Neuro Flashcards

(186 cards)

1
Q

Brain tumours

A

Can be primary / secondary (10x more common)

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2
Q

Brain tumours patho

A
Primary
Majority are gliomas
astrocytoma (85-90%)
oligodendroglioma (5%)
Also ependymomas, meningiomas, neurofibromas (Schwannomas), craniopharyngiomas
Secondary
Non-small cell lung (most common)
Small cell lung
breast
melanoma
renal cell
GI

Tumours act as space-occupying lesion, increase ICP. Initially, no symptoms, then sharp rise in ICP, symptoms, risk of coning, midline shift

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3
Q

Brain tumours Px

A

3 cardinal symptoms:

Symptoms of raised ICP
headache (increased by coughing, straining), drowsiness, vomiting, papilloedema (optic disc swelling), retinal oedema, haemorrhages

Progressive neurological deficit
Focal - motor (ataxia), sensory, speech, visual, deafness, memory, personality change
Depends on part of brain affected:

Temporal - dysphasia, amnesia
Frontal - hemiparesis, personality change, Broca’s dysphasia
Parietal - hemisensory loss, reduction in 2-point discrimination, dysphasia, astereognosis (cannot recognise object from touch alone)
Occipital - contralateral visual defects
Cerebellum - DANISH - dysdiadochokinesis (impaired rapidly alternating movt), ataxia/gait, nystagmus, intention tremor, slurred speech, hypotonia

Epilepsy
focal seizures more common with tumours

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4
Q

Brain tumour DDx

A

other causes of space-occupying lesion - abscess, haematoma, aneurysm, cyst

stroke

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5
Q

Brain tumour Ix

A

CT/MRI

Bloods - FBC, U+W, LFTs, B12 etc

Biopsy - via skull burr hole

LP CONTRAINDICATED - withdrawing CSF may provoke immediate coning

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6
Q

Brain tumour Mx

A
Surgery to remove mass
Chemotherapy, radiotherapy
Oral dexamethasone
Anticonvulsants - oral carbamazepine
Palliative care
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7
Q

Brain tumour Cx

A

acute haemorrhage into tumour, blockage of CSF outflow - hydrocephalus, coning, Cx of radiotherapy

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8
Q

Cauda equina syndrome

A

compression of cauda equina (~L1), caused by:

disc prolapse, tumour, infection, bleeding, trauma

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9
Q

Cauda equina syndrome Px

A

signs
loss of anal tone on PR
loss of anal wink reflex
loss of sensation around saddle region

symptoms
urinary retention, overflow incontinence
bilateral leg pain
impotence
loss of power in legs, gait disturbance
numbness, paraesthesia
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10
Q

Cauda equina syndrome DDx

A

difference with this and lesions higher up in cord - leg weakness is flaccid and areflexic, not spastic and hyperreflexic

conus medullaris syndrome, mechanical back pain, fracture, tumour, spinal cord compression

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11
Q

Cauda equina syndrome Ix

A

PR examination

lumbar spine MRI

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12
Q

Cauda equina syndrome Mx

A

emergency lumbar decompression

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13
Q

Dementia

A

syndrome caused by a number of brain disorders, which cause memory loss, decline in cognition, difficulties with ADL

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14
Q

Dementia causes

A

Alzheimer’s - degeneration of cerebral cortex, cortical atrophy, neurofibrillary tangles, amyloid plaque formation, reduced ACh production

Vascular - brain damage from cerebrovascular disease - stroke, multi-infarct

Dementia with Lewy bodies - deposition of abnormal proteins, has features of Parkinsonism

Frontotemporal dementia - specific degeneration of frontal and temporal lobes of the brain

Mixed dementia
Parkinson’s disease
Potentially treatable - substance misuse, hypothyroidism

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15
Q

Dementia Px

A

Cognitive impairment - difficulties with memory, language, attention, thinking

Psychiatric/behavioural disturbances - changes in personality, emotional control, social behaviour

Difficulties with ADL - driving, shopping, eating, dressing

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16
Q

Dementia Ix

A
Clinical dx
Screen for cognitive impairment - MMSE
Medication review to exclude drug cause
Identify depression, look for reversible/organic causes
MRI, functional imaging (eg PET)
EEG - in suspected delirium, FTP
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17
Q

Dementia Mx

A

AChE inhibitor - rivastigmine
Anti-glutaminergic tx - memantine (NMDA antagonist)
Challenging behaviour - trazadone/lorazepam
SSRI for depression - citalopram

Non-pharmacological - eg aromatherapy, animal assisted therapy, massage, music

Palliative care

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18
Q

Depression

A

low mood +/- loss of interest and pleasure in most activities

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19
Q

Depression patho

A

associated diseases
dysthymia (chronic depressive state 2+yrs), eating disorders, substance misuse, other psychiatric conditions, various medical conditions

causes
anxiety, alcohol abuse, substance misuse, Parkinson’s, MS, endocrine, medication

RFs
female, past history of depression, significant physical illness, other mental health problems, psychosocial issues

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20
Q

Depression Px

A
Persistent sadness/low mood
Loss of interest in most activities
fatigue, loss of energy
worthless, excessive/inappropriate guilt
recurrent thoughts of death, suicidal thoughts, suicide attempts
diminished ability to think
insomnia
changes in appetite, wt loss
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21
Q

Depression DDx

A

bipolar disorder, schizophrenia, dementia, seasonal affective disorder, bereavement, organic cause (eg hypothyroidism), drugs

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22
Q

Depression Ix

A

Assessment - PHQ-9, HAD scale, Beck’s Depression Inventory

Assess suicide risk

Past psychiatric history

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23
Q

Depression Mx

A

Mental health team - crisis team, IAPT

CBT, counselling

SSRIs - fluoxetine, sertraline, citalopram

TCAs - amitriptyline, amoxapine

Electroconvulsive therapy (ECT)

