Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

(C) - Y4 Neurology and Special Senses > 4 - Common Neurological Disorders 1 > Flashcards

4 - Common Neurological Disorders 1 Flashcards

1
Q

What is the definition of epilepsy?

A

Recurrent tendency to seizures. A seizure is an episode of transient abnormal electrical activity in the brain

Synchronus hyperexcited neuronal activity

Need 2 or more unprovoked seizures for diagnosis

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

What are some features in a history that might suggest a black out was a seizure?

A

- Prodrome: change in behaviour/mood days or hours before

- Aura: such as deja vu, strange smells

- Post ictal confusion: may also have headache, confusion

- Tongue biting

- Loss of continence

- Todd’s Palsy: temporary paralysis after

- Dysphasia

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

What are some of the causes of epilepsy? (6 main categories)

A

- Idiopathic

- Genetic predisposition

- Structural: cortical scarring from trauma, SOL, congenital cortical dysgenesis

- Metabolic:

- Immune: SLE, sarcoidosis

- Infectious: chronic infection predisposing to seizures (e.g. HIV). Different to seizures associated with an acute infection (e.g. meningitis)

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

What is the pathophysiology of epilepsy?

A
  • Acquired or inherited imbalance between inhibitory (i.e. gabanergic) and excitatory (i.e. glutamatergic) signals
  • High frequency bursts of excitatory action potentials leads to synchronous, hyperexcitable activity

- Transformation within neural networks that promote excitability and development of epilepsy

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

What are some factors that increase a persons risk of developing epilepsy?

A
  • Cerebrovascular disease
  • Head trauma
  • Cerebral infections
  • Family history: epilepsy or neurological illness
  • Premature birth
  • Congenital malformations of the brain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the three different ways of classifying epilsepy?

A

- Seizure type

- Epilepsy type (see image)

- Epilepsy syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How do you classify a seizure type using the International League against epilepsy classification?

A

1. Focal, Generalised or Focal to Bilateral Tonic Clonic (a.k.a Secondary Generalised)

2. Impaired awareness or Normal Awareness (Complex or Partial)

3. Motor or Non Motor: absence, tonic-clonic, myoclonic, atonic, spasms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the difference between a focal and generalised seizure?

A

Focal: Starts in one hemisphere of the brain, can then spread and lead to secondary generalised. Not all leads on the EEG will start at the same time

Generalised: Affects both hemispheres of the brain at the same time, always impaired awareness. All leads on the EEG start at the same time. Preferred neural pathways

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

What are some examples of epilepsy syndromes and how are they diagnosed?

A

Diagnosed based on age of onset, seizure types, EEG features, additional clinical or radiological features. Need to know to help guide treatment

- Idiopathic generalised epilepsy: myoclonus, generalised tonic-clonic, absence

- West syndrome: infantile spasms aged 3-12 months, hypsarrhythmia on EEG

- Lennox Gastaut syndrome: tonic and absence seizures usually around 6-7 years old, slow development, treatment resistant

- Juvenile Myoclonic Epilepsy: in teens myoclonic or tonic clonic seizures often on waking, no developmental issues, genetic

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

What are some triggers of seizures in juvenile myoclonic epilepsy?

A
  • Lack of sleep
  • Alcohol
  • Flashing lights (photosensitive)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the four stages of a seizure?

A

- Prodromal: change in mood or sensation e.g confusion, irritability, mood disturbances

- Early ictal: Aura, will only get in focal epilepsy, happens few seconds before and could a smell or a vision

- Ictal: depends on type of seizure e.g could be period of stiffness then rhythmic jerking

- Post-Ictal: confusion, drowsiness, memory loss, malaise that can take hours or days to recover from

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

What criteria needs to be met in order to have a diagnosis of epilepsy and what are some differential diagnoses for epilepsy?

