Neurology Flashcards

Question

How is a post-lumbar puncture headache treated?

Answer 1

* Supportive- analgesia, rest * If pain continues for \> 72 hours then specific treatment is indicated to prevent subdural haematoma * Treatment options include: blood patch, epidural saline, IV caffeine

Answer 2

* A **_seizure_** is a transient occurrence of signs + symptoms due to abnormal excessive or synchronous neuronal activity in the brain * **_Epilepsy_** is the name for occasional sudden, excessive, rapid and local discharges of grey matter. It is a disease of the brain defined by any of the following: * At least 2 unprovoked seizures \> 24 hrs apart * 1 unprovoked seizure and a probability for further seizures similar to the general recurrence risk after 2 unprovoked seizures, occurring over the next 10yrs

Answer 3

1. Genetic 2. Structural- eg chronic cerebrovascular disease, congenital malformation 3. Metabolic disorder 4. Immune disorder 5. Chronic infection eg HIV (acute infection- meningitis) 6. Unknown (1/3 pts)

Answer 4

* Seizures occur due to an imbalance between excitatory and inhibitory signals in the brain * High-frequency bursts of excitatory action potentials in neurones- synchronous, hyperexcitable activity within a neuronal population * GABA is the main inhibitory neurotransmitter, gabanergic neurones * Glutamate is the main excitatory receptor

Answer 5

* Cerebrovascular disease * Head trauma * Cerebral infections * Fhx * Premature birth * Congenital malformations of the brain * Genetic conditions associated with epilepsy

Answer 6

* 1 of 3 criteria 1. ≥2 unprovoked (or reflex) seizures occurring \> 24 hrs apart 2. 1 unprovoked (or reflex) seizure with a probability of further seizures felt to be a similar recurrence risk to pts with ≥2 unprovoked seizures over the next 10 years 3. A diagnosed epilepsy syndrome

Answer 7

* Syncope and anoxic seizures * Behavioural, psychological, psychiatric- pseudoseizures * Sleep-related conditions * Paroxysmal movement disorders * Migraine associated disorders

Answer 8

* ECG- exclude problems with the heart * Bloods- FBC, U&Es, LFT, glucose, bone profile * EEG (electroencephalogram)- assess and record electrical activity of brain * To support epilepsy diagnosis, assess recurrence risk, determine seizure type * MRI brain- to visualise the structure of the brain, assess any structural abnormalities that may be associated with the seizures

Answer 9

1. Where the seizures begin in the brain 1. Focal, generalised, or focal to bilateral tonic clonic 2. Level of awareness during a seizure 1. Impaired awareness or normal awareness (complex or partial) 3. Other features of seizures 1. Motor or non-motor 1. Absence, tonic-clonic, myoclonic, atonic, spasms

Answer 10

* Focal seizures (previously partial seizures), start in an area or network of cells on 1 side of brain * Generalised seizures: engage or involve networks on both sides of the brain at the onset * Unknown onset * Focal to bilateral seizure (previously secondary generalised): started in one side of the brain and spread to both sides

Answer 11

* Focal aware (previously simple partial): awareness remains intact, even if the pt is unable to talk or respond during the seizure * Focal impaired awareness (previously complex partial): awareness is impaired or affected at any point during seizure, even if the pt has a vague idea of what happened * Awareness unknown * Generalised seizures: presumed to affect pt’s awareness or consciousness in some way

Answer 12

* Focal motor seizure: some type of movement occurs eg twitching, jerking, stiffening movements of a body part or automatisms (licking lips, rubbing hands, walking, running) * Focal non-motor seizure: other symptoms occur first such as changes in sensation, emotions, thinking, or experiences * Auras: early symptoms may be the start of a seizure

Answer 13

* Generalised tonic-clonic seizures (grand mal)- *most common type* * Loss of consciousness * Tonic- muscle tensing * Clonic- muscle jerking * Other features- tongue biting, incontinence, groaning, irregular breathing * Pro-longed post-ictal period- confused, drowsy, irritable, depressed * Tonic * Pt becomes stiff + flexed * Pt can fall backwards * Clonic * ` * 0 * Typical absence (petit mal) * Typically occur in children * Pt becomes blank, stares into space, LoC, then abruptly returns to normal * Unaware of surroundings during episode + won’t respond * 10-20seconds * Atonic * Drop attacks * Brief lapses in muscle tone * \<3 mins * Typically begin in childhood * Myoclonic * Myoclonus = shock-like contraction of a muscle or group of muscles * Myoclonus can occur in people w/o epilepsy eg when falling asleep or just occasionally during the day * In pts with myoclonic seizure, the jerks are more frequent, often during sleep or 1^st thing in morning * May affect whole body or single limb * No aura, loss of awareness or post-ictal confusion

Answer 14

* Focal seizures begin in temporal lobe * Affect hearing, speech, memory, emotions * Features include hallucinations, memory flashbacks, déjà vu, doing strange things on autopilot TYPES: * Focal aware * Pt is awake and aware during seizure even if they can’t talk or respond * Focal impaired awareness * Lasts 30 seconds- 2 mins * Pt is confused, awareness is affected

Answer 15

* Tonic-clonic * Absence * Focal impaired awareness (complex partial)

Answer 16

* Tonic activity * Loss of consciousness when seizure begins, pt may fall * Strong tonic spasms of the muscles can force air out of lungs- cry or moan may be heard * Saliva or foam may come out of mouth, tongue, oral mucosa bites with blood in saliva * Stiffness of the chest muscles may impair breathing- cyanosis- pts face may look blue, may gasp or gurgle * Clonic activity * Jerking movements affect the face, arms and legs, becoming intense and rapid * After 1-3 mins, the jerking movements slow down and the body relaxes including the bowel or bladder * Pt may let out deep sigh or return to normal breathing * Post-ictal period * May remain unconscious for several mins as brain recovers * Gradually regain awareness, may feel confused, exhausted, sore, sad, embarrassed * Post-ictal amnesia * May have abnormal or combative or even psychotic

Answer 17

* Cerebral tumours * Intracranial infection * Head injury * Illicit drugs * Alcohol * Hypoxia * Stroke * Electrolytes * Low Na * Low Ca * Low Mg * Hypoglycaemia

Answer 18

* Focal epilepsy: any focal seizure types * Generalised epilepsy: generalised seizure types * Generalised and focal epilepsy: combination * Unknown epilepsy: insufficient evidence to conclude which type of epilepsy it is

Answer 19

* Epilepsy may be organised into a specific syndrome * Characterised by recurrent propensity to a specific seizure type or types of seizures * Important to determine a syndrome to guide medical therapy with anti-epileptic drugs (AEDs)

Answer 20

* There is a strong underlying genetic basis * 3 seizure types occur to varying degrees- myoclonus, generalised tonic clonic seizures and absence seizures

Answer 21

* West syndrome * Lennox Gastaut syndrome * Juvenile myoclonic epilepsy

Answer 22

1. Stroke 2. SoL 3. Severe head injury 4. Drug abuse or alcohol misuse 5. Brain infection

Answer 23

* PMH * Gestational * Birth * Developmental milestones * Schooling * Head injury * Drugs history * Opioids * Alcohol * Illicit drugs * FHx of epilepsy * Clinical assessment * General full examination * Full neuro examination * GCS/ MOCA * Cranial nerves * Power, tone, reflexes * Funduscopic * Ix * Bloods- FBC, U&Es, LFTs, CRP, calcium, Mg, PO4, glucose * Glucose * ECG * Urine dip * Neuro obs, normal obs including temperature.

