Neurology Flashcards
1
Q
Febrile convulsions
A
Seizures occuring in children with a high fever, between the ages of 6 months and 6 years
2
Q
Simple vs Complex febrile convulsions
A
Simple:
- generalised tonic-clonic,
- LESS THAN 15 MINUTES,
- ONLY OCCUR ONCE during a single febrile illness
Complex:
- PARTIAL/FOCAL
- MORE than 15 MINUTES
- MULTIPLE during course of same illness
- May also get Todd’s paresis
3
Q
DDx for Febrile convulsions
A
- Epilepsy
- Neuro INFECTION (mening, enceph, cerebral malaria)
- Space occupying lesions
- SYNCOPE
- ELECTROLYTE abnormalities
- TRAUMA
4
Q
Presention of febrile convulsions
A
Typically:
- Child around 18 MONTHS
- 2-5 MINUTE TONIC CLONIC
- HIgh fever
5
Q
Management of febrile convulsions
A
Identify + manage underlying infection. ALAGESIA for fever.
if simple - no further investigation - just reassure + educate.
If complex educate + investigate underlying causes/DDx
Always need hospital assessment for first instance
6
Q
Parental advice for managing febrile convulsions
A
- Stay with the child
- Put the child in a safe place, for example on a carpeted floor with a pillow under their head
- Place them in the recovery position and away from potential sources of injury
- Don’t put anything in their mouth
- Call an ambulance if the seizure lasts more than 5 minutes
If confident, can manage episodes after 1st one at home and just see GP asap
7
Q
What things must be asked about for a paediatric neuro Hx specifically
A
BIRTH HISTORY (pre/ante+post-natal)
Developmental milestones
8
Q
Paediatric neuro disorders
A
- Meuromuscular conditions
- Autoimmune
- Demyelinating
- Movement disorders
- Neurodegenerative
- Stroke
- EPILEPSY (most common in childhood specifically)
9
Q
Cerebral palseies
A
Group of developmental disorders of movement + posture - activity limitation
- attributed to NON-PROGRESSIVE pathology that occured in developing fetal/infant brain
- Sx can be progressive as child grows up (due to diff functioning needs)
10
Q
Types of cerebral palse
A
Spastic (pyramidal)
Dyskinetic: Hypotonic/Dystonic (Athetosis?) (extrapyramidal)
Ataxic
Mixed
11
Q
Patterns of cerebral palsey
A
- Monoplegia: one limb affected
- Hemiplegia: one side of the body affected
- Diplegia: four limbs are affects, but mostly the legs
- Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
12
Q
Cerebral palsey causes
A
Antenatal:
Maternal infections
Trauma during pregnancy
Perinatal:
Birth asphyxia
Pre-term birth
Postnatal:
Meningitis
Severe neonatal jaundice
Head injury
13
Q
Cerebral palsey Px
A
- Failure to meet milestones
- Increased or decreased tone, generally or in specific limbs
- Hand preference below 18 months is a key sign to remember for exams
- Problems with coordination, speech or walking
- Feeding or swallowing problems (BULBAR PROBLEMS)
- Learning difficulties
Oft have co-morb
14
Q
Complications/co-morbs of cerebral palsey
A
- Learning disability
- Epilepsy
- Kyphoscoliosis
- Muscle contractures (can get status dystonicus)
- Hearing and visual impairment
- Gastro-oesophageal reflux
- Issues with secretions due to lack of muscle control
15
Q
Classification of cerebral palsey severity
A
1 mild - 5 severe
16
Q
Cerebral palsey Mx
A
If no seizures, oft treated by neurodisability team
MSK: Positioning, Splints, Serial casting
- physio/occupational
Medical Tx for spasticity:
- Baclofen (muscle relaxants)
- Diazepam (muscle spasms)
- Glycopyrronium bromide (excessive drooling)
- Botulinium toxins, anticholinergics if specific muscle group affected + significantly affecting
Surgical: Intrathecal baclofen, dorsal rhisotomy, tendon lengthening
Feeding: Nutritional, NGT, PEG, SALT
Vision + auditiory
Social workers
17
Q
Cerebral palsey DDx
A
- Segawa disease (dopamine responsive dystonia)
- Hereditary spastic paraperesis
- Inborn errors of metabolism
- Spinal cord injuries
18
Q
Epilepsy clinical basis for diagnosis
A
2 or more unprovoked seizures occuring more than 24hrs apart
19
Q
Types of seizures
A
Generalised tonic clonic
Absence
Focal
Myoclonic
Atonic
Infantile spasms (West syndrome)
Febrile convulsions
20
Q
Infantile spasms (West syndrome) + Tx
A
Rare
Triad of:
- Spasms starting ~ 4-7 MONTHS
- full body spasms (oft affecting arms) - usually symmetrical
- hypsarrhythmia (disorganised activity) on EEG
- Developmental regression
Associated with variable encephalopathy
Poor prognosis (1/3 die by 25; 1/3 seizure free)
Tx: PREDNISOLONE + VIGABATRIN
21
Q
Infantile spasms diagnosis
A
Spasms + HYPSSARRHYTHMIA in EEG
22
Q
Rolandic epilepsy
A
Focal onset - Facial/Perioral with 2ndry generalisation
EEG - Centrotemporal spikes
23
Q
Childhood absence epilepsy
A
Brief arrest of speech + activity
EEG 3 Hz spike + slow wave activity
Check if happening in more than one setting; Having automatisms
Can affect academic achievement
24
Q
Epilepsy Ix
A
- Imaging if no evident aetiology
- Karyotyping (if intractable, learning diff, infantile onset)
- Metabolic work-up
- EEG is gold but changes only occur during episodes
- HISTORY TAKING (witness account is very important)
- Epilepsy is stereotypical so if each episode is diff, probs not epilepsy
25
Epilepsy Mx
- Anti-Epileptics (be aware of side effects + monitor)
- Surgery
- Vagal Nerve Stimulator (done via magnet)
- Daily life counselling
26
Acute causes of headaches
Systemic illness w fever
Local ENT causes
Meningitis
Trauma
Haemorrhages
27
Red flags for Headaches
- Most severe acute onset (thunderclap)
- Worsening severity
- Wakes child from sleep
- Occurs upon waking/getting up
- Morning vom/nausea
- Child younger than 4
- Associated confusion/disorietntation
- Visual abnorm
Regressed motor skills
28
Headaches Ix in kids
