Neurology Final II

Question 2

Describe the risk factors for CVT [5]

Answer 2

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 3

Describet the primary and secondary mechanisms of injury in CVT

Answer 3

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 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

Lecture

Describe the presentation of myotonic dystrophy [+]

Answer 9

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 10

Lecture

How do you treat myotonic dystrophy [+]

Answer 10

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 11

Describe the presentation of inclusion body myositis [3]

Answer 11

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 12

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

Answer 12

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 13

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

Answer 13

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 14

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

Answer 14

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

Question 15

Tx for CIDP? [2]

Answer 15

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

Question 16

Describe the presentation of MND [

Answer 16

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 17

Dx of MND? [3]

Answer 17

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 18

Describe the treatment for MND [2]

Answer 18

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 19

Describe the disease course of GBS [1]

Answer 19

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 20

Describe the investigations for GBS [2]

Answer 20

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 21

Mx for GBS? [4]

Answer 21

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

NB: Severe cases with respiratory failure may require intubation, ventilation and admission to the intensive care unit.

Question 22

How do you confirm Horner’s? [1]

Answer 22

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 23

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

Answer 23

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 24

Unilateral upper motor neurone lesions occur in: [2]

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

Answer 24

Unilateral upper motor neurone lesions occur in:
* Cerebrovascular accidents (strokes)
* Tumours

Bilateral upper motor neurone lesions are rare. They may occur in:
* Pseudobulbar palsies
* Motor neurone disease

Question 25

Describe the presentation of Bell's palsy

Answer 25

**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 26

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

Answer 26

**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 27

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

Answer 27

**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 28

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

Answer 28

**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 29

SCD occurs because of B12 deficiency affecting which affected columns? [3]

Answer 29

**dorsal column involvement**, **lateral corticospinal tracts** and **spinocerebellar tracts** of the spinal cord ## Footnote **S**pinocerebellar; **C**orticospinal and **D**orsal column

Question 30

Describe the clinical features of SCD [3+]

Answer 30

**dorsal column involvement** * **distal tingling/burning/sensory loss** is **symmetrical** and tends to affect the legs more than the arms * **impaired proprioception and vibration sense** **lateral corticospinal tract involvement**: * **muscle weakness, hyperreflexia, and spasticity** * **upper motor neuron** signs typically develop in the **legs first** * **brisk knee reflexes** * **absent ankle jerks** * **extensor plantars** **spinocerebellar tract involvement** * **sensory ataxia → gait abnormalities** * **positive Romberg's sign**

Question 31

Describe the classic presentation of syringomyelia [+]

Answer 31

Classical presentation of a syrinx is a patient who has a **'cape-like' (neck and arms)** **loss of sensation to temperature** but **preservation** of **light touch, proprioception and vibration.** - Classic examples are of **patients who accidentally burn their hands without realising** - This is due to the **crossing spinothalamic tracts in the anterior commissure** of the **spinal cord being the first tracts to be affected.** **Other symptoms:** * **spastic weakness** (predominantly of the upper limbs) * **paraesthesia** * **neuropathic pain** * **upgoing plantars and bowel and bladder dysfunction** **Prolonged syringomyelia:** **Scoliosis** will occur over a matter of years if the syrinx is not treated. It may cause a **Horner's syndrome** due to compression of the sympathetic chain, but this is rare

Question 32

Which spinal tract is classically affected first in syringomyelia [1]

Answer 32

**crossing spinothalamic tracts** in the **anterior commissure of the spinal cord** are the **first tracts to be affected.**

Question 33

How do you Ix syringomyelia? [2]

Answer 33

Investigation requires a **full spine MRI** with **contrast to exclude a tumour or tethered cord.** A **brain MRI** is also needed to **exclude a Chiari malformation.**

Question 34

Clinical features of HD? [4]

Answer 34

Features typical develop after 35 years of age * **chorea** * **personality changes** (e.g. irritability, apathy, depression) and intellectual impairment * **dystonia** * **saccadic eye movements** ZtF: * **Chorea** (involuntary, random, irregular and abnormal body movements) * **Dystonia** (abnormal muscle tone, leading to abnormal postures) * **Rigidity** (increased resistance to the passive movement of a joint) * **Eye movement disorders** * **Dysarthria** (speech difficulties) * **Dysphagia** (swallowing difficulties)