Answer 24

* Stop driving, inform DVLA * Inform occ health at work * Safety advice- no swimming, heights, occupation * Contact GP if further seizures * OP MRI and baseline EEG

Answer 25

* ABCDE * Protect airway, recovery position * Give high flow O2 * Fluids * BM-10% or 50% glucose if low * Hypoglycaemia is one of the commonest reversible causes of seizures, so always check blood glucose level and correct * ECG * U&Es, glucose, Ca, Mg, LFTs, FBC * Anticonvulsant levels, tox screen * Seizures \> 5 mins * Lorazepam, IV 4mg bolus, repeat once after 10 mins if seizures continuing * If seizure continues- start preparing phenytoin * Establishes status \> 25 mins * Phenytoin 20mg/kg over 20mins * IV valproate, levetiracetam * Take advice from neurologist * Alert ITU * Refractory status \> 30 mins * General anaesthesia, high dose propofol * Continue for 12-24 hrs after last clinical or EEG seizure, then wean sedation

Answer 26

* Status epilepticus * Sudden unexpected death in epilepsy (SUDEP)

Answer 27

* Uncontrolled or frequent seizures * Generalised tonic-clonic seizures * Seizures begin at young age, many years of living with epilepsy * Missed doses of medicine * Alcohol * Nocturnal seizures

Answer 28

* First seizure- do not drive for 6 months, reapply if no seizure * Epilepsy- do not drive for 12 months, reapply if no seizure

Answer 29

* A single seizure lasting \> 5 minutes * Or more than or equal to 2 seizures within a 5 minute period w/o the person returning to normal between them

Answer 30

* Pre-existing diagnosis of epilepsy: * AED withdrawal * Non-compliance * Alcohol use + withdrawal * Illicit drugs * Intercurrent infection * Progression of underlying disease- tumour, encephalitis, vasculitis

Answer 31

* ABC * Airway adjunct * O2 * Check blood glucose * First-line drugs benzodiazenes- diazepam, lorazepam * Pre-hospital setting rectal diazepam * Hospital setting IV lorazepam * Can be repeated once after 10-20 minutes * If on going or established status start second line drug such as phenytoin or phenobarbital infusion * If no response (refractory status) within 45 mins from onset, best way to rapidly achieve control is induce a general anaesthetic

Answer 32

* Mode of onset * Duration * Nature of headache- sharp, dull, throbbing * Site of headache- localised, variable, generalised * Pattern & timing- including time of day, relation to menstruation * Provoking & relieving factors- coughing, posture * Associated symptoms- aura * Drug hx- analgesics

Answer 33

* Primary headache- migraine, tension type headache, cluster headaches and other rarer trigemino-autonomic cephalgia * Secondary headache- caused by a separate underlying pathological process that may be amenable to treatment

Answer 34

* Vascular * Haemorrhage * Infective * Neoplastic * Drugs * Inflammation * Raised ICP * Trauma * Metabolic * Toxins * Glaucoma * Sinus disease * Hypertension

Answer 35

* Thunderclap headache * Sudden means vascular until proven otherwise * SAH is the first differential * Meningism- neck stiffness & photophobia * Meningitis * Blood in subarachnoid space * Non-blanching purpuric rash (*doesn’t blanch due to bleeding into the tissues*) * Meningococcal septicaemia * Fever * Intracranial infection- meningitis, encephalitis * Viral infection * Focal neurology * Serious causes consider infection, vascular, inflammation, SoL, severely raised ICP * Features of raised ICP- headache present on waking & worse lying down, possible N&V, CSF pressure is increased in supine pressure, may be associated confusion, cognitive slowing, diplopia, papilloedema, other focal signs * Exacerbation by valsalvala/ bending/ cough * Raised ICP * Chiari malformation * Recent onset or change in character * Secondary causes * Previous malignancy * Mets? * Constitutional symptoms- malaise, night sweats, wt loss, jaw claudication, scalp tenderness, headache over temple, tenderness or redness or swelling over temporal artery, over age 50 * Temporal arteritis * New onset headache in older pt

Answer 36

* Initially designed for use in head injury, now used to measure consciousness * Score 3-15 * Motor 6- (1) no response to pain, (2) extensor posturing to pain (3) abnormal flexor response to pain (4) withdraws to pain (5) localising response to pain (6) obeying command * Verbal 5- (1) no response (2) incomprehensible speech- moaning but no words (3) inappropriate speech- random words (4) confused conversation (5) oriented * Eye opening 4- (1) no eye opening (2) opening to response to pain to limbs (3) open in response to speech (4) spontaneous eye opening

Answer 37

* Severe unilateral throbbing headache * Associated with nausea, photophobia and phonophobia * Attacks may be up to 72 hrs * Can occur w/ or w/o aura * Migraine w/ aura- sometimes preceded/ accompanied by focal neurological symptoms eg visual symptoms such as zigzag lines, scrotoma (*area of partial alteration in visual field*), sensory symptoms such as pins and needles

Answer 38

ICHD criteria- 1. At least 5 attacks fulfilling criteria B-D 2. Headache attacks lasting 4-72 hrs (when untreated or unsuccessfully treated) 3. Headache has at least 2 of the following characteristics- * Unilateral location * Pulsating quality * Moderate or severe pain intensity * Aggravation by or causing avoidance of routine physical activity eg walking or climbing stairs 4. During headache at least 1 of the following- * N/V * Photophobia or phonophobia * Not better accounted for by another ICHD-3 diagnosis

Answer 39

* Tired, stress * Alcohol * COCP * Lack of food/ dehydration * Cheese, chocolate, red wines, citrus fruits * Menstruation * Bright lights

Answer 40

* First line: combination therapy with an oral triptan and an NSAID, or an oral triptan and paracetamol * For young people age 12-17 consider nasal triptan over an oral triptan * If the above measures are not effective or tolerated offer non-oral metoclopramide or prochlorperazine and consider adding a n on-oral NSAID or triptan

Answer 41

* Prophylaxis given if patients are experiencing 2 or more attacks per month * NICE advise either topiramate or propranolol * Propanolol should be preferred in women of child-bearing age * 10 sessions of acupuncture over 5-8 weeks * Riboflavin (400mg once daily) may be effective in reducing migraine frequency & intensity for some people * Predictable menstrual migraine

Answer 42

* Non-tablet options * Hydration * Rest * Avoid triggers- diary of foods for ~8wks * Wt loss * Exercise * Cold and hot compress * Massage to forehead & temples * ‘4-head’ * Acute mx * Simple analgesia- NSAID, paracetamol * Triptan (5HTl-receptor agonist)- sumatriptan, take at onset of headache, can be repeated after a couple of hours 2 times a day * Contraindicated in cardiovascular disease, peripheral vascular disease, pregnancy * Causes vasoconstriction * Antiemetic * Prophylaxis * Start at low dose, gradually increase according to response to maximum tolerated dose * Amitriptyline, propranolol, atenolol, verapamil, gabapentin, topiramate * If they don’t respond to at least 3 prophylactic meds + exclude medication overuse * Botox- 3 monthly injections, headache diary * Calcitonin gene-related peptide (CGRP) receptor antagonists

Answer 43

* Headache on 15+ days of the month in a pt with pre-existing primary headache & developing as a consequence of regular overuse of acute or symptomatic headache medication for \>3mnths

Answer 44

* Simple analgesics eg NSAIDs, paracetamol- for 15 days or more per month * Ergotamine, triptans, opioids, combination analgesics- for 10 days or more per month

