NEURO - MND/Dementia/Neuropathy/Myopathy Flashcards
What is motor neurone disease?
Degenerative disease of the UMN/LMN of SC/CN + cortex
Which aspect is NOT involved in motor neurone disease?
Sensory
What are the 4 types of MND
ALS - Amlyotrophic lateral sclerosis
Progressive muscular atrophy
Primary lateral sclerosis
Bulbar
Which is the most common type of MND?
ALS
What parts of the nervous system are affected in ALS
Loss of Spinal and brainstem LMN
Loss of cortical UMN
Sx ALS (3)
LMN weakness - starting in hands + progressing to arms/legs
UMN spastic weakness - starts in legs –> arms
+/- Bulbar/pseudobulbar palsy
O/E ALS (4)
Mm wasting
Fasciculations
Brisk reflexes
Increased plantars
Features progressive muscular atrophy MND (3)
Loss = RESTRICTED TO SPINAL LMN
Hence
LMN signs only
wasting + fasciculations
Features - primary lateral sclerosis MND (2)
Loss RESTRICTED TO CORTICAL UMN
Hence only UMN signs
Features - bulbar MND
Bulbar Sx w/ PRESERVATION LIMB FCT
Why is the prognosis for bulbar MND so poor?
Early respiratory involvement
Causes of death MND (2)
Pneumonia
Ventilatory failure
Dementia - criteria for diagnosis
Impairment memory in 1+ of:
Language impairment
Apraxia (motor)
Agnosis (can’t interpret senses)
+
Impairment of fct
Present for at least 6 months
No other medical/physical causes
Modifiable RF dementia (6)
Smoking Atherosclerosis Alcohol Cholesterol Obesity Low education
Non-modibiable RF dementia (3)
Genetics - APP/PSEN 1/2
AGE
MCI
macroscopic changes - Alzheimers (2)
Atrophy of the hippocampus
Enlarged ventricles
Microscopic changes ALzheimers (2)
Decr NT function
Abnormal protein depositon –> tangles + plaques
Which 3 genes have been associated with early onset alzheimers
APP
PSEN-1
PSEN-2
Which gene has been associated with late onset Alzheimers
APOE
Subtypes of vascular dementia (3)
Multi-infarct
Subcortical vascular dementia
Post stroke
What is multi-infarct vascular dementia
Cortical vascular dementia from small strokes in the cortex
What is subcortical vascular dementia
Small vessel deep damage
RF vascular dementia (4)
Smoking
High fat diet
Diabetes
HTN
LB Dementia pathology
Lewy bodies build up in the cortex and basal ganglia -> disruption NT function
Sx LB dementia (3)
Fluctuations in cognition
Visual hallucinations
Parkinsonism
Difference between Parkinsons dementia and LB dementia
LBD - cognitive dysfunction within 2 years of motor Sx
PDD - presents later
What % of frontotemporal dementia is though to be inherited
50%
Frontotemporal dementia Sx
Changes in behaviour emotion and language
MCI
Impairment of memory more than that of expected age but not enough to affect function
Does having MCI put you at greater risk of developing dementia?
Yes
What is dysphasia
Cant find words
What is agnosia
Difficulty recognising faces
Early Dementia Sx (3)
Memory loss
Slow to grasp new ideas
Errors of judgement
Mild-Mod dementia Sx (3)
Difficulty w/ daily tasks
Decline in language
Behaviour change
End stage dementia Sx (4)
Increased need of care
COmmunication impaired
Incontinence
Swallowing difficulty
Ix dementia
FBC B12/folate ESR LFT U+E TFT Glucose Lipids ECG CT = 1st line 2nd line = MRI or HMPaO-SPECT
Drugs for Tx alzheimers dementia
ACEase inhibitors - rivastigmine
NDMA antagoists - memantine
Can you Tx vascular dementia w/ alzheimers meds
No
Use of antipsychotics in dementia
FOr behaviour and psychological Sx
Radiculopathy
Process affecting nn roots
What is a neuropathy
Peripheral nn affected
What is mononeuritis multiplex
Many single nn affected
4 most common causes of neuropathies (4)
DM
Carcinomatos neuropathy
Vit B defic
Drugs
Other caused neuropathies
Folate/thiamine deficiency Uraemia in CKD Alcohol/Pb RA/CT or myxoedema G-B, CIPD Syphillis/ HIV (infective) Vascular Neoplasm
Drugs causing neuropathies (3)
Amiodarone
Statins
Hydralazine
Inherited neuropathies (2)
Charcot Marie Tooth
Freidrich’s ataxia
Charcot Marie tooth
Before 20
W/ progresive neuropathy
Typically affecting hands + feet 1st
Features of Freidrich’s ataxia
Cerebellar ataxia in the 1st decade
UMN limb signs
Peripehral neuropathy
How are demyelinating vs axonal neuropathies differentiated?
