Neurology Flashcards
1
Q
What is pyramidal weakness
A
Power seen in UMN lesions
Extension weakness in UL and flexor weakness in LL
2
Q
Earliest sign seen in UMN lesions
A
Plantars
UMN lesions initally look like LMN lesions in hyperacute stage
3
Q
Lentiform nucleus includes
A
Globus pallidus and putamen
4
Q
Frontal lobe features
A
Primary motor cortex - dictates movement Personality Primitive reflexes Dysphasia Expressive (dominant) Anosmia Optic nerve compression Gait apraxia (gait memory)
5
Q
Parietal Lobe features
A
Primary sensory cortex Gerstmann syndrome Sensory, visual, spatial inattention Construction and dressing apraxia Lower Quadrantanopia
6
Q
Temporal lobe features
A
Pimary auditory cortex
REceptive dysphasia (dominant)
MEmory loss - hippocampus and amygdala
Upper quadrantanopia
7
Q
Occipital Lobe Features
A
Homonymous Hemianopia
Anton’s Syndrome
Alexia without agraphia
8
Q
Gerstman Syndrome
A
Dominant angular gyrus lesion
Acalculia, graphia, L-R disorientation, fingeragnosia (ALF)
9
Q
Anton’s Syndrome
A
Bilateral occipital cortical lesion
Cortical blindness with confabulation
10
Q
PITS
A
Parietal inferior quadrantanopia
Temporal superior quadrantanopia
11
Q
Non- fluent/Expressive/Broca’s/Transocrtical motor aphasia
A
Able to comprehend, paucity of words, know what they want to say, but cant
+++Frustration
Dominant frontal lobe lesion
12
Q
Fluent, repetitive, wernickes, transcortical sensory aphasia
A
Unable to comprehend, fluent but incomprehensible speech
Like a foreign language
Dominant temporal lobe lesion
13
Q
Conduction aphasia
A
Mix between broncas an wernickes aphasia
-Able to comprehend with elements of fluent aphasia and poor repetition
Arcuate fasciculus lesion (connection between the 2 areas)
14
Q
Most important part of the internal capsule and why
A
Posterior limb and Genu
-Carry the corticospinal trcts and some sensory fibres
15
Q
Blood supply of the internal capsule
A
LEnticulostriate arteries which are penetrating branches of MCA (M1)
-Common areas of atherosclerosis
16
Q
Stroke of lenticulostriate arteries in internal capsule presentation
A
pure motor stroke
Dense weakness, nil cortical signs
17
Q
Thalamus Rule of 4s: Anterior nuclei
A
language and memory function (frontal/temporal input)
18
Q
Thalamus Rule of 4s: Lateral nuclei
A
motor and sensory function
19
Q
Thalamus Rule of 4s: Medial nuclei
A
maintaining arousal and memory (midbrain)
20
Q
Thalamus Rule of 4s: Posterior nuclei
A
visual function
21
Q
Issue with bilateral thalamic lesions
A
significant arousal issues
22
Q
Thalamic stroke classical presentation
A
Pure sensory loss stroke
Terminal area for all sensory nerves
23
Q
Arterial supply of thalamus
A
PCA
24
Q
Cerebellum: Vermis lesion
A
Truncal ataxia
Nystagmus
25
Cerebellum: Hemisphere lesion
```
Ipsilateral limb ataxia
Past pointing
Dysmetria
Intention tremor
Dysdiadokinesia
Nystagmus
```
26
Brain stem Rule of 4's
4 Cranial nerves in the medulla, pons, and above pons
4 structures in the midline beginning with M
--Motor pathway, Medial lemniscus, medial longitudinal fasciculus, motor nucleus
4 structures on the side beginning with S
--Spinocerebellar pathway, spinothalamic pathway, sensory nucleus of 5th CN, Sympathetic tract
4 motor nuclei in the midline that divide equally into 12 (3.4.6.12)
27
Horner's syndrome features
miosis, ptosis, anhidrosis
Points of terminals: C8/T1, then past lung apex, then superior cervical ganglion by bifurcation of ICA and ECA where sweat gland goes with ECA, then to eye along with ICA
28
CN 1
Olfactory
| -Smell
29
CN 2
Optic
| -Vision, afferent pathway for pupil
30
CN 3
Oculomotor
- Superior, inferior, medial rectus, inferior oblique, levetor palpebrae
- Efferent pathway for pupil
31
CN 4
Trochlear
| -Superior oblique (depression, most in adduction, and intorsion)
32
CN 5
Trigeminal
| -Facial sensation, muscles of mastication
33
CN 6
Abducens
| -LAteral rectus
34
CN 7
Facial
- Muscles of expression, stapedius
- Sensation of anterior 2/3 of tongue
35
CN 8
Vestibulocochlear
| -Hearing and balance
36
CN 9
Glossopharengeal
- Sensation: middle ear, posterior 1/3 tongue
- Some swallowing
37
CN 10
Vagus
- Sensation of pharynx, larynx, oesphagus thoracic and abdominal viscera
- Motor: soft palate, larynx, pharynx
38
CN 11
Accessory
| -Sternocleidomastoid, trapezius
39
CN 12
Hypoglossal
| -Tongue movement
40
Medial longitudinal fasciculus connects what 2 CN nuclei?
3 and 6
41
INO PAthyphysiology
Problem with MLF
Want to look to one side and signal sent to CN6 nuclei but due to damage to MLF cannot send signal to contralateral eyes CN3 nuclei to look in the same direction. So one eye abducts and the other does not move
Affected side is side which eye does not move
Normal eye then gets nystagmus to catch up with affected eye
Classically due to MS, but can be due to stroke in elderly
42
ACA territory stroke
Leg weakness >arm weakness
| Pelvic floor dysfunction
43
Basilar stroke
Step wise stroke progression
Usually first medullary symptoms
Then diplopia
Then lose midbrain and drop in GCS
44
Lacunar Stroke: Pure motor stroke
Posterior limb of internal capsule r anterior portion of pons
45
Lacunar stroke: Ataxic hemiparesis stroke
Posterior limb of internal capsule, basis pontis, and corona radiata
Combination of cerebellar and motor Sx
Legs>arms
46
Lacunar stroke: Dysarthria/clumsy hand
Basis pontis lesion
47
Lacunar stroke: pure sensory
Thalamic infarct
48
Lacunar stroke: Mixed sensorimotor
Thalamus and posterior limb of internal capsule
49
Spinothalmic tract synapse and decussation
1st order neuron has cell body in dorsal root ganglion.
