Neurology – Part 2
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Neurology – Part 2. Phase 2 Kaveesha Rajapaksa Ryad Chebbout. The Peer Teaching Society is not liable for false or misleading information…. Aims. Pathology, Aetiology , Clinical Symptoms and Signs, Investigations and Management of: Epilepsy Multiple Sclerosis Guillain-Barre Syndrome

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Phase 2 Kaveesha Rajapaksa Ryad Chebbout

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Phase 2 kaveesha rajapaksa ryad chebbout

Neurology – Part 2

Phase 2

Kaveesha Rajapaksa

Ryad Chebbout

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Aims

Pathology, Aetiology, Clinical Symptoms and Signs, Investigations and Management of:

  • Epilepsy

  • Multiple Sclerosis

  • Guillain-Barre Syndrome

  • Motor Neuron Disease

  • Parkinson's Disease

  • Dementia

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Definition

Recurrent tendency to have spontaneous, intermittent and abnormal electrical activity in a part of the brain or generalised across the brain. Leading to seizures.

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Aetiology

  • Idopathic (2/3rds)

  • Structural (Trauma, SOL, Developmental, Stroke)

  • Metabolic (hypoglycaemia, hypocalcemia, hyponatraemia)

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Partial = focal onset (localising features!)

Generalised = no localising features!

Simple = aware

Complex = impaired awareness

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Partial Seizures

Commonly caused by structural pathology.

Simple Partial (Jacksonian March)

Complex Partial = classically temporal lobe!

Partial + Secondary Generalisation

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Generalised Seizures

Commonly idiopathic.

Absence = </ 10sec, childhood

Tonic-Clonic = complex, stiff->jerk, post-ictal confusion + drowsiness

Myoclonic = sudden limb/face/trunk jerk

Atonic = loss of tone->fall, no LOC

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Prodrome – Aura – Ictal – Post-Ictal

Prodrome

Mood/Behaviour Change

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Aura

Strange Smell, Flashing Lights, Déjà vu/Jamais vu

Post-Ictal

Headache, Confusion, Myalgia, Sore Tongue, Todds Palsy (hemiplegia), Dysphasia

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Ictal – Localising Features

Temporal Lobe: Automatisms (lip smacking/fiddling), Visceral Aura (abdominal rising sensation), Dysphasia, Déjà vu/Jamais Vu, Hallucinations

Frontal Lobe: Jacksonian March (tingling/muscle contractions from fingers to ipsilateral face), Todds Palsy

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Parietal Lobe: Tingling Numbness

Occipital: Visual Phenomena (spots/lines/flashes)

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Investigations

EEG (classification)

MRI (structural lesions)

MEG/PET/SPECT ictal (localise epileptogenic focus for surgery)

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Management

Partial = Carbamazepine

Generalised = Sodium Valproate OR Lamotrigine

Neurosurgical Resection

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Management

Counselling – employment, insurance, driving (1yr seizure free)

Contraception and pregnancy

Epilepsy Nurse Specialist

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Epilepsy

Complications

Sudden Unexpected Death in Epilepsy (SUDEP)

Status Epilepticus

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Discrete Plaques of Demyelination in Central Nervous System

T-cell Mediated

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Risk Factors = Women, Temperate Areas

~30yrs

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Demyelination Plaque (commonly periventricular, cervical spine, brain stem)

Heals Incompletely

Prolonged Demyelination

Axonal Loss

Clinically Progressive Symptoms

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Clinical Courses

  • Benign

  • Relapsing Remitting

  • Secondary Progressive

  • Primary Progressive

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Symptoms

Monosymptomatic!

Disseminated in Time and Space

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Optic Neuritis: Decreased Visual Acuity, Pain on Eye Movement, Dyschromatopsia

Sensory: Lhermittes Sign, Limb Numbness/Tingling

Motor: Transverse Myelitis, UMN Weakness, Uhthoff’s Phenomenon

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Other: Ataxia, Erectile Dysfunction, Urinary retention

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Investigation

Dissemination in Time and Space!

Clinically (attacks + clinical lesions, 2:2, 2:1, 1:1)

+/- Aid of MRI

CSF (Oligoclonal Bands)

Evoked Potentials

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Multiple Sclerosis

Management

Acute: Methylprednisolone IV

Chronic: Interferon/Glatiramer, Natalizumab

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Guillain-Barre Syndrome

Acute AUI Inflammatory Demyelinating Polyneuropathy

Triggers: Campylobacter jejuni, CMV, Mycoplasma

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Guillain-Barre Syndrome

Symptoms

4 wk peak!

  • Weakness – Leg, Trunk, Respiratory. Proximal, Distal. Symmetrical.

  • Back/Limb Pain

  • Autonomic Features: seating, tachycardia, arrhythmia’s.

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Guillain-Barre Syndrome

Signs

Areflexia!

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Guillain-Barre Syndrome

Investigations

Nerve Conduction Studies – slow conduction

Regular FVC

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Guillain-Barre Syndrome

Management

IV Immunoglobin or Plasma Exchange

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Selective loss of motor neurons in: Motor Cortex (UMN), Cranial Nerve Nuclei (UMN/LMN) and Anterior Horn Cells (LMN).

>40yrs.

</10% Familial. ~20% SOD1 mutation.

