NARY is a -

Ans is 'c' i.e., Mitochondria! disorder Clinical Syndromes of Mitochondrial Diseases Primary Features Chronic progressive external o External ophthalmoplegia ophthalmoplegia (CPEO) o Bilateral ptosis o PEO onset before age 20 years o Pigmentary retinopathy o One of the following CSF protein greater than I g/L, cerebellar ataxia, hea block o Sideroblastic anemia of childhood o Panytopenia o Exocrine pancreatic failure o Hypotonia in the first year of life o Feeding and respiratory difficulties o Subacute relapsing encephalopathy o Cerebellar and brain-stem signs o Infantile onset o Late-childhood or adult-onset peripheral neuropathy o Ataxia o Pigmentary retinopathy o Stroke-like episodes before age 40 years o Seizures and/or dementia o Ragged-red fibers and/or lactic acidosis o Myoclonus o Seizures o Cerebellar ataxia o Myopathy o Subacute painless bilateral visual failure o Males: females-- 4:1 o Median age of onset 24 years Bilateral deafness Myopathy Dysphagia Diabetes mellitus Hypoparathyroidism Dementia Renal tubular defects Fatal form may be associated with a cardiomyopathy and/or the Toni-Fanconi-Debre syndrome Basal ganglia lucencies Maternal history of neurologic disease of Leigh syndroms Basal ganglia lucencies Abnormal electroretinogram Sensorimotor neuropathy 0 Diabetes mellitus 0 Cardiomyopathy (initially hyperophic; later dilated) 0 Bilateral deafness 0 Pigmentary retinopathy 0 Cerebellar ataxia Dementia Optic atrophy Bilateral deafness Peripheral neuropathy Spasticity Multiple lipomata Dystonia Cardiac pre-excitation syndromes