Multi-System Atrophy Flashcards
what are the features of autonomic dysfunction
postural hypotension (dizzy, lightheaded, faint)
urinary frequency,
incontinence,
dysarytmias (palpitations),
sexual dysfunction (impotence)
bowel symptoms (constipation, diarrhoea)
problems with temperature regulation,
sweating
what is the prevalence of MSA?
1.9 to 4.9 per 100,000
what is the mean age of onset of MSA
58 years
mean survival of MSA is
7 to 9 years
what is the percentage distribution of MSA
MSA-P 58%
MSA-C 42% .
In japan 84% have MSA-C ,
equal female to male ratio
what are the principles of the MSA diagnostic criteria?
combination of clinical features of the disease and criteria, a feature (A) is characteristic of the disease, a criterion (B) is a defining feature
what are the diagnostic criteria for MSA- autonomic dysfunction
A)
(1) orthostatic hypotension
(2) urinary incontinence or incomplete bladder emptying
B: criterion: orthostatic fall of BP by 30 mmhg or 15mmhg diastolic
or urinary incontinence accompanied by erectile dysfunction
or both
what are the diagnostic criteria for MSA-parkinsonism
A)
1) bradykineasia,
2) rigidity,
3) postural instability,
4) tremor
b) criterion: bradykinesia plus at least one of items 2 to 4
what is the diagnostic criteria for crebellar dysfunction MSA
a)
1) gait ataxia,
2) ataxic dysarthria
3) limb ataxia
4) gaze evoked nystagmus
B) criterion: gait ataxia plus at least one of items 2-4
what are the diagnostic categories for MSA
possible MSA:
one criterion plus 2 features from separate other domains
PROBABLE MSA: criterion for autonomic failure plus poorly levadopa responsive parkinsonism or cerebellar dysfunction,
DEFINITE MSA: pathologically confirmed high density of glial cytoplasmic inclusions in association with degenerative changes in striatonigral and olivopontocerebellar pathways
what is the prevalence of MSA-C amongst ataxias?
familial ataxias: 31% sporadic ataxias 69%
MSA-C accounts for 9% all ataxias and 13% sporadic
what is the incidence of MSA-P amongst patients with idiopathic parkinsons disease
1.9 per 100,000 compared to 105-179 per 100,000 PD
what are the clinical features of MSA
partial or no response to L-dopa,
early instability and falls,
no dyskinesias with levadopa,
marked dysarthria early on particularly in MSA-C,
neck dystonia (anterocollis),
new snoring,
vivid dreams (REM sleep disorder)
sleep apnoea,
irregular jerky tremor or myoclonus in hands,
erectile dysfunction,
pathological laughter or crying
what are some non-supportive features of MSA
pill rolling,
peripheral neuropathy,
onset younger than 30 or over 75,
family history,
dementia/cognitive decline,
vertical supranuclear gaze palsy (PSP)
what investigations can be useful for MSA?
autonomic function tests (although sometimes not necessary),
clinical history most useful,
other tests:
sphincter EMG,
24hour BP and HR,
SPECT and PET looking at postsynaptic D2 receptors (lost in MSA)
MRI useful in diagnosis (hot cross bun) MR spectroscopy
