Neurology conditions Flashcards
(162 cards)
1
Q
Seizure
A
uncontrolled discharge of neurons within the CNS
2
Q
Prodrome
A
mood/behavioural changes that occur before a seizure
3
Q
Aura
A
symptom immediately before the seizure, helps to localise
4
Q
Postictal period
A
period of time immediately after the seizure
5
Q
Partial seizure
A
- partial - one specific location of the brain, often lobe
- frontal lobe = jacksonian march → involuntary movement of one muscle group to the neck (up the arm)
- parietal lobe = sensory disturbance in extremities or in the face
- temporal lobe = visceral disturbance, memory disturbance, motor disturbance & affective disturbance
- occipital lobe = visual hallucination before the seizure
6
Q
Generalised seizure
A
- generalised = affect both hemispheres
- absence = pt stares vacantly
- myoclonic = sudden brief generalised muscle contractions
- tonic = sudden, sustained muscular contraction
- atonic = loss of muscle tone & sudden fall
- tonic-clonic = combination of tonic, followed by a clonic phase
- partial seizure can develop into tonic clonic seizure
7
Q
Epilepsy syndromes
A
West syndrome (4-6 months) - salaam attacks, hypsarrhythmias (EEG)
Lennox-Gaustat (1-3 years) - drop attacks, LD
Childhood absence epilepsy - staring blankly, no recollection
Juvenile myoclonic syndrome - throwing drinks or food in the morning, characteristic EEG
Sturge Weber syndrome - port wine stain in region of trigeminal nerve
8
Q
Epilepsy ix
A
Neuroimaging
EEG
ECG
Additional = blood tests
9
Q
Epilepsy mx
A
Generalised seizures = 1st line is sodium valproate OR
- TC, tonic, atonic = lamotrigine
- absence = ethosuximide
- myoclonic = levetiracetam
Focal seizures = 1st line is lamotrigine OR carbamazepine
- 2nd line = levetiracetam OR oxcarbazepine OR sodium valproate
Driving advice
- 1st seizure = 6 months off if no relevant structural abnormalities, 1 year if this not met
- epilepsy & medication = 1 year free of attacks
- epilepsy without medication = 6 months free of attacks
10
Q
Migraine
A
Recurrent moderate to severe headache commonly associated with nausea, vomiting, photophobia & phonophobia
11
Q
Migraine clinical features
A
Headache - unilateral, pulsating, moderate/severe pain intensity, N&V, photophobia, phonophobia
Aura - scintillating scotoma, numbness, tingling, dysphasia
12
Q
Migraine acute mx
A
Simple analgesia
Triptans - oral sumatriptan
Anti-emetics - buccal prochlorperazine
13
Q
Migraine prevention
A
Trigger avoidance
Preventative treatment - propranolol, topiramate, amitriptyline
14
Q
Migraine complications
A
Status migrainosus - debilitating migraine that persists > 72 hours
Persistent aura without infarction - symptoms of aura for > 1 week
Migrainous infarction
Migraine aura-triggered seizure
Ischaemic stroke
15
Q
IIH
A
Disorder characterised by chronically elevated intracranial pressure
16
Q
IIH risk factors
A
Overweight & obese
Females
Reproductive age
17
Q
IIH clinical features
A
Clinical features of raised ICP
Headache - worse on lying down or bending over
Transient visual loss
Photopsia
Tinnitus (pulsatile)
Diplopia
Visual loss
Other features - neck, back and/or retrobulbar pain
Signs - papilloedema, visual loss, 6th nerve palsy
18
Q
IIH ix
A
Basic - obs, urinalysis, bloods, ophthalmoscopy
Neuroimaging - MRI, CT
LP - >25cm H20 consistent with IIH
19
Q
IIH mx
A
Weight loss
Serial LP
Pharmacotherapy - carbonic anhydrase inhibitor (acetazolamide)
- other meds: topiramate, furosemide
Surgical treatment - optic nerve sheath fenestration OR shunting
20
Q
Cluster headache
A
Severe primary headache disorder characterised by recurrent unilateral headaches centred on the eye/temporal region
21
Q
Cluster headache clinical features
A
Very severe unilateral orbital/supraorbital and/or temporal pain
Conjunctival infection and/or lacrimation
Nasal congestion
Eyelid oedema
Forehead and facial sweating
Miosis and/or ptosis
Sense of restlessness or agitation
22
Q
Cluster headache ix
A
Neurology referral
MRI brain
CT head
23
Q
Cluster headache mx
A
Trigger avoidance
Acute management
- triptans - subcut/intranasal route
- short burst oxygen therapy
Preventative management
- verapamil
- other options - glucocorticoids, lithium
Refractory disease
- greater occipital nerve blocks
- deep brain stimulation
- trigeminal nerve compression
24
Q
Trigeminal neuralgia
A