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24
Q

Depression Cx

A

Impaired QoL, social difficulties, suicide

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25
Encephalitis
infection and inflammation of the brain parenchyma
26
Encephalitis causes
Viral - HSV 1, 2, VSV, CMV, HIV, mumps, measles Bacterial meningitis, TB, malaria RFs - extremes of age, immunocompromised, history of travel / animal bite
27
Encephalitis Px
fever + headache + altered mental status Fever, headaches, myalgia, fatigue, nausea Personality and behavioural changes, decreased consciousness, confusion, drowsiness Hemiparesis, dysphasia, seizures, raised ICP, coning, coma May have signs of meningitis
28
Encephalitis DDx
meningitis, stroke, brain tumour
29
Encephalitis Ix
``` MRI Contrast-enhanced CT EEG LP - CSF elevated lymphocytes, viral detection by PCR Blood and CSF serology ```
30
Encephalitis Mx
Antiviral - acyclovir Antiseizure - primidone, phenytoin If meningitis suspected - benzylpenicillin
31
Epilepsy
recurrent tendency to spontaneous intermittent abnormal electrical activity, manifesting in seizures
32
Epileptic seizure
paroxysmal event caused by excessive hypersynchronous neuronal discharges
33
Convulsions
motor signs of electrical discharges
34
Elements of a seizure
Prodrome - change in mood/behaviour before seizure, might not occur Aura - deja vu, strange gut feeling, strange smells, flashing lights, part of seizure where patient is aware, implies focal seizure, but not necessarily, from temporal lobe Post-ictal - after, headache, confusion, myalgia, sore tongue, dysphasia if seizure in temporal lobe
35
Types of seizures
Focal (partial) seizures - small area, one hemisphere - SIMPLE - without consciousness impairment, focal motor, sensory, autonomic or psychic symptoms, no post-ictal - COMPLEX - with conscious impairment, most commonly from temporal lobe, post-ictal confusion - SECONDARY GENERALISED - bilateral, convulsive seizure, starts locally, spreads widely Generalised seizures - widespread electrical discharge in both hemispheres - ABSENCE SEIZURES - brief pauses (eg stops mid sentence), presents in childhood - TONIC-CLONIC - loss of consciousness, limbs stiffen (tonic), jerk (clonic), post-ictal confusion, drowsiness - MYOCLONIC - sudden jerk of limb, face, trunk - ATONIC (AKINETIC) - sudden loss of muscle tone, fall, no LOC - INFANTILE SPASMS - seizure in baby
36
Epilepsy causes
``` idiopathic cerebrovascular disease trauma cranial surgery neurodegenerative diseases brain neoplasm genetic diseases drugs metabolic medical disorders - electrolytes ```
37
Epilepsy RFs
FHx, premature babies small for age, childhood febrile convulsions, abnormal blood vessels in brain, Alzheimers/dementia, drugs, alcohol withdrawal, stroke, trauma
38
Epilepsy Px
``` PRIMARY GENERALISED Generalised tonic-clonic (grand-mal) no aura LOC tonic - stiff clonic - jerk eyes open, tongue bitten incontinent post-ictal - drowsy, confusion, coma ``` Absence (petit mal) childhood stops activity, stares, turns pale, carries on as normal Myoclonic sudden isolated jerk of limb, face, trunk Tonic seizure sudden sustained increased tone, stiffness, no jerking, characteristic cry/grunt Atonic (akinetic) sudden loss of muscle tone, cessation of movt, fall PARTIAL/FOCAL Simple partial No LOC or memory, focal motor, sensory, autonomic or psychic symptoms, no post-ictal symptoms Complex partial LOC, memory, commonly temporal (understanding speech, memory, emotion), post-ictal confusion Secondary generalisation Starts focally, becomes widespread, typically convulsive
39
Epilepsy Px different lobes
``` Temporal lobe (memory, emotion, speech understanding) aura - deja vu, funny smells anxiety, out-of-body experience, automatisms (lip smacking, chewing, fiddling) ``` ``` Frontal lobe (motor and thought processing) motor features, Jacksonian march (seizure 'marches' up/down motor homonculus starting in face/thumb) Post-ictal Todd's paralysis - paralysis of limbs ``` ``` Parietal lobe (interprets sensations) sensory disturbances - tingling, numbness ``` ``` Occipital lobe (vision) visual phenomena - spots, lines, flashes ```
40
Epilepsy DDx
postural syncope, cardiac arrhythmia, TIA, migraine, hyperventilation, hypoglycaemia, panic attacks, non-epileptic seizure
41
Epilepsy Ix
For clinical Dx: >2 unprovoked seizures >24hrs apart EEG MRI CT head Bloods - rule out metabolic causes, find comorbidities Genetic testing - juvenile myoclonic epilepsy Rule out provoking causes - trauma, stroke, haemorrhage, increased ICP, alcohol/benzo withdrawal, infection, drugs
42
Epilepsy Mx
Emergency ABCDE Check glucose Prolonged/repeated - benzos: diazepam/lorazepam IV phenytoin loading (anti-seizure) If still fitting - anaesthetic and ventilation Medication - all AEDs (anti-epileptic drugs) ``` Primary generalised Tonic clonic (grand mal) - sodium valproate, lamotrigine, carbamazepine ``` Absence (petit mal) - sodium valproate, ethosuximide, lamotrigine Partial/focal carbamazepine, sodium valproate, lamotrigine Neurosurgical tx - resection, vagal nerve stimulation
43
Epilepsy Cx
Status epilepticus - continuous seizures Sudden unexpected death epilepsy (SUDEP)
44
Extradural/epidural haemorrhage
Collection of blood between dura mater and bone
45
EDH patho
traumatic head injury, fracture of temporal/parietal bone, laceration of middle meningeal artery also due to any tear in dural venous sinus
46
EDH Px
