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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.