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Neurology Mushkies Flashcards

1
Q

What are 3 signs on inspection of Parkinson’s?

A
  1. Asymmetrical resting tremor (5Hz) exacerbated by reading backwards
  2. Hypomimia
  3. Extrapyramidal posture
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2
Q

Parkinson’s + Nystagmus?

A

MSA

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3
Q

Parkinson’s + Vertical gaze palsy?

A

PSP

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4
Q

What are 4 features of a Parkinsonian gait?

A
  1. Slow initiation
  2. Shuffling
  3. Festination (hurrying)
  4. Absent arm swing
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5
Q

What do you do to complete an examination in a pt with Parkinson’s?

A
  1. MMSE
  2. Drug chart
  3. Abdo exam (hepatomegaly + signs of CLD)
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6
Q

What are causes of Parkinson’s disease?

A
  1. Idiopathic
  2. Parkinson’s plus syndromes = PSP, MSA, CBD, LBD
  3. Vascular = infarcts in the substantia nigra
  4. Wilson’s disease
  5. Drugs = neuroleptics and metoclopramide
  6. Post-encephalitis
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7
Q

What are the 3 cardinal signs of parkinsonism?

A
  1. Rigidity
  2. Brady/akinesia
  3. Tremor
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8
Q

What are 4 autonomic features of parkinsonism?

A
  1. Postural hypotension
  2. Urinary problems (frequency, urgency, nocturia)
  3. Erectile dysfunction
  4. Constipation
  5. Hypersalivation and hyperhidrosis
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9
Q

What are 5 sleep related problems of parkinsonism?

A
  1. Insomnia and
  2. Turning in bed leads to
  3. Excessive Daytime Sleepiness (EDS)
  4. REM behavioural sleep disorder = loss of muscle atonia during sleep leading to violent enactment of dreams
  5. Da SEs = insomnia, drowsiness, EDS
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10
Q

How can you investigate Parkinson’s?

A
  1. Bloods = caeruloplasmin (low in Wilsons)

2. Imaging = CT/MRI (exclude vascular cause), DaTSCAN

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11
Q

What is a DaTSCAN?

A

An Ioflupane I123 injection that binds to dopaminergic neurones and allows visualisation of the substantia nigra

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12
Q

How can you classify the management of Parkinsons?

A
  1. General
  2. Specific
  3. Adjuncts
  4. Other
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13
Q

What is the ‘general’ management of Parkinson’s?

A
  1. MDT = neurologist, PD nurse, physio, OT, social worker, GP and carers
  2. Assess disability = UDPRS
  3. Physio = postural exercises
  4. Depression screening
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14
Q

What is the UDPRS?

A

Unified Parkinson’s Disease Rating Scale

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15
Q

What is the specific management of Parkinson’s? (7 drugs)

A

LDAMCAA

  1. L-DOPA + Carbidopa/benserazide
  2. DA agonists = ropinerole, pramipexole
  3. Apomorphine = SC rescue drug, also a DA agonist
  4. MAO-B inhibitors = rasagiline
  5. COMT inhibitors = tolcapone
  6. Amantadine
  7. Anti-muscarinics = procyclidine
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16
Q

What are 3 adjuncts for treatment of Parkinsons?

A
  1. Domperidone = nausea
  2. Quetiapine = psychosis
  3. Citalopram = depression
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17
Q

What are 2 ‘other’ managements for Parkinsons?

A
  1. Deep brain stimulation

2. Basal ganglia disruption

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18
Q

What is the epidemiology of idiopathic PD?

A

Mean onset 65 y/o

2% prevalence

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19
Q

What is the pathophysiology of PD?

A
  1. Destruction of dopaminergic neurones in the pars compacta of the substantia nigra
  2. Alongside formation of beta amyloid plaques and
  3. Neurofibrillary tangles composed of hyperphosphorylated tau
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20
Q

What are the features of Parkinsonism?

A

TRAPPS PD

  1. Tremor = increased by stress, decreased by sleep
  2. Rigidity = lead-pipe, cog-wheel
  3. Akinesia = 5
  4. Postural instability = stooped gait with festination
  5. Postural hypotension + other autonomic dysfunction
  6. Sleep disorders = insomnia, ED, OSA, RBD
  7. Psychosis = esp. visual hallucinations
  8. Depression/Dementia/Drug SEs
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21
Q

What are the 5 akinetic features of Parkinson’s?

A
  1. Slow initiation
  2. Difficulty with repetitive movement
  3. Micrographia
  4. Monotonous voice
  5. Mask-like face
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22
Q

What are the side effects of L-DOPA?

A 
DOPAMINE
Dyskinesia 
On-off phenomena = motor fluctuations
Psychosis
ABP reduced 
Mouth dryness
Insomnia 
N&V
EDS
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23
Q

What are two type of motor fluctuations you can get in PD?

A
  1. End-of-dose

2. On-Off effect

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24
Q

What is the end-of-dose effect?

