RESUMO
Dyke-Davidoff-Masson syndrome ( DDMS) is a rare epilepsy syndrome which is characterized by cerebral hemiatrophy with ipsilateral compensatory skull changes and contralateral hemiplegia recurrent .Here we reported a case of which the curative effect turned out to be unsatisfied after conservative treatment , physical examination re-vealed mental retardation , facial asymmetry , mild right hemiparesis , MRI scan of the head showed left cerebral hemiatrophy , calvaria thickening , PET-CT showed less functional left cerebral hemisphere .The patient finally un-derwent multi-lobe disconnection to relieve recurrent seizure , whose seizure was well controlled in the following up of one year .
RESUMO
DDMS is a rare syndrome characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Dyke-Davidoff- Masson Syndrome (DDMS) is one among the syndromes associated with refractory epilepsy. We report a case of DDMS in an 18 year old female who presented with seizures, hemiparesis of the right side and mental retardation. Computed tomography on this patient assisted in making a diagnosis of DDMS.
RESUMO
PURPOSE: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. METHODS: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. RESULTS: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. CONCLUSION: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.
Assuntos
Humanos , Pessoa de Meia-Idade , Atrofia , Encéfalo , Cérebro , Anormalidades Congênitas , Hipertrofia , Imageamento por Ressonância Magnética , Osso Nasal , Seios Paranasais , Doenças Raras , Crânio , Cirurgia PlásticaRESUMO
PURPOSE: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. METHODS: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. RESULTS: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. CONCLUSION: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.
Assuntos
Humanos , Pessoa de Meia-Idade , Atrofia , Encéfalo , Cérebro , Anormalidades Congênitas , Hipertrofia , Imageamento por Ressonância Magnética , Osso Nasal , Seios Paranasais , Doenças Raras , Crânio , Cirurgia PlásticaRESUMO
Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. We report the case of a 26-year-old man with DDMS associated with hypopituitarism who complained of polydipsia and polyuria. After an oral glucose tolerance test, he was diagnosed with type 2 diabetes. There is no report of DDMS associated with other pituitary dysfunction or hyperglycemia. Clinicians should consider the possibility of coexisting pituitary dysfunction or type 2 diabetes in patients with DDMS, as it is obviously important for the patient's outcome.
Assuntos
Adulto , Humanos , Amidas , Atrofia , Diabetes Mellitus , Assimetria Facial , Teste de Tolerância a Glucose , Hemiplegia , Hiperglicemia , Hipertrofia , Hipopituitarismo , Deficiência Intelectual , Processo Mastoide , Seios Paranasais , Paresia , Polidipsia , Poliúria , Convulsões , SulfonasRESUMO
Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.