Hereditary ataxia (G11)

• ICD-10 Index

  • Diseases of the nervous system (G00–G99)

    • Systemic atrophies primarily affecting the central nervous system (G10-G14)

      • Hereditary ataxia (G11)

        • G11 - Hereditary ataxia NON-BILLABLE CODE
        • G11.0 - Congenital nonprogressive ataxia BILLABLE CODE
        • G11.1 - Early-onset cerebellar ataxia NON-BILLABLE CODE
        • G11.10 - Early-onset cerebellar ataxia, unspecified BILLABLE CODE
        • G11.11 - Friedreich ataxia BILLABLE CODE
        • G11.19 - Other early-onset cerebellar ataxia BILLABLE CODE
        • G11.2 - Late-onset cerebellar ataxia BILLABLE CODE
        • G11.3 - Cerebellar ataxia with defective DNA repair BILLABLE CODE
        • G11.4 - Hereditary spastic paraplegia BILLABLE CODE
        • G11.8 - Other hereditary ataxias BILLABLE CODE
        • G11.9 - Hereditary ataxia, unspecified BILLABLE CODE

Clinical Information for Hereditary ataxia (G11)

Friedreich Ataxia - 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)

Narcolepsy - A condition characterized by recurrent episodes of daytime somnolence and lapses in consciousness (microsomnias) that may be associated with automatic behaviors and AMNESIA. CATAPLEXY; SLEEP PARALYSIS, and hypnagogic HALLUCINATIONS frequently accompany narcolepsy. The pathophysiology of this disorder includes sleep-onset rapid eye movement (REM) sleep, which normally follows stage III or IV sleep. (From Neurology 1998 Feb;50(2 Suppl 1):S2-S7)

Cerebellar Ataxia - Incoordination of voluntary movements that occur as a manifestation of CEREBELLAR DISEASES. Characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention TREMOR), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and GAIT ATAXIA. (From Adams et al., Principles of Neurology, 6th ed, p90)

Myoclonic Cerebellar Dyssynergia - A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)

Instructional Notations

Type 2 Excludes Type 2 Excludes
A type 2 excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.

• cerebral palsy G80
• hereditary and idiopathic neuropathy G60
• metabolic disorders E70 E88