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Long-term neurological conditions Chapter 7: Neurology 105
https://t.me/med1917
Types of epileptic seizure
9
1. GENERALISED: discharge from both hemispheres
→ No warning
→ Always have LOC
2. FOCAL: discharge from one part of one hemisphere
→ May be preceding aura
→ May or may not have LOC (focal aware or impaired awareness)
→ May lead to generalised tonic–clonic seizure if LOC
Generalised onset
Tonic phase:
Tonic–clonic
• Stiffening / tone (may fall to ground)
• Hold breath → cyanosis
Tonic Clonic phase: seconds–minutes
• Rhythmical jerking
• Irregular breathing, cyanosis, salivation
Motor onset
absence
Atypical
absence
Non-motor
Myoclonic
Clonic
Atonic
Typical
• Tongue-biting, incontinence
→ Post-ictal drowsiness/confusion
Atonic phase:
• Loss of muscle tone ± jerking → fall
• ‘Blanking-out’/staring
• Absence of motor symptoms
→ Brief onset & termination (last seconds)
• More gradual onset
• Longer lasting (up to few mins)
• May have post-ictal symptoms e.g. confusion
• Sudden, repetitive, jerky movements of upper limbs, head and body
(spill/drop things → occasionally fall)
Focal onset
Motor onset • Automatisms (lip smacking/chewing, hand movements)
• Spasms
• Tonic/clonic/atonic seizures
clonic movements spreading proximally = Jacksonian March & suggests onset in
primary motor cortex
Non-motor
onset
• Sensory (e.g. smell, taste, touch, vision)
• Autonomic (e.g. nausea)
• Cognitive
• Behavioural arrest
Advice for relatives during a seizure:
Do: place pillow under head, recovery position, move nearby objects, note time seizure began →
buccal midazolam / PR diazepam if >5min if available
Do not: restrict/move them, give food/drink until recovered
Investigations for epilepsy
→ Hx & video most important
• EEG indicated if epilepsy suspected / after 1st seizure → can help determine
type & quantify risk of seizure recurrence
• Brain scans usually normal (unless a visible cause of focal seizures e.g. stroke)
9
ILAE (2017) ILAE Classication of the Epilepsies
Medicine
106 Chapter 7: Neurology Long-term neurological conditions
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A single seizure does not indicate starting AEDs
(start if >2 seizures or 1 seizure with abnormal
EEG/MRI brain)
• Must inform DVLA
• 6m driving ban after 1st seizure
• If >1 seizure must be 12m seizure-free
(includes isolated auras)
Status epilepticus = seizure >5min
or repeated seizures with no recovery of
consciousness in between
Mx: IV BZD → IV AED → anaesthetic + ICU
Management of epilepsy
10
1. Education & advice: specialist epilepsy nurse
• Avoid baths / open water swims, alcohol & sleep deprivation
• Driving
• Conception counselling
2. Anti-epileptic drugs: until seizure-free for 2y
• Aim for monotherapy at lowest effective dose
• Counsel on side-effects
3. Rescue therapy (BZDs) – call 999 if need to use
• Given to terminate prolonged seizures (>5min)
• Buccal midazolam, PR diazepam, PO clobazam (for focal seizures)
4. Other options if refractory epilepsy
• Vagal nerve stimulation • Surgery e.g. lobectomy
AED Indication Side-effects
Sodium valproate Generalised (1st /2nd line)
Avoid in women of child-bearing age
• Weight gain
• Hair loss
• Teratogen
Lamotrigine Generalised (1st /2nd line)
Focal (1st line)
Levetiracetam Focal
• Rash (SJS)
• Insomnia
• Headaches
• Irritability
Generalised
Carbamazepine Focal • Rash
• Hyponatraemia
• Ataxia
• CYP140 inducer
Ethosuximide Absence • N&V
Medicine
10
NICE (2012, updated 2021) Epilepsies [CG137]
Long-term neurological conditions Chapter 7: Neurology 107
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Neuropathies
Pathological process affecting a peripheral nerve/nerves (LMNONLY)
Causes of peripheral neuropathy
