Neuro Flashcards
1
Q
Define a headache
A
Pain or discomfort between the orbits and the occiput, arising from pain-sensitive structures
2
Q
List 4 intracranial pain-sensitive structures
A
- Venous sinuses
- Cortical veins
- Basal arteries
- Dura of anterior, middle and posterior fossae
3
Q
List 6 extracranial pain-sensitive structures
A
- Scalp vessels and muscles
- Orbital contents
- Mucous membranes of nasal and paranadal spaces
- External and middle ear
- Teeth
- Gums
4
Q
Examination of headache?
A
Full general examination
- Ocular - acuity, tenderness, stabismus
- Teeth and scalp
- Percussion over frontal and maxillary sinuses
- Fever?
5
Q
Primary causes of headache?
A
- Migraine
- Tension
- Cluster
6
Q
Secondary causes of headache?
A
- Meningitis
- Subarachnoid haemorrhage (SAH)
- Medication overuse
- Raised ICP
- Low CSF pressure
- Trigeminal neuralgia
- GCA
7
Q
What is the most common type of headache in children?
A
A headache accompanying febrile illness or infection of the nasal passages or sinuses
8
Q
What red flags are you looking for in children and what could these infer?
A
- neck stiffness
- impaired conscious level
–> meningitis, encephalitis or cerebral abscess
9
Q
What would acute obstructive hydrocephalus and a headache suggest to you in a child?
A
Intracranial tumour, occurring in the midline (medulloblastoma, pineal region tumour)
10
Q
Red flags for urgent investigation of a headache?
A
- New headache with history of cancer
- Cluster headache (pituitary)
- Seizure
- Significantly altered consciousness levels, memory loss, confusion, co-ordination changes
- Papilloedema
- Neck stiffness
11
Q
Duration of tension headaches?
A
30 mins to 7 days
12
Q
Frequency of tension headaches?
A
- Infrequent or daily
- Worse towards end of the day
- May persist over years
13
Q
Mechanism of tension headache?
A
- ‘Muscular’
- Due to persistent contraction, e.g. clenching teeth, head posture, furrowing of brow
- Some overlap with transformed migraine
14
Q
Treatment of tension headache?
A
- Reassurance
- Short term analgesia but reduce over-use
- Stress relief
- Amitriptyline and other tricyclic antidepressants
- Beta-blockers
15
Q
Characteristics of tension headaches?
A
- Bilateral
- Non-pulsatile
- Diffuse, dull, aching
- Band-like
- Worse on touching scalp
- May be aggravated by noise
- ASSOCIATED WITH DEPRESSION and anxiety
- NOT aggravated by physical activity
16
Q
Define migraine
A
A common, often familial disorder characterised by UNILTERAL THROBBING HEADACHE
- aggravated by physical activity
17
Q
Onset of migraine?
A
Childhood or early adult life
18
Q
Incidence of migraine?
A
5 - 10% of population
19
Q
Who do migraines affect more?
A
Females
20
Q
Family history of migraines?
A
70%
21
Q
What are the two forms of migraine?
A
Migraine WITH aura, and migraine WITHOUT aura
Aura = visual, sensory of motor type followed by headache - throbbing, unilateral, worsened by bright lights, relieved by sleep, associated with nausea and sometimes vomiting
Without aura can sometimes sound like a tension headache!
Can also get aura by itself.
22
Q
Duration of migraines?
A
2 - 72 hours, rarely occur more frequently than twice a week
23
Q
What are the two types of migraine with aura?
A
- Basilar: bilateral visual symptoms, unsteadiness, dysarthria, vertigo, limb paraesthesia. Possible loss of consciousness. Affects young women.
- Hemiplegic: aura of unilateral paralysis which persists for some days after settling of headache. Often misdiagnosed as stroke. Dominant inheritance is noted.
24
Q
Triggers of migraine?
A
CHOCOLATES: C: Chocolate H: Hangovers O: Orgasm C: Cheese/caffeine O: Oral contraceptive pill/premestrual L: Lie ins A: Alcohol/anxiety T: Travel E: Exercise S: Stress
25
Management of migraines?
Identify and avoid triggers
Acute attacks:
- Simple analgesics (aspirin) with metoclopramide
- Anti-emetics (domperidone/prochlorperazine)
- Sumatriptan and other triptans (PO or SC)
- Ergotamine
- Methylprednisolone
Prophylaxis:
- Propranolol (NOT in asthmatics)
- Topiramate
- Pizotifen
- Verapamil
26
What is a medication overuse headache?
When some patients with episodic tension or migraine have headaches all the time so they start taking regular medication. This overuse itself can lead to worse headaches.
Using meds 10-15 days in a month.
27
Which drugs commonly cause drug overuse headaches?
- Analgesia - mixed (paracetamol + codeine/opiates)
- Triptans
- Ergotamine
28
Management of drug overuse headaches?
Stop the medication!
29
What is a transformed migraine?
When patients with migraine go on to develop chronic daily headaches without overusing medication. Usually responds to migraine prophylactic agents.
30
What is a cluster headache?
- The most disabling primary headache
- Caused by a pituitary tumour
- More common on middle age men
- May be precipitated by alcohol
31
Duration of cluster headaches?
- The headache itself lasts 15 mins - 2 hours
- Clusters last 4-12 weeks
- Then followed by pain-free months or 1-2 years before next attack
32
Mechanism of cluster headaches?
- Superficial temporal artery smooth muscle hyper-reactivity to 5-HT
- Hypothalamic grey matter abnormalities
- Autosomal dominant gene
33
Diagnosis of cluster headached?
At least 5 headaches fulfilling:
- Severe or very severe UNILATERAL ORBITAL, SUPRAORBITAL or TEMPORAL pain lasting 15 - 180 mins
- Accompanied by IPSILATERAL cranial autonomic features and/or agitation or restlessness
- Can be from 1 every other day to 8 a day
- No attributed to another disorder
- "Alarm clock headache": wakes them at night
Extra info:
- May have watery red eye and vomiting
- May be pacing and restless
34
Management of cluster headaches?
ACUTE:
- 100% O2 for 15 mins through non-rebreathable masks
- Sumatriptan SC at onset
PREVENTION:
- Suboccopital steroid injections
- Intravenous CIVAMIDE
- Veramapril
35
Define trigeminal neuralgia
- Chronic pain disorder that affects the third branch of the trigeminal nerve = MANDIBULAR
- Believed to be due to demyelination of the trigeminal nerve
36
Causes of trigeminal neuralgia?
- Inflammation
- Aneurysm
- Tumour!!
37
Triggers for trigeminal neuralgia?
- Shaving
- Washing
- Eating
- Talking
- Dental prostheses
38
Signs and symptoms of trigeminal neuralgia?
At least 3 attacks of UNILATERAL facial pain fulfilling:
- Occurring in 1 or 2 distributions of the trigeminal nerve
- Pain has at least three of: reoccurring paroxysmal attacks from seconds - 2 mins; severe intensity; electric-shock-like; precipitated by stimulus to face
39
Management of trigeminal neuralgia?
Carbamazepine
OR Lamotrigine
If meds fail -> microvascular decompression
40
What is giant cell arteritis?
| Describe its epidemiology?
- Autoimmune disease of unknown cause
- Throbbing headache with general malaise
- Common in the ELDERLY
- Associated with POLYMYALGIA RHEUMATICA
- Secondary causes: SLE, RA, HIV
Consider in all OVER 50s with HEADACHE!!!
41
Signs and symptoms of GCA?
- Headache (gradual onset)
- Temporal artery = tender and thickened but non-pulsatile
- Tender scalp
= JAW CLAUDICATION
- Amaurosis fugax (blind drawing down) or diplopia
42
Diagnosis of GCA?
- Raised ESR and CRP
- Raised platelets and alkaline phosphatase
- Low Hb
- Temporal artery biopsy: within 7 days of steroids
43
Mechanism of GCA?
- Large and medium-sized arteries undergo 'giant cell' infiltration with fragmentation of the lamina and narrowing of the lumen
- Results in distal ischaemia as well as stimulating pain sensitive fibres
- Occlusion of important end arteries, e.g. ophthalmic
44
Treatment of GCA?
URGENT PREDNISOLONE: 60 mg daily
if complications already occurred -> give parenteral high dose steroids
Maintenance steroids = 5 mg daily: monitor the ESR to get down to maintenance dose
45
Characteristics of a raised ICP headache?
- Aggravated by bending/coughing
- Worse in morning upon awakening or may awaken patient
- Severity progresses gradually
- Vomiting in later stages
- Transient loss of vision when sudden change in posture
- Papilloedema
- Focal signs
MRI or CT = Essential!
