Neurology Flashcards

(176 cards)

1
Q

Drug side effects:

Peripheral neuropathy

A
amiodarone
phenytoin
metronidazole
nitrofurantoin
isoniazid
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2
Q

Drug side effects:

thrombocytopenia

A

sodium valproate

heparin

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3
Q

Mechanism of action:

Phenytoin

A

binds to sodium channels increasing their refractory period

P450 inducer

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4
Q

Phenytoin

Adverse effects

Acute

A

initially: dizziness, diplopia, nystagmus, slurred speech, ataxia
later: confusion, seizures

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5
Q

Phenytoin

Adverse effects

Chronic

A

gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness
megaloblastic anaemia (secondary to altered folate metabolism)
peripheral neuropathy
enhanced vitamin D metabolism causing osteomalacia
lymphadenopathy
dyskinesia

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6
Q

Phenytoin

Adverse effects

Idiosyncratic

A
fever
rashes, including severe reactions such as toxic epidermal necrolysis
hepatitis
Dupuytren's contracture*
aplastic anaemia
drug-induced lupus
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7
Q

Phenytoin monitoring

A

Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if:
adjustment of phenytoin dose
suspected toxicity
detection of non-adherence to the prescribed medication

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8
Q

Brain lesions

Gross anatomy

Parietal lobe lesions

A
sensory inattention
apraxias
astereognosis (tactile agnosia)
inferior homonymous quadrantanopia
Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
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9
Q

Brain lesions

Gross anatomy

Occipital lobe lesions

A

homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia

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10
Q

Brain lesions

Gross anatomy

Temporal lobe lesions

A

Wernicke’s aphasia: this area ‘forms’ the speech before ‘sending it’ to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
superior homonymous quadrantanopia
auditory agnosia
prosopagnosia (difficulty recognising faces)

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11
Q

Brain lesions

Gross anatomy

Frontal lobe lesions

A
expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting
disinhibition
perseveration
anosmia
inability to generate a list
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12
Q

Brain lesions

Gross anatomy

Cerebellar lesions

A

midline lesions: gait and truncal ataxia

hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus

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13
Q

Brain lesions

Medial thalamus and mammillary bodies of the hypothalamus

A

Wernicke’s and Korsakoff’s

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14
Q

Brain lesions

Subthalamic nucleus of the basal ganglia

A

Hemiballism (coarse, violent, wide amplitude movements, ipsilateral arm and leg).

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15
Q

Brain lesions

Striatum (caudate nucleus) of the basal ganglia

A

Huntington’s Chorea

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16
Q

Brain Lesions

Substantia nigra of the basal ganglia

A

Parkinson’s disease

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17
Q

Brain lesions

Amygdala

A

Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia

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18
Q

What is Friedreich’s ataxia?

A

Friedreich’s ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich’s ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.

The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.

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19
Q

Neurological features of Friedreich’s ataxia

A

absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration

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20
Q

Other features of Friedreich’s ataxia

A
hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
diabetes mellitus (10-20%)
high-arched palate
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21
Q

uses of carbamazepine

A

focal seizures - epilepsy
trigeminal neuralgia
bipolar disordre

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22
Q

Mechanism of action - carbamazepine

A

binds to sodium channels to increase their refractory period

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23
Q

Side effects of carbamazepine

A
P450 enzyme inducer
dizziness and ataxia
drowsiness
headache
visual disturbances (especially diplopia)
Steven-Johnson syndrome
leucopenia and agranulocytosis
hyponatraemia secondary to syndrome of inappropriate ADH secretion

Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.

