Neurology Flashcards
1
Q
what is a acoustic neuroma
A
it is a rare tumour of the vestibulocochlear nerve
2
Q
what cells does an acoustic neuroma arise from
A
schwann cells of the nerve sheath
3
Q
are acoustic neuromas benign/malignant
A
they are benign and slow growing
however they can occur at the cerebellopontine angle which can compress local structures and lead to life threatening conditions
4
Q
are acoustic neuromas unilateral or bilateral
A
they are usually unilateral
if bilateral think of neurofibromatosis type 2
5
Q
what are the clinical features of an acoustic neuroma
A
hearing loss - unilateral
unilateral tinnitus
vestibular disturbance - dizziness or imbalance
sensation of fullness in the ear
can also be associated with facial nerve palsy (forehead NOT spared)
Can headaches nausea and vomiting due to increased intercranial pressure
6
Q
what are risk factors of developing an acoustic neuroma
A
high dose ionising radiation to the head and neck
neurofibromatosis type 2
7
Q
what would be differential diagnosis be for acoustic neuroma
A
meningioma
ectodermal inclusion tumours
neuromas originating from other cranial nerves
extension of nearby lesions
8
Q
what investigations should be done with suspected acoustic neuroma
A
MRI head
pure tone audiometry - sensorineural pattern of hearing loss
9
Q
what is the management of acoustic neuroma
A
active observation
microsurgery
radiosurgery
10
Q
what does the management of an acoustic neuroma depend on
A
size of tumour
patient factors - age and future quality of life
involvement of local structures
11
Q
what microsurgery options are there for acoustic neuromas
A
retrosigmoid, translabyrinthine and middle fossa approach to the tumour
- which one is chosen will depend on location and size of tumour
12
Q
what is conservative treatment for acoustic neuromas and when is it done
A
active surveillance with annual neuroimaging - MRI
this is done in patients with small tumours with no impairment or in elderly patients with multiple medical co morbidities
13
Q
what are the risks of microsurgery for acoustic neuroma
A
cerebrospinal fluid leak
cerebellar injury
meningitis
hearing loss and/or facial paralysis
death
14
Q
what is stereotactic radiosurgery for acoustic neuroma
A
it is used for tumours <3cm and it involves high energy gamma rays to deliver a singe dose of radiation to the tumour (gamma knife)
15
Q
what are the risks associated with stereotactic radiosurgery for acoustic neuroma
A
radiation necrosis to the brain
malignant change of the neuroma to glioblastoma
16
Q
what are complications of acoustic neuroma
A
hearing loss
facial paralysis
hydrocephalus
compression of the cerebellar peduncles, cerebellum, brainstem and cranial nerves IX-XI
17
Q
what is anterior cord syndrome
A
it is incomplete spinal cord injury
18
Q
how does anterior cord syndrome present
A
with impairments in the pain and temperature sensations while vibration and proprioceptive sensations are preserved
motor deficits are seen both at and below the level of injury
19
Q
what causes anterior cord syndrome
A
ischaemia within the anterior spinal artery
20
Q
what does the anterior spinal artery supply
A
the bilateral anterior and lateral horns of the spinal cord
the spinothalamic tracts
the corticospinal tracts
21
Q
what are causes of anterior cord syndrome
A
iatrogenic - cross clamping of the aorta during aneurysm repair
direct injury - crash, burst, knife
indirect injury - occlusion or hypoperfusion due to severe hypotension, atherothrombotic disease or vasculitis
22
Q
what is the mechanism of a bust injury (anterior cord syndrome)
A
forces coming from above or below the vertebral body
nucleus pulposus of the intervertebral disc is forced into the vertebral body
causes it to shatter and cause spinal cord injury
23
Q
what are the two phases of mechanical trauma to the spinal cord in anterior cord syndrome
A
the initial direct trauma results in acute compression and disruption of vasculature
second phase there is inflammation, oedema, haemorrhage, demyelination changes in the neurones
a later stage consists of scar formation, wallerian degeneration and development of cysts
24
Q
how does anterior cord syndrome present
A
varying degrees of muscle weakness and sensory loss
- pain sensation is decreased or absent
- proprioception is maintained
- loss of motor power usually mimics same pattern of pain loss
chance of autonomic dysreflexia, movement and sexual impairments, neuropathic pain, bladder and bowel dysfunction
25
what motor effects will there be in anterior cord syndrome
below the level of spinal injury there will be bilateral motor dysfunction
- flaccidity with absent reflexes which may return with increased tone or spasticity
26
what sensory effects will there be in anterior cord syndrome
alterations in pain and temperature sensation present two to three dermatomal segments below the level of the injury
27
how is anterior cord syndrome diagnosed
detailed history and exam
MRI - pencil like hyper intensities
axial MRI will show owls eye
lumbar puncture
echo
bloods and U+E
28
how is anterior cord syndrome managed
mainly supportive
need to address the limitations in mobility and activities of daily living
29
whats the non surgical management of anterior cord syndrome
management of anticoagulation
antiplatelet and thromboprophylaxis
fever and glycemic control
blood pressure support
rehabilitation focused on improving independence and quality of life
30
where does the facial nerve leave the brainstem
at the cerebellopontine angle, passes through the temporal bone and the parotid gland
31
what are the five branches of the facial nerve
temporal
zygomatic
buccal
marginal mandibular
cervical
32
what is the motor function of the facial nerve
facial expression
stapedius in the inner ear
posterior digastric, stylohyoid and platysma muscles
33
what is the sensory function of the facial nerve
taste from the anterior 2/3 of the tongue
34
what is the parasympathetic function of the facial nerve
submandibular and sublingual salivary glands
lacrimal gland
35
is the forehead spared in a lower motor neurone lesion
NO !!!!!!!!!!!!!!!!!!!!!!!!!
36
what is bells palsy
it is a unilateral lower motor neurone facial nerve palsy
37
what is the cause of bells palsy
it is idiopathic - no apparent cause
38
how long is the recovery of bells palsy
most patients fully recover over several weeks but recovery may take up to 12 months
- 1/3 are left with some residual weakness
39
what is the treatment for bells palsy
if the patient presents within 72 hours of symptom development then prednisolone for
- 50mg for 10 days
- 60 mg for 5 days followed by a 5 day reducing regime dropping the dose by 10mg each day
patients also require lubricating eye drops
40
what is Ramsay-hunt syndrome
it is caused by VZV and causes a unilateral lower motor neurone facial nerve palsy
41
what are symptoms of ramsay hunt syndrome
painful and tender vesicular rash in the ear canal, pinna and round the ear. this rash can extend to the anterior two thirds of the tongue and hard palate
have facial drop like in bells palsy
42
what is the treatment of |Ramsay hunt syndrome
aciclovir
prednisolone
lubricating eye drops
43
what are other infectious causes of lower motor neurone facial nerve palsy
otitis media
otitis externa
HIV
lyme disease
44
what are systemic causes of lower motor neurone facial nerve palsy
diabetes
sarcoidosis
leukemia
multiple sclerosis
guillian barre
45
what tumours can cause lower motor neurone facial nerve palsy
acoustic neuroma
parotid tumour
cholesteatoma
46
what trauma can cause lower motor neurone facial nerve palsy
direct nerve trauma
surgery
base of skull fractures
47
what is a brain abscess
it is a pus filled swelling in the brain
48
what are ways a brain abscess may develop
direct extension of cranial infections - osteomyelitis, mastoiditis, sinusitis
penetrating head wounds
hematogenous spread
unknown cause
49
what bacteria commonly cause brain abscess
staphylococci - due to trauma, surgery or endocarditis
enterobacteriaceae - chronic ear infections
fungi (aspergillus) and protozoa (toxoplasma gondii) in HIV infected patients
50
what are symptoms of a brain abscess
headache
nausea
vomiting
lethargy
seizures
personality changes
papilloedema
focal neurological deficits
fever
chills
leukocystosis
51
how do you diagnose a brain abscess
Contrast enhanced MRI
if unavailable then contrast enhanced CT
CT guided aspiration and culture
52
what will a brain abscess look like on MRI
oedematous mass with ring enhancement - can look similar to tumour or infarction
53
how is a brain abscess treated
antibiotics - initially cefotaxime or ceftriaxone plus metronidazole/vancomycin
CT guided aspiration/surgical drainage
corticosteroids
anti seizure medication
54
how long should antibiotics be given for with a brain abscess
4-8 weeks
cefotaxime 2g IV every 4 hours
ceftriaxone 2g IV every 12 hours
55
what are the clinical features of a brain tumour
signs relating to raised intercranial pressure are common
- headache: worse on waking, lying down, bending forward or coughing
- nausea and vomiting
- papilloedema
- coma
56
what is a meningioma
it is a slow growing tumour that forms from the arachnoid cell cap found on the meninges
57
what are the management options of a meningioma
they are usually benign and surgically viable
58
when are meningiomas most common
peak incidence occurs at 45 yrs
2;1 female ratio
59
how might someone with a meningioma present
may be asymptomatic
headache
seizure
spastic weakness or numbness in both leg
gait abnormalities
visual loss
60
what investigations are done for a meningioma
MRI with gadolinium contrast (+/- CT)
angiography
61
how is meningioma managed
usually conservative unless symptoms develop
surgical excision
radiotherapy
62
what is a pituitary adenoma
it is a cancer that occurs in the pituitary gland
they can be functional and secrete hormones
63
what are the clinical presentations of pituitary adenomas
- cushingoid symptoms
- acromegaly
- diabetes insipidus
- hyperpituitarism
- amenorheoa/galactorrhoea/impotence
- bitemporal hemianopia
- hydrocephalus
- diplopia
64
what investigations are done for suspected pituitary adenoma
endocrine lab tests
MRI with contrast (+/- CT)
65
how is a pituitary adenoma managed
dopamine agonists to treat prolactinomas
surgery
radiation therapy
66
what are complications of pituitary adenoma
pituitary apoplexy - when haemorrhage causes a rapid increase in the size of the lesion and surrounding vasogenic oedema
due to this is causes tissue necrosis with sudden onset visual loss, headache and hydrocephalus
67
what is a craniopharyngioma
it arises in the pituitary stalk and can affect the hypothalamus and the pituitary
68
what is a craniopharyngioma derived from
residual cells of Rathkes pouch
69
what is the age of onset of craniopharyngiomas
bimodal age of onset 5-14 and 50-70
70
what are the symptoms of craniopharyngiomas
growth failure in children
diabetes insipidus
sexual dysfunction in adults
hydrocephalus
bitemporal hemianopia
pituitary insufficiency
headache
71
what investigations are done for craniopharyngiomas
MRI with contrast (+/- CT)
endocrine lab investigations
72
what is the management for craniopharyngiomas
surgical removal
radiotherapy
73
what is primary CNS lymphoma
it is usually a large B cell lymphoma
74
what are the clinical features of primary CNS lymphoma
seizures
headache
altered mental status
systemic symptoms: fever, night sweats, unintentional weight loss
diplopia
vertigo
dysphagia
75
what investigations are done for primary CNS lymphoma
MRI +/- CT
HIV testing
lumbar puncture - may show increased WCC
EBV virus PCR
76
what is the management of primary CNS lymphoma
chemotherapy - methotrexate (+ cytarabine)
radiation therapy
steroids - dexamethasone
surgery
77
what is a medulloblastoma
it is thought to arise from cerebellar stem cells and tend to seed within CSF pathways giving rise to a high metastatic propensity
78
what population is medulloblastoma most common in
children
79
what are the clinical features of medulloblastoma
hydrocephalus: worsening headache and decreased consciousness
cerebellar signs - incoordination and gait abnormalities
mass effect symptoms - speech and vision issues, unilateral weakness, headache, drowsiness, nausea and vomiting
extraocular muscle palsies
80
what investigations are done for medulloblastoma
lumbar puncture
MRI +/- CT
81
what is the management for medulloblastoma
surgical removal of the tumour
radiotherapy
chemotherapy
82
what is a glioma
it is an umbrella term for a variety of tumour subtypes consisting of malignant tumours of the glial tissue of the nervous system
83
what are different types of gliomas
astrocytoma's
glioblastoma multiforme
pilocytic astrocytoma
ependymomas
oligodendrogliomas
84
what is the mean age of onset for gliomas
about 55
85
how to gliomas metastisise
all gliomas metasisise via the CSF
86
what are the clinical features of gliomas
headache
vomiting
seizures
focal neurological deficit including vision loss