A

- Syncope and anoxic seizures: LOC to impaired cerebral blood flow

- Pseudoseizures

- Sleep-related conditions

- Paroxysmal movement disorders

- Migraine associated disorders

These seizures appear the same as epileptic seizures but there is no investigational evidence for them e.g no trace on EEG, no raised lactate or prolactin after seizure

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

How can you tell the difference between a pseudoseizure (non-epileptic attack disorder) and an epileptic seizure?

A
  • No clinical evidence for a pseudoseizure e.g normal CT, MRI and EEG
  • Pseduoseizures may close eyes, periods of motionless unresponsiveness, rapid breathing, abrupt termination, lack of post-ictal phase, head side to side
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What investigations are done following a first seizure to help aid the clinical diagnosis of epilepsy?

A

First seizure needs urgent referral to first fit clinic within 2 weeks.

Investigations done to rule out any precipitating cause of seizure

- EEG: see if focal cause, cannot be used to exclude epilepsy

- MRI: structural lesions that could be causing epilepsy, do CT if MRI not available

- ECG

- Bloods: FBC, U&E, LFT, Glucose, Bone profile

- Drug screen

- LP: if suspect infection

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

What are the aspects of management in epilepsy?

A

- Education and safetynetting

- Acute control of seizures

- Long term prevention of seizures (aiming for no seizures and no/few side effects)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What counselling do you need to give a patient after any fit?

A

- Need to stop driving and inform DVLA (if first seizure no driving for 6/12, if epilepsy need to be seizure free for a year)

- Watersafety: take showers not baths, buddy system, leave door unlocked, avoid swimming

- Environment: avoid heights, avoid flames, avoid dangerous activities

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

What happens at a first fit clinic?

A

- History, Exam, MRI, EEG

  • All done to decide whether seizure is likely to represent epilepsy

- Patient education and advice

  • If 2 or more seizures or 1 seizure and high risk of another, AEDs started
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the features of the following seizures and what AEDs are used to treat them?

  • Focal
  • Generalised tonic clonic
  • Absence
  • Myoclonic
  • Tonic
  • Atonic
  • Juvenile myoclonic epilepsy:
A

Focal:

1st line - Levetiracetam or lamotrigine

2nd line - Carbamazepine

Generalised tonic clonic:

1st line - sodium valproate or lamotrigine

2nd line - clobazam, lamotrigine, levetiracetam or topiramate

Absence

1st line - ethosuximide or sodium valproate

2nd line - lamotrigine

Myoclonic

1st line - sodium valoproate

2nd line - levetiracetam or topiramate

Tonic

Sodium valproate or Lamotrigine

Atonic

Sodium valproate or Lamotrigine

Juvenile myoclonic epilepsy

1st line - sodium valproate

2nd line - lamotrigine , levetiracetam or topiramate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What antiepileptics are safest to use in pregnancy?

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

What are the complications of epilepsy?

A
  • Trauma, drowning, RTAs from actual seizure
  • Status epilepticus
  • Sudden unexpected death in epilepsy (SUDEP)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How are AEDs started, stopped and switched?

A

Start: treat with one drug and one doctor only and slowly build up until seizures controlled or maximum dose reached

Switch: Titrate new drug up and titrate old drug down

Stop: Can trial under specialist supeervision if seizure free >2 years after weighing up risks and benefits. Must decreased dose over at least 2-3 months

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

What advice do you need to give a woman with epilepsy on pregnancy?

A
  • Advise all women of child-bearing age to take folic acid daily

- Avoid sodium valproate and polytherapy completely

  • Most AEDs in breast milk apart from carbamazepine, valproate. Lamotrigine is in milk but not harmful

- Enzyme inducing AEDs make POP unreliable. Oestrogen containing contraceptives lower lamotrigine levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Apart from AEDs, what other interventions can be used in epilepsy?

A

Psychological: relaxation, CBT may benefit some

Surgical:

- Neurosurgical resection: if single epileptogenic focus e.g tumour but risk of neurological deficits

- Vagal nerve stimulation

- Deep brain stimulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How can you localise a focal seizure?

