Neurology Flashcards

Question

Typical CF of problem in neuromuscular junction

Answer 1

Fatiguable weakness, no sensory sx

Answer 2

Proximal weakness, no sensory sx

Answer 3

Suspected SAH, meningitis, encephalitis, certain immunological eg MS/GBS, therapeutic removal of CSF, measurement of CSF pressure, intrathecal injection

Answer 4

Focal neurology, RICP, suspicion of a mass lesion in CNS (as reducing pressure by removing CSF could cause coning), local infection near LP site, congenital lesions like myelomeningocele, plt<40, anticoagulation issues, if unconscious/have papilloedema need CT first

Answer 5

Lie in left lateral position Do between L3-4 or L4-5 vertebrae (L4 usually between the iliac crests) Sterile Inject lidocaine into dermis, push through skin, when feel penetrated dura withdraw + allow a few drops of CSF to escape Record pressure, collect specimens, also take blood glucose, record appearance of CSF Lie flat to avoid headaches

Answer 6

* Midline shift * Obvious hyperdense areas (bleed on CT) or hypodense (infarct on CT) * Attenuation * Burr holes * GM/WM distinction * Gyri + sulci * Calcific changes * Small vessel disease (often at ventricle edges) * MCA distribution

Answer 7

* Spinal XR * CT brain * CT angiography * MRI brain/spine * Doppler US-carotid stenosis. Less reproducible but safe+low cost * Catheter angiography-more invasive * Functional MRI-research tool * FDG-PET-for occult neoplasms or large vessel vasculitis * DAT-Parkinsonian disorders when diagnosis uncertain

Answer 8

* EEG: scalp electrodes record brain electrical activity, used to characterise epilepsy syndromes * EMG + nerve conduction studies: skilled ix of nerve function for peripheral nerve/anterior horn/NMJ disease * Cerebral evoked potentials

Answer 9

Tapping on patellar tendon - stretch receptors in quad activated - impulses in 1st order sensory neurone go to LMN (L3+L4) - contract quad

Answer 10

Positive: exaggeration of a physiological phenomenon Negative: loss of function so a destructive lesion causing failure of impulse conduction

Answer 11

u/l CN deficits, impaired consciousness, crossed/bilateral motor/sensory deficits

Answer 12

The last level where the sensation is normal is the level of the lesion in a SC problem

Answer 13

Asymmetrical as one limb spastic, toes on affected side may drag on floor + leg circumducts during swing face Causes: UMN lesions so a u/l brain lesion on contralateral side (stroke, tumour, demyelination, SOL) or hemisection of SC (Brown-Sequard)

Answer 14

b/l but similar to hemiplegic gait, legs drawn together due to spasticity Causes: SC lesion (usually also affects sensation, e.g. prolapsed disc, tumour, transverse myelitis), b/l b/l brain lesion (e.g. MS, b/l infarct, midline tumour), or MND (in which LMN signs would occur)

Answer 15

Short steps, shuffling, reduced arm swing, difficulty with stopping/starting/turning Causes: idiopathic Parkinson's disease (most features of gait + the classic triad), parkinsonian disorders (some features of gait, not all of triad, sometimes other features)

Answer 16

* Cerebellar lesions: ipsilateral (stroke or SOL), bilateral (MS, alcoholism, B12 def, some drugs, genetic, paraneoplastic, multi-systems atrophy * Sensory neuropathy: appears ataxic due to impaired sensation, no other cerebellar signs * Vestibular: a/w N+V and vertigo

Answer 17

Motor weakness of nerves supplying tibialis anterior (L4-S1 root), foot drop + toes drag, to prevent drag knee + hip flex excessively making high stepping gait Causes: isolated common peroneal nerve palsy (trauma/compression e.g. #HoF, inversion preserved), L5 radiculopathy, generalised polyneuropathy

Answer 18

Peripheral sensory nerve impairment meaning pt can't sense foot location Usually dorsal column issue like B12 def/tabes dorsalis, or peripheral nerve diseases

Answer 19

Weak hip abductors so pelvis tilts down, body compensates by flexing torso laterally + circumducting leg. 'Waddling' Causes: * Systemic: hyper/hypothyroidism, Cushing's syndrome, acromegaly, polymyalgia rheumatica, polymyositis * Muscular dystrophies

Answer 20

Normal gait with involuntary choreic movements e.g. oro-facial dyskinesia, or choreic movements of limbs (dance like semi-purposeful movements) Causes: basal ganglia disorders e.g. Huntington's disease, Sydenham's chorea, Wilson's disease, dopaminergic meds

Answer 21

Shortened length of time in stance phase on affected leg due to pain - any cause of pain e.g. OA, #, inflammation, nerve entrapment

Answer 22

Regular sinusoidal oscillation of limbs/head/trunk

Answer 23

* Postural - physiological, everyone has when outstretch hands, pronounced with anxiety/caffeine/hyperthyroidism/drugs. Higher amplitude than benign essential tremor * Essential - common, often inherited, b/l fast low-amplitude, mostly of the upper limb, postural e.g. when holding something, can be of vocal cords causing vibrating voice, anxiety worsens it so small amounts of beta blocker may help * Intention - exacerbated by action with past-pointing + dysdiadochokinesia * Rest - worse at rest, a/w Parkinsonism, often pill-rolling * Dystonic - usually u/l, worse with certain tasks * Holmes - at rest/action, lesion in red nucleus * Sympathomimetics e.g. salbutamol - make all tremors worse

Answer 24

Excessive irregular movements that flit from one body part to another

Answer 25

* Systemic - thyrotoxicosis, SLE, APS, polycythaemia vera * Genetic: HD, benign hereditary chorea * Structural or vascular issues of the basal ganglia * Drugs - levodopa, COCP * Post-infection: Sydenham's chorea, after strep/acute rheumatic fever * Pregnancy

Answer 26

Presents in middle life with 'fidgeting' chorea, then progressive psych + cognitive sx AD inheritance of a CAG repeat expansion in a protein, demonstrates anticipation (successive generations tend to have earlier onset + more severe disease) M: no DMD work, risperidone/sulpiride for the chorea, progressive neurodegenetation, dementia, death after 10-20y

Answer 27

This is violent swinging movements of one side * Usually due to infarct or haemorrhage of the contralateral sub thalamic nucles * Can also occur acutely after hyperosmolar hyperglycaemia cos of the osmotic shifts

Answer 28

Brief shock like jerks

Answer 29

* Primary: physiological when going to sleep/waking, myoclonic dystonia, myoclonic jerks in epilepsy * Secondary to metabolic disorders like asterixis, dementia or encephalitis

Answer 30

Brief stereotyped movements or vocalisations, often affecting the face + neck They can be transiently suppressed, then a build of anxiety + overflow after release

Answer 31

* Simple transient tics common in children like blinking, sniffing * Adult onset is rare and usually a secondary cause * Tourette syndrome: multiple motor tics + at least one vocal tic, a/w behavioural problems + OCD, sometimes get explosive grunting of obscenities (coprolalia) or coping what other people say (echolalia)

Answer 32

Twisting movements + abnormal postures due to muscle spasm Treated with botulinum toxin injections

Answer 33

* Primary - dystonia is the main CF, usually a genetic cause. E.g. torticollis (wry neck, neck muscle spasms), blepharospasm (forced blinking), task-specific (e.g. writer's cramp-other hand functions normally) * Secondary to brain injury, cerebral palsy or drugs (metoclopramide, antipsychotics) * Hereditary-degenerative disorders * Paroxysmal dystonia - rare

Answer 34

Disorders caused by certain antiemetics + antipsychotics May cause akathisia (restless), Parkinsonism (dopamine blockade), acute dystonic reactions (e.g. oculogyric crisis [sustained upward gaze, often develops acutely after 1 dose]), tar dive dyskinesia (often after years of medS)

Answer 35

* Cortex: post-central cause loss of bladder sensation, pre-central cause difficulty initiating micturition, frontal lesions can cause socially inappropriate micturition * Cord: b/l UMN lesions cause frequency + incontinence so bladder sensitive to small changes in pressure * LMN: sacral lesions cause flaccid atonic bladder that overflows

Answer 36

* Peripheral causes of vertigo (common) * Syncope * Unsteadiness e.g. cerebellar disorders * Central causes of vertigo: rare. E.g. vestibular nuclei infarcts, demyelination in the brainstem, posterior fossa mass lesions, migrainous vertigo, CPA mass lesions compression vestibular nerve, drugs .e.g anticonvulsants

Answer 37

* Central scotoma + u/l vision loss * Impaired colour vision * Full lesion-u/l blindness + loss of pupillary reflex * Optic atrophy (late)

Answer 38

* Inflammatory optic neuritis * Optic nerve trauma or compression from glioma or aneurysm * Toxicity from alcohol, ethambutol, quinine * Ischaemia e.g. GCA * Hereditary * Nutritional deficiency e.g. B1 or B12 * Infection e.g. orbital cellulitis, syphilis, TB * Neurodegenerative * Papilloedema

Answer 39

* RICP (papilloedema) * IIH * Hydrocephalus * Optic neuritis (papilllitis) * Ischaemia e.g. GCA * Toxicity e.g. methanol * Venous occlusion or venous sinus thrombosis * Central retinal vein thrombosis * Orbital mass lesions * Malignant HTN * SLE * Hypercapnia * Disc infiltration e.g. leukaemia

Answer 40

* Pink disc * Blurring of disc margin * Loss of spontaneous retinal vein pulsation * Obliteration of the cup * Dilated vessels * Small haemorrhages * Papilloedema (when RICP is the cause) doesn't produce visual sx except momentary visual obscurations w changes in posture

Answer 41

Subacute VL (not permanent), mild fogging of central vision, colour desaturation; rare to have complete blindness, disc looks normal unless inflammation at nerve head, common to get pain/discomfort esp on movement but shouldn't be severe Develops over a few days and recovers over 2w Commonest cause is MS but can be cos of other inflammation

Answer 42

Occlusion/hypoperfusion of posterior ciliary arteries --> infarction of optic nerve head Sudden/stuttering visual loss + disc swelling then atrophy Higher risk if small hypermetropic disc

Answer 43

Bitemporal hemianopia or quadrantonopia Causes: pituitary tumour, meningioma or craniopharyngioma

Answer 44

Monocular lesions - pre-chiasmal Homonymous lesions - post-chasmal (involve the same part of the VF in both eyes)

Answer 45

* Optic tract: homonymous hemianopia | * Optic radiation: homonymous quadrantonopia

Answer 46

* u/l infarct - homonymous hemianopia, macular cortex may be spared * cortical blindness - widespread b/l damage by infarct/trauma/coning, pt characteristically lacks insight into this, the rest of the vision pathway is fine but they cannot interpret image

Answer 47

Destructive lesion on one side means eyes driven by the opposite pathway which is intact e.g. in acute cerebral lesion contralateral hemiparesis but the eyes look to the normal side (except brainstem lesion where eyes look to abnormal side)

Answer 48

Damage to the ipsilateral MLF * side of lesion causes impaired adduction on that side, a/w nystagmus of the other eye * b/l INO is almost pathognomonic of MS * INO may also be due to vascular disease

Answer 49

Rhythmic oscillation of eye movement indicating disease in the retina/cerebellum/vestibular systems * Jerk nystagmus: fast/slow oscillation, due to vestibular/CN VIII/brainstem/cerebellar issue * Pendular nystagmus: usually vertical + in all directions of gaze, usually an eye cause e.g. severe visual impairment

Answer 50

Supplies to SR, IR, MR, IO + LPS, plus carries PNS fibres for pupil constriction Signs: u/l ptosis, down + out deviation, fixed dilated pupil. But they don't c/o diplopia because ptsosi covers the eye. If pupil is spared it indicates an internal cause like diabetes Causes: posterior communicating artery aneurysm, infarct of CN III in DM/atheroma, coning of temporal lobe, midbrain infarct or tumour

Answer 51

Supply SO, causes torsional diplopia when look down, head tilts away from that side, eye looking 'up and out'. Usual cause is head injury