Head injury Brief LOC / drowsiness Lucid interval - time between injury and decrease in consciousness, while haematoma is small Severe headache, N+V, confusion, seizures - rising ICP Hemiparesis, brisk reflexes Ipsilateral pupil dilates, coma deepens, bilateral limb weakness, deep/irregular breathing - brainstem compression Decreased GCS, coning Bradycardia and BP increase - late signs
47
EDH DDx
Epilepsy, carotid dissection, CO poisoning, SDH, SAH, meningitis, intoxication
48
EDH Ix
Head CT - hyperdense haematoma, biconcave / lens shaped adjacent to skull (blood forms more rounded shape compared to sickle-shaped SDH as the tough dural attachments to skull keep it localised) Skull xray - may be normal, fracture lines
49
EDH Mx
ABCDE IV mannitol - if increased ICP Neurosurgery - clot evacuation +/- ligation of bleeding vessel
50
Subdural haemorrhage
blood between dura and arachnoid layers can be acute, subacute, chronic Simple / complicated (underlying parenchymal injury)
51
SDH patho
Commonly following rupture of bridging vein between cortex and venous sinus Bleed from damaged cortical artery (branches of ACA, MCA, PCA) Clotting disorders At risk - infants, elderly, alcoholism, on anticoagulants Cerebral atrophy in elderly and alcoholism - tension on veins Trauma, bleeding from bridging veins between cortex and venous sinuses, bleed forms haematoma, reduces pressure and bleeding stops, later when haematoma breaks down, insoluble proteins -> soluble, increase in osmotic pressure, water sucked into haematoma, enlarges, ICP rises
52
SDH Px
Days/weeks/months between injury and symptoms Lucid interval signs increased ICP seizures localising neurological symptoms (unequal pupils, hemiparesis) - occur late Stupor (decreased consciousness), coma, coning symptoms fluctuating level of consciousness slowing, sleepiness, headache, personality change, unsteadiness
53
SDH DDx
stroke, dementia, CNS masses, EDH, SAH
54
SDH Ix
Head CT - sickle-shaped collection of blood, one hemisphere, hyperdense crescent-shaped MRI for subacute, smaller Bloods - FBC, U+E, LFT, thrombocytopenia, coagulation screen
55
SDH Mx
ABCDE Reverse clotting abnormalities Neurosurgery - irrigation/evacuation via burr hole craniotomy IV mannitol Address cause of trauma, eg falls, abuse
56
SDH Cx
coning, raised ICP, seizures, coma
57
Subarachnoid haemorrhage
bleed between subarachnoid and pia
58
SAH causes
Berry aneurysm rupture (80%), results in tissue ischaemia, raised ICP, pressure on brain (bleed acts as space-occupying lesion) Arteriovenous malformations (15%)- vascular development malformation Others - encephalitis, vasculitis, tumour, idiopathic
59
SAH RFs
HTN, known aneurysm, FHx, diseases that predispose to aneurysm (PKD, Ehlers Danlos syndrome, coarctation of aorta), smoking, bleeding disorders, post-menopausal decreased oestrogen
60
SAH Px
signs neck stiffness Kernig's - unable to extend patient's legs at knee when thigh is flexed Brudzinski's - when patient neck flexed by doctor, patient will flex hips and knees retinal, subhyaloid, vitreous bleeds Papilloedema Focal neurology - eg pupil changes, CN3 palsy Later deficits BP increase as reflex to haemorrhage ``` symptoms thunderclap headache vomiting collapse seizures coma/drowsiness ```
61
SAH DDx
meningitis, migraine, intracerebral bleed, cortical vein thrombosis, other causes of stroke
62
SAH Ix
Head CT - star-shaped lesions due to blood filling gyri patterns CT angiography - see aneurysm LP - bloody CSF, then becomes xanthochromic (yellow) due to bilirubin from Hb breakdown ECG - QT prolongation, Q waves, dysrhythmias, ST elevation
63
SAH Mx
IV fluids Nimodipine - CCB, reduces vasospasm Surgery - endovascular coiling / surgical clipping, stents
64
SAH Cx
Rebleeding, cerebral ischaemia, hydrocephalus (tx ventricular/lumbar drain), hyponatraemia
65
Guillain-Barre syndrome
autoimmune condition - acute inflammatory demyelinating ascending polyneuropathy (symmetrical) affecting PNS / Schwann cells
66
GBS patho
usually triggered by infection (eg campylobacter, CMV, VZV, HIV, EBV) - resp/GI infection auto-AB mediated nerve cell damage - damage to Schwann cells, demyelination, reduction in peripheral nerve conduction
67
GBS Px
Symmetrical ascending muscle weakness, paralysis Proximal muscles more affected, then trunk, resp muscles, cranial nerves later Pain Sensory signs may be absent Paraesthesia Reflexes lost Hypotonia Autonomic features - sweating, raised pulse, BP changes, arrhythmias Progressive for 4wks then recovery
68
GBS DDx
Other causes of acute paralysis ``` Brain - stroke, encephalitis Spinal cord - compression, poliomyelitis Peripheral nerve - vasculitis, lead poisoning NMJ - MG, botulism Muscle - hypokalaemia, polymyositis ```
69
GBS Ix
Usually clinical Dx Nerve conduction studies (NCS) - slowing of conduction LP - CSF raised protein, normal WCC Spirometry - monitor FVC, if deceases, go to ITU Maybe AB screen electrolytes, ECG abnormalities
70
GBS Mx
ITU and ventilate if FVC <80% IVIg (IV immunoglobulin) Plasma exchange Pain relief for neuropathic pain
71
Headaches
``` Primary Tension - most common, pain like hand squeezing head Cluster - pain around one eye Sinus - pain behind forehead/cheekbones Migraine - pain, nausea, visual changes ``` Secondary Could be GCA, trigeminal neuralgia, medication overuse ``` Red flags for headache HIV, immunosuppressed Fever Thunderclap headache Seizure Suspected meningitis, encephalitis Red eye? acute glaucoma Headache + new focal neurology, eg papilloedema ```
