Neurology III

Question 2

Describe the feature of a migraine without aura [5+]

Answer 2

At least five attacks fulfilling criteria B-D:

B. Headache attacks lasting 4-72 hr (untreated or unsuccessfully treated)

C. Headache has at least two of the following four characteristics:
* unilateral location
* pulsating quality
* moderate or severe pain intensity
* aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)

D. During headache at least one of the following:
* nausea and/or vomiting
* photophobia and phonophobia

E. Not better accounted for by another ICHD-3 diagnosis.

Question 3

A headache meets the diagnostic criteria for migraine with aura if: [4+]

Answer 3

At least two attacks fulfilling criteria 2 and 3

B. One or more of the following fully reversible aura symptoms:
* visual
* sensory
* speech and/or language
* motor
* brainstem
* retinal

C. At least three of the following six characteristics:
* at least one aura symptom spreads gradually over ≥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

D. Not better accounted for by another ICHD-3 diagnosis.

Question 4

Describe what aura symptoms are like [4]

Answer 4

Aura can affect vision, sensation or language. Visual symptoms are the most common. These may be:

Sparks in the vision
Blurred vision
Lines across the vision
Loss of visual fields (e.g., scotoma)

Question 5

Describe the acute treatment for migraine [+]

Answer 5

first-line: offer combination therapy with
* an oral triptan and an NSAID, or
* an oral triptan and paracetamol
* for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan
* if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide or prochlorperazine and consider adding a non-oral NSAID or triptan

In the UK, new calcitonin gene-related peptide (CGRP) inhibitors may be used in acute migraine in patients who fail to respond to triptans or other standard treatments, or that these treatments are not tolerated or contraindicated.
- The main drug licensed for this indication is Rimegepant, which can be taken orally.
- These medications bind to the CGRP receptor and subsequently block attachment of CGRP which is a potent vasodilator that can amplify and perpetuate migraine headache pain.

Question 6

Describe the prophylactic treatment for migraines [6+]

Answer 6

Usual:
* Propranolol
* Topiramate (contraindicated in pregnancy, the BNF states in women with childbearing potential ‘a highly effective’ contraception is required prior to commencement)
* Amitriptyline

More specialist options include:
* Pizotifen
* Candesartan
* Sodium valproate

Preventative treatment (newer agents)
* Erenumab (Aimovig): monoclonal antibody that blocks the CGRP receptor.
* Fremanezumab (Ajovy): monoclonal antibody that binds to the CGRP ligand preventing receptor activation
* Galcanezumab (Emgality): onoclonal antibody that binds to the CGRP ligand preventing receptor activation

Question 7

When are newer preventative treatment agents indicated for use? [2]

When should they be considered for continued treatment? [2]

Answer 7

In general, these drugs have specific guidance around their prescribing as per NICE. They may be given to patients who:
* have 4 or more migraine days a month
* have tried at least 3 other medicines and they have not worked

In addition, they should only be continued if after 12 weeks:
* episodic migraine (less than 15 headache days a month) reduced by at least 50%
* chronic migraine (15 headache days a month or more) reduced by at least 30%

Question 8

What is status migrainosus? [1]

Answer 8

: a debilitating migraine that persists for longer than 72 hours.

Question 9

Prophylactic [drug class] (e.g., [2]) are an option for menstrual migraines.

Symptoms tend to occur two days before until three days after the start of menstruation.

Answer 9

Prophylactic triptans (e.g., frovatriptan or zolmitriptan) are an option for menstrual migraines. Symptoms tend to occur two days before until three days after the start of menstruation. Regular triptans may be taken during this time.

Question 10

Which one of the following is a contraindication to the use of a triptan in the management of migraine?

Concurrent pizotifen use
Patients older than 55 years
A history of epilepsy
Previous intracranial tumour
A history of ischaemic heart disease

Answer 10

Which one of the following is a contraindication to the use of a triptan in the management of migraine?

