Neurology Aaqid Akram MBChB (2013) Clinical Education Fellow

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

• Vascular• Idiopathic/iatrogenic• Trauma• Autoimmune• Metabolic• Infective• Neoplastic• Congenital• Degenerative

Epilepsy - Investigations

• 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)– 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

• 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

• 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: – 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

• 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– 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

• 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

• 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]

– 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• A = Age > 60years old (1)• B = Blood pressure > 140/90 (1)• C = clinical features

– Unilateral weakness (2)– Speech disturbance only (1)

• D = duration– >60 minutes (2)– 10-59 minutes (1)

• D = Diabetes (1)

>3– Aspirin 300mg daily (likely reduced to 75mg after assessment)– Specialist assessment + investigation within 24 hours– Secondary prevention

Stroke Rehabilitation

• Specialist Stroke Unit• Vision• Memory• Emotion• Swallowing• Communication• Motor Function• Pain Management

Long term effects

• 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