2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Other And Unspecified Disorders Of Metabolism 277.* > 2008 ICD-9-CM Diagnosis 277.87 Disorders of mitochondrial metabolism  On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date. The 2009 version of ICD-9-CM 277.87 can be accessed here. A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984) 277.87 is a specific code that can be used to specify a diagnosis 277.87 contains 18 index entries View the ICD-9-CM Volume 1 277.* hierarchy ICD9Data's sister site ProcedureCodes.com launches with the 2008/09 CPT® codes - read more on the ICD9Data.com blog. 277.87 also known as: Kearns-Sayre syndrome Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS syndrome) Mitochondrial Neurogastrointestinal Encephalopathy syndrome (MNGIE) Myoclonus with Epilepsy and with Ragged Red Fibers (MERRF syndrome) Neuropathy, Ataxia and Retinitis Pigmentosa (NARP syndrome) Use additional code for associated conditions 277.87 excludes: disorders of pyruvate metabolism (271.8) Leber's optic atrophy (377.16) Leigh's subacute necrotizing encephalopathy (330.8) Reye's syndrome (331.81) Index entries containing 277.87: Disorder - see also Disease metabolism NEC 277.9 mitochondrial 277.87 mitochondrial metabolism 277.87 Fukuhara syndrome 277.87 Kearns-Sayre syndrome 277.87 MELAS syndrome (mitochondrial encephalopathy, lactic acidosis and stroke-like episodes ) 277.87 MERRF syndrome (myoclonus with epilepsy and with ragged red fibers) 277.87 Mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS syndrome) 277.87 Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) 277.87 Myoclonus (familial essential) (multifocal) (simplex) 333.2 with epilepsy and with ragged red fibers (MERRF syndrome) 277.87 NARP ( ataxia and retinitis pigmentosa syndrome) 277.87 Neuropathy, neuropathic (see also Disorder, nerve) 355.9 ataxia and retinitis pigmentosa (NARP syndrome) 277.87 Syndrome - see also Disease Fukuhara 277.87 Kearns-Sayre 277.87 MELAS (mitochondrial encephalopathy, lactic acidosis and stroke-like episodes ) 277.87 MERRF (myoclonus with epilepsy and with ragged red fibers) 277.87 mitochondrial neurogastrointestinal encephalopathy (MNGIE) 277.87 MNGIE (mitochondrial neurogastrointestinal encephalopathy) 277.87 NARP (neuropathy, ataxia and retinitis pigmentosa) 277.87