conditions Flashcards
(183 cards)
1
Q
are fasciculations a UMN or LMN sign
A
LMN
2
Q
Ix for MND
A
NCS - normal
EMG - reduced number of action potentials with increasing amplitude
MRI - to rule out a SCC
3
Q
what kind of drug is riluzole
A
glutamate inhibitor
4
Q
complicaitons of MND
A
fatigue, swallow problems (consider gastrostomy early), muscle cramps/stiffness, rest failure (need BIPAP), breathlessness, communication difficulties,
5
Q
why do you get wernickes encephalopathy
A
in thiamine deficiency (B1) in alcoholics or in hyperemesis or AN, cannot metabolise carbs so get neurotoxic effects
6
Q
how is wernickes encephalopathy treated
A
urgent pabrinex for 5 days IV
7
Q
features of Korsakoff syndrome
A
retrograde and anterograde amnesia and confabulation
8
Q
bacteria commonly causing GBS
A
Campylobacter jejuni (also can be from EBV, mycoplasma)
9
Q
what antibody is seen in 25% of people with GBS (this has a worse prognostic factor)
A
Anti GM1
10
Q
how does GBS present
A
initially leg and back pain, then symmetrical and progressive weakness in limbs which is ascending
-hyporeflexia or arelexia
-can have eye problems if cranial nerves effected (these are part of the peripheral nervous system too)
-may have responded muscle involvement
-may have some sensation changes but these do not dominate
11
Q
what do investigations find for someone with GBS
A
LP - rise in protein with a normal WBC
NCS - decreased motor nerve conduction velocity due to demyelination
Anti GM1
spiro
Continuous BP monitoring - cause autonomic dysregulation
12
Q
MX points for GBS
A
IVIG
plasma exchange
ventilation / nutritional support
analgesia
DVT prophylaxis
pain relief
13
Q
Complications of GBS
A
LT disability, autonomic dysfunction, resp failure, DVT
14
Q
poor prognostic factors of GBS
A
high anti GM1, older age, need for ventilatory sup[port
15
Q
what causes CJD (Creutzfeldt Jakob disease)
A
prion proteins (which cause formation of beta pleated sheets which are resistant to proteases.) - prion proteins then cause other proteins to misfiled which leads to neuronal death
16
Q
how does CJD present
A
dementia, myoclonus and mood disturbance
17
Q
Ix for CJD
A
normal LP, EEG abnormal (biphasic high amplitude sharp wave), MRI abnormal (hyper intense signals in the basal ganglia)
-but only way to confirm diagnosis is post mortem
18
Q
what causes syringomyelia
A
a syrinx which is a collection of CSF within the spinal cord
19
Q
IX for syringomyelia
A
full spine MRI
20
Q
where do dorsal column second order neurones decussate
A
medulla
21
Q
where do spinothalamic second order neurones decussate
A
at the level they enter
22
Q
when is horizontal diplopia worse in a 6th nerve palsy
A
at a greater distance
23
Q
what is Lennox gastuat epilepsy
A
affects kids 1-5 y, mental handicap and presents with tonic seizure sin sleep and drop attacks in day
24
Q
what happens in west syndrome (infantile spasm)
A
get flexion of neck and trunk and then extension of arms + progressive mental handicap which normally begins in the first few months of life
25
what does EEG show in west syndrome
hypsarrhythmia
26
MX for infantile spasms
prednisolone and vigabatrin
27
what is benign Rolandic epilepsy
seizures on waking up which normally causes unilateral paralysis of the face
28
what is juvenile myoclonic epilepsy
normally affects teenage girls and is made worse by sleep disturbance, get generalised seizures, myoclonic seizures and daytime absence
29
absence seizures EEG findings
3Hz generalised symmetrical
30
what is the definition of epilepsy
it is an umbrella term which is used to describe the tendency to have a seizure (transient episodes of abnormal electrical activity in the brain)
31
RF for epilepsy
prematurity, tuberous sclerosis, neurofibromatosis, autism, head injury, CP
32
symptoms of temporal lobe epilepsy
rising epigastric feeling, deja vu, lip smacking, may get olfactory aura
-this is an example of a focal seizure
33
what is a Jacksonian march seen in frontal lobe epilepsy
clonic movement which travels proximally
34
features of generalised seizures
affects both sides of the cerebral hemispheres and awareness is always impaired
35
firstline for focal seizures
lamotrigine
36
Investigations for when someone has a seizure s
CBG, bloods (electrolyte imbalance can cause seizures), bone profile, ECG, cultures if suspected infection, VIDEO IT, EEG and MRI
37
human costs of misdiagnosing epilepsy
driving ban, stigmatisation, teratogenic drugs
38
what is the mutation in in Duchenne and Becker muscular dystrophy
the dystrophin gene (Becker is a milder form as it does not cause a frameshift mutation)
39
what is the pattern of inheritance in muscular dystrophies
X linked recessive
40
how do muscular dystrophies present
progressive weakness which starts proximal, delayed milestones, faltering growth,. fatigue, intellectual impairment
41
O/E of someone with muscular dystrophies
waddling gait, calf pseudo hypertrophy, intellectual impairment, tip toe walking due to shortening of the achilles, gowers sign (due to pelvic muscle instability - have to use arms to get up)
42
what serious condition is DMD associated with
dilated cardiomyopathy
43
how is a definitive diagnosis given for DMD
genetic analysis, do a muscle biopsy, raised CK!!!
