Neurology Important P2 Flashcards
1
Q
What is narcolepsy:
A
- HLA DR2
- low levels of orexin (responsible for appetite and sleep patterns)
- early onset of REM sleep
2
Q
Features of narcolepsy:
A
- teenage onset
- hypersomnolence
- cataplexy
- sleep paralysis
- vivid hallucinations on going to sleep or waking up
3
Q
Investigation of narcolepsy:
A
multiple sleep latency EEG
4
Q
Management of narcolepsy:
A
daytime stimulants e.g. modafinil and nighttime sodium oxybate
5
Q
Neurofibromatosis I
A
- von Recklinghausen’s syndrome
- gene mutation on chromosome 17
- encodes neurofibromas
- autosomal dominant
- cafe au lait spots
- axillary/groin freckles
- peripheral neurofibromas
- iris hamatomas
- scoliosis
- phaeochromocytomas
6
Q
Neurofibromatosis II
A
- bilateral vestibular schwannomas
- multiple intracranial schwannomas
- meningiomas and ependymomas
- chromosome 22
- autosomal dominant
7
Q
How does neuroleptic malignant syndrome come about?
A
- antipsychotic medication
- dopaminergic drugs e.g. levodopa for Parkinson’s
- usually when suddenly stopped or dose reduced
- massive glutamate release causes neurotoxicity and muscle damage
8
Q
Typical features of neuroleptic malignant:
A
- pyrexia
- muscle rigidity
- autonomic lability: hypertension, tachycardia and tachypnoea
- agitated delirium with confusion
- raised creatinine kinase
- AKI secondary to rhabdomyolysis
- leukocytosis
9
Q
Management of neuroleptic malignant syndrome:
A
- stop antipsychotic
- ICU
- IV fluids to prevent renal failure
- dantrolene
- bromocriptine, dopamine agonist
10
Q
Which conditions commonly cause neuropathic pain?
A
- diabetic neuropathy
- post herpetic neuralgia
- trigeminal neuralgia
- prolapsed intervertebral disc
11
Q
Management of neuropathic pain:
A
- first line: amitriptyline, duloxetine, gabapentin, pregabalin (try all)
- switch drugs rather than adding
- tramadol as rescue therapy
- topical capsaicin
- pain management clinic
12
Q
What is normal pressure hydrocephalus?
A
- reversible cause of dementia
- secondary to reduced CSF absorption at arachnoid vili
- triad: urinary incontinence, dementia and bradyphrenia, gait abnormality
13
Q
Investigations and management of normal pressure hydrocephalus?
A
- hydrocephalus with an enlarged fourth ventricle
- ventriculomegaly, absence of substantial sulcal atrophy
- manage with ventriculoperitoneal shunting
14
Q
Metabolic consequences of refeeding syndrome:
A
- hypophosphataemia
- hypokalaemia
- hypomagnesaemia
- abnormal fluid balance
15
Q
Management of refeeding syndrome:
A
- start with 10kcal/kg/day increasing to full needs over 4-7 days
- oral thiamine 200-300mg/day, vit B co strong 1 tds and supplements immediately before and during feeding
- give potassium (2-4mmol/kg/day), phosphate (0.3-0.6mmol/kg/day), magnesium (0.2-0.4mmol/kg/day)
16
Q
What is Parkinson’s disease:
A
- progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in substantia nigra
- triad: bradykinesia, tremor and rigidity
- asymmetrical
17
Q
Tremor in Parkinson’s disease:
A
- most marked at rest 3-5Hz
- worse when stressed or tired, improves with voluntary movement
- typically pill rolling
18
Q
Bradykinesia in Parkinson’s disease:
A
- poverty of movement (hypokinesia)
- short, shuffling steps with reduced arm swinging
- difficulty in initiating movement
19
Q
Rigidity in Parkinson’s:
A
- lead pipe
- cogwheel
20
Q
Other characteristic features of Parkinson’s:
A
- mask like facies
- flexed posture
- micrographia
- drooling of saliva
- psychiatric features
- impaired olfaction
- REM sleep behaviour disorder
- fatigue
- autonomic dysfunction (postural hypotension)