A

Deterioration as dose wears off with progressively shorter benefit

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25
What is the on-off effect?
Unpredictable fluctuations in motor performance unrelated to timing of dose
26
What are 4 other causes of parkinsonism?
1. MSA 2. PSP 3. CBD 4. LBD
27
What is the pathology of MSA?
Papp-Lantos bodies = alpha synuclein inclusions in glial cells
28
What are 3 features of MSA?
1. Autonomic dysfunction = postural hypotension 2. Parkinsonism 3. Cerebellar ataxia
29
What might you call MSA if autonomic features predominate?
Shy Drager syndrome
30
What are 4 features of PSP?
1. Postural instability --> falls 2. Vertical gaze palsy 3. Pseudobulbar palsy = speech and swallowing problems 4. Parkinsonism = symmetrical onset, tremor is unusual
31
What are 3 features of CBD?
1. Unilateral parkinsonism esp. rigidity 2. Aphasia 3. Asterogenesis = cortical sensory loss (--> alien limb phenomenon with autonomous arm movements)
32
What is the pathology of LBD?
Alpha synuclein and ubiquitin Lewy bodies in brainstem and neocortex
33
What are 3 features of LBD?
1. Fluctuating cognition 2. Visual hallucinations 3. Parkinsonism
34
What are 4 features of vascular Parkinsonism?
1. Sudden onset 2. Worse in legs than arms 3. Pyramidal signs 4. Prominent gait abnormality
35
How can you classify the causes of tremor?
1. Resting = parkinsonism 2. Intention = cerebellar 3. Postural = worse with arms outstretched
36
What are 5 causes of a postural tremor?
BHATS 1. Benign Essential Tremor 2. Hyperthyroidism 3. Alcohol withdrawal 4. Toxins = beta agonists 5. Sympathetic = anxiety
37
What is benign essential tremor (BET)?
An predominantly autosomal dominant condition that occurs with movement and is worse with anxiety and caffeine. It does not occur with sleep and is improved by alcohol.
38
What are the features of cerebellar syndrome?
``` DANISH Dysdiadochokinesia Ataxia Nystagmus + rapid saccades Intention tremor + dysmetria Slurred speech Hypotonia ```
39
What are the causes of cerebellar syndrome?
DAISIES PT 1. Demyelination (MS) 2. Alcohol/Abscess/Atrophy 3. Infarct/Infection 4. SOL = Schwanomma + other CPA tumours 5. Inherited = Wilsons, Friedrichs, Ataxia Telangiectasia, VHL 6. Epilepsy medications = Phenytoin 7. System atrophy, multiple/Spinocerebellar ataxia 8. Paraneoplastic syndrome 9. Trauma
40
Are cerebellar signs usually ipsilateral or bilateral?
Ipsilateral
41
What are bilateral cerebellar signs more likely to represent?
Global pathology x 3 (PAM) 1. Phenytoin 2. Alcohol 3. MS
42
How does a nystagmus due to a cerebellar cause present?
Fast phase towards lesion, maximal looking towards lesion
43
How does a nystagmus due to a vestibular present?
Fast phase away from lesion, maximal looking away from lesion
44
What causes Lateral Medullary syndrome?
Occlusion of vertebral artery or PICA
45
What is the eponymous name for lateral medullary syndrome?
Wallenberg's syndrome
46
Where do you find signs in lateral medullary syndrome?
Signs are ipsilateral apart from body anaesthesia to pain
47
What are the features of lateral medullary syndrome?
DANVAH 1. Dysphagia = nucleus ambiguus 2. Ataxia = inferior cerebellar peduncle 3. Nystagmus = inferior cerebellar peduncle 4. Vertigo = vestibular nucleus 5. Anaesthesia = spinothalamic tract (contralateral) or spinal trigeminal nucleus (ipsilateral) 6. Horner's syndrome = sympathethic fibres
48
What is a vestibular schwannoma?
A benign, slow growing tumour of the superior vestibular nerve, that is the cause of 80% of CPA tumours, and is associated with NF2
49
How do vestibular schwannomas present?
1. Unilateral SNHL + tinnitus + vertigo 2. Headache (raised ICP) 3. Ipsilateral CN 5,6,7,8 palsies 4. Cerebellar signs = DANISH
50
How can you investigate vestibular schwannomas?
MRI of CPA (cerebellopontine angle)
51
What are the differentials for CPA tumours? x4
1. Vestibular Schwannoma 2. Meningioma 3. Cerebellar astrocytoma 4. Metastases
52
What are 5 features of vHL syndrome?
1. Renal cysts 2. Bilateral renal cell carcinoma 3. Phaeochromocytoma 4. Islet cell tumours 5. Haemangioblastomas (often in cerebellum)
53
What is Friedrich's Ataxia?
An autosomal recessive mitochondrial disorder which leads to progressive degeneration of the dorsal column, spinocerebellar tracts and corticospinal tracts
54
When is the typical onset of Friedrich's Ataxia and what are two of its associations?
1. Onset in teenage years | 2. Associated with HOCM and mild dementia
55
What is Ataxia telangiectasia?
An autosomal recessive disorder leading to defects in DNA repair, leading to progressive ataxia, telangectasia, lymphoproliferative disease and defective cell-mediated immunity and Ab production
56
What causes Wilson's disease?
AR mutation of ATP7B gene on Chromosome 13
57
What are the features of Wilson's disease?
CLANK 1. Cornea = Keiser-Fleischer rings 2. Liver = CLD 3. Arthritis 4. Neuro = Parkinsonism, ataxia, psych problems 5. Kidney = Fanconi's syndrome
58
What causes excessive daytime sleepiness in Parkinsons?
