Neurology Flashcards
1
Q
Differentiate between CNS & PNS?
A
CNS = brain & spinal cord, collection of cell bodies = nuclei
PNS = outside the CNS
collection of cell bodies = ganglia
2
Q
What connects the CNS & PNS?
A
- 12 cranial nerves - brain & head and neck
- 31 spinal nerves - spinal cord and periphery
3
Q
the Autonomic nervous system:
- what is it
- where is it
- what are its divisions
A
- system beyond conscious control, e.g. viscera, smooth muscle, glands, heart
- part in CNS part in PNS
- sympathetic and parasympathetic divisions
4
Q
What is the sympathetic nervous system
A
- essentially fight or flight system
- ganglia close to spinal cord
- supply visceral organs and structures of the superficial body organs
5
Q
Effect of the sympathetic nervous system
A
- **increases** heart rate & force of constriction in heart
- **vasoconstriction**
- **bronchoDILATION**
- sphincter contract
- decreased gastric secretion & motility
- male ejaculation
6
Q
Major structure of the sympathetic nervous system?
A
Sympathetic trunk - two chains of ganglia lying close to spinal cord (one on each side)
7
Q
Neurotransmitter of sympathetic nervous system and receptor?
A
Preganglionic: Acetyl-choline ACh to nicotinic receptors
Effector cell: Noradrenaline to adrenergic receptors
8
Q
What is the parasympathetic nervous system
A
- essentially the rest and digest system
- some ganglia in brain, some near the organs
9
Q
Major structure of the sympathetic nervous system?
A
Cranial nerves 10, 9, 7, 3
10
Q
Neurotransmitter of sympathetic nervous system and receptor?
A
Preganglionic: Acetyl-choline ACh to nicotinic receptors
Effector cell: Acetyl-choline ACh to muscarinic receptors
11
Q
Effect of the sympathetic nervous system
A
- **decrease** heart rate and force of contraction
- **vasodilation**
- **bronchoCONSTRICTION**
- increased gastric motility and secretion
- spincter relaxation
- male erection
12
Q
How many pairs of spinal nerves do we have
A
31 - 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, 1 coccygeal
13
Q
Where is the motor cortex located
A
Precentral gyrus, **frontal lobe**!!
14
Q
Where is the somatosensory cortex located
A
Postcentral gyrus, **parietal lobe**!!
15
Q
Where is the visual cortex located
A
**Occipital lobe**
16
Q
Where is the auditory cortex located?
A
Lateral fissure, **temporal lobe**
17
Q
What is the frontal lobe responsible for?
A
higher intellect, personality, mood, social conduct and language (in dominant hemisphere only)
remember by teens - they’ve not developed frontal lobe properly yet lol also pituitary tumour in optic chiasm can cause mood changes
18
Q
What is the parietal lobe responsible for?
A
language on dom
sensory perception and integration on nondom
19
Q
What is the temporal lobe responsible for?
A
memory and language primary auditory cortex is here!!
20
Q
What is the occipital lobe responsible for?
A
vision - primary visual cortex is here!
21
Q
If a patient has
- recognition deficits (agnosias) - e.g. cannot recognise basic sounds or faces
Where has the cerebrovascular accident occurred?
A
Temporal lobe
22
Q
If a patient has
- personality and behavorial changes
- inability to solve problems
Where has the cerebrovascular accident occurred?
A
Frontal lobe
23
Q
If a patient has
- visual field defects
and this is a cerebrovascular accident - where has this occurred?
A
Occipital lobe
24
Q
If a patient has
- attention deficits, e.g. contralateral hemispatial neglect syndrome (does not pay attention to a side of body)
Where has the cerebrovascular accident occurred and what is the relation to the side of the body neglected?
A
Parietal lobe
Lesion would be on the opposite of the neglected body side
25
What is the cerebellum responsible for?
Generally, movement
26
Which neoplasm causes bitemporal hemianopia?
pituitary adenoma
ah the good old days
27
What is a dermatome
area of skin supplied by a single spinal nerve
28
What is a myotome
muscles supplied by a single spinal nerve
29
Name cranial nerves in order!!
| (mnemonic & names)
Oh Oh Oh They Traveled And Found Voldemort Guarding Very Ancient Horcruxes.
Olfactory, optic, oculomotor, trochlear, trigeminal, abducens, facial, vestibulocochlear, glossopharyngeal, vagus, accessory, hypoglossal
30
Nerves for sensory, motor or both?
| (mnemonic)
Some say money matters but my brother says big brains matter most
31
Parasympathetic nerves?
| (mnemonic & which)
1973
10 - vagus
9 - glossopharyngeal
7 - facial
3 - oculomotor
32
Nerves of the eye?
| (mnemonic & what they stand for)
SO4 LR6 3
33
How would you interpret the results of CN I tests
If \*\*anosmia\*\*: causes are
- meningioma
- trauma - skull or cribriform plate
- Parkinson's
- mucus block / covid / genetics
34
How would you test CN II
visual acuity
- distance - Snellen chart & pinhole
- colour vision - ishihara plates
fields
- neglect / inattention
- field - formal is \*\*Amsler chart\*\*
- blind spot
optic disc
- fundoscopy
Pupil
- pupillary light reflex (direct - ipslateral, consensual - contralateral, swing check relative afferent pupillary defect)
- accomodation reflex - distant to near
- size, shape
35
Visual field defects - key in investigation?
Test one eye while covering the other! Patient often do not realise
36
\*\*bitemporal hemianopia\*\*
- what is it?
- pathology?
- loss of temporal visual field in both eyes
- optic chiasm tumour
37
\*\*Homonymous field defects\*\*
- types
- pathology?
- hemianopias or quadrantanopias
- same side of visual field in each eye
- pathology is behind optic chiasm in visual pathways: stroke, tumour, abscess
38
\*\*Scotoma\*\*
- what is it?
- pathology?
- absent or reduced vision surrounded by areas of possible vision
- wide range, including demyelinating (MS, DM)
39
\*\*Monocular vision loss\*\*
- what is it?
- pathology?
- total loss of vision in one eye secondary to optic nerve pathology or ocular disease
40
How would you interpret results of a fundoscope exam?
if papilloedema, causes are
- usually \*\*ischaemia\*\* - intracranial bleed possible
- \*\*hypertension\*\* - systemic, intracranial (benign & malignant → suspect a brain tumour!)
- \*\*headache\*\*
- \*\*brain tumour\*\* causing compression
- prolonged CNS infection
41
Nerves of the eye again!!
| (mnemonic & what they stand for)
SO4 LR6 3
42
Strabismus
- what is it?
- pathology?
- how would you assess it?
- condition where eyes do not properly align with each other when looking at an object
- pathology affecting III, IV & Vi (and/or) can all cause strabismus
- test by corneal reflex test / Hirschberg test and or cover test
43
How would you test CN VII?
By facial expressions:
- raising eyebrows
- closing eyes
- blowing out cheeks
- smiling
- pursing lips
44
How to differentiate between a Facial Palsy and a stroke?
stroke is **forehead sparing** whereas the nerve palsy is not!!
45
Differentiate between Bell's palsy and facial palsy?