Synapse onto 2nd order neuron in posterior horn and that nerve then crosses over to the contralateral side and synapses in the thalamus.
Decussation occurs over 2-3 segments while ascending
50
Posterior column synapse and decussation
1st order neuron synapses on second order neuron in the medulla
2nd order neuron is called the medial lemniscus and decussates in the medulla
Then goes to thalamus and primary sensory cortex
51
Corticospinal tract synapse and decussation
1st order neuron from primary motor cortex to the anterior horn with decussation at the medulla
2nd order neuron from anterior horn through anterior nerve root to destination
52
Brown Sequard
Ipsilateral UMN weakness below lesion
Ipsilateral vibration and proprioception impairment below lesion
Contralateral pain and temp impairment 1-2 segments below lesion
Ipsilateral LMN weakness and sensory loss AT the level of lesion
53
Central Cord Syndrome
Loss of pain and temp bilaterally at the level of lesion
"Suspended sensory level" "Cape/vest distribution"
Increase in size of lesion: LMN lesion at the level of lesion
Further increase in size: UMN weakness and temp and sensation loss below the lesion
54
Spinal cord blood supply
Anterior and posterior spinal arteries supplied by:
- Vertebral arteries of the neck
- Intercostal branches of the aorta and midthoracic region
- Great Radicular artery (of Adamkiewicz) in the lower thoracic or lumbar region
55
Anterior cord syndrome
Everything gone except posterior column
| USually ischemic with occlusion of anterior spinal artery
56
Posterior cord syndrome
Only loss of dorsal column
Rarely ischemic given bilateral blood supply
Usually demyelination, nutritional, or genetic
57
T1
Grey is grey and white is white
| Good for anatomy
58
T2
Grey is light
White is grey
Good for pathology
59
T2 Flair
Suppresses the CSF color
Grey is light and white is grey
Good for pathology
60
MRI DWI
For infarcts
Bright color is stroke
MRI ADC is the opposite and also good for strokes where dark color means stroke
61
Bloome artifact on MRI
Microhemorrhages
62
Contraindications for TPA
- Ischemic stroke or head trauma in last 3 months
- Previous ICH
- Brain cancer
- GI malignancy or hemorrhage in last 21 days
- Intracranial/spinal surgery in last 3 months
- Suspicion for SAH
- BP >185/110
- Active bleeding
- Current IE
- Suspicion of aortic arch dissection
- Platelets <100,000
- INR >1.7
- APTT >40
- Therapeutic clexane in last 24 hours
- NOAC
- Extensive hypoattenuation on CT
63
Timeframe for TPA in stroke
within 4.5 hours, sooner the better
64
Risk factors for hemorrhage post stroke
```
Large infarct
Established infarctions
Grey matter infarctions
Higher NIHHS score
Poor collaterals
Hyperglycemia
Thrombocytopenia
```
65
Dose alteplase
0.9 mg/kg - 10% as bolus and the 90% over an hour
66
What is the penumbra and what imaging is used to determine its size
-Tissue at risk and potentially salvagable
CBV: Shows how much is dead
Tmax: showed the penumbra
67
Indication for clot retrieval in stroke
CTB: does not display extensive infarct
CTA: major vessel occlusion (ICA,M1,Basilar,M2)
CTP: Large penumbra, small core
Good premorbid function
68
Time frame for thrombectomy in stroke
up to 24 hours, with no difference in group getting treatment closer to 24 hours
DAWN trial - NEJM 2018
69
Tenectaplase vs alteplase before mechanical thrombectomy
Increased rates of perfusion with tenectaplase
70
Alteplase vs placebo in patients with unknown time of onset of stroke
Ischemic lesion on DWI with normal flair suggesting stroke <4.5 hours
Improved perfusion with alteplase, but increased risk of death
71
BP control in acute stroke
Increased BP as it will improve blood flow via collaterals, may reduce extent of irreversible ischemia
Upper limit with tPA: 180/105
Non tPA may go up to 220/120
72
Most important modifiable risk factor for strokes
HTN
73
Target BP after acute setting in strokes and Tx of choice
SBP 120-140
ACEi and Thiazide have the most evidence
74
When to commence BP Mx drugs post stroke
48-72 hours post stroke
75
Antiplatelets post stroke
Short term:
-Aspirin and clopidigrel for 3 weeks, then aspirin alone (most benefit of aspirin within 6 weeks)
Long term:
- Only aspirin
- No benefit of clopidigrel over aspirin and increased risk of bleeding if both A+C used
76
When is the highest risk of new stroke with large vessel atherosclerosis
First month, afterwards plaque stablises
77
CEA vs stenting
Higher risk of peri procedural stroke and death with stenting over the age of 70s
78
Indication for CEA
PAtients with non disabling carotid artery territory stroke or TIA with ipsilateral carotid stenosis measured 70-99%.