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24
Q

Stroke symptoms

anterior cerebral artery

A

Contralateral hemiparesis and sensory loss, lower extremity > upper

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25
Stroke symptoms middle cerebral artery
Contralateral hemiparesis and sensory loss, upper extremity > lower Contralateral homonymous hemianopia Aphasia
26
Stroke symptoms | posterior cerebral artery
Contralateral homonymous hemianopia with macular sparing | Visual agnosia
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Stroke symptoms | Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain)
Ipsilateral CN III palsy | Contralateral weakness of upper and lower extremity
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Stroke Symptoms | Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss Contralateral: limb/torso pain and temperature loss Ataxia, nystagmus
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Stroke Symptoms Anterior inferior cerebellar artery (lateral pontine syndrome)
Symptoms are similar to Wallenberg's (see above), but: | Ipsilateral: facial paralysis and deafness
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Stroke symptoms retinal/ ophthalmic artery
amaurosis fugax
31
Stroke symptoms Basilar artery
Locked in syndrome
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Stroke symptoms Lacunar infarcts
present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia strong association with hypertension common sites include the basal ganglia, thalamus and internal capsule
33
Tuberous sclerosis
Autosomal dominant | Like neurofibromatosis, the majority of features seen in TS are neurocutaneous
34
Cutaneous features of tuberous sclerosis
depigmented 'ash-leaf' spots which fluoresce under UV light roughened patches of skin over lumbar spine (Shagreen patches) adenoma sebaceum (angiofibromas): butterfly distribution over nose fibromata beneath nails (subungual fibromata) café-au-lait spots* may be seen
35
Neurological features of tuberous sclerosis
developmental delay epilepsy (infantile spasms or partial) intellectual impairment
36
Other features of tuberous sclerosis
retinal hamartomas: dense white areas on retina (phakomata) rhabdomyomas of the heart gliomatous changes can occur in the brain lesions polycystic kidneys, renal angiomyolipomata lymphangioleiomyomatosis: multiple lung cysts
37
Sensory lesions of the spinal cord Neurosyphilis
affects the dorsal columns loss of vibration and proprioception
38
Motor lesions of the spinal cord ALS/MND
affects both upper (corticospinal tracts) and lower motor neurons results in a combination of upper and lower motor neuron signs
39
Motor lesions of the spinal cord | Polio
affects anterior horns resulting in lower motor neuron signs
40
Combined motor and sensory lesions of the spinal cord Brown-Sequard syndrome (spinal cord hemisection) Which tracts
1. Lateral corticospinal tract 2. Dorsal columns 3. Lateral spinothalamic tract
41
Combined motor and sensory lesions of the spinal cord Brown-Sequard syndrome (spinal cord hemisection) Clinical notes
1. Ipsilateral spastic paresis below lesion 2. Ipsilateral loss of proprioception and vibration sensation 3. Contralateral loss of pain and temperature sensation
42
Combined motor and sensory lesions of the spinal cord Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency) Which tracts
1. Lateral corticospinal tracts 2. Dorsal columns 3. Spinocerebellar tracts
43
Combined motor and sensory lesions of the spinal cord Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency) Clinical notes
1. Bilateral spastic paresis 2. Bilateral loss of proprioception and vibration sensation 3. Bilateral limb ataxia
44
Combined motor and sensory lesions of the spinal cord Friedreichs ataxia Which tracts
1. Lateral corticospinal tracts 2. Dorsal columns 3. Spinocerebellar tracts
45
Combined motor and sensory lesions of the spinal cord Friedreichs ataxia Clinical notes
1. Bilateral spastic paresis 2. Bilateral loss of proprioception and vibration sensation 3. Bilateral limb ataxia In addition cerebellar ataxia → other features e.g. intention tremor
46
Combined motor and sensory lesions of the spinal cord Anterior spinal artery occlusion which tracts
1. Lateral corticospinal tracts | 2. Lateral spinothalamic tracts
47
Combined motor and sensory lesions of the spinal cord Anterior spinal artery occlusion Clinical notes
1. Bilateral spastic paresis | 2. Bilateral loss of pain and temperature sensation
48
Combined motor and sensory lesions of the spinal cord Syringomelia Which tracts
1. Ventral horns | 2. Lateral spinothalamic tract
49
Combined motor and sensory lesions of the spinal cord Syringomelia Clinical notes
1. Flacid paresis (typically affecting the intrinsic hand muscles) 2. Loss of pain and temperature sensation
50
Combined motor and sensory lesions of the spinal tract MS Which tracts
Asymmetrical, varying spinal tracts involved
51
Combined motor and sensory lesions of the spinal tract MS Clinical notes
Combination of motor, sensory and ataxia symptoms
52
Motor and descending tracts pyrimadal tracts
Lateral corticospinal tracts anterior corticospinal tracts
53
Motor and descending tracts Extrapyrimidal tracts
rubrospinal tracts reticulospinal tracts olivospinal tracts vestibular spinal tracts
54
Sensory and ascending tracts Dorsal column medial lemniscus system
Grascile fasciculus Cuneate fasciculus
55
Sensory and ascending tracts Spinocerebellar tracts
posterior spinocerebellar tracts anterior spinocerebellar tracts
56
Sensory and ascending tracts Anterolateral tracts
anterior spinothalamic tracts lateral spinothalamic tracts spino-oliviary tract