1. Inability to turn 2. Restless legs 3. Early morning dystonia (drugs wearing off) 4. Nocturia 5. OSA
59
2 features of REM behavioural sleep disorder in Parkinsons?
1. Loss of muscle atonia during REM sleep | 2. Violent enactment of dreams
60
What causes autonomic dysfunction in Parkinsons?
Combination effects of drugs and neurodegeneration
61
Cerebellar vermis lesion features?
Ataxis trunk and gait, with normal arms
62
Vestibular Schwannoma mx?
1. Gamma knife | 2. Surgery
63
4 features of Friedrich's Ataxia?
PBLL 1. Pes cavus 2. Bilateral cerebellar ataxia 3. Leg wasting + arreflexia but extensor plantars 4. Loss of vibration and proprioception
64
UMN inspection?
1. Walking aids 2. Disuse atrophy and contractures 3. Leg = extended, internally rotated with foot plantar flexed 4. Arm = flexed, internally rotated, supinated
65
Contracture defn?
A permanent tightening of tissue
66
Unilateral UMN gait?
Circumducting
67
Bilateral UMN gait?
Scissoring
68
Pyramidal distribution of UMN leg weakness?
Extensors stronger than flexors
69
Pyramidal distribution of LMN leg weakness?
Flexors stronger than extensors
70
Causes of bilateral lower limb UMN signs (spastic paraparesis)?
1. Common = MS, cord compression, cord trauma, CP | 2. Other = familial, vascular (Becks), infection (HTLV1), tumour (ependymoma), syringomyelia
71
Beck's syndrome AKA?
Anterior spinal artery syndrome
72
Causes of spastic paraparesis with mixed UMN and LMN signs?
MAST 1. MND 2. Ataxia, Friedrichs 3. SCDC 4. Taboparesis
73
What is taboparesis?
Tabo-paresis is a form of tertiary syphilis which contains features of both tabes dorsalis and general paralysis of the insane
74
Causes of a unilateral, spastic hemiparesis?
1. Hemisphere = stroke, MS, SOL, CP | 2. Hemicord = MS, cord compression
75
Mx of contractures?
1. Baclofen 2. Botulinum injection 3. Physio
76
Features of cord compression?
1. Pain = local, deep, radicular 2. Weakness = LMN @ level, UMN below level 3. Sensory level 4. Sphincter disturbance
77
Causes of cord compression?
1. Trauma = vertebral fracture 2. Disc prolapse 3. Infection = epidural abscess, TB 4. Malignancy = primary or secondary
78
Ix of cord compression?
MRI
79
Mx of cord compression?
A neurosurgical emergency 1. Malignancy = dexamethasone IV, consider chemo/radio/decompressive laminectomy 2. Abscess = Abx and surgical decompression
80
Features of cauda equina lesions?
1. Pain = back pain, radicular pain down legs 2. Weakness = bilateral flaccid, areflexic lower limb weakness 3. Sensation = saddle anaesthesia 4. Sphincters = incontinence, poor anal tone
81
Beck's syndrome (anterior spinal artery syndrome)?
Infarction of the spinal cord in the distribution of the anterior spinal artery, resulting in loss of function of the anterior two-thirds of the spinal cord
82
Causes of Beck's syndrome?
Aortic aneurysm dissection or repair
83
Features of Beck's syndrome?
1. Para/quadriparesis 2. Impaired pain and temperature sensation 3. Preserved touch and proprioception
84
Syringomyelia defn?
Disorder in which a cyst or cavity forms within the spinal cord (syrinx = tubal cavity in central canal of the cord)
85
Where is a syrinx commonly located?
Cervical cord
86
In which direction does a syrinx expand, and thus what does it affect?
Expands ventrally, affecting: 1. Decussating spinothalamic neurones 2. Anterior horn cells 3. Corticospinal tracts
87
Causes of syringomyelia?
1. Blocked CSF circulation with reduced flow from posterior fossa = Arnold-Chiari malformation, masses 2. Spina bifida 3. Trauma 4. Myelitis 5. Cord tumours 6. AVMs
88
What is an Arnold-Chiari malformation?
When the cerebellum herniates through the foramen magnum
89
4 cardinal signs of Syringomyelia?
1. Dissociated sensory loss 2. Wasting/weakness of hands +/- claw hand 3. Loss of reflexes in upper limb 4. Charcot joints (elbow and shoulder)
90
Dissociated sensory loss in syringomyelia?
1. Loss of pain and temperature --> scars from burns 2. Preserved touch, proprioception and vibration 3. Root distribution reflects syrinx location (usually affects upper limbs and chest: 'cape')
91
'Other' Syringomyelia signs?
1. UMN weakness in lower limbs with extensor plantars 2. Horners 3. Syringobulbia 4. Kyphoscoliosis
92
Infectious cause of cord disease?
HTLV1 myelopathy (Tropical Spastic Paraplegia)
93
Stroke pathogenesis?
1. Ischaemic (80%) = atheroma or embolus | 2. Haemorrhagic (20%)
94
Bamford stroke classification?
1. TACS 2. PACS 3. POCS 4. LACS
95
TACS?
Carotid/MCA and ACA territory 1. Hemiparesis and/or sensory deficit 2. Homonymous hemianopia 3. Higher cortical dysfunction a. Dominant = aphasia b. Non-dominant = neglect, apraxia
96
PACS?
2/3 of: 1. Hemiparesis and/or sensory deficit 2. Homonymous hemianopia 3. Higher cortical dysfunction
97
POCS?
``` Vertebrobasilar territory 1 of: 1. Brainstem or cerebellar syndrome 2. LOC 3. Isolated homonymous hemianopia ```
98
LACS?
Infarct around basal ganglia, internal capsule, thalamus and pons 1. Pure sensory stroke 2. Pure motor stroke 3. Sensorimotor stroke 4. Ataxic hemiparesis
99
Mx of Stroke?
