Neuro Flashcards
1
Q
Which drugs can cause Parkinsonism?
A
Anti psychotics - clozapine, quietapine, haloperidol, risperidone
Anti emetics - Metoclopramide and prochlorperazine
Methyldopa (BP)
Calcium channel blockers - cinnarizine and flunarizine
Amiodarone
Sodium valproate
Lithium
2
Q
What is juvenile Parkinson’s?
A
Diagnosis under age 20
3
Q
What is early onset Parkinson’s?
A
Diagnosis under age 40
4
Q
What are the Parkinson’s plus syndromes?
A
Multiple system atrophy
Progressive supranuclear palsy
Corticobasal degeneration
5
Q
What are signs and symptoms of multiple system atrophy?
A
Autonomic dysfunction
Parkinsonism
Ataxia
6
Q
What is a DaTSCAN?
A
Dopamine transporter SPECT scan used in diagnosis of PD
7
Q
If a GP suspects early stages of PD, how soon should the pt see a specialist (neurologist or geriatrician)?
A
Within 6 weeks
8
Q
If a GP suspects late stages of PD, how soon should the pt see a specialist (neurologist or geriatrician)?
A
Within 2 weeks
9
Q
How is a diagnosis of Parkinson’s made?
A
Usually clinical diagnosis
Likely PD if at least 2 of: resting tremor, bradykinesia, rigidity
If symptoms improved by levodopa - more likely
DaTSCAN can be used to aid diagnosis
10
Q
What are the groups of causes of Parkinsonism?
A
Idiopathic
Drug induced
Cerebrovascular
Other progressive brain conditions - Parkinson’s plus
11
Q
What development assessment tool is used for children?
A
SOGS II - birth to 5 years
Schedule of growing skills
Screening tool
12
Q
What 9 areas are assessed in SOGS II?
A
Passive posture Active posture Locomotor Manipulative Visual Hearing and language Speech and language Interactive social Self care social
13
Q
What factors contribute to idiopathic intracranial hypertension?
A
Pregnancy
Combined oral contraceptive therapy
Obesity
Oral tetracycline therapy
14
Q
How do you treat idiopathic intracranial hypertension?
A
Weight reduction
Stopping the offending drug
More serious where sight is threatened - cerebrospinal fluid removal of shunting may be required
15
Q
What is recommended for carotid artery stenoses greater than 70% diameter?
A
Carotid endarterectomy
16
Q
What are complications of subarachnoid haemorrhage?
A
Recurrent SAH
Vasospasm
Stroke
Hydrocephalus
17
Q
What are features of subarachnoid haemorrhage?
A
Acute onset severe headache Meningeal irritation Low grade fever Localising neuro signs Neurogenic pulmonary oedema ST elevation on ECG
18
Q
What problems does maternal narcotic addiction cause in a neonate?
A
Neonatal abstinence syndrome: tremors, irritability, sleep problems, high pitched crying, hypertonia, hyperreflexia, seizures, mottled skin, fever, slow weight gain
Symptoms usually 1 to 3 days after birth
19
Q
What might convulsions occurring in first 24 hours of life signify?
A
Placental insufficiency
Cerebral palsy
20
Q
What are some causes of horners syndrome?
A
Pancoast tumour Cervical rib Goitre Syringomyelia Lateral medullary syndrome - brainstem stroke
21
Q
Which is the best choice of antiepileptic drug for generalised epilepsy in women of child bearing age? What supplement should be given alongside during pregnancy? And what screening is required?
A
Lamotrigine
High dose folate supplements
Vitamin K given to mother prior to delivery
Alpha fetoprotein and second trimester USS screening
22
Q
Why would phenytoin and valproate not be sensible choices for antiepileptic treatment in a woman of child bearing age?
A
Teratogenic effects
23
Q
What is Patterson Kelly Brown syndrome?
A
Plummer Vinson syndrome
Triad of microcytic hypochromic anaemia
Atrophic glossitis
Oesophageal webs/stricture
24
Q
What is Webers syndrome?
A
Infarction of the midbrain
Contralateral hemiplegia
Ipsilateral oculomotor nerve palsy and diplopia
25
What is Charcot Marie tooth disease?
Autosomal dominant inherited condition
| Wasting of lower limbs and small muscles of hands
26
What is diabetic amyotrophy?
Weakness, wasting and pain usually in quadriceps
Paraesthesia of the proximal lower limbs - thigh, hip, buttock
Symptoms begin on one side but usually spread to other
Vasculitic changes, microvascular insufficiency and ischaemia followed by axonal degeneration and demyelination
27
What is friedreichs ataxia? What are features of it?
Progressive ataxia, dysarthria, decreased proprioception, ascending muscle weakness, pes cavus, scoliosis, cardiomyopathy, arrhythmias, diabetes
Inheritance autosomal recessive, decreased synthesis of frataxin - mitochondrial protein
Onset before 20 years
28
What is von Hippel Lindau disease?
Mutation in tumour suppressor gene
Multiple tumours in CNS and viscera
Most commonly retinal, haemangioblastomas, renal cell carcinoma, phaeochromocytoma
May be diagnosed from FH and genetic testing
29
What causes should be considered for prolonged hiccup?
CNS disease - posterior fossa tumour, brain injury, encephalitis
Phrenic nerve or diaphragm irritation - tumour, pleurisy, pneumonia, intrathoracic adenopathy, pericarditis, GORD, oesophagitis
30
What are causes of dilated pupils?
Holmes adie pupil
Third nerve palsy
Drugs and poisons - atropine, CO, ethylene glycol
31
What are causes of small pupils?
```
Horners syndrome
Old age
Pontine haemorrhage
Argyll Robertson pupil
Drugs and poisons - opiates, organophosphates
```
32
What are features of neuroleptic malignant syndrome?
```
Fever
Rigidity
Altered mental status
Autonomic dysfunction
Elevated creatine phosphokinase
```
33
Concomitant use of which drugs may increase the risk of neuroleptic malignant syndrome?
Lithium or Anticholinergics alongside antipsychotic drugs
34
What is the treatment for neuroleptic malignant syndrome?
Withdrawal of the offending drug
Reduction of body temperature with antipyretics
Bromocriptine and amantadine - dopaminergic drugs
Dantrolene sodium - muscle relaxant
35
You are referred to a 40 year old woman in a psych ward who has a long history of schizophrenia resulting in multiple hallucinations in the past. She gives a several week history of feeling generally unwell associated with increasing stiffness affecting the jaws and arms. Over the last few years she has been on haloperidol with good symptom control. Her temp was 38.5 and BP 175/85. What does she have and what is the most important investigation to be performed?
Neuroleptic malignant syndrome
| Creatine phosphokinase
36
What causes meralgia paraesthetica?
Damage to the lateral cutaneous nerve of the thigh
| Usually due to entrapment at the lateral inguinal ligament, trauma, ischaemia or a retroperitoneal lesion
37
What is alports syndrome?
Inherited condition of sensorineural deafness and renal dysfunction - glomerulonephritis
Defect in type IV collagen
38
What are features of an acoustic neuroma?
```
Hearing loss - sensorineural
Unilateral tinnitus
Vertigo
Pressure in ear
Facial numbness and tingling
Loss of corneal reflex
```
39
What is the alternative name for an acoustic neuroma? What cells does it affect?
Vestibular schwannoma
| Schwann cells
40
What is the triad of features of wernickes encephalopathy?
Acute mental confusion
Ataxia
Opthalmoplegia
41
What are the oculomotor findings in wernickes encephalopathy?
Weakness of abduction
Gaze evoked nystagmus
Internuclear opthalmoplegia
Vertical nystagmus in primary position
42
What are some causes of wernickes encephalopathy?
```
Chronic alcohol abuse
Dialysis patients
Advanced malignancy
AIDS
Malnutrition
```
43
What is the urgent treatment for wernickes encephalopathy?
100mg fresh thiamine IV followed by 50-100mg daily
44
What can cause cerebral abscess?
Untreated or partially treated meningitis
TB
Pyogenic ear infections
Facial/orbital cellulitis
45
What is treatment for cerebral abscess?
Surgical drainage
| Abx
46
What are signs and symptoms of cluster headache?
```
Severe unilateral pain in temple and periorbital region
Attacks lasting a few moments to two hours
Ipsilateral lacrimation
Nasal congestion
Conjunctival injection
Miosis
Ptosis
Lid oedema
```
47
What is the first choice of Abx in meningitis for different patient groups?
For infants 3m - adults 50 years, cefotaxime/ceftriaxone
If been outside UK recently or multiple Abx in last 3 months - add vancomycin
Under 3m and over 50, add amoxicillin to cover for listeria
48
What might be possible causes of a post op seizure out of the blue?
```
Severe hyponatraemic encephalopathy
Hypoglycaemia
Sepsis
Hypoxia
Drug induced
```
49
What are sequelae of trigeminal nerve herpes zoster?
Trigeminal neuralgia
Corneal ulceration
Postherpetic neuralgia
50
Why might MS patients get urgency of urine?
Spastic paraparesis caused by demyelination in SC
51
Why might MS patients get episodes of visual disturbance?
Optic neuritis
52
A 63 year old hypertensive male presents with sudden onset vertigo, ataxia, diplopia and dysarthria. What is the problem?
Vertebrobasilar ischaemia
53
A 55 year old woman complains of recurrent severe stabbing pain in left cheek and jaw. The episodes last 5-10 secs and frequently occur during meals and showers. Examination of the cranial nerves is unremarkable. What is the drug of choice?
Carbamazepine
54
What medications should be used for prophylaxis of migraine?
Propranolol
Pizotifen
Topiramate
55
What medications can be used for acute attacks of migraine?
Sumatriptan
| Ergotamine
56
A 53 year old administrator attends her GP complaining of pain in her R hand which is worse at night and relieved by hanging her arm off the side of the bed. What is the most likely diagnosis?
Carpal tunnel syndrome
57
Which nerve is implicated in carpal tunnel syndrome?
Median nerve
58
By what mechanism is the nerve affected in carpal tunnel syndrome?
Compression, traction, pinching, squeezing or irritation of the median nerve as it runs through the transverse carpal ligament
59
What structures form the carpal tunnel?
Scaphoid
Lunate
Triquetrium
Flexor retinaculum
60
Which tendons run through the carpal tunnel?
Flexor digitorum profundus x 4
Flexor digitorum superficialis x 4
Flexor pollicis longus
61
In which canal does the ulnar nerve run?
Guyons canal
62
What findings would you expect on examination of a person with carpal tunnel?
Scars from previous release surgery
Sensory loss lateral 3 and a half digits
Wasting and weakness of LOAF: first and second lumbricals, opponens pollicis, abductor pollicis brevis, flexor pollicis Brewis
Positive Phalens: tingling in median nerve distribution induced by full flexion of the wrists for 60 secs
Positive Tinels: tapping over median nerve at wrist generates tingling in nerves distribution
63
What conditions can predispose to carpal tunnel syndrome?
