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PACES MRCP > Neurology > Flashcards

Neurology Flashcards

1
Q

Retinal vein occlusion

Features
Causes

A

Sudden, painless visual loss

Flame haemorrhages radiating from swollen disc
Cotton wool spots
engorged Tortuous veins
= FCT

*Look for changes relating to cause: diabetic or hypertensive retinopathy

Causes: hypertension, hyperglycaemia (DM), hyperviscosity (myeloma), high intraocular pressure (glaucoma)

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2
Q

Retinal artery occlusion

Features
Causes
Treatment

A

Sudden, painless visual loss. Preceded by amaurosis fugax.

Pale, milky with thread like arteries and cherry red macula.

*Look for causes: AF, carotid bruit

Causes: embolic (carotid plaque rupture, cardiac mural thrombus w/ AF), GCA

Tx: embolism (aspirin, anticoag, endardarectomy) ,GCA (high dose steroids)

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3
Q

Sudden painless visual loss

Causes

A

Retinal vein occlusion (HTN, DM, myeloma, glaucoma)
Retinal artery occlusion (AF, GCA)
Retinal detachment (proliferative diabetic retinopathy, trauma)
Vitreous haemorrhage (proliferative diabetic retinopathy, vitreous/retinal detachment, trauma)

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4
Q

Sudden painful visual loss

Causes

A

Acute glaucoma
Endophthalmitis
Uveitis
Trauma

Binocular: migraine, vertebrobasilar insufficiency, stroke, PRES, optic neuritis

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5
Q

Retinitis pigmentosa

Features
Cause
Associated conditions

A 
White stick
Tunnel vision (reduced peripheral vision) starting with reduced night vision

Fundoscopy: bone spicule pigmentation with optic atrophy

Congenital: AR
Acquired: post inflammatory retinitis

Associated:
Freidrichs ataxia
Refsums disease (ataxia, deafness, icthyosis)
Kearns-Sayre syndrome (ataxic, deaness, ophthalmoplegia/ptosis, PPM)
Users disease (deafness)

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6
Q

Tunnel vision

Causes

A 
Retinitis pigmentosa
Papilloedema
Glaucoma
Choroidoretinitis
Migraine
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7
Q

Age related macular degeneration

Features
Risk factors
Treatment

A

Wet (neovascular, exudative)
Dry (atrophic)

Fundoscopy: drusen, atrophy, fibrosis, neovascularisation (wet)

Risk: age, white, fhx, smoking, CHD/CVE

Tx: ophthalm, wet AMD = intravitreal injections of anti-VEGF

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8
Q

Optic atrophy

Features
Causes

A

RAPD, optic disc pallor

PALE DISCS
Pressure (tumour, glaucoma)
Ataxia (friedreichs ataxia)
Lebers hereditary optic atrophy

Dietary (B12)
Degenerative (retinitis pigmentosa)
Ischaemia (central retinal artery occlusion)
Syphilis (and other infection i.e.CMV, toxo)
Cyanide (and other toxins, etoh, lead, tobacco)
Sclerosis (MS)

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9
Q

Horner’s syndrome

Features

A

Ptosis, miosis, anhydrosis

Damage to sympathetic tract:

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10
Q

Horners syndrome

Causes

A

First order/central:
Hypothalamus - stroke, tumour
Brainstem - stroke, tumour, MS
Spinal cord - tumour, MS, syringomyelia, trauma

Second order/pre ganglionic:
Apical lung - pancoasts tumour, cervical rib
Subclavian artery aneurysm
Thyroid tumour

Third order/post ganglionic:
Carotid artery - dissection, aneurysm
Skull base - tumour, trauma
Cavernous sinus - thrombosis, tumour

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11
Q

Holmes adie pupil

A

Tonic pupil
Dilated with sluggish constriction to accommodation, poor response to light
Absent reflexes
Benign

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12
Q

Argyll Robertson pupil

A

Small irregular pupil
Accommodates but no reaction to light

*look for tabes dorsalis/sensory ataxia as associated with syphilis

Causes: quaternary syphilis or diabetes
Tx: penicillin

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13
Q

Oculomotor nerve palsy

Features
Causes

A

Full ptosis
Down and out pupil

If dilated pupil = surgical (papillary fibres and superficial and easily impinged by surgical causes)
If normal pupil = medical