-also need an annual echo for dilated cardiomyopathy
44
MX of DMD
steroids, vit D and physio, serial casting and physio, mobility aids and early palliative input
45
summarise multiple system atrophy
-combinaton of parkinsons and autonomic dysfunction (postural hypotension and incontinence)
-alpha synclin inclusions in olgiodendrocytes
-only way to prove diagnosis is post mortem
-supportive MX, no drug treatments
46
what is MS mediated by
aberrant T cell activation
47
what is primary progressive MS
steady worsening of the disease from onset without remission
48
what is secondary progressive MS
initially RR and then later declines steadily and progressively without remission
49
RF of MS
FHx, F>M, age 25-35, T1DM, smoking, previous EBV, vit D deficiency
50
what are the 4 ways in which MS can present
1) optic neuritis
2) transverse myelitis (where there is focal inflammation of the spinal cord which presents with parasthesia or motor symptoms below the level of inflam)
3) cerebellar symptoms
4) brain stem syndromes
51
what is uhthoff phenomenon
worsening of symptoms on exercise or in warm environments
52
what is lhermittes
phenomenon lightening shock pain down the spine on flexion of the neck due to cervical cord plaque formation
53
what happens INO
failure to adduct eye and nystagmus on the unaffected side
54
Ix for MS
FBC to rule out infection, Vit B12 to rule out sensory symptoms, CRP, MRI of the brain (hyper intense white matter plaques), LP (high protein content and oligoclonal bands)
55
what is a relapse defined as in RR MS
symptoms worsening >24 hours
56
acute vs LT mx of MS
acute - steroids (this can be give IV or orally and although they can shorten the relapse they do not improve recovery)
LT - beta interferon (injectable DMARDS) or monoclonal antibodies (can cause stroke/skin rash)
-exercise
-stop smoking, correct vit D deficiency
-advise on pregnancy
-specialist MS service
57
complications of MS
1) fatigue - need regular exercise, supervised exercise programs, SSRI
2) spasticity
3) ataxia and tremor
4) Oscillopsia
5) mobility + mental health
6) UUI
58
prognostic factors for MS
worse in men, worse with primary progressive, worse in higher number of relapses, worse if high lesion load on MRI
59
antibodies involved in MG
1) anti - ACHR
2) MuSK
3) LRP4 (low density lipoprotein receptor related protein 4)
60
what can make symptoms of MG worse
illness, emotional stress, drugs like beta blockers and aminoglycosides
61
Ix for MG
antibodies
EMG
Neostigmine test (Tensilon) - blocks cholinesterase enzymes
CT thorax to look for thymoma
Bedside spiro (cut off is 20ml/kg)
62
MX of MG
symptomatic - pyridostigmine (long acting ACHE inhibitor)
Prednisolone
Azathioprine is steroid sparing
63
Complications of MG
resp failure, aspiration, DVT, cholinergic crisis, myasthenia crisis
64
Tx for MG crisis
plasmapheresis and IVIG
65
what tracks are affected in SADoSC
dorsal column and lateral CST (so get UMN signs in lower limbs first like upgoing plantar reflexes) and spinocerebellar (so get positive Romberg sign)
-get absent ankle jerks and brisk knee reflexes
66
what tracks are preserved in SADoSC
spinothalamic
67
O/E of SADoSC
positive rhombergs, UMN signs, symmetrical sensory deficit
68
RF for SADoSC
malnutrition, pernicious anaemia, NO use
69
Ix for SADoSC
B12 and folate, may do MRI to rule out anything else
70
Tx for SADoSC
high dose Vit B12 supplementation (if folate deficiency too, must treat the Vit B12 deficiency first)
71
presentation of DCM
neck pain, loss of motor function (manual dexterity), loss of sensory functions, Hoffman sign (UMN sign)
72
Ix of DCM
MRI - may see disc degeneration and ligament hypertrophy
73
MX of DCM
NSAIDs and physio, cervical laminectomy (but recurrence is a problem)