Facial palsy is termed Bell's palsy when it is idiopathic
46
Define stroke?
neurological deficit lasting **_longer than_** 24 hours due to vascular compromise
47
What are ischaemic strokes? Subclassify?
Reduction in cerebral blood flow due to arterial occlusion or stenosis
= anterior / middle / posterior cerebral artery / lacunar
(thrombotic / embolic)
48
How can you distinguish haemorrhagic and ischaemic strokes?
No reliable way
tho intracerebral more often associated with **_severe headaches or coma_** → ICP ++
patients on anticoag: assumed to have haemorrhage unless proven otherwise
49
Associated risk factors for ischaemic strokes?
AF - higher risk of thrombus / embolus
atherosclerosis
shock
vasculitis
50
What are haemorrhagic strokes? Classify?
Ruptured blood vessels leading to reduced blood flow
Intracerebral / subarachnoid / intraventricular
51
Risk factors for strokes?
**Hypertension, age, AF**
Smoking, diabetes, hypercholesterolaemia, polycythaemia
**medications** - HRT, combined oral contraceptive pill
52
FAST tool to identify stroke in community?
Face, Arm, Speech, Time
53
Tool used to identify strokes in ER?
ROSIER
54
Differentiate stroke from bell's palsy?
Stroke = forehead sparing = Upper motor neuron lesion
Bell's palsy = everything on face = Lower motor neuron lesion
55
Investigations for Stroke?
**_Head CT_**
→ rule out haemorrhagic _before_ thrombolysis & LP
**→ aspirin 300mg stat post CT**
**_LP_** - MUST if CT normal
not before 12h - xanthochromia
**_Pulse, BP, ECG_**
look for AF / if high BP then must maintain!! 20% will compromise cerebral perfusion
**_Bloods_**
thrombocytopenia & polycythaemia - rule out hypoglycaemia
56
3 layers of meninges of the brain?
IN → out
Pia mater, arachnoid mater, dura mater
57
Risk factors for an extradural haemorrhage?
Young patient with traumatic head injury + ongoing headache
**periods of improval then rapid decline** (haematoma getting larger to compress intracranial contents)
58
How might an extradural haemorrhage appear on CT?
Lentil / lemon, biconvex
Limited by cranial sutures!
59
How might a subdural haemorrhage appear on CT?
Crescent
Not restricted by suture lines!
60
Risk of subdural haemorrhage?
elderly, alcoholic patients
→ atrophy in brian = more likely to rupture
61
How might an subarachnoid haemorrhage appear on CT?
Star / around the brain
62
Causes of subarachnoid haemorrhage?
Usually **ruptured cerebral aneurysm**
= **berry aneurysm = 80%**
**arterio-venous malformations**
others: enceph, vasculitis, tumour, idiopathic
63
Risk for subarachnoid haemorrhage?
Hypertension, smoking, excessive alcohol consumption, cocaine
sickle cell anaemia
fam hx, also black female 45-70
64
Symptoms of a subarachnoid haemorrhage?
**Sudden and severe thunderclap headache** hitting on the back of the head
→ Most severe at beginning, short period
N+V, collapse, seizure
can present with meningism
65
How should you treat haemorrhagic strokes (4)?
Do not give antiplatelets!!
**_Reverse anticoagulants_**
**_Control hypertension_**
**_Nimodeipine_**
**_Decompression of raised ICP_**
Mannitol (diuretic) / Burr holes
66
What is nimodepine?
a CCB
**_Prevent & tx of ischaemic neurological defects_** following aneurysmal subarachnoid haemorrhage
67
How might you reverse warfarin?
Human Prothrombin Complex (Beriplex) & Vitamin K
if unavailable - fresh frozen plasma
68
Direct Oral anticoagulants (DOAC) reversal:
Agents for Dabigatran, rivaroxaban, apixaban?
Dabigatran = Idarucizumab
Rivaroxaban & Apixaban = Andexanet alfa
(R&A = direct factor Xa inhibitor)
| (D = direct oral anticoag)
69
Surgical interventions for intracranial bleeds?
**Coiling** - place platinum coils into aneurysm and seal it off from artery
**Clipping**
→ not indicated unless ruptured
70
Complications of intracranial haemorrhages?
**Vasospasm** - can result in brain ischaema
**Hydrocephalus** - buildup of fluid in ventricles
(LP or shunt)
**Seizures**
| (prevent with nimodipine)
71
Features of anterior cerebral artery stroke?
**Limb dysfunctions**
gait apraxia, truncal ataxia, incontinence
```
**Sensory loss, mostly in lower limbs**
amarosis fugax (transient vision loss)
```
72
Features of middle cerebral artery stroke?
**Upper limbs** \> lower limbs
**Facial / speech features**
**facial droop, aphasia**
homonymous hemianopia
hemineglect in non dominant hemisphere
73
Features of posterior cerebral artery stroke?
**_Heavy visual features_**
**visual agnosia -** cannot interpret info but can see
colour naming / discriminating problems
**double vision, visual field defects**
_contralateral homonymous hemianopia w/ macular sparing_
(loss sight same side both eyes)
74
Features of a lacunar stroke?
_lacks_ **_true_** _cortical signs_: aphasia, visuospatial neglect, gaze deviation, visual field defects
**_Involves limbs more than head_**
pure sensory impairment / motor stroke, ataxic hemiparesis (weak in leg), clumsy hand etc
75
Treatment principles, ischaemic stroke?
Thrombolysis = up to 4.5 hours since onset of symptoms
Thrombecttomy = 6-24 hours since onset
**Clopidogrel 24 hours after both**
if both unsuitable: **aspirin daily 2weeks then lifelong clopidogrel**
76
What is thrombolysis?
give within **_4.5 hours of onset_**
ischaemic stroke
→ once excluded primary ICH with CT!
**Alteplase** = tissue plasminogen activator, breaksdown clots & reverse stroke
Monitor for complications e.g. IC / systemic haemorrhage = **repeat CT**
77
What is thrombectomy?
**= mechanical removal of clot**
give **_6-24 hours_** since onset of symptoms
→ must confirm occlusion on imagine
**→ clopidogrel 24 hours after**
78
How might you prevent stroke?
**_Platelet tx_**
**Clopidogrel 75mg daily** ± aspirin
(alt dipyridamole)
**_Cholesterol treatment_**
**atorvastatin 80mg**
(alt simvastatin)
**Tx modifiable risk factos**
AF = warfarin / DOAC
BP - ACEi
79
Mechanism of statins?
Inhibit HMG-CoA, thereby decreasing production of LDL levels
80
Define transient ischaemic attack?
neurological deficit lasting **_less than_** (or up to) 24 hours
81
Management for TIA's?
**If on anticoag → immediate admission**
**aspirin 300 mg stat**
daily until specialist review
82
What is epilepsy?
a recurrent tendency to have **spontaneous, intermittent, abnormal electrical activity** in parts of the brain, manifesting in **seizures**
83
What is a seizure?
transient episodes of **abnormal electrical activity in the brain** as caused by excessive, hypersynchronous neural discharge
84
Diagnostic criteria, epilepsy?
**= at least 2** or **more unprovoked seizures** occurring **\> 24hrs apart** to **DIAGNOSE EPILEPSY**
85
What is syncope? Risk factors?
