Dyke–Davidoff–Masson syndrome

Charan S Jilowa, Parth S Meena, Jitendra Rohilla, Mahendra Jain 
 Department of Psychiatry, JLN Medical College, Ajmer, Rajasthan, India

Correspondence Address:
Dr. Charan S Jilowa
Department of Psychiatry, JLN Medical College, Ajmer, Rajasthan
India

Full Text

Sir,

Dyke–Davidoff–Masson syndrome (DDMS) was first described by C.G. Dyke, L.M. Davidoff, and C.B. Masson in 1933 with the help of plain skull radiographic and pneumato-encephalographic changes.[1] DDMS is generally classified into the congenital and acquired types. It is characterized by cerebral hemiatrophy, contralateral hemiplegia or hemiparesis, thickening of the cranial vault, facial asymmetry, seizures, mental retardation and behavioural changes.[2] The diagnosis of DDMS is usually made by clinical examination and the typical radiologic findings.

A 25-year-old young man presented to us with a history of generalized tonic–clonic seizures, low intelligence, and some behavioural problems since the age of 10 years. His seizures were well controlled on a combination of carbamazepine and clobazam. He had a history of non-progressive weakness in the right half of his body since the age of 13 years. His mother revealed a history of delayed developmental milestones on all fronts. Additionally, there was a history of fall from height at the age of 10 years. The patient had developed loss of consciousness, headache, and vomiting after the fall that necessitated admission and treatment in the hospital. The details of the hospital admission were unfortunately not available with the patient.

On examination, patient was conscious, oriented and had upper motor neuron type of subtle right hemiparesis (Medical Research Council grade 4/5). His intelligence quotient was 64 indicating a mild mental retardation. No other neurological signs were present. All his routine biochemical investigations were within normal limits. Computed tomography (CT) scan of the head demonstrated prominent gyri of the left cerebral hemisphere with ipsilateral ventricular prominence indicating hemi-atrophy of the left cerebral hemisphere. The electroencephalography (32 channels) recordings were normal. On the basis of these findings, a diagnosis of DDMS was finally made [Figure 1].{Figure 1}

The behavioural problems like disturbed sleep, irritability, anger outburst, increased psychomotor activity, suspiciousness and irrelevant talks, were found in our patient similar to the observations of previous authors.[3]

Mental retardation in our patient was probably secondary to untreated generalized tonic–clonic seizures for a decade and a half. This underscores the need for an early and accurate detection of this condition and prompt initiation of the antiepileptic medications. It is important to realize that certain condition may mimic DDMS like basal ganglia germinoma, Sturge–Weber syndrome, Fishman syndrome, and Rasmussen encephalitis.[4] The treatment of DDMS is symptomatic and includes the management of convulsion, hemiplegia, hemi-paresis, and learning difficulties.

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