Neurology Flashcards
1
Q
Astrocyte and oligodendroglial tumours
A
new classification system WHO 2016. Classified on basis of IDH mutation or not. ISOCITRATE DEHYDROGENASE and other genetic snp mutations. No longer histopathological basis. High grade glioma managed surgical resection plus post op radiotherapy plus systemic chemo. TEMOZOLAMIDE
2
Q
Guillan bare synrome
A
Definition: Progressive ascending motor weakness and areflexia ascending weakness pain sensory loss
AI attack on myelin sheath of peripheral nerves
Presentation:
EARLY = BACK PAIN radiating to lower limbs ?proximal nerve root inflammation
proximal weakness
symmetrical CN palsy / diaphragmatic weakness / autonomic dysfunction - tachycardia
Pathology:
70% post infective: compylobacter jejuni classic or LRTI
Investigation:
CSF: raised protein late
nerve conduction: multifocal demyelination late
Management:
Monitor FVC - ITU if <1.2L - anaesthetic and elective intubation
RX:
plasma exchange
IV Ig
3
Q
Lambert Eaton - Lambert Eaton Myaesthenic Syndrome
A
Definition
Myaesthenic like syndrome - AI abs VS Voltage gated calcium channels - PRESYNAPTIC
Association:
50-70% underlying malignancy
majority SCLC
Presentation:
SLOW PROGRESSIVE PROXIMAL MYOPATHY
LOSS OF DEEP TENDON REFLEXES
FACILITATION AND RETURN OF DTR = PATHOGNOMIC
autonomic features more present than in myaesthenia = poor salivation and erectile dysfunction
Bulbar and resp are preserved unlike myaesthenia
Also responds to facilitation unlike myaesthnia due to increase calcium in cleft from repeated stimulation
4
Q
MITOCHONDRIAL DISORDERS
A
EPILEPSY DEAFNESS OPTHALMOPLEGIA OPTIC NEURITIS HEART DISEASE SHORT STATURE LEARNING DIFFICULTIES
inherited from mother (mitochondrial)
examples: Kearns-Sayre Leigh MELAS: mitochondrial encephalopathy lactic acidosis and stroke like episodes NARP: Neuropathy and Retinitis Pigmentosa MNGIE: Mitochondrial neuro gasto intestinal encephalopathy PEO: Progressive external opthalmoplegia
5
Q
Freidreich’s Ataxia
A
Pattern:
Autosomal Recessive
Presentation: gait disturbance pes cavus scoliosis diabetes
6
Q
Huntington’s disease
A
Pattern:
Autosomal Dominant INHERITANCE
Definition:
Huntington gene chr 4 codes for HUNTINGTIN cand contains a CAG triplet repeat coding for glutamine. Normal = 11-34 repeats
Expansion of this triplet results in abnormal protein which seems to damage brain cells
Demonstrates Anticipation with paternal inheritance
Alternate presentation:
Teenage years with parkinsonian symptoms - WESTPHAL VARIANT
Presentation: Early: mood / mental acuity MIDDLE: ataxia uncoordinated jerky choreoform movements / athitosis LATE: mental decline to dementia FT lability loss of speech loss of mobility due to worsening ataxia and poor coordination
7
Q
Miller-Fisher - SUB-TYPE OF GBS
A
DEFINITION:
Sub-type of GBS
Anti-Gq1b antibody specificity
AI demyelination of peripheral nerves
Presentation:
Opthalmoplegia
Ataxia / coordination
Loss of deep tendon reflexes
8
Q
Spinocerebellar Ataxia - SCA
A
Defintion: 4 subtypes genetic disorder SLOW PROGRESSIVE UNCOORDINATED GAIT UNCOORDINATED SPEECH, HAND AND EYE MOVEMENTS
SCA 4:
Autosomal Dominant
associated sensory neuropathy
9
Q
B12 neuropathy
A
Presentation:
Sensory loss and sensory ataxia
10
Q
Motor Neurone Disease = MND
A
Definition:
Pure Motor neurone features
Cognitive impairment and labile mood overlapping with FT dementia also seen
Amyotrophic lateral sclerosis:
Mutation in SOD-1 - superoxide dismutase
Presentation: Increasingly clumsy Slow hand movements wasting of small muscles of the hands UMN sgns: - loss of CNS inhibition Fasciculations in lower limbs Spasticity weakness brisk reflexes
Investigation:
EMG: active denervation in affected muscle
MRI may be normal
need to rule out SOL
11
Q
Headache
A
Red flags: Vision changes / FAST Thunderclap New onset progressive Early morning headaches ABOVE NEED CT IMAGING ESP IN >50s
Raised ICP: Extradural SOL Venous sinus thrombosis BIH / IIH
Ix:
CT head / CT or MRI venogram
MRA for SAH
LP once confirmed no raised ICP
12
Q
BIH / IIH - no longer BIH as associated with visual loss
A
Defintion:
Raised ICP without obvious cause
Demographic:
overweight female
child bearing age
Presentation:
visual obscurity
pulsatle tinnitus
postural headaches
exam: bilateral papilloedema enlarged blind spot impaired acuity VI palsy
Ix: Ct head to exclude SOL / causes ICP CSF: opening pressure >25cm LP improved symptoms opthalmology review for regular retinal photography and visual field assessment
management:
weight loss
acetazolamide or topiramate reduced CSF production
surgical - optic nerve sheath fenestration to allow drainage or CSF shunts
major morbidity = visual loss due to papilloedema
13
Q
Stroke prevention
A
Secondary prevention:
rate control / rhythm control if AF / ablation / anticoag rivarox / apixaban
INTERNAL CAROTID ST >50%
consider endarterectomy
>70% - European carotid surgery trial - the greater the baseline risk the greater the patient has to gain from surgery vs conservative medical managstement
e.g. male sex / hemispheric event / unstable plaque / worsening comorbidity
Northamerican symptomatic carotid endarterectomy trial = >50%
Endarterectomy advised
Aspirin 75mg
TIA on Aspirin –> Add clopidogrel 75mg
Increasing aspirin dose does not reduce stroke risk but increases UGIB RR
Aspirin and dipyridaomle was treatment of choice for secondary prevention post ESPS-2 study. However clopidogrel now superseded original guidance
14
Q
Meningitis
A
Definition:
Bacterial or viral inflammation of the meninges
