Neurology Flashcards
1
Q
The presence of headaches, nausea, and transient vision loss in an obese woman with an unremarkable MRI and a raised opening pressure on lumbar puncture indicates what disease?
A
Idiopathic intracranial hypertension
2
Q
How do you diagnose IIH?
A
1st - MRI to look for any features - if unremarkable then 2nd - LP for opening pressure
3
Q
Management of IIH
A
weight loss, acetazolamide therapy
4
Q
What group of ppl do you suspect IIH in
A
Obese females
5
Q
what do you suspect in patients with headaches, nausea and vision loss
A
SOL, tumour, IIH
6
Q
How do you differentiate between syringomyelia and degenerative cervical myelopathy
A
Cervical myelopathy - sensory loss, neck stiffness, LMN signs at level of lesion and UMN signs below the level of the lesion
Synringomyelia - Typically presents with bilateral loss of sensory function limited to the upper extremities
7
Q
what is the management of suspected cervical myelopathy
A
Immediate referral to neurosurgery for decompression as the damage can be permanent
8
Q
What blood vessel is affected in EDH?
A
Middle meningeal artery
9
Q
What are the specific side effects of ergot derived dopamine agonists such as bromocriptine, cabergoline
A
Pulmonary, retroperitoneal and cardiac fibrosis - need echo, ESR, creatinine and CXR
10
Q
What are the side effects of dopamine agonists
A
impulse control disorders (gambling) and excessive daytime somnolence
11
Q
What are the side effects of levodopa
A
dyskinesia (involuntary writhing movements), ‘on-off’ effect, dry mouth, anorexia, palpitations, postural hypotension, psychosis, drowsiness
no use in neuroleptic induced parkinsonism
12
Q
WHat is the defect seen in cranial nerve IV palsy
A
Eye deviated upwards and rotated outwards
13
Q
What is the difference between communicating and non-communicating hydrocephalus
A
Communicating - There is a problem with the secretion or absorption
Non-communicating - there is a problem of flow from the ventricles to the subarachnoid space
14
Q
What are the special features of cluster headaches?
A
Cluster headaches are typically characterized by unilateral and severe headaches often associated with autonomic symptoms such as ptosis, miosis, conjunctival injection and excessive lacrimation.
15
Q
Why are triptans contraindicated in patients with heart problems?
A
It should be avoided in patients with a history of coronary artery disease (CAD) as it has the potential to cause coronary vasospasm.
16
Q
What are the antibodies detected in myanesthia gravis
A
anti-achR antibodies
anti-muscle specific kinase (anti-MUSK) - seronegative
17
Q
What are the diagnostic tests for MG?
A
1st line - blood antibodies (anti-achR and anti-MUSK)
2nd- EMG - electromyography - decremental response to repetitive nerve stimulation
18
Q
What is the most common sequelae of meningitis
A
sensorineural hearing loss (most common)
19
Q
What is the pathophysiology of Narcoplexy?
A
Loss of lateral hypothalamic neurons, which produce orexin A and orexin B (i.e., hypocretin-1 and hypocretin-2) → severe orexin deficiency → dysregulation of sleep-wake cycles
Orexin is a neurotransmitter essential for promoting wakefulness and inhibiting REM sleep.
20
Q
What are the characteristic features of narcoplexy?
A
- typical onset in teenage years
- hypersomnolence - excessive daytime sleepiness despite night sleep
- cataplexy (sudden loss of muscle tone often triggered by emotion - laughing or crying)
- sleep paralysis
- vivid hallucinations on going to sleep or waking up
21
Q
what is the investigation for narcoplexy?
A
multiple sleep latency EEG
22
Q
What is the treatment for narcoplexy?
A
daytime stimulants (e.g. modafinil) and nighttime sodium oxybate
23
Q
What is the precaution to be taken with sodium oxybate?
A
Sodium oxybate should never be taken with alcohol or other CNS depressants because doing so may cause life-threatening respiratory depression!
24
Q
What do you suspect in Bilateral acoustic neuromas ?
A
Neurofibromatosis type II
25
What are the most common primary cancers with brain mets?
lung, breast, kidney, melanoma and colorectal cancers metastases.
26
What is the first thing to do in a patient with a newly discovered tumour?
imaging (CT chest, abdomen and pelvis).
27
What is the management for acoustic neuroma?
Immediate referral to ENT
28
How do you differentiate the acute vs chronic subdural haemorrhage?
Acute- CT imaging is the first-line investigation and will show a crescentic collection, not limited by suture lines. They will appear hyperdense (bright) in comparison to the brain. Large acute subdural haematomas will push on the brain (‘mass effect’) and cause midline shift or herniation.
Chronic- On CT imaging they similarly are crescentic in shape, not restricted by suture lines and compress the brain (‘mass effect’). In contrast to acute subdurals, chronic subdurals are hypodense (dark) compared to the substance of the brain.
29
What is the first line medication for migraine
Triptans + NSAIDs / Triptans + Paracetamol
30
What is the prophylaxis medication for migraine?