Demyelination • Antibodies against myelin – RA, CTDs, myxoedema
• Inflammatory – Guillain–Barré, chronic inflammatory demyelination
• Inherited – Charcot–Marie–Tooth Type 1
Axon degeneration
(from distal → proximal)
Compression • Entrapment of nerve e.g. carpal tunnel
Infarction • Of vasa nervorum e.g. DM, arteritis
Infiltration • Granuloma, sarcoid, cancer
• Metabolic – DM, B12/folate deficiency, CKD/CLD (urea/ammonia
build-up)
• Toxins – alcohol, lead, radiation, drugs
• Antibodies against axons – RA, CTDs
• Inherited – Charcot–Marie–Tooth Type 2
Most common causes:
1. Diabetes
2. Vit B deficiency (affects DCML)
3. Drugs/toxins
4. Alcohol
5. Demyelination (CIDP/GBS)
6. Idiopathic (50% of cases)
Investigations
• Examination: peripheral weakness, sensory loss & reflex loss (LMN signs)
• Bloods: FBC, ESR, U&Es, LFTs, glucose/HbA1c, B12/folate
• Paraproteins & antibodies: ANCA, VDRL test
• Myeloma screen
• Neurophysiology testing (EMG & NCS)
• LP: raised protein in GBS/CIDP
• Peripheral nerve biopsy: if diagnosis uncertain
Summary of patterns of neuropathy
SYMMETRICAL
Weakness
AND
sensory loss
• CIDP
• GBS
• Toxins
• Metabolic disorders (DM)
• Hereditary (CMT)
Sensory loss
WITHOUT
weakness
• B12 deficiency
• Friedreich’s ataxia
• Cervical myelopathy
Weakness
WITHOUT
sensory loss
• CMT
• Spinal muscular atrophy
Neurophysiology testing:
nerveconduction study (NCS)
electromyography (EMG) &
Weakness
AND
sensory loss
• Vasculitis
• Infections (Lyme, sarcoid)
• HNPP
• Single nerve / root damage
• Polyradiculopathy/plexopathy
ASYMMETRICAL
Weakness
WITHOUT
sensory loss
• Multifocal motor
neuropathy
Fig. 7.12
Medicine
108 Chapter 7: Neurology Long-term neurological conditions
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Guillain–Barré syndrome
Prognosis:
≈60% = full recovery in 6m
≈30% = remaining disability
5–8% mortality
Complications:
• Pneumonia
• SIADH
• Pseudo-obstruction
Miller Fisher syndrome: similar to GBS & can
also present after diarrhoeal illness
→ Classic triad of Sx: ophthalmoplegia,
areflexia, ataxia
→ most common acute polyneuropathy
PATHOGENESIS: viral infection triggers inflammatory response & autoimmune
demyelination
SYMPTOMS: 1–3w after viral infection e.g. gastroenteritis / strep throat
• Ascending paralysis – starts in legs → trunk → arms
• Loss of tendon reflexes
• Facial weakness
• Bulbar weakness – dysarthria, dysphagia
• Respiratory muscle weakness – acute respiratory failure
• ± autonomic features (postural hypotension, arrhythmias)
A motor dominant neuropathy → sensory loss = uncommon
INVESTIGATIONS: Dx usually on clinical Sx
• NCS: slowed (demyelination)
• LP: raised protein (usually normal cell counts)
MANAGEMENT11:
• Urgent – rapidly progressing paralysis → ventilatory failure
• High dose IV immunoglobulin / plasma exchange
• May need ventilatory support (on HDU/ ICU)
• Rehab: physio, OT, SALT (consider keeping NBM)
Dierential diagnosis of neuropathy
CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULOPATHY
Pathogenesis: antibody-mediated demyelination
Onset: over months
Symptoms: progressive/relapsing prox & distal weakness and sensory loss
Management: immunosuppression
MULTIFOCAL MOTOR NEUROPATHY
Pathogenesis: antibody-mediated multifocal demyelination e.g. vasculitis
Onset: over months
Symptoms: progressive asymmetrical distal limb weakness (starts hands/feet)
= difficulty gripping, foot drop
HEREDITARY SENSORIMOTOR NEUROPATHY
(CHARCOT–MARIE–TOOTH)
Pathogenesis: genetic condition causing axonal degeneration (onset in
childhood or 40s)
Symptoms:
• Progressive symmetrical numbness, tingling & weakness (starting in hands/
feet & spreads proximally)
• Clawed toes, high arches, foot drop, muscle wasting
• Family history (not always)
Investigations:
