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Key Words: National Drug Policy; World Health Organization; Bangladesh.DOI: http://dx.doi.org/10.3329/jbcps.v29i3.9442 JBCPS 2011; 29(3): 177-179

A 25 year old, short stature man of non-consanguineous parents, attended with acute respiratory tract infectionalong with progressive difficulties in walking, hearing and vision. He had the complaints of growth retardation, poorfeeding, listless attitude and delayed milestones of development since one year of age. At presentation, he was noncooperative, IQ below 50; height and weight were below 5th percentile. He had progeria with enophthalmos, cataract,corneal opacity, miotic pupils, tremor, ataxia, in-coordination of movement, diminished tendon reflexes, unsteadygait, bilateral sensory neural deafness and hepatomegaly. Lateral skull X-ray showed cortical calcification. MRI ofbrain revealed bilateral dentate nucleus and basal ganglia calcification, generalized cerebral and cerebellaratrophy and ventricular dilatation. Typical clinical and imaging findings clinched the diagnosis of classic CockayneSyndrome; which is a rare, autosomal recessive, DNA repair deficient, multisystem disorder. It has no cure and theprognosis is poor.Keywords: Classic Cockayne syndrome; Dwarfism; Basal ganglia calcification; Cerebral atrophyDOI: 10.3329/jom.v11i2.5470J MEDICINE 2010; 11 : 186-188