Neurology Flashcards
1
Q
What are the features of spastic paraparesis?
A
Increased tone bilaterally
Pyramidal weakness bilaterally
Increased reflexes
Upgoing plantars and clonus
Spastic scissoring gait
2
Q
What extra things would you look for in spastic paraparesis (if doing LL exam)? - not in normal LL exam
A
Eyes - INO, nystagmus, RAPD
Mouth - fasciculations
Hands/arms - small muscle wasting, fasciculations, co-ordination, tone, power, reflexes
UL - sensory level
Back - spinal surgery scars, tenderness
3
Q
Spastic paraparesis + cerebellar signs + dorsal column involvement?
A
Friedreich’s Ataxia
4
Q
Completing Lower Limb Exam
A
PR - anal tone + saddle anaesthesia
Examine UL and Cranial nerves
Take Hx
5
Q
What are the main causes of spastic paraparesis?
A
Demyelination
Cord Compression
Trauma
Anterior Horn Cell Disease
Cerebral Palsy
6
Q
Spastic paraparesis + sensory level
A
Cord compression
- disc disease - spinal stenosis
- tumour
- trauma
- infection - epidural abscess, TB
- Vascular issue - haematoma/haemorrhage
Cord infarction
Transverse myelitis
7
Q
What are the causes of transverse myelitis?
A
Infection - HSV, VZV, HIV
Autoimmune
Paraneoplastic
Sarcoid
Neuromyelitis optica
8
Q
Spastic Paraparesis and dorsal column loss?
A
Demyelination
Friedrich’s ataxia
Subacute combined degeneration of cord
Syphilis
Parasagittal meningioma
Cervical myelopathy
9
Q
Spastic paraparesis and spinothalamic loss
A
Syringomyelia
Anterior spinal artery infarction
10
Q
Spastic paraparesis and cerebellar signs
A
Demyelination
Friedrichs ataxia
Spinocerebellar ataxi
Arnold Chiari malformation
Syringomyelia
11
Q
Spastic paraparesis and small hand muscle wasting
A
Cervical myelopathy
Anterior horn cell disease
Syringomyelia
12
Q
Spastic paraparesis and UMN signs
A
Cervical myelopathy
Bilateral strokes
13
Q
Spastic paraparesis and absent ankle jerk
A
MND
Friedrich’s ataxia
Subacute combined degeneration of cord
Syphilis
Cervical myelopathy and peripheral neuropathy
Conus medullaris
14
Q
Other causes of spastic paraparesis
A
Hereditary spastic paraparesis
Tropical - HTLV1
Cerebral Palsy
15
Q
Spastic paraparesis without any sensory involvement
A
Consider MND and HSP
16
Q
Classic case for cerebral palsy?
A
Birth injury/illness in neonatal period
Intellectual impairment
Spastic paraparesis
Brisk reflexes
Upgoing plantars
17
Q
Classic case for hereditary spastic paraparesis
A
Spastic paraparesis
Upgoing plantars
Brisk reflexes
ABSENT sensory signs
18
Q
Classic case for Friedrich’s ataxia
A
Pes cavus
Cerebellar signs
Wasting
Spastic paraparesis
?Absent reflexes
Dorsal column loss
Peripheral sensory neuropathy
19
Q
Classical case for MS
A
Spastic paraparesis
Brisk reflexes
Upping plantars
Cerebellar signs
Hx of visual or sphincter disturbance
Poss sensory - dorsal column >spinothalamic
20
Q
Classical case for anterior spinal artery occlusion
A
Spastic paraplegia
Brisk reflexes
Upgoing plantars
Loss of pain and temperature with sensory level
Dorsal columns spared
21
Q
Classical case for MND
A
Spastic paraparesis
Brisk reflexes
Upgoing plantars
Wasting
Fasciculations
No sensory signs
22
Q
Demyelinating causes for spastic paraparesis
A
MS
Neuromyelitis optica
Subacute degeneration of cord
Transverse myelitis
23
Q
Asymmetric spastic paraparesis
A
Think Brown-Sequard
24
Q
Compressive causes of myelopathy
A
Disk herniation
Tumours
Spinal stenosis
25
Autoimmune causes of myelopathy
MS
Lupus
Sarcoid
26
Infectious causes of myelopathy
HIV
Varicella
27
Nutritional causes of myelopathy
Vitamin deficiency - B12
Copper deficiency
28
Genetic causes of myelopathy
Hereditary spastic paraparesis
29
Acute causes of spastic paraparesis (minutes to 1-2 days)
Minutes - Likely vascular cause
1-2 days
- Trauma
- Disc herniation
30
Subacute causes of spastic paraparesis - days to weeks
Autoimmune:
- Lupus
- Demyelination
31
Causes of spastic paraparesis that occur over weeks to months
Nutritional deficiencies - B12, copper
Slow growing tumour
32
Chronic causes of spastic paraparesis - month to years
Genetic causes
33
How would you investigate spastic paraparesis?