Infectoin may spread to parenchyma and brainstem
Presentation: Meningo-encephalitic picture headache pyrexia nausea cranial nerve palsy neck stiffness photophobia drowzyness / depressed GCS altered behaviour seizures
Pathogens: Elderly and immunosupressed: Listeria monocytogenes Gram positive rods mortality >60% CSF 50% sensitivity
Ix:
Ct head
LP
neuro obs
Mx: unknown aet: cefotaxime + aciclovir listeria / gram +ve = ampicillin and gent TB suspect: cefotaxime and rifampicin MDR pneumococcal - cefotax and rifamp pneumococcal = cefotaxime and dex paeds = cefotax and dex evidence of oedema = dexamethasone
Pen Ax = chloramphenicol
15
Q
Peroneal Nerve Palsy
A
Definition:
Common entrapment neuropathy
Cause:
pressure at fibular head / plaster casts and immobilisation
Diagnosis:
EMG studies
CK levels
presentation:
weakness of ankle flexion and toe extension
Foot Inversion is preserved
Foot eversion is lost
L5-S1 lesion would lose inversion as well
16
Q
Anti-GM1 antibodies
A
seen in:
multifocal moto neuropathy with conduction block
17
Q
brachial neuritis
A
definition
inflammation of brachial plexus
presentation:
classic post vaccination
symptoms:
winging of scapula
weakness in any distribution of plexus supply
18
Q
cerebral abscess
A
Definition:
abscess (walled off focal infection primary or seeded) seen in the brain which may exhibit focal neurology
CT:
Ring enhancing lesion demonstrating encapsulation and non resolution
precipitant:
mastoiditis / sinusitis / dental infection
neonatal meningitis
fungal > in immunocomp e.g. HIV / DM
symptoms: headache fever encephalopathy depressed GCS FAST +VE specific symptoms dependent on the site of the lesiont
site:
most common corticomedullary junction frontoparietal region
19
Q
Mccardle’s disease - mycophosphorylase disease
effects muscle
glycogen storage disorder V
A
Defintion:
impaired glycogen storage and metabolism in skeletal muscle
Inheritance:
autosomal recessive
PYGM gene chromosome 11
presentation: teens
impaired early exercise tolerance / fatigue / cramping
fixed weakness
prolonged bouts of exercise lead to myoglobin release and dark urine - myoglobin urea
Testing: second wind phenomenon - rest - delivery of oxygen and fatty acids and switch to non aerobic metabolism myoglobinuria raised CK muscle biopsy
MX:
high carb diet
sucrose before exercise
20
Q
Beckers Muscular Dystrophy
A
Definition:
X linked muscular dystophy
Any female offspring will be carriers at least
presentation
later than Duchennes an variable anywhere from age 3 to middle age
likely to manifest arrythmias
21
Q
Muscular Dystrophy
A
Definition:
Group of muscle disorders that result in progressive muscle weakness and muscle breakdown over time
Affects dystrophin gene product
examples:
Duchennes
Beckers
22
Q
Vertebral Artery Dissection
A
Definition
Dissection of intima layer of vertebral artery resulting in occlusion of the arterial lumen
presentation: Posterior inferior cerebral artery occlusion
traumatic
vertigo / nausea / weakness / depressed GCS / ataxia
23
Q
Benign paroxysmal positional vertigo
A
Definition:
Acute onset bouts of vertigo / nausea worse on head movement brought on by loose ortoliths.
Presentation:
commonly anyone with a history of head injury
manifestation: DIX HALLPIKE
Hold head to the right 45 degrees
rapidly move from sitting to lieing
Treatment:
Prochlorperazine
EPLEY MANEOUVRE:
Turn head to affected side as for DH
SLOW and SMOOTH go from sit to lieing and allow patients head to rest off end of cough still supported for 30secs
turn head towards unaffected side and remain for 30secs head still off couch - chin on shoulder
Patient now turns onto side to face unaffected side - head still supported off couch
sit up slowly keeping head supported chin on shoulder
Turn head to face forward and move chin to chest in one movement 30 secs
DONT DRIVE FOR 2 HOURS
DONT LIE FLAT FOR 2 NIGHTS
SLEEP ON GOOD SIDE FOR 5 NIGHTS WITH SUPPORT TO PREVENT ROLLING
24
Q
Hepatic Encephalopathy
A
Definition:
Encephalopathy manifest by depressed GCS secondary to liver failure
precipitants: HEPATICUS HYPOGLYCAEMIA ELECTROLYTE DISTURBANCE PROTEIN MEAL - GI BLEED ALCOHOL / ANALGESIA TUMOUR INFECTION CONSTIPATION URAEMIA SURGERY
EEG: TRIPHASIC REPEATS
mX: Treat cause abx itu laxatives rifaximin fluids
5 stages - 0 - nil
1 - altered sleep wake cycle / mild confusion / alt behaviour
2 - moderate confusion / dorwzy / ataxia / asterixis
3- stupor / sleeping / incoherent speech
4 - coma
25
Myotonic Dystrophy type 1
Definition:
Inherited muscular dystrophy resulting in progressive muslce weakness and muscle wasting
Unlike Beckers and DMD, it is not x linked but is autosomal dominant and MD1 is the most common.
Mutation:
trinucleotide repeat disorder like Huntington's
affects dystrophia myotonica protein kinase (DMPK)
ANTICIPATION
Diagonsis:
Muscle EMG - wax and wane "dive bomber pattern"
```
Presentation:
adulthood
bilateral ptosis
frontal balding
cataracts (not seen in DMD or BMD)
progressive weakness
loss of TDR
facial wasting
smooth muscle wasting abdo pain
dysphagia
resp muscle weakness
```
26
Parkinson's Disease
Defintion:
Longterm neuro-degenerative disorder of the substantia nigra causing a reduction in dopamine release resulting in involuntary tremor like movements / bradykinesis and stiffness
Early:
Disturbnce in REM sleep due to a lesion within the PONS is a recognized feature of early onset idiopathic PD - this results incomplete relaxation during REM sleep and classically patients are seen fighting or verbalising with invisible individuals.