• NCS = slowed due to demyelination ± axonal damage → rules out diabetes
Medicine
11
BMJ Best Practice (2022) Guillain–Barré Syndrome
Long-term neurological conditions Chapter 7: Neurology 109
https://t.me/med1917
DIABETIC NEUROPATHY
Symptoms: symmetrical distal sensory loss (can be painful/autonomic types)
B12 DEFICIENCY
Pathogenesis: degeneration of dorsal & lateral white matter of spinal cord
Symptoms:
• Sensory ataxia & subacute combined degeneration of the spinal cord
• Progressive weakness + paraesthesia
Trigeminal neuralgia
→ facial pain experienced along the distribution of the trigeminal nerve
AETIOLOGY:
Idiopathic compression of trigeminal nerve (often by vascular loop of superior
cerebellar artery) or demyelination
CLINICAL PRESENTATION:
• Facial pain – sharp/stabbing episodes ± a constant background pain
• No associated neurological deficit
INVESTIGATIONS: a clinical diagnosis
• CT/MRI
MANAGEMENT12:
1st line: anticonvulsants (carbamazepine/oxcarbazepine)
2nd line: gabapentin, pregabalin, amitriptyline, nortriptyline, lamotrigine
Medication-resistant: microvascular decompression / ablative surgery
Risk factors for trigeminal neuralgia:
• Increased age
• Multiple sclerosis
Differentials of trigeminal neuralgia:
• Dental pain
• Mandibular osteomyelitis
• Temporomandibular joint syndrome
• Migraine / cluster headache
• Temporal arteritis
• Post-herpetic neuralgia
12
American Academy of Neurology (2008 – reviewed 2021) Diagnostic evaluation and treatment of
trigeminal neuralgia
Medicine
110 Chapter 7: Neurology Long-term neurological conditions
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Motor neurone disease
Degenerative disease of UMNs & LMNs in the spinal cord & cortex
General symptoms
• Progressive weakness but no sensory deficit (dysphagia, dysphonia,
breathing difficulties)
• Mixed UMN &/or LMN signs (spasticity/fasciculations)
Investigations
→ clinical diagnosis
→ EMG: active denervation, chronic neurogenic changes
→ Bloods: r/o other causes (CK, B12, folate, paraneoplastic Ab)
→ Spinal MRI: r/o myelopathy/radiculopathy
Life expectancy significantly shortened
(6 months to 3 years from onset of symptoms)
Management
13
Supportive & palliative: MDT approach
1. Respiratory support – consider NIV
2. Nutritional support (SALT) ± PEG tube
3. Muscle cramps – quinine, baclofen
4. Disease-modifying medication: riluzole – slows progression
5. Carer assessment & psychological support
6. End-of-life care plan
Subtypes
Amyotrophic lateral sclerosis
(ALS)
Most common
Nerves affected UMN & LMN LMN UMN & LMN UMN
Pattern of weakness Focal muscle weakness
e.g. in one hand → gradual
spreading to limbs/trunk
Associated signs/symptoms Fasciculations
+ spastic paresis
± fronto-temporal dementia
Progressive muscular
atrophy
10% of cases
Limb weakness
Gradually spreads distal
→ proximal
Fasciculations
+ painless wasting
Progressive bulbar palsy
20% of cases
Dysarthria, dysphagia
(choking/regurgitation)
Tongue fasciculations + Pseudobulbar palsy
Primary lateral
sclerosis
Rare
Slowly progressing
tetraparesis
Medicine
13
NICE (2016, updated 2019) Motor neurone disease [NG42]
Long-term neurological conditions Chapter 7: Neurology 111
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Myasthenia gravis
An autoimmune disorder of the neuromuscular junctions. Pathogenesis:
autoantibodies against the post-synaptic ACh receptor block neuromuscular
junction
Symptoms
Weakness & fatiguability → worsens throughout day
• Of proximal limbs – limits walking distance
• Of bulbar muscles – dysphagia/dysarthria
• Of ocular muscles – fluctuating diplopia & ptosis (worsens with prolonged
looking up) → MG can be purely ocular
• Of respiratory muscles – breathing difficulties
No UMN/LMN signs
Investigations
