Neurology IV

Question 1

Describe the risk factors for CVT [5]

Answer 1

Prothrombotic condition: Most common risk factor seen in over 40% of CVT cases
* Genetic thrombophilias including antiphospholipid syndrome; antithrombin III deficiency; protein C deficiency; protein S deficiency; Factor V Leiden mutation and Hyperhomocysteinemia
* Acquired thrombophilias such as pregnancy and the puerperium, oral contraceptive pill use and malignancy

Infection: most commonly Staphylococcus aureus spread from infections of the sinuses. May also be caused by meningitis or a subdural empyema

Trauma & Surgery

Chronic inflammatory diseases
- SLE
- Behcet
- GPA
- Sarcoidosis

Haematological disorders:
- such as paroxysmal nocturnal haemoglobinuria; thrombotic thrombocytopenic purpura, sickle cell disease and polycythemia

Question 2

Describet the primary and secondary mechanisms of injury in CVT

Answer 2

Primary mechanism of injury:
- cerebral vein or sinus becomes partially or totally occluded by a venous thrombus, deoxygenated blood will begin to pool within the brain parenchyma
- Causes an increase in cerebral venous pressure which has 3 effects: decreased cerebral perfusion & therefore parenchymal injury and cytotoxic oedema; disruption of BBB, causing vasogenic oedma (blood plasma into interstitial space); cerebral vein and capillary rupture

Secondary mechanism of injury:
- Obstruction of the superior sagittal, jugular or lateral venous sinuses causes decreased cerebrospinal fluid reabsorption.
- This will ultimately result in raised intracranial pressure

NB: In most cases of cerebral venous thrombosis (CVT), both the cerebral veins and the sinuses are involved.

Question 3

Describe the clinical features of CVT [+]

Answer 3

Isolated intracranial hypertension (90%):
- Headache is the most frequent symptom experienced by CVT patients. It is typically subacute in onset and can be generalised or focal, and is often worse with positional or postural changes
- Papilloedema and visual disturbances are also commonly seen in this syndrome

Focal neurological abnormalities (45%):
- May include motor weakness (e.g. hemiparesis), fluent aphasia; and sensory/visual field defects

Seizures (35%):
- Focal and generalised seizures may occur, as may status epilepticus.

Encephalopathy:
- Typically seen in severe cases of CVT or with straight sinus thrombosis. Causes reduced GCS, cognitive dysfunction and delirium/confusion

Question 4

Describe the acute treatment for CVT [3]

Answer 4

Acute antithrombotic therapy:
- low molecular weight heparin or unfractionated heparin in the majority of cases to recanalise the venous / sinous occlusion. Most recover with this
- Some need fibrinolysis (but big risk of intracranial haemorrhage so needs further discussion)
- Patients who still deteriorate despite optimal anticoagulation may require surgical thrombectomy, although this is rare.

Tx of complications:

Raised intracranial pressure:
- due to the high risk of herniation and subsequent patient death, raised intracranial pressure must be treated urgently.
- Patients should have the bed elevated, have osmotic therapy (mannitol or hypertonic saline) administered and be hyperventilated in an intensive care setting. Brain herniation may need emergency decompressive surgery

Seizures:
- anticonvulsants can be used both to treat seizures, and also as prophylaxis against seizures in patients deemed at high risk on neuroimaging review (large areas of cerebral oedema or infarction)

infection/inflammation:
- antibiotic treatment for infection and glucocorticoid therapy for those with inflammatory disorders is often used

Question 5

Describe the long term treatment for CVT - How does it differ for provoked vs unprovoked CVT? [2]

Answer 5

All patients with confirmed CVT require long-term anticoagulation with warfarin with an INR target of 2.5.
- This is for 3-6 months in provoked CVT and 6-12 months in those with an unprovoked CVT.
- Women who previously were taking the oral contraceptive pill will need advice regarding non-oestrogen methods of contraception such as the progesterone-only pill

Question 6

During the acute phase (first 30-days) there is a 5% mortality rate amongst patients. Death occurs as a result us: [4]

Answer 6

Transtentorial herniation from large venous haemorrhage (most common cause)
Diffuse cerebral oedema
Status epilepticus
Pulmonary embolism

Question 7

Symptoms of IIH are usually gradual and progressive, including: [+]

Answer 7

Headache - 90%
- w N&V

Transient visual obscurations - 70%
- These last seconds at a time and can be bilateral or unilateral.

