More neurological conditions Flashcards
what are the major categories of neurological disease and how can you differentiate them
- infectious
- traumatic
- congenital/genetic
- vascular
- degenerative
- toxic
- metabolic (acquired/inherited)
- neoplastic
- inflammatory/immune
Degenerative disease – slow - yrs
Vascular and trauma are fast
Inflammatory/autoimmune/neoplastic – subacute – mo
demographic
where is the problem if you have UMN signs
From cortex to subcortex to pyramidal decussation to spinal cord
distinguishing factors for facial weakness
UMN = forehead sparing - bihemispheric innervation of both nuclei for the frontalis muscle
Bell’s - eye cant open properly, white of eye seen when try and open it
where does the facial nerve sit
in the pons
summarise the visual pathway
doesn’t go through the brainstem, it goes above it
optic nerve -> optic chiasm -> radiation -> occipital lobe
Area of hypodensity = infarct
Middle cerebral artery
L middle cerebral artery territory infarct.
aphasia
expressive - cant produce the word they want to produce.
Receptive – cant understand what other people are saying to them ie cant follow commands
dx
MND
what is MND
degenerative condition - anterior horn cells and upper motor neurons in spinal cord = mixed UMN and LMN signs
cause unknown
10% familial
epidemiology of MND
inciddence 2-4/100000
50-70yrs (can be any adult age)
presentation of MND
insidious onset (nearly always missed)
motor nerves only affected - UMN and LMN (can be just one initially, other features develop with time)
progressive
amyotrophic lateral sclerosis
typical presentation of MND (60%)
one limb initially - foot drop, clumsy weak hand
some wasting
fasiculations, brisk reflexes, extensor plantar responses
no sensory signs
progression over weeks/mo
bulbar MND
dysarthria and dysphagia - 30% of cases
bulbar and pseudobulbar sx
dysarthria -> tongue wasting
fasiculation
brisk jaw jerk
weakness of neck muscles
may be limb sx
progression more rapid than ALS
Px = 2yrs until death
where are the cranial nerves
At top of spinal cord have the medulla (bulbar) – in here have the CN 12 11 10 and 9
Pons – 5 7 6 8
Midbrain – 3 and 4
Above that – optic nerve and visual pathway and olfactory pathway.
difference between bulbar and pseudobulbar
bulbar - function in mouth and oropharynx eg dysarthria/dysphagia, absent gag reflex, tongue fasiculations, absence of jaw jerk
pseudobulbar - UMN bulber problem = dysarthria/dysphagia spastic tongue, exaggerated gag reflex
other MND presentations
primary lateral sclerosis - 5% - pure UMN onset
progressive muscular atrophy - 5% - pure LMN onset - flail limb appearance
Dx of MND
clinical - look for fasiculations
EMG/NCS - chronic nerve root denervation
imaging (MRI) - exlude other causes eg cervical/lumbar degenerative changes and myelopathy - cervical/thoracic/lumbar spine (brain sometimes)
exclude:
benign fasiculation syndrome
bulbar - cerebrovascular disease
multifocal motor neuropathy
hereditory spastic paraplegia
Mx of MND
no cure
riluzole - glutamate antagonist - extend LE (3-5mo)
palliative
nutrition
soft diet - excessive secretions/salvation
opiates/benzodiazepines
dx
MS
definition of MS
evidence of damage to the CNS that is disseminated in time and space
damage occurs at different dates and to different parts of the CNS
pathophysiology of MS
immune mediated
- T cell trigger attacks on myelin in CNS (inc optic nerve)
- scarring caused by inflammatory attacks at multiple sites in CNS
- plaques visible on MRI and autopsy
genetic predisposition with environmental triggers
- 20% MS sufferes have blood relative with MS
- low vit D, ENBV, smoking, extremes of latitude, obesity in adolescence
MS sx and signs
vary between people
- fatigue
- visual problem
- bladder and/or bowel dysfunction
- sexual dysfunction
- emotional disturbances - depression, mood swings
- cognitive difficulties - memory, attention, processing
- sensory changes - tingling, numbness
- pain - neurological, musculoskeletal
- spaticity
- gait, balance, and coordination problems
- speech/swallowing problems
- tremor (if cerebellar effected)
presentation of MS
Some presentations short lived – weeks/mo – vision loss/numbness – patient doesn’t report, battle between immune response and recovery.