Chronic pain condition characterised by severe, sudden & brief bouts of shooting/stabbing pain that follow distribution of one/more divisions of the trigeminal nerve
25
Trigeminal neuralgia aetiology
Idiopathic
Malignancy
AV malformation
MS
Sarcoidosis
Lyme disease
26
Trigeminal neuralgia clinical features
Unilateral facial pain that is sudden, severe & brief
- shooting and stabbing
Triggers = touch, eating, wind blowing, cold
Examination is normal
27
Trigeminal neuralgia ix
Clinical diagnosis
MRI - exclude secondary causes
28
Trigeminal neuralgia mx
Medical - carbamazepine
- other options: phenytoin, lamotrigine, gabapentin
Surgical - microvascular decompression, treatment of underlying cause, alcohol/glycerol injections
29
Medication overuse headache
Present for 15 days or more per month
Developed or worsened whilst taking regular symptomatic medication - down-regulation of pain receptors
Patients using opioids & triptans are at most risk
30
Medication overuse headache mx
Withdraw offending drugs
Consider restarting previously used drugs at a lower dose or different drugs not previously used
31
Temporal arteritis (also called GCA)
Condition where the arteries, particularly the temples, become inflamed
32
Temporal arteritis clinical features
Temporal headache - GCA can cause blindness & stroke
Jaw claudication
Amaurosis fugax (transient monocular blindness, often described as a dark curtain descending vertically)
Systemic features - fatigue, fevers, weight loss & malaise
GCA & PR often occur together
O/E - thickened, tender temporal artery, scalp tenderness
33
Temporal arteritis ix
Bloods - inflammatory markers, FBC & LFTs
Temporal artery biopsy - granulomatous inflammation of the inner half of the media with infiltration of inflammatory cells
Doppler ultrasonography - ‘halo sign’
34
Temporal arteritis mx
High-dose steroids immediately
Gradual taper steroids over 1-2 years
Steroid protection - bisphosphonates & PPIs
Low dose aspirin - further reduce the risk of stroke & blindness
35
Tension headaches clinical features
Bilateral, non-pulsatile headaches
Tightness sensation (like band around the head)
Scalp muscle tenderness
Associated with stress, depression, alcohol, skipping meals, dehydration
36
Tension headaches mx
Reassurance
Analgesia per the WHO pain ladder
Amitriptyline is generally first-line chronic/frequent tension headaches
37
Idiopathic Parkinson’s Disease and Parkinsonism
Chronic, progressive neurodegenerative condition resulting from the loss of dopamine-containing cells of the substantia nigra
Parkinsonism = umbrella term for the clinical syndrome involving bradykinesia & at least one of tremor, rigidity and/or postural instability
38
PD pathophysiology
Loss of dopaminergic neurons in the substantia nigra & development of Lewy bodies (a-synuclein)
LRRK2 mutations most common type in familial & sporadic PD
39
PD clinical features
Triad - rigidity, bradykinesia & tremor
Shuffling gait - impaired balance on turning, flexed posture giving stooped appearance, reduced arm swing, short stride length
Hypophonia, micrographia, pseudohypersalivation, hypomimia
Non-motor symptoms: anosmia, low mood, anxiety, constipation, REM sleep disorder, fatigue
O/E:
- tremor - worse at rest, pill-rolling, re-emergent tremor with posturing, 4-6 Hz
- bradykinesia - reduced/slow movement & difficult initiation of movement, reduced arm swing, reduction in amplitude with repetitive movements
- rigidity - increase resistance to passive movement, cogwheel due to superimposed tremor
40
PD clinical rating scales
Heohn and Yahr scale
UPDRS
41
PD ix
Clinical diagnosis
Neuroimaging (CT/MRI) - strong suspicion of a secondary cause/failed response to treatment
DaTSCAN (type of SPECT imaging) - differentiate Parkinsonism from essential tremor
42
PD mx
Treatments mainly work by increasing dopamine levels
Initial management:
- Levodopa - most improvement in motor symptoms
- more likely to get dyskinesia later down the line
- Dopamine agonists
- more specified adverse events (excessive sleepiness, hallucinations & impulse control disorders)
- MAO-B inhibitors - less effective than first two meds
Adjuvant treatment:
- COMT inhibitors - inhibit the peripheral breakdown by the COMT enzyme → more levodopa to cross the blood brain barrier
- anti-muscarinics, amantadine, apomorphine
Surgical management:
- deep brain stimulation
- specialist centres → thalamic/subthalamic surgery
MDT input - OT, physios, dieticians
Drugs to avoid:
- flupentixol
- haloperidol
