Alper DİLLİ, Hacer FIRAT, Pınar ÇELTİKCİ, Baki HEKİMOĞLU, Ümit Yaşar AYAZ

MRI Findings in an Adult Patient With Dyke-Davidoff-Masson Syndrome

Dyke-Davidoff-Masson Sendromu (DDMS) serebral hemiatrofi, konvülziyon, kontralateral hemipleji/hemiparezi vemental retardasyon ile karakterize ve nadir görülen bir hastalıktır. DDMS'nin etiyolojisinin konjenital veya edinselolmasına göre değişken olduğu öne sürülmüştür. Otuz yedi yaşında erkek hastaya tekrarlayan, tedaviye dirençliepileptik nöbetler ve görme problemi şikayetleri ile kraniyal manyetik rezonans görüntüleme (MRG) yapıldı. MRG'de solda diffüz serebral atrofi (hemiatrofi) saptandı.

Dyke-Davidoff-Masson Sendromlu Erişkin Hastanın MRG Bulguları

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare disease and characterized by cerebral hemiatrophy, convulsions,contralateral hemiplegia/hemiparesia, and mental retardation. Etiology of DDMS has been postulated to be variablein patients with being congenital or acquired. Cranial magnetic resonance imaging (MRI) was performed on a Thirtyseven-years-old male patient with complaints of recurrent, treatment-resistant epileptic seizures and visual problems.).MRI showed diffuse cerebral atrophy (hemiatrophy) on the left side.

PDF

___

1. Aguiar PH, Liu CW, Leitão H, Issa F, Lepski G, Figueiredo EG, Gomes-Pinto F, Marino Júnior R. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arq Neuropsiquiatr 1998; 56(4):803-7.
2. Tasdemir HA, Incesu L, Yazicioglu AK, Belet U, Gungor L. Dyke- Davidoff-Masson syndrome. Clin Imaging 2002; 26(1):13-7.
3. Sener RN, Jinkins JR. MR of craniocerebral hemiatrophy. Clin Imaging 1992; 16(2):93-7.
4. Sharma S, Goyal D, Negi A, Sood RG, Jhobta A, Surya M. Dyke-davidoff-masson syndrome. Indian J Radiol Imaging 2006; 16:165-6.
5. Parker CE, Harris N, Mavalwala J. Dyke-Davidoff-Masson Syndrome. Five case studies and deductions from dermatoglyphics. Clin. Pediatr 1972; 11(5):288-92.
6. Shen WC, Chen CC, Lee SK, Ho YJ, Lee KR. Magnetic resonance imaging of cerebral hemiatrophy . J Formos Med Assoc 1993; 92(11):9951000.
7. Atalar MH, Icagasioglu D, Tas F. Cerebral hemiatrophy (Dyke- Davidoff-Masson syndrome) in childhood: clinicoradiological analysis of 19 cases. Pediatr Int 2007; 49(1):70-5.
8. Unal, O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clin Imaging 2004; 28(3):163-5.
9. Chiron C, Jambaque I, Nabbout R, Lounes R, Syrota A, Dulac O. The right brain hemisphere is dominant in human infants. Brain 1997; 120(6):1057-65.
10. Koshy B, Surendrababu NR. Image in medicine. Dyke-Davidoff- Masson syndrome. Ann Acad Med Singapore 2010; 39(6): 501-2