reduce ICP) - Surgical intervention:decompression may be needed

Question 107

What are the risk factors for developing a stroke

Answer 107

Hypertension Smoking AF Vasculitis Medication e.g. hormone replacement therapy

Question 108

If the anterior cerebral artery is affected in a stroke where in the body will be affected?

Answer 108

Feet and legs

Question 109

If the middle cerebral artery is affected in a stroke where in the body will be affected?

Answer 109

Hands and arms Face Language centres in the dominant hemisphere

Question 110

If the posterior cerebral artery is affected in a stroke where in the body will be affected?

Answer 110

The visual cortex will be affected meaning the patient won’t be able to see properly

Question 111

What is a TIA?

Answer 111

A transient episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischaemia without acute infarction. It usually resolves within 24 hours

Question 112

What is the management for a TIA?

Answer 112

First line is antiplatelet initially with aspirin 300mg Carotid endarterectomy: surgery to remove blockage of >70% on doppler Manage cardiovascular risk with atorvastatin etc

Question 113

What is a crescendo TIA?

Answer 113

Where there are two or more TIAs within a week. It carries a high risk of a stroke

Question 114

How many people who have a TIA will go on to have a stroke

Answer 114

10% within 3 months

Question 115

What are the two categories a haemorrhagic stroke can be split in to?

Answer 115

Intracerebral where the bleeding occurs within the cerebrum Subarachnoid when bleeding occurs between the pia and arachnoid matter

Question 116

What is meningitis?

Answer 116

Meningitis refers to inflammation of the pia and arachnoid mater. Micro-organisms infect the cerebrospinal fluid (CSF).

Question 117

What are the 3 main features of 'meningism'?

Answer 117

Stiffness of the neck Photophobia Severe headache

Question 118

What other symptoms would present with bacterial or viral meningitis?

Answer 118

Fever Feel unwell Rash (characteristically haemorrhagic in meningococcal meningitis)

Question 119

What are the viral causes of meningitis?

Answer 119

80% viral - Enteroviruses (including Echo virus, Coxsackie virus) - Herpes simplex virus - Mumps virus - varicella zoster virus - Lymphocytic chorio meningitis virus And historically, Poliovirus (also an enterovirus)

Question 120

What are the main bacterial causes of meningitis for each age group?

Answer 120

Neonates = E.coli, Group B strep, Listeria monocytogenes Infants = N.meningitis + H.influenza, Strep pneumoniae Adults = N.meningitis + S.pneumoniae Elderly = S. pneumoniae + N.meningitis + listeris monocytogenes

Question 121

What are the causes of fungal meningitis?

Answer 121

Cryptococcus neoformans Candida

Question 122

What are some risk factors for developing meningitis?

Answer 122

Immunocompromised: such as being in the extremes of age, infection (HIV), and medication (Chemotherapy) Listeria monocytogenes M. Tuberculosis Non-immunised: at risk ofH. influenza, pneumococcal and meningococcal meningitis Crowded environments: students living in halls of residence are a commonly affected demographic

Question 123

What is encephalitis?

Answer 123

Refers to inflammation of the cerebral cortex

Question 124

What are the main clinical symptoms of encephalitis?

Answer 124

Lethargy + fatigue Decreased level of consciousness Fever Focal neurology Occasionally there may be fits

Question 125

What are the viral causes of encephalitis?

Answer 125

Herpes simplex virus Varicella zoster virus Parvoviruses HIV Mumps virus Measles virus

Question 126

Where are the signs of encephalitis?

Answer 126

Pyrexia (fever) Reduced GCS Aphasia Hemiparesis Cerebellar signs --

Question 127

What are some behavioural changes that occur in encephalitis?

Answer 127

Memory disturbance Psychotic behaviour Withdrawal or change in personality

Question 128

What are some investigations for encephalitis?

Answer 128

Throat swab HIV serology MRI of head will show evidence of inflammation will be normal in 1/3 of cases Lumbar puncture and CSF investigation including a PCR for HSV

Question 129

What are some differentials for encephalitis?

Answer 129

Meningitis Encephalopathy Status epilepticus CNS vasculitis

Question 130

What is the treatment for encephalitis?

Answer 130

Aciclovir = should be given in all cases where it is suspected Ganciclovirmay = be preferred in other herpesvirus infections, such as HHV-6

Question 131

What lab investigations are done for enterovirus?

Answer 131

Nose + throat swabs - plated out onto blood and chocolate agar PCR Stool

Question 132

What lab investigations are done for S.pneumoniae and N.meningitidis?

Answer 132

Blood cultures PCR

Question 133

What CSF cell count results would signify a bacterial cause of infection?

Answer 133

- a neutrophil predominant leucocytosis - a raised protein (from dying bacteria) - reduced CSF glucose to serum glucose ratio (metabolically active bacteria, use up glucose as a source of energy)

Question 134

What are the two tests to look for meningeal infection?

Answer 134

Kernigs Test Brudzinski’s Test

Question 135

What is Kernigs Test?

Answer 135

Involves patient lying on their back and flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed. This causes the meninges to stretch A positive test will be where there is spinal pain or resistance to the movement

Question 136

What is Brudzinski’s Test?

Answer 136

Involves lying a patient on their back and gently using your hands to lift their head and neck off the bed and flexing their chin to their chest A positive test is when a patient involuntarily flexes their hips and knees

Question 137

What is another classic sign of meningococcus meningitis?

Answer 137

non-blanching rash - that everybody worries about as it indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages. Other causes of bacterial meningitis do not cause this rash

Question 138

What is the empirical antibiotic therapy used against bacterial causes of CNS infection in hospital?

Answer 138

IV cefotaxime or ceftriaxone

Question 139

What medication should be prescribed alongside antibiotics for meningitis treatment?

Answer 139

IV dexamethasone (corticosteroids) - reduce risk of long term neurological complications from some causes of meningitis

Question 140

what medication should be given in primary care for meningitis?

Answer 140

IM benzylpenicillin then admit the patient ASAP!!

Question 141

What is the management for meningitis?

Answer 141

1. Assess GCS 2. blood culture 3. broad spectrum antibiotics = Ceftriaxone or cefotaxime 4. Steroids = IV dexamethasone 5. lumbar puncture = definitive diagnosis

Question 142

What are the contraindications for a lumbar puncture?

Answer 142

Abnormal clotting (platelets/coagulation) Petechial rash Raised intracranial pressure GCS <9

Question 143

Where is a lumbar puncture usually taken from?

Answer 143

Between L3/L4 Since spinal cord ends L1/2

Question 144

When would you do a CT head before a lumbar puncture in meningitis?

Answer 144

Aged > 60 Immunocompromised History of CNS disease Seizures <1 week GCS <14 Focal neurological signs Papilloedema Atypical history

Question 145

What are close contacts of patients with meningitis offered?

Answer 145

Notified Antibiotic prophylaxis = single dose of ciprofloxacin

Question 146

What is the CSF count and culture characteristic for a viral meningitis infection?

Answer 146

Predominant lymphocyte response Only moderately raised protein CSF glucose more than 50% of the level of serum glucose

Question 147

What antimicrobial agent is used to treat a viral meningitis or encephalitis infection?

Answer 147

IV aciclovir

Question 148

How does an infection get into the brain?

Answer 148

1. extra-cranial infection e.g. nasopharynx, ear, sinuses 2. neurosurgical complications e.g. post op, infected shunts, trauma 3. via blood stream e.g. bacteraemic

Question 149

What is the pathophysiology of meningitis?

Answer 149

1. bacteria in blood 2. bacteria enter CSF and can be isolated from immune cells due to BBB 3. replication 4. blood vessels become leaky 5. so WBCs can enter the CSF, meninges and brain 6. meningeal inflammation +/- brain swelling

Question 150

What types of meningitis are acute and which are more commonly chronic?

Answer 150

Bacterial and viral meningitis are usually acute Fungal is more chronic

Question 151

What will a lumbar puncture show for bacterial meningitis?

Answer 151

high pressure cloudy high WBCs neutrophils low glucose high protein

Question 152

What will a lumbar puncture show for viral meningitis?

Answer 152

normal or elevated pressure clear increased lymphocytes high glucose low protein

Question 153

what will a lumbar puncture results show for fungal/TB meningitis?

Answer 153

elevated pressure cloudy/fibrin web Increased lymphocytes low glucose high protein

Question 154

What are the common metastases for secondary brain tumours?

Answer 154

Lung breast melanoma renal cell GI

Question 155

What are the symptoms of secondary brain tumours?

Answer 155

- **Headache:** worse in morning, when coughing or bending - **Focal neurological signs** - **Ataxia** - **Fits** - **Nausea** - **Vomiting** - **Papilloedema** (on fundoscopy)

Question 156

What are the investigations for brain tumours?

Answer 156

CT/MRI

Question 157

What is the management for brain tumours?

Answer 157

- steroids = dexamethasone (reduce cerebral oedema) - Stereotactic radiotherapy/chemotherapy - surgery if <75 years - palliative care

Question 158

What are mononeuropathies?

Answer 158

Lesions of individual peripheral or cranial nerves - causes are usually local e.g. trauma or entrapment (tumour)

Question 159

What are the features of a median nerve lesion in the wrist and anterior interosseous?

Answer 159

C6-T1 = nerve of precision grip + LOAF Wrist = carpal tunnel syndrome, weakness of abductor pollicis brevis; sensory loss over the radial 3 1/2 fingers and palm Anterior interosseous nerve lesions = weakness of flexion of the distal phalanx of the thumb and index finger Proximal lesions = may show combined effects

Question 160

What is the presentation of an ulnar nerve palsy/lesion?

Answer 160

C7-T1 Claw hand (4th + 5th fingers 'claw' up) - sensory loss over medial 1 1/2 fingers and ulnar side of the hand - wasting of hypothenar eminence

Question 161

What is the treatment for a ulnar nerve palsy?

Answer 161

Splint for elbow simple analgesia rest + avoid pressure on nerve

Question 162

What is the presentation of a radial nerve palsy?

Answer 162

C5-T1 Wrist drop Muscles involved = BEAST Brachioradialis Extensors Abductor pollicis longus Supinator Triceps

Question 163

What is the treatment for a radial nerve palsy?

Answer 163

Splint and simple analgesia

Question 164

What are the causes of brachial plexus lesion?

Answer 164

- **Trauma** - **Radiotherapy** - **Prolonged wearing of heavy rucksack** - **Cervical rib** - **Thoracic outlet compression** - **Neuralgic amyotrophy**

Question 165

What are the signs of a brachial plexus lesion?

Answer 165

Pain/ paraesthesia and weakness in the affected arm in a variable distribution

Question 166

What are the causes of a phrenic nerve lesion?

Answer 166

C3,4,5 keeps the diaphragm alive - **Lung cancer** - **TB** - **Paraneoplastic syndrome** - **Myeloma** - **Thymoma** - **Cervical spondylosis/ trauma** - **Thoracic surgery** - **Infections e.g. HZV, HIV, Lyme disease** - **Muscular dystrophy**

Question 167

What are the signs of a phrenic nerve lesion?