A history of ischaemic heart disease
- They cause vasoconstriction of cranial blood vessels and inhibit the release of vasoactive peptides. However, they can also cause coronary artery vasospasm and are therefore contraindicated in patients with a history of ischaemic heart disease due to the increased risk of myocardial infarction.

Question 11

Describe what is meant by Foster kennedy syndrome [1]

Answer 11

unilateral optic atrophy (vision loss in one eye) and contralateral papilledema (swelling of the optic disc in the other eye), often caused by a space-occupying lesion in the brain compressing the optic nerve

Question 12

A 12-year-old child developed headaches, vomiting and a staggering gait. A cerebellar neoplasm was diagnosed.

Which is the most common cerebellar neoplasm of childhood?

Ependymoma
Glioblastoma multiforme
Neuroblastoma
Astrocytoma
Oligodendroglioma

Answer 12

Astrocytoma

Question 13

What is the histology like of a meningioma? [1]

Answer 13

• Histology: Spindle cells in concentric whorls and calcified psammoma bodies

Question 14

State and explain the standard investigation for acromegaly? [1]

Name two others [2]

Answer 14

OGTT:
- Make patient fast
- At time 0, check glucose and GH
- Give 75g dose of glucose and wait 2hrs
- Normal response: suppression of GH when glucose given
- Acromegaly response: GH increases despite glucose given

Insulin-like growth factor-1 (IGF-1):
can be tested on a blood sample. It indicates the growth hormone level and is raised in acromegaly.

MRI pituitary

Testing growth hormone directly is unreliable: fluctuates in the day.

Question 15

Describe levels of Ca2+, PO4- and glucose in acromegaly [3]

Answer 15

All raised

Question 16

Name three drug therapies for acromegaly? [3]

Answer 16

Octreotide - somatostatin analogue: lowers GH levels / blocks GH release

Pegvisomont – GH receptor antagonist; subcutaneous injection

Bromocriptine (Dopamine agonists): block growth hormone release

Question 17

What is the first line treatment for acromegaly? [1]

Answer 17

Trans–sphenoidal surgery

Question 18

Name and explain the diagnostic test of choice for Cushing’s syndrome

Answer 18

Normal: dexamethasone suppresses cortisol release from adrenal glands

Cushings: cortisol levels are high despite dexamethasone suppressing cortisol release

Question 19

Management of Cushing’s syndrome:

• Surgery? [1]
• Drugs? [2]

Answer 19

 Trans-sphenoidal surgery

Adrenolytics:
 Ketoconazole: causes steroidogenesis inhibition.
 Metyrapone: reduces the production of cortisol in the adrenals and is occasionally used in treating of Cushing’s

Question 20

There are three types of dexamethason suppression test.

Describe them [3]

Answer 20

Low-dose overnight test (used as a screening test to exclude Cushing’s syndrome)
- A normal result is that the cortisol level is suppressed.
- Failure of the dexamethasone to suppress the morning cortisol could indicate Cushing’s syndrome, and further assessment is required. THINK CAPE

Low-dose 48-hour test (used in suspected Cushing’s syndrome)
- 0.5mg is taken every 6 hours for 8 doses, starting at 9 am on the first day.
- Cortisol is checked at 9 am on day 1 (before the first dose) and 9 am on day 3 (after the last dose)
- A normal result is that the cortisol level on day 3 is suppressed
- Failure of the dexamethasone to suppress the day 3 cortisol could indicate Cushing’s syndrome, and further assessment is required.

High-dose 48-hour test (used to determine the cause in patients with confirmed Cushing’s syndrome)
- carried out the same way as the low-dose test, other than using 2mg per dose (rather than 0.5mg).
- This higher dose is enough to suppress the cortisol in Cushing’s disease, but not when it is caused by an adrenal adenoma or ectopic ACTH.