Photopsia - 50%
- Bright flashes of light that may occur following changes in position, Valsalva, bright light or eye movement.

Pulsatile tinnitus - 55%
- This symptom in association with a headache is very suggestive of IIH.

Physical signs:
* Papilloedema - 95% - Typically bilateral and symmetric, but may also be asymmetric/unilateral.
* Visual field loss - 95% - Typically peripheral , but central visual field can be involved late in the course of disease or earlier if there is concurrent macular disease.
* 6th nerve palsy
* Relative afferent pupillary defect

Question 8

Mx for IIH?

Answer 8

Conservative
- Weight loss x low Na diet
- Potentially causative medications such as tetracyclines, retinoids and thyroid replacement therapies, should be stopped.
- Regular opthalmolic follow up w visual field testing

Medical:
- Acetazolamide is first-line for all patients with visual loss on presentation
- Topiramate may be used as an alternative and has the added benefit of causing weight loss in most patients
- Refractory cases: loop diuretics may be used; repeated lumbar punctures may be used as a ‘holding’ measure in refractory cases but are not used longer-term
* Analgesia: Paracetamol/NSAIDs are recommended first-line for head or back pain.

Surgical
* If patients lose vision in spite of maximal medical therapy, surgical treatment by optic nerve sheath fenestration or CSF shunting can be done.
* + Ventriculoperitoneal Shunt: Reserved for patients with refractory IIH or those with rapidly progressive visual loss. The procedure diverts excess CSF from the brain to the peritoneal cavity.

Question 9

The principal concern in patients with IIH regards the possibility of irreversible vision loss.
- What is this specifically from? [1]
- Describe the course of the vision loss [1]

Answer 9

The visual field loss is due to post-papilloedema optic atrophy.
- The peripheries of vision are typically affected first with predominantly nerve fibre bundle type defects.

Question 10

Mneumonic for the causes of cerebellar dysfunction? [+]

Answer 10

PASTRIES:
* Posterior fossa tumours
* Alcohol
* Stroke
* Trauma
* (Rare) - PaRaneoplastic syndromes
* Inherited - Friedreich’s
* Epilepsy drugs - phenytoin
* Sclerosis (multiple)

Question 11

Do cerebellar lesions cause hypertonia or hypotonia? [+]

Answer 11

Hypotonia

Question 12

Myotonic dystrophy is a genetic disorder that usually presents in adulthood. Typical features are: [4]

Answer 12

Progressive muscle weakness
Prolonged muscle contractions
Cataracts
Cardiac arrhythmias

Question 13

Lecture

Describe the presentation of myotonic dystrophy [+]

Answer 13

Muscle wasting (face/neck + distal limb) and myotonia (cramping / delayed muscle relaxation following voluntary contraction or percussion)
- Myotonia can be generalized or focal, affecting the hands, tongue, facial muscles, or lower limbs.

Both DM1 and DM2 present with progressive muscular weakness; however, the distribution and severity may vary between subtypes:
- Distal Limb Weakness (DM1): In DM1 patients, distal limb weakness predominantly affects the flexor muscles of the fingers, wrists, and ankles. In advanced stages, proximal limb muscles may also be involved.
- Proximal Limb Weakness (DM2): Patients with DM2 typically exhibit proximal limb weakness affecting hip girdle muscles more than shoulder girdle muscles. The weakness pattern in DM2 often resembles that of limb-girdle muscular dystrophy.

Facial & Bulbar Weakness
* Facial muscle involvement in both subtypes may manifest as ptosis, facial diplegia, dysarthria, dysphagia or nasal regurgitation due to palatal insufficiency.