46
Describe sinus headache
- Well localised, worse in morning, affected by posture
- XR: opacified sinuses
- Treatment: decongestants or drainage
47
Describe ocular headache
- Refraction errors may result in 'muscle contraction' headaches
- Resolves when corrected with glasses
- Acute glaucoma can produced headache but accompanied by misting of vision
48
Describe dental disease headaches
- Discomfort localised to teeth
- Check for malocclusion
- Check temporomandibular joints
49
GCS
Eye opening:
1 = none; 2 = to pain, 3 = to speech, 4 = spontaneous
Verbal response:
1 = none; 2 = incomprehensible, 3 = inappropriate words, 4 = confused speech, 5 = orientated
Motor response:
1 = none; 2 = extension to pain, 3 = flexion to pain, 4 = withdraw from pain, 5 = localises pain, 6 = obeys commands
Max score = 15, Min score = 3
50
Definition of a stroke
Rapid onset focal CNS signs and symptoms, with presumed vascular origin, lasting more than 24 hours
51
Two types of stroke and how common is each?
Ischaemic (85%) - Infarct-thromboembolism
Haemorrhagic (15%) - Bleeding:
- Hypertension (BERRYS ANEURYSM)
- Lobar
52
Causes of stroke?
- Small vessel occlusion
- Cardiac emboli (AF/prosthetic valve/MI)
- Atherothromboembolism
- CNS bleeds
53
Risk factors for stroke?
- HTN
- Smoking
- Alcohol excess
- DM
- Heart disease
- PVD
- Previous TIA
- Carotid bruit
- Combined pill
- Hyperlipidaemia
- Increased clotting
- Syphilis
- Age and sex
54
Diagnosis of stroke
- URGENT CT HEAD: rule out haemorrhage before thrombolysis
- Pulse, BP and ECG - look for AF
- FBCs - look for thrombocytopenia and polycythaemia
- Glucose - rule out hypoglycaemia
55
Describe CT scans and how different materials present. What would you see in each stroke?
WHITE: Fresh blood, calcium, bone
DARK: Fluid (e.g. CSF), air, fat (e.g. lipomas)
Haemorrhagic stroke: fresh blood = bright white! White -> Isodense -> hypodense
All stroke eventually goes DARK (subacute - chronic stage) as the space get filled with CSF
56
List some general management of an acute stroke to maximise reversible ischaemic tissue
- Hydration
- Oxygenation (> 95%)
- Consider antihypertensive if BP > 185/110 (20% drop may compromise cerebral perfusion)
- Maintain glucose between 4 - 11 mmol/l
- Treat chest infections and cardiac failure/dysrhythmias
57
Describe thrombolysis
- Intravenous recombinant tissue plasminogen activator (ALTEPLASE)
- Give within 4.5 hours of anterior circulation ischaemic stroke
- Start antiplatelet therapy 24 hrs after thrombolysis (CLOPIDOGREL)
58
CIs of thrombolysis?
- Uncertain time of onset
- Spontaneously improving
- Head injury or previous stroke in last 3 months
- GI surgery in last 21 days
- BP > 180/110
- On an anticoagulant
- Seizure
- Hypodensity on CT
59
Management of stroke if thrombolysis not an option?
- 300 mg ASPIRIN daily for 2 weeks (or Clopidogrel if CI)
- Then lifelong Clopidogrel
- Anticoagulants should be avoided if possible as increase risk of haemorrhagic transformation
60
Non-medical management of stroke?
- Transfer to stroke unit
- Assess swallow (SALT)
- Early mobilisation
- Physiotherapy
- Home modifications
61
Management of haemorrhagic stroke?
- Frequent GCS monitoring
- Anitplatelets = contraindicated
- Any anticoagulation should be reversed
(e. g. Beriplex or Vit K for warfarin)
- Control hypertension - BETA-BLOCKERS
- Manual decompression if raised ICP
- Or diuretics to reduce ICP (MANNITOL)
- Maybe surgery
62
How to calculate stroke risk (post-TIA)?
ABCD2
1-3: 0.4%
4-5: 12%
6: 31.4%
HIGH RISK IF:
- ABCD2 > 4
- >1 TIA in previous 7 days
- New arrhythmia
- Carotid stenosis
63
Prevention of stroke?
- Lifelong platelet treatment if already had stroke: Aspirin + Dipyridamole + Clopidogrel
- Cholesterol treatment, e.g. SIMVASTATIN
- AF treatment, e.g. WARFARIN
- BP treatment, e.g. RAMIPRIL
64
Define meningitis
Inflammation of the meninges (membrane of the brain and spinal cord)
65
Common meningitis organisms?
Common:
- Meningococcus (Neisseria meningitides): Gram-negative bacteria, diplococcus
- Pneumococcus (streptococcus pnuemoniae): Gram-positive bacteria, diplococcus
- Listeria monocytogenes (In immunocompromised and babies): Gram-positive bacteria, Bacilli
NEONATES: GBS
Others:
- E.coli, Haemophilus influenzae, TB, cytomegalovirus, cryptococcus
66
Clinical presentation of meningitis?
EARLY:
- Headache
- Leg pains
- Cold hands and feet
- Abnormal skin colour
LATE:
- MENINGISM - NECK STIFFNESS, PHOTOPHOBIA, +VE KERNIGS SIGN
- Reduced consciousness
- Coma
- Seizures
- Focal CNS signs
- Papilloedema
- Brudzinski's sign
- Petechial rash (non-blanching)
67
What is Kernig's sign?
With hip at 90 degrees, can't straighten leg > 135 degrees without pain
68
What is Brudinski's sign?
Flex patients neck -> patient compromised by flexing hips and knees
69
What is different in the presentation in viral meningitis?
Does not present with a rash!
70
Diagnosis of meningitis?
- Bloods: FBC, UE, LFT, glucose, coagulation screen, lactate
- Blood cultures: before antibiotics
- Throat swabs
- CT head - check for space-occupying lesions and ICP
- Lumbar puncture - If not sign of ICP
LP:
- Opening pressure is HIGH
- Send CSF for gram stain, MCS, protein, glucose, vriology/PCR, lactate
- CSF in bacteria: high neutrophils, high protein, low glucose
- CSF in viral: high lymphocytes, high or normal protein, normal glucose
- If septic --> do NOT attempt LP
71
Treatment of meningitis?
```
MEDICAL EMERGENCY if any 2 of:
- Pyrexia
- Headache
- Neck stiffness
- Altered mental state
Give immediate IV FLUIDS and IM/IV BEN PEN
```
Immediate antibiotics:
- If < 55yrs --> CEFOTAXIME
- If > 55yrs or IC --> CEFOTAXIME and AMPICILLIN
- If returned traveller --> VANCOMYCIN
- If listeria --> add AMOXICILLIN
If viral --> ACYCLOVIR
72
How would you manage a close contact of a meningitis patient?
PROPHYLAXIS:
- 1st line = CIPROFLOXACIN
- 2nd line = RIFAMPICIN - NOT in pregnancy
73
What is meningococcal septicaemia?
Meningococcal septicaemia is the cause of the classic “non-blanching rash” that everybody worries about
This rash indicates the infection has caused:
- Disseminated intravascular coagulopathy (DIC)
- Subcutaneous haemorrhages
74
Describe the facial nerve pathway
- Exits brainstem at cerebellar pontine angle
- passes through the TEMPORAL BONE and PAROTID GLAND
- Divides into 5 branches:
o Temporal
o Zygomatic
o Buccal
o Marginal mandibular
o Cervical
75
Describe the function of the facial nerve
Motor, Sensory and Parasympathetic nerve supply
76
Describe motor function of facial nerve
- Supplies muscles of facial expression
- Stapedius of inner ear
- Posterior digastric, stylohyoid and platysma muscles of the neck
77
Describe sensory function of facial nerve
Taste from ANTERIOR 2/3rds of tongue
78
Describe parasympathetic supply of facial nerve
- Supply to SUBMANDIBULAR and SUBLINGUAL salivary glands
| - Supply to LACRIMAL gland (tear production)
79
Upper vs lower motor neuron facial palsy?
UPPER spares UPPER
| - Must refer anyone with UMN facial palsy urgently with suspected stroke
80
Name some UMN facial nerve lesions
```
Unilateral:
- Cerebrovascular event, e.g. stroke
- Tumours
Bilaterally:
- Pseudobulbar palsies
- MND
```
81
What is Bells and what is the epidemiology?