74
how does diabetic neuropathy usually present first
sensation loss happens first (check with monofilament)
loss of reflexes is a late sign
75
Dx of diabetic neuropathy
HbA1c, Vit B12, NCS (gold standard - which show decreased amplitudes and decreased conduction velocities)
76
if someone with parkinsons is not bothered by motor symptoms what should be started
MAOb inhibitor or dopamine agonist
77
protective effects of parkinsons
exercise, caffeine, smoking
78
RF of parkinsons
FHx and head injuries
79
what scan can be done to rule out a parkinsons tremor from an essential tremor
SPECT
80
what are the four stages of parkinsons
1) early - just after diagnosis
2) maintenance - good response to tx
3) advanced - poor response to Tx and more dyskinetic movements from the tx
4) palliative
81
what is end stage parkinsons
when someone's life expectancy is 6-12 months, low BMI, performance status 3 or above, speech problems --> at this point focus on advanced care planning
82
complications of parkinsons
autonomic dysfunction, cognitive impairment, recurrent falls
83
how can we distinguish drug induced Parkinsonism from (metoclopramide)
symmetrical symptoms and drug induced can cause specific symptoms like tardive dyskinesia
84
what are some differentials for parkinsons
Lewy body dementia (the dementia comes before the movements), drug induced, multiple system atrophy, cerebrovascular disease (stepwise progression)
85
what is Charcot Marie tooth syndrome
inherited, affect the peripheral SENSORY AND MOTOR
86
what is the inheritance pattern of Charcot Marie tooth
autosomal dominant
87
who does CMT syndrome present
weakness in feet, then hands, distal muscle wasting, peripheral sensory loss
-there is no cure but people normally life a normal life expectancy with mobility aids
88
RF for bells palsy
pregnancy, HTN, DM, obesity
89
what does forehead sparing indicate
the lesion is UMN and therefore is not bells palsy but stroke
90
Mx of bells palsy
good eye care and oral pred within 72 hours of onset
91
when do you refer to secondary care with bells palsy
if no improvement after 3 weeks (should completely recover within 4 months)
92
complications of bells palsy
exposure keratitis
93
complications of carpal tunnel syndrome surgery
trigger finger + persistent symptoms
94
what colour is the CSF in viral meningitis
clear
95
SE of LP
low pressure headaches which is better lying down and worse standing up - advise patients to have caffeine, best rest and fluids
96
features of essential tremor
fine tremor, better with alcohol, bilateral, made worse with anxiety
97
when is a diagnosis of essential tremor given
when it has been present for 3 years and there is no neurological signs (and other things like hyperthyroidism have been ruled out)
98
Mx for essential tremor
propanolol and primidone
99
VITAMIN C for cerebellar syndromes
Vascular - stroke in POCS
Infections - lymes
Trauma
MEtabolic - alcoholism
Iatrogenic
Neoplastic - cerebellar pontine angle tumour
Congential (Freireichs)
100
a lesion in the cerebellar produces what kind of signs
ipsilateral!!!
101
IX for suspected cerebellopontine angle lesion (acoustic neuroma)
MRI
102
if diagnosis of a Horners is unclear, what can you do?
use apraclonidine / cocaine eye drops to reverse pupillary restriction
103
how does the sympathetic chain work
3 neurones
first order from hypothalamus down to T1
Second order from T1 to superior cervical ganglion
Third order from superior cervical ganglion to effector
first and second order together are the preganglioninc (these are short and myelinated and then synapse with acetylcholine)
Third order are the post ganglionic, these are long and non myelinated and travel on ICA to eyes or ECA to sweat glands.
