Neurology
Aaqid Akram MBChB (2013)
Clinical Education Fellow
Objectives
• Understand processes behind neurological
diseases.
• Understand aetiology
• Progressive nature
• Wider impact a neurological disease has on
health
Epilepsy
• Seizure
– Transient S/S
– Abnormal electrical activity
• Epilepsy
– >1 unprovoked seizures (>24 hours apart)
– 1 unprovoked + probability of further seizures
– Epilepsy syndrome
Epilepsy - Focal
• Focal (Partial)
– Limited to one hemisphere
– Localised
• Awareness = simple
• Impaired awareness = focal dyscognitive
– May progress to generalised (tonic-clonic)
Epilepsy – Generalised
• Generalised (throughout the brain)
– Absence (Petit Mal)
– Myoclonic
– Clonic
– Tonic
– Tonic-clonic (Gran Mal)
– Atonic
Epilepsy - Causes
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Vascular
Idiopathic/iatrogenic
Trauma
Autoimmune
Metabolic
Infective
Neoplastic
Congenital
Degenerative
Epilepsy - Investigations
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Blood Tests – rule out other causes
Imaging – CT/MRI (MRI>CT)
EEG
Video of seizures
ECG
Genetic testing
Epilepsy - Management
• Education + Self Management (ESN)
• Provoked
– After illness/surgery
• Correction of electrolytes
– Alcohol/substance misuse
• Addiction services
• Anti epileptic drug (AED)
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Carbamazepine
Levetiracetam
Lamotrigine
Sodium Valproate
Motor Neuron Lesions
Motor Neurone Disease
• Anterior horn cells of spinal cord / motor
cranial nuclei
• UMN/LMN signs (LMN>UMN)
• ?abnormality of mitochondrial function
• 2/100,000
• >50 years old
• Male>female
Motor Neurone Disease
• Amyotrophic lateral sclerosis (ALS)
– Focal onset
• Limb onset: most common
• Bulbar onset: appx 20%
• Respiratory onset: least common
– Cognitive dysfunction: 15%
– Pure UMN: primary lateral sclerosis
– Pure LMN : progressive muscular atrophy
• Symptom treatment/NIV/Riluzole
Dementia
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Memory Loss
Cognitive decline
Difficulties with ADL
Progressive decline
No clouding of consciousness
Appx 800,000
£23 billion a year
Dementia
• Alzeihmer’s (50%)
– Cerebral degeneration, atrophy
– Amyloid plaque formation
– Reduced ACh production from affected neurons
• Vascular (25%)
– Cerebrovascular disease
– Stepwise degeneration
• With Lewy Body (DLB) (15%)
– Abnormal protein deposition in neurons
– Brain stem/neocortex
• Fronto-temporal (<5%)
– Protein tangles (Pick’s bodies) seen histologically
• Parkinson’s Disease (PD)
Cognitive Function
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Attention/concentration
Orientation (Time/place/person)
Memory (Short and long term)
Praxis (getting dressed/lay a table)
Language function
Executive function (problem solving)
Psychiatric features
Cognitive impairment screen
– MMSE/6CIT/GPCOG/7Minute Screen
Dementia
• Acetylcholinesterase Inhibitors (AChE)
– Donepezil/galantamine/rivastigmine
– Titrated slowly (cholinergic effects)
– Alzeihmer’s / some evidence for PD
• N-Methyl-D-Aspartate (NMDA)
– Memantine
– Moderate to severe Alzeihmer’s
• Avoid antipsychotics
– unless agitation/aggression
– Lorazepam/haloperidol/olanzapine (PO>IM>IV)
Parkinson’s Disease
• Idiopathic syndrome of parkinsonism
– Resting Tremor
– Rigidity
– Bradykinesia
• Degeneration of dopaminergic pathways
– Substantia nigra
• Drug Induced
– Block dopamine receptors/reduce storage
– Tranquilisers/anti emetics (metoclopramide)
Parkinson’s Disease
• 50-59 years old: 17/100,000
• 70-79 years old: 93/100,000
• Exposure to:
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Pesticide
manganese dust
carbon disulphide
severe CO poisoning
• Born in spring
• Males>females
• Encephalitis
Parkinson’s Disease
• Tremor (4-6 Hz)
– At rest + concentration
– Absent during activity
– Asymmetrical before generalisation
• Rigidity
– Increased resistance to passive movement
• Bradykinesia
– Slowness of voluntary movement
– Reduced automatic movements
– Reduced arm swing while walking
• Festinated shuffling gait + unsteadiness on turning
• Fixed facial expression + infrequent blinking
Parkinson’s Disease
• Levodopa
– Honeymoon period up to 10 years
– Side effects are rare
• Dopamine agonists
– Motor features/younger patients
– More common side effects
– Pramipexole / bromocriptine
• Monoamine oxidase B inhibitors
– Selegiline/rasagiline
Parkinson’s Disease
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COMT Inhibitors
Amantadine
Apomorphine (SC)
Pallidotomy
Thalamic
Subthalamic
Deep Brain Stimulation
Parkinson’s Disease
• Depression/anxiety
– TCA/SSRI
• Dementia
– AChE Inhibitors
• Compulsive behaviours
– Dopamine agonists may cause development of
• Pathological gambling
• Compulsive eating/shopping
• Hallucinations/Psychosis
– Clozapine
• Acute akinesia (Parkinson’s Crisis)
– Infection/surgery/GI disease
Parkinson’s Disease
• Multiple System Atrophy
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Appears as parkinsonism initially
Rapid progression
Inability to look down voluntarily
Autonomic dysfunction (urogenital, postural
hypotension)
• Progressive supranuclear palsy
– Paresis of conjugate gaze
– Problems looking up and down voluntarily
– Dysphagia and dysphasia.
