phase 2 kaveesha rajapaksa ryad chebbout the peer teaching society is not liable for false or...

Phase 2

Kaveesha RajapaksaRyad Chebbout

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

Pathology, Aetiology, Clinical Symptoms and Signs, Investigations and Management of:•Epilepsy•Multiple Sclerosis•Guillain-Barre Syndrome•Motor Neuron Disease•Parkinson's Disease•Dementia


Aims

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


Epilepsy

Aetiology1.Idopathic (2/3rds)2.Structural (Trauma, SOL, Developmental, Stroke)3.Metabolic (hypoglycaemia, hypocalcemia, hyponatraemia)


Epilepsy

Partial = focal onset (localising features!)Generalised = no localising features!

Simple = awareComplex = impaired awareness


Epilepsy

Partial SeizuresCommonly caused by structural pathology.

Simple Partial (Jacksonian March)Complex Partial = classically temporal lobe!Partial + Secondary Generalisation


Epilepsy

Generalised SeizuresCommonly idiopathic.

Absence = </ 10sec, childhoodTonic-Clonic = complex, stiff->jerk, post-ictal confusion + drowsinessMyoclonic = sudden limb/face/trunk jerkAtonic = loss of tone->fall, no LOC


Epilepsy

Prodrome – Aura – Ictal – Post-Ictal

ProdromeMood/Behaviour Change


Epilepsy

AuraStrange Smell, Flashing Lights, Déjà vu/Jamais vu

Post-IctalHeadache, Confusion, Myalgia, Sore Tongue, Todds Palsy (hemiplegia), Dysphasia


Epilepsy

Ictal – Localising FeaturesTemporal Lobe: Automatisms (lip smacking/fiddling), Visceral Aura (abdominal rising sensation), Dysphasia, Déjà vu/Jamais Vu, HallucinationsFrontal Lobe: Jacksonian March (tingling/muscle contractions from fingers to ipsilateral face), Todds Palsy


Epilepsy

Parietal Lobe: Tingling Numbness

Occipital: Visual Phenomena (spots/lines/flashes)


Epilepsy

InvestigationsEEG (classification)MRI (structural lesions)MEG/PET/SPECT ictal (localise epileptogenic focus for surgery)


Epilepsy

Management

Partial = Carbamazepine

Generalised = Sodium Valproate OR Lamotrigine

Neurosurgical Resection


Epilepsy

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

Contraception and pregnancy

Epilepsy Nurse Specialist


Epilepsy

ComplicationsSudden Unexpected Death in Epilepsy (SUDEP)

Status Epilepticus


Epilepsy

Discrete Plaques of Demyelination in Central Nervous System

T-cell Mediated


Multiple Sclerosis


Multiple SclerosisRisk Factors = Women, Temperate Areas

~30yrs

Demyelination Plaque (commonly periventricular, cervical spine, brain stem)Heals IncompletelyProlonged DemyelinationAxonal LossClinically Progressive Symptoms


Multiple Sclerosis

Clinical Courses•Benign•Relapsing Remitting•Secondary Progressive•Primary Progressive


Multiple Sclerosis


Multiple Sclerosis


Multiple SclerosisSymptoms

Monosymptomatic!

Disseminated in Time and Space


Multiple SclerosisOptic Neuritis: Decreased Visual Acuity, Pain on Eye Movement, Dyschromatopsia

Sensory: Lhermittes Sign, Limb Numbness/Tingling

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


Multiple SclerosisOther: Ataxia, Erectile Dysfunction, Urinary retention


Multiple SclerosisInvestigationDissemination in Time and Space!Clinically (attacks + clinical lesions, 2:2, 2:1, 1:1)+/- Aid of MRICSF (Oligoclonal Bands)Evoked Potentials


Multiple SclerosisManagementAcute: Methylprednisolone IV

Chronic: Interferon/Glatiramer, Natalizumab


Guillain-Barre SyndromeAcute AUI Inflammatory Demyelinating Polyneuropathy

Triggers: Campylobacter jejuni, CMV, Mycoplasma


Guillain-Barre SyndromeSymptoms4 wk peak!

•Weakness – Leg, Trunk, Respiratory. Proximal, Distal. Symmetrical.•Back/Limb Pain•Autonomic Features: seating, tachycardia, arrhythmia’s.


Guillain-Barre SyndromeSignsAreflexia!


Guillain-Barre SyndromeInvestigationsNerve Conduction Studies – slow conductionRegular FVC


Guillain-Barre SyndromeManagementIV Immunoglobin or Plasma Exchange


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

>40yrs.