- Idiopathic
- Unilateral
- Peak = 20 - 40 yrs
- More common in pregnant women
- LMN so forehead involvement
- May also notice altered taste, dry eyes, hyperacusis
82
How would you manage Bells palsy?
- Most recover fully within few weeks though can take 12 months and some will have residual weakness forever
- If presents within 72 hours: PREDNISOLONE
- Lubricating eye drops
- If eye pain -> review for exposure keratopathy
83
What is Ramsay-Hunt syndrome?
- Caused by herpes-zoster virus
- Painful and tender vesicular rash in ear canal, pinna and around ear on affected side
- Rash may extend to anterior 2/3rds of tongue
84
Management of Ramsay-Hunt syndrome?
Ideally within 72 hours
- Prednisolone
- Acyclovir
- Lubricating eye drops
85
What is encephalitis?
Inflammation of the brain matter itself
| - Typically affects the temporal and inferior frontal lobes
86
What are the common causes of encephalitis?
```
VIRAL:
- HSV 1 and 2 and varicella-zoster
(HSV1 responsible for 95% in ADULTS)
- HIV/EBV/measles/mumps/rabies/jap enc/arbovirus
NON-VIRAL:
- Bacterial meningitis
- TB
- Malaria
- Listeria
- Lyme disease
- Legionella
```
87
Sign and symptom of encephalitis?
- Fever
- Headache
- Lethargy
- Seizures
- Off behaviour
- Psychiatric symptoms
- Vomiting
- Focal neurological features, e.g. aphasia
88
Investigations and results for encephalitis?
- LP/CSF: lymphocytosis and elevated protein; low/normal glucose
- viral PCR for HSV
- CT: Petechial haemorrhages - white areas of enhancement in temporal and inferior frontal lobes. Normal in 1/3. Do before LP.
- MRI is better
- EEG: lateralised periodic discharges at 2 Hz
89
Management of encephalitis?
- IV ACYCLOVIR within 30 mins of admission for 2 weeks
| - Should be started in all cases of suspected encephalitis
90
What is hydrocephalus?
- An excessive volume of CSF within the ventricular system of the brain
- Caused by an imbalance between CSF production and draining or absorption
91
Presentation of hydrocephalus in adults?
Symptoms of raised ICP:
- Headache (Raised ICP)
- Nausea and vomiting
- Papilloedema
- Coma (in severe cases)
92
Presentation of hydrocephalus in children?
- Bulging and tense anterior fontanelle
- Increased head circumference
- Failure of upward gaze ('sunsetting eyes') due to compression of the superior colliculus of the midbrain
- Poor feeding and vomiting
- Poor tone
- Sleepiness
93
Two categories of hydrocephalus? The pathology of each?
Obstructive (non-communicating)
- Structural pathology blocking the flow of CSF
- Dilatation of the ventricular system is seen superior to site of obstruction
- Causes: tumours, acute haemorrhage, developmental abnormalities (e.g. AQUEDUCTAL STENOSIS = most common or Arnold-Chiari malformation)
Non-obstructive (communicating):
- Imbalance of CSF production/absorption
- Either increased production of CSF (e.g. choroid plexus) but RARE
- OR failure of reabsorption at the arachnoid granulations, e.g. meningitis or post-haemorrhage (MORE COMMON)
94
Investigations in hydrocephalus?
- CT head = FIRST LINE
- MRI offers more detail - useful is suspected underlying lesion
- LP:
o ONLY if NON-OBSTRUCTIVE due to risk of HERNIATION
o Diagnostic and therapeutic as can allow you to sample CSF, measure opening pressure, and drain CSF to reduce pressure
95
Management of hydrocephalus?
- External ventricular drain (EVD) in acute, severe cases. Inserted into R lateral ventricle.
- Ventriculoperitoneal shunt (VPS) = long-term CSF diversion technique - drains CSF from ventricles to the peritoneum
- OBSTRUCTIVE: may need surgical treatment of the obstructing pathology
96
What are the complications of a VPS shunt?
- Infection
- Blockage
- Excessive drainage
- Intraventricular haemorrhage
- Outgrowing them (need replacing every 2 yrs in children)
97
What is normal pressure hydrocephalus?
- A form of non-obstructive hydrocephalus
- Characterised by large ventricles but normal ICP
- Classical triad = dementia, incontinence and disturbed gait (similar to Parkinson's)
- Reversible cause of dementia
98
Diagnosis of normal pressure hydrocephalus?
Imaging:
- enlarged fourth ventricle
- absence of substantial sulcal atrophy
99
Management of normal pressure hydrocephalus?
- VPS shunt
| - 10% experience significant complications, e.g. seizures infection and intracerebral haemorrhages
100
What is a subarachnoid haemorrhage (SAH)?
Bleeding into the subarachnoid space
101
Causes of SAH?
- MOST COMMON (85%) = BERRY ANEURYSM (associated with PKD, Ehler-Danlos and CoA)
- AV malformation (15%)
- Others: arterial dissection, mycotic infective aneurysms, vasculitis, encephalitis, tumours
102
Risk factors for SAH?
- HTN
- Smoking
- Excess alcohol
- Cocaine use
- Family history
- Black patients
- Female patients
- Age 45-70
- Sickle cell anaemia
- Neurofibromatosis
103
Presentation of SAH?
- Sudden-onset occipital headache: 'worst of my life', thunderclap headache, 'like being hit on the back of the head with a bat'
- Neck stiffness
- Photophobia
- Vision changes
- Neurological symptoms, e.g. weakness, seizures, LOC
- Nausea and vomiting
104
Investigation of SAH?
- CT = FIRST LINE: STAR SHAPE (blood in fissures), hyperattenuation in subarachnoid space
- LP: If CT is negative and no CIs. Must be done after 12hrs of headache onset.
o Red cell count will be raised (early on)
o Xanthochromia (yellow caused by bilirubin)
- Cerebral angiography once SAH confirmed to locate source of bleeding
105
Management of SAH?
Maintain cerebral perfusion
- Hydration
- Bed-rest
- Well-controlled BP (Systolic 160)
NIMODIPINE
- 21-day course
- Reduces vasospasm (cerebral artery)
Endovascular coiling
- Using catheter/angiography
- Blocks off the blood vessel
Sometimes requires surgical clipping
106
Complications of SAH?
- Rebleeding (10% of cases, < 12hrs usually)
- Vasospasm (delayed cerebral ischaemia): 7-14 days
- Hyponatraemia (due to SIADH)
- Seizures (treat with antiepileptics)
- Hydrocephalus (treat with LP or VPS shunt)
107
Associations of SAH?
- PKD
- Ehler-Danlos (or Marfans)
- CoA
- Sickle cell
- Cocaine use
108
Risk factors for intracranial bleeds?
- Head injury
- HTN
- Aneurysms
- Ischaemic stroke can progress to haemorrhage
- Brain tumours
- Anticoagulants, e.g. Warfarin
109
Define a subdural haemorrhage (SDH)
CONSIDER IN ALL WITH FLUCTUATING CONSCIOUSSNESS
- Rupture of the bridging veins between the cortex and venous sinuses
- Results in haematoma between the dura and arachnoid mater
- Gradually rising ICP
- Midline structures shift away from the side of the clot
110
Risk factors for SDH?
- ELDERLY and ALCOHOLICS: Have cerebral atrophy which increases tension on cerebral veins
- Infants have fragile bridging so at risk (shaken baby syndrome)
- Trauma
- Falls
- Anticoagulation
111
Presentation of an SDH?
- Fluctuating consciousness level
- Sleepiness
- Headaches
- Personality/mood changes
- Raised ICP
- Seizures
- Localising neurological symptoms
- Vomiting
Sometimes get LATENT PERIOD: after head injury. 8-10 weeks later, clot breaks down --> increased oncotic pressure --> water sucked into hematoma by osmosis
112
Diagnosis of SDH?
- CT/MRI scan: CRESCENT SHAPE, clot and midline shift, not limited by suture lines
113
Treatment of SDH?
- 1st line = Clot evacuation (If develops weeks after a head injury)
- Irrigation/burr hole
- 2nd line = Craniotomy (if develops soon after a head injury)
Minor: observed conservatively, may need decompressive craniectomy
114
What is an extradural haemorrhage (EDH)?
- Collection of blood between SKULL and DURA
115
Where is an EDH most common and why?
Often in the temporal region (thin skull overlying MIDDLE MENINGEAL ARTERY)
116
Causes of EDH?