Multiple Sclerosis
• Cell mediated autoimmune
– Repeated episodes of inflammation
– Loss of insulating myelin sheath
– Sclerosis in these areas
– Slowing or blocking of signal transmission
• Relapsing Remitting (RR)(80%)
• Secondary progressive (50% from RR)
• Primary progressive (15%)
Multiple Sclerosis
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Distance from equator
Females>males
40-50 years old
Genetic predisposition - +ve FHx
Reduced relapses in pregnancy]
Clinical diagnosis + supporting MRI
Revised McDonald Criteria
Multiple Sclerosis
• Relapses
– Methylprednisolone
– Azathioprine may reduce relapses and progression
• Disease modifying therapy
– Interferon Beta/Glatiramer
– Dimethyl fumarate/Teriflunomide/Alemtuzumab
• Second line
– Natalizumab/Fingolimod/Mitoxzantrone
• Other
– Cannabinoids
– Percutaneous venoplasty
• Symptom specific medication
TIA/Stroke
• Disruption of blood supply to brain
• Rapidly developing disturbance of cerebral
function
• > 24 hours = stroke
• <24 hours = TIA
• Infarction(Ischaemic) (70%)
• Haemorrhage (Intracranial) (15)
• SAH (5%)
Brain Anatomy
TIA/Stroke
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Hypertension
Smoking
DM
Heart Disease
Peripheral vascular
disease
• Previous TIA/stroke
• Polycythaemia vera
• Carotid artery
occlusion
• COCP
• Hyperlipidaemia
• Clotting disorders
• Alcohol
TIA/Stroke
• FAST
• Cerebral (50%) [TACS/PACS]
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Contralateral hemiplegia (flaccid  spastic)
Contralateral sensory loss
Homonymous hemianopia
Higher cerebral dysfunction (dysphasia/visiospatial
disorder)
• Cerebellar/Brainstem (25%) [POCS]
– Quadraplegia/locked in syndrome/gaze disturbances
• Lacunar (25%) [LACS]
– Basal ganglia/internal capsule/thalamus/pons
– Pure motor/pure sensory/mixed/ataxia
TIA: Risk of Stroke
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A = Age > 60years old
B = Blood pressure > 140/90
C = clinical features
(1)
(1)
– Unilateral weakness
– Speech disturbance only
(2)
(1)
D = duration
– >60 minutes
– 10-59 minutes
(2)
(1)
D = Diabetes
(1)
>3
– Aspirin 300mg daily (likely reduced to 75mg after assessment)
– Specialist assessment + investigation within 24 hours
– Secondary prevention
Stroke Rehabilitation
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Specialist Stroke Unit
Vision
Memory
Emotion
Swallowing
Communication
Motor Function
Pain Management
Long term effects
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ADL
Progression + Fear of progression
DVLA
Mood – altered personality?
Independence/Reliance
Institutionalisation
DVLA
• Seizure
– 1st episode
• 6 months off driving
• high risk = 12 months
– Mulitple
• Must be seizure free for 5 years +/- medication
• Chronic Neurological Disorders
– Unable to drive
• Impairment of coordination
• Impairment of muscle power
– require medical assessment suggesting driving
performance not impaired (1-3 year licence)
DVLA
• TIA
– Single
• Cannot drive for 1 month
– Multiple over short period
• Notify DVLA
• 3 months free from further attacks
• Stroke
– Cannot drive for 1 month
• If driving performance not impaired
– If deficit >1 month
• Notify DVLA
• Cannot drive until driving performance not impaired
Objectives Were:
• Understand processes behind neurological
diseases.
• Understand aetiology
• Progressive nature
• Wider impact a neurological disease has on
health