Bedside
- Blood pressure (autonomic involvement)
- Vital capacity - resp muscle involvement
Bloods
- Bone profile
- LFT
- Immunoglobulin and protein electrophoresis
- FBC, CRP, ESR - malignancy, infection, inflammation
- Virology - HIV, HTLV1, syphilis
- Anti NMO, AMA - autoimmune
- Haematinics - B12
Imaging
- MRI spine - help find cause, localise lesion and assess for intervention
- MRI brain - ?MS
Special
- Visual evoked potentials - ?MS
- LP - ?oligoclonal bands
- EMG - fibrillation and fasciculation in MND
34
How is spastic paraparesis managed?
Non pharmacological:
- Education
- PT/OT
- Orthotics
- Social services
- DVLA
Medical
- steroids - IV methylpred/PO dex
- Radiotherapy
Surgical
- Decompress/fix depending on cause
35
Which malignancies commonly metastasise to bone?
Solid organ - Breast, Prostate, Lung, Kidney, Thyroid
Haem - myeloma
36
What are differentials for flaccid paraparesis?
Myopathies - inflammatory, toxic, inherited
NMJ disorder - botulism, myasthenia
Neuropathy - Hereditary sensory motor neuropathy, alcohol, lead poisoning, porphyria
Anterior horn cells - MND
37
How is MS defined
Demyelinating disease of the CNS which is disseminated in time and space
Doesn't affect peripheral nerves
38
What investigation findings are seen in MS?
Visual evoked potentials - reduced - slow conduction
CSF - increased proteins and lymphocytes. Unmatched oligoclonal bands
MRI - active inflammation, hyperintense on T2 imaging. Periventricular white matter lesions, corpus callosum, brainstem, cerebellar, spinal cord and optic nerve
39
Stocking distribution of sensation loss with reduced/absent reflexes?
Peripheral polyneuropathy
Usually soft touch
± pin prick
± vibration
40
How do you test for sensory ataxia?
If UL - do finger nose with eyes closed
LL - Rhombergs positive only with eyes closed = sensory
41
If thinking peripheral polyneuropathy, what should you look for on examination?
Pes cavus/charcot joint
High stepping gate - foot drop
Thickened nerves - leprosy, Charcot Marie tooth, amyloidosis, neurofibromatosis
Arms - fistula
Abdo - renal, insulin injection sites
Thyroid
Conjunctival pallor
Hands - Dupeytrens contracture, finger prick marks, clubbing, vasculitic signs, muscle wasting
42
When you have detected a peripheral neuropathy, what should you try to work out?
Sensory, motor or sensorymotor
Distal or proxima
Small or large fibre
Focal, multifocal or polyneuropathy
43
Motor causes of peripheral neuropathy?
CIDP
GBS
Hereditary sensorimotor polyneuropathy
Multifocal motor neuropathy and conduction block
Porphyria
Lead/Dapsone
Anterior Horn Cell - Progressive muscular atrophy
Botulism
Muscle issues - inclusion body myositis
44
Predominant sensory peripheral neuropathy?
B12, B1, B6
Alcohol
Diabetes
Isoniazid
Vincristine
Vasculitis
Uraemia
45
Splitting sensory peripheral neuropathy Ddx into categories:
Metabolic:
- Diabetes
- Hypothyroidism
Nutritional deficiency:
- B1, B6, B12
Toxic:
- Alcohol
- Drugs - chemo, antibiotics, antivirals - TB drugs, phenytoin, metronidazole, gold, amiodarone, ciclosporin, hydralazine
Immune mediated:
- RA
- GPA
- CIDP
- GBS
Others:
- Genetic
- Infection - HIV, Lyme
46
How would you investigate a patient with suspected diabetic neuropathy?