104
Signs of Horners
partial ptosis (loss of superior tarsel), miosis (due to loss of dilator pupillae) and anhydrosis
105
central causes of Horners
these effect the first order neurones from hypothalamus --> stroke specifically PICA (laterally medullary stroke, MS). This causes anhidrosis of face and body, miosis and ptosis
Pre ganglionic (second order neurones - these leave the spinal cord and ascend up the sympathetic chain to the superior cervical ganglion)--> pancoast/ thyroid malignancy/trauma
-this causes anhidrosis of face, miosis and ptosis
Post ganglionic (third order neurones which go from the superior cervical ganglion to the effector tissues)--> carotid artery dissection or cavernous sinus thrombosis
-this causes NO ANHIDRYOSIS, miosis and ptosis
106
symptoms of autonomic dysreflexia (if lesion is T6 or above)
hypertension, bradycardia, sweating above lesion, constipation, retention
-RISK of haemorrhagic stroke
107
which part of the spinal cord does vit B12 deficiency effect
the posterior part hence get loss of dorsal column and lateral CST
108
signs of brown sequard syndrome
hemiparalysis on same side of lesion, ipsilateral loss of dorsal column (proprioception and vibration) and contralateral loss of spinothalamic (pain, temp, crude touch)
109
what is spinal cord concussion
transient loss of function for 48 hours or less
110
Ix for SOL
MRI
111
what is normal pressure hydrocephalus
it is a reversible type of dementia where there is dementia, gait disturbance and urinary incontinence.
Imaging shows ventricle enlargement.
Thought to be due to reduced CSF absorption
112
What is the MX for normal pressure hydrocephalus
ventriculoperitoneal shunting
113
definition of cognitive impairment
high cortical impairment which causes problems with memory, thinking, language, judgement
114
what is the definition of a dementia
a cognitive impairment which is sufficient to impair ADL which must happen in clear consciousness and must be present for > 6 months
115
3 reversible causes of dementia
NPH, hypothyroidism and cushings and depression
116
5 irreversible causes of dementia
alzheimers, parkinsons, Lewy body, TBI
117
RF of dementia
age, FHx, DS, low IQ
118
what cause Alzheimers
mutations in amyloid precursor proteins which cause amyloid plaques
119
presentation of Alzheimers
-memory loss affecting ST first
-disorientation to space and time
-word finding difficulties
-apraxia (inability to do previously learnt and purposeful movement despite normal coordination
-visuospatial abnormalities
-BPSD
-difficulties with ADLs
120
what are the BPSD of dementia (behavioural and psychological symptoms of dementia)
Apathetic, Psychotic, Affective, Hyperactive
121
what are the stages of dementia
1) early --> poor memory and subtle mood change with limited impact on day to day activities
2) mid --> memory problems are more prominent, may see behaviour change, need help with instrumental ADLS
3) late --> severe disability and need help even with basic aspects of personal function
122
what would you see on post mortem exam of someone with dementia
neurofibrillary tangle and B amyloid plaques
123
MX of Alzheimers
1) acetylcholinesterase inhibtiors (donepezil, rivastigmine, galantamine)
2) memantine - NMDA inhib
-activities to promote wellbeing
-cognitive stimulation therapy (eg group therapy where they work through news articles)
-risperidone for BPSD
-sertraline for low mood
-tell DVLA
124
what SE of donepezil
bradycardia and GI upset
125
what proteins are deposited in the brain in Lewy body
alpha synuclein proteins
126
characteristics of a Lewy body dementia
rapidly progression and fluctuating, visual hallucinations, cognitive symptoms must be present > 1 year before parkinsons symptoms
127
MX of LBD
1) donepezil
2) memantine
NEVER USE NEUROLEPTICS
128
what Ix can you do to diagnose Lewy body
SPECT / DAT scan
129
what proteins are deposited in frontal temporal dementia
tau proteins
130
is memory affected in FTD
no! more behavioural symptoms in the under 65s
-also get frontal release signs
131
what will imaging show in FTS
frontotemporal atrophy
132
what is the Mx of FTD
structured routine, cognitive stimulation and caregiver education
133
what are the feature of alcoholic dementia
gradual onset and cognitive status fluctuates with drinking / withdrawal episodes
134
what nerve root is affected in a lumbar disc prolapse
transversing (due to paracentral disc prolapse)
135
what nerve root is affected in a cervical disc prolapse
the exiting nerve root (so if the C6/C7 disc prolapses, the C7 nerve is compressed)
136
O/E of cervical spondylosis
reduced ROM in all planes, poorly localised tenderness and signs of radiculopathy
137
RF for cervical spondylosis
Fhx, neck trauma, ageing, previous occupation involving repetitive work like a painter
138
MX of a cervical spondylosis
stay active, NO cervical collar, one firm pillow at night, simple analgesic, topical NSAID or oral analgesics , refer to secondary care if symptoms > 4 weeks for an MRI if any neurological symptoms. If not neurological symptoms do a cervical spine Xray.