Answer 52

Horizontal diplopia, LR palsy (cannot fully abduct), may have esotropia Long intracranial course so can be damaged in brainstem by MS/infarct, compression in RICP, tumour infiltration, microvascular ischaemia in DM

Answer 53

Immobile eye as CN 3, 4 and 6 are all paralysed. Causes: * Orbit e.g. metastasis * Cavernous sinus e.g. thrombosis, meningioma * Wernicke's encephalopathy * Myasthenia gravis

Answer 54

This is disc pallor due to loss of axons, glial proliferation + reduced vascularity Can happen after any form of optic neuropathy

Answer 55

Afferent pupillary defect: complete optic nerve lesion causes dilated pupil + APD so the direct + consensual responses for the affected eye are absent RAPD: incomplete damage to one optic nerve relative to the other, so whilst the reflexes are there they differ in strength to the other eye

Answer 56

* Dysphasia - due to a problem in the dominant hemisphere, disorder of language * Dysarthria - motor speech disorder (not comprehension/language/expression) causing slurred speech

Answer 57

Expressive dysphasia (frontal lobe) - patient knows what they want to say (comprehension is fine) but they can't find the correct words (non-fluent speech), language becomes reduced to a few disjointed words. V frustrating for pt. Can repeat words better than spontaneous speech

Answer 58

Receptive dysphasia (temporoparietal region) - fluent speech with impaired comprehension so doesn't make sense, pt lacks insight

Answer 59

Nominal/anomic: difficulty naming objects. Is an early feature Global/central aphasia: loss of language production + understanding, inc reading + writing

Answer 60

Lesions in any of the articulation/phonation/swallowing pathways causing dysarthria, dysphonia + dysphagia; sometimes non-neurological local things like VC lesions Lesions may be in motor cortex, brainstem, CN nuclei (corticobulbar fibres received from both sides), cerebellum, basal ganglia or NMJ

Answer 61

* UMN lesions: usually b/l because b/l innervation to the muscles. E.g. MS, MND, progressive supranuclear palsy. Slow indistinct strained speech * LMN lesions: damage to CN VII/X/XII or their nuclei. b/l like GBS, MND. Slurred indistinct speech, nasal quality * Basal ganglia lesions: Parkinson's disease (low volume, monotonous, trails off), chorea (loud + harsh + variable), athetosis (loud + slow) * Cerebellar lesions: slurred slow speech as impaired coordination (ataxia) * Myopathies: weakness of muscles * NMJ: fatiguability * Oropharyngeal lesions: multiple ulcers, oral candidiasis, quinsy, dental abscess, loose dentures

Answer 62

* B/l cerebral hemisphere vascular/trauma issues * Brainstem - MS, vascular, tumours * MND - from bulbar muscle palsy * NMJ - MG * Muscle - poliomyositis * Pharynx + oesophagus pathology Neuro cause - difficulty swallowing liquids then solids. Corticobulbar fibres so often occurs with dysarthria

Answer 63

* Medullary lesions involving nucleus ambiguous (where CN X nucleus is) - infarct, tumour, demyelination * RLN palsy - thyroid surgery, bronchial carcinoma, aortic aneurysm * Vocal cord lesion - polyps, tumour * Functional - psychogenic aphonia

Answer 64

Damage to medial structures of temporal lobes + brainstem connections e.g. hippocampi; amnesia due to b/l lesions Causes: dementia, amnestic mild cognitive impairment, thiamine deficiency, severe head injury, anoxic brain damage, b/l thalamic stroke, SAH, diffuse small vessel disease, b/l tumours, TL epilepsy, post-hypoglycaemia, dissociative, transient global amnesia, transient epileptic amnesia, post-traumatic amnesia

Answer 65

Pain receptors in base of brain arteries + veins, meninges, extra cranial vessels, scalp/neck/face muscles, paranasal sinuses, eyes + teeth Brain parenchyma mostly has no pain receptors

Answer 66

Damage/dysfunction of the pain/sensory pathways e.g. peripheral nerve damage, radiculopathy, post-stroke pain, post-herpetic neuralgia STT involved in pain localisation + spinoreticular tract in emotional component, project to thalamus to sensory cortex + limbic system Gate theory - transmission of afferent pain impulses is gated in SC dorsal horn synapses + brainstem Allodynia - normal sensory inputs can produce abnormal response due to central + peripheral sensitisation (increased excitability of nociceptors)

Answer 67

* Peripheral nerve injury: peripheral neuropathy, nerve compression e.g. CTS/meralgia paresthetica, post-herpetic neuralgia, nerve trauma/section, trigeminal neuralgia * Nerve root/plexus: radiculopathy from disc prolapse, plexopathy (inflammation, infiltrative e.g. breast cancer) * CNS: MS, post-stroke pain, SC lesions, PD

Answer 68

* MDT pain clinics * Diagnosis is important * Psychological: CBT to control response, ADs can help * Co-analgesics: primary use is not for pain but can help alone/as an adjunct. E.g. amitriptyline, duloxetine, anticonvulsants like carbamazepine/gabapentin, CCBs, muscle relaxants e.g. baclofen, topical capsaicin, cannabinoids, botulinum toxin * Stimulation for gating effects - acupuncture, ice, heat, US, massage, TENS * Nerve blocks e.g. LA

Answer 69

* Typical 50-60s, HTN * Compression of CN V near pons by a vascular loop, MS or a CPA tumour * CF: sudden onset knife/electric type pain for seconds in the CN V distribution, usually u/l, may have a trigger like washing/cold wind/chewing, no signs of CN V dysfunction o/e * B/L sx means brainstem pathology e.g. MS * M: carbamazepine 1st line to reduce severity, can try lamotrigine/gabapentin, some surgical options may be tried e.g. selective ablation. Usually spontaneously remits then recurs

Answer 70

* Peripheral n lesions: within distribution of a nerve, section causes LoS and entrapment causes numb/pain/tingling * Neuralgia: pain in distribution of nerve e.g. trigeminal or post-herpetic * Root pain: pain in the myotome + tingling discomfort in the dermatome, worse on stretching e.g. SLR in disc prolapse/raised pressure like coughing * Posterior cord lesions: all DCML diminished below lesion, tingling sensations * Cervical cord lesion - Lhermitte's pnenomenon * STT lesion - c/l loss of pain + temp with a clear sensory level (in compressive lesions the lower roots are damaged first so the sensory level can ascend as deeper fibres become involved) * Cord compression - progressive spastic paresis with sensory loss below lesion, often sphincter disturbance + root pain @ level of compression * Thalamic lesions - deep seated pain in paretic limbs, movement disorders * Parietal cortex lesions - sensory loss, neglect, apraxia, pain is NOT a feature

Answer 71

* Metastatic cord compression causing vertebral destruction - thyroid, lung, breast, renal, prostate, myeloma, NHL * Trauma - vertebral #, facet joint dislocation * Infection - acute or chronic, epidural abscess from osteomyelitis, TB (destroys VBs + local infection) * Disc prolapse - rare as would usually cause cauda equina (inferior to SC, as lumbar herniations are the more likely location rather than further up) * Narrowed canal e.g. RA, ankylosing spondylitis, osteophytes * Chronic degenerative disc prolapse e.g. cervical or thoracic disc potrusion * Extramedullary e.g. meningioma or intramedullary e.g. glioma tumours * Epidural haematoma: rare comp of anticoagulants/bleeding disorders

Answer 72

UMN! * Impaired sensation + proprioception at dermatomal level below lesion * Pain esp on straining * B/l or u/l weakness * UMN signs (cf LMN signs in peripheral compression of nerve or cauda equina) * Reflex may be absent at level of lesion due to compression of the LMN in the ventral horn * Over time lesions at T12 or above cause bladder spasticity and below cause flaccidity * Autonomic involvement - late. Incontinence or constipation or retention * Evidence of cause e.g. WL

Answer 73

* MRI within 24h if suspect cord compression, within 7d if features of spinal mets but no compression yet * High dose corticosteroids with gastro protection with PPI * Neuro/oncology referral - decompression is definitive management if suitable, if cancer may have CT and/or RT if sensitive * If still mobile at presentation majority remain so but if cannot walk at presentation only 1/3 will walk again

Answer 74

'Hemisection of cord' tho usually not truly this * Contralateral damage to STT - loss of pain + temp * Ipsilateral CST damage - weakness on same side of lesion below the level * Ipsilateral loss of DCML (proprioception + vibration)

Answer 75

* Motor: MND (UMN+LMN), poliomyelitis (anterior horn cells-LMN signs) * Sensory: neurosyphilis tabes dorsalis (affects DCML) * Combined motor + sensory: - Brown-Sequard - Subacute combined degeneration of cord - Friedrich's ataxia: same as SACD plus cerebellar ataxia - Anterior spinal artery occlusion: b/l spastic paresis + loss of STT - Syringomyelia - MS: asymmetrical, varying tracts involved

Answer 76

* Pupils - CN III compression (1 pupil fixed dilated to light), metabolic comas (b/l mid-point reactive pupils ie normal), opiates (b/l pinpoint light-fixed), brain death (b/l light-fixed dilated) * Eye movements + position: disconjugate (1 up 1 down=brainstem lesion), conjugate (deviate towards lesion of the frontal lobe and away from the lesion in the brainstem), vestibular-ocular reflex (passive head turning eyes also move [Doll's eye reflex], absent in brainstem death), windscreen wiper eyes (in light coma) * Brainstem reflexes - corneal, gag/cough, respiratory drive * Lateralising signs - any asymmetrical response

Answer 77

A state of unrousable unresponsive, GCS of 8 or lower * Vegetative state: usually extensive cortical damage but brainstem intact so don't need ventilating, pt has sleep/wake cycles but no response to stimuli except reflexes, permanent if no recovery after 6m/12m if trauma is the cause * Minimally conscious state: fluctuating limited awareness, inconsistent but reproducible responses e.g. crying irt emotional stimuli * Brainstem death

Answer 78

* Brainstem lesions: bleed, infection, demyelination, neoplasm, Wernicke-Korsakoff * Brainstem compression: tumour, supratentorial mass lesion, bleed * Diffuse brain dysfunction: severe metabolic/toxic disorders that depress the cortex+RAS like drug OD/CO poisoning/low or high glucose * Massive cortical damage e.g. meningitis, HIE post-cardiac arrest, diffuse axonal injury from rapid accel/decel (causes multiple haemorrhages + diffuse axon damage in WM)

Answer 79

Psychogenic coma, locked in syndrome

Answer 80

* Epilepsy * Syncope * Non-epileptic attacks (pseudo seizures). May look tonic-clonic, no rise in serum prolactin, pelvic thrusting, cry after seizure, gradual onset * Panic attacks + hyperventilation: sudden SNS activation, can lead to respiratory alkalosis. CF include dizzy, CP, feeling of choking/SOB, tingling in face+extremities, palps, feeling of impending doom * Hypoglycaemia: confusion, LoC, sometimes convulse/dysphasia/hemiparesis, warning sx beforehand * Drop attacks: usually women >60y due to sudden change in LL tone causing falling, no LoC * Hydrocephalic attacks * Basilar migraine or familial hemiplegic migraine * Severe vertigo - can cause prostration * Sleep disorders * TIAs - almost never cause a LoC

Answer 81

* Neurocardiogenic/vasovagal: sudden reflex bradycardia with peripheral + splanchnic vasodilation. Triggers, brief prodome, pt usually still during blackout or some twitching/jerking, pale, may lose continence, no post-ictal state just fatigue, recover over seconds * Cardiac (Stokes-Adams attacks): arrhythmia or left ventricular outflow obstruction, without warning or on exercise * Micturition syncope * Cough syncope - venous return to heart obstructed by severe coughing/laughter * Postural hypotension * Carotid sinus syncope - vagal response to pressure over carotid sinus baroreceptors e.g. tight collar * Convulsive syncope: secondary anoxic seizure after syncope (like if collapse in a propped up position)

