Neurology Flashcards

Question

what are 2 investigations for subarachnoid haemorrhage? -if both were negative with a high level of suspicion, what should be done next?

Answer 1

Investigations • CT: negative in 5%. • LP: done after 12 Hrs (allowing time for xanthochromia to develop) If the CSF examination did not reveal xanthochromia, or there was still a high level of clinical suspicion, then cerebral angiography would be the next step

Answer 2

Complications • Rebleeding (in 30%) • Obstructive hydrocephalus (due to blood in ventricles) • Vasospasm leading to cerebral ischemia

Answer 3

Intracranial hemorrhage can cause changes in the ECG which are typically deep symmetrical T- wave inversion and prolonged QT interval

Answer 4

• Neurosurgical opinion: no clear evidence over early surgical intervention against delayed intervention • Nimodipine (e.g. 60mg / 4 hrly, if BP allows) has been shown to ↓ the severity of neurological deficits but doesn't ↓ rebleeding

Answer 5

Bleeding into the space between the dura mater and the skull. Often results from acceleration-deceleration trauma or a blow to the side of the head (pterion region). The majority of epidural Hematomas occur in the temporal region where skull fractures cause a rupture of the middle meningeal artery.

Answer 6

Features • Features of raised intracranial pressure • Some patients may exhibit a lucid interval

Answer 7

• Can cause cerebral infarction, much less common than arterial causes • 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses

Answer 8

Features • Headache (may be sudden onset) • Nausea & vomiting • Papilledema

Answer 9

Sagittal sinus thrombosis • May present with seizures and hemiplegia • Parasagittal biparietal or bifrontal hemorrhagic infarctions are sometimes seen

Answer 10

Cavernous sinus thrombosis • Other causes of cavernous sinus syndrome: local infection (e.g. Sinusitis), neoplasia, trauma • Ophthalmoplegia due to IIIrd, IVth and VIth nerve damage • Trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain • Central retinal vein thrombosis • Swollen eyelids

Answer 11

Lateral sinus thrombosis • VIth and VIIth cranial nerve palsies

Answer 12

haloperidol 0.5mg then lorazepam

Answer 13

Marchiafava Bignami syndrome: Corpus callosum degeneration from chronic alcohol excess

Answer 14

A classical triad of features is seen • Urinary incontinence • Dementia and bradyphrenia (slowness of thought) • Gait abnormality (may be similar to parkinson's disease)

Answer 15

is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to ↓ CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid hemorrhage or meningitis

Answer 16

Imaging • Hydrocephalus with an enlarged fourth ventricle

Answer 17

Management • Ventriculoperitoneal shunting

Answer 18

Idiopathic Intracranial Hypertension (also known as pseudotumour cerebri and formerly benign intracranial hypertension) is a condition classically seen in young, overweight ♀.

Answer 19

• Headache • Blurred vision • Papilledema (usually present) • Enlarged blind spot • Sixth nerve palsy may be present

Answer 20

• Obesity • ♀sex • Pregnancy • Drugs (in this case it is not idiopathic): oral contraceptive pill, steroids, tetracycline, vitamin A

Answer 21

1. CT Scan 2. LP 3. Cerebral MRI with MR Venography

Answer 22

• Weight loss • Diuretics e.g. Acetazolamide • Corticosteroids can be given • Repeated lumbar puncture • Surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to ↓ intracranial pressure

Answer 23

The ABCD2 prognostic score has previously been used to risk stratify patients who present with a suspected TIA. However, data from studies have suggested it performs poorly and it is therefore no longer recommended by NICE Clinical Knowledge Summaries Criteria Points Age ≥ 60 years - 1 Blood pressure ≥ 140/90 mmHg - 1 Clinical features - Unilateral weakness - 2 - Speech disturbance, no weakness - 1 Duration of symptoms - > 60 minutes - 2 - 10-59 minutes - 1 Patient has diabetes - 1

Answer 24

Immediate antithrombotic therapy: give aspirin 300 mg immediately, unless 1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage) 2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist 3. Aspirin is contraindicated: discuss management urgently with the specialist team

Answer 25

If the patient has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis: discuss the need for admission or observation urgently with a stroke specialist

Answer 26

If the patient has had a suspected TIA in the last 7 days: arrange urgent assessment (within 24 hours) by a specialist stroke physician

Answer 27

If the patient has had a suspected TIA which occurred more than a week previously: refer for specialist assessment as soon as possible within 7 days

Answer 28

clopidogrel is recommended first-line (as for patients who've had a stroke) aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel these recommendations follow the 2012 Royal College of Physicians National clinical guideline for stroke.

Answer 29

-blood glucose, hydration, oxygen saturation and temperature should be maintained within normal limits -blood pressure should not be lowered in the acute phase unless there are complications e.g. Hypertensive encephalopathy -aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excluded -with regards to atrial fibrillation, the RCP state: 'anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke' -if the cholesterol is > 3.5 mmol/l patients should be commenced on a statin. Many physicians will delay treatment until after at least 48 hours due to the risk of haemorrhagic transformation

Answer 30

Thrombolysis with alteplase should only be given if: it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial) haemorrhage has been definitively excluded (i.e. Imaging has been performed)

Answer 31

- Previous intracranial haemorrhage - Seizure at onset of stroke - Intracranial neoplasm - Suspected subarachnoid haemorrhage - Stroke or traumatic brain injury in preceding 3 months - Lumbar puncture in preceding 7 days - Gastrointestinal haemorrhage in preceding 3 weeks - Active bleeding - Pregnancy - Oesophageal varices - Uncontrolled hypertension >200/120mmHg

Answer 32

- Concurrent anticoagulation (INR >1.7) - Haemorrhagic diathesis - Active diabetic haemorrhagic retinopathy - Suspected intracardiac thrombus - Major surgery / trauma in the preceding 2 weeks

Answer 33

NICE recommend a pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)

Answer 34

Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours), to people who have: Acute ischaemic stroke and confirmed occlusion of the proximal anterior circulation demonstrated by computed tomographic angiography (CTA) or magnetic resonance angiography (MRA)

Answer 35

Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes): confirmed occlusion of the proximal anterior circulation demonstrated by CTA or MRA and if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume

Answer 36

Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes): who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume

Answer 37

Recommendations from NICE include: clopidogrel is now recommended by NICE ahead of combination use of aspirin plus modified-release (MR) dipyridamole in people who have had an ischaemic stroke aspirin plus MR dipyridamole is now recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated, but treatment is no longer limited to 2 years' duration MR dipyridamole alone is recommended after an ischaemic stroke only if aspirin or clopidogrel are contraindicated or not tolerated, again with no limit on duration of treatment

Answer 38

With regards to carotid artery endarterectomy: recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis > 70% according ECST** criteria or > 50% according to NASCET*** criteria

Answer 39

Anterior cerebral artery

Answer 40

Middle cerebral artery

Answer 41

posterior cerebral artery

Answer 42

Lacunar • Present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia

Answer 43

Lateral medulla (posterior inferior cerebellar artery- PICA) • Ipsilateral: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy • Contralateral: limb sensory loss

Answer 44

Pontine • VI nerve: horizontal gaze palsy • VII nerve • Contralateral hemiparesis

Answer 45

Basilar Artery CVA is typically associated with a poor prognosis • Most of patients present with nausea, vertigo and vomiting • Some present with motor deficits, dysarthria and speech involvement or headaches • May present with visual disturbances. This includes abducens nerve palsy, conjugate gaze palsy, internuclear ophthalmoplegia and ocular bobbing • Locked-In Syndrome: patient is awake but is unable to respond in anyway except by vertical gaze and blinking (lesion is in ventral pons) • 70% of patients presenting with basilar artery territory stroke are hypertensive. • Management includes vigorous control of hypertension and antiplatelet agents.