72
Migraine
Recurrent throbbing headache often preceded by aura, associated with N+V, visual changes May be caused by changes in brainstem and its interactions with trigeminal nerve, or brain chemical imbalance
73
Migraine triggers - CHOCOLATE
``` Chocolate Hangovers Orgasms Cheese Oral contraceptives Lie ins Alcohol Tumult - loud noise Exercise ```
74
Migraine Px
Prodrome - yawning, cravings, mood/sleep changes May have aura ``` Unilateral, pulsing, pain in head Aggravated by routine physical activity N+V, photophobia, phonophobia (sound sensitive) Visual disturbances Paraesthesia ``` ``` POUND Pulsating Onset over 4-72hrs Unilateral Neurological signs, eg photophobia Disabling – patient often wants to be in a dark room ```
75
Migraine Ix
Clinical dx Examine - eyes, BP, head and neck Exclude other causes - CRP, ESR, red flags, maybe LP if indicated
76
Migraine Mx
Reduce triggers - dietary factors NSAIDs, ibuprofen, naproxen Aspirin, paracetamol +/- antiemetic - prochlorperazine Triptans - sumatriptan Prevention BB (propranolol), TCA (amitriptyline), anti-convulsant (topiramate)
77
Tension headache
most common primary headache, no organic cause
78
Tension headache RFs
stress, sleep deprivation, bad posture, hunger, eye strain, anxiety, noise
79
Tension headache Px
Bilateral, pressing/tight, non-pulsatile, like a tight band Pressure behind eyes no vomiting, no sensitivity to head movt, no aura
80
Tension headache Ix
Clinical dx
81
Tension headache Mx
Reassure, exercise, avoid triggers Stress relief - massage, acupuncture Aspirin, paracetamol, ibuprofen
82
Cluster headache
most disabling primary headache, in/around eye
83
Cluster headache Px
``` Abrupt onset Excruciating pain around one eye, temple/forehead Unilateral Eye may be watery, bloodshot, facial flushing, rhinorrhoea (blocked nose), miosis (excessive pupil constriction) +/- ptosis Pain rises to crescendo Often nocturnal +/- vomiting episodic ```
84
Cluster headache Ix
Clinical Dx
85
Cluster headache Mx
O2 Triptan - sumatriptan Prevention - CCB (verapamil), prednisolone may help
86
Chickenpox
caused by varicella-zoster virus
87
Chickenpox patho
transmitted by resp droplets invades resp mucosa, replicates in lymph nodes, infects skin epithelial cells Cx can occur in - immunocompromised, older, steroid use, malignancy, pregnancy (danger to neonates, mother can get pneumonia)
88
Chickenpox Px
Prodrome (1-2 days) - fever, malaise, headaches, abdo pain Rash - pruritic, erythematous macules -> vesicles, crust in 48hrs
89
Chickenpox DDx
generalised herpes zoster or simplex, dermatitis herpetiformis, impetigo, contact dermatitis
90
Chickenpox Ix
Clinical dx PCR Investigate Cx - CXR, LP
91
Chickenpox Mx
Fluids, avoid scratching Paracetamol Self-limiting Acyclovir if at risk of Cx
92
Chickenpox Cx
secondary infections of lesions viral pneumonia encephalitis other CNS complications, other infections
93
Shingles
Herpes zoster - reactivation of VZV
94
Shingles patho
VZV remains latent in sensory ganglia, reactivated, in dorsal root, travels down affected root in dermatomal distribution RFs - elderly, immunocompromised
95
Shingles Px
Pre-eruptive phase burning, itching, paraesthesia in one dermatome malaise, myalgia, headache, fever, maybe lymphadenopathy ``` eruptive phase rash - papules, vesicles in one dermatome neuritic pain paraesthesia does not cross midline ``` chronic phase Post-herpetic neuralgia (PHN) - persistent pain >30d after acute infection
96
Shingles Ix
Clinical dx
97
Shingles Mx
Antivirals - acyclovir Topical AB for tx of secondary bacterial infection Analgesia, ibuprofen PHN Tx - TCA (amitriptyline), anti-epileptic (gabapentin), anticonvulsant (carbamazepine) Vaccine at 70yo to prevent reactivation
98
Shingles Cx
Ophthalmic - can affect sight PHN
99
Huntington's disease
neurodegenerative disorder characterised by lack of the inhibitory NT GABA causes chorea - jerky, semi-purposeful movts, may interfere with voluntary movts
100
HD patho
autosomal dominant, repeated expression of CAG on chromosome 4, mutation in huntingtin gene cerebral atrophy, loss of neurons in caudate nucleus, putamen, decreased ACh and GABA synthesis, decreased inhibition of dopamine release, excessive thalamic stimulation, excessive movts Corpus striatum loss
101
HD Px
Prodromal - mild psychotic and behavioural symptoms Chorea - relentlessly progressive, jerky, explosive, rigid involuntary movts, stop when sleeping Dysarthria, dysphagia, abnormal eye movts Aggression, addictive behaviour, apathy, self-neglect, depression/anxiety Dementia Seizures
102
HD DDx
other causes of chorea - SLE, stroke of basal ganglia
103
HD Ix
Clinical Dx Genetic testing - many CAG repeats CT/MRI - caudate nucleus atrophy, increased frontal horn size of lateral ventricles
104
HD Mx
Counselling, genetic counselling Symptomatic Mx of chorea Benzodiazepines - attach to GABA receptors Sulpiride - neuroleptic, depresses nerve function Tetrabenazine - dopamine depleting agent SSRI for depression Antipsychotic - haloperidol (neuroleptic) Risperidone to tx aggression
105
Meningitis
inflammation of the meninges
106
Meningitis patho
Bacterial group B strep, listeria, E coli, H.influenzae, N.meningitidis, S.pneumoniae, TB, G- (Klebsiella, pseudomonas, enterobacter), staph... N.meningitidis - transmitted by droplet spread, can lead to meningococcal septicaemia (bacteria invades blood, petechial rash, sepsis) Aseptic meningitis - CSF has cells, but no bacterial cultures Viral (most common), fungal, parasites Non-infective meningitis malignant cells, chemical, medication, sarcoidosis, SLE