Answer 82

* Insomnia * Myoclonic jerks when fall asleep/wake - normal * Narcolepsy - abnormal neurotransmitter causing excessive daytime sleepiness, inappropriately sleeping, cataplexy (sudden loss of muscle tone with intact awareness), dream-like hallucinations, sleep paralysis * Parasomnias - disruptive motor/verbal behaviours during sleep inc sleepwalking + night terrors * Restless leg syndrome - wanting to move legs with throbbing/pulling, usually relieved by stretch, idiopathic but can be triggered by drugs/pregnancy/uraemia/Parkinsons disease

Answer 83

Place hand under the asymptomatic limb and ask them to move the weak one - if there is an organic cause, examiner will feel the normal leg pushing downwards as pt attempts to lift weak leg. If this doesn't happen it indicates that the weakness is functional

Answer 84

* Arnold-Chiari malformation: cerebellar tonsils descend into cervical canal. A/w spina bifida + syringomyelia * Stenosis of aqueduct of Sylvius: congenital/acquired from meningitis * Dandy-Walker syndrome: cerebellar hypoplasia + obstruction of 4th ventricle outflow Tends to cause increasing head circumference (as sutures unfused), bulging fontanelles, impaired upward gaze ('sunsetting', due to pressure on tectal plate), dilated scalp veins, bradycardias, seizures, coma

Answer 85

* Stable childhood hydrocephalus suddenly worsening * Posterior fossa/brainstem tumour * Obstructed resorption from SAH/head injury/meningitis * 3rd ventricle colloid cyst * Choroid plexus papilloma - rare, secrete CSF

Answer 86

Triad of gait apraxia + dementia + urinary incontinence Pressure may actually be raised but is low-grade. Trial prolonged CSF drainage and then VP shunt sometimes (comps of shunt are seizure, infection + ICH but only in 10%)

Answer 87

* Causes: traumatic; spontaneous --> berry aneurysms in CoW (commonest spontaneous, a/w adult PKD, EDS + coarctation of aorta), AV malformation, bleeding disorders, acute meningitis, arteritis like SLE, pituitary apoplexy, mycotic aneurysms, peremesencephalic (idiopathic venous bleed) * CF: sudden v severe headache fast peak 'thundercalp', occipital, N+V, meningism, coma, death, neck stiffness, positive Kernig's sign, papilloedema, subhyaloid haemorrhage, may have ECG changes like ST elevation * Ix: CT head (typically blood in basal cisterns, sulci or ventricles), LP if still suspect but CT negative (xanthochromia, do from 12h remains detectable for 2w) * Refer to neurosurgery * After confirmed look for a cause - CT angiogram +/- catheter angiogram * Ddx: migraine (longer onset to peak and no neck stiffness), sudden benign headache, reversible vasoconstriction, acute bacterial meningitis (rupture of a micro-abscess), cervical arterial dissection * Comps: obstructive hydrocephalus, arterial spasm causing ischaemic stroke, hyponatraemia (usually SIADH), seizures, re-bleeding * M: bed rest, control HTN, nimodipine for 3w (CCB, thought to prevent vasospasm), also prevent vasospasm by permissive hypertension + haemodilution; treat hydrocephaluus like a drain or VP shunt; neurosurgical discussion, endovascular treatment of aneurysms

Answer 88

Blood in SDS due to vein rupture, usually venous rupture (may be trivial), chronic SDH common in elderly/anticoagulants/infants as bridging veins predisposed to bleed CF: fluctuating, headache, drowsiness, confusion, focal deficits, coma + coning may follow (nb doesnt cause meningism) M: often resolve without drainage but need imaging + can cause death * Acute-decompressive hemicraniectomy * Chronic-typically over several weeks, may do burr hole drainage if symptomatic

Answer 89

Typical occur after a skull # that tears a branch of the middle meningeal artery - blood accumulates between dura + skull CF: brief unconscious after injury, improvement lucid interval, then lose consciousness with ipsilateral dilated pupil + c/l hemiparesis - rapid transtentorial coning M: need urgent imaging + neurosurgery (craniotomy + evacuation of haematoma)

Answer 90

* Infection from the ears/nasopharynx/head injury/meningeal defect/haematogenous - Bacteria: N meningitides, S pneumonia (main 2 causes), S aureus, GBS (neonates), E coli, Listeria - Viruses: enteroviruses like echo/coxsackie, HSV, mumps, HIV, EBV - Fungi: cryptococcus neoformans, candida albicans * May also be non-infectious like malignancy, intrathecal drugs, blood post-SAH

Answer 91

Headache, fever, neck stiffness, photophobia, vomiting, malaise * Meningococcal sepsis: a/w petechial or non-specific rash, meningitis may be a part of this along with shock + peripheral vascular infarcts * Viral causes tend to have less prominent signs + less severe sx

Answer 92

* Empiric: IV cephalosporin (e.g. cefotaxime), IM benzylpenicillin in community, add vancomycin in areas of high resistance * Meningococcus - benzylpenicillin if confirmed sensitive * Pneumonoccus + Haemophilus - cephalosporin e.g. cefotaxime * Immunocompromised - add ampicillin to cover Listeria * Dexamethasone to reduce oedema * LP needed unless contraindicated. Features of bacterial include polymorphs, raised glucose and low glucose, whereas in viral the CSF may have lymphocytes but other parameters are normal

Answer 93

Acute inflammation of brain parenchyma by viruses like HSV, VZV, flaviviruses like West Nile Virus. CF: fever, meningism, personality/behavioural change, reduced consciousness, coma, seizures, focal deficits CSF-high lymphocytes, viral PCR for the virus M: IV acyclovir for 14-21d, anticonvulsants for seizures Comps: RICP, midline shift/coning, long term memory impairment, personality change, epilepsy

Answer 94

Subacute inflammation of parenchyma due to autoantibodies Causes include paraneoplastic syndromes, voltage gated potassium channel antibodies (usually >50y), anti-NMDA receptor antibodies (limbic encephalitis with psychiatric disturbance + seizures) M: IV Ig, plasma exchange, steroids, rituximab, cyclophosphamide

Answer 95

Continuous seizures for >5 mins or 2 or more seizures without recovering consciousness over a similar period

Answer 96

Abrupt withdrawal of AED in a stable pt, alcohol withdrawal, hypoxia, CNS infection, fever, cancer, med change, metabolic disturbance, trauma, stroke

Answer 97

* Convulsive or not * Absence status - continuous stuporous state * Continuous seizure activity in one area without LoC, often a neoplasm/infarct

Answer 98

* Ensure airway patent, high flow O2 * Early up to 30m: O2, bloods (calcium, glucose, AED levels, drug screen), lorazepam IV 4mg bolus can repeat once/IV diazepam/buccal midazolam * Established status 30-90m: IV phenytoin infusion or fosphenytoin (specialist, need cardiac monitoring) * Ongoing seizures IV phenobarbital * Refractory >90m: GA for intubation + ventilation, CT/MRI for a cause

Answer 99

A complication of acute cord compression at or above T6 - unbalanced physiological response to stimuli such as urinary retention --> extreme hypertension, flushing, sweating above level of lesion, agitation. If untreated can cause haemorrhagic stroke cos of the HTN M: control the stimulus, treat HTN/bradycardia

Answer 100

Compression of the cauda equina which is the exit points of spinal nerves from L2 onwards (as the cord ends at L1-conus medullaris) Compression basically knocks out everything below L1. Usually progressive Causes: disc herniation esp L5/S1 and L4/5, trauma (#, subluxation), neoplasm (mets, primary), infection (discitis, Pott's), chronic inflammation e.g. ankylosing spondylitis, iatrogenic (e.g. haematoma after epidural anaesthesia)

Answer 101

LMN features! * Perianal/LL anaesthesia (often b/l) * Painless urinary retention, may present as overflow incontinence [confirmed retention=complete CES, reduced ability to void=incomplete CES] * Faecal incontinence - lose sense of fullness, lack of resting anal tone * Impotence * Reduced LL sensation (often b/l) + motor weakness * LL motor weakness * Severe back pain * Hyporeflexia Ddx: cord compression (but UMN signs), radiculopathy (painful but no urinary/anal/sexual dysfunction)

Answer 102

* Always do DRE + post-void bladder scan * Whole spine MRI within 6h * Catheterise * Neuro r/v-urgent decompression * High dose steroids e.g. dexamethasone to reduce localised oedema * Immobilise if traumatic cause * If cancer may do RT/CT

Answer 103

* Linear skull # - heal spontaneously, not always a/w brain injury * Depressed skull #: often get epilepsy, usually need surgical elevation + debridement * MAy lead to EDH (MMA rupture), SDH (dural vein tears), CSF rhinorrhoea/otorrhoea, meningitis

Answer 104

* Diffuse axonal injury from shearing/rotational forces, if on opposite side contrecoup effect * Direct trauma * Oedema + RICP * Hypoxia * Ischaemia

Answer 105

* Incomplete recovery of cognition or hemipareiss * Post-traumatic epilepsy * Post-traumatic syndrome with vague headache/dizzy/malaise/depression, even after minor injuries * BPPV * Chronic SDH * Hydrocephalus * Chronic traumatic encephalopathy - from repeated minor injuries causing cognitive impairment + motor sx

Answer 106

A granulomatous large vessel arteritis seen in people >50y, a/w polymyalgia rheumatica

Answer 107

* headache over temporal/occipital areas, tenderness, loss of arterial pulsation, thickened arteries, red scalp, rarely see gangrenous patches * jaw claudication, face/mouth pain due to inflamed branches of the ECA, worse on eating, hard to protrude tongue, rarely get tongue ischaemia * visual loss from posterior ciliary artery occlusion (causes AION, disc swollen + pale), sudden monocular partila/complete LoV or amaurosis fugax may precede * systemic: severe malaise, fever, WL due to jaw claudication Ix: normochromic normocytic anaemia, raised ESR/CRP, LFT may be abnormal cos of PMR (low albumin), temporal artery biopsy is definitive within 7d (don't delay the steroids, can get negative biopsy as lesions patchy)

Answer 108

* Corticosteroids-prednisolone, high dose then reduce gradually need for about 2y. done to reduce irreversible LoV/if LoV already happened protect the other eye * If not improving in 24-48h consider another cause

Answer 109

Acute lesion in brainstem of nuclei 9 to 12, or LMN lesions of these cranial nerves Causes include medial/lateral medullary syndrome strokes, GBS, polio, neurotoxins like botulism or from scorpion

Answer 110

* EDH: hyper dense biconvex/lentiform collection around brain surface * Acute SDH: hyperdense crescent around brain, not limited by suture lines * Chronic SDH: hypodense crescent around brain, not limited by suture lines * SAH: hyperdense area in brain cistern/sulci * Intraventricular haemorrhage: hyper density in ventricles * Intracerebral haemorrhage: hyper density within brain parenchyma

Answer 111

Epileptic seizure - sudden synchronous discharge of neurones in brain causing sx/signs Epilepsy - occurrence of spontaneous unprovoked seizures within the individual Seizures may be provoked by epilepsies or an acute cerebral insult

Answer 112

* Primary generalised epilepsies * Developmental e.g. neuronal migration problems * Hippocampal sclerosis: main cause of TL epilepsy, RF-childhood febrile convulsions. may be amenable to resection * Brain trauma or surgery * Mass lesions * Vascular e.g. stroke, AV malformation * Infections e.g. encephalitis, meningitis, TB, malaria * Immune e.g. NMDA receptor encephalitis * Genetic channelopathies * Degenerative e.g. Alzheimer's * Metabolic e.g. hyponatraemia, hypocalcaemia, hypoglycaemia, hypoxia, uraemia * Drugs: many overdoses, opioids, anti-emetics, SSRIs, quinolone Abx, antipsychotics, BZDs esp in withdrawal, alcohol withdrawal, amphetamines, cocaine

Answer 113

aka partial. Electrical discharge from a limited area of one hemisphere, can undergo secondary generalisation if inhibition fails * May have an aura * Simple - consciousness not impaired i.e. aura sx * Complex - consciousness impaired, amnesia/confusion common

Answer 114

Simultaneous involvement of both hemispheres with loss of consciousness/awareness * Tonic clonic * Absence * Myoclonic * Tonic / clonic / atonic