1. Resus 2. Monitor 3. bloods 4. Imaging 5. Medical 6. Surgery 7. Stroke unit 8. Secondary prevention 9. Rehabilitation
100
Stroke resus?
1. Airway patent, consider NGT 2. NBM until swallow assessment by SALT 3. Dont overhydrate, risk of cerebral oedema 4. BM: exclude hypogylcaemia
101
Stroke monitoring?
1. Glucose = 4-11mM, sliding scale if DM 2. BP = Rx of HTN can reduce cerebral perfusion 3. Neuro obs
102
Stroke bloods?
1. FBC = sepsis may --> stroke 2. U&E = e- disturbance may mimic stroke 3. Glucose = exclude hypoglycaemia 4. Clotting = high or low INR may indicate cause
103
Stroke imaging?
1. CT head | 2. Diffusion weighted MRI
104
Medical stroke Mx?
1. Consider alteplase if <4.5hrs since onset of Sx | 2. Aspirin 300mg PO/PR once haemorrhagic stroke excluded +/- PPI
105
Stroke surgical Mx?
1. Neurosurgical opinion if intracranial haemorrhage 2. May coil bleeding aneurysms 3. Decompressive hemicraniectomy for some forms of MCA infarction
106
Stroke unit?
1. Specialist nursing and physio 2. Early mobilisation 3. DVT prophylaxis
107
Stroke work-up?
1. ECG +/- 24hr tape 2. Bloods = FBC, U&E, Glucose, Lipids, clotting and thrombophilia screen, vasculitis (ESR, ANA) 3. Imaging = CXR, Carotid doppler, echo
108
Thrombophilia screen?
1. FBC, clotting, fibrinogen conc. 2. APC resistance/FV Leiden 3. Lupus anticoagulant 4. Anti-cardiolipin Abs 5. Protein C and S and AT3 activity assays 6. PCR for prothrombin gene mutation
109
Stroke secondary prevention/
1. Statin after 48hrs 2. Aspirin/clopi for 2 weeks after stroke and then a. Clopidogrel 75mg OD OR b. Aspirin 75mg OD + Dipyramidole MR 200mg BD 3. Warfarin instead of asp/clopi if cardioembolic stroke/chronic AF, start from 2 weeks post-stroke 4. Carotid endarterectomy if good recovery + ipsilateral stenosis >=70%
110
Stroke rehab?
MENDS 1. MDT = physio, SALT, dietician, OT, specialist nurses, neurologist, family 2. Eating = swallow screen, malnutrition screen (MUST) 3. Neurorehab = physio and SALT 4. DVT prophylaxis 5. Sores = avoided
111
MS defn?
A chronic inflammatory demyelinating disorder of the CNS characterised by two or more lesions disseminated in time and space
112
MS epidemiology?
1. Lifetime risk = 1/1000 2. Age = mean of 30 y/o 3. Sex = 3F:1M 4. Rarer in blacks
113
MS Aetiology?
1. Genetic = HLA-DRB1, HLA DR2, Il2, IL7 | 2. Environmental = Latitude, EBV
114
MS Pathophysiology?
1. CD4 cell mediated destruction of oligodendrocytes --> demyelination and eventual neuronal death 2. Initial viral inflammation primes humoral Ab response vs. MBP 3. Plaques of demyelination are hallmark
115
Classification of MS?
1. Relapsing Remitting (80%) 2. Primary Progressive 3. Secondary Progressive 4. Progressive Relapsing
116
MS presentation?
TEAM 1. Tingling 2. Eye = optic neuritis (decreased central vision and eye movement pain) 3. Ataxia + other cerebellar signs 4. Motor = usually spastic paraparesis
117
Clinical features of MS?
1. Eye = Optic neuritis, INO, RAPD 2. Sensory = paraesthesia, reduced vibration sense, trigeminal neuralgia 3. Motor = spastic weakness, transverse myelitis 4. Cerebellum = cerebellar features 5. GI = swallowing disorders, constipation 6. Sexual = ED + anorgasmia, retention, incontinence
118
Lhermitte's sign?
Neck flexion --> electric shocks in trunk/limbs
119
Optic neuritis clinical features?
1. Pain on movement, rapid decrease in central vision 2. Uhthoff's = vision decreases with heat 3. O/E = reduced acuity, reduced colour vision, white disc, central scotoma, RAPD
120
Internuclear ophthalmoplegia?
A disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction, and nystagmus in the unaffected eye
121
INO cause?
Lesion to the MLF connecting CN6 to CN3
122
MS Ix?
1. CSF = oligoclonal bands 2. MRI = periventricular white matter plaques 3. VEPs = delayed velocity but normal amplitude 4. Abs = anti-MBP, NMO-IgG
123
NMO-IgG AKA and for what condition?
Anti-AQP4, for Devic's disease
124
Devic's disease aka?
Neuromyelitis optica
125
Neuromyelitis optica?
A heterogeneous condition consisting of the inflammation and demyelination of the optic nerve (optic neuritis) and the spinal cord (myelitis). It can be monophasic or recurrent. Currently at least three different kinds are proposed based on the presence of autoantibodies thought to produce the disease: anti-AQP4, anti-MOG and anti-NF.
126
3 inflammatory conditions that may mimic MS plaques?
1. CNS sarcoidosis 2. SLE 3. NMO
127
MS acute attack Mx?
1. Methylprednisolone 1g IV/24hr for 3 days | 2. Doesnt influence long term outcome, decreases duration and severity of attacks
128
MS relapse preventers?
1. IFNB 2. Natalizumab = anti-VLA4 Ab 3. Alemtuzumab = anti-CD52
129
MS symptomatic tx?
1. Acute = methylprednisolone 2. Antispasmodics = baclofen, botulinum 3. Neuropathic pain = carbamazepine 4. Depression = SSRI 5. ED = sildafenil 6. Tremor = clonazepam 7. Laxatives and intermittent catheterisation
130
MND Examination?
1. Inspection = wasting and fasciculation 2. Tone = spastic 3. Power = weak 4. Reflexes = absent and/or brisk (e.g. absent knee jerks with extensor plantars) 5. Sensation = normal 6. Completion = speech, jaw-jerk, eye movements