Surgical: communicating artery aneurysm, cavernous sinus pathology (tumour, thrombus), cerebral herniation

Medical: mononeuritis multiplex (DM), midbrain infarction, midbrain demyelination, migraine

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14
Q

Neurofibromatosis

Features
Inheritance
Associations
Complications

A

Features: ABCDEF
Axillary freckling / Acuity (optic glioma)
Blood pressure (hypertension - RAS and phaeo)
Cutaneous neurofibromas (>2) / Cafe au lait (>6)
lisch noDules (iris hamartomas)
Enlarged palpable nerves with neuropathy
Fine crackles (fibrosis)

Inheritance: AD
Type 1 = ch 17, classical
Type 2 = ch 22, CENTRAL, BL acoustic neuroma, sensorineural deafness, no skin lesions

Association - phaeo and RAS (HTN)

Complications: epilepsy, sarcomas, scoliosis, low IQ

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15
Q

Enlarged palpable nerves and neuropathy

Causes

A 
Neurofibromatosis 
Leprosy
Amyloidosis
Acromegaly
Refsums disease (RP, ataxia, deafness, anosmia, dry scaley skin, short digits)
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16
Q

Tuberous sclerosis

Features
Inheritance
Associations
Investigations

A

Features:
Skin: butterfly adenoma sebaceum, periungual fibromas, shagreen patch (lumbar, leathery) and ash leaf macule (trunk, depigmented)
Resp: cystic lung disease
Abdo: renal enlargement (PKD/angiomyolipomata), renal transplant, evidence of dialysis
Eyes: phakomas on retina (dense white)
CNS: cognitive defects, seizures (very common)

Inheritance: AD

Associations: ADPKD (genes near each other on ch 16), renal angiomyolipomas, RCC, cysts. therefore renal failure.

Ix: railroad track calcification on skull films, CT/MRI head = tuberous calcified masses in cortex, echo and abdo USS = hamartomas and renal cysts.

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17
Q

Myasthenia gravis

Features
Cause
Associations
Investigations
Treatment
A 
Bilateral fatigable ptosis
Ophthalmoplegia
Nasal speech, poor swallow
Fatigable proximal upper limb weakness
Normal reflexes
Thymectomy scar (sternotomy)
Check FVC

Cause: anti acetylcholine receptor antibodies reduce transmission

Associations: autoimmune diseases (DM, RA, thyrotoxicosis), thymomas

Ix: anti acetylcholine R antibody (90%), anti-MuSK if anti-AChR negative, EMG (decremented response to train of implses), tensilon test (edrophonium ACh-esterase inhibitor improves weakness but causes HB and asystole!!), CT/MRI thymus, TFTs (Graves)

Tx: avoid precipitating drugs (beta blockers, antibiotics i.e. fluoroquinolones, penicillin, steroids)
Acute = IVIG or plasmapheresis
Chronic = pyridostigmine (ACH-esterase inhibitor), immuosuppression (steroids, aza), thymectomy

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18
Q

Lambert Eaton myasthenic syndrome

Cause
Features
Association

A

Diminished reflexes -better after exercise
Lower limb proximal weakness

Cause: antibodies blocking pre synaptic calcium channels

Associations: SC lung Ca

EMG: second wind phenomenom on repetitive stimulation

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19
Q

Bilateral ptosis

A 
Congenital
Myasthenia gravis
Myotonic dystrophy
BL 3rd nerve/horners
Mitochondrial disorders i.e. Kearns-Sayres
20
Q

Unilateral ptosis

A

3rd nerve palsy

Horners syndrome

21
Q

Bilateral ophthalmoplegia

A

Myasthenia gravis
Graves disease
Mitochondrial disorders i.e. Kearns-Sayres

22
Q

Facial nerve palsy

Features
Cause

A

Unilateral facial droop, can’t raise eyebrows or tightly close eyes

Causes:
Bells palsy
Varicella Zoster/Ramsay hunt (vesicular rash in ear)
Mononeuropathy (DM, sarcoid, lyme)
Tumour (parotid, acoustic neuroma)
Trauma
MS
CVE
23
Q

Bells palsy

Features
Treatment

A

Facial nerve palsy. Rapid onset. HSV-1 implicated.