Bedside:
- LS BP - ?autonomic dysfunction
- Fundoscopy
- Urinalysis
Bloods
- Renal function
- Hb1AC
47
How would you investigate sensory polyneuropathy
Bedside - fundoscopy, finger prick glucose, urinalysis
Bloods:
- FBC - anaemia, WCC, macrocytosis
- B12
- U&E - uraemia
- Hb1AC
- TFT
- LFT
- ESR/CRP
- Can do connective tissue screen - ANA, ccp, RF, ANCA, Ig, ACE, paraneoplastic
- Virology
Imaging/Special - if req.
- nerve conduction studies ?demyelinating ?axonal
- EMG
- Nerve biopsy
- LP - raised protein + normal cell in GBS. cytology if ?lymphoma
- Genetic testing - Charcot Marie Tooth
48
How can you interpret nerve conduction studies?
Reduced amplitude = axonal pathology
Slower conduction = demyelinating pathology
49
What are the non-pharmacological management options for peripheral neuropathy?
OT/PT
Orthotics
Social needs assessment
DVLA considerations
CBT - chronic pain
TENS machine
Stop drinking - if contributing
DSN/Podiatry if diabetic
Dietician
50
What is the pharmacological management of peripheral neuropathy?
Pain management
- Amitriptyline, Duloxetine
- Gabapentin/Pregablin
- Consider topical capsaicin
- Chronic pain team input
Tight glycaemic control
51
What is Charcot joint?
A neuropathic joint where impaired joint position sense and sensation of pain leads to chronic damage to joints such as the ankle and mid-foot, often accompanied by poor healing responses related to peripheral vascular changes or autonomic neuropathies.
52
How would you clinically distinguish between neuropathy and myopathy?
Myopathy
- Proximal muscle weakness
- No fasciculations + reflexes preserved until late in disease course
- Contractures
Neuropathy
- Distal
- Fasiculations and reflexes lost early
- No contractures
- Sensory signs
If myopathy - consider cardiac involvement
53
What medications cause neuropathy - consider type of neuropathy too
Sensory - Isoniazid
Sensorymotor - Vincristine, amiodarone
Motor - Dapsone
54
What is Charcot Marie Tooth Disease?
Hereditary Sensory Motor Neuropathy
Inherited - typically autosomal dominant
55
What would you look for on inspection in Charcot Marie Tooth?
- B/L Pes Cavus
- Muscle wasting - more prominent distally
- Inverted Champagne Bottle
- B/L Clawed hands/toes
- High steppage gait
- Scoliosis
56
What examination features would make you think Charcot Marie Tooth?
LMN signs:
- Reduced Tone
- Reduced reflexes
Reduced Power - distal>proximal
Normal co-ordination
Mildly impaired sensation - all modalities.
Positive Romberg's
Can palpate thicker nerves
57
What are the main differentials for Charcot Marie Tooth disease?
- Diabetes
- Alcohol
- Vasculitis with mononeuritis multiplex
58
Acquired neuropathies which are differentials for Charcot Marie Tooth?
Immune - CIDP, multifocal motor neuropathy with conduction block
Metabolic - diabetes
Toxic - alcohol, meds
Nutrition - B12
Paraneoplastic
Amyloidosis
59
Investigations to consider in Charcot Marie Tooth
Bedside
- Basic jobs - L/S for autonomic involvement
- Blood glucose
- Urine dip - glucose/amyloid
- Fundoscopy - CMT types 6 - optic atrophy
Bloods - r/o alternatives - inflammatory markers, deficiency, Diabetes etc.
Special
- Genetic testing
- Nerve conduction studies
- Nerve biopsy
60
How is Charcot Marie Tooth managed?
MDT Approach:
- PT
- OT
- Genetic counselling
- Support groups
Med - analgesia
Sure - orthopaedic procedures
61
Types of Charcot Marie Tooth?
Based on clinical findings, inheritance, nerve conduction studies and genetic testing:
Thickened peripheral nerves T1/3
Weak arm>leg = T2D
Pyramidal tract signs - T5
Optic atrophy - T6
62
What are some pyramidal tract signs
Hyperreflexia
Spasticity
Upgoing plantars
Slowed alternating movements
63
Thrombolysis contraindications
Significant head trauma within 3months
Ischaemic stroke within 3 months
Intracranial malignancy
Bleeding: active, disorders, recent
Recent surgery - esp brain/spine
HTN 200/100
Pregnancy
64
Inflammatory causes of motor neuropathy
Guillian Barre
Chronic Inflammatory Demyelinating Polyneuropathy
Multifocal motor neuropathy
Sarcoid
Vasculitis
Paraprotein
Amyloidosis
65
Infectious causes of motor neuropathy
HIV
Diptheria
Lyme disease
HTLV-1
West nile virus
Rabies
Enteroviruses
66
Toxin causes of motor neuropathy
Lead
Arsenic
Thallium
Mercury
Shellfish poisoning
67
Metabolic causes of motor neuropathy?
