Neurology Conditions Flashcards

Question

What is the driving advice for a first unprovoked seizure?

Answer 1

Can't drive for 6 months if no structural abnormality and no abnormality on EEG, if not 12 months off

Answer 2

Fit free for 12 months

Answer 3

No driving until 6 months after last dose

Answer 4

Decreased slowly over 2-3 months

Answer 5

Decreased slowly over 6 months

Answer 6

Patients on other medication - CYP inducers/inhibitors Women wishing to get pregnant Women on contraception

Answer 7

``` Leucopenia Visual disturbance Balance issues SIADH Erythematous rash ``` P450 Inducer

Answer 8

Dose changes depending on valproate and carbamazepine use alongside

Answer 9

Maculopapular Rash | Steven Johnson Syndrome - warn them to see a doctor if they have flu like symptoms

Answer 10

``` Nausea Teratogenic Liver failure Pancreatitis Hair loss Obesity Ataxia Tremor Thrombocytopenia Encephalopathy ``` P450 inhibitor

Answer 11

Bind to sodium channels to increase refractory period

Answer 12

Sodium channel blocker

Answer 13

Enhance GABA receptors

Answer 14

Bind to sodium channels to increase refractory period

Answer 15

``` Nystagmus Diplopia Tremor Dysarthria Dizzy/drowsy Peripheral neuropathy Gingival hyperplasia ``` CYP450 Inducer

Answer 16

Lamotrigine

Answer 17

Lamotrigine reduce effectiveness of Contraceptive Contraceptive reduce lamotrigine levels

Answer 18

5mg Folate OD

Answer 19

Most resolve within 5 minutes. If not resolving after 5-10 mins, administer benzodiazepine (4mg lorazepam)

Answer 20

In hospital: ABCDE ``` Secure airway High conc oxygen Assess cardiac and resp function Check blood glucose levels Gain IV access IV lorazepam 4mg, repeat after 10 mins if continues ``` If persists - IV phenobarbital or phenytoin In the community - buccal midazolam or rectal diazepam

Answer 21

Seizures lasting more than 5 minutes | Or more than 3 seizures in one hour

Answer 22

A partial seizure - affect initially only one hemisphere of the brain, or part of a lobe

Answer 23

Simple partial - only affect small region of brain, often temporal lobe or e.g. hippocampi Remain conscious Complex partial - unilateral cerebral hemisphere involvement, causes impairment of awareness or responsiveness i.e. altered consciousness

Answer 24

Absence epilepsy Starts in childhood EEG abnormalities - 3Hz generalised, symmetrical spike wave complexes Treat with sodium valproate, ethosuximide or both

Answer 25

``` Mode of onset Duration Nature of headache Site Pattern and timing Provoking and relieving factors Associated symptoms including aura Drug history ```

Answer 26

Paracetamol, aspirin, NSAID use for 15 days per month or more Triptans, opioids, ergot preparations for 10 days a month or more

Answer 27

``` Thunderclap Associated fever Meningism signs RICP New neurological deficit New cognitive dysfunction Personality change Impaired/deteriorating conscious level Recent head injury New onset headache in elderly - GCA Significant change in pattern of chronic headache History of malignancy or impaired immunity Postural headaches - worse on lying, standing, post LP ```

Answer 28

Present on waking, or wakes patient at night Exacerbated by sneezing, straining, bending, lying down Intracranial tumour - short history Effortless vomiting

Answer 29

Common in young, obese women Signs of RICP but no mass Morning headache Vomiting Visual disturbances - diplopia, visual obscurations (sudden transient bilateral visual loss with changes in posture) Bilateral papilloedema CSF examination by LP confirms RICP

Answer 30

Self-limiting, resolves with weight loss and LPs Chronic - carbonic anhydrase inhibitors e.g. acetazolamide, diuretics and corticosteroids

Answer 31

``` Severe global or occipital headache Vomiting Photophobia Nuchal rigidity - stiff neck Resistance to passive neck flexion Kernig's sign - pain and resistance to passive knee extension with flexed hip ```

Answer 32

Granulomatous inflammatory changes in branches of external carotid Headache, can localise to temples Scalp tenderness on combing hair Intermittent claudication of the jaw - impairment of blood supply to muscles of mastication Amaurosis fugax - transient loss of vision in one eye Constitutional symptoms - low grade fever, night sweats, shoulder and/or pelvic girdle pain

Answer 33

``` ESR grossly elevated CRP high Normochromic normocytic anaemia Abnormal LFTs, raised ALP Temporal artery biopsy - may be skip lesions ```

Answer 34

IV steroids once blood has been taken for ESR i.e. before biopsy 40-60mg pred per day Aspirin 75mg daily decreases visual loss and strokes PPI for gastric prevention whilst on steroids Referral to rheum, vascular surgeons, ophthalmology

Answer 35

Relapse Steroid SEs Aortitis leading to aortic aneurysm and aortic dissection

Answer 36

Mild ache across forehead in band like pattern Due to stress, depression, alcohol, skipping meals, dehydration Treatment - reassurance, basic analgesia, hot towels and relaxation techniques

Answer 37

Give similar presentation to tension headache, but clear cause Underlying medical condition e.g. infection, OSA, pre-eclampsia Alcohol Head injury Carbon monoxide poisoning

Answer 38

At least 5 headaches that: - Last 4-72 hours - Are severe, unilateral, pulsating and interrupt daily activity - Are associated with N&V or photo/phonophobia - Are not due to a secondary cause

Answer 39