Answer 115

Onset in children/early adulthood, common * Childhood absence epilepsy - spontaneously remits by 18y * Juvenile myoclonic epilepsy - 10% of all epilepsies, usually myoclonic jerks beginning in teenagers then generalised TC seizures, 1/3 also get absences, often occur on waking. Triggers like lack of sleep/alcohol/strobe lights, needs treatment for life

Answer 116

Momentary loss of awareness, vacant expression <10s then return to normal, may have slight eyelid fluttering (complex type), may have many attacks per day Can reassure that these are never due to tumours May be precipitated by hyperventilation

Answer 117

Often no prodrome, tonic phase then clonic phase with synchronous limb jerking ~2 min, may utter initial cry/bite tongue/lose continence/cyanosis Post-ictal: flaccid unresponsiveness then return of awareness with confusion + drowsiness ~15 min, headache + deep sleep common Muscles may ache for days after

Answer 118

* Myoclonic: momentary brief contractions of muscle/muscle groups * Tonic: stiffening, no jerking * Atonic: sudden collapse, loss of tone, LoC

Answer 119

* With aurA: usually stereotyped. TL auras (deja vu, fear, olfactory/gustatory/auditory hallucinations, altered perception, abdominal rising sensation, nausea etc), frontal (conjugate gaze deviation away from epileptic focus), occipital (positive visual phenomena like zigzag lines) * Focal motor seizures: jerking begins on one side then spreads (Jacksonian march), sometimes local temporary paralysis (Todd's paresis) * Focal seizures with altered awareness: usually from TL/FL, usually aura, pt doesnt remember it, may evolve to secondary generalised

Answer 120

Prolonged recovery >2min, severe bitten tongue, prolonged tonic + clonic phases, cyanosis, stereotyped attacks Pallor indicates syncope Poor discriminatorS: urinary incontinence + injury as can occur in syncope or seizure

Answer 121

Stop + inform DVLA! * First unprovoked seizure - ban is 6m seizure free or 5y if HGV * Normal car: seizure-free for 1y, with or without AED * HGV/minibus etc: seizure free for 10y * Individual bias for things like not losing consciousness, changing medsprovoked

Answer 122

* Avoid precipitants * Safety advice like don't swim alone, working from heights, but live life as normally as poss just more precautions * Stop driving, inform DVLA * Discuss recurrence risk + treatment (around 3/4 have another seizure in the first 6m) * emergency advice: prolonged >5 min can terminate with buccal midazolam

Answer 123

* After 2 unprovoked seizures * Firm diagnosis + risk of recurrence * If pt considers risk of further seizure unacceptable * Structural abnormality * Clear EEG changes

Answer 124

* Start low + titrate * Aim for mono therapy, if not working gradually withdraw whilst introducing the new, but may need combination * Prescribe by brand name to ensure consistent drug levels - esp phenytoin + carbamazepine * Monitoring not needed usually * Be aware of interactions * Phenytoin only used in emergencies * Consider withdrawal after seizure free for 2-3y, but 50% recurrence, withdraw over 2-3 months * Never use sodium valproate in women of childbearing age

Answer 125

* Generalised TC: carbamazepine, lamotrigine, sodium valproate (SV v effective but not in child-bearing age) * Absence: ethosuximide, sodium valproate, lamotrigine * Focal: carbamazepine, lamotrigine, levetiracetam, SV * Tonic/atonic: SV, lamotrigine * Myoclonic: levetiracetam, SV, topiramate

Answer 126

* Skin rashes: lamotrigine, carbamazepine, phenytoin * Blood dyscrasias: carbamazepine * SV: weight gain, nausea, alopecia, ataxia, tremor, hepatotoxicity, pancreatitis, thrombocytopenia, teratogenic * Lamotrigine can cause rash for first 8w, usually mild * Phenytoin: coarse facial features, gingival hyperplasia, enlarged lips, hirsutism (not really used now except status), peripheral neuropathy * Carbamazepine: hyponatraemia, SIADH

Answer 127

* Lamotrigine conc reduced by half with oral contraceptives * Carbamazepine halves morning after pill conc * Phenytoin, carbamazepine + phenobarbital are all enzyme inducers * Enteral feedings can interfere absorption of AEDs - give via the NG during the feed-free period

Answer 128

N+V, ataxia, fine rapid nystagmus on lateral gaze IV intoxication - bradycardia, hypotension, arrhythmia

Answer 129

May be done for uncontrolled seizures caused by hippocampal sclerosis

Answer 130

* Risk of uncontrolled epilepsy > risk of meds * Adv taking 5mg folic acid daily well before pregnancy + in T1 * Avoid SV obv (NTD), phenytoin (cleft palate), lamotrigine showing low rate of malformations * Breast feeding usually considered safe

Answer 131

* Need effective contraception if on SV * Lamotrigine can use any except COCP * Phenytoin/carbamazepine/topiramate - don't use COCP/POP/implant (depo or a coil)

Answer 132

* Not needed as leads to unnecessary alterations of dose * Monitor dose by seizure response + ADRs * May do serum plasma conc in these circ: adjusting phenytoin dose (complex pharmokinetics), check adherence to AED therapy, help confirm clinical impression of toxicity, if admitted with status epilepticus

Answer 133

* Headache lasting 4h to 3d if untreated * At least 2 of: unilateral pain, throbbing, moderate-severe intensity, motion sensitivity * At least 1 of: nausea/vomiting, photophobia/phonophobia * normal examination, no other cause At least 5 attacks of these

Answer 134

* Usually severe enough to prevent normal activities * Sleep normally improves it * May be triggered * During: tender scalp, prefer dark quiet environment, may have vertigo * May have auras - focal neurological sx immediately preceding the headache phase, usually evolves over 5-20m but rarely lasts >1h. Visual aura commonest with zigzag lines/patchy scotomas/hemianopia; positive sensory like tingling/dysphasia * In children attacks may be shorter, headache more commonly b/l and GI disturbance more prominent

Answer 135

Sleep changes, stress/letdown after stress, hormones in women (PMS, OCP, menopause), eating (skipping meals, alcohol; individual foods rarely a trigger), sensory stimuli, weather changes

Answer 136

Rare autosomal dominant condition of hemiparesis and/or coma with headache, recover in 24h but some permanent cerebellar signs

Answer 137

* Explanation, adv headache diary for triggers, avoid triggers, lifestyle like sleep hygiene/hydration/regular meals/stress management * Acute treatment ASAP after onset, if have aura take at the start of the headache not start of the aura * Don't overuse rescue remedies * Migraine suppression when getting frequent attacks + impacting QoL, usually after 3-6m will reduce frequency + severity

Answer 138

* Consider possible causes * Consider emotional problems triggering migraine and consequences like missing school * Encourage 8w headache diary * Analgesia - paracetamol or NSAID, intranasal triptan if ineffective (oral triptans not licensed for children) * Low threshold for referral

Answer 139

* Paracetamol and/or NSAIDs first line * Anti-emetic e.g. metoclopramide * Triptans where simple analgesics ineffective - these are serotonin agonists, take as soon as poss after headache onset, can be oral/orodispersible/nasal/SC. ADRs: triptan sensations (tingling, heat, tightness in throat/chest, heaviness, pressure); CI in h/o of sig RF for IHD/stroke - Oral sumatriptan first line (IN/SC if vomiting) - CI in IHD, uncontrolled HTN, CVA, prev TIA - S/e: dizziness, drowsiness, dyspnoea, flushing, nausea

Answer 140

* Beta blockers e.g. propranolol SR (safer in women of child-bearing age) * Anticonvulsants e.g. topiramate * TCAs e.g. amitriptyline (2nd line)

Answer 141

* New onset migraine is rare in pregnancy + pre-existing migraine usually improves - consider secondary causes like pre-eclampsia or cerebral venous thrombosis * Always check BP+urinalysis * If it is indeed migraine try to avoid meds, use paracetamol if needed or ibuprofen (avoid ibuprofen in T3 as can cause premature closure of ductus arteriosus)

Answer 142

Chronic headache from using acute meds like triptans/paracetamol etc for 10-15+ days per month * a/w TTH and migraine * treatment is withdrawal (adv headache may temporarily worsen) * prob due to psychological factors + acquired central sensitisation

Answer 143

Mild-moderate headache usually b/l and a tight band sensation/pressure behind eye/bursting sensations (not pulsating) * Not aggravated by ADLs, no nausea, no autonomic sx (may get photophobia), may have cranial tenderness * Can last between 30m-7d! * May be infrequent, frequent or chronic (occurring for >half the month for 3m+) * Often a/w depression

Answer 144

* Adv headache diary to monitor frequency severity + which treatments work * Simple analgesia (if needing opioids is not TTH) * Physical - massage, ice packs * Identify co-morbidities like chronic pain, sleep disorders

Answer 145

U/L pain in trigeminal distribution (usually V1) + prominent ipsilateral autonomic features Includes cluster headaches, paroxysmal hemicranial (briefer + more frequent, not in clusters, respond to indomethacin), SUNCT (v short attacks v rare)

Answer 146

Mostly affects 20-40yo men * CF: recurrent bouts excruciating u/l retro-orbital pain + parasympathetic activation (red eye, tearing, nasal congestion, transient Horner syndrome), pt rocks/moves around, last 30-90m, may get several times per day esp at night, clusters in 1-2m most nights then stop and recur ~1y later * No analgesics or prophylaxis really help, give SC/IN sumatriptan, high flow oxygen (can get at home) * Verapamil, lithium or steroids can help terminate a bout * Avoid triggers like smoking and alcohol

Answer 147

* Migraine * TTH * Trigeminal autonomic cephalgias inc clusters * Ice pick headache: primary stabbing headache, good response to indomethacin, a/w other primary headaches * Primary cough headache: sudden sharp head pain on coughing, exclude intracranial pathology then indomethacin can help * Primary sex headache: explosive headache at/before orgasm, exclude SAH after 1st episode * Chronic daily headache: headache on 15+ days per month for at least 3m, many causes but majority are chronic migraine +/- medication overuse

Answer 148

* RICP * IIH * Low pressure headache * Post-traumatic * Local pathology like dental reasons, sinusitis

Answer 149

Present on waking, worse on cough/straining, a/w vomiting, visual obscurations (momentary LoV when bend/cough), papilloedema (often not present if rise is rapid) Causes include mass lesions, venous sinus thrombosis and hydrocephalus

Answer 150

Typically younger overweight women. A/w tetracyclines, isotretinoin, contraceptives, steroids, levothyroxine, lithium, cimetidine * CF: headaches, transient visual obscurations, CN VI palsy (a false localising sign), papilloedema, CSF pressure very elevated, imaging normal * M: self-limiting, but monitor visual fields as chronic papilloedmea can damage optic nerve. If needed may try repeated LP, acetazolamide or thiazide diuretics to reduce CSF. Adv weight loss. Sometimes need VP shunt/optic nerve sheath fenestration to protect vision

Answer 151

Reduced CSF volume following LP/spontaneous intracranial hypotension/vigorous Valsalva/straining/coughing Causes a postural headache that is worse on sitting/standing and relieved by lying flat. Don't do LP but if you did would have a low opening pressure M: 'blood patch' (inject autologous blood into epidural space to seal the leak), occasionally do surgical repair of dural tear, sometimes IV caffeine + bed rest can help

Answer 152

May worsen pre-existing migraine or be a new development but usually goes; consider SDH + low pressure headaches

Answer 153

Thunderclap headache, fever, meningism, non-blanching rash, RICP signs, new neuro deficit, new cognitive dysfunction, personality change, impaired conscious level, recent head injury, new onset in elderly (GCA), significant change in pattern of a chronic headache (SOL), h/o malignancy, immunosuppression, pregnancy (pre-eclampsia)

Answer 154

Progressive degenerative disorder of neurone loss in brainstem + basal ganglia Highest risk >70y, M>F, environmental like pesticides, non-smokers, genetic component in early-onset but usually sporadic

Answer 155

* Lewy bodies (neuronal inclusions) * Loss of dopaminergic neurones from pars compact of substantia nigra in midbrain --> inadequate dopamine transmission * Degeneration in other basal ganglia nuclei

