Neurology Flashcards
Retinal vein occlusion
Features
Causes
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)
Retinal artery occlusion
Features
Causes
Treatment
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)
Sudden painless visual loss
Causes
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)
Sudden painful visual loss
Causes
Acute glaucoma
Endophthalmitis
Uveitis
Trauma
Binocular: migraine, vertebrobasilar insufficiency, stroke, PRES, optic neuritis
Retinitis pigmentosa
Features
Cause
Associated conditions
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)
Tunnel vision
Causes
Retinitis pigmentosa Papilloedema Glaucoma Choroidoretinitis Migraine
Age related macular degeneration
Features
Risk factors
Treatment
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
Optic atrophy
Features
Causes
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)
Horner’s syndrome
Features
Ptosis, miosis, anhydrosis
Damage to sympathetic tract:
Horners syndrome
Causes
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
Holmes adie pupil
Tonic pupil
Dilated with sluggish constriction to accommodation, poor response to light
Absent reflexes
Benign
Argyll Robertson pupil
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
Oculomotor nerve palsy
Features
Causes
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
Neurofibromatosis
Features
Inheritance
Associations
Complications
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
Enlarged palpable nerves and neuropathy
Causes
Neurofibromatosis Leprosy Amyloidosis Acromegaly Refsums disease (RP, ataxia, deafness, anosmia, dry scaley skin, short digits)
Tuberous sclerosis
Features
Inheritance
Associations
Investigations
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.
Myasthenia gravis
Features Cause Associations Investigations Treatment
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
Lambert Eaton myasthenic syndrome
Cause
Features
Association
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
Bilateral ptosis
Congenital Myasthenia gravis Myotonic dystrophy BL 3rd nerve/horners Mitochondrial disorders i.e. Kearns-Sayres
Unilateral ptosis
3rd nerve palsy
Horners syndrome
Bilateral ophthalmoplegia
Myasthenia gravis
Graves disease
Mitochondrial disorders i.e. Kearns-Sayres
Facial nerve palsy
Features
Cause
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
Bells palsy
Features
Treatment
Facial nerve palsy. Rapid onset. HSV-1 implicated.
Tx: prednisolone within 72 hrs of onset, eye protection
70-80% full recovery
Bilateral facial palsy
Causes
GBS Sarcoidosis Lyme disease Myasthenia gravis Bilateral bells palsy
Friedrichs ataxia
Features
Inheritance
Associations
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
Extensor plantars with absent reflexes
Freidriechs ataxia
Sabacute combined degeneration of spinal cord (B12)
MND
Taboparesis
Combined lesions i.e. cervical radiculopathy and peripheral neuropathy
Peripheral neuropathy
Causes
Sensory: metabolic type
DM, alcohol, drugs, vitamin deficiency (b12, thiamine)
Motor: rare types
GBS, lead toxicity, porphyria, HSMN (charcot marie tooth)
Mononeuritis multiplex
Causes
DM CTD (SLE, RA) Vasculitis (PAN, Churg Strauss) Infection (HIV) Malignancy (paraneoplastic)
HSMN/Charcot Marie Tooth
Features
Types
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
Parkinson’s disease
Features Associated signs Causes Pathology Treatment
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
Tremor
Causes
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)
MND
Features History Types Investigation Treatment Prognosis
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
Wasting of hand muscles
Causes
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
Syringomyelia
Features
Cause
Associations
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
Charcot joint
Causes
Treatment
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
Biceps/supinator jerk
Triceps jerk
C5/6
C7/8
Spastic paraparesis i.e. myelopathy
Features
Cause
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)
Cord compression
Features
Cause
Investigation
Treatment
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
Wallenberg/lateral medullary syndrome
Features
Cause
IPSILATERAL: cerebellar signs, vertigo, horners, palatal paralysis, trigeminal pain and temp loss.
CONTRALATERAL: loss of pain and temp
Cause: occlusion of PICA
Stroke
Features Look for causes Definition CVE/TIA Investigation Management Bamford classification
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
Visual field defects
Defect and location of lesion
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
Multiple sclerosis
Features Diagnostic criteria Investigations Treatment Prognosis Pregnancy
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
Cerebellar syndrome
Features
Cause
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
Myotonic dystrophy
Features
Inheritance
Investigations
Management
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
Ulnar nerve lesion
Features
Causes
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
Main peripheral nerves of upper limb
Ulnar - finger abduction
Median - abductor pollicis brevis
Radial - wrist extension
Posterior interossei nerve - finger extension