``` Oestrogen (COCP and menstruation) Foods (cheese, red wine, citrus fruits) Stress Bright lights Alcohol ```

Answer 40

ACUTE: 1. oral triptan + NSAID or paracetamol 2. prochlorperazine + nasal triptan PROPHYLAXIS if >2/month: 1. propranolol (preferred in women) or topiramate (teratogenic) 2. acupuncture MENSTRUAL MIGRAINE frovatriptan

Answer 41

- transient hemianopic disturbance | - spreading scintillating scotoma

Answer 42

Severe, sudden onset, unilateral pain around the eye Ipsilateral autonomic features - rhinorrhoea, sweating, partial horner's, lacrimation, lid swelling Typically occur at night 1-2 hour bouts daily over 6-12 weeks

Answer 43

Triggers - alcohol, histamine, heat, exercise

Answer 44

Acute - subcutaneous sumatriptan, 100% O2 Prophylaxis - verapamil (some evidence for prednisolone) Surgery - trigeminal nerve blockade

Answer 45

``` V: temporal arteritis I: meningitis, sinusitis, malaria, HIV T: head injury, SAH A: M: hypothyroid I: N: brain metastasis, primary tumour D: medication induced, CO poisoning ``` Other: dental/ jaw

Answer 46

- loss of dopaminergic neurones in substantia nigra - leads to increased activity of basal ganglia and so hyperkinesia - most are sporadic with many gene loci identified - mean onset is in 60s

Answer 47

- TREMOR: worse at rest, pinrolling At 4-6Hz, or can be induced by concentration, usually apparent in one limb or limbs on one side for months - HYPERTONIA: rigidity (lead pipe- not velocity dependent), when combined with tremour gives cogwheeling during rapid pronation/ supination - BRADYKINESIA: slow to initate movement, actions slow and decreased in amplitude with repitition, slow blink rate and micrographia - GAIT: shuffling, pitched forward, loss of arm swing, en bloc turning

Answer 48

- expressionless face - autonomic dysfunction (postural hypotension, constipation, urinary frequency, urgency, drooling) - sleep disturbance (REM sleep disorder) - reduced sense of smell - neurpsychiatric complications: dementia, depression, psychosis - signs are almost always worse on one side- if bilateral look for other causes

Answer 49

- Levodopa: efficacy reduced over time so start it late as poss - dopa decarboxylase inhibitors (carbidopa/ co benledopa) will increase levo dopa's efficacy - dopamine agonists like ropinerole can help delay starting levodopa in early PD - apomorphine: potent DA agonists used to even out end of dose effects or as rescue pen for dose effects - anticholinergics: young pts only as confuse old ppl - MAO- B inhibitors: alternative to DA's in early PD - COMT inhibitors eg etacapone- help motor complications in late disease and lessen off time in end of dose wearing off - clonazepam and melatonin for REM sleep disorder - fludrocotisone if postural hypotension - quetiapine and clozapine for distressing hallucinations (worsens parkinsons) - Acetylcholinesterase inhibitor for dementia (donepezil and rivastigmine)

Answer 50

- deep brain stimulation: may help but only if partially dopamine responsive still - surgical ablation of basal ganglia circuits eg subthalamic nucleus

Answer 51

- Progressive supranuclear palsy: early postural instability, vertical gaze palsies +/- falls, rigidity of trunk> limbs, symmetrical onset, little tremor, speech and swallowing problems - Multiple systems atrophy: early autonomic features eg incontinence, postural hypotension + cerebellar + pyramidal signs, rigidity > tremor - Corticobasal degeneration: akinetic rigidity involving one limb, cortical sensory loss, apraxia -Lewy body dementia Early onset dementia <1 yr with features of parkinonism Dementia usually proceeding feature prior to motor symptoms, with visual hallucinations and fluctuating consciousness

Answer 52

- vascular parkinsonism: diabetic/ HTN pt with postural instability and falls - drugs: antipsychotics, metoclopramide - toxins: manganese - wilsons disease - trauma - encephalitis - neurosyphilis

Answer 53

Basal ganglia - gray matter cell bodies - contains caudate nucleus, putamen, globus pallidus, substansia nigra and subthalamic nucleus Basal ganglia helps kick start and fine tune movement initiated by the motor cortex Inhibition of muscle tone Coordinated slow sustained movement Suppression of useless patterns of movement Initiation of movement

Answer 54

Process of modulation dependent on direct and indirect pathways Basal ganglia needed for the modulation of pyramidal motor output Direct - mostly stimulatory pathway, shorter, mostly off, predominantly associated with D1 receptors Dopamine released from SN via dopaminergic neurones to activate D1 receptors Indirect - inhibitory pathway Longer, mostly on, D2 receptors Allows inhibition of muscular tone to prevent unnecessary movement

Answer 55

Step 1: diagnosis of Parkinsonian syndrome Bradykinesia + at least one: muscular rigidity, 4-6Hz resting tremor, postural instability Step 2: exclusion criteria for PD - history of repeated strokes, history of repeated head injury, definite encephalitis, sustained remission, cerebellar signs, dementia, Babinski's, hydrocephalus, negative response to LDOPA Step 3 - supportive prospective positive criteria of Parkinson's Three or more for definite: Unilateral onset, rest tremor present, progressive disorder, persistent asymmetry, excellent response to LDOPA, hyposmia, visual hallucinations

Answer 56

Occurs more than one year after diagnosis Similar to Alzheimer's Presence of Parkinsonism in the limbs Frequent visual hallucinations Frequent fluctuations in lucidity

Answer 57