cranial nerve dysfunction
87
what investigations are done for suspected glioma
MRI with contrast
88
what is the management for gliomas
surgery - complete excision often challenging. Carmustine implants can be added which can help reduce rate of tumour spread
Radiotherapy
Chemotherapy - temozolomide
89
what is the prognosis for gliomas
overall poor prognosis as they are rarely curable
90
what are causes of increased intercranial pressure
brain tumours
intercranial haemorrhage
idiopathic intercranial hypertension
abscess of infection
91
what are symptoms of raised intercranial pressure
constant headache which is nocturnal, worse on waking, worse on coughing, straining or bending forward
vomiting
papilloedema on fundoscopy
altered mental status
visual field defects
seizures
unilateral ptosis
third and sixth nerve palsies
92
what is papilloedema
it is swelling of the optic disc secondary to raised intercranial pressure
93
how is papilloedema seen on fundoscopy
blurring of the optic disc margin
elevated optic disc
loss of venous pulsation
engorged retinal veins
haemorrhages around the optic disc
patons lines - creases or folds in the retina around the optic disc
94
what are the three main types of glioma
astrocytoma - most common and aggressive
oligodendroglioma
ependymoma
95
what cancers most often spread to the brain
lung
breast
renal cell carcinoma
melanoma
96
what is bulbar palsy
it is a lower motor neurone impairment in nerves 9, 10, 11 and 12
97
what are symptoms of bulbar palsy
depends on nerve damaged
- swallowing difficulties
- reduced gag reflex
- difficulty chewing
- nasal regurgitation
- slurred speech
- difficult in handling secretions
- aspiration of secretions
- altered vocal ability
- difficulty articulating words
- nasal speech that lacks in modulation
- difficulty with consonants
- wasting tongue
- drooling
- weakness of jaw and facial muscles
- absent or abnormal jaw reflex
98
what are the two types of bulbar palsy
progressive and non progressive
- progressive is more common
99
what is pseudobulbar palsy
it is a result of damage to the upper motor neurons
100
what is the difference between bulbar palsy and pseudobulbar palsy
in pseudobulbar palsy the patient often has atypical expression of emotion displaying unusual outbursts of laughing or crying
101
what are causes of bulbar palsy
brainstem strokes
tumours
degenerative diseases - ALS
autoimmune diseases - guillian barre
genetic diseases - kennedy disease, brown-vialetto-van laere syndrome, fazio-londe syndrome
102
what is Kennedy disease
it is a lower motor neurone disease that can affect transmission signals between the brain and spinal cord
- x linked
103
how do you diagnose bulbar palsy
history and exam
CSF analysis
MRI of brain
104
how is bulbar palsy treated
currently no known treatments
supportive management - feeding tube, speech and language therapy, medications to prevent drooling
condition specific treatment
105
is bulbar palsy fatal
it can be in progressive cases - can occur 1-3 years from the start of the disorder and normally attributed to aspiration pneumonia
106
what are the three parts of the cerebellum
Archicerebellum/vestibulocerebellum
midline vermis
lateral hemispheres
107
what is the role of the archicerebellum
maintains equilibrium and coordinated eye, head and neck movements
108
what is the role of the midline vermis (cerebellum)
coordinates trunk and leg movements
109
what is the role of the lateral hemispheres (cerebellum)
control quick and finely coordinated limb movements predominantly the arms and legs
110
what is the most common cause of cerebellar disorders
alcoholic cerebellar degeneration
111
what are causes of cerebellar disorders
alcoholic cerebellar degeneration
congenital malformations
hereditary ataxias
acquired ataxias - MS, stroke, traumatic brain injury, toxin exposure, celiac disease, hypothyroidism
paraneoplastic syndrome
112
what toxins can cause cerebellar dysfunction
carbon monoxide
heavy metals
lithium
phenytoin
solvents
113
what are symptoms of cerebellar degeneration
balance problems
decreased muscle tone
eye problems - double vision and nystagmus
poor muscle coordination in arms and legs
problems with motor skills such as holding your head up
slow and slurred speech
114
how id cerebellar degeneration diagnosed
MRI or CT
CSF analysis
bloods - immune issues
115
how is cerebellar degeneration managed
treatment of possible cause
usually supportive
Riluzole 50mg every 12 hours for short term ataxia treatment
chemo if paraneoplastic syndrome
alcohol abstinence
physical, occupational or speech therapy
116
what is cerebral palsy
it is a non progressive permanent neurological condition commonly affecting normal movement and posture
117
what causes cerebral palsy
it is caused by damage to the developing brain which can occur while the baby is in utero, during birth or in the neonatal period
- hypoxia, haemorrhage, infection
118
what are the antenatal risk factors of cerebral palsy
multiple gestation
chorioamnionitis
maternal infections - toxoplasmosis, rubella, CMV and herpes simplex
119
what are the perinatal risk factors for cerebral palsy
prematurity
low birth weight
birth asphyxia
neonatal sepsis
120
what are the clinical features of cerebral palsy
delayed motor milestones
- not siting by 8 months
- not walking by 18 months
- hand preference before 12 months
tone abnormalities
abnormal movements
feeding issues such as choking/dysphagia
persistent toe walking
121
what are postnatal risk factors for cerebral palsy
meningitis
severe hyperbilirubinaemia
122
what are the different types of cerebral palsy
spastic cerebral palsy
dyskinetic cerebral palsy
ataxic cerebral palsy
123
what is spastic cerebral palsy
it is charactised by velocity dependent hypertonia and hyperreflexia
it is the most common type
when a limb is moved quickly the muscle can suddenly increase in tone and stop further movement
124
what is dyskinetic cerebral palsy
it is charactised by involuntary, uncontrolled, recurring movements, fluctuating muscle tone and persistent primitive reflexes
125
what are the two subtypes of dyskinetic cerebral palsy
dystonic - abnormal posturing and hypertonia
choreoathetotic - chorea and athetosis
126
what is ataxic cerebral palsy
it is characterised by a loss of muscle coordination resulting in ataxia and tremor
127
what are the classifications of cerebral palsy depending on limbs affected
monoplegic
hemiplegic
dipelgic
quadraplegic
128
what is hypertonia
it is a general term for increased resistance to movement
129
what is spasticity
velocity dependent
the faster you move a limb the higher the tone you will feel
130
what is dystonia
it is abnormal postures which are worse on intention
131
what investigations are done for cerebral palsy
it is a clinical diagnosis
MRI brain - may show damage
132
what is conservative management for cerebral palsy
physiotherapy - improve function
occupational therapy
speech and language therapy
dietician input
133
what is the medical management for cerebral palsy
hyoscine hydrobromide - excess drooling
diazepam - pain
baclofen: hypertonia
botulinum toxin type A injections for spasticity
134
what surgical management is done for cerebral palsy
hip displacement is very common and surgical intervention may be required
135
what are complications of cerebral palsy
problems with eating or drinking
drooling
constipation
virtual and hearing issues
epilepsy
learning disability
speech difficulty
osteopenia and osteoporosis
sleep disturbance
136
what is hypoxic ischaemic encephalopathy
it is brain injury that occurs when the brain doesnt get enough oxygen/blood and permanently affect brain unction
137
what are causes of hypoxic ischaemic encephalopathy
traumatic birth
neonatal encephalopathy
perinatal hypoxia
138
what are signs/symptoms of hypoxic ischaemic encephalopathy
a floppy, weak muscle tone or tense muscles
difficulty feeding
fatigue
irritability
a weak cry
a pale blue or gray tone to the skin, fingers and lips
little to no response to sound
poor reflexes
irregular or slow HR
abnormal breathing
seizures
LOC
139
what are causes of hypoxic ischaemic encephalopathy during foetal development
abnormal development or function of fetus heart or lung
an infection - toxoplasmosis or CMV
blood pressure thats too high or too low
low oxygen levels in pregnant parent
140
what acre causes of hypoxic ischaemic encephalopathy during birth
umbilical cord prolapse
umbilical cord compression
placental abruption
uterine rupture
placenta praevia
141
what are causes of hypoxic iscaemic encephalopathy in older children and adults
arrhythmia
asphyxiation
overdose
poisoning
shock
142
what are risks factors of developing hypoxic ischaemic encephalopathy
low birth weight
amniotic fluid contamination
delayed fetal development
preeclampsia
gestational diabetes
prolonged labour
substance use disorder
143
what are complications of hypoxic ischaemic encephalopathy
developmental delays
problems with growth
hearing or vision loss
issues with cognitive function
difficulty paying attention
epilepsy
cerebral palsy
infections
144
how is hypoxic ischaemic encephalopathy diagnosed
medical emergency
physical exam
fetal heart monitoring
placenta bloods
newborn wellness assessment
cord blood and gasses
MRI
EEG
145
how is hypoxic ischaemic encephalopathy treated
cooling babies body followed by rewarming body to protect brain from further injury
EEG monitoring and antiseizure medication
supportive treatment - oxygen
physical and occupational therapy
speech therapy
early intervention educational programs
146
what is chronic fatigue syndrome
it is extreme fatigue that lasts for at least six months
symptoms worsen with physical or mental activity but dont fully improve with rest
147
what are the symptoms of chronic fatigue syndrome
extreme exhaustion after physical or mental exercise
problems with memory or thinking
dizziness that worsens with moving from lying down or sitting to standing
muscle or joint pain
unrefreshing sleep
headaches
sore throats
tender lymph nodes
148
what are causes of chronic fatigue syndrome
genetics
infections
physical or emotional trauma
problems with energy storage
149
what are risk factors of chronic fatigue syndrome
age - young to middle aged adults
sex- women more common
other medical conditions - fibromyalgia or PoTS
150
what is the diagnostic criteria for chronic fatigue syndrome
- so severe that it interferes with the ability to engage in pre illness activities
- new or definite onset
- not substantially alleviated by rest
- worsened by physical, mental or emotional exertions
- difficulties with memory, focus and concentration
- dizziness that worsens with moving from lying down o from sitting to standing
151
how long do symptoms need to be present for before a diagnosis of chronic fatigue syndrome is made
at least six months and occur at least half the time at moderate, substantial or severe intensity
152
what is the treatment for chronic fatigue syndrome
there is no cure treatment focuses on symptom relief
- pain: ibuprofen, naproxen, pregabalin, gabapentin
- orthostatic intolerance: bp regulation
- depression: antidepressants
153
what is diabetic neuropathy
it is a type of nerve damage that can occur with diabetes
154
what are the four main types of diabetic neuropathy
peripheral neuropathy
autonomic neuropathy
proximal neuropathy
mononeuropathy/ focal neuropathy
155
what is peripheral neuropathy
it is the most common type of diabetic neuropathy and it affects the feet and the legs first
156
what are symptoms of peripheral neuropathy
numbness or reduced ability to feel pain or temperature changes
tingling or burning feeling
sharp pains or cramps
muscle weakness
extreme sensitivity to touch
serious foot problems
157
what are symptoms of autonomic neuropathy
a lack of awareness that blood sugar is low
drops in blood pressure when rising from sitting or lying down that may cause dizziness
bladder or bowel issues
slow stomach emptying causing N+V
difficulty swallowing
changes in the way the eyes adjust to light/dark/things far away
increased or decreased sweating
problems with sexual response, vaginal dryness in women and erectile dysfunction in men
158
what is proximal neuropathy
it is a type of neuropathy that affects the nerves in the thighs, hips, buttocks or legs
symptoms are normally on one side of the body
159
what are symptoms of proximal neuropathy
severe pain in the buttock, hip or thigh
weak and shrinking thigh muscles
difficulty rising from a sitting position
chest or abdominal wall pain
160
what is mononeuropathy
it is damage to a single specific nerve
161
what are signs of mononeuropathy
difficulty focusing or double vision
paralysis on one side of the face
numbness or tingling in hands or fee
weakness in the hand
pain in the shin or foot
foot drop
pain in the front of the thigh
162
what are risk factors or developing diabetic neuropathy
poor blood sugar control
diabetes history
kidney disease
being overweight
smoking
163
what are complications of diabetic neuropathy
hypoglycaemic unawareness