Answer 46

LACS (lacunar stroke): Pure motor or pure sensory syndrome or Ataxic hemiparesis or Dysarthria or Clumsy-hand syndrome.

Answer 47

PACS (partial anterior circulatory stroke): 2 of the 3 elements below, or new higher cerebral dysfunction. Higher cerebral dysfunction Contralateral sensory/motor deficit Visual field defect.

Answer 48

TACS (total anterior circulatory stroke): combination of the 3 elements Higher cerebral dysfunction Contralateral sensory/motor deficit Visual field defect.

Answer 49

POCS (posterior circulation syndrome): Bilateral motor/sensory deficit or Disorder of conjugate eye movement, or Solitary visual field defect, or Cerebellar dysfunction, or Crossed cranial nerve and sensory/motor signs.

Answer 50

Lateral Medullary Syndrome: also known as Wallenberg's syndrome, occurs following occlusion of the posterior inferior cerebellar artery, resulting in sensory and sympathetic disturbances Cerebellar features • Ataxia • Nystagmus Brainstem features • Ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner's • Contralateral: limb sensory loss (pyramidal tract signs)

Answer 51

Pituitary Apoplexy: sudden enlargement of pituitary tumour secondary to hemorrhage or infarction

Answer 52

Features • Sudden onset headache similar to that seen in subarachnoid hemorrhage • Vomiting • Neck stiffness • Visual field defects: classically bitemporal superior quadrantic defect • Extraocular nerve palsies • Features of pituitary insufficiency e.g. Hypotension secondary to hypoadrenalism • Electrolytes disturbance.

Answer 53

Management: IV Hydrocortisone should be given to prevent adisonian crisis

Answer 54

Brown-Séquard syndrome: is a loss of sensation and motor function (paralysis and ataxia) that is caused by the lateral hemisection of the spinal cord.

Answer 55

• Ipsilateral loss of fine touch, vibration and proprioception • Ipsilateral hyper-reflexia and extensor plantar reflex • Contralateral loss of pain and temperature sensation occurs affecting the side. • Segmental anaesthesia at the level of the lesion • Complete syndrome picture is rare and many patients may only exhibit some features. • Trauma is a common cause; demyelination due to multiple sclerosis is another common cause but any lateral cord lesion (ischaemia, hemorrhage, granuloma, tumour etc) may give this picture.

Answer 56

Syringomyelia is a developmental, slowly enlarging cavitaryexpansion of the cervical cord that produce progressive myelopathy. Symptoms begin insidiously in adolescence or early adulthood, progress irregularly, and may undergo spontaneous arrest for several years; most patients acquire a cervical-thoracic scoliosis. • Development of cavity (syrinx) within the spinal cord • If extends into medulla then termed syringobulbia • Strongly associated (>50%) with the arnold-chiari malformation

Answer 57

MRI is the investigation of choice Myelography used to confirm the diagnosis but was associated with more deterioration

Answer 58

Transverse Myelitis: is an inflammatory lesion that can affect the cord. Constitutional symptoms such as headache and fever are common as is pain. Signs are indistinguishable from those caused by cord compression and again all sensory aspects are equally affected with no sparing of proprioception.

Answer 59

Subclavian Steal Syndrome: is associated with retrograde flow in the vertebral artery due to proximal subclavian artery stenosis. Neurological symptoms are precipitated by vigorous exercise with the arm above the head, such as painting a wall.

Answer 60

• Diagnosis is often confused with transient ischemic attacks or epilepsy. • Duplex ultrasound and MRA are the investigations of choice. • Endarterectomy and stenting are common surgical methods involved in relieving symptoms associated with this condition.

Answer 61

Subacute Combined Degeneration of Spinal Cord, also known as Lichtheim's disease, refers to degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency (most common), vitamin E deficiency or Friedrich's ataxia. It is usually associated with pernicious anemia.

Answer 62

Features: • Patchy losses of myelin in the dorsal and lateral columns. • Present with progressive weakness of legs, arms, trunk, tingling and numbness. • Visual & Mental changes may also be present. • Bilateral spastic paresis may develop and pressure, vibration and touch sense are diminished. • Positive Babinski sign may be seen. • Prolonged deficiency (> 3 months) of vitamin B12 leads to irreversible nervous system damage. • If someone is deficient in vitamin B12 and folic acid, the vitamin B12 deficiency must be treated first to avoid precipitating subacute combined degeneration of the cord. • Therapy with vitamin B12 results in partial to full recovery,depending on the duration and extent of neurodegeneration

Answer 63

Narcolepsy is a condition causing excessive daytime somnolence and an overwhelming desire to sleep. Symptoms include excessive daytime sleepiness (EDS), involuntary sleep episodes –microsleeps- cataplexy (70%), sleep paralysis hallucinations - hypnagogic (at the onset of sleep) and hypnopompic (on awakening).

Answer 64

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3 Retinal and cerebellar hemangiomas are key features of Von Hippel-Lindau syndrome. Retinal hemangiomas are bilateral in 25% of patients and may lead to vitreous hemorrhage

Answer 65

• Cerebellar hemangiomas (Can secret crythyropiotiene that causes secondary polycythemia) • Retinal hemangioma: vitreous hemorrhage • Renal cysts (premalignant) • Pheochromocytoma • Extra-renal cysts: epididymal, pancreatic, hepatic • Endolymphatic sac tumours

Answer 66

Friedreich's Ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterized by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich's ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation. The typical age of onset is 10-15 years old

Answer 67

Neurological features • Absent ankle jerks/extensor plantars • Cerebellar ataxia • Optic atrophy • Spinocerebellar tract degeneration

Answer 68

Other features • Hypertrophic obstructive cardiomyopathy (90%, most common cause of death) • Diabetes mellitus (10-20%) • High-arched palate

Answer 69

Tuberous Sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neuro-cutaneous

Answer 70

Cutaneous features • Depigmented 'ash-leaf' spots which fluoresce under UV light • Roughened patches of skin over lumbar spine (Shagreen patches) • Adenoma sebaceum: butterfly distribution over nose • Fibromata beneath nails (subungual fibromata) • Café-au-lait spots may be seen

Answer 71

Neurological features • Developmental delay • Epilepsy (infantile spasms or partial) • Intellectual impairment

Answer 72

Also • Retinal hamartomas: dense white areas on retina (phakomata) • Rhabdomyomas of the heart • Gliomatous changes can occur in the brain lesions • Polycystic kidneys, renal angiomyolipomata

Answer 73

Neurofibromatosis: both types are inherited in an autosomal dominant fashion. NF1 is also known as von Recklinghausen's syndrome. It is caused by a gene mutation on chromosome 17 which encodes neurofibromin and affects around 1 in 4,000. NF2 is caused by gene mutation on chromosome 22 and affects around 1 in 100,000

Answer 74

Café-au-lait spots (= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris: Lisch nodules in > 90% Scoliosis

Answer 75

Bilateral acoustic neuromas

Answer 76

Hereditary Sensorimotor Neuropathy (HSMN) is a relatively new term which encompasses Charcot-Marie-Tooth disease (also known as peroneal muscular atrophy). Over 7 types have been characterized - however only 2 are common to clinical practice • HSMN type I: primarily due to demyelinating pathology • HSMN type II: primarily due to axonal pathology

Answer 77

HSMN type I • Autosomal dominant • Due to defect in PMP-22 gene (which codes for myelin) (PMP = Peripheral Myelin Protein) • Features often start at puberty • Motor symptoms predominate • Distal muscle wasting, pes cavus, clawed toes • Foot drop, leg weakness often first features • Nerve Conduction Velocity is greatly ↓ <30m/second

Answer 78

Motor Neuron Disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognized including amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy and bulbar palsy. In some patients however, there is a combination of clinical patterns

Answer 79

• Typically LMN signs in arms and UMN signs in legs • In familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase

Answer 80

Primary lateral sclerosis • UMN signs only

Answer 81

Progressive muscular atrophy • LMN signs only • Affects distal muscles before proximal • Carries best prognosis

Answer 82

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

Answer 83

motor neurone disease

Answer 84

• Doesn't affect external ocular muscles • No cerebellar signs • Abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature.

