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FRCS Part 1 > Ch10 Neurology > Flashcards

Ch10 Neurology Flashcards

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1
Q

What is the definition of dementia?

A

The loss of intellectual abilities severe enough to interfere with social and occupational functioning

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2
Q

What are risk factors for dementia?

A

Age, family history and apolipoprotein E-4 allele

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3
Q

What is delirium?

A

An acute confusional state with motor signs, altered consciousness and hallucinations. EEG shows diffuse slowing.

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4
Q

What is the diagnostic yield for brain biopsy in neurodegenerative disease?

A

20% - higher where there is a focal MRI abnormality. Biopsy should include GM, WM and dura

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5
Q

What are the two broad classifications of chronic headache?

A

Migraine and Tension (muscular))

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6
Q

What are the features of a common migraine?

A

Episodic headache with nauseas and photophobia. No aura or neurological deficit.

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7
Q

What are the features of a classic migraine?

A

Common migraine with an aura. Transient neurological deficit (mostly visual) resolve completely within 24 hours.

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8
Q

What is a complicated migraine?

A

Same as a classical migraine but neurological changes can take up to 30 days to resolve.

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9
Q

What is a migraine equivalent?

A

Acephalgic migraine, mostly seen in children and develops into a typical migraine with age.

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10
Q

What is cluster headache?

A

Recurrent unilateral attacks of severe pain, usually oculofrontal / temporal associated with ipsilateral autonomic symptoms. Occasionally assoc with a Horners. M:F = 5:1. Occur for 1-3 months and have remission for ~ 1 year.

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11
Q

What is the treatment for cluster headache?

A

Prophylaxis is only minimally effective with Lithium being the drug of choice. Treatment of an acute attack is with 100% O2, ergotamine and sumatriptan

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12
Q

What are the surgical treatment options for cluster headache?

A

Percutaneous radiofrequency ablation of the pterygopalatine ganglion Occipital nerve stimulation DBS - hypothalamus and ventral tegmental area (just ant to the red nucleus)

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13
Q

What is the diagnosis in an adolescent with transient neurological deficits in the distribution of the basilar artery?

A

Basilar migraine - 90% have a family history. Most commonly cuases vertigo, ataxia and visual disturbance followed by headache and n&V

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14
Q

What causes Parkinsonism?

A

Relative loss of dopamine-mediated inhibition (i.e. overactivation) of the effects of acetylcholine on the basal ganglia

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15
Q

What is the triad of Parkinsonism?

A

Bradykinesia Tremor Rigidity (also have postural and gait disturbances, Micrographia, mask-like facies and emotional lability)

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16
Q

How can primary and secondary parkinsons be distinguished?

A

Primary - gradual onset bradykinesia and tremor which is asymmetrical and responds well to levodopa. Secondary - rapidly progressive with poor response to levodopa. Early midline symptoms eg. gait, balance, sphinter disturbance etc and associated with dementia, orthostatic hypotension and extra-ocular muscle weakness

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17
Q

Where are the DA neurons in the basal ganglia?

A

Pars compacta of the substantia nigra

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18
Q

What is the direct pathway in PD?

A

In normal circumstances the direct pathway causes movement and the indirect pathway prevents movement.

Direct pathway - motor cortex stimulates the striatum (Caudate + putamen). The striatal GABA release inhibits the GPi. The GPi release of GABA on the thalamus is reduced so excitign the motor cortex and allowing movement. The STN excites the SNc. DA neurons from the SNc then release DA on the D1 striatal neurons and increases the GABA release on the GPi. There is negative feedback between the SNc and STN.

*The DA from the SNc on the D1 in the striatum amplifies the DIRECT pathway

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19
Q

What is the indirect pathway in PD?

A

Indirect pathway inhibits motor movement.

Motor cortex stimulates the striatum. The striatum GABA inhibits the GPe. The GPe release of GABA on the STN is therefore reduced so the STN activation of the GPi increases. The GPi inhibition of the thalamus increases which then inhibits the motor cortex. This prevents movement. The STN activity also stimulates the SNc. The SNc then releases DA on the striatum and activates the DIRECT pathway i.e. negative feedback loop.

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20
Q

What are the different actions of D1 and D2 receptors in the striatum?

A

D1-R activate the DIRECT pathway and therefore causes movement

D2-R activate the INDIRECT pathway and therefore prevents movement

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21
Q

What are Lewy bodies?

A

Eosinophilic intraneuronal hyaline inclusions composed of alpha-synuclein

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22
Q

What are the causes of secondary Parkinsonism (Parkinson plus syndromes)?