Answer 45

* Withdrawal of the overused drug * Stop simple analgesics abruptly for 6 wks minimum * Withdraw codeine over 2-4 wks- can use naproxen for 2 weeks intermittently to help the pain * Advice for pt- * Pt may feel worse initially * Keep a headache diary * After 6 weeks, simple analgesics can be used on no more than 10 days a month * Codeine should be avoided in chronic headache

Answer 46

* Clinical features: tight band around the head or pressure sensation, bilateral symptoms, lower intensity than migraine, no aura/ N&V/ aggravated by routine physical activity * Mx- aspirin, paracetamol or NSAID first line for acute treatment * Prophylaxis- up to 10 sessions of acupuncture over 5-8 weeks * Low dose amitriptyline is widely used but not recommended by NICE * If there is no improvement or diagnostic uncertainty, refer to neurology

Answer 47

* Patients in their 20s * Stress, mental tension * Fatigue * Missing meals * Female \> male

Answer 48

* A severe primary headache disorder characterised by recurrent unilateral headaches centred on the eye or temporal region * Occur in short attacks (15-180mins) * Associated w/ ipsilateral autonomic signs * Conjunctival injection (enlargement of conjunctival vessels) * Nasal congestion * May be episodic with periods of remission or chronic (no periods of remission for \>3 months) * Most common trigeminal autonomic cephalgia (TAC)

Answer 49

* TACs are a group of primary headache disorders characterised by: * Unilateral pain within the trigeminal distribution * Associated ipsilateral cranial autonomic features * Such as lacrimation, conjunctival injection, rinorrhoea, nasal congestion, eyelid oedema, ptosis * Most common type of TAC: cluster headache * Other types- paroxysmal hemicrania (PH), SUNCT (short-lasting uniltaral neuralgiform headache attacks with conjunctival injection and tearing), hemicrania continua

Answer 50

1. At least 5 attacks fulfilling criteria 2-4 2. Severe or very severe unilateral orbital, supraorbital +/- temporal pain lasting 15-180 mins (when untreated) 3. At least one of the following symptoms/ signs ipsilateral to the headache * Conjunctival injection or lacrimation * Nasal congestion or rinorrhoea * Eyelid oedema * Forehead and facial sweating * Miosis +/- ptosis * Sense of restlessness or agitation 4. Occurring with a frequency between 1 to 8 a day 5. Not better accounted by another ICHD-3 diagnosis

Answer 51

* Age \>50: GCA, SoL * Age \<10: secondary causes? * Immunodeficient: malignancy, infection * Active/ previous cancer: metastatic spread * Pregnant: pre-eclampsia, venous sinus thrombosis

Answer 52

* Male * Smokers * Alcohol

Answer 53

* First-line: sumatriptan for acute attacks * 5HT1-receptor agonist * Route: subcutaneous or intranasal * Short burst O2 therapy- 15 L/min via non-rebreather mask for 15-20 mins * Avoid triggers * Prevention medication: verapamil * Calcium channel blocker * Refractory disease- where medical options haven’t helped, there are a few therapies available- * Greater occipital nerve blocks * Deep brain stimulation * Trigeminal nerve compression

Answer 54

* Basal ganglia * Insufficiency of neurotransmitter dopamine

Answer 55

* The basal ganglia is a series of cell bodies (grey matter) that are located together within the deep subcortical white matter of the brain * Stimulate motor cortex * Responsible for coordinating habitual movements such as walking or looking around, controlling voluntary movements and learning specific movement patterns * Part of the basal ganglia called the substantia nigra produces a neurotransmitter: dopamine, which is essential for the correct functioning of the BG * In PD, there is gradual but progressive fall in dopamine production * The thalamus is not formally classified as part of the basal ganglia, forms extensive connections with nuclei

Answer 56

* Inhibition of muscle tone * Co-ordinated, slow, sustained movement * Suppression of useless patterns of movement * Initiation of movement

Answer 57

* Hypokinetic- Parkinson’s disease, Parkinson’s plus syndromes- PSP, MSA, CBD, AD with Lewy bodies * Hyperkinetic- tremor, dystonia, myoclonus, chorea, tics, akathisia

Answer 58

* IPD is a progressive degenerative disorder characterised by neuronal loss in the brain stem and basal ganglia * There is loss of dopaminergic neurones in the substantia nigra that leads to inadequate dopamine transmission * PD may not be apparent until a substantial number of neurones (50-80%) have been lost within the substantia nigra * Lewy Body formation in affected neurones is a characteristic finding

Answer 59

1. Bradykinesia 1. General slowing of voluntary movements 2. ↓ arm swing 3. ↓ in amplitude with repetitive movements 2. Tremor (unilateral) 1. Resting & pill-rolling 2. 4-6Hz in frequency 3. Can be induced by distraction 3. Rigidity 1. ↑ resistance to passive movement in a joint 2. Cogwheel due to superimposed tremor

Answer 60

* Motor symptoms * Akinetic rigid amalgamation of symptoms: * Unilateral rolling tremor * Rigidity * Bradykinesia- slowness of voluntary movements * Unsteady gait- slowness of movements, reduced arm swing (can be asymmetrical at the beginning of PD), forward flexion (later), small shuffling steps, defragmentation of turns, reduced postural control and stability, hesitancy in initiating movement * Postural instability- most disabling features * Non motor symptoms * Fatigue * Orthostatic hypotension * Bladder & bowel dysfunction * REM sleep disorders * Saliva drool * Depression * Dementia * Hallucinations, delusions

Answer 61

* Older aged man around 70 years old

Answer 62

* Unilateral * Pill rolling: looks like they are rolling a pill between their fingertips & thumb * More pronounced when resting * Improves on voluntary movement * Worsened if pt is distracted

Answer 63

* If you take their hand & passively flex & extend their arm at the elbow, you feel a tension in their arm that gives way to movement in small increments (like little jerks) à cogwheel

Answer 64

* Handwriting gets smaller and smaller * Can only take small steps when walking- shuffling gait * Difficulty initiating movement- eg when standing still to walking * Difficulty in turning around when standing- have to take lots of small steps * Reduced facial movements and facial expression- hypomimia

Answer 65

* Slow movement * Reduced arm swing * Forward flexion * Shuffling gait * Defragmentation of turns * Reduced postural control & stability * Hesitancy in initiating movement

Answer 66

* Bradykinesia * At least one of the following- * Muscular rigidity * 4-6 Hz rest tremor * Postural instability not caused by primary visual, vestibular, cerebellar or proprioceptive dysfunction * 3 or more required for diagnosis of definite PD in combination with the above * Unilateral onset * Rest tremor present * Progressive disorder * Persistent asymmetry affecting side of onset most * Excellent response (70-100%) to levodopa * Severe levodopa-induce chorea * Levodopa response for 5 years or more * Clinical course of 10 years or more

Answer 67

* Multiple system atrophy- where the neurones of multiple systems in the brain degenerate, affects BG & other areas, leads to Parkinson’s presentation * Degeneration of other areas à autonomic dysfunction: postural hypotension, constipation, abnormal sweating, sexual dysfunction * Cerebellar dysfunction à ataxia * Dementia with Lewy Bodies: associated features of Parkinsonism, progressive cognitive decline, visual hallucinations, delusions, disorders of REM sleep & fluctuating consciousness * Progressive supranuclear palsy * Corticobasal degeneration

Answer 68

* Levodopa * Reduces the motor symptoms (not effective on non-motor) * Carbidopa prevents peripheral breakdown of levodopa * à combination drugs: co-benyldopa, co-careldopa * Doesn’t alter disease progression * More motor complications * COMT inhibitors- entacapone, tolcapone * Dopamine agonists- ropinirole * Doesn’t reduce motor symptoms as much as levodopa * Amantadine * MAO inhibitors * Surgical * Deep brain stimulation

