totally .. out of this world .. behaviour IN .. those WITH
porphyria .. then you TOO will understand the meaning OF the ..
word .. premenstral .. syndrome .. <<

 "In our experience Demerol, together with ACTH and chelating agents,
such as Versene, has been of greatest value."

Now Versene is EDTA .. a nonselective metal chelator ..
coincidentally .. again .. of course.

Psychosomatic Medicine 21:34-39 (1959)
? 1959 American Psychosomatic Society

Stress and the Precipitation of Acute Intermittent Porphyria
ELLIOT D. LUBY M.D.1, J. GARTH WARE M.D.1, RITA SENF Ph.D.1, and
CHARLES E. FROHMAN Ph.D.1
1 Lafayette Clinic and Wayne State University College of Medicine,
Detroit, Mich.

Relationships between emotional stress and the precipitation of acute
intermittent porphyria have rarely been suggested. Eldahl reported an
episode occurring only two days after a traumatic event.6 Visher and
Aldrich10 correlated urinary coproporphyrin levels with the emotional
state of a patient and concluded that anxiety was important in
determining the onset of her symptoms. Roth,9 after stating that
porphyria occurred almost exclusively in people with severe neurotic
personality disorders, speculated that "psychoneurosis plays an
important part in the pathogenesis of the disease, and in determining
the time of onset of the acute attack." On the other hand, Cross1 has
cautioned that "Sweeping assertions about the importance of
psychogenic factors, especially in diseases of metabolism, are open to
the charge of belonging to the post-hoc, ergo-propter-hoc category."
Such caution is well advised, particularly in a disease with such a
multiplicity of precipitants as acute intermittent porphyria. In
addition to precipitating factors, gene penetrance is of great
importance in determining which members of a tainted family develop
the manifest illness.

The six patients described in this report all became ill at a time
when either anxiety or depression was produced by disturbing life
situations. In three instances the relationship between the emotional
stressor and the onset of the manifest disease was immediate and
dramatic. For two of these patients, the loss of an important
interpersonal relationship was the triggering factor. The other three
patients gradually developed symptoms in settings where stress was of
a continuing nature, associated with either an unhappy marriage or the
pressure of an unmanageable work load. Alcoholism in the history of
one very probably contributed to his morbidity and eventual mortality.

The correspondence between the mean porphobilinogen level and
emotional stress in one family group presents additional evidence of
the importance of disturbed life situations in the pathogenesis of
this disease. At a time when one member was moribund and in a
respirator, the porphobilinogen excretion of the family rose
dramatically. As he improved it gradually diminished. The unexplained
peak at the seventh week may have been a result of other unknown
stress factors, or there may be cyclic variations in porphobilinogen
excretion. Longer and more detailed followup might resolve this
question.

Studies of stress have been concerned primarily with the adrenal
cortex rather than with specific enzyme systems. It is apparent that a
multitude of nonspecific stressors, emotional, pharmacologic, or
physical, are capable of making manifest a latent biochemical lesion.
The investigation of the vicissitudes of other enzyme systems in
stressful situations would seem warranted and potentially profitable
in understanding disease mechanisms.

The literature would imply classical personality configurations for
these patients and their families. This was not observed in our study.
The MMPI's of thirteen members of KB's family, all of whom had
positive Schwartz-Watson tests at one time or another, revealed almost
no abnormal scores in any of the scales. The members of HA's family
who became ill manifested sociopathic and schizophrenic behavior,
while KB's relatives presented with predominantly neurotic symptoms.
These observations suggest that much of the behavior seen in patients
with acute intermittent porphyria will depend on their basic premorbid
personality. Thus psychiatric symptomatology to be seen in these
patients can be expected to vary from one person to another.

Unfortunately, this disease is frequently misdiagnosed. Measures taken
to produce symptomatic relief, such as somatic therapy or sedation
with barbiturates, only serve to intensify the process. Often surgery
is performed with pentothal induction; this has had dire consequences.
Although chlorproniazine has been helpful for some patients, it has
harmed others. In our experience Demerol, together with ACTH and
chelating agents, such as Versene, has been of greatest value. The
danger of addiction to Demerol is great, and caution should be used
with its exhibition.

Submitted on October 10, 1958

Who loves ya.
Tom

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