loss of toe, foot or leg
urinary tract infections and urinary incontinence
sharp drops in blood pressure
digestive issues
sexual dysfunction
increased/decreased sweating
164
how can you prevent diabetic neuropathy
blood sugar control
foot care
165
how is diabetic neuropathy diagnosed
filament testing
sensory testing
nerve conduction testing
electromyography
autonomic testing
166
how is diabetic neuropathy treated
slow progression of disease - maintaining blood sugars, maintaining healthy weight, regular physical activity
relieving pain - gabapentin, pregabalin, amitriptyline, SNRIs
managing complications and restoring function
167
what is encephalitis
it is inflammation of the brain
168
what are symptoms of infective encephalitis
headache
fever
chills
aches in muscles
fatigue or weakness
stiff neck
confusion, agitation, hallucinations
seizures
irregular movements
loss of consciousness
169
how might infective encephalitis preset in an infant
bulging of the fontanelle
nausea and vomiting
stiffness affecting the whole body
poor feeding or not waking for feeding
irritability
170
what are symptoms of autoimmune encephalitis
changes in personality
memory loss
psychosis
hallucinations
seizures
changes in vision
sleep issues
muscle weakness
trouble waking
irregular movements
bladder and bowel dysfunction
171
what are causes of encephalitis
infections - viruses mc
autoimmune - can be triggered by tumours, infection or idiopathic
172
what are viruses that can cause encephalitis
HSV 1 and 2
EBV
VZV
enteroviruses
mosquito borne viruses - west nile
tick borne viruses
rabies virus
173
what are risk factors for developing encephalitis
age - extremes of age
weakened immune system - HIV/AIDS
geographical regions - mosquito/tic causes
autoimmune disease
smoking
174
what are complications of encephalitis
chronic disease
weakness or lack of muscle coordination
personality change
memory issues
hearing or vision changes
trouble with speech
175
how is encephalitis diagnosed
MIR/CT
CSF analysis
bloods, U+E, sputum sample
EEG
brain biopsy (rare)
body imaging
176
how is encephalitis managed
bed rest
fluids
antivirals- acyclovir, ganciclovir
steroids and IV Ig
177
what follow up therapy may be done for those with encephalitis
brain rehabilitation
physical therapy
occupational therapy
speech therapy
psychotherapy
178
what is the pathophysiology of seizures
imbalance between excitatory (glutamatergic) and inhibitory (GABAergic) neuronal signaling at the synaptic level resulting in a reduced threshold for neurotransmission
179
what are structural abnormalities which can increase risk of seizures
head injury
stroke
space occupying lesion - tumours
neurodegenerative disease
genetic syndromes - tuberous sclerosis or neurofibromatosis
180
what genetic disorders can affect neurological conduction and cause epilepsy
Dravet syndrome - mutation affecting sodium voltage gated channels
tuberous sclerosis
neurofibromatosis
181
what can change seizure threshold in patients with epilepsy/all patients
metabolic or electrolyte abnormalities - hyponatraemia, hypoglycaemia
central nervous system infection - encephalitis, meningitis
alcohol withdrawal
182
How does the international league against epilepsy classify seizures
according to onset
- focal
- generalised
- unknown
183
what is a focal seizure
it is a seizure that begins from a specific focus
184
what are features of a focal seizure
the patient remains conscious during
awareness may be impaired
may have motor or non motor onset
may spread to a generalised seizure
185
what is a general seizure
it affects both hemispheres
often motor and have characteristic abnormal movements
186
what are types of generalised seizures
tonic clonic
myoclonic
atonic
absence
187
what may be seen in a motor onset focal seizure
automatisms - repeated stereotypes movements
atonic movement
clonic movement
epileptic spasms
hyperkinetic
myoclonic
tonic
188
what what might be seen in a non motor onset focal seizure
autonomic behaviours
behaviour arrest
cognitive change
emotional change
sensory issues
189
what are key questions to ask in a seizure history
prodrome - any sensory aura pre fit
seizure itself - what happened
post ictal period - confusion/disorientation. how long for
190
what is Todds paralysis
post ictal limb weakness
191
what are dissociative seizures
type of functional neurological disorder which have distinctive features and lack abnormal electrical activity in the brain
192
what are features of functional seizures
long duration of seizure
quick recovery
retained awareness with bilateral arm movement
back arching
eye opening during events
dissociation
often very emotional after the event as well
193
what investigations should be done post epileptic fit
basic obs
capillary blood glucose
FBC, U+E, Bone profile, venous blood gas
CT/MRI head
EEG
video telemetry
patient videos
194
what are the criteria that needs to be met for someone to be diagnosed with epilepsy
two or more unprovoked seizures more than 24 hours apart
195
what does anti seizure medication primarily target
sodium channels or modulate GABAergic neurotransmission
196
what are complications of epilepsy
accidental self injury
status epilepticus
sudden death in epilepsy
197
whats something you always need to check with someone who has had a seizure (non medical)
DRIVING
first seizure = no driving for 6 months
epileptic seizure = no driving for 1 year
198
what is the general treatment for tonic clonic seizures
Sodium valproate
in women - lamotrigine or levetiracetam
199
what is the general treatment for partial seizures
lamotrigine or levetiracetam
200
what is the treatment for myoclonic seizures
sodium valproate
in women levetiracetam
201
what is the treatment for tonic and atonic seizures
sodium valproate
in women lamotrigine
202
what is the treatment for absence seizures
ethosuximide
203
what is carbamazepine used for
epilepsy - partial seizures with complex symptoms, general tonic clonic and mixed patterns
trigeminal neuralgia
acute manic and mixed bipolar disorder
204
what are the different antiseizure medications
sodium valproate
lamotrigine
levetiracetam
ethosuximide
carbamazepine
phenytoin
topiramate
205
how does sodium valproate work
it increases activity of GAMA which reduces activity in the brain
206
what are the side effects of sodium valproate
teratogenic
liver damage and hepatitis
hair loss
tremor
reduced fertility - not given to under 55 anymore
207
how is sodium valproate teratogenic
it causes neural tube defects and developmental delay
208
what is status epilepticus
it is a seizure lasting more than 5 minutes or multiple seizures without regaining consciousness in the interim
209
how do you treat status epilepticus
ABCDE approach
IV benzodiazepine repeat after 5-10 mins
IV phenytoin, levetiracetam or sodium valproate
general anaesthesia
210
what are the different benzodiazepine options in status epilepticus
IV lorazepam
rectal diazepam
buccal midazolam
211
what is essential tremor
it is a fine tremor that affects all voluntary muscles
- common in elderly
212
what are the features of essential tremor
fine tremor - 6-12 Hz
symmetrical
more prominent with voluntary movement
worse when tired, stressed or after caffeine
improved by alcohol
absent during sleep
213
what are differential diagnosis for essential tremor
parkinsons
multiple sclerosis
huntingtons
hyperthyroidism
fever
dopamine antagonists - antipsychotics
214
what is the treatment for benign essential tremor
no definitive treatment - not harmful so doesnt require treatment unless its causing functional or psychological issues
- propranolol
- primidone
215
what is an extradural haemotoma
it is an acute haemorrhage between the dura and the inner surface of the skull
216
what is an extradural haemotoma commonly caused by
skull trauma in the temporal region - fall, assault, sporting injury
217
what is the most common artery to rupture in extradural haemotomas
middle meningeal artery
218
what does an extradural haemotoma look like on CT
lemon shaped haemotoma
- bi-convex
- can get midline shift and brainstem herniation
219
what is the pathophysiology of an extradural haemotoma
as blood leaks into extradural space it begins to strip the dura mater away from the skull
as this grows it increases the intercranial pressure
this can cause midline shift and tentoria herniation
this can eventually lead to brainstem death
220
what is the typical history of someone with extradural haemotoma
headache
nausea and vomiting
confusion
loss of consciousness followed by a period of lucidity
progressive decreasing level of consciousness
221
what are the typical clinical findings in extradural haematoma
tenderness of the skull
confusion
reduced GCS
cranial nerve deficits
motor or sensory deficits of the upper and/or lower limbs
hyperreflexia and spasticity
upgoing planter reflex
cushings triad
222
what is cushings triad
it is a physiological response to raised intercranial pressure
bradycardia
hypertension
irregular breathing pattern
223
what investigations would be done on someone with suspected extradural haemorrhage
capillary blood glucose
ECG
FBC, U+E, CRP, coagulation, group and save
CT head
224
what is the initial management for an extradural haemorrhage
ABCDE
patients on anticoagulation will require reversal
prophylactic antibiotics
temporary anticonvulsant medication
IV MANNITOL to reduce intercranial pressure
225
what is the definitive management for extradural haematoma
conservative - small bleeds with minimal mass effects
burr hole craniotomy
trauma craniotomy
will need close observation during post operative period
226
what are complications of an extradural haematoma
infection
cerebral infarct
seizures
cognitive impairment
hemiparesis
hydrocephalus
brainstem injury
227
what clinical features in extradural haemorrhage are associated with poorer prognosis
low GCS at presentation
no history of lucid interval
pupil abnormalities
decerebrate rigidity
pre-existing brain injury
228
what is a febrile seizure
it is a convulsion that occurs in a febrile child (between 6 months and 5 years) and is not caused by a CNS infection
229
what are most febrile seizures caused by
viral infections
- human herpes virus 6 and influenza mc
230
what are risk factors for febrile seizures
family history of febrile seizures
high fever
viral infection
recent immunisation (rare)
231
what are the clinical features of febrile seizures
most occur within 24 hours of the child developing a fever
depends on whether it is a simple or complex febrile seizure
232
what is a simple febrile seizure
most common (80-85%)
the duration is less than 15 minutes
generalised seizure - normally tonic-clonic
occurs only once in 24 hours
233
what is a complex febrile seizure
15-20%
duration longer than 15 minutes
focal seizure
prolonged post ictal state
more than one seizure in 24 hours
234
what is febrile status epilepticus
a seizure lasting over 30 minutes or multiple seizures without recovery
235
what is the immediate management of a febrile seizure
most will have resolved by the time of presentation
same management of normal seizure in practice - ABCDE
236
what is the management of febrile seizure after the seizure has occurred
need to find the source of the fever !
parental education - explain the benign nature
give info on managing seizures and basic first aid
can give rescue medication - rescue midazolam
237
what are complications of febrile seizures
injury while seizing
aspiration while seizing
small increased risk of epilepsy compared to the general population (2% risk)
risk of recurrence
238
what is giant cell arteritis
it is a systemic vasculitis that can affect large and medium sized vessels
it commonly affects the extracranial branches of the carotid artery
239
what other condition in giant cell arteritis associated with
polymyalgia rheumatica
240
what are risk factors of giant cell arteritis
age: mostly round 50 years old
female sex
polymyalgia rheumatica
241
what are typical symptoms of giant cell arteritis
subacute onset unilateral headache typically affecting temporal region
tongue and jaw claudication
scalp tenderness
painless complete or partial vision loss in one/both eyes
diplopia
systemic symptoms - malaise, weight loss, fatigue, anorexia, depression
242
what are symptoms of polymyalgia rheumatica
bilateral shoulder stiffness
pelvic girdle pain
worse in morning
243
what are clinical findings seen in giant cell arteritis
scalp/temporal artery tenderness
reduced or absent temporal artery pulse
fundoscopy: oedema and pallor of the optic disc
auscultation: axillary, brachial and carotid bruits may be heard
asymmetrical blood pressure
244
what investigations are done for suspected giant cell arteritis
full blood count
CRP - typically increased
ESR - over 50mm/hour seen as significant
LFTs - alkaline phosphatase and transaminases may be slightly increased
temporal artery ultrasound - thickening of wall (halo sign)
temporal artery biopsy - multinucleated giant cells
245
what is the initial management of giant cell arteritis
Medical emergency !
