Neurology Flashcards
(74 cards)
1
Q
How do tension headaches present? How should they be managed?
A
Bilateral headache, tight band
Paracetamol or NSAID
2
Q
How do migraines present? How should they be managed?
A
Unilateral, throbbing, severe headache; last 4-74hrs
Acute: oral triptan + NSAID/paracetamol + metoclopramide
Prophylaxis *: topiramate/propranolol/TCAs
* only if ≥4 attacks/month
3
Q
What are episodic vs chronic cluster headaches?
A
Episodic — attacks occur in periods lasting from 7 days to one year and are separated by pain-free periods lasting at least 3 months.
Chronic — attacks occur for one year or longer without remission, or with remission periods lasting less than 3 months.
4
Q
How do cluster headaches present? How should they be managed?
A
- At least five attacks of severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15 minutes to 3 hours (untreated) and either or both of the following:
At least one of the following ipsilateral symptoms or signs: - Conjunctival injection and/or lacrimation.
- Nasal congestion and/or rhinorrhoea.
- Eyelid swelling.
- Forehead and facial sweating.
- Forehead and facial flushing.
- Sensation of fullness in the ear.
- Miosis (excessive pupillary constriction) and/or ptosis.
Management
- Offer drug treatment with a triptan for acute attacks,
- Offer short-burst oxygen therapy for acute attacks.
- Encourage the use of drug prophylaxis such as verapamil
5
Q
What is giant cell arteritis?
A
Giant cell arteritis (GCA) is a chronic vasculitis characterized by granulomatous inflammation in the walls of medium and large arteries.
6
Q
How does giant cell arteritis present? How should they be managed?
A
- Visual disturbances such as loss of vision, diplopia, or changes to colour vision.
- Scalp tenderness.
- Intermittent jaw claudication.
- Features of PMR (such as proximal muscle pain, stiffness, and tenderness) are present in about 40% of people with GCA.
- Ix: Bloods (High CRP + ESR + normochromic normocytic anaemia and an elevated platelet count are common.), Biopsy and US
-
Giant cell arteritis (GCA) is a medical emergency.
- Early treatment with effective doses of glucocorticoids may prevent serious complications such as vision loss. (PREDNISOLONE)
7
Q
How do medication overuse headaches present? How should they be managed?
A
≥15 days / month, worsened with medication
Stop medication
8
Q
Define trigeminal neuralgia? Which branches are commonly affected?
A
Trigeminal neuralgia is typically defined as severe, episodic facial pain, in the distribution of one or more branches of the fifth (trigeminal) cranial nerve.
- Typically, the maxillary or mandibular branches are affected, either alone or in combination. Involvement of the ophthalmic branch alone is uncommon.
9
Q
How does trigeminal neuralgia present? How should it be managed?
A
- Severe — often described as electric shock-like, sharp, or shooting. Some people experience continued aching after the acute pain has resolved.
- Unilateral — trigeminal neuralgia is bilateral in only 3% of people and is rarely active bilaterally.
- Short-lived — lasting a few seconds to 2 minutes and stopping suddenly.
- Recurrent — the person may experience many attacks a day, with a refractory period between each attack.
- Episodic — the pain may go into remission for weeks or months before returning. Pain free periods tend to gradually shorten between episodes.
- Provoked by factors such as light touch to the face, eating, talking, or exposure to cold air.
- If there are red flag symptoms and signs that may suggest a serious underlying cause, admit, or refer urgently for specialist assessment, depending on clinical judgement.
- Screen for depression
- If there are no red flag symptoms and signs, offer the person carbamazepine.
10
Q
What are the RFs for trigeminal neuralgia?
A
- Multiple sclerosis.
- Advancing age — trigeminal neuralgia is rare in people under 40 years old. Peak incidence is between age 50 and 60 years.
- Female sex.
- Family history — although rare, familial clusters of trigeminal neuralgia have been reported.
- Hypertension and stroke — however, this association could be due to chance as hypertension and degenerative disease share common epidemiology.
11
Q
What is Idiopathic intracranial pressure? What causes it?
A
Raised intracranial pressure in the absence of a mass lesion or of hydrocephalus.
Idiopathic intracranial hypertension (IIH) appears to be due to impaired cerebrospinal fluid (CSF) absorption from the subarachnoid space across the arachnoid villi into the dural sinuses.
