Neurology - Level 3 Flashcards

1
Q

Classes of brain tumours?

A

o High-grade – grows rapidly and is aggressive

o Low-grade – grows slowly but which may/may not be successfully treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Epidemiology of brain tumours?

A
  • 2% of all tumours
  • Lifetime risk is 1 in 77
  • Common 50-70
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Risk factors of brain tumours?

A
o	Ionising radiation
o	Vinyl chloride
o	Immunosuppression
o	Oil refining, embalming, textiles
o	Neurofibromatosis, VHL disease, TSC, Li-Fraumeni syndrome, Cowden’s disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Types of brain tumours - malignant?

A

o Metastases most commonly from other sites 10x

 Lung, breast, stomach, prostate, thyroid, colorectal, melanoma, kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Types of brain tumours - high grade?

A

 Gliomas, glioblastoma multiforme
 Primary cerebral lymphomas
 Medulloblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Types of brain tumours - low grade?

A
	Meningioma
	Acoustic neuroma
	Neurofibromas
	Pituitary tumours
	Pineal tumours
	Craniopharyngioma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Symptoms of brain tumours?

A
o	Headache
	Worst in mornings, coughing
o	Nausea and vomiting
o	Seizures
o	Progressive focal neurological deficits
	Diplopia, visual field defect, upper/lower limbs
o	Behavioural changes
o	Papilledema
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Symptoms related to location of mass of brain tumours - temporal?

A

dysphasia, contralateral homonymous hemianopia, amnesia, odd phenomenon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Symptoms related to location of mass of brain tumours - frontal?

A

hemiparesis, personality change (indecent, indolent, facetious), Broca’s dysphasia, unilateral anosmia, concrete thinking, perseveration, executive dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Symptoms related to location of mass of brain tumours - parietal?

A

hemisensory loss, sensory inattention, dysphasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Symptoms related to location of mass of brain tumours - occipital?

A

contralateral visual field defects, seeing multiple images

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Symptoms related to location of mass of brain tumours - cerebellum?

A

DASHING (dysdiadochokinesia, ataxia, slurred speech, hypotonia, intention tremor, nystagmus, gait abnormalities)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Investigations in brain tumours?

A

• Urgent direct access MRI scan of brain (CT if MRI CI)
 Progressive, sub-acute loss of central neurological function
• Urgent referral appointment for children within 48 hours
• Others: Technetium brain scan, Magnetic resonance angiography, PET

  • Stereotactic biopsy via skull burr-hole
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Investigations in gliomas?

A

 MRI
 MDT team
 MR perfusion/spectroscopy
 Molecular markers (IDH1&2, ATRX, 1p/19q, H3.3K27M, BRAF, MGMT, TERT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Management of glioma - low grade?

A
  • 5-ALA-guided resection (within 6 months)
    • If not appropriate: Biopsy to attain histology and molecular diagnosis
  • If very low grade – active monitoring – then resection if progressing
  • Post-surgical Radiotherapy followed by 6 cycles of PCV chemotherapy
    • If 1p/19q, IDH-low grade glioma and >40 or residual tumour
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Management of glioma - Grade 3?

A

5-ALA-guided resection (within 6 months)
- Post-surgical radiotherapy followed by PCV chemotherapy
• If newly diagnosed grade 3 glioma with 1p/19q codeletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Management of glioma - Grade 4?

A
  • Radiotherapy with temozolomide chemotherapy

• If newly diagnosed glioma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Management of glioma - Recurrent grade 3 or 4?

A
  • PCV or lomustine chemotherapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Investigations in meningioma?

A

 MRI (CT if assessing bones)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Management of meningioma?

A

 5-ALA-guided resection (within 6 months)

 Radiotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Investigations in brain metastases?

A

 MRI (CT if assessing bones)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Management of brain metastases - single?

A
  • Systemic anti-cancer therapy if likely to respond (germ cell, small-cell lung)
  • Surgical excision
  • Stereotactic radiosurgery or radiotherapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Management of brain metastases - multiple?