Answer 85

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 ↓ number of action potentials with ↑ amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy

Answer 86

Riluzole • Anti-glutamate drug • Used mainly in amyotrophic lateral sclerosis • Prolongs life by about 3 months • Expensive Respiratory care • Non-invasive ventilation (usually BiPAP) is used at night • Studies have shown a survival benefit of around 7 months Prognosis • Poor: 50% of patients die within 3 years

Answer 87

Epilepsy is more likely to occur in young children or people over the age of 65 years; however it can occur at any time.

Answer 88

Basics • Two main categories: generalised and partial seizures • Partial seizures may progress to general seizures • Other types: myoclonic, atypical absence, atonic and tonic seizures are usually seen in childhood

Answer 89

- no focal features, consciousness lost immediately • Grand mal (tonic-clonic) • Petit mal (absence seizures) • Partial seizures progressing to generalised seizures

Answer 90

- focal features depending on location • Simple (no disturbance of consciousness or awareness) • Complex (consciousness is disturbed) • Temporal lobe → aura, déjà vu, jamais vu; motor → jacksonian Complex partial seizures can take the form of automatisms, such as chewing and swallowing, repeatedly scratching the head or searching for an object. Some people may even undress. They can occur as a result of seizure activity in any part of the brain but most commonly arise in the temporal lobes

Answer 91

Generalised tonic-clonic seizures sodium valproate second line: lamotrigine, carbamazepine

Answer 92

Absence seizures (Petit mal) sodium valproate or ethosuximide sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy carbamazepine may exacerbate

Answer 93

Myoclonic seizures sodium valproate second line: clonazepam, lamotrigine carbamazepine may exacerbate these

Answer 94

Caution should be exercised when combining sodium valproate and lamotrigine as serious skin rashes such as Steven- Johnson's syndrome may be provoked.

Answer 95

Vigabatrin – rarley used anti-epileptic • 40% of patients develop Visual field defects, which may be irreversible • Visual fields should be checked every 6 months

Answer 96

Absence Seizures (Petit Mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old and girls are affected twice as commonly as boys

Answer 97

Features • Absences last a few seconds and are associated with a quick recovery • Seizures may be provoked by hyperventilation or stress • The child is usually unaware of the seizure • They may occur many times a day • EEG: bilateral, symmetrical 3Hz (spike and wave) pattern

Answer 98

Management • Sodium valproate and ethosuximide are first-line treatment • Good prognosis - 90-95% become seizure free in adolescence

Answer 99

Focal seizures carbamazepine or lamotrigine second line: levetiracetam, oxcarbazepine or sodium valproate

Answer 100

AED cessation can be considered if seizure free for > 2 years – Stop AEDs over 2-3 months

Answer 101

Therapy stopped: chance of recurrence in the next 2 years is 43% Therapy cont.: 10% Factors that have been shown to increase the risk of seizures include: • Older age • Use of multiple anticonvulsants • History of myoclonic or tonic clonic seizure • Previous abnormal imaging or EEG • Seizure while on therapy.

Answer 102

The risks of uncontrolled epilepsy during pregnancy generally outweigh the risks of medication to the fetus. All women thinking about becoming pregnant should be advised to take folic acid 5mg/day well before pregnancy to minimise the risk of neural tube defects. Around 1-2% of newborns born to non- epileptic mothers have congenital defects. This rises to 3-4% if the mother takes antiepileptic medication

Answer 103

• Aim for monotherapy • There is no indication to monitor antiepileptic drug levels • Sodium valproate: associated with neural tube defects • Phenytoin (Epanutin®): associated with cleft palate • Lamotrigine: studies to date suggest the rate of congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy • Carbamazepine: often considered the least teratogenic of the older antiepileptics

Answer 104

Breast feeding is generally considered safe for mothers taking antiepileptics with the possible exception of the barbiturates

Answer 105

It is advised that pregnant women taking phenytoin are given vitamin K in the last month of pregnancy to prevent clotting disorders in the newborn

Answer 106

• Occurs between 3-12 month of age, managed by Vegabatrin (S.E causes alopecia, ↓ visual acuity diplopia). • West syndrome is the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia), and mental retardation - although the international definition requires only two out of these three elements.

Answer 107

Non-epileptic attacks are broadly divided into: • Hyperkinetic/thrashing attacks • Akinetic/motionless attacks.

Answer 108

pseudoseizure

Answer 109

Video telemetry Prolactin is often elevated 15–20 min after a tonic–clonic seizure and should be normal in non-epileptic attacks, but there are a number of problems: serum rises are seen in syncopal episodes; it may be normal, especially in partial epileptic seizures; and the test is often badly carried out in practice (too early or too late).

Answer 110

Parkinson's Disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra

Answer 111

bradykinesia, asymetrical tremor and rigidity. The symptoms of Parkinson's disease are characteristically asymmetrical

Answer 112

Bradykinesia • Poverty of movement also seen: mask-like face • Difficulty in initiating movement

Answer 113

Tremor • Most marked at rest, 3-5 Hz • Typically 'pill-rolling'

Answer 114

Rigidity • Lead pipe • Cogwheel: due to superimposed tremor

Answer 115

• Flexed posture • Short, shuffling steps • Micrographia • Drooling of saliva • Psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur • Impaired olfaction • REM sleep behaviour disorder

Answer 116

Parkinsonion syndrome?

Answer 117

Drug-induced Parkinsonism has slightly different features to Parkinson's disease: • Motor symptoms are generally rapid onset and bilateral • Rigidity and rest tremor are uncommon

Answer 118

Drugs causing Parkinsonism • Phenothiazines: e.g. Chlorpromazine • Butyrophenones: haloperidol, droperidol • Metoclopramide

Answer 119

Domperidone does not cross the blood-brain barrier and therefore does not cause extra-pyramidal side-effects

Answer 120

Currently accepted practice in the management of patients with Parkinson's disease (PD) is to delay treatment until the onset of disabling symptoms

Answer 121

For first-line treatment: if the motor symptoms are affecting the patient's quality of life: levodopa if the motor symptoms are not affecting the patient's quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO‑B) inhibitor

Answer 122

Dopamine receptor agonists (favored for patients > 75yrs old) • E.g. Bromocriptine, Pramipexole, Ropinirole, Cabergoline, Apomorphine • Ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an ESR, creatinine and CXR should be obtained prior to treatment and patients should be closely monitored • Ropinirole is least associated with tissue fibrosis. • Patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence Levodopa • Usually combined with a decarboxylase inhibitor (e.g. Carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopamine • ↓ effectiveness with time (usually by 2 years) • Unwanted effects: dyskinesia, 'on-off' effect • Not used in neuroleptic induced parkinsonism • Favoed for patients < 75yrs MAO-B (Monoamine Oxidase-B) inhibitors • E.g. Selegiline • Inhibits the breakdown of dopamine secreted by the dopaminergic neurons Amantadine • Mechanism is not fully understood, probably ↑ dopamine release and inhibits its uptake at dopaminergic synapses COMT (Catechol-O-Methyl Transferase) inhibitors • E.g. Entacapone • COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy • Used in established PD Antimuscarinics • Block cholinergic receptors • Now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease • Help tremor and rigidity • E.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)