107
Meningitis Px
Fever, headache, neck stiffness Leg pains, cold hand+feet, abnormal skin colour Photophobia Reduced GCS, coma Seizures, focal CNS signs, petechial rash (stays red when glass pressed) Shock, papilloedema, vomiting Kernig's - pain + resistance on passive knee extension with hip fully flexed Brudzinski's - passive flexion of neck causes patient to raise knees/hips Opisthotonos - spasm of muscles causing backwards arching of head, neck, spine
108
Meningitis DDx
malaria, encephalitis (altered mental state is predominant symptom), septicaemia, dengue, tetanus, SAH
109
Meningitis Ix
Blood culture (before LP and any tx) Bloods - FBC, U+E, CRP, glucose LP - to MC&S - Bacteria - polymorphs, raised protein, low glucose, turbid/yellow colour - TB - lymphocytes, raised protein, low/normal gluc - Virus - lymphocytes, normal/high protein, normal gluc CT head Throat swabs Serum PCR for virus
110
Meningitis Mx
ABs - IV ceftriaxone/cefotaxime, add amoxicillin if >60yo or immunocompromised Chloramphenicol if allergic to beta lactams Dexamethasone for meningism, seizures, ICP Maybe IV vancomycin for return travellers Prophylaxis for contacts - oral ciprofloxacin/oral rifampicin AB for specific org
111
Meningitis Cx
Meningococcal septicaemia - IV benzylpenicillin / IV cefotaxime Septic shock, DIC, coma, cerebral oedema, raised ICP
112
Motor neuron disease
cluster of neurodegenerative diseases - destruction of motor neurons loss of neurons in motor cortex, cranial nerve nuclei, anterior horn cells
113
MND patho
Usually sporadic, possibly oxidative stress from abnormality of mitochondrial function 5% have familial SOD1 mutation Amyotrophic lateral sclerosis (ALS) Most common (<80%) Loss of motor neurons in motor cortex and anterior horn of cord (UMN + LMN) Progressive bulbar palsy (PBP) 10-20% CN9-12 (LMN) Progressive muscular atrophy (PMA) <10% Anterior horn lesion (LMN) Affects distal muscle groups before proximal Primary lateral sclerosis (PLS) Loss of bets cells in motor cortex (UMN) Spastic leg weakness, pseudobulbar palsy, no cognitive decline
114
MND Px
ALS - UMN+LMN Weakness, LMN wasting/fasciculations - usually one limb Cramps Split hand sign - thenar wasting, thumb side of hand seems adrift Wrist and foot drop UMN signs - brisk reflexes, positive Babinski, spasticity ``` PMA - LMN Everything goes down Muscle tone reduced - flaccid Muscle wasting Fasciculation Reflexes depressed/absent Usually starts in one limb, gradually spreads Affects distal muscles before proximal ``` PBP - LMN Dysarthria, dysphagia, nasal regurgitation of fluids, choking LMN lesion of tongue - flaccid, fasciculating, jaw jerk normal/absent, speech quiet, hoarse, nasal PLS - UMN Everything goes up Spasticity Brisk reflexes - tendon and jaw reflexes, positive Babinski Upper limb extensors weaker than flexors, lower limb flexors weaker than extensors, finer movt impaired Velocity dependent spasticity - clasp knife Less prominent muscle wasting FTD in 25% Weakness and atrophy spreads from start to other parts of body
115
MND DDx
MS and polyneuropathies - have sensory loss, sphincter disturbance MG - eye movts affected Diabetic amyotrophy, GBS, spinal cord tumours
116
MND Ix
Clinical findings Brain/cord MRI, LP - exclude structural causes Nerve conduction studies (NCS), electromyography (EMG) - shows denervation of muscles
117
MND Mx
Oral riluzole (inhibits glutamate release, thought to play role in cell death) Drooling - oral propantheline (anti-muscarinic) or oral amitriptyline (dries mouth) Dysphagia - blend food, NG tube Spasm - oral baclofen (CNS depressant, skeletal muscle relaxant) Analgesia - NSAIDs, opioids Palliative MDT care (death from resp failure in 3yrs)
118
Multiple sclerosis
Autoimmune inflammatory disorder of CNS - multiple plaques of demyelination in brain and spinal cord, occurring sporadically over years
119
MS patho
T cell mediated, oligodendrocytes targeted, auto-ABs against myelin created Multiple areas of scar tissue form along neurons - slow/block transmission acute attacks, period of remission where there is remyelination, but is less efficient, temp dependent Types - relapsing/remitting, 2 progressive, 1 progressive RFs - race (caucasian), genetics (HLADR2), FHx, maybe low sunlight and vit D
120
MS Px
Usually monosymptomatic Unilateral optic neuritis - pain on eye movt, decreasing central vision Symptoms may worsen with heat, eg bath, exercise sensory dysaesthesia - unpleasant sensation when touched pins/needles decreased vibration sense trigeminal neuralgia GI - dysphagia, constipation Eye - diplopia, hemianopia, optic neuritis, visual phenomena, pupil defects motor spastic weakness transverse myelitis - weakness/paralysis of both legs Cerebellum - trunk/limb ataxia, intention tremor, scanning speech (monotonous), falls sexual/GU erectile dysfunction anorgasmia, urine retention, incontinence Malaise, N+V, positional vertigo, seizures, aphasia, meningism
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MS Ix
Clinical Dx with McDonald's criteria - 2+ attacks affecting different parts of CNS Exclude differentials - FBC, inflammatory markers, U+E, glucose, HIV serology, auto-ABs, Ca, vit B12 MRI brain, spinal cord LP - oligoclonal IgG bands Electrophysiology - visual evoked potential studies