Propanolol + Topiramate
31
What prophylaxis is preferred in women of child bearing age?
Propanolol is preferred
| Topiramate - teratogenic effects and it can reduce the effects of hormonal contraceptives
32
How do you differentiate Multiple system atrophy from Parkinson's disease?
Parkinsonism with associated autonomic disturbance (atonic bladder, postural hypotension) points towards Multiple System Atrophy
Key features to help you differentiate are the presence of unilateral symptoms, and more severe/early onset autonomic dysfunction (postural hypotension/erectile dysfunction).
33
How do you differentiate parkinson plus syndromes from parkinson's disease?
1. Poor response to levodopa
2. Early involvement of the autonomic nervous system (orthostatic hypotension, impotence, incontinence, anhidrosis)
3. Early onset of postural instability with frequent falls = although common in parkinsons, it occurs late
4. Dementia
34
What is Multiple system atrophy?
Multiple system atrophy (MSA) is a rare, adult-onset, neurodegenerative disease characterized by neuronal degeneration in the substantia nigra
35
What are the subtypes of multiple system atrophy MSA?
MSA-P: predominantly parkinsonian features
| MSA-C: predominantly cerebellar features (ataxia, tremor, dysarthria)
36
How do you differentiate multiple system atrophy from lewy body dementia?
MSA - Autonomic dysfunction with urogenital problems (othostatic hypotension, erectile dysfunction and atonic bladder)
Lew body dementia- visual hallucinations
37
What could be the causes of postural hypotension in a man with diagnosed parkinsons disease
Could be due to parkinsons disease progression
Could be a side effect of co-careldopa
Could be a parkinson's plus syndrome (presents earlier)
38
What is neuroleptic malignant syndrome?
Life-threatening neurological disorder usually associated with antipsychotics that is characterized by a tetrad of features (fever, muscle rigidity, autonomic instability, and mental status changes) as well as rhabdomyolysis and elevated creatine kinase
39
What are the features of neuroleptic malignant syndrome?
1. Fever,
2. Muscle rigidity
3. Autonomic instability - hypertension, tachycardia and tachypnoea
4. Mental status changes (delerium)
FALTER": Fever, Autonomic instability, Leukocytosis, Tremor, Elevated enzymes (creatine kinase, transaminases), Rigidity
40
What causes Neuroleptic malignant syndrome?
Use antipsychotics, dopaminergic drugs (eg. levodopa)
41
What is the important complication in NMS?
ACUTE KIDNEY INJURY secondary to rhabdomyolsis in severe cases
42
What is the diagnostic testing of NMS?
↑↑ Creatine kinase
Leukocytosis
↑ Transaminases
43
What is the management of the NMS?
1. Discontinue/Stop the antipsychotic drugs
2. IV fluids if in renal failure
3. Dantrolene (ryanodine receptor antagonist) - might be helpful (still dubious)
4. bromocriptine, dopamine agonist, may also be used
44
When does NMS present?
Hours to days after the start of a new antipsychotic drug
45
What type of drug is ondensetron and where does its action occur?
Serotonin receptor antagonists
(5-HT3 antagonists)
1. Strong central antiemetic effect at the area postrema
2. Peripheral antiemetic effect via inhibition of the vagus nerve
46
What are the settings where ondensetron are used?
1. Management of generalised nausea
| 2. Management of chemotherapy-related nausea.
47
What are the side effects of odensetron?
Headaches
constipation is common
prolonged QT interval
48
What is the mechanism of action of Procyclidine and when is it used in Parkinsons's disease?
Antimusucranic - block cholinergic receptors
Now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease
Help tremor and rigidity
e.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)
49
What are the side effects specifically in ergot derived dopamine agonists such as cabergoline and bromocriptine?
pulmonary, retroperitoneal and cardiac fibrosis
echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored
50
What are the general side effects of dopamine agonists?
1. Patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence
2. More likely than levodopa to cause hallucinations in older patients. Nasal congestion and postural hypotension are also seen in some patients
51
What are the possible aetiologies of restless leg syndrome?
Primary: positive family history in 50% of patients with idiopathic RLS
Secondary:
1. iron deficiency with or without anaemia
2. uraemia - end stage renal disease
3. diabetes mellitus - peripheral neuropathy
4. pregnancy
5. Inflammatory conditions: celiac disease, rheumatoid arthritis, inflammatory bowel diseases
52
What are the clinical features of restless leg syndorme
1. A recurrent urge to move the legs that is typically relieved by movement and worsened by rest. (Akathisia)
Often worse in the evening and at night. May occur exclusively at night.
2. Paraesthesias e.g. 'crawling' or 'throbbing' sensations
3. movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
53
Management of restless leg syndrome
1. simple measures: walking, stretching, massaging affected limbs
2. treat any iron deficiency
3. Dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
4. Benzodiazepines
5. Gabapentin
54
What are drugs that make MG worse?