Some get single episode of inflammation and some more progressive
loss of vision in 1 eye, with painful eye movements (optic neuritis)
double vision - brainstem syndromes
sensory disturbance or weakness, spastic parapesis, weakness and gait, increased tone and clonus, hyperreflexia -spinal cord lesion, transverse myelitis
balance related problems or clumsiness - cerebellar
altered sensation travelling down back when bend neck forward - Lhermitte’s syndrome
Dx of MS
clinical
PMHx and Ex
evidence of damage disseminated in time and space
McDonalds critera - more reliant on MRI appearances of plaques
- MRI - hyperdense plaques - Pathognomonic in corpus collosum
- LP - in CSF see abnormal Ig present, or immune response in the CSF
- visual evoke potentials - optic nerve slowed down by loss of myelin
clinical course of MS
in young adults <40yrs
4 patterns of disease:
- relapsing remitting - most common, gets better but return to worse baseline
- primary progressive - worse Px
- secondary progresive
- progressive-relapsing remitting MS
Mx of MS
neurology annual review
MDT
look for incection in relapse - IV or PO steroids
disease modifying drugs eg B interferon when not in relapse as prophylaxis
dx of parkinson’s
clinical
summarise parkinson’s
idiopathic
substantia nigra degeneration -> dopamine deficiency in striatum/basal ganglia -> motor sx = abnormality of movement, not loss
basal ganglia fasicilitates movement normally - extrapyramidal tract
dopaminergic therapy relieves motor sx
3 cardinal sx of parkinson’s
tremor - high frequency resting tremor (5movements/sec) - pinrolling (finger and thumb), or supination-pronation
rigidity - different from increase in tone/spasticity in UMN. In spasticity – tone more notable in particular dirn. Parkinsons – leadpipe rigidity – equal in both directions.
Cogwheel rigidity – tremor superimposed on the tone.
Bradykinesia – don’t blink very often, paucity of movement, gait. Get them to tap fingers and see if it is slow
features of parkinsons
Freeze when mobilise
Shuffling hait
Poor arm swing on one side
Poor posture
Problem swallowing postural hypotension, bowel and urinary function
Hypophonia
Need 2/3 of bradykinesia, tremor, rigidity
causes of parkinsonism
parkinson disease - idiopathic or genetic
parkinson-plus degenerations
drug induced parkinsonism
vascular parkinsonism
brain trauma
CNS infection
parkinson-plus degenerations
progressive supranuclear palsy - impaired gaze (cant look down), balance and dysarthria
multiple system atrophy - autonomic issues, urinary problems and orthostatic hypotension
drug induced parkinsonism
anti-dopaminergics
drugs that reduce dopamine transmission - antipsychotics/antiemetics:
- resperidone
- haloperiodol
- metoclopramide
ways to differentiate Parkinson’s disease from other causes of parkinsonism
asymmetrical findings
no atypical features - eye fine, automomic shlouldnt be the predominant sx
not on/had neuroleptics
responds to L Dopa
Mx of Parkinson’s disease
L-dopa (with carbidopa) - most effective and best tolerated
dopamine agonists - ropinirole, pramiplexole
MAO-B inhibitors, anti-cholinergics, amantadine have modest benefits
drugs are for sx relief, not neuroprotective/neurotoxic
response to L dopa is best diagnostic test for PD
L-Dopa SE
non-motor:
- nausea
- orthostasis
- sleepiness
- hallucinations
dyskinesias
Mx of PD as disease progresses
more motor complications as disease progresses - including dyskinesias and on-off fluctuations
drug resistant motor sx - impaired balance with falls
non-motor sx - dementia and hallucinations
hemiplegic
typical of pyramidal weakness from stroke or UMN problem
Corticospinal tract affected – reflex arc exaggerated and out of control – spasticity in pyramidal pattern
Everything flexed in upper limb and extend in lower limb
swing leg out to prevent the foot drop
Hemiplegic stroke – extended position and arcs outward
highstepping gait
foot drop - lift foot high so dont rip
stomping gait
sensory problem – have to stomp so you get vibration – peripheral neuropathy
Hoffman’s sign
antalagic gait
painful leg - compensate by lifting leg up a bit
L occipital lobe = R sided homonymous hemianopia
sx to check for with visual field defects
clarify field of vision loss in transient sx
any positove phenomona
any associated sx
dx
MS – internuclear ophthalmoplegia (INO) – brainstem and medial longitudinal fasiculus – associated with MS.
R eye didn’t adduct, L eye looked like it had nystagmus. Relate to the midbrain and pons – plaque occur in MS
BPPV
Very short lived period of vertigo, related with head movement.
Manouvre – Dix-Hallpike gold standard test
Epley manouvre to make them feel better
fatiguability
Myasthenia gravis
Eye becomes misaligned with other eye = squint – fatiguability
Diplopia in evening
And speech in consultation
summarise myasthenia gravis
autoimmune synapto-pathy affecting NMJ of skeletal muscle
affecys ocular, face and swallowing
sx and signs worse with use - fatiguability
NCS and Ab testing - AChR and MUSK Ab test
CXR - thyoma, ice test
Mx for myasthenia gravis
ACh inhibitors
immunosuppresssion
plasmapharesis and IVIG
surgery? thymectomy
R middle cerebral artery stroke
Cerebral met
Think about the mode of onset
SAH – all or nothing event – thunderclap headache
TIA – resolves in <24hrs
Cerebral infarct – wouldn’t progress – over mo language would get better.
R brainstem:
Not cervical spine because face problem – and tracks above this
Not occipital – because would be vision of R of both eyes
R motor cortex – L sided face weakness
Have crossed signs here – face not equating to the body
Very typical for brainstem - Nucleus on one side, then pyramidal decussation
spinal cord
T10
GBS
Ascending motor radiculopathy
Peripheral = areflexia
Contact the stroke team – do scan where pt get treatment
L cerebellum – cerebellum is ipsilateral innervation – L sided coordination.