= **loss of consciousness due to hypoperfusion to brain**
Risk - prolonged upright position, sweating, nausea, issues w/ heart / BP etc
86
Differentiate non-epileptic seizures to epileptic seizures?
**Non epileptic** = situational, situational, with pre-ictal anxiety
**Epileptic** = result from sleep, can have incontinence & sleep
87
General advice for patients with epilepsy?
→ **CANNOT DRIVE = inform DVLA**
until free of day time seizures for minimum a year
→ try not to swim alone, avoid dangerous sports, leave door open when taking bath
88
Causes of epilepsy?
⅔rd idiopathic, often familial
Post CNS infection → cortical scarring ‘sclerosis’
Space occupying lesion, stroke, alzheimer's / dementia, alcohol withdrawal
89
Risk factors for epilepsy?
Fam hx
Premature born babies
Abnormal blood vessel in brain
arterio-venous malformations
Use of drugs - cocaine
Alzheimer's / dementia
stroke / brain tumour / infection
90
DIfferentials for epilepsy?
**Syncope** = most common
* *Dissociative (non-epileptic) seizures**
- situational, durational, dissociative
- eyes closed, can cry / speak, same f but larger amp
91
Investigations & Diagnosis for epilepsy?
\> 2 seizures 24 hours apart
→ diagnosis of exclusion
**EEG** (checks for which type)
**MRI brain** (structural problems / tumours)
**CT head** - space occup lesion
exclusions - **ECG** for heart
92
4 stages of epileptic attacks?
**Prodrome** = change of mood or behaviour
**aura** = deja vu, strange feeling in gut, flashing light
**ictus** = the seizure, 30-120s
**Post-ictally** = after seizure
93
Examples of post-ictally behaviour?
**Amnesia**
**Headache, confusion, myalgia and sore tongue** (might be bitten)
**Temporary weakness** after _focal seizure_ in motor cortex = **todd's palsy**
**dysphagia** following _temporal_ lobe seizure
94
What are generalised seizures?
Bilateral, symmetrical & synchronous motor manifestations
95
What are focal seizures?
Features referable to one part of a hemisphere
96
Floaters in vision field / visual flashes
Which lobe can the seizure be located to?
Occipital lobe
= visual
97
Pins and needles, pricking, ants are crawling over skin
Which lobe can the seizure be located to?
Parietal lob
= sensory seizures
98
Hallucinations, lip smacking / grabbing, plucking, sense of deja vu
Which lobe can the seizure be located to? **Feature post-ictally?**
Temporal lobe
Post ictally = dysphagia
99
Head / leg movements, posturing, start from one point then → whole body
Which lobe can the seizure be located to? Post-ictally features?
Frontal lobe
= motor
* *Post ictally = weakness**
assoc. _Todd's palsy_ = paralysis of limbs involved for hours
100
What is status epilepticus? How would you manage it?
→ seizures lasting **\> 5 minutes** or **\> 3 seizures in one hour**
= medical emergency
**IV lorazepam 4mg**, repeat aft 10 minutes if seizure continues
If still = **IV phenobarbital or IV phenytoin**
**Other options**
buccal midazolam, rectal diazepam
101
What are Tonic-clonic seizures?
```
tonic = muscle tensing
clonic = muscle jerking
```
102
What are absent seizures?
→ **typically in children**
blank, stares into space and abruptly returns to normal
often don't realise, 10-20s,
\> 90% grow out of it
103
Feature of absent seizure on EEG?
3Hz spike and wave activity!
104
What are atonic seizures?
**Drop attacks** = sudden loss of muscle tone and cessation of movement = fall
typically \> 3 minutes, begin in childhood
differential is lennox-gastaut syndrome
105
What are myoclonic seizures?
→ usually in children, as a part of juvenile myoclonic epilepsy
**sudden brief muscle contractions, like a sudden ‘jump‘**
= a violently disobedient limb or be suddenly thrown to the ground
106
If a child presents with myoclonic seizure, test you should do?
Genetic testing
107
Treatment principles for epilepsy?
For generalised seizures
→ 1st = sodium valproate
→ 2nd = lamotrigine or carbamazepine
Reverse for focal seizures
108
Sodium valproate
Class / mechanism / SE / CI?
**antiepileptics**
increase GABA activity → relax the brain
**SE** = teratogenic, liver damage & hepatitis, hair loss, tremor
**CI** = girls & women of reproductive age!
109
Carbamazepine
Class / Mechanism / SE?
antiepileptic
Inhibit neuronal sodium channels
**SE** - agranulocytosis (carbimazole also)/ aplastic anaemia / CYP450
| (also for trigeminal neuralgia)
110
Lamotrigine
class / SE
antiepileptic
also for bipolar disorder
**SE** Stevens-Johnson syndrome / DREE = life threatening rashes!!
+ leukopenia
111
Surgical options to treat seizures?
**Surgical resection** = if single defined cause or area
## Footnote
**Vagal nerve stimulation / disconnection**
112
What are infantile spams?
West syndrome, rare
start around 6m infancy, clusters of full body spasms
113
Treatment for infantile spams?
1st line prednisolone & vigabatrin
114
What is Parkinson's?
Degeneration of the _pars compacta_ in **substantia nigra** of brain leading to **decrease in dopamine**
115
Causes & risk factors of Parkinson's
**No known cause**
Genetic mutations - PINK1, Parkin etc
**Risk**
Pesticide exposure, MPTP in illegal opioids
male, age, fam history
! non smoker = higher !
116
Presentation of Parkinson's?
**TRAP**
+ sleep disturbances / anosmia
**T = tremor** = _resting_!
pill rolling tremor
**R = rigidity**
cogwheel rigidity, esp in arms
**A = akinesia / dyskinesia**
reduced arm swing, shuffling gait, soft voice, reduced blinking, drooling
**P = postural instability**
& forward tilting
→ NO WEAKNESS
117
Differentials for Parkinson?
**Benign essential tremor**
**Dementia with lewy bodies**
**Progressive suprenuclear palsy**
**_if on Levodopa_**
**high response = lewy body dementia**
(hallucinations, agitation, confusion)
**low response = Multi-system atrophy**
| (_symmetrical_, _worse_ on _mvt_, no other parkinson features, better with alcohol)
118
Investigations for Parkinson's?
**CT / MRI**
**PET with fluorodopa**
localises dopamine deficiency in basal ganglia
119
What might you find on an autopsy of a Parkinson patient?
**Lewy body depositions** = eosinophilic protein aggregations in substantia nigra, cytoplasm rich in eosin
## Footnote
**disappearance / fading of the substantia nigra**
120
Most effective tx for Parkinson's?
Levodopa = 1st line
if motor symptoms not affecting patient's QoL then others or levodopa
121
Levodopa
class / mechanism / SE
**synthetic dopamine (precursor)**
→ boost patient's dopamine level
→ give with COMT
**SE** less effective over time, if dose too high = dyskinesia (N+V, arrhyth, chorea, dystonia)
122
COMT inhibitors
Examples / Mechanism?