Diabetic amyotrophy
Porphyria
68
Drug causes of motor neuropathy?
Penicillamine
Gold
Ciclosporin
69
Congenital causes of motor neuropathy
Charcot-Marie Tooth
70
Neuromuscular junction disorders associated with flaccid paraparesis?
Myasthenia gravis
Lambert Eaton Syndrome
Botulism
Organophosphate poisoning
Tick paralysis
Snake venom
71
Features of Guillian Barre Syndrome
Acute inflammatory demyelinating neuropathy
Ascending paralysis
Onset over days - weeks, typically after an infection - chest (Mycoplasma) or GI - Campylobacter
72
Symptoms of Guillain Barre
Spine pain
Distal paraesthesia - sensory loss is patchy
Fatigue
Ascending flaccid paralysis
Absent reflexes
73
What are the other features of Guillian Barre (apart from flaccid paralysis)
Cranial nerves - ptosis, ophthalmoplegia, facial nerve and bulbar weakness
Autonomic - tachycardia, labile BP, arrhythmia, bladder/bowels
Resp - SOB, fatigue
74
What is Miller Fischer Syndrome?
Proximal variant of GBS
Ataxia
Ophthalmoplegia
Areflexia
anti-GQ1b antibodies
75
How would you investigate Guillian Barre
CSF Cytology - raised protein, normal WCC
Nerve conduction study - reduced velocity + conduction block
Anti GM1 antibodies
FVC
ECG
76
How is Guillian Barre managed?
IVIG
Methylpred
Plasma exchange
ITU - if FVC <1.3L or Bulbar dysfunction - may need tracheostomy/ventilationW
77
What is spina bifida
Neural tube defect with incomplete closure of the vertebral canal
78
How does spina bifida present?
Asymmetrical flaccid paraparesis with sensory loss (usually L5/S1)
Bladder and bowel involvement
Cutaneous - tufts of here/dimples
79
What would you expect with old polio?
Flaccid mononeuropathy
Often associated limb shortening
Limb hypoplasia and wasting
Reduced reflexes
No sensory loss
80
What is a differential for old polio?
Infantile hemiplegia
Motor neurone disease
81
What is polio, how is it transmitted?
Enterovirus transmitted via faecal oral route
Causes predilection of anterior horn cells
82
Clinical signs of Parkinson's Disease
Expressionless face
Coarse, pill rolling resting tremor - 3-5hz
Bradykinesia
Absence of arm swing
Stooped, shuffling gait + postural instability
Cog-wheel rigidity + lead pipe rigidity
Slow monotonous faint speech
Micrographia
83
How is it best to illicit cogwheel rigidity?
- Best felt at wrist
At elbow - felt on extension motion
Ask patient to do somehting with other hand - tap on knee
84
Screening tests to do in UL neuro exam?
Pronator drift - help detect UMN lesion
Tap arms - over shoot on bounce back = cerebellar
Extension of fingers rapidly - difficult = myotonic dystrophy
Tap index finger and thumb - small = Parkinson's
Shoulder abduct against resistance one side. Then ask pt. to hold up both arms in shoulder abduction. If one side falls - myasthenia
85
What other tests would you do to assess for Parkinsons Plus syndromes?
BP - MSA or cerebellar syndromes - Parkinsonism with postural hypo + cerebellar and pyramidal signs
Verticle eye movements - impaired in PSP
Dementia + PD = Lewy body dementia
Visual or cognition - Lewy body
Gait failure/LL impairment - vascular dementia
Medication hx - drugs can cause parkinsonism - metoclopramide, haloperidol, chlorpromazine
86
Causes of Parkinsonism?
Parkinsons disease
PP syndromes - MSA, PSP, Corticobasal degeneration (unilateral)
Drug induced
Anoxic brain injury
Post encephalitis
MPTP toxicity
87
Pathophysiology of Parkinson's disease?
Degeneration of dopaminergic neurones in the substantia nigra and basal ganglia.
88
Non motor manifestations of Parkinsons Disease?
Constipation
Urinary incontinence
Autonomic instability - postural hypotension
Mood disorders
Anosmia
Cognitive effects
Pain
Sleep disorders - REM, broken sleep
89
How is PD investigated?
