Neurological Disorders

1. NEUROLOGICAL
DISORDERS

2. Neurological disorders
Nerve and muscle
Multiple sclerosis
Parkinson’s disease
Stroke
Epilepsy
Headache and facial pain
Neurological infections
Head injury and tumour
Spinal conditions
Congenital disorders
Dementia

3. Nerve and muscle
Neuropathy
Myaesthenia gravis
Muscular dystrophy
Myopathy

4. Neuropathy
Mononeuropathy
damage by trauma eg pressure
Diabetics – nerves sensitive to pressure
damage to blood supply (vasa nervorum)
Vasculitic diseases
Polyneuropathy
Multiple peripheral nerves
Distal, symmetrical pattern
Lower limbs before upper limbs
Causes -
Inflammatory
Metabolic
toxic

5. Diabetic neuropathy
30 – 40% of diabetic patients in UK will develop
significant nerve damage during their lives due to sub-
optimal control
Small fibre neuropathy
Autonomic neuropathy
Motor neuropathy
Large fibre neuropathy
Complications
Callus and plantar neuropathic ulcers
Infection

6. Diabetic neuropathy
Acute painful neuropathies, radiculopathies and
mononeuropathies
Constant burning, parasthesiae, shooting pains
Either symmetrical sensory stocking distribution (both feet) or
single or adjacent nerve roots affecting:
feet and/or legs or one or both thighs
Often wasting
May be weakness leading to falls
‘diabetic amyotrophy’/ proximal motor neuropathy due to
radiculopathy or femoral neuropathy
Contact discomfort (eg. clothes)
Insomnia

7. Nerve compression

Median nerve compression
 in carpal tunnel syndrome occurs in up to 10% patients
Diagnosis difficult if severe neuropathy involving hands
Requires nerve conduction studies
Surgical decompression
Ulnar nerve compression
Usually at elbow (groove of medial epicondyle)
Pain and paraesthesia along medial aspect of forearm and numbness in little
and ring fingers
May be wasting and weakness of ulnar-innervated small muscles of hand
Common peroneal nerve neuropathy
Nerve runs around head of fibula
Presents with foot drop and may have wasting of tibialis anterior (weak foot
dorsiflexion, toe extension and foot eversion)

8. Guillain-Barre syndrome
Acute peripheral neuropathy affecting motor more than
sensory nerves
Usually follows infection
Clinical features
Symptoms over days/weeks
Bilateral flaccid weakness
Loss of tendon reflexes
May affect muscles of respiration
Burning pains and numbness

9. Myaesthenia gravis
Autoimmune disorder
Most patients have antibodies to acetylcholine receptors
at neuromuscular junction
Rare (annual incidence 0.4 in 100000)
Clinical features
Fatiguable ptosis
Diplopia with limitation of eye movements
Facial weakness
Dysphagia
Dysarthria
Neck and limb muscle weakness (fatiguable)
Can involve respiratory muscles

10. Myaesthenia gravis

11. Myopathy
Weakness of trunk and proximal limb muscles
May be weakness of neck flexion and/or
extension and muscles of facial expression
Gait waddling

12. Inflammatory myopathies
Polymyositis
May occur in association with autoimmune connective tissue
disorders
Dermatomyositis
Rash affects face and knuckles
In minority of cases may have associated malignancy
Inclusion body myositis
Selective involvement of finger flexors and quadriceps

13. Dermatomyositis

14. Spinal root disease (radiculopathy)
Cervical radiculopathy
Prolapse of intervertebral disc may compress cervical nerve as exits
Neck pain, muscle weakness, loss of tendon reflex, sensory impairment
Drugs, physiotherapy, may need surgery
Prolapsed lumbar intervertebral disc
S1 root
Low back pain and tenderness
Pain down leg from buttock to ankle (sciatica)
Wasting and weakness of gastrocnemius and soleus)
S1 sensory loss
Depressed ankle reflex
L5 root
Sciatic pain
Foot drop (weakness extensor hallucis longus)
L5 dermatomal sensory impairment

15. Spinal cord disease
Inherited
Hereditary spastic paraplegia
Congenital
Arnold-Chiari (develop syringomyelia)
Trauma
Disc protrusions, vertebral fracture
Infection
Epidural abscess
Inflammation
Post-viral transverse myelitis
Neoplasm
Vetebral metastases, cord tumour
Vascular
Spinal cord infarct, epidural haematoma
Metabolic
Subacute combined degeneration of the cord
Degenerative
Cord – motor neurone disease
Spine – spondylosis with cord compression