Answer 123

More improvement in motor symptoms More improvement in ADLs More motor complications Fewer specified adverse events Adverse effects of L-Dopa • Dyskinesia • 'On-off' effect • Postural hypotension • Cardiac arrhythmias • Nausea & vomiting • Psychosis • Reddish discolouration of urine upon standing

Answer 124

Less improvement in motor symptoms Less improvement in activities of daily living Fewer motor complications More specified adverse events More off‑time reduction More risk of hallucinations

Answer 125

Less improvement in motor symptoms Less improvement in activities of daily living Fewer motor complications Fewer specified adverse events Off‑time reduction Fewer adverse events Lower risk of hallucinations

Answer 126

Improvement in motor symptoms Improvement in activities of daily living Off‑time reduction More adverse events Lower risk of hallucinations

Answer 127

No evidence of improvement in motor symptoms No studies for: Off-time Adverse events (excessive sleepiness, hallucinations and impulse control disorders) risk of hallucinations

Answer 128

Consider glycopyrronium bromide to manage drooling of saliva in people with Parkinson's disease.

Answer 129

If excessive daytime sleepiness develops then patients should not drive. Medication should be adjusted to control symptoms. Modafinil can be considered if alternative strategies fail.

Answer 130

If orthostatic hypotension develops then a medication review looking at potential causes should be done. If symptoms persist then midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance) can be considered.

Answer 131

Multiple system atrophy: Shy-Drager syndrome is a type of multiple system atrophy Features • Parkinsonism • Autonomic disturbance (atonic bladder, postural hypotension) • Cerebellar signs

Answer 132

Progressive Supranuclear Palsy is degenerative disease involving the gradual deterioration and death of selected areas of the brain. Both ♂ and ♀ are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000. • AKA Steele-Richardson-Olszewski syndrome • A 'Parkinson Plus' syndrome

Answer 133

Features • Impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs) • Parkinsonism • Falls • Slurring of speech • Cognitive impairment

Answer 134

Management • Poor response to L-dopa

Answer 135

Lewy Body Dementia is an increasingly recognised cause of dementia, accounting for up to 20% of cases. The characteristic pathological feature is cytoplasmic neuronal inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas

Answer 136

Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible Parkinsonism. Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agent

Answer 137

Features • Progressive cognitive impairment • Parkinsonism • Visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen)

Answer 138

Essential tremor: (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs.

Answer 139

Features • Postural tremor: worse if arms outstretched (usually 6-8 Hz) • Improved by alcohol and rest • Most common cause of titubation (head tremor)

Answer 140

Management • Propranolol is first-line • Primidone (anticonvulsant ) is sometimes used when propanolol is contraindicated

Answer 141

Creutzfeldt - Jakob disease (CJD): Basics & Features: • Rapidly progressive, severe and invariably fatal (usually within few months) • Dementia • Cerebellar ataxia • Defuse myoclonic jerks • Other neurological abnormalities. Myoclonus is typical and progressive, even during the later stage when the patient is stuporous or comatose.

Answer 142

Investigation: • The EEG patern is characteristic (diffuse non specific slowing periodic sharp wave complexes- PSWCs of 1 – 2 Hz), but diagnosis relies on either espiecalized tests for prion protein in CSF or direct brain biopsy. • Pulvinar Sign on cranial MRI > 90% pathological in vCJD (Not Sporadic) • Prion protien in tonsils • 14-3-3 protien in CSF

Answer 143

Sporadic CJD predominantly affects late mid-aged individuals with mean age of death in the late 60s. Memory impairment and cerebellar ataxia are common early features, subsequently, rapidly progressive dementia and myoclonus. The median duration of illness is 4 months and about 65% of caeses die within 6 months.

Answer 144

Genetics • Most cases are sporadic • 5% are inherited as an autosomal dominant trait • Mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form • Apoprotein E allele E4 - encodes a cholesterol transport protein

Answer 145

Pathological changes • Macroscopic = widespread cerebral atrophy, particularly involving the cortex and hippocampus • Microscopic = intraneuronal neurofibrillary tangles, neuronal plaques, deficiency of neurons • Biochemical = deposition of type A-β-amyloid protein in cortex, deficit of Ach from damage to an ascending forebrain projection

Answer 146

Neurofibrillary tangles • Paired helical filaments are partly made from a protein called tau • In Alzheimer: tau proteins are excessively phosphorylated

Answer 147

Management • NICE now recommend the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer’s disease. Donepezil may cause bradycardia and atrioventricular node block. • Memantine is reserved for patients with moderate - severe Alzheimer’s.

Answer 148

Hemiballism occurs following damage to the subthalamic nucleus.

Answer 149

Features - Ballisic movements: • Involuntary, sudden, jerking movements which occur contralateral to the side of the lesion • Primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements. • Symptoms may decrease during sleeping.

Answer 150

• Etiology: stroke in elders, infection or inflammatory in young.

Answer 151

• Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment. Tetrabenazine may be considered when long-term therapy is required.

Answer 152

Supply - 'face, ear, taste, and 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

Answer 153

Facial Palsy + convergent squint - lesion is in Pons as VIth is encircled by VIIth

Answer 154

Causes of bilateral facial nerve palsy • Sarcoidosis • Guillain-Barre syndrome • Polio, Lyme disease

Answer 155

• Bell's palsy • Ramsay-Hunt syndrome (due to herpes zoster) • Acoustic neuroma • Parotid tumours • HIV • Multiple Sclerosis • Diabetes Mellitus NO

Answer 156

stroke YES

Answer 157

Bell's palsy may be defined as an acute, unilateral, idiopathic, facial nerve paralysis. The aetiology is unknown although the role of the herpes simplex virus has been investigated previously.

Answer 158

Features • Lower motor neuron facial nerve palsy - forehead affected • Patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes

Answer 159

Management • In the past a variety of treatment options have been proposed including no treatment, prednisolone only and a combination of aciclovir and prednisolone • Following a National Institute for Health randomised controlled trial it is now recommended that prednisolone 25mg BD for 10 days should be prescribed for patients within 72 hours of onset of Bell’s palsy. Adding in aciclovir gives no additional benefit • Eye care is important - prescription of artificial tears and eye lubricants should be considered

Answer 160

Prognosis • If untreated around 15% of patients have permanent moderate to severe weakness

Answer 161

Multiple Sclerosis: (it is an UML) also known as disseminated sclerosis or encephalomyelitis disseminate, is an autoimmune disease in which the body's immune response attacks a person's central nervous system (brain and spinal cord), leading to demyelination. Disease onset usually occurs in young adults, and it is more common in ♀.

Answer 162

Relapsing-remitting: Characterized by unpredictable relapses followed by periods of months to years of relative quiet (remission) with no new signs of disease activity. Deficits suffered during attacks may either resolve or leave sequelae. This describes the initial course of 85–90% of individuals with MS. When deficits always resolve between attacks, this is sometimes referred to as benign MS.