```
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
```
55
What is the difference between the effects of suxamethonium and rocuronium in MG
Suxamethonium = succinylcholine (depolarizing NMA) It is a strong Ach agonist and produces sustained depolarization, so much so that muscle cannot repolarize to depolarize, so patient cannot have further muscle contraction
It acts by binding to and activating the receptor, at first causing muscle contraction, then paralysis.
Suxamethonium needs to bind to the receptors AND depolarise the muscle to work, and as in MG they're being taken up by antibodies there's fewer places to bind and so more Sux will be needed to depolarise. Thus it cannot be used in the patients as a neuromuscular blocker in patients with NMD
Rocuronium instead works by binding to the receptors and stopping acetylcholine from binding, BUT does not need to depolarise the cell. This basically means it's doing the same thing as the MG antibodies and so you need less Roc as it's already having it's work done by the MG
56
What is the aetiology of essential tremor?
Positive family history (50–70%; autosomal dominant inheritance) or sporadic
57
What are the features of essential tremors?
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
58
What is the management of essential tremors?
1st line - propanolol
| 2nd line- primidone
59
When do you treat for status epilepticus?
If the seizure hasn't stopped for >5 mins, then it is unlikely to stop. so despite the definition being 30 mins, treat if seizure is unstopped after 5 mins
60
What is the treatment protocol for status epilepticus?
ABC
1. Benzodiazepines such as diazepam or lorazepam (diazepam- rectally, lorazepam - IV - repeated after 10-20mins)
2. If ongoing (or 'established') status it is appropriate to start a second-line agent such as phenytoin or phenobarbital infusion
3. If no response ('refractory status') within 45 minutes from onset, then the best way to achieve rapid control of seizure activity is induction of general anaesthesia.
61
What are the side effects of phenytoin?
PHENYTOIN: cytochrome P-450 interaction, Hirsutism, Enlarged gums (gingival hyperplasia), Nystagmus, Yellow-browning of skin (melasma), Teratogenicity, Osteomalacia, Interacts with folate, Neuropathy
Can't PHEEL-y-TOE-n
Inducer of the P450 system
62
which condition is commonly associated with MND
Frontotemporal dementia and MND are linked by the mutation in the same gene.
C9orf72 gene mutation is associated with both ALS and frontotemporal dementia
63
What is the pathophysiology of Lambert-Eaton syndrome
Autoantibodies are directed against presynaptic voltage-gated calcium channels → impaired acetylcholine release in the NMJ.
64
What is associated with Lambert-Eaton syndrome?
small-cell lung carcinoma (in ⅔ of LEMS cases)
65
How do you differentiate drug induced parkinsons from idiopathic parkinsons disease?
Symmetrical tremor, young age - drug induced
| Asymmetrical tremor - Idiopathic
66
What are the causes of parkinsonism
1. Idiopathic Parkinson's disease
2. drug-induced e.g. antipsychotics, metoclopramide*
3. progressive supranuclear palsy
4. multiple system atrophy
5. Wilson's disease
6. post-encephalitis
7. dementia pugilistica (secondary to chronic head trauma e.g. boxing)
8. toxins: carbon monoxide, MPTP
67
What is the secondary prevention in stroke?
1st line - clopidogrel
2nd line - if clopidogrel CI / aspirin + modified release dipyridamole
3rd - if both clopidrogel and aspirin CI - only modified release dipyridamole
68
How do you differentiate progressive supranuclear palsy (PSP) from MSA and Lew body dementia
PSP - vertical gaze palsy, frontal lobe problems (apathy, disinhibition, impaired reasoning)
MSA - autonomic dysfunction with urogenital problems
Lewy body dementia - visual hallucinations
69
What is the management of generalised tonic-clonic seizures?
sodium valproate
second line: lamotrigine, carbamazepine
Monotherapy with another drug should be attempted before combination therapy is started. Caution should be exercised when combining sodium valproate and lamotrigine as serious skin rashes such as Steven-Johnson's syndrome may be provoked
70
What are the nerves affected in acoustic neuroma and what are the features it presents with?
cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
cranial nerve V: absent corneal reflex
cranial nerve VII: facial palsy
71
What is the mechanism of action of lamotrigine and what are its side effects?
Inhibition of voltage-gated Na+channels → ↓ glutamate release
Side effect = Stevens-Johnson syndrome (slow titration is necessary to prevent skin and mucous membrane reactions)
72
What are the drugs that can trigger steven johnson syndrome?
Antibiotics: sulfonamides (e.g., TMP/SMX), aminopenicillins, rifampicin
Corticosteroids
Antiretroviral drugs (e.g., nevirapine)
Antiepileptics: phenytoin, phenobarbital, lamotrigine, valproic acid, carbamazepine, ethosuximide
Oxicam NSAIDs (e.g., piroxicam)
Allopurinol
Sulfasalazine
73
What are the common causes of stevens johnson syndrome in children?
Infections: mycoplasma pneumonia, CMV, herpes
74
What are the features of of stevens-johnson syndrome?