Answer 167

- **Orthopnoea** - **Raised hemi-diaphragm** on chest x-ray

Question 168

What nerve causes a 'foot drop'?

Answer 168

Common peroneal nerve L4-S1

Question 169

What are the effects of a tibeal nerve lesion?

Answer 169

L4-S3 - inability to stand on tiptoes, invert foot and flex the toes - sensory loss over the sole

Question 170

What is mononeuritis mulitplex? And causes?

Answer 170

- Involvement of two or more peripheral nerves - Causes tend to be systemic: diabetes mellitus, connective tissue disorders, vasculitis, sarcoidosis, amyloidosis, leprosy - Electromyography helps define the anatomic site of lesion

Question 171

What are the signs of cranial nerve 1 lesion?

Answer 171

- Reduced taste and smell - Ammonia taste remains as it stimulates the pain fibres carried in the trigeminal nerve

Question 172

What are the causes of a cranial nerve lesion?

Answer 172

- Trauma - Frontal lobe tumour - Meningitis - fracture - Raised ICP - Diabetes - hypertension

Question 173

What are the signs of cranial nerve 2 lesion?

Answer 173

1. Visual field defects: - Start as small areas of visual loss (scotomas). - Monocular severe sight impairment: lesions of one eye or optic nerve - Bilateral severe sight impairment - Bitemporal hemianopia - Homonymous hemianopia: loss of the same half (left or right) of the visual field of both eyes, on the opposite side to the lesion (eg, a right side lesion causes loss of the left side of the visual field of both eyes). 2. Pupillary abnormalities 3. Optic neuritis - Pain on moving the eye - Loss of central vision - Afferent pupillary defect - Papilloedema 4. Optic atrophy - Pale optic discs - Reduced acuity

Question 174

What are the signs of cranial nerve 3 lesion?

Answer 174

- Fixed dilated pupil which doesn't accommodate - Ptosis - Complete internal ophthalmoplegia - Unopposed lateral rectus causes outward deviation of the eye - If the ocular sympathetic fibres are also affected behind the orbit, the pupil will be fixed but not dilated.

Question 175

What are the signs of cranial nerve 4 lesion?

Answer 175

- Diplopia due to weakness of downward and inward eye movement (pure vertical diplopia) - Compensation by tilting the head away from the affected side

Question 176

What are the signs of cranial nerve 5 lesion?

Answer 176

- Reduced sensation or dysasthesia over the affected area - Weakness of jaw clenching and side-to-side movement. - If LMN lesion, the jaw deviates to the weak side when the mouth is opened - May be fasciculation of temporalis and masseter

Question 177

What are the causes of sensory CN5 lesions?

Answer 177

Trigeminal neuralgia HSV Nasopharyngeal carcinoma

Question 178

What are the signs of cranial nerve 6 lesion?

Answer 178

- Inability to look laterally - Eye is deviated medially because of unopposed action of the medial rectus muscle

Question 179

What are the signs of cranial nerve 7 lesion?

Answer 179

- Facial weakness - LMN lesion: - Forehead is paralysed - the final common pathway to the muscles is destroyed - UMN lesion: - Upper facial muscles are partially spared because of alternative pathways in the brainstem

Question 180

What is Bell’s palsy/

Answer 180

Neurological condition that presents with rapid onset of unilateral facial paralysis

Question 181

Why is the top half of the face sometimes spared in Bell’s palsy?

Answer 181

The lower half of the faces only has contralateral innervation Top half has bilateral. forehead sparing

Question 182

How can you tell between an UMN and LMN Bell’s palsy?

Answer 182

UMN injured: means the contralateral side is weak but the forehead is not LMN - weakness of all the muscles on the ipsilateral side of the face

Question 183

What are the signs of cranial nerve 8 lesion?

Answer 183

- Unilateral sensorineural deafness - Tinnitus - Slow-growing lesions seldom present with vestibular symptoms as compensation has time to occur

Question 184

What are the signs of cranial nerve 9 lesion?

Answer 184

- Unilateral lesions do not cause any deficit because of bilateral corticobulbar connections (wasting and deviation of tongue) - Bilateral lesions result in pseudobulbar palsy

Question 185

What are the causes of a CN9/CN10 lesion?

Answer 185

Jugular foramen lesion

Question 186

What are the signs of cranial nerve 10 lesion?

Answer 186

- Palatal weakness: - Nasal speech - Nasal regurgitation of food - Palate moves asymmetrically when the patient says 'ahh' - Recurrent nerve palsy: - Hoarseness - Loss of volume - Bovine cough (non- explosive cough of someone unable to close their glottis)

Question 187

What are the signs of cranial nerve 11 lesion?

Answer 187

Weakness and wasting of sternocleidomastoid and trapezius muscles

Question 188

What are the signs of cranial nerve 12 lesion?

Answer 188

- LMN lesion: - Wasting of the ipsilateral side of the tongue, with fasciculation - Attempted protrusion of tongue causes deviation towards the affected side

Question 189

What are conditions that may affect cranial nerves?

Answer 189

- Diabetes mellitus - Stroke - MS - Tumours - Sarcoid - Vasculitis (e.g. polyarteritis nodosa) - Systemic lupus erythematosus (SLE) - Syphilis - Chronic meningitis (malignant, TB, or fungal)

Question 190

How do you get a tetanus infetion?

Answer 190

Inoculation through skin with Clostridium tetani spores (found globally in soil) Clostridium tetani - gram positive anaerobe with spores e.g. stepping on a nail, dirty wounds

Question 191

What toxins does a tetanus infection produce?

Answer 191

Tetanolysin (tissue destruction) Tetanospasmin (clinical features )

Question 192

What are the symptoms of tetanus?

Answer 192

Unopposed flexion of limbs spasm of muscles

Question 193

What is the management of tetanus?

Answer 193

Prevention = vaccinate!! Supportive Muscle relaxants Paracetamol/cooling Immunoglobulin to mop up toxin Metronidazole to clear any residual bacteria

Question 194

How do you become infected with Rabies?

Answer 194

Inoculation through skin withsaliva of rabid animal(dogs, cats, foxes etc) e.g. lick, bite, splash Travels retrogradelyalong nerves

Question 195

What is the clinical manifestation of Rabies?

Answer 195

Incubation depends on site and size of inoculation symptoms can first present within Min 2 weeks to Max years! Paraesthesia at bite site Reaches CNS… Furious or paralytic presentation

Question 196

What is the management for rabies?

Answer 196

Once symptomatic invariably fatal (>99.9%) Managed with sedatives ++++ PROPHYLAXIS IS KEY!!! pre-exposure prophylaxis(vaccination) post-exposure prophylaxis (vaccination and immunoglobulin)

Question 197

What are polyneuropathies?

Answer 197

Motor and/or sensory disorder of multiple peripheral or cranial nerves Usually symmetrical, widespread and worse distally (glove and stocking distribution)

Question 198

What are the classification of polyneuropathies?

Answer 198

- Chronicity e.g. acute or chronic - Function e.g. sensory, motor, autonomic or mixed - Mostly motor e.g. - Guillain-Barre syndrome - Lead poisoning - Charcot-Marie-Tooth syndrome - Mostly sensory e.g. - Diabetes mellitus - Renal failure - Leprosy - Pathology e.g. demyelination, axonal degeneration or both

Question 199

What are the causes of polyneuropathies?

Answer 199

- Metabolic: diabetes mellitus, renal failure, hypothyroidism, hypoglycaemia, mitochondrial disorders - Vasculitides: polyarteritis nodosa, rheumatoid arthritis, GPA - Malignancy: paraneoplastic syndromes, polycythaemia rubra vera - Inflammatory: Guillain-Barre syndrome, sarcoidosis - Infections: leprosy, HIV, syphilis, lyme disease - Nutritional: decreased; vit B12, B1, B6, E - Inherited syndromes: Charcot-marie-tooth, porphyria, leucodystrophy - Drugs/toxins: lead, arsenic, alcohol, vincristine, cisplatin, metronidazole, isoniazid, phenytoin, nitrofurantoin - Other: paraproteinaemias, amyloidosis

Question 200

What are the differential diagnoses for epilepsy?

Answer 200

- postural syncope - migraine - hypoglycaemia - benign paroxymal positional vertigo - dystonia - cataplexy - TIA - parasomnia - cardiogenic syncope - non-epileptic seizure - hyperventilation

Question 201

Define epilepsy?

Answer 201

A neurological disorder in which a person experiences recurring seizures. (needs to have at least 2 seizures to be diagnosed as epilepsy)

Question 202

What are the main excitatory and inhibitory neurotransmitters? And their function?

Answer 202

main excitatory neurotransmitter = glutamate - binds to NMDA (primary receptor) - opens ion channels that cause calcium and sodium influx. main inhibitory neurotransmitter = GABA - binds to GABA receptors - inhibits the signal by opening channels that cause a chloride influx

Question 203

What are the different types of seizure?

Answer 203

1. Generalised:when both hemispheres are affected always a loss of consciousness 2. Focal : when the affected area is limited to one half of the brain or sometimes even smaller like a single lobe can progress to bilateral

Question 204

What are the different subtypes of generalised seizure?

Answer 204

Tonic Atonic Clonic Tonic-clonic Myoclonic Absence

Question 205

What are the two types of focal seizure?

Answer 205

Simple (without impaired awareness) Complex (with impaired awareness)

Question 206

What are the general clinical manifestations of seizures for each stage?

Answer 206

Prodromal phase: Confusion, irritability or mood disturbances Early-ictal phase: Aura: warning felt before a seizure. These can include sensory, cognitive, emotional or behaviour changes. Ictal phase: Will vary depending on seizure type Post-ictal phase: Confused, drowsy and irritable during recovery

Question 207

What is a tonic, clonic and tonic-clonic seizure?

Answer 207

Tonic seizure: the muscles become stiff and flexed which will cause the patients to fall backwards Clonic seizures: violent muscle contractions (convulsions) Tonic-clonic: there is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking episodes). Typically the tonic phase comes before the clonic phase. (tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolongedpost-ictal periodwhere the person is confused, drowsy and feels irritable or depressed.

Question 208

What is the medication for generalised tonic-clonic seizures?

Answer 208

1st line: Sodium valproate 2nd line: Lamotrigine or Carbamazepine

Question 209

What is an Atonic seizure

Answer 209

Known as drop attacks. This is where the muscles suddenly relax and become floppy which can cause the patient to fall usually forward. They don’t usually last longer than 3 minuets. They typically begin in childhood. They may be indicative ofLennox-Gastaut syndrome.

Question 210

What is the management for an Atonic seizure?

Answer 210

First line: sodium valproate Second line: Lamotrigine

Question 211

What is a myoclonic seizure?

Answer 211

They present as sudden brief muscle contractions like a sudden jump. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy.

Question 212

What is the management for a myoclonic seizure?