- chlorpromazine
- prochlorperazine
- metaclopramide
43
PD complications
Motor:
- motor fluctuations
- dyskinesia
- freezing of gait
- ‘wearing off’ phenomenon
- falls
Non-motor complications:
- aspiration pneumonia
- bladder, bowel and sexual dysfunction
- pressure sores
- impulse control disorders & psychosis
44
Parkinsonism differentials
Vascular
Drug-induced - anti-psychotics
Benign essential tremor - responds to alcohol, 1st line treatment is propranolol
Dementia with Lewy bodies - presence of parkinsonism & dementia within 1 year
Parkinson plus conditions - progressive supranuclear palsy, multi-system atrophy, dementia with lewy-body, corticobasal degeneration
45
GBS
An acute, inflammatory polyneuropathy typically characterised by a progressive, ascending neuropathy
Refers to a number of ‘variants’ with unique features & pathogenesis → AIDP
46
GBS triggers
Campylobacter jejuni - common cause of food poisoning & gastroenteritis
Other infections - CMV, EBV, hepatitis E & mycoplasma pneumoniae
47
Acute inflammatory demyelinating polyneuropathy (AIDP)
Classical symptoms:
- progressive symmetrical weakness in limbs
- reduced or absent tendon reflexes
- reduced sensation
Symptoms tend to ascend from distal muscles of the limbs
Commonly history of preceding viral illness
48
Acute motor axonal neuropathy (AMAN)
Form of GBS that results from axonal involvement typically following infection with campylobacter
Progresses more rapidly than AIDP & does not demonstrate sensory nerve involvement
49
Acute motor and sensory axonal neuropathy (AMSAN)
Severe form of AMAN that also demonstrates involvement of sensory nerves
Characterised by axonal degeneration of both sensory & peripheral nerves
50
Miller Fisher syndrome
Variant characterised by unique presentation:
- ataxia
- areflexia
- ophthalmoplegia
25% will also develop some weakness in the extremities
51
GBS ix
Bedside - vital signs, blood sugar, pregnancy test (if indicated), stool culture (if indicated), urine porphobilinogen
Bloods - FBC, renal function, LFTs, CRP, bone profile & Mg, HbA1c, thyroid profile, B12/folate/thiamine
Imaging - CXR, MRI spine
Nerve conduction studies, electromyography, LP, antibodies (anti-GQ1b), spirometry
52
GBS mx
Supportive care
- monitor for respiratory failure
- 4 hourly FVC is an important component
- cardiovascular monitoring
- DVT prophylaxis
- analgesia
Immunotherapy - speed up recovery
- IV immunoglobulin
- plasma exchange - removes circulating antibodies & other immune modulators
53
GBS clinical course
Symptoms develop rapidly over two weeks before plateauing, with recovery around week four
Factors associated with poorer prognosis - old age, preceding campy infection, rapid onset & severe presenting symptoms
54
MND
Clinical syndrome characterised by prominent and progressive muscular weakness
55
MND risk factors
Increasing age
Male
Genetic predisposition
56
MND phenotype
Amyotrophic lateral sclerosis (ALS) - affects both upper and lower motor neurones
Progressive bulbar palsy - primarily affecting bulbar muscles (speech & swallowing)
Primary lateral sclerosis (PLS) - predominately affects UMNs
Progressive muscular atrophy (PMA) - predominantly affects LMNs
57
MND pathophysiology
Progressive degenerative disorder of both upper and lower motor neurones
Sensory neurones are spared
58
MND genetics
TDP-43 → pathological feature
C9orf72 gene - most common genetic abnormality → both loss and gain of function mutations
59
MND UMN vs LMN signs
UMN - hypertonia, pyramidal pattern of weakness, hyperreflexia, positive jaw jerk, slow tongue movements, disuse atrophy, Hoffman’s and extensor plantar responses
LMN - hypotonia, areflexia, wasting, fasciculations
60
MND clinical presentation
Presence of both upper & lower motor neuron signs with an insidious onset
Limb onset
Bulbar phenotype → progressive dysarthria & dysphagia, tongue fasciculations
Triceps & finger flexors are comparatively spared
Wasting of thenar eminence & 1st dorsal interosseous
Hip flexion & ankle dorsiflexion often affected
Cognitive impairment
Overlap with FTD
61
MND diagnosis
Clinical diagnosis
Electromyography - active denervation with compensatory reinnervation
62
MND ix
MRI brain, spine
LP
Bloods
Resp function → can’t ventilate properly
ECG - riluzole can cause tachycardia
63
MND mx
Riluzole (glutamate inhibitor) - can prolong life by approx. 3 months
Supportive/palliative treatment:
- NIV
- gastrotomy