1. Prodromal phase: begins 1–3 weeks after the intake of medication): high fever (usually > 39°C or 102°F), malaise, sore throat, myalgia and/or arthralgia (like a viral infection)
2. Mucocutaneous lesions begin 1-3 days after the onset of prodromal symptoms
i. painful erythematous/purpuric macules and may have targetoid apperance
ii. Lesions form bullae or vesicles - positive Nikolsky sign
iii. Extensive epidermal sloughing
iv. Healing by re-epithelisation
Mucosal membranes are almost always involved:
Oral and pharyngeal mucosa : stomatitis, cheilitis, oral ulcers, odynophagia
Severe conjunctivitis, corneal ulcers : photophobia, eye pain
Urethritis, genital ulcers, and/or perianal ulcers
Esophageal ulcers and/or strictures (less common): dysphagia
Tracheal involvement (less common)
Small bowel, colon involvement (rare): diarrhea, melena, intussusception, bowel perforation
75
How do you differentiate steven-johnson syndrome from staphylococcal scaled skin syndorme?
The involvement of mucous membranes differentiates SJS from staphylococcal scalded skin syndrome (SSSS) in which mucous membranes are spared!
SSSS is also much more commonly seen in a younger age group (especially infants).
SKin biopsy - The split is intradermal in the case of SSSS, while the split appears to be subepidermal in the case of TEN and SJS.
76
What is the management of SJS?
The immediate management of SJS includes ceasing the culprit drug, in this case lamotrigine. Hospital admission is required, ideally to an intensive care unit or burns unit. Fluid replacement via intravenous and nasogastric access is required, as there is significant fluid loss from the blisters and red, oozing dermis.
77
What is the treatment criteria for carotid artery endarterectomy performed in a TIA patientst?
Recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled
Should only be considered if carotid stenosis > 70% according ECST* criteria or > 50% according to NASCET** criteria
78
What is the pharmacological management of a patient who is suspected to have TIA based on the history?
Immediate antithrombotic therapy:
give aspirin 300 mg immediately, unless
1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage)
2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
3. Aspirin is contraindicated: discuss management urgently with the specialist team
79
What is the management of a patient who has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis
discuss the need for admission or observation urgently with a stroke specialist
80
What is the management of a patient who has had a suspected TIA in the last 7 days
arrange urgent assessment (within 24 hours) by a specialist stroke physician
81
What is the management of a patient who has had a suspected TIA which occurred more than a week previously
refer for specialist assessment as soon as possible within 7 days
82
What is the most common type of brain tumour?
Glioblastoma multiformae
* Glioblastoma is the most common primary tumour in adults and is associated with a poor prognosis (~ 1yr).
* On imaging they are solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.
* Histology: Pleomorphic tumour cells border necrotic areas
* Treatment is surgical with postoperative chemotherapy and/or radiotherapy. Dexamethasone is used to treat the oedema.
83
What type of tumour typically arises from falx cerebri and pushes on the brain. There is a well-defined border between the tumour and the brain parenchyma.
Meningioma (2nd most common type of brain tumour in adults)
Meningiomas are typically benign, extrinsic tumours of the central nervous system. They arise from the dura mater of the meninges and cause symptoms by compression rather than invasion.
* They typically are located at the falx cerebri, superior sagittal sinus, convexity or skull base.
* Histology: Spindle cells in concentric whorls and calcified psammoma bodies
* Investigation is with CT (will show contrast enhancement) and MRI, and treatment will involve either observation, radiotherapy or surgical resection.
84
What are the risk factors for idiopathic intracranial hypertension?
```
Obesity
Certain medications, including:
1. Growth hormone
2. Tetracyclines
3. Excessive vitamin A or derivatives
```
85
What is the most common paediatric brain tumour?
Craniopharyngioma - supratentorial tumour
* A craniopharyngioma is a solid/cystic tumour of the sellar region that is derived from the remnants of Rathke’s pouch. It is common in children, but can present in adults also. It may present with hormonal disturbance, symptoms of hydrocephalus or bitemporal hemianopia.
* Histology: Derived from remnants of Rathke pouch
* Investigation requires pituitary blood profile and MRI. Treatment is typically surgical with or without postoperative radiotherapy.
86
A 75-year-old male is found unconscious by his wife. He had previously been fit and well. He has a past medical history of long standing uncontrolled hypertension. On examination you note quadriplegia and small reactive pupils. He has a Glasgow coma score of 6 (M2, E2, V2).
Pontine haemorrhage as a result of chronic hypertension
Pinpoint Pupils points to pons!
Patients often present with reduces Glasgow coma score, quadriplegia, miosis, and absent horizontal eye movements.
87
What are the causes of bell palsy?
```
Trauma (e.g., temporal bone fracture)
Infection (e.g., herpes zoster, borreliosis, HSV infection, HIV infection)
Tumors
Pregnancy
Diabetes mellitus
Guillain-Barré syndrome
Sarcoidosis
Amyloidosis
Stroke
```
88
What is the management of bell palsy?
i) in the past a variety of treatment options have been proposed including no treatment, prednisolone only and a combination of aciclovir and prednisolone
ii) following a National Institute for Health randomised controlled trial it is now recommended that prednisolone 1mg/kg for 10 days should be prescribed for patients within 72 hours of onset of Bell's palsy. Adding in aciclovir gives no additional benefit
iii) eye care is important - prescription of artificial tears and eye lubricants should be considered
89
What is the management of migraine in patients during pregnancy?
