A neuropsychiatric and genetical investigation of acute intermittent porphyria / by Lennart Wetterberg.

  • Wetterberg, Lennart.
Date:
1967
Catalogue details

Licence: Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)

Credit: A neuropsychiatric and genetical investigation of acute intermittent porphyria / by Lennart Wetterberg. Source: Wellcome Collection.

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    with a functional pyridoxine deficiency (Druyan and Haeger-Aronsen 1966, Elder and Mengel 1966, Hamfelt and Wetterberg 1967). Disturb¬ ances of lipid metabolism (Schwartz 1955), thyroid function (Hellman et al. 1963), iron metabolism (Kramer 1963), amino acid metabolism (Mellinkoff et al. 1959, Druyan et al. 1965) and an inappropriate secre¬ tion of antidiuretic hormone (Hellman et al. 1962, Ludwig and Goldberg 1963, Nielsen and Thorn 1965, Perlroth et al. 1966) are some other occasional conditions reported in AlP-patients. Further studies of the biochemical lesions in AIP have been summariz¬ ed by Watson (1964), Tschudy (1965), Rimington (1966) and Schmid (1966). The clinical manifestations show preference for certain abdominal and neurological symptoms which may reflect non-randomized target areas in the nervous system, although the link between such lesions and the observ¬ ed disturbance in porphyrin synthesis is unknown. The clinical symptoms apparently are not correlated with the excreted amount of ALA and PBG (Ackner et al. 1961). In fact, the excretion may be higher in asymp¬ tomatic individuals than in patients with clinical manifestations. During an acute attack of porphyria psychotic symptoms, sometimes of a schizophrenic type, may occur (Waldenstrom 1937, Peters 1962). This observation may be of interest for research on the etiology of psychotic states in general. Psychiatric manifestations The first extensive investigation of AIP was reported by Günther (1911, 1922) who noted abdominal pain, constipation, vomiting and psychiatric disturbances as the prominent symptoms in the disease he referred to as Hämatoporphyrie. Waldenstrom (1937) made a clinical survey of 103 cases of acute porphyria in Sweden. Five of these cases had been admit¬ ted to mental hospitals because of psychotic manifestations and about 20 cases displayed other mental symptoms. In 1954 Markovitz reported five cases of AIP and reviewed an addi¬ tional 64 cases reported between 1941 and 1953. Mental changes had been observed in 80 per cent of these patients, and in 14 per cent they were the initial symptoms of the attacks. Minor changes such as irrita- 15