A
  • Adjuvant stereotactic radiosurgery/radiotherapy if 1-3 metastases
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Follow up in brain tumours?

A
  • MRI scans and clinical assessment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Other medications used in management of brain tumours?

A
  • Analgesics – codeine
  • Anticonvulsants – phenytoin
  • Corticosteroids – dexamethasone, mannitol for raised ICP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Complications of brain tumours?

A
  • Acute haemorrhage
  • Hydrocephalus
  • Increases in ICP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Definition of encephalitis?

A
  • Inflammation of brain parenchyma, often caused by viral infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Risk factors of encephalitis?

A
  • <1 or >65
  • Immunodeficiency
  • Viral infection
  • Animal/Insect bites
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Viral types of encephalitis?

A
  • Acute (caused by direct viral infection)

- Post-infectious (autoimmune process)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Viral causes of encephalitis?

A
  • Herpes Simplex 1&2
  • CMV
  • EBV
  • VZV
  • Measles
  • Mumps
  • Adenovirus
  • Influenza
  • Polio
  • Rubella
  • Rabies
  • HIV – toxoplasmic meningoencephalitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Bacterial causes of encephalitis?

A

 Any cause of bacterial meningitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Fungal causes of encephalitis?

A

 Cryptococcosis, histoplasmosis, candidiasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Parasitic causes of encephalitis?

A

 Trypanosomiasis, toxoplasmosis, schistosomiasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Symptoms of encephalitis?

A
  • Triad:
    • Fever, headache, altered mental status
  • May have confusion, seizures, focal neurological signs
  • Symptoms of raised ICP – headache, vertigo, nausea, convulsions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Investigations of encephalitis?

A
-	Bloods
•	FBC and film
•	U&amp;E
•	LFTs
•	CRP/ESR
•	Viral PCR
•	Malaria film
  • Blood Cultures
  • Throat swab
  • CT Scan
    • Focal bilateral temporal lobe involvement – HSV
    • Before LP
  • Lumbar Puncture
    • Send for cells, protein, glucose, lactate and PCR
    • Viral – lymphocytosis, normal glucose ratio
  • EEG
    • Diffuse slowing with periodic discharges
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What to send for in LP of encephalitis?

A
  • Send for cells, protein, glucose, lactate and PCR

* Viral – lymphocytosis, normal glucose ratio

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Management of encephalitis - initial management?

A
  • Assess using ABCDE and check glucose
  • Immediate LP
    • If contraindications for LP then urgent CT scan
    • Send for opening pressure, CSF and serum glucose, CSF protein, 2x M, C &S, virology PCR, lactate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Management of encephalitis - if CSF findings do not suggest encephalitis?

A

• Repeat LP in 24 hours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Management of encephalitis - if CSF findings suggest encephalitis?

A

• IV Aciclovir (within 30 mins of arrival)
 10mg/kg/8h
- Neonate 20mg/kg
- 3 months – 12 years – 500mg/m2
 14 days if >12 years
 21 days if immunosuppressed or <12 years old
 Adjust dose according to eGFR every 12/24h

  • MRI within 24-48 hours if not already performed
  • ICU or HDU if severe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Management of encephalitis - symptomatic treatment?

A
  • Intubation and ventilation
  • Phenytoin for seizures
  • Sedatives
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Management of encephalitis - if elevated ICP?

A
  • Elevate head of bed to 45 degrees
  • Hyperventilation
  • Corticosteroids
  • Mannitol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Management of encephalitis - specific treatments to CMV, Syphilis, Rocky Mountain fever?

A
  • CMV – ganciclovir + foscarnet
  • Syphilis – Benzylpenicillin
  • Rocky Mountain spotted Fever – Doxycycline
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Clinical contraindications to LP?

