A woman, 51 years of age, presented
as the first most striking mental symptom, ideas of jealousy concerning
her husband. Soon after, a rapidly developing mental weakening was noticed;
she would lose her way about in her own home, throw things around and hide
herself for fear of being killed.
In hospital she seemed perplexed, was disoriented
for time and place, occasionally complained that she understood nothing
and of an inabillty to express her thoughts.
She frequently greeted the physician as a social caller, making excuses
meanwhile that her housework was still unfinished. At other times
She would cry out in fear thinking that the physicjan would cut her or
evidence distrust of hlm, believing that her honor would be assailed.
At times she was delirous; tossed the bed-clothing about,called out
for her husband and daughter and appeared to have auditory hallucinations.
Frequently she shouted loudly for hours at a time.
Whenever she was unable to mentally grasp a situation
she would cry out loudly, this too, whenever an examination was attempted
. Only through repeated and patient effort was anything finally obtained
from her. Retention (Merkfähigkeit) was markedly impaired.
When shown objects she named them for the most part correctly, but immediateiy
forgot them. In reading she went from line to line spelling out the words
or read without inflection. In writing she repeated many syllables, left
out others, but executed the tests rapidly. In speaking she misplaced words--occasional
paraphasia-- and perservation was frequent. Many questions asked her were
apparently not understood. The gait was undisturbed and use of the hands
was equally good. Patellar reflexes were present; the radial arteries firm;
no increase in the area of cardiac dullness; no albumin in the urine.
In the further course of the disease the disease the focal symptoms
were sometimes more prononced, sometimes less so, but throughout never
intense. The patient finally was completely demented; confined to bed with
contractres of the lower extremities; and passed urine and feces involuntarily.
In spite of greatest care decubitus developed. Death afer a duration of
41/2 years.
The autopsy revealed a diffusely atrophied
brain without macroscopic focal lesions, the larger cerebral arteries sclerotic.
In sections handled after the Bielschowsky silver impregantion
method a striking alteration of the neurofibrils was shown. In an otherwise
seemingly normal cell there appears at first one or more fibrils which
on account of increased thickness and increased tingibility stand out prominently.
In the further course of the alteration many neighboring fibrils are similarly
affected. These, thene, form thick bundles which gradually come to the
surface of the cell. Finally the nucleus and cell disisntgrate and only
a tangled bundle of fibrils remains to indicate the site of a former ganglion
cell.
That these fibrils are colored by other staining methods which
do not display neurofibrils indicates a chemical alteration in the fibril
substance. This can well be, for the fibrils survive the destruction of
the cell. The alterations in the neurofibrils appears to go hand in hand
with a deposition of not yet definitely determined pathological metabolic
stuffs. About 1/4 to 1/3 of all ganglion cells of the cerebral cortex exhibited
this peculiar alteration of the fibrils. Many ganglion cells, particularly
in the upper cell laminae, had disapperaed.
Throughout the entire cortex, especially numerous in the outer
layers, were found many miliary foci, the result of a deposition of peculiar
stuffs in the brain substance. These foci may be recognized without staining,
but are very refractory to staining methods.
There was a rich proliferation of glia fibers and many glia cells
exhibited large fat sacs. There was no infiltration of the walls of vessels,
but proliferative changes of the endothelium were demonstrable and occasionally
vessel proliferation was encountered.