Answer 69

* Dopaminergic drugs- levodopa (as co-careldopa, co-beneldopa) * L-dopa is a precursor of dopamine that can enter the brain via a membrane transporter * In PD there is a deficiency of dopamine in the nigrostriatal pathway that links the substantia nigra in the midbrain to the corpus striatum in the basal ganglia – this causes the basal ganglia to exert greater inhibitory effects on the thalamus, ↓ the excitatory input to the motor cortex, hence the features of PD such as bradykinesia and rigidity – replacing the dopamine will counteract this * Levodopa: wearing off effect where the pt’s symptoms worsen towards the end of the dosage interval- can be overcome by increasing the dose/ frequency, but this can cause dyskinesia and excessive movements at the beginning of the dosage interval = the on-off effect * When the dose of dopamine is too high, pts can develop dyskinesia- excessive motor activity * Dystonia: excessive muscle contraction leads to abnormal postures or exaggerated movements * Chorea: abnormal involuntary movements that can be jerking or random * Athetosis: involuntary twisting or writhing movements usually affecting fingers, hands or feet * COMT inhibitors (entacapone) * Inhibition of catechol-o-methyltransferase which metabolises levodopa in both the body and brain * Entecapone is taken with levodopa to slow breakdown of the levodopa in the brain, extends the effective duration of levodopa * Dopamine agonists (ropinirole, pramipexol) * Ropinirole and pramipexol are relatively selective agonists for D₂ receptor * Usually used to delay the use of levodopa and are then used in combination with levodopa to reduce the dose required * One notable side effect is pulmonary fibrosis * All dopaminergic drugs can cause nausea, drowsiness, confusion, hallucinations, hypotension * Monoamine oxidase inhibitors (selegiline, rasagiline) * Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin & adrenaline; most specific to dopamine, helps increase circulating dopamine

Answer 70

**Why is levodopa given with a peripheral dopa-decarboxylase inhibitor?** * To reduce its conversion to dopamine outside the brain * This ↓ nausea and lowers the dose needed for therapeutic effect

Answer 71

* First line is levodopa if motor symptoms are affecting pt’s QoL * If motor symptoms are not affecting QoL, dopamine agonist, levodopa or MAO B inhibitor can be used

Answer 72

* Nearly all pts with IPD respond well to drug treatment but as the disease advances response becomes poorer and side effects from the drugs can be disabling, in these instances non-oral treatments are sometimes used

Answer 73

* Medical specialist- neurologist or geriatrician * PD specialist nurse * Physiotherapist * Occupational therapist * Speech and language specialist * Dietician later states * Psychiatrist sometimes required

Answer 74

1. Early stage- soon after diagnosis, mild symptoms, normal life possible 2. Maintenance- good response to treatment, no major disability 3. Advanced stage- poor response to drugs with motor side effects 4. Palliative stage- unable to live independently and in need of MD support

Answer 75

* Motor complications: deteriorating function, loss of drug effect, motor fluctuations, dyskinesia, freezing of gait, falls * Non-motor: * Mental health: depression, anxiety, apathy * Dementia, cognitive impairment * Autonomic dysfunction: constipation, orthostatic hypotension, dysphagia, weight loss, XS sweating & salivation, bladder & sexual problems * Other: N&V, pain, sleep disturbance, aspiration pneumonia, pressure sores

Answer 76

* A syndrome which manifests as bradykinesia and rigidity +/- tremor +/- postural instability

Answer 77

* Multiple System Atrophy (MSA) * Progressive Supranuclear Palsy (PSP) * Dementia with Lewy bodies * Drug-induced Parkinsonism- antipsychotics, metoclopramide, antidepressants * Wilson’s disease * Post-encephalitis * Toxins: carbon monoxide, MPTP

Answer 78

* Doesn’t cross Blood Brain Barrier

Answer 79

* Typically an essential tremor is a postural tremor (worse if arms outstretched) but may also be intentional * Postural- ask pt to hold their hand it happens, intention- trying to move hand and can’t * Typically bilateral- generally affects the dominant side more than the non-dominant side * Fine tremor, symmetrical, more common on voluntary movements * Worse when tired, stressed or after caffeine * Improved by alcohol * Absent during sleep * Most common cause of titubation (head tremor) * FHx * No features of PD present- none of the postural stability, masked faces, cog wheeling, non-motor symptoms * Check thyroid- hyperthyroidism can lead to tremor

Answer 80

* Ageing * Genetics * Environmental toxins- pesticides, lead, mercury

Answer 81

* Alcohol & rest both improve tremor * Exacerbated by anxiety, excitement, adrenergic stimulation

Answer 82

* Archimedes spiral test for tremor diagnosis * Essential tremor: 8 to 2 O’clock

Answer 83

* Parkinson’s disease * Multiple sclerosis * Huntington’s chorea * Hyperthyroidism * Fever * Medications- antipsychotics

Answer 84

* 1^st line: propranolol * Primidone is also sometimes used * Both the above medications may reduce the tremor amplitude by up to 50% however the tremor may not completely subside

Answer 85

* Midbrain (mesencephalon) * Cerebral peduncles- connect cerebral hemisphere to midbrain, descending motor fibres down into cord * Pons * Medulla * Medullary pyramids- decussation of descending motor fibres- lateral corticospinal tracts

Answer 86

* Primary motor cortex (precentral gyrus) * Upper limb- lateral aspect of motor cortex * Lower limb- medial aspect of motor cortex

Answer 87

* Inhibition

Answer 88

* The facial motor nucleus is split into 2 halves, 1 supplies superior face and one inferior face * The part of the facial motor nucleus that supplies the upper half of the face receives UMNs from both hemispheres, whereas the part that supplies the lower face only receives a contralateral UMN input * Hence UMN lesions involving the face spare the forehead * True face nerve palsies- affects all muscles of facial expression

Answer 89

* *Damage to upper motor neurones means loss of descending inhibition of lower motor neurones* * Weakness (loss of direct excitatory inputs onto LMNs from UMNs) * Hypertonia (loss of descending inhibition)- all muscles affected equally however in the U/L the flexors are more powerful so they win * Flexed upper limb * Extended lower limb * Hyperreflexia (brisk reflexes due to * Extensor plantar reflexes * Positive Babinski sign (loss of descending modulation of spinal reflexes) * Clonus (exaggerated stretch reflexed causes a series of contractions in a muscle when it is suddenly stretched & held in that position) * Clasped knife rigidity- gives way * Long term disuse of a muscle due to paralysis à disuse atrophy * Acute phase of UMN lesion: * Flaccid paralysis- spinal shock – converts to hypertonia after a few weeks

Answer 90

* UMN: hypertonia, hyperreflexia, spasticity, positive Babinski’s sign, clonus * LMN: hypotonia, hyporeflexia, flaccid weakness, fasciculations, atrophy

Answer 91

* The cell body of a LMN lies with the central nervous system * Ventral horn of spinal cord * Brainstem motor nuclei of the cranial nerves which have motor modalities- oculomotor nucleus, trochlear nucleus, trigeminal motor nucleus

Answer 92

* Weakness (due to denervation) * Hyporeflexia/ areflexia (due to denervation) * Wasting (due to loss of trophic support to the muscle from the LMN across the NMJ) * Hypotonia (due to loss of muscle activation) * Fasciculation (involuntary muscle twitches, often compared to having a bag of worms under the skin, due to loss of up-regulation of muscle nAChRs to try to compensate denervation)