- manage other symptoms eg. spasticity, cramps, head drop
64
MG
Autoimmune disease marked by production of antibodies that target the nicotinic acetylcholine receptors on muscle fibres
65
MG clinical features
Limb muscle weakness
Extra-ocular muscle involvement - drooping eyelids, diplopia
Facial muscle involvement - difficulty in smiling or chewing
Bulbar muscle involvement - change in speech or difficulty swallowing
O/E - fatigable muscle weakness, bilateral ptosis, myasthenic snarl, head droop & bulbar features
Symptoms typically worsen with prolonged movement/by end of the day
66
MG ix
Ice-pack test
Bloods - serum acetylcholine receptor antibody & muscle-specific tyrosine kinase antibodies
Imaging investigations - CT scan of the chest to identify thymic hyperplasia/thymoma
Nerve conduction studies/EMG
Myasthenic crisis → serial pulmonary function tests
67
MG mx
R/w in neurology, MDT
Medical management - steroids, anticholinesterase inhibitors (pyridostigmine)
Acute cases - IVIG or plasmapheresis
Surgical management with thymectomy → in patients with thymic hyperplasia/thymoma
68
Spinal muscular atrophy
Inherited disease that affects LMN
Autosomal recessive defect in SMN1 gene
Hypotonia, areflexia, poor feeding, not meeting milestones, respiratory failure
69
MS
Relapsing-remitting MS (most common) - unpredictable attacks which may or may not leave permanent deficits followed by periods of remission
Neuroinflammatory disorder characterised by demyelination of the central nervous system
69
MS classification
Mcdonald’s criteria (2017) - classification of MS
- dissemination in space
- dissemination in time
70
MS risk factors
F>M
Age (<50)
Genetics
Geographical distribution
Infections
71
MS aetiology
Multiple genes
EBV
Low vitamin D
Smoking
Obesity
72
MS presentation
Unilateral optic neuritis - demyelination of the optic nerve & presents with unilateral reduced vision, developing over hours to days
- central scotoma (enlarged central blind spot)
- pain with eye movement
- impaired colour vision
- relative afferent pupillary defect - pupil in the affected eye constricts more when shining a light in the contralateral eye than when shining in the affected eye
Painless binocular diplopia
Focal brainstem/cerebellar syndrome
Partial transverse myelitis with sensory and/or motor symptoms
Examination - focus on eye examination (check vision, light perception, extraocular movements)
73
MS ix
Obs
Bloods
MRI brain (gold standard for diagnosis)
LP - CSF will show oligoclonal bands
74
MS mx
Acute treatments - steroids (5 days of IV methylprednisolone) → makes attack shorter, improves recovery, minimises risk of permanent symptoms
Chronic treatments - disease modifying therapies
MDT approach
75
Polyneuropathy
Refers to damage or dysfunction of multiple nerves of the peripheral nervous system
Typically lead to symmetrical involvement of distal peripheral nerves in a ‘length-dependent’ manner → meaning the longer nerves of the legs & arms are affected first
76
Polyneuropathy classification (onset)
Acute - rapid progression from onset to worst clinical features within 4 weeks
- eg. GBS, vasculitis, certain toxins or critical illness
Chronic
- eg. diabetes mellitus, CKD, hereditary neuropathies, alcohol excess
77
Polyneuropathy classification (pathology)
Demyelination - refers to damage to the myelin sheath that insulates nerves & improves conduction
- eg. autoimmune diseases, hereditary causes or myeloma
Axonal degeneration - refers to damage to the nerve axon that extends from the cell body & transmits electrical conduction
- eg. systemic diseases → diabetes mellitus, connective tissue disease, vitamin B12 deficiency
78
Polyneuropathy classification (clinical presentation)
Motor - refers to damage/dysfunction of motor nerves
- weakness & atrophy of affected muscles
- eg. chronic demyelinating inflammatory polyneuropathy, GBS & Charcot-Marie-Tooth
Sensory - damage or dysfunction of sensory nerves
- may be ‘negative’ referring to loss of normal sensation eg. touch, proprioception or vibration
- may be ‘positive’ referring to dysregulated function eg. pain, paraesthesia or burning
- diabetes mellitus, alcohol excess, CKD, paraneoplastic syndromes
79
Polyneuropathy classification (small and large fibre)
Large fibres - carry information to control movement & important sensory information
- touch, vibration & proprioception
