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|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 5 | Page : 774-776
Dyke-Davidoff-Masson syndrome: A classical case with additional skeletal findings
Sanjay Pandey, Shruti Jain
Department of Neurology, GB Pant Hospital, New Delhi, India
|Date of Web Publication||6-Oct-2015|
Department of Neurology, GB Pant Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pandey S, Jain S. Dyke-Davidoff-Masson syndrome: A classical case with additional skeletal findings. Neurol India 2015;63:774-6
Dyke-Davidoff-Masson syndrome More Details (DDMS) is a congenital, neonatal or early infantile condition characterized clinically by variable degrees of facial asymmetry, contralateral hemiplegia or hemiparesis, mental retardation and seizures. This condition results from atrophy or hypoplasia of one the cerebral hemispheres (hemiatrophy), which is usually due to an insult to the developing brain in the fetal or early childhood period. The typical radiological features are cerebral hemiatrophy with ipsilateral compensatory hypertrophy of the skull and sinuses.
A 19-year-old boy was referred for management of recurrent seizures and right hemiparesis. He was born full-term, after a normal delivery in the hospital. At 2 months of age, he developed paralysis of right upper and lower limbs. He subsequently had delayed sitting, standing, and speech milestones and fared poorly in scholastic activities. At the age of 12 years, he developed focal motor seizures involving the right upper and lower limbs without generalization at a frequency of 1/year despite being on a high dose of carbamazepine (900 mg/d). On examination, he had microcephaly, relative atrophy of the right half of the body, high arched palate, right-sided torticollis and thoracic scoliosis with convexity toward left [[Figure 1] and Video 1]. Neurological examination revealed a mild right upper motor neuron facial weakness, spastic weakness of right upper and lower limbs with a spastic deformity of the right upper limb and a spastic right hemiplegic gait. His magnetic resonance imaging (MRI) showed diffuse atrophy of the left cerebral hemisphere and left half of the cerebral peduncle, pons and medulla with thickening of the overlying calvarium and hyper-aeration of the frontal sinuses [Figure 2]. There was also a large ill-defined area showing encephalomalacic changes involving the left perirolandic, parietal and ganglio-capsulo-thalamic regions with loss of adjacent white matter and dilatation of the ipsilateral lateral ventricle with a midline shift toward the left side.
|Figure 1: Right hemiatrophy, spastic posturing of right upper limb, right sided torticollis (a) and scoliosis with convexity toward the left ( b)|
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|Figure 2: T1-weighted and T2-weighted axial sections of magnetic resonance imaging of the brain showing atrophy of left temporal lobe and left half of the midbrain (a and e), hyperpneumatization of the left frontal sinus (b and f), gliosis of the left frontoparietal lobe with ipsilateral dilatation of the lateral ventricle and thickening of the overlying skull bone (c and g), large ill-defined area showing encephalomalacic changes involving the left perirolandic area and the parietal and ganglio-capsulo-thalamic region with loss of adjacent white matter and dilatation of ipsilateral lateral ventricle (d and h)|
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Our patient, therefore, had non-progressive infantile hemiplegia, mental retardation and well-controlled late-onset focal motor seizures and MRI findings characteristic of the rare syndrome known as DDMS with additional musculoskeletal changes not described in the previously published literature.
Dyke-Davidoff-Masson syndrome is a syndrome characterized by cerebral hemiatrophy and a contralateral hemiplegia, hemiatrophy, epilepsy and mental retardation with characteristic skull changes. In their original work, Dyke, Davidoff and Masson described the radiological skull findings in 9 patients with infantile hemiplegia. They observed cranial vault thickening and hyper-aeration of frontal, mastoid and ethmoid air cells ipsilateral to the side of the lesion. They postulated that these changes were a secondary phenomenon that compensated for the loss of cerebral volume secondary to congenital or acquired brain insults. Subsequent studies showed additional findings of elevation of the orbital roof, sphenoidal wing and petrous ridge ipsilateral to the lesion. In addition to diffuse cerebral atrophy, case reports have shown an ipsilateral brainstem atrophy, an ipsilateral or contralateral cerebellar atrophy, pachygyria and/or porencephalic cysts. Shen et al., depicted three MRI patterns of cerebral hemiatrophy: MRI pattern I corresponds to diffuse cortical and subcortical atrophy; pattern II corresponds to diffuse cortical atrophy coupled with porencephalic cysts; and pattern III corresponds to previous infarction with gliosis in the middle cerebral artery (MCA) territory. Our patient showed pattern III on MRI.
This syndrome occurs secondary to a cerebral insult early in life, which may occur in the intrauterine, perinatal or infantile period. The most common cause is a vascular occlusion or malformation involving the MCA, which manifests as hemiplegia at birth or during early infancy. Other causes may include infections, trauma, prolonged febrile seizures, or ischemic/hemorrhagic insults later in life.
The differential diagnosis for the triad of hemiplegia, hemiatrophy and epilepsy include Rasmussen encephalitis, Sturge– Weber syndrome More Details and hemimegalencephaly. Rasmussen encephalitis is characterized by its progressive course of hemiplegia with worsening documented clinically as well as on MRI on separate time occasions and is associated with intractable epilepsy. Sturge–Weber syndrome is characterized by the classical port wine stain and the cerebral venous malformations and tram track calcification. Hemimegalencephaly is a congenital condition characterized by the defective cellular organization and neuronal migration resulting in hamartomatous overgrowth of the brain. Patients typically present with intractable seizures in the 1st year of life and subsequently develop progressive contralateral hemiparesis. Imaging shows a dysplastic enlarged cerebral hemisphere, enlarged ipsilateral lateral ventricle and thickened cranial vault.
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[Figure 1], [Figure 2]