1st line - paracetamol
2nd line - NSAIDs can be used second-line in the first and second trimester
avoid aspirin and opioids such as codeine during pregnancy
90
What is weber syndrome?
Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain)
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
91
What is wallenberg syndrome?
Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
92
What are the localising feature of the temporal lobe focal epilepsy
HEAD
Hallucinations (auditory/gustatory/olfactory), Epigastric rising/Emotional, Automatisms (lip smacking/grabbing/plucking), Deja vu/Dysphasia post-ictal)
93
What are the localising feature of the frontal lobe focal epilepsy
Head/leg movements, posturing, post-ictal weakness, Jacksonian march
94
What are the localising feature of the occipital lobe focal epilepsy
Floaters and flashes
95
What is the antibody that is detected in lambert eaton syndrome?
Voltage-gated calcium-channel antibodies
96
Who does Bell palsy affect the most
The peak incidence is 20-40 years and the condition is more common in pregnant women.
97
When can you stop anticonvulsants in epileptic patients
Can be considered if seizure free for > 2 year, with AEDs being stopped over 2-3 months
98
Which is the best assessment tool for differentiating between stroke and stroke mimics?
The Recognition of Stroke in the Emergency Room (ROSIER) scale is effective in the initial differentiation of acute stroke from stroke mimics.
99
What is the use of the ABCD2
ABCD2 score is used for predicting the occurrence of a stroke in the short term period following a transient ischaemic attack.
100
What is the NIHSS
National Institutes of Health Stroke Scale (NIHSS) is a tool used to objectively measure the impairment caused by a stroke.
101
What are the features of normal pressure hydrocephalus
wet, wobbly and whacky -urinary incontinence, gait ataxia, and dementia respectively.
A classical triad of features is seen
urinary incontinence
dementia and bradyphrenia (reversible)
gait abnormality (may be similar to Parkinson's disease)
102
What is the management of a patient with normal pressure hydrocephalus
ventriculoperitoneal shunting
around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
103
What is expected in the blood tests of neuroleptic malignant syndorme?
Raised CK and leucocytosis
104
What is the sign used to differentiate between organic and inorganic lower leg weakness?
Hoover sign
105
What are the clinical features of wernickes encephalopathy?
CAN OPEN
```
Confusion
Ataxia
Nystagmus
Ophthamoplegia
PEripheral
Neuropathy
```
106
What happens if wernicke's encephalopathy goes untreated and what are its symptoms?
If not treated Korsakoff's syndrome may develop as well. This is termed Wernicke-Korsakoff syndrome and is characterised by the addition of antero- and retrograde amnesia and confabulation in addition to the above symptoms.
107
What is the drug for generalised tonic clonic seizures?
sodium valproate
| second line: lamotrigine, carbamazepine
108
What is the drug for absense seizures?
sodium valproate or ethosuximide
| sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy
109
What is the drug for myoclonic seizures?
sodium valproate
| second line: clonazepam, lamotrigine
110
What is the drug for focal seizures?
carbamazepine or lamotrigine
| second line: levetiracetam, oxcarbazepine or sodium valproate
111
What are the seizure types that carbamazepine cannot be used in?
carbamazepine may exacerbate absence seizures and myoclonic seizures
112
Which anticonvulsant is associated with weight gain?
Sodium valporate
113
What is the investigation of GBS?
1. lumbar puncture- rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66% - In GBS, elevated protein levels result from impaired CSF protein resorption. Nevertheless, normal CSF findings may present in early stages of the disease.
2. nerve condution studies may be performed
114
what type of drug is acetazolamide and how does it function?
Acetazolamide is a carbonic anhydrase inhibitor that is used in IIH to reduce the production of cerebrospinal fluid in order to reduce intracranial pressure.
115
A 30-year-old woman is admitted to hospital with an unprovoked first seizure. She attends neurology clinic and asks for advice regarding driving as she uses her car to drop her children to school. The neurologist has not made a formal diagnosis of epilepsy but will review the patient again in the clinic in 6 months time. Can she still drive?
Following a first seizure, patients must be seizure for 6 months before they may reapply to the DVLA for their license to be reissued. If the patient has a formal diagnosis of epilepsy they must be seizure-free for at least 12 months before they may reapply to the DVLA for their license to be reissued.
116
What are the features of facial nerve palsy?