- Fractured temporal/parietal bone (middle meningeal artery) - typical after trauma to temple just lateral to eye
- Tear in dural venous sinus
- Rapid rise in ICP --> ventricles get rid of CSF to prevent this rise
117
Sign and symptoms of EDH?
- Fall in consciousness
- Followed by lucid interval (temp. improvement)
- Severe headache
- Vomiting
- Confusion
- Brisk reflexes
- Seizures
- Late signs: raised BP, bradycardia
118
What happens if left untreated and what later signs might you see if bleeding continues in an EDH?
Coning (squeezing of brain and brainstem through the foramen magnum because of swelling)
- Ipsilateral pupil dilates (compression of parasymp fibres of 3rd cranial nerve
- Coma deepens
- Bilateral limb weakness
- Breathing = deeper and irregular (brainstem compression)
- -> Death
119
Diagnosis of EDH?
- CT: LENS-SHAPED (round). Limited to suture lines of skull.
| - XR: Fracture lines crossing the course of middle meningeal artery
120
Treatment of EDH?
- Clot evacuation
- Ligation of bleeding
- Decrease ICP
121
Define coma
Unconsciousness = not aware and not rousable
122
Commom causes of coma?
- Drugs/toxins (opiates/EtOH/CO)
- Anoxia (post-arrest)
- Mass lesions (bleeds - head injury)
- Infections (bacterial meningitis)
- Metabolic (hypoglycaemia/DKA/uraemia)
- SAH
- Epilepsy
123
Uncommon causes of coma?
- Mass lesions (tumour)
- Venous sinus occlusions
- Hypothermia
- Psychiatric (catatonia)
124
History of an unconscious person?
- Collateral history from relatives/friends/ambulance/nursing staff/GP
- Previous events, e.g. suicide notes, travel history
- PMH: general, psychiatric, history of head injury, alcohol abuse,
- Drug history: recreational, insulin, antiepileptics
125
General examination of an unconscious person?
- Trauma - skull#, head injury
- Fever
- Hypothermia
- Breath odour (alcohol/ketoacidosis)
- Needle marks
- Stigmata of liver disease
- Evidence of convulsion
- Skin colour
- ASSESSMENT OF GCS
- Check for MENINGISM
126
Brainstem examination of an unconscious patient?
- Pupil size
- Pupil reactions
- Eye movements
- Corneal reflexes
127
Focal examination of an unconscious patient?
- Asymmetry of motor function?
- Tendon reflexes
- Plantar responses
128
Investigations in an unconscious patient?
- Bloods: FBC, UE, glucose, Ca, Phosphate, LFT, clotting, toxicology (incl. alcohol), ABGs, anion gap
- Imaging: CT head, MRI head, ECG
- LP
- EEG: Only if strongly indicated!
- Rarely: cortisol, TFTs
129
Differentials of sudden or subacute new headache?
- SAH
- Cerebral venous thrombosis
- Dissection: carotid/vertebral
- Infection: meningitis, encephalitis, cerebral abscess
- Acute haemorrhage
130
Management of acute headache?
- Need to know when it escalated to 10/10 pain
- 2 commonly used treatments:
o Surgical clipping
o Endovascular coiling
131
Define status epilepticus
Official definition: Persistent seizure activity for 30 mins+
- Continuous
- Intermittent attacks without the recovery of consciousness
Practical definition: convulsive seizure activity for > 10 mins
= MEDICAL EMERGENCY
132
Causes of status epilepticus?
- Anticonvulsant withdrawal (21%)
- Cerebrovascular disease (21%)
- Alcohol withdrawal (18%)
- Metabolic disorders (13%)
- Haemorrhage (7%)
- Infectious disorders (4%)
- Hypotension (4%)
- Tumours (3%)
- Anoxia (2%)
133
Why is status epilepticus a medical emergency?
Because if the seizure activity is prolonged, it can lead to irreversible brain damage
134
Management of status epilepticus?
- ABCDE + GLUCOSE (oxygen, airway adjunct, IV access)
- 1ST LINE: BENZODIAZEPINES
e. g. Lorazepam IV 4 mg, repeated after 10 mins if continues
- If seizures persist: PHENYTOIN or PHENOBARBITAL IV infusion
- If not response within 45 mins - general anaesthetic (e.g. Propofol)
135
List 5 neurological emergencies
1. Coma
2. Sudden/subacute new headache
3. Weakness
4. Visual loss
5. Status epilepticus
136
Basic management of neurological emergencies?
ABCDE(f)G
(DEFG = Don't Ever Forget Glucose)
```
A: Airway
B: Breathing
C: Circulation
D: Deficits
E: Environment (hypo/hyperthermic)
(f)
G: Glucose
```
137
What is myasthenia gravis?
AUTOIMMUNE disease against NICOTINIC ACETYLCHOLINE RECEPTORS in the neuromuscular junction --> reduced muscle contraction (85%)
In 15% of those with myasthaenia gravis:
- Muscle-specific kinase
- Low-density lipoprotein receptor-related protein 4 (LRP4)
138
Presentation of myasthenia gravis (MG)?
- WEAKNESS: Worse with muscle use, improves with rest (better in the morning)
Mainly in head and neck:
- Extraocular muscles --> diplopia
- Eyelid muscles --> partial ptosis
- Facial muscles --> reduced facial movements (snarly smile)
- Bulbar muscles --> swallowing/chewing problems and slurred speech
139
What does MG only cause partial ptosis?
Because the levator palpebrae superioris is 50/50 smooth muscle and striatal muscle
140
Examination of MG?
- Repetitive blinking to elicit ptosis
- Count to 50 to elicit speech tiring
- Upward gazing to elicit double vision
- Repetitive adduction of arm X20 will elicit unilateral weakness
- Check for thymectomy scar
- Can check FVC to check breathing muscle strength
141
Epidemiology of MG?
Peak in women < 40 yrs
| Peak in men > 60 yrs
142
Pathophysiology of MG?
- Autoimmune disease mediated by ANTIBODIES to the acetylcholine receptor (Anti-AChR)
- Interferes with neuromuscular junction via depletion of working post-synaptic receptor sites
- Both T-cell and B-cells are implicated
- Blocks the excitatory effect of ACh on the nicotinic receptors --> MUSCLE WEAKNESS
143
Diagnosis of MG?
ANTIBODIES:
- Anti-AChR in 85%
- Anti-MUSK in 10%
- Anti-LRP4 in <5%
CT/MRI of Thymus gland to look for hyperplasia, atrophy or tumour (thymoma)
Single fibre electromyography:
- High sensitivity (92-100%)
Edrophonium test:
- IV dose of Edrophonium Chloride
- Briefly and temporarily improve weakness
144
Management of MG?
Symptom control:
- Anticholinesterase inhibitors (ORAL PYRIDOSTIGMINE)
Immunosupression:
- ORAL PREDNISOLONE
Steroids may be combined with:
- ORAL AZATHIOPRINE or METHOTREXATE
Thymectomy:
- Removal of the thymus if onset <50yrs and poorly controlled
Monoclonal antibodies:
- Rituximab (B-cells)
- Eculizumab (C5 cells) (NOT on NHS)
145
What is a myasthenic crisis?
Severe and potentially life-threatening complication
- Acute worseing of symptoms
- Including RESPIRATORY muscles --> can lead to RESP FAILURE
- Causes: infection, natural disease cycle, under/over dosing on meds
146
Management of MG crisis?
- Urgent neurologist review
- Non-invasive ventilation with BiPAP
- Or full intubation and ventilation
- IV IMMUNOGLOBULINS
- PLASMAPHERESIS (plasma exchange and antibody removal)
147
Associations with MG?
- THYMOMAS (15%)
- Autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, RA, SLE
- Thymic hyperplasia (50-70%)
148
What is Guillian Barre syndrome (GBS)?
- An acute inflammatory demyelinating polyneuropathy --> SCHWANN CELLS
- Often triggered by an infection
149
Pathophysiology of GBS?
- Molecular mimicry
- B cells of the immune system create antibodies against pathogens that cause the preceding infection
- These antibodies also match the proteins on the myelin sheath of motor nerve cells or nerve axons
- Cross reaction of antibodies with gangliosides in the peripheral nervous system
- Correlation between the anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
- anti-GM1 antibody in 25% of patients
150
What are the most common causes of GBS?
- CAMPYLOBACTER JEJUNI
| - Also CMV, or EBV
151
Presentation and course of GBS?