Small fibres - carry information about pain & temperature → painful polyneuropathies
80
Polyneuropathy aetiology
Idiopathic
Diabetes mellitus
Systemic illness - CKD, chronic liver disease, amyloidosis
Autoimmune - GBS
Inflammatory - CIDP
Toxic - alcohol, chemo, heavy metals
Neoplastic - myeloma, paraneoplastic syndrome
Hereditary - Charcot-Marie-Tooth
Nutritional - vitamin B12, folate, pyridoxine, vit E deficiencies
Vasculitis
Medications
81
Polyneuropathy - diabetes mellitus
Most common cause of polyneuropathy
Can cause a wide range of peripheral neuropathies:
- distal, symmetrical polyneuropathy
- autonomic neuropathy
- radiculopathies
- mononeuropathies
- mononeuritis multiplex
Nerve damage occurs due to oxidative stress as part of a byproduct of different metabolic pathways that occur due to persistent hyperglycaemia
82
Polyneuropathy - charcot-marie-tooth
Refers to a collection of peripheral neuropathies due to an inherited mutation
Slowly progressive lower limb neurological changes
- distal calf atrophy
- pes cavus
- clumsy walking
- weakness
- reduced sensation
Family history of lower limb abnormalities
Examples: CMT1A, CMT2A
83
Polyneuropathy pathophysiology
Demyelination - degeneration of the myelin sheath is seen
Axonal degeneration - damage to nerve axons, typically a symmetrical polyneuropathy with weakness
84
Polyneuropathy clinical features
Symmetrical, ‘length-dependent’ neuropathy in a ‘glove and stocking’ distribution
Paraesthesia, sensory loss and/or weakness
Sensory features - burning sensation, paraesthesia, sensory loss, ataxia, loss of light touch, loss of vibration, loss of proprioception
Motor features - weakness, reduced/absent reflexes, hypotonia, fasciculations, muscle atrophy, muscle cramping, deformity
85
Polyneuropathy ix
Bloods test - FBC, U&Es, LFTs, vitamin B12, folate, vit E, myeloma screen
Electrodiagnostic tests:
- electromyography - refers to an assessment of the electrical activity of muscles
- nerve conduction studies - refers to an assessment of individual peripheral nerve function
- can determine the type of injury
86
Polyneuropathy mx
Treat the underlying cause - avoidance of precipitating drug/toxin, targeted treatments
Manage the symptoms - neuromodulating drugs such as gabapentin/duloxetine
87
Cervical spondylosis
Degeneration of the vertebral column in the cervical region
88
Cervical spondylosis risk factors
Ageing
Occupation which involves repetitive neck movement/overhead work (eg. painting/decorating)
Previous traumatic neck injury
FHx
89
Cervical spondylosis clinical features
Pain and/or stiffness in cervical region
Referred pain: retro-orbital, temporal, occipital, interscapular, upper limbs
O/E:
- reduced range of movement of neck (all directions)
- poorly localised tenderness
- radiculopathy (most common - C5-C7): unilateral neck, shoulder/arm pain, paraesthesia/hyperaesthesia, diminished arm reflexes
90
Cervical spondylosis ix
Clinical diagnosis
Cervical XR - osteophyte formation, narrowed disc spaces, narrowing of intervertebral foramina
MRI
91
Cervical spondylosis mx
Lifestyle - postural advice, maintain normal activities, avoid use of neck collar
Pharmacological - analgesia, specialist pain clinic, epidural injection
Physiotherapy - symptoms > 4 weeks
OT/psychologist
Surgical - decompressive surgical procedures
92
Cervical spondylosis complications
Neurological - radiculopathy & myelopathy
Surgical complications - reduced ROM, accelerated progression of degeneration of adjacent levels
93
Bell’s palsy
Unilateral facial nerve palsy of unknown cause
Thought to be related to inflammation and oedema of the facial nerve secondary to a viral infection/autoimmunity
94
Bell’s palsy clinical features
Rapid onset (< 72 hours) of unilateral facial weakness
Post-auricular/ear pain
Difficulty chewing
Incomplete eye closure
Drooling
Tingling
Hyperacusis (weakness of the stapedius muscle)
Signs - loss of nasolabial fold, drooping of the eyebrow, drooping of the corner of the mouth, asymmetrical smile, Bell’s sign (upward movement of the eye maintained on attempt to close the eye)
95
Bell’s palsy ix
Clinical diagnosis - unilateral facial weakness of rapid onset without forehead sparing
Routine blood tests
Neuroimaging
Specialist tests - LP, lyme serology
96
Bell’s palsy mx
Largely supportive but prednisolone may be used in those presenting within 72 hours of onset
General points - patients should be reassured that the prognosis is good, but can take several months to recover