Multisystem disease – cataracts, diabetes, cardiac conduction defects, respiratory failure, endocrine dysfunction
- atrioventricular block or atrial fibrillation, dilated cardiomyopathy, and sudden cardiac death.
- Respiratory insufficiency due to diaphragmatic or intercostal muscle weakness is a significant concern in DM patients.
- DM patients may exhibit insulin resistance or type 2 diabetes mellitus, hypogonadism with testicular atrophy (DM1), primary ovarian failure (DM2)

Question 14

Lecture

How do you treat myotonic dystrophy [+]

Answer 14

Management
* Cataract surgery, diabetic meds,
* Pacemaker/Defibrillator,
* Non-invasive ventilation.
* Na+ blockers for myotonia (mexiletine/phenytoin)

PM:
* Mexiletine can be used for myotonia, but its use should be guided by a neurologist. Steroids are not recommended due to potential worsening of myotonia.

Question 15

Describe the presentation of inclusion body myositis [3]

Answer 15

Pattern of weakness:
- Forearm, wrist, finger flexors, quadriceps, foot dorsiflexors.
- Can be asymmetrical (think distal asymmetrical weakness)
- The disease is progressive by nature, meaning those affected are likely to require a walking aid or wheelchair within 15 years to help conserve energy and stay mobile.
- Depression and a general feeling of unhappiness is very noticeable and can be an indication of the disease before any sign of muscle weakness.

Question 16

Describe the electrolyte disturbance seen in rhabdomyolysis [3]

Describe why [3]

Answer 16

High serum P043- and high serum K+ (can be life threatening) and Low serum Ca2+

hypocalcaemia (myoglobin binds calcium)
elevated phosphate (released from myocytes)
hyperkalaemia (may develop before renal failure)

Question 17

Describe the presentation of steroid-induced myopathies [3]

What dose & length of tx causes it? [2]

What would CK, EMG and Histology look like? [3]

What is the tx? [1]

Answer 17

Pattern of weakness:
- Proximal, usually symmetrical, muscle weakness – particularly quadriceps.
- Cushingoid appearance
- Spares cranial nerve muscles

Risk with >40mg/day for >6 weeks
Serum CK usually normal
EMG usually normal
Histologically: Selective atrophy of type II muscle fibres
Treatment:
- Dose reduction or withdrawal of corticosteroid

Question 18

What does a normal, myopathic and neuropathic EMG look like? [3]

Answer 18

Neuropathic EMG – polyphasic, long duration, large amplitude

Myopathic EMG – polyphasic, short duration, small amplitude

Question 19

Describe what is meant by Charcot Marie Tooth disease [1]

Answer 19

Charcot-Marie-Tooth disease is an inherited disease that affects the peripheral motor and sensory neurones.
- It is also known as hereditary motor and sensory neuropathy.
- There are various types, with different genetic mutations and pathophysiology, causing myelin or axon dysfunction.
- The majority of mutations are inherited in an autosomal dominant pattern.

Question 20

Describe the presentation of Charcot-Marie-Tooth disease [+]

Answer 20

There may be a history of frequently sprained ankles
- Lower leg weakness, particularly loss of ankle dorsiflexion (with a high stepping gait due to foot drop)

Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity
Distal muscle wasting causing “inverted champagne bottle legs”
Peripheral sensory loss

Lecture:
- Symmetrical, length dependent (distal –> proximal gradient).
- Lower limbs before upper limbs.
- Deformity - claw toes, pes cavus, charcot joints, scoliosis
- Continuum from pure motor –> motor/sensory/pure sensory)

Question 21

Other causes of peripheral neuropathy can be remembered with the ABCDE mnemonic [5]

Answer 21

A – Alcohol
B – B12 deficiency
C – Cancer (e.g., myeloma) and Chronic kidney disease
D – Diabetes and Drugs (e.g., isoniazid, amiodarone, leflunomide and cisplatin)
E – Every vasculitis

Question 22

CIDP – Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Describe the typical disease course and presentation [2]

Answer 22

Progressive weakness (proximal + distal) and sensory loss (distal) – Over >8 weeks – relapse - remitting