Answer 212

First line: sodium valproate Other options: lamotrigine, levetiracetam or topiramate

Question 213

What is an absence seizure?

Answer 213

Impaired awareness or responsiveness. Patient becomes blank and stares into space before returning to normal. Motor abnormalities are either absent or very minor e.g. eyelid flutters or repetitive lip smacking. Common in children. Most patients (> 90%) stop having absence seizures as they get older.

Question 214

What is the management for an absence seizure?

Answer 214

First line: sodium valproate or ethosuximide

Question 215

What are infertile spasms?

Answer 215

Known as West syndrome. - It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. - It is characterised by clusters of full body spasms. - There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free.

Question 216

What is a simple focal seizure (focal aware seizure)?

Answer 216

No loss of consciousness The patient is aware and awake Will have uncontrollable muscle jerking

Question 217

What is a complex focal seizure (focal impaired awareness seizure)?

Answer 217

There is loss of consciousness Patient is unaware

Question 218

What is the most common region of the brain affected in a focal seizure?

Answer 218

Temporal lobe

Question 219

What are the features of a temporal lobe seizure?

Answer 219

They affect hearing, speech, memory and emotions: - Hallucinations - Memory flashbacks - Déjà vu - Doing strange things on autopilot Can also include audio symptoms such as buzzing, ringing and vertigo

Question 220

What are the features of a frontal lobe seizure?

Answer 220

Motor symptoms: pelvic thrusting, bicycling and tonic posturing Bizarre behaviour Vocalisations Sexual automatisms

Question 221

What are the features of a parietal focal seizure?

Answer 221

Paraesthesia Visual hallucinations Visual illusions More subjective and difficult to diagnose than other areas

Question 222

What are the features of a Occipital focal seizure?

Answer 222

Visual hallucinations Transient blindness Rapid and forced blinking Movement of head or eyes to the opposite side

Question 223

What can happen if a focal seizure spreads?

Answer 223

Focal to bilateral tonic-clonic: a focal seizure may spread to affect a wider network of neurons involving both hemispheres. Traditionally termed a secondary generalised seizure.

Question 224

What is the medication for focal seizures?

Answer 224

1st line:  Carbamazepine  or  Lamotrigine 2nd line:  Sodium valproate  or  Levetiracetam

Question 225

What is the management for infantile/west syndrome seizures?

Answer 225

Prednisolone Vigabatrin

Question 226

What is the mechanism of Carbamazepine?

Answer 226

sodium channel blocker; prevents repetitive and sustained firing of action potentials

Question 227

What are the side effects of Carbamazepine?

Answer 227

- Reduce effect of Oral Contraceptive Pill (by CYP450 induction > faster metabolism) - leucopenia, thrombocytopenia, hydronatraemia + liver dysfunction

Question 228

Can you take Carbamazepine when pregnant?

Answer 228

DONT STOP - risk of harm from seizures is greater than risk of foetal malformations (very low risk) - immediately start taking folic acid

Question 229

What is the mechanism of sodium valproate?

Answer 229

increases the activity of GABA, which has a relaxing effect on the brain, teratogenic

Question 230

What are the investigations for seizures?

Answer 230

urinalysis or urine cultures, blood cultures (exclude sepsis, encephalitis, or meningitis) CT scan Blood: blood glucose (rule out hypoglycaemia) ECG (to exclude a heart problem) EEG (only useful while having a seizure to track electrical patterns in brain, won't visualise much afterwards)

Question 231

What are the other treatments for epilepsy?

Answer 231

Vagus nerve stimulation = send electrical stimulations to left vagus nerve to calm down irregular electrical brain activity Surgery: Temporal lobe resection MC Corpus callosotomy = cut callosum to stop epilepsy spreading to other side of brain Lesionectomy Hemispherectomy or hemispherotomy Ketogenic diet - high fat, low carbs, controlled protein protein diet - brain use ketone bodies instead of glucose

Question 232

Which anti-epileptic medication is teratogenic?

Answer 232

sodium valproate - drugs which cause structural/functional malformations in a developing fetus

Question 233

What is required for a diagnosis of epilepsy?

Answer 233

Must have had 2 or more seizures more than 24 hours apart MRI/CT: examine the hippocampus look for underlying cause EEG: 3H2 wave absence Bloods: rule out metabolic/infection

Question 234

What are the different causes of seizures? (pneumonic)

Answer 234

VITAMIN DE - Vascular - Infection - Trauma - Autoimmune- SLE - Metabolic - Idiopathic - Neoplasms - Dementia and drugs (cocaine) - Eclampsia

Question 235

What is the medical emergency associated with epilepsy?

Answer 235

Status Epilepticus

Question 236

How do you treat status Status Epilepticus?

Answer 236

ABCDE Give IV lorazepam 4mg and repeat 10 minuets after if it doesn’t work If seizure persist then give IV phenobarbital or phenytoin

Question 237

What are the driving rules for a seizure?

Answer 237

Adults who present with an isolated seizure: Should stop driving for6 months, providing no cause is found on brain imaging and there is no epileptiform activity on EEG If the above conditions are not met or the patient has known epilepsy: Patients must be seizure-free for12 monthsbefore they may qualify for a driving license If seizure-free for 5 years, a ‘til 70 licence is usually reinstated

Question 238

What is Multiple Sclerosis?

Answer 238

An autoimmune cell-mediated demyelinating disease of the central nervous system

Question 239

What is the epidemiology of MS?

Answer 239

More common in women 20-40 most common age for diagnosis More common in white More common in northern latitudes Symptoms will improve in pregnancy and post-partum period

Question 240

What are some risk factors for developing MS?

Answer 240

Vitamin D deficiency Family history: HLA-DR2 is implicated; 30% monozygotic twin concordance EBV infection: the virus with the greatest link to MS Smoking Obesity

Question 241

Describe the pathophysiology of MS

Answer 241

(don't fully know the cause) T-cells get through the blood brain barrier and are activated by myelin. The T-cell then changes the BBB to allow more immune cells to get in the brain MS is a type IV hypersensitivity reaction: T-cells release cytokines and these recruit more immune cells whilst also damaging the oligodendrocytes (+ axones) B-cells will make antibodies that will destroy the myelin of the oligodendrocytes. leaving behind areas of plaque/sclera

Question 242

How does MS progress over time?

Answer 242

In early disease, re-myelination can occur and symptoms can resolve. (thin myelin forms so conduction reduced in heat) In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent. A characteristic feature of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time MS lesions change location over time is that they are “disseminated in time and space”.

Question 243

What are the different types of MS?

Answer 243

Relapsing-remitting: Secondary progressive Primary progressive Progressive relapsing Clinically isolated syndrome (kind of counts)

Question 244

What is Relapsing-remitting: MS?

Answer 244

The most common pattern 85% of cases Episodic flare-ups separated by periods of remission. There isn’t full recovery after flare ups so disability increases over time. 60% will develop secondary within 15 years

Question 245

What is secondary progressive MS?

Answer 245

Initially, the disease starts with a relapsing-remitting course, but then symptoms get progressively worse with no periods of remission

Question 246

What is primary progressive MS?

Answer 246

Symptoms get progressively worse from disease onset with no periods of remission Accounts for 10% of cases and is more common in older patients

Question 247

What is Progressive relapsing MS?

Answer 247

One constant attack but there are bouts superimposed during which the disability increases even faster

Question 248

What is clinically isolated syndrome MS?

Answer 248

This describes the first episode of demyelination and neurological signs and symptoms. - MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”. - Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress to MS

Question 249

What are the signs and symptoms of MS?

Answer 249

Optic nerve affected: Optic neuritis Eye movement abnormalities- double vision VI nerve Spinal cord affected: Focal weakness (incontinence, limb paralysis, Bells palsy) Focal sensory symptoms ( numbness, pins and needles, Lhermitte’s sign is an electric shock sensation that travels down the spine and into the limbs when flexing the neck.) Cerebellar white matter affected Ataxia Cerebral hemispheres: - large variety but can be silent Medulla + pons - dysarthria, double vision, vertigo, nysatgmus Uhthoff’s phenomenon: worsening of symptoms following a rise in temperature, such as a hot bath

Question 250

What is optic neuritis?

Answer 250

Demyelination of the optic nerve which present with: - Unilateral reduced vision - Central scotoma (enlarged blind spot) - Pain on eye movement - Impaired colour vision (red)

Question 251

What is the primary investigation for MS and what would it show?

Answer 251

MRI of brain and spine: - Will show demyelinating plaques called Dawson’s fingers - High signal L2 lesions - Old lesions will not enhance with contrast, whereas newer lesions will. This provides evidence of dissemination of lesions in time and space which is required for a diagnosis of MS

Question 252

What other investigations are done for MS?

Answer 252

Lumbar puncture - can detect oligoclonal IgG bands in the CSF Nerve conduction studies - delayed conduction speeds Evoked Potentials (VEP) - measures speed of messages along nerves

Question 253

What is the diagnostic criteria used to diagnose MS?

Answer 253

McDonald criteria

Question 254

What is the McDonald criteria based on?

Answer 254

2 or more relapses and either: - Objective evidence of two or more lesions - Objective evidence of one and a reasonable history of a previous relapse ‘Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potentials

Question 255

What is used to treat a MS relapse?

Answer 255

Oral or IV methylprednisolone (reduce time to recover from illness but don't treat illness) Plasma exchange: to remove disease-causing antibodies

Question 256

What is used for maintenance of MS?

Answer 256

Disease-modifying drugs- Beta-interferon: decreases the level of inflammatory cytokines Monoclonal antibodies e.g. alemtuzumab (anti-CD52) and natalizumab (anti-α4𝛃1-integrin) Glatiramer acetate: immunomodulator drug which acts as a ‘decoy’ Fingolimod: a sphingosine-1-phosphate receptor modulator that keeps lymphocytes in lymph nodes so they can’t cause inflammation

Question 257

Why is stem cell transplant used in MS?

Answer 257

- use chemotherapy to kill off all their immune cells - and give them a stem cell transplant - MS is a inflammatory disease so new immune cells should not attack myelin - doesn't improve demyelination as damage is already done but stops the disease progressing

Question 258

What are some complications of MS?

Answer 258

Genitourinary: urinary tract infections, urinary retention and incontinence Constipation Depression: offer mental health support if required Visual impairment Mobility impairment: offer physiotherapy, orthotics and other mobility aids Erectile dysfunction

Question 259

What is Guillain-Barré syndrome?

Answer 259

An acute paralytic polyneuropathy. It is an autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system

Question 260

What are the risk factors for developing Guillain-Barré syndrome?

Answer 260

Male Age 15-35 and 50-75 Malignancies Vaccines (flu) Infections

Question 261

What are the most common infections that trigger Guillain-Barré syndrome?

Answer 261

Campylobacter jejuni (most common) Cytomegalovirus EBV

Question 262

What causes Guillain-Barré syndrome?