Medical therapy - in patients presenting within 72 hours, prednisolone may be given; anti-viral treatment may be considered
Eye care - critical to provide good eye care which includes drops, advice on taping the eye when sleeping, sunglasses outdoors
- incomplete eye closure = referred to ophthalmology
97
Bell’s palsy complications
Majority make a full recovery within four months
Eye symptoms - corneal ulcer
No improvement after three weeks/develop late problems with aberrant reinnervation
- voluntary muscle contraction leading to involuntary contraction of another muscle
98
Carpal tunnel syndrome
Median nerve neuropathy due to compression as it passes through the carpal tunnel in the wrist
Most common upper limb mononeuropathy
99
Carpal tunnel syndrome risk factors
Females
Diabetes
Osteoarthritis
Obesity
Hypothyroidism
Pregnancy
Trauma
100
Carpal tunnel syndrome clinical features
Symptoms - pain, paraesthesia, weakness, clumsiness, difficulty with tasks, sensory loss
Signs - sensory loss, thenar eminence wasting, weak thumb abduction & opposition
Positive Phalen’s & Tinel’s test
101
Carpal tunnel syndrome ix
Usually clinical
Electromyography
Nerve conduction studies
Imaging reserved for patients with a suspected structural abnormality
102
Carpal tunnel syndrome mx
Non-surgical - wrist splints, physical therapy and/or corticosteroids
Surgical - surgical decompression: open carpal tunnel release, endoscopic carpal tunnel release, US guided minimally-invasive release
- complications
- wound infection
- scar formation
- vascular injury
- nerve injury
- complex region pain syndrome
103
Ulnar nerve palsy aetiology
Direct trauma/injury to the nerve
Compression/entrapment - cubital tunnel syndrome/Guyon’s canal syndrome
Iatrogenic
Systemic diseases that affect peripheral nerves - DM
104
Ulnar nerve palsy clinical features
Weakness or paralysis of most of the intrinsic hand muscles & two muscles in the forearm
Numbness, tingling or pain in sensory distribution of the ulnar nerve (skin of the medial 1.5 digits & associated palm area
105
Ulnar nerve palsy ix
Neurological examination
EMG/NCS
MRI/CT scan
106
Ulnar nerve palsy mx
Conservative measures - rest, physical therapy, splints
Analgesia
Surgical interventions
107
Radiculopathies
Conduction block in the axons of a spinal nerve or its roots, with impact on motor axons causing weakness & on sensory axons causing paraesthesia
108
Radiculopathies aetiology
Anything that causes nerve compression
Intervertebral disc collapse
Degenerative diseases of the spine
Fracture
Malignancy
Infection
109
Radiculopathies clinical features
Sensory features - paraesthesia & numbness
Motor features - weakness
Radicular pain is often present → burning, deep, strap-like/narrow pain
O/E - important to identify dermatomal & myotomal involvement, ensure to examine for CES
110
Radiculopathies mx
Definitive long-term management depends on underlying cause
- Symptomatic management
Analgesia - neuropathic pain medications are commonly utilised
Amitriptyline, with pregabalin/gabapentin as alternatives
Physiotherapy
111
Essential tremor
Most common type of action tremor that typically involves the hands & is bought out by anti-gravity
Associated with family history & shows an autosomal dominant pattern of inheritance
112
Essential tremor clinical features
Characterised by a bilateral kinetic and/or postural tremor of the hands and arms
- when outstretched or required to perform a specific task
Can affect head & voice
Improves with a small amount of alcohol
Absence of other neurological signs
113
Essential tremor diagnosis
Typically based on the following four features:
1) isolated upper limb action tremor
2) with/without tremor in other sites
3) duration > 3 years
4) no other neurological features
114
Essential tremor mx
Removing any potential precipitant that makes the tremor worse
- Pharmacological treatment
Propranolol
Primidone - anti-epileptic drug
- Additional therapies
Neuromodulation
Botulinum toxin injections
Deep brain stimulation
115
Horner’s Syndrome
Classic triad of miosis, partial ptosis and anhidrosis
Due to a lesion anywhere along the sympathetic pathway
116
Horner’s Syndrome oculosympathetic pathway
Composed of three neurons: first, second & third order
First order neuron (central neuron)
- fibres travel from the hypothalamus to the spinal cord (C8-T2)
Second order neuron