Question 35

# HD **Neuroimaging**: - While not diagnostic, neuroimaging can support the clinical findings. **MRI or CT scans may show [] nucleus atrophy**, but these changes are more evident in advanced stages of **HD**

Answer 35

**Neuroimaging**: - While not diagnostic, neuroimaging can support the clinical findings. **MRI or CT scans may show caudate nucleus atrophy**, but these changes are more evident in advanced stages.

Question 36

Describe the management for HD: - Neurological management [3] - Pyschiatric management [2] - Nutrional [2] and dysphagia [3]

Answer 36

**Neurological Management**: - **Tetrabenazine** is the first-line treatment for chorea in HD (works by depleting dopamine) - **Deutetrabenazine and valbenazine** are alternatives if tetrabenazine is not tolerated. - For patients with **bradykinesia** or **rigidity**, consider a trial of **levodopa**. - **Amantadine**: May also help reduce chorea and has some mood-stabilizing effects. - Drugs like **olanzapine, risperidone, or quetiapine** are sometimes used to manage chorea and psychiatric symptoms if associated **Psychiatric Management** * **Selective serotonin reuptake inhibitors (SSRIs) or mirtazapine** can be used to manage **depression** and **irritability** in HD patients. * **Cognitive behavioural therapy (CBT)** should be considered for managing obsessive-compulsive disorder symptoms. * Mood stabilisers like **valproate or lamotrigine** can be helpful for mood swings or irritability. * **Antipsychotics**: These are also used to treat psychosis, severe agitation, or aggression. **Dysphagia Management** * A **speech and language therapist** should assess swallowing function regularly to minimise **risk of aspiration pneumonia.** Dietary modifications may be necessary. * If oral feeding becomes unsafe despite interventions, **percutaneous endoscopic gastrostomy (PEG)** feeding should be considered.

Question 37

Describe the medical [3] surgical [2] and other treatments [2] for dystonia

Answer 37

**Medications**: * **Anticholinergics**: Drugs like trihexyphenidyl and benztropine help reduce muscle contractions by blocking the neurotransmitter acetylcholine. * **Benzodiazepines**: Drugs like diazepam or clonazepam can help relax muscles and reduce spasms. * **Dopaminergic Agents**: Levodopa or tetrabenazine may be used in certain forms of dystonia. **Surgical treatments:** - **Deep Brain Stimulation (DBS):** This is the most common surgical treatment for dystonia. Electrodes are implanted in specific brain areas, like the globus pallidus or subthalamic nucleus, to modulate abnormal brain signals. DBS is often used for generalized dystonia or severe focal dystonia unresponsive to other treatments. * **Selective Peripheral Denervation**: Involves cutting nerves to specific muscles to reduce contractions. This is sometimes used for cervical dystonia. **Other** * **Physiotherapy/Occupational tx / exercise** * **Botox injections** - treat focal dystonia by temporarily paralyzing the overactive muscles. The effects last for 3-4 months, after which the injections may need to be repeated.

Question 38

What is the Westphal variant of HD? [1]

Answer 38

**rare juvenile-onset cases:** - **parkinsonism, dystonia, myoclonus, epilepsy, chorea may be absent**

Question 39

Describe the tx of tics: - Behavioural therapy [2]

Answer 39

**1. Behavioral Therapy** **Habit Reversal Training (HRT):** Helps individuals recognize the urge to tic and replace the tic with a more appropriate, competing response. **Relaxation Techniques:** Stress and anxiety can exacerbate tics, so learning relaxation strategies like deep breathing and progressive muscle relaxation can help manage tics **2. Medications**: * **Alpha-2 Adrenergic Agonists**: Such as **clonidine**. These are often used as a first-line medication, especially in children, because they have fewer side effects. Particularly useful in tics with coexisting ADHD. * **Antipsychotic Medications**: Such as **aripiprazole, risperidone, and haloperidol.** These can be effective in reducing tics but may have side effects like weight gain and sedation. * **Dopamine Depleters: Such as tetrabenazine.** These reduce the amount of dopamine in the brain, which can help control tics.