Tx: prednisolone within 72 hrs of onset, eye protection

70-80% full recovery

24
Q

Bilateral facial palsy

Causes

A 
GBS
Sarcoidosis
Lyme disease
Myasthenia gravis
Bilateral bells palsy
25
Q

Friedrichs ataxia

Features
Inheritance
Associations

A 
Features:
Young, wheelchair
Ataxia, dysarthria, nystagmus
LMN: leg wasting, weakness, absent reflexes
UMN: upgoing plantars
PC loss: vibration and proprioception
MSK: pes cavus, kyphoscoliosis
Eye and ears: optic atrophy, sensorineural deafness
Cardiac: HOCM
Diabetes

Inheritance: AR, 20yr from diagnosis

Association: HOCM, dementia

26
Q

Extensor plantars with absent reflexes

A

Freidriechs ataxia
Sabacute combined degeneration of spinal cord (B12)
MND
Taboparesis
Combined lesions i.e. cervical radiculopathy and peripheral neuropathy

27
Q

Peripheral neuropathy

Causes

A

Sensory: metabolic type
DM, alcohol, drugs, vitamin deficiency (b12, thiamine)

Motor: rare types
GBS, lead toxicity, porphyria, HSMN (charcot marie tooth)

28
Q

Mononeuritis multiplex

Causes

A 
DM
CTD (SLE, RA)
Vasculitis (PAN, Churg Strauss)
Infection (HIV)
Malignancy (paraneoplastic)
29
Q

HSMN/Charcot Marie Tooth

Features
Types

A

Neuropathy - predominantly motor, with MSK effects

Distal wasting of lower limbs, preserved thigh muscle bulk (inverted champagne bottles)
Weakness distally
Socking sensory loss
Gait: high stepping (foot drop) and stamping (loss of proprioception)
Hand muscle wasting
Palpable nerves
MSK: pes cavus

Types: I (demyelinating) and II (axonal) most common
AD inheritance

Ix: NCS/EMG (demyelinating vs axonal)

Tx: PT/OT, podiatry, genetic counselling

30
Q

Parkinson’s disease

Features
Associated signs
Causes
Pathology
Treatment
A

Expressionless face
Tremor: coarse, pill rolling, asymmetrical
Bradykinesia: oppose digits quickly
Cogwheel rigidity: synkinesis enhances it (simultaneous movements of other hand tapping on leg)
Gait: shuffling, reduced arm swing
Speech: slow, quiet

Associated signs:
BP (MSA - postural drop, cerebellar and pyramidal signs)
Eye movements (PSP - reduced up/down eye movements)
Dementia (DLB)
Drug history

Causes:
Idiopathic Parkinsons disease
Parkinsons plus: MSA, PSP, CBD, DLB
Drug induced Parkinsonism (phenothiazines)

Pathology: degeneration of dopaminergic neurons in substantia nigra

Treatment:
L dopa with dopa decarboxylase inhibitor (madopar) - nausea, dyskinesia, wear off, on/off motor fluctuation
Dopamine agonist (pergolide) - less side effects, apomorphine is SC injection/infusion
MAO-B inhibitor (selegiline)
Anti cholinergic (reduce tremor)
COMT inhibitor (entacapone)
Amantadine (increase DA release)
Surgery: DBS
31
Q

Tremor

Causes

A

Resting: Parkinsons (check eye movements, bradykinesia, cogwheel rigidity, BP)
Intention: cerebellar (check eye movements, past pointing, dysdiadokokinesia, ataxia, hypotonia, dysarthria)
Postural: benign essential tremor (better with alcohol), anxiety, thyrotoxicosis (thyroid examination), metabolic (CO2/asterixis)

32
Q

MND

Features
History
Types
Investigation
Treatment
Prognosis
A

Wasting, fasciculation
Increased tone
Weakness
Absent knee with extensor plantar
NORMAL SENSATION
Speech: bulbar (nasal) or pseudobulbar (hot potato due to spastic tongue)
Tongue: wasting and fasciculation (if bulbar), spastic (if pseudobulbar)

History: asymmetrical, rapid, aggressive, behavioural/cognitive dysfunction (FTD = MND).
MFMN = distal weakness
Family history - Kenendys