27
Chronic Inflammatory Demyelinating Neuropathy:
inherited - charcot marie tooth
drug related - amiodarone
paraprotein
Definition:
Chronic demyelinating sensorimotor neuropathy
EMG:
latency
F waves
slowed conduction
treatment:
oral steroid
IV Ig
azathioprine as a steroid sparing agent
28
Periodic Paralysis
| thyrotoxicosis with periodic hypokalaemic paralysis
Definition:
periodic attacks of global motor weakness usually starting in the second half of the night following strenuous exercise the day before or a high carb meal
common:
chinese / japanese heritage
associated:
thyrotoxicosis
hypokalaemic paralysis
Mx:
potassium chloride supplements restore muscle power
euthyroid state prevents further attacks
29
Basilar stroke
Definition
Ischaemic occlusion of middle / proximal / distal basilar artery affecting brainstem function
risk factors:
HTN
atherosclerotic disease
AF
```
symptoms:
LOCKED IN SYNDROME - middle and proximal
consciousness preserved but paralysis globally
eye movement preserved
bulbar involvment
```
mortalty
85%
thrombolysis
35% restore reasonable QOL
30
Ramsay Hunt Syndome
Definition:
Hearing loss / hyperacusis / ptosis
see herpetic lesions on TM
VII palsy resulting from HERPES ZOSTER INFECTION of GENICULATE GANGLION
viii MAY ALSO BE AFFECTED = SENSORINEURA HEARING LOSS AND VERTIGO
IX:
schirmer test strip swab of pinna and viral pcr
vesicular blisters over tympanic membrane - NOT PAINFUL as bullous myringitis - mycoplasma pneumonia
Mx:
pred
aciclovir
31
Autonomic myopathy
Definition:
Group of conditions affects SNS / PNS or both
```
presentation:
postural drops
hypotension
tachycardia - loss of vagal tone
anhydryasis
impotence
nocturia
impotence
urgency
horners sign - ptosis with constricted pupil and decreased sweating on side affected
```
```
Primary:
DM
amyloidosis
GBS - poly motorneuropathy
infections - HIV / chagas
metabolic - uraemic neuropathy / hepatic related / alcohol
immune - RA /
```
RX: dependent on cause
1. raise bed at night to increase renin production and raise baseline bp
2. slow standing exercises to reduce falls risk
3. increase salt intake
4. fludricortisone
5. eat little and often to reduce post prandial syncope
6.diabetic control
etc
32
Holmes - Adie Syndrome
Definition:
inherited polyneuropathy - degeneration of cilia ganglion and post synaptic ganglionic fibres
signs:
purely motor
ipsilateral ptosis
blurred vision
Rx:
0.1% pilocarpine to constrict pupil
33
CADASIL
| cerebral autosomal dominant arteriopathy with subcortical infarcts
Arteriopathy - media thickening and stenosis with loss of smc
Definition:
syndrome of multiple early onset TIAs / stroke like episodes and complete strokes with significant periventricular white mater changes on MRI T" weighted imaging
early onset dementia
FMH migraine
Definitive Ix:
NOTCH-3 gene testing
34
lyme disease
definition:
| Tick borne parasitic infection characerrised by meningitis / motor or sensory radiculopathy / cranial neuropathy
35
botulism
Definition:
bilateral cranial nerve neuropathy with descending weakness caused by botulinum toxin release from clostridium botulinum
diplopia
opthalmoplegia / blurred vision - CN involvement
desending weakness
neurotoxin blocks acetylcholine at NMJ
i.e. FLACCID PARALYSIS
Therefore EMG shows incremental increase in response to repeated stimulation as more AcH enters NMJ
PURE MOTOR - no sensory / remains responsive
```
signs:
symmetrical descending paralysis
CN:
diplopia / ptosis / dysarthria / dysphagia / facial weakness
RESP:
diaphragmatic paralysis
IC muscle weakness
AUTONOMIC:
Diarrhea / abdo pain / fixed or dilated pupils / tachycardia / urinary symptoms
```
36
macular sparing homonomymous hemianopia
definition in stroke:
macular sparred single sided hemianopia affecting both sets of optic radiaotin therefore must originate in cerebellum
macular sparred as supplied by distant branch of MCA
location of occulusion
posterior cerebellar artery
37
inferior quadrantinopia
definition in stroke
homonymous hemianopia knocking out parietal optic radiations supplying information from the inferior quadrants of the visual fields
associated:
same sided neglect or inattention
38
superior quadrantinopia
defintion in stroke:
homonymous hemianopia knocking out temporal optic radiations supplying information from the superior quadrants of the visual fields
associated:
AV hallucinations / dysphasia
39
Juvenile monoclonic epilepsy
Definition
Early onset periodic a)GTCS b)myoclonic jerking of one or more limbs c)absence seizures
strong genetic component - 50% have another affected relative
most common of all generalised epilepsies
Presentation:
On waking typically
Precipitant:
exacerbated by a)sleep deprivation b) alcohol c)anxiety
Mx:
lamotrigine - non teratogenic
avoid precipitants
AVOID CARBAMAZEPINE - WORSENS SEIZURES
AVOID PHENOBARBITONE
EEG: BURST WAVES / POLYSPIKE COMBINATIONSFDIVE
40
Myaesthenia Gravis
Definition
A progressive autoimmune myopathy resulting from antbodies directed against the AcH receptor on the on post synaptic termini (ant acHR) or against a post synaptic protein muscle specific kinase (anti-MUSK) in 40% where ach abs are negative
thymectomy may be required.