Ideally within 2 weeks
79
Indications for stenting over CEA
Unfavorable anatomy
Symptomatic restenosis post CEA
Previous radiotherapy
Only if Age < 70
80
Mx of intracranial large vessel atherosclerosis
DAPT
No role for warfarin
No role for stenting
Aggressive medical Mx
81
Risk factors for dissection
Trauma (can be minimal) with hyperextension of the neck
Genetic predisposition
Fibromuscular dysplasia
CTDs
82
Clinical features of dissection
```
NEck pain
HEadache
Stroke
Partial Horner's Syndrome
SAH if rupture
```
83
Tx of dissection
Aspirin
| Generally good prognosis with resolution of dissection in majority
84
When to start anticoagulation post acute stroke
0, 3, 6, 12 rule
0 days - TIA
3 days - Small stroke
6 days - medium stroke
12 days - large stroke
85
50% of cryptogenic strokes are due to:
PFO
86
Mx of PFO in stroke setting
PFO closure and antiplatelet therapy for patients <60 with no other cause of stroke found is indicated if associated with atrial septal aneurysm or moderate to large R to L shunt
Increased risk of AF in first 1-1.5 months post closure, but transient
0 % recurrence of stroke in PFO closure group
87
Risk of developing stroke post TIA
3.7% due to improved medical therapy
Risk stratification based on:
- Age >60
- BP >140/90
- Clinical features: unilateral weakness, speech impairment
- Duration: + points if >60 mins
- Diabetes
- Dual TIA
- Imaging: >50% stenosis of ICA, acute DWI restriction
88
BP management in acute ICH
Aim 140-160 (closer to 140)
| Higher renal adverse events in 7 days if intensive BP control
89
REducing haematoma size in ICH
Evidence for lowering bp to <160 and INR <1.3 within 4 hours
90
Platelets in ICH
Worse outcomes. Do not use
91
Tranexamic acid in ICH
Fewers deaths by day 7, but same amount of deaths by 90 days
92
Primary causes of ICH
Deep perforating vasculopathy (atheroscleosis of small vessels)
- Basal ganglia or brainstem
- White matter lesions, lacuna strokes
Cerebral amyloid angiopathy
-Lobar inctra cerebral hemorrhage (small vessel blockage)
93
Causes of secondary ICH
```
Mets
AVM
aneurysm
Cerebral venous sinus thrombosis
IE
```
94
Cerebral Amyloid angiopathy Features
Symptomatic lobar hemorrhages
Cortical microhemorrages
White matter disease and cortical infarcts
Dementia
CAn present with TIA symptoms with positive symptoms
95
Cerebral Amyloid angiopathy Pathophysiology
Combo of ischemia and bleeds
Genetic association with APOE E4 and E3 allele
Amyloid deposition in capillaries, arterioles, and small arteries leads to necrosis of vessel wall and leads to rupture and small vessel occlusion
96
Mx of CAA
Avoid anticoagulation, antiplatelets, and thrombolysis
Mainstay BP control: Perindopril/Indapamide most evidence
97
Cerebral Venous thrombosis Risk factors
```
Hormonal therapy
Post partum
Thrombophilia
Local infections
Chronic inflammatory diseases
Malignancy
```
98
Cerebral Venous thrombosis presentation
Isolated intracranial HTN (90% of cases)
SEizures
Focal neurology
Encephalopathy
99
Cerebral Venous thrombosis Mx
Heparin infusion or therapeutic clexane
Then transition to warfarin
3-6 month duration in provoked, 6-12 months if unprovoked
Increased risk of recurrence if occured during post partum and may require prophylactic anticoagulation in future pregnancy
100
Extradural haematoma on CT
Lemon
101
Subdural haematoma on CT
Banana
102
Heerfordt Syndrome
Sarcoid
| Facial palsy combined with uveitis, fever, parotid enlargement, and transverse myelitis
103
Features of optic neuritis
```
Subacute onset
Pain on eye movement
Decreased visual acuity and colour vision
Sparkles of light
Relative afferent pupillary defect
```
(Retrobulbar - no swelling, anterior -swellin)
104
Leber's Optic neuritis
Acute onset visual loss and a week later the other eye too
Mitochondrial disorder
Nil pain on eye movement
105
Causes of bilateral optic disc swelling
HTN
Raised ICP (papillodema)
Bilateral ON/neuropathy - NMOSD
106
MS HLA type
HLA DR2 (HLA DRB1*15)
107
Environmental factors associated with MS
EBV
Smoking
Latitude
Sunlight exposure and Vit D are protective
108
Pathophysiology of MS
Early axonal loss as well as demyelination
Involves cortical grey matter lesions and white matter
Both T and B cell involvement
109
Duration of demylinating attack in MS
LAsting greater than 24-48 hours
Nadir within 2 weeks
Resolution by 4 weeks (may not return to baseline)
Pseudo relapse can occur in setting of fevers/heat
110
Lhermitte's phenomenon
an electric shock-like sensation that occurs on flexion of the neck. This sensation radiates down the spine, often into the legs, arms, and sometimes to the trunk.
111
Uhthoff's phenomenon
the worsening of neurologic symptoms in multiple sclerosis (MS) and other neurological, demyelinating conditions when the body gets overheated from hot weather, exercise, fever, or saunas and hot tubs.
112
63% of patients with a clinically isolated syndrome will develop MS. What are the RFs to develop MS
```
Younger age
High cerebral lesion load
Asymptomatic infratentorial or spinal cord lesions
GAD enhancing lesions
Oligoclonal bands in CSF
Abnormal visual evoked potentials
```
113
Optic neuritis and MS
20% initial presentation of MS is optic neuritis
| 50% of MS patients will develop optic neuritis
114
MS on MRI
```
T2 Flair is best modality
Contrast enhancing of acute lesions lasts up to 1 month
Typical location of lesions:
-Periventricular
-Juxtacortical
-Infratentorial
-Spinal cord
```
115
McDonalds Criteria
For Diagnosis of MS
NEED 2 LESIONS IN TIME AND SPACE
Time
- 2 seperate attacks, or even a Hx of an attack
- MRI with contrast enhancement of a lesion and no enhancement of another
- Oligoclonal bands
Space:
- 2 different locations in the CNS through objective clinical evidence
- 2 different locations in the CNS through MRI
116
Oligoclonal bands in CSF with CIS
High predictor of conversion to MS
117
Factors that are associated with an increased risk of development of primary progressive MS in radiologically isolated syndrome
Older age
Man
Spinal cord lesions
118
Acute treatment of MS
3 days of IV/PO MEthyprednisolone
- Accelerates rate of recovery from relapse
- No change in disability or disease
PLEX for severe disease
119
Interferon Beta for MS
| -MOA, SE, efficacy
Modualtes T cell and B cell function, decreases expression of matrix metalloproteinases and reverses BBB disruption
Annually ~30% reduction in relapses, disability, MRI lesion activity
Reduces mortality
SE:
- Flu like symptoms
- Injection site reaction
- Depression, leukopenia, LFT derrangement, Thyroid disorders
- Some will develop Abs
120
Glatiramer Acetate for MS
| -MOA, SE, efficacy
MOA
- Functions as an altered peptide ligand for MHC Class II
- Stimualtes TREG cells
Annually ~30% reduction in relapses, disability, MRI lesion activity
SE:
-Injection site reaction
121
Laquinimod for MS
| -MOA, SE, efficacy
unknown MOA
-reduces leuks in CNS or modualtes cytokines
23% reduction in relapse and reduces disability and MRI lesions
SE
0Mild LFT derrangeent
122
Teriflunomide for MS
| -MOA, SE, efficacy
From LEF
-Antimetabolite that interferes with de novo synthesis of pyramidines, blocks replication and rapidly dividing cells
31% reduction in relapse, disability progression and MRI lesions reduced by 80%
SE
-Thinning hair, GI upset, teratogenic
123
Dimethyl Fumerate for MS
| -MOA, SE, efficacy
MOA:
- Hydrolyzed into monomethyl fumerate
- Unknown mechansm
53% reduction in relapse, 90% reduction in MRI lesions
SE:
Flushing and diarrhea
Nausea and abdo pain
PML
124
Fingolimod
| -MOA, SE, efficacy
MOA
- Sphingosine 1 phosphate receptor modulator
- Inhibits the migration of T cells from lymphoid tissue into peripheral circulation and CNS
54% reduction in relapse and 82% reduction in MRI lesions
SE:
- First dose bradycardia
- VZV reactivation
- Macular oedema (need 3 monthly OCT)
- HTN
- LFT derrangment
- Lymphopenia
- Risk of rebound relapse after ceasing (1-2 months post) that can be more severe than prev. relapses
- PML
125
Cladribine for MS
| -MOA, SE, efficacy
MOA
- Immunosuppresive purine antimetabolite that targets lymphocytes, folloed by reconstitution
- 2x5 da treatment one month apart then yearly
58% reduction in relase, 82% reduction in MRI lesions
SE
- HEadaches
- Lymphopenia
- Herpes Zoster
- Malignancy (increased risk with treatment >2 years)
126
Natalizumab for MS
| -MOA, SE, efficacy
MOA
- targets a4b1 integrin
- Inhibits the leucocyte migration across the BBB by blocking the interaction between a4 integrin on leuks and the vascular cell adhesion molecule 1 on endothelial cells
68% reduction in relapse, 92% reduction in MRI lesions
SE:
- Anxiety, pharyngitis, peripheral oedema, infusion related symptoms
- 6% develop persistant anti natalizumab ab
- PML
- Risk of rebound relapse with cessation
127
PML features
Subacute worsening visual, motor, or cognitive change with gradually enlarging T2 hyperintensities with minimal or no gad enhancement
128
Mx of PML
PLEX
CAn trial Pembrolizumab
Can develop IRIS post PLEX abou 2-5 weeks post PLEX
129
Alemtuzumab for MS
| -MOA, SE, efficacy
MOA
- Targets CD52 (T and B cells) and leads to lyphopenia
- yearly infusion for 2 years
SE:
- Infusion reaction
- ITP
- Graves disease
- Anti GBM
- Increased infection risk, herpes
130
Ocrelizumab for MS
| -MOA, SE, efficacy
MOA
- Targets CD20
- Similar mechanism to rituximab
Effective in Primary Progressive MS (need Hx of OGB in CSF previously)
Slows the disability, doesn't cure it.
131
Siponimod for MS
| -MOA, SE, efficacy
MOA
- Selective Sphingosine 1 phosphate receptor 1 and 5 modulator
- 2nd gen Fingolimod
- may prevent neurdegeneratio nand promote remyelination
Indication: Secondary progressive MS
```
SE:
-First dose bradycardia
-VZV dissemination
-Macualr oedema
HTN
-LFT derrangement
Lypmhopenia
```
132
Mitoxantrone for MS
| -MOA, SE, efficacy
MOA
-Chemo agent: inhibition of B cell, T cell, and macropage proliferation
For aggressive RRMS, secondary progressive MS
```
SE:
Cardiac toxicity (CI if EF <50%) or drop during treatment
```
133
MS and pregnancy
MS relapses are reduced in pregnancy, but not completely.
| Increased risk of relapse post partum
134
Tx of MS in pregnancy
```
CAn use:
IFB
Glateramer Acetate
Alemtuxumab
Rituximab/Ocrelizumab - avoid in 2nd/3rd trimester
```
135
Features of NMO
Relapsing B cell mediated disease targeted agaisnt astrocytes
-More severe than MS with likely permanent residual damage
PRsentation
- Optic neuritis
- Acute myelitis (contiguous spinal cord MRI lesion over 3 vertebral segments)
- Area postrema syndrome (episodes of otherwise unexplained hiccups or nausea and vomiting)
136
Anti AQP4
NMO
137
MOG Ab
NMO, ADEM
| -Steroid responsive
138
Tx of NMO
Acute: IV Methylpred or PLEX
Chronic:
- Eculizumab, Ritux, AZA, MTX
- Inebilizumab (CD19 depletion)
- Satralizumab (Anti IL 6 receptor)
139
ADEM
USually preceeded by viral infection
Resolves after 3 months
Neurological symptoms assocaited with encephalopathy
140
SOD1 mutation
Gain of function mutation for motor neuron disease
141
C9orf72
Motor neuron disease
142
TDP-43 cytoplasmic inclusions
Dementia in ALS - Tau process
ALS patients get frontotemporal dementia - executive function and verbal fluency loss
Ocassional overlap with PSP/PArkinsons
143
NCS in Motor neuron disease
Decreased CMAP
| Normal SNAP
144
Tx of MND
Riluzole - inhibitor of glutamate relase
-Extends survival by 3-6 months
Edaravone
- Potential free radical scavenger to reduce oxidative stress
- Mild reduction in progression (only safe to use up to 6 months)
145
What is a motor unit
a single motor nerve and all of the muscle fibres it innervates
146
Fibrillation potentials and positive sharp waves on EMG
Spontaneous firing of individual muscle fibres
Sign of ACTIVE DEMYELINATION
Can occur in inflammatory myopathies
147
Faciculations on EMG
Firing of all parts of the motor unit
- MND
- CIDP
- Chronic nerve entrapment
- Cal also occur in benign fasciculation syndrome
148
EMG - Neurogenic changes
Spontaneous: Faciculations
Morphology: Large amplitude , widened duration
Recruitment: Decrased
149
EMG - Myogenic changes
Morphology: small amplitude
Recruitment: Increased
150
MG Antibodies
AChR (85%) - Correlates with thymoma or thymic hyperplasia
| MuSK (6-10%)
151
Features of MG with MuSK ab
Non-white, young female
Early onset
Not related to thymoma
Generalised MG
Severe disease with resp and bulbar involvement
Poor response to treatment, good response to rituximab
152
How does Tensilon Test work
Administration of edrophonium (short acting acetylcholinesterase inhibitor)
Given with atropine at bedside
153
Diagnostic findings on Repetitive nerve stimulation for MG
10% decline in amplitude with repitation
-Good for generalised MG
Single fibre EMG best for ocular MG
154
Mx of MG
1. Pyridostigmine (Cholinesterase inhibitor)
2. Steroids - reduce risk of developing generalised MG - can worsen before improving usually after 4-5 days of starting
3. AZA/MMF/Tac/RTX/IVIG/PLEX
4. Eculizumab in severe cases
Thymoma removal if present
MG Crisis: IVIG, PLEX, Steroids, Supportive care and intubation
155
Meds that can cause G crisis
```
BB
CCB (verapamil)
Aminoglycosides
Fluroquinolones
Tetracyclines
Macrolides
Phenytoin
Lithium
```
156
MG and pregnancy
Exacerbation in 1st trimester and post partum
| Settled during 2nd and 3rd trimester
157
LEMS Clinical features
Prosimal weakness - improved post exercise
Autonomic features - Dry mouth, erectile dysfunction, constipation, urinary issues, orthostatic hypotension
Areflexia
158
VGCC antibodies
LEMS
159
Tx of LEMS
Tumor resection (SCLC usually paraneoplastic)
3-4 diaminopyridine - Blocks efflux of K+ ions prolonging duration of depolarisation
IVIG
PRed
160
MOA of Botulism
Anaerobic spore forming Gram positive bacillus
| Inhibition of presynaptic acytelcholine release
161
Features of Botulism
```
Incubation 2 days
N+V
Symmetric descending flaccid paralysis
Autonomic involvement - Dry eyes, mouth, paralytic ileus, urinary retention
Mentation and reflexes preserved
```
162
Mx of botulism
Equine antitoxin
- Avoid aminoglycosides
- REmove toxin if ileus and not absorbed
Recovery over months
163
Anti synthetase syndrome
```
ILD
FEver
Polyarthritis
MEchanic hands
Raynauds
```
Anti Jo1 ab
164
Anti cN1A
Inclusion body myositis
165
Anti SRP
Necrotising autoimmune myositis
166
Types of generalised seizures
Tonic clonic
Absence
Myoclonic
Atonic
167
Juvenile absence epilepsy features and Mx
Absece seizures and occasional GTCS
Generally cease in late teens
Mx - Valproate
168
JME features and Mx
ICK Gene in 7 % (Intestinal cell kinase)
Myoclonus in nearly waking states
CAn have GTCS and absence seizures
Wose with sleep deprivation and ETOH
Mx
1. Valproate
2. Lamotrigine - women of child bearign age
3. Zonisamide
Prognosis - poor remission
169
3 Hz spike/polyspike wave discharges
Buzzword for JME
170
Mesial Temporal Lobe Epilepsy with Hippocampal Sclerosis
Most common form of epilepsy
Risk factors: Prolonged febrile convulsions, CNS infections
Typical auras, focal seizures with impaired awareness with or without automatisms
-Deja vu, gustatory/olfactory hallucinations, epigastric rising sensation
Usually medically refractory and requiring surgery
171
EEG: Alpha 9-12 Hz
Normal Wakefullness
172
EEG: Beta 13-15 Hx
Too much Benzos
173
EEG: Theta 5-8 Hz
Drowsy or encephalopathic
174
EEG: Delta 1-4 Hz
Sleep or encephalopathic
175
EEG: Spike and wave
Epileptogenic
176
When to treat seizures
After 2 seizures no more than 1-2 years a part
| EArly treatment if high risk of recurrence or in vulnerable population
177
Tx of choice for focal seizures
1. Carbamazepine
2. Lamotrigine
3. Gabapentin
4. Keppra
5. Valproate
6. Phenytoin
178
Tx if choice for absence seizures
1. Valproate
| 2. Ethosuzimide
179
Tx of choice for Generalised epilepy
1. Valproate
2. Lamotrigine for women of child bearing age
3. Zonisamide
180
Carbamazepine and SJS HLA type
HLA B*1502 - Han Chinese, thai, malay, filopino
| HLA A *3103 - Europeans
181
Best known combo for dual therapy in epilepsy
lamotrigine and valproate
182
Role of hormones in epilepsy
Estrogen promotes neuroexcitatory properties
Progresterone promotes neuroinhibitory properties
Lamotrigine levels lowered by OCP
OTher AEDs induce rapid clearance of OCP
183
Best tolerated AEds in pregnancy
Lamotrigine and carbamazepine
Note that lamotrigine and keppra undergo increased clearance and will need increased dose at some point in pregnancy
184
Risk factors for SUDEP
GTCS > 2 years
Nocturnal seizures
Treatment resistant seizures
Long duration of epilepsy and early stage of onset
Dravet Syndrome - Na channel gene mutation
185
Mx of Status epilepticus
1. Benzos - IV Loraze 2-4 mg; Midaze 10 mg IM
2. Load with AED - Rule of thumb 20 mg/kg
3. Midaze/propofol infusion
4. Pentobarbitol
Monitor BSL as will drop with prolonged status
186
MOA Carbamazepine and SE
Sodium channel
```
SE:
-Diplopia/dizziness/drowsiness
-SJS/Rash
-Aplastic anaemia
Hyponatremia
-Hepatotoxicity
-Lupus like syndrome
-WEight gain
-Decreased bone mineral density
```
187
MOA and SE Keppra
Inhibits presynaptic calcium channels reducing neurotransmitter release and acting as a neuromodulator
SE:
- Psychosis/irritability/hostility/depression
- Dizziness
188
MOA and SE Phenytoin
Sodium channel
SE:
- CAn worsen absence and myoclonic seizures
- Ataxia, nystagmus, diplopia
- Blood dyscrasia
- Behavior changes
- Skin thickening/gingival hyperplasia/hirsutism/corsening facial features/Acne
- Peripheral neuropathy
- Bone disease
189
MOA and SE Valproate
PIP3 reduction, sodium channel, Increases GABA
SE:
- WEight Gan/PCOS like syndrome/ menstrual cycle issues
- Thrombocytopenia
- Teratogenic ++
- Acute liver failure/pancreatitis
- Alopecia
- PArkinsons/dementia
190
CGRP
Calcitonin Gene Related Peptide
- Produced by trigeminal ganglion and released in nerve endings
- Most potent dilator of cerebral and diral blood vessels
- Release of inflammatory mediators from mast cells
- Increased levels during a MIGRAINE
191
Hemiplegic migraine
weakness tkes up to 72 hours to reseolve
Need to rule out stroke or TIA
FMH1 mutation of CANCNL1A4 gene (coding for calcium channel)
192
Migraine prophylaxis medication options
```
Amitryptylline
Propanolol
Topiramate
Candesartan
Pizotifen
```
New role for CGRP inhibitors
193
Cluster headaches
Male predominence
Occur nearly daily lasting 15-180 mins
Follow circadian cycle - predominantly nocturnal
Sharp stabbing pain with ipsilateral autonomic features
Restlessness and agitation
Acute Tx - Triptans, High flow O2 with non rebreather
Bridging Tx - steroids, occipital nerve block
Prevention - Verapamil SR TDS
194
Paroxsymal Hemicrania
Similar to cluster but..
Female predominant
5-10 times per day lasting 2-20 mins
Complete an absolute response to indomethocin (diagnostic)
Melatonin trial if indomethocin responsive
195
Hemicrania continua
Constant unilateral, side locked headache
assoacited with autonomic features and restlessness
Daily to near daily lasting 30 mins to 3 days
Tx: indomethicin responsive
196
SUNCT/SUNA
| Short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing
Stabbing lancinating burning pain, orbital pain
Up to 100 times per day lasting 1-600 seconds
Tx: Lamotrigine
O2 and indomethocin do nothing for this
197
Trigeminal neuralgia
20 x increase risk in MS - esp. bilateral TN
V2 distribution most common
Usually due to neurovascular compression
Tx: CArbamazepine, microvascular decompression
198
Idiopathic intracranial HTN RF and secondary causes
Unknown cause
RF: Female, obese, reproductive age
```
SEcondary causes:
Venous sinus thrombosis
Tetracycliens, fluroquinolones
Vit A - tretinoin
Iron deficiency
OSA
Raised CSF protein
```
199
Diagnsotic criteria for IIH
Papillodema
Normal neuro exam except CN abnormalities
Normal MRI and CSF
CSF opening pressure > 25 mmHg
200
IIH management
```
WEight loss
Acetazolamide
Topiramate
VP shunt
Optic nerve seath fenestration
Venous stenting
```
201
Aceazolamide MOA
Potent ezyme inhibitor of carbonic anhydrase, and it impedes the activity at the choroid plexus reducing CSF secretion
202
Intracranial hypotension
Postural headache worse on standing or sitting and better when laying flat
Most common is post LP headache
Mx:
bed rest, fluids, coffee, epidural blood patch, surgical repair
203
Eseential tremor
Bilateral, slow progression
ETOH may help, but not diagnostic
Mx: Propanolol, DBS, USS Thalamotomy, Primidone
204
Intention tremor
Worse on end of movement and suggests cerebellar involvement
205
Holmes tremor
Unilateal, rest, postural, movement, and worse on intention
| Seen in wilson's disease, stroke, MS
206
Dystonia
Sustained r intermittant muscle contractions causing abnormal often repetitive movements, postures, or both
Movments are typically twisting
Initiated or worsened by voluntary action
Mx: Botox
207
Chorea
Involuntary movmeent of limbs, trunk, neck, or face wjich rapidly flit from region to region in an irregular pattern
208
Dyskinesia
Chorea movement with parkinsons
209
FEatures of presymptomatic stage of PArkinsons
Anosmia
Constipation
REM sleep disorder
Mood changes, increasing fatigue
210
Inclusion criteria for PArkinsons
Decrementing bradykinesia
Rigiditiy
4-6 Hx rest tremor
211
Side effects of L Dopa
```
Sedation, N+V
Dyskinesia, mtor fluctuations
Impulse control disorders (in high doses)
Punding
Dopamine dysregulation - abuse
```
212
Motor complications in PArkinsons
On - Well
Off - Full blown parkinsons
Dyskinesias - too much drugs
Will have mtr fluctuations and dyskinesias after 5 years of treatment
213
Risk factors for motor fluctuations in PD
Longer disease duration
Higher levels of L Dopa used
Younger onset of PArkinsons
214
Mx of motor fluctiations in PD
Divide dose into increased freq.
| Add on COMT or MAO
215
Dyskinesia treatment in PD
minimise dopaminergic meds
Amantadine
Break up doses into higher frequency
216
Amantadine MOA
Weak antagnist of the NMDA type glutamate receptor, Increases dopamine release and blocks dopamine reuptake
217
DA agonist - names, MOA
MOA: Stimulate dopamine by binding directly to post synaptic dopamine receptors in the striatum
MEds:
-Non Ergot DAs: Pramiprexole, Ropinirole, Transdermal rotigotine, Apomorphine
-Ergot derived: CAbergoline, bromocriptine, pergolide
218
SE of CAbergoline, bromocriptine, pergolide
Heart valve complications and retroperitoneal fibrosis
219
SE DA agonists
Nausea, somnolence, oedema, dizziness, hallucinations, hypotension
Worsens delirium
Impulse control disorders
220
MAO B inhibitors names and SE
Rasagline, selagiline
USed in early/young patients
```
SE:
LFT derrangement
INSOMNIA - most common
Higher overall mortality
Tyrasine hypertensive crisis
-Tyrasine releases NA and ordinarily MAO A breaks down excess, if MAOI then adrenergic crisis
```
221
Exenatide in Parkinsons
GLP1 receptor agonst
Improves medication motor scores
Likely symptomatic improvement rather than neuroprotective
222
Order of ceasing PD meds in setting of delirium and hallucinations
1. Anticholinergic meds
2. Amantadine
3. DA
4. MAOI
5. L Dopa
223
Why can meds not be stopped abruptly in PD
PArkonsonian crisis - NMS
224
Strongest predictor of dementia in PD
Age not duration
225
Alpha synneuclianopathies
PD
DLB
MSA
226
Tau protein opathies
PSP
| Corticobasalar degeneration
227
PSP Triad and MRI findings
Supranuclear palsy, postural instability, dementia
- Early falls
- Psuedobulabar palsy: dysarthria anddysphasia
Hummingbird sign on MRI (midbrain atrophy)
228
MSA FEatures and MRI findings
Autonomic failure, Parkinsonism, Cerebellar signs
Hot cross bun sign
229
Corticobasal syndrome
Cortical dysfunction and basal ganaglia dysfunction
Asymetric progressive ideomotor aprazia that frequently affects the hand and is associated with rigidity myoclonus and dystonia
Alien hand
230
FMR protein deficiency
FXTAS/Fragile X syndrome
231
HTT Protein
Huntingtons
-CAG repeat, paternal anticipation
HTT protein is toxic to striatal neurons
232
Spinocerebellar ataxia
CAG repeat disorder
CAn be AD, AR, X linked
Variable syndromes, but predominantly ataxia and UMN signs
233
Differentiating Serotonin syndrome from NMS
SS:
- Acute onset <24 hours
- Myoclonus, Hyperreflexia, rigidity
NMS
- Slower onset
- CK rise and rigidity
234
HSV encephalitis
Herpes PCR can be negative in first 24-48 hours and should recheck if high suspicion
MRI: Y2 hyperintensity in medial and inferior temporal lobe extending up insular cortex
EEG: focal slowing
Tx: IV Aciclovir 14-21 days
235
Listeria encephalitis
Most commonly rhomboencephalitis - brainstem involvement
Incidence higher in pregnancy, elderly, immunosuppressed
Tx: Benpen, ampicillin
236
Anti Hu
Limbic encephalitis, peripheral neuropathy
| SCLC
237
Anti Yo
Cerebellar degeneration
| Breast/Ovarian cancer
238
Anti Ri
Opsoclonus myoclonus, rhomboencephalitis
| SCLC
239
Anti Ma/Ta
Rhomboencephalitis - NArcolepsy, Vertical gaze disorder
| Testicular cancer
240
NMDA encephalitis
F>M
50% of young women have ovarian teratoma
Most common cause of AI encephalitis
PResent with preceeding viral prodrome and then associated with psychosis, hallucinations, behavioral changes
NMDA antibodies oresent
MRI: Temporal lobe limbic changes
241
Tx of NMDA encephalitis
```
Remove cancer
IV methylpred, then PO pred
IVIG or PLEX
Rituximab
Cyclophophamide
```
242
LGI1 antibody
Voltage gate potassium channel encephalitis
Cognitive and memory changes
Faciobracial dystonic seizures
243
CASPR2 ab
Voltage gate potassium channel encephalitis
244
VGKC Ab
Voltage gate potassium channel encephalitis
245
PRES RFs
Clincial and radiological syndrome of encephalopathy and posterior circulation oedema
```
RFs:
HTN
Pre-eclampsia
REnal failure
Autoimmune conditions
```
246
PRES Tx
Aggressive BP control
| Seizure Mx
247
Effect on NCS in radiculopathy and pre ganglionic lesions
Normal NCS
248
What does a low amplitude on NCS represent
Axonal loss
249
What does increased latency or decreased conduction velocity on NCS represent
Demyelination
250
NCS in Compression lesions
Focal demyelination - drop in velocity at lesion site
| Oeer time will get drop in amplitude, then change in motor conduction
251
What is SNAP for NCS
Sensory nerve action potentials - the sum of the resultant APs
252
What is CMAP for NCS
Compound muscle action potential - sum of all the APs in the muscle fibre
253
Conduction Block on NCS
Specific point that the nerve has slowed conduction
| Drop in CMAP amplitude between proximal and distal sites of the same nerve
254
F wave on NCS
Only way of looking at proximal segment of nerve
May be only abnormality in early GBS
Delayed F wave if dysfunction
255
Risk factors for carpel tunnel
```
Female
Diabetes
PRegnancy
RA
Hypothyroidism
HAemodialysis
Steroid use
```
256
Mononeuritis multiplex presentation
painful neuropathy which is not at a place of compression
Due to inflammation of blood vessel within the nerve - both small and large fibres are affected - motor and sensory
Definitive testing is biopsy
257
3rd nerve palsy determining compression vs vascultitis
Compression - dilates first
| Vasculitis - Motor, then pupil dilates later or can be pupil sparing altogether
258
Sarcoid induced neuropathies
Polyradiculopathy
Peripheral neuropathy
Mononeuropathy multiplex
Bilateral CN7 palsies - Always consider sarcoid
259
Hereditary neuropathy with liability to pressure palsy
| HNPP
PMP22 gene reciprocal deletion
AD
Characterised by transient and recurrent motor and sensory mononeuropathies, typically occuring at entrapment sites
260
Peripheral neuropathy always affects feet before hands. If it is the other way around who do you need to consider as the cause
Dorsal root ganglion or cervical injury
261
DDX for autonomic failure
Diabetes
MSA
Amyloid
262
Paraproteinemic Peripheral neurpathy causes
- MGUS - If present not always cause - IgM most common
- Waldenstroms - distal acquired demyelinating symmetric neuropathy
- MM
- POEMS
- Amyloidosis - Painful length dependent PN with generalised autonomic failure
263
Chemo agents that cause PN
Platinum based chemo
Taxanes
Vinca ankaloids
Bortezomib
264
Neuropathies in B12 deficiency
PN
Corticospinal tract
Dorsal column
265
RAdiculopathy features
Asymmetric
Follow a dermatome or myotome
PAINFUL
Can be sensory or motor
266
Zoster sine herpete
Neuralgiform pain with no rash
267
Miller Fisher Syndrome
Type Of GBS
| Opthalmoplegia, areflexia, ataxia
268
Anti GQ1b
Miller Fisher Syndrome
269
GBS presentation
Prodrome in the 4 weeks prior to onset of symptoms
Symmetric ascending weakness
CAn be associated with radicular pain
Proximal and distal weakness is usually the predominant feature
Hyporeflexia or areflexia can be delayed by 1 week
WEakness nadir by 2-4 weeks
--If progressive Sx post 4 weeks then rethink Diagnosis ?CDIP
270
NCS in GBS
Prolonged F wave latency
Prolonged distal latencies (demyelinating) - motor before sensory
Sural nerve is often preserved
271
Anti GD1a and GM1
Acute motor axonal neuropathy/Multifocal motor neuropathy with conduction block
(GBS varient)
272
Tx of GBS
1. PLEX - Reduces time to walk unaided and time on ventilator - USe within 2-4 week onset
2. IVIG -use within 2 weeks onset for most benefit in those unable to walk unaided
NO STEROIDS - worse outcomes
273
Poor prognostic markers in GBS
```
Rapid onset prior to presentation
High diability nadir
Severely reduced CMP
Older than 40 yo
PReceeding diarrheal illness, C. Jejuni
PReceeding CMV infection
Inexcitable nerves
```
274
CIDP
| Chronic inflammatory demylinating polyradiculopathy
Chronic version of AIDP
Nadir > 4 weeks
Usually milder phenotype and rarely has resp involvement
Similar NCS findings to AIDP
Tx
-Steroids, steroid sparing agents, IVIG, PLEX
275
NF155
| Contactin 1 Ab
Severe CIDP
Mx: Rituximab
276
Multifocal Motor neuropathy with conduction block
Immune mediated condition with asymmetric peripharl motor loss
Usually upper extremeties
Atrophy and EMG evidence of axon loss over time
Deep tendon reflexes usually decreased
Anti GM1 in 40-80%
IVIG and steroids can worsen the condition
277
Dorsal root Gangiolopathy
Pure sensory loss
UL before LL or same time as LL
Causes: Sjogrens, Anti Hu, B6 deficiency
278
Parsonage-Turner Syndrome
Neuralgic amyotrophy, brachial neuritisL Pain and muscle atrophy
-Severe shoulder and arm pain followed by weakness and numbness
Usually middle aged men, but can be anyone
Tx: steroids and analgesics
279
Diabetic Amyotrophy (Lumbosacral Radiculoplexopathy)
Age >50 and men usually
SEvere unilateral pain in the back, hip, or thigh that spreads to involve the entire limb and can involve the other leg within weeks or months
-Proximal weakness shortly after the onset of pain, and then can become widespread
Not related to glucose control or duration of diabetes
280
LEvel above which consciousness is associated
medulla
281
Topiramate MOA and SE
stabilises presynaptic neuronal membranes by blocking voltage-dependent sodium channels. Enhances activity of GABA on postsynaptic chloride channels.
SE:
-Psych stuff
Renal stones
282
by rapidly progressive dementia, myoclonus and periodic tri-phasic sharp waves on EEG?
JC Virus
283
Stroke Syndrome:
| MCA inferior devision
Contralateral visual loss - homonymous hemianopia Contralateral visual loss - upper quadrant anopia
Contralateral constructional apraxia (non-dominant hemisphere)
Contralateral aphasia - receptive (dominant hemisphere - Wernicke's area)
284
Stroke Syndrome:
| MCA superior devision
Contralateral weakness - upper and lower limb (Face, arm>leg) Contralateral weakness - face - lower half Contralateral hemisensory loss - upper and lower limb Contralateral sensory loss - face - all modalities Contralateral hemineglect (non-dominant hemisphere) Contralateral aphasia - expressive (dominant hemisphere - Broca's area)
285
Balint Syndrome
If affecting bilateral posterior cerebral arteries
Affects bilateral parietal-occipital lobes
Bilateral loss of voluntary but not reflex eye movements
Bilateral optic ataxia - poor visual-motor coordination
Bilateral asimultagnosia - inability to understand visual objects
286
Claude syndrome
Ipsilateral eye movement weakness (oculomotor palsy)
Contralateral ataxia - arm and leg (cerebellar tracts)
Contralateral tremor
287
Posterior cerebral artery - Unilateral occipital
Contralateral visual loss - homonymous hemianopia (optic pathway, calcarine cortex)
288
Thalamic Pain syndrome
| Dejerine-Roussy Syndrome
Penetrating branches to thalamus
Contralateral hemisensory loss - all modalities
Contralateral hemi-body pain
289
Weber Syndrome
Penetrating arteries to midbrain
| Contralateral weakness - upper and lower limb (corticospinal tract) Ipsilateral lateral gaze weakness (CnIII)
290
Lateral pontine syndrome
| Marie-Foix syndrome
AICA
Ipsilateral ataxia - arm and leg (cerebellar tracts) Contralateral weakness - upper and lower limbs (corticospinal tracts) Contralateral pain and temperature loss (spinothalamic tracts)
291
Lateral medullary syndrome
| Wallenberg syndrome
PICA
Small penetrating arteries
Ipsilateral loss of pain and temperature from face + facial pain (Cn5 nucleus)
Ipsilateral ataxia - arm and leg, gait ataxia (Restiform body, cerebellum)
Ipsilateral nystagmus, N&V, vertigo (Vestibular nucleus)
Ipsilateral hoarseness and dysphagia (nucleus ambiguus)
Ipsilateral Horner's syndrome (descending sympathetics)
Contralateral loss of pain and temperature from body (Spinothalamic tract)
Hiccoughs
292
Medial medullary syndrome
| Dejerine syndrome
Vertebral ARtery/anterior spinal
Contralateral weakness of upper and lower extremity (Pyramidal tract) Contralateral hemisensory loss - vibration and proprioception (Medial lemniscus) Ipsilateral tongue weakness +/- atrophy (Cn12 nucleus)
293
Inferior medial pontine syndrome
| Foville syndrome
Basilar artery
Unilateral lesion affecting the dorsal pontine tegmentum in caudal third of pons
Contralateral weakness - upper and lower limbs (corticospinal tract)
Ipsilateral weakness - face - entire side (CnVII nucleus/fascicle) Ipsilateral lateral gaze weakness (PPRF or CnVI nucleus)
294
Locked-in syndrome
Basilar artery
Affects bilateral ventral pons Bilateral weakness - upper and lower limb (Bilateral cortical spinal tracts) - quadriplegia Bilateral weakness - face - entire side (bilateral corticobulbar tracts) Bilateral lateral gaze weakness (bilateral fascicles of CnVI)
Dysarthria (bilateral corticobulbar tracts)
295
Ventral pontine syndrome
| Raymond syndrome
Basilar artery
Affects ventral medial pons
Ipsilateral lateral gaze weakness (CnVI) Contralateral weakness - upper and lower limb (pyramidal tract)
296
Ventral pontine syndrome
| Millard-Gubler syndrome
Basilar artery
Affects basis pontis and fascicles of CN VI and VII Contralateral weakness - upper and lower limb (pyramidal tract) Ipsilateral lateral gaze weakness (CnVI) Ipsilateral facial weakness (whole side) (CnVII)