21
Q
Drug induced parkinsonism:
A
- motor symptoms generally rapid onset and bilateral
- rigidity and rest tremor uncommon
22
Q
Diagnosis of Parkinson’s:
A
- usually clinical
- 123I-FP-CIT SPECT
- Lewy bodies (stained brown)
- discolouration of substantia nigra due to loss of pigmented nerve cells
23
Q
Causes of Parkinsonism:
A
- Parkinson’s disease
- drug induced e.g. antipsychotics, metoclopramide
- progressive supra nuclear palsy
- multiple system atrophy
- Wilson’s disease
- post-encephalitis
- dementia pugilistica
- toxins: carbon monoxide, MPTP
24
Q
Predominantly motor loss peripheral neuropathy:
A
- Guillain-Barre
- porphyria
- lead poisoning
- hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- diphtheria
25
Predominately sensory loss peripheral neuropathy:
- diabetes
- uraemia
- leprosy
- alcoholism
- vitamin B12 deficiency
- amyloidosis
26
Alcohol neuropathy:
- secondary to both direct toxic effects and reduced absorption of B vitamins
- sensory symptoms prior to motor symptoms
27
How does vitamin B12 deficiency cause peripheral neuropathy:
- subacute combined degeneration of spinal cord
| - dorsal columns usually affected first (joint position, vibration) prior to distal paraesthesia
28
Idiosyncratic ADR of phenytoin:
- fever
- rashes e.g. toxic epidermal necrolysis
- hepatitis
- Dupuytren's contracture
- aplastic anaemia
- drug-induced lupus
29
What teratogenic effect dose phenytoin have?
- cleft palate
| - congenital heart disease
30
What is progressive supra nuclear palsy?
- Steele-Richardson-Olszewski syndrome
- Parkinson Plus syndrome
- poor response to L-dopa
31
Features of progressive supra nuclear palsy:
- postural instability and falls: stiff, broad-based gait
- impairment of vertical gaze (difficulty reading or descending stairs)
- parkinsonism: bradykinesia prominent
- cognitive impairment: primarily frontal lobe dysfunction
32
Factors favouring psychogenic non-epileptic seizures:
- pelvic thrusting
- family member with epilepsy
- much more common in females
- crying after seizure
- not when alone
- gradual onset
33
Factors favouring true epileptic seizures:
- tongue biting
| - raised serum prolactin
34
Origination of radial nerve:
continuation of posterior cord of brachial plexus (root values C5-T1)
35
Regions innervated by radial nerve:
```
motor (main nerve):
-triceps
-anconeus
-brachioradialis
-extensor carpi radialis
motor (posterior interosseous branch)
-supinator
-extensor carpi ulnaris
-extensor digitorum
-extensor indicis
-extensor digiti minimi
-extensor pollicis longis and brevis
-abductor pollicis longus
sensory: proximal phalanges on dorsal hand (not little finger and part of ring finger)
```
36
Effect of radial paralysis at shoulder:
- affects long head of triceps (extension of elbow)
| - minor effect on shoulder stability in abduction
37
Effect of radial paralysis in arm:
- affects triceps
| - loss of elbow extension
38
Effect of radial paralysis in forearm:
- supinator, brachioradialis, extensor carpi, radialis longus and brevis
- weakning of supination of prone hand and elbow flexion in mid prone position
39
Patterns of damage in radial nerve:
- wrist drop
| - sensory loss to small area between dorsal 1st and 2nd metacarpals
40
Normal ICP in supine position:
7-15mmHg
41
Causes of raised ICP:
- idiopathic intracranial hypertension
- traumatic head injuries
- infection
- tumours
- hydrocephalus
42
Features of raised ICP:
- headache
- vomiting
- reduced consciousness
- papilloedema
- Cushing's triad: widening pulse pressure, bradycardia, irregular breathing
43
At what ICP level is treatment considered?