A
  • GCS <13 or fall or 3 or more
  • Focal neurological signs (unequal, poorly dilating or responsive pupils)
  • Abnormal posture
  • Papilloedema
  • After seizures until stabilised
  • Relative bradycardia and hypertension
  • Abnormal doll eyes movements
  • Immunocompromise
  • Systemic shock
  • Coagulopathies – platelets <100, anticoagulation therapy
  • Local infection at puncture site
  • Respiratory insufficiency
  • Suspected meningococcal septicaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Pathology of fibromyalgia?

A
  • Chronic pain disorder

o Aberrant peripheral and central pain processing
o Allodynia and hyperaesthesia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Epidemiology of fibromyalgia?

A
  • Women 10x

- Aged 20-50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Risk factors of fibromyalgia?

A
o	Female
o	Middle Aged
o	Low income
o	Divorced
o	Low educational status
o	Psychosocial:
	Sickness behaviours, social withdrawal, emotional problems, problems at work
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Associations of fibromyalgia?

A

o Chronic fatigue syndrome, IBS, chronic headaches, RA/SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Symptoms of fibromyalgia?

A

o Chronic widespread pain
 Multiple sites, low back pain
 Worse in cold and stress

o Morning stiffness

o Fatigue

o Poor concentration

o Low mood

o Sleep disturbances

o Numbness, tingling, cold/heat insensitivity, TMJ dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Investigations to perform in fibromyalgia?

A

o Normal – FBC, TFTs, CRP/ESR, RF, Anti-CCP, ANA

o Other tests all normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Diagnosis made of fibromyalgia when?

A

o >3 months
o Widespread (left and right sides, above and below waist and axial skeleton) with no inflammation
o Pain on palpation (4kg/cm2)of at least 11/18 tender points (left and right):
 Suboccipital muscle insertion
 Anterior aspect of inter-transverse spaces at C5-7
 Midpoint of upper border of trapezius
 Origin of supraspinatus near medial border of scapular spine
 Costochondral junction of 2nd rib
 2cm distal from lateral humeral epicondyle
 Upper outer quadrant of gluteal
 Posterior to greater trochanter
 Knee, at medial fat pad proximal to joint line

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Management of fibromyalgia - general points?

A

o Explain diagnosis and reassure no serious underlying pathology/joint damage
o MDT approach – GP, rheumatologists, psychologist, psychiatrists, physios

52
Q

Management of fibromyalgia - non-pharmacological?

A

o CBT

o Graded-exercise programmes

53
Q

Management of fibromyalgia - drug therapy?

A
o	Paracetamol/Tramadol
o	Low-dose amitriptyline or pregabalin
o	Venlafaxine
o	SNRIs (duloxetine)
54
Q

Diagnostic criteria of chronic fatigue syndrome?

A
  • Persistent disabling fatigue lasting >4 months, affecting mental and physical function, present in >50% of time with 4 or more of:
o	Myalgia
o	Polyarthralgia
o	Poor memory
o	Unrefreshing sleep, insomnia, hypersomnia
o	Fatigue after exertion >24h
o	Persistent sore throat
o	Tender lymph nodes
55
Q

Investigations of chronic fatigue syndrome?

A

o Urinalysis

o Bloods – FBC, LFT, U&E, TFT, ESR, CRP, glucose, coeliac, Ca, CK, ferritin

56
Q

Management of chronic fatigue syndrome - symptoms control?

A

 Nausea – eating little and often, sipping fluids, anti-emetic if severe
 Pain – paracetamol
 Sleep - Sleep hygiene advice, rest periods during day, relaxation techniques

57
Q

Management of chronic fatigue syndrome - when to refer?

A
o	Refer to specialist within 6 months if mild, 4 months if moderatre and immediately if severe
	Graded-exercise programme and,
	CBT
	Activity Management
	Amitriptyline if poor sleep or pain
58
Q

Definition of hydrocephalus?

A

o Increase in volume of CSF occupying cerebral ventricles
 Usually due to reduced absorption or excessive secretion
o Ventricle dilatation and CSF permeates through ependymal lining in periventricular white matter – white matter damage and gliotic scarring

59
Q

Classification of hydrocephalus?