Answer 156

* Typically initially u/l then slowly progresses through the stages over a 10-20y course * Prodrome ~7y before full PD where 90% get anosmia; may also get depression, aches, REM sleep behaviour disorder, restless legs, autonomic (urinary urgency, hypotension), constipation * Bradykinesia: slow movement. E.g. issues standing up, micrographia, difficulty with buttons, impassive face, serpentine stare, reduced blinking * Tremor: typically in hand then spreads to leg of same side then opposite side. Is a resting tremor, 'pill rolling', worse with emotion/stress, improved by movement * Rigidity: lead pipe (stiffness throughout passive ROM, not speed-dependent unlike spasticity), cogwheel rigidity (if also tremor as jerkiness) * Posture + gait disturbance: stooped, shuffling gait, slow turns, freezing, difficulty starting/stopping, reduced arm swing; falls are a late feature * Speech + swallowing: quiet, loss of intonation, may have drooling, late swallowing difficulty * Cognitive + psychiatric in late stage, may develop dementia, visual hallucinations on treatment can happen

Answer 157

Clinical diagnosis, MRI only used if suspect another cause, DAT sometimes used (ligand binding to dopaminergic terminals to see extent of loss)

Answer 158

* Education, encourage physical activity * Dopamine replacement (when sx begin to cause disability): levodopa (combined w dopa decarboxylase inhibitor), dopamine agonists (non-ergot e.g. ropinirole as less chance of fibrotic reactions, give domperidone on initiation as may cause nausea, less effective but fewer motor ADRs), MAO-B inhibitors (e.g. selegiline/rasagiline, mild effect reduce catabolism of dopamine), amantadine (improve dyskinesias in advanced), apomorphine (short acting dopamine agonist, SC for 'rescue' or continuous infusion in advanced) * Deep brain stimulation: usually <70, selected pt with disabling dyskinesias not controlled with meds, can lower med dosage after this * Levodopa intestinal gel infusion via jejunostomy in patient-controlled pump, severe/palliative when apomorphine + DBS contraindicated * Non-motor: treat depression, pain, constipation, sleep disorders * For delirium use lorazepam not anti-psychotic * PT, OT, SALT, physical aids, PD specialist nurse

Answer 159

* Wearing off - duration of effect of individual doses becomes progressively shorter * Dyskinesias * On/off phenomena: sudden unpredictable transitions from mobile to immobile Possible strategies include dose fractionation of levodopa, adding a COMT inhibitor (entacapone) to prolong duration of action, slow-release levodopa for overnight sx, avoiding protein-rich meals which impair levodopa absorption, deep brain stimulation if eligible, levodopa intestinal gel Should not be stopped acutely as can precipitate neuroleptic malignant syndrome

Answer 160

Neurodegenerative disorders affecting the basal ganglia with similar CF to Parkinson's disease, may also be the result of drugs V progressive and usually lead to death within 10y. A few types have a response to levodopa Features: symmetrical presentation, no tremor, poor response to levodopa, early falls within the 1st year (PD this is a late sign), additional neurological features

Answer 161

* Vascular PD * Dementia with lewy bodies (whereas in PD dementia the cognitive features are late) * Multisystem atrophy: Parkinsonism, autonomic + cerebellar signs (cerebellar/Park sx predominate), no response to levodopa * Progressive supranuclear palsy: parkinsonism, postural instability, vertical supra nuclear gaze palsy, pseudobulbar palsy, dementia * Corticobasal degeneration: alien limb phenomena, myoclonus, dementia * Drug-induced: dopamine blocking drugs. Antipsychotics (not clozapine), antiemetics (except domperidone). Antimuscarinics help like procyclidine as they block cholinergic receptors, but may make tardive dyskinesia worse * Dementia pugilistic: multiple head injuries causing dementia but also tremors, reduced coordination, gait changes * Wilson's disease: rare but treatable with penicillamine to chelate copper. Copper deposited in basal ganglia, cornea (Kayser Fleisher rings), and liver. Test serum copper + caeruloplasmin

Answer 162

* Tumours e.g. medulloblstoma, haemangioma, mets, vestibular schwannoma compression * Friedreich's ataxia, ataxic telangiectasia * Alcohol * Stroke - POCS * MS - demyelination * Hypothyroidism * Drugs: phenytoin, lead poisoning * Paraneoplastic e.g. secondary to lung cancer

Answer 163

* Lateral: distorted movement on same side, rebound overshoot when limb pressed down + released, ataxic gait towards side of lesion, action tremor with past-pointing, dysdiadochokinesia, imprecise movements, coarse horizontal nystagmus, dysarthria * Central: truncal ataxia, broad-based ataxic gait * Complications: hydrocephalus from obstructed acqueduct, coning of cerebellar tonsils through foramen magnum causing respiratory arrest, tonic seizures

Answer 164

Progressive degeneration of motor neurones in the motor cortex + SC anterior horns, and degeneration of cells in CN nuclei of the brainstem --> UMN + LMN are affected Relentless progression over 1-5y with no cure Features: fasciculations, no sensory features (tho early on may get vague things like pain), mix of UMN+LMN signs, wasting of small hand muscles/tibialis anterior Discriminating features: doesn't affect extra ocular muscles, no cerebellar signs, abdominal reflexes usually preserved. Nerve conduction studies: normal motor function, help exclude neuropathy EMG: reduced number of APs, increased amplitude MRI: to exclude cervical cord compression myelopathy

Answer 165

5-10% familial, rest sporadic, 50-75y commonest group, before age 50 M>F but after equal

Answer 166

* Amyotrophic lateral sclerosis: mixture of UMN+LMN signs in limbs, head + neck, normal sensation * Progressive muscular atrophy: LMN signs that later progress to UMN * Progressive bulbar + pseudobulbar palsy: bulbar palsy of the brainstem motor nuclei, pseudobulbar of bilateral UMN. Affect lower CN nuclei + their supra nuclear connections * Primary lateral sclerosis: pure UMN, then over time progress to involve LMN

Answer 167

* Counselling, careful discussions * Speech therapy + communication aids, altered food for safe swallow * Riluzole (glutamate inhibitor) - increases survival by 2-3m * Baclofen or tizanidine - spasticity * Pain management * PT, walking aids, splints, wheelchairs, home adaptation * EOLC - full palliative care * NIPPV, CPAP for nocturnal hypoventilation

Answer 168

An acquired immune-mediated inflammatory condition of the brain + SC (does not affect the PNS) Peak onset 20-30y and F>M

Answer 169

Areas of demyelination (plaques) in the WM of the brain + SC around venules, axons relatively preserved Inflammatory infiltrate of cells, interstitial oedema in acute lesions Common sites are around ventricles, corpus callosum, brainstem, cerebellum, cervical cord, optic nerves Demyelination is rare, functional recovery occurs but unsure of mechanism. Some relapses are irreversible due to loss of axons + cell bodies

Answer 170

* Relapsing + remitting - lesions in different parts of CNS at different times, 90% begin as this * Secondary progressive: starts as R-R then recovery from each relapse becomes less complete * Primary progressive: little/no recovery from relapses, cumulative disability from the beginning

Answer 171

* Optic neuritis: u/l subacute visual loss, central scotoma, pain on eye movement, papillitis if optic nerve head involved, colour vision defects, usually no residual defects except optic atrophy (scarring, if get RICP in future doesnt show papilloedema). Ix anyone presenting with this for MS (tho may also be transverse myelitis or isolated) * Retrobulbar neuritis when the optic nerve behind the head is inflamed * Brainstem presentation: demyelination causes diplopia (often INO), nystagmus (cerebellar connections), vertigo, dysarthria (cerebellar), facial numbness/trigeminal neuralgia (CN V effects), dysphagia, limb + truncal ataxia, pyramidal signs (CST involvement), hemisensory/patchy sensory changes in limbs * Spinal cord lesion (myelopathy): spastic paresis (T) or tetra paresis (C), tonic limb spasms, bladder sx, sensory sx, sx worse in heat * Neuropathic pain e.g. trigeminal neuralgia (demyelination affecting the sensory root next to the brainstem)

Answer 172

* MRI: hyperintense lesions on T2 images, if different contrast levels suggests separated in time so support MS * CSF: oligoclonal bands of IgG; may also have mild lymphocytosis, raised protein * Evoked potentials: visual evoked potentials seeing demyelination along optic nerve, somatosensory, brainstem auditory evoked potentials

Answer 173

*McDonald criteria to diagnose: lesions consistent with inflammatory process, no alternative diagnosis, multiple lesions in space + time, progressive neurological deterioration for >1y (primary progressive) Relapses: new sx, rapid worsening of sx or sx for >24h in absence of infection/another cause, after a stable period of 1y or more

Answer 174

* Anti-inflammatory: high dose IV methylprednisolone for acute relapses to reduce duration * Immunomodulators/suppressants to reduce relapses: multiple trials * Spasticity: baclofen, dantrolene, diazepam, tizanidine (don't reduce tone too much as some pt use tone to walk, PT to prevent contractures, botulinum toxin injection) * Bladder dysfunction: oxybutynin, tolteridone, intermittent self-catheterisation, early UTI treatment * Carbamazepine or phenytoin for paroxysmal sx like burning pains or tonic spasms * Clonazepam may help intention tremor

Answer 175

* Acute disseminated encephalitis after viral illness or vaccine. Confluent symmetrical WM change * Progressive multifocal leucoencephalopathy (due to a virus) * Leucodystrophies (of WM) * Subacute sclerosing pan encephalitis: rare + deadly delayed complication of measles * Vit B12 deficiency * Central pontine myelinolysis from too-rapid correction of sodium, often in h/o alcohol abuse

Answer 176

Acute cord inflammation usually of the full cross section for 1/2 segments * Onset over days, recovery weeks-months (often incomplete) * Affects all modalities at that level * Causes: para-infectious autoimmune response, systemic inflammation like SLE, infection like TB/syphilis/Lyme disease, MS * M: high dose steroids, other immunosuppression, antimicrobial if infective

Answer 177

Rare condition causing recurrent oral/genital ulceration, uveitis, neurological sx (brainstem + cord lesions), aseptic meningitis/encephalitis, cerebral venous thrombosis People from Turkey, Middle East + Asia most affected

Answer 178

ALS: brisk jaw jerk, dysarthria, dysphagia, wasting + fasciculation of tongue, progressive focal muscle wasting + weakness, fasciculations, brisk reflexes, extensor plantar responses, cramps PMA: wasting weakness + fasciculations but preserved tendon reflexes, begins asymmetrically in small muscles then spreads, later get UMN signs Bulbar + pseudobulbar palsy: dysarthria, dysphagia, nasal regurgitation, choking, 'Donald Duck' voice, wasting + fasciculation of tongue Eye movements + sphincters almost never involved in MND so this excludes it May get painful cramps at night, may have sensory symptoms without sensory signs, resp muscles can be involved causing T2RF

Answer 179

Brief electric sensation down limbs upon neck flexion, indicating SC lesion - demyelination, vit B12 deficiency

Answer 180

Intermediate state between normal cognition + dementia, with memory impairment more than expected for age but w/o other features of dementia

Answer 181

* Degenerative: AD, DLB, FTD, Huntington's, PD, prion disease * Vascular dementia, cerebral vasculitis * Metabolic: uraemic, liver failure * Toxins: alcohol, solvent misuse, heavy metals * Vitamin deficiency: B12 or thiamine (B1) * Trauma: severe/repeated brain injury * Brain lesions - SDH, tumours, hydrocephalus * Infection: HIV, neurosyphilis, TB

Answer 182

* RF: age, FH 1st degree relative 2x risk, some rare inherited forms, environmental * Deposition of beta amyloid plaques, structural changes in tau protein + ACh deficiency -- cortical atrophy + neuronal death * CF: insidious onset, gradual progression, episodic memory impairment initially, word-finding, apraxia, agnosia, frontal executive function impairment, visuospatial/navigatory problems; late non-cognitive features like myoclonus, sleep-wake reversal, incontinence (but usually motor still fine - this is why they wander) * MRI shoes TL atophy esp hippocampal, generalised atrophy. may be normal in early stages