A

Temporal Lobe

  • Automatisms (lip smacking, chewing, fiddling, grabbing)
  • Dysphasia
  • Deja-vu
  • Emotional disturbance
  • Hallucinations of smell, taste, sound

Frontal Lobe

  • Motor features like Jacksonian March
  • Subtle behavioural disturbances
  • Speech arrest

Parietal Lobe

  • Sensory disturbances e.g numbness, pain

Occipital Lobe

  • Visual phenomena like spots, lines, flashes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What is the mechanism of action of the following AEDs and what are some side effects are each of them? - Sodium Valproate - Carbamazepine - Lamotrigine - Levetiracetam - Phenytoin - Phenobarbitone - Topiramate
NEED TO MONITOR FOR DEPRESSION AND SUICIDAL THOUGHTS ON ALL AEDs
26
What are some symptoms of phenytoin toxicity?
- Nystagmus - Diplopia - Tremor - Dysarthria - Ataxia
27
What is Idiopathic Parkinson's disease?
***Chronic progressive neurodegenerative disease*** that occurs due to a ***loss of dopaminergic neurones*** in the ***substantia nigra*** Most common cause of ***Parkinsonism*** (see image) Starts unilateral then as it progresses becomes bilateral. Has non-motor symptoms too e.g depression, sleep disturbance, autonomic dysfunction
28
What is Parkinsonism?
Bradykinesia plus of one: - Resting tremor - Postural instability - Rigidity
29
What is the pathophysiology of Idiopathic Parkinson's disease?
Loss of dopaminergic neurones in the substantia nigra so less dopamine in the direct and indirect pathway. **Basal ganglia functions:** - Inhibition of muscle tone - Coordinated, slow, sustained movement - Suppression of useless patterns of movement - Initiation of movement
30
What are the clinical features of IPD?
**- Bradykinesia** (slowing of voluntary movements, loss of arm swing) **- Resting 'pill-rolling' tremor** of 4-6Hz **- Cog Wheel rigidity** **- Shuffling gait** **- Micrographia** **- Parkinsonian mask** **- Non motor:** sleep disorder, bowel/bladder symptoms, sexual dysfunction, glabellar tap, depression, psychotic symptoms, anosmia
31
How is a diagnosis of IPD made?
Refer to neurologist! **UK Parkinson's Disease Society (PDS) Brain Bank Clinical Diagnosis Criteria** **_Step 1_** Bradykinesia plus one of: resting tremor, postural instability, muscle rigidity **_Step 2_** Exclusion of other causes of Parkinsonism e.g review drug chart **Step 3** 3 or more supportive criteria: - Unilateral onset - Rest tremor present - Progressive - Persistent asymmetry - Excellent response to levodopa - Levodopa induce chorea - Levodopa response for 5 years or more - Clinical course of 10 years or more
32
What are some exclusion criteria for IPD?
- Repeated strokes and stepwise progression - History of head trauma - \> 1 relative affected - Sustained remission - Unilateral features after 3 years - Antipsychotic or dopamine-depleting drugs - Negative response to levodopa - Oculogyric crisis - Exposure to neurotoxin - Cerebral tumour or hydrocephalus
33
What is the MDS (movement disorder society) criteria for diagnosing IPD?
**- Confirmation of Parkinsonism:** bradykinesia + tremor or rigidity **- No absolute exclusion criteria present** **- ≥2 supportive criteria** **- Absence of red flags:** rapid development gait impairment, early bulbar dysfunction, non-progressive motor symptoms ≥ 5 years while not on treatment, respiratory dysfunction, early severe autonomic dysfunction
34
What are some causes of Parkinsonism?
- IPD - Multisystem Atrophy - Progressive Supranuclear Palsy - Dementia with Lewy Body - Corticobasal degeneration - Vascular - Drug induced - Post encephalitis - Neurosyphillis
35
How do the following Parkinson's plus syndromes present? - MSA - PSP - DLB - CBD