Imaging
LP - +manometer for pressure
Bloods
29
Management of headaches
Lifestyle changes
Pharm:
- Analgesia
Triptans
Topiramate
Propanolol
Be mindful of medication overuse headache
30
Examples of lifestyle changes for headaches
- sleep habits + routine
- fluid intake
- regular meals
- eliminate precipitants: chocolate, cheese, tomates, sugary snacks, caffiene, alcohol, citrus fruit
- regular exercise
- reduce stressors
31
Movement disorders
- Abnormal movement in absence of weakness
- Hyperkinetic:
- Tremor, chorea, athetosis, hemiballismus
- Hypokenetic:
- Parkinsonism: Rigid, Resting tremor, Bradykinesis
- Dopa-responsive dystonia; drug induced
32
Chorea
Spontaneous, rapid, brief, jerky
- **UNSUSTAINED + RANDOM** movement
Present at rest and INCREASED by ACTIVITY, STRESS and SELF-CONSIOUSNESS
- BUT **disappears in sleep**
33
Causes of chorea
- Syndenham's chorea (from RHEUMATIC FEVER)
- Vascular disease of basal ganglia
- Benign hereditary chorea
34
Athetosis
IRREGULAR, coarse, **RHYTHMIC + WRITHING**
- SLOWER + MORE SUSTAINED + LARGER AMPLITUDE than chorea
Sort of flowy, looks like dancing - disappears in sleep
35
Hemiballismus
Wild, FLINGING
- INCESSANT movement of one side of body
36
Myoclonus
Rapid, ARRYTHMIC, Repetitive, JERKY involuntary contractions
37
Examples of myoclonus
Sleep myoclonus, hypnic jerks
Juvenile Myoclonic Epilepsy (idiopathic + hereditary)
38
Active epilepsy
Has had a seizure in past 12 months
39
Epilepsy Diagnostic definition
- 2 or more unprovoked seizures more than 24 hours apart
- one unprovoked seizure with >60% risk of recurrance in the 10 years following 2 previous seizures
- Dx of Epilepsy Syndrome
40
Common causes of epilepsy misdiagnosis
- Lack of eye witness
- Presence of clonic jerks/incontinence
- Past Hx of seizures
- Influenced by +ve FHx
- Over-interpretation of EEG (the normal population can have variations in EEG)
41
Syncope pathophys
Arrest of cortical activity due to transient impaired blood/O2
42
Syncopal disorder
- Cyanotic breath-holding attack
- Reflex anoxic seizure (in response to pain - typically in infants)
- Vasovagal
- Valsalva manoeuvre
- Cardiac arrhythmia (so EVERYONE with LOC/seizure NEEDS ECG)
- esp LONG QT SYNDROME
43
Pre-Syncope Sx
- lightheadedness
- nausea
- visual 'gray' / black spots etc
- trouble hearing
- palpitations
- weakness, sweaty
44
Causes of syncope
- Impaired orthostatic control
- Resp syncope
- Stimulated by valsalva manouver
45
DDx for night time seizure-like Sx
- Parasomnias (night terrors, sleep talking, sleep walking - during deep sleep, won't remember)
- Epilepsy (frontal/temporal lobe)
- Psychogenic
- Cardiogenic
Phisiological = Sleep myoclonus (jerkig only in sleep; NO FACIAL JERKING)
46
REM Parasomnias
Uncommon - early hours of morning; can remember
- REM sleep behaviour disorder
- REM sleep related sinus arrest
- NIGHTMARES
- SLEEP PARALYSIS
47
Frontal lobe seizures
Typically get multiple stereotypical events throughout the night for multiple nights per week
- Initial Arm posturing (easily missed)
- Hypermotor features + fearful response
48
How can MI cause cardioembolic stroke?
Wall becomes hypokinetic -> buildup of blood -> LV aneurysm
- Esp in anterior wall
- 2nd most common cardioembolic after AFib
49
RFx Primary intracranial haemorrhage
- Intracranial small vessel disease - - HTN
- Cerebral amyloid angiopathy
- Arteriovenous Malformations (AVMs)
- CNS neoplasms - esp if multiple small haemorrhages
- Anticoagulation
50
What is the BP aim for HTN with stroke risk
< 130/80
51
Give specific lifestyle advice for HTN/diabetes in stroke risk
Reduce sodium salt in diet (high sodium : low potassium salt is a big RFx for HTN)
At least 150 mins of light/moderate exercise (intense enough to make you sweat)
52
Causes of Cerabellar dysfunction
VITAMIN C (is the mnemonic)
- Vascular
- Infectious (LYME DISEASE; Cerebellar ABSCESS
- Trauma
- ALCOHOL (chronic -> cerabellar atrophy)
- MULTIPLE SCLEROSIS (Inflam)
- Iatrogenic (**Phenytoin; Carbamazepine**)
- NEOPLASTIC
- Congenital / Hereditary (Friedrich's ataxia; Spinocerebellar ataxias)
53
Cereabellar dysfunction Sx
DANISH
- Dysdiadochokinesia
- Ataxia
- Nystagmus
- Intention tremor
- Slurred / Staccato speech
- Heel-Shin test / HYPOTONIA
+ Reduced reflexes
Location of lesion -> specific Sx:
- Vermis -> truncal ataxia + unstable gait (few limb signs)
- Hemispheric -> Limb signs
54
Cerebellar dysfunction Ix
NEURO EXAM/Hx + further tests as required
- CT / MRI
- Serology for infection / MS markers (Intrathecal/blood IgG Oligoclonal banding)
- Lumbar Puncture (infection, inflam or malig)
- Genetic testing
55
Cerebellar dysfunction Mx
Tx underlying:
- Meds / Abx for infections/inflam
- Surgery to remove tumours / treat trauma
- Rehabilitation (physio/occupational; SALT etc)
- Lifestyle mod - esp CEASE ALCOHOL
56
Friedrich's ataxia
Autosomal RECESSIVE hereditary NEURODEGENERATIVE disorder -> PROGRESSIVE damage, mainly to CORTICOSPINAL + SPINOCEREBELLAR tracts, DORSAL column + peripheral nerves
57
Friedrich's ataxia epid
- rare (1 in 40 000) but most common hereditary ataxia
- **5-15 y/o**
58
Friedrich's ataxia pathophys
Autosomal RECESSIVE - trinucleotide (GAA) repeat EXPANSION in FRATAXIN gene (chromosome 9)
- reduced frataxin synthesis - it is normally used for mitochondrial iron homeostasis - low levels = iron accumulation + oxidative stress -> neuronal + muscle cell death
59
Freidrich's ataxia Sx
- Neurological symptoms: lower limb weakness, gait abnormalities, frequent falls, cerebellar signs (ataxia, dysarthria, dysmetria, etc.), and mixed upper and lower motor neuron signs such as absent knee/ankle reflexes and upgoing plantars.