Answer 262

A pathogenic antigen resembles myelin gangliosides in the peripheral nervous system. The immune system targets the antigen and attacks the myelin sheath of sensory and motor neurones It occurs in patches along the length of the axon so is called segmental demyelination

Question 263

What antibodies are found in 25% of people with Guillain-Barré syndrome?

Answer 263

Anti-ganglioside antibodies (anti-GMI)

Question 264

Describe the disease course of Guillain-Barré syndrome?

Answer 264

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Question 265

What are the signs and symptoms of Guillain-Barré syndrome?

Answer 265

Symmetrical ascending weakness (starting at feet and moving up the body) Reduced reflexes Loss of sensation and pain Cranial nerve involvement such as facial nerve weakness Autonomic features (sweating, raised pulse) Struggling to breathe

Question 266

What is used to diagnose Guillain-Barré syndrome?

Answer 266

A clinical diagnosis that is evidenced by progressive weakness and hyporeflexia in the weaker limbs. The Brighton criteria is used for diagnosis.

Question 267

What are the differentials of Guillain-Barré syndrome?

Answer 267

Myasthenia gravis Transverse myelitis Polymyositis

Question 268

What is the treatment for Guillain-Barré syndrome?

Answer 268

IV immunoglobulins IV IG Plasma exchange (alternative to IV IG) Venous thromboembolism prophylaxis (PE is the leading cause of death)

Question 269

What is the prognosis for Guillain-Barré syndrome?

Answer 269

80% will fully recover 15% will be left with some neurological disability 5% will die

Question 270

What is Parkinson’s disease (PD)

Answer 270

A neurodegenerative disorder characterised by the loss of dopaminergic neurons within the substantia nigra pars compacta (SNPC) of the basal ganglia.

Question 271

What are the risk factors for developing PD?

Answer 271

Age: prevalence is 1% in 60-70 and 3% in those above 80 Gender: men are 1.5 times more likely than females to develop PD Family history

Question 272

Describe the pathophysiology of PD

Answer 272

1. progressive loss of dopamine-producing neurons meaning there is a reduction in the amount of dopamine produced at the substantia nigra 2. Loss of these neurons results in reduction in action of the direct pathway and a resultant increase in the antagonistic indirect pathway which has a restrictive action on movement. 3. Therefore bradykinesia and rigidity are key symptoms 4. There is also formation of protein clumps Lewy bodies

Question 273

What are the 3 key presentations of PD?

Answer 273

Bradykinesia Tremor Rigidity PD symptoms usually start unilaterally and then become bilateral later in the disease course.

Question 274

What does the bradykinesia look like in PD?

Answer 274

Handwriting gets smaller Only take small steps (shuffling gait) Difficulty initiating movement Difficulty turning around when standing Reduced facial movements and expressions

Question 275

What does the tremor look like in PD?

Answer 275

A unilateral resting tremor. Described as pill rolling tremor looks like they are rolling pill between thumb and finger The tremor is worse at resting and when they are distracted like using the other hand Frequency of 4-6 times a second

Question 276

What is the difference between an essential and Parkinson's tremor?

Answer 276

Essential - active tremor + worsens with movement Parkinson's - pill rolling, resting tremor

Question 277

What is the rigidity like in PD?

Answer 277

If you take their hand and passively flex and extend their arm at the elbow you will feel tension in their arm that gives way to movement in small increments (little jerks) Described as cogwheel

Question 278

What are some other symptoms of PD?

Answer 278

Depression Sleep disturbance and insomnia no REM Loss of sense of smell Postural instability Cognitive impairment and memory problems

Question 279

What are the differences between a PD tremor and a benign essential tremor?

Answer 279

PD= asymmetrical BET= symmetrical PD= frequency= 4-6 BET= 5-8 PD= worse at rest BET= better at rest PD= improves with intentional movement BET= worse PD= no change with alcohol BET= better with alcohol

Question 280

What is used to diagnose PD?

Answer 280

PD is a clinical diagnosis and should be suspected in a patient with bradykinesia and at least one of the following: - Tremor - Rigidity - Postural instability

Question 281

What is the management for PD?

Answer 281

Motor symptoms not affecting quality of life: A choice of one of the following: 1. Dopamine agonist(non-ergot derived) - Pramipexole, ropinirole 2. Monoamine oxidase B inhibitor - Selegiline, rasagiline - Stop breakdown of circulating dopamine Motor symptoms affecting the quality of life: 3. L.Dopa Synthetic dopamine levodopa given with a drug that stops it being broken down. - These are peripheral decarboxylase inhibitors. Carbidopa and benserazide. Co-benyldopa (levodopa and benserazide) Co-careldopa (levodopa and carbidopa)

Question 282

What is the prognosis for PD?

Answer 282

PD is a chronic and progressive condition with no cure. Overall, life expectancy is reduced with the mortality being 2-5 times higher for those aged 70-89 years old. Also, the risk of dementia is up to 6 times higher in PD patients.

Question 283

What is Huntington’s disease?

Answer 283

An autosomal dominant genetic neurodegenerative condition that causes a progressive a progressive deterioration in the nervous system.

Question 284

What causes HD?

Answer 284

- It is a trinucleotide repeat disorder that involves a genetic mutation in the HTT gene on chromosome 4 - There is a repeat of CAG which codes for glutamine 36 times in a row so patients have 36 glutamine in a row on the Huntington protein - These mutated proteins aggregate within neuronal cells of the caudate and putamen causing neuronal cell death. - This leads to decreased ACh and GABA synthesis. - This leads to dopamine increase leading to excessive movement

Question 285

What is anticipation and how is it linked to HD?

Answer 285

It is a feature of trinucleotide repeat disorders. When copying the HTT gene, DNA polymerase can lose track of which CAG it’s on and so add extra CAGs. This is called repeat expansion. This leads to successive generations having more repeats in the gene resulting in: - Early age of onset - Increased severity of the disease

Question 286

What are the symptoms of HD?

Answer 286

Patients will be asymptomatic until 30-50. Symptoms: - Begin with Cognitive, psychiatric or mood problems - Chorea (involuntary, abnormal movements) - Eye movement disorders - Dysarthria: speech difficulties - Dysphagia: swallowing difficulties - Dementia

Question 287

How is diagnosis of HD made?

Answer 287

Made at a specialist genetic centre that looks for the number of CAG repeats. This will involve pre and post test counselling

Question 288

What is the treatment for HD?

Answer 288

No treatment - supporting person and family - maintaining quality of life = OT + PT - speech and language therapy - genetic counselling - end of life care planning

Question 289

What medications are given for symptoms relief in huntington's?

Answer 289

Medications that can suppress the disordered movement: Antipsychotics (e.g. olanzapine) Benzodiazepines (e.g. diazepam) Dopamine-depleting agents (e.g. tetrabenazine)

Question 290

Describe the epidemiology of Alzheimer’s Disease?

Answer 290

Most common form of dementia More than 500,000 people with it in the UK More common in women

Question 291

What are the risk factors for developing AD?

Answer 291

Age CVD Depression Low educational attainment - Low social engagement and support - head trauma, learning difficultie

Question 292

What genes put you at risk of AD?

Answer 292

APP and presenilin genes (PSEN1, PSEN2) - APP on chromosome 21, so increased risk for people with down syndrome alleles of apolipoprotein E4 = not as good at clearing beta plaques

Question 293

What are the amyloid beta plaques and how do they contribute to AD?

Answer 293

1. Senile plaques deposits of beta amyloid aggregate. 2. Amyloid precursor protein is found on neurons (helps it to grow and repair after injury). 3. If it is broken down normally by alpha secretase and gamma secretase it is soluble and easy to remove 4. However if it is broken by beta secretase the leftover fragment isn’t soluble and creates a monomer called beat amyloid. 5. These are sticky and can in between neurones and their signalling, They also increase inflammation which damages surrounding neurones

Question 294

What are neurofibrillary tangles and how do they contribute to AD?

Answer 294

- Neurons held together by cytoskeleton = is made up of microtubules a protein 'tau' makes sure they stay together. Beta amyloid build-up outside the neurones initiates pathways which leads to the activation of kinase. This leads to the phosphorylation of tau The tau protein changes shape, stops supporting the microtubules, and clumps up with other tau proteins, forming neurofibrillary tangles Neurones with tangles and non-functioning microtubules can’t signal as well, and sometimes end up undergoing apoptosis. As neurones die, the brain starts to atrophy.

Question 295

What are some of the symptoms of AD?

Answer 295

- Agnosia- can’t recognise things - Apraxia can’t do basic motor skills - Aphasia speech difficulties Cognitive impairment Agitation and emotional lability Depression and anxiety Sleep cycle disturbance: Motor disturbance: wandering is a typical feature of dementia

Question 296

How can the activities of daily life be affected in AD?

Answer 296

Loss of independence: increasing reliance on others for assistance with personal and domestic activities: - Early stages: problems with higher level function (e.g. managing finances, difficulties at work) - Later stages: problems with basic personal care (e.g. washing, eating, toileting) and motor function (e.g. walking, transferring)

Question 297

What is the diagnostic criteria for AD?

Answer 297

Functional ability: inability to carry out normal functions Impairment in 2 or more cognitive domains Differentials excluded

Question 298

What imaging is used for AD?

Answer 298

CT/MRI: exclude other diagnosis help determine type of dementia; will show medial temporal lobe atrophy

Question 299

What is the definitive diagnosis for AD?

Answer 299

Brain biopsy after autopsy

Question 300

What are the two cognitive assessments that can be performed to asses AD?

Answer 300

Mini mental state examination (MMSE) Montreal cognitive assessment scale (MoCA) Primary care: 6 Cognitive Impairment Test (6CIT)

Question 301

What is assessed in the cognitive assessments?

Answer 301

Attention and concentration Recent and remote memory Language Praxis (planned motor movements) Executive function Visuospatial function

Question 302

What are the different score on the MMSE and MoCA and what do they represent?

Answer 302

Mild: MMSE 21-26, MoCA 18-25, CDR 1 Moderate: MMSE 10-20, MoCA 10-17, CDR 2 Severe: MMSE <10, MoCA <10, CDR 3

Question 303

What is the treatment for AD?

Answer 303

Non-pharmacological: programmes to improve/maintain cognitive function Mild to moderate AD: acetylcholinesterase inhibitors e.g., Donepezil and rivastigmine Moderate to severe AD: N-methyl-d-aspartic acid receptor antagonist memantine (anti-glutamate transmitter)

Question 304

What is frontotemporal dementia?

Answer 304

A neurodegenerative disorder characterised by focal degeneration of the frontal and temporal lobes. It is a heterogenous condition with various subtypes e.g., Pick’s disease

Question 305

What are the genes associated with FTD?

Answer 305

- C9ORF72 gene: found on chromosome 9. Most common genetic cause of inherited FTD. Also implicated in hereditary motor neuron disease. - MAPT (microtubule associated protein)- found on chromosome 17 - Granulin precursor(GRN): found on chromosome 17.

Question 306

Describe the pathophysiology of FTD?