**_MSA_** - Adult-onset rapidly progressive disease with parkinsonism +profound autonomic dysfunction leading to severe postural hypotension, urogenital dysfunction, cerebellar and corticospinal features - Poor response to treatment **_PSP_** - Begins at age 50-60 years with vertical gaze dysfunction (strugle looking down and up), postural instability (stiff broad based gait), dysarthria and cognitive decline, parkinsonism, cognitive impairment - Tremor is rare, **_DLB_** - Early onset dementia (\< 1 year) with features of parkinsonism - Dementia prior to motor symptoms and has visual hallucination and fluctuating consciousness - REM sleep disorder **_CBD_** - Progressive dementia, parkinsonism and limb apraxia - Alien limb syndrome
36
What are some drugs that can induce Parkinsonism?
- Neuroleptics e.g Risperidone, Olanzapine, Clozapine - Metoclopramide - Prochlorperazine - Manganese
37
What investigations can be done when a patient has Parkinsonism to find if it is IPD or there is an underlying cause?
**- CT/ MRI:** can look for secondary cause **- PET with Fluorodopa:** to localise dopamine deficiency in basal ganglia **- DaTscan:** differentiate Parkinsonism from essential tremor **- Always review drug chart**
38
How can you distinguish between a Parkinson's tremor and a Benign Essential tremor?
39
How can you make a resting tremor more visible in IPD?
Unilateral so distract their other hand by asking them to mime painting a fence or something
40
What are the principles of management in Parkinson's disease?
**- Initial Therapy** **- Adjuvant Therapy** **- Surgery** **- Non motor symptoms:** e.g laxatives for constipation, catheter for bladder dysfunction, modafinil for excessive tiredness
41
Why should you not withdraw medication suddenly in Parkinson's disease?
Risk of: - Akinesia - Neuroleptic malignant syndrome
42
Why do you not give a young patient with PD Levodopa initially?
- Side effects gets worse the longer it is used e.g dyskinesia - Larger and more frequent dosing needed the more you use it - Unpredictable freezing and end-of-dose reduced response the more it is used - Try to delay use of Levodopa by using MAO-B Inhibitors and DA
43
How do you treat drug-induced dyskinesia in IPD?
- Alter dose of levodopa to extended release - Give **Amantadine** (Dopamine agonist)
44
What are the main medications used to initially treat Parkinson's disease and what are the side effects of these treatments?
**_Levodopa:_** - Given as ***Co-careldopa*** or ***Co-beneldopa*** (Dopa De-carboxylase inhibitors) **- SE:** Psychosis, Visual hallucinations, Dyskinesia, Dystonia, Vomiting (give domperidone) **_Dopamine Agonists (younger patients as less SE):_** ***- Ropinorole*** and ***Pramipezole*** transdermal **- SE:** drowsiness, hallucinations, impulse control disorders (gambling, hypersexualitity) - Ergot derived agonists e.g bromocriptine, not favoured as risk of *PULMONARY FIBROSIS* **_MAO-B Inhibitors_** - MAO-B involved in breaking down dopamine ***- Rasagiline*** and ***Selegiline*** - **SE**: postural hypotension, AF
45
What is some adjuvant therapy used in PD?
**_COMT Inhibitors_** ***- Entacapone*** - Stop the breakdown of levodopa in peripheries so more gets into BBB - Adjuvant to levodopa and help with end-of-dose response **_Amantadine_** - Dopamine agonist that can help with dyskinesia **_Apomorphine_** - Non selective dopamine agonist - Used SC for freezing episodes or for end-of-dose effects - Postural hypotension
46
What surgical managament options are available for IPD?
**- Thalamic or Subthalamic surgery** **- Deep brain stimulation:** Electrodes placed in basal ganglia. Can reverse akinesia, tremor and rigidity but can cause confused and intracerebral haemorraghes
47
What are some complications with Parkinson's disease?
Motor and Non-Motor
48
Levodopa can cause dyskinesias. What is the meaning of the following dykinesias: - Chorea - Dystonia - Athetosis