**entacapone, tolcapone**
Inhibition of breakdown of dopamine by Catechol-O-Methyl Transferase enzymes
used in **conjunction w/ levodopa**
123
Dopamine Receptor Agonists
Examples / mechanism / SE
**bromocriptine / cabergoline**
= stimulate dopamine receptors
**SE** impulse control disorders, hallucinations, excessive daytime somnolence
124
MAO-B inhibitors
Examples? Mechanism?
Selegiline, rasagiline
Inhibits breakdown of dopamine by Monoamine oxidase-B enzymes
125
Surgical tx options for Parkinson's?
Deep brain stimulation
126
Define dementia?
Clinical syndrome characterised by **significant deterioration in cognitive skills → impairment of normal function**
127
Reversible causes of dementia & tx (5)?
aka differentials of dementia?
alcohol
hypothyroidism (THR)
vitamin B12 deficiency
Syphilis (penicillin)
Normal pressure hydrocephalus
(ventriculoperitoneal shunt)
128
What is an Normal pressure hydrocephalus?
= abnormal venous drainage of cerebrospinal bluid → backup of fluid & hydrocephalus
**symptoms** = dementia, urinary incontinence, magnetic gait
**MRI** - no cortical atrophy, ventriculomegaly of all ventricles, patent cerebral aqueduct
Tx ventriculoperitoneal shunt
129
Irreversible causes of dementia? (Types of dementia)?
Alzheimer's disease
Vascular dementia
Lewy body dementia
Frontotemporal dementia
130
Briefly outline pathophysio of Alzheimer's?
**neurofibrillary tangles & beta amyloid plaques** affect function of neurons → interrupt key neuron process → **cell death**
131
What can be found on a brain biopsy in someone with Alzheimer's?
**Neurofibrillary tangles** (tau protein aggregations) & **beta amyloid plaques**
Widespread **cerebral atrophy esp. _cortex & hippocampus_**
132
Key presentation of Alzheimer's?
**_Memory loss_**
Loss of personality, intellect & language
133
Investigations for Alzheimer's?
= Exclude
**_CT / MRI_** → hippocampus & cortex atrophy
definitive is actually brain biopsy but obv impossible until death
134
Treatment option for Alzheimer's?
_Symptomatic_
Cholinesterase inhibitors
(+ACh levels)
NMDA receptor antagonists
_Depression_ = SSRI
135
Key presentation vascular dementia?
Step wise steady & gradual deterioration related to region (and a history) of stroke / TIAs
136
Symptoms of vascular dementia?
Urinary incontinence, cranial nerve palsy, focal neurological deficits
137
Treatment for vascular dementia?
None to reverse
**→ Prevent another stroke**
= BP, lifestyle, smoke, antiplatelet = aspirin
| (nimodepine is ischaemic)
138
Key presentation frontotemporal dementia?
\< 65 years
Personality change, lack of social inhibition
139
What might you find on an imaging scan of frontotemporal demential patient?
**Atrophy of frontal & temporal lobes**
sparing other lobes!
140
Treatment for frontotemporal dementia?
No tx available to delay progression
**SSRI** = behavoural symptoms
(setraline, citalopram)
**Atypical antipsychotics** = psychosis
141
Symptoms of Parkinson's disease & lewy body dementia?
* *= Parkinsonism**
* *= TRAP**
Tremor (resting)
Rigidity (esp in arms)
Akinesia / bradykinesia
Postural instability
142
Differentiate Parkinson's disease dementia x Lewy body dementia?
→ sequence of events
Parkinson's = Parkinsonism THEN dementia
Lewy body = dementia THEN Parkinson
143
If on Levodopa, how might you differentiate Lewy body dementia x Parkinson's disease dementia?
Responds well = Parkinson's d.d.
If 3 tions: **hallucination, agitation & confusion** → Lewy body dementia as patients are dopamine sensitive
144
Other features of lewy body dementia?
Recurrent visual hallucinations
Flunctuating attention / alertness / cognition
Sleep disturbances
145
Tx for Lewy body dementia?
**Cholinesterase inhibitors** = improve symptoms, but don't slow progression
**Levodopa** = helps with motor impairment but risk of developing delirium
→ CI antipsychotics (worsen!)
146
What is Huntington's disease?
**Autosomal dominant**
genetic condition, progressive deterioration in the nervous system
147
Genetic stuff for Huntington's?
Trinucleoside repeat disorder = CAG
Genetic mutation on HTT gene on chromosome 4
148
What is anticipation?
= in Huntington's
each successive generation have more repeats in gene
= earlier age of onset
= increased severity of disease
149
Presentation - Hungtington's?
30-50 years, but can be earlier or later
**Chorea**
= involuntary, purposeless movements
= _parakinesia_ = try to incorporate into purposeful mvt
**Dementia**
= cognitive decline
**Behavior changes**
= cognitive, psychiatric, mood problems,
dysarthria / dysphagia / dystonia + parkinson's
150
How might you diagnose Hungtington's chorea?
**blood PCR** for CAG repeats
**MRI** = atrophy of caudate & putamen, enlargement of the lateral ventricles
151
Tx for Hungtington's?
**_No tx for slow or stop_**
Symptomatic relief / MDT in QoL
speech & language / genetic counselling / advanced directives
**chorea** = antipsychotics, benzodiazepine
152
Prognosis for Huntington's?
Progressive condition
LE 15-20 years after onset
death often due to respiratory, e.g. pneumonia, but also common is suicide 😞
153
Before any headache investigation - one thing to rule out?
Idiopathic cranial hypertension
154
Signs of raised intracranial pressure?
headache, vomitting, reduced levels of consciousness, papilloedema
**_Cushing's triad_**
widening pulse pressure, bradycardia, irregular breathing
155
Presentation & risk - idiopathic intracranial hypertension
headache, blurred vision, enlarged blind spot, **_papilloedema_**, ± 6th nerve palsy
risk → **obesity, female, pregnancy, drugs (COCP, steroids, tetracyclines, lithium)**
156
Signs of papilloedema?
Optic nerve swelling & blurring of the disc margins
cotton wool spots, silver wiring,
hard exudates, retinal haemorrhage
**_→ seen on a fundoscope_**
157
Tx Idiopathic intracranial hypertension?
1st line = **Weight loss**
Diuretics = **acetazolamide**
**Topiramate** (anticonvulsant) = also cause weight loss
Surgery
optic nerve sheath decompression and fenestration
158
Thunderclap headache?
Subarachnoid haemorrhage!
159
Thunderclap headache?
Subarachnoid haemorrhage!
160
Presentation of tension headaches?
Aches across the forehead in a band pattern
Pressing & tightening
come and go
no N&V
161
Tx Tension headaches?
* *Basic analgesia**
* *= aspirin / paracet / ibuprofen**
Consider amitriptyline
Relaxation techniques & hot towels to local area
162
Tension headache associations
stress / depression / alcohol / skipping meals / dehydration
163
Triggers of migraines?
= chocolate
Chocolate
Hangovers
Orgasms
Cheese
Oral contraceptives
Lie in's
Alcohol
Tumult - loud noise
Exercise
164
Risk factors for migraines?
females, positive family history
165
Presentation of migraine?
moderate to severe intensity
* *pounding or throbbing in nature**
* *nausea & vomiting**
± aura
± photophobia, phonophobia
166
What is aura & examples?