Answer 93

* Stroke, MS, traumatic brain injury, cerebral palsy, spinal cord injury, Huntington’s disease

Answer 94

* Motor neurone disease * Peripheral neuropathy * Poliomyelitis * Spinal cord injury with nerve root compression * Spinal muscular atrophy

Answer 95

* Autosomal recessive * Loss of motor neurones within ventral horn of spinal cord and motor nuclei of cranial nerves in pons/ medulla * Signs of LMN syndrome * Often accompanying bulbar palsy

Answer 96

* Can be divided into conditions which predominantly cause a motor or sensory loss * Predominantly motor loss * Guillain-Barre syndrome * Porphyria * Lead poisoning * Charcot-Marie-Tooth * Chronic inflammatory demyelinating polyneuropathy (CIDP) * Diphtheria * Predominantly sensory loss * Diabetes * Uraemia * Leprosy * Alcoholism * B12 deficiency * Amyloidosis * ABCDE mnemonic: * Alcohol * B12 deficiency * Cancer and CKD * Diabetes and Drugs (isoniazid, amiodarone, cisplatin) * Every Vasculitis

Answer 97

* Immune-mediated demyelination of the peripheral nervous system, acute paralytic polyneuropathy * Triggers- often infection- Campylobacter jejuni * Molecular mimicry * Antibodies against the antigen on the pathogen that causes the pre-ceding infection * The antibodies also match the proteins on the nerve cells * They may target proteins on the myelin sheath or the nerve axon * Cross-reaction of antibodies with gangliosides in the PNS * Correlation between anti-ganglioside antibody (eg anti-GM1) and clinical features has been demonstrated

Answer 98

* Initially- back or leg pain * Characteristic features- progressive, symmetrical weakness of all limbs – starts at feet and moving up body * The weakness is classically ascending i.e. legs affected first * Reduced or absent reflexes * Sensory symptoms tend to be mild- eg distal paraesthesia * Neuropathic pain may be present * May be a hx of a gastroenteritis * Respiratory muscle weakness * Cranial nerve involvement- * Diplopia4 * Bilateral facial nerve palsy * Oropharyngeal weakness * Autonomic involvement- * Urinary retention * Diarrhoea * Less common- papilledema secondary tor educed CSF resorption

Answer 99

* Symptoms start within 4 weeks of preceding infection * Symptoms typically start in feed and progresses upward * Peaks 2-4 weeks * Recovery period months- years

Answer 100

* Clinical diagnosis- Brighton criteria to diagnose & distinguish high/ low risk * Nerve conduction studies- reduced signal through the nerves * Lumbar puncture for CSF- raised protein w/ normal cell count + glucose

Answer 101

* IV immunoglobulins * Plasma exchange- removes the circulating antibodies * Supportive care * Respiratory failure- monitor FVC * Cardiovascular monitoring * VTE prophylaxis (PE is a leading cause of death) * Analgesia

Answer 102

* AIDP: acute inflammatory demyelinating polyneuropathy * Classical symptoms of GBS: progressive symmetrical weakness in limbs, reduced or absent tendon reflexes, reduced sensation * Over a period of weeks to months remyelination will heal the lesions

Answer 103

* Rare variant of GBS

Answer 104

* Chronic inflammatory demyelinating polyneuropathy * Symmetrical * Slowly progressive & cyclical

Answer 105

* Hereditary peripheral neuropathy – affects peripheral motor and sensory nerves * Causes dysfunction in the myelin or in the axons * Hx of frequently sprained ankles * Foot drop * High arched feet pes cavus * Hyporeflexia * (relatively common- 1 in 2500 people- likely to appear in OSCE due to good neuro signs)

Answer 106

* High foot arches (pes cavus) * Distal muscle wasting causing inverted champagne bottle legs * Weakness in lower legs- loss of ankle dorsiflexion * Weakness in hands * Reduced tendon reflexes * Reduced muscle tone * Peripheral sensory loss

Answer 107

* Supportive * Orthopaedics: correct disabling joint deformities, physiotherapists: maintain muscle strength and joint range of motion, podiatrists: help with foot problems & prescribe insoles and other orthoses to improve symptoms

Answer 108

* Progressive degeneration of both upper and lower motor neurones- due to degeneration in both UMN and LMN (anterior horn cells) in the spinal cord and brainstem; rapid progression & poor prognosis * Sensory neurones are spared * Types- * Amyotrophic lateral sclerosis- most common, LMN signs in arms and UMn signs in legs * Progressive bulbar palsy- second most common, loss of brainstem motor nuclei, affects primarily muscles of talking + swallowing, worst prognosis * Progressive muscular atrophy- LMN signs only, affects distal muscles first, best prognosis * Primary lateral sclerosis- UMN signs only * Typical presentation- * Late middle-aged man (eg age 60) * Insidious, progressive weakness of muscles throughout the body affecting limbs, trunk, face, speech * Weakness often first noticed in upper limbs * Increased fatigue when exercising * Clumsiness, dropping things more often, dysarthria (slurred speech) * No cerebellar signs * Signs of LMN- muscle wasting, reduced tone, fasciculations, reduced reflex * Signs of UMN- increased tone or spasticity, brisk reflexes, upgoing plantar responses

Answer 109

* Cervical radiculopathy * Syringomyelia * Syphilic pachymeningitis * Motor neuropathy * Spinal muscular atrophies * Kennedy’s syndrome “spinal and bulbar muscular atrophy)

Answer 110

* Amyotrophic lateral sclerosis: typically LMN in arms and UMN in legs, prognosis 3-5 years * Progressive bulbar palsy: palsy of tongue, chewing/ swallowing muscles and facial muscles due to loss of brainstem motor nuclei * Carries worse prognosis, 1-3 years

Answer 111

* Diagnosis * Clinical diagnosis- hx and examination * Nerve conduction studies will show normal motor conduction; excludes neuropathy, shows evidence of wide spread denervation * NCS is the most useful test * Electromyography (EMG)- confirms diagnosis- reduced a.p.’s with increased amplitude * MRI cervical spine & brain- exclude cervical cord compression, myelopathy, spinal spondylitis * Incurable * MDT- * Respiratory support- NIV to support breathing at night- BIPAP * Nutritional support * Psychological support * Riluzole * Glutamate inhibitor * Can slow progression in ALS * Prolongs life by ~3 months * End of life care planning

Answer 112

* Respiratory failure * Breathlessness on exertion or when lying flat * O/E: poor inspiratory effort, lack of abdo movement w/ respiration * Ix: ABG, CO2 retention * CXR: hemi-diaphragm * Treatment: ventilation support (NIV) * Aspiration pneumonia * Difficulty swallowing secretions * Chronic (silent aspiration)

Answer 113

* Autoimmune condition that causes muscle weakness that gets progressively worse w/ activity & improves w/ rest

Answer 114

* Acetylcholine receptor antibodies are produced * These bind to the post-synaptic nmj receptors * This blocks the receptor and prevents the Ach from being able to stimulate the receptor & trigg er muscle contraction * As the receptors are used more during activity, more of them become blocked up * This leads to less effective stimulation of the muscle with increased activity * There is more muscle weakness the more the muscles are used * This improves w/ rest as the receptors are freed up again * These antibodies also activate the complement system within the nmj leading to damage to cells at the postsynaptic membrane, this further worsens the symptoms * There are 2 other antibodies which cause 15% of MG cases * Muscle-specific kinase (MuSK) * Antibodies against low-density lipoprotein receptor-related protein 4 (LRP4)