**Dystonia:** excessive muscle contraction leads to abnormal postures or exaggerated movements. **Chorea:** These are abnormal involuntary movements that can be jerking and random. **Athetosis:** Involuntary twisting or writhing movements usually in the fingers, hands or feet.
49
How can IPD be staged?
**Hoehn and Yahr scale** or **Unified Parkinson's Disease Rating Scale** Response to treatment gets worse as stages go on. Only treat with Levodopa when seriously affecting their life. Could start with dopamine agonists
50
What is vascular parkinsonism and how does it present?
***Symmetrical lower-body*** parkinsonism with ***gait unsteadiness*** and ***absence of tremor***s and is usually associated with pyramidal signs Patient may be diabetic or hypertensive Treat by controlling stroke risk
51
What is Motor Neurone Disease and how can it be distinguished from MS, Polyneuropathies and MG?
Group of neurodegenerative diseases characterised by **loss of motor neurones** in **motor cortex, cranial nerve nuclei and anterior horn cells.** Mixed UMN and LMN signs **_No sensory loss or sphincter disturbance_** like in MS and polyneuropathies. **_Never affects eye movements_** but does in MG
52
What are the four patterns of MND?
NEVER SENSORY SIGNS! **_Amylotrophic Lateral Sclerosis (ALS - 80%)_** - Loss of motor neurones in motor cortex and anterior horn of cord so mixed UMN/LMN signs - Worse prognosis if bulbar onset, increased age, decreased FVC **_Progressive Bulbar Palsy (10%)_** - Only loss of cranial nerves IX-XII - Bulbar and Corticobulbar (Pseudobulbar) palsy **_Progressive Muscular Atrophy_** - Anterior horn cell lesion so LMN signs only - Affects distal to proximal muscle groups - Better prognosis than ALS **_Primary Lateral Sclerosis_** - Loss of Betz cells in motor cortex so usually UMN sgns - Spastic leg weakness and pseudobulbar palsy - No cognitive decline
53
What presentation would make you consider MND?
**- \>40 with stumbling spastic gait** **- Foot drop** **- Proximal myopathy** **- Weak grip and shoulder abduction** **- Aspiration pneumonia** **- LMN signs**: wasting, fasiculation, weakness, hyporeflexia **- UMN signs:** spasticity, brisk reflexes, babinski, clonus, weakness **- Bulbar signs:** speech or swallowing
54
What is the aetiology and epidemiology of ALS?
- Usually men 50-60 years old **- Sporadic (90%)** **- Familial (10%):** * C9orf72 expansion. Excess of the hexanucleotide repeat, GGGGC \>30. * Men \> Women in genetic cases * Autosomal dominant
55
What is the average survival with MND?
3-5 years after onset Starts as asymmetrical limb weakness, eventually leads to respiratory muscles being affected so respiratory failure **Respiatory failure or Aspiration Pneumonia is leading cause of death!**
56
How does ALS typically present?
**_First symptoms:_** **- Asymmetrical limb onset:** hand weakness and loss of dexterity or foot drop and loss of balance running **- Bulbar symptoms:** dysarthria, dysphagia/choking/aspiration **_Other symptoms:_** **- Frontotemporal dementia** **- LMN and UMN signs**
57
What investigations are done if you suspect MND?
Clinical diagnosis but Ix can rule out other things **- Electromyograph (EMG):** look for chronic/acute dennervation and fasiculations **- NCS:** motor conduction altered, sensory conduction fine **- Genetic testing**: C9orf72 and SOD1 **- Bloods:** blood borne viruses, vit B12, folate, CK **- Antibodies (rule out others):** AchRA, VGCC, Anti-GM-1 **- T2 Weighted MRI:** will see enhancment of corticospinal tracts in MND **- LP:** look for infective causes
58
How is MND diagnosed?
**Clinical: Revised El Escorial diagnostic criteria for ALS**
59
How is ALS managed?
