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  2. ICD-10-CM Codes List
  3. G00–G99
  4. G60-G65
  5. Hereditary and idiopathic neuropathy (G60)

Hereditary and idiopathic neuropathy (G60)

ICD-10 code G60 covers hereditary and idiopathic neuropathies, which are nerve disorders caused by genetic factors or unknown origins. These codes specifically categorize various inherited and unexplained peripheral neuropathies affecting motor, sensory, and autonomic nerves.

Within this section, G60.0 refers to hereditary motor and sensory neuropathy, commonly known as Charcot-Marie-Tooth disease and its many genetic subtypes. This code includes related conditions like Déjérine-Sottas disease and hereditary liability to pressure palsies, helping coders capture the diverse forms of inherited nerve damage affecting movement and sensation. G60.1 identifies Refsum's disease, a rare metabolic disorder causing neuropathy along with associated symptoms like cardiomyopathy and ophthalmoplegia.

The codes G60.2 and G60.3 pinpoint neuropathies linked to hereditary ataxia and idiopathic progressive neuropathy, respectively, useful for disorders presenting with coordination and nerve function decline of unknown cause. G60.8 includes other hereditary and idiopathic neuropathies such as hereditary sensory and autonomic neuropathies, small fiber neuropathies, and rare syndromes like giant axonal neuropathy. Finally, G60.9 is for hereditary and idiopathic neuropathy when specific details are unavailable, supporting coding in unclear cases. This classification aids healthcare professionals and coders in accurately identifying inherited or unexplained nerve disorders for proper diagnosis and management.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Giant Axonal Neuropathy

Rare autosomal recessive disorder of INTERMEDIATE FILAMENT PROTEINS. The disease is caused by mutations in the gene that codes gigaxonin protein. The mutations result in disorganization of axonal NEUROFILAMENT PROTEINS, formation of the characteristic giant axons, and progressive neuropathy. The clinical features of the disease include early-onset progressive peripheral motor and sensory neuropathies often associated with central nervous system involvement (INTELLECTUAL DISABILITY, seizures, DYSMETRIA, and CONGENITAL NYSTAGMUS).

Small Fiber Neuropathy

Disorder of the peripheral nerves that primarily impair small nerve fibers. The affected small nerve fibers include myelinated A-delta fibers (see A FIBERS) and unmyelinated C FIBERS. Because these small fibers innervate skin and help control autonomic function, their neuropathy presents with neuropathic pain, reduced thermal and pain sensitivity, and autonomic dysfunction (e.g. abnormal sweating or facial flushing). Small fiber neuropathy can be idiopathic or associated with underlying diseases (e.g., AMYLOIDOSIS; DIABETES MELLITUS; SARCOIDOSIS; or VASCULITIS).

Vocal Cord Paralysis

Congenital or acquired paralysis of one or both VOCAL CORDS. This condition is caused by defects in the CENTRAL NERVOUS SYSTEM, the VAGUS NERVE and branches of LARYNGEAL NERVES. Common symptoms are VOICE DISORDERS including HOARSENESS or APHONIA.

Waardenburg Syndrome

Rare, autosomal dominant disease with variable penetrance and several known clinical types. Characteristics may include depigmentation of the hair and skin, congenital deafness, heterochromia iridis, medial eyebrow hyperplasia, hypertrophy of the nasal root, and especially dystopia canthorum. The underlying cause may be defective development of the neural crest (neurocristopathy). Waardenburg's syndrome may be closely related to piebaldism. Klein-Waardenburg Syndrome refers to a disorder that also includes upper limb abnormalities.