>20mmHg
44
Management of raised ICP:
- underlying
- head elevation 30 degrees
- IV mannitol as osmotic diuretic
- controlled hyperventilation: aim to reduced pCO2 - vasoconstriction of cerebral arteries (caution for ischaemic parts of brain)
- drain from intraventricular monitor
- repeated lumbar puncture
- ventriculoperitoneal shunt (for hydrocephalus)
45
Ankle reflex root:
S1-2
46
Knee reflex root:
L3-L4
47
Biceps reflex root:
C5-6
48
Triceps reflex root:
C7-8
49
Acute seizures rectal diazepam dose adults:
10-20mg
50
Acute seizures midazolam oromucosal solution adults:
10mg (unlicensed)
51
Motor lesions:
ALS:
-both upper and lower motor neurons
Poliomyelitis:
-affects anterior horns resulting in lower motor neurone signs
52
Brown sequard syndrome tracts affected:
-lateral corticospinal tract
-dorsal columns
-lateral spinothalamic tract
(ipsilateral spastic paresis below, ipsilateral loss of proprioception and vibration, contralateral loss of pain and temp sensation)
53
Friedrich's ataxia tracts affected:
-same as subacute combined degeneration of spinal cord
-lateral corticospinal tracts
-dorsal columns
-spinocerebellar tracts
(bilateral spastic paresis, bilateral loss of proprioception and vibration sensation, bilateral limb ataxia)
54
Anterior spinal artery occlusion tracts affected:
-lateral corticospinal tracts
-lateral spinothalamic tracts
(bilateral spastic paresis, bilateral loss of pain and temperature sensation)
55
Syringomyelia tracts affected:
-ventral horns
-lateral spinothalamic tract
(flaccid paresis, loss of pain and temp sensation)
56
Multiple sclerosis tracts affected:
- asymmetrical
- varying spinal tracts
- combination of motor, sensory and ataxia
57
Neurosyphilis tracts affected:
- dorsal columns
| - loss of proprioception and vibration sensation
58
Risk factors spontaneous ICH:
CTD - Marfan's
59
Features of spontaneous ICH, investigations and management:
- strong postural relationship
- worse upright
- MRI with gadolinium: pachymeningeal enhancement
- manage conservatively or epidural blood patch
60
Management status epilepticus:
- ABC
- benzodiazepines: diazepam or lorazepam
- prehospital: rectally
- hospital: IV lorazepam (repeat after 10-20 minutes)
- on going: phenytoin or phenobarbital infusion
- no response within 45 minutes: general anaesthesia
61
What sign can you use to differentiate between organic and non-organic lower leg weakness?
Hoover's sign
62
Paralysis of which nerve is likely to result in hyperacusis?
facial nerve
63
What type of dementia does motor neurone disease put you at increased risk of?
frontotemporal dementia
64
What is subacute combined degeneration of spinal cord?
- due to vitamin B12 deficiency
- dorsal and lateral columns affected
- joint position and vibration sense lost first then distal paraesthesia
- UMN signs in legs (extensor planters, brisk knee reflexes, absent ankle jerks)
- if untreated stiffness and weakness persist
65
Acute subdural haematoma:
- most commonly high impact trauma
- CT first line
- crescenteric collection not limited by suture lines
- hyperdense
- can cause midline shift or herniation
- small or incidental - conservative
- monitor ICP and decompressive craniectomy
66
Chronic subdural haematoma:
- wekks to months
- rupture of small bridging veins in subdural space rupture and cause slow bleeding
- elderly and alcoholics (brain atrophy)
- shaken baby syndrome
- crescenteric not restricted by suture lines
- hypodense
- if neurological deficit or severe image findings: surgical decompression with burr holes
67
What is syringomyelia:
- collection of CSF in spinal cord
- caused by Chiari malformation, trauma, tumours, idiopathic
- cape like loss of sensation to temperature
- preserved light touch proprioception and vibration
- 'accidentally burning without realising'
- due to crossing spinothalamic tracts in anterior commissure of spinal cord being first affected
- spastic weakness upper limbs, paraesthesia, neuropathic pain, upping planters and bowel and bladder dysfunction
- scoliosis over years, Horner's syndrome
- full spine MRI
68
Characteristics of tension-type headaches:
- tight band around head
- bilateral
- lower intensity than migraine
- no aura, n&v, aggravation by routine physical activity
- may be stress related
- may co-exist with migraine
69
What qualifies as chronic tension headaches?
15 or more days per month
70
Treatment of tension headaches:
aspirin, paracetamol or NSAID
71
Third nerve palsy features:
- down and out eye
- ptosis
- may have dilated pupil
72
Causes of third nerve palsy:
- diabetes mellitus
- vasculitis e.g. temporal arteritis, SLE
- false localising sign due to uncle herniation through tentorium if raised ICP
- posterior communicating artery aneurysm: pupil dilated, often associated pain
- cavernous sinus thrombosis
- Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia caused by midbrain strokes
- other possible causes: amyloid, MS
73
What is thoracic outlet syndrome?