- Sensory symptoms: impaired joint position and vibration sense due to dorsal column involvement.
- Physical signs: high-arched palate, pes cavus (high arch of the foot), and kyphoscoliosis.
- Non-motor features: **hypertrophic obstructive cardiomyopathy**, reduced visual acuity, type 1 diabetes mellitus, and deafness.
60
Friedrich's ataxia Mx
primarily supportive and symptomatic
Physical therapy for gait and coordination problems.
Speech therapy for dysarthria.
Cardiac surveillance and management of hypertrophic cardiomyopathy.
Regular screening for diabetes mellitus and management if present.
Assistive devices for
mobility as the disease progresses.
Genetic counselling to discuss implications for family members and potential offspring.
61
Friedrich's ataxia prognosis
most patients become wheelchair-bound by their 20s and face premature death, usually in their 40s to 50s, primarily due to cardiac complications
62
Spinocerebellar ataxia
Autosomal DOMINANT PROGRESSIVE neurodegen - mostly in cerebellum but also in spine
- Ataxia
- Nystagmua
- Dysarthria
- Typically presents in ADULTHOOD
63
Narcolepsy
Early onset of REM sleep - associated with **HLA-DR2** + LOW levels of OREXIN (HYPOCRETIN) - protein responsible for controlling appetite + sleep patterns
64
Narcolepsy Sx
- Hypersomnolence
- Cataplexy (sudden loss of muscle tone oft triggered by strong emotion)
- Sleep paralysis
- Vivid hallucinations on going to sleep / waking up
65
Narcolepsy Ix
Multiple sleep latency EEG
66
Narcolepsy Mx
daytime stimulants (e.g. modafinil) and nighttime sodium oxybate (sedative)
67
Herpes zoster ophtalmicus
Shingles affecting the ophthalmic division of trigeminal nerve
- Painful red eye
- Fever, malaise + headache
- Erythematous vesicular rash over ophthalmic distribution
Ocular involvement may be indicated by a lesion on the nose ()HUTCHINSON'S sign)
68
Complications of shingles
- Secondary bacterial infection
- CORNEAL ULCERS / scarring + blindness if eye involved
- POST-HERPETIC NEURALGIA
- NEUROPATHIC PAIN at site of healed shingles (burning / pins + needles)
- ALLODYNIA (pain from non-painful stimulus e.g. light touch)
69
Normal Pressure Hydrocephalus (NPH)
Excess CSF accumulates in ventricles and causes pressure effects leading to Sx, HOWEVER pressure oft appears normal on lumbar puncture
70
NPH epid
- older adults (big cause of dementia - reversible tho)
71
NPH aet
Uncertain but oft associated with things blocking CSF absorption:
- SAH
- Meningitis
- Head trauma / Iatrogenic post-surg
72
NPH Sx
Classic triad:
- DEMENTIA (global impairment + attention / memory defecits)
- MAGNETIC GAIT (difficulty lifting feet off floor)
- INCONTINENCE (usually urinanry)
("Wet, Whacky + wobbly")
73
NPH Dx
- CT / MRI
- DILATED LATERAL ventricles (tho can see in other forms of dementia due to cortical atrophy)
- Lumbar puncture
- measuring walking ability and cognitive assessment before and after can help identify which patients would benifit from surgical management
74
NPH Mx
- Ventriculoperitoneal shunt (permenantly shunts excess CSF to abdo)
- THeraputic lumbar puncture (if can't do VP shunt)
75
Normal ICP in adults in supine position
7 - 15 mmHg
76
Cerebral perfusion pressure
Net pressure gradient causing cerebral blood flow to brain
CPP = mean arterial pressure - ICP
77
Causes for raised intracranial pressure
- Idiopathic intracranial HTN
- TRAUMA
- INFECTION (meningitis)
- Tumours
- Hydrocephalus
78
Raised ICP Sx
- Headache (esp on lying, in morning, on valsalva etc)
- Vomiting
- Reduced consiousness
- Papilloedema
- CUSHING'S TRIAD
- Wide pulse pressure
- Bradycardia
- Irregular breathing
79
Raised ICP Ix
- CT / MRI (underlying cause)
- Invasive ICP monitoring
- CATHETER into LATERAL ventricles
- can take CSF samples / drain small amounts
- Further Tx only given if pressure **>20 mmHg**
80
Raised ICP Mx
- Tx underlying cause
- Elevate head to **30 degrees**
- **IV MANNITOL** (osmotic diuresis)
- Controlled hyperventilation
- reduces pCO2 -> casoconstriction of cerbral arteries + reduced ICP - HOWEVER risk of further ischaemia
- Remove CSF
- intraventricular drain monitor
- Repeated lumbar puncture
- Ventriculoperitoneal shunt
81
Hydrocephalus (not NPH) epid
more common in INFANTS + ppl OVER 60
82
Types of hydrocephalus
- OBSTRUCTIVE - flow of CSF blocked along one / more narrow passages connecting ventricles
- foramen of Monro (e.g. colloid cysts)
- cerebral aquaduct (e.g. aquaduct stenosis)
- 4th ventricle (e.g. posterior fossa tumour)
- COMMUNICATING hydrocephalus - Impaired absorption of CSF into blood
- oft from subarachnoid issues e..g. SAH or infective meningitis
83
Hydrocephalus Sx
Early morning headaches
Nausea and vomiting
Lethargy
Vision disturbances
Balance problems
Cognitive difficulties
(all caused by pressure squishing brain) - but NOTE - incontinence is NOT typical in hydrocephalus but is a classic finding in NPH
84
Hydrocephalus Ix
- CT (ventricular enlargement - confirms Dx)
- MRI (further info for underlying cause)
85
Hydrocephalus Tx
- ventriculoperitoneal/-atrial SHUNT
- needs regular follow-up to check for malfunction / infection
- potentially endoscopic THIRD VENTRICULOSTOMY (ETV) - hole in bottom of ventricle -> CSF can drain out of brain