Referral to specialist for diagnosis
Clinical diagnosis - need 3 core features - bradykinesia, rigidity and tremor + r/o other causes
Some Ix may include:
- CT/MRI head
- SPECT - differentiate between PD and essential tremor
- Caeruloplasmin and urinary copper
90
How is Parkinson's Disease managed?
MDT - neurology, psych, PD nurses, PT/OT, GP, SALT, Social care
Medications:
- Dopamine agonist - Ropinirole, Rotigotine, Apomorphine
- Levodopa - usually with carbidopa
- MAO-i - Selegiline, Phenelzine
- COMT inhibitory - Entacapone
- Amantadine
Surgical
- Pallidotomy - create scar in globus pallidus to reduce activity
- Thalmic surgery
- Deep brain stimulation - in subthalmic nucleus or globus pallidus
91
How is medication therapy chosen in Parkinson's Disease?
Early disease - MAOI or Dopamine agonists
Less side effects
Spare L-DOPA for later in disease course
92
Important complications of Levodopa therapy?
Weaning off
On-off fluctuations
Dyskinesia
Hallucinations and psychosis
Compulsive behaviours
93
What are the symptoms associated with post polio syndrome?
Fatigue
Cramps
Progressive muscle weakness
94
How would you investigate a patient with suspected post polio syndrome?
Exclude concurrent or alternative pathologies:
- MRI lumbosacral spine
- Nerve conduction studies
- EMG
95
How is post polio syndrome managed?
MDT approach:
- Neurology - symptom management
- PT/OT
- Neuropsychologist
Regular review
Assess patient function and help make adjustments for maintaining independence
96
What does pet cavus indicate and give some causes?
Suggests chronic neuromuscular disease
Charcot-Marie Tooth
Freidreich's Ataxia
Old polio
Syringomyelia
Cerebral Palsy
97
Features of Horners Syndrome?
Partial ptosis
Apparent enopthalmos
Miosis (constricted) - causes anisocoria (not equal)
Anhidrosis and flushing
98
What should you mention if you mention ptosis
unilateral/bilateral
complete or partial
fatiguable or not
99
How can you try localise lesion in Horners Syndrome based on clinical signs?
Using presence of anhidrosis/flushing
1st order - ipsilateral body and face affected
2nd order - Ipsilateral face only affected
3rd order - No anhidrosis or flushing
100
Things to examine when suspecting Horners syndrome?
General - hemiparesis, hearing aids
Face - heterochromia, eye movements
Neck - scars/signs of trauma, lymphadenopathy, aneurysm, thyroid nodules, tracheal deviation, carotid bruits
Upper thorax - scars (superior/posterior), lung bases, vocal resonance
Ipsilateral arm - wasting, fasciculations, claw hand
Neuro UL - motor, cerebellar, sensory
101
Possible causes for Horners Syndrome
Congenital - birth injury - Klumpke's, Hereditary
First order (from hypothalamus, to midbrain, to spinal cord) - often associated with other neurological signs
- Trauma
- Stroke - Wallenberg syndrome
- Tumour
- Demyelination
- Syringomyelia
Second order - spinal cord, exit C8-T2, top of lung, into neck
- Trauma
- Pancoast tumour
- Schwannoma
Third Order - neck into face
- Trauma
- Carotid artery dissection
- Cavernous sinus thrombosis or inflammation
- Cluster headache
- Migraine
102
How would you investigate Horners Syndrome?
Bedside - review old photos
Bloods - depend on cause. Consider LFT/Bone profile for malignancy
Imaging
- MRI Brain
- CXR - ?Pancoast
- MRA/Carotid doppler
- MRI cavernous sinus and orbit
Special tests
- Apraclonidine test - reverse anisociria by dilating affected pupil - ptosis may resolve
- Cocaine test - exacerbate anisocoria - dilate normal pupil but not affected
- Hydroxyamphetamine test - localise lesion - if absent or poor dilation --> 3rd order
103
How is Horners syndrome managed?
Education
Treat underlying cause
104
How is hereditary Horners syndrome inherited?
Autosomal Dominant
105
What is Wallenberg's Syndrome?
Lateral Medullary syndrome:
- Posterior inferior cerebellar artery infarct
Dysphagia
Sensory loss - ipsilateral face and contralateral trunk and limbs
Rotatory Nystagmus
106
If a patient has face or neck pain prior to onset of Horners, what should you consider?