Question 23

Describe the examination findings of CIDP – Chronic Inflammatory Demyelinating Polyradiculoneuropathy. [3]

Answer 23

Examination
- Symmetrical proximal >distal weakness
- Absent reflexes
- Symmetrical glove and stocking sensory loss

Question 24

Tx for CIDP? [2]

Answer 24

Treatment: Steroids/Intravenous Immunoglobulin
(Immune mediated attack on myelin sheath

Question 25

Describe the presentation of MND [

Answer 25

The typical patient is a **late middle-aged (e.g., 60) man,** possibly with an affected relative. There is an **insidious, 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 can develop **slurred speech** Also * **fasciculations** * the absence of sensory signs/symptoms * the **mixture of lower motor neuron and upper motor neuron signs** * **wasting of the small hand muscles/tibialis anterior is common** * **doesn't affect external ocular muscles** * **no cerebellar signs** * **abdominal** **reflexes** are **usually preserved and sphincter dysfunction if present is a late feature**

Question 26

Dx of MND? [3]

Answer 26

The diagnosis of motor neuron disease is **clinical**, but **nerve conduction studies will show normal motor conduction** and can **help exclude a neuropathy**. - **Electromyography** shows a **reduced number of action potentials with increased amplitude.** - **MRI** is usually performed to **exclude** the differential diagnosis of **cervical cord compression and myelopathy**

Question 27

Describe the treatment for MND [2]

Answer 27

**Riluzole** * prevents stimulation of glutamate receptors * used mainly in **amyotrophic lateral sclerosis** * prolongs life by about 3 months **Respiratory care** * non-invasive ventilation (usually BIPAP) is used at night * studies have shown a survival benefit of around 7 months

Question 28

Describe the presenting features of each type of MNDs: ALS [2] Primary lateral sclerosis [1] Progressive muscular atrophy [3] Progressive bulbar palsy [2]

Answer 28

**ALS** - **LMN** in **arms** - **UMN** signs in **legs** **Primary lateral sclerosis**: - UMN signs only **Progressive muscular atrophy** - LMN signs only - affects distal muscles before proximal - carries best prognosis **Progressive bulbar palsy** * palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei * carries worst prognosis

Question 29

Describe the clinical features of essential tremor

Answer 29

**Tremor** * Action tremor * **Exacerbated on intentional movements**, and usually **absent on rest** * Common **daily tasks** where the tremor is exacerbated includes **writing, handling utensils and small objects, drinking, reaching out for objects** * On examination, **will worsen on holding the arms** **outstretched** and on finger-to-nose testing * **Typically bilateral** * **Asymmetric** in **nature** and generally affects the **dominant side more than the non-dominant side** * Primarily affects **hands** and **arms** in early stages * Can sometimes progress over a long time to involve the **head, voice, trunk** * **Very rarely affects the lower limbs** * **Head tremor can be either vertical** (nodding head yes) in **25**%, or **horizontal** (shaking head no) **in 75%** **Tremor frequency** * Moderate to high frequency * **6-12 Hz** **Relieving/exacerbating factors** * **Relieving**: usually relieved somewhat by **alcohol** (in approximately 65%) * **Exacerbating**: exacerbated by **anxiety, excitement, adrenergic stimulation**

Question 30

In some situations, the essential tremor is called 'essential tremor plus.' What features would be included in this? [4]

Answer 30

**Difficulty with tandem gait** **Mild cognitive impairment** (typically mild memory impairment) **Slight resting tremor** alongside action tremor ## Footnote **NB**: - Often, the presence of these symptoms may confuse the diagnosis and usually will prompt investigation for another differential prior to making the diagnosis of essential tremor plus. This is because the diagnostic criteria below indicates that for the diagnosis of essential tremor, these must be absence of other neurological signs

Question 31

Medical [2] and surgical [2] treatment of essential tremor?

