[State of dementia due to intermittent acute porphyria: a case report]. / Une porphyrie démentielle ou manifestations psychiatriques chroniques de la porphyrie. À propos d'un cas.

INTRODUCTION: Porphyrias are rare inherited disorders due to specific enzyme abnormalities of heme biosynthesis. We can classify them as hepatic or erythropoïetic, the type depends on the organ in which the enzyme is deficient. Among the hepatic forms, three of them are clinically characterised by acute abdominal pain and neuropathy, sometimes associated with psychiatric disorders. CASE REPORT: This 58-year-old woman has been hospitalized frequently since 1995 with depression, apathy, or for suicide attempts. She has a history of alcohol addiction and since 1992 suffers from non-insulin dependant diabetes. She had been treated by benzodiazepines and neuroleptic medication for several years. Psychiatric examination revealed persecution and megalomania delirium associated with a Capgras syndrome, confusion, disorientation, depression and blunted affect. Results of neurologic examination showed reduced deep tendon reflexes, and nerve conduction revealed sensitive neuropathy. Moreover, she lay down, remained non-responsive and resisted care. The whole clinical picture evoked early dementia. Coloured urine was noticed and porphyria tested. When the diagnosis of intermittent acute porphyria was made, she gradually improved by stopping the drugs not indicated for the case, and by treating her diabetes. A few months later, while hospitalized for a similar episode, she choked on her food and died, probably caused by autonomic neuropathy. DISCUSSION: Our hypothesis was that chronic and debilitating psychopathology may be due to acute intermittent porphyria. Probably, among patients treated for psychiatric illness, some of them may suffer from this enzyme abnormality. Above all, the psychopathology associated with intermittent acute porphyria is polymorphous such as atypical psychosis, schizoaffective disorder, depression, apathetic withdrawal, and in this case report dementia. In like manner, organic manifestations are numerous and varied, leading to erroneous diagnosis. We suspect that patients with porphyria are common among mentally ill subjects. Do we have to test all our psychiatric patients for porphyria? It seems that the prevalence of intermittent acute porphyria in the psychiatric patient population is 0.21%, whereas the prevalence in the normal population is from 0.001 to 0.002%. CONCLUSION: The early detection of porphyria may diminish morbidity and mortality rates, and perhaps heal some chronic atypical psychiatric illnesses.

[Late-onset schizophrenia or chronic delusion]. / Schizophrénie tardive ou délires chroniques.

HISTORICAL ASPECTS: Chronic delusion occurring late in life has essentially been studied by European psychiatrists. "Late-onset schizophrenia" was first described and defined by Manfred Bleuler in 1943, as a form of schizophrenia which occurs after the age of 40. Later, British psychiatrists often used the term "Late-onset paraphrenia" interchangeably with "Late-onset schizophrenia" to designate this disorder. However, late-onset paraphrenia is a British concept which includes all delusional disorders starting after age 60. American psychiatrists had little interest in this patient group, so it is only within the DSM III-R that a separate category was created for patients who developed schizophrenia after age 44. There is now no longer a "late-onset" category for schizophrenia within the DSM IV, nor age criterion for the diagnosis of schizophrenia. In the French nosography, schizophrenia is excluded when a non-affective, non-organic psychosis begins after the age of 40. These chronic delusion syndromes fall into a specific French category: "Psychose Hallucinatoire Chronique" (chronic hallucinatory psychosis). LITERATURE FINDINGS: Basing themselves on the analysis of many studies, the authors endeavor to define the characteristics of late-onset schizophrenia. The exact prevalence is not known, but is considered lower than 1%. There is a preponderance of women over men in this form of disease, that could be explained by the relative excess of dopamine type 2 (D2) receptors in young men (compared with young women), and by a protective role played by estrogens until the menopause, among women predisposed to schizophrenia. Studies of families reveal a lower lifetime risk of schizophrenia in first degree relatives of patients with late-onset schizophrenia, than those with an early onset. Most of these patients have been or are married, and had worked for a long time. Generally at the onset of the illness they are isolated and unemployed. Paranoid and schizoid abnormal premorbid personality traits are frequently noted with the diagnosis of late-onset schizophrenia. An association between late-onset schizophrenia and sensory impairment (visual and auditory) is frequently observed and appear to be in link with auditory and visual hallucinations. The analysis of clinical features reveal that the later the schizophrenia breaks out, the more the patient shows delusion and hallucinatory symptoms, which remain limited to his surroundings, whereas in younger patients, delusion has no limit. Moreover, late-onset schizophrenic patients have a lower prevalence of looseness of associations and negative symptoms than those with an earlier onset. The authors note from the few studies on the treatment, that a number of patients responded well to low dose of antipsychotic agents. The use of "atypical" anti-psychotic drugs is recommended, in order to reduce the adverse effects, notably the extrapyramidal symptoms which are frequent in elderly people. CONCLUSION: Finally, they conclude that patients with late-onset schizophrenia have symptoms very similar to those of patients with early-onset schizophrenia. But, when taking the different forms of schizophrenia - even the late onset ones - into account, raises the question of the role of trigger factors that could guide research on predictive factors and early diagnosis. This may be one explanation for the survival of the French entity "Psychose Hallucinatoire Chonique".