Carotid artery dissection --> need urgent CTA or MRA
107
What is pseudoathetosis and what does it represent?
Involuntary slow writing movements when eyes are shut
Evidence of sensory ataxia secondary due to loss of proprioception
108
What are some red flags that a lower motor neurone neuropathy is not a typical length dependent neuropathy?
Non-length discrepancy
Marked asymmetry
Prominent sensory ataxia
109
Common causes of peripheral neuropathy?
Diabetes
Alcohol
Hypothyroidism
110
Common causes of inflammatory peripheral neuropathy?
Acute - Guillain-Barre
Chronic - CIDP
111
Key features of Guillain-Barre syndrome
Ascending bilateral weakness
Loss of reflexes
Reduced sensation
112
How would you investigate a patient with a concerning peripheral neuropathy?
Bloods
- FBC, UE, LFT, CRP, ESR
- HbA1C/TFT/B12/Virology (rule out common causes)
- Paraneoplastic screen
EMG - ?axonal or demyelinating
Nerve conduction studies
CSF - raised protein, normal cell count in GBS/CIDP
113
How would Guillain-Barre be managed?
A-E approach in acute setting
Mostly supportive
Frequent FVC monitoring - if <1.5 - consider whether ventilatory failure present - ABG's/discuss with ITU
Severe Sx - may need Plasma Exchange or IVIG
114
How would CIDP be managed?
MDT approach - PT, OT, Orthotics
Regular neurology review
Symptomatic therapies:
- Neuropathic pain
Disease modifying:
- Plasma exchange
- IVIG
- Steroids
- Immunosuppression
115
What are the symptoms of spastic paraparesis due to myelopathy?
Weakness
Upgoing plantars
Increased tone
Brisk reflexes
Reduced Sensation
116
What things should you do as screening tests for an UL examination?
Pronator drift
Myotonic dystrophy
Parkinsons
Winging of scapula
Cerebellar - past pointing/push arms down
117
How is spinocerebellar ataxia inherited?
Autosomal dominant
Has anticipation - present earlier throughout generations
118
Tips for checking co-ordination on UL/LL exam
Ask patient to extend finger as far as possible. Put finger at end of reach to look for tremor. Then move a little closer for past pointing
LL
- Tap feet against hands - look for irregularity
- Put finger at maximal foot reaching distance - get them to touch toe to finger --> past pointing/tremor
119
How would you test the key nerves in UL examination?
Radial - finger extension
Ulnar - abductor digiti minimi
Medial - thumb abduction
120
How does pattern of weakness help you localise the cause?
Proximal - myopathic cause
Distal - neuropathic cause
121
What are the key differentials for distal weakness with preserved reflexes, normal sensation and normal tone?
This indicates a muscle pathology:
- Myotonic dystrophy
- Inclusion body myositis
122
What are some features of inclusion body myositis?
Elderly male patients
Long finger flexors affected
Proximal impairment in legs - quads
123
Features of myotonic dystrophy
Distal weakness
Atrophic temples
Partial ptosis
Frontal balding
Percussion myotonia
Look for PPM - associated with conduction delay (heart block/Long QT)
Listen to lungs - bibasal creps
Associated with diabetes, peripheral oedema and gonadal atrophy
124
What is percussion myotonia?
Tap on thenar eminence - thumb move inwards
125
Important when testing UL tone?
Do full 180 degree motion
Fast and slow - look for catch.
Catch is often late. If throughout --> cogwheel rigidity
126
What is pyramidal weakness?
Flexors stronger than extensors
127
Key things to look and comment on in diabetic neuropathy?
Neurologically impairment - sensory motor but predominantly sensory
Vasculature - check pulses and listen for femoral bruit
Look for ulcers and infections
Look and comment on toes - ?amputations
Charcot joint
128
What order is sensation lost in diabetic neuropathy?
Temperature first then proprioception and vibration
Dorsal columns protected by myelin sheath
129
Causes of dystonia?
Idiopathic
Parkinsons
Parkinsons plus
If a patient is dystonic - ask r.e. signs of PD
130
What are some features of dystonia?
Botox is helpful
Symptoms resolve with rest
Worse when stressed or tired
Develop neck flexion and head drop
131
How would you differentiate between neurofibromatosis type 1 and 2?
NF1 - childhood. Many cutaneous features
NF2 - hearing loss. minimal cutaneous. early adulthood
132
Features of a Parkinsons Gait?