late in disease there is thymic atrophy
```
Mx:
pyridostigmine 30mg qds
increasing incremental steroid o alternate days
IV Ig
FVC monitoring +/- ITU
```
41
5 causes of extensor plantar and absent knee and ankle jerks
extensor plantars = UMN - babinksi sign
absent reflexes = LMN
5 causes
1. subacute combined degeneration of the cord
2. Hereditary Cerebellar Ataxia - Friedreichs / spinocerebellar ataxia (heterogenous group of inherited ataxia -
3. MND
4. Combined pathology neuropathy and myelopathy- DM to give sensory loss of reflexes and Cervical spondlylolithiasis / trauma / syrinogmyellia causing compression to give UMN
5. Syphillis taboparesis
6. Conus medullaris
42
What is Webers Syndrome
IPSILATERAL COMPLETE CN 3 PALSY
CONTRALATERAL HEMIPARESIS
MIDBRAIN STROKE POST CEREBRAL ARTERY
substantia nigra gives contralateral PD
43
Stroke Investigations
```
CT HEAD / CTA WITH CONTRAST
CREAT AND INR
THROMBOLYSIS WITHIN 4 HR
THROMBECTOMY WITHIN 6 HR
24 HR TAPE
ECHO
DOPPLER STUDIES
THROMBOPHILIA SCREEN
MRI HEAD TO CONFIRM
```
44
Stroke Differentials
```
Tia
SOL
CVA
Meningitis
Todds paresis
Functional
MS
```
45
Rule of 4
| Brainstem anatomy and brainstem vascular syndromes
Blood supply
Paramedian supply = median syndromes
circumferential supply = lateral syndromes
Anterior inferior cerebellar artery (AICA),
Posterior inferior cerebellar artery (PICA)
Superior cerebellar artery (SCA)
46
Brainstem blood supply
paramedian and circumferential branches of anterior and posterior inferior cerebellar arteries and superior cerebellar artery
47
Brainstem anatomy and cranial nerves
Midbrain
2 CN nuclei medial - 3 - 4
superior cerebellar artery
CN3 KO = ptosis / meiosis / eye down and out
CN4 KO = failed adduction / failed vertical downsize = Vertical diplopia
```
Pons
4 CN nuclei
1 medial - 6 - INO ipsilateral
3 lateral - 5 - 7 - 8
5 - masseter weakness and sensory loss
7 - facial weakness - ant 2/3 taste lost
8 - sensorineural loss + vertigo
```
```
Medulla
4 CN nuceli
1 medial - 12
3 lateral - 9 - 10 - 11
12 - weak tongue
9 - loss 1/3 post taste
10 - loss Gag reflex - sensory and motor
11 - trapezius weak
```
48
What structures lie in the midbrain
blood supply
Superior cerebellar artery
medial CN 3-4
4 medial structures
1 - Motor nuclei
2 - Corticospinal tract - motor tract - not decussated yet
3 - Medial longitudinal fasciculus - INO ipsilat
4 - Medial Lemniscus - Posterior columns - light touch / vibration / proprioception
4 lateral structures
1 - Sympathetic chain - ipsilat Horners
2- Sensory pathway - 5
3- Spinothalamic lateral - pain / temp - crosses in cord
4- Spinocerebellar - coordination - ipsilateral
49
what structures lie in the pons
blood supply
Anterior inferior cerbellar artery
4 CN nuclei
5-6-7-8
Medial
1. Motor nuclei - 6
2. Corticospinal tract - not decussated yet
3. Medial longitudinal fasciclus - ipsilat INO
4. Medial lemniscus - Posterior columns
Lateral
1. Sympathetic chain - ipsilat horners
2. Sensory pathway - 5 (masseter and sensation_
3. Lateral spinothalamic - crosses in cord
4. posterior spino cerebellar
7- facial weakness - X 2/3 ant taste
8 - vertigo + sensorineural hearing loss- ipsilateral
50
what structures lie in the medulla
Blood supply
Post inferior cerebellar artery
4 CN nuclei
9-10-11-12
```
medial
1. motor nuclei - 12 - tongue weakness
2. Corticospinal tract - DECUSSATE HERE
3 . Medial longitudinal fasciculus
4. Medial Lemniscus - posterior column - Decussate here
```
Lateral
1. Sympathetic chain - ipsilat horners
2. Sensory pathway
3. Spinothalamic - crosses in cord
4. Spinocerebellar - coordination - ipsilateral
51
What does the corticospinal tract supply and where does it decussate
Motor pathway in medial brainstem
crosses in medulla to contralateral side
travels in CST and gives signals from anterior horn
lesions above medulla therefore will give contralateral signs - left lesion - right hemiparesis
lesions below the medulla will give ipsilateral signs
e.g. cord lesion on right gives right hemiplegia
52
What does the spinothalamic tract supply and where does it decussate
Lateral brainstem
sensory pain and temp
crosses at level of cord at supply dermatome level
53
What does the spinocerebellar tract supply and where does it decussate
lateral brainstem
does not decussate
sensory info on coordination
54
What does the Medial Lemniscus supply and where does it decussate
medial brainstem
decussates in medulla pyramids
forms posterior columns
sensory information on light touch / vibration and proprioception
55
How would a midbrain stroke present
superior cerebellar stroke
MEDIAN STROKE - 3rd nerve palsy +/ 4
1. contralateral hemiplegia (above decussation Corticospinal tract)
2. ipsilateral complete CN 3 palsy
eye down and out / meiosis / ptosis
3. ipsilateral complete CN 4 palsy
failure of done gaze and adduction - V diplopia
4.contralateral light touch / vibration and proprioception loss due to medial lemniscus
5. INO if MLF affected
LATERAL STROKE
1. Ipsilateral Horners
2. contralateral loss of pain and temp - spinothal
3. Ipsilateral coordination problems - spinocerebellar
56
How would a pons stroke present
anterior inferior cerebellar artery
MEDIAN STROKE
1. contralateral hemiplegia (above decussation Corticospinal tract)
2. median longitudinal fasciculus - INO ipsilateral
3.contralateral light touch / vibration and proprioception loss due to medial lemniscus
```
LATERAL STROKE
Facial weakness - 7 + 2/3 ant taste
sensorineural deafness + vertigo - 8
Facial paraesthesia
1.Ipsilateral Horners
2.ipsiliateral facial paraesthesia - 5
3. contralateral loss of pain and temp - cross cord
4. Ipsilateral coordination problems - spinocerebellar
```
57
How would a medulla stroke present
MEDIAN STROKE - NO INO (MLF pons and midbrain)
1. contralateral hemiplegia (above decussation Corticospinal tract)
2. contralateral light touch / vibration and proprioception loss due to medial lemniscus
3. tongue weakness - 12
LATERAL STROKE - Face spared (7 above in pons)
10-loss of gag and pallet weakness
9-loss of 1/3 post taste + pharyngeal sensation
11 - trap weakness + SCM
1.Ipsilateral Horners