A

Olivopontocerebellar degeneration

Striatonigral degeneration

Post-encephalitic parkinsonism

PSP

MSA

Drug induced

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23
Q

What are the features of MSA?

A

Parkinsonism + postural hypotension or other signs of autonomic dysfunction. Do not respond to L-Dopa. Associated with degeneration of neurones in the lateral horn of the thoracic spine.

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24
Q

What are the features of PSP?

A

Progressive vertical gaze palsy (Doll’s eye remains the same)

Pseudobulbar palsy (dysarthria / dysphasia / hyperactive jaw jerk)

Axial dystonia (neck and upper trunk)

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25
What is the main difference between Parkinsons and PSP?
Normal gait and no tremor. They tend to fall backwards.
26
What are the classical findings of MS?
Optic neuritis Paraesthesias Internuclear opthalmoplegia Bladder dysfunction
27
What are the diagnostic criteria for MS?
McDonald criteria
28
What are the MRI findings in MS?
Multiple lesions of different age (some enhancing, some not) involving the periventricular WM, spinal cord, optic nerve and cerebellum
29
What are the clinical subtypes of MS?
Relapsing-remitting (acute episodes with stable courses between relapses) Secondary progressive (gradual deterioration in a patient with relapsing-remitting disease previously) Primary progressive (gradual continuous deterioration) Progressive relapsing (Similar to primary progressive but with acute exacerbations superimposed)
30
What causes scanning speech (where words are broken up into their individual syllables with pauses in between)?
Cerebellar lesions - represents an ataxic dysarthria
31
What is dysarthria?
Poor articulation of words in someone who is otherwise linguistically normal
32
What is the DD of MS?
CNS lymphoma Devic's disease ADEM Vasculitis
33
What is Uhthoff sign?
In MS, where the neurological deficit is worse with a hot bath / shower)
34
What is McDonald's criteria for MS?
Based on the number of clinical attacks (0, 1 or 2) Lesions on T2 MRI disseminated in space and time Additional criteria include CSF positive for OCBs but not in the serum or elevated IgG index
35
What are the 3 patterns of MND?
Amyotrophic lateral sclerosis - mixture of UMN and LMN (most common) Primary lateral sclerosis - UMN degeneration with no LMN signs. Pseudobulbar palsy is common. Slower progression than ALS. Progressive muscular atrophy / Spinal muscular atrophy - LMN degeneration
36
What are the clinical features of ALS?
UMN causing spasticity (LLs) LMN causing atrophy and fasciculations (ULs)
37
What is the pathology of MND?
Degeneration of alpha-motor neurones in the anterior horn and CST therefore producing LMN and UMN signs, respectively.
38
What is the differential diagnosis of ALS?
Cervical myelopathy as this may result in hand weakness (LMN) and lower limb weakness with spasticity and hyperreflexia (UMN)
39
What is suggested by fibrillation potentials in the tongue?
ALS
40
What is GBS?
Guillain-Barre syndrome - acute onset peripheral motor neuropathy with no sensory involvement. Onset 3 days - 5 weeks after viral URTI, immunisation or campylobacter enteritis. Focal segmental demyelination with raised protein in CSF.
41
What is the characteristic neurological deficit in GBS?
Symmetrical ascending weakness with areflexia
42
What antibodies are found in GBS?
Anti-ganglioside antibodies
43
What is the tremor frequency in PD?
4-6 Hz
44
How do you manage PD?
1) Assess functional status and cognition 2) Start drugs when PD seriously interferes with life - use the lowest dose that provides symptom relief Start with L-Dopa (dopamine precursor) and a Dopa-decarboxylase inhibitor (carbidopa). The carbidopa acts peripherally and cannot cross the BBB so prevents the conversion of L-Dopa to dopamine preventing the peripheral effects of dopamine. Dopamine cannot cross the BBB. (100 mg L-Dopa : 25 mg Carbidopa TDS). COMT inhibitors can be used in conjunction to prevent peripheral L-Dopa breakdown. MAO-B inhibitors are used to manage 'end of dose' off-effects of L-Dopa. 3) After a few years, the L-dopa becomes less effective. May help to start slow-release L-Dopa and add in Dopamine-3 agonists e.g ropinorole / cabergoline / pramipexole / Pergolide. These may be used as first-line in younger patients to reserve L-Dopa. Anti-muscarinics can be used to help with extrapyramidal symptoms but are associated with cognitive impairment. 4) Advanced disease may be treated with an apomorphine pump 5) DBS