57
Bitemporal hemianopia
lesion of optic chiasm upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
58
Homonymous quadrantanopias
superior: lesion of the inferior optic radiations in the temporal lobe (Meyer's loop) inferior: lesion of the superior optic radiations in the parietal lobe mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
59
homonymous hemianopia
incongruous defects: lesion of optic tract congruous defects: lesion of optic radiation or occipital cortex macula sparing: lesion of occipital cortex
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what are congruent visual defects
A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric. incongruous defects = optic tract lesion; congruous defects = optic radiation lesion or occipital cortex
61
Key features of myasthenia gravis
The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest: extraocular muscle weakness: diplopia proximal muscle weakness: face, neck, limb girdle ptosis dysphagia
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Associations with myasthenia gravis
thymomas in 15% autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE thymic hyperplasia in 50-70%
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Investigations of myasthenia gravis
single fibre electromyography: high sensitivity (92-100%) CT thorax to exclude thymoma CK normal autoantibodies: around 85-90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia
64
Management of myasthenia gravis
long-acting acetylcholinesterase inhibitors pyridostigmine is first-line immunosuppression: prednisolone initially azathioprine, cyclosporine, mycophenolate mofetil may also be used thymectomy Management of myasthenic crisis plasmapheresis intravenous immunoglobulins
65
Pharmacology of triptans
Triptans are specific 5-HT1B and 5-HT1D agonists used in the acute treatment of migraine. They are generally used first-line in combination therapy with an NSAID or paracetamol. _ take after the onset of headache, not aura
66
adverse effects of triptans
'triptan sensations' - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure
67
triptan contraindications
patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease
68
What is restless leg syndrome
Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present
69
Features of restless leg syndrome
uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest paraesthesias e.g. 'crawling' or 'throbbing' sensations movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
70
Causes and associations of restless leg syndrome
``` there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy ```
71
Management of restless leg syndrome
``` there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy ```
72
Features of von-Hippel Lindau
cerebellar haemangiomas: these can cause subarachnoid haemorrhages retinal haemangiomas: vitreous haemorrhage renal cysts (premalignant) phaeochromocytoma extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours clear-cell renal cell carcinoma
73
Vestibular neuritis
a 30-year-old woman who is recovering from an upper respiratory tract infections presents with recurrent attacks of vertigo associated with nausea and vomiting. There is no hearing loss or tinnitus
74
Viral labyrinthitis | e.g. Hx
a 30-year-old woman who has recently developed an upper respiratory tract infection presents with vertigo and vomiting. Her hearing is also affected. The symptoms came on suddenly this morning
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Viral labyrinthitis
Recent viral infection Sudden onset Nausea and vomiting Hearing may be affected
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Vestibular neuritis
Recent viral infection Recurrent vertigo attacks lasting hours or days No hearing loss
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Acoustic neuroma
Hearing loss, vertigo, tinnitus Absent corneal reflex is important sign Associated with neurofibromatosis type 2
78
Vertebrobasilar ischaemia
Elderly patient | Dizziness on extension of neck
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Meniere's disease
Associated with hearing loss, tinnitus and sensation of fullness or pressure in one or both ears
80
Benign paroxysmal positional vertigo
Gradual onset Triggered by change in head position Each episode lasts 10-20 seconds
81
becker muscular dystrophy
develops after the age of 10 years intellectual impairment much less common non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form
82
Duchene muscular dystrophy
progressive proximal muscle weakness from 5 years calf pseudohypertrophy Gower's sign: child uses arms to stand up from a squatted position 30% of patients have intellectual impairment there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form
83
Dystrophinopathies
X-linked recessive due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21 dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleto
84
Stereotypical history of Becker's dystrophy
onset > 10 years old, pseudohypertrophy of calf muscles, trouble standing from sitting, no learning disability
85
Presenting features of Freidreich's ataxia
typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.
86
History of posterior cerebral artery stroke eye symptoms
contralateral homonymous hemianopia with macular sparing and visual agnosia
87
History of middle cerebral artery stroke
contralateral hemiparesis and sensory loss with the upper extremity being more affected than the lower, contralateral homonymous hemianopia and aphasia
88
Von Hipple lindau chromosome
Chromosome 3
89
Typical history of MS
conjugate horizonal gaze palsy, nystagmus in abducting eye | lethargy, optic neuritis, paraesthesia, spastic weakness
