Split Hand / Foot Malformation Syndrome with Cerebral Degeneration.

Received; 08.10.2014; Revised: 20.05.2015; Accepted: 02.06.2015 A 36 years old male presented in medicine outpatient department of our hospital with episode of generalized tonic-clonic seizures. History and clinical examination revealed that he was born with deformed hands and feet in the form of absent middle finger in both the upper limbs and syndactyly of great toe and 2nd toe with absence of 3rd toe in the left lower limb. Right lower limb did not revealed any malformation. There was no facial dysmorphism. Rest of the physical and systemic examination was clinically unremarkable. Malformations o f both hands and fee t gave the appearance of lobster claw hands and feet (Figure 1). When searched about the family history, similar claw hand-feet malformations were present in his father and grandfather also showing continuous three generations involvement most probably as a result of autosomal mode of inheritance.