Question 21

Answer 21

Question 22

Answer 22

Question 23

Answer 23

Question 24

Answer 24

Question 25

How do you calculate CPP / cerebral perfusion pressure? [1] What is normal CPP? [1]

Answer 25

**CPP = MAP – ICP** - CPP normally **60-80 (150) mmHg**

Question 26

Describe the clinical features and investigations for EDH [4]

Answer 26

patient who **initially** **loses**, briefly **regains** and then **loses** **again** **consciousness** after a **low-impact head injury.** - The brief regain in consciousness is termed the '**lucid** **interval**' and is **lost eventually** due to the **expanding haematoma and brain herniation**. - As the **haematoma** **expands** the **uncus of the temporal lobe** **herniates** around the **tentorium** **cerebelli** and the **patient develops a fixed and dilated pupil** due to the **compression** of the **parasympathetic fibers of the third cranial nerve.** **On imaging:** - An extradural haematoma appears as a **biconvex (or lentiform)**, hyperdense collection around the surface of the brain. **They are limited by the suture lines of the skull.** - AKA lemon

Question 27

SDH is caused by damage to which blood vessels in the brain? [1]

Answer 27

Subdural haemorrhage occurs between the dura mater and arachnoid mater and is caused by a **rupture of the bridging veins in the outermost meningeal layer**. On a CT scan, they have a crescent shape and are not limited by the cranial sutures (they can cross over the sutures).

Question 28

Describe the differences in clinical features between acute and chronic SDH

Answer 28

An **acute subdural haematoma** is a collection of fresh blood within the subdural space and is **most commonly caused by high-impact trauma**. - Since it is associated with high-impact injuries, there is often other brain underlying brain injuries. A **chronic subdural haematoma** is a collection of blood within the subdural space that has been present for **weeks to months.** - Rupture of the small bridging veins within the subdural space rupture and cause slow bleeding. **Elderly and alcoholic patients are particularly at risk of subdural haematomas since they have brain atrophy** and therefore **fragile or taught bridging veins.** - Presentation is typically a several week to month progressive history of either **confusion, reduced consciousness or neurological deficit.**

Question 29

Acute and chronic SDH Mx? [2]

Answer 29

**Acute subdural haematoma** - Small or incidental acute subdurals can be **observed conservatively.** - **Surgical options** include **monitoring of intracranial pressure** and **decompressive craniectomy.** **Chronic subdural haematoma** - If the chronic subdural is an **incidental** **finding** or if it is **small in size** with no associated neurological deficit then it can be **managed conservatively with the hope that it will dissolve with time.** - If the patient is **confused**, has an associated **neurological** **deficit** or has **severe imaging findings** then **surgical decompression with burr holes is required.**

Question 30

What are the two types of SAH? [2] What causes them? [+]

Answer 30

The most common cause of SAH is **head injury and this is called traumatic SAH** In the absence of trauma, SAH is termed **spontaneous SAH** . The rest of this note focuses on spontaneous SAH. * **Intracranial aneurysm (saccular 'berry' aneurysms)** accounts for around 85% of cases * conditions associated with berry aneurysms include **hypertension,adult polycystic kidney disease, Ehlers-Danlos syndrome and coarctation of the aorta** * **arteriovenous malformation** * **pituitary apoplexy** * **mycotic (infective) aneurysms**

Question 31

Describe the clinical features of SAH [+]

Answer 31

**headache** * usually of sudden-onset ('thunderclap' or 'hit with a baseball bat') * severe ('worst of my life') * occipital * typically peaking in intensity within 1 to 5 minutes * there may be a history of a less severe 'sentinel' headache in the weeks prior to the presentation **nausea and vomiting** **meningism (photophobia, neck stiffness)** **altered consciousness:** Levels can range from slight drowsiness to coma, indicative of the severity of the haemorrhage or associated complications like hydrocephalus **focal neurological signs** * **cranial nerve palsies**: particularly affecting the **third, fourth, and sixth cranial nerves**, leading to visual disturbances or diplopia * **hemiparesis or hemiplegia**: indicative of extensive bleeding or secondary ischaemia speech disturbances: aphasia may occur if the dominant hemisphere is affected. seizures