45
What classes of drugs are used in PD?
1) L-Dopa (dopamine precuror) with dopa decarboxylase inhibitor (e.g. co-careldopa) 2) Dopamine agonists (cabergoline, bromocriptine, premipexole, ropinerole, apomorphine and pergolide) 3) COMT inhibitors 4) MAO-B inhibitors 5) Antimuscarinics
46
What are the side effects of L-Dopa?
Nausea / vomiting Dyskinesia and fluctuation in motor performances Palpitations / postural hypotension Drowsiness Anxiety / depression / psychosis
47
What are the side effects of Bromocriptine?
Drowsiness / hypotension / Nausea / constipation / headache Pulmonary and retroperitoneal fibrosis Cardiac valvular disease and pericarditis Dyskinesias Loss of inhibitions and impulsivity - increased libido and gambling Peripheral vasospasm so don't use in Raynaud's syndrome
48
What CSF markers can be sent for investigation of dementia?
CSF tau, prophorylated tau and beta-amyloid are suggestive of AD 14-3-3 for CJD
49
What proportion of MS patients suffer with TN?
2%, more ofter bilateral and occur at a younger age
50
What are Dawson's fingers?
Ovoid periventricular FLAIR lesions that are perpendicular to the ependymal surface characteristic of MS
51
What are the diagnostic criteria for GBS?
Progressive weakness of more than one limb and areflexia
52
What is Miller-Fisher variant of GBS?
Ataxia, areflexia and opthalmoplegia. Associated with anti GQ1b antibodies
53
What is CIDP?
Chronic immune demyelinating polyneuropathy - chronic relapsing form of GBS
54
What are the MRI spine findings with GBS?
Diffuse enhancement of the cauda equina and nerve roots
55
What is the treatment for GBS?
IVIG Plasmapheresis may hasten recovery in severe cases
56
What are the causes of myelitis?
Post-infectious (polio, herpes zoster) Post-traumatic Paraneoplastic (mostly lung) Metabolic (DM / Liver disease) Toxins Arachnoiditis Autoimmune (MS / Devics) Collagen vascular diseases
57
Where is the most common site for transverse myelitis?
Thoracic spine
58
What is the treatment of transverse myelitis?
Steroids Plasma exchange Immunosuppression (cyclophosphamide)
59
What is sarcoidosis?
Systemic granulomatous disease Neurosarcoidosis mainly involves the leptomeninges and presents with multiple cranial nerve palsies and diabetes insipidus. Treated with immunosuppression
60
What marker is raised in Sarcoidosis?
Serum and CSF ACE
61
Which familial form of this condition is associated with a SOD1 mutation?
Motor neurone disease (Superoxide dismutase). Histologically only the posterior columns remain intact and preserve the myelin staining.
62
What are the features of Huntingtons on gross pathology?
Marked atrophy of the caudate and putamen associated with enlarged ventricles due to ex vacuo hydrocephalus.
63
Which condition is associated with degeneration of the dorsal columns and lateral CST?
Freidrich's ataxia (spinocerebellar degeneration due to mutation in the frataxin gene - GAA repeats)
64
Which condition shows marked thinning of the corpus callosum?
Vascular dementia - may also have white matter cavitation
65
What condition is characterised by juxtacortical and deep white matter periventricular lesions?
MS! Note histologically myelin is stained blue with LUXOL fast blue stain so areas with loss of staining suggest focal demyelination.
66
What are the histological features of CO poisoning?
Diffuse brain swelling and pink discolouration from CO-Hb in the tissues. The colour is most notable in the GPi bilaterally.
67
What is superior vermis atrophy associated with?
Chronic ETOH
68
What is toxic leukoencephalopathy associated with?
Cocaine overdose
69
Which condition causes atrophy and discolouration of the putamen and GPi (lentiform nucleus)?
Wilson's disease
70
What is CADASIL?
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
71
What is metachromic leukodystrophy?
Wide spread myeline loss with sparing of subcortical U fibres
72
What is ulegyria?
Focal hypoperfusion causing atrophy at the depth of the sulcus with preservation of the gyrus
73
What mutations are found in inherited forms of Alzheimer's disease?
Amyloid precursor protein, Presenilin 1 and 2. Only 5% are inherited. Autosomal dominant. Increased risk with Apolipoprotein E4.
74
What are the features of Lewy body dementia?
Cognitive impairment Parkinsonism Visual hallucinations Treat with acetylcholine esterase inhibitors. Don't use neuroleptics!
75
What is Riluzole?