139
what runs through the cavernous sinus
III,IV, Va, Vb, VI and ICA
140
where does neuronal cell death occur in Huntingtons
the caudate and the putamen of the basal ganglia
141
what are the features of Huntingtons
chorea, personality change, dystonia and saccadic eye movements
142
name a drug that can be used in the Tx of Huntingtons
tetrabenazine
143
definition of syncope
transient loss of consciousness characterised by fast onset and spontaneous recovery caused by cerebral hypoperfusion
144
what is the role of the thalamus
relays sensory and motor info to the cerebral cortex
145
pathway of the pyramidal tracts
start in the PMC, travel through the internal capsule, decussate at the meduallary pyramids and then travel in the ventral horn down.
146
what does a T1 weighted MRI show
fat is bright
147
what does a T2 weighted MRI show
fat and water are bright
148
how does the posterior circulation enter the brain
via the foramen magnum
149
what part of the motor system does the basal ganglia form part of
the extrapyramidal
150
what are the motor system functions in the basal ganglia
maintain posture, regulate involuntary movements and fine tune voluntary movement s
151
what is the basal ganglia made of
5 pairs of nuclei:
1) caudate
2) putamen
3) globus pallidus
4) subthalamic
5) STN
-1+2 = the striatum
152
in the parasympathetic NS where is the ganglia
close to target organs
153
which opioid can be used to tx neuropathic pain when standard tx have failed
tramadol
154
apart from bells palsy, what are other causes for a facial LMN palsy?
acoustic neuroma, otitis media, diabetes
155
what happens in an uncal brain herniation and what is a cause of this
the medial temporal lobe herniates over the tenstorial notch. This causes a CNIII palsy.
156
what happens in a tonsillar herniation?
this is a terminal event where the medulla is compressed band this is where the cardio respiratory centre is
157
what lobe of the brain is the uncus form
temporal
158
what is hoovers sign
used for distinguishing between organic and non organic causes of leg weakness. Put hands under either heel, ask pt to lift up leg with weakness, if the patient is making a genuine effort there will be a downward force on the good leg due to synergistic contraction.
159
complications of epilepsy
sudden unexplained death in epilepsy, injury, not being able to drive
160
when can you start withdrawing anti epileptic drugs
after being seizure free for two years
161
if someone has a diagnosis of epilepsy, how long must they not drive for
12 months
162
if someone has a single unprovoked seizure, how long must they not drive for
6 months
163
what is friedrichs ataxia
inherited condition where there is impairment of mitochondrial functionjn , trinucleotide repeat, cause gait ataxia and kyphoscoliosis, does not show anticipation
164
what is parkinsons
disease of the basal ganglia where there are a loss of dopaminergic neurones in the pars compact of the substantia nigra
165
how is parkinsons normally diagnosed
clinically, only do investigations if there is diagnostic uncertainty - MRI
166
side effects of all parkinsons drugs if asked in exams for one
hallucinations
167
features of progressive supranuclear palsy
a parkinsons plus syndrome -> inability to look upwards, postural instability along with the normal symptoms
POOR RESPONSE TO LEVO DOPA
168
do multiple systems atrophy and progressive supra nuclear palsy have a good or bad response to levodopa
bad
169
triad of meningitis symptoms
necks stiffness, fever, altered mental state
170
cause of meningitis that causes no blanching rash
meningococcal septicaemia so Neisseria meningitidis
171
when do you treat meningitis with cefotaxime and amoxicillin
< 3 months or over 50 years
172
how does klumpkes palsy present
claw hand (damage to C8/T1) from birth or from grabbing branches when falling
173
what is conduction dysphasia
speech is fluent but repetition is poor
174
what causes a conduction dysphasia
a stroke affecting the arcuate fasciculus which is the area connected brocas and wernickes area
175
impulse control disorders can be a complication of parkinsons, when are these more common
-starting dopamine agonist
-history of alcohol abuse
176
when do you start anticoagulation for a stroke caused by afib
after 14 days of an ischaemic stroke
177
when is migraine prophylaxis offered
if having 2+ migraines a month
178
why is propanol preferred for migraine prophylaxis in women of child bearing age
topiramate associated with clef lip
179
MND associated with what type of dementia
FTD
180
why can you get emotional lability in MND
UMN lesion to the pseudo bulbar tract
181
firstline for neurogenic bladder dysfunction in MS
US KUB, do not start on anticholinergics without this as if there is a large residual volume this can cause retention.
If there is a large residual volume then would need to do an intermittent self catheterisation
182
firstline for scalp psoriasis
topical steroids
183
MX of acute necrotising gingivistis (severe gum disease)
chlorhexidine, good oral hygiene and metronidazole
