[Parry Romberg syndrome associated with refractory epilepsy, atrophy of the dura mater and cystic leukoencephalopathy]. / Síndrome de Parry-Romberg asociado a epilepsia refractaria, atrofia de la duramadre y leucoencefalopatía quística cerebral.

CASE REPORT: We report the clinical and radiologic observation in a man patient of 20 years old. He developed gravity left progressive facial hemiatrophy. In associated form, he presented numerous visual sensories and complex partials seizures. The single and combined treatment with several antiepileptic drugs was ineffective for the control of the epilepsy. The magnetic resonance investigation of the cranium facial structures showed the gravity and intensivity of the dysmorphia. The same procedure showed the strong of the duramadre atrophy in the convexity of the brain, as well as, the existence of the cystic formation in the white matter of the left temporo occipital region at the face affected. CONCLUSION: We considered, that this is the first report documented fully, about the Parry Romberg syndrome with refractory epilepsy, duramadre atrophy and cystic leukoencephalopaty, published in Peru.

[Wilson's disease: a clinical case is presented with neuropsychiatric dominant form and probable physiopathology interpretation with magnetic resonance of the brain]. / Enfermedad de Wilson: forma neuropsiquiátrica dominante. Presentación de un caso e interpretación fisiopatológica basada en la resonancia magnética del encéfalo.

CASE REPORT: A clinical case of Wilson's disease is presented. She was a 26 years old woman who began to show psychological symptoms, and later developed neurological signs such as asymmetrical hand tremor, parkinsonism, dystonia and later dysphagia and mutism. The ophthalmological examination found a Kayser Fleischer ring in the Descemet membrane. There was disturbance of copper metabolism documented with reduction of serum ceruloplasmin and increase of the urinary excretion of copper. Cirrhosis was demonstrated through laparoscopy and liver biopsy. RESULTS: The brain magnetic resonance showed frontotemporal atrophy and a degenerative process at the basal ganglia, cerebellum and brain stem, data which could de used to suggest the probable neuropsychiatric physiopathology. The stenosis and intense cervical dysphagia, associated with crycopharyngeal membrane, has not been mentioned previously.

Enfermedad de Wilson: forma neuropsiquiátrica dominante. Presentación de un caso e interpretación fisiopatológica basada en la resonancia magnética del encéfalo / Wilson's disease: a clinical case is presented with neuropsychiatric dominant form and probable physiopathology interpretation with magnetic resonance of the brain

Caso clínico. Se presenta un caso clínico de la enfermedad de Wilson. Una mujer de 26 años de edad presentó trastornos psíquicos a los que se añadieron luego signos neurológicos tales como temblor asimétrico de las manos, parkinsonismo y distonía; más tarde tuvo mutismo y disfagia. La exploración oftalmológica demostró la presencia del anillo de Kayser-Fleischer en la membrana de Descemet. Se comprobó una alteración en el metabolismo del cobre que consiste en reducción de la ceruloplasmina sérica y aumento de la excreción urinaria de cobre. Mediante laparoscopia y biopsia se diagnosticó cirrosis hepática. Resultados. La investigación de las estructuras del encéfalo con resonancia magnética reveló atrofia frontotemporal del cerebro y proceso degenerativo de los ganglios basales, el cerebelo y el tronco encefálico, datos que podrían explicar la sintomatología neuropsiquiátrica. La asociación con membrana cricofaríngea, causante de estenosis y disfagia cervical intensa, no se ha mencionado anteriormente (AU)

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