Types
Axonal degeneration of upper an lower motor neurons
AML: 1/2, CS tracts, spastic paraparesis
PMA: 1/4, anterior horn cells, wasting/fasciculation/weakness. Best prognosis.
PBP: 1/4, lower CN and suprabulbar nuclei (speech and swallow problems). Worst prognosis.
Kennedys = tongue fasciculations
SMA: LMN

** differentials: multifocal motor neuropathy with conduction block.

Ix: EMG (fasciculation, fibrillations, in MFMN = demyelinating, conduction block), MRI brain and spine (exclude cord compression, myelopathy, brainstem lesions)

Tx: supportive (PEG, NIV), MDT
Riluzole (glutamate antagonist) - 3 months benefit, doesn’t improve function or QoL
Screen for FTD.

Prognosis: 3 yrs from diagnosis due to pneumonia and resp failure

33
Q

Wasting of hand muscles

Causes

A

Anterior horn cell: MND, syringomyelia, cervical cord compression, polio

Brachial plexus: trauma, pancoast’s tumour, cervical rib

Peripheral nerve: median and ulnar nerve lesions, peripheral neuropathy

Muscle: disuse atrophy i.e. RA

34
Q

Syringomyelia

Features
Cause
Associations

A

UL: Weakness/wasting small hand muscles
Areflexia upper limbs
Dissociated sensory loss (pain and temp lost) with scars
Charcot joint

LL: UMN weakness with upgoing plantars

Kyphoscoliosis, Horner’s, if syringobulbia -> cerebellar/lower CN

Cause: progressively expanding fluid filled cavity in cervical spinal cord spanning several levels

Associations: arnold-chiari, spina bifida

Ix: spinal MRI

35
Q

Charcot joint

Causes
Treatment

A

Painless deformity and destruction of joint with new bone formation after repeated minor trauma secondary to loss of pain sensation (spinothalamic)

Cause: tabes dorsalis (hip/knee), diabetes (foot/ankle), syringomyelia (elbow/shoulder)

Tx: bisphosphonates

36
Q

Biceps/supinator jerk

Triceps jerk

A

C5/6

C7/8

37
Q

Spastic paraparesis i.e. myelopathy

Features
Cause

A 
Wheelchair/stick, ?scars (trauma), urinary catheter (?Spinal)
Increased tone with clonus 
Weakness
Hypereflexia and extensor planters
Scissoring gait
?sensory level (spinal)
?signs of MS (cerebellar, optic atrophy)

Broadly: trauma, ischaemia, inflammation, neoplastic, infection, metabolic.

Commonly: MS, spinal cord compression, cervical myelopathy, trauma, MND (normal sensation)

Other: anterior Spinal artery thrombosis (dissociated sensory, DC preserved), syringomyelia (LMN signs in upper limb, UMN in lower limb, shawl sensory loss), HSP (fhx, stiff>weak), subacute combined degeneration of cord (absent reflexes, upcoming plantars), Friedrich ataxia (cerebellar, DC loss, UMN, MSK, eye, heart)

38
Q

Cord compression

Features
Cause
Investigation
Treatment

A

Sensory level
Spastic paraparesis
Upgoing planters and clonus
Weakness

Causes: disc prolapse, trauma, infection (abscess, tb), malignancy

Ix: mri spine

Tx: surgical decompression, steroids/radio if malignancy

39
Q

Wallenberg/lateral medullary syndrome

Features
Cause

A

IPSILATERAL: cerebellar signs, vertigo, horners, palatal paralysis, trigeminal pain and temp loss.

CONTRALATERAL: loss of pain and temp

Cause: occlusion of PICA

40
Q

Stroke

Features
Look for causes
Definition CVE/TIA
Investigation
Management
Bamford classification
A

Flexed upper limb/extended lower limbs
Increased tone and clonus
Weakness
May have reduced coordination 2 to weakness or cerebellar involvement (PC)
Brisk reflexes and extensor plantars
Gait - tip toesing
Look for - facial weakness, swallow, visual fields/neglect, higher cortical

Look for causes: AF, carotid bruits, murmur, HTN

Definition:
CVE; rapid onset focal neurological deficit due to vascular lesion >24 hrs
TIA; rapid onset focal neurological deficit <24 hrs