**prodrome / postdrome to migraine**
**visual** = flashing light, zigzag lines, lose part of vision, black holes in visual field
**sensory** = parasthesia
**speech or language** = problems saying / understanding words
167
Diagnostic criteria for migraines
5 attacks
168
Acute management for migraines?
oral triptan ± paracetamol
or other analgestics = aspirin / paracetamol
169
Preventive management for migraines?
**Topiramate / propanolol**
→ **amitriptyline**
→ 10 sessions of **acupuncture** over 5-8 weeks
* *Riboflavin** (vit B12)
* *Botulinum Toxin type A** = last line
| (CI teratogenic / asthma)
170
Presentation of cluster headaches?
→ **headache + unilateral,**
**ipslateral eye concentrated**
red, swollen, watering eye
```
miosis = constrict,
ptosis = eyelid droop
```
nasal discharge, facial sweating
→ 5 to diagnose
→ episodic, can wake pt
171
Triggers of cluster headaches?
Alcohol, strong smells, exercise
172
Acute treatment for cluster headaches?
**100% 15L O2**
15-20 minutes via non-rebreathable mask
CI if COPD
**Triptan** = SC **sumatriptan** 6mg
→ serotonin receptor agonist = reduce vascular inflammation
173
Preventive measures for cluster headaches?
**1st line CCB** = **verapamil**
**Avoid alcohol** during cluster period
**Corticosteroids** may help during cluster period (prednisolone short course 2-3 weeks to break cluster)
174
Presentation - trigeminal neuralgia?
Intense facial pain, similar to **electric shock / shooting pain** lasting seconds to minutes
related to eating
175
Branches of the trigeminal nerve?
Ophthalmic (v1)
Maxillary (V2)
Mandibular (V3)
→ any combination can be affected)
176
Tx trigeminal neuralgia?
**1st** = **oral carbamazepine**
**2nd** oral phenytoin, gabapentin & lamotrigine
**Surgical** - microvascular decompression / gamma knife surgery
| (anticonvulsants)
177
Associations & triggers - trigeminal neuralgia?
5-10% multiple sclerosis
triggers: cold weather, spicy food, caffeine, citrus fruits, brushing teeth, cough / sneeze
178
Medication overuse headache - diagnostic criteria? triggers?
**Headache present ≥ 15 days / month**
Triggers = opioids, triptanss, combination analgesic (paracetamol + codeine / opiates)
179
Present sinusitis?
**Headache** associated with inflammation of sinuses
## Footnote
**→ if tender over the sinus = DIAGNOSTIC**
**facial pain behind nose, forehead and eyes**
180
Treatment sinusitis?
Resolve in 2-3 weeks, mostly viral
tx
* *nasal irrigation with saline**
* *prolonged = steroids nasal spray**
181
Cervical spondylosis - present & diagnose?
Degenerative changes in cervical spine = diag by exclusions
## Footnote
**Neck pain worsened by mvt**
182
Hormonal headache - present & tx?
related to **_low oestrogen,_** nonspecific tension like headache
→ 2 days before & first 3 days of menstruation, menopause, pregnancy
= **2 out of 3 menstrual cycles** for diagnosis → headache diary
183
What is Horner's syndrome?
* *_ptosis, miosis, anhydrosis_**
* *_→ all on the same side_**
flushing on one side of the face and that side doesn’t sweat. The eye on the same side has a smaller (constricted) pupil with a drooping or weak eyelid.
184
Define meningitis?
inflammation of meninges
185
What is meningism?
Neck stiffness / photophobia /
Kernig's sign
= pain & resistance on passive knee extensions with hips fully extended
186
Presentation - meningitis?
**_triad_**
Fever, headache, neck stiffness / leg pains
N + V, **photophobia**, rash (non blanching)
187
Causes of meningitis?
Bacterial / viral / fungal / parasitic
188
How might you treat primary meningitis?
IV / IM Benzylpenicillin
or IM ceftriaxone
189
Secondary tx for meningitis?
**GCS assessment**
**Blood cultures + broad spectrum antibiotics**
**Steroids**
= IV dexamethasone to reduce tissue inflammation, damage & reduce morbid
usually - **CT** before LP
**DIAGNOSTIC = _Lumbar puncture_**
CI: coagulation disorders, rash, raised intracranial pressure
190
Initial antibiotics for meningitis? Any to add?
**Ceftriaxone or cefotaxime**
if allergy = (erythro?)
if _immunocompromised_ + **amoxicillin**
(risk of listeria)
if _travel_ hx **+ vancomycin**
(risk of penicillin resistance)
191
If there is a rash - most likely causative organism? special response?
Rash = most likely
**neisseria meningitidis**
→ inform **PHE**
→ identify close contacts: prophylactic antibiotics = **ciprofloxacin / rifampicin**
192
Causative bacteria for meningitis? (6+2)
**_GRAM NEG_**
Neisseria meningitidis
E coli
Haemophilius influenza B
**_GRAM POS_**
Listeria spp.
Strep pneumoniae
Group B strep.
**_Chronic_**
Mycobacterium Tuberculosis
Syphilis
193
Meningitis in - neonates, pregnant ladies, immunosuppressed patients, elderly
Listeria spp.
194
Causes of meningitis in adults (2)?
Neisseria meningitidis
Strep pneumoniae
195
Viral causes of meningitis?
```
Herpes Simplex (HSV)
Varicella Zoster (VZV)
Enterovirus
```
196
Fungal causes of meningitis?
Cryptococcal spp.
197
Cause of meningitis when LP / CSF is
‘fibrin web’ appearance
high WBC = lymphocytes
high protein
low glucose
Fungal / TB
→ PCR for M. TB
198
Cause of meningitis when LP / CSF is
cloudy
high WBC = neutrophil / granulocytes
high protein
low glucose
Bacterial
199
Cause of meningitis when LP / CSF is
clear
high WBC = lymphocytes
high protein
normal glucose
Viral
PCR for HSV, VZV & enterovirus
200
How might you test for TB?
Phenol auramine / Ziehl-Neelson stain
201
Differentiate between TB / bacterial causes of meningitis as appeared on CSF?
Bacterial = neutrophils / other granulocytes
TB / fungal = neutrophils = lymphocytes
202
Differentiate between TB / fungal and viral caused meningitis as appeared on CSF?
viral = glucose normal
TB / fungal = high glucose
203
Signs of sepsis?
= prolonged refill time
cold hands and feet
decreasing BP
evolving rash
204
Sepsis 6 tx?
**_Give_**
oxygen
fluid resuscitation
IV antibiotics
**_Take_**
Blood cultures
Urine putput
Lactate
205
What is encephalitis?
Inflammation of the cerebral cortex
= severe abnormalities in function of brain
206
Causes of encephalitis?
| (name essential at least)
**_usually Viral_**
→ Herpes Simplex virus
→ Varicella zoster virus
MMR, EBV, HIV if immunocompromised, CMV, Coxsackie
* *Tropical**
* *mozzie bite**: Japanese encephalitis / West Nile
* *animals**: tick borne / rabies
207
Present - encephalitis?