Question 40

Describe the clinical features of brachial neuritis [2]

Answer 40

**acute, severe pain in the shoulder region** - udden onset and may be preceded by an antecedent event such as infection, surgery, or trauma - many cases have no identifiable precipitating factor - sharp or burning nature - exacerbated by movement **Following the initial phase of intense pain, which can last from hours to weeks, patients may experience progressive weakness and atrophy** in one or more muscles innervated by the brachial plexus. **Sensory disturbances are less common** but can manifest as numbness, tingling (paresthesia), or altered sensation (dysesthesia) in areas corresponding to affected nerves

Question 41

Describe the difference between Erb-Duchenne and Klumpke's paralysis [2]

Answer 41

**Erb-Duchenne paralysis** * damage to C5,6 roots * winged scapula * may be caused by a breech presentation **Klumpke's paralysis** * damage to T1 * loss of intrinsic hand muscles * due to traction

Question 42

Describe the different symptomatic classifications of FTD [3]

Answer 42

**Behavioural variant Frontotemporal Dementia (bvFTD):** - Most common form - **changes** in **personality** and **behaviour**, including **disinhibition**, **apathy**, **loss of empathy or compulsive behaviours** **Semantic Dementia (SD):** - **loss of semantic knowledge** - patients have difficulty understanding words or recognising familiar people or objects **Progressive Nonfluent Aphasia (PNFA):** * Characterised by **progressive language disorder** with **non-fluent speech and grammatical errors.** * Patients may also show signs of **agrammatism** or **apraxia** of **speech**.

Question 43

Describe the different neuropathological classifications of FTD [3]

Answer 43

**Tau-positive FTLD:** - **tau proteins accumulate** abnormally **within** **neurons** leading to **neurofibrillary** **tangles**. - Three major types exist based on the morphology and distribution of tau inclusions: **Pick's disease, corticobasal degeneration, and progressive supranuclear palsy.** **TDP-43 positive FTLD**: - Characterised by the **accumulation** of transactive response DNA binding protein 43 (**TDP-43)**. - **Four subtypes exist (A to D)** based on the distribution and morphology of TDP-43 inclusions. **FUS-positive FTLD:** - Less common, characterised by the **presence** of **fused in sarcoma (FUS) protein** within **inclusion** **bodies** - These are typically seen in younger patients and often associated with a more aggressive disease course.

Question 44

Describe the typical features in neuroimaging of bvFTD; nonfluent PPA; semantic PPA [3]

Answer 44

**bvFTD**: - **frontal and temporal atrophy** in up to 65%. Particularly anterior insula, anterior cingulate cortex, and amygdala. May be asymmetrical. **Nonfluent PPA**: - early atrophy and hypoperfusion in the **left posterior fronto-insular cortex.** **Semantic PPA**: - **significant anterior temporal atrophy.** Often asymmetric.

Question 45

Describe the clinical features of Holmes-Adie pupil [6+]

Answer 45

**Anisocoria**: - Patients with Holmes-Adie pupil commonly present with anisocoria, i.e., unequal size of pupils. - The affected **pupil appears dilated in comparison to the contralateral side.** **Tonic Pupillary Response:** - The **hallmark feature** of this **condition is the tonic response observed upon direct light stimulation. When exposed to light, the affected pupil demonstrates slow and prolonged constriction followed by gradual redilation**. - This sluggish reaction is best appreciated when comparing it to the normal brisk constriction of the unaffected side. **Light-Near Dissociation:** - Another characteristic feature is **light-near dissociation,** where there is a **reduced or absent pupillary reaction** to **light** while maintaining a **relatively preserved response to near stimulus (accommodation).** - This phenomenon occurs due to the **selective involvement of parasympathetic fibres innervating the iris sphincter muscle**. **Vermiform Movements:** - Upon **close examination of the affected iris under slit-lamp biomicroscopy**, one may observe irregular, undulating movements known as **vermiform movements**. These subtle movements are attributed to segmental denervation and reinnervation of iris sphincter muscle fibres. **Accommodative Dysfunction** - Patients may report difficulty focusing on near objects or experience blurred vision at near distances due to impaired accommodation reflex. **Associated Neurological Findings**: - Holmes-Adie syndrome, a variant of this condition, is characterized by the presence of additional neurological findings such as areflexia or hyporeflexia, particularly in the lower extremities. This manifestation results from damage to the peripheral nerve fibres that supply the muscle spindle stretch receptors.