2. ipsilateral facial sensory loss
2. contralateral loss of pain and temp - cross cord
4. Ipsilateral coordination problems - spinocerebellar
58
Horners syndrome
Pupil constricted - meiosis
partial ptosis
anhidriasis
side ipsilateral to lesion
brainstem stroke at highest
Causes:
classic - pan coast tumour infiltration of sympathetic chain
lateral brainstem syndromes
59
lateral brainstem syndromes
```
Think
4 S's
ALL:
sympathetic chain
- ipsilateral horners
sensory pathway
- 5 sensation loss + Masster weakness
spinothalamic
- contralateral pain and temp loss (cross cord)
spinocerebellar
- ipsilateral ataxia / past point / dysdiadochokinesia
```
midbrain:
pons: FACIAL WEAKNESS / VOMITING / VERTIGO / HEARING LOSS
CN 5/7/8
ipsilateral facial paraesthesia
ipsilateral facial weakness - forehead spared
ipsilateral sensorineural hearing loss and vertigo + vomit
```
medulla: DYSPHAGIA / DYSARTHRIA
CN 9/10/11
9 - 1/3 post taste loss
pharyngeal sensory loss
10-gag reflex loss
hoarseness
11- trapezlus and SNC weakness
ipsilateral cerebellar signs
```
60
LATERAL MEDULLARY SYNDROME
ALL LATERAL SYNDROMES HAVE POTENTIAL FOR:
1. Ipsilateral horners
2. Facial paraesthesia
3. SPINOTHALAMIC
- contralateral loss pain and temp
4. SPINOCEREBELLAR
- Ipsilateral ataxia / dysdiadochokinesia /
Medulla Specific based on CN
lateral = 9-10-11
DYSPHAGIA (gag and pharyngeal sensation / DYSARTHRIA (x) /
STC and Trap weakness
61
Describe what an INO is
INTERNUCLEAR OPTHALMOPLEGIA
describes a failure of yolked eye movements in which there is a lesion affecting the MLF preventing abduction of the eye on the affected side and contralateral nystagmus in the unaffected eye with lateral gaze diplopia
Cause:
Medial Brainstem stroke - pons or higher
Pons median stroke
- ant inferior cerebellar artery
- INO
- contralateral hemiplegia /
- contralateral loss light touch, proprioception and vibration
Midbrain
- CN3 - ptosis / meiosis / eye down and out
- CN 4 - failure to accommodate / vertical gaze = diplopia
- INO
- contralateral hemiplegia /
- contralateral loss light touch, proprioception and vibration
IF NO LATERAL STRUCTURES ARE AFFECTED THERE SHOULD NOT BE ANY OTHER CEREBELLAR SIGNS
62
What would you suspect if both lateral and medial brainstem signs were present
e. g. INO + ataxia (pons or midbrain)
e. g. DYSPHAGIA / DYSARTHRIA / HEMIPLEGIA (MEDULLA)
THINK this means BASILAR STROKE
63
CORTEX PROBLEM
HIGHER FUNCTION AND REASON
| BEHAVIOURAL CHANGES
64
SUBCORTEX PROBLEM
UMN SIGNS
- hypertonia
- pyramidal weakness - i.e. contralateral hemiplegia / hypertonia / spasticity / clonus / brisk reflexes / upping plantar
- processing problems - slow thought
65
Basal Ganglia Problem
Extra-pyramidal signs - Parkinsonism
- Cog wheeling / resting coarse tremor
- Rigidity - clasp knife or lead pipe - not velocity dependent
- Bradykinesia
- Dyskinesia
- Dystonia - muscular spasm or posture problems (stooped)
- tapping on floor with foot / marching on spot
- postural instability
66
Brain stem problem
Divide into
1. median or lateral affects or both
2. CN involvement = decide on level
CN3/4 - midbrain
INO or vertigo / vomiting / facial weakness / facial paraesthesia = pons and above
dysphagia / dysarthria = medulla
Classically all medial:
contralateral hemiplegia - corticospinal
contralateral hemiparesis - posterior columns - medial lem
Classically all lateral
Horners - miosis / partial ptosis / anihidriasis
spinothalamic - contra pain / temp loss
spinocerebellar - ipsilateral ataxia / dysdiadochokinesia /
67
Cerebellum problem
CEREBELLUM receives spinocerbellar inputs from PONS
lesion therefore gives ipsilateral cerebellar signs for coordination due to this tract never crossing
DANISH
Dysdiadochokinesia - failure to perform rapid alternating movements e.g. walking
DYSMETRIA - past pointing - fails to judge distance
Ataxia - broad based gait + REBOUND - IPSILATERAL
Nystagmus at rest
Intention tremor
Slurred speech - dysarthria
Hypotonia arms and legs
68
Explain Rebound phenomenon
The Cerebellum calibrates muscular force
Dysfunction therefore results in an imbalance of forces controlling a limb that is destabilised or in motion
Rebound phenomenon demonstrates the dysfunctional attempt to rebalance a limb in space that has had an external force applied
This manifests as the upper limb oscillating in an exaggerated manner before coming to rest again in the neutral plain
69
What is pseudoathetosis
Involuntary writhing of fingers / limbs due to impaired proprioception (Posterior columns of medial lemniscus)
check for difference between cerebellar sensory loss and other causes of peripheral neuropathy
70
Causes of cerebellar Syndrome
1. Global changes? - toxic / MSA / drug induced (carbamazepine / barbiturate /
Surgical sieve - VITAMIN C
VASCULAR
Cerebellar stroke - ipsilateral to ataxia side / coordination loss
Cerebellar aneurysm or bleed
HBI
Infective
Cerebellar abscess - endocarditis / hydatid cyst / toxoplasmosis
autoimmune
MULTISYSTEM ATROPHY - PREDOMINANCE FOR CEREBELLUM
-Extrapyramidal features bilaterally
-autonomic dysfunction - post drops +incontinence/retent
-muscle rigidity
-involuntary flexion
Multiple Sclerosis - INO / RAPD / UMN
```
metabolic
drugs - carbamazepine / phenytoin / barbituates
alcohol
b12
Wilsons / PSC
Vit E deficiency
Hypothyroidism
Nutritional - coeliac / SBS /
```
neoplastic
SOL
paraneoplastic - associated with Hodgkins / gynae and testicular germ cell tumours / SCLC / breast
```
Congenital
SPINOCEREBELLAR ATAXIA - AUT DOM (25 types)
-extrapyramidal signs
-UMN
-peripheral neuropathy
-INO
FRIEDREICHS ATAXIA - AUT REC - 5-18 years
-optic atrophy
-pes cavus
-DM
-HOCM
-deafness
-spasticity - WHEELCHAIR
-peripheral neuropathy
ATAXIC TELANGIECTASIA - AUT REC
-skin and eye telangiectasia
-dystonia
-chorea
```
71
Surgical sieve - vitamin C
```
vascular
infective
trauma / tumour
autoimmune
metabolic
inflammatory - iatrogenic
neoplastic
```
congenital
72
What anti-epileptics can cause cerebellar syndromes
carbamazepine / phenytoin / barbituates
73
What are the absolute contraindications to thrombolysis in stroke?