``` Benign essential tremor Drug or toxin induced Huntington's Wilson's Corticobasal degeneraiton Creutzfeldt Jakob disease Multi-infarct dementia Lewy body dementia Pick's disease Cerebellar tumour ```

Answer 58

CT or MRI if fail to respond to therapeutic (600mg/day) of L-DOPA MRI to rule out rare secondary causes PET with flurodopa to localise dopamine deficiency Genetic testing - Huntington's Olfactory testing Measurement of ceruloplasmin levels - Wilson's, or syphilis serology

Answer 59

Resistance to passive movement of a joint Take their hand, passively flex and extend arm at the elbow, creates a tension in their arm that gives way to movement in small increments - little jerks

Answer 60

Parkinson's is asymmetrical, worse at rest, improves with intentional movement, no change with alcohol Benign essential tremor is symmetrical, improves at rest, worse with intentional movement, improves with alcohol

Answer 61

Synthetic dopamine Given orally to boost own dopamine levels Combined with other drug that stops levodopa being broken down in the body before it gets to the brain

Answer 62

Develops dyskinesias - abnormal movements associated with excessive motor activity Dystonia - excessive muscle contraction leads to abnormal postures or exaggerated movements Chorea - abnormal involuntary movements that can be jerking and random Athetosis - involuntary twisting or writhing movements usually in the fingers, hands or feet

Answer 63

Inhibitors of catechol-o-methyltransferase COMT enzyme metabolises levodopa in the body and brain e.g. entacapone taken with levodopa and a decarboxylase inhibitor to slow breakdown of levodopa in the brain

Answer 64

Pulmonary fibrosis

Answer 65

Mimic dopamine in the basal ganglia Stimulate dopamine receptors Less effective than levodopa Used in combination with levodopa to reduce the dose of levodopa required Bromocryptine Pergolide Carbergoline

Answer 66

Monoamine oxidase B enzyme - specific to breakdown of dopamine So these medicatoins bllock this enzyme, increasing circulating dopamine Usually used to delay use of levodopa Examples - selegiline, rasagiline

Answer 67

- Both may have mixed UMN and LMN signs - There is NO sensory loss or sphincter disturbance in MND - It also NEVER effects eye movements- helps distinguish from MG

Answer 68

- amylotrophic lateral sclerosis (ALS): Loss of motor neurones in motor cortex AND anterior horn of cord (so UMN +LMN signs). Definite if in 3 regions, probably if in 2. - Progressive bulbar palsy: bulbar palsy (LMD) , often mixed with pseudobulbar palsy (UMN) Affects primarily the muscles of talking and swallowing - progressive muscular atrophy: only affects anterior horn cells so LMN signs only, distal before proximal muscle groups affected - primary lateral sclerosis: rare, loss of betz cells in motor cortex, mainly UMN

Answer 69

- age >40 - stumbling spastic gain, foot drop +/- proximal myopathy, weak grip, poor shoulder strength or aspiration pneumonia are usually 1st presentations - UMN signs AND LMN signs (tongue fasiculation common) - frontotemporal dementia occurs in 25% - no sensory disturbance, eyes movements not effected Wasting usually begins at hands then spreads, may initially be unilateral Fasciculations, cramps Usually NO PAIN Bulbar and pseudobulbar features - progressive Dysphagia, dysarthria, nasal regurg, choking Swallowing solids difficult Reflex problems, lost or hyperreflexic (due to loss of corticospinal neurons) Sphincters not affected

Answer 70

- no diagnostic test - MRI brain/ cord to exclude structural cause - LP excludes inflammatory causes - neurophysiology studies can detect subclinical denervation and exclude mimicking motor neuropathies

Answer 71

- Riluzole: inhibits glutamate release and NMDA receptor antagonist, is the only drug proven to improve survival but only by 2-4 months but has significant impact on tracheostomy free survival - Positioning, oral care and anti muscarinic (glycopyrronium) can help with the excess saliva - Blended and thickened foods as well as gastrostomy can help with dysphagia - Exercise, orthotics can help with spasticity - Augmentative and alternative communication equipment can aid comms difficulty - Involve palliative care from point of diagnosis as median survival from diagnosis is only 2-4 yrs - Positive pressure ventilation can aid with breathing greatly - Muscle cramps- quinine, baclofen, gabapentin, diazepam - communication aids - Early discussion about end of life care are esp important with ALS as many will develop frontotemportal dementia and not be able to express wishes later on in the disease