urgent referral to rheumatologist
oral prednisolone - visual symptoms
IV methylprednisolone - vision loss
246
if someone has vision symptoms in GCA what is given to them
same day 60-100mg one off dose of prednisolone
247
what is given to someone with GCA that presents with vision loss
500mg IV methylprednisolone OD for 3 days
follow with prednisolone regime
248
what is given to someone who presents with GCA but doesnt have visual symptoms
a dose 40-60mg prednisolone per day
249
what is the on going management for GCA
gradually taper prednisolone down to 0 over a period of 12-18 months
patient has regular monitoring every 2-8 weeks after diagnosis
side effects of prednisolone are monitored
250
what are complications of temporal arteritis
irreversible vision loss
aortic dissections, aortic aneurysms, large artery stenosis
cardiovascular events - stroke/MI
complications from steroid treatment - bruising, diabetes, hypertension, osteoporosis
251
what is Guillian barre syndrome
it is an acute inflammatory neuropathy which primarily affects the peripheral nervous system
252
what is the cause of GBS
about 66% o cases involve previous history of either upper respiratory tract infection or gastrointestinal infection
- CMV, EBV, mycoplasma, campylobacter jejuni
253
what is the pathophysiology of GBS
it is considered an autoimmune condition
immune system activation against pathogens where molecular mimicry occurs and immune system attacks components of the peripheral nerves
this results in demyelination of motor and sensory peripheral nerves
254
what are the 4 subtypes of GBS
1. acute inflammatory demyelinating polyneuropathy - MC
2. acute motor axonal neuropathy - motor presentation
3. Acute motor sensory axonal neuropathy
4. Miller fisher syndrome - antibodies to GQIb ganglioside
255
what is the triad which is typically associated with Miller-Fisher GBS syndrome
ophthalmoplegia
ataxia
areflexia
256
what are the typical symptoms of GBS
rapidly progressive symmetrical weakness typically in an ascending pattern
paraesthesia in lower limbs and hands (tingling)
issues with balance or coordination
back pain or limb pain which can be worse at night
difficulties with vision
difficulties with speech, swallowing or chewing
autonomic dysfunction - palpitations, heart failure, bowel and bladder dysfunction
257
what typical examination findings are found in those with GBS
- symmetrical bilateral weakness ascending from the lower limbs first up to arms, trunk, bulbar and ocular muscles
- reduced sensation over area of weakness
- areflexia: absent/reduced reflexes
- autonomic dysfunction
258
what are differential diagnosis to think of or GBS
stroke
encephalitis
myasthenia gravis
polymyositis
myelopathy
botulism
259
what investigations should be done on someone with suspected GBS
clinical diagnosis
serial lung function tests - regular FVC
electrocardiogram
continuous BP monitoring
FBC, U+E, LFT, glucose, creatinine kinase
MRI spine to rule out myelopathy
nerve conduction studies
lumbar puncture - increased protein and normal cell count
260
what is the general management of GBS
serial lung function tests
supportive treatment - IV fluids
VTE prophylaxis
eye care
pressure sore screening and relief
physiotherapy
swallow assessment
plasma exchange
IV IG
261
when should someone with GBS be admitted to intensive care
requirement of significant respiratory support
rapidly progressing muscle weakness
rapidly fluctuating blood pressure/arrhythmias
swallow dysfunction
262
what are complications of GBS
respiratory compromise
VTE
infection
aspiration pneumonia
cardiac arrhythmias
ileus
long term disability
persistent neuropathic pain or fatigue
psychological trauma
263
what is looked at in the motor section of the GCS
6 - obeys commands
5 - localises to pain/moves to localised pain
4 - flexion withdrawal from pain
3 - flexes to pain
2 - extends away from pain
1 - none
264
what is looked at in the verbal portion of the GCS
5 - orientated to time place and person
4 - confused
3 - inappropriate words
2 - incomprehensible sounds
1 - none
265
what is looked at in the eye response portion of the GCS
4 - eyes open spontaneously
3 - eyes open to speech
2 - eyes open to pain
1 - none
266
what causes shingles
re activation of herpes zoster virus
267
who is more at risk of shingles
those with a weakened immune system
those over 50
other illness
trauma
those under stress
268
what are the symptoms of shingles
fever
chills
headache
fatigue
sensitivity to light
stomach upset
itching, tingling or burning in area of skin
redness on affected skin area
raised rash
fluid filled blisters
mild to severe pain
269
what is the shingles disease course
1. few days before rash there may be pain on the skin which is itching, burning, stabbing or shooting
2. raised rash occurs on one side of body only on one dermatome
3. within 3-4 days the rash develops into red, fluid filled painful open blisters
4. these blisters begin to dry out/scab over within about 10 days
5. scabs clear up about 2-3 weeks later
270
what is the most common long term complication of shingles
long term nerve pain - postherpetic neuralgia
271
how is shingles diagnosed
history and exam - clinical diagnosis
can scape/swab blisters and do lab tests
272
how is shingles treated
no cure
can use acyclovir, famciclovir or valacyclovir within 72 hours of first signs of shingles
ibuprofen can help with pain
prednisolone can be used if shingles affect eyes or other parts of the face
273
how many people who get shingles develop postherpetic neuralgia
over 10% of people
274
other than postherpetic neuralgia what other complications can occur because of shingles
numbness or itching
bacterial infection of the shingles rash
eye/ear inflammation
275
how is postherpetic neuralgia treated
lidocaine or capsaicin cream
antidepressants - amitriptyline
antiepileptics
nerve blocks
steroid injections
276
what is Horners syndrome
it is a characteristic triad of ptosis, anhidrosis and miosis caused by a lesions in the sympathetic chain supplying the eye
277
what three structures does the sympathetic nervous system innervate in the eye
1. dilator pupillae: involved in mydriasis or dilation of the pupil
2. superior tarsal muscle: aids in elevating the upper eyelid with the levator palpebrae superioris
3. sweat glands
278
how can you tell if Horners syndrome is caused by first, second or third order neurone lesions
by the sites affected by anhidrosis
Central/first order = anhidrosis of the face, arm and trunk
Anhidrosis of face = second order
No anhidrosis = third order
279
what causes central horners
a lesion in the first order neurone which spans from the hypothalamus to the T1 segment of the spinal cord
280
what can cause central horners
multiple sclerosis
brain tumours - Wallenbers syndrome
281
what causes second order horners
a lesion in the second order neurone which leaves the spinal cord and travels towards head via the cervical sympathetic chain, terminating at the superior central ganglion
282
what can cause second order horners syndrome
pancoast tumours - apical lung cancer
thyroid malignancies
iatrogenic
trauma causes
283
what causes third order horners syndrome (postganglionic)
lesion in the postganglionic neurone which arises from the superior cervical ganglion and travels along the internal carotid artery to the cavernous sinus where it joins the ophthalmic nerve V1
284
what are causes of postganglionic horners syndrome
carotid artery dissection
cavernous sinus thrombosis
cluster headaches - rare
285
what is congenital horners
this is very rare, when horners is present at birth
it can be due to birth trauma or due to an unknown cause
classic finding is iris heterochromia
286
what investigations are done for horners syndrome
clinical diagnosis + presence of anhidrosis
if unclear can be diagnosed using eye drops
- Apraclonidine given which reverses the pupillary constriction in horners as its an aloha 2 receptor agonist
- hydroxyamphetamine drops can be used to identify the location of the lesion as it will dilate a constricted horners pupil if there is an underlying preganglionic lesion
287
what is the management of horners
management depends on underlying cause
accurate and timely investigation aids in reducing morbidity and mortality
288
what is the genetic inheritance pattern of huntingtons
autosomal dominant
289
what is the genetic abnormality in huntingtons
a trinucleotide repeat disorder
genetic mutation in the HTT gene on chromosome 4
290
when do symptoms typically begin in huntingtons
30-50
291
what are other examples of trinucleotide repeat disorders
fragile X syndrome
spinocerebellar ataxia
myotonic dystrophy
friedrich ataxia
292
what is anticipation
it is when successive generations have
- earlier age of onset
- increased severity of disease
due to more repeats
293
how does huntingtons present
cognitive, psychiatric or mood problems
chorea - involuntary, random, abnormal
dystonia - abnormal muscle tone
rigidity
eye movement disorders
dysarthria - speech issues
dysphagia - swallowing issues
294
how is huntingtons diagnosed
genetic testing
- involves pre and post test counselling
295
how is huntingtons managed
there is currently no treatment options for slowing or stopping the progression of the disease
- counselling
- physiotherapy
- speech and language therapy
- tetrabenazine : helps chorea
- antidepressants
- advanced directives
- end of life care
296
what is the prognosis of huntingtons
life expectancy is about 10-20 years after the onset of symptoms
297
what is malaria caused by
plasmodium family of protozoan parasites
298
how is malaria spread
through bites from the female anopheles mosquitos
299
what are the types of malaria
plasmodium falciparum - most common and severe
plasmodium vivax
plasmodium ovale
plasmodium malariae
plasmodium knowlesi
300
what is the malaria life cycle
1. sporozoites from the mosquitos gut are injected into someone as they are bitten
2. they travel to the liver of the infected person
3. here they mature into merozoites which enter the blood and infect red blood cells
4. in the RBC the merozoites reproduce, and the RBC ruptures releasing them
5. this causes haemolytic anaemia
301
what malaria types can lie dormant in someones liver
P. vivax and P. ovale
302
what are symptoms of malaria
fever - up to 41oC with sweats and rigors
fatigue
myalgia
headache
nausea and vomiting
303
what signs on examination would you see in someone with malaria
pallor due to anaemia
hepatosplenomegaly
jaundice
304
how is malaria diagnosed
malaria blood film - in EDTA bottle
- three negative samples over three consecutive days is requires to exclude malaria
305
how is uncomplicated malaria managed
inform infectious diseases
- artemether with lumefantrine
- quinine plus doxycycline
- quinine plus clindamycin
- chloroquine (increasing resistance)
306
what is the management of severe or complicated malaria
admission to HDU or ICU
IV artesunate
IV quinine dihydrochloride
307
what are the complications of malaria (p.falciparum)
cerebral malaria
seizures
reduced consciousness
AKI
pulmonary oedema
disseminated intravascular coagulopathy
severe haemolytic anaemia
multi organ failure and death
308
what prophylaxis is given to those travelling to areas where malaria is present
- mosquito spray
- mosquito nets and barriers when sleeping
- seek medical advice
- take antimalarials: proguanil with atovaquone, doxycycline, mefloquine, chloroquine with proguanil
309
what is the treatment course of proguanil/atovaquone
taken two days before until 7 days after travel to an endemic area
310
what are the side effects of doxycycline
skin sensitivity to sun
diarrhoea
thrush
311
what is the treatment course of doxycycline for malaria prophylaxis
two days before until four weeks after travel to an endemic area
312
what are the side effects of mefloquine
psychiatric side effects - anxiety, depression, abnormal dreams
313
what is the treatment course of mefloquine
two weeks before until four weeks after travel to an endemic area
314
what is Merieres disease
it is a common cause of peripheral vertigo
315
what are the symptoms that Menieres presents with
vertigo
tinnitus
hearing loss
316
what is the cause of Menieres disease
not fully known
significant number of patients have endolymph build up in the membranous labyrinth
317
what are the typical symptoms of menieres disease
recurrent spontaneous vertigo lasting between 20 minutes to several hours
progressive or fluctuating hearing loss
tinnitus
aural fullness
318
what are risk factors of Menieres disease
family history of the disease
319
what are differential diagnosis for menieres disease
vestibular migraine
labyrinthitis
benign paroxysmal positional vertigo
stroke
vestibular schwannoma
320
what investigations are done to diagnose menieres disease
no specific investigations are required to diagnose the disease
- most patients receive formal audiology assessment
- MRI imaging to exclude abnormalities
321
what hearing loss is common in menieres
in early disease either low frequency sensorineural hearing loss or combined low and high frequency sensorineural hearing loss
later disease the hearing loss is at all frequencies
322
how is menieres disease diagnosed
- two or more spontaneous episodes of vertigo, with each episode lasting between 20 minutes to 12 hours
- audiological assessment demonstrating low to moderate sensorineural hearing loss on affected side
- aural fullness
323
what is the medical management of menieres disease
lifestyle and dietary changes - minimise salt and caffeine intake
betahistine can be used
vestibular sedatives (prochlorperazine) can be used in acute episodes of vertigo
vestibular rehabilitation
324
what is the surgical management of Menieres disease
involves the injection of medication intratympanically - either dexamethasone or methylprednisolone or gentamicin
intratympanic steroids
labyrinthectomy
vestibular neurotomy
325
what are complications of Menieres disease
intermittent flares
long term intermittent tinnitus
long term hearing loss
326
what is meningitis
it is inflammation o the meninges- the outer membranes that cover the brain and spinal cord
327
what can meningitis be caused by
bacteria
viruses
fungo
leukaemia and lymphoma
autoimmune disease
drugs
328
what is the common causes of bacterial meningitis
in neonates - Group B streptococcus, E.coli, Strep.pneumoniae, listeria monocytogenes
in children and adults - Neisseria meningitidis, strep. pneumoniae and haem. influenzae B
329
what is aseptic meningitis
this is when the CSF has white cells on microscopy but the gram stain is negative
- viral
- fungal
330
what are the common causes of viral meningitis
enteroviruses - echovirus, coxackievirus
mumps
HSV
herpes zoster
HIV
measles
influenza
331
what are causes of fungal meningitis
cryptococcus
332
what are clinical features of meningitis
neck stiffness
non blanching rash in meningococcal disease
photophobia
leg pain
altered mental state
ever
seizures
general flu like illness
bulging fontanelle in infants
Kernig and Brudzinski sign
333
what is Kernigs sign
pain and resistance on passive knee extension with hips fully flexed
334
what is brudzinskis sign
knees and hips flex on bending the head forward
335
what investigations should be done for suspected meningitis