Absolute contraindications for alteplase therapy in AIS>
- History or evidence of ICH
- Clinical presentation suggestive of SAH
- Known arteriovenous malformation
- Systolic BP exceeding 185 mm Hg or diastolic BP exceeding 110 mm Hg despite repeated measurements and treatment
- Seizure with postictal residual neurologic impairment
- Platelet count below 100,000/µL
- Prothrombin time (PT) above 15 or INR above 1.7
- Active internal bleeding or acute trauma (fracture)
- Head trauma or stroke in the previous 3 months
- Arterial puncture at a noncompressible site within 1 week
- Relative contraindications for alteplase therapy for AIS include the following:
Pregnancy
Rapidly improving stroke symptoms
Myocardial infarction (MI) in the previous 3 months
Glucose level lower than 50 mg/dL or higher than 400 mg/dL
The eligibility criteria in the extended time period of 3 to 4.5 hours are similar to those for patients treated at earlier time periods. In addition, the following exclusion criteria must be considered: patients older than 80 years, those taking oral anticoagulants regardless of their INR, those with an NIH Stroke Scale score higher than 25, and those with both a history of stroke and diabetes.
74
What is the NIH stroke scale?
National Institutes of Health Stroke Scale
https://stroke.nih.gov/documents/NIH_Stroke_Scale.pdf
11 items scored on presentation / post thrombolysis / post thrombectomy
score 0-4 in most categories
0= normal function
4= severe impairment
score /42
```
Score Stroke severity
0 No stroke symptoms
1-4 Minor stroke
5-15 Moderate stroke
16-20 Moderate to severe stroke
21-42 Severe stroke
```
75
What are the NIHSS categories - Detail
11 categories:
```
1 - Alertness
2 - Opthalmoplegia
3 - Visual field defects
4 - Face
5 - power arm
6 - power leg
7 - coordination
8 - sensory
9 - recognition
10 - speech
11 - Neglect
```
1. Level of Consciousness
A- responsiveness. - AVPU
B-answer questions - Age and month
C-follow verbal commands - open close eyes / grip release hand
```
2. Horizontal Eye Movement
Conjugated eye deviation is present in approximately 20% of stroke cases.
-right hemispheric strokes a
- basal ganglia
- temporoparietal cortex.
- midbrain and pons - CN3/4/6
-OPTHALMOPLEGIA
```
```
CN3 = ptosis / eye down and out / pupil dilated and fixed
CN4 = cannot invert gaze or look down
CN6 = INO cannot adduct eye on affected side - MLF lesion = MS or midbrain or pons medial brainstem syndrome - compensatory nystagmus with good eye
Horners = ptosis / miosis / anhidriasis - ipsilateral brainstem syndrome due to damage of sympathetic chain
```
3. Visual field test
- Visual field defect may point to lesion
Bitemporal hemianopia - optic chaism = tumour / pit apoplex
-Bilateral cortical blindness - bilateral cerebellar stroke
-superior quadrantinopia - temporal infarct of optic rad
-inferior quadrantinopia - parietal infarct of optic rad
-homonymous hemianopia - cortical infarct on contralateral side to vision loss
-homonymous hemianopia with macula sparing - cerebellar lesion - macula region of cerebellum supplied by MCA
4. Facial Palsy - forehead sparing
- paralysis is most pronounced in the lower half of one facial side.
- Minor paralysis; function is less than clearly normal, such as flattened nasolabial fold or minor asymmetry in smile
- Complete facial Hemiparesis
5. Motor power arm
- hold arms out for 10 seconds
6. Motor power leg
- Hold leg up for 5 seconds
7. coordination
- unilateral cerebellar lesion, and distinguishes a difference between general weakness and coordination.
- finger nose - dysmetria
- heel to shin - dysmetria
- Disdiadochokinesia
UNILATERAL
-lesion is ipsilateral!
Vascular - embolic / ICH or /cva
- brainstem or cerebellum
Brainstem = midbrain / pons / medula
Midbrain - superior cerebellar artery
Pons - anterior inferior cerebellar artery
Medulla - posterior inferior cerebellar artery / basillar / vertebral
Cerebellar - PICA / AICA / SCA
-SOL-->cerebellum or cerebellar pontine angles / abscess and granuloma
-MS and demyelination
localise for brainstem
INO = pons and above - medial syndrome - CN 6
Horners = ipsilateral anywhere - lateral syndrome
Dyshagia / dyasarthria - Lat Medulla - CN9/10/12
vertial gae palsy / ptosis / down + out - Midbrain CN3/4
Cerebellum = DANISH
Disdiadochokinesis and Dysmetria (pastpointing and heelshin) / Ataxia / Nystagmus / Intention Tremor / Scanning speech / Hypotonia
BILATERAL - VITAMIN C
Vascular
-Bilateral stroke
-Bilateral CVA
Inflammation
-bilateral demyelination
Trauma
- vertibral artery dissection
- SAH
Metabolic
- b12
- alcohol
Iatrogenic
- phenytoin
- carbemazepine
8. Sensory
- pinpricks in the proximal portion of all four limbs.
WHEN DOES IT FEEL NORMAL?
Sensory level or hemiparesis
- neglect
Brain damage leading to neglect usually involves infarcts in the inferior parietal lobe, temporo-parietal junction and/or the superior temporal lobe.
9. Language
Object recognition
```
10. Speech
baby hippopotamus
british constitution
lalala
mamama
bababa
- scanning speech - cerebellar
- monotonic speech - PD
```
Dysarthria typically affect areas such as the anterior opercular, medial prefrontal and premotor, and anterior cingulate regions
- Broca's area - Left frontal lobe = speech production
- Wernicke's area - Left temporal gyrus = speech comprehension
11. Extinction and Inattention
- "double simultaneous stimulation".
- During this time the examiner is alternating between touching the patient on the right and left side.
- examiner touches the patient on both sides at the same time.
- To test extinction in vision, the examiner should hold up one finger in front of each of the patient's eyes and ask the patient to determine which finger is wiggling or if both are wiggling. The examiner should then alternate between wiggling each finger and wiggling both fingers at the same time.