- Few weeks after infection (~4 weeks)
- Symmetrical ascending muscle weakness
- Reduced reflexes
- May be peripheral loss of sensation or neuropathic pain
- May progress to cranial nerves and cause facial nerve weakness
- If autonomic dysfunction (involvement of heart): sweating, increased pulse, BP changes, arrhythmias
152
Investigations of GBS?
- Brighton criteria for a clinical diagnosis
Supportive investigations:
- Nerve conduction studies: SLOW conduction
- LP of CSF: high protein, normal WCC and glucose
(change in WCC would indicate a different diagnosis)
- Monitor FVC and ECG (resp failure and autonomic dysfunction are biggest GBS killers)
153
Management of GBS? And prognosis?
- IV IMMUNOGLOBULINS for 5 DAYS
- Plasma exchange (Plasmapheresis)
- If resp involvement --> ventilation and measure FVC
- 85% will make a good recovery, however, 10% are unable to walk after 1 year
154
What is myasthenic Lambert-Eaton syndrome?
- Progressive muscle weakness with increased use as result of damage to the NMJs
- Antibodies directed against the PRE-synaptic VOLTAGE-GATED CALCIUM CHANNELS
- Similar set of symptoms to MG but more insidious and less pronounced
155
What is LE syndrome associated with?
- Small cell lung cancer
| - Less so: Breast/ovarian cancer
156
Presentation of LE syndrome?
- Same as MG
- Proximal leg muscle weakness is the key feature
- Reduced reflexes
- Sometimes see an increase in muscle strength and normal reflexes (not seen in MG) = post-tetanic potentiation
- Autonomic features: dry mouth, impotence, difficult micturating
157
Management of LE syndrome?
- Tx of underlying cancer
- Immunosuppression: Prednisolone +/- Azathioprine
- AMIFAMPRIDINE
- Plasma exchange
158
What is cataplexy?
The sudden and transient loss of muscular tone caused by strong emotion, e.g. laughter, being frightened
- Around 2/3rds of patients with narcolepsy have cataplexy
- Features range from buckling knees to collapse
159
What is narcolepsy?
- Associated with HLA-DR2
- Associated with low levels of orexin (hypocretin) - a protein responsible for controlling appetite and sleep patterns
- Early-onset of REM sleep
160
Features of narcolepsy?
- Typical onset in teenage years
- Hypersomnolence
- Cataplexy
- Sleep paralysis
- Vivid hallucinations on going to sleep or waking up
161
Investigation for narcolepsy?
- Multiple sleep latency EEG
162
Management of narcolepsy?
- Daytime stimulants (e.g. modafinil)
| - Nighttime sodium oxybate
163
Define multiple sclerosis (MS)?
- Chronic and progressive
- Inflammatory plaques of demyelination in the central nervous system
- Disseminated in space and time
- Occurring at multiple sites
164
In whom and when is MS most common?
- Young adults < 50 yrs
- Peak = 30 yrs
- More common in women
- Symptoms tend to improve in pregnancy and the postpartum period
- Much more common at higher latitudes
165
Pathophysiology of MS?
- T-cell mediated immune response
- T-lymphocyte is primed -> gets through blood-brain barrier -> starts immune response cascade -> discrete plaques of demyelination at multiple CNS sites -> heals poorly -> remitting and relapsing symptoms
- Demyelination: some recovery but THIN
- Prolonged demyleination -> Axonal loss: these do NOT recover -> progressive symptoms/disability
166
MS disease patterns?
- Clinically isolated syndrome: first episode. May or may not go on to develop MS. If lesions are seen on MRI they are more likely to progress to MS
- Relapsing-remitting: 85%. Acute episodes (1-2 months) followed by recovery periods.
- Secondary-progressive: Started as relapsing-remitting but now deteriorated to have neuro symptoms between relapses. Around 65% of RR go on to develop SP within 15 yrs. May see gait/bladder problems.
- Primary progressive: worsening of the disease and neuro symptoms without initial relapses and remissions. More common in older people.
167
Causes of MS
- Genetics + chance + environment
- EBSTEIN BARR VIRUS (EBV)
- Low vitamin D
- Smoking
- Obesity
168
Presentation of MS
VISUAL
Visual:
- optic neuritis (UNILAT)
- optic atrophy
- Internuclear ophthalmoplegia = conjugate lateral gaze disorder (6th cranial nerve): affected eye will not be able to ADDUCT when looking laterally
169
Presentation of MS
SENSORY
Sensory:
- Numbness, tingling in limbs
- Trigeminal neuralgia
- Pins and needles (paraesthesia)
- Lhermitte's sign: electric shock sensation down the spine when flexing the neck - indicates disease in the cervical spinal cord in dorsal column
170
Presentation of MS
MOTOR
- Spastic weakness, most commonly seen in legs
- Bells Palsy
- Horners syndrome
171
Presentation of MS
CEREBELLAR
- Ataxia: more often seen during acute relapse
| - Tremor
172
Presentation of MS
OTHERS
- Urinary incontinence
- Sexual dysfunction
- intellectual deterioration
- CHARCOTS TRIAD:
o Nystagmus
o Intention tremor
o Dysarthria
173
Diagnosis of MS
- Clinically: 2 or more lesions of CNS disseminated in time and space
- MRI: high signal T2 lesions; periventricular plaques; Dawson fingers - hyperintense lesions perpendicular to the corpus callosum
- LP/CSF: OLIGOCLONAL BANDS of IgG
- Visual evoked potential: delayed but well-preserved waveform
174
Treating relapses of MS
- High dose steroids, e.g. PO/IV methylprednisolone for 5 days
- Shorten relapse but do not alter the degree of recovery
175
Disease-modifying drugs in MS
- BETA-INTERFERON
- Monoclonal antibodies, e.g. NATALIZUMAB or Alemtuzumab
- Immunosuppression: Azathioprine
176
Symptom control in MS
Spasticity:
- Baclofen or Gabapentin (or Diazepam)
- Physiotherapy
Fatigue:
- Amantadine once other possible causes excluded
Incontinence:
- If not residual volume (do US) -> anticholinergic (e.g. oxybutinin)
Depression: SSRIs
Neuropathic pain:
- Gabapentin or Amytryptilline
Tremor:
- Botulinum Toxina
Exercise to maintain activity and strength
IF SEVERE --> bone marrow transplant
177
What is cerebellar syndrome? Features?
- Unilateral cerebellar lesions cause ipsilateral signs
DANISH:
D: Dysdiadokinesia, Dysmetria (past-pointing), 'Drunk' appearance
A: Ataxia (limb, truncal)
N: Nystagmus (horizontal = ipsilateral hemisphere)
I: Intention tremor
S: Slurred staccato speech
H: Hypotonia
178
Causes of cerebellar syndrome?
- Toxic (alcohol/lithium/phenytoin/lead poisoning)
- Idiopathic
- Neurodegenerative
- MS
- Hypothyroidism
- Paraneoplastic (2ndry to lung or breast cancer) -
Friedrich's ataxia:
- Autosomal recessive
- Presents early in childhood
- Patient at increased risk of CVD disease
Spino-cerebellar ataxia 6 (SCA6)
- Autosomal dominant
- Episodic ataxia
- Difficulty focusing and migraines
179
Diagnosis of cerebellar syndrome?
- MRI: cerebellar atrophy and excludes other causes
180
Management of cerebellar syndrome?
- Speech therapy
| - Physiotherapy
181
What is Wernicke's encephalopathy (WE)
- Neuropsychiatric illness caused by THIAMINE (Vit B1) deficiency
- Mainly seen in ALCOHOLICS, but rarely in persistent vomiting, stomach cancer or dietary deficiency
- Petechial haemorrhages occur in the brain
182
Features of WE?
CLASSIC TRIAD - Confusion, Ataxia and Nystagmus (CAN)
- Ophthalmoplegia
- Peripheral sensory neuropathy
183
Investigations of WE?
- Decreased red cell transketolase
| - MRI
184
Treatment of WE?
Urgent replacement of THIAMINE
| Medical emergency - high mortality rate if untreated!
185
What is Korsakoffs syndrome? Management?
- Complication of WE if it is left untreated
- Addition of antero- and retrograde amnesia and confabulation in addition to symptoms of WE
- Often irreversible and requires full-time institutional care
- Can give thiamine supplementation and abstain from alcohol
186
What is shingles?
An acute, unilateral, painful blistering rash caused by reactivation of the Varicella Zoster Virus (VZV)
- Occurs in ONE DERMATOME
187
Management of shingles?
