MSA Flashcards
What are the phenotypes of Multiple system atrophy
- Predominantly cerebellar involvement (up to substantia nigra)
- Predominantly substantia nigra (down to cerebellar)
Name the clinical features of MSA
Neurology - Parkinsonism, cerebellar features
ENT - stridor, dysphonia, dysphasia
Pneumology - aspiration pneumonia
Dermatology - hypohydrois / anhidrosis, vasomotor abs
Psychiatry - depression, anxiety
Sleep - REM sleep behaviour disorder, sleep apnea, restless leg syndrome
Cardiology - syncope, OH, postprandial hypotension, nocturnal hypotension
Gastroenterology - dysphasia, constipation, diarrhea
Urology - sexual dysfunction, urinary urgency and frequency, retention, nocturia
Diagnosis procedure of MSA
Generally poor diagnostic accuracy
LEVELS OF CERTAINTY:
1. Neuropathologically established MSA
- Clinically established MSA
- Clinically probable MSA
- Possible prodromal MSA
Supportive features:
Motor:
Within 3yrs of motor onset
- rapid progression
- severe speech impairment
- moderate to severe postural instability
Unexplained Babinski sign
Jerky myoclonic postural or kinetic tremor
Postural deformities
Non motor
Stridor
Inspiratory sighs
Cold discoloured hands and feet
Erectile dysfunction below the age of 60
Pathological laughter or crying
What is the diagnostic accuracy of MSA
Study : DLB, PD and PSP masquerade as MSA
Clinical diagnosed MSA: 134
MSA - 83
Non-MSA - 51 (DLB,PD,PSP, other)
MSA mimicking Parkinson’s disease or progressive supranuclear palsy
Natural history and progression of MSA
General findings:
Age onset - over 50
7r prognosis
Start with autonomic failure
Earlier AF, earlier mortality
Preclinical MSA
- no symptoms, positive biomarkers
Prodromal MSA
- autonomic sumps
- subtle Parkinsonism or cerebellar signs
- pyramidal signs
- stridor
- biomarkers
CLINICALLY DEFINED MSA
Early stage MSA (diagnosis)
- Parkinsonian, cerebellar and autonomic symptoms in different combinations (preserved functional independence)
Advanced-stage MSA
- severely impaired speech and swallowing
- difficulties in walking unaided
- postural instability and falls
Late stage MSA
- daily falls
-wheelchair bound
-severe OH and frequent syncope
- need for indwelling catheter
- loss of intelligible speech
- tube feeding
- institutional care
Premotor stage of MSA:
- RBD
- ED
- UD
- OH
- RD/stridor
Both MSA and pd experience (RBD) not a feature to define one from another.
Loss of time due to symptoms addressed as singularly and at specific clinics rather than neurologist.
MSA investigation methods:
Neuroimaging: FDG-PET scan
- cerebellum atrophy
- peduncle atrophy
^ non MSA specific, but not present in PD
MSA diagnostic criteria
- hyper metabolism in putamen nucleus, mesencephalic region and cerebellum (supportive for MSA-P)
Autonomic dysfunction
-multiple tests
Sphincter EMG
PET
- SN reduction in MSA. (Pattern does not distinguish disorder from others like PD)
What are the management types for MSA and why
Aim of management is to make people comfortable
Drug management is symptom specific
Parkinsonism:
L-dopa
Amantadine
OH:
Midodrine
Droxidopa
Sleep apnea:
CPAP
REM behaviour disorder:
Melatonin
Depression:
SSRI
Name some types of autonomic dysfunction testing
Cardiovascular
Standing test:
(OH with blunted heart-rate increase on standing)
24hr ambulatory blood pressure monitoring:
(Nocturnal hypertension)
Respiratory domain:
Polysomnography
(Sleep apnea)
Thermoregulatory domain:
Thermoregulatory sweat test
(Rapidly progressive failure of whole body sweating (differentiating MSA-P and PD)
What are the pathological findings of MSA
- a-synuclein positive inclusions
(Glia and oligodendrocyte inclusions)
2.cerebrum and cerebellum fibre loss
- Atrophy of areas different between phenotypes