vital signs
blood sugar
FBC, U+E, CRP, blood culture
CT
lumbar puncture
336
what are contraindications to performing a lumbar puncture in meningitis
raised intercranial pressure - reduced or fluctuating consciousness, bradycardia and hypertension, focal neurological signs, abnormal posturing, abnormal pupil reflexes, papilloedema, bulging fontanelle
convulsions
coagulation abnormalities
shock
extensive purpura
337
how do you treat bacterial meningitis in hospital
children three months and older - IV ceftriaxone
children under 3 months - IV ceftriaxone + amoxicillin/ampicillin (covers listeria)
338
how do you treat bacterial meningitis in primary care
urgent transfer to hospital
IM/IV benzylpenicillin if meningococcal sepsis is suspected
339
how do you treat contacts of someone with bacterial meningitis
ciprofloxacin/rifampicin within 24 hours
340
what is the treatment of viral meningitis
supportive therapy
if concerns of encephalitis then IV aciclovir
341
what are the acute complications of meningitis
sepsis
septic shock
disseminated intravascular coagulation
coma
cerebral oedema
raised ICP
subdural effusions
SIADH
seizures
peripheral gangrene
death
342
what are complications of bacterial meningitis
30-50% survivors have permanent neurological sequelae
- hearing loss
- seizures
- motor deficit
- cognitive impairment
- hydrocephalus
- visual disturbance
343
what are complications of viral meningitis
usually complete resolution within 10 days but you can get
- headaches
- cognitive and psychological issues
344
what are the four main types of migraine
migraine with aura
migraine without aura
silent migraine
hemiplegic migraine
345
what are the five stages of a migraine
premonitory or prodromal stage
aura
headache
resolution
postdromal or recovery stage
346
what are the typical symptoms of a migraine
unilateral
- moderate to severe in intensity
- pounding or throbbing
- photophobia
- phonophobia
- osmophobia - discomfort of strong smells
- aura
- nausea and vomiting
347
what is an aura
it is a sensation change that occurs pre migraine
it can affect vision, sensation and language
348
what are examples of aura in migraine
sparks in the vision
blurred vision
lines across the vision
loss of visual fields
dysphagia
tingling or numbness
349
what is a hemiplegic migraine
where you get unilateral limb weakness (can also include ataxia and LOC) associated with the migraine
350
what is familial hemiplegic migraine
it is an autosomal dominant genetic condition characterised by hemiplegic migraines which run in families
351
what are migraine triggers
stress
bright lights
strong smells
certain foods - chocolate, cheese, caffeine
dehydration
menstruation
disrupted sleep
trauma
352
what is the acute management of a migraine
dark, quiet room, sleeping
NSAIDS
paracetamol
triptans
antiemetics - metoclopramide or prochlorperazine
353
what are triptans used for in migraines
they are used to abort migraines when they start to develop (5HT receptor agonists)
- taken as soon as the headache starts
354
what are the main contraindications o triptan use
relate to risks associated with vasoconstriction
- hypertension
- CAD
- previous stroke or TIA
- MI
355
what prophylaxis can be used for migraines
propranolol - not in asthmatics
amitriptyline
topiramate - teratogenic and very effective contraception is needed
356
what specialist medications can be used in migraine prophylaxis if regular prophylaxis doesnt work
pizotifen
candesartan
sodium valproate
monoclonal antibodies - erenumab
357
when might prophylactic triptans be used in migraine
for menstrual migraines
- taken two days before until three days after the start of menstruation
358
what is mononeuropathy
it is damage to a single nerve, which results in the loss of movement, sensation or other function of that nerve
- damage that occurs outside the brain and spinal cord
359
what can cause mononeuropathy
most common cause is injury/trauma
systemic disease can damage isolated nerves
360
what are common forms of mononeuropathy
axillary nerve dysfunction
common peroneal nerve dysfunction
carpal tunnel syndrome
cranial mononeuropathy III,IV
cranial mononeuropathy VI
cranial mononeuropathy VII
femoral nerve dysfunction
radial nerve dysfunction
sciatic nerve dysfunction
ulnar nerve dysfunction
361
what does axillary nerve dysfunction cause
loss of movement or sensation in the shoulder
362
what does common peroneal nerve dysfunction cause
foot drop
weak eversion
absent ankle jerk reflex
- can be caused by fibular head fracture (L4-S1)
363
what causes carpal tunnel syndrome
median nerve dysfunction (C6-T1)
due to repetitive overuse, hypothyroidism, acromegaly, rheumatoid arthritis, pregnancy
364
what are symptoms of median nerve palsy
numbness, tingling, weakness in hand and fingers
wake and shake
thenar muscle wasting
365
what does cranial mononeuropathy VI cause
double vision
366
what does cranial mononeuropathy VII cause
facial paralysis
367
what does femoral nerve dysfunction cause
loss of movement or sensation in part of the leg
368
what does radial nerve (C5-T1) dysfunction cause
Wrist drop (saturday night palsy)
week dorsiflexion
change in sensation over back of hand and arm
369
what does sciatic nerve dysfunction cause
problems with the muscles of the back of the knee and the lower leg, and sensation to the back of the thigh, part of the lower leg and the sole of the foot
370
what does ulnar nerve (C8-T1) dysfunction cause
cubital tunnel syndrome
- numbness, tingling, weakness of outer and underside of the arm, palm, ring and little fingers
- Claw hand
371
what are symptoms of mononeuropathies
loss of sensation
paralysis
tingling, burning, pain, abnormal sensations
weakness
372
what investigations can be done for a mononeuropathy
electromyograms
nerve conduction studies
nerve ultrasound
X-ray, MRI, CT
bloods - glucose
nerve biopsy
CSF examination
skin biopsy
373
what are treatment options of mononeuropathy
painkillers
antidepressants - amitriptyline
steroid injections
surgery to relieve the pressure on the nerve
physical therapy to maintain muscle strength
braces, splints
TENS to improve nerve pain
374
what are possible complications of mononeuropathy
deformity - loss of tissue mass
medicine side effects
repeated or unnoticed injury to the affected area due to lack of sensation
375
what is motor neurone disease
it is a term that encompasses a variety of specific diseases affecting the motor nerves
it is progressive and eventually fatal condition where motor neurones stop functioning
376
what are the different types of motor neurone disease
amyotrophic lateral sclerosis (ALS)
progressive bulbar palsy
progressive muscular atrophy
primary lateral sclerosis
377
how does ALS present
begins with muscle twitching and weakness in an arm or leg, trouble swallowing and slurred speech
- tripping and falling
- weakness in legs, feet or ankles
- hand weakness or clumsiness
- slurred speech or trouble swallowing
- muscle cramps and twitching
- thinking or behavioural change
378
what are risk factors for developing ALS
genetics - 10%
age
sex - men>women
smoking
environmental toxin exposure - lead
379
what are the symptoms of progressive bulbar palsy
wasting of tongue
weakness/stiffness of tongue
difficulty speaking
difficulty swallowing
problems with the thickness/amount of saliva
380
how does motor neurone disease often present
late middle aged man
insidious progressive weakness or the muscles though out the body affecting the limbs, trunk, face and speech
weakness often first noticed in the upper limbs
increased fatigue when exercising
clumsiness
dropping things or tripping over
dysarthria
381
what are signs of motor neurone disease
have both upper and lower signs
lower - muscle wasting, reduced tone, fasciculations, reduced reflexes
upper - increased tone or spasticity, brink reflexes, upgoing plantar reflex
382
how is motor neurone disease managed
no effective treatment in halting/reversing the disease
- Riluzole can help slow progression and extend survival by several months in ALS
- non invasive ventilation
- symptom control: baclofen for muscle spasticity and antimuscarinics for saliva
- benzodiazepines
- advanced directives
- end of life care
383
what is multiple sclerosis
autoimmune inflammatory disease of the CNS which is characterised by demylination
384
what is the pathophysiology of MS
thought to be mediated by T cell activation although underlying cause is unclear
- attacks central nervous system causing demyelination and eventually cell death
385
what are the four main groups of MS
relapsing remitting
secondary progressive
primary progressive
clinically isolated syndrome
386
what is clinically isolated syndrome in MS
this is the first episode of demyelination and neurological signs that cant be explained by something else
patients with this may never go on to having MS but they are more likely to than the general population
387
what is relapsing remitting MS
this is when there are unpredictable attacks of neurological dysfunction lasting over 24 hours followed by relief of symptoms
- patients may not fully return to previous baseline
388
what is secondary progressive MS
this is when MS initially presents as relapsing remitting and then later steadily declines and progressively without remission in between attacks
389
what is primary progressive MS
this is when there is a steady, progressive worsening of disease severity from the onset without remission
390
what are risk factors for developing MS
family history
sex F>M
age - between 25-35
other co existing auto immune disease
smoking
previous EBV infection
latitude of habitat
vitamin D deficiency
391
what are common symptoms of MS
limb weakness
limb tingling/numbness
cerebellar symptoms
fatigue
nystagmus
optic neuritis
diplopia
dysphagia
slurring/stuttering speech
spasm
urinary incontinence
retention
constipation/diarrhoea
392
what two phenomina are characteristic to MS
Uhthoffs phenomenon
Lhermittes phenomenon
393
what is Uhthoffs phenomenon
this is when there is worsening of symptoms on exercise/in warm environments such as the bath
394
what is Lhermittes phenomenon
this is a lightning shock sensation down the spine on flexion of the neck secondary to cervical cord plaque formation
395
what is often the first sign of MS
optic neuritis
396
what is optic neuritis
it is demyelination of the optic nerve and presents with unilateral reduced vision developing over hours
397
what are key features of optic neuritis
central scotoma - enlarged central blind spot
pain with eye movement
impaired colour vision
relative afferent pupillary defect -
398
what is a relative afferent pupillary defect
this is when the pupil of the affected eye constricts more when shining light in the contralateral eye then when shining it in the affected eye
399
what other eye movement abnormalities can you get with MS
- lesions affecting CN III, IV, VI can cause double vision and nystagmus
- internuclear ophthalmoplegia caused by a lesion in the medial longitudinal fasciculus causing impaired adduction on the ipsilateral eye and nystagmus in the contralateral eye
- CN VI lesion causes conjugate lateral gaze disorder
400
what focal weaknesses may present in MS
incontinence
horners syndrome
facial nerve palsy
limb paralysis
401
what focal sensory symptoms may be present in multiple sclerosis
trigeminal neuralgia
numbness
paraesthesia (pins and needles)
Lhermittes sign
402
what cerebellar signs may be present in MS
nystagmus
intention tremor
scanning dysarthria
403
what investigations are done to diagnose MS
FBC, CRP, U+E, LFT, calcium, TFT, vit B12, HIV
MRI of the brain and spinal cord
lumbar puncture with CSF analysis
404
what is the typical CSF finding in someone with MS
high protein content
oligoclonal bands of immunoglobulin
405
what is the McDonald criteria for MS
for a diagnoses the symptoms need to be disseminated in both time and space
406
what is the management of an acute MS attack
if symptoms are severe - high dose steroid therapy with methylprednisolone (500mg oral 5 days)
plasmapheresis
look to see if there is an underlying cause i.e infection
407
what is the long term management of MS
Beta interferon and glatiramer acetate - DMARDS which reduce relapses in RRMS
other DMARDS - dimethyl fumarate, cladribine
moAbs - alemtuzumab, natalizumab
exercise
physiotherapy
neuropathic pain - amitriptyline or gabapentin
spasticity - gabapentin
urge incontinence - antimuscarinics: solifenacin
408
what are complications of MS
majority of those diagnosed with MS become unemployed within 15 years
depression (50%)
cognitive deficits
reduced life expectancy
409
what is muscular dystrophy
it is a group of genetic diseases associated with progressive weakness and loss of muscle mass
- occurs with mutations in genes responsible for the production of proteins key to healthy muscle development
410
what are the types of muscular dystrophy
duchenne muscular dystrophy
becker muscular dystrophy
emery dreifuss muscular dystrophy
limb girdle muscular dystrophy
facioscapulohumeral muscular dystrophy
411
what are the two most common types of muscular dystrophy
Duchennes muscular dystrophy
Beckers muscular dystrophy
412
what is the genetic inheritance of duchennes muscular dystrophy and becker muscular dystrophy
x linked recessive conditions
413
what are typical presenting features of muscular dystrophy
progressive weakness - starting proximally and moving distally with the lower limbs being affected before upper limbs
delayed motor milestones
waddling gait
faltering growth
fatigue
intellectual impairment
behavioural issues
leg pain
414
what investigation should be done in children who cannot walk by 18 months of age
their creatinine kinase levels
415
at what age to people with duchennes muscular dystrophy become wheelchair users
typically about 12 years of age
416
what are clinical findings in patients with muscular dystrophy
weakness - typically the proximal and distal leg muscles
calf pseudohypertrophy - accumulation of connective tissue and fat replacing muscle
waddling gait
tip toe walking
hyporeflexia or areflexia
loss of arches of the feet
difficulty of inability to squat
417
what are clinical findings associated with facioscapulohumeral MD
winging of the scapula
upper extremity weakness
facial weakness
418
what are clinical findings associated with oculopharyngeal MD
dysarthria
dysphagia
419
what are clinical findings associated with emery-dreifuss MD
contractures affecting the ankles, elbows and neck extension
increased tone in the lumbar and paravertebral muscles
cardiac abnormalities
420
what investigations are done in suspected muscular dystrophy
serum creatinine kinase - increases with more muscle damage
genetic analysis
muscle biopsy
clinical exam
electromyography
ECG and echo
lung function testing
421
what is the early management of muscular dystrophy - when the child is still walking
corticosteroids - prolong walking by 6-24 months
vitamin D and calcium supplements
physiotherapy
orthoses - stabilise the knee, ankle and foot
serial casting of the ankles - prevent shortening of the achilles tendon
422
what is the later stage management of muscular dystrophy
wheelchair aid
counselling
orthopaedic input - orthotics and surgery
cardiac and respiratory surveillance