Answer 72

Progressive degeneration of both upper and lower motor neurones The sensory neurones are spared Genetic component Take a good family history as 5-10% inherited Oxidative neuronal damage Aggregation of abnormally large amounts of protein inside a cell Glutamate problems Apoptosis Prolonged caspase activity - contributing to the apoptosis

Answer 73

Smoking Exposure to heavy metals Certain pesticides

Answer 74

Bulbar palsy - LMN palsy that affects CNs 9, 10, 11 and 12 Pseudobulbar palsy - UMN palsy affecting the corticobulbar tracts of 5, 7, 9, 10, 11, 12 Any condition which disrupts or damages the cranial nerve nuclei or corticobulbar tracts can cause this - stroke, MS, infections, tumours

Answer 75

Amyotrophic lateral sclerosis Lateral sclerosis refers to damage to the lateral corticospinal tracts Leads to spastic parapesis Amyotrophic - loss of muscle tone May be pyramidal weakness

Answer 76

Motor neuropathy - will cause distal weakness and LMN signs There will be conduction block Spinal muscular atrophy Kennedy's syndrome Hyperthyroidism and hyperparathyroidism can both result in muscle wasting and hyperreflexia Pseudobulbar palsy Cervical spondylitis and spinal tumours both exhibit both UMN and LMN signs

Answer 77

Bladder never affected No sensory signs Occular muscles never affected

Answer 78

Sodium channel blocker Can slow progression very slightly, especially if bulbar features Thought that by blocking sodium channels, effect of glutamate greatly reduced

Answer 79

GABA agonist | Helps reduce spasticity

Answer 80

Cell mediated autoimmune demyelination of the white matter CNS. This leads to reduced conduction velocity Disseminated (over time and space) - lesions vary in their location over time, meaning different nerves affected, symptoms change over time

Answer 81

The central nervous system (the oligodendrocytes) | Inflammation around myelin and infiltration of immune cells

Answer 82

``` Multiple genes EBV Low Vitamin D Smoking Obesity ```

Answer 83

Older patient Motor signs at onset Many MRI lesions

Answer 84

Optic Neuritis - pain on eye movement with greying and blurring of vision Odd sensations such as wetness or burning

Answer 85

Corticospinal path - motor pathway

Answer 86

Dysaesthesia (abnormal unpleasant feeling when touched) Pins and needles Decreased vibration sense Trigeminal neuralgia Lhermitte's syndrome - electric shock sensation that travels down the spine and into the limbs when flexing the neck, indicates disease in cervical spinal cord in DC Up to 75% report bladder dysfunction, may occur leading to incontinence, poor bladder emptying or urinary retention

Answer 87

Spastic weakness Myelitis Hyper-reflexia Transverse myelitis - local inflammation within the spine, leads to sensory and motor symptoms below the level of the lesion Cerebellar syndrome - disease of the cerebellum causes ataxia, slurred speech, intension tremor, nystagmus, vertigo, clumsiness

Answer 88

Erectile dysfunction Anorgasmia Urinary frequency Incontinence

Answer 89

Swallowing difficulty | Constipation/diarrhoea

Answer 90

``` Diplopia Optic neuritis Nystagmus Opthalmoplegia Uhthoff's Phenomenon - transient worsening of neurological symptoms in increase in temp ```

Answer 91

Trunk/limb ataxia Intention tremor Scanning speech (ataxic dysarthria)

Answer 92

Amnesia Executive dysfunction - emotional and behavioural difficulty Depression/mood disorders cognitive impairment

Answer 93

Relapsing-remitting (most common) Secondary progressive disease Primary progressive disease

Answer 94

Acute 1-2 month attacks followed by periods of remission

Answer 95

R-R patients don't fully remit and over time the disability accumulates (around 65% of R-R patients progress within 15 years) See gait and bladder problems

Answer 96

Progressive deterioration seen from the onset

Answer 97

Regular exercise Stop smoking Avoid stressful situations

Answer 98

High dose IV methylprednisolone

Answer 99

Not suitable for primary progressive. | Have to have had 2 relapses in past 2 years.