90
Examples of 5-HT3 antagonists
ondansetron | granisetron
91
What are 5-HT3 antagonists
5-HT3 antagonists are antiemetics used mainly in the management of chemotherapy-related nausea. They mainly act in the chemoreceptor trigger zone area of the medulla oblongata.
92
Side effects of 5-HT3 antagonists
Constipation | QT prolongation
93
Non-neurological associations with Friedreich's ataxia
``` hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate ```
94
Kluver-Bucy syndrome: stereotypical history
a man who has recently been treated for herpes simplex encephalitis presents with a sudden increase in appetite and sexual desire
95
Edrophonium
readily reversible acetylcholinesterase inhibitor. It prevents breakdown of the neurotransmitter acetylcholine and acts by competitively inhibiting the enzyme acetylcholinesterase, mainly at the neuromuscular junction
96
What is ataxic telangiectasia
Ataxia telangiectasia is an autosomal recessive disorder caused by a defect in the ATM gene which encodes for DNA repair enzymes. It is one of the inherited combined immunodeficiency disorders. It typically presents in early childhood with abnormal movements.
97
Features of ataxic telangiectasia
cerebellar ataxia telangiectasia (spider angiomas) IgA deficiency resulting in recurrent chest infections 10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
98
Stereotypical history of Branchial cyst
a 20-year-old presents with a painless swelling on the lateral aspect of the neck. On examination it is smooth, non-tender and fluctuant
99
What is a branchial cyst
An oval, mobile cystic mass that develops between the sternocleidomastoid muscle and the pharynx Develop due to failure of obliteration of the second branchial cleft in embryonic development Usually present in early adulthood
100
What is a cervical rib
More common in adult females | Around 10% develop thoracic outlet syndrome
101
Cystic hygroma
A congenital lymphatic lesion (lymphangioma) typically found in the neck, classically on the left side Most are evident at birth, around 90% present before 2 years of age
102
Pharyngeal pouch
More common in older men Represents a posteromedial herniation between thyropharyngeus and cricopharyngeus muscles Usually not seen but if large then a midline lump in the neck that gurgles on palpation Typical symptoms are dysphagia, regurgitation, aspiration and chronic cough
103
Thyroglossal cyst
More common in patients < 20 years old Usually midline, between the isthmus of the thyroid and the hyoid bone Moves upwards with protrusion of the tongue May be painful if infected
104
Idiosyncratic side effects of phenytoin
``` fever rashes, including severe reactions such as toxic epidermal necrolysis hepatitis Dupuytren's contracture* aplastic anaemia drug-induced lupus ```
105
Acute side effects of phenytoin
initially: dizziness, diplopia, nystagmus, slurred speech, ataxia later: confusion, seizures
106
Chronic side effects of phenytoin
common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness megaloblastic anaemia (secondary to altered folate metabolism) peripheral neuropathy enhanced vitamin D metabolism causing osteomalacia lymphadenopathy dyskinesia
107
Ethosuximide
Ethosuximide is an antiepileptic that is particularly indicated in patients with absence seizures blocks T-type calcium channels in thalamic neurons
108
Riluzole- used in management of Amyotrophic lateral scelosis
prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis prolongs life by about 3 months BIPAP at night - prolongs life by approx 7 months
109
Side effects of lamotrigine
Stevens-Johnston syndrome
110
Stereotypical history of mytonic dystrophy
a 25-year-old man with muscle weakness is reviewed in clinic. On shaking his hand he has difficultly loosening his grip. On examination he has frontal balding, bilateral ptosis and cataracts
111
What is myotonic dystrophy
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
112
Genetics of myotonic dystrophy
autosomal dominant a trinucleotide repeat disorder DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19 DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
113
Myotonic dystrophy DM 2
- ZNF9 gene on chromosome 3 - Proximal weakness more prominent - Severe congenital form not seen
114
Myotonic dystrophy DM1
DM1 DM2 - DMPK gene on chromosome 19 - Distal weakness more prominent
115
General features of myotonic dystrophy
``` myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria ```
116
Other features of myotonic dystrophy
myotonia (tonic spasm of muscle) weakness of arms and legs (distal initially) mild mental impairment diabetes mellitus testicular atrophy cardiac involvement: heart block, cardiomyopathy dysphagia
117
Treatment of SAH
- coil aneurysms - strict bed rest and BP control - Vasospasm is prevented using a 21-day course of nimodipine (a calcium channel inhibitor targeting the brain vasculature) and treated with hypervolaemia, induced-hypertension and haemodilution**
118
Complications of SAH
Re-bleeding Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset Hyponatraemia (most typically due to syndrome inappropriate anti-diuretic hormone (SIADH)) Seizures Hydrocephalus Death
119
Chromosome for neurofibromatosis type 1
Chromosome 17
120
Chromosome for neurofibromatosis type 2
Chromosome 22
121
Conductive hearing loss
Rinne's test Bone conduction > air conduction in affected ear Air conduction > bone conduction in unaffected ear Webber's- Lateralises to affected ear
122
Sensorineural hearing loss
Rinne's test- Air conduction > bone conduction bilaterally | Webber's test - Lateralises to unaffected ear
123
Stereotypical history of cavernous sinus thrombus
