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PACES 2.0 > Neurology > Flashcards

Neurology Flashcards

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1
Q

Cranial nerves affected in cavernous sinus syndrome

A

Ipsilateral
III
IV
VI
V1

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2
Q

Associated antibodies in myasthenia gravis

A

Acetylcholine receptor antibodies
- 75%

Anti-muscle-specific kinase antibodies

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3
Q

Diagnostic tests for myaesthenia gravis

A

AChR Ab
Striated muscle Ab
MuSK Ab

Electrophysiological studies
- EMG

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4
Q

Signs of UMN lesion

A

Increased tone
Increased reflexes
Upgoing plantars

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5
Q

Signs of LMN lesion

A

Reduced tone
Reduced reflexes
Downgoing plantars

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6
Q

Unilateral UMN lesion

A

Brain
- stroke
- tumour
- MS
- abscess
- severe subdural haemorrhage

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7
Q

Bilateral LMN lesion

A

Cauda equina
- disc
- tumour

Polyneuropathy

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9
Q

Unilateral LMN lesion

A

Nerve root radiculopathy
- disc
- tumour

Mononeuropathy
- entrapment
- trauma

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10
Q

Signs of dystrophia myotonica

A

Myopathic facies
- long, thin, expressionless
Wasted facial, sternocleidomastoid, and distal muscles
Bilateral ptosis
Frontal balding
Dysarthria
Myotonia
Areflexia

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11
Q

Associated with dystrophia myotonica

A

Cataracts
Cardiomyopathy
Brady/tachyarrhythmias
Diabetes
Testicular atrophy
Dysphasia

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12
Q

Long, thin, expressionless facies
Bilateral ptosis
Frontal balding
Myotonia

A

Dystrophia myotonica

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13
Q

Genetics of dystrophia myotonica type 1

A

Common
AD
Anticipation
Expansion of CTG trinucleotide repeat
DMPK gene
Chromosome 19

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14
Q

Genetics of dystrophia myotonica type 2

A

Rare
AD
Anticipation
Expansion of CCTG trinucleotide repeat sequence
ZNF9 gene
Chromosome 3

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15
Q

Diagnosis of dystrophia myotonica

A

Clinical features
EMG
Genetic testing

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16
Q

Management of dystrophia myotonica

A

MDT
No treatment for weakness
Mexiletine/phenytoin for myotonia
CI: general anaesthetic
Premature death from cardio/resp complications

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17
Q

Clinical signs of cerebellar syndrome

A

DANISH

Dysdiadochokinesis
Ataxia
Nystagmus
Intention tremor
Scanning dysarthria
Hypotonia/hyporeflexia

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18
Q

Cerebellar hemisphere lesions - side of signs

A

Ipsilateral

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19
Q

Causes of cerebellar syndrome

A

PASTRIES

Paraneoplastic cerebellar syndrome
Alcohol
Sclerosis (MS)
Tumour
Recessive (FA, AT) or dominant (SCA)
Iatrogenic (phenytoin)
Endocrine (hypothyroidism)
Stroke

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20
Q

Signs of MS

A

Wheelchair/catheter
Optic atrophy
Internuclear ophthalmoplegia
Optic neuritis (reduced colour vision/acuity/pain on eye movements)
UMN signs
Cerebellar signs

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21
Q

Location of lesion in internuclear ophthalmoplegia

A

Medial longitudinal fasiculus

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22
Q

Anatomy of conjugate eye movements

A

Medial longitudinal fasciculus
Paramedian pontine reticular formation
VI
III

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23
Q

Signs of internuclear ophthalmoplegia

A

Ipsilateral eye unable to adduce in response to abduction of normal eye
Contralateral nystagmus
Ipsilateral abduction normal
Convergence normal

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24
Q

Diagnosis of MS after a single attack is with

A

McDonald criteria

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25
Q

Subtypes of MS

A

Relapsing-remitting (85%)
- eventually convert to secondary progressive
Primary progressive (15%)