**_acute flu-like illness, hours to days_**
fevers, seizures
**altered GCS** - confusion, agitation, drowsiness, coma
* *± meningism**, mostly absent
* *check travel tx / animal bite**
208
Investigations / initial management encephalitis?
IV Acyclovir
**_Contrast enhanced CT_**
must preceed LP
**_Lumbar Puncture_**
high lymphocytes
high protein
normal glucose
**_viral PCR_**
(throat swab & MSU)
blood culture, toxoplasma IgM titre, malaria film, gram film → negative
209
Treatment for encephalitis?
**if HSV or VZV = acyclovir**
PCR at the end of cycle
others mostly supportive
→ lots of neuro rehab
210
Cause of tetanus?
**_Clostridium tetani_**
gram pos rod, anaerobic spore
in soil & house dust
inoculation through skin
211
TETANUS CARDS
212
RABIES CARDS
213
CJD CARDS
214
What is Guillan Barre syndrome?
Acute, inflammatory polyneuropathy
→ **ascending neuropathy resulting in weakness & reduced reflexe**s
215
Risk factors for Guillan Barre syndrome?
peak 15- 35, 50-75
males \> females
after pregnancy (delivery)
216
Causes of Guillan Barre?
**History of preceeding illness**
Diarrhea or a respiratory illness
**→ Campylobacter jejuni = most common**
**→** causes **gastroenteritis**
**Viral causes**
Cytomegalovirus (CMV), Epstein Barr, Zika, HIV
**Post vaccinations**
217
Pathophysio - Guillan Barre?
Immune mediated damage to peripheral nerves
**= molecular mimicry**
= antibodies generated in response to pathogen incorrectly cross react with body's own cells
218
Presentation - Guillan Barre?
**Prodrome** = usually gastroenteritis & flu like illness
**_Ascending neuropathy = symmetrical_**
feet → facial / respiratory
**→ reduced reflexes**
**→ weakness**
**→ peripheral neuropathy**
**→ loss of sensation**
219
What is Miller Fisher syndrome?
Variant of Guillan Barre
= **ataxia, areflexia, ophthalmoplegia**
with **anti-GQ1B** antibodies
(component of nerves)
220
Differentials for Guillan Barre?
Lyme disease
sarcoidosis
Thiamine deficiency
Neoplasms
221
Investigations for Guillan Barre?
**_DIAGNOSTiC = Nerve conduction studies_**
= reduced signal through nerve, prolonged distal motor latency ± conduction block
**Lumbar puncture at L4**
high protein, normal cell count, normal glucose
**Spirometry = monitor FVC**
decrease FVC = admit to ITU for maintenance of airways
**Bloods** → check for **IgA deficiency**, if yes cannot tx IV IG = severe allergic reaction
222
Treatment of Guillan Barre?
**Plasmaphoresis**
**IV Immunoglobulin**
CI: IgA deficient patients → anaphylaxis!
| (plasma exchange)
223
Complications of Guillan Barre?
**Respiratory failure, infection, cardio dysfunction & pulm embolism**
→ monitor BP, FVC, low limb stockings & LMWH prophylaxis for venous thrombosis
224
Tx neuropathic pain?
gabapentin / carbamazepine
| (anticonvulsants)
225
Criteria for assessment for tx pathways, Guillan Barre?
Hughes Disability score
226
What is myasthenia gravis?
Neuromuscular autoimmune disorder, due to antibody mediated blockage of neuromuscular transmission
227
Risk & aetiology, myasthenia gravis?
20-30's females
60-70's males
228
Pathophysio of myasthenia gravis?
**Acetylcholine receptor antibodies (ACH`r-Ab / anti ACh)** binds to **ACh receptors** at neuromuscular junction
= **prevents binding of ACh** & subsequent depolarisation needed for muscle contraction
229
AChR-Ab
produced by? ways it affects NMJ?
produced by **B lymphocytes**
prevents binding by ACh
cross link ACh-R = internalisation & destruction
Complement mediated damage of post synaptic membrane
230
What type of hypersensitivity reaction is myasthenia gravis?
antibody mediated = type II
231
Other antibodies in Myasthenia Gravis?
AChR-Ab = 80-90%
anti MuSK protein
anti LRP protein
232
Associations - Myasthenia Gravis?
**Thymus abnormalities**
85% hyperplasia, 15% thymoma
autoimmune associations
Grave's SLE, rheum art
**Transient MG** - caused by D-Penicillamine treatment for Wilson's disease
233
Clinical presentation - Myasthenia Gravis
hallmark = **_fatiguability of muscles_**
increasing weakness with use
→ common **_at the end of the day_** or **following exercise**
**Signs by region**
Limbs = arms \> legs
can't hang up laundry / comb in one go
Myasthenic sneer = facial muscles
expressionless, poor smile
dropped head at the end of day
bulbar = count to 50 = voice smaller
ophthalmic
234
Investigations for myasthenia gravis?
**Ice pack test**
ptosis improve after application of ice
(neuromuscular transmission better at lower muscle temp)
**Cogan's lid twitch**
patient to follow pen down … and suddenly up = lid will overshoot and twitch
**Bloods for AChR-Ab, MuSK & LRP4**
if negative = **Electromyography** (90% sensitivity)
**Thymus assessment (CT chest)**
+ thyroid function test (highly assoc)
235
Pharmacological treatment (with class) for myasthemia gravis?
**1st = pyridostigmine**
**2nd = Prednisolone**
corticosteroids
**3rd = immunosuppressants**
azathioprine = inhibit purine synthesis
risk of marrow failure
236
Class of pyridostigmine? Mechanism? SE?
**Acetylcholinesterase Inhibitors**
_prevent hydrolysis_ or acetylcholine and increase effect at NMJ
**SE** cholinergic = secretions, diarrhoea, GI upset, bronchospasm, sweating, urinary incontinence
→ avoid during acute myasthenic crisis
237
Other management options, myasthenia gravis?
FVC manage, speech and language team
**IV Immunoglobulin**
if severe respiratory or bulbar symptoms
alt plasmaphoresis
**Thymectomy for thymomas**
**High dose steroids if ventilated**
initially worsen then better
238
What is a myasthenic crisis?
Worsening weakness that requires respiratory support, e.g. intubation / ventilation
239
Presentation - myasthenic crisis?
**Severe limb weakness, diplopia**
**Hypoxia**
quiet breathing, reduced chest expansion, tachy, HP
sats & ABG can be normal
240
Tx myasthenic crisis?
Urgent admission to hospital
**_intubation / non invasive ventilation_**
**FVC / single breath count = BEST investigate !!!**
all to be assessed by speech & language team & regularly FVC assess
241
What is Lambert Eaton syndrome?
Antibodies against **voltage gated calcium channels** on presynaptic junction
ACh release reduced = muscle weakness
242
Causes of Lambert Eaton syndrome?
**Autoimmune related**
both MNJ & autonomic ganglia
**90% seropositive**
**50-60% have underlying malignancy**
small cell lung CA, lymphoproliferative
**STRONG relate w/ T1DM, Thyroid diseases**
243
Presentation Lambert Eaton syndrome?
hallmark = **_strength increases with repeated effort / exercise!_**
Proximal muscle weakness
**→ in the morning**
dry eyes, dry mouth, sexual dysfunction, sphincter issues, postural hypotension
244
Management - Lambert Eaton syndrome?