```
Diabetes
Hypothyroidism
RA
Pregnancy
Obesity
Wrist fracture/trauma
```
64
What investigations can be done for carpal tunnel syndrome? What will they show?
EMG: electromyography shows focal slowing of conduction velocity in the median nerve across carpal tunnel, prolongation of the median distal motor latency, decreased amplitude of median sensory and/or motor nerves
USS wrist: space occupying lesion may be identified
MRI wrist: space occupying lesion may be identified, useful pre op
65
Describe EMG as you would to a patient
Electrodes attached to skin one which emits a signal, one which records
Electrical impulses given to nerve - feel like sharp tapping sensation on skin, bit unpleasant but doesn't last long
Time it takes for muscle to contract is recorded - conduction velocity
If you take muscle relaxants or anti cholinergics, may need to stop them
Tell person doing test if you have a pacemaker or defibrillator
Avoid hand lotions/creams
Wear loose fitting clothing that can be rolled above elbow
66
What are management options for carpal tunnel syndrome?
Avoid activities which make symptoms worse
Treating underlying condition
Wrist splint: improvement in 4 weeks
Corticosteroid injection into wrist
Carpal tunnel release surgery when other treatments have failed
67
What are possible complications of carpal tunnel release surgery?
```
Infection
Persisting symptoms
Bleeding
Nerve injury
Scarring
Persistent wrist pain different from original
Complex regional pain syndrome
```
68
What advice should be given after carpal tunnel release surgery?
Keep hand bandaged and raised for 2 days - sling
Gentle finger, shoulder and elbow exercise to prevent stiffness
Avoid using hand to do demanding activities until it is completely recovered which might take several weeks
69
A 35 year old man attends his GP complaining of severe pain in his L leg and numbness in his foot. His symptoms began suddenly 2 days ago when he bent down to pick up his child. He is otherwise fit and well. He also has back pain but less severe than his leg. What is the likely cause of his symptoms?
Sciatic nerve compression
70
What anatomical change causes sciatica?
Vertebral disc prolapse which causes compression on the sciatic nerve
71
What clinical test can you do to diagnose sciatica? What sign does this elicit?
Straight leg raise
Lasegues sign: pain in distribution of sciatic nerve reproduced with passive flexion of straight leg between 30 and 70 degrees
72
What is the management for sciatica?
```
Keeping active - exercise
Physio
NSAIDs
Amitriptyline and duloxetine
Gabapentin and pregabablin
Corticosteroid injections
Muscle relaxants
Surgery: Open discectomy, microdiscectomy
```
73
A patient with a sciatic pain returns to the GP 3 days later with pain in both legs. What diagnosis needs to be urgently excluded and why? How do exclude it?
Cauda equina syndrome
Needs to be excluded quickly as can lead to paralysis, sensory changes, bladder bowel and sexual dysfunction
MRI
74
What questions do you need to ask to exclude cauda equina syndrome?
```
Unilateral or bilateral sciatica
Saddle and perineal anaesthesia
Urinary retention/incontinence
Faecal incontinence
Lower extremity motor weakness and sensory deficits
```
75
What examination findings would you expect in cauda equina syndrome?
```
Back tenderness to palpation
Radicular sensory loss
Saddle anaesthesia
Asymmetrical paraplegia
Loss of tendon reflexes
Poor anal sphincter tone
```
76
What is the management for cauda equina?
Urgent spinal decompression
77
A 54 year old dentist attends her GP complaining of cramps and weakness in her R hand which are starting to affect her work. On examination there is general wasting of the hand muscles with muscle fasciculation and a brisk wrist reflex. What is the most likely diagnosis? Why?
Motor neurone disease - mixed upper and lower motor neurone signs
ALS
78
Which nerve cells are affected in motor neurone disease?
Motor neurones in the ventral spinal cord
79
What is the likely prognosis for ALS?
Life expectancy 2 to 5 years
80
What symptoms do you see with progressive bulbar palsy?
```
Progressive dysphagia
Dysphonia
Dysarthria
Wasted tongue with fasciculations
Drooling
```
81
What are symptoms of psuedobulbar palsy?
```
Slow and slurred speech
Dysphagia
Spastic tongue
Brisk jaw jerk
Dysarthria
```
82
A fit and well 25 year old woman attends A and E complaining of weakness in her legs. 2 weeks ago she had an episode of food poisoning from which she made a full recovery. 5 days ago she started to get numbness in her toes which has spread to her feet ankles and shins. This morning she woke up and was unable to move her feet. She is very anxious. She has a mild low back ache. On examination tone is reduced in her lower limbs, there is sensory loss, weakness, reduced knee and absent ankle reflexes. What is the differential?
```
Guillain Barre syndrome
Cauda equina and conus medullaris syndromes
Chronic inflammatory demyelinating polyradiculoneuropathy
Myasthenia gravis
Heavy metal toxicity
Lyme disease
MS
Botulism
```
83
What is the pathological process involved in Guillain barre syndrome?
Post infective immune mediated
| Antibodies that cross react with glycolipids and gangliosides distributed through myelin in peripheral nervous system
84
What investigations can be used to confirm guillain barre syndrome?
Nerve conduction studies: slowed conduction
LP: elevated CSF protein
LFT: elevated AST and ALT
Spirometry: reduced vital capacity, max inspiratory pressure
Antiganglioside antibody
85
What is the management for guillain barre syndrome?
IV immunoglobulin 400mg/kg/day for 5 days
Plasma exchange if IgA deficiency or renal failure
DVT prophylaxis
Intubation and ventilation
Gabapentin or carbamazepine for pain
Fluid boluses if hypotensive
86
What are the characteristic features of myasthenia gravis?
Skeletal muscle weakness
| Increased fatigability
87
What thymus problems do people with myasthenia gravis have?
65% hyperplasia of thymus
| 12% thymoma
88
How do you treat myasthenia gravis?
Rest
Pyridostigmine - acetylecholinesterase inhibitor
Prednisolone
Azathioprine, mycophenolate or methotrexate
Thymectomy - 25% chance remission, 50% improve symptoms
Severe/life threatening - plasmapheresis, IV immunoglobulin
89
What is the charity which provides useful information and support to people living with myasthenia gravis?
Myaware
90
What are side effects of pyridostigmine?
```
Cholinergic crisis if overdose
Diarrhoea
Urinary frequency
Meiosis
Excessive salivation and lacrimation
```
91
What might cause a myasthenic crisis?
Drug omission
Infection
Stress
92
What are indications for an immediate CT head after head injury?
GCS < 13 on initial assessment
GCS < 15 at 2 hours post-injury
Suspected open or depressed skull fracture
Any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign)
Post-traumatic seizure
Focal neurological deficit
More than 1 episode of vomiting
93
What are indications for a CT head within 8 hours of head injury?
Adults with risk factors who have experienced loss of consciousness or amnesia since injury:
Age 65 years or older
Any history of bleeding or clotting disorders
Dangerous mechanism of injury (pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs)
More than 30 minutes' retrograde amnesia of events immediately before the head injury
Patient on warfarin
94
Which nerve is at risk during a carotid endarterectomy procedure?
Ipsilateral hypoglossal
95
What haemodynamic combination is most likely to be seen prior to coning? What is this reflex called?
Hypertension and bradycardia
| Cushing's reflex - pre terminal event
96
What are causes of primary brain injury?
Focal: contusion, haematoma
Diffuse: diffuse axonal injury
97
What are causes of secondary brain injury?
Cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation
98
What are risk factors for subdural haematoma?
Old age
Alcoholism
Anticoagulation
99
A 22-year-old mechanic is involved in a fight. He is hit on the head with a hammer. On examination he had clinical evidence of an open depressed skull fracture and a GCS of 6/15. What imaging is indicated?
Urgent neurosurgical review (even before CT head performed)
| GCS less than 8
100
A 67-year-old retired lawyer falls down the stairs. His GCS is 15/15 and he has some bruising over the mastoid. What imaging is indicated?
CT head within an hour
| Basal skull fracture, indicated by a positive Battle's sign
101
A 52-year-old secretary falls down '10-11' stairs. She complains of neck pain. She has a GCS of 15/15 and no neurology. She is unable to rotate her c-spine 45 degrees to the left and right. What imaging is indicated?
C spine immobilisation and CT c spine
102
What is Kernigs sign?
Positive when the thigh is flexed at the hip and knee at 90-degree angles and subsequent extension of the knee elicits pain. This can be indicative of meningism of subarachnoid haemorrhage
103
An elderly patient with a precious history of spinal canal stenosis sustains an extension injury of the cervical spine. The upper limbs are more affected than the lower and perianal sensation is preserved. What is the likely diagnosis?
Central core syndrome - motor and sensory loss, upper limbs mainly
104
What are some causes of a spastic paraparesis?
Metastatic infiltration of the spine
Vascular disorders
Osteoporotic collapse of vertebrae
105
What are some causes of facial nerve palsy?
Intracranial: brain stem tumour, stroke, ms, acoustic neuroma
Intratemporal: otitis media, Ramsay hunt, cholesteatoma
Infratemporal: parotid tumour, trauma
Other: sarcoidosis, guillain barre, diabetes, Bell's palsy
106
In a patient presenting with amaurosis fugax but is otherwise fit and well, what treatment should they be started on?
Clopidogrel 75mg od
OR
Aspirin 75mg od plus dipyridamole mr 200mg bd if intolerant to clopidogrel
107
In a patient presenting with amaurosis fugax and af, what treatment should they be started on?
Warfarin - stroke risk
108
What are the layers of the scalp?
S: Skin from which head hair grows. Contains numerous sebaeceous glands and hair follicles
C: Connective tissue. A dense subcutaneous layer of fat and fibrous tissue containing nerves and vessels
A: Aponeurosis (epicranial or galea aponeurotica). Tough layer of dense fibrous tissue which runs from frontalis anteriorly to occipitalis posteriorly
L: Loose areolar connective tissue layer, easy plane of separation between upper three layers and pericranium. Referred to as danger zone because of ease by which infectious agents can spread through it to emissary veins which drain into the cranium. Made up of collagen I, collagen III, glycosaminoglycans
P: Pericranium is the periosteum of skull bones and provides nutrition to bone and the capacity for repair
109
What is Hutchinson's sign?
Vesicles on tip or side of the nose, precedes the development of ophthalmic herpes zoster - sight threatening
Nasociliary branch of the trigeminal nerve innervates both the cornea and the lateral dorsum of the nose as well as the tip of the nose
110
What is the Le Fort fracture classification?
Fractures of midface, which involve separation of all or a portion of the midface from the skull base. In order to be separated from the skull base the pterygoid plates of the sphenoid bone need to be involved as these connect the midface to the sphenoid bone dorsally
Le Fort 1 is a horizontal maxillary fracture, separating the teeth from the upper face (floating palate)
Le Fort 2 is a pyramidal fracture, with the teeth at the pyramid base, and nasofrontal suture at its apex (floating maxilla)
Le Fort 3 is craniofacial dysjunction (floating face)
111
What is the Monroe Kellie principle?
