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3.
Rev. neurol. (Ed. impr.) ; 55(2): 87-90, 16 jul., 2012. tab, ilus
Article in Spanish | IBECS | ID: ibc-101773

ABSTRACT

Introducción. La neuromielitis óptica o enfermedad de Devic es una enfermedad inflamatoria y desmielinizante del sistema nervioso central que afecta selectivamente a los nervios ópticos y a la médula espinal, con alta frecuencia de recidivas. Los anticuerpos antiacuaporina 4 (AQP4) son un marcador altamente específico de esta entidad. Caso clínico. Mujer de 66 años de edad con una mielitis transversa longitudinalmente extensa dorsal con remisión completa tras tratamiento esteroideo y recidiva aguda posterior, con plejía de una extremidad. Entre los diagnósticos diferenciales se consideraron los de tumoración espinal y malformación arteriovenosa medular. La positividad de AQP4 fue el factor determinante en el diagnóstico final. Conclusión. La detección precoz de los anticuerpos anti-AQP4 junto con una inmunoterapia apropiada puede representar la clave de un mejor pronóstico. Es muy importante un diagnóstico precoz de cara a un inicio temprano del tratamiento y así evitar recurrencias y secuelas graves (AU)


Introduction. Neuromyelitis optica, or Devic’s disease, is an inflammatory, demyelinating disease of the central nervous system that selectively affects the optic nerves and the spinal cord, with a high rate of relapses. Anti-aquaporin-4 (AQP4) antibodies are a highly specific marker for this condition. Case report. A 66-year-old female with longitudinally extensive dorsal transverse myelitis with complete remission following steroidal treatment and later acute relapse, with palsy in one limb. The differential diagnoses considered included a spinal tumour and arteriovenous malformation of the spinal cord. Being positive for AQP4 was the decisive factor in the final diagnosis. Conclusions. Early detection of anti-AQP4 antibodies together with appropriate immunotherapy can be the key to a better prognosis. An early diagnosis is essential to be able to start treatment at an early stage and thus prevent relapses and severe sequelae (AU)


Subject(s)
Humans , Female , Aged , Neuromyelitis Optica/diagnosis , Aquaporins/antagonists & inhibitors , Myelitis, Transverse/diagnosis , Diagnosis, Differential , Demyelinating Diseases/diagnosis , Immunotherapy
4.
J Pediatr Endocrinol Metab ; 25(11-12): 1165-8, 2012.
Article in English | MEDLINE | ID: mdl-23329765

ABSTRACT

Central precocious puberty (CPP) is fairly common in girls. In most girls, the etiology for the CPP is unknown. Among the more rare causes of CPP in girls are central nervous system tumors and hamartomas. Osteolipoma of the tuber cinereum, which is the most commonly diagnosed at autopsy, has been reported as a cause of CPP. We describe an 8-year-old girl with central precocious puberty in whom MRI demonstrated a lesion compatible with osteolipoma. Her symptom was breast development that begun at age 7 years and 9 months. Her case history, laboratory studies and imaging are presented. Her puberty was rapidly progressive. She was treated successfully with a GnRHa (Triptorelin 3.75 mg IM q 4 weeks). Her case brings to the forefront the need to perform an MRI in children with rapidly progressing puberty.


Subject(s)
Hypothalamic Neoplasms/diagnosis , Lipoma/diagnosis , Puberty, Precocious/diagnosis , Tuber Cinereum/pathology , Adrenal Gland Neoplasms/diagnosis , Algorithms , Child , Diagnosis, Differential , Female , Fibrous Dysplasia of Bone/diagnosis , Gonadotropin-Releasing Hormone/analogs & derivatives , Gonadotropin-Releasing Hormone/therapeutic use , Humans , Hypothalamic Neoplasms/complications , Hypothalamic Neoplasms/drug therapy , Lipoma/complications , Lipoma/drug therapy , Magnetic Resonance Imaging , Ovarian Cysts/diagnosis , Ovarian Neoplasms/diagnosis , Puberty, Precocious/drug therapy , Puberty, Precocious/etiology , Treatment Outcome
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