Answer 163

Secondary progressive: Describes those with initial relapsing-remitting MS, who then begin to have progressive neurologic decline between acute attacks without any definite periods of remission. Occasional relapses and minor remissions may appear. The median time between disease onset and conversion from relapsing-remitting to secondary progressive MS is 19 years.

Answer 164

Primary progressive: Describes the approximately 10–15% of individuals who never have remission after their initial MS symptoms. It is characterized by progression of disability from onset, with no, or only occasional and minor, remissions and improvements. The age of onset for the primary progressive subtype is later than other subtypes.

Answer 165

Progressive relapsing: Describes those individuals who, from onset, have a steady neurologic decline but also suffer clear superimposed attacks. This is the least common of all subtypes.

Answer 166

Non-standard behaviors: Have also been described. Sometimes referred to as borderline forms of MS, these include Devic's disease, Balo concentric sclerosis, Schilder's diffuse sclerosis and Marburg MS. MS also behaves differently in children. There is debate whether these are atypical variants of MS or different diseases

Answer 167

Visual • Optic neuritis: common presenting feature • Optic atrophy • Uhthoff's phenomenon: worsening of vision following rise in body temperature (hot bath) • Internuclear ophthalmoplegia

Answer 168

Sensory • Pins/needles • Numbness • Trigeminal neuralgia • Lhermitte's syndrome: paraesthesiae in limbs on neck flexion

Answer 169

Motor • Spastic weakness

Answer 170

Cerebellar • Ataxia, Tremor

Answer 171

Urinary incontinence

Answer 172

Sexual dysfunction

Answer 173

Intellectual deterioration

Answer 174

Good prognosis features • ♀sex • Young age of onset • Relapsing-remitting disease • Sensory symptoms • long interval between first two relapses TYPICAL PRESENTATION

Answer 175

Diagnosis requires demonstration of lesions disseminated in time and space MRI • High signal T2 lesions • Periventricular plaques CSF • Oligoclonal bands (and not in serum) • ↑ intrathecal synthesis of IgG Visual evoked potentials • Delayed, but well preserved wave form

Answer 176

Treatment in multiple sclerosis is focused at reducing the frequency and duration of relapses. There is no cure. High dose steroids (e.g. IV methylprednisolone) may be given for 3-5 days to shorten the length of an acute relapse. Baclofen is helpful in controlling spasticity. Hallucinations are occasionally seen on the withdrawal of baclofen

Answer 177

Beta-interferon has been shown to reduce the relapse rate by up to 30%. Certain criteria have to be met before it is used: relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided) reduces number of relapses and MRI changes, however doesn't reduce overall disability Other drugs used in the management of multiple sclerosis include: glatiramer acetate: immunomodulating drug - acts as an 'immune decoy' natalizumab: a recombinant monoclonal antibody that antagonises Alpha-4 Beta-1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available

Answer 178

Fatigue once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine other options include mindfulness training and CBT

Answer 179

baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine physiotherapy is important cannabis and botox are undergoing evalulation

Answer 180

may take the form of urgency, incontinence, overflow etc guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients if significant residual volume → intermittent self-catheterisation if no significant residual volume → anticholinergics may improve urinary frequency

Answer 181

Oscillopsia (visual fields apper to oscillate) gabapentin is first-line

Answer 182

Ptosis + dilated pupil = third nerve palsy

Answer 183

Horner's syndrome

Answer 184

• Opiates • Parasympathomimetics: pilocarpine • Organophosphate toxicity

Answer 185

constricts

Answer 186

Argyll-Robertson: small irregular pupils that do not react to light but react to accommodation. Causes: Multiple Sclerosis, Sarcoidoisis, DM Referred to as the "Whore's Eye" because of the association with tertiary syphilis and because of the convenient mnemonic that, like a prostitute, they “accommodate but do not react”

Answer 187

Horner's syndrome - anhydrosis determines site of lesion: • Head, arm, trunk = central lesion: stroke, syringomyelia • Just face = pre-ganglionic lesion: Pancoast's, cervical rib • Absent = post-ganglionic lesion: carotid artery It is caused by an interruption of the sympathetic pupillomotor fibres

Answer 188

central: STEMS stroke tumour encephalitis multiple sclerosis syringomyelia

Answer 189

pre-ganglionic: PCT pancoast tumour cervical rib trauma thyroidectomy

Answer 190

Post-ganglionic: all the C's Carotid artery dissection Carotid aneurysm Cavernous sinus thrombosis Cluster headache

Answer 191

• Myotonic dystrophy • Myasthenia gravis* • Syphilis • Congenital *ptosis is much less common in Lambert-Eaton syndrome than myasthenia gravis

Answer 192

• Third nerve palsy • Horner's

Answer 193

Painful third nerve palsy = posterior communicating artery aneurysm

Answer 194

• Eye is deviated 'down and out' • Ptosis • Pupil may be dilated (sometimes called a 'surgical' third nerve palsy) → PCA aneurysm

Answer 195

• Diabetes Mellitus • Vasculitis e.g. Temporal arteritis, SLE • False localizing sign due to uncal herniation through tentorium if raised ICP • Posterior communicating artery aneurysm (pupil dilated) • Cavernous sinus thrombosis • Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes (cerebral peduncle) • Other possible causes: amyloid, multiple sclerosis

Answer 196

Myasthenia Gravis: Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 90% of cases (antibodies are less commonly seen in disease limited to the ocular muscles).

Answer 197

Myasthenia is more common in women (2:1). It is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the post-synaptic neuromuscular junction.

Answer 198

At 200–400 cases per million it is one of the less common autoimmune disorders. The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis. Symptoms become more marked during the day

Answer 199

Features • Extraocular muscle weakness: diplopia • Proximal muscle weakness: face, neck, limb girdle • Ptosis • Dysphagia

Answer 200

Associations • Thymomas in 15% • Autoimmune disorders: pernicious anemia, hypothyroidism, rheumatoid, SLE • Thymic hyperplasia in 50-70%

Answer 201

Management • Long-acting anticholinesterase e.g. Pyridostigmine • Immunosuppression: prednisolone initially (although sometimes it can worsen the symptoms) • Thymectomy

Answer 202

Management • Long-acting anticholinesterase e.g. Pyridostigmine • Immunosuppression: prednisolone initially (although sometimes it can worsen the symptoms) • Thymectomy

Answer 203

myaesthenia gravis patients - may worsen symptoms

Answer 204

Lambert-Eaton Myasthenic Syndrome is seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system

Answer 205

Features • Repeated muscle contractions lead to ↑ muscle strength* (in contrast to myasthenia gravis) • Limb girdle weakness (affects lower limbs first). Proximal muscles more commonly affected. • Hyporeflexia • Autonomic symptoms: dry mouth, impotence, difficultly micturating • Ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis) *in reality this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually ↓

Answer 206

EMG • Incremental response to repetitive electrical stimulation

Answer 207

Management -treatment of underlying cancer -immunosuppression, for example with prednisolone and/or azathioprine -3,4-diaminopyridine is currently being trialled works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate intravenous immunoglobulin therapy and plasma exchange may be beneficial

Answer 208

Anti-Hu (imagine H sticks as 2 lungs or 2 brain hemispheres)

Answer 209

Anti-Yo (imagine Y as lady’s private organ

Answer 210

Anti-GAD antibody

Answer 211

It is believed to be due to an autoimmune reaction targeted against components of the central nervous system (specifically Purkinje cells and large brain stem nuclei). It is thought to be caused by an anti-neuronal Antibody known as anti-Yo. It typically presents as a subacute progressive cerebellar ataxia, both truncal and appendicular.