Answer 100

Reduce number of relapses and MRI changes No effect on disability

Answer 101

``` Interferon-B Glatiramer acetate Dimethyl fumerate Alemtuzumab Natalizumab Fingolimod ```

Answer 102

Baclofen and gabapentin | Physio

Answer 103

Botulinum toxin | Primidone

Answer 104

If significant residual volume - self catheterise | Frequency symptoms - Anticholinergics

Answer 105

Gabapentin

Answer 106

Amantadine and CBT

Answer 107

MRI contrast: demyelination plaques (hyperintense plaques in the periventricular region) Electrophoresis of CSF: oligoclonal bands of IgG Evoked potentials: prolonged conduction

Answer 108

Demyelinating diseases: neuromyelitis optica, tranverse myelitis Infections: Lyme's, tertiary syphillis Metabolic diseases: B12 deficiency, DM, copper deficiency SLE, sarcoidosis Neoplasia, vasculitis, stroke

Answer 109

- Optic neuritis: pain on eye movement, reduced central vision, red desensitisation- esp centrally, usually unilateral, sometimes swollen disc on fundoscopy acutely and after the disc appears white (scarred), RAPD - Intranuclear ophthalmoplegia (MLF syndrome)- when one eye looks laterally the other doesn’t turn medially or lags, often with nystagmus of the laterally moved eye, due to demyelination of medial longitudinal fasciculus connecting the IV and VI cranial nerves in the brain stem, can be bilateral

Answer 110

- worsen with heat (hot weather, shower, bath) - SENSORY: dysaesthsia, pins n needles, decreased vibration sense, trigeminal neuralgia - MOTOR: spastic weakness, myelitis, CN palsies-> diplopia - SEXUAL/ GU: ED, incontinence, urinary retention - EYE: diplopia, hemianopia, pupil defects, visual phenomena on exercise - cerebellum signs - cognitive: visual spacial neglect, accidnet, low mood, poor executive functioning

Answer 111

- clinical- McDonald criteria can be used - MRI sensitive but not specific for detecting lesions and can help exclude other causes - oligoclonal bands of IgG on electrophoresis of CSF can help indicate MS - Visual evoked response - measuring electrical activity via a transducer over the occipital cortex in response to a light stimulus

Answer 112

- Regular exercise, stop smoking and avoid stress may help - Legal obligation to inform DVLA - Dimethyl fumarate then interferon B for mild- moderate RR MS - Natalizumab if more active RR MS - Interferon B for primary and secondary progressive MS - Cannaboids effective in some - Metylprednisolone IV for 3-5 days shortens acute relapses but use sparingly (only for attacks lasting >24 hrs and moderate- severe), doesn’t alter overall progress - Baclofen/ gabapentin/ benzos for spasticity - Botulinum toxin injections for tremors - Self catheterisation if retainer - Amantadine, CBT and exercise can help with the fatigue - gabapentin for oscillopsia - exercise, cbt then amantadine for chronic fatigue

Answer 113

Optic nerves Spinal cord - majority associated with concomitant brain lesions Brainstem - may present with ophthalmoplegia Cerebellum - ataxia and gait disturbance Juxtacortical white matter Periventricular white matter near the ventricular system

Answer 114

90% characterised by relapsing-remitting disease course

Answer 115

First clinical episode of suspected MS No previous evidence of demyelination clinically or on neuroimaging Oligoclonal bands in CSF can be used as supportive criteria to help come to diagnosis

Answer 116

Abducens palsy - absence of lateral abduction of the eye If the right abducens nerve is affected, when looking to the right, the right eye will remain central

Answer 117

Diagnosis based on MRI findings - brain +- spinal cord and clinical presentation >2 attacks with objective clinical evidence of >2 lesions - MS diagnosed

Answer 118

NMO spectrum disorders Series of neuroinflammatory demyelinating conditions preferentially affecting the optic nerve and spinal cord Hallmark is acute attack of bilateral optic neuritis or transverse myelitis In NMOSD the presentation is more severe Associated with formation of IgG autoantibody to aquaporin 4 Treatment is similar to MS with corticosteroids, but plasma exchange may be required for refractory cases

Answer 119

Motor - Freezing of Gait, Falls | Non Motor - Aspiration Pneumonia, Pressure Sores

Answer 120

Guillaine Barre Syndrome Myasthenia Gravis Motor Neurone Disease Muscular Dystrophy

Answer 121

Acute inflammatory polyneuropathy characterised by progressive ascending neuropathy with weakness and reduced reflexes Often triggered by infections such as Campylobacter

Answer 122

Immune mediated damage to peripheral nerves (proposed antigenic mimicry)

Answer 123

Acute Inflammatory Demyelinating Polyneuropathy (90%) Acute Motor Axonal Neuropathy Acute Motor Sensory Neuropathy Miller Fisher Syndrome

Answer 124

Progressive symmetrical weakness (often ascending from distal muscles) Reduced/absent reflexes Reduced sensation

Answer 125

Ataxia Areflexia Opthalmoplegia

Answer 126

- Viral Screen - CXR (Rule out Sarcoidosis) - Electrophysiology (confirms diagnosis and differentiates axonal and demyelinating) - LP (increased protein and normal WCC) - AB for Miller Fisher

Answer 127

FVC<20ml/kg - ITU and intubation IVIG/Plasma Exchange

Answer 128

``` Severity scoring for GBS 0 - Healthy 4 - Bedridden 5 - Assisted Ventilation 6 - Death ```

Answer 129

Antibody mediaed blockage of Neuromuscular Transmission due to ACh receptor AB

Answer 130

- Type II Hypersensitivity reaction - Cross links receptors causing destruction and activates --Membrane Attack Complexes to destroy membrane - Disease fluctuates as NMJ can repair itself and create more receptors easily

Answer 131

1 - Fluctuating muscle weakness with most severe symptoms happening here 2 - Persistent but stable symptoms 3 - Remission