a young man presents with an acute headache associated with unilateral periorbital edema. On examination there is a lateral gaze palsy
124
Features of cavernous sinus thrombus
headache (may be sudden onset) nausea & vomiting, cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma periorbital oedema ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain central retinal vein thrombosis
125
Features of sagittal sinus thrombus
headache, vomiting seizures and hemiplegia parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen
126
lateral sinus thrombus features
6th and 7th cranial nerve palsies
127
benzhexol
antiparkinsonian agent of the antimuscarinic class
128
Stereotypical history of syringomelia
a 30-year-old man presents with progressive weakness of his hands. On examination you notice wasting of the small muscles of the hand. Also pain and temperature sensation is reduced but light touch is preserved.
129
What is syringomelia
a collection of cerebrospinal fluid within the spinal cord.
130
Syringobulbia
similar phenomenon to syringomyelia which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding.
131
Presentation of syringomelia
‘cape-like’ (neck and arms) loss of sensation to temperature but preservation of light touch, proprioception and vibration. spastic weakness (predominantly of the upper limbs), paraesthesia, neuropathic pain, upgoing plantars and bowel and bladder dysfunction. Scoliosis in long term
132
selegiline
MOAB used to treat parkinsons
133
Stereotypical history of normal pressure hydrocephalus
an elderly man presents with gradual worsening dementia and bradyphrenia associated with a slow, shuffling gait. He has also recently suffered with urinary incontinence
134
Stereotypical history of multisystem atrophy
an elderly man presents with parkinsonism atonic bladder, postural hypotension and cerebellar signs
135
Stereotypical history of conduction aphasia
a patient is noted to speak fluently but have difficulty repeating phrases. He is aware of the errors he is making and comprehension remains normal
136
Stereotypical history of Wernicke's aphasia
a patient is noted to speak fluently but makes little sense with mulitple word substitutions and neologisms. He also has difficulty understanding what people are saying to him
137
Features of parietal lobe lesions
``` sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation ```
138
stereotypical history of posterior inferior cerebellar artery stroke
sudden onset vertigo and vomiting, dysphagia, ipsilateral facial pain and temperature loss, contralateral limb pain and temperature loss and ataxia
139
Stereotypical history of anterior inferior cerebellar artery stroke
sudden onset vertigo and vomiting, ipsilateral facial paralysis and deafness
140
Features of neuroleptic malignant syndrome
``` pyrexia muscle rigidity autonomic lability: typical features include hypertension, tachycardia and tachypnoea agitated delirium with confusion AKI secondary to raised CK ```
141
Management of neuroleptic malignant syndrome
``` stop antipsychotic patients should be transferred to a medical ward if they are on a psychiatric ward and often they are nursed in intensive care units IV fluids to prevent renal failure dantrolene bromocriptine ```
142
Features of Meniere's disease
recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom a sensation of aural fullness or pressure is now recognised as being common other features include nystagmus and a positive Romberg test episodes last minutes to hours typically symptoms are unilateral but bilateral symptoms may develop after a number of years
143
Natural history of Meniere's disease
symptoms resolve in the majority of patients after 5-10 years the majority of patients will be left with a degree of hearing loss psychological distress is common
144
Investigation of Meniere's disease
ENT assessment is required to confirm the diagnosis patients should inform the DVLA. The current advice is to cease driving until satisfactory control of symptoms is achieved acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required prevention: betahistine and vestibular rehabilitation exercises may be of benefit
145
Features of CJD
dementia (rapid onset) | myoclonus
146
Investigations of CJD
CSF is usually normal EEG: biphasic, high amplitude sharp waves (only in sporadic CJD) MRI: hyperintense signals in the basal ganglia and thalamus
147
Sporadic CJD
accounts for 85% of cases 10-15% of cases are familial mean age of onset is 65 years
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New variant CJD
younger patients (average age of onset = 25 years) psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features the 'prion protein' is encoded on chromosome 20 - it's role is not yet understood methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD - all patients who have so far died have had this median survival = 13 months
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Reye's syndrome
Reye's syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas. The aetiology of Reye's syndrome is not fully understood although there is a known association with aspirin use and a viral cause has been postulated
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Features of Reye's syndrome
The peak incidence is 2 years of age, features include: there may be a history of preceding viral illness encephalopathy: confusion, seizures, cerebral oedema, coma fatty infiltration of the liver, kidneys and pancreas hypoglycaemia
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Stereotypical history of cluster headaches