- fibres exit the spinal cord via the dorsal roots & enter the sympathetic chain
- travel upwards over the apex on the lung to the superior cervical ganglion
Third order neuron
- fibres pass within the adventitia of the internal carotid artery
- join the ophthalmic portion of the trigeminal nerve at the cavernous sinus & subsequently pass to the pupillary dilator via ciliary nerves
- post-ganglionic sympathetic fibres innervate a small muscle → ptosis
117
Horner’s Syndrome aetiology
First order - lesion anywhere between the brainstem and cervicothoracic spinal cord
- eg. stroke, SOL, MS, syringomyelia, cervical cord trauma
Second order
- eg. pancoast tumour, thoracic outlet lesion, trauma, thoracic aneurysm
Third order
- eg. carotid artery dissection, cavernous sinus pathology, neck mass
118
Horner’s Syndrome mx
Neurological emergency = acute, painful Horner’s
Directed towards the underlying cause
Eg. hyperacute stroke unit, thrombolysis, mechanical therapies
119
Subacute combined degeneration of the spinal cord
Rare neurological disorder that affects the dorsal columns, lateral corticospinal tracts & spinocerebellar tracts of spinal cord due to vitamin B12 deficiency
120
Subacute combined degeneration of the spinal cord aetiology
Vitamin B12 deficiency
- malabsorption
- inadequate dietary intake
- other underlying medical conditions
121
Subacute combined degeneration of the spinal cord clinical features
Dorsal column involvement
- distal tingling/burning/sensory loss is symmetrical & tends to affect the legs more than the arms
- impaired proprioception & vibration sense
Lateral corticospinal tract involvement
- muscle weakness, hyperreflexia & spasticity
- UMN signs typically develop in the legs first
- brisk knee reflexes
- absent ankle jerks
- extensor plantars
Spinocerebellar tract involvement
- sensory ataxia → gait abnormalities
- positive Romberg’s sign
122
Subacute combined degeneration of the spinal cord ix
Assessment of B12 and folate levels
Homocysteine level analysis - increased level despite normal B12 = functional B12 deficiency
MRI spine
Nerve conduction studies
123
Subacute combined degeneration of the spinal cord mx
Vitamin B12 replacement
- hydroxocobalamin 1mg IM on alternate days until no further improvement
- maintenance treatment with hydroxocobalamin 1mg IM every two months
124
Cavernous sinus syndromes
Constellations of clinical signs and symptoms referable to pathology within or adjacent to the cavernous sinus
125
Cavernous sinus syndromes clinical features
Multiple unilateral cranial neuropathies
- ophthalmoplegia (CN III, IV, VI) - diplopia
- facial sensory loss (CN V1 and V2)
- Horner syndrome
Pain
Chemosis
Proptosis
126
Cavernous sinus syndromes aetiology
Neoplastic - meningioma, neurofibroma
Inflammatory - sarcoidosis
Infection
Vascular - cavernous malformation, cavernous sinus thrombosis
Traumatic
127
Pituitary tumours classification
Adenomas can be classified according to:
- size (< 1 cm = microadenoma)
- hormonal status
Prolactinomas are most common types
128
Pituitary tumours clinical features
Excess of hormone - Cushing’s disease, acromegaly, galactorrhoea
Depletion of a hormone
Headaches - stretching of dura
Bitemporal hemianopia - compression of optic chiasm
Incidental finding of neuroimaging
129
Pituitary tumours ix
Pituitary blood profile - GH, prolactin, ACTH, FSH, LSH, TFTs)
Formal visual field testing
MRI brain with contrast
130
Pituitary tumours mx
Medical management:
- prolactinomas: dopamine agonists (cabergoline or bromocriptine)
- GH-secreting adenomas: somatostatin analogues & GH receptor antagonists
- ACTH-secreting adenomas: cortisol synthesis inhibitors
Transsphenoidal surgery
Radiotherapy
131
Wernicke’s encephalopathy
Neurological disorder caused by thiamine (vitamin B1) deficiency
132
Wernicke’s encephalopathy aetiology
Chronic alcohol abuse
Malnutrition
Bariatric surgery
Hyperemesis gravidarum
133
Wernicke’s encephalopathy clinical features
Confusion
Oculomotor abnormalities - gaze-evoked nystagmus, spontaneous upbeat nystagmus & horizontal/vertical ophthalmoplegia
Ataxia (difficulties with coordinated movements)
134
Wernicke’s encephalopathy ix
Neurological examination
MRI head
Blood tests - can reveal low thiamine levels
135
Wernicke’s encephalopathy mx
High dose IV thiamine (Pabrinex)
136
Wernicke’s encephalopathy complications
Permanent horizontal nystagmus
Inability to walk
Deficits in learning & memory