Answer 212

Acoustic Neuromas account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle. AKA Vestibular schwannoma, it is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (CN VIII). The term "acoustic" is a misnomer, as the tumor rarely arises from the acoustic (or cochlear) division of the vestibulocochlear nerve

Answer 213

Features can be predicted by the affected cranial nerves • Cranial nerve VIII: hearing loss, vertigo, tinnitus • Cranial nerve V: absent corneal reflex • Cranial nerve VII: facial palsy

Answer 214

Bilateral vestibular schwannomas are seen in neurofibromatosis type 2.

Answer 215

Patients with a suspected vestibular schwannoma should be referred urgently to ENT. It should be noted though that the tumours are often slow growing, benign and often observed initially.

Answer 216

MRI of the cerebellopontine angle is the investigation of choice. Audiometry is also important as only 5% of patients will have a normal audiogram.

Answer 217

Management is with either surgery, radiotherapy or observation.

Answer 218

Glomus Jugulare Tumours are slow-growing, highly vascular tumours derived from neural tissue and arising within the jugular foramen of the temporal bone.

Answer 219

♀:♂ ratio between 3 and 6:1 - Annual incidence is around 1 in 1.3 million. Tend to present between 40 and 70 years of age.

Answer 220

Presents with deafness and pulsatile tinnitus Rarely causes hearing loss (4%) with vertigo (if extends to middle ear)

Answer 221

Cranial nerves IX to XI pass through the jugular foramen, and so are commonly involved. Less commonly, affect cranial nerves VII and XII.

Answer 222

Otoscopic examination may reveal a characteristic pulsatile reddish-blue mass behind the tympanic membrane.

Answer 223

Surgical resection is the treatment of choice, embolisation and radiotherapy have been used.

Answer 224

Demyelinating pathology

Answer 225

axonal pathology

Answer 226

Predominately motor loss

Answer 227

• Secondary to both direct toxic effects and ↓ absorption of B vitamins • Sensory symptoms typically present prior to motor symptoms

Answer 228

• Subacute combined degeneration of spinal cord • Dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

Answer 229

• Antibiotics: nitrofurantoin, metronidazole • Amiodarone • Isoniazid • Vincristine and most of the anti-cancer chemotherapy • Tricyclic antidepressants

Answer 230

Demyelinating • Reduced conduction velocity • Normal amplitude

Answer 231

Axonal • Normal conduction velocity • Reduced amplitude

Answer 232

Features • Impotence, inability to sweat • Postural hypotension e.g. Drop of 30/15 mmHg • Loss of ↓ in heart rate following deep breathing • Pupils: dilates following adrenaline instillation

Answer 233

autonomic neuropathy

Answer 234

Guillain-Barre Syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). It is usually a subacute neuropathy.

Answer 235

Pathogenesis • Cross reaction of antibodies with gangliosides in the peripheral nervous system • Correlation between anti-ganglioside antibody (e.g. Anti-GM1) and clinical features has been demonstrated • Anti-GM1 antibodies in 25% of patients

Answer 236

• Campylobacter jejuni • Chlamydia • HBV • Mycoplasma Pneumoniae • CMV, EBV, HZV, HIV

Answer 237

• Usually presents subacutely (over days to weeks) as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. It may involve respiratory muscles leading to their paralysis.

Answer 238

• Plasma exchange • IV immunoglobulins • Steroids and immunosuppressants have not been shown to be beneficial • FVC regularly to monitor respiratory function

Answer 239

Prognosis • 20% suffer permanent disability, 5% die

Answer 240

• Age > 40 years • Poor upper extremity muscle strength • Rapid symptoms progression. • Previous history of a diarrhoeal illness (specifically campylobacter jejuni) • High anti-GM1 antibody titre • Need for ventilatory support There is currently contradictory evidence as to whether a gradual or rapid onset of GBS is associated with a poor outcome

Answer 241

Miller-Fisher syndrome • Variant of Guillain-Barre syndrome • Associated with areflexia, ataxia, ophthalmoplegia • Usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome • Anti-GQ1b antibodies are present in 90% of cases

Answer 242

Herpes Simplex (HSV) Encephalitis is a common topic in the MRCP. The virus characteristically affects the temporal lobes - questions may give the result of imaging or describe temporal lobe signs e.g. aphasia

Answer 243

Features • Fever, headache, psychiatric symptoms, seizures, vomiting • Focal features e.g. Aphasia • Peripheral lesions (e.g. Cold sores) have no relation to presence of HSV encephalitis

Answer 244

Pathophysiology • HSV-1 responsible for 95% of cases in adults • Typically affects temporal and inferior frontal lobes

Answer 245

Investigation • CSF: lymphocytosis, elevated protein • PCR for HSV • CT: medial temporal and inferior frontal changes (e.g. Petechial hemorrhages) - normal in one-third of patients • MRI is better • EEG pattern: lateralised periodic discharges (LPD, not LAPD☺) at 2 Hz

Answer 246

Treatment • IV aciclovir

Answer 247

The prognosis is dependent on whether aciclovir is commenced early. If treatment is started promptly the mortality is 10-20%. Left untreated the mortality approaches 80%

Answer 248

Sub-acute Sclerosing Panencephalitis: It is recognized to be the result of chronic measles infection. It is now an uncommon disease after the widespread use of measles vaccination. There is usually a history of measles very early in life followed by 8 years of asymptomatic period.

Answer 249

• Evolves in several stages. " Initially: decline in proficiency in school. " Followed by diffuse myoclonic jerks in association with focal and generalised seizures and visual deterioration due to choiroidoretinitis. " Followed by pyramidal signs, rigidity and progressive unresponsiveness. " Finally the patient lies decorticated followed by death.` The course of the illness is 1 to 3 years. No effective treatment is available

Answer 250

1. Encephalitis • May be due to CMV or HIV itself • HSV encephalitis but is relatively rare in the context of HIV • CT: edematous brain

Answer 251

2. Cryptococcus • Most common fungal infection of CNS • Headache, fever, malaise, nausea/vomiting, seizures, focal neurological deficit • CSF: high opening pressure, India ink test positive • CT: meningeal enhancement, cerebral edema → • Meningitis is typical presentation but may occasionally cause a space occupying lesion

Answer 252

3. Progressive multifocal leukoencephalopathy (PML) • Widespread demyelination • Due to infection of oligodendrocytes by human papovirus (jc virus) • Symptoms, subacute onset : behavioural changes, speech, motor, visual impairment • CT: single or multiple lesions, no mass effect, don't usually enhance, Low attenuation diffusely • MRI is better - high-signal demyelinating white matter lesions are seen in advanced HIV.

Answer 253

4. AIDS dementia complex • Caused by HIV virus itself • Symptoms: behavioural changes, motor impairment • CT: cortical and subcortical atrophy

Answer 254

1. Toxoplasmosis • Accounts for around 50% of cerebral lesions in patients with HIV • Constitutional symptoms, headache, confusion, drowsiness • CT: usually single or multiple ring enhancing lesions, mass effect may be seen • Management: sulfadiazine and pyrimethamine

Answer 255

2. Primary CNS lymphoma • Accounts for around 30% of cerebral lesions • Associated with the Epstein-Barr virus • CT: single or multiple ring enhancing lesions

Answer 256

3. Tuberculosis • Much less common than toxoplasmosis or primary lymphoma • CT: Single enhancing lesion

Answer 257

Myotonic dystrophy (also called dystrophia myotonica) DM1

Answer 258

Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.