Slow shuffling
Reduced arm swing
Stooped posture
Reduced stride length
133
Important additional examination things in suspected Parkinson's?
Clasp fingers - big and fast as possible - screening test
Use synkinesis - look for tremor. Worse when doing other things
If have paper - draw spiral/write sentence
Ask for BP - considering autonomic features of MSA
Mention but don't do
- Globolar tap - lose blink suppression
- Pull test - take many steps back in PSP/MSA
134
What would a symmetrical tremor in a patient with Parkinsonian features make you think?
Less likely to be PD
- Parkinson's plus
- Vascular parkinson's
- Drug related - antipsychotic
135
What can cause a cranial nerve VII palsy?
Bells
Ramsay Hunt
Cerebellopontine angle tumour
Lyme disease
Sarcoidosis - bilateral bells palsy
Guillain Barre - bilateral
Lupus - bilateral
136
What is important about aciclovir use?
Nephrotoxic - needs regular U&E monitoring
137
If a patient has double vision, how should you approach this?
Check eye movements
- Look for clear CN III, IV or VI palsy
If not fitting into clear nerve pathology --> complex ophthalmoplegia. Need to consider other causes
138
If cranial nerve VI is affected, what should you check and why?
Assess CN VII --> ?cerebellopontine angle tumour
139
How can cranial nerve III pathologies be categorised?
Compressive vs Vascular
If evidence of pupil dilation --> need urgent admission
Vascular has no pupil involvement
140
When checking eyelids, what should you be looking for?
Drooping - ?MG ?myotonic dystrophy
Lid lag - ?thyroid eye disease
Proptosis
141
If a patient has sudden loss of vision and a RAPD, what should you always consider?
GCA
142
What can cause tunnel vision?
Glaucoma
Retinitis Pigments
Tunnel vision crosses the midline
143
How can eye pathologies be split into eye vs systemic issues?
If unilateral --> optic nerve/retinal issue.
If B/L
- Hemianopic --> vascular/cranial issue
- If cross midline --> Eye issue
144
What can cause angioid streaks on fundoscopy?
Pseudoxanthoma elasticum
Ehler's Danlos
145
Additional things to examine in a patient with proximal myopathy
Fatiguability
Inspect eyes - ?ptosis
Fasciculations - ?MND
Check skin - ?dermatomyositis
Look for cushingoid appearance - ?steroid use
Wasting - ?Hereditary/MND/Long Standing
146
What are some causes of proximal myopathy?
Inflammatory - dermatomyositis/polymyositis
Connective tissue - SLE, RA, Systemic sclerosis, Mixed, vasculitis
Cancer
Drugs - statins, steroids
Infections - HIV, EBV, CMV, Hepatitis, Bacterial
Endocrine - thyroid, acromegaly, Addisons, cushings, diabetic
Toxins - alcohol
Metabolic - liver/renal failure, electrolyte abnormalities
Misc - mitochondrial, rhabdo, sarcoidosis
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Investigations for proximal myopathy
Bloods
- FBC, U&E, Ca, Phosphate, Mg, LFT
- CK, extended LFT's
- TFT, Vit D, HbA1C, cortisol
- HIV/Hepatitis screen/CMV/EBV/Serum ACE
- Rheum screen
Urine dip/PCR
Nerve conduction studies and EMG
Muscle MRI
Muscle biopsy
Ca screen if dermatomyositis/polymyositis
Systemic assessment - CXR, ECG, Echo, Lung function etc.
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When would a patient with a proximal myopathy need admission?
Evidence of respiratory involvement
Evidence of rhabdomyolysis
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What increase risk of myopathy in patient's taking statins?
CYP450 inhibitors:
- Fibrates
- Ciclosporin
- Amiodarone
- Macrolides (clarithromycin)
- Protease inhibitors
Old age
ETOH XS
Renal/liver failure
Hypothyroid
Strenuous exercise
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What factors predispose to vitamin D deficiency?
Dark skin
Low exposure to sunlight
Old age
Renal failure
Poor absorption - coeliac
Meds - reduce breakdown - Rifampicin, Phenytoin
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What can cause a raised CK?
Rhabdo
Afro-caribbean ethnicity
IM injections
Exercise
MND
Hypothyroid
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What would you expect on examination in a cerebellar syndrome?
Tone - REDUCED - if increased ?corticospinal tract involvement
Power - Normal - if reduced ?corticospinal
Reflexes - pendular (less brisk)
Intention tremor
Dysdiadocokinesia
Nystagmus
Past pointing
Scanning/slurred speech
Gait - broad based. Struggle with tandem
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What should you test if you detect unilateral cerebellar signs?