Supply - 'face, ear, taste, tear'
face: muscles of facial expression - bell palsy
ear: nerve to stapedius - hyperacusis
taste: supplies anterior two-thirds of tongue
tear: parasympathetic fibres to lacrimal glands, also salivary glands
117
What are the causes of bilateral facial nerve palsy?
sarcoidosis
Guillain-Barre syndrome
Lyme disease
bilateral acoustic neuromas (as in neurofibromatosis type 2)
as Bell's palsy is relatively common it accounts for up to 25% of cases f bilateral palsy, but this represents only 1% of total Bell's palsy cases
118
Causes of unilateral facial nerve palsy
```
Bell's palsy
Ramsay-Hunt syndrome (due to herpes zoster)
acoustic neuroma
parotid tumours
HIV
multiple sclerosis*
diabetes mellitus
```
UMN - stroke
119
How do you differentiate seizures from non-epileptic
About 20% of patients with (apparently) treatment‑resistant epilepsy also or solely suffer from psychic seizures. Important differential criteria: closed eyes, no or only slight increase in CK and prolactin, duration often considerably longer (up to hours).
Factors favouring true epileptic seizures
1. tongue biting
2. raised serum prolactin (why prolactin is raised following seizures is not fully understood. It is hypothesised that there is spread of electrical activity to the ventromedial hypothalamus, leading to release of a specific prolactin regulator into the hypophyseal portal system)
120
What is the cause of bitemporal superior quandrantopia?
inferior chiasmal compression, commonly a pituitary tumour
121
What is the cause of bitemporal inferior quandrantopia?
superior chiasmal compression, commonly a craniopharyngioma
122
What do you need to worry about in parkinsons patients admitted to the ward?
SHould not with-hold colbenyldopa - can cause neuroleptic malignant syndrome - raised Creatinine kinase
123
What is the management of trigeminal neuralgia?
1st line - Carbamazepine
2nd line - failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
124
A 72-year-old man presents to his general practitioner with tremor in his right hand, and stiffness in his right arm. Over the past 4 months he has experienced episodes of urinary incontinence, which he has found quite distressing. Additionally, he complains of being light headed when getting up in the morning, and feels as if he is going to pass out. He has a history of hypercholesterolaemia, which is controlled with atorvastatin.
What is the most likely diagnosis?
Parkinsonism with associated autonomic disturbance (atonic bladder, postural hypotension) points towards Multiple System Atrophy
Parkinson's disease presents with tremor, rigidity and bradykinesia
Lewy body dementia would present with Parkinsonism and visual hallucinations
Olivopontocerebellar atrophy presents with Parkinsonism and cerebellar signs
Progressive supranuclear palsy presents with Parkinsonism and ophthalmoplegia
125
What is the criteria for oxford stroke classification
1. Unilateral hemipariesis and hemosensory loss
2. Homonymous hemianopia
3. Higher cognitive dysfunction
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Commonest cause of lacunar stroke?
Hypertension
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What are the signs of PICA?
Bilateral weakness
cerebellar signs
visual problems
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What are the complications of stroke?
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Raised ICP (Cerebral oedema, haemorrhage)
Aspiration
Pressure sores
depression
cognitive impairment
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What is the threshold BP for thrombolysis CI
185/110 mmHg
130
When do you start anticoagulation in stroke patients with AF?
2 weeks after
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How do you check if thrombolysis is successful
24hr after CT head
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When do you prefer thrombectomy?
1. High NIHSS (pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)
2. Independently mobile
3. Time window
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What is the driving restriction for stroke patients?
4 weeks /1 month
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Management of respiratory failure in MND
O2 with care
Consider infection
Non-invasive ventilation
ITU not appropriate
135
What is the mode of inheritance of neurofibramatosis
Autosomal dominant inheritantace
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What are the two types of neurofibramatosis and which chromosomes and genes are affected?
NF1 is also known as von Recklinghausen's syndrome. It is caused by a gene mutation on chromosome 17 which encodes neurofibromin gene and affects around 1 in 4,000
NF2 is caused by gene mutation on chromosome 22 and encodes merlin protein.
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What are the features of NF1
1. Multiple neurofibromas - soft, painless nodules; age of onset - adolescence
2. Café au lait spots - brown, flat macule or patch (onset before 2 years) - melanocyte dysfunction
3. Lisch nodules - pigmented iris harmatomas (onset 5-10 years) brown - melanocyte dysfunction
4. Scoliosis, short stature
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CAFE SPOTS - mnemonic
Café au lait spots
Axillary freckling
neuroFibromas
nodules in the Eye (Lisch nodules)
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Skeletal abnormalities (e.g., Scoliosis, sphenoid dysplasia)
high blood Pressure , pheochromocytoma
Optic Tumor - optic glioma
Stature (usually Short).
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What are the features of NF2
Age of onset - 18-24 yrs
1. Bilateral vestibular schwannomas (acoustic neuromas) - can cause tinnitus, hearing loss and vertigo
2. Early onset cataracts, usually bilateral
3. multiple cerebral and spinal tumours (meningiomas, ependymosmas)
In NF Type 2, the mutation is in chromosome Twenty-2 and the clinical findings (schwannoma and cataracts) manifest on 2 sides.