Answer 31

**Medical treatment** - **first-line medical treatment** for essential tremor is either **propranolol and primidone** - If required, **step-up to using both propranolol and primidone in combination** may be trialled - For patients refractory to these medications, reasonable second-line medications according to UptoDate and BMJ best practice include **gabapentin, topiramate and nimodipine** **Surgical treatment** - **Deep brain stimulation** may be indicated for patients w severe disability (directed at nucleus ventralis intermedius of the thalamus) - **Botulinum toxin type A injections** ## Footnote **NB**: Medical tx witll reduce the tremor amplitude by up to 50%, therefore it is important to educate patients that it is unlikely that their tremor will completely subside

Question 32

**[]** is the preferred way to support nutrition in patents with motor neuron disease

Answer 32

**Percutaneous gastrostomy tube (PEG)** is the preferred way to support nutrition in patents with motor neuron disease

Question 33

What is the mechanism of action of ondansetron? 5-HT3 antagonist 5-HT2 antagonist Dopamine antagonist NK1 receptor antagonist Antihistamine

Answer 33

What is the mechanism of action of ondansetron? **5-HT3 antagonist** 5-HT2 antagonist Dopamine antagonist NK1 receptor antagonist Antihistamine

Question 34

Describe the pathophysiology of GBS [2]

Answer 34

**Guillain-Barré** is thought to occur due to a process called **molecular mimicry**: - The **B cells** of the immune system create **antibodies** against the **antigens** on the **triggering** **pathogen**. - These **antibodies also match proteins on the peripheral neurones.** - They may **target proteins on the myelin sheath or the nerve axon itself.** - correlation between **anti-ganglioside antibody (e.g. anti-GM1)** and clinical features has been demonstrated; **anti-GM1 antibodies in 25% of patients**

Question 35

Describe the clinical feature of GBS [+]

Answer 35

**progressive weakness of all four limbs.** - The weakness is classically **ascending** i.e. the **lower extremities are affected first**, however it tends to affect **proximal** **muscles** **earlier** than the **distal ones** **Reduced reflexes** **Neuropathic pain** **Sensory symptoms tend to be mild** (e.g. distal paraesthesia) with very few sensory signs. Some patients experience back pain in the initial stages of the illness There may be **peripheral loss of sensation or neuropathic pain**. It may **progress to the cranial nerves and cause facial weakness**. Autonomic dysfunction can lead to **urinary retention, ileus or heart arrhythmias.** **Lecture**: - Often involves **cranial nerves** (eyes - III/IV/VI and face – bilateral VIIth) - **Ascending weakness** (Legs>arms) over days. - Often **starts** with **paraesthesiae** in **hands and feet + back pain**

Question 36

Describe the disease course of GBS [1]

Answer 36

**Symptoms** usually **start** **within four weeks of the triggering infection**. They **begin** in the **feet** and **progress upward.** - **Symptoms peak within 2-4 weeks**. Then, there is a **recovery** **period** that can **last months to years.**

Question 37

Describe the investigations for GBS [2]

Answer 37

**The diagnosis of Guillain-Barré syndrome** is made **clinically** **(using the Brighton criteria)**, supported by investigations: * **Nerve conduction studies** (showing **reduced signal through the nerves**) * **Lumbar puncture for cerebrospinal fluid** (showing **raised protein with a normal cell count and glucose**)

Question 38

Mx for GBS? [4]

Answer 38

**Management**: * **plasma exchange** * **VTE prophylaxis** (pulmonary embolism is a leading cause of death) * **IV immunoglobulins (IVIG)**: as effective as plasma exchange. No benefit in combining both treatments. IVIG may be easier to administer and tends to have fewer side-effects * **Plasmapheresis** is an **alternative to IVIG** * **steroids and immunosuppressants** have **NOT** been shown to be **beneficial** * **FVC regularly to monitor respiratory function** ## Footnote **NB: Severe cases** with respiratory failure may require **intubation, ventilation and admission to the intensive care unit.**

Question 39

What are the underlying causes of Horner's syndrome? [2] What's the difference in cause between First, Second & Third order Horner's? [2]

Answer 39

The underlying causes of Horner's syndrome can vary, including **tumours, injuries, or neurological disorders** affecting the **sympathetic nerves.** **First-order (central) Horner's** - lesion from hypothalamus to T1 spinal cord segment; causes include multiple sclerosis, brain tumours, and strokes (e.g., Wallenberg’s syndrome). **Second-order Horner's** - lesion from spinal cord to superior cervical ganglion; causes include Pancoast tumours, thyroid malignancies, iatrogenic, and traumatic causes. **Third-order (postganglionic) Horner's**: - lesion from superior cervical ganglion to the eye; causes include carotid artery dissection, cavernous sinus thrombosis, and cluster headaches.