MDT (neurologist, palliative nurse, physio, OT, speech therapist, dietician) **_Medical:_** ***Riluzole*** (inhibitor of glutamate release) is the only drug that can slightly improve survival by few months **_Dietician:_** Blend food, Gastrotomy to prevent aspiration **_Ventilation:_** Discuss NIV at night and eventual mechanical ventilation **_End of Life Planning:_** discuss early, see if can get an advanced care direction or LPA **_Help with communication needs_**
60
What is bulbar palsy and what are the signs and casues of this?
**LMN lesion** of the nuclei of CN IX-XII in the medulla. - Flaccid fasiculating tongue - Jaw jerk normal or absent - Quiet, hoarse or nasal speech **_Causes:_** MND, GBS, MG, syringobulbia, brainstem tumours, central pontine myelinolysis
61
What is Pseudobulbar palsy and what are the signs and causes of this?
UMN palsy affecting the corticobulbar tracts of the same nerves as a bulbar palsy UMN lesion due to bilateral lesions above the mid-pons **- Slow tongue movements** **- Slow speech** **- Increased jaw jerk** **- Increased pharyngeal and palatal reflexes** **- Pseudobulbar affect:** crying or laughing unprovoked **_Causes:_** MS, MND, Stroke, Central pontine myelinolysis
62
What are the differences between bulbar and pseudobulbar palsy?
63
What is Guillain-Barre Syndrome?
**Acute inflammatory demyelinating polyneuropathy** that usually is **symmetrically** ascending. **Ascending muscle weakness from lower limbs** and reduced reflexes Usually preceded by illness e.g gastroenteritis or flu
64
What are some triggers for GBS?
- Campylobacter jejuni (Gram -ve rod) - Mycoplasma - CMV - HIV - EBV - Vaccinations - UNKNOWN
65
What is the pathophysiology of GBS?
Trigger causes formation of antibodies which cross react and attack myelin sheath (AIDP) or axons (AMAN,AMSAN) **_Variants:_** **- Acute inflammatory demyelinating polyneuropathy** (AIDP) (90%) **- Acute motor axonal neuropathy** (AMAN) **- Acute motor and sensory axonal neuropathy** (AMSAN) **- Miller Fisher Syndrome** (MFS)
66
How does AIDP, most common form of GBS, present?
PRODROME OF ILLNESS 4 WEEKS BEFORE: **- Symmetrical ascending weakness** (starting at the feet) **- Reduced reflexes** - May be **peripheral loss of sensation** or **neuropathic pain** - May progress to the cranial nerves and cause **facial nerve weakness** **- Autonomic neuropathy:** increased BP, sweating
67
What is the timeline of symptoms in GBS?
- Symptoms within 4 weeks of preceding infection - Symptoms peak after 2-4 weeks (can lead to paralysis) - Recovery period starts 2 weeks after symptom peak
68
What investigations should you do if you suspect GBS and what will they show?
Brighton Criteria ## Footnote **- NCS and EMG:** reduced signal through the nerves **- Lumbar puncture for CSF:** raised protein (\>5.5) with a normal WCC and glucose **- Spirometry (FVC):** monitor disease progression and respiratory muscle involvement **- Antibodies:** Anti-GQ1b, specific for Miller Fisher syndrome - May also do CXR (BHL for sarcoidosis) and MRI spine to rule out other pathologies
69
How is GBS managed?
**_Definitive_** **- IV Immunoglobulin** for 5 days **- Plasma Exchange** **_Supportive_** **- Intubation** if resp failure **- Cardiovascular monitoring** as may have labile BP or arrhythmias due to autonomic dysfunction. Telemetry **- DVT prophylaxis** with stockings and LMWH **- Analgesia** like gabapentin for neuropathic pain
70
How do you monitor for respiratory involvement in GBS and how do you act on this if there is involvement?
- Do **FVC every 4 hours** **- Transfer to ITU** if respiratory involvement **- Ventilate if FVC\<1.5L, PaO2\<10kPa, PaCO2\>6kPa**
71
How do you score someones functional level after GBS?
**Hughes Disability Score**
72
What is the prognosis with GBS?
- Complete paralysis can have complete recovery. After 6 months 80% of patients can walk unaided - 10% relapse **- Poor prognosis:** old age, receding campylobacter jejuni infection, rapid, need for mechanical ventilation