Answer 306

In Pick disease 3R isoforms of the tau protein become hyperphosphorylated. - This means they change shape and stop being able to tie together the tubulins in the neurones cytoskeleton. These proteins start to clump together forming tangles of tau proteins known as pick bodies

Question 307

What are the frontal lobe effects of FTD?

Answer 307

Personality and behaviour change: - Disinhibition - Loss of empathy - Apathy - Hyperorality(e.g. dietary changes, attempt to consume non-edible products, eat beyond satiety) - Compulsive behaviour(e.g. cleaning, checking, hoarding)

Question 308

What are the temporal lobe effects of FTD?

Answer 308

Language problems: - Effortful speech - Halting speech - Speech sound errors - Speech apraxia - Word-finding difficulty - Surface dyslexia or dysgraphia: mispronouncing difficult words (e.g. yacht)

Question 309

How would you diagnose FTD?

Answer 309

Diagnosis based on cognitive assessment Imaging: MRI: exclude other pathology; indicates changes in the frontal and temporal lobes Definitive diagnosis = brain biopsy after a person has died

Question 310

What are the pharmacological treatments for FTD?

Answer 310

Serotonin reuptake inhibitors (SSRI) = used for difficult behavioural symptoms. Have been shown to decrease disinhibition, anxiety, impulsivity and repetitive behaviours. Atypical anti-psychotics = can help with agitation and behavioural symptoms.

Question 311

What is the epidemiology of vascular dementia?

Answer 311

Makes up 20% of dementias It is the second most common form

Question 312

What can cause vascular dementia?

Answer 312

Ischaemic stroke Small vessel disease Haemorrhage Other: cerebral amyloid, which is a cause of small vessel disease. Deposition of amyloid in small arteries. CADASIL, which is due to mutation in the NOTCH3 gene and leads to arterial thickening and occlusion.

Question 313

Normal Physiology - how much of the hearts cardiac output goes to the brain? What is unique about the brain’s metabolism?

Answer 313

The brain uses around 20-25% of oxygen Neurons can only function in aerobic conditions and don’t have long term energy stores so need a constant supply

Question 314

Describe the pathophysiology of VD?

Answer 314

- Once blood supply to the brain falls below the demands to the tissue, it’ considered an ischaemic stroke. - The tissue damage is permanent because the dead tissue liquifies in a process called *liquefactive necrosis** - Brain tissue necrosis leads to a loss of mental function in the area where the loss has occurred

Question 315

How do the symptoms of VD appear?

Answer 315

They appear suddenly and the brain function decline is STEP-WISE e.g. it decreases with each stroke Symptoms will vary depending on which vessels are affected in stroke/atherosclerosis

Question 316

What is Lewy body dementia?

Answer 316

A type of dementia characterised by fluctuating cognitive impairment, visual hallucinations and parkinsonism

Question 317

What % of dementia does Lewy Body Dementia make up?

Answer 317

15-25%

Question 318

What causes LBD?

Answer 318

- Neurons contain a protein called alpha synuclein and in LBD this proteins is misfolded within the neurons - This misfolded protein aggregates to form Lewy bodies that deposit inside the neurones particularly in the cortex and the substantia nigra.

Question 319

What other disease are Lewy bodies seen?

Answer 319

Parkinson’s Multiple system atrophy

Question 320

What are the early symptoms in LBD?

Answer 320

Alzheimer’s like cognitive ones: Difficulty focusing Poor memory Visual hallucinations Disorganized speech Depression

Question 321

What are the later symptoms of LBD?

Answer 321

Later symptoms are typically motor ones (Parkinson’s-like) Resting tremors Stiff and slow movements Reduced facial expressions Some patients may have sleep disorders e.g. sleep walking or talking in their sleep

Question 322

What is the management for LBD?

Answer 322

Dopamine analogue e.g. levodopa: for Parkinson’s like motor symptoms Cholinesterase inhibitors e.g. donepezil: increases acetylcholine availability, used for Alzheimer’s-like cognitive symptoms

Question 323

What drugs should be avoided in LBD?

Answer 323

Antipsychotics as they can have harmful side effects

Question 324

What are primary headaches and give some examples?

Answer 324

No underlying cause relevant to the headache: - Migraine - Cluster - Tension - (Trigeminal Neuralgia)

Question 325

What are red flags for headaches in regard to the features of the headache?

Answer 325

Sudden-onset reaching worse severity within 5 minuets (subarachnoid haemorrhage) New-onset over 50 (GCA/ space occupying lesion) Progressive/persistent headache or one that has changed dramatically (space-occupying lesion/ subdural haematoma)

Question 326

What are red flag precipitating factors for headaches?

Answer 326

Recent head trauma within 3 months (subdural haematoma) Headache worse lying down (raised ICP) Headache worse on standing (CSF leak) Household contacts with similar symptoms (CO poisoning)

Question 327

What are red flag associated symptoms of headaches?

Answer 327

Fever, photophobia or neck stiffness (meningitis or encephalitis) New neurological defect (raised ICP/stroke) Visual disturbance (GCA/ acute closure glaucoma) Vomiting (raised ICP, brain abscess and CO poisoning)

Question 328

What is an important investigation to carry out for a headache?

Answer 328

fundoscopy = (look at retina at back of eye) look for papilledema which indicates a raised intracranial pressure + idiopathic intracranial hypertension

Question 329

What are the features of idiopathic intracranial hypertension?

Answer 329

females - headache, intermittent, generalised - worse lying down - visual obscurations - usually high BMI - papillodema I = normal CT T = modify risk factors, acetazolamide/diuretics/topiramate

Question 330

What are the risk factors for migraines?

Answer 330

Family history Female gender 3 times more common Obesity Triggers

Question 331

What are some triggers for migraines?

Answer 331

CH- Chocolate OC- Oral contraceptive OL- alcohOL A- anxiety T- travel E- exercise CHOCOLATE Other triggers can be red wine, bright lights and menstruation

Question 332

What are the different types of migraine?

Answer 332

Migraine without aura Migraine with aura Silent migraine (just the aura without the headache) Hemiplegic migraine

Question 333

What causes migraines?

Answer 333

- Headache is thought to be due to neuronal hyperexcitability. - This leads to trigeminal nerves initiating an inflammatory response with dilation of meningeal blood vessels and sensitisation of surrounding nerve fibres leading to pain - Aura is thought to occur due to cortical spreading depression which is a propagating wave of depolarisation across the cerebral cortex causing the brain to become hypersensitive to certain stimuli

Question 334

What are the headache symptoms of a migraine?

Answer 334

Last between 4-72 hours: - Pounding or throbbing in nature - Usually unilateral (can be bilateral more common in children) - Photophobia - Phonophobia (loud noises) - Aura - Nausea and vomiting

Question 335

What is aura?

Answer 335

Aura is the term used to describe the visual changes associated with migraines symptoms can be: - Sparks in vision - Blurring vision - Line across vision - Loss of different visual fields

Question 336

What is a hemiplegic migraine?

Answer 336

They can mimic a stroke. Need to rule out if patient has symptoms: - typical migraine - Sudden onset - Hemiplegia - Ataxia - Change in consciousness

Question 337

What are the 5 stages of a migraine?

Answer 337

- Prodromal stage subtle symptoms such as yawning, fatigue or mood changes - Aura - headache - Resolution the headache can fade away and be relieved by vomiting or sleeping - Postdromal stage

Question 338

What is the diagnostic criteria for migraines with aura?

Answer 338

At least TWO headaches filling criteria: One or more typical fully reversible aura symptoms including: - Visual symptoms such as zigzag lines and/or scotoma visual aura is the most common type of aura. - Sensory symptoms such as unilateral pins and needles or numbness. - Speech and/or language symptoms such as dysphasia. At least three of the following: - At least one aura symptom spreads gradually over at least 5 minutes. - Two or more aura symptoms occur in succession. - Each individual aura symptom lasts 5-60 minutes. - At least one aura symptom is unilateral. - At least one aura symptom is positive. - The aura is accompanied, or followed within 60 minutes, by headache. (can be confused with stroke until headache starts)

Question 339

What is the diagnostic criteria for migraines without aura?

Answer 339

FIVE attacks fulfilling this criteria Duration = Headache lasting 4–72 hours in adults or 2–72 hours in adolescents. Headache with at least TWO of the following characteristics: - Unilateral location (more commonly bilateral in children). - Pulsating quality — may be described as ‘throbbing’ or ‘banging' in young people. - Moderate or severe pain intensity. - Aggravation by, or causing avoidance of, routine activities of daily life (for example walking or climbing stairs). Headache with associated symptoms including at least one of: - Nausea and/or vomiting. - Photophobia (sensitivity to light) and phonophobia (sensitivity to sound).

Question 340

What is the management for migraines?

Answer 340

Analgesia (avoid opioids) Oral triptan ( 500mg sumatriptan) as the headache starts Antiemetics metoclopramide

Question 341

What are triptans?

Answer 341

They 5HT (serotonin) receptor agonists and they cause: - smooth muscle contraction in arteries - Peripheral pain receptors to inhibit activation of pain receptors - Reduce neuronal activity in the central nervous system

Question 342

What are the medications used for migraine prophylaxis?

Answer 342

Propranolol Topiramate (don’t use in pregnancy as it is teratogenic and can cause cleft lip) Amitriptyline

Question 343

What are the non-pharmacological treatments for migraines?

Answer 343

Acupuncture: if bothpropranololandtopiramateare ineffective or unsuitable Riboflavin (vitamin B2): **may be effective in some people, but avoid in pregnancy

Question 344

What is a tension headache?

Answer 344

The most common form of a primary headache - Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)

Question 345

What are the causes of tension headaches?

Answer 345

Missed meals Stress Overexertion Lack of sleep Depression

Question 346

What are the symptoms of tension headaches?

Answer 346

Bilateral with a pressing/tight sensation of mild-moderate intensity Nausea or vomiting Photophobia Phonophobia

Question 347

What is the main risk factor for a tension headache?

Answer 347

stress

Question 348

What are cluster headaches?

Answer 348

Severe unilateral headaches often periorbital that come in clusters of attacks

Question 349

What is the typical presentation for cluster headaches?

Answer 349

A typical patient with cluster headaches in your exams is a 30 – 50 year-old male smoker. - Attacks can be triggered by things like alcohol, strong smells and exercise.

Question 350

How long can a cluster headache last?

Answer 350

Patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years. Attacks last between 15 minutes and 3 hours.

Question 351

What are the symptoms of a cluster headache?

Answer 351

Severe and intolerable pain unilateral Red swollen watering eye Pupil constriction Eyelid dropping Nasal discharge Facial sweating

Question 352

What is the acute management of cluster headaches?

Answer 352

Triptans (6mg sumatriptan subcut) High flow oxygen 100%

Question 353

What are the prophylaxis for cluster headaches?

Answer 353

Verapamil (CCB) Lithium Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)

Question 354

What are the risk factors for Trigeminal Neuralgia?

Answer 354

females 50-60 increases with age unilateral MS

Question 355

What are the causes of Trigeminal Neuralgia?

