Other degenerative diseases of basal ganglia (G23)
Clinical Information
Machado-Joseph Disease - A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96)
Striatonigral Degeneration - A sporadic neurodegenerative disease with onset in middle-age characterized clinically by Parkinsonian features (e.g., MUSCLE RIGIDITY; HYPOKINESIA; stooped posture) and HYPOTENSION. This condition is considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Pathologic features include a prominent loss of neurons in the zona compacta of the SUBSTANTIA NIGRA and PUTAMEN. (From Adams et al., Principles of Neurology, 6th ed, p1075-6)
Instructional Notations
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.
- multi-system degeneration of the autonomic nervous system G90.3
Diseases of the nervous system (G00–G99)
Extrapyramidal and movement disorders (G20-G26)
G23 Other degenerative diseases of basal ganglia
- G23.0 Hallervorden-Spatz disease
- G23.1 Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]
- G23.2 Striatonigral degeneration
- G23.3 Hypomyelination with atrophy of the basal ganglia and cerebellum NEW CODE
- G23.8 Other specified degenerative diseases of basal ganglia
- G23.9 Degenerative disease of basal ganglia, unspecified
Other degenerative diseases of basal ganglia (G23)