Question 40

Describe the clinical features of Horner's syndrome [5+]

Answer 40

**Anisocoria** * Miosis (constricted pupil) on the affected side * Relative pupillary dilation lag in darkness (2-8 seconds) **Ptosis** * Partial ptosis due to weakness of Muller's muscle * Involvement of levator palpebrae superioris may lead to more pronounced ptosis **Facial Anhidrosis and Vasodilation** * Ipsilateral loss of sweating (anhidrosis) over forehead, face, and neck regions * Facial vasodilation due to loss of sympathetic tone **Heterochromia Iridum (in congenital cases)** * Lighter iris colour in the affected eye compared to contralateral eye * Due to a lack of melanin deposition during development **Enophthalmos (rare)** * Slight posterior displacement of the eyeball within the orbit * Attributed to the loss of sympathetic innervation to orbital smooth muscles

Question 41

How do you confirm Horner's? [1]

Answer 41

**The presence of systemic features** with Horner’s can help guide the identification of lesion location and inform further investigations. For example, patients with cough and weight loss should undergo a chest X-ray to screen for a Pancoast tumour. **Apraclonidine** is now the commonly used alternative to confirm Horner’s, which reverses the pupillary constriction in Horner’s due to its agonistic effect on alpha-2-receptors. **Hydroxyamphetamine drops** can subsequently be used to identify the location of the lesion to guide imaging. Hydroxyamphetamine will dilate a constricted Horner’s pupil if there is an underlying preganglionic lesion (first or second-order). **If the pupil fails to dilate**, this suggests that the lesion is in the third order or postganglionic neuron.2

Question 42

Describe the features of central lesions of Horner's [1] What are the specific causes? [5]

Answer 42

Anhidrosis of the face, arm and trunk **S**troke **S**yringomyelia Multiple **s**clerosis **Tumour** **Encephalitis**

Question 43

Describe the features of pre-ganglionic lesions of Horner's [1] What are the specific causes? [4]

Answer 43

Anhidrosis of the **face** Pancoast's **t**umour **T**hyroidectomy **T**rauma **Cervical rib**

Question 44

Describe the features of post-ganglionic lesions of Horner's [1] What are the specific causes? [4]

Answer 44

No anhidrosis **C**arotid artery dissection **C**arotid aneurysm **C**avernous sinus thrombosis **C**luster headache

Question 45

What does the facial nerve supply? [4] - What is the motor [3]; sensory [1] and parasympathetic [2] functions?

Answer 45

Supply - 'face, ear, taste, tear' **face**: muscles of facial expression **ear**: nerve to stapedius **taste**: supplies anterior two-thirds of tongue **tear**: parasympathetic fibres to lacrimal glands, also salivary glands **Motor function for:** * Facial expression * Stapedius in the inner ear * Posterior digastric, stylohyoid and platysma muscles **Sensory function** for **taste** from the **anterior 2/3 of the tongue.** **Parasympathetic supply to the:** * Submandibular and sublingual salivary glands * Lacrimal gland (stimulating tear production)

Question 46

Unilateral upper motor neurone lesions occur in: [2] Bilateral upper motor neurone lesions are rare. They may occur in [2]

Answer 46

**Unilateral** **upper** **motor** **neurone** lesions occur in: * **Cerebrovascular accidents (strokes)** * **Tumours** **Bilateral upper motor neurone lesion**s are rare. They may occur in: * **Pseudobulbar palsies** * **Motor neurone disease**