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26
Causes of MS
Genetic (>200 alleles) Environmental (EBV, smoking, latitude, Vit D) Mixed <2% risk of MS in children
27
Uthoff’s phenomenon
MS Worsening of symptoms with heat or exercise
28
Lhermitte’s sign
Shock like pain on neck flexion
29
Management of MS
MDT Disease modifying treatments - RR: interferon-B, fingolimod, monoclonal Abs reduce relapses and delay conversion - PP: octelizumab (anti-CD20) modestly effective Symptomatic treatments - methylprednisolone (shortens relapses) - anti-spasmodics (baclofen) - gabapentin, pregabalin, amitriptyline - laxatives - intermittent catheterisation
30
Investigations for stroke/TIA
FBC CRP/ESR HbA1c Cholesterol Renal function ECG CXR CT Head MRI Head Holter ECHO Carotid Doppler Angiogram Vasculitic screen (if young)
31
Indications for thrombolysis in stroke
<4.5 hours from onset of acute ischaemic stroke
32
Indications for thrombectomy in stroke
Performed by IR Acute ischaemic stroke CTA shows proximal occlusion Within 24hrs
33
Management of stroke
Thrombolysis (4.5hr) Thrombectomy (24hr) DAPT MDT Carotid endarterectomy Anticoagulation Cardiovascular risk factors Social care DVLA
34
Indications for carotid endarterectomy
Symptomatic - anterior circulation stroke/TIA - TLOC Ipsilateral carotid artery >50% stenosis
35
Lateral medullary syndrome (AKA)
Wallenberg syndrome
36
Lateral medullary (Wallenberg) syndrome signs
Acute vertigo/nystagmus Horner’s syndrome Ipsilateral loss of face pain and temperature sensation Contralateral loss of limb pain and temp sensation
37
Lateral medullary (Wallenberg) syndrome pathophysiology
Brainstem stroke (lateral medulla) - inferior cerebellar peduncle - vestibular nucleus - descending sympathetic tract - nucleus ambiguous - trigeminal nerve nucleus - spinothalamic tract Occlusion of PICA
38
Common causes of spastic legs
MS Spinal cord compression Myelopathy Trauma MND
39
Rare causes of spastic legs
Anterior spinal artery stroke Inflammatory - neuromyelitis optica - SLE - Sjögren’s Syringomyelia Hereditary spastic paraplegia B12 deficiency Friedreich’s ataxia Parasaggital falx meningioma
40
Spastic legs Pain/temperature loss Weakness
Anterior spinal artery stroke
41
Optic neuritis Spastic legs
Neuromyelitis optica
42
Spastic legs Cerebellar signs Peripheral neuropathy Pes cavus Optic atrophy
Friedreich’s ataxia
43
Spastic legs Dorsal column sensory loss
B12 deficiency (subacute combined degeneration of the cord)
44
Lumbosacral roots, motor: L1/2
Hip flexion
45
Lumbosacral roots, motor: hip flexion
L1/2
46
Lumbosacral roots, motor: L2/3
Hip adduction
47
Lumbosacral roots, motor: hip adduction
L2/3
48
Lumbosacral roots, motor: L3/4
Knee extension
49
Lumbosacral roots, motor: knee extension
L3/4
50
Lumbosacral roots, reflex: knee jerk
L3/4
51
Lumbosacral roots, motor: L4/5
Ankle dorsiflexion Ankle inversion Ankle eversion
52
Lumbosacral roots, motor: ankle D/I/E
L4/5
53
Lumbosacral roots, motor: L5/S1
Knee flexion Hip extension
54
Lumbosacral roots, motor: knee flexion
L5/S1
55
Lumbosacral roots, motor: hip extension
L5/S1
56
Lumbosacral roots, motor: S1/2
Ankle plantarflexion
57
Lumbosacral roots, motor: ankle plantarflexion
S1/2
58
Lumbosacral roots, reflex: ankle jerk
S1/2
59
Weakness and wasting of small muscles of the hand Loss of reflexes in upper limbs Loss of pain/temperature sensation in upper limbs Preservation of joint position and vibration Charcot joints Pyramidal weakness in lower limbs Upgoing plantars Horner’s syndrome