A glutamate release inhibitor that extends life expectancy and ventilatory dependence in MND
76
What are the features of spinomuscular atrophy?
Autosomal recessive condition. Mutation in the survival of motor neurone gene. 4 forms based on the onset. 1 - acute, 2 - intermediate, 3 - \>18 months and able to walk independently and 4 - adult-onset.
77
What is the role of thallium spect scan in immunocompromised patients?
To distinguish cerebral toxoplasmosis (negative) from a primary CNS lymphoma (positive)
78
What does India ink stain?
Cryptococcus
79
How do you diagnose Devics disease?
2 or more of the following: Spinal lesion \>3 levels Normal MRI Aquaporin 4 antibodies Treatment is with steroids, plasma exchange and immunosuppression
80
How do you distinguish ADEM from MS?
ADEM tends to be after a viral illness, occurs in young people and is monophasic (not relapsing and remitting)
81
How do you treat an acute relapse in MS?
High dose steroids for 3-5 days. Shortens the length of the relapse but does not alter the recovery.
82
When do you start disease modifying treatment for MS?
When you diagnose a relapsing and remitting form. Interferon beta (not if liver dysfunction) Glatiramer acetate (acts as a myelin decoy) Fingolimod (sphinosone-1-phosphate R modulator preventing lymphocyte migration) & Natalizumab are 2nd line and associated with PML.
83
What is Marcus Gunn phenomenon?
Jaw winking phenomenon - after facial nerve injury aberrant reinnervation cause the eyelids to blink
84
What condition causes cerebellar signs, haematuria and hepatosplenomegaly?
VHL! Polycystic liver and kidney disease, cerebellar tumour, haemangioblastomas and retinal angiomas / telangiectasia are all features. Autosomal dominant. M\>F
85
What are the features of diabetic amyotrophy
Painful wasting of the proximal lower limb muscles
86
How do you distinguish polymyositis from inclusion body myositis?
Polymyositis is painful, CK is very high and distal weakness occurs at advanced stages. Inclusion body myositis is most common in the elderly. Mainly affects finger and wrist flexion. CK is only minimally raised.
87
How does Duschenne muscular dystrophy present?
Muscle weakness \<5 y Pseudo calf hypertophy Gower's sign Death around 25 years due to respiratory dysfunction. X-linked mutation in the dystrophin gene
88
How do you distinguish Becker's and Duschenne muscular dystophy?
Becker occurs after age of 10 years. No intellectual impairment. Survival 40-50 years. CK and cardiac disease are prominent in both,
89
What is facioscapulohumeral muscular dystrophy?
Autosomal dominant condition Weakness of the face, scapula causing winging, wasting of the biceps and triceps. Calf atrophy and foot drop.
90
What is Emery-Driefuss musclar dystrophy?
X-linked muscular dystrophy Severe cardiac involvement. Skeletal muscle weakness is mild. Triad of elbow contractures, achilles tendon and posterior cervical muscle weakness.
91
Where does the chorda tympani leave the skull?
Pterygotympanic fissure
92
How does EMG distinguish myaesthenia gravis from Lambert-Eaton or botulism?
In LE and Botulism the Ach blockade is presynaptic and low frequency repetition causes a decrement in the muscle response. High frequency repetition causes a build up of Ca and increases the muscle response. This is not seen with myaesthenia gravis!
93
What is the NIHSS score?
Stroke severity score important for prognosis Range from 0-42. \>25 = very severe impairment 15-24 = severe (more likely to be discharged to a nursing home) 5-14 = moderate (more likely to be discharged to rehab) \<5 = mild (more likely to be discharged home) An increase of score of 2 or more points indicates stroke progression. If the initial score is \>7 then 2/3 will have stroke progression. If the score is \>20 then the chance of an ICH is 17% A score \>15 is needed to consider decompressive hemicraniectomy!
94
What are the indications for cartoid endarterectomy?
\>50% stenosis in a man or \>70% stenosis in a female with TIA or stroke should be referred for CES within 14 days. Reduces 5-year stroke rate by 18.5% if CES \<2 weeks, 10% if 2-4 weeks and 5% 4-12 weeks. Benefit only 1% if after 12 weeks!
95
What are the CHADS2-VASC and HAS-BLED scores?
CHADS2-VASC = CHF, Hypertension, Age, DM, Stroke/Sex, Vascular disease. If score 2 or more then offer anticoagulation HAS-BLED = Hypertension, Abn liver / renal function, Stroke, Bleeding, Labile INR, Elderly, Drugs predisposing to bleeding
96
