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Table 1 Clinical types of alpha-mannosidosis

From: Alpha-mannosidosis

Type II: Less severe, late onset form involving hearing loss, coarse face, mental retardation, and hepatosplenomegaly.

Type I: Severe infantile form which is fatal at <3–8 years of age.

 

Type 1: Mild form clinically, recognized after 10 years of age, without skeletal abnormalities and very slow progression.

Type 2: Moderate form, clinically recognized before 10 years of age, with skeletal abnormalities, and slow progression with development of ataxia at age 20–30.

Type 3: Severe form, immediately recognized, with skeletal abnormalities, and obvious progression, leading to an early death from primary CNS involvement or myopathy.

  1. Staging of disease severity of mannosidosis according to Desnick et al., 1976 [9] (Upper panel) and according to Chester et al., 1982 and Malm & Nilssen, 2006, [14, 16] (Lower panel).
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