A

o Non-communicating – flow of CSF is obstructed within ventricles or between ventricles and subarachnoid space

o Communicating – communication between ventricles and subarachnoid space and problem lies outside of ventricular system (reduced absorption or blockage of venous drainage)

60
Q

CSF physiology of production and flow?

A

o Produced at rate of 500ml per day in adults, by choroid plexus of lateral, third and fourth ventricles
o Flow is caudal through ventricles and into subarachnoid space through the foramina of Luschka and Magendie
o Passes through tentorial hiatus and over hemispheric convexity where it is absorbed into venous system through arachnoid granulations

61
Q

Risk factors for congenital hydrocephalus?

A

o Pre-Eclampsia
o Hypertension during pregnancy
o Alcohol use during pregnancy

62
Q

Causes of hydrocephalus - non communicating?

A

 Congenital (aqueduct stenosis, atresia of outflow foramina (Dandy-Walker), Chiari malformation)
 Posterior fossa neoplasm
 Intraventricular haemorrhage

63
Q

Causes of hydrocephalus -communicating?

A

 SAH
 Meningitis
 Choroid plexus papilloma

64
Q

Causes of hydrocephalus - others in adults?

A

 Idiopathic (1/3)

 Normal pressure hydrocephalus

65
Q

Symptoms of hydrocephalus - in infants?

A

o Rapid increase in head circumference
o Dysjunction of sutures, dilated scalp veins and tense fontanelle
o Setting-sun sign (fixed downward gaze, upper lids retracted and sclera visible above iris)
o Macewen’s sign (cracked pot soung on percussion of head)
o Increased limb tone

66
Q

Symptoms of hydrocephalus - in adults - acute?

A

 Headache
 Nausea and Vomiting
 Papilloedema or impaired upward gaze

67
Q

Symptoms of hydrocephalus - in adults - gradual onset?

A
	Unsteady gait
	Large head
	Blurred/double vision
	Cognitive decline
	Incontinence
68
Q

Investigations in hydrocephalus - antenatally?

A

US scan

69
Q

Investigations in hydrocephalus - infants?

A

USS +/- CT

70
Q

Investigations in hydrocephalus - adults?

A

CT
o If dilated lateral + 3rd ventricle:
 With normal 4th ventricle – aqueduct stenosis
 With abnormal 4th ventricle – posterior fossa mass

71
Q

Management of hydrocephalus - if acute deterioration and communicating hydrocephalus?

A

Lumbar puncture

72
Q

Management of hydrocephalus - medical management?

A
o	Furosemide (inhibits CSF secretion)
o	Isosorbide (promotes reabsorption)
73
Q

Management of hydrocephalus - surgical management?

A

o External ventricular drain (temporary)
o Insertion of shunt (majority of cases, ventricular catheter drains CSF through reservoir down to right atrium of heart (ventriculoatrial shunt) or peritoneal cavity (ventriculoperitoneal shunt))
o Other – Choroid plexectomy, endoscopic cerebral aqueductoplasty

74
Q

Complications of hydrocephalus?

A
  • Often fatal if untreated
  • Epilepsy
  • Learning difficulties
  • Of shunt surgery – infection, subdural haematoma, shunt obstruction
75
Q

Definition of normal pressure hydrocephalus?

A
  • Form of communicating hydrocephalus with ventricular dilatation in absence of raised CSF pressure on LP
  • Characterised by triad of gait abnormality, urinary incontinence and dementia
76
Q

Aetiology of normal pressure hydrocephalus?

A
o	50% idiopathic
o	SAH
o	Meningitis
o	Head injury
o	CNS tumour
77
Q

Symptoms of normal pressure hydrocephalus?