Pressure-volume relationship that aims to keep a dynamic equilibrium among the essential non-compressible components inside the rigid compartment of the skull
Average intracranial volume in an adult is around 1700 mL, composed of brain tissue (1400 mL), CSF (150 mL), and blood (150 mL). The volume of these three components remains nearly constant in a state of dynamic equilibrium. Thus, a decrease in one component should be compensated by the increase in other and vice-a-versa
112
What should be given to prevent vasospasm in a subarachnoid haemorrhage?
Nimodipine - CCB
113
What complications are we aiming to prevent in management of SAH?
```
Rebleeding
Vasospasm
Hydrocephalus
Hyponatraemia
Seizures
Acute pulmonary oedema
Cardiac dysfunction
```
114
What is the difference between a communicating and obstructive hydrocephalus?
Communicating: impaired cerebrospinal fluid reabsorption in absence of any CSF-flow obstruction between the ventricles and subarachnoid space - SAH, meningitis. Functional impairment of the arachnoidal granulations
Obstructive: CSF-flow obstruction. Foramen of Monro obstruction may lead to dilation of lateral ventricles
Aqueduct of Sylvius, normally narrow to begin with, may be obstructed and lead to dilation of both lateral ventricles as well as the third ventricle
Fourth ventricle obstruction will lead to dilatation of the aqueduct as well as the lateral and third ventricles
Foramina of Luschka and foramen of Magendie may be obstructed due to congenital malformation
115
How is brain stem death confirmed?
Preconditions: apnoeic coma, defined cause of severe and irreversible brain damage, core temperature above 34
Tests by 2 doctors, repeated
Fixed unresponsive pupils with absence of direct and consensual light reflexes
Absent corneal reflexes
Absent vestibulo ocular reflexes
Absent motor activity after painful stimuli
Absent gag reflex
Absent spontaneous respiration
116
What are causes of neck stiffness?
Meningitic conditions
Tumours
Subarachnoid haemorrhage
Bony abnormalities: cervical spondylosis
117
What are features of benign essential tremor?
Postural tremor worse if arms outstretched
Improved by alcohol and rest
Cause titubation
118
What is the management for essential tremor?
Propranolol
| Primidone
119
What are the criteria for ABCD2 for prognosis in TIA?
Age > 60
Blood pressure >140/90
Clinical features: unilateral weakness (2), speech disturbance (1)
Duration of symptoms: >60 mins (2), 10-59 mins (1)
Diabetes
120
What does an ABCD2 score of 4 or above tell you?
Had TIA and are at higher risk of stroke
Need aspirin started immediately
Specialist assessment and investigations within 24 hours of symptoms
Secondary prevention
121
What needs to be done if an ABCD2 score is 3 or below?
Specialist assessment within 1 week of symptoms
| If vascular territory or pathology uncertain, refer for imaging
122
What should be done with patients with crescendo TIAs? (Two or more episodes in a week
Treated as high risk of stroke even if ABCD2 score is 3 or less
123
Why does myeloma increase risk of stroke?
Paraproteinaemia - hyperviscosity of blood
124
Which drugs are used for migraine prophylaxis?
Topiramate or propranolol
125
Which migraine prophylaxis drug is preferable in a woman of child bearing age?
Propranolol preferable to topiramate due to teratogenic effects and reduced effectiveness of hormonal contraceptives
126
What is first line management for an acute migraine?
Combination therapy with oral triptan and NSAID/paracetamol
127
An 18 year old female presents with tremor and dysarthria, there is a family hx of early onset liver disease. Blood tests show a raised ALT. What is the most likely diagnosis?
Wilson's disease
128
How is Wilson's disease inherited?
Autosomal recessive
129
What is the defect in Wilson's disease?
ATP7B gene on chromosome 13 leading to excessive copper deposition in tissues due to increased absorption and decreased hepatic excretion
130
What are features of Wilson's disease?
Liver: hepatitis, cirrhosis
Neuro: basal ganglia degeneration, speech and behavioural problems, asterixis, chorea, dementia
Kayser-fletcher rings
Renal tubular acidosis (fanconi syndrome)
Haemolysis
Blue nails
131
How is a diagnosis of Wilson's disease made?
Reduced serum caeruloplasmin
Reduced serum copper
Increase 24h urinary copper excretion
132
What is the management of Wilson's disease?
Penicillamine (chelates copper)
| Trientine hydrochloride
133
What is the preferred benzodiazepine to give IV in the presence of ongoing seizures?
Lorazepam
134
What is the preferred benzo to give in a child with ongoing seizures?
Buccal midazola,
135
What is the next line of management if seizures were to continue despite two doses of benzodiazepine?
Phenytoin
| Senior help as intubation and ventilation may be required
136
What is Charcots triad in the context of MS?
Suggestive of cerebellar lesions in MS
Nystagmus
Intention tremor
Staccato speech
137
In a patient presenting with headache and amenorrhoea combined with some visual field loss, what needs to be investigated and how?
Pituitary lesion
| Assessment of pituitary function, MRI and serum prolactin
138
What are the International Headache Society diagnostic criteria for migraine without aura?
At least 5 attacks fulfilling:
Headache lasting 4-72 hours
Headache that is: unilateral, pulsating, moderate to severe, aggravated by activity
During headache: nausea/vomiting, photo/phonophobia
Not attributed to another disorder
139
Give examples of typical auras that precede migraines
Transient hemianopic disturbance
Spreading scintillating scotoma (jagged crescent)
Develop over at least 5 mins and last 5-60 mins
140
What is the oxford stroke classification? (Bamford)
Total anterior circulation infarct: middle and anterior cerebral arteries. Hemiparaesis/hemisensory loss, homonymous hemianopia, higher cognitive dysfunction
Partial anterior circulation infarct: smaller artistes of anterior circulation. 2 of above criteria
Posterior circulation infarct: vertebrobasilar arteries. Cerebellar/brainstem syndromes, loss of consciousness, isolated homonymous hemianopia
Lacunar: perforating arteries around internal capsule, thalamus, basal ganglia. Unilateral weakness/sensory deficit, pure sensory, ataxic hemiparesis
141
What is lateral medullary syndrome?
Posterior inferior cerebellar artery stroke
Wallenbergs syndrome
Ipsilateral ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy - horners
Contralateral limb sensory loss
142
What is webers syndrome?
Ipsilateral III palsy
| Contralateral weakness
143
Which conditions are associated with berry aneurysms?
Adult polycystic kidney disease
Ehlers-Danlos syndrome
Coarctation of the aorta
144
What are some causes of subarachnoid haemorrhage?
Berry aneurysm rupture
AV malformations
Trauma
Tumours
145
How should subarachnoid haemorrhage be investigated?
CT
| Lumbar puncture after 12 hours (time for xanthochromia to develop)
146
What are complications of subarachnoid haemorrhage?
Rebleeding
Obstructive hydrocephalus
Vasospasm leading to cerebral ischaemia
147
What is the management of subarachnoid haemorrhage?
Neurosurgical opinion
| Post op nimodipine 60mg/4h if BP allows
148
What is an Adie pupil?
Tonically dilated pupil
Slowly reactive to light with more definite accommodation response
Damage to parasympathetic innervation of eye due to viral or bacterial infection
Accompanied by absent knee or ankle jerks
149
What is a Marcus-Gunn pupil?
Relative afferent pupillary defect seen during swinging light examination
Pupil constricts less and therefore appears to dilate when like swung from unaffected to affected eye
Damage to optic nerve or severe retinal disease
150
What signs does horners produce?
Miosis (pupil constriction)
Ptosis (droopy eyelid)
Apparent enopthalmos
Anhydrosis
151
What is Hutchinsons pupil?
Unilaterally dilated pupil unresponsive to light
| Compression of oculomotor nerve by intracranial mass
152
What is an Argyll Robertson pupil?
Bilaterally small pupils that accommodate but don't react to bright light
Prostitutes pupil - neurosyphilis
Diabetic neuropathy
153
Which infection classically triggers Guillain Barre?
Campylobacter jejuni
154
What are characteristic features of Guillain Barre?
Progressive weakness of all 4 limbs, ascending
Proximal muscles before distal
Very few sensory signs
Areflexia
Cranial nerve involvement - diplopia
Autonomic involvement - urinary retention
155
Aneurysm where needs to be ruled out with an acute onset painful third nerve palsy?
Posterior communicating artery
156
Which type of motor neurone disease carries the worst prognosis?
Progressive bulbar palsy
157
What is the most common presentation of MS?
Optic neuritis
158
In which tubes from a lumbar puncture should red blood cell count be determined?
4 tubes altogether
| RBC count in 1 and 4
159
What features of CSF suggest SAH?
Elevated opening pressure
RBC that does not diminish between tubes 1 and 4
Xanthochromia after 12 hours
160
What are the different forms of motor neurone disease?
Amyotrophic lateral sclerosis
Primary lateral sclerosis
Progressive muscular atrophy
Progressive bulbar palsy
161
What is a rosier scale assessment?
```
Differentiate patients with stroke and stroke mimics
LOC -1
Seizure -1
Facial weakness +1
Arm weakness +1
Leg weakness +1
Speech disturbance +1
Visual field defect +1
Stroke likely if score >0
```
162
Which medications are associated with IIH?
```
Tetracycline antibiotics
Isotretinoin
Contraceptives
Steroids
Levothyroxine
Lithium
Cimetidine
```
163
What are risk factors for IIH?
Obesity
Female
Pregnancy
Drugs: OCP, steroids, tetracycline, vit A, lithium
164
What are features of IIH?
```
Headache
Blurred vision
Papilloedema
Enlarged blind spot
Sixth nerve palsy
```
165
What is the management of IIH?
```
Weight loss
Diuretics - acetazolamide
Topiramate
Repeated lumbar puncture
Surgery - optic nerve sheath decompression and fenestration, lumboperitoneal or ventriculoperitoneal shunt
```
166
In a patient with a stroke and AF with a slow ventricular response, what is the management?
Aspirin 300mg OD for 2 weeks then lifelong anti coagulation
167
Where is the lesion which leads to hemiballismus?
Contralateral subthalamic nucleus
168
What are some causes of hemiballismus?
```
Stroke
Traumatic brain injury
Amyotrophic lateral sclerosis
Hyperglycaemia
Malignancy
Vascular malformations
Tuberculomas
Demyelinating plaques
```
169
What are features of Wilson's disease?
```
Liver: hepatitis, cirrhosis
Neuro: basal ganglia degeneration, speech and behavioural problems, asterixis, chorea, dementia
Kayser fletcher rings
Renal tubular acidosis
Haemolysis
Blue nails
```
170
How is wilsons inherited?
Autosomal recessive
171
What are the underlying problems in Wilson's disease?
Increased copper absorption from small intestine
Decreased hepatic copper excretion
Defect in ATP7B gene on chromosome 13
172
How is Wilson's disease diagnosed?
