NABOKV-L post 0013594, Sat, 14 Oct 2006 10:21:51 -0400

Subject
Re: Cerebral sclerosis and a bit of neuroanatomy
From
Date
Body
I'm neither a neurologist nor a physician yet, but am working on it - so

I may be able to help with the cerebral sclerosis / neuralgia questions.

Schilder's cerebral sclerosis is also known by many of the names already

given, perhaps most commonly as diffuse sclerosis, but is not the same
as tuberous sclerosis - it's closer to MS or forms of encephalomyelitis.

Cerebral sclerosis is a strange condition because it's not clear whether

it is actually a disease in its own right, or just a category into which

lots of people with other conditions were lumped before we could
diagnose them properly. There are only a hundred or so cases in the
literature from 1913 or so to now (which may make Shade's doctor less
culpable for going for neuralgia than on a first reading?), and a review

of these (CM Poser, Diffuse-disseminated sclerosis in the adult. J
Neuropathol Exp Neurol. 1957 Jan;16(1):61-78. PMID 13398842) suggested
only nine of them could definitely be called cerebral sclerosis, and 70%

of the rest were actually MS. As things stand, therefore, it still
exists as a clinical entity, but not one you'd want to diagnose in a
hurry.

The presentation - in as much as we can draw conclusions from nine
confirmed cases - involves headache, malaise, and fevers as a prodrome.
These tend to come on over weeks, but can can come on abruptly on the
back of an infectious illness. When the condition kicks in properly, you

can get a wide variety of neurological abnormalities, although seizures
are described as "not common". Interestingly some patients manifest
psychosis, and memory problems, slowed mental processes, irritability,
confusion, disorientation, and personality and behavioural changes are
relatively common.

Diagnosis at the moment (the most recent case in the literature is 2002,

then 1994 prior to that) is by MRI scan of the head and biopsy, so if
the question is "could Shade have had it", the answer is "just
possibly", but for the reasons above coupled with Matthew Roth's
reasonable suggestion that Shade's dormant period between fits is too
long, I'd suggest no one in their right mind would diagnose him with
it...

Sticking with Matthew's post, there are various lemnisci in the brain,
but (brutally denying poetic licence on neuroanatomical grounds) none
would be affected in cerebral sclerosis, as they're all (second-order)
neurones which connect the spinal cord (medial lemniscus) or the
trigeminal nuclei (trigeminal lemniscus) to the thalamus, or the
cochlear nuclei to the inferior colliculi (lateral lemniscus). Because
the lesions of cerebral sclerosis are subcortical, they'd be "above" the

thalamus. All this neuroanatomy, of course, doesn't remove the
connection between "lemniscate" and the brain.

Personally, my gut feeling would be not to go into that much detail and
to chuck out the cerebral sclerosis theory on the grounds that it's so
rare - but it isn't impossible and it is mentioned specifically in the
text - so who knows?

Nick Grundy

Search the archive: http://listserv.ucsb.edu/archives/nabokv-l.html
Contact the Editors: mailto:nabokv-l@utk.edu,nabokv-l@holycross.edu
Visit Zembla: http://www.libraries.psu.edu/nabokov/zembla.htm
View Nabokv-L policies: http://web.utk.edu/~sblackwe/EDNote.htm