A

o Gait disturbance (distortion of corona radiata by dilated ventricles)
 Slow, broad-based and shuffling gait OR gluing-to-floor on attempting to walk
 Gait apraxia

o Sphincter Disturbance (sacral nerve supply)
 Urinary incontinence (+/- bowel incontinence)

o	Dementia (distortion of periventricular system)
	Memory loss, inattention, inertia, bradyphrenia
78
Q

Signs of normal pressure hydrocephalus?

A

o Pyramidal tract signs
o Reflexes brisk
o NO PAPILLOEDEMA

79
Q

Investigations of normal pressure hydrocephalus - initial?

A

o MRI/CT Scan – ventricular enlargement

o Lumbar Puncture (5-18mmHg) – CSF pressure normal or mildly elevated

80
Q

Investigations of normal pressure hydrocephalus - if PArkinson’s suspected?

A

o Levodopa Challenge – if suspected Parkinsons’ disease – if no response then NPH possible

81
Q

Investigations of normal pressure hydrocephalus - further testing?

A

o Intraventricular pressure monitoring

o Lumbar infusion test (Intrathecal infusion test) – abnormal sustained rise in CSF suggest NPH

82
Q

Diagnosis of normal pressure hydrocephalus made when?

A

o Clinical triad
o No papilloedema
o No evidence of raised ICP

83
Q

Management of normal pressure hydrocephalus - general measures?

A

o Control blood pressure
o Smoking cessation
o Statin

84
Q

Management of normal pressure hydrocephalus - surgical management?

A
  • Surgery (1st line)

o CSF shunt – to RA or peritoneum

85
Q

Management of normal pressure hydrocephalus - medical management?

A
  • Medical (if surgery unsuitable)
    o Acetazolamide (carbonic anhydrase inhibitors)
    o Repeated lumbar punctures
86
Q

Definition of myasthenia gravis?

A
  • IgG antibody-mediated autoimmune disease to nicotinic acetylcholine receptors, interfering with neuromuscular transmission via depletion of post-synaptic receptor sites
  • Patient present with muscle weakness, worsens with continued activity and improves on rest
87
Q

Physiology of normal neuromuscular junction?

A

• When action potential arrives at pre-synaptic terminal, depolarisation opens voltage-gated Ca channel (In Lambert-Eaton – this is disrupted)
• Influx of Ca through VGCCs triggers fusion of synaptic vesicles with pre-synaptic membrane (botulinum interferes with this), and neurotransmitter released into cleft
• Transmitters cross by diffusion and bind to receptors on post-synaptic membranes causing depolarisation
• Triggers onward transmission of action potential or muscle contraction at NMJ
 In myasthenia gravis, antibodies block post-synaptic Ach receptors, preventing end-plate potential from becoming large enough
• Termination occurs by acetylcholinesterase degradation, uptake into pre-synaptic membrane or glial cell, or diffusion away
• Anticholinesterase treatments (pyridostigmine) reduce rate of degradation of Ach increasing chance of end-plate potential

88
Q

Epidemiology of myasthenia gravis?

Most common in people under and over 50?

A
  • Peak incidence in 30s women, 60s men
  • <50 – commoner in women
    • Associated with thymic hyperplasia
  • > 50 – commoner in men
    • Association with thymic atrophy/tumour, RA, SLE
89
Q

Aetiology of myasthenia gravis?

A

• IgG1-dominant antibodies to Ach receptors cause weakness of skeletal muscles

90
Q

Symptoms of myasthenia gravis?

A

• Increasing muscular fatigue
 Order of muscle groups – extra-ocular, bulbar (swallowing, chewing), face, neck, limb girdle, trunk
 Worsened by pregnancy, low K, infection, over-treatment, change of climate, emotions, exercise, drugs (opiates, gentamicin, tetracyclines, quinine, B-blockers)
• Slurred speech
• Eating/chewing problems
• Difficulty walking/sitting

91
Q

Signs of myasthenia gravis?

A
  • Diplopia
  • Ptosis
  • Myasthenic snarl (smile)
  • Peek sign
  • On counting to 50 – voice fades
92
Q

Examination of myasthenia gravis?