**ACh inhibitor = pyridostigmine**
(goes both ways\_
3, 4 diaminopyridine = blocks K channel in nerve terminal & potentiates action
treat cancer / use steoids may also help
245
What is Isaac's syndrome?
**Peripheral nerve hyperexcitability** due to **antibodies against voltage gated _potassium_ channels**
cramps, fasciculations, hyperhydrosis, myokymia, exercise intolerance
246
What is Multiple Sclerosis?
Chronic progressive condition that involves **demyelination** of the myelinated neurones in the CNS
247
Causes & risk of Multiple Sclerosis?
**Autoimmune** = **T cell mediated**
**20-40's, women!!!**
_Others_
genetics / Epstein Barr virus / low sun or vit D / smoking & obesity
248
What are myelins? Produced by which cells?
Myelin = **sheath of the axons** of neurones in CNS
→ produced by **oligodendrocytes** = affected in MS
249
Pathophysio of multiple sclerosis?
**Inflammation around myelin** & infiltration of immune cells = damage to myelin
**Plaques** **of demyelination** occur around veins (perivenular) & have a predilection for distinct CNS sites:
optic nerves, ventricles of brain, corpus callosum, brainstem & cerebellar connections etc
250
Type of reaction for Multiple Sclerosis?
Type 4 = cell mediated hypersensitivity
| (T cells / B cells / macrophages)
251
Symptoms of Multiple Sclerosis?
Charcot's neurologic triad +3
**Dysarthria** = plaques in brainstem
speech / eat / swallow / talk = throat
**Intention tremor** = plaques in motor pathways
limb weakness & spasms, tremors, ataxia
**Nystagmus** = plaques in eye nerves
optic neuritis, diplopia
loss of vision / pain
**Paraesthesia** = plaques in sensory pathways
**Lhermitte's sign**
electric shock runs down back & radiate to limbs when bending neck
**Plaques in ANS**
Incontinence / sexual dysfunc / bowel symptoms
252
Mneumonic for Multiple Sclerosis symptoms?
**LOVDIP**
Lhermitte's sign, limb weakness
Optic neuritis
Vertigo
Diplopia
Incontinence
Paraesthesia
253
Immediate treatment with optic neuritis?
seen urgently by ophathalmologist
Steroid treatment, 2-6 weeks recovery
254
What are the four disease patterns of multiple sclerosis? Briefly describe each one? Which is the most common?
**Replasing / remitting MS** (80%)
no increase in disability between bouts months apart
**Secondary progressive MS**
over time from RRMS disability progression becomes constant
**Primary Progressive MS**
steady disability progression
**Progressive relapsing MS**
steady disability + bouts superimposed
255
Differentials for MS?
SLE
Parkinson's
Motor neurone disease
256
Diagnostic criteria & investigations?
**Criteria** = 2 or more attacks disseminated in time & space (affecting diff parts of CNS)
**DIAGNOSTIC = MRI**
90%+ white matter plaques
**Lumbar puncture** = CSF shows IgG bands (antibodies)
**Electrophysiology** = visual evoked potential studies → delayed conduction = demyelination
257
How might you differentiate between active & chronic lesions of MS?
use **Gadolinium contrast in MRI**
Lesions that are enhancing = break in BBB & uptake gd = active
Not enhancing = old
258
Current pharmacological treatment for MS?
→ disease modifying = induce remission with no evidence of disease activity
**Monoclonal antibodies** = IV alemtuzumab, natalizumab
**dimethyl fumarate**
259
How might you treat an acute MS attack?
**steroids** = oral methylprednisolone 500mg daily for 5 days / IV 1g / day if severe
if frequent relapses = **anti-inflammatory cytokines**
SC interferon 1A / 1B reduce relapse and lesion accumulation
→ 2nd line = **plasmaphoresis / stem cell transplant**
260
How might you symptomatically treat MS?
**exercise to maintain activity & strength**
**neuropathic pain** = amitriptyline / gabapentin
**depression** = SSRI
**Urgen incontinence** = anticholinergic medications = oxybutynin
**spasticity** = gabapentin / botox
261
What is Charcot Marie Tooth syndrome?
dysfunction in myelin or axons causes weakness and impaired sensory perception due to improper signalling
262
Nature of Charcot Marie Tooth syndrome? aetiology?
inherited = auto dom!
affects peripheral motor & sensory pathways
before 10 or later than 40
263
Pathology of CMT?
Loss of myelin on peripheral neurones OR loss of peripheral neurones
**progressive loss of**
→ motor in feet & legs
→ sensory in hands & feet
264
Present - CMT?
High foot arches = **pes cavus**
Distal muscle wasting = **inverted champagne bottle legs**
Weakness, sharp pain & numbness in hands and legs
= _trip a lot, clumsy_, **feet slap the ground when walking**, _can't run_
265
Management for CMT?
no tx, for cause or prevent progression
physio, occup, podiatrist
ortho surgeon to correct disabling joint deformities
266
Differentiate Charcot Marie Tooth & MS?
**MS** = disease of brain & spinal cord
→ **CNS**
mvt, sensory, thinking
**CMT** = disease of peripheral nerves
→ **PNS**
weakness & sensory perception
signal can't get to brain
267
Causes of peripheral neuropathy?
A alcohol
B B12 deficiency
C cancer & chronic kidney disease
D diabetes
& drugs - isoniazid, amiodarone, cisplatin
E every vasculitis
268
What are Duchenne & Becker's muscular dystrophies
Genetic disorder characterised by **progressive muscle degeneration and weakness**
## Footnote
**both → _x-linked recessive_**
269
What are dystrophins?
**proteins which keeps muscle cell intact**
lack of dystrophins = become fragile & easily damaged
270
Pathophysio of Duchenne's & Becker's?
**Duchenne** = no functional dystorphins made
**Becker** = dystrophin protein only partially functional
271
Characteristic presentation of Duchenne & Becker's muscular dystrophies?
**_hallmark = muscle weakness_**
**Gower's manoeuvre** = walks hands to legs before getting up
**Enlargement of calves**
Difficulty jumping, running & walking, **clumsy**, waddling gait, **lumbar lordosis** (spine curve inwards)
272
Compare and contrast Duchenne & Becker muscular dystrophy in terms of presentation?
**_Duchenne_** = early childhood, usually 2-3, boys
→ proximal body muscle to distal
**_Becker_** = teens to early adulthood, men 5-60
→ begins hips and pelvic, thighs & shoulders
273
Complications of Duchenne & Becker muscular dystrophies?
**Cardiomyopathy & respiratory symptoms** (weakened organs)
**Adrenal insufficiency** (from steroids)
**Growth retardation**
late puberty, lack of testosterones,
274
Investigations for the MD's?
**_Screen = Creatinine kinase_**
= enzyme released by muscle when damaged
= usually 10-100x value (\>200)
**_Diagnostic = genetic testing_**
then test for liver function
275
How might you treat the muscular dystrophies?
