[Dichloromethylenbiphosphonic acid in the therapy of myositis ossificans progressive (MOP)]. / L'acido diclorometilenbifosfonico nella terapia della miosite ossificante progressiva.

The authors report 3 cases of myositis ossificans progressiva (MOP) treated with i.v. dichloromethylenbiphosphonic acid. Two of these patients presented a severe development of the disease so that they could not walk anymore. After a few months of treatment with dichloromethylenbiphosphonic acid (300 mg/die for 10 continual days every month) sufficient recovery in motor capacity was achieved that walking was possible again. The favourable effect of therapy has been shown only by clinical features because there is no biological marker to monitor in the follow-up. During the treatment no side effect or modification of the markers of liver and kidney activity was observed.

[18q syndrome with deficiency of myelin basic protein (MBP)]. / Sindrome 18q- con carenza di myelin basic protein (MBP).

The Authors present a patient with 18q- Syndrome in which lymphatic cell karyotype could resume development of extrapyramidal degeneration signs before they appeared. Severity range of phenotypic manifestations in the 18q- syndrome is correlated with chromosomic breakpoint and with genetic background. Many chromosome 18's distal arm genes have been mapped Myelin Basic Protein gene (MBP) has been located in 22-23 position; it forms about 30-40% of myelinic sheath proteins. Failure in MBP gene expression would be correlated in the central white matter with extrapyramidal system degeneration signs: in 18q- patients with involuntary movements studied by MRI or by post-mortem autopsy unmyelinated areas in central white matter tracts have been put in evidence. As MBP absence in peripheral nervous system does not appear to have a functional effect, it has been suggested that some specific component of peripheral myelin is functionally equivalent to MBP and capable to substitute this protein in its absence.

[A case of organic psychosis in dermatomyositis. Etiopathogenetic hypothesis]. / Un caso di psicosi organica in corso di dermatomiosite. Ipotesi eziopathogenetiche.

The authors describe a girl affected by dermatomyositis, with a quick and grave evolution, which required urgent tracheostomy to reduce the dysphagic symptoms. During the observation the patient showed psychotic symptoms. After the revision of literature, in which no such complication has ever been described, the authors propose an etiopathogenetic interpretation for a generalized acute psycho-organic syndrome, supposing a cerebral hypoxic damage, as vasculitic manifestation, probably complicated by symptoms of hysterical conversion, due to the isolation of the patient in the Department of Intensive Therapy and to the severe physical conditions.