16. Multiple sclerosis
Most common chronic neurological disorder affecting
young people
In most typical form is characterised by lesions
separated in time and space in central nervous system
More common in temperate than tropical climate
More common in females (M:F 1.5:1)
Usual age of presentation is between ages of 20 – 40
Prevalence in UK of 1 in 1000

17. Multiple sclerosis
Pathophysiology
Affects white matter of brain and spinal cord
Inflammatory cells present and myelin damaged
Foci of inflammation and demyelination known as plaques
Initially inflammation and oedema, followed by loss of myelin and
then gliosis (scar tissue)
Leads to reduction in conduction velocity
Thought that environmental agent (eg. virus) triggers condition in
genetically susceptible individual

18. Multiple sclerosis
Presentation
Visual disturbance
Optic neuritis caused by inflammatory demyelination of one optic
nerve
Pain around one eye especially on eye movement
Blurred vision which may progress to monocular blindness over days
or weeks
Loss of colour vision
Limb weakness and sensory disturbance
Lesion in spinal cord or cerebral hemispheres
May have tingling sensation down back +/or limbs on neck flexion
Symptoms may be worse after hot bath

19. Multiple sclerosis
Course
Relapsing-remitting
After episode may be complete or near-complete
resolution of symptoms (80% patients)
After further episodes may be some residual disability
Secondary progressive
Steady progression without resolution
Primary progressive
10% patients have this form of disease with no clear
relapses and remissions

20. Multiple sclerosis

21. Multiple sclerosis
Management
If relapse severe enough to limit function – treat
with steroids
Symptom control
Spasticity, fatigue, bladder disturbance, depression,
pain
Disease modifying therapy
Interferon beta and glatiramer acetate

22. Parkinson’s disease
Degenerative condition affecting extrapyramidal pathways
(neurotransmitter dopamine)
Affects dopaminergic neurones in substantia nigra of midbrain
projecting to striatum of basal ganglia
Symptoms occur when 60-80% nigrostriate neurones lost
Mean age of onset 60 years
Positive family history unusual but some gene mutations identified
Clinical triad:
Akinesia
Rigidity
Tremor

23. Parkinson’s disease
Gait
Flexed/stooped posture
Difficulty defending balance
Difficulty initiating walking (‘freezing’)
Steps small and shuffling
Festinant
Normal arm swing lost
Risk of falls

24. Posture

25. Parkinson’s disease
Treatment
Medical
Levodopa (often in combination with DOPA decarboxylase inhibitor)
Dopamine receptor agonists
Selegiline (monoamine oxidase inhibitor type B)
Entacapone (catechol-O-methyltransferase inhibitor)
Amantadine
Surgical
Stereotactic thalamotomy (severe tremor)
Pallidotomy (drug-induced dyskinesias)

26. Other movement disorders
Chorea
Randomly distributed, irregular timed muscle jerks
Athetosis
Inability to sustain body part in one position (eg fingers)
Tremor
Dystonia
Tics
Drug-induced
Restless legs syndrome
Stiff person syndrome

27. Stroke
Third most common cause of death in developed world
(annual incidence 2 per 1000 population)
Rapidly developing symptoms or signs
If resolve within 24 hours – transient ischaemic attack
Mechanism
Infarction
thrombotic (thrombosis of arteries in CNS – degenerative,
inflammatory, trauma)
Embolic (cardiac valve disease, AF, recent MI)
Haemorrhage

28. Vascular territories

29. Stroke
Cerebral arteries
Anterior
Middle
posterior
Clinical features
Anterior circulation infarct
Hemiplegia
Hemianopia
Cortical deficits (dysphasia, visuospatial loss)
Lacunar infarct
Pure motor stroke
Pure sensory stroke
Ataxic hemiparesis
Posterior circulation infarct
Brainstem lesion
Homonymous hemianopia

30. Stroke
Complications
Pneumonia
Deep venous thrombosis and pulmonary embolism
Myocardial infarction
10% of patients with cerebral infarction die in first 30 days post-
stroke
50% remain dependent
Long-term disability
Pressure sores, seizures, falls, spasticity, depression

31. Stroke
Treatment
If no haemorrhage start aspirin (+/- dipyridamole)
Clopidogrel can be used in patients intolerant of aspirin
Thrombolysis
iv tissue plasminogen activator (alteplase)
Within 3 hours of stroke onset
Prevention
Modify risk factors
Smoking
Diet (inc lower cholesterol)
Blood pressure
Diabetic control