Korsakoff syndrome - irreversible deficits in anterograde and retrograde memory
137
Argyll Robertson pupil
Specific finding in neurosyphilis
Constricted pupil that accommodates when focusing on a near object but does not react to light
Often irregularly shaped
138
Creutzfeldt-Jakob Disease (CJD)
Neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein - prion
139
CJD classification
Sporadic CJD
New variant CJD - younger patients, psychological symptoms are most common presenting features
Other prion diseases - kuru, fatal familial insomnia, Gerstmann Straussler-Scheinker disease
140
CJD clinical features
Rapid onset dementia
Neurological symptoms - ataxia, weakness & visual disturbances
Psychiatric impairment - precedes neurological symptoms
Myoclonus
141
CJD ix
Diagnosis confirmed by tissue biopsy
Supportive ix:
- EEG
- MRI
- LP
142
CJD mx
No cure for CJD
Symptom control
Palliative care
143
Syringomyelia
Development of a fluid-filled cyst in the spinal cord
144
Syringomyelia aetiology
Chiari malformation
Trauma
Tumours
Idiopathic
145
Syringomyelia clinical features
‘cape-like’ (neck and arms) loss of sensation to temperature but preservation of light touch, proprioception & vibration
- accidentally burn hands without realising
Spastic weakness
Paraesthesia
Neuropathic pain
Upgoing plantars
Bowel & bladder dysfunction
146
Syringomyelia ix
Full spine MRI with contrast
Brain MRI - Chiari malformation
147
Syringomyelia mx
Directed at treating the cause of the syrinx
Patients with persistent/symptomatic syrinx → shunt into syrinx can be placed
148
Third nerve palsy
Neurological condition characterised by weakness or paralysis of the muscles innervated by the CN III
149
Third nerve palsy aetiology
Ischaemic (microvascular) third nerve palsy:
- present without pupillary involvement
- commonly associated with DM, HTN
- tend to resolve spontaneously over weeks to months
Compressive (mass lesion) third nerve palsy:
- typically presents with pupillary involvement
- can be due to aneurysms, tumours or other SOLs
- cases require urgent evaluation & management to prevent life-threatening complications
150
Third nerve palsy ix
MRI
CTA
151
Third nerve palsy mx
Ischaemic CN III palsy:
- patients should be managed conservatively with observation & control of risk factors
- spontaneously resolve within a few months
Compressive CN III palsy:
- urgent evaluation and intervention are required to address underlying cause
Symptom management:
- patching
- prism therapy
- botulinum toxin injections
- eyelid surgery
152
Facial nerve palsy
A condition involvement the unilateral (or sometimes bilateral) dysfunction of the seventh cranial nerve, leading to facial weakness/paralysis
153
Facial nerve palsy aetiology
CNS involvement - distinguished due to forehead-sparing weakness (UMN lesion)
Physical lesions of cerebellopontine angle
Basal meningitis
Ramsey Hunt syndrome
Trauma
Diseases of middle/inner ear
Mononeuritis multiplex
154
Facial nerve palsy clinical features
Facial weakness or paralysis
Difficulty closing the eye on affected side
Altered sense of taste
Hypersensitivity to sound in one ear
Decreased tearing and salivation
Loss of the nasolabial fold
Drooping of mouth on one side
155
Facial nerve palsy ix
Clinical examination & neurological assessment
Imaging - MRI/CT
Serologic tests for Lyme disease/other relevant infectious agents
NCS & EMG → neuromuscular differentials
156
Facial nerve palsy mx
Dependent on underlying cause
Bell’s palsy - corticosteroids
RHS - antiviral agents
Lyme disease & basal meningitis - abx
Acoustic neuroma & trauma - surgical intervention
Physical therapy & rehab to restore facial muscle function
Addressing underlying conditions eg. diabetes, htn
157
Sixth nerve palsy
Dysfunction of the abducens nerve causing a lateral rectus palsy
158
Sixth nerve palsy aetiology
Ischaemia
Neoplastic
Trauma
Cavernous sinus pathology
Deficiency in thiamine & raised ICP - bilateral abducens nerve palsy
Diabetes/HTN
159
Sixth nerve palsy clinical features
Diplopia - worse on horizontal gaze in direction of lesion
Blurred vision
Dizziness
Eye pain
Failed eye abduction - ipsilateral to the lesion
Strabismus - abnormal eye alignment
160
Sixth nerve palsy ix
Clinical diagnosis
MRI/CT
ESR/CRP
161
Sixth nerve palsy mx
Treatment directed towards underlying cause
Persistent symptoms → patching, use of prisms, strabismus, botox injections (referral to ophthalmology)