Nn conduction studies
Demyelinating neuropathie
Damages spare axons
Affects swann cells
–> decreased conduction velocity
E.g. demyelinating neuropathy
GBS
Common in immune-mediatedidsease
Prognosis demyelinating neuropathy
Schwann cells can regrow hence improve w/ Tx
Axonal neuropathy
.Nn cell bodsies untable to maintain long axonal processes
Degenerating starts in the periphery ==> neuronal cells
Leading to decrease amplitude of impulses
Prognosis of axonal neuropathy
Axons cant regrow
Hence poor outcome
Wallerian degeneration
Aftect nerve section or microinfarction
Compression neuropathy
Leads to focal demyelination
Nn infiltration
Malignant or granulomatous infilatration
Ix neuropathies (7_
FBC U+E LFT Glucose B12/folate Autoantibodies Nn covnduction studies LP - incr GBS/CIDP Peripheral nn biopsy
What is the most common acute polyneuropathy
GBS
Is GBS demyelinating or axonal
Demyelinating
How long after 1’ infection does GBS occur
3 ish weeks
PS GBS (4)
Ascending paralysis
Loss of reflexes
Affecting lower limbs
Progresses over several days to weeks
Complications of GBS (3)
VTE
BP irregularities
Arrhythmias
Mx GBS (5)
Admit to ITU/HDU Monitor FVC reg Ventilatory support SC heparin + TEDS High dose IVIG within 2w
What % GBS make a complete recovery over months
90%
Mortality rate in acute phase GBS
10%
What is shingles
Reactivation of VXV within dosral root ganglion
Which dermatomes are most commonly affected by shingles
Low thoracic
What is Ramsay Hunt syndrome
infection of geniculate ganglion –? facial palsy, facial/ear pain + vesicules in ear canal/pinna + soft palate
Mx Shingles
Aciclovir 5-7days
Paracetamol/amitriptylline - pain
What is post-herpetic neuralgia
Pain in a previous shingles zone
Mx post-herpetic neuralgia
Amitriptylline
What is myasthenia gravis
Disorder of neuromuscular transmission resulting from autoantibodies to components of the NM junction
Which receptor is most commonly affected in myasthenia gravis?
Acetylcholine receptor
PS myasthenia gravis (6)
Mm weakness + FATIGABILITY - gets worse throughout day
Fluctuating proximal weakness (> upper limb)
Symmetrical diplopia + ptosis
Dysphagia + speech difficulties
Wasting + resp difficulties after many years
What is preserved in myasthenia gravis
Tendon reflexes
Heart also not affected
What are the 3 types of muscle disease
Muscular dystrophies
Myopathies
Neurogenic mm diseases
DMD genetics
X linked recessive
mutation in dystriphin gene - mm breaks down
Features DMD (3)
Global weakness
Calf pseudohypertrophy
Gowers sign
Ix DMD
CK increased
What is Becker Muscular dystrophy
Milder + better prog version of DMD
Gene issue Becker Muscular dystrophy
Partially functioning dystrophin
Prognosis Becker Muscular dystrophy
40s
Myotonic dystrophy genetics
AD
What is the issue with in Myotonic dystrophy
Cl channelopathy
PS Myotonic dystrophy
Mm weakness
Myotonia - can’t relax
Ix mm disease
CK
Electromyography
Mm biopsy
Spinothalamic tract - test (2)
Pain w/ neurotip
Temp - cool and warm object
Dorsal column - test (3)
Vibration - tuning fork 128hz
Joint proprioception - joint movement
Light touch - cotton wool
Pyramidal pathway - anatomy
Pathways from cerebral cortex to mm
Physiological movement - pyramidal vs extrapyramidal
pyramidal = voluntary extrapyramidal = involuntary
Pathological movements in pyramidal pathology (4)
Paralysis
Hyperreflexia
Spasticity
Paresis
Pathological movements in extrapyramidal pathology (5)
Chorea Dystonia Myoclonus Tremor Akathisia