Answer 132

10-15% have a Thymoma Up to 85% have Thymic Hyperplasia There are myoid cells in the thymus which may start the target for autoimmunity

Answer 133

Ocular | Generalised

Answer 134

AChR - Ab Anti MuSK Anti - LRP4

Answer 135

Diplopia (improved on occluding one eye) Ptosis Weak eye movements Pupillary sparing

Answer 136

- Bulbar - Fatiguable chewing - Facial - Expressionless - Neck - Dropped head towards EOD - Proximal limb weakness more than distal

Answer 137

Improvement of ptosis after ice applied to closed eye for one minute Neuromuscular transmission is better at lower temperatures

Answer 138

- Tensilon Test (improvement after AChesterase inhibitors) - Serological Antibodies - Repetitive Nerve Stimulation (showing decline) - CT/MRI thymus

Answer 139

Pyridostigmine Prednisolone Azathioprine Could also do a Thymectomy

Answer 140

Worsening of weakness requiring respiratory support Precipitants include warmth/surgery/stress/infection

Answer 141

Acetylcholinesterase inhibitor | Blocks the action of acetylcholinesterase and increases the levels of acetylcholine

Answer 142

IVIG Plasma Exchange Steroids

Answer 143

Umbrella term that causes gradual wasting and weakness of muscles

Answer 144

- X linked inherited abnormal gene - If mother is a carrier, son has 50% chance of being affected - Boys present at 3-5y with pelvic muscular weakness - Life expectancy of 25-35y

Answer 145

Steroids to slow progression and creatine supplements

Answer 146

Dystrophin gene is less severely affected than in Duchennes | Symptoms start around 8-12y and may require a wheelchair by 20-30y

Answer 147

- Genetic disorder presenting in adulthood | - Progressive muscle weakness, prolonged contractions, cataracts, cardiac arrhythmia

Answer 148

In childhood, weakness around face progressing to shoulders and arms Sleep with eyes open, can't purse lips, can't blow out cheeks

Answer 149

Bilateral Ptosis Restricted eye movements Swallowing problems

Answer 150

- Contractures (usually at elbow and ankles) | - Progressive weakness and muscle wasting

Answer 151

Relatively common condition characterised by fine trmor affecting voluntary muscles, most noticable in hands but can affect other areas

Answer 152

Fine Tremor Symmetrical Improved by alcohol More prominent on voluntary (eg outstretched)

Answer 153

No definitive management | Propranolol or Primidone can be tried

Answer 154

Idiopathic facial nerve palsy

Answer 155

Difficulty chewing Tingling Ear Pain Hyperacusis

Answer 156

Loss of nasolabial folds Drooping corner of mouth Drooping eyebrow Asymmetric smile

Answer 157

A stroke affected UMN so is forehead sparing

Answer 158

House Brackmann Grading System

Answer 159

Overt pain Systemic Illness Hearing abnormalities

Answer 160

Prednisolone if within 72 hours Eye drops and tape for protection May take several months to recover

Answer 161

Corneal Abrasion Aberrant Reinnervation (voluntary contraction of one muscle leads to involuntary contraction of another muscle)

Answer 162

- Cervical pain worsened by movement - Cervical stiffness - Vague numbness/tingling/weakness of upper limbs - Radiculopathy - Retro orbital/Temporal pain

Answer 163

Lateral flexion and pressure on top of head

Answer 164

- Age of onset <20 ir >55 - Weakness/ Sensory loss in more than one Dermatome/Myotome - Constitutional symptoms

Answer 165

First 3-4 weeks reassure patient that it is common and will resolve Stay active One firm pillow at night

Answer 166

Physiotherapy, Occupational health referral, Pain Clinic, Surgery

Answer 167

Sensory/Motor symptoms in response to nerve root damage by any cause

Answer 168

Pain along posterior thigh and posterolateral leg due to L5/S1 radiculopathy

Answer 169

Pain along anterior leg/thigh due to L3/L4 radiculopathy

Answer 170

``` Peripheral Sensorimotor Acute Diffuse Painful Autonomic Mononeuropathy Diabetic Amyotrophy ```

Answer 171

Smoker >40 Periods of poor glycaemic control

Answer 172

Sensory nerves affected more (glove and stocking) Loss of ankle jerks and later knee Hands only in long standing

Answer 173

Abrupt onset but can resolve completely Burning foot pain (worse at night) Associated with poor glycaemic control

Answer 174

Cardiac abnormalities Exercise intolerance Reduced respiratory drive Reduced baroreceptor sensitivity

Answer 175

E.G Carpal Tunnel

Answer 176

Pain and paraesthesia in upper legs with weakness and wasting of muscle

Answer 177

Diabetic Control Measuring BP Nerve conduction studies Electromyography

Answer 178

Regular surveillance and foot care Good diabetic control bed foot cradles Neuropathic pain relief if required

Answer 179

Personal - Washing/Dressing/Toileting/Continence Domestic - Cooking/Cleaning/Shopping

Answer 180

Progressive neurodegenerative disorder that causes reduced mental performance and impairment in social and occupational function

Answer 181

A combination of factors Older Age, Genetics (Sporadic/APP/Presenilin), CVS Disease

Answer 182

Senile Plaques (Beta Amyloid deposits extracellularly, they are seen in normal ageing) Neurofibrillary Tangles (Hyperphosphorylated Tau Proteins in regions involved in memory, promoting cell death)

Answer 183

Cognitive - Poor Memory, Language Non Cognitive - Agitation, Emotional Lability

Answer 184

- Mini - Cog (three item word memory and clock drawing) - AMT (ten item tool) - MMSE (eleven item tool) - MoCA (several domains including executive function, attention, language, memory and visuospatial)

Answer 185