Answer 115

* Most symptoms affect the proximal muscles and small muscles of the head and neck * Diplopia due to extraocular muscle weakness * Ptosis due to eyelid weakness * Facial movements weakness * Dysphagia * Fatigue in jaw when chewing * Slurred speech * Progressive weakness with repetitive movements * Strong link with thymoma

Answer 116

* Repeated blinking- ptosis * Prolonged upward gazing- diplopia * Repeated abduction of 1 arm 20 times- unilateral weakness when comparing both sides

Answer 117

* Ach-R antibodies (85% pts) * MuSK antibodies (10% pts) * LRP4 antibodies (\<5% pts) * CT or MRI of thymus for thymoma * Edrophonium test if there is doubt about the diagnosis * Given IV edrophonium chloride or neostigmine * Stops breakdown of Ach * Relieves the weakness temporarily & briefly

Answer 118

* Long-acting Ache inhibitors: pyridostigmine is first line * Immunosuppression: initially prednisolone, azathioprine * Thymectomy * Monoclonal antibodies

Answer 119

* Acute worsening of symptoms * Often triggered by RTI * Mx- NIV with BiPAP or full intubation and ventilation * Immunomodulatory therapies- IV immunoglobulins and plasma exchange

Answer 120

* Penicillamine * Quinidine, procainamide * Beta blockers * Lithium * Phenytoin * Abx: gentamicin, macrolides, quinolones, tetracyclines

Answer 121

* Central nervous system- oligodendrocytes * Inflammation around myelin and infiltration of immune cells causing demyelination * Characterised by plaques

Answer 122

* Viral infections- EBV * Geographic latitude- prevalence of MS increases the greater the distance north or south from the equator * Sunlight exposure- inverse relationship- low vitamin D * Other- obesity during adolescence, smoking, gender (females)

Answer 123

* Optic nerves * Spinal cord- cervical spine * Brainstem- ophthalmoplegia * Cerebellum- ataxia & gait disturbance * Juxtacortical white matter- near the cerebral cortex * Periventricular white matter * A characteristic feature of MS is that lesions vary in their location over time

Answer 124

* Optic neuritis (mx = high dose steroids, recovery within 4-6 weeks) * Diplopia * Ascending sensory disturbance +/- weakness particularly in face or limbs * Weakness due to UMN * Problems with balance, unsteadiness, clumsiness * Altered sensation travelling down the back and sometimes into the limbs when bending the neck forwards (lhermitte’s symptom) * Neuropathic pain eg trigeminal neuralgia * Bladder symptoms * Cognitive impairment (usually a late sign) * Pts are often \<50 years old, have a hx of previous neurological symptoms, have symptoms that have evolved over \>24 hours, have symptoms that may persist over several days/ weeks and then improve

Answer 125

* Visual loss * Blurred vision * Pain typically behind the eye and on movement * Scotoma- partial visual field loss * Poor colour differentiation * Relative afferent pupillary defect * Optic nerve swelling * INO: intranuclear ophthalmoplegia * Abducens palsy

Answer 126

* Multiple sclerosis: the commonest associated disease * Sarcoidosis * SLE * Diabetes * Syphilis * Measles * Mumps * Lyme disease

Answer 127

* Unilateral decrease in visual acuity over hours or days * Poor discrimination of colours, red desaturation * Pain worse on eye movement * RAPD * Central scrotoma

Answer 128

* Severe unilateral pain * Brief electric shock-like pains, abrupt in onset & termination, limited to 1 or more divisions of trigeminal nerve * Pain commonly evoked by light touch, washing, shaving, smoking, talking, brushing teeth or can be spontaneous * Small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas) * The pins usually remit for variable periods

Answer 129

* Disorder of conjugate lateral gaze due to demyelination of the medial longitudinal fasciculus (MLF) * MLF is heavily myelinated * Connects the abducens nucleus complex with the contralateral oculomotor nucleus * If affected, when looking to the R, the R eye will abduct but the L will remain central (failure in adduction)

Answer 130

* Progressive paraparesis * Evidence of UMN signs- spasticity, reduced power, hyper-reflexia * Transverse myelitis * Cerebellar syndrome

Answer 131

* Paraesthesia * Pain * Heat sensitivity (Uhthoff phenomenon) * Sexual dysfunction * Bladder & bowel dysfunction

Answer 132

* Cognitive impairment * Depression * Fatigue * The depression can have a negative impact on memory, attention & concentration

Answer 133

* Relapsing-remitting MS (RMMS) * Most common at initial diagnosis * Active: new symptoms are developing or new lesions are appearing on MRI or not active where no new symptoms or lesions are appearing on MRI * Overall worsening disability over time or no worsening * Secondary progressive MS (SPMS) * Where there is relapsing-remitting MS but with progressive worsening of symptoms with incomplete remissions, symptoms become more and more permanent * Primary progressive MS (PPMS) * Where there is worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions, classified as active or worsening

Answer 134

* MS attack- an episode of neurological symptoms that relate to an inflammatory demyelinating lesion, lasts \>24 hours +/- recovery, typically sensory/ motor/ visual disturbances, 30+ days between episodes

Answer 135

* Lesions consistent with an inflammatory process * No alternative diagnosis * Multiple lesions in time and space (relapsing-remitting MS) * Progressive neurological deterioration for 1 year (primary progressive MS)

Answer 136

* Develops new symptoms * Has rapid worsening of existing symptoms * Symptoms last for more than 24 hours in the absence of infection or other cause after a stable period of 1 month or more

Answer 137

* Demyelinating diseases- transverse myelitis, acute disseminated encephalomyelitis * Infections- lyme disease * Metabolic disease- B12 deficiency, diabetic neuropathy, adult onset leukodystrophies, copper deficiency * Systemic diseases: SLE, sarcoidosis * Other: neoplasia, vasculitis, stroke

Answer 138

* MRI of brain or spinal cord- demonstrates demyelination plaques * Immunoelectorphoresis of CSF- shows oligoclonal bands of IgG

Answer 139

* Analyse CSF to assess for CSF-specific oligoclonal bands * Oligoclonal bands = bands of immunoglobulins * The test is positive if oligoclonal bands identified in the CSF are not present in the serum

Answer 140

* General care- modifiable risk factors * Exercise to maintain strength * Vaccinations- live vaccines may be contraindicated * Possible benefit of flu vaccine * Advise not to smoke * Advise that MS-related fatigue may be precipitated by heat, overexertion, stress, time of day * Managing acute relapses * Methylprednisolone 500mg po od for 5 days, 1g IV for 3-5 days where oral treatment has failed previously/ severe relapse * Disease modifying therapies * Symptomatic treatments * Neuropathic pain: amitryptiline, gabapentin * Depression: SSRIs * Urge incontinence: tolterodine, oxybutynin * Spasticity: baclofen, gabapentin, physiotherapy

Answer 141

* Spasticity: baclofen and gabapentin are 1^st line, physiotherapy * Neuropathic pain: amitriptyline, gabapentin * Depression: SSRIs * Incontinence: anticholinergics – oxybutynin * Fatigue: amantadine

Answer 142

* Beta-interferons reduce relapse rate by up to 30% * Natalizumab: a recombinant monoclonal antibody that antagonises alpha-4 Beta-1-integrin found on surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the BBB * Fingolimod: prevents lymphocytes from leaving lymph nodes

Answer 143

* The general degeneration of the spine that can occur in joints, discs and bones of the spine as we age * Results from osteoarthritis