Answer 355

Normally due to compression of the trigeminal nerve by a vascular loop often the superior cerebellar artery

Question 356

What are triggers for TGN?

Answer 356

Light touch Washing Shaving Talking Cold weather

Question 357

What is the headache like in Trigeminal Neuralgia?

Answer 357

Electric Shock Pain that lasts for seconds to minutes across the face 90% unilateral 10% bilateral

Question 358

What is the diagnostic criteria for trigeminal neuralgia?

Answer 358

Clinical Dx 3 or more attacks with characteristic unilateral facial pain and Symptoms MRI - exclude secondary causes/other pathology

Question 359

What is the treatment for TGN?

Answer 359

- First line: Carbamazepine - Second line: microvascular decompression or ablative surgery may be considered in refractory patients

Question 360

What is spinal cord compression SCC?

Answer 360

Results form processes that compress or displace arterial, venous and cerebrospinal fluid spaces as well as the cord itself.

Question 361

What is the main cause of SCC?

Answer 361

Metastatic cancer lesions

Question 362

Where are the metastasis most commonly from the cause SCC?

Answer 362

Breast Lung Prostate Thyroid Kidney Myeloma Lymphoma

Question 363

What area of the spine is most commonly affected by metastasis in SCC?

Answer 363

Thoracic 60% Lumbar 30% Cervical 10%

Question 364

What are some other causes of SCC?

Answer 364

Disc herniation Disc prolapse Primary spinal cord tumour Infection Haematoma

Question 365

What are the symptoms of SCC?

Answer 365

Back pain Progressive weakness of legs with UMN signs Sensory loss 1-2 cord segments below level of lesion UMN signs below level of lesion LWN signs at the level of the lesion

Question 366

What is a late sign of SCC?

Answer 366

Bladder and anal sphincter involvement: hesitancy, frequency and painless retention

Question 367

What are the nerve routes for the ankle Jerk reflex?

Answer 367

S1/S2

Question 368

What are the nerve routes for the Knee jerk reflex?

Answer 368

L3/L4

Question 369

What are the nerve routes for the Big toe reflex?

Answer 369

L5

Question 370

What would the features of a complete spinal cord compression be?

Answer 370

Loss of all motor and sensory function below the level

Question 371

What would be the features of an anterior spinal cord compression?

Answer 371

Disruption of the anterior spinal cord: - Loss of motor function below the level - Loss of pain and temperature sensation - Preservation of fine touch and proprioception

Question 372

What would be the features of a posterior spinal cord compression?

Answer 372

Disruption of posterior spinal cord or posterior spinal artery (rare) - Loss of fine touch and proprioception (posterior column) - Preservation of pain and temperature sensation (anterior column)

Question 373

What are the features of brown-sequard syndrome?

Answer 373

Hemi section of spinal cord: - Ipsilateral paralysis - Ipsilateral loss of vibration and proprioception - Contralateral loss of pain and temperature

Question 374

What is the primary investigation if suspecting a Spinal cord compression?

Answer 374

MRI of spinal cord Biopsy/surgical exploration

Question 375

What is the treatment for SCC?

Answer 375

Surgical decompression - Laminectomy: removal of the lamina/spongy tissue between the discs to relieve pressure - Microdiscectomy: removal of the herniated tissue from the disc

Question 376

What is sciatica?

Answer 376

L4/S3 lesion Sensory loss and pain in the back of the thigh and lateral aspect of little toe

Question 377

What nerves are branches of the sciatic nerve?

Answer 377

Common peroneal Tibial

Question 378

What are the symptoms of sciatica?

Answer 378

Unilateral pain from the buttock radiating down the back of the thigh to below the knee or feet Paraesthesia numbness and motor weakness

Question 379

What are the main causes of sciatica?

Answer 379

Herniated/prolapsed disc Tumours Spinal stenosis Piriformis syndrome

Question 380

What is bilateral sciatica a red flag for?

Answer 380

Cauda equina syndrome

Question 381

What is the main treatment for sciatica?

Answer 381

Physiotherapy + Analgesia: Amitriptyline Duloxetine

Question 382

What is cauda equina syndrome?

Answer 382

It is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed

Question 383

What are the causes of CES?

Answer 383

Lumbar disc herniation: the most common cause of CES Trauma Spinal tumour Lumbar spinal stenosis: narrowing of the spinal cord may result in CES. This can be congenital or acquired e.g. spinal osteoarthritis (spondylosis), rheumatoid arthritis, and a slipped vertebra (spondylolisthesis) Epidural abscess or haematoma

Question 384

What are the key presentations of CES?

Answer 384

Saddle anaesthesia = (loss of sensation in the perineum) can you feel wiping after poo Loss of sensation in bladder and rectum (not knowing when they’re full) Urinary retention or incontinence Faecal incontinence

Question 385

What are the investigations for CES?

Answer 385

Examinations: PR exam, knee and ankle reflexes MRI spine is gold standard Bladder ultrasound: to determine whether there us urinary retention

Question 386

What is the treatment for CES?

Answer 386

Emergency decompressive laminectomy: surgery should be performed within24-48 hoursof symptom onset. The incidence of thromboemboli in patients with CES is remarkably high, therefore patients require adequate thromboprophylaxis

Question 387

What is the main differential of CES?

Answer 387

Conus medullaris

Question 388

What is motor neurone disease?

Answer 388

Motor neurone disease is an umbrella term that encompasses a variety of specific diagnoses. They are neurodegenerative diseases that affect both upper and lower motor neurones but sensory neurons are spared

Question 389

What is the most common cause of Motor Neurone Disease?

Answer 389

Amyotrophic lateral sclerosis (ALS) accounts for 50% of cases

Question 390

What are some other causes of MND?

Answer 390

Progressive muscular atrophy: LMN only Primary lateral sclerosis: UMN only Progressive bulbar palsy: affects muscles of talking and swallowing (second most common) LMN only ALS: affects both UMN and LMN

Question 391

What gene is implicated in some cases of ALS?

Answer 391

SOD1

Question 392

What are the risk factors for MND?

Answer 392

Increasing age (over 60) Male Pesticides Heavy metals Rugby

Question 393

What are the general symptoms of MND?

Answer 393

- Progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. - The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. - They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).

Question 394

What are the signs of LWN disease?

Answer 394

Muscle wasting Reduced tone Reduced reflex Fasciculations Loss of power Babinski reflex negative

Question 395

What is the cause of LMN injury?

Answer 395

motor neurone - MND< spinal muscular atrophy, polio infection motor nerve roots - radiculopathy, guillain barre motore nerves - neuropathies Neuromuscular junction - myasthenia gravis Muscles disorder - myositis, myopathies

Question 396

What are the signs of a UMN lesion?

Answer 396

Increased tone (no UMN to stop actions of LMN) Brisk reflexes Rigidity + spasticity Babinski reflex positive

Question 397

What is never affected in MND?

Answer 397

Eye muscles (oculomotor movements) Sensory function and sphincters (onuf's nucleus)

Question 398

What are the symptoms of MND?

Answer 398

Progressive weakness Fatigue - nocturnal respiratory insufficiency (so wake up in night when CO2 builds up) Falls Speech and swallow issues

Question 399

What are the investigations for MND?

Answer 399

MND is a clinical diagnosis Electromyography: in MND there will be evidence of fibrillation potentials Nerve conduction studies: may show modest reductions in amplitude MRI spine: imaging can help exclude spinal pathology which may mimic MND, such as cervical cord compression and myelopathy

Question 400

What are some differentials for MND?

Answer 400

MS Polyneuropathies Myasthenia gravis GBS

Question 401

What is the management for MND

Answer 401

No treatment - large amount of neurones have died before symptoms present Riluzole prolongs survival by 2-4 months by protecting motor neuron damage form glutamate Respiratory support: patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months Supportive treatment: Antispasmodics: such as baclofen

Question 402

What is the main cause of death in motor neurones disease?

Answer 402

death 75-80% within 5 years - most commonly due to respiratory failure

Question 403

What is spinal muscular atrophy?

Answer 403

- genetic condition that makes the muscles weaker + causes problems with movement - gets worse over time - unstable SMN protein produced - muscle weakness appears symmetrical + proximal

Question 404

What is Myasthenia Gravis?

Answer 404

A chronic autoimmune disorder of the postsynaptic membrane at the neuromuscular junction of skeletal muscle

Question 405

When does Myasthenia Gravis affect men and women?

Answer 405

Symptoms peak in women in 20/30s Symptoms peak in men 50/60s

Question 406

What are the risk factors for developing Myasthenia Gravis?

Answer 406

Female 2x as common Autoimmune: linked to rheumatoid and SLE -Thymoma or thymic hyperplasia: 10-15% have a thymoma and 70% have thymic hyperplasia

Question 407

Describe the normal physiology of a neuromuscular junction

Answer 407

Axons of motor nerves are situated across a synapse from from the post-synaptic membrane on the muscle cell The axons release a neurotransmitter from the pre-synaptic membrane. This neurotransmitter is acetylcholine. ACh travels across the synapse and attaches to nicotinic receptors on the post-synaptic membrane stimulating muscle contraction

Question 408

What is the main cause of disease in Myasthenia Gravis?

Answer 408

Acetylcholine receptor antibodies present (85%) These bind to the receptors on the post-synaptic membrane and prevent ACh from binding and causing muscle contraction The problem is worsened during exercise as more of the receptors become blocked up. So the more the muscles are used the weaker they get. Gets better with rest These antibodies also activate the complement system which damages cells further making the problem worse

Question 409

What are the two other antibodies that can cause MG and how do they do it?

Answer 409

Muscle specific kinase (MuSK) low-density lipoprotein receptor-related protein 4 (LRP4). They are both proteins that are important for making the acetylcholine receptor. These antibodies lead to inadequate acetylcholine receptors causing MG

Question 410

What are the signs of MG?

Answer 410

Proximal muscle weakness: often affecting the face and neck Ptosis (drooping eyelid) Complex ophthalmoplegia: cannot be isolated to one cranial nerve Head drop: a rare sign due to weakness of cervical extensor muscles Myasthenic snarl: a ‘snarling’ expression when attempting to smile

Question 411

What are the symptoms of MG?

Answer 411

Muscle weakness that gets worse throughout the day Diplopia: double vision Slurred speech Fatigue in jaw while chewing

Question 412

What is are the investigations for MG?

Answer 412

Look for presence of autoantibodies: - Acetylcholine receptor (ACh-R) antibodies (85% of patients) - Muscle-specific kinase (MuSK) antibodies (10% of patients) - LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%) CT/MRI of thymus gland to look for thymoma

Question 413

What is another test that can be performed if unsure about MG?

Answer 413

Edrophonium test: patients given IV Edrophonium chloride (neostigmine). Will prevent breakdown of ACh by cholinesterase enzymes and improve symptoms temporarily

Question 414

What are the treatments for MG?

Answer 414

First-line: Reversible acetylcholinesterase inhibitors (neostigmine/pyridostigmine) Second-line: Immunosuppressants: prednisolone/azathioprine Consider monoclonal antibodies (rituximab) Thymectomy can also improve symptoms.

