Utilidad del SPECT cerebral en el estudio de la hipersomnia recurrente: síndrome de Kleine-Levin / Usefulness of brain SPECT imaging in the study of recurrent hypersomnia: Kleine-Levin syndrome

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Enfermedad de Graves en un paciente de 3 años con agranulocitosis asociada a fármacos antitiroideos: terapia ablativa con radioyodo como una alternativa eficaz / Graves’ disease in a 3 year-old patient with agranulocytosis due to anti-thyroid drugs: radioiodine ablation therapy as an effective alternative

Se presenta el caso de una niña de 3 años pluripatológica, con una enfermedad genética mitocondrial (encefalomiopatía necrosante subaguda o síndrome de Leigh), insuficiencia renal crónica estadio v por una esclerosis mesangial difusa y alteraciones del desarrollo, que fue diagnosticada de hipertiroidismo autoinmune por enfermedad de Graves-Basedow. A las 6 semanas del inicio terapéutico con neocarbimazol, la paciente presentó un cuadro de agranulocitosis severa que obligó a suspender la medicación con fármacos antitiroideos, motivo por el cual fue tratada de manera eficaz con terapia metabólica con 131I. La relevancia del artículo radica en la infrecuencia de la enfermedad de Graves en niños prepuberales (especialmente menores de 6 años), las complicaciones en el desarrollo derivadas de un posible diagnóstico tardío, la aparición de agranulocitosis como efecto adverso potencialmente grave tras el uso de antitiroideos y los pocos casos reportados de terapia ablativa con 131I a esta edad, que otorgan singularidad al caso (AU)

The case is presented of a 3 year-old girl with mitochondrial disease (subacute necrotizing encephalomyelopathy of Leigh syndrome), v-stage chronic kidney disease of a diffuse mesangial sclerosis, as well as developmental disorders, and diagnosed with hyperthyroidism Graves-Basedow disease. Six weeks after starting the treatment with neo-carbimazole, the patient reported a serious case of agranulocytosis. This led to stopping the anti-thyroid drugs, and was treated successfully with 131I ablation therapy. The relevance of the article is that Graves’ disease is uncommon in the paediatric age range (especially in children younger than 6 years old), and developing complications due to a possible late diagnosis. Agranulocytosis as a potentially serious adverse effect following the use of anti-thyroid drugs, and the few reported cases of ablation therapy with 131I at this age, makes this case unique (AU)

Graves' disease in a 3 year-old patient with agranulocytosis due to anti-thyroid drugs: Radioiodine ablation therapy as an effective alternative. / Enfermedad de Graves en un paciente de 3 años con agranulocitosis asociada a fármacos antitiroideos: terapia ablativa con radioyodo como una alternativa eficaz.

The case is presented of a 3 year-old girl with mitochondrial disease (subacute necrotizing encephalomyelopathy of Leigh syndrome), v-stage chronic kidney disease of a diffuse mesangial sclerosis, as well as developmental disorders, and diagnosed with hyperthyroidism Graves-Basedow disease. Six weeks after starting the treatment with neo-carbimazole, the patient reported a serious case of agranulocytosis. This led to stopping the anti-thyroid drugs, and was treated successfully with 131I ablation therapy. The relevance of the article is that Graves' disease is uncommon in the paediatric age range (especially in children younger than 6 years old), and developing complications due to a possible late diagnosis. Agranulocytosis as a potentially serious adverse effect following the use of anti-thyroid drugs, and the few reported cases of ablation therapy with 131I at this age, makes this case unique.

Linfedema primario en miembro superior asociado a síndrome de Klippel-Trénaunay (displasia angiectasia congénita) / Primary upper limb lymphedema associated with Klippel-Trénaunay syndrome (congenital dysplastic angiectasia)

Se presenta el caso de una paciente de 41 años que se diagnostica de flebolinfedema primario con localización en miembro superior izquierdo asociado a síndrome de Klippel-Trénaunay, que no presentó manifestación clínica hasta los 37 años de edad, momento de aparición de la tríada clínica característica de angiomas cutáneos, varicosidades e hipertrofia de los tejidos blandos. La relevancia de este artículo radica en que se trata de una enfermedad rara y congénita, que se manifiesta en la infancia o adolescencia, con afectación más frecuente en miembros inferiores. Su etiopatogenia es aún desconocida, surgiendo casos esporádicos, aunque se cree que podría ser debido a una alteración en el desarrollo del mesodermo en el feto, que afecta a las líneas angioblásticas, linfoblásticas y osteoblásticas. Es importante conocer los diferentes signos clínicos para un diagnóstico y tratamiento precoz, el cual suele ser sintomático y la rehabilitación constituye un pilar fundamental dentro del tratamiento multidisciplinar (AU)

The case is presented of a 41 years old patient with primary lymphedema diagnostic in her left upper limb associated with Klippel-Trénaunay syndrome, who did not present any clinical manifestations until 37 years old, time of appearance of the characteristic clinical triad of cutaneous angiomatosis, varicose veins and hypertrophy. The relevance of this article is that it is a rare and congenital disease. This syndrome usually manifests in childhood or adolescence with more frequent involvement of the lower limbs. Its etiopathogeny is still unexplained, emerging sporadic cases, although it is believed it could be due to fetal's mesoderm development alteration, which affecting angioblastic, lymphoblastoid and osteoblastic lines. The importance of an early correct diagnosis and its symptomatic treatment, where rehabilitation has a fundamental role within the multidisciplinary approach is emphasized (AU)

Utilización de la gammagrafía hepatobiliar en la evaluación del quiste hepático / Hepatobiliary scintigraphy for the evaluation of hepatic cyst

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Neurolinfomatosis como manifestación inicial de recidiva en linfoma / Neurolymphomatosis as initial manifestation of recurrence in lymphoma

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The usefulness of the scan with 67Ga-citrate in the multicentric reticulohistiocytosis

En este caso la gammagrafía con 67Ga-citrato se presenta como una herramienta útil para valorar la reactivación y la extensión de la enfermedad. La reticulohistiocitosis multicéntrica es una enfermedad sistémica rara de etiología no conocida. Se caracteriza por provocar artropatía erosiva, así como lesiones cutáneas. Su diagnóstico y tratamiento deben realizarse precozmente para evitar secuelas. Se presenta el caso de un varón de 67 años con antecedentes de reticulohistiocitosis multicéntrica y artroplastia en la cadera izquierda que acude por dolor en el miembro inferior izquierdo, siendo diagnosticado de luxación de prótesis de cadera izquierda. Durante el ingreso se realiza una gammagrafía ósea con 99mTc-HDP y una gammagrafía con 67Ga-citrato donde se evidencia la ausencia de actividad inflamatoria ósea en la pelvis que contraindique la cirugía. Además, se observa una captación bilateral en las partes blandas de las grandes articulaciones compatible con la persistencia de la actividad inflamatoria secundaria a reticulohistiocitosis multicéntrica(AU)

Multicentric reticulohistiocytosis is a rare systemic disease of unknown etiology. It is characterized by erosive arthritis and skin lesions. Diagnosis and treatment should be performed early to avoid sequelaes. We report the case of a 67-year-old man diagnosed with multicentric reticulohistiocytosis and left hip arthorplasty. The patient consulted for local pain in his left leg. Final diagnosis was left prosthetic dislocation. During admission, a bone scintigraphy with 99mTc-HDP and scintigraphy with 67Ga-citrate was performed, showing no evidence of inflammatory activity in pelvis that contraindicating surgery. Besides it showed bilateral uptake in soft tissue of large joints compatible with persistent inflammatory activity secondary to multicentric reticulohistiocytosis. In this case scintigraphy with 67Ga-citrate, is a useful tool to assess the recovery and extent of disease(AU)

The usefulness of the scan with 67Ga-citrate in the multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis is a rare systemic disease of unknown etiology. It is characterized by erosive arthritis and skin lesions. Diagnosis and treatment should be performed early to avoid sequelaes. We report the case of a 67-year-old man diagnosed with multicentric reticulohistiocytosis and left hip arthorplasty. The patient consulted for local pain in his left leg. Final diagnosis was left prosthetic dislocation. During admission, a bone scintigraphy with (99m)Tc-HDP and scintigraphy with (67)Ga-citrate was performed, showing no evidence of inflammatory activity in pelvis that contraindicating surgery. Besides it showed bilateral uptake in soft tissue of large joints compatible with persistent inflammatory activity secondary to multicentric reticulohistiocytosis. In this case scintigraphy with (67)Ga-citrate, is a useful tool to assess the recovery and extent of disease.