CN V, VI, VII, VIII - CP angle tumour
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What should you consider if you detect BL cerebellar signs?
Spinocerebellar ataxia
Freidrichs ataxia - look for pacemaker, dorsal columns, pes cavus, Diabetes
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Differentials for unilateral cerebellar signs
Demyelination
Vascular
Space occupying lesions
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Differentials for B/L cerebellar signs
Inherited:
- Spinocerebellar ataxia
- Friedreich's ataxia
- Ataxic Telangiectasia
Metabolic - alcohol, B12 deficiency, Wilson's
Iatrogenic - phenytoin, carbemazepine
Paraneoplastic (anti hu/yo)
Endocrine - hypothyroid
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Important professionals in management of Friedreich's ataxia
Cardiologist - HCM, HF, Arrhythmia
Diabetes
Orthopaedics
Neurologist
PT/OT/Social workers/Genetic counsellors, Orthotics
GP
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How does Huntington's disease present?
Chorea - hyperkinetic movements. Large movements. Jerky, Unpredictable
Hemiballism - violent flinging movements. Single arm +-ipsilateral leg
Athetosis - slow writhing
Dysarthria
Facial grimace
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Differentials for Huntington's Disease?
Inherited - Huntington's, Wilsons
Vascular - stroke, polycythaemia rubra vera
Immune - Sydenham's chorea, SLE, antiphospholipid
Hormonal - Pregnancy (Gravidarum chorea), OCP, Hyperthyroid
Toxic - antipsychotics, dopaminergic in PD, anti epileptics
Infectious - HIV, lyme's
Paraneoplastic
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How is Huntington's disease managed?
Non Medical:
- Education, genetic counselling etc.
- PT/OT + SALT
- DVLA considerations
Medical:
- Tetrabenazines - anti-choretic
Treat complications:
- Depression - SSRI
- Rigidity - dopamine agonist, baclofen
- psychosis - atypical antipsychotics
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What is the inheritance of Huntington's?
Autosomal dominance with anticipation
Trinucleoside repeat of CAG on chromosome 4
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What are some complications of Huntington's?
Seizures
Psychosis
Depression
Rigidity
Progressive dementia
Aspiration pneumonia
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What happens in internuclear ophthalmoplegia?
Medial longitudinal fasciculus (heavily myelinated tract) normally allow communication between contralateral CN III and CN VI for co-ordinated lateral gaze.
In INO - loss of MLF unilaterally or bilaterally --> lose of conjugate adduction.
- R MLF loss = R adduction loss - nystagmus of L eye when looking left. No issues when looking right. (L MLF still working).
If left eye covered, then R eye should be able to abduct and adduct without issues - muscles and nerves unaffected, just the communication for conjugate gaze
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Causes of internuclear ophthalmoplegia?
MS and Stroke are most common
Others:
- Sarcoid/SLE
- Infections
- Wernicke's
- Fabry's
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Examples of stroke mimics
Hypoglycaemia
Hemiplegic migraines
Seizures
Hyponatraemia
SOL
Intoxication/Infection
MS
Todd's Paresis
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Completing examination with hemiparetic signs?
Examine cranial nerves, UL and LL (whichever haven't done)
Urine dip
Fingerprick glucose
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How can different strokes be classified?
Bamford classification:
Anterior - partial/total
Posterior
Lacunar
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Classical case of anterior circulation stroke?
- Motor sensory deficit affecting 2/3 of face, arm and leg
- Higher cortical dysfunction
- Visual field defect
Total = 3/3, Partial = 2/3
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Classical case for posterior circulation stroke?
Cranial nerve loss with contralateral motor sensory deficit
Conjugate eye movement disorder
Bilateral motor-sensory deficit
Cerebellar dysfunction
Isolated homonymous hemianopia
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Classical case for a lacunar stroke?
Discrete symptoms affecting 2/3 arm, leg or face (NO HIGHER CORTICAL SIGNS OR VISUAL FIELD IMPAIRMENT)
Pure sensory
Pure motor
Sensory motor
Ataxic hemiparesis
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How can you assess speech?
1 - Simple commands
2 - 2 step commands
3 - Expressive dysphasia/dysarthria - baby hippopotamus
4 - Speech production e.g. what did you have for breakfast?
5 - Test naming - hold up pen and ask them to identify/what use it for
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