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Management of NF1?
conservative
Multidisciplinary team with geneticist, neurologist, surgeon and physio
Yearly cutaneous survery and measurement of BP
If neurofibromas catch on clothing - excise some
genetic counselling required
140
Management of NF2?
Conservative
Hearing tests yearly from puberty in affected families, with MRI brain if abnormality is detected
A normal MRI in late teens - helpful in assessing risk to any offspring
Clear scan at 30yrs indicates that the gene has not been inherited
Complicated neurosurgery - to fix hearing loss and facial palsy
mean survival from diagnosis - 15yrs
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Important Differential diagnosis of NF?
Schwannomatosis consists of multiple peripheral and intracranial schwannomas. Bilateral vestibular schwannomas are rare.
142
Differentials of Cafe au lait spots?
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Normal (up to age of 5)
McCune-Albright syndrome
Fanconi anemia
Tuberous sclerosis
Ataxia telangiectasia
Noonan syndrome
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What is the mode of inheritance of tuberous sclerosis?
Autosomal dominant inheritance
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What are the features of tuberous sclerosis?
HAMAARTOMASS –
Hamartomas - skin
Ash leaf spots - hypopigmented macules on the trunk and extremities -which fluoresce under UV light
Mind (intellectual disability) - developmental delay
Adenoma sebaceum - benign tumour - acne like appearance around nose and cheeks - butterfly like
renal Angiomyolipoma - may manifest feeling of abdo fullness
Rhabdomyoma (cardiac) and retinal hamartomas: dense white areas on retina (phakomata)
Tumor suppressor genes (TSC1 gene and TSC2 gene)
autosomal dOminant
Mitral regurgitation
Astrocytomas
Seizures - infantile spasms
Shagreen patches - roughened patches of skin over lumbar spine
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What is the mode of inheritance of the von-hippel lindau syndrome and what are the features?
autosomal dominant inheritance affecting the VHL gene - short arm of chromosome 3 - tumour and cyst development
Von HIPPEL-Lindau syndrome: (HIPPEL)
Hemangioblastoma - cerebellar mostly - these can cause subarachnoid haemorrhages
Increased risk of bilateral renal cell carcinoma -clear-cell renal cell carcinoma
Pheochromocytoma
Pancreatic, renal and adrenal lesions (cysts, cystadenomas, and neuroendocrine tumors)
Eye Lesions (retinal angiomas or hemangioblastomas - vitreous haemorrhage).
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What is the mode of inheritance and the features of Sturge weber syndrome?
Congenital noninherited developmental anomaly of neural crest derivatives - GNAQ gene
Port wine stain
Leptomeningeal angioma
seizures
intellectual disability
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What is the mode of inheritance of ataxia telengectasia and what are the clinical features of the disease?
It is autosomal recessive and affects the ATM gene
4As:
1. ATM gene
2. Ataxia - cerebellar involvement
3. Spider Angiomas - involves the conjunctiva and face
4. IgA deficiency
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A 79-year-old man presents to the emergency department with weakness in his right arm and right leg, as well as drooping of right side of his lip. These symptoms lasted for about 1 hour and have now resolved. He hasn't had any similar previous episodes. His past medical history includes a pulmonary embolism 3 months ago, for which he is being treated with warfarin. Examination is unremarkable.
What is the next step in the management of this man?
Admit for CT scan
If a patient is on warfarin/a DOAC/ or has a bleeding disorder and they are suspected of having a TIA, they should be admitted immediately for imaging to exclude a haemorrhage
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What is the management of TIA?
Give aspirin 300 mg immediately, unless
1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage)
2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
3. Aspirin is contraindicated: discuss management urgently with the specialist team
150
What is the management of the patient who has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis?
Discuss the need for admission or observation urgently with a stroke specialist
151
What is the management of the patient who has had a suspected TIA in the last 7 days?
Arrange urgent assessment (within 24 hours) by a specialist stroke physician
152
What is the management of the patient who has had a suspected TIA more than a week ago?
Refer for specialist assessment as soon as possible within 7 days
153
What is the DVLA rules for TIA?
Advise the person not to drive until they have been seen by a specialist.
154
What is the long term management of TIA?
- Clopidogrel is recommended first-line (as for patients who've had a stroke)
- Aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel
155
What can trigger cluster headaches?
Alcohol
156
What is the acute management of cluster headaches?
100% oxygen + subcutaenous sumatriptans
157
A 23-year-old man presents to neurology with an 8 month history of increasing muscle wasting in his calves and forearms. Alongside this, he has also described difficulty walking and has sprained his ankle of several occasions in the past 6 months as well noticing some tingling in his fingers. He also states his mother has a similar problem.
On examination, there is clear evidence of wasting in his calf muscles and high arches in his feet.
What is the most likely diagnosis?
Charcot marie tooth syndrome
Charcot-Marie-Tooth Disease is the most common hereditary sensorimotor peripheral neuropathy. Autosomal dominant
Inverted champagne bottle sign - calve wasting
- There may be a history of frequently sprained ankles
- Foot drop
- High-arched feet (pes cavus)
- Hammer toes
- Distal muscle weakness
- Distal muscle atrophy
- Hyporeflexia
- Stork leg deformity
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What are the drugs that cause serotonin syndrome?