Reduced serum caeruloplasmin
Reduced serum copper
Increased 24h urinary copper excretion
173
How is Wilson's disease managed?
Penicillamine (copper chelator)
| Trientine hydrochloride
174
Which nerve is at risk in saphenopopliteal ligation for varicose veins?
Common peroneal nerve
175
What is the difference between simple and complex partial seizures?
Simple: no disturbance of consciousness
Complex: consciousness disturbed
176
What are presenting features of MS?
```
Spastic paraparesis
Cerebellar signs
Optic atrophy
Nystagmus
Internuclear opthalmoplegia
Urinary incontinence
```
177
What does CSF examination show in MS?
Raised protein and lymphocyte count
Oligoclonal bands of IgG on electrophoresis
Delayed visual, auditory and somatosensory evoked potentials
178
What is transient global amnesia?
Transient vascular insufficiency of both hippocampi
Usually affects people over 50
Lasts less than 24h
Awareness of personal identity is retained, along with normal cognition
179
How can the weakness in lambert eaton myasthenia be differentiated from that of myasthenia gravis?
Lambert eaton: weakness affects legs more than arms, gets better with exertion
Myasthenia: weakness arms more than legs, gets worse on exertion, easily fatigued
180
Which nerve root is likely damaged with weakness of ankle dorsiflexion, eversion and inversion, decreased ankle jerk and sensory loss confined to dorsum of the foot and anterior leg?
L5
181
What are features of neuroleptic malignant syndrome? Which neurotransmitters/brain regions are responsible for each symptom?
```
Muscle rigidity - dopamine
Parkinsonism - dopamine
Tremor - dopamine
Hyperreflexia - calcium
Rhabdomyolysis - calcium
Renal failure - calcium
Agitation - mesolimbic system
Confusion - reticular activating system
Labile blood pressure - dopamine
Tachycardia - dopamine
Pyrexia
Sweating
White cell count raised
```
182
What is the management of neuroleptic malignant syndrome?
IV fluids
Dantrolene (Ryanodine receptor antagonist)
Bromocriptine (dopamine agonist)
183
What are some adverse effects of sodium valproate?
```
Nausea
Increased appetite and weight gain
Alopecia
Ataxia
Tremor
Hepatitis
Pancreatitis
Thrombocytopenia
Teratogenic
Hyponatraemia
```
184
What are important factors required for a diagnosis of dementia?
Two or more cognitive impairments, one being memory
Sufficiently effected to cause functional impairment
No physical abnormality effecting cognition
Patient fully alert when assessed
185
How is dementia staged?
Mild: difficulties with ADLs, score over 23
Moderate: unable to manage independent living, score 11 to 22
Severe: personal ADLs severely impaired, score under 10
186
What is the triad of symptoms for wernickes?
Confusion
Ataxia
Opthalmoplegia
187
What is the treatment for wernickes?
IV high potency 2-3 pairs TDS for 2 days
188
What is a tensilon test?
Diagnosis of myasthenia gravis
| Administration of intravenous edrophonium rapidly reverses symptoms
189
Why is a diabetic 3rd nerve palsy painful?
Infarction of the nerve
190
What are features of cavernous sinus syndrome?
Opthalmoplegia
Pain
Proptosis
Prominent vessels
191
What is the treatment for Huntingtons and other hyperkinetic movement disorders?
Tetrabenazine - depletes dopamine
192
What are causes of optic neuritis?
MS
Diabetes
Syphilis
193
What are features of optic neuritis?
Unilateral decrease in visual acuity over hours or days
Poor discrimination of colours - red desaturation
Pain worse on eye movement
Relative afferent pupillary defect
Central scotoma
194
What is the management for optic neuritis?
High dose steroids
195
What is Hoffmans sign?
Flicking the distal phalanx of the middle finger to cause momentary flexion
Positive result is exaggerated flexion of the terminal phalanx of the thumb
Sign of upper motor neuron lesion
196
What is a tensilon test?
Tensilon is an anticholinesterase inhibitor
| People with myaesthenia gravis get stronger when injected with tensilon
197
What is bulbar palsy? What are clinical features?
Lower motor neurone lesion of cranial nerve 9-12
| Tongue flaccid and fasciculating, dysarthria, absent palatal movement, bovine cough, dysphagia, nasal regurgitation
198
What are some causes of bulbar palsy?
```
Motor neurone disease
Syringobulbia
Guillain Barre syndrome
Poliomyelitis
Neuro syphilis
```
199
What are some indications for an urgent CT head? (Within 1 hour)
GCS <13 on initial assessment
GCS <15 at 2 hours post injury
Suspected open or depressed skull fracture
Any sign of basal skull fracture: haemotympanum, panda eyes, csb rhinorrhoea, battles sign
Post traumatic seizure
Focal neurological deficit
More than 1 episode of vomiting
200
What are some indications for CT head within 8 hours of head injury?
Adults who have had a LOC or amnesia since injury and have any of these risk factors:
Over 65
History of bleeding or clotting disorder
Dangerous mechanism of injury: pedestrian hit by car, ejected from motor vehicle, fall from height greater than 1m or 5 stairs
More than 30 mins retrograde amnesia of events immediately before the injury
Patient on warfarin
201
What are features of myotonic dystrophy?
Frontal baldness
Atrophy of temporalis, masseters and facial muscle
Neck muscle atrophy
Cardiac abnormalities: first degree heart block, complete heart block
202
How does subclavian steal syndrome present?
Posterior circulation problems such as dizziness or vertigo during exertion of an arm
203
What is subclavian steal syndrome?
Subclavian artery steno occlusive disease proximal to origin of vertebral artery
Flow reversal in vertebral artery
204
What is a stroke?
Rapidly developing clinical signs of focal disturbance of cerebral function, lasting more than 24 hours or leading to death
205
What is the most common cause of stroke?
Arterial embolism or thrombus
206
What is the most common cause of stroke in younger adults?
Carotid or vertebral artery dissection
207
Which scoring systems are used in TIA and stroke?
ABCD2 in TIA
| ROSIER in Stroke
208
What are the categories of the ABCD2 score?
A – Age >60 years – 1 pt
B – Initial BP reading either SBP >140 or DBP >90 - 1 pt
C – Clinical features of TIA, Unilateral weakness – 2pts, Speech disturbance without weakness – 1pt
D – Duration of symptoms 10-59 minutes – 1pt, >60 minutes – 2 pts
D – Diabetes – 1pt
209
Which TIAs are considered high risk?
```
ABCD2 score >4
Crescendo TIA (2 or more TIAs in 1 week)
```
210
How is a high risk TIA managed?
```
Aspirin 300mg daily
Specialist assessment and investigation within 24hr
Make decision on brain imaging – MRI
Carotid imaging, endarterectomy
Secondary prevention
```
211
What are the different categories of stroke?
Cerebral hemispheric infarcts: Total/partial Anterior circulation infarction (TACI/PACI)
Brainstem infarcts: Posterior circulation infarct (POCI)
Lacunar: Lacunar infarct (LACI)
212
What are the clinical features of total anterior circulation syndrome/infarct?
All 3 of:
Unilateral weakness +/- sensory deficit
Higher dysfunction (dysphasia)
Homonymous hemianopia
213
How does a partial anterior circulation syndrome/infarct present?
2 of:
Unilateral weakness +/- sensory deficit
Higher dysfunction (dysphasia)
Homonymous hemianopia
214
What are features of a posterior circulation syndrome/infarct?
1 of:
Cerebellar/ brainstem syndromes: DANISH
LOC
Isolated homonymous hemianopia
215
How can lacunar infarcts present?
Pure motor hemiparesis – contralateral weakness of face, arm and leg with no sensory deficit
Pure sensory stroke – involving contralateral half of body
Dysarthria
Ataxic hemiparesis – on ipsilateral side
216
What are indications for immediate imaging in acute stroke?
Thrombolysis indicated
On anticoagulation
Bleeding tendency
Reduced GCS
Unexplained progressive or fluctuating symptoms
Papilloedema, neck stiffness or fever
Severe headache at onset of stroke symptoms
217
What is the management of acute stroke?
Thrombolysis with Alteplase: exclusion criteria
Aspirin 300mg (oral or rectally if dysphagic) - for 2 weeks
Homeostasis: O2 if SpO2 <95%, BM maintained between 4-11 mmol/L, Lower BP only if hypertensive emergency, <185/110 mmHg if for thrombolysis
Swallow assessment – need for NG
Dietician input
Early mobilisation when able
Surgical referral for acute intracerebral haemorrhage
218
What is multiple sclerosis?
Autoimmune demyelinating disease of the CNS characterised by repeated episodes of inflammation leading to reduced signal transmission
219
What are the patterns of MS?
Relapsing-remitting – symptoms come and go
Secondary progressive – follows on from relapsing-remitting - gradually worsening of symptoms with fewer remissions
Primary progressive – gradually worsening of symptoms from onset
Clinically isolated syndrome
220
What are signs and symptoms of MS?
Sensory symptoms of limbs and face
Unilateral visual loss with painful eye movements
Motor weakness
Diplopia
Balance problems (unsteadiness, clumsiness)
Lhermitte's sign (electric shock down spine triggered by bending head forward)
Autonomic dysfunction
221
How should MS be investigated?
```
Exclusion of differentials
Standard bloods – inflammatory markers (ESR, CRP), LFTs, U&Es
HIV serology in at risk groups
B12
Calcium
Referral to a consultant Neurologist
Electrophysiology
MRI – 95% periventricular lesions / white matter abnormalities
```
222
How is a diagnosis of MS made?
McDonald Criteria
Takes into account number of attacks and evidence of lesions on MRI (lesions at different times and different anatomical locations)
Dissemination in time and space
223
What is the management of MS?
```
Support (medical/social) - advance care planning
Lifestyle advice
Methylprednisolone for relapses
Disease modifying therapy
Interferon – no longer recommended
Dimethyl fumarate (relapsing-remitting)
Management of general symptoms: Fatigue, Pain - neuropathic, Visual problems
Physio and occupational therapy
```
224
What is motor neurone disease?
Destruction of anterior horn cells of the spinal cord and motor cranial nuclei
Leading to upper and lower motor neuron dysfunction
225
What are clinical features of motor neurone disease?
Limb weakness – wasting, fasciculations (thighs), distal weakness
Bulbar involvement – wasting and fasciculations of tongue
Respiratory involvement
Absence of major sensory symptoms and pain
226
What are the clinical patterns of motor neurone disease?
Amylotrophic lateral sclerosis: Combination of lateral corticospinal tracts and anterior horn cell disease, Progressive spastic tetraparesis, Combination UMN and LMN
Progressive muscular atrophy: Predominantly LMN lesion of the cord, weakness, wasting, fasciculations, Leading to UMN signs later in course (2 years)
Progressive bulbar palsy: UMN and LMN disorder of cranial muscles, Dysarthria and dysphagia
Primary lateral sclerosis: Isolated UMN, Slower progression
227
How does MND typically present?