- disorder involving compression of brachial plexus, subclavian artery or vein at site of thoracic outlet
- neurogenic or vascular (most neurogenic)
- neck trauma, soft tissue anomalies (scalene muscle hypertrophy and anomalous bands) or osseous structure e.g. cervical rub
74
Clinical presentation of neurogenic thoracic outlet syndrome:
- painless muscle wasting go hands and weakness
- sensory symptoms such as numbness and tingling
- autonomic nerve: cold hands, blanching, swelling
75
Clinical presentation of vascular thoracic outlet syndrome:
- subclavian vein compression leads to painful diffuse arm swelling with distended veins
- subclavian artery compression leads to painful arm claudication and sometimes ulceration and gangrene
76
What does a parkinsonism tremor look like?
- resting, pill rolling
- bradykinesia
- rigidity
- flexed posture, short, shuffling steps
- micrographia
- mask like face
- depression and dementia
- anti-psychotic history
77
What does an essential tremor look like?
- postural: worse if outstretched
- improved by alcohol and rest
- titubation
- often strong family history
78
What does tremor look like in cerebellar disease:
- intention tremor
| - cerebellar signs: past pointing, nystagmus etc.
79
Characteristics of trigeminal neuralgia:
- unilateral, brief, electric shock-like pains, abrupt
- limited to one or more division of trigeminal nerve
- evoked by light touch
- trigger areas
- pains remit for variable periods
80
Management of trigeminal neuralgia:
- carbamazepine
| - failure to respond or atypical features: referral to neurology
81
What are triptans, indications, ADR, CONTRA:
5HT1b and 5HT1d agonists
- acute migraines
- with NSAID or paracetamol
- ASAP after onset of headache rather than at onset of aura
- ADR: triptan sensations - tingling, heat, tightness, heaviness, pressure
- CONTRA: ischaemic heart disease or cerebrovascular disease
82
Cutaneous features of tuberous sclerosis:
- depigmented ash leaf spots which fluoresce under UV
- roughened patches of skin over lumbar spine (Shagreen patches)
- adenoma sebaceous: butterfly distribution over nose
- fibromata beneath nails
- cafe au lait spots
83
Neurological features of tuberous sclerosis:
- developmental delay
- epilepsy
- intellectual impairment
84
Other features of tuberous sclerosis:
- retinal hamartomas: dense white areas of retina
- rhabdomyomas of the heart
- gliomatous changes can occur in lesions
- polycystic kidneys, renal angiomyolipomata
- lymphangioleiomyomatosis: multiple lung cysts
85
Where does the ulnar nerve arise from:
medial cord of brachial plexus (C8, T1)
86
Ulnar nerve innervates:
motor:
- medial two lumbricals
- adductor pollicis
- interossei
- hypothenar muscles: abductor digit minimise, flexor digiti minimi
- flexor carpi ulnas
sensory: medial 1 1/2 fingers palmar and dorsal
87
Presentation of vestibular schwannoma:
- CN VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
- CN V: absent corneal reflex
- CN VII: facial palsy
88
Investigation of vestibular schwannoma:
cerebellopontine angle MRI
89
What does homonymous hemianopia indicate?
- incongruous defects: lesion of optic tract
- congruous defects: lesion of optic radiation or occipital cortex
- macular sparing: lesion of occipital cortex
90
What does homonymous quadrantopia indicate?
-suprior: lesion of inferior optic radiations in temporal lobe
-inferior: lesion of the superior optic radiations in parietal lobe
PITS
91
What does bitemporal hemianopia indicate?
- lesion of optic chiasm
- upper quadrant defect > lower quadrant defect = inferior chasmal compression, commonly pituitary tumour
- lower quadrant defect > upper quadrant defect = superior chasmal compression, commonly a craniopharygioma
92
What is Von Hippel-Lindau syndrome and what are the features:
- autosomal dominant condition predisposing to neoplasia
- abnormality in VHL gene on short arm of chromosome 3
- cerebellar haemangiomas: SAH
- retinal haemangiomas: vitreous haemorrhages
- renal cysts (premalignant)
- phaeochromocytomas
- extra renal cysts: epididymal, pancreatic, hepatic
- endolymphatic sac tumours
- clear-cell renal cell carcinoma
93
How does Wernicke's encephalopathy present?
- thiamine deficiency
- persistent vomiting, stomach cancer, dietary deficiency
- triad: opthalmoplegia/nystagmus, ataxis and confusion
- can get petechial haemorrhages
- peripheral sensory neuropathy
94
Investigations for Wernicke's and treatment:
- decreased red cell transketolase
- MRI
- urgent replacement of thiamine
