PACNS - Part 3: Clarification, Please

[Dave M]

Thank you for your response to my questions of April 25, 2001.  I found it helpful, and I will review the literature you mentioned.  We also will check to find out which vessels are affected and more specifically where.

TO ANSWER YOUR QUESTIONS:  The diagnosis was made via brain biopsy, MRI and lumbar puncture and confirmed by Dr. Calabrese.  No angiography.  The initial diagnosis, based on the MRI at emergency admission, was an inoperable brain tumor with six months to live.  Two subsequent MRIs showed improvement.  That is, the first of those MRIs, very soon after the initial MRI and in preparation for the biopsy, showed fewer "spikes" (my term) and a smaller, softer-looking mass.  The most recent MRI (about a month ago) showed very little mass, but the neurologist said there were some "abnormalities" but has not elaborated.

I was confused in reading your discussion of small, medium and large vessel involvement, so I'll ask if you could check the following summary for accuracy and completeness.  I'm not sure that I have the statements assigned to the correct type.

Small Vessel Types:
-- Most adults have this form of the disease.
-- Symptoms are progressive and, depending on where the lesions are, affect those brain areas.
-- Lesions are best picked up by high resolution MRI.
-- Multifocal neurologic defects, cognitive decline and often seizures are seen in this type of angiitis.
-- Aneurysm also can occur.
-- The course of the disease would reflect generalized brain damage and a "gradual but variable course,"  (WHAT DOES GRADUAL BUT VARIABLE MEAN?) with headaches, focal seizures, multifocal neurologic deficits and neurobehavior impairment.
-- Responses to treatment range from remission to gradual, but progressive, decline.

Medium to Large Vessels:
-- Usually involve strokes, TIAs, or subarachnoid hemorrhage (isn't the last another term for stroke?).
-- Track changes with MRI.
-- This type is more aggressive than the small vessel variety.
-- The brain region compromised by the lesion would show neurological signs, so check the vision cortex or optic tracts.

Thanks, again, for your helping us to understand this disease and its effects.

[CCF Neuro[P]-M.D.-RPS]

Dear Dave:

Gradual but variable means that each patient is variable, or unique in the progression of disease.  Some patients have a rapid down hill course, while others it is more gradual over a period of time, while in others there is a rapid progression but then a leveling off of symptoms (static and non-progressive) but in time symptoms will progress.  So, it is variable depending on the patient but always progressive in nature.  Subarachnoid hemorrhage can be considered a hemorrhagic stroke, but certainly not an embolic stoke or lacunar stroke.  Bleeding is not into the parenchymial matter but in the subarchnoid space.  Just a choice in terms so to delineate the difference.  

Sincerely,

CCF Neuro MD