Major clinical signs | Other common clinical signs |
---|---|
Diabetes mellitusa [40]  Average age of diagnosis 6 years | Urinary tract problems and renal dysfunction [13, 38]   Neurogenic bladder   Bladder incontinence   Urinary tract infection Average age of diagnosis 12–20 years |
Optic atrophya [40]  Average age of diagnosis 10–11 years | Psychiatric symptoms [52]   Depression   Psychosis   Panic attacks   Sleep abnormalities   Mood swings |
 Average age of diagnosis 14–15 years | Neurological manifestation/autonomic dysfunction [12, 38]   Central apnea   Dysphagia   Areflexia   Epilepsy   Decreased ability to taste and detect odors   Headache   Orthostatic hypotension   Hypothermia, hyperpyrexia   Gastroparesis, constipation |
Sensorineural hearing loss [13, 38]  Average age of diagnosis 16 years |  Hypogonadism  Deficient growth hormone secretion  Deficient corticotropin secretion  Delayed menarche in female |
Neurological manifestation [38]: ataxia  Average age of diagnosis 15 years |  |
Dominant disease with or without diabetes mellitus and recessive Wolfram like disease without diabetes mellitus | |
Severe gastrointestinal ulcers, bleeding and defective platelet aggregationb [12] |