``` Functional Inability (and decline from previous) Cognitive (impairment in >2 domains) Differentials excluded (via neuroimaging etc) ```

Answer 186

Advanced Care Planning Inform DVLA Encourage groups and activities

Answer 187

Mild to Mod - AChesterase inhibitors (Donepazil) Mod to Severe - NMDA Antagonists (Memantine)

Answer 188

Behavioural Variant - Progressive personality and behavioural change Primary Progressive Aphasia (Non Fluent or Semantic)

Answer 189

Phosphorylated Tau Proteins/Pick Bodies Some familial cases with AD Inheritance Atrophy of frontal and temporal lobes

Answer 190

Disinhibition, Loss of empathy, Apathy

Answer 191

Effortful/Halting Speech Speech Apraxia Difficulty finding words

Answer 192

- MND - Corticobasal Syndrome (Dystonia, Asymmetrical Akinesia, Alien Limb) - Progressive Supranuclear Palsy (difficulty looking up)

Answer 193

Frontal and temporal lobe atrophy

Answer 194

Financial advice, SALT input, Supervision SSRIs (decrease impulsivity)/Atypical Antipsychotics

Answer 195

- Subcortical - Stroke Related (Large Cortical) - Single/Multiple Infarct - Mixed (with Alzheimers) - Autosomal Dominant

Answer 196

Stepwise cognitive decline | Can have focal neurological symptoms

Answer 197

Donepazil/Memantine | Control CVS factors

Answer 198

Classic triad of Miosis/Ptosis and anhidrosis/enopthalmos due to a lesion along oculosympathetic pathway

Answer 199

First Order - Hypothalamus to Spinal Cord Second Order - Preganglionic (spinal cord to sympathetic chain) Third Order - Post Ganglionic (within adventitia of IC, travelling to dilator pupillae and muller's muscle)

Answer 200

Stroke multiple Sclerosis SOL Syringomyelia Anhidrosis on one half of body

Answer 201

Tumour Trauma Thyroidectomy Top Rib

Answer 202

Carotid Aneurysms Carotid Artery Dissection (no other sx) Cavernous Sinus Thrombosis No Anhidrosis

Answer 203

CT Angiography (exclude dissection) MRI (if brainstem features) CXR (Pancoast)

Answer 204

Cocaine Eye Drops - blocks NA reuptake, only unaffected eye constricts Apraclonidine - Alpha agonist causing affected pupil to dilate and normal pupil to constrict Hydroxyamphetamine - all dilate except post ganglionics

Answer 205

Acoustic Neuroma | Meningioma

Answer 206

Tumours of the vestibulocochlear nerve arising from schwann cells Typically benign and slow growing but causes pressure on surrounding tissues

Answer 207

Neurofibromatoses | High dose ionising radiation

Answer 208

Unilateral/Asymmetric hearing loss/tinnitus Impaired facial sensation Balance problems without explanation

Answer 209

Facial Pain/Numbness Earache Ataxia Hydrocephalus

Answer 210

Audiology | MRI

Answer 211

Microsurgery (can cause CSF leak or stroke) | Radiotherapy (sterotactic, fractionated, proton beam)

Answer 212

Slow growing benign tumours arising from Dura Mate Normally well circumscribed

Answer 213

I - Generally Benign II - Higher rate of recurrence post surgery III - Anaplastic

