Regarding ANCIPS 2020

1. Dyke Davidoff Masson Syndrome,a rare
entity- Case report
Authors: Dr.Shikha Singh, Prof. Sudhir Kumar, Dr. Ajay Kumar
Institute Of Mental Health and Hospital, Agra
INTRODUCTION:Dyke Davidoff Masson Syndrome is a condition characterised
by hemi cerebral atrophy/ hypoplasia secondary to brain insult in fetal or early
childhood and accompanied by ipsilateral compensatory osseous hypertrophy
and contralateral hemiparesis.
AIM:Management of a case of Dyke Davidoff Masson Syndrome with behavioural
problems.
CASE REPORT: A 13 yr old female belonging to lower middle socio economic status and
urban background presented to OPD with complaints of irritability, stubborness,increased
talkativeness ,anger outbursts,delayed developmental milestones ,seizure
episodes(complex partial type),low intelligence,weakness in right side of body and self
biting tendency.Antenatal reports of the patient's mother revealed that she was diagnosed
with eclampsia during last trimester of pregnancy.

2. Physical examination showed -microcephaly,facial
asymmetry, head turned to one side(rt. side),
squint in rt. eye.Neurological examination revealed
right sided hemiparesis with brisk tendon reflexes
and extensor plantar response.Other systemic
examination being normal.Vision and hearing were
normal and cranial nerves were intact.She scored
poorly on the Mini-Mental Status Examination
(15/30) .NCCT head showed -atrophy of left
cerebral hemisphere with fronto-parieto-temporal
cystic encephalomalacia and ex-vacuo dilatation of
left lateral ventricle.EEG showed- abnormal
interictal tracing.Her treatment was started on Tab
Aripiprazole (10mg), Tab Sodium valproate
(300mg) and Tab Clonazepam (1mg).She was
discharged on Tab Sodium Valproate (500mg), Tab
Aripiprazole (10mg) and Tab Clonazepam(1mg)
with an advice to continue regular physiotherapy
sessions.Patient gradually improved over 3 months
with these medicines .

3. DISCUSSION:The index patient presented with a rare syndrome- 'Dyke Davidoff Masson
Syndrome' along with behavioural problems .According to review of literature, in 1933 , Dyke,
Davidoff and Masson described 9 patients with clinical features of hemiparesis,facial asymmetry,
seizures and mental retardation noted to have pnuemoencephalographic changes on skull
radiograph.It is an uncommon clinical entity, refractory seizure remaining the usual concern in
early childhood .Common causes include congenital anomalies, perinatal hypoxia , intracranial
hemorrhage and infections.Hemispherectomy remains the treatment of choice along with
physiotherapy , speech therapy and occupational therapy besides medications.There have been
only a few reports of cases of DDMS in adulthood in the medical literature.
4. Dyke CG, Davidoff LM, Masson CB. Cerebral hemiatrophy
and homolateral
hypertrophy of the skull and sinuses. Surg Gynecol Obstet.
1933;57:588–600.
5.Dyke CG, Davidoff LM, Masson CB: Cerebral hemiatrophy
and homolateral hypertrophy of the skull and sinuses. Surg
Gynecol Obstet 1933;57:588-600.
6.Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M: Left
hemisphere and male sex dominance of cerebral
hemiatrophy (DDMS). External Resources
Pubmed/Medline (NLM)
Crossref (DOI)
References:1. Verma R, Sahu R. Dyke-Davidoff-
Masson syndrome. BMJ Case Reports. 2012.
https://doi.org/10.1136/bcr-2012-006729.
2. Piro E, Piccione M, Marrone G, Giuffre M, Corsello
G. Dyke-Davidoff-Masson
syndrome: case report of fetal unilateral
ventriculomegaly and hypoplastic
left middle cerebral artery. Ital J Pediatr. 2013;39:32.
3. Roy U, Panwar A, Mukherjee A, Biswas D. Adult
presentation of dyke