Winner of CoW 18 September 2016
MULTISYSTEM ATROPHY -C
Findings
Disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle) pons: hot cross bun sign –T2 hyperintensity in pons.
Discussion
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterised by varying degrees of cerebellar ataxia, autonomic dysfunction, parkinsonism andcorticospinal dysfunction Shy-Drager syndrome is used when autonomic symptoms predominate striatonigral degeneration shows predominant parkinsonian features olivopontocerebellar atrophy demonstrates primarily cerebellar dysfunction MSA has been divided clinically into two forms according to the dominant non-autonomic symptoms: MSA-C: predominance of cerebellar symptoms (olivopontocerebellar atrophy). MSA-P: predominance of Parkinsonian signs and symptoms (striatonigral degeneration). MRI FINDINGS T2: hyperintensities typically present in the pontocerebellar tracts pons: hot cross bun sign (MSA-C) middle cerebellar peduncles cerebellum putaminal findings in MSA-P : reduced volume reduced GRE and T2 signal relative to globus pallidus reduced GRE and T2 signal relative to red nucleus abnormally high T2 linear rim surrounding the putamen ("putaminal rim sign"), seen at 1.5T (this is normal at 3T) MSA-C disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle) ADC values: higher in the pons, cerebellum, and putamen than in Parkinson disease or controls fractional anisotropy (FA): lower in the pons, cerebellum, and putamen than in Parkinson disease or controls.
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Findings
Disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle) pons: hot cross bun sign –T2 hyperintensity in pons.
Discussion
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterised by varying degrees of cerebellar ataxia, autonomic dysfunction, parkinsonism andcorticospinal dysfunction Shy-Drager syndrome is used when autonomic symptoms predominate striatonigral degeneration shows predominant parkinsonian features olivopontocerebellar atrophy demonstrates primarily cerebellar dysfunction MSA has been divided clinically into two forms according to the dominant non-autonomic symptoms: MSA-C: predominance of cerebellar symptoms (olivopontocerebellar atrophy). MSA-P: predominance of Parkinsonian signs and symptoms (striatonigral degeneration). MRI FINDINGS T2: hyperintensities typically present in the pontocerebellar tracts pons: hot cross bun sign (MSA-C) middle cerebellar peduncles cerebellum putaminal findings in MSA-P : reduced volume reduced GRE and T2 signal relative to globus pallidus reduced GRE and T2 signal relative to red nucleus abnormally high T2 linear rim surrounding the putamen ("putaminal rim sign"), seen at 1.5T (this is normal at 3T) MSA-C disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle) ADC values: higher in the pons, cerebellum, and putamen than in Parkinson disease or controls fractional anisotropy (FA): lower in the pons, cerebellum, and putamen than in Parkinson disease or controls.
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Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!