Answer 214

``` Seizures Raised ICP Changes in personality Nerve palsies Can be spinal - Brown Sequard ```

Answer 215

Well defines Central Cystic Degeneration Oedema of nearby white matter

Answer 216

Endovascular Embolisation (Coil or Glue) Surgical Removal Stereotactic Radiotherapy

Answer 217

Degeneration of the posterior and lateral columns as a result of B12/Vitamin E/Copper deficiency

Answer 218

Dietary Deficiency Lack of IF Low Gastric pH

Answer 219

B12 and folate deficiency leading to megaloblastic anaemia

Answer 220

Weakness of leg/arms/trunk Progressive tingling and numbness Posterior Column - reduced vibration and proprioception Lateral Column - UMN signs, Ataxia

Answer 221

Replace B12 If folate also deficient, treat B12 first

Answer 222

Any pathology involving Cavernous Sinus which may present as a combination of unilateral opthalmoplegia, autonomic dysfunction, or sensory loss

Answer 223

Meningioma Sarcoidosis Basal Skull Fracture Cavernous Sinus Thrombosis

Answer 224

Can compress any nerves leading to isolated palsies or post ganglionic Horners May get Proptosis and Chemosis secondary to pressure

Answer 225

Bloods MRI CT

Answer 226

Tumour - Surgery or Radio Traumatic - Orbital Decompression Inflammatory - Steroids Vascular - Embolisation/Clipping

Answer 227

Spectrum of diseases resulting from Thiamine deficiency (usually related to alcohol abuse although can be secondary to Bariatric Surgery/Hyperemesis Gravidarum etc)

Answer 228

Inadequate nutritional thiamine/decreased absorption from GI tract/impaired thiamine utilisation Thiamine is a cofactor required by three enzymes for carbohydrate synthesis so causes metabolic disruption

Answer 229

Ataxia, Opthalmoplegia, Confusion Unexplained hypotension/hypothermia Hallucinations

Answer 230

At least 2 of: Dietary Deficiency Oculomotor Abnormalities Cerebellar Dysfunction Altered Mental State/Impairment

Answer 231

Thiamine + Pabrinex Alcoholics should have prophylactic thiamine as long as they are malnourished/have decompensated liver disease May have to use Mental Health Act

Answer 232

Confabulation Anterograde and Retrograde Amnesia Psychosis

Answer 233

Small irregular pupils that have little/no constriction to light but do accommodate Classically caused by neurosyphilis

Answer 234

Damage to dorsal aspect of EWN disrupts inhibitory neurones causing constant constriction

Answer 235

Best known Human Prion Disease | Accumulation of small infectious pathogens, containing protein but lacking nucleic acids

Answer 236

- Sporadic (85% of cases) - Hereditary (Familial clusters with dominant inheritance) - Iatrogenic (Neurosurgery, tissue grafts) - New Variant (linked to eating BSE infected cattle products)

Answer 237

- Myoclonus - Progressive Ataxia/Choreiform - Visual Disturbance - Rapidly progressing cognitive and functional impairment

Answer 238

- Brain Biopsy (can use tonsil biopsy if new variant) - CSF markers - EEG (Periodic wave complexes in sporadic) - MRI (increased intensity in certain brain regions depending on subtype)

Answer 239

Fluid filled tubular cyst in central spinal cord that can elongate/enlarge and expand into grey/white matter, compressing tracts

Answer 240

Where Syringomyelia has extended into brainstem | Can cause nerve palsies

Answer 241

Blockage of CSF (normally secondary to Chiari malformation) SOL, Arachnoiditis, Post Traumatic

Answer 242

Spinothalamic lost in a shawl like distribution Extends into Dorsal Column Dysaesthesia - Pain when skin is touched

Answer 243

As it extends and damages LMN of anterior horn Muscle wasting and weakness beginning in hand Reduced tendon reflexes Claw hand

Answer 244

Can affect bowel/bladder/sexual organs | Horners

Answer 245

MRI - shows soft tissue causes | CT - shows bony causes

Answer 246

Physio and rehab Taught to avoid damage which may occur in the absence of pain Surgery - Shunt/Laminectomy/Syringotomy

Answer 247

Entire brain is derived of adequate oxygen supply but loss is not total. Normally associated with neonates but can occur in adults (eg post cardiac arrest)

Answer 248

Cyanosis as blood is redirected | Fainting/Coma/Seizures/Brain Death

Answer 249

Diffuse oedema | Reduced cortical grey matter

Answer 250

Non functioning adenoma Prolactinoma GH secreting ACTH secreting

Answer 251

Headache (retro-orbital or bitemporal, worse on waking) Visual Field Defects Facial Pain If extending into hypothalamus - Diabetes Insipidus etc

Answer 252

If initially lower quadrants affected - Craniopharyngoma If upper - Pituitary Adenoma

Answer 253

LH, GH, TSH, ACTH, FSH

Answer 254

SIADH DI Addisons CSF leak

Answer 255

Acromegaly - Octreotide | Prolactinoma - Bromacriptine

Answer 256

Incomplete resection

Answer 257

Trauma Frontal Lobe Tumour Meningitis

Answer 258

DM GCA PCA

Answer 259

Rare -Orbital trauma, Diabetes

Answer 260

MS Pontine CVA Raised ICP

Answer 261

Upper - Stroke | Lower - Bells

Answer 262

Loud Noises Pagets Acoustic Neuroma

Answer 263

Trauma GBS Polio

Answer 264

Trauma | Brainstem pathology

Answer 265

Polio Syringomyelia TB

Answer 266

Levetiracetam (Keppra)

Answer 267

Migraines Todd’s Paresis (post seizure) Bells Palsy

Answer 268

Bilateral thalami stroke Bilateral occipital stroke Limb shaking TIA

Answer 269

Medications Drugs Sleep Flashing Lights

Answer 270

At least 1 form of contraception (ideally 2) Should be started Atleast one month before starting the medication

Answer 271

Contralateral Lower limb > upper limb Disorder of executive function

Answer 272

Spike and wave discharge Hz is how many spikes and waves in a second

Answer 273

Early Maintenance Advanced Palliative

Answer 274

Glutamate - main excitatory transmitter in the central nervous system - NMDA are glutamate receptors GABA - inhibitory in the brain Glycine - inhibitory in the spinal cord