When do you start antiplatelets or anticoagulation following a stroke?
Anti-platelets immediately (Aspirin or clopidogrel) Anticoagulation after 2 weeks
97
How do you treat a stroke?
Aspirin immediately If no bleed and within 4.5 hours then thrombolysis and send for mechanical thrombectomy if available.
98
What is the mechanism behind subacute degeneration of the spinal cord?
Vit B12 converts propionic acid to succinic acid. Lack of Vit B12 results in accumulation of methylmalonic acid and homocysteine in the blood and causes demyelination in the dorsal columns, peripheral nerves and subcortical white matter.
99
What are the feature of mercury poisoining?
Limb and facial tremor Decreased pain and temperature sensation Memory disturbance and personality change Associated with felt-processing
100
What are the features of lead poisoning?
Peripheral motor neuropathy
101
What are the features of Arsenic poisoning?
Insecticide exposure Loss of tendon reflexes
102
What are the features of ergot poisoning?
Farm worker Fasciculations Myoclonus Sensory neuropathy
103
What are the features of Manganese poisoning?
Miner Drooling Temor Shuffling gait Cog wheel rigidity Becomes quieter as he talks
104
What are the features of fragile X?
Affects boys Moderate retardation ADHD Long face, big ears and macroorchidism
105
What are the features of Prader-Willi?
Obesity from hyperphagia Small hands and feet Hypogonadism Mental retardation
106
Which condition is associated with elfin facies?
Williams syndrome - high levels of calcium in the blood and urine.
107
What condition has loss of language, cognitive decline and stereotypic hand movements?
Rett syndrome
108
What are the features of acute intermittent prophyria?
Recurrent attacks of abdominal pain, psychosis and neuropathy (motor and autonomic)
109
What are the features mononeuritis multiplex?
Diabetic with individual nerves that are transiently disabled. Recovery of function takes weeks to months.
110
Which condition is associated with ataxia, posterior column neuropathy, acanthocytosis (red cells have thorn-like projections) and retinitis pigmentosa?
Abetalipoproteinaemia. Due to the inability to absorb fat-soluble vitamins.
111
What is deficient in an alcoholic with confusion and conjugate gaze deficiency?
Thiamine (Wernicke's encephalopathy)
112
What are the features of Vitamin E deficiency?
Prox. myopathy Opthalmoparesis Loss of pain perception in the feet Absent deep tendon reflexes High CK Liver dysfunction Ataxia
113
What condition is associated with high levels of N-AA in the brain, blood and urine?
Canavan disease
114
What condition is associated with macrocephaly, retardation, blindness and a cherry-red spot in the retina?
Tay-sachs
115
What are the features of Hurler's syndrome?
Alpha-L-Iduronidase deficiency causing accumulation of heparan and dermatan. Presents with dwarfism, facial dysmorphism, cataracts and mental retardation.
116
What is Hunter syndrome?
A mild form of Hurler's syndrome without mental retardation and cataracts.
117
What is Morquio syndrome?
Type 4 mucopolysaccharidosis. Due to deficiency in Galactose-6-sulphate sulphatase deficiency. Skeletal dysplasia, cord compression, cardiac abnormalities and corneal clouding.
118
What is Kearns-Sayre syndrome?
Opthalmoplegia, retinitis pigmentosa, ataxia, heart block, deafness, myopathy, dementia.
119
What is MELAS?
Mitochondiral encephalopathy, lactic acidosis and stroke like symptoms. Commonest mitochondrial disorder. Presents with strokes in patients \<40 years.
120
What condition is associated with bilateral painless visual loss, dystonia and cardiac pre-excitation?
Leber's hereditary optic neuropathy
121
How do you classify aphasias?
Fluent \> Good comprehension \> Good repetition = anomic aphasia with isolated word finding difficulty Fluent \> Good comprehension \> poor repetition = Conduction aphasia due to arcuate fasciculus damage Fluent \> Poor comprehension \> good repetition = Transcortical sensory aphasia due to temporal white matter damage Fluent \> Poor comprehension \> poor repetition = Wernicke's aphasia Non-fluent \> Good comprehension \> Good repetition = Transcortical motor aphasia Non-fluent \> Good comprehension \> poor repetition = Broca's aphasia Non-fluent \> Poor comprehension \> good repetition = Mixed transcortical aphasia Non-fluent \> Poor comprehension \> poor repetition = Global aphasia

FRCS Part 1 (85 decks)

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