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2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Nervous System And Sense Organs 320-389 > Hereditary And Degenerative Diseases Of The Central Nervous System 330-337 > Spinocerebellar Disease 334.* > 2008 ICD-9-CM Diagnosis 334.0 Friedreich's ataxia  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 Therefore, this code may be out of date.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 334.0 can be accessed here. An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75) 334.0 is a specific code that can be used to specify a diagnosis 334.0 contains 12 index entries View the ICD-9-CM Volume 1 334.* hierarchy Index entries containing 334.0: Ataxia, ataxy, ataxic 781.3 family, familial 334.2 spinal (Friedreich's) 334.0 Friedreich's (heredofamilial) (spinal) 334.0 hereditary NEC 334.2 spinal 334.0 spinal hereditary 334.0 Disease, diseased - see also Syndrome Friedreich's combined systemic or ataxia 334.0 Friedreich's ataxia 334.0 combined systemic disease 334.0 disease 333.2 combined systemic 334.0 sclerosis (spinal cord) 334.0 Sclerosis, sclerotic Friedreich's (spinal cord) 334.0 hereditary spinal 334.0 spinal (cord) (general) (progressive) (transverse) 336.8 hereditary (Friedreich's) (mixed form) 334.0