Schizencephaly. .

45 melanin, but also slow nail growth rate resulting in larger accumulation of the pigment. The presence of HHN neither correlates with degree of renal impairment nor with blood urea nitrogen or creatinine levels. HHN can be an important clue in making the diagnosis of renal disease. 3 0 year old male presented with history of generalised tonic clonic seizures since the age of 16 poorly controlled with antiepileptic drugs and involuntary eye movements observed by his parents since childhood. He is the third child born at full term to a non-consanguineous couple with no family history of genetic disease and mental retardation. No history of delayed milestones. There was no history of fever, radiation exposure or any significant drug intake or any major illness during pregnancy period of his mother. On examination patient was conscious and cooperative. Vitals were normal. Neurological examination revealed normal higher mental functions, Mini mental score of 29. Visual acuity Rt eye was 6/60, Lt eye was finger counting at 1 meter. Fundus examination revealed optic atrophy in both eyes (Figure 1). Horizontal nystagmus present in both eyes on looking at both directions. Ocular movements were normal, pupils were bilaterally equally reacting normally. R e s t o f t h e C N S e x a m i n a t i o n wa s normal. Respiratory, cardiovascular a n d a b d o m i n a l e x a m i n a t i o n w a s unremarkable. Male external genitilia and secondary sexual characteristics were normal. Routine investigations like haemogram, blood sugar, LFT, RFT, Electrolytes, ECG were normal. MRI brain with orbits showed absence of septum pellucidum, CSF filled cleft lined by gray matter noted in the right inferior frontal gyrus, not communicating with the ipsilateral ventricular system suggestive