Loss of dexterity, falls, trips
Speech or swallowing problems (bulbar presentation)
Muscular weakness, wasting, twitching, cramps, stiffness
Breathing problems and effects of reduced respiratory function: Orthopnea, Fatigue, Morning headache
Cognitive features
228
What is the management of MND?
No specific investigations – electrophysiology may show characteristic pattern of fibrillations and fasciculation’s
MDT approach
Respiratory function testing: SpO2 – at rest on room air plus Measurement of FVC
SpO2 of 92-94% on room air then do ABG for assessment of pCO2 and need for ventilatory support: NIV
229
What usually causes death in patients with motor neurone disease?
respiratory failure
230
What is the median survival of patients with motor neurone disease?
2-4 years
231
What is degenerative cervical myelopathy?
Cervical spinal cord compression due to cervical spondylosis
232
What is the most common cause of non traumatic tetraparesis in developed countries?
Degenerative cervical myelopathy
233
What is the name of the disability score used in MS?
Kurtzke scale
234
What features would support a diagnosis of Korsakoff's syndrome?
Anterograde amnesia
Confabulation
Lack of insight
Chronic alcoholism
235
What is brown sequard syndrome and how does it present?
Hemi-section of the cord / lateral injury of the cord
Ipsilateral spastic paresis and loss of proprioception and vibration sense
Contralateral loss of pain and temperature
236
What is guillain barre and how does it present?
Demyelination and axonal degeneration
Ascending pattern of progressive symmetricalweakness
Usually 3 weeks after viral illness (resp / GI)
Sensory symptoms may be present
237
What is Webers syndrome?
Midbrain stroke the involves fascicles of the oculomotor nerve resulting in ipsilateral cranial nerve 3 palsy and contralateral hemiplegia
238
Which medications can be used for migraine prophylaxis?
Topiramate
| Propranolol
239
Which medications should be used in an acute migraine attack?
Triptan
NSAID
Paracetamol
240
In which patients should migraine prophylaxis be offered?
2 or more attacks per month producing disability lasting 3 days or more
241
What should be offered for migraines if propranolol/topiramate fail as prophylaxis?
A course of up to 10 sessions of acupuncture over 5-8 weeks
| Gabapentin
242
What is a shagreen patch?
Roughened area of skin found in tuberous sclerosis
243
How can you differentiate between myasthenia gravis and lambert eaton?
Myasthenia gravis: weakness worsens with exercise
| Lambert eaton: weakness improves with exercise
244
How does lateral medullary syndrome present?
```
Vertigo with vomiting
Dysphagia
Nystagmus on looking to side of lesion
Ipsilateral hypotonia
Ataxia
Horners syndrome
Paralysis of soft palate
Dissociated sensory loss
```
245
What are features of myotonic dystrophy?
```
Muscle weakness
Myotonia
Ptosis
Frontal balding
Cataracts
Cardiomyopathy
Impaired intellect
Testicular atrophy
Diabetes mellitus
Dysarthria
```
246
What does a patient with Alzheimer's need to do with regards their driving?
Attend a driving assessment centre
247
For how long does a patient with first attack idiopathic epilepsy need to stop driving?
Fit free for 6 months
248
What are differential diagnoses for ring enhancing lesions in the brain?
```
Cerebral mets
Toxoplasmosis
Histoplasmosis
Abscess
Some primary brain tumours
```
249
What is miller fisher variant of guillain barre syndrome?
Starts by affecting cranial nerves
Manifests with eye signs - opthalmoplegia, areflexia and ataxia
Tend to be preceded by an infection - campylobacter jejuni
250
After what time period should anticoagulation start in patients with ischaemic stroke with AF?
14 days after onset - risk of secondary haemorrhage in early phase
251
What are absolute contraindications to thrombolysis?
```
Previous intracranial haemorrhage
Seizure at onset stroke
Intracranial neoplasm
Suspected SAH
Stroke or traumatic brain injury in previous 3 months
Lumbar puncture in previous 7 days
GI bleed in previous 3 weeks
Active bleeding
Pregnancy
Oesophageal varices
Uncontrolled HTN >200/120
```
252
What are relative contraindications to thrombolysis?
```
Concurrent anticoagulation INR >1.7
Haemorrhagic diathesis
Active diabetic haemorrhagic retinopathy
Suspected intracardiac thrombus
Major surgery/ trauma in past 2 weeks
```
253
What is the appropriate management for a patient identified to have degenerative cervical myelopathy?
Cervical decompressive surgery
254
Why does hypocalcaemia cause tetany?
Low extracellular calcium increases permeability of neuronal membranes to sodium, causing progressive depolarisation which increases possibility of action potentials
255
What is shy drager syndrome?
Type of multiple system atrophy
256
What are features of cavernous sinus syndrome?
```
Pain
Opthalmoplegia
Proptosis
Trigeminal nerve lesion (opthalmic branch)
Horners syndrome
```
257
What are features of multiple system atrophy?
Parkinsonism
Autonomic disturbance: atonic bladder, postural hypotension
Cerebellar signs
258
What are criteria for migraine without aura?
A: at least 5 points fulfilling criteria B-D
B: headache lasting 4-72 hours
C: headache with 2 of- unilateral, pulsating, moderate/severe, aggravated by or causing avoidance of routine
D: nausea/vomiting, photophobia, phonophobia
E: not attributed to another disorder
259
With which virus is nasopharyngeal carcinoma associated?
EBV
260
Why are spinal discs so much more likely to herniate posteriolaterally rather than directly posterior?
Posterior longitudinal ligament
261
Which blood tests are involved in a confusion screen?
```
FBC
U and Es
LFTs
INR
TFTs
Calcium
B12/folate/haematinics
Glucose
Blood cultures if suspicion of sepsis
```
262
What factors can trigger migraines?
```
Hormone changes
Stress
Certain foods: cheese, aspartame, MSG
Alcohol
Sensory stimuli
Changes to sleep pattern
Physical exertion
Weather changes
COCP
```
263
What are features of lateral medullary syndrome?
```
Ipsilateral ataxia
Nystagmus
Dysphagia
Facial numbness
Cranial nerve palsy
Contralateral hemisensory loss
```
264
What is Bell’s palsy?
Acute unilateral idiopathic facial nerve paralysis
Peak incidence 20-40 years
More common in pregnancy
265
What are features of Bell’s palsy?
```
Lower motor neurone facial palsy, forehead affected
Post auricular pain
Altered taste
Dry eyes
Hyperacusis
```
266
What is the management of Bell’s palsy?
Prednisolone 1mg/kg for 10 days within 72 hours of onset
| Eye care: artificial tears and eye lubricant
267
Describe the pathophysiology of papilloedema?
Axoplasmic flow stasis with intraaxonal oedema
Subarachnoid space of brain continuous with optic sheath
As CSF pressure increases, pressure is transmitted to optic nerve
Sheath acts as a tourniquet to impede axoplasmic transport
268
Which conditions are associated with a waddling gait?
Proximal myopathy - osteomalacia, diabetic amyotropy
Cushing's syndrome
Polymyositis
Metabolic derangements
269
What antiplatelets should be given when a patient has an acute stroke?
Aspirin 300mg daily for 2 weeks
Clopidogrel 75mg daily long term
Statin if not on one
270
What is an epileptic seizure?
A transient occurrence of signs and/or symptoms, the result of a primary change to the electrical activity (abnormally excessive or synchronous) in the brain
271
What is epilepsy?
A condition in which a person is prone to recurrent epileptic seizures
272
What is an epilepsy syndrome?
A distinctive disorder identifiable on the basis of a typical age of onset, seizure types, specific EEG characteristics, and often other features. Identification of epilepsy syndrome has implications for treatment, management and prognosis
273
What is status epilepticus?
When a convulsive seizure continues for a prolonged period (longer than 30 minutes), or when convulsive seizures occur one after the other with no recovery between. Convulsive status epilepticus is an emergency and requires immediate medical attention
274
What is SUDEP (sudden unexpected death in epilepsy)?
Sudden, unexplained, witnessed or unwitnessed, non-traumatic and non-drowning death in people with epilepsy, with or without evidence for a seizure, and excluding documented status epilepticus, in which post-mortem examination does not reveal a toxicological or anatomic cause for death
275
What is non epileptic attack disorder (NEAD)?
A disorder characterised by episodes of change in behaviour or movement, not caused by a primary change in electrical activity of the brain. Movements are varied, and the attacks can be difficult to differentiate from epileptic seizures
276
What are differential diagnoses for the cause of a seizure?
```
Epileptic seizure
Hypoglycaemia
CNS infection: meningitis, encephalitis, cerebral abscess, TB, malaria
Febrile convulsion
Trauma/head injury
Child abuse
Raised intracranial pressure: tumour
Recreational drug use or poisoning or self-harm (overdose)
```
277
What is the most common cause of neonatal seizures?
Hypoxic ischaemic encephalopathy
278
What are causes of neonatal seizures?
Hypoxic ischaemic encephalopathy
Intracranial haemorrhage
Metabolic disturbance: hypoglycaemia, drug withdrawal
TORCH infection
Malformation of cortical development
Benign neonatal or infantile familial convulsions
279
Why can damage occur to the brain in epilepsy?
The underlying disease
Systemic complications of the convulsions especially hypoxia from airway obstruction and later acidosis when systemic hypotension occurs
Direct injury from repetitive neuronal discharge
Increased cerebral metabolism by a factor of 3
Initially increased sympathetic activity with release of catecholamines
which leads to peripheral vasoconstriction and increased systemic blood pressure
There is loss of cerebral arterial regulation and consequential increase in cerebral blood flow
If fits continue, systemic blood pressure falls which leads to fall in cerebral blood flow
Lactic acid accumulates with subsequent cell death, oedema, free radical release, mitochondrial dysfunction and increased ICP which leads to worsening of cerebral perfusion
This associated with loss of systemic compensatory mechanisms with
resulting biochemical, renal, hepatic and cardiac failure
280
What are complications of status epilepticus?
```
Airway obstruction
Hypoxia
Aspiration
Respiratory depression secondary to excess benzodiazepines
Cardiac arrhythmias
Pulmonary oedema
Hyperthermia
Hypertension
Disseminated intravascular coagulation (DIC)
```
281
What are risk factors for the development of SUDEP?
```
Frequent seizures
Long epilepsy duration
Young adults
Early epilepsy onset
Lack of AED use
Subtherapeutic levels
Polytherapy >3 drugs
>2 AED changes per year
Rapid AED withdrawal
```
282
What are red flags for headache?
Worsening headache with fever
Sudden-onset headache reaching maximum intensity within 5 minutes
New-onset neurological deficit
New-onset cognitive dysfunction
Change in personality
Impaired level of consciousness
Recent head trauma
Headache triggered by cough, valsalva or sneeze
Headache triggered by exercise
Orthostatic headache
Symptoms suggestive of giant cell arteritis
Symptoms and signs of acute angle closure glaucoma
Substantial change in the characteristics of their headache
283
What is cerebral palsy?