131
MND eyes?
MND does not involve the eyes
132
MND speech?
1. Bulbar = nasal | 2. Pseudobulbar = hot potato
133
MND jaw-jerk?
1. Bulbar = absent | 2. Pseudo-bulbar = brisk
134
MND DDx?
1. Cervical cord compression --> myelopathy 2. Brainstem lesions 3. Polio = asymmetrical LMN paralysis 4. Mixed UMN and LMN signs
135
Mixed UMN and LMN signs?
MAST 1. MND 2. Ataxia, Friedrichs 3. SCDC: b12 4. Taboparesis
136
MND Ix?
1. LP = exclude inflammatory cause 2. Brain/cord MRI = exclude structural cause 3. EMG = fasciculation
137
MND diagnostic criteria?
Revised El Escorial criteria
138
MND Mx?
1. General 2. Specific 3. Supportive
139
MND General Mx?
1. MDT | 2. Discussion of end-of-life decisions = Advanced directive, DNAR
140
MND Specific Mx?
Riluzole = antiglutaminergic that prolongs life by approx. 3m
141
MND Supportive Mx?
DR DPS 1. Drooling = amitryptiline 2. Resp. failure = NIV 3. Dysphagia = NG/PEG feeding 4. Pain = analgesic ladder 5. Spasticity = baclofen, botulinum
142
MND prognosis?
Most die within 3 years due to bronchopneumonia and respiratory failure
143
MND classification?
1. Amyotrophic Lateral Sclerosis (50%) 2. Primary Lateral Sclerosis (30%) 3. Progressive Muscular Atrophy (10%) 4. Progressive Bulbar Palsy (10%)
144
Amyotrophic lateral sclerosis?
Disorder of corticospinal tracts --> UMN and LMN signs and fasciculation
145
Primary lateral sclerosis?
1. Loss of Betz cells in motor cortex --> mainly UMN signs 2. Marked spastic leg weakness and pseudobulbar palsy 3. No cognitive decline
146
Progressive muscular atrophy?
1. Anterior horn cell lesion --> LMN signs only 2. Distal to proximal 3. Better prognosis cf. ALS
147
Progressive bulbar palsy?
Only affects CN 9-12 --> bulbar palsy
148
LMN pathology?
Anywhere from anterior horn cell to muscle itself
149
LMN signs?
1. Inspection = wasting, fasciculations 2. Tone = reduced 3. Power = weak 4. Reflexes = hyporeflexia
150
Bilateral, symmetrical, distal LMN signs name?
Motor peripheral polyneuropathy
151
Motor peripheral polyneuropathy DDx?
1. HSMN 2. Paraneoplastic 3. Lead poisoning 4. Acute = GBS, Botulism
152
Bilateral, symmetrical, proximal LMN signs name?
Proximal myopathy
153
Proximal myopathy DDx?
1. Inherited = muscular dystrophy 2. Inflammation = polymyositis, dermatomyositis 3. Endocrine = Cushings, Acromegaly, Thyrotoxicosis, Osteomalacia, Diabetic Amyotrophy 4. Drugs = alcohol, statins, steroids 5. Malignancy = paraneoplastic
154
Unilateral LMN signs isolated to single limb with no sensory signs?
Old polio
155
Unilateral LMN signs localised to group of muscles with same supply?
1. Segmental = nerve roots, plexus | 2. Peripheral = mononeuropathy
156
Hand wasting DDx?
1. Anterior horn = syringomyelia, MND, polio 2. Roots (C8-T1) = spondylosis 3. Brachial plexus = compression (cervical rib), avulsion (Klumpke's palsy) 4. Neuropathy = Generalised (HSMN), mononeuritis multiplex (DM), compressive mononeuropathy 5. Muscle = disuse (RA), distal myopathy (myotonic dystrophy)
157
Proximal myopathy Ix?
1. Bloods = DM, muscle damage (CK, ESR, LDH), Endo (TSH, Ca, 9am cotrisol, IGF1), Abs (anti-Jo1) 2. CXR = paraneoplastic 3. EMG 4. Genetic analysis 5. Muscle biopsy
158
Mononeuropathy Ix?
1. Bloods = DM, B12, Folate, vasculitis (ESR, ANA, ANCA) | 2. EMG + Nerve conduction
159
Radiculopathy Ix?
MRI
160
Peripheral polyneuropathy causes?
1. Mainly sensory | 2. Mainly motor
161
Mainly sensory peripheral polyneuropathy cause?
1. DM 2. Alcohol 3. B12 4. CKD 5. Ca (paraneoplastic) 6. Vasculitis 7. Drugs = isoniazid, vincristine
162
Mainly motor peripheral polyneuropathy?
1. HMSN (CMT) 2. Paraneoplastic 3. Lead poisoning 4. Acute = GBS and botulism
163
Peripheral polyneuropathy Ix?
1. Bedside = dipstisk, glucose 2. Bloods = DM, B12, FBC, U&E, LFT, TFTs, vasculitis 3. CXR = paraneoplastic 4. Nerve conduction studies 5. EMG 6. Genetic = PMP22 in CMT 7. Nerve biopsy
164
Nerve conduction study findings and interpretations?
1. Demyelination --> reduced conduction speed | 2. Axonal degeneration --> decreased conduction amplitude
165
DM examination of LL findings?
1. Bilateral loss of ankle jerks secondary to sensory neuropathy 2. Mononeuritis multiplex --> foot drop
166
Femoral neuropathy due to DM?
Painful asymmetric weakness and wasting of the quads with loss of knee jerks
167
5 features of diabetic autonomic neuropathy?
1. Postural hypotension 2. Gastroparesis 3. Diarrhoea 4. Urinary retention 5. ED
168
Charcot-Marie-Tooth syndrome aka?
1. Hereditary Motor and Sensory Neuropathy | 2. Peroneal Muscular Atrophy
169
CMT syndrome examination?
1. Inspection = pes cavus, symmetrical distal muscle wasting, thickened nerve (esp. common peroneal around fibula) 2. Motor = high stepping gait due to foot drop, weak foot and toe dorsiflexion, absent ankle jerks 3. Sensory = variable loss of sensation in a stocking distribution
170
What is CMT?
A group of inherited motor and sensory neuropathies 1. HMSN1 = most common form, demyelinating, AD mutation in PMP22 gene 2. HMSN2 = second commonest form, axonal degeneration, AD
171
CMT syndrome nerve conduction studies?