Major symptoms and differential diagnosis of neuroleptic malignant syndrome: three case reports.

The authors describe the cases of three patients presenting a major extrapyramidal symptom with rising CPK, inconstant hyperthermia and autonomic dysfunction. Through a brief review of the literature, the authors question the unity of the malignant syndrome. If hypertonia is quite constant, the rise in the seric CPK is aspecific and hyperthermia is inconstant.

[Atypical neuroleptic malignant syndrome]. / Atypicité du syndrome malin.

Mr A ..., 38 years old, follow up for five years for an obsessional nevrosis, hospitalized for a serious breakdown with experiences of derealization, discordance and apragmatism, was treated with haloperidol (15 mg), cyamemazine (200 mg), aenethol trithione (25 mg), associated with clorazepate (150 mg), fluvoxamine (300 mg), trimipramine (200 mg). One month later, the appearance of an extrapyramidal syndrome induced a treatment with biperidone (2 mg). The following day, he was in a state of sub-confusion with temporo-spatial disorientation and anxious perplexity. The extrapyramidal syndrome was evident with major hypertonia and generalized shaking. The appearance of bedsores on the heels, and vesicular symmetrical lesions on the ear lobes evoked a toxidermia. A large muscular atrophy of the lower limbs inducing an important muscular weakness and rising of the seric C.P.K. up to 12300 UI/l complete the clinical picture. One could notice the absence of any general symptoms, particularly a persisting apyrexia. The paraclinical evaluation was normal with no indication of renal deficiency. The treatment with neuroleptics was stopped but in spite of the rising of biperidone (8 mg), then giving trihexyphenidyle (30 mg), the extrapyramidal syndrome regressed only after 2 months, the seric C.P.K. and the dermatologic lesions within a month. Through a brief review of literature, the authors question the unicity of the malignant syndrome. If hypertonia is quite constant, the rising of the seric C.P.K. is aspecific and hyperthermia is inconstant. A few theoric informations differentiate extrapyramidal symptoms with fever and neuroleptic malignant syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

[Post-traumatic schizophrenia. A case]. / Schizophrénie post-traumatique. Une observation.

We present a case of post-traumatic schizophrenia the interest of which lies in the plurality of aetiological factors and in the signs announcing the disease observed: many mental patients among collaterals and obsessive disorders during childhood. After a brief review of the literature, this case incites us to question the existence of such a thing as post-traumatic schizophrenia and to wonder about the role played by multifactoriality in the determinism of schizophrenia.

[Statistical study of mental disorders in New Caledonia]. / Etude statistique des maladies mentales en Nouvelle-Calédonie.

315 patients admitted to the psychiatric hospital of Nouville, New-Caledonia, during the year 1987, are examined. There are several ethnies: Melanesians, Europeans, Wallisians. Europeans are admitted for depression, suicide and neurotic troubles. On the contrary Melanesians and Wallisians are admitted for acute and chronic psychotic troubles above all. Maniac-depressive illness exists in every ethny. There are a lot of psychopathological hypothesis. Indigenous medicine of Melanesians in New-Caledonia has a great part in the distribution of diagnostics.

[Psychiatric hospital. The years 1935-1945. A computerized study]. / Hôpital psychiatrique. Années 1935-1945. Etude informatisée.

Studying the files collected during a 11-years stay in a psychiatric hospital, the authors wonder about the patients' characteristics in those days, and especially about the recorded diagnoses. They show the patients' evolution and notably the fact that most of them became institutionalized. They also underline the huge percentage of psychotics as opposed to the small number of deteriorations resulting from age, times and rural environment allowing older people to continue their life at home.