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


Motor Neuron DiseaseKey FeaturesUMN + LMN SignsNo Sensory Loss/Sphincter DisturbanceNo Eye Involvement


Motor Neuron DiseaseClinical PatternsAmyotrophic Lateral SclerosisProgressive Bulbar Signs – CN IX-XII, bulbar/pseudobulbar palsyProgressive Muscular Atrophy – no UMN signsPrimary Lateral Sclerosis – mainly UMN


Motor Neuron DiseaseAssociationsFronto-Temporal Dementia (10-35%)!


Motor Neuron DiseaseSignsStumbling Spastic GaitFoot Drop +/- Proximal MyopathyWeak GripSpasticity/Hypereflexia/Upwards Plantars + Wasting/Fasciculations


Motor Neuron DiseaseInvestigationsMRI/LP/Neurophysiology – exclude other causes

ManagementRiluzole – prolong life by ~ 3monthsSymptomatic + MDT (Ventilation, PEG)


Parkinson's DiseaseDecreased Dopaminergic Neurons in Substantia Nigra (Pars Compacta)

Decreased Striatum Dopamine LevelsDecreased Basal Ganglia – Cortex CommunicationDecreased Movement


Parkinson's Disease~65yrs. Associated with Lewy Bodies


Parkinson's DiseaseClassic TriadTremor – resting, pill-rollingRigidity – increased tone, cogwheel rigidity, leadpipe rigidityBradykinesia – slowness of movement initiation-Expressionless Face, Monotonous Hypophonic Speech, Micrographia-Gait: Festinant, Reduced Arm Swing


Parkinson's DiseaseOther SymptomsAnosmiaDepressionSleep disturbancesVisual Hallucinations (animals, children)Dementia (late stage)


Parkinson's DiseaseManagementL-Dopa + Dopa-Decarboxylase Inhibitor (e.g. Madopar)-Efficacy reduces with time = Increased Dose-Dyskinesia’s, Off Freezing, End-of-Dose Reduced Relapse


Parkinson's DiseaseManagementDopamine Agonist – Ropinirole/PramipexoleApomorphine – potent DA agonist, acuteAnticholinergics – tremor

Deep brain stimulation


DementiaSyndrome 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%


DementiaTypesAlzheimer's DiseaseVascular DementiaLewry Body DementiaFronto-Temperal Dementia


Alzheimer's DementiaIncreased Beta-Amyloid Peptide = Progressive Neuronal Damage (hippocampus, amygdala, temporal neocortex)-Neurofibillary Tangles-Amyloid Plaques-Decreased Ach


Alzheimer's DementiaRisk FactorsFamily History, Downs Syndrome, Homzygosity for ApoE e4 Allele, DM/HTN/AF

Protective FactorsSmoking, Oestrogen


Alzheimer's DementiaSymptomsProgressive Global Cognitive ImpairmentAphasiaAnosognosia (lack of insight)IrritabilityMood Disturbance – Depression, EuphoriaBehavioural Change – Wandering, Aggression


Alzheimer's DementiaInvestigationsMMSE or Addenbrooks Cognitive ExamCT – temporal/parietal atrophy, ventricular enlargementMRI – hippocampus/amygdala/medial temporal lobe grey matter atrophyCSF – phosphorylated tau protein


Alzheimer's DementiaManagementAcetylcholinesterase Inhibitors (Donepezil, Rivastigmine, Galantamine) – help lay down new memories

Memantine (Antiglutamatergic)

BP Control


Vascular DementiaCumulative effect of many small strokes.

Vascular RF’s – Stroke Hx, HTN

Sudden Onset + Stepwise Deterioration -Emotional/Personality Changes-Cognitive Defecits-Depression/Labile Mood


Lewy Body DementiaFluctuating Cognitive ImpairmentVisual Hallucinations (animals/children) +/- ParkinsonismRepeated Falls/Syncope

CT/MRI – relative sparing of medial temporal lobeHistology – Lewy Bodies in Brainstem/Neocortex


Fronto-Temporal DementiaFrontal + Temporal Atrophy. AD Histology. Early, 45-65yrs

Behavioural/Personality ChangeDisinhibitionChange in Diet (sweets, overeating)Emotional BluntingPick bodies on histology


DementiaManagementCare CoordinatorCapacityDevelop Routines, Plan AheadChallenging Behaviour – Lorazepam, Risperidone (not Lewy Body!)Depression - Citalopram

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.

phase 2 kaveesha rajapaksa ryad chebbout the peer teaching society is not liable for false or...

Documents

misleading information

peer teaching society

management partial

partial seizures

complex partial

generalised seizures

parietal lobe

structural pathology