Question 46

NICE recommend that we suspect CFS if: [3]

Answer 46

* the person has had **all of the persistent symptoms for a minimum of 6 weeks in adults and 4 weeks in children and young people and** * the **person's ability to engage in occupational, educational, social or personal activities is significantly reduced from pre-illness levels** and * symptoms are **not explained by another condition.**

Question 47

Describe the management for CFS [4]

Answer 47

**refer to a specialist CFS service** if the diagnostic criteria are met and symptoms have persisted for 3 months **energy management** * a self-management strategy that involves a person with ME/CFS managing their activities to stay within their energy limit, with support from a healthcare professional **physical activity and exercise** * do not advise people with ME/CFS to undertake exercise that is not part of a programme overseen by an ME/CFS specialist team * should only be recommended if patients 'feel ready to progress their physical activity beyond their current activities of daily living' * graded exercise therapy used to be recommended but is now specifically not recommended by NICE **cognitive behavioural therapy** * NICE stress this is 'supportive' rather than curative for CFS

Question 48

Focal seizures not responding to first-line drug - try **[2]** (i.e. the first-line drug not already tried) and if neither help then **[1]**

Answer 48

Focal seizures not responding to first-line drug - **try lamotrigine or levetiracetam** (i.e. the first-line drug not already tried) and if neither help then **carbamazepine**

Question 49

Speech fluent; comprehension impaired Broca's aphasia Global aphasia Conduction aphasia Wernicke's aphasia

Answer 49

Speech fluent; comprehension impaired Broca's aphasia Global aphasia Conduction aphasia **Wernicke's aphasia**

Question 50

auditory agnosia would occur from a lesion in Frontal lobes lesions Temporal lobe lesion Occipital lobe lesions Parietal lobe lesions Cerebellum lesions

Answer 50

auditory agnosia would occur from a lesion in Frontal lobes lesions **Temporal lobe lesion** Occipital lobe lesions Parietal lobe lesions Cerebellum lesions ## Footnote Auditory agnosia refers to impairments in sound perception and identification despite intact hearing, cognitive functioning, and language abilities

Question 51

inferior homonymous quadrantanopia would occur from a lesion in Frontal lobes lesions Temporal lobe lesion Occipital lobe lesions Parietal lobe lesions Cerebellum lesions

Answer 51

**Parietal lobe lesions**

Question 52

superior homonymous quadrantanopia would occur from a lesion in Frontal lobes lesions Temporal lobe lesion Occipital lobe lesions Parietal lobe lesions Cerebellum lesions

Answer 52

superior homonymous quadrantanopia would occur from a lesion in Frontal lobes lesions **Temporal lobe lesion** Occipital lobe lesions Parietal lobe lesions Cerebellum lesions

Question 53

Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia **would occur from a lesion in which specific brain area?**

Answer 53

**Amygdala**

Question 54

Wernicke and Korsakoff syndrome occur from lesions in which brain areas? [1]

Answer 54

**Medial thalamus and mammillary bodies of the hypothalamus**

Question 55

Hemiballism is a movement disorder characterized by rapid, flailing, and jerky movements on one side of the body, often involving the arm and leg. It is usually caused by a lesion in the **[]**

Answer 55

Hemiballism is a movement disorder characterized by rapid, flailing, and jerky movements on one side of the body, often involving the arm and leg. It is usually caused by a lesion in the **subthalamic nucleus**

Question 56

Describe the presentation of central cord syndrome [2]

Answer 56

There is a **flaccid weakness of the arms (both)**, but **motor and sensory fibres to the lower limb are comparatively** preserved as these are located **more peripherally in the spinal cord**