1. HMSN1 = demyelinating = reduced conduction velocity | 2. HMSN 2 = axonal degeneration = reduced conduction amplitude
172
Mx of CMT syndrome?
Supportive 1. MDT 2. Foot care and careful shoe choice 3. Orthoses = e.g. ankle brace
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Myasthenia Gravis exam?
1. Inspection = thymectomy scar 2. Eyes = bilateral ptosis worse on sustained upward gaze, complex ophthalmoplegia 3. Facial movements = myasthenic snarl on smiling 4. Voice = nasal, fatigue (count backwards) 5. Limbs = fatiguability (flap arm) 6. Completion = resp function (spirometry)
174
Myasthenia Gravis Ix?
1. Bloods = Anti-AChR, Anti-MuSK, TFTs 2. Tensilon test (improvement with edrophonium, an anticholinesterase) --> can cause heart block and even asystole 3. EMG = decremented response to a titanic train of impulses 4. CT mediastinum
175
Myasthenia associations?
1. <50 y/o, female = AI --> DM, SLE, RA, Graves | 2. >50 y/o, male = Thymoma
176
Myasthenia Mx?
1. Acute = Plasmapharesis/IVIG, consider ventilation | 2. Chronic = Pyridostigmine, Immunosuppression (steroids and azathioprine), thymectomy (benefit even if no thymoma)
177
LEMS?
1. Abs vs. VGCC 2. Often paraneoplastic e.g. SCLC 3. Lower limb girdle weakness 4. Weakness improves upon repetitive testing
178
DDx for bilateral ptosis?
1. MG 2. Myotonic dystrophy 3. Congenital 4. Senile 5. Bilateral Horner's
179
GBS classification?
1. AIDP = acute inflammatory demyelinating polyneuropathy | 2. Miller fisher = ophthalmolplegia + ataxia + areflexia
180
Pathophysiology of GBS?
1. Molecular mimicry: Abs cross react with ganglioside 2. Bacteria = C.jejuni, mycoplasma 3. Viruses = CMV, EBV
181
Features of GBS?
1. Symmetrical ascending flaccid paralysis 2. Sensory disturbance = paraesthesia 3. Autoimmune neuropathy = labile BP
182
GBS Ix?
1. Bedside = stool MC + S 2. Bloods = Infection, anti-ganglioside Abs 3. LP = raised CSF protein 4. Nerve conduction studies = demyelination
183
GBS Mx?
1. Supportive = 4As 2. Immunosuppression = IVIG, Plasma exchange 3. Physio = prevent flexion contractures
184
GBS supportive Mx?
4As 1. Airway/ventilation = ITU if FVC <1.5L 2. Analgesia = NSAIDs, gabapentin 3. Autonomic = inotropes, catheter 4. Antithrombotic = TEDS, LMWH
185
GBS prognosis?
1. 85% complete recovery 2. 10% unable to walk alone at 1 year 3. 5% mortality
186
Facial nerve palsy examination?
1. Inspection | 2. Weakness
187
Facial nerve palsy examination on inspection?
1. Unilateral facial droop 2. Absent nasolabial fold 3. Absent forehead creases 4. Scar or parotid mass 5. Ear rash
188
Facial nerve palsy weakness examination?
1. Raising eyebrows = frontalis 2. Screwing up eyes = orbicularis oculi (Bell's sign = eyeball rolls back on closure) 3. Smiling = orbicularis oris
189
UMN or LMN facial palsy?
UMN --> sparing of frontalis and orbicularis oculi due to bilateral cortical representation
190
Millard-Gubler syndrome?
Due to unilateral lesion in ventral pons 1. CN6 nucleus --> ipsilateral lateral rectus palsy 2. CN7 nucleus --> ipsilateral LMN facial palsy 3. Corticospinal tracts --> contralateral hemiparesis
191
CPA lesions?
Ipsilateral CN 5,6,7,8 palsies and cerebellar signs 5. Facial anaesthesia and absent corneal reflex 6. Lateral rectus palsy 7. LMN facial nerve palsy 8. SNHL Cerebellar. DANISH
192
Causes of facial nerve palsy?
1. Idiopathic Bell's Palsy 2. Supranuclear = vascular, SOL, MS 3. Pontine = vascular, SOL, MS 4. CPA = vestibular schwannoma, meningioma, mets 5. Intra-temporal = Ramsay-Hunt, cholesteatoma, trauma 6. Infra-temporal = Parotid tumour, trauma 7. Systemic a. Neuropathy = DM, Lyme, Sarcoidosis b. Pseudopalsy = MG
193
5 causes of bilateral facial nerve palsy?
1. Infection = Lyme 2. Inflammation = GBS, Sarcoidosis 3. Bilateral Bell's 4. Pseudopalsy = MG, myotonic dystrophy
194
Hyperacusis path in facial nerve palsy?
1. Chorda tympani and nerve to stapedius arise just distal to geniculate ganglion w/in the temporal bone 2. Hyperacusis thus indicates a proximal lesion 3. Common in Ramsay Hunt due to VZV at geniculate ganglion
195
Facial Nerve Palsy Ix?
1. Bedside = urine dip for glucose 2. Bloods = DM, VZV serology, Lyme serology, anti-AChR 3. Imaging = MRI posterior cranial fossa 4. Pure tone audiometry 5. LP = exclude infection 6. Nerve conduction studies = may predict delayed recovery when performed @ 2 weeks
196
Facial nerve palsy Mx?
1. Prednisolone w/in 72hrs 2. Valaciclovir if VZV suspected 3. Protect eye = dark glasses, artificial tears, tape closed @ night
197
Facial nerve palsy prognosis?
1. Incomplete paralysis = recovers completely w/in weeks 2. Complete paralysis = 80% have full recovery, remained have delayed recovery/permanent neurological/cosmetic abnormalities
198
Complications of facial nerve palsy?
1. Synkinesis = blinking causes upturning of mouth | 2. Crocodile tears syndrome = eating stimulates unilateral lacrimation, not salivation
199
4 key causes of facial nerve palsy?
1. Bell's Palsy 2. Ramsay Hunt Syndrome 3. Cholesteatoma 4. Lyme disease
200
Bell's palsy quick facts?