advanced planning and palliative care
423
what is Gowers sign
this is a technique children with proximal muscle weakness use to stand up
- get onto their hands and knees
- then push their hips up and back
- they shift their weight back and put their hands onto their knees
- whilst keeping their legs straight they then walk their hands up their legs to stand up
424
what is the genetic abnormality that causes duchennes muscular dystrophy
defective dystrophin gene which normally holds muscle together at a cellular level
425
what is beckers muscular dystrophy
it is similar to duchennes however the dystrophin gene is less severely affected and maintains some function
- clinical course is less predictable and symptoms only appear between 8-12
426
what are contractures (seen in emery-dreifuss MD)
they are the shortening of muscles and tendons that restrict the range of movement in limbs
- patients suffer with progressive weakness and washing of muscles
427
what is myasthenia gravis
it is an autoimmune condition affecting the neuromuscular junction
causes muscle weakness and progressively worsens with activity and improves with rest
428
what other condition does myasthenia gravis have a strong link with
thymomas - thymus gland tumours
429
what is the pathophysiology of myasthenia gravis
In the NMJ acetylcholine is released and binds to acetylcholine receptors on the post synaptic membrane stimulating muscle contraction
in MG there are acetylcholine receptor antibodies which bind to the post synaptic receptors, blocking them and preventing stimulation, and muscle contraction
430
what is the link between exercise and myasthenia gravis
the more activity the more acetylcholine receptors are used, and therefore the more they become blocked
- MG worsens with exercise
431
other than blocking postsynaptic preceptors what else do the autoantibodies do in MG
they activate the compliment system within the NMJ leading to cell damage at the postsynaptic membrane and further worsening symptoms
432
what two other antibodies can cause myasthenia gravis other than acetylcholine receptor antibodies
muscle specific kinase antibodies
low density lipoprotein receptor related protein 4 (LRP4)
433
how does myasthenia gravis present
difficulty climbing the stairs, standing from a seat or raising their hands above their head
extraocular muscle weakness causing dipolpia
eyelid weakness causing ptosis
weakness in facial movements
difficulty with swallowing
fatigue in the jaw when chewing
slurred speech
434
what muscles are most affected in myasthenia gravis
proximal muscles of the limbs and small muscles of the head and neck
435
how do you elicit muscle weakness in people with myasthenia gravis
repeated blinking
prolonged upward gazing
repeated abduction of one arm 20 times will result in unilateral weakness
436
what investigations are done in someone with suspected myasthenia gravis
AchR antibodies
MuSK antibodies
LRP4 antibodies
CT/MRI of the thymus to look for thymoma
edrophonium test
437
what is the edrophonium test
patient is given IV edrophonium chloride (neostigmine) which blocks cholinesterase enzymes and reduces acetylcholine breakdown. As a result there is temporary relied of weakness
438
How do you treat myasthenia gravis
pyridostigmine - cholinesterase inhibitor
immunosuppression - prednisolone
thymectomy - even without thymoma
rituximab - monoclonal antibody against B cells
439
what is a myasthenic crisis
it is a potentially life threatening complication of myasthenia gravis
it is an acute worsening to symptoms often triggered by another illness
respiratory muscle weakness can lead to respiratory failure
440
what is the treatment for a myasthenic crisis
non invasive ventilation or mechanical ventilation
IV immunoglobulins
plasmapheresis
441
what is narcolepsy
it is a sleep disorder that makes people very drowsy during the day - suddenly fall asleep or loss of muscle tone (cataplexy)
442
what are the two types of narcolepsy
type 1 - with cataplexy
type 2 - without cataplexy
443
what are symptoms of narcolepsy
excessive daytime sleepiness - can fall asleep without warning
sudden loss of muscle tone - can cause slurred speech or complete weakness of most muscles
triggered by intense emotions
sleep paralysis
hallucinations
changes in REM sleep
444
what is the cause of narcolepsy
not fully known
people with type 1 narcolepsy have low levels of hypocretin (orexin) which is a chemical in the brain that helps control being awake and when you enter REM sleep
445
what is the link between narcolepsy an REM sleep
in narcolepsy you may suddenly enter REM sleep without going through non REM sleep
446
what are risk factors for narcolepsy
age - between 10-30
family history - 20-40 times higher if a close family has it
447
how is narcolepsy diagnosed
clinical history - sleep history (epworth sleepiness scale)
looking at sleep records
a sleep study - polysomnography
multiple sleep latency test
genetic tests and lumbar puncture
448
what is the treatment for narcolepsy
there is no cure - medication and lifestyle changes
- stimulants: modafinil or armodafinil/ solriamfetol (new)
- SNRI/SSRI
- tricyclic antidepressants
sodium oxybate and oxybate salts: relieve cataplexy
449
what is neurofibromatosis
it is a genetic condition that causes nerve tumours to develop throughout the nervous system
- benign but can cause neurological and structural issues
450
what are the two types of Neurofibromatosis
type 1 - chromosome 17, Auto dominant
type 2 - chromosome 22, Auto dominant
451
what does the neurofibromatosis type 1 gene code for
a protein called neurofibromin which is a tumour suppressor protein
452
what are the features of neurofibromatosis type 1
CRABBING
Cafe au lait spots (>15mm diameter in adults)
Relative with NF1
Axillary or inguinal freckling
Bony dysplasia such as Bowing of long bone/sphenoid wing dysplasia
Iris hamartomas - yellow/brown spots on iris
Neurofibromas
Glioma of the optic pathway
453
where can neurofibromas be seen
they can be seen on the skin - skin coloured, raised nodules or papules with a smooth regular surface
- two or more are significant
- one plexiform neurofibroma is significant (larger, irregular, complex)
454
what is the management of neurofibromatosis
no treatment
- managing symptoms, monitoring and treating complications
455
what are complications of neurofibromatosis
migraines
epilepsy
renal artery stenosis - hypertension
learning disability
behavioural issues
vision less - secondary to optic nerve gliomas
malignant peripheral nerve sheath tumours
gastrointestinal stromal tumours
brain tumours
spinal cord tumours
increased risk of cancer
456
what does neurofibromatosis type 2 gene code for
merlin which is a tumour suppressor protein which is important in schwann cells
- mutations lead to schwannomas
457
how does a temporal lobe seizure present
flushing sweating, pallor, butterflies in stomach
hallucinations
feeling intense emotions
stereotypic behaviours - chewing, lip smacking, swallowing, scratching, fumbling
458
how does a frontal lobe seizure present
motor symptoms
- arm/hand stiffness or rigidity and abnormal posturing
- abnormal movements of the arm
- head turning or abnormal posturing
459
how does a parietal lobe seizure present
sensory symptoms - warmth, paraesthesia, pain up one side of the body
460
how does an occipital lobe seizure present
visual disturbance - lashes, floaters, vision loss, change in colour vision
461
How does primary lateral sclerosis present (MND)
movements becoming slow
pseudobulbar effect
- affects the upper motor neurones of the arms, face and legs
462
how does progressive muscular atrophy present (MND)
muscle wasting, clumsy hand movements, fasciculations, muscle cramps
- progressive degeneration of only the lower motor neurones
463
who are at the highest risk of carpal tunnel syndrome
females between the age of 40-60
464
what are risk factors for carpal tunnel
smoking
over 30 years old
high BMI
smoking
acromegaly
hypothyroidism
rheumatoid arthritis
diabetes
pregnancy
occupation
465
what is normal pressure hydrocephalus
this is when CSF slowly builds up within your skull putting pressure on the brain
there is a slow build up meaning while pressure increases it remains within normal range
466
what are symptoms of normal pressure hydrocephalus
symptoms start gradually and develop over about 6 months
Triad:
Gait issues, Urinary incontinence, Cognitive issues
467
what gait issues are present in normal pressure hydrocephalus
trouble lifting the feet
steps are shorter and unsteady
freeze/walk uncertainty
rotate toes out as they walk
468
what cognitive difficulties may be present in normal pressure hydrocephalus
mental or physical slowness
memory issues
executive dysfunction
emotional change
469
what are causes of primary normal pressure hydrocephalus
idiopathic - think its to do with age
may have a connection to lewy body dementia or Alzheimer's
470
what are causes of secondary normal pressure hydrocephalus
brain aneurysm
intercranial haemorrhage
brain tumours
encephalitis or meningitis
stroke
traumatic brain injury
471
how do you diagnose normal pressure hydrocephalus
history and exam
MRI and lumbar puncture
472
how do you treat normal pressure hydrocephalus
primary - use a shunt. They drill a hole in the skull and the shunt runs down under the skin and drains into the abdomen or the chest
secondary - need to treat underlying cause
473
what are side effects of a shunt to treat normal pressure hydrocephalus
infection
shunt malfunction or failure
position shit of the valve/catheters
too much CSF draining out the shunt
474
is normal pressure hydrocephalus reversible
if it is caught and treated early then it is reversible however delay can lead to more permanent brain damage due to prolonged pressure and compression of the brain
475
what are causes of headache
tension headache
migraines
cluster headaches
secondary headaches - drug induced
sinusitis
GCA
glaucoma
intercranial haemorrhage
subarachnoid haemorrhage
venous sinus thrombosis
hormonal headache
cervical spondylitis
trigeminal neuralgia
raised ICP
tumour
meningitis
encephalitis
abscess
pre eclampsia
476
what are red flags in a headache
fever, photophobia or neck stiffness
new neurological symptoms
visual disturbance
sudden onset occipital headache
postural, worse on standing, lying or bending over
vomiting
history of trauma
history of cancer
pregnancy
477
what is a tension headache
it is common type of headache that typically causes a mild ache or pressure in a band like pattern around the head
- develop and resolve gradually and dont produce visual change
478
what may tension headaches be associated with
stress
depression
alcohol
skipping meals
dehydration
479
what are symptoms of tension headache
Dull, aching head pain.
Feeling of tightness or pressure across the forehead or on the sides and back of the head.
Tenderness in the scalp, neck and shoulder muscles.
480
what is the management for tension headache
simple analgesia - ibuprofen or paracetamol
amitriptyline is offered first line in chronic or frequent headaches
481
what are causes of secondary headache
infections
obstructive sleep apnoea
pre elampsia
head injury
carbon monoxide poisoning
482
what is sinusitis
it is inflammation in the paranasal sinuses which typically causes pain and pressure following a viral upper resp. tract infection
483
what is hormonal headache
it is a headache related to low oestrogen
- similar features to migraine
- occur two days before and the first three days of the menstrual period, in the perimenopausal period and in early pregnancy
484
what is the treatment for hormonal headaches
triptans and NSAIDS (mefenamic acid)
485
what is cervical spondylosis
it is a common condition caused by degenerative changes in the cervical spine. It usually causes neck pain made worse by movement and headaches
486
what are cluster headaches
severe unilateral headaches centered around the eye which come on in clusters of attacks
487
how long does a cluster headache last for
between 15 minutes and 3 hours
488
what are symptoms of cluster headaches
severe pain - suicide headaches
read swollen and watering eye
pupil constriction (miosis)
eyelid drooping (ptosis)
nasal discharge
facial sweating
489
what is the treatment for an acute cluster headache attack
Triptans - subcut or intranasal sumatriptan
high flow 100% oxygen - reservoir mask
490
what is used for cluster headache prophylaxis
verapamil (calcium channel blocker)
occipital nerve block
prednisolone
lithium
491
what is parkinson's disease
it is a neurodegenerative disorder occurring due to the loss of dopaminergic cells from the substantia nigra
492
what is the aetiology of parkinsons (who is it found in)
typically presents in those above 55 years old
higher prevalence in men
493
what is the pathophysiology of parkinsons disease
- in parkinsons there is a progressive loss of dopaminergic neurones
- the motor signs of parkinsons occur due to the loss of dopaminergic neurons in the nigrostriatal pathway
- physiologically dopamine acts to upregulate the thalamus and the motor cortex via the direct pathway which results in increased movement
- loss of neurones results in a reduction in the action of the direct pathway and increase in the antagonistic indirect pathway which restricts movement
494
what are the symptoms of parkingsons
motor symptoms: bradykinesia, stiffness, resting tremor, instability, falls
constipation
lightheadedness
excess salivation or sweating
urinary and sexual dysfunction
mood symptoms: low mood, anxiety, sleepiness
sleep disturbance
anosmia or reduced sense of smell
micrographia
495
what are the typical clinical findings in someone with parkinsons
bradykinesia
tremor: pill rolling
rigidity (cog wheel)
abnormal gait: shuffling
postural instability
hypomimia - decreased facial activity
confusion
reduced dexterity
496
what are differential diagnosis for parkinsonism
lewy body dementia
multiple system atrophy
progressive supranuclear palsy
vascular parkinsonism
drug induced parkinsonism
497
what drugs can cause drug induced parkinsonism
antipsychotics
anti emetics - prochlorperazine or metoclopramide
498
what investigations do you do in someone with suspected parkinsons disease
bedside investigations and cognitive assessment
FBC, U+E, LFT, CRP, Glucose, TFT, calcium, B12
Dopamine active transporter scans
499
what medical management can be used for parkinsons
Levodopa
Monoamine oxidase B inhibitors
dopamine agonists
catechol-o-methyl transferase inhibitors - always taken with levodopa
500
what is often used with levodopa to extend its life
it is often used with carbidopa which is a dopamine decarboxylase inhibitor to reduce metabolism of levodopa
501
what else can be done for parkinsons other than medication
physiotherapy
speech and language therapy
deep brain stimulation
502
what are complications of parkinsons
increased fall risk
increased risk of dementia with lewy bodies
hallucinations
aspiration pneumonia
503
what type of tremor is present in parkinsons
pill rolling, worse on one side, 4-6 hz
504
what is the main side effect of levodopa
dyskinesia
- dystonia
- chorea
- athetosis
505
what can be used to manage the dyskinesia associated with levodopa
amantadine - glutamate antagonist