73
What are some causes of death in GBS?
- Respiratory failure - Infection **- PE (MOST COMMON)** - Cardiovascular dysfunction
74
What does Miller Fisher syndrome present with?
75
What symptoms make you doubt a diagnosis of GBS?
76
What is myasthenia gravis and the pathophysiology of this?
**Autoimmune neuromuscular disorder** characterised by **weakness** and **fatiguability** **Acetylcholine receptor antibodies** block AChR
77
What is the epidemiology and aetiology of MG?
**_Epidemiology:_** - Females 20-30 - Males 60-70 **_Aetiology:_** - Other autoimmune conditions e.g RA, SLE - Thymoma (10%) - Thymic hyperplasia (85%) - Genetic HLA-B8, DRw3, and DQw2
78
What cells in the thymus contribute to the development of MG?
79
What are the different subtypes of MG?
**- Clinical subtypes:** ocular or generalised **- Antibody subtypes:** AChR-Ab, Anti-MuSK, Anti-LRP4, Seronegative MG **- Thymic abnormalities:** Normal, Thymoma, Thymic Hyperplasia, Atrophy **- Age:** Neonatal, Juvenile, Early Onset (\<50), Late onset (\>50)
80
What autoantibodies are associated with MG?
- AChR - MuSK - LRP4
81
How may MG present?
**Fluctuating skeletal muscle weakness with fatiguability** (symptoms often worse at end of day) **_Occular (often progresses to generalised in 2 years):_** - Ptosis, diplopia, pupil sparing **_Generalised_** **Bulbar:** fatiguable chewing, dysarthria, dysphagia **Facial muscles:** expressionless face, myasthenic sneer **Neck muscles:** extensor and flexor muscles can be affected. May get ‘dropped-head syndrome’ towards end of day. **Limb muscles**: proximal muscle weakness. Arms affected more than legs **Respiratory muscles:** Can lead to respiratory failure, which is life-threatening
82
What are some precipitants of MG that can cause a crisis?
- Pregnancy - Infection - Surgery - Drugs e.g opioids, tetracyclines, B blockers - Exercise - Emotions
83
What are some investigations you should do if you suspect MG?
**- CT/MRI!!!!!!!** - Exclude thymoma as 5 yr survival **- Ice Pack test:** improve ptosis **- Autoantibodies:** Anti-AChR. If this is negative try for MuSK and LRP4 **- Repetitive nerve stimulation (RNS) and electromyography (EMG):** progressive decline in amplitude showing fatiguability **- TFTs, Rheumatological screen**
84
How is MG managed?
**_Pyridostigmine_** - AChE inhibitor so cholinergic - Slowly uptitrate **- SE:** salivation, lacrimation, sweats, vomiting, miosis **_Immunosuppressants_** **- Prednisolone:** if above not working, give with bone protection, alternate day strategy and dose increased until symptomatic improvement. Different dose if ocular or generalised **- Azathioprine**: check TPMT levels to see if risk of bone marrow suppression **_Thymectomy_** - If thymoma OR - without thymoma, \< 45 years old and have AChR antibodies
85
What are some differentials for MG?
- Polymyositis - Myopathies - SLE - Botulism - Takayasu's arteritis
86
How can you exhibit muscle fatiguability in MG?
- Repeated blinking will exacerbate ptosis - Prolonged upward gazing will exacerbate diplopia on further eye movement testing - Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides **ALWAYS CHECK FVC!!!!**
87
What is a myasthenic crisis and who are at risk of these crises?
**Life threatening weakness of respiratory muscles** during a relapse of MG that requires **respiratory support**
88
What is the key investigation if you suspect myasthenic crisis and what values tell you this is crisis?
FVC!
89
How is a myasthenic crisis managed?
**- Urgent IVIG** **- Plasma exchange** if poor response to above - Consider ventilation - NG prednisolone AVOID PYRIDOSTIGMINE AS EXCESS SECRETIONS SO RISK OF ASPIRATION
90
What are the three main stages of MG?