32. Subarachnoid haemorrhage
Causes
Rupture of aneurysm
Arteriovenous malformation
Trauma
Blood vessels weakened by infection (v rare)
Features
Sudden severe headache with photophobia, vomiting, neck
stiffness
Management
30-40% die within few days of onset
Signif risk of rebleeding in 6 weeks after onset
Nimodipine
Aneurysm clipped or coiled

33. Intracerebral haemorrhage
10% of all strokes
Large haematomas have poor prognosis (>50%
mortality) – risk of hydrocephalus and coning
Causes
Hypertension
Bleeding into tumours
Trauma
Blood disorders
Blood vessel disorders

34. Epilepsy
1% of population suffer from epilepsy (recurring
tendency to have seizures)
Definition
‘ paroxysmal disorder in which cerebral cortical neuronal
discharges result in intermittent, stereotyped attacks of altered
consciousness, motor or sensory function, behaviour or emotion’
Classification
Partial (simple or complex)
Generalised (absence, myoclonic, tonic-clonic, tonic, atonic)

35. Partial seizures
Simple partial seizure
Arise in one cortical area (most commonly temporal lobes)
Usually brief
Discharge remains localised (eg focal motor, sensory or psychic
symptoms)
No loss of awareness
Complex partial seizure
Impairment of awareness during attack
Frequently altered or ‘autonomic’ behaviour
Reactive automatisms – may be able to do simple task
Partial onset with secondary generalisation
Epileptic discharge spreads to both cerebral hemispheres

36. Generalised seizures
Absence
Myoclonic
Clonic
Tonic
Tonic-clonic
Atonic

37. Tonic-clonic seizures
May have prodrome or no warning
Tonic phase – rigidity
Often cyanosed
Clonic phase – jerking (about 2 mins)
May be incontinent or bite cheek/tongue
Post-ictal confusion and tiredness

38. Conditions that mimic epilepsy
Syncope
Lasts <30 secs, pale, little/no confusion after event
Cardiac
arrhythmia, outflow obstruction
Transient ischaemic attacks
Usually last longer, rarely LOC
Narcolepsy, cataplexy
Metabolic disturbance
Non-epileptic attacks

39. Headache and facial pain
Headache
Primary (uncertain cause)
Migraine
Cluster headache
Tension-type headache
Secondary (known cause)
Raised intracranial pressure
Idiopathic intracranial hypertension
Meningeal irritation
Giant cell arteritis
Metabolic disturbances
Facial pain
Trigeminal neualgia
Post-herpetic neuralgia

40. Migraine
Unilateral headache
Associated with nausea, vomiting, visual disturbance
Typical onset teens and twenties
Aura may be phase of vasoconstriction
Then vasodilatation of extracerebral vessels may cause
headache
Treatment
simple analgesia initially
triptan (5HT1 R agonist)
Prophylactic agents
Propranolol
pizotifen

41. Other headaches
Cluster headache
Unilateral headache
Severe attacks pain around one eye
Lasts 20 – 120 mins
Usually recurs several times a day
Pattern continues for days, weeks or months
Then symptom-free for weeks-months
Treatment
Steroids initially, then methysergide, verapamil or pizotifen
Tension-type headache/ chronic daily headache
May be due to neck muscle contraction
Amitriptyline may help

42. Facial pain
Trigeminal neuralgia
Compression of trigeminal sensory root
Unilateral facial pain with trigger areas
Treat with carbamazepine initially
May require surgery
Post-herpetic neuralgia
Post-shingles of a branch of trigeminal nerve
Persistent facial pain after rash healed
May respond to amitriptyline, carbamazepine

43. Neurological infections
Bacterial
Meningitis
Neisseria meningitidis, Haemophilus influenza, Streptococcus
pneumoniae
Brain abscess
May complicate otitis media
Raised intracranial pressure, focal signs, seizures
Parameningeal infections
Pus in epidural space
Tuberculosis
Syphilis
Lyme disease
Leprosy

44. Neurological infections
Viral infections
Viral meningitis
Mumps, enterovirus
Viral encephalitis
Commonest cause Herpes simplex
Headache, fever, reduced LOC, may have seizures
Herpes zoster
Dormant in dorsal root ganglion after chickenpox
May reactivate as shingles
Pain and itching of single or adjacent dermatomes, following by
vesicular rash
Retroviral infections
Meningitic illness may occur at seroconversion
Slowly progressive dementia
Risks of immunocompromise (infection, tumour)

45. Head injury
Damage at impact
Contusion and laceration
Diffuse axonal injury
Secondary complications
Haematoma
Cerebral oedema
Cerebral ischaemia
Coning
Infection
Post-traumatic epilepsy