Question 32

Examination fndings of SAH? [4] ECG Changes? [2]

Answer 32

**fundoscopy**: - **subhyaloid haemorrhages** may be visible **meningeal signs**: positive Kernig's or Brudzinski's signs may be elicited **papilloedema**: though uncommon, may be present indicating raised intracranial pressure vital signs: **hypertension** is commonly observed; however, **hypotension** is a **poor** **prognostic** sign **ECG changes:** * **transient** ECG changes including **ST elevation** may be seen * this may be secondary to either autonomic neural stimulation from the hypothalamus or elevated levels of circulating catecholamines

Question 33

Ix for SAH? [3]

Answer 33

**non-contrast CT head** is the first-line investigation of choice - if **CT head is done within 6 hours of symptom onset** and is **normal**: consider an alternative diagnosis (DON'T do an LP) - if **CT head is done MORE than 6 hours** after symptom onset and is **normal**: **DO an LP** (should occur within 12hrs) **if the CT shows evidence of a SAH** * **referral** to **neurosurgery** to be made as soon as SAH is confirmed After **spontaneous SAH is confirmed**, the aim of investigation is to **identify a causative pathology** that needs urgent treatment: * **CT intracranial angiogram** (to identify a vascular lesion e.g. aneurysm or AVM) * **+/- digital subtraction angiogram (catheter angiogram)**

Question 34

Describe the Mx of a confirmed aneurysmal subarachnoid haemorrhage [3]

Answer 34

**supportive** * bed rest * analgesia * venous thromboembolism prophylaxis * discontinuation of antithrombotics (reversal of anticoagulation if present) **vasospasm** is prevented using a course of **oral nimodipine** **intracranial aneurysms are at risk of rebleeding** and therefore require **prompt intervention**, preferably within 24 hours * most intracranial aneurysms are now treated with a **coil by interventional neuroradiologists**, but a minority require a **craniotomy** and clipping by a neurosurgeon

Question 35

Complications of aneurysmal SAH? [5]

Answer 35

**re-bleeding** * happens in around 10% of cases and most common in the first 12 hours * if rebleeding is suspected (e.g. sudden worsening of neurological symptoms) then a repeat CT should be arranged * associated with a high mortality (up to 70%) **hydrocephalus** * hydrocephalus is temporarily treated with an external ventricular drain (CSF diverted into a bag at the bedside) or, if required, a long-term ventriculoperitoneal shunt **vasospasm** (also termed delayed cerebral ischaemia), typically 7-14 days after onset * ensure euvolaemia (normal blood volume) * consider treatment with a vasopressor if symptoms persist **hyponatraemia** (most typically due to syndrome inappropriate anti-diuretic hormone (**SIADH**)) **seizures**

Question 36

Describe the current NICE guidelines on CT imaging for head trauma [+]

Answer 36

Question 37

Head trauma comes to A&E. What would be an alarming sign and why?

Answer 37

**Unequal pupils** ---> Alarming sign , **indicates increased ICP & asymmetric mass effect.**

Question 38

A patient with head trauma comes in with unequal pupils. What would be next immediate appropriate steps? [5]

Answer 38

**Measures to lower high ICP:** * **Head elevation** (30 degrees) * **Remove neck restrains** (unless unstable spine injury) * **Hyperventilation** (pCO2: 4-4.5 kpa) for **cerebral vasoconstriction** * **Mannitol, hypertonic saline**

Question 39

Describe what the Cushing reflex is and what it is composed of [3]

Answer 39

**physiological response to increased intracranial pressure** (ICP), characterized by the Cushing triad: **hypertension, bradycardia, and irregular respirations**

Question 40

How do you tx Cushing's triad? [

Answer 40

**Mannitol**, an osmotic diuretic medication, is often provided intravenously and can be highly effective in lowering ICP and increasing CPP **diuretics** (e.g., furosemide), **steroids** (e.g., methylprednisolone), and **sedatives** (e.g., propofol) Rarely, a **craniotomy**, or removal of a small portion of the skull, may be performed to alleviate the rising pressure.