12
Q
What are the RFs for IIH?
A
- Being overweight (90% patients).
- There is an increased risk in women with menstrual irregularity.
- Female-to-male ratio is between 3:1 to 8:1.
- In women it may coincide with recent weight gain, fluid retention, the first trimester of pregnancy and the postpartum period.
- It is increasingly prevalent in deprived populations
13
Q
Describe the presentation of IIH. How do we investigate it?
A
- Headache
- Throbbing (worse in the morning and last thing at night)
- Aggravated by coughing or a change in position
- Gradual field defects
- Nausea, vomiting and drowsiness
- Diplopia
IX
- Exclude other causes of Raised ICP
- CT MRI scanning
- Visual field charting
- LP (pressure > 250 mm CSF)
14
Q
How do we manage IIH?
A
3 aims
- Treatment of underlying disease
- Weight reduction
- Protection of vision
- Acetazolamide or topiramate
- Headache morbidity - migraine preventers, analgesia
Surgical intervention may be considered if other measures are ineffective or there is a rapid or progressive decline in visual function
- Optic nerve sheath fenestration
- CSF shunting
- Intracranial venous sinus stenting
15
Q
What is normal ICP?
A
Normal ICP 7-12 mmHg
16
Q
What causes raised ICP?
A
- Tumours (primary, metastatic)
- Head injury
- Haemorrhage (subdural, extradural, subarachnoid, intracerebral, intraventricular)
- Infection (meningitis, encephalitis, brain abscess)
- Hydrocephalus
- Cerebral oedema
- Status epilepticus
- IIH
17
Q
How does raised ICP present?
A
- Headache (worst on leaning forward) / vomiting
- Altered GCS, focal neurological deficit
- Trauma history
-
Cushing’s response
- Widening pulse pressure
- low HR
- Cheyne-Stokes respiration
- Unilateral ptosis or 3rd or 6th nerve palsy
- Eye changes:
- Pupil constriction early → dilation late
- Reduced visual acuity, peripheral field loss
- Papilloedema ± visible venous pulsation LOSS (absent in 50% normally, but LOSS is a useful sign)
- Blurring of disc margins
- Monroe-Kelly doctrine = the cranium is a rigid box; therefore, the total volume of the intracranial contents must remain constant if ICP is not to change – the CSF/blood compensatory mechanism can compensate for ~100mL
18
Q
How do we investigate raised ICP? How do we monitor it?
A
- CT/MRI scanning to determine any underlying lesion.
- Check and monitor blood glucose, renal function, electrolytes and osmolality.
ICP Monitoring is used in patients with severe head injury (Glasgow Coma Scorebetween 3 and 8 after cardiopulmonary resuscitation) and an abnormal CT scan.
Also in patients with severe head injury and a normal CT scan if two or more of the following features are noted on admission: age over 40 years, unilateral or bilateral motor posturing, systolic blood pressure <90 mm Hg.
- a cut-off of > 20 mmHg is often used to determine if further treatment is needed to reduce the ICP
19
Q
How do we manage raised ICP?
A
- Urgent neurosurgery referral
- Head elevation to 30 degrees
- IV mannitol may be used as an osmotic diuretic
- controlled hyperventilation
- aim is to reduce pCO2 → vasoconstriction of the cerebral arteries → reduced ICP
- leads to rapid, temporary lowering of ICP. However, caution needed as may reduce blood flow to already ischaemic parts of the brain
- removal of CSF, different techniques include:
- drain from intraventricular monitor (see above)
- repeated lumbar puncture (e.g. idiopathic intracranial hypertension)
- ventriculoperitoneal shunt (for hydrocephalus)
20
Q
Define meningitis.
A
Meningitis is inflammation of the two inner meninges (the pia and arachnoid mater) of the brain and spinal cord.
21
Q
What causes meningitis?
A
Causes of meningitis can be infective (bacterial, viral, and fungal) and non-infective (certain cancers, autoimmune disorders, and drugs)
22
Q
What are the most common causative organisms of meningitis in the UK?
A
- Neisseria meningitidis (meningococcus)
- Streptococcus pneumoniae (pneumococcus)
- Haemophilus influenzae type b (Hib)
23
Q
What are the most common causative organisms of meningitis in neonates in the UK?