86
Neurofibromatosis aet
Both are AUTOSOMAL DOMINANT Loss-of-function mutations
- NF1 - in NEUROFIBRIMIN gene (Chromosome **17**)
- NF2 - in SCHWANNOMIN TUMOUR SUPRRESSOR gene (**Chromosome 22**)
87
NF1 Px
Skin:
- Cafe-au-lait spots (grow throughout life - need at least 6 > 5mm in kids to be a feature of NF1)
- Axillary / inguinal freckling
- Neurofibromas (nodular tumours in skin)
Non-skin:
- **Lisch nodules** (hamartomas on IRIS - brown patches typically visible by 6 y/o)
- Optic glioma
- Scoliosis
- Learning diff
- Hypertension
- GI bleeding / obs from tumours
- Epilepsy from brain tumour
88
NF2 Sx
- Bilateral VESTIBULAR SCHWANNOMAS (acoustic neuroma)
- sensorineural hearing loss, tinnitus, vertigo
- Meningiomas
- Spinal Ependymomas
- Posterior LENS OPACITIES
- Cerebral calcification
- ASTROCYTOMAS
- Glial hamartomas
(i.e. more aggressive w tumours than NF1, but NF1 has more other Sx)
89
NF Ix
- GENTIC TESTING
- Neuroimaging (tumours / abnormal anatomy?)
- Slit lamp examination (for LISCH nodules )
90
NF Mx
- Surveillance
- Sx treatment (e.g. HTN, epilepsy, other complications etc)
- Surgical intervention to remove tumours when necessary
91
Squint
aka Strabismus
Misalignment of the eyes causing double vision
92
Amblyopia
Lazy eye - usually caused by childhood squint
- high acuity vision is affected by abnormal visual stimulation until age 8-10
- abnormal vision in one or both eyes -> vision loss in that eye + more passive + reduced function due to disconnection from brain
93
Types of squint
- Concomitant (due to differences in control of extraoccular muscles)
- Paralytic squint (rare - paralysis in one/more of extra occular muscles)
94
Causes of squint
Usually idiopathic
- Hydrocephalus
- Cerebral palsey
- Space occupying lesions
- Trauma
95
Examination for squint
- General + eye movement
- Fundoscopy
- Visual acuity
HIRSCHBERG'S TEST - looking at if reflection on a light shone 1M away is equal + symetrical in both eyes
COVER TEST - cover one eye + get kid to focus on somethin
- then switch to other eye + see if they have to move their previously covered eye to focus on the object
96
Squint Mx
Needs to be before age 8 (when visual fields development stops)
- Occlusive path - covers good eye + forces weaker eye to develop
- alternative is atropine drops in healthy eye -> blurs vision in that eye
Co-ordinated by opthalmologist
Tx any underlying
97
Causes of amblyopia
- Squint
- Unequal refractive error
- Congenital cataracts
- Tumour blocking visual axis
98
Amblyopia Sx
- Reduced acuity
- Blurred / cloudy vision
- Double vision
-Poor depth perception
- Squinting / closing one eye to focus
- Eye fatigue
- Visible strabismus (squint)
May SUPPRESS image from weaker eye to avoid double vision
- may have reduced contrast sensitivity
99
CNS tumours
most common tumours in KIDS (20% of childhood cancer)
100
Most common types of brain tumours:
- Astrocytoma - 40% (from GLIAL cells)
- Medulloblastoma - 13% (usually in posterior fossa / cerebellum)
- Ependymoma - 7% (from CSF making EPENDYMAL cells)
- Craniopharyngioma - 5% (close to pit gland)
- Germ cell tumours - 4% (close to pit + pineal gland)
- Choroid plexus tumours - 2% (from ependymal cell network)
101
RFz for childhood CNS tumours
- **PMx / FHx** - brain tumour, leukaemia, sarcoma OR early onset breast cancer
- Prior theraputic CNS IRRADIATION
- NEUROFIBROMATOSIS 1 & 2
- TUBEROUS SCLEROSIS 1 & 2
- Familial genetic syndromes (e.g. von Hippel-Lindau)
102
Common Sx of brain tumours in kids
- Headache
- N+V
- Seizures
- Altered GCS
- Behavioural change (frontal lobe)
- Polyuria / -dipsia (tumours can interfere with ADH production from pit gland -> DIABETES INSIPIDUS)
On examination:
- Diplopia; reduced acuity / fields; abnormal eye movement / fundoscopy
- Abnormal gait / co-ord; swallowing diff; weakness
- DELAYED GROWTH / Abnormal puberty
- Increased head circum in <2 y/o
N/B: may seem completely normal on examination
103
Red flag Sx for brain tumours in kids
- Persistent/recurrent HEADACHE (less so in kids < 5)
- Persistent / recurret VOMITING
- BALANCE / co-ord / walking problems
- ABNORMAL EYE movements / IMPAIRED/LOSS of VISION
- BEHAVIOUR CHANGE (esp if particularly lethargic)
- Fits or SEIZURES
- Abnormal HEAD POSITION in younger children
- Delayed / arrested PUBERTY
Increasing head circumerence in kids < 5
104
CNS malig management (kids)
initial: Analgesia, Antiemetics, Anticonvulsants, Fluid / dietary support
- consider STEROIDS to REDUCE ICP
- Surg resection
- Radio
- Chemo
- consider Proton therapy or Stem Cell Transplants
SURVEILLANCE for RELAPSE + effects of Tx; Fertility support + Neuro-rehab
105
Potential complications of CNS tumour Tx in kids
- Epilepsy / seizures
- Sleep disturbance
- Effects on puberty / fertility
- Hearing loss
- Impaired growth
- Cognitive impairment
- Secondary malig (if the kid recieved radiotherapy)
106
CNS tumour prognosis (kids)
5 year survival rate:
- Low grade astrocytoma = 95%
- High grade astrocytoma = 25%
- Ependymoma = 80%
- Medullablastoma = >60%
107
Tuberous sclerosis
Rare genetic condition which causes (mainly) benign tumours to develop through body.