- Oral aciclovir = first line
| - Treatment reduces the incidence of post-herpetic neuralgia (more common in elderly)
188
Describe the shingles vaccine
- Offered to all patients aged 70 - 79 years
- Is a live-attenuated vaccine given sub-cutaneously
- CI = immune suppression
- SEs = injection site reactions, chickenpox (1 in 10,000)
189
What is Horner's syndrome?
- Damage to the sympathetic nervous system supplying the face
190
Features of Horner's syndrome?
- Ptosis
- Miosis (small pupil)
- Anhidrosis (loss of sweating on one side)
+/- Enopthalmos (sunken eye)
191
Causes of Horner's and how to distinguish?
```
Central: 4S's
Stroke
Swelling (tumour/encephalitis)
multiple Sclerosis
Syringomyelia (cyst in the spinal cord)
```
```
Pre-ganglionic: 4T's
Trauma
Tumour (Pancoast's)
Thyroidectomy
Top rib (cervical rib above clavicle)
```
```
Post-ganglionic: 4C's
Carotid artery dissection
Carotid aneurysm
Cavernous sinus thrombosis
Cluster headache
```
Anhidrosis:
Central: face, trunk, arms
Pre-ganglionic: face
Post-ganglionic: NONE
Hereditary cause:
Heterochromia
192
Diagnosis of Horner's syndrome?
- Cocaine eye drop WON'T cause any changes to the pupil (would normally)
- Adrenaline eye drop will cause DILATION of the pupil (would NOT normally)
193
What is Huntington's?
- Inherited neurodegenerative genetic condition
- Autosomal dominant
- Progressive and incurable
- Degeneration of cholinergic and GABAnergic neurons in the striatum of basal ganglia
- Due to a defect in the huntingtin (HTT) gene on chromosome 4
- Presents in middle age
194
What is genetic anticipation in Huntington's?
- A feature of TRINUCLEOTIDE REPEAT DISORDERS
- Successive generations have more repeats in the gene, resulting in:
o Earlier age of onset
o Increased severity of the disease
195
Presentation of Huntington's?
Prodrome:
- Irritability
- Depression
- Incoordination
- Personality changes
Progresses to:
- Chorea: abnormal involuntary movements
- Athetosis: twisting and writhing
- Abnormal eye movements: broken pursuit
- Ataxia: problems with heel to toe walking
- Some Parkinsonism
Within 15 yrs:
- Dementia
- Psychiatric problems - personality change, depression, psychosis
- Fits
- Death
196
Management of Huntington's?
No treatment that prevents progression
Supportive:
- MDT involvement
- SALT
- Genetic counselling
- Advanced life directives
- End of life care planning
Symptom management:
- Chorea: dopamine antagonists, e.g. TETRABENAZINE
- Depression: SSRIs, e.g. SEROXATE
- Psychosis: Olanzapine, Haloperidol
- Aggression: Risperidone
- Benzodiazepines, e.g. Diazepam
197
Prognosis of Huntington's?
- Typically results in death after 20 yrs after initial symptoms
- Suicide is a more common cause of death than for the general population
198
What is epilepsy?
- Umbrella term for conditions where there is a tendency to have unprovoked epileptic seizures
- Seizure = paroxysmal event in which changes of behaviour, sensation or cognitive processes are caused by excessive, hypersynchronous neuronal discharges in the brain
199
Which conditions have an association with epilepsy?
- Cerebral palsy (30% have epilepsy)
- Tuberous sclerosis
- Mitochondrial diseases
200
Characteristics of an epileptic seizure?
- Duration: 30 - 120 seconds
- Positive ictal symptoms: seeing/hearing/feeling things that are not there
- Postictal symptoms
- Stereotypical seizures - follow patterns
- May occur from sleep
- May be associated with other brain dysfunction
- Lateral tongue bite
- Deja vu
201
Basic classification questions in epilepsy?
1. Where the seizures begin in the brain
2. Level of awareness during a seizure (important as can affect safety during seizures)
3. Other features of the seizure
202
Features of a generalised tonic-clonic (grand mal) seizure?
Involves networks on both sides of the brain at onset
- LOC
- Tonic: muscle tensing; Clonic: muscle jerking
- May be associated with tongue biting, incontinence, groaning, irregular breathing
- Post-ictal confusion, drowsiness or irritability
203
Features of an absence (petit mal) seizure?
- Brief pauses (<10 secs), e.g. stop talking mid-sentence
- Presents in childhood
- Unaware of surroundings and won't respond
- Last 10-20 seconds
- EEG: 3Hz spike & wave oscillations
204
Features of an atonic seizure?
- Sudden loss of muscle tone, causing a fall
- No loss of consciousness
- Don't usually last > 3 mins
- May be indicative of Lennox-Gastaut syndrome
205
Management of generalised tonic-clonic seizure?
1st line = Sodium Valproate (AVOID in pregnancy)
| 2nd line = Lamotrigine or Carbamazepine
206
Features of a focal seizure/partial seizure?
- Specific area on one side of the brain
- Level of awareness can vary
- Focal aware (simple partial), focal impaired awareness (complex partial) and awareness unknown can further describe focal seizures
- Can also be classed as motor, non-motor or as having other features, e.g. aura
207
Management of focal seizures?
1st line = CARBAMAZEPINE
| 2nd line = LAMOTRIGINE, sodium valproate, oxcarbamazepine, topimerate
208
Management of absence seizures?
- Most (>90%) will stop having these as they get older
| - 1st line = Sodium Valproate or Ethosuximide
209
Management of atonic seizures?
- 1st line = Sodium Valproate
| - 2nd line = Lamotrigine
210
Feature of myoclonic seizures?
- Sudden brief muscle contractions
- Like a sudden jump
- Patient usually remains awake during these episodes
- Happen in children as part of Juvenile myoclonic epilepsy
211
Management of myoclonic seizures?
- 1st line = Sodium Valproate
| - 2nd line = Lamotragine, Topiramate, Levetiracetam
212
Temporal lobe seizure features?
- Lip-smacking
- Chewing
- Fumbling
- Grabbling
- Abdomen rising sensation
- Dysphasia
- Hallucinations
- Deja vu
- Postictal confusion
- Impaired awareness
213
Frontal lobe seizure features?
- Asymmetrical posturing
- Pedalling legs
- Post-ictal weakness
- Jacksonian march
- Do NOT lose consciousness
214
Parietal lobe seizure features?
- Paraesthesia
215
Occipital lobe seizure features?
- Floaters/flashers
216
Which antiepileptics should not be used in pregnancy and why?
- Sodium valproate - neural tube defects
| - Phenytoin - cleft palate
217
Briefly describe Infantile spasms (West's syndrome)
- Brief spasms starting in the first few months of life
- More common in males
- Usually secondary to a serious neurological condition
- Poor prognosis
- Vigabatrin/steroids
1. Flexion of the head, trunk, limbs --> extension of arms
Lasts 1-2 seconds, repeat up to 50 times
2. Progressive mental handicap
3. EEG: hypsarrthymia
218
Briefly describe Lennoz-Gastaut syndrome
- May be an extension of infantile spasms
- Onset 1-5 years
- Atypical absences, falls, jerks
- Usually have a history of more than one seizure type. For example, both atonic & myoclonic seizures.
- 90% moderate-severe mental handicap
- EEG: 1.5-2.5Hz (SLOW) spike & wave pattern
- Ketogenic diet may help
219
Briefly describe Juvenile myoclonic epilepsy
- Onset = teens
- More common in females
1. Infrequent generalised seizures, often in the morning
2. Daytime absences
3. Sudden, shock-like myoclonic seizures
Usually good response to sodium valproate
220
What differences might you see in non-epileptic seizures to help differentiate them from epilepsy?
- Last longer (10-20 mins)
- Eyes and mouth closed
- No cyanosis
- May get crying/weeping/speaking
- Recovery is quicker
- Pelvic thrusting
- History of psychiatric illness
- Situational
221
Diagnosis of epilepsy?
- Detailed description of attack by eye witness
- EEG
- CT scan: to look for space-occupying lesions
222
What is motor neurone disease?
An umbrella term encompassing neurological conditions of unknown cause that can present with both upper and lower motor neurone signs
- A progressive, ultimately fatal condition
- NO effect on SENSORY neurones
223
Epidemiology of MND?
- More common in MALES
- Median age of onset = 60yrs
- Prevalence = 6/100,000
224
Pathophysiology of MND?
- Genetic component - so good family history is important
- There's an increased risk associated with smoking, exposure to heavy metals and certain pesticides
- Cluster of major degenerative diseases
- Upper AND Lower MNs affected
- Selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells
- Sensory nerves are spared
225
How might you differentiate MND from other differentials?