A
- Streptococcus agalactiae
- Escherichia coli
- S. pneumoniae
- Listeria monocytogenes
24
Q
Name all bacteria that can cause meningitis?
A
25
How do patients with meningitis present?
* **Common non-specific symptoms/signs:**
* Fever.
* Vomiting/nausea.
* Lethargy.
* Irritability/unsettled behaviour.
* Ill appearance.
* Refusing food/drink.
* Headache.
* Muscle ache/joint pain.
* Respiratory symptoms/signs or breathing difficulty.
* **Less common non-specific symptoms/signs include:**
* Chills/shivering.
* Diarrhoea, abdominal pain/distension.
* Sore throat/coryza or other ear, nose, and throat symptoms/signs.
* **More specific symptoms/signs include:**
* [Non-blanching rash](https://cks.nice.org.uk/topics/meningitis-bacterial-meningitis-meningococcal-disease/diagnosis/assessing-the-rash/).
* Stiff neck.
* Capillary refill time of more than 2 seconds, cold hands and feet.
* Unusual skin colour.
* Shock and hypotension.
* Leg pain.
* Back rigidity.
* Bulging fontanelle.
* Photophobia.
* Kernig's sign (person unable to fully extend at the knee when hip is flexed).
* Brudzinski's sign (person’s knees and hips flex when neck is flexed).
* Unconsciousness or toxic/moribund state.
* Paresis.
* Focal neurological deficit including cranial nerve involvement and abnormal pupils.
* Seizures.
26
What does L.monocytogenes cause?
* **meningitis _and_ encephalitis**
27
How should you manage a suspected bacterial meningitis with a non-blanching rash?
* **Arrange emergency medical transfer to hospital by telephoning 999.**
* **Administer a single dose of parenteral benzylpenicillin (intravenously or intramuscularly) at the earliest opportunity]**
* **In hospital: IV Ceftriaxone**
28
How should you manage a suspected bacterial meningitis without a non-blanching rash?
* **Arrange emergency medical transfer to hospital by telephoning 999.**
* **_Do not_ give parenteral antibiotic treatment unless urgent transfer to hospital is not possible**
* **Elderly** - *L. monocytogenes* IV amoxicillin / ampicillin
* **Neonate** - *L. monocytogenes, GBS, E. coli* IV cefotaxime + IV amoxicillin
29
What should be done for close contacts of someone with bacterial meningitis?
* Prophylaxis against meningococcal disease should be considered for the following close contacts, regardless of meningococcal vaccination status:
* People who have had prolonged close contact with the case in a household-type setting during the 7 days before onset of illness (for example, people who are living or sleeping in the same household, pupils in the same dormitory, boy/girlfriends, or university students sharing a kitchen in a hall of residence).
* People who have had transient close contact with a case only if they have been directly exposed to large particle droplets/secretions from the respiratory tract of a case around the time of admission to hospital.
* PO ciprofloxacin
30
Who get chronic meningitis? What organisms causes it?
(immunosuppressed) = TB, syphilis, cryptococcus
31
How do we treat meningitis caused by cryptococcus neoformans?
**Ambisome** ± **flucytosine**
32
What would you expect to see on a TB meningitis MRI?
* _leptomeningeal enhancement_, _basal cistern enhancement_, _dilatation of ventricles_
33
What is the investigation of choice for meningitis?
Investigations suggested by NICE
* full blood count
* CRP
* coagulation screen
* blood culture
* whole-blood PCR
* blood glucose
* blood gas
Lumbar puncture if no signs of raised intracranial pressure
34
35
What else should be given for meningitis?
Intravenous dexamethasone should also be given to reduce the risk of neurological sequelae, but the BNF advise to withhold if:
* septic shock
* meningococcal septicaemia
* immunocompromised
* meningitis following surgery
36
What are the viral causes of meningitis? Who is at risk of getting it?
Causes
* non-polio enteroviruses e.g. coxsackie virus, echovirus
* mumps
* herpes simplex virus (HSV), cytomegalovirus (CMV), herpes zoster viruses
* HIV
* measles
Risk factors
* patients at the extremes of age (\< 5 years and the elderly)
* immunocompromised, e.g. patients with renal failure, with diabetes
* intravenous drug users
37
Describe the management of viral meningitis.