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Key Features

UMN + LMN Signs

No Sensory Loss/Sphincter Disturbance

No Eye Involvement

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Clinical Patterns

Amyotrophic Lateral Sclerosis

Progressive Bulbar Signs – CN IX-XII, bulbar/pseudobulbar palsy

Progressive Muscular Atrophy – no UMN signs

Primary Lateral Sclerosis – mainly UMN

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Associations

Fronto-Temporal Dementia (10-35%)!

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Signs

Stumbling Spastic Gait

Foot Drop +/- Proximal Myopathy

Weak Grip

Spasticity/Hypereflexia/Upwards Plantars + Wasting/Fasciculations

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Motor Neuron Disease

Investigations

MRI/LP/Neurophysiology – exclude other causes

Management

Riluzole – prolong life by ~ 3months

Symptomatic + MDT (Ventilation, PEG)

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

Decreased Dopaminergic Neurons in Substantia Nigra (Pars Compacta)

Decreased Striatum Dopamine Levels

Decreased Basal Ganglia – Cortex Communication

Decreased Movement

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

~65yrs. Associated with Lewy Bodies

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

Classic Triad

Tremor – resting, pill-rolling

Rigidity – increased tone, cogwheel rigidity, leadpipe rigidity

Bradykinesia – slowness of movement initiation

  • Expressionless Face, Monotonous Hypophonic Speech, Micrographia

  • Gait: Festinant, Reduced Arm Swing

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

Other Symptoms

Anosmia

Depression

Sleep disturbances

Visual Hallucinations (animals, children)Dementia (late stage)

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

Management

L-Dopa + Dopa-Decarboxylase Inhibitor (e.g. Madopar)

  • Efficacy reduces with time = Increased Dose

  • Dyskinesia’s, Off Freezing, End-of-Dose Reduced Relapse

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Parkinson's Disease

Management

Dopamine Agonist – Ropinirole/Pramipexole

Apomorphine – potent DA agonist, acute

Anticholinergics – tremor

Deep brain stimulation

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Dementia

Syndrome of progressive deficits in 2 or more higher cognitive domains. (Memory, language, apraxia, agnosia, visuospatial function, personality)

Interferes with social functioning.

Occurs in clear consciousness.

>80yrs=20%, >100yrs=70%

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Dementia

Types

Alzheimer's Disease

Vascular Dementia

Lewry Body Dementia

Fronto-Temperal Dementia

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Alzheimer's Dementia

Increased Beta-Amyloid Peptide = Progressive Neuronal Damage (hippocampus, amygdala, temporal neocortex)

  • Neurofibillary Tangles

  • Amyloid Plaques

  • Decreased Ach

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Alzheimer's Dementia

Risk Factors

Family History, Downs Syndrome, Homzygosity for ApoE e4 Allele, DM/HTN/AF

Protective Factors

Smoking, Oestrogen

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Alzheimer's Dementia

Symptoms

Progressive Global Cognitive Impairment

Aphasia

Anosognosia (lack of insight)

Irritability

Mood Disturbance – Depression, Euphoria

Behavioural Change – Wandering, Aggression

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Alzheimer's Dementia

Investigations

MMSE or Addenbrooks Cognitive Exam

CT – temporal/parietal atrophy, ventricular enlargement

MRI – hippocampus/amygdala/medial temporal lobe grey matter atrophyCSF – phosphorylated tau protein

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Alzheimer's Dementia

Management

Acetylcholinesterase Inhibitors (Donepezil, Rivastigmine, Galantamine) – help lay down new memories

Memantine (Antiglutamatergic)

BP Control

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Vascular Dementia

Cumulative effect of many small strokes.

Vascular RF’s – Stroke Hx, HTN

Sudden Onset + Stepwise Deterioration

  • Emotional/Personality Changes

  • Cognitive Defecits

  • Depression/Labile Mood

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Lewy Body Dementia

Fluctuating Cognitive Impairment

Visual Hallucinations (animals/children) +/- Parkinsonism

Repeated Falls/Syncope

CT/MRI – relative sparing of medial temporal lobe

Histology – Lewy Bodies in Brainstem/Neocortex

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Fronto-Temporal Dementia

Frontal + Temporal Atrophy. AD Histology.

Early, 45-65yrs

Behavioural/Personality Change

Disinhibition

Change in Diet (sweets, overeating)

Emotional Blunting

Pick bodies on histology

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

Dementia

Management

Care Coordinator

Capacity

Develop Routines, Plan Ahead

Challenging Behaviour – Lorazepam, Risperidone (not Lewy Body!)

Depression - Citalopram

The Peer Teaching Society is not liable for false or misleading information…


Phase 2 kaveesha rajapaksa ryad chebbout

A 65 year old man with walking difficulties presented to his general practitioner. He complained of worsening tremor in the right arm with stiffness, which he said on occasion spread to his right leg. He said that the tremor was much worse when he was stressed, or in public. Examination revealed a man with a resting tremor, marked cogwheeling rigidity of the right side and an inability to perform repetitive tasks with the right arm. His gait was not normal and he had a reduced arm swing on the right.


Phase 2 kaveesha rajapaksa ryad chebbout

A 36 year old male patient presents with increasing unsteadiness which started two days ago. Two years ago he had blurred vision in his left eye which improved considerably within a few weeks, but left him with some minor deficit. Eight years ago he had a 3 week episode of numbness in his left arm.


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