Most commonly in:
- Brain
- Skin
- Kidneys
- Heart
- Eyes
- Lungs
108
Bell's palsey
Idiopathic syndrome of damage to facial nerve / CN7
- potentially inked to viral infections, particularly: HSV-1; EBV, VZV
Peaks at age 15-45
109
Bell's palsey Px
- Acute (**but not sudden**) UNILATERAL LMN FACIAL WEAKNESS - **not forehead sparing**
- Postauricular otalgia (pain from behind ear) may preceed
- Hyperacusis (reduced tolerance to sound)
- Nervus intermedius Sx ( altered taste, dry eyes/mouth)
- Sometimes numbness / heaviness feeling without obgective facial somatosensory disturbances
110
Bell's palsey DDx
- Ramsay-Hunt Syndrome (prominent otalgia + vesicular rash)
- Lyme disease (erythem migrans + systemic Sx)
- Herpes zoster
- Acute otitis media
- Stroke
- GUillain Barre syndrome (asscending symmetrical weakness + autonomic dysfunction)
111
Bell's palsey Dx
Clinical. Tests of exclusion.
- FBC
- ESR / CRP
- Viral serology (HSV-1; EBV; VZV)
- Lyme serology (if suspected)
- Otoscopy (vesicular lesions? - ramsay-hunt)
- Electromyography (EMG)
- Ct /MRI
112
Bell's plasey Mx
- 50mg ORAL PREDNISOLONE OD for 10 DAYS - then taper down
- can consider aciclovir sometimes
- Supportive:
- Artificial tears / ocular lubricants
- Eye patch / tape if protective Bell's phenomenon is absent (prevent corneal exposure + injury)
- consider analgesia; physio; psych support
113
Bulbar palsey
LMN lesion affecting:
- 9th, 10th, and 12th cranial nerves
Results in impaired speech and swallowing
114
Main causes bulbar palsey
- MND (esp bulbar palsey varient)
- Myesthenia gravis
- Guillain Barre syndrome
- Brainstem stroke (esp lateral medullary syndrome or Wallenberg's syndrome)
- Syringobulbia (rare - fluid-filled cavity (syrinx) within spinal cord)
115
Bulbar palsey Sx
- Absent OR normal jaw jerk reflex
- Absent gag reflex
- Flaccid, fasciculating tongue
- Nasal speech, often described as "quiet"
- Other signs related to the underlying cause e.g. limb weakness in MND
116
Bulbar palsey DDx
- Pseudobulbar palsey (UMN CN lesion instead of LMN - additional emotional lability)
- Brainstem tumour (long tract signs more common e.g. spastic, hyperreflexia, Babinski)
- MS (variable Sx depending on lesion)
- Poly/dermamyositis
117
Bulbar palsey Dx
find underlying cause:
- Neuro exam
- Electromyography (EMG) + nerve conduction studies (MND + MG)
- BLOODS - FBC, electrolytes, CK, Autoantibodies
- MRI
- LP - rule out infection / identify autoimmune
118
Bulbar palsey Mx
- SALT + swallowing therapy
- NUTRITION support
- Meds depending on underlying condition
- REGULARLY MONITOR
119
Anterior cord syndrome
**Anterior Spinal Artery ischaemia / infarct**, affecting ANTERIOR 2/3 of cord, leading to MOTOR DEFECITS + LOSS of PAIN / TEMP +/- autonomic Sx
- normally supplies blood to anterior and lateral horns (T1-L2 - Sympathetic chain cell bodies); and spinothalamic + corticospinal tracts
120
Anterior cord syndrome Sx
Typically acute
- Motor dysfunction + loss pf PAIN / TEMP below level of lesion
- usually bilateral
- Loss of autonomic functions below lesion if lesion occurs between T1 - L2 (location of sympathetic chain)
- neurogenic bladder / bowel
- hypotension
- sexual dysfunction
Oft also get acute onset back pain at level of lesion - in cord stokes in general
121
Anterior cord syndrome Ix
MRI -> hyperintese areas on T2-weighted images in anterior 2/3 of cord
- may see owl's eyes sign in spinal cord if axial MRI done
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Complications of anterior cord syndrome
- Peristent neuro deficits
- CHRONIC PAIN
- Urinary / bowel dysfunction
- PRESSURE SORES from prolonged immobility
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Anterior cord syndrome Mx
Supportive/prevent complications + treat underlying
- e.g. surgery for aortic dissection
- ANALGESIA
- PHYSIO
- Mx of autonomic dysfunction e.g. urinary catheter to manage neurogenic bladder
- Skin care to prevent pressure sores
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Causes of Anterior cord syndrome
- Aortic damage: aneurysm or dissection
- Atherosclerosis