Neurological disorder caused by a non-progressive brain
injury or malformation that occurs while the child’s brain is under development. Primarily affects body movement and
muscle coordination
284
What are the different types of cerebral palsy?
Spastic: hemiplegia, diplegia, quadriplegia
Athetoid
Dyskinetic
Ataxic
285
Which key professionals might need to be involved in the care of a child with cerebral palsy?
```
Health visitor and GP
Community Paediatrician/ Paediatric neurology
AHPs- OT, Physio, SALT
Orthopaedic surgeon/Spinal surgeon
Ophthalmology
Maxillofacial surgery
Community Nursing team
Educational Psychology
Special school
Social care
Respite services
Wheelchair services
Housing organisations
Family Fund and other charities
Youth services
```
286
When should treatment for epilepsy be commenced?
2 or more unprovoked seizures
287
Why is it important to classify epilepsy syndromes?
Determines choice of drug
Need for investigations
Prognosis
288
Which is the most common form of adult epilepsy?
Focal (partial) onset syndromes
289
What are the 3 main mechanisms of action of anti epileptic drugs?
Modulation of voltage gated ion channels: sodium, calcium
Enhancement of GABA mediated inhibitory neurotransmission
Decrease of glutamate mediated excitatory neurotransmission
290
Which are first line AEDs for generalised onset seizures?
Sodium valproate if male
Lamotrigine
Topiramate
Levetiracetam
291
Which are first line AEDs for focal onset seizures?
Carbamazepine
Lamotrigine
Levetiracetam
Topiramate
292
Which drugs can interact with carbamazepine?
OCP, warfarin, antispychotics,antidepressants, other AEDs, erythromycin
293
What is the main mechanism of action of carbamazepine?
inhibition of voltage-dependent Na conductance
294
What is autoinduction which occurs with carbamazepine?
Marked increase in clearance and drop in serum half-life in the first few weeks of therapy; auto-induction finished after 1 month
295
In which conditions is carbamazepine contraindicated?
AV conduction abnormalities, porphyria
296
What are adverse effects of carbamazepine?
```
5-10% skin rash
Nausea, headache, ataxia, diplopia
Hyponatraemia
Leucopenia
Rare: SJS, Haematological; thrombocytopenia, pancytopenia– Hepatotoxicity
```
297
What is the mechanism of action of lamotrigine?
Inhibition of Na channel conductance and block release glutamate
298
What are side effects of lamotrigine?
```
Headache
Nausea
Dizziness
Diplopia
Ataxia
Rash 3% approx (if started too quickly)
GI symptoms
Rarely behavioural change
Rare: Hypersensitivity syndrome, SJS, Aplastic anaemia
```
299
Which AED are strong enzyme inducers?
Carbamazepine
Phenytoin
Phenobarbitone
Primidone
300
What is the main mechanism of action of sodium valproate?
Effects on GABA and glutamergic activity, and calcium and potassium conductance
301
In which conditions is sodium valproate contraindicated?
Acute liver disease, porphyria
302
What are common side effects of sodium valproate?
```
Weight gain
Hair loss
Tremor
GI SE
PCOS
Teratogenic effects
Rare: Hepatotoxicity, Encephalopathy, Pancreatitis, thrombocytopenia
```
303
Which AEDs are category 1? What does that mean?
Phenytoin, carbamazepine, phenobarbital, primidone
| Specific measures are necessary to ensure consistent supply of a particular product
304
What is the mechanism of action of phenytoin?
Inhibition of voltage dependent Na channels
305
What are contraindications to phenytoin?
Sinus bradycardia
Sinoatrial node block
2nd and 3rd degree heartblock
Porphyria
306
What are possible IV side effects pf phenytoin?
```
Hypotension
Arrhythmias – especially prolonged QT
Thrombophlebitis at injection site
Purple glove syndrome – hand damage distal to injection site
Agranulocytosis
```
307
What are common side effects of oral phenytoin?
```
Rash 5%
Acute toxicity: lethargy, ataxia, dysarthria
Gum hypertrophy
Coarsening of face, hirsutism, acne
Macrocytosis, leucopenia
Mental slowing, drowsiness
```
308
What are potential long term consequences of phenytoin?
Cerebellar ataxia and cerebellar atrophy
Peripheral neuropathy
Osteoporosis
Folate deficiency and megalobalstic anaemia
309
What are side effects of phenobarbitone?
Sedation and cognitive side effects
Reduction of folate levels
Connective tissue (frozen shoulder, plantar fibromatosis)
Rash
Rare: Ataxia, Osteomalacia, Megaloblastic anaemia, Neuropathy
310
How long should a patient be seizure free before they can consider coming off anti epileptic drugs?
Eligible for coming off medication - seizure free for 2 years minimum
311
What are indications for monitoring drug levels of antiepileptic drugs?
```
Non-adherence
Suspected toxicity
Adjustment of phenytoin dose
Management of interactions
Specific clinical conditions. (organfailure, pregnancy, status epilepticus, interactions eg with NG feed)
```
312
Who is at increased risk of epilepsy relapse after coming off AEDs?
Epilepsy since childhood
Patients who require more than one drug to control epilepsy
Had seizures while on medication
Suffered myoclonic or tonic-clonic seizures
Had an abnormal EEG in remission
Have known underlying brain damage
313
What are rules on driving while coming off AEDs?
DVLA recommends that patients do not drive during drug withdrawal and for 6 months following withdrawal
314
What are the criteria for diagnosis of Alzheimer's?
Two or more than two cognitive impairments one being memory
Sufficiently effected to cause functional impairment
No other abnormality effecting cognition
Patient fully alert when assessed
315
What are the different stages of Alzheimer's?
MILD: Difficulties with IADL’s , score over 23
MODERATE : Unable to manage independent living : score 11 to 22.
SEVERE : PADL’s severely impaired. Score under 10
316
What is mild cognitive impairment?
Clinically difficulties with new information,as a result slow, take longer but manage functional responsibilities
317
What are different types of dementia?
Cortical : Alzheimer’s Disease, Pick’s Disease (Frontotemporal Dementia), Frontal Dementias
Sub-cortical Dementias: Extrapyramidal Syndromes (Parkinson's
Disease, Huntington’s Disease, Progressive supranuclear palsy, Wilsons Disease, Spinocerebellar degeneration, Idiopathic basal ganglia calcification)
Hydrocephalus
White Mater Diseases: Small vessel disease, (Binswanger’s Disease), Multiple Sclerosis, HIV encephalopathy
Multi Infarct dementias
Infectious dementias
Toxic and metabolic encephalopathies, systemic illnesses, Endocrinopathies,
Deficiency states, Drug intoxications, Heavy metal exposure
Miscellaneous: Post traumatic, Post anoxic, Neoplastic
318
What investigations should be done for suspected dementia?
Base Line: FBC, U and Es, LFTs, TFTs, Vitamin B12 and Folate, Glucose, Lipid Profile
Specific: Syphilis Serology, CSF, Serum copper
EEG
Brain Scanning: Structural (CT, MRI), Functional (FDG PET, FMRI, Amyloid )
319
Why are older people more at risk of delirium?
Ageing or disease of the brain
Impairment of vision and hearing
Changes in pharmacokinetics and dynamics of drugs
High prevalence of chronic diseases.
High susceptibility to acute diseases
Reduced capacity for homeostatic regulation
320
What are some non neuro complications of Parkinson's disease?
Constipation, pain, urinary dysfunction, dysphagia, drooling, speech problems, depression, anxiety, dementia, psychosis, sexual dysfunction, sleep disturbance, orthostatic hypotension and hyposmia
321
What are the clinical stages of Parkinson's disease?
Diagnosis: recognition, treatment initiated
Maintenance: monitor symptoms, avoid complications
Complex: motor fluctuations, drug side effects, non motor symptoms, falls
Palliative: reduced efficacy of treatment, QOL/end of life issues
322
What motor fluctuations can occur in the complex stage of Parkinson's disease?
Freezing: Common during “offs” but can also occur when “on”
Wearing off: End of dose deterioration (motor and non motor), May be predictable or unpredictable, sudden or gradual
On and Off phenomena
Delayed “on” or no “on” (ie dose failures)
Dyskinesia: fidgety, wriggly snake like movements
Dystonia: May occur off (wearing off, early morning) but can occur at peak of dose, Painful
323
What is the striatum?
Caudate nucleus and the putamen which are separated by the internal capsule
324
What is the function of the striatum?
Responsible for bringing together the dopamine signals
| from the substantia nigra with the voluntary movement signals from the prefrontal cortex
325
What classes of drugs can be used to treat Parkinson's?
```
Levodopa/carbidopa
Dopamine agonists
MAO-B inhibitors
COMT inhibitors
Amantadine
Continuous dopaminergic stimulation (CDS)
Acetylcholinesterase inhibitors
```
326
What are the 2 main preparations of levodopa used for Parkinson's?
Sinemet (carbidopa) co-careldopa
| Madopar (benserazide) co-beneldopa
327
What are some short term side effects of levodopa?
GI: N and V, Loss of appetite
Cardiovascular: Postural hypotension
Sleep: Somnolence, Insomnia, Vivid dreams, nightmares, Inversion of sleep-wake cycle
Psychiatric: Confusion, Visual hallucinations, Delusions, illusions
328
What are some long term side effects of levodopa?
Involuntary movements: Peak-dose dyskinesia, Diphasic dyskinesia, Dystonia
Response fluctuations: Wearing off, Unpredictable on/off
Psychiatric: Confusion, Visual hallucinations, Delusions, illusions
329
What are side effects of MAOIB drugs such as selegiline?
Hallucinations, insomnia, nightmares, vivid dreams
| Postural hypotension, nausea, confusion
330
What are some benefits of using rasagaline alongside other Parkinson's drugs?
Reduces off time by 48-56 mins/day
Increases on time without dyskinesias
Similar in efficacy and tolerability to entacapone
331
What are some problems with ergot derived dopamine agonists like bromocriptine, pergolide and carbergoline?
Cardiac valvulopathy
| Pulmonary, retroperitoneal, and pericardial fibrotic reactions
332
What are some common side effects of dopamine agonists?
```
N and V, loss of appetite
Postural hypotension
Confusion, hallucinations
Somnolence
Impulse control disorders in up to 20%
```
333
What are some examples of dopamine agonists used in Parkinson's?
Ropinirole
Pramipexole
Rotigotine patch
Apomorphine s/c
334
What are side effects of COMT inhibitors?
```
Dyskinesia (so decrease levodopa)
Diarrhoea
Nausea
Somnolence
Abdo pain
Discoloured urine (body fluids orange)
Hepatic toxicity (tolcapone): Rigorous blood monitoring, Stop if AST or ALT exceed upper limit of normal
```
335
What forms of continuous dopaminergic stimulation are available for Parkinson's?
Apomorphine
Duodopa
Deep brain stimulation
336
Which drugs are usually used in early Parkinson's disease?