1. Thorough history
2. Examination: fatiguable tasks, full PNS & CNS exam
3. Serum antibodies: anti-ACh receptor, anti-Musk
4. EMG: repeated nerve stimulation → decreasing response
5. Imaging: CT for thymus masses
can be antibody negative
EMG can be DIAGNOSTIC
Associated with other autoimmune
conditions
• 25% also have thymoma
• 50–80% also have thymic hyperplasia
Monitoring of MG:
Course = lifelong & fluctuating
Routine monitoring: 4-monthly as an
outpatient
Monitoring acute flare: regular forced vital
capacity (FVC)
Lambert–Eaton myasthenic syndrome
Pre-synaptic NMJ disorder associated with small
cell carcinoma of the lung
Management
1. Oral anticholinesterases: prolong ACh action ( breakdown)
e.g.pyridostigmine
2. Immunosuppression: steroids ± disease-modifying drugs (azathioprine,
methotrexate, mycophenolate)
3. Thymectomy: if hyperplasia/thymoma
14
Acute crisis: sudden worsening of weakness (± respiratory muscles)
→ Rapid plasmapheresis &/or IV immunoglobulins
→ ± Ventilation
→ ± NG feeding
14
Sussman, et al. (2015) Myasthenia gravis: Association of British Neurologists’ management
guidelines. Pract Neurol, 15:199.
Medicine
112 Chapter 7: Neurology Long-term neurological conditions
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Myopathies
= muscular disorder/weakness
Myositis: inflammatory disease
↳
Muscular dystrophy: inherited disorder of protein dysfunction
Channelopathies: ion channel disorder of muscle
General features
• Symmetrical weakness
• Muscle wasting or pseudohypertrophy
• Muscle pain/aches/cramps
± ptosis, ataxia, dysphagia, dysarthria, cardiac signs
Categories of muscle disease:
• Inflammatory
• Hereditary
• Metabolic
• Drug-induced
General investigations
1. Thorough Hx & clinical examination: incl. FHx
2. Serum CK: raised
3. EMG: myopathic features
4. Muscle MRI/biopsy: shows inflammation / muscle changes
5. Neurogenetic tests
Muscular dystrophies
DUCHENNE & BECKER: X-linked recessive
Duchenne Becker
Onset From 5y From 10y
Symptoms • Progressive proximal weakness
• Calf pseudohypertrophy
• Waddling / slow running
• Gower’s sign
• 30% = intellectual impairment
Prognosis Early 20s (respiratory failure / cardiomyopathy)
MYOTONIC DYSTROPHY: most common dystrophy → autosomal dominant
Symptoms:
• Myotonic grip: slow to let go / open fist
• + distal muscle weakness
• + ptosis & facial muscle weakness
• ± respiratory & cardiac complications
• Milder phenotype
• Not associated with
intellectual impairment
Late 40s → normal
Medicine
Differentiating myopathic & neurogenic cause of weakness
Neurogenic Myopathic
Weakness Distal or focal Proximal or bulbar
Muscle pain / fatigue – Pain in myositis
Fatigue in myasthenia
Sensory loss / burning + –
Reflex loss + –
CK
Fasciculation In MND
Clues in Hx Diabetes Sx
/ normal
–
Not in peripheral neuropathy
Often FHx
Viral Sx (GBS)
Long-term neurological conditions Chapter 7: Neurology 113
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Essential tremor
Involuntary, rhythmical shaking movement most commonly affecting the
upper limbs
Clinical presentation
1. Progressive tremor
• Bilateral & symmetrical (4–12Hz frequency)
• Usually affects hands/arms, but can also affect voice & head
• Present during action & when holding a position (postural)
• Not usually present at rest (but can be when very severe)
• Improves with alcohol
2. No associated neurological symptoms e.g. dystonia, ataxia, or parkinsonism
Investigations
• Examination – including Archimedes’ spiral
• CT/MRI brain – if associated neurological symptoms
• TFTs – r/o hyperthyroidism
• DAT scan – if tremor is particularly asymmetrical &/or signs of Parkinson’s
disease
essential tremor is a clinical diagnosis
Tremor may impact fine motor control: writing,
eating, drinking etc.