Answer 259

Genetics • Autosomal dominant • A trinucleotide repeat disorder • DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19 • DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3

Answer 260

General features (typically in the above figure) • Myotonic facies (long, 'haggard' appearance) • Frontal balding • Bilateral ptosis • Cataracts • Dysarthria Slow-relaxing grip may be noticed on initial hand-shake with the patient and is typical of myotonic dystrophy. Dysarthric speech is secondary to myotonia of the tongue and pharynx

Answer 261

Myotonia (tonic spasm of muscle) Weakness of arms and legs (distal initially) Mild mental impairment Diabetes mellitus Testicular atrophy Cardiac involvement: heart block, cardiomyopathy Dysphagia

Answer 262

Spastic Paraparesis: describes a upper motor neuron pattern of weakness in the lower limbs

Answer 263

Trigeminal neuralgia is a pain syndrome characterized by severe unilateral pain. The vast majority of cases are idiopathic but compression of the trigeminal roots by tumours or vascular problems may occur. The International Headache Society defines trigeminal neuralgia as: • A unilateral disorder characterized by brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve • The pain is commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously • Small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas) • The pains usually remit for variable periods

Answer 264

Management carbamazepine is first-line failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology

Answer 265

Sensory changes Deafness or other ear problems History of skin or oral lesions that could spread perineurally Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally Optic neuritis A family history of multiple sclerosis Age of onset before 40 years

Answer 266

Causes multiple sclerosis diabetes syphilis

Answer 267

unilateral decrease in visual acuity over hours or days poor discrimination of colours, 'red desaturation' pain worse on eye movement relative afferent pupillary defect central scotoma

Answer 268

Management high-dose steroids recovery usually takes 4-6 weeks

Answer 269

Prognosis MRI: if > 3 white-matter lesions, 5-year risk of developing multiple sclerosis is c. 50%

Answer 270

NICE updated their guidance on the management of neuropathic pain in 2013: first-line treatment: amitriptyline, duloxetine, gabapentin or pregabalin if the first-line drug treatment does not work try one of the other 3 drugs in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia) pain management clinics may be useful in patients with resistant problems

Answer 271

Features • Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours • Clusters typically last 4-12 weeks • Intense pain around one eye (recurrent attacks 'always' affect same side) • Patient is restless during an attack • Accompanied by redness, lacrimation, lid swelling • Nasal stuffiness • Miosis and ptosis in a minority

Answer 272

Management • Acute: 100% oxygen, subcutaneous sumatriptan (5-HT1D receptor agonist), nasal lidocaine • Prophylaxis: verapamil, prednisolone • Consider specialist referral

Answer 273

• Unilateral pain which is generally oculofrontotemporal in location. • Can occur at any time, and patients often describe a throbbing, boring, pulsating or claw-like pain • Frequency of attacks is usually 10–20 per day. Episodes usually last 2–25 min, but may last as long as 60 min • Patients may also complain of ipsilateral conjunctival injection, ptosis and lid swelling, lacrimation and rhinorrhoea, occasionally photophobia

Answer 274

• The most effective treatment is with indomethacin

Answer 275

short-lasting unilateral neuralgiform headache with conjunctival injection and tearing

Answer 276

CH has male preponderance, unlike CPH, which is more common in females. In CPH, frequency of attacks is higher, usually more than 15 in 24 hours, whereas CH has an attack frequency of 1-4 (maximum 8) in 24 hours. The duration of headaches is shorter in CPH (2-25 min) than in CH (15-60 min).

Answer 277

A At least 5 attacks fulfilling criteria B-D B Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated) C Headache has at least two of the following characteristics: 1. unilateral location 2. pulsating quality (i.e., varying with the heartbeat) 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs) D During headache at least one of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia E Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)

Answer 278

In children, attacks may be shorter-lasting, headache is more commonly bilateral, and gastrointestinal disturbance is more prominent

Answer 279

Standard analgesia, Triptans & Ergotamine. Standard analgesia? • First-line therapy • E.g. Paracetamol, ibuprofen, aspirin, may be poorly absorbed, often combined with anti-emetic e.g. Metoclopramide (to relieve associate nausea) → caution should be exercised with young patients as acute dystonic reactions may develop Avoid aspirin in children < 16 years as risk of Reye's syndrome Triptans • Second-line therapy • Specific 5-HT1 agonists - opposes Vasodilation Ergotamine • α-blocker and a partial 5-HT1 agonist • Now rarely used due to high incidence of adverse effects (e.g. Nausea and vomiting) • Listed in the BNF as 'less suitable for prescribing'

Answer 280

Prophylaxis should be given if patients are experiencing ≥ 2 attacks/month. Treatment is effective in about 60% of patients First-line • β-blockers: propranolol 80-240mg OD Also recommended in the SIGN guidelines • Sodium valproate • Topiramate (CKS recommend this is used under specialist supervision) • Gabapentin • Amitriptyline • Venlafaxine The SIGN guidelines also suggest that stress management and acupuncture may be useful 5-HT2 antagonists • Pizotifen: used less commonly now due to adverse effects (weight gain and drowsiness) • Methysergide: very rarely used as associated with retroperitoneal fibrosis

Answer 281

Migraine during pregnancy • Paracetamol 1g is first-line • Aspirin 300mg or ibuprofen 400mg can be used second-line in the first and second trimester

Answer 282

Migraine and the combined oral contraceptive (COC) pill • If patients have migraine with aura then the COC is absolutely contraindicated due to an increased risk of stroke (relative risk 8.72)

Answer 283

Migraine and menstruation • Many women find that the frequency and severity of migraines increase around the time of menstruation • SIGN recommends that women are treated with mefanamic acid or a combination of aspirin, paracetamol and caffeine. Triptans are also recommended in the acute situation

Answer 284

Migraine and hormone replacement therapy (HRT) • Safe to prescribe HRT for patients with a history of migraine but it may make migraines worse

Answer 285

Normal values of cerebrospinal fluid (CSF) are as follows: • Pressure = 60-150 mm (patient recumbent) • Protein = 0.2-0.4 g/l • Glucose = > 2/3 blood glucose • Cells: red cells = 0, white cells < 5/mm3

Answer 286

• Viral meningitis/encephalitis • TB meningitis • Partially treated bacterial meningitis • Lyme disease • Behcet's, SLE • Lymphoma, leukemia

Answer 287

• Tuberculous, fungal and bacterial meningitis • Viral encephalitis • Guillain-Barre syndrome • Spinal block (Froin's syndrome)

Answer 288

Post-LP headaches are more common in young ♀ with a low BMI. Typical features • Usually develops within 24-48 hours following LP but may occur up to one week later • May last several days • Worsens with upright position • Improves with recumbent position

Answer 289

↑ needle size Direction of bevel Not replacing the stylet ↑ number of LP attempts

Answer 290

↑ volume of CSF removed Bed rest following procedure ↑ fluid intake post procedure Opening pressure of CSF Position of patient

Answer 291

• Supportive initially (analgesia, rest) • If pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural hematoma • Treatment options include: blood patch, epidural saline and intravenous caffeine

Answer 292

Oppenheim's sign is seen when scratching of the inner side of leg leads to extension of the toes. It is a sign of cerebral irritation

Answer 293

• Polymyositis: reduced amplitude and duration of motor unit

Answer 294

MND: fibrillation due to denervation

Answer 295

Myasthenia Gravis: diminished responses to repitative stimulations

Answer 296

Lambert Eaton Myasthenia: enhanced responses to repitative stimulations.