A
  • No muscle wasting/fasciculations
  • Tone, sensation and tendon reflexes normal
  • Upward gaze – starts to lower after a while
93
Q

Classification criteria of myasthenia gravis?

A

• Class 1 – any eye muscle weakness, ptosis, all other muscle strength normal
• Class 2 – Mild weakness of other muscles, +/- eye muscle weakness
 A – predominantly limb or axial muscles or both
 B – predominantly oropharyngeal/respiratory muscles
• Class 3 – moderate weakness of other muscles, +/- eye muscle weakness
 A – predominantly limb or axial muscles or both
 B – predominantly oropharyngeal/respiratory muscles
• Class 4 – severe weakness, +/- eye muscle weakness
 A – predominantly limb or axial muscles or both
 B – predominantly oropharyngeal/respiratory muscles
• Class 5 – Intubation needed to maintain airway

94
Q

Investigations to perform in myasthenia gravis - bloods?

A
  • Anti Ach-R antibodies
  • If seronegative – look for anti-MUSK & anti-LRP4 antibodies
  • TFTs
95
Q

Investigations to perform in myasthenia gravis - neurophysiology?

A
  • Decremental muscle response to repetitive nerve stimulation
  • If negative – single-fibre electromyography
96
Q

Investigations to perform in myasthenia gravis - imaging?

A
  • CT thymus

* MRI brain – if seronegative and negative electrophysiology but compatible symptoms

97
Q

Investigations to perform in myasthenia gravis - others?

A
  • Used less now, Edrophonium (Tensilon test)
    • IV short-acting acetylcholinesterase inhibitor and watch improvement of muscle strength
    • Rarely done as can results in bradycardia
98
Q

Management of myasthenia gravis - symptoms control?

A

Anticholinesterase inhibitor (Pyridostigmine 30-120mg PO up to 6x daily, max 1.2g/day)

99
Q

Management of myasthenia gravis - immunosuppression?

A

Prednisolone
 Regimens:
- 10mg on alternate days, increase by 10mg/week up to 1-1.5mg/kg on treatment day
- 5mg daily, increased in steps of 5mg daily, maintenance 60-80mg (0.75-1mg/kg)
- Lower dose (10-40mg) if ocular myasthenia only
 Give bisphosphonate prophylaxis

Azathioprine

Methotrexate

100
Q

Management of myasthenia gravis - surgical?

A

• Thymectomy (if <50 or thymomas on CT)

101
Q

Complications of myasthenia gravis?

A

Aspiration pneumonia

Myasthenic crisis

102
Q

Definition and triggers of myasthenic crisis?

A

• Definition
 Emergency where worsening muscle weakness results in respiratory failure

• Triggers
 Infection, post-surgery, medications (antibiotics, beta blockers, quinidine, verapamil, atracurium, vecuronium, lithium, opiates, phenytoin, statins, steroids)

103
Q

Investigations and management of myasthnic crisis?

A

• Investigations
 Monitor arterial O2 sats & force vital capacity

• Management
 Intubation & Ventilation
 Immunoglobulins, steroids or Plasmaphoresis

104
Q

Definition of motor neurone disease?

A
  • Cluster of major degenerative diseases characterised by selective loss of neurons in motor cortex, cranial nerve nuclei and anterior horn cells
  • Upper and lower motor neurones affected
105
Q

How to distinguish MND from other neurological conditions?

A
  • MS as never affected sensory nerves

* Myasthenia as never affects eye movements

106
Q

Epidemiology of motor neurone disease?

A
  • 50% ALS
  • Women more
  • Mean onset 60 years
  • Often fatal in 2-4 years
107
Q

Risk factors of motor neurone disease?

A

• ALS-FTD locus on 9p21

108
Q

When to suspect MND?

A
  • Think MND in over 40, stumbling spastic gait, foot drop and proximal myopathy, weak grip
109
Q

Types of motor neurone disease - Amyotrophic lateral sclerosis?