a male smoker presents with recurrent episodes of severe pain around one eye which typical lasts around 1 hour. These episodes have occured daily for the past 6 weeks and are associated with ocular erythema and lacrimatio
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Features of cluster headaches
pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours clusters typically last 4-12 weeks intense sharp, stabbing pain around one eye (recurrent attacks 'always' affect same side) patient is restless and agitated during an attack accompanied by redness, lacrimation, lid swelling nasal stuffiness miosis and ptosis in a minority
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Management of cluster headaches
acute: 100% oxygen (80% response rate within 15 minutes), subcutaneous triptan (75% response rate within 15 minutes) prophylaxis: verapamil is the drug of choice. There is also some evidence to support a tapering dose of prednisolone
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stereotypical history of freidreich's ataxia
a teenage boy presents with clumsy walking. On examination he has gait ataxia, an intention tremor and loss of lower limb reflexes
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Pathophysiology of Lambert-Eaton syndrome
Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.
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Features of Lambert-Eaton Syndrome
repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis) in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease limb-girdle weakness (affects lower limbs first) hyporeflexia autonomic symptoms: dry mouth, impotence, difficulty micturating ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)
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Kluver-Bucy syndrome
hypersexuality, hyperorality, hyperphagia, visual agnosia | disease of the amygdala
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Features of Ramsay Hunt syndrome
auricular pain is often the first feature facial nerve palsy vesicular rash around the ear other features include vertigo and tinnitus
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What is Ramsay Hunt syndrome
Ramsay Hunt syndrome (herpes zoster oticus) is caused by the reactivation of the varicella zoster virus in the geniculate ganglion of the seventh cranial nerve.
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Management of Ramsay Hunt Syndrome
oral aciclovir and corticosteroids are usually given
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Stereotypical history of BPPV
a 55-year-old woman presents with recurrent episodes of vertigo that are often triggered by a change in head position. Each episode lasts around 10-20 seconds and is associated with nausea
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sub acute combined degeneration of the spinal cord
due to vitamin B12 deficiency dorsal columns + lateral corticospinal tracts are affected joint position and vibration sense lost first then distal paraesthesia upper motor neuron signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks if untreated stiffness and weakness persist
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Complications of SAH (aneurysmal)
``` Re-bleeding Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset Hyponatraemia (SIADH) Seizures Hydrocephalus Death ```
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Klumpke's palsy (claw hand)
traction or tear of the lower trunk of the brachial plexus (C8 to T1) wasting of intrinsic hand muscles and flexion deformities of the proximal interphalangeal (PIP) & distal interphalangeal (DIP) joints.
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Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
rare cause of multi-infarct dementia | patients often present with migraine
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Causes of gingival hyperplasia
phenytoin ciclosporin calcium channel blockers (especially nifedipine)
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Normal pressure hydrocephalus
urinary incontinence dementia and bradyphrenia gait abnormality (may be similar to Parkinson's disease)
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Lambert-Eaton myasthenic syndrome
associated with small cell lung cancer (also breast and ovarian) antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system can also occur independently as autoimmune disorde
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Lambert-Eaton myasthenic syndrome
associated with small cell lung cancer (also breast and ovarian) antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system can also occur independently as autoimmune disorder
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Anti-Hu
associated with small cell lung carcinoma and neuroblastomas sensory neuropathy - may be painful cerebellar syndrome encephalomyelitis
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Anti-Yo
associated with ovarian and breast cancer | cerebellar syndrome
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Anti-GAD antibody
associated with breast, colorectal and small cell lung carcinoma stiff person's syndrome or diffuse hypertonia
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Anti- Ri
associated with breast and small cell lung carcinoma | ocular opsoclonus-myoclonus
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Purkinje cell antibody
peripheral neuropathy in breast cancer
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hemiballismus
type of chorea which is caused by a decreased activity in the subthalamic nucleus of the basal ganglia in most case
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Wernicke's area
Brodmann area 22 in the superior temporal gyrus