* whilst awaiting the results of the lumbar puncture, treatment should be supportive and if there is any question of bacterial meningitis or of encephalitis, the patient should be commenced on broad-spectrum antibiotics with CNS penetration e.g. ceftriaxone and aciclovir intravenously. This is particularly the case if the patient has risk factors e.g. elderly, immunocompromised
* generally speaking, viral meningitis is self-limiting, with symptoms improving over the course of 7 - 14 days and complications are rare in immunocompetent patients
* aciclovir may be used if the patient is suspected of having meningitis secondary to HSV
38
What are the complications of meningitis?
* sensorineural hearing loss (most common)
* seizures
* focal neurological deficit
* infective
* sepsis
* intracerebral abscess
* pressure
* brain herniation
* hydrocephalus
Patients with meningococcal meningitis are at risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).
39
What is the meningitis/meningococcal sepsis management algorithm?
40
Define encephalitis
Brain parenchyma inflammation
41
How does encephalitis usually present? How does it defer to meningitis?
* fever, headache, psychiatric symptoms, seizures, vomiting
* focal features e.g. aphasia
42
Describe the pathophysiology of encephalitis. What is the most common causative organism in the UK and worldwide?
* **UK** : HSV-1 (viral); non-viral causes like *L. monocytogenes* and *Naegleria fowleri* exist
* **Worldwide :** arboviruses; however, West Nile virus is becoming a leading cause of encephalitis
43
What investigations should be done for encephalitis?
* CSF: lymphocytosis, elevated protein
* PCR for HSV
* neuroimaging
* medial temporal and inferior frontal changes (e.g. petechial haemorrhages)
* normal in one-third of patients
* MRI is better
* EEG pattern: lateralised periodic discharges at 2 Hz
44
How do you manage encephalitis?
* Treatment (often treat as ‘meningoencephalitis’ as you cannot differentiate them):
* _IV aciclovir_ (10mg/kg, TDS) + _IV ceftriaxone_ (2g, BD) ± _IV amoxicillin_ (immunocompromised **or** \>50yo)
45
Define Guillain Barre Syndrome.
Guillain-Barre syndrome describes an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically *Campylobacter jejuni*)
46
Describe the pathogenesis of Guillain Barre syndrome.
* cross-reaction of antibodies with gangliosides in the peripheral nervous system
* correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
* anti-GM1 antibodies in 25% of patients
47
What is Miller Fisher syndrome?
* variant of Guillain-Barre syndrome
* associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
* usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
* anti-GQ1b antibodies are present in 90% of cases
48
How does Guillain Barre syndrome present?
Initial symptoms
* around 65% of patients experience back/leg pain in the initial stages of the illness
The characteristic features of Guillain-Barre syndrome is progressive, symmetrical weakness of all the limbs.
* the weakness is classically **ascending** i.e. the legs are affected first
* reflexes are reduced or absent
* sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
Other features
* there may be a history of gastroenteritis
* respiratory muscle weakness
* cranial nerve involvement
* diplopia
* bilateral facial nerve palsy
* oropharyngeal weakness is common
* autonomic involvement
* urinary retention
* diarrhoea
Less common findings
* papilloedema: thought to be secondary to reduced CSF resorption
49
What are the investigations for Guillain Barre syndrome?
* lumbar puncture
* rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66%
* nerve condution studies may be performed
* decreased motor nerve conduction velocity (due to demyelination)
* prolonged distal motor latency
* increased F wave latency
50
How is Guillain Barre syndrome managed?
A multidisciplinary approach to the acute phase is required, combining supportive therapy and disease-modifying therapy with either high-dose intravenous immunoglobulin (IVIG) or plasma exchange
Respiratory failure is common in GBS, and approximately 20% to 30% of patients need ventilatory support in an intensive care unit (ICU).
DVT prophylaxis
51
What is Multiple sclerosis?
Multiple sclerosis is chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system.
52
Who does MS present in?
* 3 times more common in women
* most commonly diagnosed in people aged 20-40 years
* much more common at higher latitudes (5 times more common than in tropics)
53
What are the subtypes of MS?
Relapsing-remitting disease
Secondary progressive disease
Primary progressive disease
54
How common is relapsing-remitting MS? What is the main feature?