Answer 297

Myotonia Dystophia: High frequency action potentioals, Kamikaze discharge or dive bombers frequency which is heard

Answer 298

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

Answer 299

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

Answer 300

Unicentric Castleman's disease is a lymphoproliferative disorder associated in a subset of cases with HIV and HHV-8.

Answer 301

Patient's with unicentric Castleman's disease tend to be asymptomatic and lymphadenopathy is constrained to one lymph node group.

Answer 302

The most common sites of the disease being the chest (24 percent), neck (20 percent), abdomen (18 percent), and retroperitoneum (14 percent).

Answer 303

Biopsy of the lymph node commonly shows regressed germinal centres surrounded by prominent mantle zones.

Answer 304

Possible causes include: common peroneal nerve lesion - the most common cause L5 radiculopathy sciatic nerve lesion superficial or deep peroneal nerve lesion other possible includes central nerve lesions (e.g. stroke) but other features are usually present

Answer 305

A common peroneal nerve lesion is the most common cause . This is often secondary to compression at the neck of the fibula. This may be caused by certain positions such as leg crossing, squatting or kneeling. Prolonged confinement, recent weight loss, Baker's cysts and plaster casts to the lower leg are also known to be precipitating factors.

Answer 306

Examination if the patient has an isolated peroneal neuropathy there will be weakness of foot dorsiflexion and eversion. Reflexes will be normal weakness of hip abduction is suggestive of a L5 radiculopathy

Answer 307

Bilateral symptoms, fasiculations or other abnormal neurological findings (e.g. hyperreflexia) are indications for specialist referral.

Answer 308

If the examination suggests a peroneal neuropathy then conservative management is appropriate. Leg crossing, squatting and kneeling should be avoided. Symptoms typically improve over 2-3 months

Answer 309

a cause of horizontal disconjugate eye movement due to a lesion in the medial longitudinal fasciculus (MLF) controls horizontal eye movements by interconnecting the IIIrd, IVth and VIth cranial nuclei located in the paramedian area of the midbrain and pons

Answer 310

Features impaired adduction of the eye on the same side as the lesion horizontal nystagmus of the abducting eye on the contralateral side

Answer 311

Causes multiple sclerosis vascular disease

Answer 312

This area of the hypothalamus along with the paraventricular nucleus of the hypothalamus is responsible for the synthesis of antidiuretic hormone and oxytocin, which are transported to the posterior hypothalamus for storage and release.

Answer 313

The posterior hypothalamus is responsible for heat generation to maintain core body temperature.

Answer 314

3: The anterior hypothalamus is responsible for heat dissipation to cool down the body to prevent a rise in temperature which would be detrimental to body's internal environment.

Answer 315

4: The ventromedial area of the hypothalamus is often invaded by craniopharyngiomas. This area of the thalamus controls the satiety center and it is removed during surgery, the patient can have uninhibited hunger leading to significant weight gain.

Answer 316

5: This area of the hypothalamus along with the supraoptic nucleus of the hypothalamus is responsible for the synthesis of antidiuretic hormone and oxytocin, which are transported to the posterior hypothalamus for storage and release.

Answer 317

Meniere's disease is a disorder of the inner ear of unknown cause. It is characterized by excessive pressure and progressive dilation of the endolymphatic system. It is more common in middle- aged adults but may be seen at any age. Meniere's disease has a similar prevalence in both men and women

Answer 318

Features • Recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom • A sensation of aural fullness or pressure is now recognised as being common • Other features include nystagmus and a positive Romberg test (patient can’t stand-alone when eyes closed and feet together) • Episodes last minutes to hours

Answer 319

Natural history • Symptoms resolve in the majority of patients after 5-10 years • Some patients may be left with hearing loss • Psychological distress is common

Answer 320

Management • ENT assessment is required to confirm the diagnosis • Patients should inform the DVLA. The current advice is to cease driving until satisfactory control of symptoms is achieved • Acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required • Prevention: betahistine may be of benefit

Answer 321

Rinne's test • Tuning fork is placed over mastoid process, followed by repositioning just over external acoustic meatus • Air conduction (AC) is normally better than bone conduction (BC) • If BC > AC then conductive deafness

Answer 322

Weber's test • Tuning fork is placed over middle of forehead, patient is asked which side is loudest • In unilateral sensorineural deafness, sound is localised to the 'good' side • In unilateral conductive deafness, sound is localised to the 'bad' side

Answer 323

otosclerosis

Answer 324

acoustic neuroma

Answer 325

Aspirin Aminoglycosides Loop diuretics Quinine

Answer 326

Ramsay Hunt syndrome: (herpes zoster oticus) is caused by the reactivation of the varicella zoster virus in the geniculate ganglion of the seventh cranial nerve.

Answer 327

Features • Auricular pain is often the first feature • Facial nerve palsy • Vesicular rash around the ear • Other features include vertigo and tinnitus

Answer 328

Management • oral aciclovir and corticosteroids are usually given

Answer 329

Management options include • Topical aluminium chloride preparations are first-line. Main side effect is skin irritation • Iontophoresis: particularly useful for patients with palmar, plantar and axillary hyperhidrosis • Botulinum toxin: currently licensed for axillary symptoms • Surgery: e.g. endoscopic transthoracic sympathectomy. Patients should be made aware of the risk of compensatory sweating

Answer 330

Features • Vertigo triggered by change in head position (e.g. Rolling over in bed or gazing upwards) • May be associated with nausea • Each episode typically lasts 10-20 seconds • Positive halpike manoeuvre

Answer 331

BPPV has a good prognosis and usually resolves spontaneously after a few weeks to months. Symptomatic relief may be gained by: • Epley maneuvre (successful in around 80% of cases)

Answer 332

• ANCA positive vasculitis e.g. Wegener's, Churg-Strauss • Cryoglobulinemia • Goodpasture's syndrome • Guillain-Barre syndrome • Hyperviscosity syndrome e.g. Secondary to myeloma • Myasthenia gravis • TTP/HUS

Answer 333

Reflex syncope (neurally mediated) • Vasovagal: triggeres; emotion, pain, stress or orthostatic factors. Often referred to as 'fainting' • Situational: cough, micturition, gastrointestinal • Carotid sinus syncope

Answer 334

Carotid sinus hypersensitivity (CSH): diagnosis is only made after ischemic heart disease or rhythm disturbance have been excluded. CSH may be predominantly cardioinhibitory (bradycardia), vasodilatory (hypotension), or a mixture of the two. Cardioinhibitory is usually managed with insertion of a dual-chamber pacemaker, and vasodilator is managed with support stockings, fludrocortisone and midodrine.

Answer 335

Orthostatic syncope • Primary autonomic failure: Parkinson's disease, Lewy body dementia • Secondary autonomic failure: e.g. Diabetic neuropathy, amyloidosis, uraemia • Drug-induced: diuretics, alcohol, vasodilators • Volume depletion: hemorrhage, diarrhoea

Answer 336

Cardiac syncope • Arrhythmias: bradycardias (sinus node dysfunction, AV conduction disorders) or tachycardias (supraventricular, ventricular) • Structural: valvular, myocardial infarction, hypertrophic obstructive cardimyopathy • Others: pulmonary embolism

Answer 337

Features: • Excessive somnolence • Lethargy and clumsiness • Tends to occur around 11-21 or 31-35 days after high dose cranial radiotherapy. • No focal cause is identified and it is postulated that the condition may occur due to post irradiation demyelination. • No specific therapy is required, some case reports suggest that corticosteroids may be of use but the evidence is not firmly established.

Answer 338

To differentiate it from cerebral mets or cerebral edema There should be no new focal neurological signs in post irradiation SS.

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