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Neurodegenerative Disease - Coggle Diagram
Neurodegenerative Disease
Dementia
Aquired global impairement of intellect, memory and personality
Impairement of intellectual function in the presence of nrmal conciousness affecting
Language
Visuospatial skills
Emotion or personality
Cognition
Presentation
Progressive memory dysfunction
Stepwise neurological decline
Behavioural / personality changes
Language dysfunction
Weakness , spasticity
Movement disorder
Investigations
Histopathology
Amyloid plaques
Neurofibrillary tangles
Cerebrovascular disease
TSP-43
Tau
Lewy bodies
Imaging
MRI
Volume loss
Signal Change
Blood flow abnormalities
Types
Alzheimer's Disease
Vascular Dementia
Lewy Body Dementia
Frontotemporal dementia
Creutzfeldt–Jakob disease
Host Risk Factor
Age
Education
Genetics
Vascular risk factors
ApoE ε4
Diabetes
Parkinson's Disease
Cardinal Features (3)
Bradykinesia
Resting tremor
Rigiditiy
Clinical diagnosis requires 2/3 cardinal features
Presentation
Bradykinesia
Resting tremor
Rigidity
Autonomic dysfunction
Cognitive disturbance
Dysphagia
Pill rolling finger movements
Shuffling gait
Poor balance
Dribbling
Expressionless face, flat soft voice
Mneumonic
Parkinsons
TRAPSS
the body
T - Tremor (pill rolling)
R - Rigidity (cogwheeling)
A - Akinesia / bradykinesia
P - Postural instability
S - Shuffling gait
S - Small handwriting (micrographia)
Vascular Dementia
Epidemiology
Elderly
Synergistic with other forms of dementia
Presentation
Impact on cognition unclear
Rarely the equivalent of clinical dementia in post mortem studies
Causes
Atherosclerosis
Microvascular diseases
Reduced CBF
Oxidative stress
Blood brain barrier dysfunction
Endothelial disorders
Alzheimer's
Presentation
Dominant problems in
memory
Problams in word retrieval
Perceptuospatial and constructional difficulties
Preserved social skills
Investivations
Imaging
MRI
Marked atrophy of hippocampus
Pathology
TAU
Ba4
Staging
ABC Score
A - Amyloid
B - Braak and braak staging / Tau deposition
C - CERAD Plaque scoring, correlate with age
Genetics
Early Onset
Chr1 PS2
Chr14 PS1
CHr21 APP
Late Onset
Chr19
APOE
Chr6 TREM2
Lewy Body Disease
DLBL
Presentation
Progressive cognitive decline
Repeated falls
Syncope
Neuroleptic sensitivitu
Systematised delusions
Hallucinations
Diagnostic Criteria
Progressive cognitive decline
Plus 2/3:
Fluctuating cognition
Recurrent visual hallucinations
Spontaneous motor features of Parkinsonism
Frontotemporal Dementia
Presentation
Breakdown in social behaviour
Breakdown in exceutive functions
Preserved perceptuospatial skills
Patients with progressive frontal lobe dysfuntion
Spongiform Encephalopathies
KURU
Infection
Gerstmann–Sträussler–Scheinker syndrome (GSS)
Fatal Familial Insomnia
PRP mutation
Creutzfeldt–Jakob disease
Classification
Definite sporadic
Probable sporadic
Iatrogenic
Familial
GSS
Presentation
Dementia
Characteristic ECG changes
sCJD vs nvCJD
Motor Neuron Disease (MND) /
Amyotrophic Lateral Sclerosis (ALS)
Presentation
Generalised wasting and fasiculation
Bulbar muscle involvement common
Associated upper motor neurone symptoms and signs
Steadily progressive and fatal
Symmetrical and proximal
Genetics
Cu/Zn superoxide dismutase gene on Ch 21q
accounts for 25% of all familial cases
Peripheral Neuropathy
Causes
Deficiency
Vit B1 in alcoholics
Vit B6 in pts taking isoniazid
Vit B12 in pts with PA and bowel disease
Toxic
Alcohol
Drugs - Isoniazid, Vincristone
Metabolic
DM
CRF
Post-infection
Guillain-Barre syndrome
Collagen Vascular
Rheumatoid arthritis
Systemic lupus erythematosus
PA
Hereditary
Charcot-Marie
Tooth disease
Idiopathic
50% of cases
Presentation
Asymmetrical and Distal
Guillain-Barre Syndrome
Causes
Post-infection
CAmpylobacter
Cytomegalo virus
EBV
Autoimmune Demyelinating polyneuropathy and polyradiculopathy
Progression
Rapid evolution over several days
Life threatening weakness
Occurs within 2 weeks of infection
Weakness and sensory symptoms worsen daily for 1-2 weeks
Presentation
Paralysis of acute onset
Acending and symmetrical paralysis
Myasthenia Gravis
Presentation
Muscle weakness without wasting
Fatiguability
Ocular and bulbar muscle involvment
Responds well to treatment
Pathogenesis
Autoimmune degeneration of motor end plate
