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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.53 no.1 São Paulo Mar. 1995

http://dx.doi.org/10.1590/S0004-282X1995000100016 

Ressonância magnética do envolvimento cerebral na hemiatrofia facial progressiva (doença de Romberg) reconsideração de uma síndrome

 

Magnetic resonance of brain involvement in progressive facial hemiatrophy (Romberg's disease): reconsidering a syndrome

 

 

K. TersteggeI; B. KunathII; S. FelberIII; J.G. SpecialiIV; M. BeckertI; H. HenkesIII; N. HostenI

IDepartament of Radiology, Universitätsklinikum Rudolf Virchow, Freie Universität Berlin (Spandauer Damm 130, 1000 Berlim 19, Deutschland)
IIKlinik und Poliklinik für Neurologic Medizinische Akademie "Carl Gustav Carus" (Fetscherstrasse 74, D-O-8019 Dresden, Deutschland)
IIIInstitut für Neuroradiologie, Universitätskliniken der Universität Homburg (D-W-6650 Homburg/Saar, Deutschland)
IVDepartamento de Neuropsiquiatria, Hospital das Clínicas, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (14049-900 Ribeirão Preto SP, Brasil)

 

 


RESUMO

A hemiatrofia facial progressiva (HFP) é doença esporádica de etiologia não esclarecida, caracterizada por progressiva atrofia e deformação de um dos lados da face. Os relatos e interpretações de comprometimento do sistema nervoso central na HFP, conforme deduzido pela ocorrência de crises epilépticas em alguns pacientes e pela documentação por pneumencefalografia e CT em pequenas séries de pacientes, são contraditórios. Examinamos três pacientes do sexo feminino com HFP, uma com epilepsia, com o objetivo de obter mais informações sobre a patogênese da doença.
MÉTODOS: Realizamos exames de ressonância magnética nuclear (RMN) de rotina da cabeça e face.
RESULTADOS:Apenas a paciente com epilepsia apresentou achados patológicos no cérebro. Estes eram confinados ao hemisfério homolateral à hemiatrofia facial: dilatação monoventricular, dismorfismo meningo-cortical e alterações na substância branca.
CONCLUSÕES: As alterações morfológicas verificadas à RMN assim como os achados neurorradiológicos e histopatológicos mostrados em revisão da literatura indicaram que a hemiatrofia homolateral é achado típico para um subgrupo de pacientes com HFP, mas não indica um modelo de simples processo atrófico. Reconsideramos a possibilidade de uma meningoencefalite crônica com acometimento vascular como possível causa do ocasional envolvimento cerebral na HFP.

Palavras-chave: hemiatrofia facial progressiva, cérebro, ressonância magnética, esclerodermia localizada da face.


SUMMARY

Progressive facial hemiatrophy (PFH) is a sporadic disease of unclear etiology, characterized by shrinking and deformation of one side of the face. Reports and interpretations of CNS involvement in PFH, as deduced from the occurrence of seizures in some patients and documented by pneumoencephalography and CT findings in small series of patients, are contradictory. We examined three female patients with PFH, one with partial epilepsy, with the view to gaining further insight into the pathogenesis of the disease.
METHODS: Routine MR examinations of the head and face were performed.
RESULTS:Only the patient with epilepsy showed pathological findings, confined to the cerebral hemisphere homolateral to the facial hemiatrophy, and including monoventricular enlargement, meningo-cortical dysmorphia and white matter changes. CONCLUSIONS: The MR morphology, and corresponding neuroradiological and histopathological findings disclosed by a review of the literature, indicate that homolateral hemiatrophy is a typical finding for a subgroup of PFH patients, but do not support the model of a simple or nutritive atrophic process. We reconsider chronic localized meningo-encephalitis with vascular involvement as possible underlying cause of the occasional brain involvement in PFH.

Key words:progressive facial hemiatrophy, brain, magnetic resonance, localized sclerodermia.


 

 

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Aceite: 27-juIho-1994.

 

 

Dr. K. Terstegge - Universitätsklinikum Rudolf Virchow - Augustenburger Platz 1-13 353 Berlin - Deutschland. Fax (49) (4505) 2078.