3 types of PLS?

[Guest guest]

Here is a repost, it talks about childhood PLS, HSP

and PLS. Kind of backs up what I posted to .

Subject: excerpts from Dr. Finks article

This is taken from Dr. Fink's paper.

" In this author's experience, it is clear that PLS is

a separate disorder (or group of related disorders),

that is clinically distinct from HSP, motor neuron

disease(ALS), and any other recognizable cause of the

spastic paraplegia syndrome. This observation is

based on the sometimes striking differences between

HSP and PLS. HSP is an inherited disorder; PLS is

typically sporadic or autosomal recessive. Subjects

with HSP often exhibit dorsal column disturbance and

urinary urgency, signs which are absent in PLS.

Moreover, HSP does not cause functional weakness in

the arms, dysphagia or dysarthria. In contrast,

symptomatic involvement of the upper extremities and

corticobulbar muscles eventually occurs in PLS. It

must be recognized, however, that the early stages of

HSP (prior to development of dorsal column

disturbances, urinary urgency, and ascertainment of

other affected relatives) may not be distinguished

from early stages of PLS (prior to development of

upper extremity weakness, dysarthria, and dysphagia). "

" there are exceptions to the generalizations that HSP

invariably is a hereditary disorder and PLS a sporadic

disorder. There is not always a family history in

subjects who otherwise have all signs and symptoms of

uncomplicated HSP. somtimes this is because of

incomplete penetrance, variable age of symptom onset,

mistake paternity, or incomplete family ascertainment.

For other subjects with " apparently sporadic " HSP,

absent family history is due to autosomal recessive or

X-linked inheritance, or the presence of a new

mutation. Although usually sporadic, PLS is

occasionally familial. We (J.K.Fink, unpublished

observations) and others have observed infantile and

early childhood onset lower extremity spastic weakness

among siblings that for several years was consistent

with autosomal recessive uncomplicated HSP; and which

was followed later by progressive involvement of the

upper extremities, speech and swallowing, consistent

with the diagnosis of PLS. "

=====

Thomson, Solana Beach, CA

www.geocities.com/mdmfoo/pls.html

__________________________________________________