Answer 144

* Neck pain * Referred pain may mimic headaches, shoulder blades/ upper limb pain * Orbital/ temporal pain * Neck stiffness * Poor balance * Limited range of movement * Radiculopathy due to compression or stretching of the nerve roots- unilateral pain in one dermatome * Clinical diagnosis

Answer 145

* Encourage neck movement and refrain from work absence etc * Refer to physiotherapist, psychologist * Might need surgery if pain \> 12 weeks

Answer 146

* Pain * Stiffness * Referred pain to the buttocks or legs * Restricted movement

Answer 147

* Encourage good posture * Keep moving- avoid staying in 1 position * Take care when lifting- bend at knees * Engage in physiotherapy exercises * Lumbar facet injections * Corticosteroid injections * Surgery- spinal fusion, laminectomy, disc replacement

Answer 148

* Median nerve compression in the carpal tunnel * Idiopathic * Pregnancy * Oedema- eg HF * Lunate fracture * Rheumatoid arthritis

Answer 149

* Pins and needles or pain in thumb, index, middle finger * Symptoms may ascend proximally * Pt shakes hand to obtain relief- classically at night * Examination: weakness of thumb abduction (abductor pollicis brevis) * Wasting of thenar eminence * Tinel’s sign: tapping à paraesthesia * Phalen’s sign: wrist flexion causes symptoms

Answer 150

* Corticosteroid injection * Wrist splints at night * Surgical decompression (flexor retinaculum division)

Answer 151

* Ulnar nerve entrapment at the elbow- cubital tunnel syndrome * Ulnar nerve entrapment at wrist- Guyon’s canal syndrome * Due to repetitive compression from leading on elbows or wrists and prolonged elbow flexion * Can also occur from trauma, swelling, fractures, vascular and bony pathologies

Answer 152

* Loss of sensation in hand- ring and little fingers * Loss of finger coordination * Pain * Hand weakness may get worse with physical activity * Loss of grip strength * Severe cases- ulnar claw

Answer 153

* Splinting * NSAIDs * Physiotherapy * Corticosteroid injection * Surgical intervention

Answer 154

* High ulnar nerve injury à less prominent ulnar claw * Low ulnar nerve injury à more prominent ulnar claw

Answer 155

* The meninges cover the brain and the spinal cord- there are three layers * Dura mater (outermost) * Arachnoid mater * Pia mater (innermost- tightly adhered to surface of brain & spinal cord, followed gyri & fissures) * 2 major functions- * Supportive framework for cerebral & cranial vasculature * Protect CNS from mechanical damage * Subarachnoid space * Underneath the arachnoid mater * Contains cerebrospinal fluid- cushion to brain * Arachnoid mater projections (arachnoid granulations\_ allow CSF to re-enter the circulation via dural venous sinuses

Answer 156

* Hypertension * Smoking * Excessive alcohol consumption * Cocaine use * FHx * Black female patients, age 45-70 * Sickle cell anaemia * Connective tissue disorders- Marfan’s, Ehlers-Danlos * Neurofibromatosis

Answer 157

* 90% sensitivity in first 24 hrs * 50% sensitivity by 72 hrs

Answer 158

* Lumbar puncture * Do not perform within first 12 hrs post headache onset * Plain CT head is not 100% sensitive and the consequences of missing a SAH and leaving it untreated are serious

Answer 159

* The CSF is analysed by spectrophotometry looking for the bilirubin peak- bilirubin is the breakdown product of blood produced as the blood breaks down over several hours * When you perform an LP it is unavoidable there will be some bleeding in the CSF caused by the procedure * When the sample is analysed, provided there has been time for the blood from the SAH to start to break down, if there are RBC you can still ascertain that there has been a SAH by the presence of a bilirubin peak * If you take the sample too early then there’s no chance for the cells to start to break down & you may not be able to tell whether the RBCs in the CSF are due to a SAH or whether they have been introduced during the LP

Answer 160

* 70% berry aneurysms * 10% arteriovenous malformations * 10% hypertension * 5% idiopathic * Trauma

Answer 161

* Headache- sudden-onset, thunderclap, severe, occipital * N&V * Meningism- photophobia, neck stifness * Coma * Seizures * Sudden death * ECG- ST elevation

Answer 162

* Immediate transfer to interventional neuroradiologist or neurosurgical ward for a cerebral angiogram & treatment of any remaining aneurysm or AVM if one is found * This will help prevent a re-bleed * Give nimodipine (CCB, targets brain vasculature) to prevent cerebral vasospasm (21 days) * Hydrate to avoid poor cerebral perfusion & avoid the extremes of BP * Control blood pressure * Close neuro observation- * Hydrocephalus can occur early or later * If neuro status deteriorates, urgent head CT required to identify hydrocephalus, which will need a neurosurgical procedure to release the pressure- CSF shunt

Answer 163

* Re-bleeding * Happens in ~10% cases& most common in first 12 hours * If rebleeding is suspected (eg sudden worsening of neurological symptoms) then arrange a repeat CT * Very high mortality * Vasospasm: delayed cerebral ischaemia, typically 1-2 weeks after onset * Hyponatraemia (mostly due to SiADH) * Seizures * Hydrocephalus * Death

Answer 164

* Conscious level on admission * Age * Amount of blood visible on CT head

Answer 165

* 7-15 mmHg in adults in supine position

Answer 166

* Present on waking * May wake them up at night * May improve during the day * Exacerbated by sneezing, straining, bending, lifting or lying down- all these may further raise the ICP * Short hx- days, weeks, few months * N&V * Effortless vomiting- posterior fossa mass close to 4^th ventricle, irritating the vomiting centre * Reduced levels of consciousness * Cushing’s triad: widening pulse pressure, bradycardia, irregular breathing

Answer 167

* Idiopathic intracranial hypertension * Traumatic head injury * Infection- meningitis * Tumours * Hydrocephalus

Answer 168

* CT/ MRI of the brain * Invasive ICP monitoring: catheter placed into lateral ventricles of brain to monitor the pressure, may also be used to take samples of CSF and also to drain small amounts of CSF to reduce the pressure

Answer 169

* Ix and treat underlying cause * Head elevation to 30 degrees * IV mannitol as an osmotic diuretics * Controlled hyperventilation * Aim is to reduce pCO2 à vasoconstriction of the cerebral arteries à reduced ICP * Leads to rapid, temporary lowering of ICP- but caution as may reduce blood flow to already ischaemic parts of the brain * Removal of CSF- eg intraventricular monitor, repeated lumbar puncture, ventriculoperitoneal shunt (hydrocephalus)

Answer 170

* Commonly occurs in young, obese women, reproductive age * Associated conditions * Medications: growth hormones, retinoids * Dietary: XS vit A * Systemic illness: sleep apnoea, hypercoaguable disorders, PCOS, SLE, Behcet syndrome, endocrine eg Addison’s, hypoparathyroid * Signs & symptoms of raised ICP but no mass lesion on CT/ MRI * Pathophysiology- impaired CSF absorption * Morning headache, vomiting, visual disturbance- diplopia, visual obscurations (sudden, transient bilateral visual loss with changes in posture), tinnitus, bilateral papilloedema, may have 6^th nerve palsy & no other focal neurological signs

Answer 171

* Clinical features of raised ICP, identification of raised pressures on lumbar puncture, exclusion of alternative cause such as SoL, hydrocephalus, venous sinus thrombosis via neuroimaging g * Mx- weight loss, lumbar puncture, may require carbonic anhydrase inhibitor (acetazolamide)

