Neuro Flashcards
What disease causes a rapidly progressive decline and what other features would you see?
Creutzfeldt-Jacob disease
prion infection causing spongiform encephaopathy
causes rapidly fata demetia - death within 1 year
myoclonic jerks and extra-pyramidal signs
causes - sporadic, infected hosipital infection, familial, blood transfusions in 1995
What is Huntington’s disease?
autosomal dominant disease with 100% peetrance - trinucleotide expansion repeat of CAG.
symptoms - cognitive decline –> progresses to subcortical dementia, personality change, choreinform involuntary movements, dysarthria, psychiatric disturbance.
genetic test - children must wait until old enough to decide
What is the pathophysilogy of HD?
reduced GABA (reduced inhibition) causing dopamine hypersensitvity and increase in dopamine transmission increased stimualtion at thalamus and cortex --> involuntary movements
Name 2 medical conditions that can cause psychiatric symptoms?
neurosyphilis - grandiosity, euphoria, mania, personality change
Wilson’s disease - copper excess leading to both neuro and psych changes, liver disease and kayser-fleischer rings
Name a potentially reversible cause of dementia?
Normal pressure hydrocephalus
triad of - ataxia, dementia and urinary incontinence
causes - idiopathic, SAH, head trauma, meningitis
tx with ventriculoperitoneal shunt
What are the features of an ataxc gait?
wide-based
falls
cannot walk heel-to-toe
often worse in the dark or with eyes closed
What are the 2 main causes of an ataxic gait?
- cerebellar problem
2. issue with proprioception
What are the cerebellar causes of an ataxic gait?
MS
posterior fossa tumour
alcohol
phenytoin toxicity
deficits are ipsilateral to cerebellar lesion
What are the features of cerebellar syndrome?
ataxia + nystagmus
What are the proprioceptive causes of an ataxic gait?
sensory neuropathies - low B12
inner ear problems affecting the vestibular system
How do you distinguish between a cerebellar and proprioceptive cause of ataxic gait?
walk normally with eyes open, problems start when eyes closed –> proprioceptive
problems exists all the time –> cerebellar
What are the features and causes of a circumduction (spastic) gait?
features - stiff gait, circumduction of the legs +/- scuffing of the toe of the shoes
cause - strone (hemiplegia)
What are the features and causes of a shufflinf (extra-pyramidal) gait?
features - flexed posture, shuffling feet, postural instability, slow to start
cause - Parkinson’s diease, PD+ syndromes, other causes of Parkinsonism such as antipsychotic medications
What are the causes of an antalgic gait?
AKA limping
MSK cause - painful limb
What are the features and causes of high stepping gait?
features - trip over often, struggle with dorsiflexion of the foot, lift ffeet high whilst walking to avoid tripping over
cause - foot srop (common peroneal nerve pasly)
What are the features and causes of Trendelenberg gait?
Features - unstable hip, sound side sags on Tredelenberg test
Causes - congenital hip dislocation, DDH, gluteus medius muscle weakness, superior gluteal nerve damage
What are the features and causes of an apraxic gait?
features - glued to the floor when attempting to walk, wide based unsteady gait with a tendency to fall (novice on ice)
causes - normal pressure hydrocephalus, multi-infarct states, Alzheimer’s disease
How does the onset of an episode of weakness help you assess the cause of it?
Sudden onset - likely to be a vascular event
medium onset - likely to be related to demyelination
insidious onset - slow-growing tumours etc
What are the patterns and distributions of muscle weakness?
- proximal weakness - muscle problem - hair, chair, stairs - struggle to do things close to their trunk
- distal weakness - nerve problem - nueropathy starts distally and works its way up - glove and stocking distribution
- symmetrical - genetic or metabolic cause eg. diabetes, muscular dystrophy
- asymmetrical - vasculitis or inflammatory
- mononeuropathy - entrapment
- polyneuropathy - systemic like diabetes
What are the features of peripheral neuropathy?
chronic and slowly progressive
length-dependent
sensnory, motor or both
glove and stocking distribution
How does mononeuritis multiplex present and what are some causes?
individually erves picked off randomly - wrist drop, leg numbness, foot drop
subacute presentation (months) inflammatory/immune-mediated
causes - inflammation of the vasa nervorum can block off the blood supply to the nerve causing sudden deficit
vasculitis (Wegner’s, PAN, RA), sarcoidosis
Give some examples of entrapment mononeuropathies.
Median nerve at wrist = carpal tunnel syndrome
ulnar nerve at elbow
radial nerve at axilla
common peroneal nerve in leg
What causes a myasthenic crisis?
infection
natural part of the disease
under or overdosing of medication
How should a myasthenic crisis be treated?
urgent neuro review
monitor breathing - serial FVC measurements
anaesthetic review