Risk factors for essential tremor:
• Increasing age • FHx
Archimedes’ spiral: by asking a patient to
draw this spiral, the frequency, amplitude
& direction of tremor can be assessed
Management
No psychological or functional impact: no intervention indicated
Psychological or functional impact:
• 1st line: propranolol or primidone
• 2nd line: gabapentin or topiramate
Treatment-resistant tremor: deep brain stimulation (DBS) → has associated
complications/risks
Normal Essential tremor
15
Parkinson’s disease
Differentials of essential tremor:
• Parkinson’s disease
• Dystonic tremor
• Psychogenic tremor
• Drug-induced tremor
(lithium, antipsychotics, caffeine)
15
BMJ Best Practice (2021) Essential Tremor
Medicine
114 Chapter 7: Neurology Long-term neurological conditions
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Headaches
Classifying headaches
Describing headaches:
S: Site
O: Onset
C: Characteristic
R: Radiation
A: Associations
T: Timing
E: Exacerbating factors
S: Severity
Symptoms S: Unilateral/bilateral
Who? • 10% of population
Risk factors /
associations
Management
Migraine Cluster headache Tension
O: Hours (± worsening aura)
C: Throbbing
A: Photo-/phonophobia, N&V
T: 24–72h
E: Movement
Typically, aura develops from
visual → sensory → motor
• F:M = 3:1
• Menstruation/OCP
• Physical exercise
• Emotional stress
• Foods (cheese, chocolate, red wine)
14
r/o other differentials:
e.g. raised ICP, SAH, meningism
1st line:
Lie in dark room plus
oral NSAIDs/paracetamol
± anti-emetic
± 900mg soluble aspirin
(avoid in asthmatics)
2nd line: triptans
Prophylaxis: if ≥4 a month or ≥1 a
week, topiramate or propranolol
Primary headaches Secondary headaches
• Migraine
• Tension / medication overuse
• Cluster
• Subarachnoid haemorrhage
• Raised ICP
• Infection
• GCA
Benign/primary headaches
S: Above eye (unilateral)
O: Sudden
C: Severe
A: Tearing, nose running, sweating,
conjunctival injection
T: 15–180min & recurs 1–8 × a day
Relief: Pacing (have restlessness)
• M:F = 3:1 • 30–70% of the population
• Alcohol
• Smoking
r/o other causes of eye pain:
e.g. acute glaucoma / GCA
At start of attack: SC triptan
During attack: home O2 via non-rebreathe
mask
Prophylaxis:
→ alcohol avoidance
→ verapamil/topiramate/prednisolone
S: Frontal bilateral ‘band’
O: With stress/tiredness
C: Tightness
• Medication overuse
• Stress
• Depression
Episodic: <15d/m
Paracetamol & NSAIDs
Advise on medication overuse
If use medications >2d/w
Low dose amitriptyline
(start at 10mg and slowly )
Chronic: >15d/m
• Exclude medication overuse
• Exclude depression
• Reassurance, relaxation
Medicine
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