Inattention on one side in one modality; visual, tactile, auditory, or spatial
Hemi-inattention; does not recognize stimuli in more than one modality on the same side.
76
What is the oculocephalic manoeuvre
DOLLS EYE REFLEX
If unable to assess for opthalmoplegia due to paralysis / non communication use OCULOCEPHALIC MANOEUVRE.
This is done by manually turning the patient's head from midline to one side and assessing the eye's reflex to return to a midline position.
If the eye position remains fixed in orientation of turned head this is a negative reflex and implies damage to the nuclear gaze centres - voluntary and reflexes damaged
IIf there is damage to the cortical gaze centre then voluntary movement is lost but the doll's eyes reflex is retained.
77
What is a Lacunar infarct
A Lacunar stroke results from an occlusion of a deep penetrating artery
```
Lesions occur in deep nuclei of
Brain
-PUTAMEN
-THALAMUS
-CAUDATE
```
Brainstem
-PONS
POSTERIOR LIMB INTERNAL CAPSULE
- internal capsule is mainly corticospinal tract
- motor fibres from motor cortex--> corona radiata-->internal capsule-->cerebral crus-->into midbrain corticospinal tract
The corresponding lesions occur in the deep nuclei of the brain (37% putamen, 14% thalamus, and 10% caudate) as well as the pons (16%) or the posterior limb of the internal capsule (10%)
NB - internal capsule also carries corticobulbar tract
-most CN are innervated bilaterally so single lesion will usually not cause a weakness
HOWEVER this does not apply 7 2/3
Therefore facial palsy with forehead sparing will localise leision to contralateral side
Same is true for CN12
By definition the watershed supply loss
78
Whata re the 4 classic lacunar syndromes
Recognised symptomatically
lesion localisation for these can be anywhere in the following structures
1. posterior IC / corona radiata
2. putamen / thalamus / caudate nucleus
3. pons
1. Isolated motor paresis - 35-55%
contralateral cst
2. Isolated sensory loss
contralateral posterior columns
3. Clumsy hand and dysarthria syndrome
4. Ataxic Hemiparesis - pons / post IC / corona radiata
contralateral cst and pc plus fibres that would intereact wth spinocerebellar tract efferents in pontine nucleus in vermis
79
What is Shingles
Shingles is a cutaneous manifestation of reactivated Herpes Zoster from a dorsal root ganglion
It therefore typically manifests within a specific dermatome
sensory loss
Trigeminal root = Gasserian ganglion
80
What is Ependymoma and what else could the symptoms represent?
Tumour of CNS
Typically presents within the cord or intracranially
Paeds = >intracranial
signs:
new onset back pain
Bilateral UMN signs - CST
Bilateral sensory signs - STT
Contralateral or bilateral coordination problems
loss of pain and temp sens - contra
potentially sensory level if whole cord involved at a level
If hemi umn and sensory always consider unilateral SOL
Ix:
MRI cord - weighted - look for high intensity signal change
```
Diffs:
SOL - any CN involvement / behaviour change / dysphagia / dysarthria / facial weakness etc
Disc prolapse
corda equina
corda medullaris
vertebral fracture / compression / mets
```
81
Describe a typical complex partial seizure
2/3 seizures arising from temporal lobe with typical patterns
```
associated:
1) Aura - visual or perceptual
-deja vu - strange familiarity
-jamais vu - unfamiliarity
2)Motor element
-lip smacking
-gestural
-chewing
3) Evolution
60% evolve to GTCS
```
Typically
loss of awareness but not LOC unless evolves to 2ndary GTCS
last 2-3 mins
post ictal phase after
BG:
Febrile convulsion as a child = MESIAL TEMPORAL SCLEROSIS
SOL
Old stroke
82
Catoplexy
Transient paralysis in response to emotional outburst
fits of crying / laughter
Also associated with narcolepsy
- uncontrolled sudden LOC - pt falls asleep
83
Treatment for acne vulgaris
Isotretinoin
synthetic 13 cis isomer of natural trans-retinoic acid
decreases sebum production and sebaceous gland szie
```
issues:
reduce efficacy of carbemazepine
base line LFT
fasting ;ipids
mood changes
```
```
Biggest issue
VERY TERATOGENIC
COUNSEL PRIOR
BHCG BEFORE STARTING
COMMIT TO CONTRACEPTIVE 1 MONTH PROR TO STARTING AND 1 MONTH POST CESSATION
```
84
What is the typical presentation of GBS
ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY
(AIDP)
Description:
Ascending demyelinating milder sensory and predominantly motor neuropathy typically post infective with prescence of anti-ganglioside antibodies and raised CSF protein.
Typically infectious trigger - viral / myocplasma / compylobacter
Presentation:
rapidly evolving ascending weakness
mild sensory loss
hypo- or areflexia
Diagnosis:
CSF = albuminocytosolic differentiation in 90% of cases
-also rules out other causes
Anti GM1 or GQ1b - anti-gnaglioside antibodies
Antibodies:
anti-ganglioside - GM1
Varient - MILLER FISHER - anti G1QB
85
How do you diagnose GBS
1. LP
- Raised protein in 90%
- aka albuminocytosolic dissociation (i.e. exudative CSF)
2. Antiganglioside antibodies in GBS - anti GM1
- Miller Fischer variant - Anti G1Qb
GBS= anti-Gm1 - post viral / c.jejuni infection
pain
ascending weakness and sensory loss
MFS - Anti-G1qb - rarer
opthalmoplegia
loss of DTR
ataxia
86
What is the treatment for GBS
1. supportive
- monitor FVC - <1.5L = ITU discussion
- intubation and ventilation
2. IV immunoglobulin / plasmaphoresis
3. dalteparin
4. ECG monitoring for arrhythmias
87
How does anterior spinal artery occlusion present
RF = AF
Acute cord ischaemia
ANTERIOR SPINAL ARTERY SYNDROME
paraparesis and sensory loss
PRESERVE POST COLUMNS = vibration AND PROPRIO
LOSS
SPINOTHALAMIC = MOTOR
LATERAL SPINOTHALAMIC = PAIN AND TEMP
POSTERIOR SPINOCEREBELLAR = COORINATION
88
How does Bells palsy present?
hemi facial weaknes
+/- hyperacusis
Hyperacusis occurs if there is paralysis of stapedius muscle
Treatment with pred < 72 hours post onset
hypromellose eye drops / artificial tears to prevent ulceration
89
What is the difference between bells and facial weakness in stroke
Bells = LMN lesion - no forhead sparing
Stroke = UMN - collateral supply from contralteral VII intact and spares forhead
90
What does HTLV-1 cause
Tropical spastic paraparesis
sexually transmitted
retention
constiptation
hyperreflexia
increased tone
upgoing plantars
NORMAL COORDINATION (Post spinocerebellar intact
91
How does MS present
inflammatory demyelinating disease
typically presents in relapsing and remitting fashion
optic neuritis
UMN pattern / lmn pattern
opthalmoplegia - INO via MSF
Ataxia
92
How does optic neuritis present
Acute inflammatory demyelination of optic nerve
Blurred vision and loss of acuity
strong MS association
unilateral
pain worse on eye movement and loss of colour differentiation
may be retrobulbar so no features of optic nerve changes
typically ON is pale
Mx:
IV methyl pred 1g/24hr
93
Neuroleptic malignant syndrome
Typical secondary to OD on anti-psychotics
UMN rigidity
Pyrexia
rhabodmyolysis - myoglobinurea
AKI
Mx:
cooling with IV fluids and hyratoin
stop meds
Bromocriptine and dantrolene to promote dopaminergic interactions
94
What is a common association between MG and pregnancy
Relaspe of myaesthenis gravis which was in remission / controlled
diplopia
fatigue esp end of day
ptosis
remember MG - fatigue on repetitive action due to depletion of ACtH from pre-synaptic terminal and receptors blocked by Ab - anti - ACTHR ABs and Anti MUSK abs (Vs muscle tyr kinase receptor)
LEMS - repeated excitation will result in greater release of ACTH and a fortifying response as abs are vs vgcc - more excitation / more calcium / more acth degranulation /more propogation of AP to NMJ
95
What is STEAL syndrome
Restricted arterial flow resulting in watershed ischaemia to supplied territory
variety of reasons
```
Subclavian steal due to atheromatous disease
steal due to vascula access device
Takayasu's ateritis
thoracic outlet ocmpression
aortic repair
```
96
What are the neurological manifestations of Wilsons disease
Typically present in 30s
Gene defect = ATP7b = copper transporter protein constitutive activation
Copper overload
diagnosis = reduced Caeruloplasmin / urinary Cu increased
```
Neuro:
extrapyrmidal parkinsonian due to Cu deposition int he BG
-tremor
-micrographia
-bradykinesia
-lead pipe rigidity
-gait disturbance
-speech disturbance
-dementia
-death
```
also hepatic manifestations and KF rings
97
Parkinsonian tremor
Asymmetric
extinguishes on movement
worse at rest
98
essential tremor
action tremor - symmetrical
worse on movement
worse with anxiety
propranolol
99
Consequence of terminating parkinsons medication abruptly
neuroloeptic malignant syndrome
```
Pyrexia
rigidity
labile BP
dropped GCS
rasied CK and rhabdo
```
15-30% mortality
Associated with anti-psychotics - promote dopa
Management:
stop antipsychotics
reinstate dopa drugs
fluids for slow cooling
100
How does cluster headache present
```
clusters of headache
severe
last up to 1 hour
come in clusters - symptom free inbetween
present over several weeks
```
lacrimation of eye
conjunctival injection
severe hemicranial pain
nasal conjestion
Mx:
triptans
high flow oxygen
101
How does CIDP present?
Chronic inflammatory demyelinating polyradiculopathy- MOTOR
MRI gad - enhancement of anterior horns
proximal weakness
reduced tone
loss of reflexes
LOWER MOTOR NEURONE PICTURE
```
steroids
azathioprine
Ig
plasmaphoresis
gabapentin and amitriptyline for neuropathic pain
```
102
How do CPA tumours present?
deafness / tinitus / vertigo e.g. acoustic neuroma
103
What are chiari malformations?
3 types
anatomical variants with symptoms
1 cerebellar tonsil extends out of foramun magnum
- Hydrocephalus - nausea / ataxia / vertigo / papilloedema
- compression of cord
shawl sensory loss
weakness of small muscles of the hand
2 Further projection of the cerebellum and medulla into the cervical canal - pons and 4th ventricle tensed and stretched.
3 Cervical spinabifida
104
What is sporadic CJD and how does it present?
Spontaneous formation of prion protein
i.e. not transmitted
Typically occurs post travel / recent infection
14-3-3 protein in CSF
Ataxia
Behaviour change and memory loss
UMN signs
dystonia
MRI uptake signal in putamen and caudate (corresponding to parkinsonian type extrapyramidal signs)
105
What are causes of UMN and LMN mixed signs
e. g. increased tone / absent reflexes
e. g. upgoing plantars / flaccid weakness
e. g. fasciculations / increased tone
MND
- AML - hits motor cortex / cord / lmn
- Classical AML = anterior horn and LMN only
- progressive muscular atrophy - all LMN too
SCDC
Dual pathology - DM1 + cervical fracture
Syphillis taboparesis
Conus Medullaris
106
How is myaesthenia treated?
Symtoms:
pyridostigmine acetylcholinesterase i
30mg TDS
may precipitate cholinergic crisis with too much ACh
rapid reversal needed as main Sx is increased weakness
```
Major issues in MG = myasethenic crisis which is weakness of resp muscles requiring intubation and ventilation
rapid imunomodulation is key
IVIG
induction
thymectomy
```
also
cyclophos
mycofenolate
rituximab
107
Recurrent stroke already on aspirin. What should be added / removed / swapped for secondary prevention?
After 3 months stop aspirin and switch to clopi long term
alt if clopi not tolerated continue aspirin and dipyridamole
108
Causes of UMN lesion
Ischemic or haemorrhagic
SOL
Demyelination
109
Investigation muscular dystrophy acute presentation
```
EMG divebomber potentials
Muscle biopsy
Ct head in acute setting
Mri head and spine with gadalinium
Baseline bloods
```
110
Treatment of muscular dystrophy
Supportive care
Peg
Steroids may slow down weakness for 6months to 2 years
Gene targetted therapies for specific MD to increase protein
111
Key Muscular dystrophy examination give away
FSHMD
winging of scapula
Weakness of eye closure
BMD
Pes Cavus
Calf hypertrophy
```
MD
Mask face
Ptosis bilaterally
Percussion myoclonus
Slow grasp release
```