- Monoamine oxidase inhibitors
- SSRIs
- St John's Wort, often taken over the counter for depression, can interact with SSRIs to cause serotonin syndrome
- Ecstasy
- Amphetamines
159
what are the drugs used in neuropathic pain?
1st line - amitriptyline, duloxetine, gabapentin or pregabalin (if one doesnt work, try 1 of the other 3)
Rescue therapy - tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain
topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia)
160
A 65-year-old male with known nasopharyngeal carcinoma presents with double vision over a few weeks. On examination he is found to have left eye proptosis and it is down and out. He reports pain on attempting to move the eye. There is an absent corneal reflex. What is the most likely diagnosis?
Cavernous sinus syndrome
161
What are the effects of the various anti-epileptic medications of pregnancy?
Sodium valproate: associated with neural tube defects
Phenytoin: associated with cleft palate (if still taking during pregnancy, give vitamin K in the last month of pregnancy)
Carbamezapine - less teratogenic
Lamotrigine - shown to be relatively safe atm. (dose may need to increased during pregnancy)
162
What is the CI for triptans?
Ischaemic heart disease
163
What are the differentials for spastic paraparesis?
Cerebral palsy and multiple sclerosis
| Cord compression, disc prolapse, infection, trauma
164
what is the criteria to send for CT head immediately?
- 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
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what is the criteria to send for CT head within 8hrs?
For adults with any of the following risk factors who have experienced some loss of consciousness or amnesia since the injury:
- Age 65 years or older
- Any history of bleeding or clotting disorders
dangerous mechanism of injury (a 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
If a patient is on warfarin who have sustained a head injury with no other indications for a CT head scan, perform a CT head scan within 8 hours of the injury.
166
What is Ménière's disease? Mx?
Endolymphatic system pressure increases causes recurrent episodes of vertigo, sensorineural hearing loss, tinnitus, and a feeling of aural fullness.
Treatment involves antihistamines and bed rest.
167
What is BPPV? Dx? Mx?
The presence of debris in the semicircular canals of the ears causes vertigo upon head movement.
The Hallpike manoeuvre is diagnostic, where certain movements of the head causes fatiguable nystagmus.
Epley manoeuvres treat BPPV by clearing the debris.
168
What is Acute labyrinthitis? Dx? Mx?
Inflammation of the vestibular nerve causes an acute severe vertigo, which may be associated with vomiting. It does not involve deafness or tinnitus.
It is often associated with a recent viral illness or a vascular lesion, in which case it may involve other cranial nerve deficits.
It often resolves completely over a month, so treatment is conservative, although sedatives may help in severe cases.
169
A 74-year-old diabetic male presents to the clinic. He has had type 1 diabetes controlled with insulin for the past 60 years. He gives a history of transient left-sided facial weakness, which recovered spontaneously after two weeks. He previously presented three months earlier with a left common peroneal nerve palsy leading to motor and sensory loss. Again this recovered spontaneously after three to four weeks. On examination his BP is 110.70mmHg with pulse 70 bpm and regular. There is peripheral sensory loss affecting both feet. What is the most likely diagnosis?
Mononeuritis multiplex
170
What is the first step to do if suspected giant cell arteritis?
Oral Prednisolone 40-60mg
171
What do you do if giant cell is suspected and pred is given?
Send to ophthalmology same day or within 3 days as there is risk of Anterior ischaemic optic neuritis
172
CI for triptan?
Pregnancy, IHD
| Can be drowsy during driving
173
What is the prophylaxis for cluster headache?
Verapamil
174
What is the treatment for Ramsay Hunt syndrome?
oral aciclovir and corticosteroids
175
Red flag's for headache
-compromised immunity, caused, for example, by HIV or immunosuppressive drugs
- age under 20 years and a history of malignancy
- a history of malignancy known to metastasis to the brain
- vomiting without other obvious cause
- worsening headache with fever
- sudden-onset headache reaching maximum intensity within 5 minutes - 'thunderclap'
- new-onset neurological deficit
- new-onset cognitive dysfunction
- change in personality
- impaired level of consciousness
- recent (typically within the past 3 months) head trauma
- headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked), sneeze or exercise
orthostatic headache (headache that changes with posture)
- symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma
- a substantial change in the characteristics of their headache
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what are the drugs that precipitate IIH
oral contraceptive pill, steroids, tetracycline, vitamin A, lithium
177
What are the features of lacunar stroke?
involves perforating arteries around the internal capsule, thalamus and basal ganglia
presents with 1 of the following:
1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
2. pure sensory stroke.
3. ataxic hemiparesis
178
What are the features of posterior circulation stroke?
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involves vertebrobasilar arteries
presents with 1 of the following:
1. cerebellar or brainstem syndromes
2. loss of consciousness
3. isolated homonymous hemianopia
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179
Whats the difference in the
Vermix - ataxia
| Central - past pointing