122
MS Mx
Exercise, stop smoking, reduce stress Dimethyl fumarate (decreases worsening episodes), MABs Methylprednisolone - for relapses Spasticity - baclofen/gabapentin Tremor - botox Urgency/frequency - self-catheterisation/tolterodine Fatigue - amantadine, CBT Stem cell transplant
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Myasthenia gravis
autoimmune disease with ABs against nicotinic ACh receptors in NMJ
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MG patho
If <50yo, commoner in women, associated with other autoimmune disease - pernicious anaemia, SLE, RA, thymic hyperplasia IF >50yo, commoner in men, associated with RA, SLE, thymic atrophy/tumour Transient MG sometimes caused by penicillamine tx for Wilson's Destruction of AChR, immune complex deposition, blocks excitatory effect of ACh on nicotinic receptors - muscle weakenss
125
MG Px
Increasing muscular fatigue Muscle groups affected in order: extra-ocular, bulbar, face, neck, trunk Proximal limb muscles, speech, facial expression commonly affected Ptosis, diplopia, myasthenic snarl Resp difficulties Tendon reflexes normal, may be fatigable Ask to count to 50, voice becomes less audible Hold finger up high, ask patient to look up, after a while will not be able to keep eyes raised Worsened by - pregnancy, hypokalaemia, infection, emotion, drugs
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MG DDx
Other causes of generalised muscle weakness - MS, hyperthyroidism, acute GBS Lambert-Eaton myasthenic syndrome (defective ACh release, weakness tends to improve after exercise)
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MG Ix
Serum anti-AChR, if negative look for anti-MuSK (muscle specific tyrosine kinase) EMG, NCS CT thymus - hyperplasia, atrophy, tumour
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MG Mx
Anti-cholinesterase, so more ACh remains in NMJ - oral pyridostigmine Immunosuppression - prednisolone, azathioprine, methotrexate Thymectomy
129
MG Cx
Myasthenic crisis - weakness of resp muscles during relapse, tx plasmapheresis (AB removal), IV Ig aspiration pneumonia, acute resp failure, ADL restrictions
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Carpal tunnel syndrome
Compression of median nerve as it passes through carpal tunnel in wrist
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CTS patho
entrapment of median nerve - anything that causes swelling/compression of tunnel DM, pregnancy, amyloidosis, obesity, RA, acromegaly, hypothyroidism
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CTS Px
Intermittent symptoms, gradual onset Aching in hand and arm, esp at night, can wake patient up Paraesthesia in thumb, index, middle, 1/2 ring finger and palm Relieved by dangling hand over bed May be sensory loss, weakness of abductor pollicis brevis +/- wasting of thenar eminence Light touch, 2-point discrimination and sweating may be impaired
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CTS DDx
peripheral neuropathy, MND, MS
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CTS Ix
Electroneurography (ENG) Electromyography (EMG) Phalen's test - patient can only maximally flex wrist for 1min and will produce symptoms of carpal tunnel Tinel's test - tapping on nerve at wrist induces tingling
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CTS Mx
Wrist splint at night Local steroid injection Decompression surgery
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Sciatica
L5/S1 root compression
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Sciatica causes
Spinal - disc prolapses, spinal stenosis, spondylolisthesis Piriformis syndrome, pregnancy, trauma, back injury OA
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Sciatica Px
``` signs unilateral weak plantar flexion absent right ankle jerk decreased sensation over lateral edge and sole of right foot ``` symptoms pain in buttock, back of thigh, lateral aspect of little toe (sciatic nerve distribution)
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Sciatica Ix
MRI/CT Examination - straight leg raise test (gently raise leg, if pain then positive)
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Sciatica Mx
Analgesia Surgical decompression
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Olfactory nerve palsy (CN1)
anosmia - can't smell
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optic nerve palsy (CN2)
visual defects depend on location of lesion
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oculomotor nerve palsy (CN3)
ptosis fixed dilated pupil (loss of psym outflow) Eye looks down and out causes - raised ICP, diabetes, HTN, GCA, berry aneurysm
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trochlear nerve palsy (CN4)
Innervates superior oblique Palsy results in head tilt to correct extortion that results in diplopia on looking down Causes - trauma to orbit (rare)
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abducens nerve palsy (CN6)
innervates LR, eye will be adducted, looking in Causes - MS, Wenicke's encephalopathy, pontine stroke (presents with small fixed pupils +/- quadriparesis)
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CN3,4,6 palsy
non-functioning eye Causes - stroke, tumours, Wernicke's encephalopathy
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Trigeminal nerve palsy (CN5)
jaw deviates to side of lesion loss of corneal blink reflex Causes - trigeminal neuralgia, herpes zoster, nasopharyngeal cancer
