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How we arrived
at this working hypothesis. |
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A friend and colleague,
Loretta S. Loftus M.D., suggested AIP for van Gogh in December 1988, after
she read my paper "Vincent van Gogh and the thujone connection"
in JAMA.4 As a practicing internist she was immediately impressed
by the frequency of references therein to neuropsychiatric and gastrointestinal
complaints, the sensitivity to alcohol, and the intermittent nature of
his psychosis. Together we searched the van Gogh letters for everything
on illness and systematized the data. The artist's symptomatic agreement
with the hallmarks of acute intermittent porphyria, the recognition of
precipitant factors in his life, and the van Gogh family history of mental
illness, all supported the case for AIP, which we adopted as a working
hypothesis, tested, and documented.1,2 |
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Overview
of acute intermittent porphyria (AIP) as a disease entity. |
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*
AIP is one member of a class of metabolic abnormalities, the porphyrias,
which are characterized by the excessive production of porphyrins,
or related compounds.5,6 Individuals who suffer from these
diseases are prone to excrete elevated concentrations
of these same compounds in their urine and feces. The abnormal excretion
per se is of no intrinsic medical import but it is a reflection of elevated
concentrations circulating within the body, and therein lies the potential
for cutaneous photosensitivity (due to porphyrins), neurological abnormalities
(due to porphyrin precursors), or both. |
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*
In the case of AIP, all of the symptoms are neurological
and the specific, overly-produced compounds are delta-aminolevulinic
acid and porphobilinogen.These are intermediates in the metabolic
pathway to porphyrins, which in turn are used in the biosynthesis of the
heme of hemoglobin, and other heme-containing proteins (see the biochemical
pathway that is presented in the following section). |
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*
"Acute" refers to the rapid onset,
and abrupt cessation, of expressed symptoms. (The underlying cause of
AIP is present from birth, so in that other sense it is chronic.) "Intermittent"
refers to the periodicity, which is typical, and emphasizes the distinct
periods of normalcy which intercede between the episodes of illness. |
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Symptoms rarely occur before puberty; the peak decade for onset
of symptoms is from age 20 to 29, i.e. the third decade,
and is somewhat later for males than females, but the disease sometimes
remains latent throughout a lifetime. |
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*
Tabulations of the most common hallmarks emphasize abdominal
pain and other gastrointestinal complaints, symptoms referable
to the peripheral and central nervous systems, and signs of autonomic
neuropathy including tachycardia and hypertension (see figure 1). Porphyria-induced
hypertension can cause early-onset renal failure. Bladder dysfunction
may result in urinary retention. Effects on optic nerves or the occipital
lobes have been documented for AIP cases. Sexual impotence has occasionally
been reported. Premonitory symptoms include restlessness and irritability;
attacks develop rapidly; resolution may occur in days or sometimes weeks,
in an unpredictable fashion. Seizures do not always attend severe crises,
but when they do many antiseizure drugs, with the notable exception of
bromides, may adversely affect the outcome.7,8 |
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Main
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