Atypical form of melorheostosis improved by pamidronate.

BACKGROUND: Melorheostosis is a rare chronic bone disease of unknown etiology that often affects a single limb. AIM: Report a new case of melorheostosis of the ribs improved by pamidronate infusions CASE: A 36-year-old man without any medical history was admitted for a history of one month painful tumefaction on the 7th left rib. The diagnosis of melorheostosis of the rib and the tibia was made. Patient was treated by pamidronate infusions with useful and satisfactory outcome.

[Pseudo-tumoral neuro-Behçet's disease]. / Neuro-Behçet pseudo tumoral.

INTRODUCTION: Clinical involvement of the central nervous system occurs in about 10 to 30 percent of patients with Behçet's disease. Neurological pseudo tumoral presentation is rare. CASE REPORT: We report a case of pseudo tumoral neuro-Behçet disease in a 38-year-old man, with past history of facial and dorsal folliculitis, who presented suddenly headache, right hemiplegia, aphasia and disturbed consciousness. Neuroradiological investigations showed a pseudo tumoral lesion in the left capsulo-thalamic region extending to the homolateral peduncle. The patient improved with steroid and immunosuppresseur therapy. CONCLUSION: The clinical and radiological presentation of neuro-Behçet's disease can mimic a brain tumor.

[Primary hypertrophic osteoarthropathy without cutaneous involvement (Currarino's disease)]. / Ostéoarthropathie hypertrophiante primitive sans atteinte cutanée (maladie de Currarino).

INTRODUCTION: Hypertrophic osteoarthropathy (HOA) is a syndrome associating hippocratic fingers, arthropathy and periostosis of long bones. Currarino's disease, considered at present as a clinical form of primary HOA, is characterized by the absence of pachydermia. OBSERVATION: A 24-year-old Caucasian man, consulted for a painful swelling of both ankles that had developed over the past year. Clinical examination revealed hippocratic fingers without pachydermia. The ankles were swollen. The X-rays showed periosteal apposition and an acro-osteolysis. In view of this triad: arthropathy, hippocratic fingers and periostosis, primary HOA without cutaneous involvement or Currarino's disease was diagnosed. The search for a secondary cause remained negative. Clinical improvement was obtained after 15 months with non-steroidal anti-inflammatory drugs (NSAID) and colchicine. COMMENTS: Although exceptional, primary HOA without cutaneous involvement is a genetic disease which must not be ignored.

[Bronchiectasis in a patient with ulcerative colitis]. / Bronchectasies chez un patient porteur d'une rectocolite ulcéro-hémorragique.

Bronchopulmonary involvement is uncommon in ulcerative colitis. Bronchiectasis is a rare manifestation of the disease. We report a case of a 65-year-old patient with ulcerative colitis in whom we diagnosed bronchiectasis. The evidence of a relationship between bronchiectasis and ulcerative colitis was established on the basis of 3 criteria: the late occurrence of bronchiectasis in a patient without pulmonary history, their appearance after the ulcerative colitis developed and their improvement after inhaled corticosteroid treatment. This observation points out that ulcerative colitis investigations should be extended to the respiratory structures. The respiratory involvement is not always concomitant with the bowel disease. This observation suggests the efficiency of the inhaled corticosteroids in this disease.

[Disseminated tuberculosis in non-immunocompromised host: three case reports]. / Tuberculose disséminée sur terrain non immunodéprimé: à propos de trois cas.

INTRODUCTION: Disseminated tuberculosis, i.e., tuberculosis involving lung, liver, spleen, bone marrow and lymph nodes is rare (2.8%), particularly when immunocompromised diathesis is lacking. EXEGESIS: We report three cases of disseminated tuberculosis confirmed by bacteriology or histology, which occurred in non-immunocompromised patients. Disease evolution under antituberculous treatment was favorable in two cases and fatal in the third one. CONCLUSION: Disseminated tuberculosis must be suspected when miliary pulmonary lesions are associated with hematologic abnormalities, even in non-immunocompromised host. Early treatment is mandatory to avoid fatal outcome.

[Sleep apnea syndromes and Arnold-Chiari malformation]. / Syndrome d'apnées du sommeil et malformation d'Arnold-Chiari.

The Arnold-Chiari malformation (MAC), ectopic medulla of the cerebral hemispheres, may be the basis of respiratory problems provoked by compression of the respiratory centre. It is associated with numerous respiratory problems in children; there are however few publications related to the association of MAC and ventilatory problems in adults. It was recently suggested and subsequently shown that the syndrome of sleep apnoea (SAS) could occur in relation to MAC. We report a new observation documenting the association of MAC and SAS. The surgical cure of MAC enabling a remission of SAS suggests the existence in this case of a causal relation between the two pathologies. A search for such a malformation by imagery of the occipito-medullary crest should be a part of the work up in SAS when the cause remains undetermined.