- Cardiac arrest
- Cardiac emboli
- Vasculitis
- Shock
basically anything that can cause ischaemia
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Anterior cord syndrome epid
Mostly in elderly
Higher prevelance if co-morb:
- Atherosclerosis
- HTN
- Diabetes
Or if having surgical procedures done on aorta
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Causes of brain abscess
- Extension of sepsis from middle ear or sinuses
- Trauma / surgery to head
- Embolic events from endocarditis
and more
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Brain abscess Sx
Oft raised ICP and focal Sx
- Dull, persisting headache
- FEVER (may be absent)
- Usually NOT a SWINGING pyrexia (common with other types of abscesses)
- Nausea
- Papilloedema
- Seizures
- Focal neurology e.g. oculomotor nerve palsey from raise ICP , motor dysfunction etc
127
Brain abscess Ix + findings
CT head:
- RING ENHANCING hypoattenuating lesion with SURROUNDING OEDEMA (usually)
- basically very dark in middle, white ring, darker gray than usual brain around it
Sometimes brain mets can necrose in centre and look like absecess -> MRI to differentiate
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Brain absecess Mx
- Surgery
- CRAINIOTOMY + DEBRIDEMENT of abscess cavity
- tho abscess could reform once head is closed
- IV ABX - typically 3rd gen Cephalosporin e.g. **CEFTRIAXONE / CEFOTAXIME + METRONIDAZOLE** for **6-8 wks**
- Manage ICP e.g. with Dexamethasone (reduces swelling)
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Which drugs can make MG worse
- Some antibiotics (GENTAMICIN)
- BETA-BLOCKERS
- CALCIUM-channel blockers
(remember ABC)
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What can trigger myesthenic crisis
- Infection
- Anaesthetics
- Under/overdosing meds
- Severe disease
- CI drugs
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Cape-like distribution of weakness; pain + temp - affecting arms
SYRINGOMYELIA
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Total anterior circulation infarct (TACI) definition (Bamford/Oxford classification)
- Contralateral hemiplegia / hemiparesis AND
- Controlateral homonymous hemianopia AND
- Higher cerebral dysfunction (e.g. aphasia, neglect)
Involves anterior AND middle cerebral arteries
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Partial anterior circulation infarct (PACI) definition (Bamford/Oxford classification)
Any 2 of:
- Contralateral hemiplegia / hemiparesis AND
- Controlateral homonymous hemianopia AND
- Higher cerebral dysfunction (e.g. aphasia, neglect)
OR
- Just higher ccerebral dysfunction by itself
Only either anterior OR middle cerebral arteries affected (on affected side)
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Lacunar infarct (LACI) definition (Bamford/Oxford classification)
Any one of following:
- pure motor stroke
- pure sensory stroke
- senorimotor stroke
- ATAXIC HEMIPARESIS
- DYSARTHRIA-CLUMPSY HAND SYNDROME
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Posterior circulation infarct (POCI) definition (Bamford/Oxford classification)
Any one of the following:
- Cerebellar dysfunction
- Conjugate eye movement disorder
- Bilateral motor/sensory deficit
- Ipsilateral cranial nerve palsey with conteralateral motor/sensory deficit
- Cortical blindness / isolated hemianopis
Involves vertebrobasilar arteries + associated branches which supply cerebellum, brainstem + occipital lobe (e.g. cerebelar arteries)
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Posterior stroke syndromes
- Basilar artery occlusion (locked in syndrome, LoC, sudden death)
- Anterior Inferior Cerebellar artery (lateral pontine syndrome)
- Wallenburg's / Lateral medullary syndrome
- Weber's / medical midbrain syndrome
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What is the most common brain tumour in kids
Pilocytic Astrocytoma
Defferentiated by seeing 'Rosenthal fibres' (corkscrew eosinophilic bundle) on histology
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Medulloblastoma
Aggressive PAEDIATRIC brain tumour arising from INFRATENTORIAL COMPARTMENT.