Answer 183

* Multi-infarct, diffuse small vessel disease or post-stroke * CF similar to AD but cognitive sx vary depending on site of damage, stepwise deterioration, apraxic gait disorder, pyramidal signs, urinary incontinence * MRI shows widespread small vessel disease

Answer 184

* Group of disorders, asymmetrical FL + TL atrophy, usually onset <65y + FH * Frontal presentations: personality change, apathy, disinhibition, behavioural change; episodic memory preserved * Temporal presentations: aphasia, fluent speech lacking meaning, increasing telegrammatic speech * Eventually they merge

Answer 185

* CF: visual hallucinations (often animals/people), fluctuating cognition, variation in attention, sleep disorders like REM sleep behaviour disorder, autonomic dysfunction, parkinsonism (occurs later + more mild than in PD), delusions, transient LoC * Memory often preserved in early stages * May respond to AChE inhibitors * Dramatically worsened by neuroleptic drugs

Answer 186

Cognitive problems a late feature in PD, parkinsonism predominates. May respond to AChE inhibitors

Answer 187

* Exclude dementia mimics like depression * General: supportive, familiar environment for as long as poss, carer support * Medical treatments for dementia * Psychiatric management - depression can trial antidepressants, agitation only use antipsychotics as a last resort due to high stroke risk * Legal: may wish to set up LPA if they still have capacity, may be entitled to financial benefits * Driving: must inform DVLA then do a driving safety assessment

Answer 188

* Cholinesterase inhibitors: donepezil, rivastigmine, galantamine. Increase ACh by reducing destruction, improves memory + cognition in AD, DLB and Parkinson's dementia. S/e are the cholinergic GI sx; CI in cardiac conduction defects so do ECG before * Memantine: NMDA receptor antagonist. Used in severe AD or if cholinesterase inhibitors CI * Disease modifying therapies are in development

Answer 189

- Neuropathy: peripheral nerve affected by pathology - Mononeuropathy: a single nerve - Mononeuritis multiplex: several individual nerves affected. In DM, leprosy, vasculitis, amyloidosis, malignancy, neurofibromatosis, HIV, HCV. When it is symmetrical is hard to distinguish from polyneuropathy - Radiculopathy: disease affecting nerve roots - Plexopathy: brachial/lumbosacral plexus - Polyneuropathy: see below

Answer 190

Diffuse symmetrical disease that can be motor, sensory, sensorimotor or autonomic, and may be demyelinating or axonal

Answer 191

Acute polyneuropathy (usually demyelinating) Anti-ganglioside antibodies in 25% Antibody responses against peripheral nerves, usually post-infectious (often trivial or unidentified). Common culprits are Campylobacter jejuni and CMV. Onset 1-3w after the infection Does not recur

Answer 192

* weakness of distal limb muscles and/or distal numbness; ascending weakness is typical over days-6w * low back pain * loss of tendon reflexes * may require ventilatory support if face/resp muscles involved

Answer 193

* IV immunoglobulin in first 2w (but screen for IgA def beforehand as if they have it risk severe reaction to the IgG antibodies) * Plasma exchange if can't have IV Ig * LMWH heparin + compression stockings to reduce VTE * Gradual improvement over months-years, but 30% left disabled and 5% die

Answer 194

Paraproteins can cause myeloma: * IgM paraproteins cause a demyelinating distal neuropathy, ataxia + tremor * POEMS syndrome

Answer 195

Common, progressive symmetrical numbness+tingling in hands+feet, spreads proximally in glove + stocking distribution, and an ascending distal weakness. Lose tendon reflexes NCS - axon degeneration

Answer 196

* DM: microvascular complication. Distal symmetrical sensory changes, acute painful sensory, mononeuropathy, mono neuritis multiplex (inc CN lesions), diabetic amyotrophy (reversible plexopathy), autonomic neuropathy * Uraemia: progressive sensorimotor neuropathy * Porphyria: episodes of severe proximal neuropathy in limbs, sometimes abdo pain/confusion/coma * Malignancy * Critical illness

Answer 197

* Motor: phenytoin, dapsone * Sensory: anti-retrovirals, vincristine, paclitaxel, isoniazid, metronidazole * Both: amiodarone, disulfiram, chloroquine

Answer 198

* Polyneuropathy * Myopathy * Acute intoxication * Withdrawal: morning shakes, tremor, delirium tremens, seizures * Thiamine def: polyneuropathy, Wernicke-Korsakoff syndrome * Epilepsy * Cerebral infarction * Cerebellar degeneration * Cerebral atrophy, dementia * Cardiomyopathy, other myopathy * Hepatic stupor + coma

Answer 199

* B1 (thiamine): Beriberi=polyneuropathy + HF; Wernicke-Korsakoff syndrome * B6 (pyridoxine): sensory neuropathy (this is why you give pyridoxine when prescribing isoniazid) * Nicotinic acid * B12: damage to SC, peripheral nerves + brain

Answer 200

Spondylosis (degenerative changes), trauma, herpes zoster, tumours (neurofibroma, mets), meningeal inflammation, malignant infiltration (painful radiculopathy +/- plexopathy e.g. Pancoast's apical bronchial carcinoma --> T1 + sympathetic outflow lesion --> wasting of small hand muscles, pain + T1 sensory loss, ipsilateral Horner's syndrome)

Answer 201

* Trauma * Malignant infiltration * Neuralgic amyotrophy: severe pain + wasting + demyelinating brachial plexopathy * Thoracic outlet syndrome: fibrous band/cervical rib extends from tip of C7 transverse process towards 1st rib, compresses lower BP roots (C8+T1) --> forearm pain at ulnar border, T1 sensory loss, thenar muscle wasting esp APB. Can lead to Horner syndrome or subclavian artery/vein occlusion

Answer 202

Acute facial palsy likely caused by a virus causing CN VII compression within the facial canal * CF: u/l LMN facial weakness (ie frontalis affected) over 24-48h, may alter taste, hyperacusis (1/3), pain behind ear, O/E sensation normal (but pt often report vague altered sensation) * Bell's phenomenon: the normal upward conjugate eye movement when eyes closed is accentuated when orbicularis is weak * Outside UK may be caused by Lyme disease, or HIV seroconversion * Majority get complete/near recover over 4-12w + unlikely to recur * M: lubricating eye ointments/tape at night (if can't blink to avoid keratitis, may need ophthalmology), prednisolone for 10d (improves outcomes if start within 72h), if incomplete plastic surgery can help

Answer 203

Median n compression, usually idiopathic but can be seen in hypothyroidism, T3 pregnancy, RA, acromegaly, amyloidosis CF: numbness, tingling + pain in median n distribution, may radiate to forearm, fingers feel swollen, wasting of APB, sensory loss in radial 3.5 fingers. Tinel's (tapping carpal tunnel) + Phalen's (hold wrist in flexion) tests may reproduce the pain M: splint overnight in dorsiflexion, steroid injection into carpal tunnel (not the nerve!), if persisting may need surgical decompression

Answer 204

Ulnar n compressed in cubital tunnel (elbow) from prolonged/recurrent pressure or # CF: clawing of hand, wasting interossei + hypothenar muscles, weak interossei + medial 2 lumbricals, sensory loss in little finger and 0.5 ring finger; or can just affect the deep motor branch Decompression doesn't normally work

Answer 205

At spinal groove of humerus, acutely like 'Saturday night palsy' or more chronic, recovers in 1-3m CF: wrist drop, weakness in brachioradialis + finger flexors

Answer 206

Compression of the lateral cutaneous nerve of the thigh @ the inguinal ligament --> numbness + burning dyaesthesia (more sensitive to light touch) over anterolateral thigh Causes: sudden weight gain, injury, pelvic surgery

Answer 207

Compressed at neck of fibula e.g. yoga, prolonged squatting, plaster cast CPN supplies anterior + lateral leg motor + sensation to dorsum CF: foot drop, weak ankle eversion, unable to extend toes; ankle jerk preserved (as S1 spinal root not the nerve), patch of numbness on anterolateral border of foot dorsum; may confuse with L5 radiculopathy, usually recover

Answer 208

Compression to the sympathetic nerves causing unilateral partial ptosis (lose superior tarsal part of LPS but CN III innervates the rest), miosis, anhidrosis (dep on level), enophthalmos Causes: * Brain: massive infarction, brainstem demyelination * Cervical SC: syringomyelia, cord tumours * T1 root: apical lung tumour, TB, cervical rib, brachial plexus trauma * Sympathetic chain + carotid: after thyroid/laryngeal/carotid surgery, carotid artery dissection, neoplastic cervical sympathectomy * Misc: cluster headache, congenital

Answer 209

May compress CN VIII, V, VII, IX, X, XI (in that order of likelihood) --> vestibular schwannoma, meningioma, arachnoid cyst

Answer 210

Cord + peripheral nerve damage affecting the CSTs, DCs + spinocerebellar tracts Caused by Addisonian pernicious anaemia, and rarely other causes of B12 deficiency or vitamin E deficiency *NO abuse may cause a functional B12 deficiency

Answer 211

Numbness + tingling of fingers/toes, distal sensory loss (esp DC), absent ankle jerk, b/l spastic paresis, limb ataxia; if cord is involved may have exaggerated ankle jerks + extensor plantar; may cause optic atrophy + retinal haemorrhage Late stage: sphincter disturbance, severe generalised weakness, dementia M: parenteral B12 to reverse nerve damage (little effect on cord + brain)

Answer 212

Fluid-filled cavity (syrinx) in the SC central canal; syringobulbia when occurs in the brainstem. Expands so destroys ST neurones, anterior horn cells + lateral CSTs; syringobulbia lower CN nuclei Causes: Arnold-Chiari malformation (abnormality @ foramen magnum allows CSF pressure waves to be transmitted to fragile tissues in cervical cord/brainstem), or spinal cord trauma or intrinsic cord tumours

Answer 213

* UL pain worse on exertion * STT sensory loss e.g. burn self without noticing. Classically 'cape like' distribution (neck + arms) * Maintain DCML - so may not notice the ST losses * Loss of UL reflexes, muscle wasting, spastic paraparesis mostly UL * Syringobulbia: may progress to this with brainstem signs, tongue atrophy + fasciculation, bulbar palsy, rarely Horner syndrome * Bowel/bladder dysfunction * Scoliosis

Answer 214

* Full spine MRI - cavity + herniation of cerebellar tonsils * Brain MRI to exclude Chiari malformation * M: is slowly progressive over decades but minor trauma/surgery may cause sudden deterioration; treat cause + if symptomatic place shunt into the syrinx

Answer 215

Clinical signs due to pathology in/adjacent to cavernous sinus (on either side of pituitary fossa) Causes: neoplastic, inflammation e.g. GPA/sarcoidosis, infection B/V/F, vascular (cavernous haemangioma, cavernous sinus thrombosis-a/w prothrombotic states, causes eye pain fever proptosis chemosis ophthalmoplegia papilloedema, M-heparin, oral anticoagulants for 6m, AED if seizing), congenital, traumatic

Answer 216

Oculomotor n, Trochlear n, Ophthalmic + Maxillary nerves (divisions of trigeminal) C (ICA), Abducens nerve, Trochlear n Dural venous sinus divided into multiple small 'caves' by fibrous bits

Answer 217

Multiple u/l CN palsies, any combination of: * Ophthalmoplegia - usually p/w diplopia * Facial sensory loss in CNV1/2 * Horner syndrome * Pain if inflammatory cause * Vascular signs like chemosis, proptosis

Answer 218

* Metastases (35%): usually from bronchus, breast, stomach, prostate, thyroid, kidney * Primary malignant (35%): gliomas (include astocytoma [vary in aggression], oligodendroglioma [often grow slowly over decades]; usually in the hemispheres + spread in CNS not outside), ependymoma, lymphoma, medulloblastoma * Benign (15%): meningioma (commonly find symptomless ones incidental e.g. in pituitary fossa), neurofibroma (schwannomas, of Schwann cells most commonly CN VIII vestibular n sheath, may be b/l in NF2)