506
what is the main side effect of dopamine agonists
pulmonary fibrosis with prolonged use
507
what are examples of dopamine agonists
bromocriptine
pergolide
cabergoline
508
what are examples of monoamine oxidase B inhibitors
selegiline
rasagiline
509
what are symptoms of a motor nerve peripheral neuropathy
muscle weakness
cramps
muscle twitching
510
what are symptoms of a sensory nerve peripheral neuropathy
numbness or tingling
trouble walking or keeping your balance
issues fastening buttons
issues with sensing pain or changes in temperature
injury of sensory nerves may cause pain
511
what can autonomic nerve peripheral neuropathy cause
excessive sweating
changes in blood pressure
inability to tolerate heat
GI symptoms
512
what are causes of peripheral neuropathy
injury - compress, crush or cut nerves
medical conditions - diabetes, guillian barrre, carpal tunnel
autoimmune disease - lupus, RA, sjogren syndrome
513
how are peripheral neuropathies diagnosed
history and exam
electromyography
nerve conduction studies
MRI
US
glucose monitoring
514
what treatment may be required for a peripheral neuropathy
medication - aspirin, ibuprofen, amitriptyline
physical therapy
surgery - nerve repair or graft
braces or splints
electrical stimulators
exercise
515
what is radiculopathy
it describes a range of symptoms which is produced by the pinching of a nerve root in the spinal column
516
what is a lumbar radiculopathy
it is sciatica
517
what are different types of radiculopathy
cervical - affects arms and hands most commonly
thoracic - pain and numbness that wraps round to the front of your body
lumbar - sciatica
518
what are symptoms of radiculopathy
sharp pain in the back, arms, legs or shoulders that worsen with certain activities
weakness or loss of reflexes in the arms or legs
numbness of the skin, pins and needles or other abnormal sensations
519
what are causes of radiculopathies
herniated disc
bone spurs - extra bone growth
ossification of spinal ligaments
spinal infection
cancer/non cancer growths
520
how is radiculopathy diagnosed
physical exam and history
imaging - X-ray, CT, MRI
nerve conduction studies
521
how is radiculopathy treated
NSAIDs, opioids, muscle relaxants
weight loss
physical therapy
steroid injections
surgery - releases the pressure on the nerve root by widening the foramina it exits out of
522
what is the difference between a radiculopathy and myelopathy
radiculopathy is a temporary issue caused by a pinched nerve root near your spine
myelopathy is compression of your spinal cord caused by trauma, tumour, generation or infection
523
what is the acute treatment for a seizure
insert nasogastric tube into the nose
provide supplemental oxygen
ensure IV access
attach O2 sats and watch for hypoxia
524
whats the best benzodiazepine to use in status epilepticus
IV midazolam as its short acting
- test dose of 1ml
525
what is mannitol
an osmotic diuretic and is used short term to treat rising ICP
526
what is given before an operation for patients undergoing surgery for intercranial tumours
dexamethasone
- 4mg QDS or 8mg BD
527
what can be used in cranial diabetes insipidus to help prevent sx
desmopressin
528
what is often used to treat radicular pain
gabapentin and pregabelin
529
what is first line treatment for trigeminal neuralgia
carbamazepine
can also be used in combination with amitriptyline
530
when might intubation/ventilation be required in neurosurgery
1. agitated and CNS imaging required
2. in circumstances of acute neurologic deterioration
3. persisting poor level of consciousness
4. where the patient is at risk or has resp. failure
5. status epilepticus
6. significant intra-operative brain swelling
531
where is the pain/numbness felt in a patient with carpal tunnel syndrome
thumb, index and middle finger and the palm
532
what will a patient with vertebral pain syndrome complain of
localising aching pain with limited radiation
stiffness
restriction of movement
worse with activity
abscence of associated neurological sx
533
what are causes of vertebral pain syndrome
most commonly just due to wear and tear/degenerative changes
TB
bacterial discitis
osteomyelitis
osteoporotic fractures
metastatic disease
534
what two nerve roots are commonly affected in cervical radiculopathy
C6 and 7
535
what to nerve roots are commonly affected in lumbar radiculopathy
L5 and S1
536
how will patients with spinal claudication present
bilateral radiating leg pain or paraesthesia that comes on with walking
symptoms often relieved by sitting forward
537
what are red flag symptoms of spinal claudication
constant bilateral leg pain
perineal numbness
foot weakness
urinary incontinence
538
what is the c6 myotome, dermatome and reflex
myotome - thumb
dermatome - biceps
reflex - biceps jerk
539
what is the c7 myotome, dermatome and reflex
myotome - middle finger
dermatome - triceps
reflex - triceps reflex
540
what is the L5 myotome, dermatome and reflex
myotome - dorsum of the foot/big toe
dermatome - dorsiflexion (stand on heels)
reflex
541
what does L5 radiculopathy cause
foot drop and weak inversion
present ankle reflex
542
what is the S1 myotome, dermatome and reflex
myotome - ankle, lateral aspect of foot/sole of foot/ little toe
dermatome - plantar flexion (stand on toes)
reflex - ankle jerk
543
what will be found on examination if someone has raised ICP
drowsiness
papilloedema
paralytic squint
fixed dilated pupil
reduced levels of consciousness
544
what area of the brain is broccas area in
frontal
545
how do you calculate the cerebral perfusion pressure
the mean arterial pressure - intercranial pressure
546
what are predisposing factors for chronic subdural haemotoma
aspirin
anticoagulation
547
what can cause late secondary brain injury
brain swelling
delayed hydrocephalus
CNS infection
post operative haemotoma
systemic infection - pneumonia
548
what is the most common brain tumour type
gliomas
549
what is the most common form of glioma
glioblastoma multiform - high grade/grade 4
550
what are the major categories of extra-axial/extrinsic neurological tumours
meningiomas
pituitary adenomas
nerve sheath tumours - schwannomas
551
what causes spinal degenerative disease
1. hard disc osteophyte formation - osteoarthritis or wear and tear
2. soft disc prolapse
both of which result in canal stenosis, lateral recess stenosis or foraminal stenosis
552
what are causes of spinal cord compression
osteoarthritis - wear and tear
abnormal spine alignment - scoliosis
injury to the spine
spinal tumour
bone diseases
rheumatoid arthritis
infection
553
what are symptoms of spinal cord compression
pain and stiffness in the back, neck or lower back
burning pain that spreads to the arms, legs, buttocks
numbness, cramping or weakness
loss of sensation in the feet
trouble with hand or leg coordination
foot drop
loss of sexual ability
554
how is spinal cord compression diagnosed
X ray of the spine
CT/MRI
bone scan, myelogram, electromyography
555
how is spinal cord compression treated
NSAIDs
high dose steroids
radiation used to shrink tumours
physical therapy
surgery - removal or bone spurs
556
what is cauda equina syndrome
it is compression of the nerve roots in the cauda equina
- surgical emergency
557
what are causes of cauda equina syndrome
lumbar disc herniation - L4/5 L5/S1
spinal vertebral fractures of subluxation
malignancy
spinal infection: abscess, meningitis, tuberculosis
iatrogenic: anaesthesia, post operation
558
what cancers most commonly metastasise to the spine
breast, prostate, lung
559
what are the typical features of cauda equina
severe back pain
bilateral sciatica
perineal (saddle) anaesthesia
bowel and bladder dysfunction
sexual dysfunction
560
what is the difference between incomplete and complete cauda equina syndrome
incomplete - urinary difficulties, altered urinary sensation, loss of desire to void, hesitancy, urgency
complete - definitive urinary retention with associated overflow incontinence
561
what is the management of cauda equina syndrome
ABCDE
analgesia
catheter insertion
surgery = surgical decompression (laminectomy - removal of vertebral lamina, or discectomy - removal of intervertebral disc, or both)
562
what are complications of cauda equina syndrome
paraplegia
lower limb numbness
chronic urinary retention or incontinence
chronic sexual dysfunction
563
what are the ascending tracts of the spine
dorsal columns - fine touch, vibration sense and proprioception
spinothalamic tracts - pain and temperature
564
what are the descending tracts
corticospinal tract - motor information
corticobulbar
rubrospinal
tectospinal
reticulospinal
vestibulospinal
565
what is brown sequard syndrome
it is damage to one lateral half of the spinal cord - mostly occurs in cervical region
566
what is the classical clinical presentation of brown-sequard syndrome
ipsilateral spastic paralysis below level of lesion
ipsilateral loss of fine touch, proprioception and vibration
contralateral loss of pain and temperature sensation
567
what deficiency is posterior cord syndrome seen in
B12 deficiency - due to subacute combined degeneration of the spinal cord
568
What is a TIA
it is a sudden onset focal neurological deficit of vascular origin that lasts less than 24 hours
569
what are the most important causes of TIA to identify
atrial fibrillation
carotid stenosis
570
what are risk factors for developing a TIA
age
sex: M>F
smoking
hypertension
atrial fibrillation
diabetes
family history of TIA
previous TIA
vasculitis
571
what are the clinical features of a TIA
sudden onset and short duration
weakness
numbness or tingling
speech issues
vision changes
dizziness or loss of balance
headache
572
what investigations need to be done in someone with suspected TIA
12 lead EGC
FBC, blood glucose, lipid profile, coagulation screen
CT brain, MRI brain, carotid US and echo
573
what is the management of a TIA
300mg aspirin immediately
referral for assessment within 24 hours
screen for AF and carotid stenosis
if TIA confirmed and not at high risk of haemorrhage then duel antiplatelet for 21 days - aspirin and clopidogrel 75mg daily
secondary prevention: lifestyle advice, statins, smoking cessation, htn and diabetes management
574
what is carotid endarterectomy and when is it performed
it is a surgical procedure that removes atherosclerotic plaque from the carotid artery
recommended if:
70-99% internal carotid artery stenosis
50-69% stenosis recommended but only at centres with perioperative complication rate of <6%
575
what is trigeminal neuralgia
it is a form of neuropathic pain that is characterised by sharp, shooting sensation affecting the face
the pain is very intense
576
what are the three branches of the trigeminal nerve
ophthalmic branch - cornea, scalp and forehead
maxillary branch - cheek, upper jaw, upper lip, teeth, gums, lateral aspect of nose
mandibular branch - lower jaw, teeth, gums and lower lip
577
what are three types of trigeminal neuralgia
classic trigeminal neuralgia - vascular compression by artery/vein
secondary trigeminal neuralgia - tumour
idiopathic trigeminal neuralgia
578
what artery/vein most commonly causes classic trigeminal neuralgia
superior cerebellar artery
579
what are risk factors for trigeminal neuralgia
female
age (>50)
multiple sclerosis
580
what are symptoms of trigeminal neuralgia
unilateral, sharp facial pain
581
what are common triggers of trigeminal neuralgia
talking
washing the face
shaving
brushing teeth
exposure to cold air
582
how is trigeminal neuralgia managed
1st line - carbamazepine
other treatments: oxcarbazepine, lamotrigine, gabapentin, pregabalin
surgery - decompression, gamma ray knife
583
what is idiopathic intracranial hypertension
it is a raised intracranial pressure in the absence of intracranial mass or hydrocephalus
584
what is idiopathic intracranial hypertension associated with
female sex
obesity
585
what is the pathophysiology of idiopathic intracranial hypertension
several mechanisms proposed to explain it:
- overproduction of CSF
- obstruction of outflow of CSF
- raised pressure in the venous sinuses
- dysfunction in the glymphatic pathway
- hormonal alteration
586
what is the normal mechanism of CSF production and distribution
CSF is secreted by the choroid plexus in the lateral, third and fourth ventricles
this then moves into the subarachnoid space and acts as a cushion
CSF absorption mainly occurs via the arachnoid granulations when pressure exceeds the venous sinus pressure
587
what are risk factors for idiopathic intracranial hypertension
obesity
female sex
medications - COCP, tetracyclines, vitamin A, retinoids, lithium, thyroxine, nitrofurantoin
588
what are symptoms of idiopathic intracranial hypertension
headache - most common (non specific, diffuse)
nausea and vomiting
retrobulbar pain
pressure like sensation behind the eyes, worse on bending forward
transient visual loss/burring of vision
pulsatile tinnitus
visual disturbance
ocular motility disturbance - horizontal diplopia
589
what clinical findings might you see in idiopathic intracranial hypertension
papilloedema - bilateral and symmetrical
enlargement of the blind spot
peripheral visual field defects
horizontal diplopia
590
what are differential diagnosis for IIH
intracranial mass lesions
increased CFS production
reduced CSF absorption
obstruction of venous outflow
malignant hypertension
obstructive sleep apnoea
591
what investigations should be done for IIH
MRI/CT
lumbar puncture
blood pressure
magnetic resonance venogram of the head
perimetry
ocular coherent tomography
592
how is IIH diagnosed
signs and symptoms of raised intracranial pressure
absence of localising findings on neurological examination
normal CSF composition
absence of deformity, displacement or obstruction of ventricular system
normal neuroimaging
no other cause of increased ICP
593
how is IIH managed
conservative - weight loss
medical - Acetazolamide (first line), topiramate, furosemide, serial lumbar punctures
surgical - given to those with rapidly declining function: CSF diversion procedures, optic nerve sheath fenestration
594
what are complications of IIH
permanent visual loss
595
what is the most of action of acetazolamide
it is a carbonic anhydrase inhibitor and is believed to reduce the rate of CSF production
596
what is a stroke
it is an acute neurological deficit attributed to an acute focal injury of the brain, spinal cord or retina by either a vascular occlusion or haemorrhage
597
what are causes of ischaemic stroke
large vessel atherosclerosis
small vessel occlusion
cardioembolic ischaemic stroke
arterial dissection
non atherosclerotic arterial wall disease
non inflammatory vasculopathy
haematological disorders
cryptogenic
598
in western population where is the most common site of atherosclerosis with stroke
the internal carotid artery, however the circle of willis, vertebral arteries and the aortic arch may also be sources
599
what are risk factors for stroke
high BMI
high fasting plasma glucose
high systolic blood pressure
high LDL
kidney dysfunction
tabacco smoking
low physical activity
diet high in sodium and red meat, low in fruit and veggies
alcohol consumption
air pollution, lead exposure
600
what initial investigations should be done for someone presenting with a stroke
blood sugar - check for hypoglycaemia
CT head !!