Syringomyelia
60
Signs of syringomyelia
Weakness and wasting of small muscles of the hand Loss of reflexes in upper limbs Loss of pain/temperature sensation in upper limbs Preservation of joint position and vibration Charcot joints Pyramidal weakness in lower limbs Upgoing plantars Horner’s syndrome
61
Syringomyelia definition
Progressively expanding fluid-filled cavity in the cervical cord Spans several levels
62
Investigation for syringomyelia
Spinal MRI
63
Causes of Charcot joint
Tabes dorsalis Diabetes Syringomyelia
64
Treatment of Charcot joint
Bisphosphonates can help
65
Cervical roots, motor: C5
Shoulder abduction
66
Cervical roots, motor: shoulder abduction
C5
67
Cervical roots, motor: C5/6
Elbow flexion Supination
68
Cervical roots, motor: elbow flexion
C5/6
69
Cervical roots, motor: supination
C5/6
70
Cervical roots, reflex: biceps jerk
C5/6
71
Cervical roots, reflex: supinator jerks
C5/6
72
Cervical roots, motor: C6/7
Elbow extension Wrist extension
73
Cervical roots, motor: elbow extension
C6/7
74
Cervical roots, motor: wrist extension
C6/7
75
Cervical roots, motor: triceps jerk
C7/8
76
Cervical roots, motor: C7
Finger extension
77
Cervical roots, motor: finger extension
C7
78
Cervical roots, motor: C8
Finger flexion
79
Cervical roots, motor: finger flexion
C8
80
Cervical roots, motor: T1
Finger abduction
81
Cervical roots, motor: finger abduction
T1
82
UMN and LMN signs Preserved sensation
Motor neuron disease
83
Hot potato speech
Pseudo-bulbar UMN Spastic tongue
84
Donald Duck, nasal speech
Bulbar LMN Flaccid tongue
85
Definition of motor neuron disease
MND is a progressive disease of unknown aetiology Axonal degeneration of upper and lower motor neurons
86
MND with best prognosis
Progressive spinal muscular atrophy
87
MND with worst prognosis
Progressive bulbar palsy
88
MND types
Amyotrophic lateral sclerosis - combined UMN and LMN Primary lateral sclerosis - UMN signs Progressive spinal muscular atrophy - LMN in limbs Progressive bulbar palsy - LMN in brainstem
89
Investigations in MND
EMG - denervation MRI brain and c-spine - exclude mimics
90
Management of MND
MDT - psych, dietician, PT, OT, SLT PEG feeding NIPPV Riluzole (glutamate antagonist) - slows disease progression
91
Signs of Parkinson’s disease
Expressionless face Pill rolling tremor Bradykinesia Cogwheel rigidity Shuffling gait Monotonous, quiet speech
92
Parkinson’s plus syndromes
Multiple system atrophy Progressive supranuclear palsy Lest body dementia
93
Additional signs to elicit in PD
LSBP -> postural drop = MSA Vertical eye movements -> PSP
94
Causes of Parkinsonism
PD PD+ - MSA, PSP, LBD Drug-induced - prochlorperazine, metoclopramide, antipsychotics Vascular Post-encephalitis
95
Pathology of Parkinson’s Disease
Degeneration of dopaminergic neurons between the substantia nigra and basal ganglia
96
Treatment of Parkinson’s Disease
MDT L-Dopa with a peripheral dopa-decarboxylase inhibitor - wearing off -> add in entacapone or MAOi (selegiline) - peak dose dyskinesia - nausea Dopamine agonists Deep brain stimulation Dopamine agonist -> apomorphine
97
Hereditary motor sensory neuropathy (CMT) signs
Wasting of distal LL Pes cavus Weakness of distal muscles Distal sensory loss High stepping gait Palpable lateral popliteal nerve
98
Hereditary motor sensory neuropathy (CMT) subtypes
Axonal Demyelinating
99
Most common form of hereditary motor sensory neuropathy (CMT)
Type 1A AD Demyelinating 17p duplication
100
Signs of Friedreich’s ataxia