Histology: small, blue cells in ROSETTE pattern with many MITOTIC figures
Surgical resection + chemo - can spread THROUGHOUT CNS
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Where is ependymoma often found + histological appearance
In 4th ventricle -> can cause hydrocephalus
Perivascular pseudorosettes
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Oligodendroma
BENIGN, SLOW-growing tumour - oft in FRONTAL lobes
Histology: Calcification with 'FRIED-EGG' appearance
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Haemagioblastoma meaning + histology
VASCULAR tumour - of CEREBELLUM
Associated with **von Hippel-Lindau syndrome**
Histology: foam cells + HIGH VASCULARITY
142
Microadenomas vs macroadenomas of pituitary (size comparison)
- micro = <1cm
- macro = >1 cm
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Craniopharyngioma meaning
Most common SUPRATENTORIAL tumour in kids (kids commonly have infratentorial tumours)
- SOLID or CYSTIC tumour
- found in SELLAR REGION
- DERIVED from RATHKE'S POUCH remnants (can present at any age technically)
144
Craniopharyngioma Px
- hormonal disturbance (can compress pituitary)
- hydrocephalus
- bitemporal hemianopia
145
Which cancers most commonly metastesize to the brain
- LUNG
- Breast
- Bowel
- Melanoma
- Kidney
146
Glioblastoma multiforme meaning / epid
most common PRIMARY brain tumour in ADULTS
- poor prognosis (~1 yr)
- SOLID tumours with CENTRAL NECROSIS (ie RIM ENHANCING LESION on CONTRAST brain scan)
- disrupt blood-brain barrier -> associated with VASOGENIC OEDEMA
147
What medication can be used to treat brain oedema
Dexamethasone
148
Meningioma epid + pathophys
2nd most common ADULT PRIMARY brain tumour
- BENIGN (usually)
- Extrinsic tumours
- Arise from ARACHNOID CAP CELLS of meninges
- Typically located next to DURA
- at FALX CEREBRI, Superior sagittal sinus, convexity or skull base
- Sx from compression of parencyma
149
Meningioma Ix + Tx
- CT with contrast (will show enhancement)
- MRI
- Tx with observation, radiotherapy / surgical resection
(If histology taken (not done in clinical practice) -> Spindel cells in concentric whorls + calcified psammoma bodies)
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Vestibular schwannoma / acoustic neuroma
- BENIGN tumour arising from CN8
- oft seen in CEREBELLOPONTINE ANGLE
- Px:
- Hearing loss
- TInnitus
- Bells palsey (via compression as both CN 7 + 8 are in same sheath)
BILATERAL vestibular schwannomas associated with NEUROFIBROMATOSIS TYPE 2
151
Myotonic dystrophy
AUTOSOMAL DOMINANT tinucleotide repeat disorder
- multisystem inherited disorder characterised by progressive muscle wasting + weakness
2 main types: type 1 and 2 (DM1 + DM2)
152
Myotonic dystrophy epid
commonly presents in **20s** but can present at any age (is autosomal dominant so hereditary)
153
Myotonic dystrophy pathophys
- type 1 = CTG repeat on DMPK (a protein kinase gene) on chromosome 19
- type 2 = repeat expansion of ZNF9 gene on chromosome 3
Creates abnormal protein which disrupts muscle-specific CHLORIDE CHANNALS which affects skeletal, cardiac and smooth muscle
154
Myotonic dystrophy Px
- Face:
- Frontal balding
- Myopathic facies (long thin face)
- Bilateral ptosis
- Cataracts
- Neck - wasting of sternocleidomastoid
- DYSARTHRIA (from myotonic tongue + pharyngeal muscles)
- Dysphagia
- Hands:
- Slow relaxing grip
- Percussion myotonia (thumb flexes on percussion of thenar eminence)
- Internal:
- Insulin resistance / metabolic syndrome
- Cardiomyopathy / arryhthmia
- Testicular atrophy
- mild mental impairment
Muscles:
- Distal weakness more prominent in type 1
- initially distal in both type 1 + 2
- Proximal weakness more prominent in type 2
155
Myotonic dystrophy Ix + Mx
Genetic analysis
- Sx control
- Physio / OT
- Meds
- Prevent complications
- Regular screening for heart disease + diabetes
156
Statin induced myopathy
Statin use can cause myalgia, myositis, rhabdomyolysis + asymptomatic increase in CK
- low incidence (1.2 / 10000 person years in the 90s)
157
Px of statin induced myopathy
- Fatigue, myalgia / tenderness
- Muscle weakness
- Nocturnal cramping
- Tendon pain
Tend to be PROXIMAL, GENERALISED and worse with EXERCISE
All occuring within months of starting statins (unlikely to be caused by statins if been taking for years)
158
Ix + Mx of statin induced myopathy
- check CK
- check TSH
- if CK >10x the upper limit of normal -> check renal function + urine myoglobulin as this is Rhabdomyolysis
If Sx tolerable -> continue statin w/ frequent monitoring
If Sx intolerable -> stop statin
- consider repeat statin challange / alt drug option
If Sx / raised CK persists after stopping statin -> EMG + muscle biopsy
159
Myositis vs Rhabdomyolysis
- Myositis = CK is higher than normal but <10x the upper limit of normal
- Rhabdomyolysis = CK >10x upper limit of normal
e.g. if upper limit of normal is 10: 10-99 = Myositis; >100 = rhabdomyolsysis
160
Causes of myopathy
- Inflammation (polymyositis)
- Inherited (Duchenne, Becker, myotonic)
- Endocrine (Cushings, thyrotoxicosis)
- Alcohol
- Drugs (steroids - causes Cushing's syndrome, statins)
- Infection (polio)
161
1st line Tx for diff types of seizures
- Tonic clonic = **sodium valproate** / Laotrigine
- Myoclonic = Sodium valproate (or levetiracetam)
- Absence = **Ethosuximide** (sodium valproate 2nd line)
- Focal = **Lamtotrigine** / Levetiracetam
- Atonic / tonic = Sodium valproate / lamotrigine
Carbemazepine, topiramate, and phenytoin are not 1st line for anything
162
Tx for status epilepticus
In hospital:
- IV lorazepam
- seek expert advice
- IV lorazepam again if seizure doesn't stop 5-10 minuts after 1st dose
- Still no response:
- levetiracetam, phenytoin or sodium valproate bolus
- Still no response:
- expert advice + consider phenobarbital / general anaesthesia
Patient's may have ann individualised emergancy Mx plan - in which case just follow what it says in the plan
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Side effects of Sodium Valproate
- Ataxia
- Anaemia
- Confusion
- Gastric irritation
- Haemorrhage
- Hyponatraemia
- Tremor
- Weight gain
- Teratogenicity
164
Side effects of lamotrigine
- Blurred vision
- Arthralgia
- Ataxia
- Dizziness
- Headache
- Insomnia
- Diarrhoea
- Rash
- Tremor
165
What is included in a confusion screen?
