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CNSLF Path Neurodegeneration (ii) (MND (aka ALS - amyotrophic lateral…
CNSLF Path Neurodegeneration (ii)
Delirium
acute disorder of attention + global cognition
reversible by txing underlying cause (unlike dementia which is irreversible progressive decline in @ least 2 cognitive areas)
Investigations
excl acute cause of cognitive decline e.g. infection
detailed dx + cognitive assessment to identify affected cognitive domains
MMSE
Montreal cognitive assessment +/- psych evaluation
excl metabolic cause
TFTs
B12
U+E
LFTs
autoab workup (vasculitis)
brain imaging
CT, MRI +/- PET
exl other causes e.g. stroke + assess distribution of any atrophy
rarely brain bx to identify cause
vasculitis
lymphoma
Alz
macroscopy: atrophy due to neuronal loss, eso medial temporal lobe (esp hippocampus), cortex affected
microscopy: tau + amyloid (neurofibrillary tangles + amyloid plaques)
early signs + symptoms
memory loss esp recent events
language problems, difficulty finding the right word
changed mood/behaviour
confused
difficulty managing money + everyday tasks
difficulty problem solving (e.g. doing puzzles)
loss of interest in hobbies, lack of initiative
repeating Qs/stories without realising
PD
3 cardinal features
bradykinesia
resting tremor
rigidity
+/- autonomic dysfunction
cognitive decline
dysphasia
macroscopy: pale substantia nigra
microscopy: alpha synclein, Lewy body
MND
aka ALS - amyotrophic lateral sclerosis
neurodegeneration of UMNs + LMNs (brain, cord, N)
clinical features = mixture of UMN (spasticity, hyperreflexia) + LMN (fasciculations) symptoms + signs, incl bulbar (e.g. swallowing)
mean age of onset = 60yrs
progressive motor failure incl resp failure which is cause of death
median survival = 3-5 yrs
microscopy: ant horn cell protein TDP43
macroscopy: corticospinal tract degeneration, N root atrophy