Table 1.

Clinical manifestations of WS

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
Diabetes insipidus [13, 38]  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	Endocrine disorders [12, 17]  Hypogonadism  Deficient growth hormone secretion  Deficient corticotropin secretion  Delayed menarche in female
Neurological manifestation [38]: ataxia  Average age of diagnosis 15 years
Atypical forms [6–8]	Dominant disease with or without diabetes mellitus and recessive Wolfram like disease without diabetes mellitus
Severe gastrointestinal ulcers, bleeding and defective platelet aggregationb [12]

^aRequired for diagnosis

^bAssociated with WFS 2