CT angiogram and perfusion
chest X ray
ECH - AF
bloods - U+E, LFT, pregnancy
601
how is stroke diagnosed
history of symptom onset - acute deficit in keeping with a vascular event
radiological data to back it up
602
what is the management for ischaemic stroke
ABCDE !!!
- thrombolysis with administration of a tissue plasminogen activator such as alteplase. This is given within 4.5 hours or may be given up to 9 hours after stroke
- thrombectomy can be performed up to 24 hours after the stroke
- aspirin - 300mg immediately or after 24 hours if thrombolysis has been given
- decompressive hemicraniectomy
603
what is the initial ward care for patients coming in with stroke
should be admitted to acute or hyperacute stroke unit
need to manage aspiration risk, blood sugar control and VTE risk
blood pressure control in intercranial haemorrhage
cardiac rhythm monitoring
604
when is endarterectomy used in ischaemic stroke
if the carotid vessel is occluded by over 50% on the side where a stroke has happened
605
what is the long term medical care for ischaemic stroke
modifying risk factors
blood pressure - want systolic <130mmHg
lipids - 80mg atorvastatin
antiplatelets - 75mg clopidogrel OD long term
anticoagulation - DOAC
606
what is a total anterior circulation stroke
large cortical stroke affecting areas of the brain supplied by both the middle and anterior cerebral arteries
607
what are the criteria for something to be diagnosed as a total anterior stroke
1. unilateral weakness (and/or sensory deficit) of the face, arm and leg
2. Homonymous hemianopia
3. higher cerebral dysfunction (dysphagia, visuospatial disorder)
608
what is a partial anterior circulation stroke
it is a less severe form of TACS in which only part of the anterior circulation has been compromised
609
what is the criteria needed for a partial anterior circulation stroke to be diagnosed
two of the following need to be present:
1. unilateral weakness (and/or sensory deficit) of the face, arm and leg
2. Homonymous hemianopia
3. higher cerebral dysfunction (dysphagia, visuospatial disorder)
610
what is posterior circulation syndrome
it involves damage to the area of the brain supplied by the posterior circulation (e.g cerebellum and the brainstem)
611
what needs to be present for a diagnosis of posterior circulation syndrome
one of the following need to be present:
1. cranial nerve palsy and contralateral motor/sensory deficit
2. bilateral motor/sensory deficit
3. conjugate eye movement disorder
4. cerebellar dysfunction
4. isolated homonymous hemianopia
612
what is a lacunar stroke
this is a subcortical stroke that occurs secondary to small vessel disease. there is no loss of higher cerebral functions
613
what needs to be present for a diagnosis of lacunar stroke
one of the following needs to be present:
1. pure sensory deficit
2. pure motor deficit
3. sensori-motor stroke
4. ataxic hemiparesis
614
what is lateral medullary syndrome
clinical syndrome caused by acute ischaemia or infarction of the lateral medulla oblongata due to occlusion of the intracranial portion of the vertebral artery, posterior inferior cerebellar artery or its branches
- also known as wallenberg syndrome
615
what are risk factors of lateral medullary syndrome
hypertension is the commonest risk factor
smoking
diabetes
616
what is the clinical presentation of lateral medullary syndrome
1. vestibulocerebellar symptoms: ipsilateral hemiataxia, vertigo, falling towards the side of the lesion, multidirectional nystagmus
2. autonomic dysfunction: ipsilateral horner syndrome, hiccups
3. sensory symptoms: loss of pain and temperature over the ipsilateral face and contralateral side of the body
4. ipsilateral bulbar muscle weakness: hoarseness, dysphonia, dysphagia and dysarthria, reduced gag reflex
617
what structures can be affected in lateral medullary syndrome
inferior cerebellar peduncle
dorsolateral medulla
descending spinal tract
nucleus of the trigeminal nerve
vagus nucleus and nerve
glossopharyngeal nucleus and nerve
descending sympathetic tract fibres
618
what causes lateral medullar syndrome
vertebral artery disease - atherosclerosis, cerebral embolism, dissection, hypoplastic vertebral artery
619
how is lateral medullary syndrome diagnosed
clinical history and examination
MRI evaluation including diffusion weighted imaging (will look bright)
620
how is lateral medullary syndrome treated
similar management to any acute stroke
- rapid evaluation and treatment
621
what are the two pared arteries which are responsible for the blood supply to the brain
the vertebral arteries and the internal carotid arteries
622
where do the internal carotid arteries originate
at the bifurcation of the left and the right common carotid arteries at the level of C4
623
what arteries does the internal carotid artery give rise to
ophthalmic artery - structures in the orbit
posterior communicating artery - connecting vessel in circle of willis
anterior choroidal artery - brain important for motor and vision
anterior cerebral artery - cerebrum (parts)
the internal carotids then continue as the middle cerebral artery and supplies the lateral portions of the cerebrum
624
what are the branches of the vertebral arteries
meningeal branch - supplies falx cerebelli
anterior and posterior spinal arteries
posterior inferior cerebellar artery - supplies the cerebellum
the two vertebral arteries converge to form the basilar artery which supplies the pons and cerebellum
625
what is given long term to prevent further stroke
High dose statin - 80mg simvastatin
lifelong clopidogrel - 75mg
626
what is a key symptom of basilar skull fracture
CSF rhinorrhoea
627
when do you do a head CT post head injury if:
A) there is laceration or swelling
B) there is no signs, well in self +/- headache
A) within 1 hour
B) within 8 hours
628
what are complications of head injury
epilepsy
mood disorder
personality change
629
what are signs of a syncope episode
pre: pale, clammy, autonomic symptoms, chest pain
Ictal: floppy, loss of consciousness, eyes closed
post: immediate recovery and no amnesia
630
What are causes of epilepsy
V: Vascular
I: Infection
T: Trauma
A: AV malformation
M: Metabolic
I: Idiopathic
N: Neoplasm
631
what are risk factors for developing epilepsy
congenital: NFM1, tuberous sclerosis, CP
acquired: meningoencephalitis, febrile convulsions
dementia
632
what is brugada syndrome
a rare but potentially life-threatening inherited disease that predisposes patients to fatal cardiac arrhythmias
633
what are the characteristic ECG findings in Brugada syndrome
right bundle branch block and ST-segment elevations in the right precordial leads (V1-V3).
634
what are causes of non epileptic seizures
cardiogenic - MF, arrhythmias, heart block
reflex - neurogenic: postural hypotension, vasovagal
artery insufficiency
systemic
hypoglycaemia
635
what other diseases is parkinsonism seen in
lewy body dementia
parkinsons plus diseases - multisystem atrophy, progressive supernacula palsy, and corticobulbar degeneration
Wilsons disease
drug induced
N.P.H
636
what are symptoms of multisystem atrophy
parkinsonism plus cerebellar and autonomic symptoms
637
what are symptoms of progressive supranuclear palsy
parkinsonism plus fixed upward gaze
638
what are symptoms or corticobulbar degeneration
parkinsonism plus alien limb
- also get dementia and difficulty speaking and swallowing
639
what are examples of COMT inhibitors
entacapone
640
what are side effects of entacapone
orange urine
dyskinesia - worsens L-dopa effect
641
what is an example of dopamine agonists
ropinerole - non ergot derived
642
what are side effects of dopamine agonists
Ergot - pulmonary and CV fibrosis
non ergot - hallucinations
Dopamine receptor agonists are associated with the highest chance of inhibition disorders out of the antiparkinsonian medications
643
what are side effects of L-DOPA
postural hypotension
confusion
psychosis
chorea
644
what antipsychotics are used in huntingtons
vesicular monoamine transporter 2 (VMAT 2) inhibitors
645
what can cause medication overuse headache
over 15 days/month ibuprofen, paracetamol, triptans
over 10 days/month codeine, tramadol
646
what causes spinal muscular atrophy
auto recessive 5q SMN1 mutation
647
how does spinal muscular atrophy present
progressive muscle weakness
648
how can you treat spinal muscular atrophy
Nusinersen - increases the amount of the SMN protein helping to compensate for the defect
649
do the DVLA need to be informed in MS
yes they need to be informed but the patient is okay to drive
650
what is a key differential diagnosis for optic neuritis/ms
neuromyelitis optica
- see aquaporin 4 antibodies in the csf
651
when is someone considered brain dead
apnoea > 5 minutes
brainstem signs - no corneal, caloric cough reflexes, fixed dilated pupils, no pain response
GCS = 3
652
what are the two types of comatose posturing
decorticate = arms flexed to chest, cortical lesion (better prognosis)
Decerebrate - arms extended and pronated, brainstem lesion (poor prognosis)
653
what does transition to decorticate to decerebrate coma posturing indicate
that there is tonsillar herniation
654
what are causes of mono multiplex neuropathy
WARDS PLC
Wegners, AIDS, RA, DM, sarcoidosis, polyarteritis nodosa, leprosy, carcinoma
655
what are causes of polyneuropathy
ABCDE
alcohol
B12 deficiency
carcinoma
DMT2
every vasculitis
GB syndrome
656
what are risk factors for dural venous sinus thrombosis
factor V leiden, aPL, SLE, COCP, pregnancy, DIC
657
what are symptoms of dural venous sinus thrombosis
headache (acute, sudden N+V)
focal neurological symptoms (motor)
seizures (GTC)
658
how do you diagnose dural venous sinus thrombosis
CT contrast = empty delta sign
(filling defect in superior sagittal sinus)
659
how do you treat dural venous sinus thrombosis
low molecular weight heparin
660
what are signs of central retinal artery occlusion
amorosis fugax (painless)
rapid
cherry red macular spot
661
what is anterior ischaemic optic neuropathy
it is a thrombosis in the short post ciliary arteries and it is associated with GCA
662
what is acute angle glaucoma
it is a blockage in the aqueous humour causing raised intraocular pressure and optic nerve damage
663
what are signs of acute angle glaucoma
painful red eye
halos around lights
hazy cornea
nausea and vomiting
664
how is acute angle glaucoma treated
pilocarpine and acetazolamide
665
what can cause flash and floaters in the eye
retinal detachment
vitreous haemorrhage
666
what is a syringomyelia
it is cape like loss of the spinothalamic tract - in a cape distribution - upper limb, shoulders, posterior trunk and is due to a lesion of the anterior white commissure
667
what would be seen on MRI in syringomyelia
paraspinal cord fluid filled cysts
668
what are the symptoms of pituitary apoplexy
sudden onset headache
visual field defects
evidence of pituitary insufficiency (hypotension)
669
what is the management of pituitary apoplexy
1st = urgent steroids are needed (IV hydrocortisone)
definitive = pituitary adenoma resection
670
what is tuberous sclerosis
is an autosomal dominant genetic condition that affects multiple systems
671
what is a characteristic feature of tuberous sclerosis
the development of hamartomas which are benign tissue growths which cause problems based on where the lesion is: skin, brain, lungs, heart, kidneys, eyes
672
what are the mutations which cause tuberous sclerosis
TSC1 gene on chromosome 9, which codes for hamartin
TSC2 gene on chromosome 16, which codes for tuberin
673
what is the role of hamartin and tuberin in cells
Hamartin and tuberin interact with each other to control the size and growth of cells. Abnormalities in one of these proteins lead to abnormal cell size and growth.
674
what are the skin features of tuberous sclerosis
Ash leaf spots (depigmented areas of skin shaped like an ash leaf)
Shagreen patches (thickened, dimpled, pigmented patches of skin)
Angiofibromas (small skin-coloured or pigmented papules that occur over the nose and cheeks)
Ungual fibromas (circular painless lumps that slowly grow from the nail bed and displace the nail)
Cafe-au-lait spots (light brown “coffee and milk” coloured flat pigmented lesions on the skin)
Poliosis (an isolated patch of white hair on the head, eyebrows, eyelashes or beard)
675
what are the neurological features of tuberous sclerosis
Epilepsy
Learning disability
Brain tumours
676
How does tuberous sclerosis affect different body organs (heart, kidney, lungs, brain, eyes)
Rhabdomyomas in the heart
Angiomyolipoma in the kidneys
Lymphangioleiomyomatosis in the lungs
Subependymal giant cell astrocytoma in the brain
Retinal hamartomas in the eyes
677
what is the management of tuberous sclerosis
no treatment for underlying defect
supportive - monitoring and treating complications
mTOR inhibitors can be used to suppress growth of tumours
678
what is Webers syndrome
it is a midbrain stroke which is characterised by an ipsilateral third nerve palsy and contralateral hemiparesis
679
what blood supply is affected in Webers syndrome
the branches of the posterior cerebral artery that supply the midbrain
680
What is broccas dysphagia
speech is non fluent
comprehension normal
repetition impaired
- problem with inferior frontal gyrus
681
What is Wernickes aphasia
fluent but nonsensical speech
difficulty understanding language
- damage to superior temporal gyrus
682
what is conduction aphasia
speech is fluent but repetition is poor
aware of the errors
comprehension is normal
- damage to the arcuate fasiculus