Dopamine agonist
Levodopa
MAO B inhibitors
337
What can be causes of confusion and agitation in a Parkinson's disease patient?
Co-morbid medical illness (especially infection, metabolic upset)
Side-effects of the PD medication itself
Progression of Parkinson's disease (development of PD dementia)
338
What can be used to manage confusion and agitation in Parkinson's disease?
```
Lorazepam prn (0.5-1mg PO, max 4mg in 24 hours)
Quetiapine (12.5mg od to 25mg bd)
Clozapine (12.5 - 50mg daily)
Cholinesterase inhibitors
Do not use anti dopaminergics!
```
339
What acetylcholinesterase inhibitors are used in dementia?
Rivastigmine licensed for use in mild to moderate PD dementia
Tablets: 1.5mg bd (max 6mg bd)
Patch: 4.6mg/24 hours (max 9.5mg/24 hours)
Donepezil, Galantamine (not licensed for PD dementia)
NMDA receptor antagonists: Memantine
340
Which drugs should be avoided in Parkinson's?
Anything that blocks dopamine
Anti-emetics: Prochlorperazine, Metoclopramide, cyclizine
Antipsychotics: Chlorpromazine, promazine. Fluphenazine, perphenazine, prochlorperazine, trifluoperazine, Haloperidol
341
What are the aims of treatment of stroke?
Reduce the ongoing neurological injury
Reduce mortality and long-term disability
Prevent complications
Prevent stroke recurrence
342
When is tPA licensed for stroke treatment?
When given within 4.5 hours of symptom onset in 18-80 year olds with no evidence of haemorrhage
Stroke symptoms present at least 30 minutes
A clearly measurable deficit (NIHSS - national institute of health stroke scale)
343
What should be given in acute stroke if thrombolysis is not an option?
Aspirin 300mg stat (PO, PR, NG)
344
What are contraindications to thrombolysis for stroke?
Other life threatening illness
Haemorrhagic retinopathy
Oesophageal varices
Recent (within 10 days) traumatic external cardiac massage, puncture of a non-compressible vessel
Manifest or recent severe or dangerous bleeding
Recent major surgery or trauma
Neoplasm with increased bleeding risk
Severe liver disease
Recent ulcerative GI disease
Acute pancreatitis
Bacterial endocarditis, pericarditis
Abnormal clotting/haemorrhagic diathesis
Platelet count <100x109/l
CT evidence of haemorrhage
Glucose >22.2mmol/l or <2.8mmol/l
Pretreatment systolic BP >185 mmHg or diastolic BP >110 mmHg
Severe stroke assessed by NIHSS (>25) or by imaging
Unknown time of onset
Symptoms suggestive of subarachnoid haemorrhage, even if CT is normal
345
What are relative contraindications or times when caution should be used in thrombolysis for stroke?
Pregnant
Minor symptoms e.g. NIHSS < 5
Significant symptoms may be present even with NIHSS 0-4 e.g. dysphasia
Symptoms rapidly improving, consider treatment if significant symptoms still present after 30 mins
On Warfarin/heparin /LMW heparin/other anticoagulants, INR should be 1.7 or less
NOAC: consider tPA if starting 24 hours after last dose of NOAC (12-24 hrs if Dabigatran provided APTT is 1.3 or less)
Seizure at stroke-onset (seizure per se is not a CI but make sure weakness is not due to post-seizure paralysis)
Age > 80 years, consider ‘biological’ age
Patient dependent prior to stroke, consider level of dependency
Prior stroke and concomitant diabetes, consider current diabetic control, time interval between previous and current stroke,
level of dependency prior to current stroke
Stroke within the last 3 months, 6-8 weeks if the initial stroke was minor
Arterial aneurysm, AV malformations: small, incidental cerebral aneurysms may be allowed
346
What are complications of thrombolysis for stroke?
Hypotension (slow infusion down)
Nausea and vomiting
Stop tPA infusion if: Major systemic bleeding, Anaphylaxis, Urticaria, bronchospasm, angioedema, hypotension/shock, Suspected ICH
Neuro deterioration (GCS drop or rise >2 NIHSS points)
New headache
Sustained hypertension
347
After considering thrombolysis for stroke, what is the next management?
If given thrombolysis: Repeat CT head at 24 hours, If no haemorrhage start Aspirin 300mg OD
If not thrombolysed and no haemorrhage: Start Aspirin 300mg OD
Continue Aspirin 300mg od for total 2 weeks (then clopidogrel 75mg /day thereafter)
If Aspirin cannot be given: Clopidogrel 300mg single dose on day 1 and then Clopidogrel 75mg OD thereafter
Avoid anticoagulation - even in AF, Consider if required 2 weeks after acute stroke
348
How should blood pressure be managed in acute stroke?
Aim initially for BP of 160-180 / 90-105
Treat if BP > 220/120 or if diastolic > 140
Labetalol iv 2-10mg over 2-5 mins
GTN infusion: bolus 5mg iv, then 1-4mg/hour
349
What do new guidelines say about when patients should be seen after a TIA?
All patients with suspected TIA should be seen in 24hrs IRRESPECTIVE of risk– Seen in 7 days if symptoms from over 1 wk ago
Previously used ABCD2 score to determine risk
High risk (4-7): seen within 24 hours
Crescendo: may need admission
Low risk (0-3): assess and investigate within 7 days
350
What are the components of the ABCD2 score?
```
Age 60 or over
Blood pressure over 140/90
Clinical features: unilateral weakness (2), speech disturbance (1)
Duration: >60 mins (2), 10-59 mins (1)
Diabetes
```
351
How should TIA be managed?
All patients with suspected TIA get aspirin 300mg at presentation unless contraindicated
All patients with proven TIA clopidogrel 300mg
Day 2 onwards: Clopidogrel 75 mg OD, High intensity statin therapy (eg atorvastatin 20-80mg daily)
All patients need carotid dopplers within 24 hrs (if significant stenosis use second mode of imaging that is non invasive to confirm)
Anticoagulation: If patient is in AF, start anticoagulation immediately if no contraindications (CT head first to exclude haemorrhage)
Blood pressure: Aim for secondary prevention target of < 130/80
352
What are risk factors for stroke?
Non-modifiable: age, male, familial predisposition
Blood pressure: risk doubles for every 7.5 mmHg increase in diastolic BP
Cholesterol: increase with total cholesterol, LDL cholesterol and low HDL levels
Cigarette smoking: doubles risk of stroke death
AF: 4% annual risk (increase to 12% if previous TIA/stroke), 5 fold increase risk of stroke
Diabetes: 2-4 fold increase risk of stroke
Valvular heart disease: risk of 10-20% per yr
Carotid stenosis: risk is 2% per yr in asymptomatic stenosis of 75%, increase to 15% if recent event
Other: OCP, HRT, physical inactivity, obesity
353
What are contraindications to the use of NOACs?
Presence of prosthetic heart valve (bioprosthetic and mechanical valves although edoxaban is approved with bioprosthetic valves after the first 3 months)
Renal impairmen
Concomitant use of prohibited medications including HIV protease
inhibitors, systemic azoles, dronedarone, rifampicin, carbamazepine, phenytoin
Liver disease eg enzymes > x2 upper limit of normal
354
What are the components of the CHA2DS2VASC score?
```
Congestive heart failure
HTN
Aged 75 or more (2)
Diabetes
Stroke/TIA (2)
Vascular disease
Aged 65-74
Sex female
```
355
What are the components of the has bled score?
```
HTN
Abnormal renal and liver function (1 point each)
Stroke
Bleeding
Labile INR
Elderly
Drugs or alcohol (1 point each)
```
356
How may a brain tumour present?
Space occupying lesion / raised intracranial pressure
Headache resistant to pain relief
Vomiting
Epilepsy
Altered state of consciousness
Frontal lobe: changed mood behavior reasoning disturbed speech
Temporal lobe: disturbed memory, language, reasoning, temporal
lobe epilepsy
Parietal lobe: aphasia, disturbed sensory or motor function poor spatial visual perception
Occipital lobe: disturbed vision and visual loss
Cerebellum: disturbed balance and gait
357
What is the WHO grading system for CNS tumours?
Grade I tumors are slow-growing, nonmalignant, and associated with long-term survival, Low cellularity, No pleomorphism
Grade II tumors are relatively slow-growing but sometimes recur as higher grade tumors. They can be nonmalignant or malignant, Low to moderate cellularity, Mild pleomorphism
Grade III tumors are malignant and often recur as higher grade tumors, High cellularity, Infiltrative, Definite pleomorphism, mitoses
Grade IV tumors reproduce rapidly and are very aggressive malignant
tumors, Vascular proliferation, Tumour necrosis
358
What is MEN1?
Pituitary adenoma
Pancreas tumours
Parathyroid hyperplasia
359
What is tuberous sclerosis?
Multi-system disease
Benign tumours (hamartomas) in brain, heart (rhabdomyomas) kidney (angiofibroma) lung (lymphangioleiomyomatosis), skin (adenoma subaceum)
Mutations in tumour supressor genes TSC1 (Hamartin) TSC2(Tuberin)
CNS involvement: Cortical Tubers, Sub-ependymal nodules, Giant cell astrocytomas, Seizures, developmental delay, behavioural problems
360
What is neurofibromatosis type1?
```
Autosomal dominant
Deletion in neurofibromin gene Chr 17q11.2
Negative regulator of RAS
Multiple schwannomas and neurofibromas
Peripheral nerves
Multiple musculoskeletal abnormalities
Café au-lait patches on the skin
Lisch nodules on the iris
Cognitive learning disability
```
361
What is neurofibromatosis type2?
NFII merlin or schwannomin chr 22q11-13.1
Acoustic neuroma (vestibular neuroma), Cerebropontine angle
50% develop tumours in other cranial nerves and spinal cord
Skin tumours
cataracts
362
What is carneys complex?
Myxoma of heart and skin
Hyperpigmentation of skin (lentiginosis)
Primary pigmented nodular adrenocortical disease (pituitary tumour)
363
What is the different between a macro and micro pituitary adenoma?
10mm or more macro-adenomas, under 10mm microadenomas
364
What are the 4 main types of glial cell?
Astrocytes, oligiodendrocytes, ependyma and microglial cells
365
What is a craniopharygioma?
Arises in the pituitary stalk
Derived from Rathke’s pouch
Sqamous epithelial tumour
Locally invasive
366
Which spinal level is affected to cause paraplegia?
T1-L5
367
Which spinal level is affected to cause tetraplegia?
C1-C8
368
What are red flags in headache?
```
Early Morning Headache
Vomiting with Headache
Vision Problems
Sudden Onset Headaches
Thunderclap Headache
Fever and rash, neck stiffness/photophobia
```
369
What is management for tension headache?
```
Simple analgesics
Stress reduction
Acupuncture
Lifestyle changes: Reduce caffeine, cheese, red meat, weight loss, stay hydrated
? amitriptyline
```
370
What is the management of migraine?