**Stage one (active)**: Fluctuating muscle weakness with most severe clinical features. Majority of crises occur in this phase. **Stage two (stable):** Persistent, but stable symptoms. Serious exacerbations may develop due to infection, medications, or altering treatment. **Stage three (remission)**: A minority of patients may develop complete remission without need for treatment.
91
What is a myopathy and what symptoms favour a myopathy?
Primary disorder of muscle with ***gradual onset symmetrical weakness*** ## Footnote - Gradual onset symmetrical proximal weakness (difficulty combing hair, climbing stairs) - Specific muscle groups affected - Preserved tendon reflexes - No paraesthesia or bladder issues - No fasiculation
92
If there is gradual progressive symmetrical weakness and the following symptom, what myopathy would you consider: - Rapid onset - Spontaneous pain at rest - Pain on exercise - Oddly firm muscles
**- Rapid onset:** Toxic, drug or metabolic myopathy **- Spontaneous pain at rest:** Inflammatory myopathy **- Pain on exercise:** Ischaemia or metabolic (e.g McArdle's) **- Oddly firm muscles:** Pseudohypertrophic muscular dystrophies (fat or CT infiltrations) e.g Duchennes
93
What are some different classifications of myopathies?
- Muscular dystrophies - Myotonic disorders - Inflammatory myopathies - Metabolic myopathies - Acquired myopathies - Drug induced ALWAYS CONSIDER GENETIC COUNSELLING IF INHERITED
94
What investigations should you do if you suspect a myopathy and what will they show?
- ESR, CK, AST and LDH: raised - EMG - Muscle biopsy - Genetic testing
95
What is a muscular dystrophie and what are some examples of this?
Group of genetic diseases with **progressive degeneration and weakness of specific muscle groups**. Abnormality often in muscle membrane **Muscles can be unusually firm** due to infiltration of fat or connective tissue
96
What are the presentations of the following muscular dystrophies: - DMD - Becker's - Facioscapulohumeral muscular dystrophy
**_DMD_** - Presents around 4 years with clumsy walking, difficulty in standing and then respiratory failure - Pseudohypertrophy - CK raised 40 fold **_Becker's_** - Similar to above but milder symptoms, later age, better prognosis **_Facioscapulohumeral (FSHD)_** - Onset 12-14 years with inability to puff out cheeks and raise hands above head - Weakness of face, shoulders, upper arms, foot drop, scapular winging, scoliosis, anterior axillary folds, horizontal clavicles
97
What is a myotonic disorder and an example of this?
**Tonic muscle spasms** due to long chains of **central nuclei within muscle fibres**- autosomal dominant **_Myotonic Dystrophy_** **DM1**: 20-40 years with distal weakness (hand/foot drop), weak SCM, myotonia, testis atrophy, cardiomyopathy **DM2** **General features** myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria **Other features** myotonia (tonic spasm of muscle) weakness of arms and legs (distal initially) mild mental impairment diabetes mellitus testicular atrophy cardiac involvement: heart block, cardiomyopathy dysphagia
98
What are some examples of inflammatory myopathies?
May have pain at rest and local tenderness on palpation **- Inclusion body myositis** **- Polymyositis** **- Dermatomyositis**
99
What is polymyositis and how is it managed?
Important revision from rheumatology
100
What are some examples of the following myopathies: - Metabolic - Acquired of late onset - Drug induced
**Metabolic**: McArdle's disease pain and weakness after exercise due to defective glycogen storage **Acquired:** part of systemic disease e.g hyperthyroidism, malignancy, Cushing **Drug**: alcohol, statin, steroids, chloroquine, cocaine

(C) - Y4 Neurology and Special Senses (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions