Giugno 2011 - Volume XXX - numero 6
Pagine elettroniche ; Caso Contributivo
Dipartimento Materno Infantile, Unità Clinica Pediatrica, Azienda Ospedaliero-Universitaria di Parma
Indirizzo per corrispondenza: giuliabarbato@libero.it
Key words: Transverse myelitis, Paresthesia, Weakness, Spinal cord MRI
Transverse myelitis is a neurologic syndrome caused by acute or subacute inflammation of the spinal cord. Since its incidence is about 0.5/100,000 children/year, transverse mielitis is included in the group of rare diseases. The pathogenesis of the disease includes an abnormal activation of the immune system of the spinal cord, which leads to axonal demyelination. Transverse mielitis occurs expecially after infections or vaccines; it is often due to autoimmune diseases; rarely, it is a paraneoplastic syndrome. When a direct cause cannot be found, it is called idiopathic. The clinic includes limb and leg weakness, sensory disturbance, bowel and bladder dysfunction, back and radicular pain. The prognosis is extremely variable: about one third of the patients recovers without consequences, one third has some disability left, one third gets an important rate of disability without recovery. We report a case of a 13-year-old girl affected by transverse myelitis who rapidly developed leg paresthesia and weakness, difficulty in deambulation, sensor and sphincter deficits. Since all the tests made during the hospitalization to find a possible cause were negative, no pathogenetic association could be done.
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Pagine elettroniche ; Caso Contributivo
1Scuola di Specializzazione in Pediatria, IRCCS “Burlo Garofolo”, Università di Trieste
2UO Pediatria, Ospedale “M. Bufalini”, Cesena
3UO Medicina Trasfusionale, Ospedale “M. Bufalini”, Cesena
Indirizzo per corrispondenza: angela.decunto@libero.it
Key words: Acquired haemophilia, Paediatric age, Case report
The case of a boy who presented with an isolated gross haematuria is reported. In the previous days he referred a muscular haematoma after a minimal trauma. Coagulation studies showed a prolonged activated partial thromboplastin time (90 sec), not corrected by incubating a sample of his blood with equal volumes of normal plasma (mixing study). A factor VIII level (FVIII) less than 1% was detected and a FVIII inhibitor of 2 Bethesda unit was dosed. Steroid treatment was started with prompt resolution of “acquired haemophilia”.
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