46. Brain tumour
Intracranial neoplasms:
Benign
Usually extra-axial (eg. Meninges, cranial nerves)
Compress brain
Malignant
Usually intra-axial (ie. brain parenchyma)
Primary (>50% adult intracranial neoplasms)
gliomas
Secondary (15-20% adult intracranial neoplasms)
metastases

47. Glioma

48. Brain tumour - management
Surgery
Benign tumours
Complete excision may be possible -cure
Malignant
Histology
Symptomatic
Radiotherapy
Gliomas – direct towards tumour
Metastases – whole-brain radiation
Drug treatment
Anti-convulsants
Dexamethasone
Chemotherapy

49. Congenital disorders
Cerebral palsy
Spinal dysraphism
Infantile hydrocephalus
Cerebral structural disorders
Intrauterine infection

50. Cerebral palsy
Pre- or peri-natal insult
Fetal hypoxia or infection
Prematurity
Traumatic delivery (intracranial haemorrhage)
Clinical features:
Spastic diplegia
May have shortening and deformity of legs
Spastic hemiplegia
Associated with hemisensory deficits, learning difficulties and
epilepsy
Athetoid cerebral palsy
Movement disorder develops in early childhood. Usually normal
cognitive function

51. Spinal dysraphism (spina bifida)
Failure of closure of neural tube during development
Defect of overlying skin
Abnormal development of bony structures
Particularly affects lumbosacral region
Myelomeningocoele
Parts of spinal cord in meningeal sac
Paraplegia and incontinence
Spina bifida occulta
Mildest form
Failure of fusion of vertebral arches

52. Congenital disorders (continued)
Infantile hydrocephalus
Enlargement of head
Delayed development, learning difficulties, seizures, spastic
paraparesis
Neurosurgery – eg. ventricular shunt
Cerebral structural disorders
May be incidental findings or may delay development
Intrauterine infection
Rubella
Cataracts, hearing loss, learning difficulties, congenital heart disease
Neurosyphilis
Adult disease plus deafness, keratitis, deformed teeth

53. Neurogenetics
Huntington’s disease
Wilson’s disease
Friedreich’s ataxia
Hereditary spastic paraplegia
Leber’s hereditary optic atrophy
Hereditary spinal muscular atrophies
Hereditary motor and sensory neuropathy (HMSN)
Muscular dystrophies

54. Friedreich’s ataxia
Autosomal recessive
Trinucleotide repeat (GAA)
Progressive ataxia, areflexia, extensor
plantars
Kyphoscoliosis
Pes cavus
Cardiomyopathy

55. Pes cavus

56. Muscular dystrophy
Dystrophinopathies (mutations of X-linked gene for
muscle protein dystrophin)
Duchenne muscular dystrophy
Boys develop proximal weakness in early childhood
Difficulty rising from squatting position (use hands to ‘climb’ up legs –
Gowers’ sign)
Pseudohypertrophy of calf muscles (muscle replaced by fatty tissue)
Progressive disability
Becker muscular dystrophy
Presents in adolescence or adult life
Can have normal lifespan but progressive disability
Limb-girdle dystrophies

57. Muscular dystrophy
Myotonic dystrophy
Autosomal dominant
Abnormally sustained muscle contraction (myotonia)
Bilateral ptosis
Facial weakness
Wasting and weakness of sternomastoids
Cataracts
Endocrine associations (diabetes mellitus, frontal balding, testicular atrophy)
Facioscapulohumeral muscular dystrophy
Autosomal dominant
Bilateral facial weakness
Winging of scapulae
Weakness of spinal and pelvic muscles - waddling gait
Lumbar lordosis

58. Hereditary motor and sensory
neuropathy (HMSN)
Inherited neuropathies
Genetic defects discovered
Incidence 1:2500
Clinical features:
Atrophy of muscles supplied by peroneal nerves (‘inverted
champagne-bottle legs’)
Areflexia
Pes cavus
Claw toes
Distal sensory loss and/or paraesthesia

59. Dementia
Inherited
Familial Alzheimer’s disease, Huntington’s disease
Trauma
Infection
Syphilis, AIDS-related,
Inflammation
Multiple sclerosis
Neoplasm
Frontal tumours
Vascular
Multi-infarct dementia
Metabolic
hypothyroidism
Drugs/toxins
Barbiturates, alcohol
Degenerative
Alzheimer’s disease, Prion disease

60. Neurorehabilitation
Aim ‘to restore patients to maximum capability and
independence within limits set by their disability and
their needs’

Multidisciplinary teams
Physiotherapy
Occupational therapy
Speech therapy
Neuropsychology
Social work