Question 41

Describe the pathophysiology of Cushing's reflex [+]

Answer 41

As the **ICP begins to increase,** it eventually becomes **greater** than the **mean arterial pressure** (i.e., average pressure in arteries during a heartbeat), which typically **must be greater than the ICP in order for the brain tissue to be adequately oxygenated**. - This **difference in pressure** causes a **decrease** in the **cerebral perfusion pressure (CPP)**, or the **amount of blood and oxygen the brain** is receiving, therefore leading to **brain ischemia, or inadequate blood supply to the brain** - To **compensate** for the **lack of oxygen, the sympathetic nervous system** is activated, causing an **increase in systemic blood pressure** and an **initial increase in heart rate.** - The **increased blood pressure** then signals the **carotid** and **aortic** **baroreceptors** to **activate the parasympathetic nervous system**, causing the **heart rate to decrease.** - As the **pressure in the brain continues to rise, the brainstem** may start to **dysfunction**, resulting in **irregular respirations** followed by periods where breathing ceases completely.

Question 42

State what type of herniation A-D are [4]

Answer 42

A: **Cingulate** (Subfalcine) B: **Central** (**transtentorial**) C: **Uncal** D: Downward cerebellar (**Tonsillar**)

Question 43

What type of herniation is depicted? Uncal Central (transtentorial) Cingulate (Subfalcine) Transcalvarial Upward cerebellar (transtentorial) Downward cerebellar (Tonsillar)

Answer 43

What type of herniation is depicted? Uncal Central (transtentorial) **Cingulate (Subfalcine)** Transcalvarial Upward cerebellar (transtentorial) Downward cerebellar (Tonsillar)

Question 44

What is a Chiari malformation? [1]

Answer 44

describes the downward displacement, or **herniation**, of the **cerebellar tonsils** through the **foramen magnum**. Malformations may be **congenital or acquired through trauma.**

Question 45

What are the features of a Chiari malformation? [3]

Answer 45

**Features** * non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow * headache * syringomyelia

Question 46

What is a Cavernoma? [1]

Answer 46

They're sometimes known as cavernous angiomas, cavernous haemangiomas, or cerebral cavernous malformation (CCM). A typical cavernoma looks like a raspberry. It's filled with blood that flows slowly through vessels that are like "caverns".

Question 47

What is normal ICP [1] Was can a ICP of greater than cause herniation? [1]

Answer 47

Normal adult **ICP: 5 - 15 mmHg** (supine position). A mass lesion with an ICP **of ≥ 20 mmHg can cause herniation.**

Question 48

Name the 6 types of brain herniation

Answer 48

Uncal Central (transtentorial) Cingulate (Subfalcine) Transcalvarial Upward cerebellar (transtentorial) Downward cerebellar (Tosillar)

Question 49

What is the difference between decorticate and decerebrate brain injury location and presentation? [4]

Answer 49

**Decerebrate**: damage to midbrain, brainstem and/or pons – anything below the red nucleus . **Decorticate**: damage to cerebral cortex and/or internal capsule.

Question 50

# 5- Acute management of TBI (**Severe TBI**) Which drugs can you use to start seizure prophylaxis? [2] Which drugs can you use to induce coma? [2]

Answer 50

Start on seizure prophylaxis: **phenytoin/levetiracetam** Sedation/Induce coma with **propofol or benzodiazepines**

Question 51

How could you manage severe ICP: Acutely [2] Long term [1]

Answer 51

Short term: * **mannitol** * **hypertonic saline** (shift of water from extravascular space to intravascular space across the BBB-controversy which therapy is better.) Long term: * **extraventricular drain/ external ventricular drain (EVD)** or **ventriculostomy**