Answer 219

* Direct infiltration: impairment of local function, depends on area. E.g. in frontal lobe can disturb personality/intellect/expressive aphasia/hemiparesis; parietal lobe can cause optic radiation homonymous vision defect/sensory loss/hemiparesis/partial seizures; CPA can cause deafness/facial numbness/facial weakness/cerebellar ataxia * Secondary effects: RICP (papilloedema, vomiting, headache), shift of intracranial contents (impaired consciousness, coning), false localising signs that don't point to site of mass (e.g. CN VI lesion (first on side of mass then b/l) * Seizures: esp partial that evolve into generalised

Answer 220

* Ix: MRI/CT/PET, LP CI as withdrawing CSF can cause coning, stereotactic biopsy via burr hole * M: dexamethasone to reduce oedema, surgical removal sometimes possible, radiotherapy often in glioma/mets, chemo has limited role

Answer 221

``` Prolactinoma - 50% GH-secreting ACTH-secreting Gonadotrophin-secreting (rare) Thyrotroph secreting (rare) Non-secreting - 25% ```

Answer 222

* Mass effects: headache, VF defects (superior temporal quadrantonopia or bitemporal hemianopia), cavernous sinus compression (usually CN III palsy, may compress IV/VI and V1+V2), erosion through sphenoid sinus causing CSF rhinorrhoea * Hormone over-secretion (usually one): hyperprolactinaemia (v high levels, amenorrhoea/galactorrhoea/breast pain/infertility/hirsutism/loss of libido, or in men impotence/loss of libido/reduced seminal fluid/galactorrhoea/gynaecomastia); gigantism/acromeglay; Cushing's disease; hyper secretion of TSH/FSH/LH * Hormone under-secretion (one or multiple): GH deficiency (dwarfism in kids, wrinkling of skin + hypoglycaemia in adults), gonadotrophin def (delayed puberty, amenorrhoea, impotence), TSH def (usually mixed with others, hypothyroidism sx), ACTH def (reduced skin pigment, poor appetite, N+V, fatigue, loss of body hair), prolactin def (failure of lactation), loss of PP hormones (ADH deficiency causing polyuria + thirst)

Answer 223

Haemorrhage/infarction of pituitary * CF: severe acute headache, neck stiffness, rapidly progressive visual impairment, extra ocular muscle palsies, primary pituitary insufficiency, coma * M: IV hydrocortisone, fluid balance, trans-sphenoidal surgery; if survive lifelong hormone replacement

Answer 224

Neuropsychiatric disorder caused by thiamine (B1) def --> petechial haemorrhages in brain structures Usually in alcoholics, may also be seen in persistent vomiting, gastric cancer or a dietary deficiency

Answer 225

* Nystagmus * Ophthalmoplegia + conjugate gaze palsies * Ataxia, cerebellar signs, vestibular paralysis * Confusion, altered GCS * Peripheral sensory neuropathy * Vomiting, fever, hypothermia, ptosis, non-reacting miotic pupil * Korsakoff's syndrome: if not treated --> retrograde amnesia, confabulation, irreversible short-term memory loss

Answer 226

Urgent oral/IV thiamine replacement | Untreated mortality 20%

Answer 227

Small irregular pupil that is fixed to light but consticts on convergence -- due to a brainstem lesion around aqueduct of Sylvius Caused by neurosyphilis, DM or MS

Answer 228

Most common human prion disease * Sporadic: rapidly progressive dementia + death within 6m, myoclonus, usually ~65y * Iatrogenic: prions resistant to sterilisation so from neurosurgical instruments or transplant like corneal grafts, long incubation * Familial: rare, different phenotypes like fatal familial insomnia

Answer 229

Young pt were affected in an outbreak caused by feeding cows mechanically recovered 'meat' containing spinal cords of cows infected with these prions - average onset 25y - neuropsychiatric sx, ataxia + dementia with myoclonus/chorea Course up to several years All pt who have died so far have had a specific feature of the prion protein, uncertain for the future

Answer 230

* higher stroke risk * seroconversion - meningo/encephalitis, Guillain Barre, Bell's palsy * chronic meningitis from fungi/TB/listeria * AIDS-dementia complex * Abscess * CNS lymphoma * Peripheral neuropathy

Answer 231

Reactivation of VZV in DRG (sensory ganglia), often a/w reduced cell-mediated immunity (ageing, cancer etc) * Dermatomal shingles * Post-herpetic neuralgia: pain >4m after shingles, burning, poor response to painkillers, lasts ~2y * CN involvement: CN V1-corneal scarring, CN VII -Ramsay Hunt syndrome * Myelitis if spreads to adjacent SC

Answer 232

* Meningovascular: subacute meningitis with CN palsies + papilloedema, gumma (chronic expanding mass in brain), paraparesis * Tabes dorsalis: demyelination in dorsal roots causing lightning pains, ataxia, sensory loss, wasting, Charcot joints, Argyll Robertson pupils, ptosis, optic atrophy * “General paralysis of the insane”: dementia + weakness M: benzylpenicillin IM

Answer 233

Epilepsy due to the pork tapeworm, M-control seizures + anti-helminth agent

Answer 234

Usually a/w Strep angionsus and Bacteroides from sinuses/teeth, or Staph from penetrating trauma, multiple a/w HIV, can be due to fungi CF: headache, focal signs, epilepsy, RICP, fever, leucocytosis, raised CRP, ring-enhancing mass with oedema Do not do LP M: aspiration + high dose Abx Can also get brain tuberculoma, subdural empyema from ME infections, intracranial epidural abscess or spinal epidural abscess (fever, back pain, paraparesis, root lesions)

Answer 235

Anosmia | Due to head trauma, olfactory groove tumours, URTI or local nose disorders

Answer 236

* Causes: brainstem problem, cerbellopontine angle tumours, cavernous sinus + skull base tumour/infection, peripheral branches * CF: u/l sensory loss on face, scalp, anterior 2/3 tongue, buccal mucosa, jaw motor fibres interrupted so deviates to side of lesion, reduced corneal reflex

Answer 237

* CF: usually unilateral sx. UMN-contralateral weakness, frontalis spared, eye closure mostly unaffected; LMN-ipsilateral weakness, frontalis affected, eye closure weak, taste often impaired * Causes: brain (stroke, UMN, other stroke sx), pons (LMN, may also have hemiparesis), cerebellopointine angle (can compress V, VI and VIII nerves like VS or meningioma), petrous temporal bone problem (Bell's palsy, trauma, middle ear infection, Ramsay Hunt), skull base/parotid gland/within face * B/L weakness rare but more likely identifiable cause, less obvious as no asymmetry. E.g. Infection like Lyme disease, sarcoidosis, tumours, pontine lesions, NM disorders like GBS/MG, dystrophies

Answer 238

* brainstem: infarct, syringobulbia, MND * skull base: nasopharyngeal carcinoma, glomus tumour, neurofibroma, jugular venous thrombosis, trauma * neck/nasopharynx: nasopharyngeal carcinoma, mets, carotid artery dissection (CN XII), trauma, LN biopsy in posterior triangle (CN XI)

Answer 239

Usually occur together as disease @ jugular foramen affects both: * U/L CN IX sx: reduced sensation on same side of pharynx * U/L CN X sx: i/l failure of soft palate elevation so drawn to opposite side, i/l VC paralysis * B/L CN IX + X: palatal weakness, reduced sensation, absent gag reflex, dysphonia, choking

Answer 240

Weakness of SCM + trapezius, neuralgic pain if due to a nerve section e.g. LN biopsy

Answer 241

* U/L: tongue weakness, wasting + fasciculation, protruded tongue deviates to weaker side * B/L supra nuclear lesions (UMN): slow limited tongue movements, stiff tongue

Answer 242

* Bulbar: LMN weakness of muscles with CN nuclei in the medulla (lower CN nuclei, the nerves themselves or NMJ malfunction). causes dysphagia, dysarthria, dysphonia * Pseudobulbar: UMN (b/l supranuclear) lesions of lower CNs causing weak tongue + pharynx muscles, resembles bulbar palsy, often have emotional lability + exaggerated jaw jerk. Usually caused by MND, multiple infarcts or progressive supranuclear palsy

Answer 243

* Infarction in anterior 2/3 of SC: atherosclerosis, aortic dissection, trauma, cross-clamping in surgery, vasculitis, emboli, thrombotic disorders, severe hypotension * CF: acute paraplegia + loss of STT sensation * Occlusion of the artery of Adamkiewicz (supplies thoracic ASA): watershed infarction of the cord, typically at T8 level (perfusion relatively poor)

Answer 244

Rare but potentially curable, acquired or congenital -- direct connection between A+V causing oedema CF: progresive myelopathy over months-years, thoracic back pain M: endovascular ablation or can resolve w/o treatment as long as permanent damage not already occurred

Answer 245

AD, 50% sporadic * CF: multiple skin neurofibromas, cafe au lait patches, axillary freckling, Lisch nodules on iris, plexiform neurofibromas on nerves/roots/SC (remove if pressure) * Comps: LD, malignant transformation, scoliosis

Answer 246

AD, much less common than NF1. Causes many neural tumours like b/l vestibular schwannomas, meningiomas, gliomas inc of optic nerve, 30% skin neurofibromas, cafe au lait spots

Answer 247

Rare multi system AD condition causing depigmented ash leaf spots which depigment under UV, roughened skin patches over lumbar spine, fibromata beneath nails, angiofibromas, renal tumours, glial overgrowth, epilepsy + LD + developmental delay May also see retinal hamartomas, rhabdomyomas in heart, polycystic kidneys, multiple lung cysts

Answer 248

Rare disease causing cerebellar/spinal/retinal haemangioblastomas, RCC, phaeochromocytoma, polycythaemia

Answer 249

Can accompany cancer without there being mets, often a/w SCLC/breast/ovarian: * sensorimotor neuropathy * Lambert-Eaton myasthenic myopathic syndrome, MG (thymoma) * MND variants * Spastic paraparesis * Cerebellar syndrome * Limbic encephalitis * Paraneoplastic stiff person syndrome

Answer 250

Autoimmune disorder of NMJ transmission - anti-AChR antibodies + complement deposits at post-synaptic membranes so ACh receptors are destroyed. Peak 30y, a/w other autoimmune conditions (pernicious anaemia, thyroid, RA, SLE), often a/w hyperplasia/tumour of thymus gland (50-70% hyperplasia, 15% thymomas) Fluctuating course, mostly life-long, exacerbations by infection/aminoglycosides/beta blockers/lithium/penicillamine/phenytoin/macrolides/tetracyclines/quinolones/quinidine Comps: resp impairment, nasal regurgitation, dysphagia

Answer 251

* Weakness + fatiguability of proximal limb, bulbar + extra-ocular muscles, worse at end of day - diplopia, proximal muscle weakness, ptosis, dysphagia * Fluctuating diplopia, ptosis * Resp sx * Reflexes initially preserved but may fatigue * May get wasting after many years * Ix: serum anti-AChR and anti-MuSK (anti muscle specific tyrosine kinase) antibodies, EMG-high sensitivity, mediastinal MRI/CT to check for thymoma

Answer 252

* Oral anticholinesterases: pyridostigmine, lasts 3-4h, inhibits AChE to prolong ACh action. ADRs are muscarinic like colic/diarrhoea, oral atropine helps relieve this (antimuscarinic). doesnt alter course * Immunosuppresion with steroids/azathioprine in severe * Thymectomy: improves MG if have hyperplasia + positive AChR antibodies * Plasmapheresis + IV Ig in myasthenic crisis

Answer 253

Paraneoplastic syndrome a/w SCLC due to defective ACh release at the NMJ (occasionally can be autoimmune with no malignancy-a/w voltage gated calcium channel antibodies) CF: proximal limb muscle weakness, sometimes affects ocular/bulbar muscles, some absent reflexes, weakness tends to improve after a few mins of contraction + absent reflexes return (whereas in MG they don't)

Answer 254

* Inflammatory e.g. polymyositis, viral myositis, granulomatosis * Metabolic e.g. steroids- proximal weakness, thyroid, calcium + vitamin D-proximal myopathy, hypokalaemia, alcohol, drugs like steroids, lithium (weak), pain (fibrates), rhabdomyolysis (stains/interactions), malignant hyperpyrexia * Malignant hyperpyrexia – widespread skeletal muscle rigidity + hyperpyrexia due to GA/neuroleptics like haloperidol * NMJ e.g. MG * Muscular dystrophies * Myotonias – continued involuntary movement (failure of relaxation) * Channelopathies: ion channel disorders

Answer 255