**_Corticosteroids = slow progress_**
prednisolone
**_ataluren_** = if genetic defect
→ allows ribosome to read through premature stop codons that causes incomplete dystrophin synthesis
**Supportive** = bone health & hormones (from steroids), supply with vitamins
276
What are motor neurone diseases?
= umbrella of rare degenerative neurological illness affecting the motor neurones
→ rare progressive ultrimately fatal
277
Risk & pathology of motor neurone diseases?
**Genetic component**
→ surviving motor neurones contain abnormal protein **TDP-43**
**Fam hx**
**links**: smoking, heavy metal exposure, certain pesticides
278
How are MND classified? Examples?
Upper motor neurones, lower motor neurones, mixed neurones (ALS, progressive bulbar)
279
Differentiate UMN lesion from LMN lesion?
**UMN** = spasticity (+tone), hypereflexia, plantars,
**LMN** = wasting, fasciculation, absent nerve conduction
weakness corresponding to lower cord nerves
Diff = in UMN forehead will be spared = stroke
280
Example of an **upper** motor neurone disease?
**Primary lateral sclerosis**
= loss of Betz cells in motor cortex, mainly UMN signs, marked leg weakness & pseudobulbar palsy
281
Example of a lower motor neurone disease?
Progressive muscular atrophy
= weakness & wasting of distal muscle, fasciculations & absent reflexes
282
Example of a mixed motor neurone disease?
**Amylotrophic lateral sclerosis ALS**
= classic & most common
= distal & proximal muscle weakness & wasting, spasticity, hyperreflexia
283
Typical presentation of ALS?
_Triad of_
Limbs
Bulbar
Respiratory
284
The motor neurone disease on its own & second most common?
**_Progressive bulbar palsy_**
= only bulbar muscles = talking & swallowing
→ involves cranial nerve nuclei
285
Typical presentation for MNND?
**_Limb onset = 75%_**
→ **distal asymmetric weakness of the arms / hands**
= grip / shoulder abduction issues
* *→ lower limb symptoms**
* *= stumbling gait, foot drop,** tripping, loss of power, wasting
286
Typical progressive presentation of MND?
**_Bulbar onset (25%)_**
→ dysarthria, wasting & fasciculations of tongue, dysphagia, slurred speech
**_Respiratory (5%)_**
dyspnoea, orthopnoea, poor sleep, reduced appetite, weight loss
287
Distinguish MND from Myasthenia Gravis & Multiple sclerosis?
MND never affects eyes movements - MG
MND will not have sphincter disturbances - MS
288
How might you investigate motor neurone disease?
Upper = **imaging, from the lesion upward**
Lower = **nerve conduction studies NCS / Electromyography (EMG)**
**ALS = + creatinine kinase**
289
Diagnostic criteria of ALS?
**_El Escorial diagnostic criteria_**
**Definite** = LMN + UMN in 3 regions
**Probable** = LMN + UMN in 2 regions
or (LMN +) UMN in 1 region
+ EMG acute denervation in ≥ 2 limb
* *Possible** = LMN + UMN in 1 region
* *Suspected** = LMN, or UMN, only
290
Complications / links of MND?
Frontotemporal dementia
Death causes = respiratory failure or pneumonia
291
Only medication to slow progression of ALS / MND?
Riluzole
prolongs life for 3-4 months
292
Tx for MND muscle cramps?
1st = quinine
SE prolongs QT interval
2nd = baclofen
→ consider exercise programs
293
Tx for MND salivation problems?
**1st = antimuscarinics**
propantheline, SC **glycopyrroate** (less cognitive effects = preferred for cognitive impairment)
botox type A if doesn't work
294
Tx for respiratory symptoms in MND?
treat reversible causes of worsening respiratory impairment before considering meds
**opioids / benzodiazepines**
**non-invasive ventilation at home** for those with respiratory impairment
295
Supportive measures for ALS?
Bulbar - blend food, gastrostomy
non invasive ventilation
296
What is corticobulbar palsy / pseudobulbar palsy?
UMN lesion of bulbar muscles due to bilateral lesions above the mid pons
297
Presentation of corticobulbar / pseudobulbar palsy?
**Slow tongue movements & speech**
**jaw jerk**
**pharyngeal & palatal reflexes**
**Pseudobulbar effect**
→ weeping unprovoked by sorrow
→ mood incongruent giggling
= emotional incontinence without mood change
298
Tx corticobulbar palsy?
dextromethorphan + quinidine
299
Which spinal tract is the descending upper motor neurone?
Corticospinal
decussate at the medulla
300
Which spinal tract is responsible for proprioception, vibration & 2 point discrimination?
Dorsal column
= ascending sensory tract
decussate at medulla
301
Which spinal tract is responsible for pain and temperature?
Spinothalamic tract
= ascending sensory tract
decussate IMMEDIATELT at spinal cord
302
What are common causes of spinal cord compression?
acute = **vertebral body neoplasm**
most common
**Disc herniation**
**Disc prolapse**
(bulge within the disc itself)
303
Presentation - spinal cord compression?
Sensory loss below the level of lesion (**_sensory level_**)
304
What is sciatica?
**S1** nerve root compression
Sensory loss in back of leg to lateral aspect of little toe (sciatic nerve distribution)
305
How will L5 nerve root compression present?
Sensory loss / pain in lateral leg & medial side of big toe
306
Investigations for spinal cord damage?
do not delay!!
**_MRI = gold standard_**
biopsy / surgical exploration for identifying masses
307
Treatment for spinal cord compression?
**if malignancy = IV dexamethasone**
**Epidural abscess = surgically decompress** and antibiotics given
Neurosurg refer stat
→ decompression
laminectomy / microdisectomy
| (reduce inflammation & oedema)
308
How many vertebrae and pairs of spinal cord are there in the spinal coloumn?
33 vertebrae, 31 spinal cords
7 (8) cervical, 12 thoracic, 5 Lumbar, 5 sacral, 4 (1) coccygeal
309
How many intervertebrae discs are there?
23
310
Where does the spinal cord end?
L2, conus medullaris
311
What is the cauda equina?
5 lumbar 5 sacral 1 coccygeal
nerve roots distal to the level of termination at L1/L2
LMN!!!
312
Muscular presentation of someone affected by cauda equina?
Flaccid & areflexic
313
Causes of cauda equina?
**Spondylolisthesis** = top vertebra slips forward (sink into body), nerve root comes above the vertebra below
**Herniation of lumbar disc**
L4/5 or L5/S1
**Tumours / mets**
**spinal stenosis / ankylosing spondylitis**
**trauma / post op haematoma**
314
Present cauda equina?
**Variable leg weakness**
→ flaccid & areflexic (LMN signs)
## Footnote
**Bilateral sciatica**
**Saddle anaesthesia**
**Bladder / bowel dysfunction, erectile dysfunction**
315
Investigations of cauda equina?
MRI to localise lesion
knee flexion - tests L5-S1,
ankle plantar flexion tests S1-S2
straight leg raising = L5 S1
femoral stretch = L4
316
Treatment for cauda equina syndrome?
= EMERGENCY
refer to neuro immediately
**decompression of the nerve** **_stat_** to avoid irreversible paralysis
→ microdisectomy, epidural steroid for pain, surgical spine fixation if slipped disk, spinal fusion = reduce pain from motion → nerve root
