Alien hand is seen in
a. Cortico Basalganglia degeneration.
b. Non dominant Middle cerebral artery infact.
c. Multi system atrophy.
d. Normal pressure hydrocephalus.
Oh no!! I think I didn't need to go that far!! See what our Harrison's says:
Alien limb phenomena is a characteristic sign present in many cases in CBD..
Something more on CBD..
C/F:
6th-7th decade
insiduous onset
asymmetric progressive apraxia, rigidity, dystonia, bradykinesia..
myoclonic jerks
may be cortical sensory loss
Alien limb phenomena
progresses to become bilateral in 2-5 yrs------> paraplegia in flexion
may present with
frontotemporal dementia, progressive aphasia f/b asymmetric corical sensory signs, including abnormalities of graphaesthesia & astereognosis
Imaging:
MRI: focal cortical loss in c/l superior frontal & parietal lobes, hyperintense signal abnormalities in white matter & atrophy of corpus callosum.. (atrophy of corus callosum plays important part in alien hand phenomenon)
Yea parin.
U r correct...
Good explanation of urs..
Here is the expantion of ur summary from harrison...
Corticobasal Degeneration (CBD)
CBD, another sporadic tauopathy, is less common and has a broader range of clinical presentations than PSP25. As with most atypical forms of parkinsonism, it begins insidiously in the sixth to seventh decades with varying degrees of asymmetric progressive apraxia, rigidity, dystonia, bradykinesia, and myoclonic jerks with or without cortical sensory loss. Alien limb phenomena is a characteristic sign present in many cases. The disorder progresses to become bilateral over 2 to 5 years, leading to total incapacity with, ultimately, paraplegia in flexion. A significant number of cases present with frontotemporal dementia or progressive aphasia, followed by asymmetric cortical sensory signs, including abnormalities of graphesthesia and astereognosis (Chap. 350). Brain MRI26 reveals focal cortical loss in the contralateral superior frontal and parietal lobes with corresponding hypometabolic changes on PET27 scan, as well as hyperintense signal abnormalities in white matter and sometimes atrophy of the corpus callosum. Treatment is largely ineffective.
Grossly, CBD28 is a focal cortical degenerative process with asymmetric Pathology
and volume loss in the parietal and frontal regions. Most of the damage is in the dorsal peri-Rolandic, superior frontal, and superior parietal cortices, whereas cases with aphasia show abnormalities in the peri-Sylvian regions. Histologically, gliosis and swollen (ballooned) achromatic neurons and neuronal loss are present in these cortical regions as well as in the nigra, caudate, putamen, and thalamus. Recent clinicopathologic evidence indicates the syndrome can occur in the absence of basal ganglia or nigral degeneration.
|
Topic Tags: 
