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  2. ICD-10-CM Codes List
  3. G00–G99
  4. G10-G14
  5. Spinal muscular atrophy and related syndromes (G12)

Spinal muscular atrophy and related syndromes (G12)

The ICD-10 code section G12 is used to classify spinal muscular atrophy (SMA) and related motor neuron diseases. These codes cover a range of hereditary and progressive neuromuscular disorders affecting motor neurons, leading to muscle weakness and wasting.

This section includes specific codes for different types of SMA and motor neuron diseases, such as G12.0 for infantile spinal muscular atrophy type I (Werdnig-Hoffman disease), and G12.1 covering other inherited forms like Kugelberg-Welander disease and distal hereditary motor neuropathies. The code for amyotrophic lateral sclerosis (ALS), an often-fatal degenerative disease, is G12.21. Related disorders such as progressive bulbar palsy (G12.22), primary lateral sclerosis (G12.23), and familial motor neuron disease (G12.24) are also included. These codes help healthcare professionals assign accurate diagnoses for conditions like anterior horn cell disease, progressive muscular atrophy, and other motor neuron syndromes. Unspecified or less common forms fall under codes G12.8 and G12.9. Understanding the synonyms, like Werdnig-Hoffmann disease or Kugelberg-Welander disease, assists in precise coding for spinal muscular atrophy and related syndromes.

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.

Amyotrophic Lateral Sclerosis

A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)

Motor Neuron Disease

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)

Multiple System Atrophy

A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)

Pseudobulbar Palsy

A syndrome characterized by DYSARTHRIA, dysphagia, dysphonia, impairment of voluntary movements of tongue and facial muscles, and emotional lability. This condition is caused by diseases that affect the motor fibers that travel from the cerebral cortex to the lower BRAIN STEM (i.e., corticobulbar tracts); including MULTIPLE SCLEROSIS; MOTOR NEURON DISEASE; and CEREBROVASCULAR DISORDERS. (From Adams et al., Principles of Neurology, 6th ed, p489)