1. Idiopathic facial nerve palsy responsible for 75% 2. Is a Diagnosis of exclusion 3. Inflammatory oedema --> compression of CN7 in narrow facial canal 4. Probably of vial origin (HSV1)
201
Features of Bell's palsy?
1. Sudden onset 2. Complete LMN facial palsy 3. Ageusia = corda tympani 4. Hyperacusis = stapedius 5. Associated with other CN involvement in 8%
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Ramsay Hunt syndrome defn?
Facial nerve palsy caused by reactivation of VZV in the geniculate ganglion of CNVII
203
Ramsay Hunt syndrome features?
1. Preceding ear pain or stiff neck 2. Vesicular rash in auditory canal +/- TM, pinna, tongue, hard palate 3. Ipsilateral facial nerve weakness, ageusia, and hyperacusis 4. May affect CN8 --> vertigo, tinnitus, deafness
204
Ramsay Hunt mx?
Prednisolone and valaciclovir w/in 72hrs
205
Ramsay Hunt prognosis?
1. Rxed w/in 72hrs = 75% full recovery | 2. Otherwise: 1/3rd recover, 1/3rd partial, 1/3rd poor
206
Cholesteatoma defn?
1. Locally destructive expansion of stratified squamous epithelium within the middle ear 2. Usually secondary to attic perforation in chronic suppurative otitis media
207
Cholesteatoma presentation?
1. Foul smelling white discharge 2. Vertigo, deafness, headache, pain, facial paralysis 3. Appears pearly white with surrounding inflammation
208
3 complications of cholesteatoma?
1. Deafness (ossicle destruction) 2. Meningitis 3. Cerebral abscess
209
Early features of lyme disease?
1. Erythema chronicum migrans | 2. Systemic malaise
210
Late features of lyme disease?
1. Neuro = facial palsy, polyneuropathy, meningoencephalitis 2. Heart = myocarditis, heart block 3. Joints = Arthritis
211
Causes of facial anaesthesia?
1. Supranuclear = Infarct, MS, SOL 2. Nuclear = CPA lesion, lateral medullay syndrome 3. Peripheral mononeuropathy (DM, sarcoid, vasculitis, cavernous sinus)
212
Horners examination?
1. Face = miosis, ptosis, anhydrosis, enophthalmos 2. Neck = central lines/carotid endarterectomy 3. Hands = complete claw hand + intrinsic hand weakness, decreased/absent sensation in T1 3. Completion = cerebellum, CNs, PNS
213
Horners defn?
AKA oculosympathetic paresis, is a combination of symptoms that arises when the sympathetic trunk is damaged
214
Horners DDx?
1. Central = MS, Wallenberg's 2. Preganglionic (neck) = Pancoast's tumour (T1 nerve root lesion), Trauma (CVA insertion or carotid endarterectomy) 3. Post-ganglionic = Cavernous sinus thrombosis (usually 2ary to spreading facial infection via the ophthalmic veins --> CN 3,4,5,6 palsies)
215
3rd nerve palsy features?
1. Ptosis 2. Down and out 3. Fixed, dilated pupil 4. Ophthalmoplegia and diplopia
216
Medical 3rd nerve palsy?
1. MS 2. Mononeuritis multiplex (Mellitus) 3. Midbrain infarct 4. Migraine
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Surgical 3rd nerve palsy?
1. Cavernous sinus thrombosis 2. Posterior communicating artery aneurysm 3. Cerebral uncal herniation 4. Cancer
218
Holmes-Adie (Myotonic) Pupil?
1. Dilated pupil that has no response to light and sluggish response to accomodation 2. Reduced or absent ankle and knee jerks 3. Benign condition, more common in young females
219
Argyll-Robertson Pupil features?
1. Small, irregular pupils 2. Accommodate but doesnt reaction to light 3. Atrophied and depigmented iris
220
Causes of Argyll-Robertson pupil?
1. Quaternary syphilis | 2. DM
221
Marcus Gunn pupil aka?
RAPD
222
RAPD features?
1. Minor constriction to direct light | 2. Dilatation on moving light from normal to abnormal eye
223
Features of optic atrophy?
1. Decreased visual acuity 2. Decreased colour vision 3. Central scotoma 4. Pale optic disc 5. RAPD
224
Causes of RAPD?
CAC VISION 1. Congenital = Leber's hereditary optic neuropathy, CMT, Friedrich's, DIDMOAD 2. Alcohol and Toxins = ethambutol, lead, B12 def. 3. Compression = neoplasia, glaucoma, Paget's 4. Vascular = DM, GCA, thromboembolic 5. Inflammatory = Optic neuritis = MS, Devic's, DM 6. Sarcoid = or other inflammatory 7. Infection = HZV, TB, syphilis 8. Oedema = papilloedema 9. Neoplastic infiltration = lymphoma, leukaemia
225
DIDMOAD?
Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness
226
Visual pathway?
1. Retina 2. Optic nerve 3. Optic chiasm 4. Optic tract 4. LGN of thalamus 5. Optic radiation a. superior field = temporal b. inferior field = parietal 6. Visual cortex
227
Visual pathway Ix?
1. Perimetry | 2. CT/MRI brain
228
Homonymous hemianopia quick facts?
1. Retrochiasmatic 2. Greater defect = larger lesion or closer to chiasm 3. Contralateral
229
Homonymous hemianopia extra examination?
1. Examine for ipsilateral hemiparesis 2. Examine for cerebellar signs 3. Right = test for neglect 4. Left = test for aphase
230
How does MCA stroke affect vision?
MCA supplies optic radiation in the temporal and parietal lobes
231
How does PCA stroke affect vision?
PCA supplies occipital love and visual cortex --> homonymous hemianopia with macula sparing
232
Causes of homonymous hemianopia?
1. Vascular = ischaemia or haemorrhage 2. SOL = tumour, abscess 3. MS
233
Bitemporal hemianopia lesion?
Chiasmatic lesion