- No sensory loss or sphincter disturbances: differentiate from MS and polyneuropathies
- No eye movement disturbances: differentiate from MG
- Wasting of the small hand muscles/tibialis anterior is common in MND
- No cerebellar signs in MND
- No sensory signs in MND
- Nerve conduction studies will show normal motor conduction and can help exclude a neuropathy
- MRI can exclude the differential diagnoses of cervical cord compression and myelopathy
226
What are the four clinical patterns of MND?
- ALS (80%)
- Progressive bulbar palsy (10-20%)
- Progressive muscle atrophy (<10%)
- Primary lateral sclerosis (rare)
227
Features of ALS?
- Loss of motor neurons in motor cortex AND anterior horns SO combined UMN and LMN signs
- Typically LMN signs in arms and UMN signs in legs
- Progressive spastic tetra-paresis
- Worse prognosis if:
Bulbar onset
Increased age
Low FVC
228
Features of progressive bulbar palsy?
- ONLY affects CRANIAL NERVES
- LMN lesion of tongue and muscles of talking/swallowing
- Flaccid, fasciculating tongue
- Jaw jerk = normal/absent
- Speech = quiet, hoarse or nasal
- Dysarthria
- Dysphagia
- Carries the worst prognosis
229
Features of progressive muscle atrophy?
- Anterior horn cell lesions ONLY
- So LMN signs ONLY
- Affects distal muscle groups before proximal
- Fasciculations, weakness, wasting
230
Features of primary lateral sclerosis?
- Loss of Betz cells in the motor cortex
- Mainly UMN signs
- Marked spastic leg weakness and pseudobulbar palsy
- No cognitive decline
231
Diagnosis of MND?
Mainly clinical
- Nerve conduction studies: normal motor conduction (can help exclude a neuropathy)
- Electromyography: reduced number of action potentials with increased amplitude
- MRI: can exclude cervical cord compression and myelopathy
- LP: may exclude inflammatory causes
232
Management of MND?
Antiglutamatergics, e.g. RILUZOLE
- Used mainly in ALS
- Prolongs life by ~ 3 months
For resp failure:
- Non-invasive ventilation (BIPAP) at night/home
- Survival benefit of ~ 7 months
Death:
- Usually in sleep due to hypercapnia
- Give LORAZEPAM (prevent choking) and MORPHINE
Drooling: Amitryptiline
Dysphagia: Blend food/NG tube/PEG
Spasticity: Baclofen/Diazepam
Joint pain: Analgesic ladder
233
What is hereditary spastic paraplegia?
UMN problems:
- Abnormal gait
- Bladder problems
- High arched feet
- Increased tone in legs and minimal muscle wasting
- Family history
234
Define cerebral palsy (CP)
- Permanent neurological problems resulting from damage to the brain around birth
- A disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain
- NOT progressive, however, the nature of the symptoms and problems may change over time during growth and development
235
Causes of cerebral palsy?
Antenatal (80%):
- Maternal infections (TORCH)
- Trauma during pregnancy
Perinatal (10%):
- Birth asphyxia
- Pre-term birth
- Trauma
Postnatal (10%):
- Intraventricular haemorrhage
- Meningitis
- Head trauma
- Severe neonatal jaundice
236
Types of cerebral palsy?
- Spastic (70%): Hypertonia and reduced function from UMN damage.
- Dyskinetic: problems controlling muscle tone (hypo- and hypertonia), athetoid movements and oro-motor problems. Basal ganglia damage.
- Ataxic: problems with coordinated movement from cerebellar damage
- Mixed: Mix of some or all of the above
237
Patters of spastic CP?
- Monoplegia: one limb affected
- Hemiplegia: one side of the body affected
- Diplegia: four limbs affected, but mostly legs
- Quadriplegia: four limbs affected more severely, often with seizures, speech disturbance and other impairments
238
Presentation of CP?
- UPPER MOTOR NEURONE (differentials: acquired brain injury or a tumour)
- HAND PREFERENCE BEFORE 18 MONTHS
- Failure to meet milestones
- Feeding or swallowing problems
- Learning difficulties
- Abnormal gait
- Abnormal tone in early infancy
- Leg will be extended with PLANTAR FLEXION
239
Complications of CP?
- Learning difficulties
- Epilepsy (30%)
- Squints (30%)
- Hearing impairment (20%)
- GORD
- Kyphoscoliosis
- Muscle contractures
240
Management of CP?
- MDT approach: PT/SALT/OT
- Spasticity: Baclofen, Diazepam, Botulim toxin type A
- Anticonvulsants and analgesia as required
MDT approach:
- PT/OT/SALT
- Dieticians
- Ortho surgeons
- Paediatricians
- Charities and support groups
241
Briefly discuss Benign Rolandic epilepsy
- Also known as Childhood Epilepsy with Centrotemporal Spikes (CECTS)
- Centrotemporal spike formations
- Daytime symptoms: tingling, numbness and twitching (often of the face)
- Night symptoms (more common and noticeable) are often full body convulsions
242
How would a third nerve palsy present?
- Diplopia
- Ipsilateral fixed pupil
- Ptosis
- Inability to adduct or supraduct the eye
243
When might an extended period of time for treating a stroke with a thrombectomy be considered?
For thrombectomy in acute ischaemic stroke, an extended target time of 6-24 hours may be considered if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
NICE recommends thrombectomy should be considered as soon as possible for people who fulfil the following criteria:
- Last known to be well up to 24 hours previously (including wake-up strokes)
- Who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA
- There is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
244
What is peripheral neuropathy?
- CHRONIC and SLOWLY progressive
- Starts in the LEGS and lower limbs
- SENSORY or MOTOR or BOTH
- Symmetrical!
245
Predominantly motor loss types of peripheral neuropathy?
- GBS
- Porphyria
- Lead poisoning
- Hereditary sensorimotor neuropathies (HSMN): Charcot-Marie-Tooth
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
- Diptheria
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Predominantly sensory loss types of peripheral neuropathy?
- Diabetes
- Uraemia
- Leprosy
- Alcoholism
- Vit B12 deficiency
- Amyloidosis
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What is alcoholic neuropathy?
- Secondary to both direct toxic effects and reduced absorption of B vitamins
- Sensory symptoms typically present prior to motor symptoms
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What is Vit B12 deficiency neuropathy?
- Subacute combined degeneration of the spinal cord
| - Dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia
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What is Charcot-Marie-Tooth disease?
| Management?
- The most common hereditary peripheral neuropathy
- Predomaninatrly motor loss
- No cure so management = PT/OT/podiatrists/ortho surgeons
Features:
- History of a frequently sprained ankle
- Foot drop (high-stepping gait)
- High-arched feet (pes cavus)
- Hammer toes
- Distal muscle weakness
- Distal muscle atrophy
- Hyporeflexia
- Stork leg deformity
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Briefly describe porphyria
- Photosensitive rash with blistering on face and dorsal of hands
- Hyperpigmentation
- Hypertrichosis
- Treat with chloroquine
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Briefly describe lead poisoning
Features:
- Abdominal pain
- Peripheral neuropathy (mainly motor)
- Fatigue
- Constipation
- Blue lines on gum margin (only 20% of adult patients, very rare in children)
The blood lead level is usually used for diagnosis: > 10 mcg/dl
Management:
- Dimercaptosuccinic acid (DMSA)
- D-penicillamine
- EDTA
- Dimercaprol
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Describe carpal tunnel syndrome
Caused by compression of median nerve in carpal tunnel
- Pain/pins and needles in THUMB, INDEX FINGER, MIDDLE FINGER
- Wake and shake - patient shakes hand to obtain relief, classically at night
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Examination of carpal tunnel syndrome
- Weakness of thumb abduction (abductor pollicis brevis)
- Wasting of thenar eminence (NOT hypothenar)
- Tinel's sign: tapping causes paraesthesia
- Phalen's sign: flexion of wrist causes symptoms
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Causes of carpal tunnel syndrome
- Idiopathic
- Pregnancy
- Oedema (HF)
- Lunate fracture
- RA
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Treatment of carpal tunnel syndrome
- Corticosteroid injection
- Wrist splints at night
- Surgical decompression (flexor retinaculum division)
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Presentation of ulnar nerve damage
Damage at wrist:
- 'Claw hand'
- Wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals)
- Wasting and paralysis of hypothenar muscles
- Sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects)
Damage at the elbow:
- Same as above
- Clawing is more severe in distal lesions
- Radial deviation of the wrist
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Presentation of radial nerve damage
- Wrist drop
- Sensory loss to small area between the dorsal aspect of 1st and 2nd metacarpals
Axillary damage:
- As above
- Paralysis of triceps
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Presentation of common peroneal nerve damage
--> FOOT DROP
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What is neurofibromatosis?