Question 415

What is the main complication of MG?

Answer 415

Myasthenic crisis often triggered by another illness such as respiratory tract infection . Causes an acute worsening of symptoms and can lead to respiratory failure

Question 416

What is the treatment for a Myasthenic crisis?

Answer 416

Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation. Medical treatment of myasthenic crisis is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.

Question 417

What is the main differential for MG?

Answer 417

Lambert-Eaton myasthenic syndrome

Question 418

What drugs should be avoided in MG?

Answer 418

Antibiotics; - aminoglycosides (e.g. gentamicin) - fluoroquinilones (e.g. ciprofloxacin) - Macrolides (e.g. azithromycin) - tetracyclines (e.g. doxycycline) - quinolones Neuromuscular blocking agents Magnesium sulfate penicillamine Cardiac drugs - BB neurological - lithium + phenytoin

Question 419

What is Lambert-Eaton myasthenic syndrome?

Answer 419

Lambert-Eaton myasthenic syndrome has a similar set of features to myasthenia gravis. It causes progressive muscle weakness with increased use as a result of damage to the neuromuscular junction. The symptoms tend to be more insidious and less pronounced than in myasthenia gravis.

Question 420

What causes Lambert-Eaton myasthenic syndrome?

Answer 420

Typically occurs in patients with SCLC. - It is a result of antibodies produced by the immune system against voltage gated calcium channels in SCLC but this also targets the pre-synaptic terminal These channels are responsible for assisting the release of ACh at the synapse

Question 421

What is the presentation of Lambert-Eaton myasthenic syndrome?

Answer 421

Proximal muscle weakness that develops more slowly All other same symptoms really e.g., double vision ptosis, jaw tiredness, slurred speech

Question 422

What is the difference clinically between Lambert-Eaton myasthenic syndrome and MG?

Answer 422

Lambert Eaton Syndrome symptoms tend to improve following a period of strong muscle contraction. Post Tetanic Potentiation

Question 423

What is the treatment for Lambert Eaton Syndrome?

Answer 423

Amifampridine - allows more ACh to be released in the neuromuscular junction synapses by blocking voltage gated potassium channels

Question 424

What are some other treatments for Lambert Eaton Syndrome?

Answer 424

Immunosuppressants (e.g. prednisolone or azathioprine) IV immunoglobulins Plasmapheresis

Question 425

What is syncope?

Answer 425

A term used to describe the event of temporarily losing consciousness due to disruption in blood flow. Known as vasovagal episodes

Question 426

What are the causes of primary syncope?

Answer 426

Dehydration Missed meals Extended standing in warm environment Vasovagal response to stiumli

Question 427

What are the causes of secondary syncope?

Answer 427

Hypoglycaemia Anaemia Hypovolaemia e.g. due to haemorrhage, GI bleeding, ruptured aortic aneurysm Infection Anaphylaxis Arrhythmias Valvular heart disease Hypertrophic obstructive cardiomyopathy Pulmonary embolism: causing hypoxia

Question 428

What are some risk factors for syncope?

Answer 428

Elderly Pregnant women Certain medications such as - that block vasoconstriction e.g. calcium channel blockers, beta blockers, alpha blockers, and nitrates Affect the volume status e.g. diuretics That prolong the QT interval e.g. antipsychotics and antiemetics

Question 429

What is a vasovagal episode (neurocardiogenic) syncope?

Answer 429

Caused by problems with the autonomic nervous system regulating blood flow to the brain When the vagus nerve receives strong stimulus it causes an increase in parasympathetic activation which causes vasodilation and reduction in BP and blood flow to brain leading to the loss of consciousness

Question 430

What is Carotid sinus hypersensitivity?

Answer 430

This occurs when mild external pressure on the carotid bodies in the neck is enough to induce this reflex response. - Mild pressure could be due to shaving, neck turning, tight collar etc

Question 431

What is s orthostatic hypotension syncope?

Answer 431

- A drop of blood pressure of more than 20mmHg or a reflex tachycardia of more than 20 beats. When a person goes from lying down to standing - It occurs when there’s a delay in constriction of the lower body veins, which is needed to maintain an adequate blood pressure when changing position to standing. As a result, blood pools in the veins of the legs for longer and less is returned to the heart, leading to a reduced cardiac output.

Question 432

What are the prodrome symptoms for syncope?

Answer 432

Hot or clammy Sweaty Heavy Dizzy or lightheaded Vision going blurry or dark Headache

Question 433

What is loss of consciousness defined as?

Answer 433

Suddenly losing consciousness and falling to the ground Unconscious on the ground for a few seconds to a minute as blood returns to their brain Twitching, shaking or convulsion activity, which can be confused with a seizure

Question 434

What are the primary investigations for syncope?

Answer 434

Clinical Hx and examination Ix to rule out pathological causes: Bloods - infection FBC - anaemia ECG- arrythmia Glucose - Hypoglycaemia B-hCG - ectopic pregnancy

Question 435

What is a mononeuropathy?

Answer 435

A process of nerve damage that affects a single nerve

Question 436

What is polyneuropathy?

Answer 436

Disorders of peripheral or cranial nerves whose distribution is usually symmetrical and widespread

Question 437

What is mononeuritis multiplex?

Answer 437

A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes

Question 438

What are the causes of mononeuritis multiplex?

Answer 438

WARDS PLC - Wegner’s granulomatosis - AIDS - Rheumatoid arthritis - Diabetes - Sarcoidosis - Polyarteritis nodosa - Leprosy - Carcinoma

Question 439

What is peripheral neuropathy?

Answer 439

Nerve pathology outside of the CNS that affects the peripheral nerves

Question 440

What are the mechanisms that cause peripheral neuropathy?

Answer 440

Demyelination Axonal damage Nerve compression

Question 441

What can cause peripheral neuropahty?

Answer 441

ABCDE A- Alcohol B- B12 deficiency C- Cancer and CKD D- Diabetes and drugs E- Every vasculitis

Question 442

What drugs can cause peripheral neuropathy?

Answer 442

amiodarone isoniazid vincristine nitrofurantoin metronidazole

Question 443

What are the main symptoms of peripheral neuropathy?

Answer 443

numbness and tingling in the feet or hands burning, stabbing or shooting pain in affected areas loss of balance and co-ordination muscle weakness, especially in the fee

Question 444

What is carpal tunnel syndrome?

Answer 444

Compression of the median nerve as it travels through the carap tunnel in the wrist

Question 445

What two things can cause carpal tunnel syndrome?

Answer 445

Swelling of the contents e.g. swelling of tendons putting pressure on nerve Narrowing of the tunnel

Question 446

Who is more commonly affected by carpal tunnel syndrome?

Answer 446

Females due to narrower wrists so more likely to have compression Over 30s

Question 447

Where does the median nerve provide sensory innervation for in the hand?

Answer 447

The palmar aspects and full fingertips of the: - Thumb - Index and middle finger - The lateral half of the ring finger Note that the palmar cutaneous branch of the median nerve provides sensation to the palm. However, this branch originates before the carpal tunnel and does not travel through the carpal tunnel. Therefore, it is not affected by carpal tunnel syndrome.

Question 448

Where does the median nerve provide motor function for in the hand?

Answer 448

The three thenar muscles which make up the muscular budge at the base of the thumb: - Abductor pollicis brevis (thumb abduction) - Opponens pollicis - Flexor pollicis brevis (thumb flexion) The other muscle that controls thumb movement is the adductor pollicis (thumb adduction). However, this is innervated by the ulnar nerve.

Question 449

What are the causes of carpal tunnel syndrome?

Answer 449

Most cases are idiopathic but can be caused by: - Repetitive strain - Obesity - Perimenopause - RA - Diabetes - Acromegaly - Hypothyroidism If it is bilateral then think underlying cause

Question 450

What are the sensory symptoms of carpal tunnel syndrome?

Answer 450

Numbness Paraesthesia (pins and needles or tingling) Burning sensation Pain worse at night wakes from sleep This occurs in areas innervated so most of the palmar aspects of everything but little finger. Patients may shake hand to relieve pain

Question 451

What are the motor symptoms of carpal tunnel syndrome?

Answer 451

Weakness of thumb movements Weakness of grip strength Difficulty with fine movements involving the thumb Weakness of the thenar muscles

Question 452

What are the two tests for carpal tunnel syndrome?

Answer 452

Phalen’s test Tinel’s test Durkan’s test

Question 453

What is Phalen’s test?

Answer 453

Involves fully flexing the wrist and holding it in this position. Often this is done by asking the patient to put the backs of their hands together in front of them with the wrists bent inwards at 90 degrees. The test is positive when this position triggers the sensory symptoms of carpal tunnel, with numbness and paraesthesia in the median nerve distribution.

Question 454

What is Tinel’s test?

Answer 454

Involves tapping the wrist at the location where the median nerve travels through the carpal tunnel. This is in the middle, at the point where the wrist meets the hand. The test is positive when this position triggers the sensory symptoms of carpal tunnel, with numbness and paraesthesia in the median nerve distribution.

Question 455

What is the management for carpal tunnel syndrome?

Answer 455

Rest and altered activities Wrist splints that maintain a neutral position of the wrist can be worn at night (for a minimum of 4 weeks) Steroid injections Surgery (last resort)

Question 456

What are the nerve roots of the radial nerve?

Answer 456

C5-T1

Question 457

What is the classical presentation of a radial nerve palsy?

Answer 457

wrist drop

Question 458

What can cause wrist drop?

Answer 458

Damage to the radial nerve e.g. mid shaft fracture of humerus or compression of radial nerve at humerus

Question 459

What is the classical presentation of an ulnar nerve palsy?

Answer 459

Claw hand (4th/5th fingers claw up)

Question 460

What is the sign of a Sciatic/Common peroneal nerve Palsy?

Answer 460

Foot drop

Question 461

What is Charcot-Marie-Tooth disease?

Answer 461

A group of diseases that affect the peripheral motor and sensory nerves

Question 462

What are the most common forms of Charcot-Marie-Tooth disease?

Answer 462

CMT1 and CMT2

Question 463

What inheritance pattern are CMT1 and CMT2?

Answer 463

Autosomal dominant

Question 464

What causes CMT1?

Answer 464

Caused by mutations in the PMP22 and MPZ genes which encode proteins that are part of the myelin sheath made by Schwan cells. Loss of myelin slows down the transmission of nerve impulses. Over time Schwann cells try and replace myelin. As a result, under a microscope, there’s often onion bulb formation - the axon is surrounded by layers of new myelin with underlying damaged layers of myelin.

Question 465

What causes CMT2?

Answer 465

Caused by mutations in MFN2 gene which encodes for a protein in neuronal mitochondria. When the protein is defective mitochondrial function is disrupted leading to neuronal death

Question 466

What are the affects of CMT1 and CMT2?