Question 47

Describe the presentation of Bell's palsy

Answer 47

**Acute onset** (within 72 hours) Involves the **forehead and lower parts** of the face on the affected side * **Upper facial signs** include the inability to wrinkle forehead and close eye fully on the affected side * **Lower facial signs** include the **loss of the nasolabial labial fold, and drooping of the mouth**, which is more **pronounced when the patient tries to smile** * The severity of the **paralysis can be quantified by the House-Brackmann facial paralysis scale**, which is **graded from 1 (normal) to 6 (complete paralysis).** **Pain in the ear and surrounding area** **Loss of taste in the anterior tongue** in 35% of patients **Hyperacusis** (increased sensitivity to noise) is a rarer symptom

Question 48

Describe the investigations for Bell's palsy? [+]

Answer 48

**Bell's palsy** is usually a **diagnosis of exclusion** Therefore a comprehensive clinical review is required - **Examination** * Forehead sparing suggests an upper motor neurone cause and therefore not Bell's palsy, which is a lower motor neurone pathology. * Any masses, particularly parotid swelling may suggest a tumour. * Any other cranial nerve abnormalities or neurology of the upper or lower limbs may suggest a central or systemic pathology. **Imaging** should be used if clinical features are not in keeping with Bell's palsy or the patient is not recovering as expected. * **MRI Head** is the modality of choice to view the facial nerve.

Question 49

How would a stroke present similarly / differently to Bell's palsy? [2]

Answer 49

**Similarities** - present with unilateral facial droop . **Differences** - there will be sparing of the forehead (patient will still be able to frown) as this is an upper motor neurone lesion and may also be accompanied by weakness of the arm/leg on the affected side, slurred speech and visual disturbances.

Question 50

Describe the management plan for Bell's palsy

Answer 50

**Prednisolone** for patients who present **within 72 hours of symptom onset.** - **Antivirals** can be considered for use in **conjunction** with prednisolone (should **not be used as monotherapy**). **Good eye care** is very important on the affected side as the inability to completely close the eyelid can cause dry eyes and other complications: * **Artificial tears or ocular lubricants** to prevent dry eyes. * Using **tape** (e.g.micropore) to **close the eye overnight.** * Wearing sunglasses and **avoiding irritants** to the **eye such as dust.** * If a patient has **eye pain or irritation, they should be referred to ophthalmology** (secondary care) for review as these patients are at risk of problems such as **corneal ulceration** which can **lead to visual loss** if not treated correctly

Question 51

Describe disease course of Bell's palsy and what this means clinically [2]

Answer 51

The **majority of patients will recover with the acute management** measures. However, if **no signs of recovery** are seen after **3 weeks of initial symptoms** (i.e. no improvement in House-Brackmann score), **NICE recommends referral to secondary care** (either to ENT or neurology) for further investigation and management. Therefore it is very important that all patients diagnosed with **Bell's Palsy have a follow-up** appointment arranged to **monitor recovery.**

Question 52

While the majority of Bell's palsy cases resolve spontaneously, a number of complications can occur. The most common complications are: [2]

Answer 52

**Synkinesis**: - This is characterised by **involuntary muscular movements accompanying voluntary movements**. It occurs due to aberrant regeneration of the facial nerve and may present as **crocodile tears syndrome (lacrimation during eating), gustatory sweating or hemifacial spasm**. **Persistent facial weakness**: - Despite treatment, some patients may experience persistent facial weakness which can lead to functional impairment and cosmetic concerns. Severity varies from mild to severe disfigurement.

Question 53

Bell's palsy can also lead to several ocular complications including [3]

Answer 53

**Corneal keratitis**: Due to impaired eyelid closure, corneal exposure may result in desiccation, ulceration or even perforation if not managed promptly. **Lagophthalmos**: Inability to close the eye completely could lead to exposure keratopathy. **Abrasive keratopathy:** This results from irregular blinking leading to trauma on the corneal surface.

Question 54

What would CSF results look like in GBS? [2]

Answer 54

**rise in protein** with a **normal white blood cell** count (albuminocytologic dissociation) - found in 66%