Ataxic gait Young adult Pes cavus Cerebellar signs UMN and LMN Loss of vibration and position joint sense Kyphoscoliosis Optic atrophy HOCM murmur DM Sensorineural deafness High-arched palate
101
Ataxic gait Young adult Pes cavus Cerebellar signs UMN and LMN Loss of vibration and position joint sense
Friedreich’s ataxia
102
Friedreich’s ataxia inheritance and prognosis
Autosomal recessive Onset as teenager Survival for ~20 years HOCM and mild dementia
103
Facial weakness sparing forehead
UMN
104
Facial weakness involving forehead
LMN
105
Commonest cause of facial weakness
Bell’s palsy
106
Pathophysiology of Bell’s palsy
Demyelination Swelling and compression of nerve in facial canal
107
Treatment of Bell’s palsy
Prednisolone within 72hrs Eye protection - artificial tears - tape eye closed at night
108
Prognosis of Bell’s palsy
70-80% make a full recovery
109
Pathophysiology of myaesthenia gravis
Anti-AChR or anti-MuSK Abs Reduce NMJ transmission
110
Diagnostic tests for myaesthenia gravis
Anti-AChR Anti-MuSK EMG - reduced potentials with repeat stimulation
111
Other tests in myaesthenia gravis
CT or MRI of mediastinum - thymoma TFTs TPMT - if requiring azathioprine
112
Treatment for myaesthenia gravis
Acetylcholinesterase inhibitor - pyridostigmine +/- immunosuppression - steroids - azathioprine or mycophenolate IVIG Plasma exchange Thymectomy
113
Cancer associated with Lambert-Eaton myaesthenic syndrome
Small cell lung cancer
114
Pathophysiology of LEMS
Abs block pre-synaptic calcium channels
115
Treatment of LEMS
Treat cause Immunosuppress if no cancer 3,4-diaminopyridine for symptom control
116
Tuberous sclerosis signs
Perinasal angiofibromata Periungal fibromas Leathery skin over lumbar region - Shagreen patch Ash leaf macules May have signs of RRT or transplant Retinal phakomas LD Seizures
117
Ash leaf macules fluoresce with
UV/Wood’s light
118
Complications of tuberous sclerosis
Cystic lung disease Renal - angiomyolipomas - cysts - RCC Learning difficulties Seizure
119
Investigations in tuberous sclerosis
Skull films - railroad track calcification CT/MRI Head - tuberous masses ECHO - hamartomas USS - renal cysts
120
Genetics of tuberous sclerosis
AD TSC1 chromosome 9 TSC2 chromosome 16
121
Signs of Neurofibromatosis
6 cafe au lait macules 2 neurofibromas Axillary or inguinal freckling 2 Lisch nodules Optic glioma Osseous lesion Pulmonary fibrosis Neuropathy
122
Inheritance of neurofibromatosis
Autosomal dominant
123
Genetics of NF1
Chromosome 17
124
Genetics of NF2
Chromosome 22
125
Neurofibromatosis associated diseases
Phaeochromocytoma Renal artery stenosis
126
Complications of Neurofibromatosis
Epilepsy Sarcomatous changes Scoliosis Mental retardation
127
Absent/diminished ankle/knee jerks Moderately dilated pupil Poorly responsive to light
Holmes-Adie pupil Benign condition seen in females Reassurance
128
Small, irregular pupil Accommodates Does not react to light
Argyll Robertson pupil
129
Argyll Robertson pupil is a sign of
Quaternary syphilis
130
Investigation of syphilis
TPHA or FTA Positive for duration of illness
131
Management of syphilis
Penicillin
132
Causes of oculomotor (III) nerve palsy
MMMM CCC Mononeuritis multiplex Midbrain infarction (Weber’s) Midbrain demyelination Migraine Communicating artery aneurysm Cavernous sinus pathology Cerebral uncus herniation
134
Causes of mononeuritis multiplex
Diabetes mellitus SLE RA Vasculitis Infection (HIV)
135
Top causes of spastic paraparesis
Demyelination Cord compression Trauma MND Cerebral palsy

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