https://www.jstage.jst.go.jp/article/internalmedicine1992/42/8/42_8_629/_pdf

Flexion Contractures Possibly Reflect the Existence of Hypocortisolism

'Dementia with contractures' as presenting signs of secondary adrenocortical insufficiency.

Les méningites chroniques: un carrefour à baliser analyse de 5 observations et revue de la littérature

Leucémie lymphoblastique aiguë révélée par une méningite lymphocytaire

SUMMARY The aetiology of the clinical stiff-man syndrome is likely to be heterogeneous, but until we have more precise methods of identifying an individual cause the need will continue for this rather flippant appellation in patients whose condition cannot be described in any other way. It is also important because patients may otherwise become labelled as suffering from a psychiatric disorder and may even be falsely accused of abusing diazepam (Westblom, 1978). The reverse is also true, and patients may masquerade as stiff men or women (Price and Allott, 1958; Casati and Rossi, 1969). The endocrine dimension remains and should be tested for carefully, particularly in patients with predominantly lower-limb rigidity whose spasms are a relatively minor aspect of their clinical syndrome. Clearly those patients described by George et al (1984) and Slater (1960) as suffering from the stiff-man syndrome need to be reclassified as examples of the hormonal stiff muscle syndrome, and there may be others so misclassified. An endocrine aetiology may easily be missed in a patient with relatively minor muscle stiffness, pain and cramps, such as the man described by Yunus et al (1981) whose myalgia, ‘arthralgia’ and muscle tenderness vanished completely within four days of taking physiological replacement doses of cortisone acetate as treatment for his hypopituitarism. The rarity of the stiff-man syndrome makes prospective studies of its aetiology and treatment impossible, yet the dramatic and devastating nature of the syndrome suggests that such cases may be extreme examples of a much more common condition. On the other hand, it is possible to argue that once the psychiatric, the overtly neurological and the endocrine cases are omitted we are left with nothing. However, this is just where Moersch and Woltman came in; they could not explain 14 of their cases. Despite modern technology, despite refinements of diagnosis and despite the increasing recognition of the stiff-man syndrome as a heterogeneous condition, there still remains—albeit very rarely—a cohort of patients with progressive proximal muscular stiffness and spasms who defy proper scientific explanation, but who are likely to suffer from a chronic myelitis which destroys normal feedback mechanisms between muscle spindles and the spinal cord. Experience over the last 30 years has served at least to alert people to the psychiatric possibilities, to remove any question of primary muscle or tendon disease and to point to the usefulness of diazepam. With hope, this chapter provides an endocrine dimension which offers an actual cure and therefore deserves to be more widely recognized.

Muscle 'contractures' and the 'stiff-man' syndrome.

Acute leukemias. Intensive treatment followed by a graft of autologous hematopoietic stem cells treated in vitro with an active derivative of cyclophosphamide

The course of acute leukaemias, particularly of the lymphoblastic type, is very frequently complicated by the development of neuromeningeal localisations of the disease. They may, more rarely, be presenting initial lesions, their origin being confirmed clinically and/or biologically, but it is exceptional that they remain a strictly isolated expression of the affection. A case is reported of a 16-year-old adolescent with leukaemic meningitis, in whom haematological tests (blood counts, bone marrow smear, iliac medullary biopsy, lymph node biopsy) gave negative results over a period of at least two months. Based on the findings in this case, the significance of early neuromeningeal localisations of acute leukaemias is discussed.

[Primary neuromeningeal manifestation, isolated for a long period, of acute lymphoblastic leukemia].

Serous otitis disclosing periarteritis nodosa

Otite séreuse révélant une périartérite noueuse.

Traitement de l'encéphalopathie addisonienne: apparition d'une contracture abdomino-crurale

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