Answer 172

* The superficial temporal artery is a terminal branch of the external carotid artery * It originates at the level of the neck of the mandible * After traversing the parotid gland, it runs superficially to the zygomatic process of the temporal bone

Answer 173

* A systemic vasculitis of the medium and large arteries * Typically affects temporal arteries à temporal arteritis * There is a strong link with polymyalgia rheumatica

Answer 174

* Unilateral temporal headache * Jaw claudication * Scalp tenderness * Constitutional symptoms- fever, weight loss, fatigue, anorexia, muscle aches

Answer 175

* When 3 of the following criteria are met- * Age of disease onset \>50 * New headache * Temporal artery abnormality * Elevated ESR (\>50mm/hr) * Abnormal temporal artery biopsy

Answer 176

* Multinucleated giant cells

Answer 177

* High-dose corticosteroids * With visual symptoms: 60-100mg one off prednisolone * W/o visual symptoms: 40-60mg daily prednisolone * Other medications: aspirin 75mg daily to ↓ visual loss & strokes, and PPI while on steroids (GI protection)

Answer 178

* Inflammation of the meninges (lining of brain and spinal cord) * Neisseria meningitidis- Gneg diplococcus bacteria

Answer 179

* Fever * Neck stiffness * Vomiting * Headache * Photophobia * Altered consciousness * Seizures * Non-blanching rash (meningococcal septicaemia)

Answer 180

* Kernigs test * Lying patient on back, flex 1 hip & knee to 90 degrees and then slowly straighten the knee keeping the hip flexed * This creates a slight stretch in the meninges and where there is meningitis it will produce spinal pain or resistance to this movement * Brudzinski’s test * Lying pt flat on back and gently using your hand to lift their head and neck off the bed and flex their chin to chest * Positive test: when this causes the pt to involuntarily flex their hips and knees

Answer 181

* FBC, CRP, coagulation screen * Blood culture * Whole-blood PCR * Blood glucose * Blood gas * If no signs of raised ICP- lumbar puncture

Answer 182

* Community * Children with suspected meningitis and non-blanching rash à urgent stat IM or IV benzylpenicillin prior to hospital transfer * Hospital * Ideally, perform LUP for CSF before starting abx * Send bloods for meningococcal PCR * \< 3 months or \>50 years: IV cefotaxime + amoxicillin * \> 3 months: IV cefotaxime or ceftriaxone * Meningococcal meningitis: IV benzylpenicillin or cefotaxime * IV dexamethasone given to reduce risk of neurological sequelae and hearing loss * Withhold if: septic shock, meningococcal septicaemia, immunocompromised, meningitis following surgery

Answer 183

* Prophylaxis to close contacts/ household members, and to those exposed to respiratory secretion regardless of closeness of contact * Oral ciprofloxacin or rifampicin * Highest risk in first 7 days, persists for 4 weeks

Answer 184

* Most common cause central disc prolapse- typically L4/5 or L5/S1 * Other causes include: * Tumours: primary or metastatic * Infection: abscess, discitis * Trauma * Haematoma

Answer 185

* Lower back pain * Bilateral sciatica * Reduced sensation/ pins and needles in perianal area * Reduced anal tone * Urinary incontinence, reduced awareness of bladder filling, loss of urge to void * Incontinence is a late sign

Answer 186

* Urgent MRI

Answer 187

* Surgical decompression

Answer 188

* Caused by increased permeability of alveolar capillaries leading to fluid accumulation in the alveoli ie non-cardiogenic pulmonary oedema * Causes- infection: sepsis, pneumonia, massive blood transfusion, trauma, smoke inhalation, acute pancreatitis, cardio-pulmonary bypass

Answer 189

* Dyspnoea * Elevated respiratory rate * Bilateral lung crackles * Low O2 sats

Answer 190

* CXR * ABG

Answer 191

* ITU * Oxygenation/ ventilation * Vasopressors as needed * Abx for sepsis

Answer 192

* Miosis (small pupil) * Ptosis * Enophthalmos (sunken eye) * Anhidrosis (loss of sweating on one side)

Answer 193

* Vitamin B12 deficiency * Vitamin E deficiency * Copper deficiency

Answer 194

* Lateral corticospinal tracts * Dorsal columns * Spinocerebellar tracts

Answer 195

* Usually affects people \> 40 * Begins w/ general feeling of weakness * Tingling, pins & needles, numbness in both hands and feet * Constant and gradually worsens * Limbs feel stiff, clumsy movements, difficulty to walk * Reflexes decreased, increased, or absent

Answer 196

* Injections of vitamin B12

Answer 197

* One of the (paired) dural venous sinuses of the head * Located within the middle cranial fosse, either side of the sella turcica of the sphenoid bone (which contains the pituitary gland)

Answer 198

* Caused by any pathology involving the cavernous sinus, may present as a combination of unilateral ophthalmoplegia (CN III, IV, VI), autonomic dysfunction (Horner syndrome) or sensory trigeminal (V1-V2) loss

Answer 199

* Benign tumour of pituitary gland * 10% of all people- common * Most cases never found * Most common type: prolactinoma, produce XS prolactin * Non-secreting adenomas are the 2^nd most common * Then GH secreting and ACTH secreting * Symptoms caused by excess of hormone: Cushing’s disease due to ACTH, acromegaly due to GH or amenorrhoea and galactorrhoea due to XS prolactin * Stretching of the dura within or around pituitary fossa can cause headaches * Compression of optic chiasm- causing bitemporal hemianopia due to crossing nasal fibres

Answer 200

* Pituitary blood profile: GH, prolactin, ACTH, FH, LSH, TFTs * Formal visual field testing * MRI brain with contrast * Treatment includes a combination of hormonal therapy, surgery and radiotherapy

Answer 201

* Acute neurological condition Triad of ophthalmoparesis with nystagmus, ataxia and confusion * Caused by thiamine deficiency which primarily affects the peripheral and central nervous systems

Answer 202

* Characterised by small and irregular pupils that have little to no constriction to light but constricts briskly to near targets (light-near dissociation) * Pupils are noticeably able to accommodate to near targets * Mostly bilateral

Answer 203

* It is a specific finding in neurosyphilis

Answer 204

* Rapidly progressive neurological condition caused by prion proteins * These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases

Answer 205

* Rapid onset dementia * Myoclonus

Answer 206

* CSF usually normal * EEG: biphasic, high amplitude sharp waves (only in sporadic CJD) * MRI: hyperintense signals in the basal ganglia and thalamus

Answer 207

* Sporadic * 85% all cases * Mean age of onset 65 * New variant CJD * Younger pts- 25 years old * Psychological symptoms- anxiety, withdrawal, dysphonia

Answer 208

* Syringomyelia describes a collection of CSF within the spinal cord * Causes: Chiari malformation, trauma, tumours, idiopathic

Answer 209

* Cape like- neck shoulders & arms * Loss of sensation to temperature but preservation of light touch, proprioception & vibration * Classic examples- burning hands w/o realising * This is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected * Spastic weakness (predominantly lower limbs) * Neuropathic pain * Upgoing plantars * Autonomic features: * Horner’s syndrome due to compression of sympathetic chain (rare) * Bowel and bladder dysfunction * Scoliosis over years if the syrinx is not treated

Answer 210

* Full spine MRI * Brain MRI to exclude Chiari malformation

Answer 211

* Treat cause of the syrinx * Shunt may be placed into the syrinx

Answer 212

* Type of brain dysfunction that occurs when the brain doesn’t receive enough oxygen or blood flow for a period of time * Occurs in neonates during labour