Porphyria Educational Services
PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 38
September 15, 1999
FOCUS: The Neuropsychological Aspects of Porphyria
While care to avoid relapses of remission in patients diagnosed
with acute
porphyrias and the improved glucose protocol for intervention
therapy during
acute attacks have reduced the mortality of porphyria patients
considerably
over the last decade, there are still problems with the
nurological aspects
of porphyria.
When porphyria patients are in remision there are usually little
or
no neuropsychological problems except in patients with a long
history of
acute attacks and of course in those who have a chronic prognosis.
The three main types of porphyria that give rise to
neuropsychiatric
disorders include the acute intermittent porphyria [AIP],
variegate
porphyria [VP] and the heriditary coproporphyria [HEP].
In a medical publication entitled "The Little Imitator"
written by
H.L. Crimlisk, of the Department of Neuropsychiatry at the
Institute of
Neurology in London, England, the author states that
nerological or
psychiatric symptoms occur in most acute attacks.
While true of
symptomology of earlier years, today these neuropsychological
symptomologies can be treated early on, by porphyric patients
learning to be "in tune" with their mind and bodily
functions.
The early use of propranolol, adequate rest, control of seizures,
nausea and vomiting and lab tests checking electrolyte balance,
all can
correct if not inhibit the neuropsychological problems of earlier
etiology
of acute attacks. In times past many porphyria patients
experience
bizarre psychological behavior and often would be hospitalized in
neuropsychological wards due to the behavior rather than the
treatment of the porphyria.
The management of patients with porphyria and the psychiatric
symptoms do cause considerable problems unless the porphyria
is actively addressed first and foremost. This calls
for adequate
testing and diagnosis.
With a confirmed diagnosis today a porphyria patient
can undergo
PREVENTATIVE treatments which most usually can be administered
at home and alleviate the necessity of triggering attacks which
require costly
hospitalization for observation and INTERVENTION treatments
of either glucose or heme. Such preventative treatment when shown
to be cost effective, are usually handled under "case
management"
of most major medical insurers.
While the physical medical treatment and careis much better for
the
porphyric patient, so too is the neuropsychological.
Avoiding acute attacks
means avoiding the chances of neuropsyhcological impairments and
exercerbations.
In the earlier days many porphyria patients were
institutionalized
due to the neuropsychological aspects of their disease, thus
accounting for the early
day large porphyria populations in mental institutions.
Today however with the
advances in molecular biology which permit the identification of
porphyria patients
early on, in both acute and latent carriers alike within a family,
acute attacks can
be avoided for the most part. This means that the
neuropsychological elements
of the acute porphyrias can be addressed as well and for the most
part
treated in advance, with little or no permanency.