*variant of Guillain-Barre syndrome a/w ophthalmoplegia, areflexia and ataxia. *eye muscles are typically affected first *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 256

* Predominantly motor loss: GBS, porphyria, lead poisoning, hereditary sensorimotor neuropathies (inc Charcot Marie Tooth), chronic inflammatory demyelinating polyneuropathy, diphtheria * Predominantly sensory loss: DM, uraemia, leprosy, alcoholism, vitamin B12 def, amyloidosis

Answer 257

Spondylosis means vertebral + ligamentous degenerative changes during ageing/trauma, like osteophytes, thickened ligaments, disc herniation + protrusion predisposed to neuro sequelae if already have spinal stenosis

Answer 258

Most common C7 protrusion --> root pain to triceps/extensor forearm + tingling/numbness in C7 distribution may also cause weakness/wasting and loss of triceps reflex initial pain can be v severe but usually recover , may do root decompression

Answer 259

L4-5 prolapse = L5 root compression, L5-S1 prolapse = S1 root compression [root below the disc interspace is compressed] CF: limited SLR, reflex loss (S1-ankle jerk), weak PF (S1) or great toe extension (L5), sensory loss in affected dermatome Most resolve with rest + analgesia + early mobilisation

Answer 260

A common disorder of older adults where posterior disc protrusion --> SC compression (common at C4-5, C5-6 and C6-7) because of congenital narrowing, osteophytes, thick ligaments + ischaemia CF: slowly progressive difficulty walking (spastic paraparesis), usually no neck sx, may have a reflex level in UL Ix: XR cannot diagnose it as can't visualise soft tissues, need MRI M: avoid neck manipulation, if more than mild needs decompression by anterior cervical discectomy (any degenerative cervical myelopathy need urgent referral as early treatment gives best chance of recovery)

Answer 261

E.g. CN VI lesion (first on side of mass then b/l), CN III lesion (TL uncus herniates, compressing nerve, first sign is ipsilateral pupil dilation as parasympathetic fibres compressed), hemiparesis on same side as hemisphere tumour (from compression of c/l cerebral peduncle on edge of tentorium; would normally expect hemiparesis on opposite side)

Answer 262

herpes zoster of geniculate ganglion. * CF: facial palsy identical to Bell’s, vesicles around EAM/soft palate, may have HL/vertigo/unsteadiness * Complete recovery less likely than in Bell’s * M: antivirals (aciclovir/valaciclovir) + steroids

Answer 263

Irregular painless u/l spasm of facial muscles, usually after middle age * Starts in orbicularis oculi and moves over years to other facial muscles on same side. Varies from mild to severe * Caused by compression of root entry zone, usually by vascular structures like vertebral/basilar artery/branches, but rarely CPA mass lesions * Tic convulsif: HFS + i/l trigeminal neuralgia * M: mild no treatment, botulinum toxin injection every 3-4m into affected muscles is main treatment, surgical decompression possible but risks of facial weakness + deafness

Answer 264

* Systemic changes of auto regulation to try to meet perfusion needs - usually hypertension * CN palsies e.g. unreactive pupils * Cardiac centre involvement-bradycardia * Resp centre involvement - Cheyne Stokes respitation * Coma Cushing's triad: widened pulse pressure, resp changes, bradycardia - pre-terminal

Answer 265

Immediately: * GCS < 13 on initial assessment/<15 at 2 hours post-injury * suspected skull fracture or signs of basal # (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign). * post-traumatic seizure. * focal neurological deficit. * more than 1 episode of vomiting If GCS is 8 or less need urgent neurosurgical r/v even before CT Within 8 hours: * If on warfarin * If have any of these risk factors plus LoC/amnesia: >65y, h/o bleeding or clotting disorders, dangerous mechanism of injury (eg hit by car), >30 mins retrograde amnesia of events before getting the injury

Answer 266

* deep coma of known cause, reversible causes excluded, no sedation and normal electrolytes * pupillary reflex, corneal reflex, oculo-vestibular reflex, cough reflex, absent response to supraorbital pressure, and no spontaneous respiratory effort * done by 2 appropriate doctors on 2 occasions, both experienced with 5y+ postgrad experience, one must be a consultant, neither can be a member of the transplant team

Answer 267

Similar to GBS but more chronic over weeks-months, antibody-mediated inflammation causing segmental demyelination of peripheral nerves. Motor features predominate. High protein in CSF. M-steroids, immunosuppressants

Answer 268

.*motor weakness * diplopia * visual symptoms in one eye only * poor balance * reduced consciousness

Answer 269

Rare condition seen in people taking antipsychotic meds, or dopaminergic drugs (usually when suddenly stopped/dose reduced)

Answer 270

* young males most common * onset in 10d of beginning/increasing dose, over hours-days * pyrexia * rigidity, reduced reflexes * tachycardia * usually raised creatinine kinase, may have leucocytosis * severe cases may get AKI due to rhabdomyolysis

Answer 271

Stop the drug, IV fluids to prevent renal failure, dantrolene in certain cases (reduces contraction in skeletal muscle), bromocriptine may be used (dopamine agonist)

Answer 272

Fast onset irt SSRIs/MAOIs/ecstasy/novel psychoactive substances Causes hyperreflexia, clonus, dilated pupils M: cyproheptadine, chlorpromazine

Answer 273

* Rectal diazepam 10-20mg (max dose 30mg if have to repeat); 10mg in elderly * Buccal midazolam 10mg

Answer 274

* Exertion: most common * Penicillamine * quinidine, procainamide * beta blockers * lithium * phenytoin * some abx like gentamicin, macrolides, quinolones, tetracyclines * suxamethonium in GA

Answer 275

Pain after damage/disruption to nervous system, eg diabetic neuropathy, post-herpetic neuralgia, prolapsed IV disc, trigeminal neuralgia * 1st line: amitriptyline, duloxetine, gabapentin or pregabalin (not trigeminal neuralgia in which 1st line is carbamazepine) * if not working try another of those 3 * tramadol as a rescue for exacerbations * topical capsaicin for localised e.g. post-herpetic * pain management clinics if resistant

Answer 276

* Temporal lobe (HEAD): Hallucinations (auditory/gustatory), Epigastric rising/Emotional, Automatisms (lip smacking, grabbing), Deja-vu or Dysphasia (post-ictal) * Frontal lobe (motor): head/leg movements, posturing, post-ictal weakness (Todd's paresis), Jacksonian march * Parietal lobe (sensory): paraesthesia * Occipital lobe: floaters/flashes

Answer 277

* Increases GABA activity * ADR: P450 inhibitor, nausea, weight gain, alopecia, ataxia, tremor, hepatotoxicity, pancreatitis, thrombocytopenia, teratogenic, hyponatraemia, hyperammonemic encephalopathy (treat with L-carnitine)

Answer 278

The main points for the exam are: left homonymous hemianopia means visual field defect to the left, i.e. Lesion of right optic tract; the homonymous hemianopia is always the same side as the paresis homonymous quadrantanopias: PITS (Parietal-Inferior, Temporal-Superior) incongruous defects = optic tract lesion; congruous defects = optic radiation lesion or occipital cortex A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric. Please see the link for an excellent diagram. More congruous the further posterior the defect is Homonymous hemianopia incongruous defects: lesion of optic tract congruous defects: lesion of optic radiation or occipital cortex macula sparing: lesion of occipital cortex Homonymous quadrantanopias* superior: lesion of temporal lobe inferior: lesion of parietal lobe mnemonic = PITS (Parietal-Inferior, Temporal-Superior) Bitemporal hemianopia lesion of optic chiasm upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma *this is very much the 'exam answer'. Actual studies suggest that the majority of quadrantanopias are caused by occipital lobe lesions. Please see the link for more details.

Answer 279

*inducer of the P450 system. *Acute: dizziness, diplopia, nystagmus, slurred speech, ataxia, confusion, seizures *Chronic: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness megaloblastic anaemia (secondary to altered folate metabolism), peripheral neuropathy, enhanced vitamin D metabolism causing osteomalacia, lymphadenopathy, dyskinesia *Idiosyncratic: fever, rashes (including severe reactions such as toxic epidermal necrolysis), hepatitis, Dupuytren's contracture, aplastic anaemia, drug-induced lupus *Teratogenic: associated with cleft palate and congenital heart disease Monitoring Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if: adjustment of phenytoin dose suspected toxicity detection of non-adherence to the prescribed medication

Answer 280

* Motor response: 6. Obeys commands 5. Localises to pain 4. Withdraws from pain 3. Abnormal flexion to pain (decorticate posture) 2. Extending to pain 1. None * Verbal response: 5. Orientated 4. Confused 3. Words 2. Sounds 1. None * Eye opening: 4. Spontaneous 3. To speech 2. To pain 1. None Glasgow coma scale (GCS) scores are generally expressed in the following format 'GCS = 13, M5 V4 E4 at 21:30'. Take the best response out of both sides

Answer 281

* Vomiting more than once with no other cause. * New neurological deficit (motor or sensory). * Reduction in conscious level (as measured by the Glasgow coma score). * Valsalva (associated with coughing or sneezing) or positional headaches. * Progressive headache with a fever. NICE suggest that if two or more of the ‘red-flag’ criteria are present then an urgent CT scan should be performed as the likelihood of a serious intracranial pathology being the cause of a presentation is high

Answer 282

* L5 radiculopathy: a/w weak hip abduction, web space paraesthesia, weakened DF + inversion + eversion of ankle * Sciatic nerve lesion: weak DF, inversion, eversion + PF * Common peroneal nerve lesion: most common, often compression @ neck of fibula. A/w reduced DF + ankle eversion * Superficial/deep peroneal nerve lesion * Central lesions-usually other features If peroneal neuropathy: avoid leg crossing, squatting = kneeling, usually improves over 2-3m

Answer 283

Weak plantar flexion, reduced sensation around lateral malleolus

Answer 284

Umbrella term for neuro + other sx that follow surgery/minor injury Type I: most common, no demonstrable lesion Type II: lesion to a major nerve CF: progressive disproportionate sx to original injury, allodynia, temp + skin colour changes, oedema, sweating, motor dysfunction Budapest Diagnostic Criteria M: early PT, neuropathic analgesia (so 1st line amitriptyline), specialist pain team management

Answer 285

SNHL - most common Epilepsy, paralysis Sepsis, intracerebral abscess Brain herniation, hydrocephalus

Answer 286

* If unilateral then signs of ipsilateral * Dysdiadochokinesia, Dysmetria (past-pointing), "Drunk" * Ataxia (limb, truncal) * Nystagmus * Intention tremor * Slurred staccato speech, Scanning dysarthria * Hypotonia

Answer 287

Spontaneous continuous LL movements, +/- paraesthesia, v common, FH in 50% CF: uncontrollable urge to move legs (akathisia) initially at night but may occur in day, worse at rest, paraesthesias e.g. 'crawling' or throbbing sensation, limb movements during sleep Causes include idiopathic, iron-def anaemia, uraemia, DM and pregnancy M: adv walking/stretching/massage, treat iron def anaemia, dopamine agonists e.g. ropinirole 1st line, then BZDs, then gabapentin

Answer 288

* u/l dilation, sluggish/fixed to light - 3rd nerve compression from tectorial herniation * b/l dilated pupils, sluggish/fixed to light - poor CNS perfusion or b/l 3rd nerve palsy * u/l dilated or equal, RAPD - optic nerve injury * b/l miosis - opiates, pontine lesions, metabolic encephalopathy * u/l constriction, preserved light reflexes - sympathetic pathway disruption

Answer 289

* Rapidly rising like in EDH use IV mannitol/furosemide * Diffuse cerebal oedema- may need decompresive craniotomy * May need to do Burr holes where scans unavailable but otherwise little use in modern practice ICP monitoring needed if GCS or 8 or less