* most common form, accounts for around 85% of patients
* acute attacks (e.g. last 1-2 months) followed by periods of remission
55
What are the main features of secondary progressive MS? How common is it?
* relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses
* around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis
* gait and bladder disorders are generally seen
56
Who do we see primary progressive MS in? How common is it? What is the main feature?
* accounts for 10% of patients
* progressive deterioration from onset
* more common in older people
57
How does MS present?
* 75% of patients have significant lethargy.
Visual
* optic neuritis: common presenting feature
* optic atrophy
* Uhthoff's phenomenon: worsening of vision following rise in body temperature
* internuclear ophthalmoplegia
Sensory
* pins/needles
* numbness
* trigeminal neuralgia
* Lhermitte's syndrome: paraesthesiae in limbs on neck flexion
Motor
* spastic weakness: most commonly seen in the legs
Cerebellar
* ataxia: more often seen during an acute relapse than as a presenting symptom
* tremor
Others
* urinary incontinence
* sexual dysfunction
* intellectual deterioration
58
What is the clinical diagnosis of MS based on?
Diagnosis can be made on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse.
Diagnosis requires demonstration of lesions disseminated in time and space
59
MRI showing multiple white matter plaques penpendicular to the corpus callosum giving the appearance of Dawson fingers
60
Widespread periventricular, juxtacortical, post fossa and upper cervical cord high T2 lesions are noted. Note the difference in the lesions with varying degrees of contrast enhancement and restricted diffusion indicating active/recent demyelination. This satisfies the diagnostic criteria in terms of separation in terms of time space.
61
What other ix could be done for MS diagnoses?
CSF
* oligoclonal bands (and not in serum)
* increased intrathecal synthesis of IgG
Visual evoked potentials
* delayed, but well preserved waveform
62
What is the purpose of the management in MS?
Treatment in multiple sclerosis is focused at reducing the frequency and duration of relapses. There is no cure.
63
How do we split the management options in MS?
* Acute Relapse
* Disease modifying drugs
* Specific symptomatic
64
What do we give for an acute replase?
High dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse.
It should be noted that steroids shorten the duration of a relapse and do not alter the degree of recovery (i.e. whether a patient returns to baseline function)
65
What are the indications for starting disease-modifying drugs?
* relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
* secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
66
What are the disease-modifying drugs?
* natalizumab
* fingolimod
* beta-interferon - not as effective. Given SC/IM
* glatiramer acetate
* immunomodulating drug - acts as an 'immune decoy'
* given subcutaneously
* along with beta-interferon considered an 'older drug' with less effectiveness compared to monoclonal antibodies and S1P) receptor modulators
67
What is natalizumab? How is it given?
* a recombinant monoclonal antibody that antagonises alpha-4 beta-1-integrin found on the surface of leucocytes
* inhibit migration of leucocytes across the endothelium across the blood-brain barrier
* generally considered to have the strongest evidence base for preventing relaspe of the disease-modifying and hence is often used first-line
* given intravenously
68
What is fingolimod? How is it given?
* sphingosine 1-phosphate (S1P) receptor modulator
* prevents lymphocytes from leaving lymph nodes
* oral formulations are available
69
how do we treat fatigue in MS?
* once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine
* other options include mindfulness training and CBT
70
how do we treat spasticity in MS?
* baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine
* physiotherapy is important
71
how do we treat bladder dysfunction in MS?
* may take the form of urgency, incontinence, overflow etc
* guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients
* if significant residual volume → intermittent self-catheterisation
* if no significant residual volume → anticholinergics may improve urinary frequency
72
how do we treat oscillopsia in MS?
* gabapentin is first-line
73
What are the causes of cauda equina syndrome?
* the most common cause is a central disc prolapse
* this typically occurs at L4/5 or L5/S1
* other causes include:
* tumours: primary or metastatic
* infection: abscess, discitis
* trauma
* haematoma
74
How can cauda equina present?
* low back pain
* bilateral sciatica
* present in around 50% of cases
* reduced sensation/pins-and-needles in the perianal area
* decreased anal tone
* it is good practice to check anal tone in patients with new-onset back pain
* however, studies show this has poor sensitivity and specificity for CES
* urinary dysfunction
* e.g. incontinence, reduced awareness of bladder filling, loss of urge to void
* incontinence is a late sign that may indicate irreversible damage
