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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 Motor Neuron Disease Parkinson's Disease Dementia The Peer Teaching Society is not liable for false or misleading information…
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…
Epilepsy
Aetiology
1. Idopathic (2/3rds) 2. Structural (Trauma, SOL, Developmental, Stroke) 3. Metabolic (hypoglycaemia, hypocalcemia, hyponatraemia) The Peer Teaching Society is not liable for false or misleading information…
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…
Epilepsy
Partial Seizures
Commonly caused by structural pathology.
Simple Partial (Jacksonian March) Complex Partial = classically temporal lobe!
Partial + Secondary Generalisation
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Epilepsy
Generalised Seizures
Commonly idiopathic.
Absence = 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…
Epilepsy
Prodrome – Aura – Ictal – Post-Ictal
Prodrome
Mood/Behaviour Change The Peer Teaching Society is not liable for false or misleading information…
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…
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…
Epilepsy
Parietal Lobe: Tingling Numbness Occipital: Visual Phenomena (spots/lines/flashes) The Peer Teaching Society is not liable for false or misleading information…
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…
Epilepsy
Management
Partial = Carbamazepine Generalised = Sodium Valproate OR Lamotrigine Neurosurgical Resection The Peer Teaching Society is not liable for false or misleading information…
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…
Epilepsy
Complications
Sudden Unexpected Death in Epilepsy (SUDEP) Status Epilepticus The Peer Teaching Society is not liable for false or misleading information…
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…
Multiple Sclerosis
Risk Factors = Women, Temperate Areas ~30yrs The Peer Teaching Society is not liable for false or misleading information…
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…
Multiple Sclerosis
• • • •
Clinical Courses
Benign Relapsing Remitting Secondary Progressive Primary Progressive The Peer Teaching Society is not liable for false or misleading information…
Multiple Sclerosis
The Peer Teaching Society is not liable for false or misleading information…
Multiple Sclerosis
Symptoms
Monosymptomatic!
Disseminated in Time and Space The Peer Teaching Society is not liable for false or misleading information…
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…
Multiple Sclerosis
Other: Ataxia, Erectile Dysfunction, Urinary retention The Peer Teaching Society is not liable for false or misleading information…
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…
Multiple Sclerosis
Management
Acute: Methylprednisolone IV Chronic: Interferon/Glatiramer, Natalizumab The Peer Teaching Society is not liable for false or misleading information…
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…
Guillain-Barre Syndrome
Symptoms
4 wk peak!
• Weakness – Leg, Trunk, Respiratory. Proximal, Distal. Symmetrical.
• Back/Limb Pain • Autonomic Features: seating, tachycardia, arrhythmia’s.
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Guillain-Barre Syndrome
Signs
Areflexia!
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Guillain-Barre Syndrome
Investigations
Nerve Conduction Studies – slow conduction Regular FVC The Peer Teaching Society is not liable for false or misleading information…
Guillain-Barre Syndrome
Management
IV Immunoglobin or Plasma Exchange The Peer Teaching Society is not liable for false or misleading information…
Motor Neuron Disease
Selective loss of motor neurons in: Motor Cortex (UMN), Cranial Nerve Nuclei (UMN/LMN) and Anterior Horn Cells (LMN).
>40yrs.
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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…
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…
Motor Neuron Disease
Associations
Fronto-Temporal Dementia (10-35%)!
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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…
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…
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…
Parkinson's Disease
~65yrs. Associated with Lewy Bodies The Peer Teaching Society is not liable for false or misleading information…
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…
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…
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…
Parkinson's Disease
Management
Dopamine Agonist – Ropinirole/Pramipexole Apomorphine – potent DA agonist, acute Anticholinergics – tremor
Deep brain stimulation
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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…
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…
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…
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…
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…
Alzheimer's Dementia
Investigations
MMSE or Addenbrooks Cognitive Exam CT – temporal/parietal atrophy, ventricular enlargement MRI – hippocampus/amygdala/medial temporal lobe grey matter atrophy CSF – phosphorylated tau protein The Peer Teaching Society is not liable for false or misleading information…
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…
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…
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…
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…
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…
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.
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.