Lifestyle: avoid triggers, headache diary
Terminate: Sumitriptan, NSAID, paracetamol
Sickness: Metoclopramide, Prochlorperazine, domperidone
Prevention: Propranolol, topiramate, up to 10 sessions of acupuncture over 5-8 weeks if medication ineffective, riboflavin
371
What are triggers for migraine?
```
Menstruation
Altered sleep
Stress
Food: chocolate, cheese, caffeine, alcohol
Strong smells
Bright light
Dehydration
Missed meals
Jet lag
Strenuous exercise
```
372
In which migraine patients should prevention medication be considered?
>4 attacks per month
Producing disability lasting 3 days or more
Person at risk of medication overuse headache
Standard analgesia and triptans are contraindicated/ineffective
373
What specific questions should you ask to diagnose GCA?
```
Headache?
Jaw claudication?
Scalp tenderness?
Visual disturbances?
Proximal limb muscle pain and tenderness?
```
374
What clinical findings might you expect with temporal arteritis?
Non-pulsatile temporal artery
Tenderness over temporal artery
As it is a vasculitis need to examine whole peripheral vascular system
375
How do you manage GCA?
Take blood for inflammatory markers: ESR
Start prednisolone 60mg daily oral
Counsel with regard to medication and side-effects
Start aspirin 75 mg daily (unless contra-indications)
Start gastroprotection with PPI in view of added risk of peptic ulceration with high-dose steroids and aspirin
Low-dose aspirin has been shown to decrease rate of visual loss and strokes in patients with GCA
Arrange temporal artery biopsy
Arrange review in next few days
Safety-net: if visual problems, go to A and E
376
What are characteristics of a migraine headache?
Paroxysmal headaches, severe unilateral, although 30-40% bilateral
Premonitory phase in 20-60%
Aura
Photophobia, vomiting, nausea with marked headache but course highly variable
Resolution phase occurs as headache gradually fades
Feel tired, irritable, depressed and have difficulty concentrating
377
What is a premonitory phase in migraine?
Different from an aura, occurs hours to days before headache
Feeling that a migraine is imminent
Depression, tiredness, difficulty concentrating, irritability, stiff neck and food cravings
Many different features can be present but they tend to be consistent for the individual
378
What is a migraine aura?
Progressive, gradual onset over minutes, last five minutes to an hour and occur before headache
Visual disturbance starts in one eye and may spread. It may be homonymous. Is fully reversible
Sensory symptoms (paraesthesia or numbness) are unilateral and fully reversible. Numbness usually starts in hand and moves up arm before involving face, lips and tongue. Leg is sometimes affected. Numbness may follow paraesthesia. Sensory auras rarely occur alone and usually follow visual auras
Aura has no motor symptoms
Headache either begins before end of aura or within an hour of the end and has the same features as migraine without aura
One patient may at different times have migraine attacks with and without aura
Sometimes no headache may follow a typical aura - but always consider possibility of transient ischaemic attack (TIA) rather than migraine in these patients, particularly if the patient says the aura was different from their usual aura
379
What are contraindications to use of triptans for migraine?
Uncontrolled hypertension
Coronary heart disease or cerebrovascular disease
Risk factors for coronary heart disease or cerebrovascular disease
Coronary vasospasm (Prinzmetal's angina)
380
Which migraine medications can be used in pregnancy?
Paracetamol
381
What is a medication overuse headache?
Headache developing as a consequence of regular overuse of acute or symptomatic headache medication for over 3 months
Use of simple analgesics and NSAIDs on 15 days or more per month
Triptans, opioids and combination analgesics on 10 days or more per month
382
What is the management of medication overuse headache?
Stop analgesics abruptly or
Wean down by increasing, by 1 day each week, number of
medication-free days
Opiates and codeine-based medication may need to be gradually reduced, as withdrawal symptoms can be severe
383
In patients with total loss of consciousness, which features would warrant urgent referral for cardiovascular assessment?
An ECG abnormality
Heart failure (history or physical signs)
TLoC during exertion
Family history of sudden cardiac death in people aged younger than 40 years and/or an inherited cardiac condition
New or unexplained breathlessness
A heart murmur
Consider referring within 24 hours for cardiovascular assessment, as above, anyone aged older than 65 years who has experienced TLoC without prodromal symptoms
384
Which patients with total loss of consciousness need referring for assessment of epilepsy?
A bitten tongue
Head-turning to one side during TLoC
No memory of abnormal behaviour that was witnessed before, during or after TLoC by someone else
Unusual posturing
Prolonged limb-jerking (note that brief seizure-like activity can often occur during uncomplicated faints)
Confusion following the event
Prodromal déjà vu, or jamais vu
385
Which medications can cause tremor?
Atypical antipsychotics
Anti-nausea agents (metoclopramide, prochlorperazine, promethazine)
Calcium-channel blockers (flunarizine, cinnarizine)
Dopamine depletors (reserpine, tetrabenazine)
Antidepressants (SSRI, TCA, MAOI)
Mood stabilisers (lithium)
Antiepileptic drugs (valproic acid)
Cardiac drugs (amiodarone)
Immunosuppressants (ciclosporin, tacrolimus, corticosteroids)
Anti-asthma drugs (salbutamol, theophylline)
Stimulants (amphetamines)
386
What investigations can be done for tremor?
Renal, liver, bone biochemistry
TFTs
May need MRI or CT brain
May need 24-hour urinary copper and serum caeruloplasmin if suspect Wilson's disease
387
What are causes of tremor?
```
Parkinson's disease
Lewy body dementia
Hypoglycaemia
Thyrotoxicosis
Alcohol withdrawal
Essential tremor
Drug-induced
Uncommon: Multiple system atrophy, Progressive supra-nuclear palsy, Cortical basal degeneration, Toxin-induced, Phaeochromocytoma, Cerebellar tremor (MS, trauma, stroke) Fragile X tremor ataxia syndrome (FXTAS), Orthostatic tremor, Primary writing tremor, Neuropathic tremor, Wilson's disease, Rubral tremor (combined coarse rest and action tremor), Psychogenic tremor
```
388
What is Todds paresis?
Transient weakness of hand, arm or leg after focal seizure activity within that limb
389
Which tests are part of a confusion screen?
```
AMTS
FBC
U and Es
LFTs
Calcium
Glucose
TFTs
B12
Folate
```
390
What are features of myasthenia gravis?
```
Muscle fatigability
Extraocular muscles: diplopia
Proximal muscle weakness: face, neck, limb girdle
Ptosis
Dysphagia
```
391
What are associations of myasthenia gravis?
Thymoma
Thymic hyperplasia
Autoimmune disorders: pernicious anaemia, autoimmune thyroid disease, rheumatoid, SLE
392
How should myasthenia gravis be investigated?
Single fibre electromyography
CT thorax to exclude thymoma
CK (normal)
Autoantibodies to ACh receptor, muscle specific tyrosine kinase antibodies
Tensilon test: IV edrophonium reduces muscle weakness temporarily (risk of cardiac arrhythmia)
393
What is the management of myasthenia gravis?
Long acting anticholinesterase: pyridostigmine
Immunosuppressants: prednisolone initially
Thymectomy
Myasthenic crisis: plasmapheresis, IV immunoglobulins
394
Which drugs are associated with idiopathic intracranial HTN?
Isotretinoin
Tetracycline antibiotics
Hormonal contraceptives
395
Which migraine auras would be considered atypical and may prompt further investigation?
```
Motor weakness
Double vision
Unilateral visual symptoms
Poor balance
Decreased consciousness
```
396
What are features of multiple system atrophy?
Parkinsonism
Autonomic disturbance: atonic bladder, postural hypotension
Cerebellar signs
397
What does the facial nerve supply?
Muscles of facial expression
Stapedius
Taste anterior 2/3 tongue
Parasympathetic to lacrimal and salivary glands
398
What are causes of bilateral facial nerve palsy?
Sarcoidosis
Guillain barre syndrome
Polio
Lyme disease
399
What are causes of unilateral facial nerve palsy?
```
Stroke (UMN)
Bell’s palsy
Ramsay hunt syndrome
Acoustic neuroma
Parotid tumour
HIV
MS
Diabetes
Sarcoidosis
Guillain barre syndrome
Polio
Lyme disease
```
400
What is the difference between upper and lower motor neuron lesions of the facial nerve?
UMN: spares upper face
LMN: affects all facial muscles
401
What are neurological causes of SIADH?
```
Surgical or traumatic damage to brain
Encephalitis/brain abscess
Brian tumour
Meningitis
Guillain barre
Cerebrovascular accidents
Subdural haematoma
Tumour of 4th ventricle
Neonatal hypoxia
Hydrocephalus
```
402
What is Gaucher’s disease?
Inherited autosomal recessive disorder of lipid metabolism due to beta glucosidase deficiency
Causes accumulation of glycolipids in phagocytic cells and cells of CNS
Features: hepatosplenomegaly, neuro dysfunction, bone erosions of long bones, pingueculae (orange wedge shaped deposits in subconjunctiva and yellow brown discolouration of skin in areas exposed to sun
403
What is the difference between Guillain barre and miller fisher syndrome?
Guillain barre: paralysis without sensory loss, areflexia
| Miller fisher: areflexia, ataxia, opthalmoplegia, no weakness
404
What are type 1 and 2 Charcot Marie tooth?
Inherited motor and sensory neuropathy
Type 1: demyelinating, autosomal dominant
Type 2: axonal, can be dominant or recessive
405
What abnormalities would you expect with superficial peroneal nerve damage?
Paraesthesia in central dorsum of foot and proximal toes except 5th
Weakness of eversion of foot
406
What are chronic neurological sequelae from kernicterus?
Athetosis
Gaze disturbance
Hearing loss
407
What is the world federation of neurological societies grading system for SAH?
```
Grade 1: GCS 15
Grade 2: GCS 13-14 no deficit
Grade 3: GCS 13-14 focal deficit
Grade 4: GCS 7-12
Grade 5: GCS <7
```
408
Which part of the cerebellum is affected if there is truncal ataxia with minimal limb signs?
Cerebellar vermis
| Hemispheres if limb signs and less truncal
409
What are causes of Cerebellar syndrome?
MS
Stroke
Alcohol
Phenytoin
410
What are differences between Cerebellar and vestibular nystagmus?
Cerebellar: fast towards lesion and worse towards affected side
Vestibular: fast away from lesion and worse looking away
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In a patient with upper motor neurone signs in the legs only, where is the lesion?
In spinal cord, above L1
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What is pyramidal weakness?
Flexors in arms stronger than extensors
| Extensors in legs stronger than flexors
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What are differentials for a mixed picture of upper and lower motor neurone signs?
MND
Freidreichs ataxia
Subacute combined degeneration of the cord
Tabes dorsalis
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How can you examine for myotonia?
Thenar eminence tap, slow to relax
| Grip of hands, difficult to release
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What is pronator drift a sign of?
UMN pyramidal lesion