- Genetic condition
- Autosomal dominant
- Tumours of nerves (neuromas)
- Benign but cause neurological symptoms
- NF1 and NF2
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Briefly describe NG1 gene neurofibromatosis?
- Also known as Recklinghausen's syndrome
| - CHROMOSOME 17 - codes for NEUROFIBROMIN
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Presentation of NG1 gene neurofibromatosis?
CRABBING. For diagnosis, need 2:
- C: Cafe-au-lait spots (at least 6, >5mm in children, >15mm in adults
- R: Relative with NF1
- A: Axillary and inguinal freckles
- BB: Bone dysmorphia, e.g. Bowing of long bones or sphenoid wing dysplasia
- I: Iris hamartoma/Lisch nodules (at least 2)
- N: Neurofibromas (at least 2) or one plexiform neurofibroma
- G: Gliomas of the optic nerve
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Investigations into NG1 gene neurofibromatosis?
CLINICAL DIAGNOSIS so do not need investigations but some could be helpful:
- CT/MRI for lesions in the brain/spinal cord/anywhere
- Gene testing if unsure
- XR for bone lesions/pain
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Management of NG1 gene neurofibromatosis?
- No treatment for the underlying condition or to prevent complications
- Management = control symptoms, treat complications, monitor the disease
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Complications of NG1 gene neurofibromatosis?
- Renal artery stenosis (leads to HTN)
- Malignant peripheral nerve sheath tumours
- GI stromal tumour (type of sarcoma)
- Migraines
- Epilepsy
- Learning difficulties/behavioural problems
- Scoliosis
- Vision loss (2ndry to gliomas of optic nerve)
- Brain tumours
- Spinal cord tumours with associated neurology
- Increased risk of cancers. e.g. breast
- Leukaemia
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Briefly discuss aetiology, presentation and management of NG2 gene neurofibromatosis?
- Found on CHROMOSOME 22 which codes for MERLin
- Important in SCHWANN CELLS
- Present with BILATERAL ACOUSTIC NEUROMAS/SCHWANNOMAS
- May, therefore, have hearing loss, tinnitus or balance problems
- Also: schwannomas in brain, meningiomas, ependymomas
- SURGERY can be used to resect tumours but carries a risk of permanent nerve damage
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What is tuberous sclerosis?
- A GENETIC condition
- AUTOSOMAL DOMINANT inheritance
- Characesterist feature = HAMARTOMAS (abnormal growth and size of cells
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Where does tuberous sclerosis most commonly affect?
- Brain
- Kidneys
- Eyes
- Lungs
- Skin
- Heart
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Types of tuberous sclerosis?
- TSC1 on CHROMOSOME 9, encodes for HAMARTIN
| - TSC2 on CHROMOSOME 16, encodes for TUBERIN
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Skin signs in tuberous sclerosis?
- Ash leaf spots
- Shagreen patches
- Angiofibromas
- Subungual fibromata
- Cafe-au-lait spots
- Poliosis
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Neuro signs in tuberous sclerosis?
- Developmental delay
- Epilepsy
- Intellectual impairment
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Other signs in tuberous sclerosis?
- Retinal hamartomas
- Rhabdomyomas of the heart
- Gliomas
- Polycystic kidneys
- Lymphangioleiomyomatosis
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What is the classical presentation of tuberous sclerosis?
- A CHILD presenting with EPILEPSY with the characteristic SKIN FEATURES of TS
- Can also present in adults
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Causes of brain abscess?
- Extension of sepsis from middle ear or sinuses
- Trauma or surgery to the scalp
- Penetrating head injuries
- Embolic events from infective endocarditis
274
Features of a brain abscess?
- Depends upon the site if the abscess
- Those in critical areas, e.g. motor cortex will present earlier
- Raised ICP is common
- Fever, headache and focal neurology
- Fever may be absent and isn't like the pyrexia seen in other abscesses
275
Investigation of a brain abscess?
- CT
276
Management of a brain abscess?
- Surgery: craniotomy with debribement of abscess
- Antibiotics: IV 3rd-gen CEPHALOSPORIN and METRONIDAZOLE
- ICP management, e.g. Dexamethasone
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Briefly describe neoplastic spinal cord compression
- Oncological emergency and affects up to 5% of cancer patients
- Extradural compression accounts for the majority of cases, usually due to vertebral body metastases.
- It is more common in patients with lung, breast and prostate cancer
278
Features of neoplastic spinal cord compression
- Back pain - the earliest and most common symptom; may be worse on lying down and coughing
- Lower limb weakness
- Sensory changes: sensory loss and numbness
Neurological signs depend on the level of the lesion:
- Lesions above L1 usually result in upper motor neuron signs in the legs and a sensory level.
- Lesions below L1 usually cause lower motor neuron signs in the legs and perianal numbness.
- Tendon reflexes tend to be increased below the level of the lesion and absent at the level of the lesion
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Investigation of neoplastic spinal cord compression
URGENT MRI: whole spine MRI within 24hrs of presentation
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Management of neoplastic spinal cord compression
- High-dose oral dexamethasone
| - Urgent oncological assessment for consideration of radiotherapy or surgery
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Features of myopathies?
- Symmetrical muscle weakness (Proximal > distal)
- Common problems rising from chair/getting out of bath
- Sensation = normal, reflexes = normal
- No fascisulations
282
Causes of myopathies?
- Inflammatory: poliomyelitis
- Inherited: Duchenne's/Becker muscular dystrophy, myotonic dystrophy
- Endocrine: Cushing's (Steroids), thyrotoxicosis
- Alcohol
283
Duchenne vs Becker muscular dystrophy
Duchenne muscular dystrophy
- Progressive proximal muscle weakness from 5 years
- Calf pseudohypertrophy
- Gower's sign: child uses arms to stand up from a squatted position
- 30% of patients have an intellectual impairment
- Associated with dilated cardiomyopathy
Becker muscular dystrophy
- Develops after the age of 10 years
- Intellectual impairment much less common
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Define brain tumours
Abnormal growths within the brain
| Many different types from benign to highly malignant
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What are the three cardinal symptoms of a brain tumour?
1. Symptom of a raised ICP
- Headache, reduced GCS, nausea and vomiting
- Papilloedema
2. Progressive neurological defects
- Depending on the location of the tumour
3. Epilepsy
- Focal seizures
- Sinister in adults when new-onset
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What does papilloedema look like on fundoscopy?
- Loss of crisp white optic nerve head margins
- Venous engorgement
- Retinal oedema
- Retinal haemorrhages
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Diagnosis of brain tumours?
- CT/MRI
- Consider biopsy
- PET-scan: good for primary cause
- DO NOT LP --> RISK OF CONING
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What is a secondary brain tumour?
- Metastases
- Spherical and multiple lesions
- Most commonly from: LUNG, breast, bowel, skin, prostate, kidney
289
What kind of headache may follow a LP and how would you manage this?
- Low pressure headache
- Manage with fluids and caffeine
- If does not improve: blood patch
290
What is idiopathic intracranial hypertension (IIH)?
- Idiopathic ICP
| - Classically seen in young, overweight females
291
What are the risk factors for IIH?
- Obesity
- Female
- Pregnancy
- Drugs: OCP, Steroids, tetracycline, Vit A, Lithium
292
What are the features of IIH?
- Headache
- Blurred vision
- Papilloedema
- Enlarged blind spot
- Sixth nerve palsy may be present
293
How would you manage IIH?
- Weight loss
- Diuretics, e.g. ACETAZOLAMIDE
- TOPIRAMATE
- Repeated LP
- Surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve
- A lumboperitoneal or ventriculoperitoneal shunt may also be performed
294
What is multiple system atrophy?
- Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system and movement.
Two types:
- MSA-P: predominantly Parkinsonian features
- MSA-C: predominantly Cerebellar features
295
What are the features of Multiple system atrophy?
- Parkinsonism
- Autonomic disturbance:
o Erectile dysfunction
o Postural hypotension
o Atonic bladder
- Cerebellar signs
296
How to differentiate multiple system atrophy from Parkinson's?
Key features to help you differentiate are the presence of UNILATERAL symptoms, and more severe/early onset AUTONOMIC dysfunction (postural hypotension/erectile dysfunction).
Drug-induced Parkinsonism can also cause unilateral symptoms so this would present with history of drug use.