Answer 466

When motor neurones are affected muscles begin to atrophy. When sensory neurones are affected it first affects the feet and toes as those are the longest axons and most sensitive to damage

Question 467

What are the signs of CMT1 and CMT2?

Answer 467

Pes cavus: high foot arches Hammer toes Distal muscle wasting Hand and arm muscle wasting Thickened palpable nerves

Question 468

What are the symptoms of CMT?

Answer 468

Weakness in the lower legs, particularly loss ofankle dorsiflexion Foot drop High-stepped gait Weakness in the hands Reduced tendon reflexes Reduced muscle tone Peripheral sensory loss Tingling and burning sensations in the hands and feet May be neuropathic pain

Question 469

What are the investigations for CMT?

Answer 469

Nerve conduction studies: measures ability of nerves to conduct impulses Neurologists and geneticists make the diagnosis by looking for mutations

Question 470

What is Duchenne’s muscular dystrophy?

Answer 470

A genetic condition that cause gradual weakening and wasting of muscles.

Question 471

What is another type of muscular dystrophy?

Answer 471

Becker’s

Question 472

What is the inheritance pattern of Duchenne’s muscular dystrophy?

Answer 472

X-linked recessive

Question 473

What causes Duchenne’s muscular dystrophy?

Answer 473

There is a mutation which codes for the dystrophin protein which is used to stabilise the muscle fibres. Without it the sarcolemma wilts and becomes unstable. Overtime cellular proteins e.g. creatinine kinase starts escaping the damaged cell and calcium starts to enter the cell leading to its death In the short term there is muscle regeneration resulting in muscle fibres of different sizes but in the long term the muscles atrophy and are infiltrated by fat and fibrotic tissue making them really weak

Question 474

What will a muscle biopsy in Duchenne’s muscular dystrophy show?

Answer 474

Biopsy of the tissue show changes in the muscle itself but not in the nerves which is used to distinguish it between neuropathies

Question 475

What are the symptoms of Duchenne’s muscular dystrophy?

Answer 475

Symptoms usually present at around 3-5: - Weakness is muscles around pelvis - Walking late - Waddling gait - Enlarged calves

Question 476

What is Gower’s sign?

Answer 476

Children with proximal muscle weakness use a specific technique to stand up from a lying position. This is called Gower’s sign. To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect. This is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.

Question 477

What are the investigation for DMD?

Answer 477

High creatinine kinase level Genetic testing looking for dystrophin mutations (can be diagnostic) Muscle biopsy Electromyogram: distinguish between neuropathic and myopathic pathology

Question 478

What are the treatments for DMD?

Answer 478

Occupational therapy Oral steroids have been shown to slow the progression of muscle weakness Creatine supplementation can give an improvement in muscle strength

Question 479

What are the complications of DMD?

Answer 479

Respiratory failure because of a weak diaphragm Scoliosis Dilated cardiomyopathy and arrhythmias: dystrophin protein is also expressed in heart muscle.

Question 480

What is the life expectancy for DMD?

Answer 480

Duchenne’s muscular dystrophy: patients have a life expectancy of around 25 – 35 years with good management of the cardiac and respiratory complications. Becker’s muscular dystrophy has a better prognosis

Question 481

What are the different types of brain tumour?

Answer 481

Gliomas Meningiomas Pituitary Hemangioblastoma Acoustic neuroma Accounts for less than 2% of all malignant tumours but 20% of childhood cases

Question 482

What are glioblastomas?

Answer 482

They are tumours in the brain or spinal cord there are 3 types

Question 483

What are the 3 types of glioblastoma?

Answer 483

Astrocytoma Glioblastoma multiformeis the most common Oligodendroglioma Ependymoma

Question 484

What is an astrocytoma?

Answer 484

Glioblastoma multiformeis the most common Most commonly found in cerebral hemisphere Histologically has pseudo-palisading pattern - peripheral tumour cells lined up around necrotic centre Grade IV (most malignant)

Question 485

What is an Oligodendroglioma?

Answer 485

Most common in 40s-50s Adult oligodendrogliomas typically form in the frontal lobes Categorised as grade II or III, slow-growing tumours, Histologically, prominent features can vary from fairly small, round nuclei, surrounded by well-defined “halos” or thick white borders of cytoplasm giving them a “fried egg” appearabce

Question 486

What is an Ependymoma?

Answer 486

Arise from ependymal cells (form the epithelial lining of the ventricles in the brain and the central canal of the spinal cord)

Question 487

What is a Meningioma?

Answer 487

They grow from cells found in the arachnoid mater of the meninges They are usually benign and this can lead to raised intercranial pressure and lead to the neurological symptoms These tumours may also cause the formation of calcifications called psammoma bodies

Question 488

What are the symptoms of brain tumours?

Answer 488

They are initially asymptomatic . - As they develop they present with **focal neurological symptoms. - Will show signs of raised ICP

Question 489

What are the focal symptoms of a frontal lobe tumour?

Answer 489

Hemiparesis personality change, Broca’s dysphasia, lack of initiative, unable to plan tasks

Question 490

What are the focal symptoms of a temporal lobe tumour?

Answer 490

Dysphagia Amnesia

Question 491

What are the focal symptoms of a parietal lobe tumour?

Answer 491

Hemisensory loss Dysphasia reduction in 2-point discrimination astereognosis (unable to recognise object from touch alone

Question 492

What are the focal symptoms of a cerebellum tumour?

Answer 492

DASHING - - Dysdiadochokinesis (impaired rapidly alternating movement), - Ataxia, - Slurred speech (dysarthria), - Hypotonia, - Intention tremor, - Nystagmus, - Gait abnormality

Question 493

What are the symptoms of raised ICP?

Answer 493

Headache Vomiting Visual field defect Seizures Papilloedema (on fundoscopy)

Question 494

What are the investigations for a brain tumour?

Answer 494

CT/ MRI Blood tests e.g. FBC, U&Es, LFT’s, B12 Tissue biopsy: to determine cancer grade and guide management

Question 495

What is used to grade brain tumours?

Answer 495

Severity is classified by the World Health Organisation’s (WHO) scale The scale goes from I to IV based on the morphologic and functional features of the tumour cells; a grade IV tumour being the most abnormal looking cells that also tend to be the most aggressive.

Question 496

How common are secondary brain tumours?

Answer 496

Brain metastasis affects up to 40% of patients with cancer 10 times more common than primary brain tumours

Question 497

What are the most common metastases of secondary brain tumours?

Answer 497

Lung Breast Melanoma Renal cell GI

Question 498

What is ataxia?

Answer 498

problems with balance and coodination - heterogenous group of disorders - many different causes - majority permanent and progressive

Question 499

What are the types of ataxia?

Answer 499

Inherited - autosomal dominant =SCA6 and EA2 - autosomal recessive = Friedreich's ataxia, - X-linked - Mitochondrial - metabolic Acquired - toxic/metabolic = alcohol, vitamin deficiencies - immune mediated = paraneoplastic cerebellar degeneration, gluten related - infective = post infectious - degenerative = multi-system atrophy cerebellar variant - structural

Question 500

What are the symptoms of cerebellar dysfunction?

Answer 500

- dizzy - falls, stumble - difficulty focusing/double vision/'oscillopsia' ( - slurred speech - problems with swallowing - tremor - problems with dexterity/ fine motor skills

Question 501

What are the clinical signs of cerebellar dysfunction?

Answer 501

- nystagmus - dysarthria - intention tremor/myoclonus - dysmetria (under/overshoot of movements) - heel - shin ataxia - wide gait/limb/truncal ataxia - tone/reflexes

Question 502

What is the scale for ataxia?

Answer 502

Scale for the assessment and rating of ataxia (SARA) - mild = mobilising independently or with one walking aid - moderate = mobilising with 2 walking aids or walking frame - severe = predominantly wheelchair dependent

Question 503

What are the investigations for ataxia?

Answer 503

Blood = FBC, LFTs, ESR, CRP, Gluten, B12, HbA1c, folate HLA typing for DQ2/DQ8 MRI brain = demonstrate cerebellar atrophy CT if MRI contraindicated

Question 504

What is the management of cerebellar ataxia?

Answer 504

no cure so tailored to the individual patient - specialist services

Question 505

What is the Cushing Relfex?

Answer 505

A physiological nervous system response to increased intracranial pressure Triad: 1. Hypertension 2. Bradycardia 3. Irregular breathing

Question 506

Explain the 3 stages of the Cushing Reflex

Answer 506

1. Sympathetic stage = ICP -> sympathetic stimulation -> vasoconstriction -> hypertension 2. parasympathetic stage = stimulate baroreceptors in aortic arch -> reduce heart rate 3. ICP -> press on resp centre in brain stem -> irregular breathing

Question 507

Why is there an increase in intracranial pressure after a TIA/stroke?

Answer 507

Ischaemia + necrosis of brain cells -> inflammation + swelling --> increase ICP --> press against blood vessels

Question 508

Define tremor

Answer 508

involuntary, rhythmic oscillatory movement of a body part

Question 509

what are the cause of tremors?

Answer 509

- physiological, - drugs, - metabolic (hyperthyroidism), - essential tremor = puts hands out in front of them and can see tremor, carrying cup, faster then PD, family history (autosomal dominant), archimedes spiral (on axis, diagonal) - dystonic tremor = jerky, variable axis, may be task specific, little finger hyperextended, head tremor - functional tremor = distractibility (reduce tremor if asked to perform motor task), entrainability (Train tremor to follow rhythm if following someone else’s movement) , variability, unusual pattern, no 2 spirals are the same

Question 510

Define dystonia

Answer 510

movement disorder characterised by sustained or intermittent muscle contractions causing abnormal, repetitive, movements, postures or both

Question 511

What are the features and causes of dystonia?

Answer 511

Cause: - Idiopathic - Functional - Genetic = usually starts in childhood in a lower limb - Secondary Can affect any part of the body or if after stroke can affect one side of body or focal (writers cramp, cervical,) - Task specific - Stop when they touch their face - Spontaneous remission - Exacerbation with stress

Question 512

Define chorea

Answer 512

involuntary, irregular, flowing movement that doesn’t follow a pattern (figidity + restless) e.g. Huntington's

Question 513

Define Tic

Answer 513

brief stereotyped movements or vocalisations that resemble voluntary actions

Question 514

Define myoclonus

Answer 514

lightning fast, jerky involuntary movements - Positive - movement contraction - negative - loss of tone, in liver cirrhosis = asterixis

Question 515

What is the difference between an organic and functional neurological movement?

Answer 515

Organic = follows a set pattern and fits the definition Functional = more fluid and doesn't fit a set definition, can't be classed as one thing

Question 516

WHat is the main medication for depression?

Answer 516

SSRI - Selective Serotonin Reuptake Inhibitors e.g. fluoxetine

Question 517

What is St John's Wort and why does it need to be considered when prescribing medication?

Answer 517

Herbal medication - potent inducer of cytochrome P450 - increases potency = SSRIs - decreases effect = Contraceptive pill, warfarin, statins, digoxin, anticonvulsants, HIV medication