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2007 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 330-338 > Other Extrapyramidal Disease And Abnormal Movement Disorders 333.* > 2007 ICD-9-CM Diagnosis 333.0 Other degenerative diseases of the basal ganglia This code may be outdated. View the most recent version of ICD-9-CM 333.0 A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7) A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085) A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord, which differentiates this condition from other forms of idiopathic orthostatic hypotension (HYPOTENSION, ORTHOSTATIC). This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with orthostasis and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536) A rare autosomal recessive degenerative disorder which usually presents in late childhood or adolescence. Clinical manifestations include progressive MUSCLE SPASTICITY; hyperreflexia; MUSCLE RIGIDITY; DYSTONIA; DYSARTHRIA; and intellectual deterioration which progresses to severe dementia over several years. Pathologic examination reveals neuronal atrophy in the globus pallidus and iron deposition in blood vessels and perivascular spaces. (From Adams et al., Principles of Neurology, 6th ed, p972; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp972-929) A rare neuroaxonal dystrophy, histologically characterized by axonal spheroids, iron deposition, lewy body (LB)-like intraneuronal inclusions and neurofibrillary tangles. 333.0 is a specific code that can be used to specify a diagnosis 333.0 contains 24 index entries View the ICD-9-CM Volume 1 333.* hierarchy 333.0 also known as: Atrophy or degeneration: olivopontocerebellar [Déjérine-Thomas syndrome] pigmentary pallidal [Hallervorden-Spatz disease] striatonigral Parkinsonian syndrome associated with: idiopathic orthostatic hypotension symptomatic orthostatic hypotension Progressive supranuclear ophthalmoplegia Shy-Drager syndrome Index entries containing 333.0: Atrophy, atrophic Déjérine-Thomas 333.0 olivopontocerebellar 333.0 Degeneration, degenerative basal nuclei or ganglia NEC 333.0 olivopontocerebellar (familial) (hereditary) 333.0 pallidal, pigmentary (progressive) 333.0 pigmentary (diffuse) (general) pallidal (progressive) 333.0 strionigral 333.0 Déjérine-Thomas atrophy or syndrome 333.0 Disease, diseased - see also Syndrome basal ganglia 333.90 degenerative NEC 333.0 Hallervorden-Spatz 333.0 Hallervorden-Spatz disease or syndrome 333.0 Hypotension (arterial) (constitutional) 458.9 orthostatic (chronic) 458.0 dysautonomic-dyskinetic syndrome 333.0 Insufficiency, insufficient idiopathic autonomic 333.0 Ophthalmoplegia (see also Strabismus) 378.9 supranuclear, progressive 333.0 Palsy (see also Paralysis) 344.9 progressive supranuclear 333.0 supranuclear NEC 356.8 progressive 333.0 Parkinsonism (arteriosclerotic) (idiopathic) (primary) 332.0 associated with orthostatic hypotension (idiopathic) (symptomatic) 333.0 Shy-Drager syndrome (orthostatic hypotension with multisystem degeneration) 333.0 Steele-Richardson (-Olszewski) Syndrome 333.0 Syndrome - see also Disease Déjérine-Thomas 333.0 Hallervorden-Spatz 333.0 orthostatic hypotensive-dysautonomic-dyskinetic 333.0 progressive pallidal degeneration 333.0 Shy-Drager (orthostatic hypotension with multisystem degeneration) 333.0