Ix: bloods (inc CRP/ESR if young), glucose, renal function, ECG (AF), CXR (cardiomeg, aspiration), CTH (infarct, bleed)
Consider: echo, carotid dopplers, MRI/MRA/MRV (dissection, venous sinus thrombosis), clotting, vasculitis screen if young

Mx:
Acute: thrombolysis <4.5 hrs and no CI in ischaemic
Aspirin 300 2/52 then clopi, PPI, MDT on st unit, IPCS

Chronic: secondary prevention (statin, ACEI), carotid endartarectomy (70-99% occlusion), anticoag if AF

Bamford:
TACS: hemiplegia, homonomous hemianopia, high cortical dysfunction (swallow, neglect)
PACS: 2/3
Lacunar: pure hemisensoy/motor

41
Q

Visual field defects

Defect and location of lesion

A

Unilateral - ipsilateral optic nerve
Bitemporal hemianopia - optic chiasm
Homonymous hemianopia - contralateral optic tract
Upper homonymous hemianopia - contralateral temporal optic radiation
Lower homonymous hemianopia - contralateral parietal optic radiation

Macula spared in posterior cerebral artery stroke
Otherwise middle cerebral artery

42
Q

Multiple sclerosis

Features
Diagnostic criteria
Investigations
Treatment
Prognosis
Pregnancy
A

Wheelchair
INO (can be bilateral - cant adduct, other eye nystagmus), optic atrophy, reduced acuity, any other CN pathology
UMN with altered sensation - spastic paraparesis
Cerebellar signs
Autonomic features - bladder and bowel
Uthoffs - heat worsens
Lhermittes - lightening pain on neck flexion

Diagnostic: CNS demyelination causing neurological impairment disseminated in space and time. Unknown cause.

Ix: CSF (oligoconal bands), MRI (periventricular white matter plaques), VEPs (delayed velocity, normal amplitude - previous optic neuritis)

Tx: MDT
DMARD: IFN b, glatiramer reduce relapse rate. Monoclonal ab reduce progression (natalizumab).
Symptoms: high dose steroids i.e. methylpred shortens relapse, antispasmodics, carbamazepine (neuro pain), manage bowel and bladder

Prognosis: variable, majority ambulant 10 yrs

Pregnancy: reduced relapse rate but increased in post partum

43
Q

Cerebellar syndrome

Features
Cause

A

Dysarthria, rebound phenomenon, incoordination, hypotonia and hyporeflexia, nystagmus, broad based gait
Ipsilateral limbs signs

Causes:
Alcohol and drugs (PT)
CVE
SOL or paraneoplastic
Inflammation: MS
Endocrine: hypothyroidism
Rare: Freidreichs, ataxic telangiectasia
44
Q

Myotonic dystrophy

Features
Inheritance
Investigations
Management

A

Face: myopathic, frontal balding, temporalis wasting, BL ptosis, cataracts, dysarthria
Hands: myotonia (delayed grip release, tapping on thenar eminence), wasting and weakness, areflexia
Other: cardiomyopathy, heart block, PPM, diabetes, testicular atrophy

Inheritance: AD.
DM 1 = DMPK gene ch 19, DM 2 = ZNF9 gene ch 3
Anticipation in DM1

Ix: EEG (dive bomber), genetics

Mx: resp and cardiac complications, PPM, PT for myotonia, avoid GA

45
Q

Ulnar nerve lesion

Features
Causes

A 
Wasting of hypothenar eminence and small muscles of hand
?scars
Claw of 4th/5th fingers
Normal tone
Weakness finger abduction
Altered sensation -ulnar border

**Mimic - C8-T1 radiculopathy, check for other muscles of hand (abductor pollicis brevis) and sensation T1.

Causes:
Trauma - at elbow
Surgical procedure
Malignancy - paraneoplastic
Mononeuritis multiplex (would likely get areflexia if inflammatory)

Ix: neuro-physiological testing, NCS to localise lesion, EMG for prognosis in terms of recovery

Treatment
Neuropathic pain - tricyclic antidepressant (amitriptyline), gabapentin/pregabalin, duloextine, capsacin

46
Q

Main peripheral nerves of upper limb

A

Ulnar - finger abduction
Median - abductor pollicis brevis
Radial - wrist extension
Posterior interossei nerve - finger extension

PACES MRCP (6 decks)

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