A
  • Loss of motor neurons in motor cortex and anterior horn of cord
  • Weakness and UMN signs (upgoing plantars)
  • LMN wasting and fasciculations
  • Thenar wasting
110
Q

Types of motor neurone disease - Progressive bulbar palsy?

A
  • Only affects cranial nerves 9-12

- LMN lesion of tongue and muscles of talking/swallowing, flaccid, fasciculating tongue

111
Q

Types of motor neurone disease - progressive muscular atrophy?

A
  • Anterior horn cells only thus no UMN signs

- Distal muscle groups before proximal

112
Q

Types of motor neurone disease - primary lateral sclerosis?

A
  • Loss of Betz cells in motor cortex thus mainly UMN signs and marked spastic leg weakness
113
Q

Signs in motor neurone disease?

A

UMN Signs
- Spasticity, brisk reflexes, increased plantars
LMN Signs
- Wasting, weakness, fasciculations, speech and swallowing affected

114
Q

Diagnostic criteria of motor neurone disease?

A

El Escorial Diagnostic Criteria for ALS
- Definite
• LMN and UMN signs in 3 regions

  • Probable
    • LMN and UMN signs in 2 regions
  • Probably with lab support
    • LMN and UMN signs in 1 region and EMG shows acute denervation in >2 limbs
  • Possible
    • LMN and UMN signs in 1 region
  • Suspected
    • LMN OR UMN signs only in 1 or more region
115
Q

Imaging performed in motor neurone disease?

A
  • Brain/Cord MRI
  • LP
  • Neurophysiology
116
Q

How to manage motor neurone disease?

A
  • Referral to neurologist

- MDT approach – doctor, palliative nurse, hospice, physio, OT, SALT, dietician

117
Q

Management of motor neurone disease - drug therapy?

A

• Antiglutaminergic (riluzole) – prolongs life by 3 months in ALS

118
Q

Management of motor neurone disease - symptoms control - muscle problems?

A

 Exercise programme
 Quinine for muscle cramps/stiffness/spasticity
 Baclofen, dantrolene, gabapentin alternatives

119
Q

Management of motor neurone disease - symptoms control - nutrition?

A

 Assess early and refer appropriately if needed

 Gastrostomy

120
Q

Management of motor neurone disease - symptoms control - psychological support?

A

• Psychological support with counselling and psychological assessment

121
Q

Management of motor neurone disease - symptoms control - saliva problems?

A

 Propantheline/amitriptyline – stops drooling

122
Q

Management of motor neurone disease - symptoms control - Speech and communication?

A

 Provide alternative communication equipment a patient needs (alphabet, word or picture board and PC/tablet based voice output aids)

123
Q

Management of motor neurone disease - symptoms control - respiratory symptoms - assessment and lung function tests?

A

 Assessment of function

  • Symptoms – SOB, orthopnoea, recurrent infections, disturbed sleep, nightmares, daytime sleepiness, poor concentration, morning headaches, fatigue
  • Signs – increased RR, shallow breathing, weak cough/sniff, use of accessory muscles

 Baseline Lung function tests and repeat every 2-3 months

  • SpO2
  • Forced vital capacity
  • ABG/CBG if SpO2 <92% with lung disease or <94% without lung disease
124
Q

Management of motor neurone disease - symptoms control - respiratory symptoms - cough, SOB and ventilation?

A

 If sleep related symptoms – refer to respiratory ventilation service for nocturnal oximetry

 Ineffective Cough
- Manual assisted cough or unassisted breath stacking

 Breathlessness

  • Opioids
  • Benzodiazepines if anxiety related

 Non-Invasive Ventilation

  • When respiratory impairment if likely to benefit
  • Need risk assessment and care plan
  • Perform during day at start then regular treatment during night and build up hours
125
Q

Management of motor neurone disease - general advice?

A
  • End of life care
  • Advanced decisions
  • LPAs