234
3 conditions to look for with bitemporal hemianopia?
1. Prolactinoma 2. Acromegaly 3. Cushings
235
Causes of bitemporal hemianopia?
1. Pituitary tumour = from below = descending visual loss | 2. Craniopharyngioma = from above = ascending visual loss
236
What is a craniopharyngioma?
A benign suprasellar tumour originating from Rathke's pouch, that calcifies as it arises from the odontogenic epithelium
237
How to check for monocular blindness?
Check counting fingers, movement and light perception
238
Cause of monocular blindness?
Lesion proximal to optic chiasm 1. Eye itself = cornea, vitreous, retina 2. Optic nerve = e.g. optic neuropathy
239
Ophthalmoplegia defn?
Weakness or paralysis of one of the extraocular muscles
240
Ophthalmoplegia exam?
1. Inspection = ptosis, alignment, pupil sizes 2. Ask pt to tell you if they get double vision 3. H test = ophthalmoplegia, nystagmus, diplopia (do cover test) 4. Saccades = vertical and horizontal
241
Diplopia key facts?
1. Maximal in direction of pull of affected muscle | 2. Cover test = outer image disappears with affected eye
242
3rd nerve palsy?
1. Ptosis 2. Down and out at rest 3. Fixed, dilated pupil 4. Diplopia maximal = up and in
243
4th nerve palsy?
1. Slight head tilt = ocular torticollis 2. Normal at rest 3. Failure to depress eye during adduction 4. Diplopia maximal = down and in
244
6th nerve palsy?
1. Normal in resting position 2. Failure to abduct 3. Diplopia maximal in abduction 4. Commonly a false localising sign of raised intracranial pressure (contralateral lesion)
245
Causes of simple eye nerve palsies?
1. CNS = Vascular, MS, SOL | 2. Peripheral = DM, compression, trauma
246
Ophthalmoplegia Ix?
1. Bedside = urine dip for glucose 2. Bloods = glucose and HbA1c 3. Imaging = MRI brain
247
4 causes of Internuclear ophthalmoplegia?
1. MS 2. Infarct = ischaemic or haemorrhagic 3. Syringomyelia 4. Phenytoin toxicity
248
Complex ophthalmoplegia?
Diagnosis of exclusion when ophthalmoplegia doesnt fit a single pattern
249
Causes of Complex ophthalmoplegia?
1. DM 2. MS 3. MG 4. Thyrotoxicosis
250
Complex ophthalmoplegia Ix?
1. Bedside = urine dip for glucose 2. Bloods = BMs, TFTs, anti-AChR 3. MRI brain = plaques in periventricular white matter
251
Hearing loss tuning fork?
512hz
252
Rinne's test positive?
AC > BC (Normal)
253
Rinne's test negative?
BC > AC 1. true = conductive deafness 2. false = complete SNHL
254
Weber's test normal?
Central
255
Weber's test SNHL?
Lateralises to normal ear
256
Weber's test CHL?
Lateralises to abnormal ear
257
Causes of conductive hearing loss?
Impaired conduction anywhere b/w auricle and round window 1. Canal obstruction = wax, FB 2. TM perforation = trauma, infection 3. Ossicle defects = otosclerosis, infection 4. Fluid in middle ear
258
Causes of sensory hearing loss?
Defects of cochlea, cochlear nerve, or brain 1. Congenital 2. Acquired
259
Congenital SNHL?
1. Alports = SNHL + haematuria | 2. Jervell Lange-Nielsen = SNHL + LQT
260
Acquired SNHL?
1. Presbyacusis 2. Drugs = gentamicin, vancomycin 3. Infection = meningitis, measles 4. Tumour = vestibular schwannoma
261
Speech test components?
1. Quick screen 2. Dysphonia 3. Dysarthria 4. Dysphasia
262
Dysphonia?
Impaired production of voice sounds
263
Dysarthria?
Impaired articulation of sound into words
264
Dysphasia?
Impairment of language
265
Quick screen for speech exam?
How did you get here today? 1. Listen to volume, rhythm, clarity and content 2. Any striking abnormality?
266
Dysarthria?
1. Repetition a. Yellow lorry = lingual sounds b. Baby hippopotamus = labial sounds c. The Leith police dismisseth us = multiple processes 2. Count to 30 = muscle fatigue in MG
267
Dysphonia?
1. Say ahh = vocal cord tension 2. Cough = bovine 3. Voice = quiet or hoarse
268
Dysphasia?
1. Name 3 animals = nominal dysphasia 2. 3 step command = receptive dysphasia 3. Repeat sentence = conductive dysphasia
269
Dominant parietal lobe speech lesion additional exams?
1. Dyslexia = read a paragraph | 2. Dysgraphia = write a sentence
270
Causes of dysphonia?
1. Vocal cord pathology = laryngitis, tumour, nodule | 2. Recurrent laryngeal nerve palsy
271
Dysarthria pathophysiology?
Lesion in tongue, lips, mouth or disruption of NM pathway 1. Bulbar = unilateral UMN weakness, palatal weakness --> donald duck speech 2. Pseudobulbar = bilateral UMN lesions --> spastic dysarthria, difficult lingual sounds --> hot potato speech, brisk jaw jerk
272
Bulbar dysarthria causes?
1. Brainstem infarct 2. MND 3. GBS
273
Pseudobulbar dysarthria causes?
1. CVA e.g. bilateral internal capsule infarcts 2. MND 3. MS
274
Dysphasia causes?
1. Expressive = Broca's, frontal lobe, non-fluent speech, comprehension intact 2. Receptive = Wernicke's, temporal lobe, fluent but meaningless speech, comprehension impaired 3. Conductive = Arcuate fasiculus, comprehension intact, unable to repeat words or phrases
275
Parkinsons deep brain stimulation happens to structures?
Subthalamic nucleus or globus pallidus

Medicine and Surgery Mushkies (52 decks)

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