|Year : 2015 | Volume
| Issue : 2 | Page : 150-152
Dyke-Davidoff-Masson syndrome: A case report of cerebral hemiatrophy with crossed cerebellar diaschisis
Ramakrishna Narra, Susheel Kumar Kamaraju, Bheemeswarao Pasupaleti, Naga Narasimha Raju Jukuri
Department of Radiology, Katuri Medical College, Guntur, Andhra Pradesh, India
|Date of Web Publication||21-Nov-2015|
Department of Radiology, Katuri Medical College, Guntur, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Refractory epilepsy is common in practice of neurology. When associated with hemiparesis and hemiatrophy a possibility of Dyke-Davidoff-Masson syndrome (DDMS) could be considered and imaging helps in confirmation. We present a case report of a 10-year-old female who presented with seizures, right-sided hemiparesis, hemiatrophy of the right side of the body, and mental retardation. Magnetic resonance imaging (MRI) brain revealed characteristic features diagnostic of congenital type of cerebral hemiatrophy or DDMS. Few case reports are described in literature highlighting crossed cerebellar diaschisis an imaging finding associated with the syndrome on MRI. The associated MRI findings of the syndrome are described.
Keywords: Cerebellar diaschisis, cerebral hemiatrophy, Dyke-Davidoff-Masson syndrome, magnetic resonance imaging
|How to cite this article:|
Narra R, Kamaraju SK, Pasupaleti B, Jukuri NR. Dyke-Davidoff-Masson syndrome: A case report of cerebral hemiatrophy with crossed cerebellar diaschisis. Afr J Med Health Sci 2015;14:150-2
|How to cite this URL:|
Narra R, Kamaraju SK, Pasupaleti B, Jukuri NR. Dyke-Davidoff-Masson syndrome: A case report of cerebral hemiatrophy with crossed cerebellar diaschisis. Afr J Med Health Sci [serial online] 2015 [cited 2021 Jan 17];14:150-2. Available from: http://www.ajmhs.org/text.asp?2015/14/2/150/170195
| Introduction|| |
Refractory epilepsy is common in neurologic practice. Dyke-Davidoff-Masson syndrome (DDMS) is one of the syndromes associated with refractory epilepsy.  Originally described by Dyke-Davidoff-Masson in 1933 in a case series of nine patients with epilepsy, hemiparesis, and mental retardation radiographical features of the skull were asymmetry, ipsilateral osseous hypertrophy of the calvarium, and hyperpneumatization of the sinuses. Few case reports are described in literature highlighting crossed cerebellar diaschisis an imaging finding associated with the syndrome on magnetic resonance imaging (MRI). The associated MRI findings of the syndrome are described.
| Case Report|| |
A 10-year-old female presented with seizures, right-sided hemiparesis, hemiatrophy of the right side of the body, and mental retardation. On examination, she had facial asymmetry, right-sided hypertonia, brisk deep tendon reflexes, an extensor plantar reflex, and hemiatrophy on the right side. Her routine hematological and biochemical investigations were within normal limits (Hb-11.4 g/dl, red blood cell-4.5 mil/μl, white blood cell-9800 cells/μl, neutrophils-66%, eosinophils-6%, lymphocytes-25%, basophils-2%, monocytes-2%, platelets-3 lakhs/μl, random blood sugar-90 mg/dl, blood urea-36 mg%, serum creatine-0.8 mg/dl, sodium-140 meq/l, potassium-4 meq/l, chloride-70 meq/l, bicarbonate-24 meq/l, serum calcium-9 mg/dl, urine analysis: Color-pale yellow, pH-5, specific gravity-1.020, pus cells-2-3, epithelial cells-1-2, casts-nil, cerebrospinal fluid analysis: Glucose, proteins within normal limits.).
Electroencephalogram showed continuous irregular theta slow activity and low amplitude waves on the left side.
Magnetic resonance imaging of brain done fluid-attenuated inversion recovery, T2W, T1W sequences revealed left cerebral hemiatrophy with areas of gliosis secondary to old vascular insult in fronto-temporo-parieto-occipital region on left side (middle cerebral artery [MCA] distribution), thickening of bony calvarium of left hemicranium with hyperpneumatized ipsilateral frontal sinus and ipsilateral cerebral peduncle atrophy, contralateral middle cerebellar peduncle, and cerebellar atrophy [Figure 1], [Figure 2] and [Figure 3]. MR angiogram done showed complete occlusion of left MCA M1 segment [Figure 4]. All these features are suggestive of DDMS.
|Figure 1: Axial fluid-attenuated inversion recovery magnetic resonance imaging (MRI) shows diffuse atrophy of left cerebral hemisphere with gliosis and chronic infarct in left middle cerebral artery distribution (black arrow). Note dilation of left lateral ventricle (red arrow) (1.5T GE HDE MRI scanner)|
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|Figure 2: Axial T2W magnetic resonance imaging at the level of temporal horn showing chronic infarct in left middle cerebral artery distribution with prominent sulci and temporal horn of lateral ventricle (black arrow). Also note pneumosinus dilatans (red arrow) of frontal sinus and atrophy of left cerebral peduncle (blue arrow)|
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|Figure 3: Axial T1W magnetic resonance imaging at cerebellar level showing prominent right cerebellar folia (atrophy) (black arrow) with reduced width of right middle cerebellar peduncle (red arrow)|
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|Figure 4: Time-of-flight magnetic resonance angiogram showing occlusion of M1 segment of left middle cerebral artery (MCA) and the paucity of left MCA cortical branches (red arrow)|
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| Discussion|| |
Clinically, patients present with seizures, facial asymmetry, contralateral hemiparesis, and mental retardation. ,, 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 MCA territory. In our case, Pattern III was present. The atrophied cerebral hemisphere will have prominent sulcal spaces if the vascular insult occurs after birth or after end of sulcation. However, if ischemia occurs during embryogenesis when the formation of gyri and sulci is deficient, prominent sulcal spaces will be absent. 
Children with medically refractive epilepsy may be candidates for hemispherectomy, which is helpful in eradicating or significantly reducing seizures in 85% of patients. 
Magnetic resonance imaging is a valuable imaging technique in the evaluation of cerebral hemiatrophy as it has the ability to bring to light changes in the cerebral hemispheres as well as highlighting bony structural changes.
Crossed cerebellar diaschisis is a condition in which cerebellar hypometabolism is a result of functional disconnection of the cerebellar hemisphere from the cerebral cortex. Interruption of the cerebellopontine-cerebellar pathway is thought to be the most likely mechanism of this remote transneuronal metabolic depression.  This phenomenon can be diagnosed by positron emission tomography, as there is hypometabolism in the affected cerebellar hemisphere and on MRI they present as cerebellar atrophy.
Differential diagnosis is Sturge-Weber syndrome, Rasmussen encephalitis which have unique clinical and radiological features.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]