Bloods:
- FBC (infection, anaemia)
- U+E (electrolytes)
- LFTs (hepatic encephalopathy)
- Coag / INR (intracranial haemorrhage)
- TFTs (hypothyroidism)
- **Calcium** (hypercalcaemia)
- **B12 + folate / haematinics**
- **GLUCOSE**
- Blood cultures (sepsis)
**Urinalysis** (NB - +ve dipstick alone is not enough for UTI diagnosis in elderly)
**Imagining**:
- CT head (bleeds, stroke, abscess etc)
- Chest X-ray
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Non-epileptic attack disorder (NEAD)
AKA functional / dissociative seizures
Belived to be triggered by significant STRESS (emotional or physical) - tho not necessarily when the patient is actively feeling stressed
- may proceed specific traumatic event
- may gradually accumulate over time (sort of like c-PTSD)
May be idiopathic
Often occur from rest
Main complication is the psychosocial impact
167
NEAD Dx + Mx
Dx via thorough Hx + EEG to rule out underlying physical pathology
- videos are always useful
Mx = psychotherapy:
- CBT
- Grounding / distraction techniques
- Eye movement desensitization and reprocessing (EMDR) - if trauma triggers being investigated
May use anti -anxiety / -depressants as these are common co-morbs
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Post-herpetic neuralgia meaning
- Pain at site of healed shingles infection - can Px with:
- Neuropathic pain (burning, pins + needles)
- Allodynia (perception of pain from normally non-painful stimulus)
169
Post-herpetic neuralgia Mx
Conservative:
- Soft fabrics (e.g. silk)
- Compression / covering of area
- Cold packs (can make allodynia worse)
- Pain diary to better manage
Pharm (v important to titrate carefully):
- Mild-mod = paracetamol / co-codamol
- Uncontrolled = neuropathic pain meds (amytriptyline, gabapentin, pregabalin) + titrate as able
- Consider topical capsaician cream / lidocaine plasters (if mild; concerned about systemic SE of meds in old ppl; as an adjucnt to oral therapy)
- Tramadol ONLY for acute rescue Tx
Follow-up after every dose change / new med
Consider referral to specialist pain service / CBT if severe pain that significantly affects ADLs
170
Characteristic Px of ALS
- ASYMMETRIC limb weakness
- WASTING of small muscles in hand / tibialis anterior
- Fasiculations
- mixed UMN, LMN Px
NB:
- there are **no sensory Sx** (tho might get vague limb pain in early disease)
- **NO external OCULAR muscle involvement**
- NO CEREBELLAR signs
- **Abdo reflexes + sphincter function remains intact** - tho might go in very late severe disease
171
Guillain Barre syndrome Px
- Progressive ascending weakness (tends to affect proximal muscle more tho) affecting all 4 limbs
- SYMMETRICAL
- Sensory Sx tend to be mild (some paresthesia / pain)
- May present with BACK PAIN initially
- Hx of GI Sx
- AREFLEXIA
- Cranial nerve involvement (DIPLOPIA, FACIAL weakness)
- Autonomic Sx:
- URINARY RETENTION
- DIARRHOEA / Ileus
- Arrhythmias
(May get papilloedema 2ndry to reduced CSP resorption)
172
GBS pathophys
- Cross reaction of ANTIBODIES with GANGLIOSIDES (mediate cell communication - most abundant in nervous system)
- anti-GM1 (anti-ganglioside Ab) in 25% of pts
Thought to be caused by molecular mimicry
173
GBS Ix + Dx
- Lumbar puncture
- Rise in PROTEIN with NORMAL WCC (**albuminocytological dissociation**) found in 66% + NOMRAL GLUCOSE (ie raised protein but no other acute signs of infection)
- Can do nerve conduction studies
- REDUCED SIGNAL
Diagnosed clinically via Brighton criteria and confirmed with Ix
174
Guillain Barre syndrome definition
Acute Ascending Paralytic POLYNEUROPATHY affecting PERIPHERAL nerves
175
Pathogens commonly associated with GBS
- Campylobacter jejuni
- Cytomegalovirus
- Epsetin Barr virus
176
Natural history of GBS
- Sx within 4 WEEKS of trigerring infection
- Symptoms PEAK within 2-4 WEEKS
- Recovery period = MONTHS - YEARS
177
Brighton criteria for GBS
- **FLaccid limb weakness**
- AREFLEXIA
- Monophasic course lasting less than 28 days
- **Absence of alternative diagnosis**
- **Albuminocytological disassociation**
- CSF cell count <50/ml
- CSF protein conc >60 mg/dL
- Suggestive findings on Electrophysical studies (e.g. reduced signal on nerve conduction)
178
GBS Mx
- IVIg
- Plasma exchange
- REGULAR FVC + supportive care as required
- may need intubation, ventilation and ICU admission if go into resp failure
- VTE prophylaxis
179
What factors can lead to worse prognosis in GBS
- age > 40
- Poor upper extermity muscle stregth
- Hx of diarrhoeal illness (esp from campy)
- high-anti GM1 Ab titre
- NEED FOR VENTILATORY SUPPORT
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181
Risk factors for recurrence of febrile convulsions
- age of onset **< 18 months**
- SHORTER DURATION of fever (<1 hour)
- Relatively low grade fever (<40 C)
- complex convulsions (multiple seizures during same illness)
- FHx in 1st degree relative
182
Red flags suggestive of CNS infection
- Complex febrile seizures
- HISTORY of LETHARGY, IRRITABILITY or DECREASED FEEDING
- Physical signs of mening/encephalitis (typical signs may be absent in babies)
- Previous/current treatment with Abx
- Incomplete immunisation in kids 6 - 18 months against HiB and Strep Pneum
183
*Relative* contraindications to thrombolysis in ischaemic stroke
- Previous stroke / head injury in last 3 months (underlying cause)
- Rapidly improving NIHSS (National Institute of Health Stroke Scale) score (recovering by self)
- Seizure at onset of symptoms (epileptiform vs stroke)
- Lumbar puncture or arterial puncture at non-compressible site in last 7 days (bleeding risk)
184
*Absolute* contraindications to thrombolysis for ischaemic stroke
- INR >1.7
- Past history of intracranial haemorrhage
- Major surgery in the last 14 days
- GI or urinary tract bleeding in the last 21 days
- Suspicion of subarachnoid haemorrhage, even if CT normal
- Uncontrolled blood pressure >185 sysolic and/or >110 diastolic
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