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Facial nerve palsy (CN7)
facial droop and weakness Forehead sparing if lesion is central, before facial nerve nucleus Causes - Bell's palsy, fractures of petrous bones, middle ear infections, inflammation of parotid gland (facial nerve passes through) Tx with steroids
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vestibulocochlear nerve palsy (CN8)
hearing impairment, vertigo, lack of balance Causes - surrounding tumour, skull fracture, toxic drug effects, ear infections
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glossopharyngeal (CN9) and vagus (CN10) palsy
gag reflex issues, swallowing issues, vocal issues causes - jugular foramen lesion
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accessory nerve lesion (CN11)
SCM and trapezius | can't shrug shoulder or shake head
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hypoglossal nerve palsy (CN12)
tongue deviates towards side of lesion
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Spinal cord tract
Corticospinal motor descending UMN decussates at medulla DCML ascending sensory tract fine touch, proprioception, vibration, 2-point discrimination decussates at medulla Spinothalamic ascending sensory pain, temp, crude touch decussates almost immediately in spinal cord
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Brown-Sequard Syndrome
Hemisection of spinal cord ``` Causes Space-occupying lesions Intervertebral disc prolapses Vertebral bone fractures Trauma - GSW, knife Infectious - HIV MS ``` Px Level of lesion: - ipsilateral spinothalamic dysfunction - loss of temp, pain, pressure sense Below lesion: - ipsilateral corticospinal dysfunction - loss of motor function (weakness) - ipsilateral DCML dysfunction - loss of fine touch, proprioception, vibration, 2-point discrimination - contralateral spinothalamic dysfunction - loss of temp, pain, pressure Overall - ipsilateral loss of fine touch, proprioception, motor, contralateral loss of pain, temp, crude touch Ix MRI spine Mx physical and occu therapy Steroids - reduce swelling inflammation
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Paraplegia
paralysis of both legs - always spinal cord lesion
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Hemiplegia
paralysis of one side of body - brain lesion
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UMN signs
signs contralateral to lesion - indicates lesion is above anterior horn cell - in spinal cord, brainstem, motor cortex Increased muscle tone - spastic paralysis, velocity dependent, non-uniform, clasp-knife Weakness - flexors weaker than extensors in legs, extensors weaker than flexors in arms Hyperreflexia - reflexes increased, are brisk
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LMN signs
Signs ipsilateral to lesion decreased muscle tone, flaccid paralysis (nerve to muscle cut, will go floppy for life...) wasting/atrophy, fasciculations reflexes reduced/absent
159
Parkinson's disease
degenerative movt disorder caused by reduction in dopamine in substantia nigra
160
PD patho
associated with dementia and depression combination of environ, genetics, oxidative stress, mito dysfunction progressive loss of dopaminergic neurons from pars compacta of SN in midbrain - less dopamine, thalamus inhibited - decrease of movt neural inclusions present RFs- FHx, environmental factors
161
PD Px
gradual, asymmetrical onset of symptoms, commonly present with impaired dexterity anosmia, depression, anxiety, aches and pains, REM sleep disorders, urinary urgency, hypotension and constipation Tremor worse at rest, asymmetrical issue with repetitive hand movts, worsening in rhythm the longer attempted Rigidity increased tone in limbs and trunk limb resists passive movt throughout (as opposed to UMN lesion spasticity - clasp knife) Bradykinesia slow to initiate movt reduced blink rate, monotonous hypophonic speech, writing smaller, difficulty with fine movt, narrow gait, reduced asymmetrical arm swing, problems with balance, stooped posture, shuffling steps expressionless face
162
PD DDx
benign essential tremor, multiple cerebral infarcts, drug induced, Wilson's disease, trauma, HD
163
PD Ix
Clinical dx, can confirm by levodopa response CT/MRI head
164
PD Mx
Oral levodopa, with deoxycarbolase inhibitor (co-careldopa / co-beneldopa) which prevents peripheral conversion of l-dopa to dopamine (cannot cross BBB) Dopamine agonists - oral ropinirole / oral pramipexole Mono oxidase B (MAO-B) inhibitors - oral selegiline / oral rasagiline Catechol-O-methyltransferase (COMT) inhibitors - oral entacapone / oral tolcapone COMT and MAO-B break down dopamine Surgical - ablation of overactive basal ganglia circuits, eg subthalamic nuclei
165
PD Cx
Depression, dementia, psychosis SSRI - oral citalopram Anti-psychotic - oral quetiapine
166
Radiculopathy
pinched nerve - compression of nerve root and LMN
167
Mononeuropathy
disease affecting single nerve - peripheral/cranial nerve causes usually local - eg trauma, entrapment, tumour Common - CTS, ulnar neuropathy, peroneal neuropathy, cranial neuropathies
168
Mononeuritis multiplex
2+ peripheral nerves affected (separate areas) ``` when causes tend to be systemic - WARDS PLC Wegener's granulomatosis AIDs/amyloid RA DM Sarcoidosis PAN Leprosy Carcinoma ```
169
Polyneuropathy
disease of many nerves diffuse, symmetrical, usually starts peripherally Can be sensory/motor/autonomic/mixed Mostly motor - GBS, lead poisoning, Charcot-Marie-Tooth syndrome Mostly sensory - DM, renal failure, leprosy ``` Px SENSORY numbness, paraesthesia glove+stocking loss of dexterity maybe signs of trauma (not noticed by patient) ``` MOTOR progressive weak/clumsy hands difficulty in walking, breathing, weakness, wasting
170
6 mechanisms that can cause nerve malfunction
Demyelination Axonal degeneration - causes nerve to die back from periphery Compression Infarction Infiltration - by inflammatory cells Wallerian degeneration - nerve fibre cut, distal part of axon separated from cell body dies
171
Peripheral neuropathies Px
symptoms sensory - loss of pain, warmth/cold, proprioception, balance, pressure, vibration (depends on fibre affected) motor - muscle cramps, weakness, fasciculations, atrophy, ataxia, high arched feet from excessive atrophy signs - symmetrical sensorimotor - commonest neuropathy, starts distally, initially sensory, then sensorimotor - asymmetrical sensory - patchy, dorsal root ganglia affected, uncommon - paraneoplastic, Sjogrens, coeliac disease - asymmetrical sensorimotor - mononeuritis multiplex, very uncommon - vasculitis
172
Peripheral neuropathy Ix
H+E | Neurophysiological examination - nerve conduction studies/quantitative sensory test
173
Peripheral neuropathy Mx
Anti-neuralgic for pain - gabapentin, amitriptyline, pregabalin Cramps - quinine
174
Spinal cord compression
compression can occur in any part of spinal cord Causes spondylosis (degeneration of spine), disc prolapse, spondylolisthesis, tumour, stenosis, abscess, TB, trauma, infection RFs osteoporosis, trauma, tumour, RA, ankylosing spondylitis Px At level of lesion - back pain, nerve root pain, numbness, paraesthesia, LMN signs Below lesion - UMN signs Sphincter disturbance, loss of bladder and bowel control Ix MRI Mx Surgical - decompressive laminectomy Dexamethasone/chemo/radio if malignancy
175
Stroke
disruption of blood supply to the brain, leading to neurological deficit from focal infarction
176
Stroke patho
``` Ischaemic/infarction (80%) loss of blood flow, lack of oxygen - thrombosis in situ - emboli from atheroma (carotid bifurcation commonly) - heart emboli - AF, valve disease, IE, fat emboli after long bone fracture - large artery stenosis - watershed stroke - drop in BP - venous sinus thrombosis (1%) ``` Haemorrhage (17%) Bleeding in CNS - trauma, aneurysm rupture, thrombolysis, anticoagulation, carotid artery dissection, SAH, HTN -> microaneurysm rupture RFs HTN, smoking, DM, heart disease, PAD, post-TIA, polycythaemia, combined OCP, hyperlipidaemia, exess alcohol
177
Stroke Px
Worse at onset Distinguish between the two (not reliable) Haemorrhagic - meningism, severe headache, coma Ischaemic - carotid bruit, AF, past TIA, IHD FAST ``` ACA Leg weakness > arm weakness leg sensory disturbances gait apraxia truncal ataxia incontinence drowsiness akinetic mutism - stuporous state ``` MCA contralateral arm and leg weakness, sensory loss hemianopia aphasia (cannot understand/produce speech) dysphasia (deficiency in speech generation) facial droop PCA contralateral homonymous hemianopia cortical blindness (brain tissue causes blindness, eye is healthy) visual agnosia (cannot interpret visual info) prosopagnosia - cannot see faces ``` Posterior circulation brainstem, cerebellum locked in syndrome - aware, cannot respond motor deficits - hemiparesis, tetraparesis, facial paralysis dysarthria, speech impairment vertigo, N+V visual disturbance altered consciousness ``` ``` Lacunar stroke 25% basal ganglia, internal capsule, thalamus, pons unilateral weakness +/- sensory deficit ataxic hemiparesis cerebellar and motor symptoms ```
178
Stroke DDx
head injury, hypo/hyperglycaemia, SDH, hepatic encephalopathy
179
Stroke Ix
Head CT BP, ECG - look for AF Bloods - FBC (polycythaemia), glucose, ESR
180
Stroke Mx
ABCDE Aspirin (once haemorrhagic excluded) Thrombolytic drugs - alteplase Thrombectomy - surgical removal Haemorrhagic Surgery Mannitol for raised ICP Control HTN ``` Prevention Aspirin, clopidogrel Simvastatin AF tx HTN tx ```
181
Transient ischaemic attack
temporary inadequacy of circulation to part of brain <24hrs, most <30 mins
182
TIA causes
atherothromboembolism cardioembolism - post-MI, valve disease, AF hyperviscosity - polycythaemia, sickle cell, myeloma Vasculitis Hypoperfusion - cardiac dysrhythmia, postural hypotension
183
TIA RFs
HTN, smoking, DM, heart disease, PAD, polycythaemia, hyperlipidaemia
184
TIA Px
sudden loss of function, lasts minutes FAST Anterior circulation weak, numb contralateral leg, maybe similar arm symptoms hemiparesis hemisensory disturbance dysphagia amaurosis fugax - temporary loss of vision, eg emboli in retinal, ophthalmic, ciliary blood flow ``` Posterior circulation diplopia vertigo vomiting choking, dysarthria ataxia hemisensory loss hemianopia LOC transient global amnesia tetraparesis (muscle weakness at all 4 extremities) ```
185
TIA Ix
Bloods - FBC (polycythaemia), ESR (vasculitis), glucose, creatinine, electrolytes, cholesterol Carotid artery doppler USS - look for atheroma/stenosis MRI/CT angiography ECG - AF or MI ischaemia Calculate ABCD2 score - risk of stroke after TIA
186
TIA Mx
Control CV risk factors - ACEi/ARB, simvastatin, DM, stop smoking Aspirin, clopidogrel If CV source of emboli - heparin, warfarin Carotid endarterectomy - correct stenosis